ALLERGY and IMMUNOLOGY

IMMUNODEFICIENCY 10 Warning signs of Immunodeficiency 8 ear infections or more within a year 2 sinus infections within a year 2 months antibiotics with little effect 2 pneumonias within 1 year failure gain weight; grow normally Recurrent abscessess Thrush in mouth or skin IV antibiotics for infections 2 deep-seated infections family history of primary immunodeficiency General Screening of Immunity CBC, differential, platelets IgG,IgA,IgM,IgE levels Baseline Antibody Titers Phagocytic Defect NBT test Rebuck skin window Chemotaxis Bacterial assay Complement Defect CH50 C3 C4 assay HIV Screening (ELISA) Positive- Probable AIDS Verify diagnosis by: repeat ELISA HIV test Western blot analysis CD4 T cell count Negative- Non-AIDS T cell defect CMI skin test (PPD, Candida antigen, etc.) CD4, CD8 assay ratio Lymphocyte blastogenic assay T cell enumeration ANAPHYLAXIS Criteria for rapid recognition of Anaphylaxis 1. Exposure to an allergen within 1 hour & 1 systemic sign 2. Urticaria or angioedema & 1 systemic sign Systemic signs: hypotension bronchospasm or dyspnea laryngeal/pharyngeal edema, stridor or dysphonia increased gastrointestinal tract motility Patterns Acute explosive onset within seconds to minutes of exposure to triggering event Biphasic followed by a reaction 3 to 8 hours after initial reaction (5-20% of cases) Protracted lasts 3 to 21 days from onset of acute reaction Laboratory findings Elevated plasma histamine Elevated serum tryptase - longer half-life Treatment EPINEPHRINE IS THE DRUG OF CHOICE! potent cathecholamine with both and adrenergic properties Reverses all pathophysiologic features of anaphylaxis Pedia Notes

-hypotension,peripheral vasodilation, increased vasopermeability, urticaria, angioedema -positive inotropic & chronotropic effects, bronchodilation, increase cAMP Epinephrine 1:1000 0.01 ml/kg SC/ IM (ped) or 0.3 to 0.5 ml (adult) given q 20 mins prn Px on blockers may be resistant to epinephrine so higher does may be required or glucagon given insect sting or injected drug: infiltrate 0.1 - 0.2 ml locally to retard absorption of the residual allergen tourniquet applied proximally if injection or sting is on an extremity Immediate Therapy Rapid ABCs of resuscitation Epinephrine IV (1:100,000) = 0.01 mg/kg or continuous drip 0.1-0.2 g/kg/min titrated q 0.1 g/kg/min to max of 1.5 g/kg/min Separate IV line no HCO3 infusion Continuous monitoring of CVS status and O2 Rapid HX of triggering event, current medications, HX of asthma, allergies and concomitant medical conditions Subacute H1 blocker Diphenhydramine 1-2 mg/kg PO,IM,IV Chlorpheniramine 10-20 mg IV,IM Corticosteroids Hydrocortisone 4-8 mg/kg/dose or methylprednisolone 1-2 mg/kg Iv q 6 h 2 agonist nebulization q 20 mins of continuous Secondary H2 blocker Ranitidine IV or PO 2-4 mg/kg/day q 8 h Glucagon 0.1 mg/kg IV if refractory to initial TX Observe at least 4 hours for biphasic anaphylaxis Fluids Loss of up to 50% intravascular volume may occur resulting in profound hypotension not responsive to epinephrine Antihistamines are not appropriate monotherapy for the Tx of acute anaphylaxis Corticosteroids are used to prevent the biphasic response and to control bronchospasm Bronchodilators are useful adjuncts in TX esp in those with asthma

CARDIOLOGY
Heart Rate Age Awake Mean NB-3mos 85-205 140 3mos-2yrs 100-190 130 2-10yrs 60-140 80 >10yrs 60-100 75 Prob SVT (nQRS) >220 infants, >180children Sleeping 80-160 75-160 60-90 50-90

Cardioversion/ Defibrillation: 0.5-1J/kg (VT); 2-4J/kg (VF/ Pulseless VT) ECG

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Normal Axis Newborn 0- (+)180 1- 6 m0 (+)10- (+)125 6mo- 3yr (+)10- (+)110 >3yr 0- (+)90 PR 0.12-0.20sec QRS 0.08-0.12sec ST not >1mm in limb leads; not >2mm in precordial QTc 0.44sec 3-4days; 0.45 <6mos; 0.44 children Q <0.04sec; <25% of QRS <5mm in L precordial & aVF; 8mm in LII for <3yo T (+) in I, II & V6 >48hrs abn if >7mm in LL or 10mm in precordial P <2.5mm amp; <3yo 0.03-0.09sec; >3yo 0.05-0.1sec Chamber Enlargements: RAE Steeple; Peaked P >3 mm in L2 & V1 LAE Wide, notched, biphasic P >2.5 mm in L2 & V1 RVH RVH in Newborn SL1 12mm Pure RV1 >10mm, RV1 >25 qR in V1 upright T in V1 >3day R in avR8mm RVH in Children RV1 >20, SV6 >7 qR in chest leads upright T >3yo RV110mm T wave inversion in avF R/S ratio in V1 >1 RsR in V1 RAD >3mos LVH SV1 >20, RV6 >25 Asymmetric T wave inversion inV5 & V6 SV1 + RV6 >50mm Qwave >30mm in II, III, aVF, V5-6 CVH Direct signs of RVH & LVH LVH + RAD & tall R in V1 RVH + q 2 mm in V5 & V6, tall R in V6, & inverted T in V6 Large equiphasic QRS in V2- V4, R + S >60 mm- KatzWachtel phenomenon QTc (corrected QT) - Bazetts Formula: ____QTa______ RR interval where RR interval = # of small squares between R-R x 0.04 sec First Degree AV Block There must be P waves There must be one P wave to each QRS complex P waves have morphology and axis usual for the subject QRS complex must have morphology and axis usual for the subject P-R interval is constant P-R interval is prolonged (i.e. >0.20 sec.)

Second degree AV block Mobitz type 1- Wenkebach phenomenon there must be P waves there must be QRS complexes P waves must have morphology and axis usual for the subject Progressive prolongation of P-R interval with each succeeding beat until there is a dropped beat

Second degree AV block Mobitz type 2 there must be P waves & QRS complexes P waves have morphology and axis usual for the subject QRS complex must have morphology axis usual for the subject P-R interval of conducted beats may be normal or long but fixed, then there is a dropped beat

High Grade AV Block Some P waves are followed by QRS complexes and some are not Atrio-ventricular conduction ratio is 3:1 or higher P-R interval of beats in which a QRS complex follows a P wave may be normal or long but must be constant

Third degree AV block Any form of atrial activity may be seen or there may be no atrial activity no consistent or meaningful relationship between atrial and ventricular activity. Variable PR and RP intervals. QRS may be normal in shape, duration and axis but more often are abnormal and are of constant morphology QRS rate is usually constant and lies within the range of 15-70 beats/min.

Pedia Notes

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CHEST XRAY ABNORMAL PATTERN RAE AP: >1/3 of the right RVE AP: apex upturned / rounded Lateral: obliteration of the retrosternal space; filled only normally. Displacement of LV posteriorly. Behind shadow of IVC LAE AP: increased distance between the right wall of the left atrium and left main stem bronchus (double density); 3.5cm for infants, 4.5cm for children; Prominence of left atrial appendage or so called disappearance of cardiac waistline; elevation of left main stem bronchus. Lateral: elevation of left main stem bronchus; discrete bulge in the region of the left atrium which pushes the esophagus posteriorly LVE AP: prominent left heart border and mid-left heart concavity with apex displaced posteriorly and meets IVC at the diaphragm level MYOCARIDAL INFARCTION IN CHILDREN ECG Findings New onset wide Q waves (>0.035 sec) seen within first few hours and persistent over several years ST-segment elevation (>2mm) seen within the first few hours Diphasic T waves seen within first few days (beginning sharply inverted) then normalizing over time Prolonged QT interval (>0.44 sec) with accompanying abnormal Q waves Deep wide Q waves in Leads I, avL, or V6 contrast Q waves in II, III, avF Other criteria Elevated creatinine kinase / MB although this is not specific for detection of acute MI in children Cardiac Troponin I is a more sensitive indicator of early myocardial damage in children elevated within hours of cardiac injury, persists for 4-7 days, specific for cardiac injury CARDIOVASCULAR MEDICATIONS Inotropes: agents that improve myocardial contractility and enhance stroke volume Pressors: agents that increase systemic vascular resistance and increase blood pressure Chronotropic: Increase heart rate Lusotropic: improve relaxation during diastole and decrease EDP in the ventricles ALPHA-ADRENERGIC MEDICATIONS Alpha1-adrenergic effects: Vascular smooth muscle contraction Alpha2-adrenergic effects: Vascular smooth muscle relaxation--this is a very mild effect only at low doses of an alpha-adrenergic agent like epinephrine. BETA-ADRENERGIC MEDICATIONS Beta1-adrenergic effects:Direct cardiac effects: (a) Inotropy (improved cardiac contractility) (b) Chronotropy (increased heart rate) Beta2-adrenergic effects: (a) Vasodilation (b) Bronchodilation CARDIAC MEDS VIA CONTINUOUS INFUSION EPINEPHRINE Both an alpha- and beta-adrenergic agent Indications for its use as a continuous infusion are: o low cardiac output state beta effects will improve cardiac function alpha effects may increase afterload and decrease cardiac output septic shock - useful for both inotropy and vasoconstriction Actions are dose dependent (mcg/kg/min): o 0.02-0.08 = mostly beta1 and beta2 stimulation. increased cardiac output mild vasodilation o 0.1-2.0 = mix of beta1 and alpha1 increase cardiac output increase SVR = vasoconstriction o > 2.0 = mostly alpha1 increase SVR, and may decrease CO by increasing afterload Side effects include: Anxiety, tremors,palpitations Tachycardia and tachyarrhythmias Pedia Notes

Increased myocardial oxygen requirements and potential to cause ischemia Decreased splanchnic and hepatic circulation (AST, ALT) Anti-Insulin effects: lactic acidosis, hyperglycemia NOREPINEPHRINE Employed primarily for its alpha agonist effect - increases SVR (and B.P.) without significantly increasing C.O. Used in cases of low SVR and hypotension such as profound warm shock with a normal or high C.O. state Infusion rates titrated between 0.05 to 1 mcg/kg/min o In general, norepinephrine differs from epinephrine in that at doses used in clinical practice, the vasoconstriction outweighs any increase in cardiac output. i.e. norepinephrine usually increases blood pressure and SVR, often without increasing cardiac output. Side Effects: Similar to those of Epinephrine Can compromise perfusion in extremities and may need to be combined with a vasodilator e.g. Dobutamine or Nipride More profound effect on sphlancnic circulation and myocardial oxygen consumption DOPAMINE Intermediate product in the enzymatic pathway leading to the production of norepinephrine; thus, it indirectly acts by releasing norepinephrine. Directly has alpha, beta and dopaminergic actions (dose-dependent) Indications are based on the adrenergic actions desired. Improve renal perfusion 2-5 mcg/kg/min Improve C.O. in mild to moderate Cardiogenic or Distributive Shock 510mcg/kg/min Post-resuscitation stabilization in patients with hypotension (in conjuction with fluid therapy) 10-20mcg/kg/min DOBUTAMINE Synthetic catecholamine with inotropic effect (increases stroke volume) and peripheral vasodilation (decreases afterload) Positive chronotropic effect (increases HR) Some lusotropic effect Overall, improves Cardiac Output by above beta-agonist acitivity o Major metabolite is 3-O-methyldobutamine, a potent inhibitor of alphaadrenoceptors.Therefore, vasodilation is possible secondary to this metabolite. Usual starting infusion rate is: 5 mcg/kg/min, with the dose being titrated to effect up to 20 mcg/kg/min. Used in low C.O. states and CHF e.g. myocarditis, cardiomyopathy, myocardial infarction If BP adequate, can be combined with afterload reducer (Nipride or ACE inhibitor) In combination with Epi/Norepi in profound shock states to improve Cardiac Output and provide some peripheral vasodilatation MILRINONE/AMRINONE Belong to new class of agents Bipyridines Non-receptor mediated activity based on selective inhibition of Phosphodiesterase Type III enzyme resulting in cAMP accumulation in myocardium cAMP increases force of contraction and rate and extent of relaxation of myocardium Inotropic, vasodilator and lusotropic effect AMRINONE First generation agent - limited use now Long half-life (4.4 hours) with potential for prolonged hypotension after loading dose Associated with thrombocytopenia Dosage: Load with 0.75 mg/kg with infusion rate of 5-10 mcg/kg/min Milrinone is preferred drug from this group MILRINONE Increases CO by improving contractility, decreased SVR, PVR (?), lusotropic effect; decreased preload due to vasodilatation Unique in beneficial effects on RV function Half-life is 1-2 hours Load with 50 mcg/kg over 30 mins followed by 0.3 to 0.75 mcg/kg/min No increase in myocardial O2 requirement

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VASODILATORS Classified by site of action Venodilators: reduce preload - Nitroglycerin Arteriolar dilators: reduce afterload Minoxidil and Hydralazine Combined: act on both arterial and venous beds and reduce both pre- and afterload Sodium Nitroprusside (Nipride) NITROPRUSSIDE Vasodilator that acts directly on arterial and venous vascular smooth muscle. Indicated in hypertension and low cardiac output states with increased SVR. Also used in post-operative cardiac surgery to decrease afterload on an injured heart. Action is immediate; half-life is short; titratable action. Toxicity is with cyanide, one of the metabolites of the breakdown of nipride. Severe, unexplained metabolic acidosis might suggest cyanide toxicity. Dose starts at 0.5 mcg/kg/min and titrate to 5 mcg/kg/min to desired effect. May go higher (up to 10 mcg/kg/min) for short periods of time. NITROGLYCERIN Direct vasodilator as well, but the major effect is as a venodilator with lesser effect on arterioles. Not as effective as nitroprusside in lowering blood pressure. Another potential benefit is relaxation of the coronary arteries, thus improving myocardial regional blood flow and myocardial oxygen demand. Used to improve myocardial perfusion following cardiac surgery Dose ranges from 0.5 to 8 mcg/kg/min. Typical dose is 2 mcg/kg/min for 24 to 48 hours post-operatively Methemoglobinemia is potential side effect ISOPROTERENOL Synthetic catecholamine Non-specific beta agonist with minimal alpha-adrenergic effects. Causes inotropy, chronotropy, and systemic and pulmonary vasodilatation. Indications: bradycardia, decreased cardiac output, bronchospasm (bronchodilator). No longer available in some markets Occasionally used to maintain heart rate following heart transplantation. Dose starts at 0.01 mcg/kg/min and is increased to 1.0 mcg/kg/min for desired effect. INHALED NITRIC OXIDE Selective Pulmonary vasodilator Dilates only pulmonary capillaries to alveoli participating in gas exchange Decreases intrapulmonary shunt and improves V/Q matching Rapidly inactivated by Hgb in pulm. cap. so no systemic side effects (eg hypotension) Potential for use in ARDS and Pulmonary Hypertension Currently only approved for use in neonatal Pulmonary Hypertension Expensive Special monitoring equipment required Dose: Concentration of 0.5-60 ppm in inhaled gas CARDIAC ARREST MEDICATIONS EPINEPHRINE Both an alpha- and beta-adrenergic agent During an cardiac arrest, most think it has the greatest benefit by alpha-adrenergic actions, increasing afterload and thus diastolic blood pressure, leading to improved coronary artery perfusion. Indications: o Cardiac arrest o Severe bronchospasm o Anaphylactic reactions Route of Administration o IV or IO o SQ or IM (for bronchospasm) o ET (cardiac arrest without IV or IO access) Pedia Notes

Dosage: o initial (low) dose: 0.01 mg/kg o = 0.1 cc/kg of 1:10,000 o subsequent (high) doses: 0.1 mg/kg o = (0.1 cc/kg of 1:1,000) ATROPINE Parasympathetic (not an alpha- or beta-adrenergic) agent--acts by blocking cholinergic stimulation of the muscarinic receptors of the heart. Results in an increase in the sinus rate of the heart. Little effect on systemic vascular resistance or myocardial contractility. Indications: o Bradycardia o Second or third degree heart block o Asystole o Pulseless electrical activity (electrical mechanical dissociation) o Route of Administration: IV, IO, ET, SQ, IM, nebulization Dosage: o 10 to 20 mcg/kg o minimum dose is 0.1 mg--smaller doses may cause reflex bradycardia (central stimulatory effect on the medullary vagal nuclei) o maximum (adult) dose is 2 mg SODIUM BICARBONATE Use during CPR remains a controversial issue due to lack of evidence showing benefit from receiving bicarbonate. Elevates blood pH by binding with hydrogen to form water and CO2 HCO-3 + H+ => H2CO3 => H2O + CO2 Must have adequate ventilation to remove CO2 or respiratory acidosis will worsen Adverse effects of acidosis: o Cardiac Decrease contractility Lower threshold for ventricular fibrillation Decrease responsiveness to catecholamines o Vascular Decrease systemic vascular resistance Decrease systemic vascular responsiveness to catecholamines Increase pulmonary vascular resistance Indications: o Pre-existing acidosis o Prolonged CPR (after 10 minutes) o Pulmonary hypertensive crisis o Hyperkalemia Route of administration: IV, IO o Dosage: 1-2 meq/kg/dose (1 meq/cc or 0.5 meq/cc) CALCIUM Current recommendations for the use of calcium during CPR are restricted to a few specific situations. Intracellular calcium plays an important role in the process of cell death, but no studies have shown that transient hypercalcemia worsens outcome after cardiac arrest. Adverse Effects of Hypocalcemia o Decreased myocardial contractility o Decreased systemic vascular resistance o Decreased catecholamine release o Decreased cardiovascular response to catecholamines Indications: o Hypocalcemia Ionized hypocalcemia may result from severe alkalosis or after large transfusions of citrated blood products. o Hyperkalemia o Hypermagnesemia o Calcium channel blocker overdose Route of administration: o IV, IO only o Calcium chloride--central venous line o Calcium gluconate--peripheral venous line Dosage: o Calcium chloride = 10-20 mg/kg o Calcium gluconate = 100-200 mg/kg

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LIDOCAINE Class 1B antiarrhythmic Decreases automaticity threshold and ventricular fibrillation threshold. Effective in terminating PVCs. Rarely used in pediatric arrests as ventricular tachycardia and ventricular fibrillation are not commonplace. Indications: o Ventricular Tachycardia o Ventricular Fibrillation o Frequent PVCs Route of Administration: IV, IO, ET o Dosage: 1 mg/kg/dose (may need up to 2.5 mg/kg ET) ENDOTRACHEAL MEDICATIONS (LEAN) o Lidocaine o Epinephrine o Atropine o Naloxone (Narcan) CONGENITAL HEART DISEASE

RHEUMATIC FEVER Guidelines for the Diagnosis of Initial Attack of Rheumatic Fever (Jones Criteria, Updated 1992) SUPPORTING EVIDENCE OF ANTECEDENT GROUP A MAJOR MINOR STREPTOCOCCAL MANIFESTATIONS MANIFESTATIONS INFECTION Clinical features: Carditis Positive throat culture or rapid streptococcal antigen test Polyarthritis Arthralgia Fever Elevated or increasing streptococcal antibody titer Erythema marginatum Laboratory features: Subcutaneous Elevated acute phase nodules reactants: Erythrocyte sedimentation rate C-reactive protein Prolonged PR interval Chorea intended only for the diagnosis of the initial attack of acute rheumatic fever and not for recurrences 5 major and 4 minor criteria and an absolute requirement for evidence (microbiologic or serologic) of recent GAS infection. Diagnosis of acute rheumatic fever: 2 major criteria or 1 major and 2 minor criteria and meets the absolute requirement. Chorea may occur as the only manifestation of acute rheumatic fever. Indolent carditis may be the only manifestation in patients who 1st come to medical attention months after the onset of acute rheumatic fever Criteria for determining activity: joint symptoms new significant murmur increasing heart size congestive heart failure in the absence of old valvular disease subcutaneous nodules rectal temperature >100.4 F for at least 3 consecutive days sleeping pulse of >100/min positive C-reactive protein *considered active if any one of the following findings is present RHEUMATIC HEART DISEASE MR/MS is appreciated on PE LVH/RVH on ECG irregular cardiac borders on CXR *In RF there is also cardiomegaly but with normal ECG findings Rheumatic Carditis Mild carditis no cardiomegaly & no CHF Moderate carditis cardiomegaly & CHF Severe carditis severe CHF & pulmonary edema Valvulitis Apical systolic murmur (Mitral regurgitation) Apical mid-diastolic murmur (Carey-Coombs) Basal diastolic murmur (Aortic regurgitation) Basal systolic murmur (Tricuspid regurgitation) Resting tachycardia Muffled heart sounds Gallop rhythm Pericardial friction rub Congestive heart failure Treatment 1. Mild carditis ASA 80-100mg/kg/day X 2 weeks then 60mg/kg/day X 2 weeks 2. Moderate to severe carditis Prednisone 2mg/kg/day X 2 weeks, Tapering until discontinued On the last week of prednisone, start ASA 80-100mg/kg/day X 2 weeks then 60mg/kg/day X 2 weeks Page 5 /epcapul

Approach to Congenital Heart Disease: by location of murmur

Aortic Valve Stenosis
-Supravalvar AS -Subvalvar AS

Pulmonary valve stenosis Atrial septal defect Pulmonary ejection murmur (innocent) Pulmonary flow murmur
-PA stenosis

-Aortic stenosis -PDA, PAPVR, TAPVR

Ventricular Septal Defect Endocardial Cushion Defect Vibratory Innocent Murmur

-HOCM (IHSS) -Tricuspid regurgitation -TOF

Mitral Regurgitation Vibratory innocent murmur MVP syndrome Aortic stenosis IHSS

ACYANOTIC DEFECTS

INC.PBF L to R Shunts

NORMAL PBF Obstructive Lesions

LVH or CVH

RVH

LVH

RVH

- VSD - PDA - ECD

- ASD - PAPVR

- AS or AR - CoA - MR

- PS - CoA - MS

(infants)

CYANOTIC DEFECTS

PBF
LVH or CVH RVH CVH

PBF
LVH RVH

- PTA - SV - TGA-VSD

- TGA-IVS - TAPVR - HLHS

- TGA-PS - PTA w/

- TVA - TOF - PVA-IVS - Ebsteins a. hypopl. PAs - PVOD - SV w/PS

Pedia Notes

INFECTIVE ENDOCARDITIS (Duke criteria) Major criteria (1) positive blood cultures (two separate cultures for a usual pathogen, two or more for less typical pathogens) and (2) evidence of endocarditis on echocardiography (intracardiac mass on a valve or other site, regurgitant flow near a prosthesis, abscess, partial dehiscence of prosthetic valves, or new valve regurgitant flow) Minor criteria (1) predisposing conditions (2) fever (3) embolic-vascular signs (4) immune complex phenomena (glomerulonephritis, arthritis, rheumatoid factor, Osler nodes, Roth spots) (5) a single positive blood culture or serologic evidence of infection (6) echocardiographic signs not meeting the major criteria. Definite Endocarditis: Two major criteria, one major and three minor, or five minor criteria KAWASAKI DISEASE Clinical and Laboratory Features EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA) Fever persisting at least 5 days Presence of at least 4 principal features: Changes in extremities Acute: Erythema of palms, soles; edema of hands, feet Subacute: Periungual peeling of fingers, toes in weeks 2 and 3 Polymorphous exanthema Bilateral bulbar conjunctival injection without exudates Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral Exclusion of other diseases with similar findings 3 Clinical phases: Acute Febrile Phase ( 1-2 weeks ) fever, conjuctivitis, erythema, rash Subacute Phase ( 2-4 weeks ) begins when the fever stops desquamation, thrombocytosis, coronary aneurysms Convalescent Phase ( 4-6 Weeks ) resolution of all sign & symptoms labs return to normal coronary aneurysms persists Coronary Aneurysms Classification of CAA Small - <5mm internal diameter Medim - 5-8 mm internal diameter Large - >8 mm internal diameter Treatment Acute Stage Intravenous Immunoglobulin: 2g/kg over 10-12 hours With Aspirin (80-100 mkd) q6, until day 14 of illness and afebrile for 48 to 72 hrs This therapy should be instituted within the 1st 10 days of illness and if possible within 7 days of illness. Convalescent Stage Aspirin (3-5 mkd) OD, until the patient shows no evidence of CA changes by 6-8 weeks after the onset of illness. IVIG also should be administered to children presenting after the 10th day of illness Long Term: Coronary Abnormalities Aspirin (3-5 mkd) divided dosed, continued indefinitely High dose intravenous gammaglobulin (IVIG) effective in preventing the occurrence of coronary artery abnormalities in KD. Patients treated with IVGG have a significant increase in T suppressor cells, a decrease in circulating activated T helper cells, and a decrease in spontaneous IgG and IgM synthesis. suggest that IVGG reduces the vasculitis in KD by suppressing the marked immune activation associated with this disease. Pedia Notes

Measles and Varicella immunization should be deferred for 11 months after child receives high-dose of IVIG Even when treated with high-dose of IVIG within the 1st 10 days of illness, 5% of children develop at the least transient coronary artery dilation and 1% develop giant aneurysms. Careful monitoring is necessary during the administration of gamma globulin because it rarely can cause an allergic-like reaction.

DEVELOPMENT
Anterior fontanelles closed at 7-19 months Posterior fontanelle closed at 3 months ANTHROPOMETRICS Length/Height Average Birth Length: 50cm Length: 9-8-5-3cm Height: agex5+80

Head Circumference Average 13-14in 0-4 mos 2in 5-12mos 2in 1-2 yrs 2 in Weight 2-5 yrs 2 in 5-20 yrs 2 in Average BW: 3000 1-6mos= age in mos x 600 + BW OR 7-12mos= age in mos x 500 + BW Average: 35cm 1-6yrs=agex2+8 0-3mos 2cm/mo 3-6 1cm/mo 7-12yrs=agex7-5/2 6-9 0.5cm/mo 9-12 0.5cm/mo BSA: square root of (wt x ht / 3600) 1-3yrs 0.25cm/mo 4-6yrs 1cm/yr Height age age points on the growth curve where the childs height falls on the 50th percentile Weight age age point on the weight curve where the childs weight falls on the 50th percentile Midparental height 7 (for girls) 10 Midparental height + 7 (for boys) 10 OR For Males: (mothers height + 13cm + fathers height) 5 2 For Females: (Fathers height - 13cm + mothers height) 5 2 Growth Velocity (cm/yr) Ht (cm) measured at Time 2 - Ht (cm) measured at Time 1 X 12 (mos/yr) Number of months between time 2 and time 1 Age Rate (cm/yr) 1-2 month 38 4 months 28 1 year 12 2 years 10 3-4 years 7 5-6 years 6 7-puberty 5 Arm Span Age Boys: <10-11 years Girls: <11-14 years Adult Male Adult Female Body Mass Index (BMI) Wt (kg) / ht2 (m2) Overweight: >85th percentile Obesity: >95th percentile Body proportions Upper segment sitting height (measure using Harpenden sitting table) Lower segment measure from upper border of symphysis pubis to floor in standing position US/LS: Birth = 1,7; 10 years 1 Page 6 /epcapul Arm Span <height <height >Height by 5.3cm >Height by 1.2cm

Sexual Maturity Rating Girls Stage Breast 1 Preadolescent 2 Breast and papilla elevated as small mound, diameter of areola is increased 3 Breast and areola enlarged, no contour separation 4 Areola and papilla form secondary mound 5 Mature nipple projects, areola part of general breast contour Boys Stage 1 2

ENDOCRINOLOGY
Pubic Hair Preadolescent Sparse, lightly pigmented, straight, medial border of labia Darker, beginning to curl, increased amount Coarse, curly, abundant but less than in adult Adult feminine triangle, spread to medial surface of thighs Pubic Hair None Scanty, long, slightly pigmented Darker, beginning to curl, small amount Resembles adult type, but less quantity; coarse, curly Adult distribution, spread to medial surface of thighs IDF definition of Metabolic Syndrome in children and adolescents Age 6 to <10 years Obesity 90th percentile as assed by WC. MS cannot be diagnosed but further measurements should be made if family history of MS, T2DM, dyslipidemia, CVD, hypertension, or obesity Age 10 to <16 years Obesity 90th percentile as assessed by WC Triglyceride 1.7mmol/L (150mg/dL) HDL Cholesterol < 1.03 mmol/L (40mg/dL) BP 130mmHg systolic or 85mmHg diastolic Glucose 5.6 mmol/L = 100mg/dL (OGTT recommended) or known T2DM Age >!6 yo Use existing IDF criteria for MS DIABETES MELLITUS Positive findings from any two of the ff. tests on different days: Symptoms of DM plus casual plasma glucose 200 mg/dl (11.1 mmol/l) or Fasting plasma glucose 126 mg/dl (7 mmol/l) or 2hrsPPG 200 mg/dl (11.1 mmol/l) after a 75g glucose load Clinical manifestation HYPERGLYCEMIA Osmotic diuresis Dehydration Electrolyte losses Na, K, PO4 Volume contraction Azotemia KETOSIS ACIDOSIS Hyperventilation Hypocapnia Dec. cerebral blood flow Circulatory depression Ileus Gastric dilatation DIABETIC KETOACIDOSIS hyperglycemia (BG >200 mg/dl (11.1 mmol/l) heavy glycosuria (>55 mmol/l) ketonuria acidosis (pH < 7.3) ( HCO3 < 15 mmol/l) 5% or more dehydrated vomiting / drowsy Principle 1:Restoration of vascular volume In shock with poor peripheral perfusion or coma: give 10 cc/kg x 10-30 min Repeat if poor pulses remain Fluid of choice: 0.9 NSS Fluid input > 4li/m2 : incrd risk for cerebral edema IV therapy MODEL 1 Reqts = Deficit + Maintenance Maintenance: 3 9 kg 80 cc/kg/d 10-19 kg 70 cc/kg/d 20-30 kg 60 cc/kg/d 30-50 kg 50 cc/kg/d >50kg 35 cc/kg/d Add deficit to 48 hr MTN; Replace for 48 hrs w/ PNSS

Penis Preadolescent Minimal change / Enlargement Lengthens

Testes Preadolescent Enlarged scrotum, pink texture altered Larger

Larger; Glans and breadth increase in size Adult size

Larger, scrotum dark

Adult size

RED FLAGS Motor Delay poor head control by 3 months hands still fisted by 4 months unable to hold objects by 7 months does not sit independently by 10 months cannot stand on one leg by 3 years Language Delay does not turn to sound by 6 months does not babble or use gestures by 12 months no single word utterances by 16 months No 2-word phrases by 2 years No 3-word sentences by 3 years Psychosocial Delay No social smile by 3 months Not laughing in playful situation by 6 months Hard to console, stiffens when approached by 1 year In constant motion, resists discipline Does not play with other children at 3 years Cognitive delay - 2 months Not alert to mother -6 months Not searching for dropped objects - 12 months No object permanence - 18 months No interest in cause-and-effect games - 2 years Does not categorize similarities - 3 years Does not know full name -4 years Cannot count sequentially - 5 years Does not know letters or colors -5 years Does not know birthday or address

Pedia Notes

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MODEL 2 Covers maintenance + 10% deficit, give evenly for 48 hrs. 3 9 kg 6 cc/kg/hr 10 19 kg 5 cc/kg/hr 20 kg 4 cc/kg/hr (max 250 cc/hr) Compute for the fluid requirement of VJ ( BW=35 kg) Deficit: 35 x 60cc= 2100 ml (assume mod dehydration unless shocky) Maintenance: (35 x 50) x 2=35000 Total fluid for 48h: 5600 ml Monitor urine output especially during the first 4-6 hours of therapy Replace urine losses volume per volume Entails frequent changing rate of infusion Potassium supplementation Start as early as the 3rd hour or even earlier as long as the patient is voiding Shift to a glucose containing solution when the blood glucose is down to 200 mgs% Principle 2:Inhibition of lipolysis and correction of hyperglycemia Insulin therapy Only short-acting insulin is used ( Humulin R, Actrapid ) Should not be started until shock has been reversed IV route only Target fall in blood glucose: 50-100 mg per hour Low dose continuous Insulin Infusion 0.1 u/kg/hr (consider 0.05 u/kg/hr for a young child) Hourly blood glucose, fluid input and output Neurological status at least hourly Electrolyte 2 hrs after start of IV therapy Monitor ECG for T wave changes How to prepare insulin infusion? Mix 10 U SA insulin in 100 cc plain NSS 0.1 u/ml Flush tubings with solution For VJ: 35 kg x .1u/kg/hr= 3.5 u 35 ml/hr So, always prepare a solution as above so that infusion rate is equal to the weight of the patient When do you stop insulin infusion? acidosis is resolved patient is awake How to shift to subcutaneous route? Compute at .15-.25 u/kg/dose q6h to be given 30 min pre-meals and at MN D/C infusion 30 min after 1st SQ dose Do not increase insulin levels >100% Dosing Schedule AM 2/3 PM 1/3 Principle 3:Correction of acidosis Sodium Bicarbonate therapy pH < 7.0 HCO3 < 5meq/li Half-correction over 30 minutes - 1 hour HR 1/3 HN 2/3 HR 1/3 HN 2/3

THYROID STORM Precipitating factors for thyroid storm Infection Surgery (thyroidal and nonthyroidal) Therapy with radioactive iodine Administration of iodinated contrast dyes or ingestion of large, stable iodine loads Withdrawal of antithyroid medication Amiodarone therapy Ingestion of excessive amounts of exogenous thyroid hormone Diabetic ketoacidosis Congestive cardiac failure Hypoglycemia Toxemia of pregnancy Parturition and the immediate postpartum state Severe emotional stress Acute manic crisis Pulmonary embolism Cerebral vascular accident Bowel infarction Acute trauma Tooth extraction Vigorous palpation of thyroid gland The predictive clinical scale for thyroid storm (Burch and Wartofsky) Scoring Parameter taken into consideration points Thermoregulatory dysfunction, Temperature (oral) 99-99.9F 37.2-37.7 C 5 100-100.9F 37.8-38.2 C 10 101-101.9F 38.3-38.8 C 15 102-102.9F 38.939.3C 20 103-103.9F 39.4-39.9 C 25 >104F >40 C 30 CNS effects Absent 0 Mild (agitation) 10 Moderate (delirium, psychosis, extreme 20 lethargy) Severe (seizures, coma) 30 GI-hepatic dysfunction Absent 0 Moderate (diarrhea, nausea/vomiting, 10 abdominal pain) Severe (unexplained jaundice) 20 Tachycardia (beats/min) 99-109 5 110-119 10 120-129 15 130-139 20 >40 25 Congestive cardiac failure Absent 0 Mild (pedal edema) 5 Moderate (bibasal rales) 10 Severe (pulmonary edema) 15 Atrial fibrillation Absent 0 Present 10 Precipitating event Absent 0 Present 10 A cumulative score of >45 is highly suggestive of thyroid storm, 25-44 is suggestive of impeding storm, and <25 is unlikely to represent thyroid storm.
From Sarlis NJ, Gourgiotis L. Thyroid emergencies. Rev Endocr Metab Disord 2003;4:129-36.

Treatment 1.Phenobarbital - may be used for sedation because it stimulates metabolic clearance of thyroid hormone by the liver 2. PTU - 600-1000 mg (12-20 mg/kg) loading dose, followed by 200-300 mg (4-6 mg/kg) every 4-6 hrs orally. The drug of choice because of its inhibition of peripheral conversion of T4 to T3 in addition to its inhibition of synthesis of thyroid hormone Pedia Notes Page 8 /epcapul

3. Methimazole (alternative) - given as a loading dose of 60-100 mg (1.22 mg/kg), followed by 20-30 mg (0.4-0.7 mg/kg) every 6-8 hrs orally 4. Inorganic iodine -Ideally, iodine therapy should be administered 2 hrs after initial thiourea dosing, to allow for initial blockade of iodine organification. Saturated solution of potassium iodide (children, 5 drops, 250 mg, 2-4 times/day, infants 2 drops 4 times/day) and Lugol solution (4-8 drops 3 times/day) 5. Lithium therapy - (300 mg or 6 mg/kg every 6 hrs) may be used in addition to iodine to block thyroid hormone release 6.High-dose corticosteroids - Hydrocortisone 50-100 mg, IV, every 6-8 hrs or 25-50 mg/m2 body surface - effective in blocking peripheral conversion of T4 to T3 7.-adrenergic blocking agent - -blockers (e.g., propranolol, 40-80 mg, 0.5 mg/kg, orally, or 1-3 mg/dose IV, every 4-8 hrs; Given in the absence of cardiac failure, effective in reducing tachycardia, hypertension, and adrenergic symptoms associated with thyrotoxicosis * medical therapy should be used for weeks to months before definitive treatment with radioactive iodine or thyroidectomy Indications for thyroidectomy Thyroid cancer Prophylactic thyroidectomy in children with MEN-2 A large multinodular goiter Graves disease (including young children, not responding to antithyroid drugs, in whom radioactive iodine is contraindicated) Steroids Hydrocortisone 50-100mg/m2 as loading dose then 50-100mg/m2 in divided doses q6 4 Hydrocortisone = 1 Prednisone 25 Hydrocortisone = 1 Dexamethasone Taper 25% every week Glucocorticoid Comparison Chart Name Equivale Relative Relative Plasm Biologi nt Dose AntiMineralocorticoi a c (mg) inflammator d Potency Half- Halfy Potency life life Short-acting Cortisone 25 0.8 2 0.5 8-12 Hydrocortisone 20 1 2 1.5-2 8-12 Intermediate-acting Methylprednisolo 4 5 0 1.5-3 18-36 ne Prednisone 5 4 1 1 18-36 Prednisolone 5 4 1 2-3.5 18-36 Triamcinolone 4 5 0 3.5-4 18-36 Long-acting Betamethasone 0.6 20-30 0 5.5 36-54 Dexamethasone 0.75 20-30 0 2-3.5 36-54 -HCG : 3000mg/BSA

Body Surface Method Water Na+ K+ Requirements 1500 ml/m2/day 30-50 meq/m2/day 20-40 meq/m2/day

Factors Modifying Fluid Requirements Additional Fluids Needed fever 12% for each C > 37.5C sustained hyperventilation or excessive 25-50% muscular activity hypermetabolic states 25-75% for burns: 2% increase per 1% BSA with burns diarrhea and vomiting volume per volume sweating 10-25% room temperature > 31C 30% per C rise > 31C newborn under radiant warmer or 25% phototherapy Less Fluids needed hypothermia very high humidity humidified inspired air oliguria or anuria sedated or paralyzed Electrolyte Na+ K+

12% per C fall below 37.5C 30% 25% Individualized 40%

Daily Requirement (meq/kg/day) 2.5-3.0 2.0-2.5

Determine the fluid deficit Severity of Dehydration Infant Child (>10 kg) mild 50 cc/kg 30 cc/kg moderate 100 cc/kg 60 cc/kg severe 150 cc/kg 90 cc/kg determine the maintenance fluid requirement give the of the fluid deficit over the 1st 8 hours then over the next 16 hours re-assess hydration status periodically for moderate to severe dehydration, check serum electrolytes ELECTROLYTES Na 135-145; K 3.5-5; Ca 2.1-2.6; HCO3 22-26 IVF IVF pLR

Na+ (meq/L) 130 154 51 25 140 40

K+ (meq/L) 4 20 5 13

Cl(meq/L) 109 154 51 22 98 40

FLUIDS & ELECTROLYTES

Daily Water Loss Area obligatory urine volume stool water Skin Lungs

ml/100 cal expended 50-55 0-5 30 15

pNSS D5 0.3NaCl D5IMB D5NR D5NM

HCO3(meq/L) 28 (lactate) 27 (acetate) 16 (acetate)

Mg++ (mg/dL) 3 3

Ca++ (mg/dL) 3 -

Holliday-Segar Method Weight Daily Requirements 3-10 kg 100 ml/kg 11-20 kg 1000 ml + 50 ml/kg for each kg > 10 kg > 20 kg 1500 ml + 20 ml/kg for each kg > 20 kg

HYPONATREMIA Fast correction: -4mL/kg/dose of 3% NaCl -3% NaCl= 1mL (2meqs/mL NaCl + 4mL sterile water) -Total Na required= (M+D) bolus M= 3meqs/kg/day D= (desired Na actual Na) x o.6 x wt Page 9 /epcapul

Pedia Notes

HYPERNATREMIA Total water required for 2 days = (M for 2 days +D) bolus Ideal TBW (in liters)= wtx 0.6; ideal serum Na 140 Water deficit= ideal TBW actual TBW Actual TBW= ideal TBW x ideal serum Na/actual serum Na CORRECTED SODIUM Glucose in mg/dL Na+ + Glucose -100 x 1.6 100 Glucose in mmol/L Na+ + Glucose -5.6 x 1.6 5.6 HYPOKALEMIA Fast correction 0.5meqs/kg/dose in PNSS diluent x 1hour x 3-5 doses (max 40meqs/L) Example: Wt 20kg: (0.5meg/kg/dose K? x 20kg =10meq/hr Compute how much diluent is required. Central line (200meq/L concentration) 200meq = 10meq 1000mL x X=50mL Order: Give 10meq K in 50mL NSS x 1hr Peripheral line (60meq/L concebtration) 60meq = 10meq 1000mL x X=170mL Order: Give 10meq K in 170mL NSS x 1hr Bedside Pediatric Nephrology PO correction is potassium chloride of 4-6meg/kg/day given in divided doses. Parenteral correction Intermittent Dosing: (for symptomatic hypokalemia) 0.5 to 1.0meq/kg/hr (maximum 30meq/hr) with maximum infusion rate of 0.5meg/kg/hr and given Q2-4hours until symptoms resolve. Continuous Dosing: (for non-symptomatic hypokalemia) 0.2-0.3meg/kg/hr for 24hours *always consider the possibility of Magnesium deficiency especially among patients with refractory hypokalemia. Magnesium is a important co-factor for the activity of the Na-K-ATPase pump which is necessary for potassium homeostasis. Hariett Lane: Oral: Child: 1-4meg/kg/24hrBID-QID Adult: 40-100meq/24hrBID-QID IV: Child: 0.5-1meq/kg/dose given as an infusion of 0.5meq/kg/hr x 1-2hr Max: 1meq/kg/hr. This may be used in critical situations(i.e. hypokalemia with arrhythmia) Adult: Serum K >2.5meq/L: Replete at rates up to 10meq/hr. Total dosage not to exceed 200meq/24hr Serum K <2meq/L: Replete at rates 40meq/hr. Total dosage not to exceed 400meq/24hr Maximum peripheral IV solution concentration: 40meq/L Maximum concentration for central line administration : 150-200meq/L Kalium durule 10meq/durule 10% Oral KCl=1.34meq/mL HYPERKALEMIA 10% Calcium Gluconate (100mg/kg/dose) + equal diluent x1 hour - aims to stabilize cell membrane and opposes the negative inotropic effect of hyperkalemia Glucose-insulin drip: 5mL/kg D10 or 1mL/kg of D50 + 0.1unit/kg Humulin R over 30-60minutes Beta2 adrenergic agonist- Salbutamol administration at 1-5mcg/kg/min IV or nebulized at 10-20mg over 15 minutes - drive potassium into the cells just like insulin Pedia Notes

sodium bicarbonate- 2meq/kg IV over 30minutes (except for ERD patients) Polystyrene sulphonate resins-0.5-1gm/kg PO or PR Q4-6hours Sodium Bicardonate Base deficit Wt (kg)x distribution of NaHCO3(0.3) HYPOCALCEMIA -100mg/kg/dose Calciumgluconate/IV + equal diluent to run for 1 hour x 4-6doses, Q6hours (Max 2g) -Corrected Calcium: = (40-albumin) x 0.002+ actual serum calcium INFUSIONS / DRIPS Dopamine amount (cc) = dose (mcg/kg/min) x BW (kg) x 480 40,000 Dobutamine amount (cc) = dose (mcg/kg/min) x BW (kg) x 480 12,500 Epinephrine amount (cc) = 0.6(BW) + sterile water to make 100 cc 0.1 mcg/kg/min = 1 cc/hr general formula drip rate (cc/hr) = dose x BW x 60 x total volume___ preparation Preparation Dose (mcg/kg/min) Albumin 20% 10mg/50mL Abumin 25% 12.5mg/50mL Aminosteryl 6% 6g/100mL Dobutamine 250mg/20mL 5-20 (250,000 mcg/20mL) Dopamine 200mg/5mL 5-10 (200,000mcg/5mL) Epinephrine 1mg/mL 0.1-0.5 (1000mcg/mL) Furosemide 20mg/2mL 2 Midazolam 15mg/3mL 1 (15,000mcg/3mL) Milrinone 10mg/10mL 0.25-1 Nitroglycerine(NTG) 10mg/mL 1-5 (10,000mcg/mL) Norepinephrine 2mg/mL Max: 2mcg/kg/min Thiopental 20mg/mL (20,000mcg/mL) Voluven 6% Max 50cc/kg/day DEXTROSITY Dextrosity of Fluids D5W contains 5 g of glucose per 100 ml changing dextrosities of fluids factor = desired dextrosity lower dextrosity higher dextrosity lower dextrosity amount of D50 to be added to actual IVF = (factor)(actual IVF being given) peripheral line D12 central line D20 Glucose Infusion Rate determines adequacy of infused glucose (dextrosity)(drip rate in cc/hr)(0.167) body weight (kg) nv: infants 6-8; children 4-6 GIR normal = 5-8 GIR = rate (gtts/min) x dextrosity x 1,000 Weight x 60 BURN - Parkland D1: 4ml/ k/ %BSA burned x hrs, x 16hrs + maintenance D2: 50-75% of above Maintenance fluids should be estimated for children who weigh <40kg For adults and children who weigh >40kg, maintenance fluids are not included in the estimate of fluid requirements Half of this volume is given in the first 8 hours after injury and the other half is given in the following 16 hours Page 10 /epcapul

GASTROENTEROLOGY
NUTRITIONAL STATUS ASSESSMENT WATERLOWE CLASSIFICATION S: 90-95 (Mi); 80-90 (Mod); <80 (S) W: 80-90 (Mi); 70-80 (Mod); <70 (S) Stunting x 100 Actual height Ideal ht for age > 95% normal 90-95 mild 85-90 mod <85 severe Wasting Actual weight x 100 Ideal wt for ht >/= 90% normal 80-90 mild 70-80 mod < 70 severe DIARRHEA Plan A <2k 50-100ml 2-10k 100-200ml >10k as much

Plan B 75ml/kg x 4hrs Plan C Infants 30ml/k x 1hrs, 70ml/k x 5hrs Older 30ml/k x 30mins, 70ml/k x 2hrs

CALORIC COMPUTATION Protein: gm/k/d x wt x 4; requirement 0.5-3gm/k/d Lipid: gm/k/d x wt x 9; requirement 0.5-4gm/k/d CHO: gm/100cc x vol x 4 (eg. D5=5gm/100cc) CALORIC DISTRIBUTION OF CHO, COOH, CHON OF TOTAL CALORIES GIVEN CHO 60-70% CHON 10-15% Fats 20-30% Breastmilk: 20cal/oz; VCO: 7.7cal/cc; Cereal 12.4cal/scoop 1gm Nitrogen = 6.25gm protein Supplements for Severe Malnutrition: 50% MgSO4 2ml (2mmol/mL) IM x 1 dose; Zn 1mkd until diarrhea stops; Cu 0.1mkd; Folic acid 5mcg/k/d; Fe 3mkd; Vit A (if not given w/in 6mos) <6mos 50,000iu, 6-12mos 100,000iu, >1yr 200,000iu Age REE Multiplication factor 0-1 55 Maintenance 0.2 1-3 57 Acitivity 0.1-0.25 4-6 48 Fever 0.13/deg >38C 7-10 40 Simple Trauma 0.2 11-14 32M/ 28F Multiple Injuries 0.4 15-18 27M/ 25F Burns/ GI surgery 0.5-1 Total Daily Energy Req: Sepsis 0.4 REE + REE x Total factors Growth 0.5 FORMULA FOR CALORIC REQUIREMENT FOR CATCH-UP GROWTH Get the height, weight get ideal weight for actual height kcal/kg=RDA for age (kcal/kg) x ideal wt/ht actual wt CHON=CHON recommended for age x ideal wt/ht Actual wt * start by 50-70% of caloric requirement * increase calories by 20 kcal/kg/k every 2 days until caloric requirement is reached * increase protein by 0.5 g/kg every 2 days until catch up is reached RDA for CHON (g/kg/day) 0-6 mos 2.2 7-12 mos 1.5 1-2 yrs 1.1 3-8 yrs 0.95 9-13 yrs 0.95 14-18 yrs 0.85 RECOMMENDED ENERGY INTAKE (kcal/kg) per kg per day Infants 0-6mos 108 650 6-12 mo 98 852 Children 1-3 yr 102 1,300 4-6 yr 90 1,800 7-10 yr 70 2,000 Males 11-14 yr 55 2,500 15-18 45 3,000 19-24 40 2,900 25-50 30 2,900 > 50 30 2,300 Females 11-14 yr 47 2,200 15-18 40 2,200 19-24 38 2,200 25-50 36 2,200 > 50 30 1,900 Pregnant +300 Lactating +500 CALORIC REQUIREMENT FOR PARENTERAL NUTRITION Neonate 90-120 cal/kg </= 10 kg 100-170 cal/kg 11-20 kg 1000 cal + 50 cal/kg in xces of 10kg > 20 kg 1500 + 20 cal/kg in excess of 20 kg Page 11 /epcapul

ReSoMal: 1Lpack Oresol, 1L water; 45mL 10% KCl, 50gm sucrose 1st 2hrs 5ml/k q30mins; 4-10hrs 5-10ml/k/hr VOMITING WARNING SIGNALS in the vomiting infant Bilious vomiting GI bleeding (hematemesis, hematochezia) Forceful vomiting Onset at 6 mos of life Failure to thrive Diarrhea Constipation Fever Lethargy Hepatosplenomegaly Bulging fontanelle Macro/microcephaly Seizures Ab tenderness/distention Genetic disorders (trisomy 21) Other chronic d/o (eg HIV) GER passage of gastric contents to esophagus GERD gastric contents to esoph/oropharynx and produce symptoms such as: Recurrent vomiting hematemesis Wt loss/FTT dysphagia Irritability feeding refusal Regurgitation cough apnea/ALTE anemia recurrent pneum Hoarseness Heartburn/chestpain wheezing/stridor Esophagitis Barrets esoph OMEPRAZOLE - duodenal ulcer : 20-40 mg OD x 2-4 wks - benign gastric ulcer, reflux esophagitis 20-40 mg OD x 4-8 wks - children >2yrs serious reflux esophagitis 1 mkd for 4-8 wks > 20 kg 20 mg OD 10-20kg 10 mg OD - NSAID induced gastric and duodenal ulcer : 20 mg OD x 4-8 wks - GERD 10-20 mg OD x 2-4 wks - symptomatic GERD w/ esophageal lesions 20 mg OD x 4 wks -maintenance of healing of erosive esophagitis 20 mg OD up to 12 mos Eradication of H. pylori BID x 1 wk: Omeprazole 20 mg + Amox 1000 mg + clarithromycin 500 mg BID x 1 wk: Omeprazole 20 mg + Metro 500 mg + clarithromycin 250 mg Pedia Notes

FAT requirement in parenteral nutrition 0-12 mos 2 g/kg/day 1-8 yr 4 g/kg/day > 8 yr 2.5 g/kg/day CARBOHYDRATE reqt VLBW (<1.5 kg) 2.25 0-12 mo 2.5 1-8 yr 1.5-2 > 8 yr 1-1.5 NORMAL ELECTROLYTE REQT Na 2-4 meq/kg/day K 2-3 Cl 2-3 Mg 0.25-0.5 Ca Infants 300-400 mg/kg/day Children 100-200 Adolescent 50-100 Phosphorous Infants 1-1.5 mmol/kg/day Children 1 Adolescent 0.5-1 F75 DIET: 75 cal/100 cc Skimmed milk powder 25g Veg. oil 20g Sugar 60g Rice (cereal) powder 60g Water to make 1,000 ml *give 100-130 cc/kg/day F100 DIET: 100 cal/100 cc Skimmed milk powder 80g Veg. oil 60g Sugar 50g Water to make 1,000 ml * minimum daily intake of 120-200 ml/kg RESOMAL (ORS for malnourished patients) Dilute 1 L of ORS with 1 L water Add 45 ml of 10% KCl Add 50 g sucrose Composition: Na 45 mmol/L K 40 mmol/L Sugar 25 g/L FEEDING REGIMEN (ENTERAL NUTRITION) 1. Intermittent/bolus more physiologic - should only be used for gastric feed - start at 1-5 ml/kg/bolus - every 3-6 hrs - deliver over 30-120 min (2 hrs) 2. Continuous better tolerated in px w/ feeding intolerance & significant GER - for critically ill px - start 1-2 ml/kg/hr in child/adol - can be increased by 1-2 ml/hr - concentration shld be inc before volume

(or whole liquid milk 85-295) Rice 15g Vegetable oil 3-5g Cane sugar 3g Water to make 200 ml * 130 ml/kg provides 110 cal/kg 2nd Diet: Lactose free w/ reduced starch 75cal/100g Whole egg 64g Rice 3g Vegetable oil 4g Glucose 3g Water to make 200 ml * if finely ground cooked chicken meat is used instead of egg. Provides 70 cal/100 * 145 ml/kg provides 110 cal/kg MILK FORMULAS Alfare: 72 cal/100 65 cal/100 Fats Linolento CHON CHO

1:1 dilution 15g:100 ml 72cal/100ml 3.6 g 0.42 g 2.5g 7.8 g Cal/100 ml 22 44 65 72

65cal/100ml 3.3g 0.38g 22g 7g

D1: 1 scoop +100 ml D2: 2 scoops + 100 ml D3: 3 scoops + 100 ml D4: 3 scoops + 90 ml

PEPTAMEN JUNIOR (1cal/ml) 7 scoops in 210 ml to make 250 ml 14 scoops in 425 ml to make 500 ml 28 scoops in 850 ml to make 1000 ml Per 1L preparation Fat 38.5g CHON 30.1 g CHO 138.4 g * 60% MCT * 100% hydrolyzed when CHON 30 g /L NUTREN JUNIOR (same prep as peptamen) 1 cal/ml 12% CHON 35% COOH 53% CHO MCT 25% Lactose free/ gluten free Per 100 ml CHON 3g CHO 13.3g COOH 3.9 g PEDIASURE standard dilute Per 100 ml Caloric content 100cal CHON 3g COOH 4.78g CHO 43.8g * 190 ml of water + 5 scoops to make 225 ml MICRONUTRIENTS Vitamin A single dose < 6mos 50,000 IU 6-12 mos 100,000 IU > 12 mos 200,000 IU Zinc 1mg/kg/d Copper (infants) 0.2-0.6 mg/day (child/adol) 1-2 mg/day MgSO4 50% - 2ml IM/SQ Folic acid 5 ucg/kg/d Iron to start only in the 2nd week of illness when infection is better controlled at a dose of 3mg/kg/d Page 12 /epcapul

NUTRITIONAL GUIDELINE Energy caloric goal = 125% RDA based on wt/ht at 50th percentile * glucose polymer to in to 24-27 cal/mg formula * MCT infant formula * MCT oil supplement 1-2 ml/k/d 2-4 doses * supplemental nighttime NGT feeding Essential Fatty acids corn oil Protein intake (infants) 2-3 g/k/d (child) 0.5-1 g/k/d Children Hospital Formulary - started at 10-20 cc/kg/d as bolus or cont. - advance by </= 20-25 ml/kg/d PERSISTENT DIARRHEA First Diet: Reduced Lactose Full fat dried milk Pedia Notes

70 cal/100g 11g

MICRONUTRIENTS FOR UPBUILDING Vitamin A Folic Acid 800 ucg/prep (5 ucg/kg/d) D1-LD 5 mg or 5 tabs D2 1 mg or 1 tab Zinc 1-2 mg/kg/d Copper 0.2-0.6 mg/d (infant) 1-2 mg/d (children) FOR ACUTE DIARRHEA Zinc <6mo : 10 mg/d for 10-14 days >6mo : 20 mg/d for 10-14 days Test dose for Intralipid < 5kg : 0.1 g/kg x 1 hr > 5kg 0.01 g/min x 10-15 min TOTAL PARENTERAL NUTRITION (TPN) amino acids make fluid D7.5/D10 NaCl (2.5 meq/ml) 3 meq/kg KCl (2 meq/ml) 2 meq/kg Ca gluconate 10% - wt x 3, or wt x 300/100 MgSO4 (25% 1meq/ml, 50% 2meq/ml) -0.2 meq/kg NEONATAL CHOLESTASIS CHOLERETIC DRUGS UDCA 250mg/tab, 15-45 mkd Rifampicin 5mkd Cholestyramine 4-16 g/d Phenobarital 3-10 mkd Vitamin A 2,500-25,000 IU/day Clusivol drops /0.6ml = 4,000 IU Clusivol syrup /5ml = 2,500 IU Nutrilin drops /ml = 5,000 IU Nutrilin syrup /5ml = 1,500 IU Enervon C drops /ml = 3,500 IU Enervon C syrup /5ml = 100 IU Vitamin D 400-1,200 IU/day as D3 Clusivol drops /0.6ml = 400 IU Clusivol syrup /5ml = 500 IU Nutrilin drops /ml = 333.33 IU Nutrilin syrup /5ml = 100 IU Enervon C drops /ml = 200 IU Enervon C syrup /5ml = 200 IU Rocaltrol (Calcitriol) 0.25ucg/cap = 0.05-0.2 ucg/kg/d Vitamin E 15mg/d -200 mg/kg/d or alpha tocopherol acetate (squibb) [100 or 200 or 400 IU/cap] 25-200 IU/kg/d, 1 cap at least q5 days in infants 100 IU = 65 mg Vitamin K 1-5 mg/d Ca (elemental) 50-200 mg/kg/d 25-100 mg/kg/d Up to 800-200 mg/d Ca Sandoz /5ml =110mg elemental Ca Sandoz /tab =500mg elemental *Corrected Ca = (40-actual)x.02 + actual Phosphorous (elemental) 25-50 mg/kg/d up to 500 mg/d Mg Mg oxide 1-2 meq/kg/d PO deficiency: serum Mg <1.4 mg/L 50% solution of MgSO4 0.3-0.5 meq/kg IV over 3 hrs (max 3-6 meqs) Zinc - 1mg/kg/day PO x 2-3 mos elemental Gluconate 7 mg/kg/d Sulfate 5mg/kg/d MEDICATIONS: Midazolam 5mg/ml 0.1 mkdose Nalbuphine 10 mg/ml - 0.1 mkdose Propranolol (10 mg, 40 mg) - 0.6- 8 mkd Somatostatin 3.5 ucg/kg/hr (250 ucg +50 cc or 3mg + 250 cc D5W or pNSS) Adult 3mg in 500cc D5W x 12 hr Pedia Notes

Octreotide 30 mg/m2/hr Spironolactone (25 mg, 50 mg) 3-5 mkd furosemide (20 mg, 40mg) 1-4 mkd Atenolol (25, 50mg) 1.2 mkd MOTILITY DRUGS Domperidone (Motilium) 1mg/ml -0.3 mkdose, 3-4 doses, max 0.6 mkdose. Erythromycin estolate 3mkdose or 20 mgd 2-4 doses Metoclopramide 0.1mkdose up to 4x, max dose 0.5mkd. Adult: 10mg before meals & at bedtime Loperamide 0.5-1.5mkd, 3-4 doses *Prophylaxis for cholangitis Sultamicillin (Unasyn) 10mkd Cotrimoxazole 4mkd Somatostatin dec splanchnic blood flow in normal subjects, in cirrhotic pts not uniformly; used to control acute variceal hemrhge Propranolol beta blocker studied in use for recurrent variceal hmrhge; dec splanchnic blood flow and portal HPN by splanchnic vasoconstriction and cardiac output LIVER FUNCTION LIVER SPAN (Nelson) 12 wk 4.5-5 cm 12 yr (female) 6-6.5 cm 12 yr (male) 7-8 cm After 12 yr (female) 0.27 x wt (lbs)+ 0.22 x ht (in.) -10.75 (male) .032 x wt (lbs)+0.18 x ht (in.) -7.86 PTT measures generation of thrombin through Intrinsic pathway (uses all coag factors including factor IX vit K dependent, andVIII, EXCEPT for factor VII) PT measures time for prothrombin (factor II) to be converted into thrombin factors 1,2,5,7,10 - prolongation of >2sec is pathologic - >3 sec indicate risk for bleeding - evaluates extrinsic pathway - prolonged when facter 1,2,5,7,10 deficient - if prolonged in chronic liver dse suggest poor prognosis NORMAL PT/PTT IN HEALTHY PRETERM PT PTT Day1 13(10.6-16.2) 53(27.5-79.4) 5 12.5(10-15.3) 50.5(26.9-74) 30 11.8(10-13.6) 44.7(26.9-62.5) 90 12.3(10-14.6) 37.5(28.3-50.7) 180 12.5(10-15) 37.5(21.7-53.3) Adult 12.4(10.8-13.9) 33.5(26.6-40.3) Factor VIII- non-hepatic - only factor not made in liver - can be used to differentiate liver dse fr DIC (may be N or inc in liver dse) Vit K deficiencies Give Vit K 1mg/kg IM/IV, min: 1mg in FT Measure PT 4-6 hrs after ROLE OF LIVER IN COAGULATION produce coag factors except von willebrand produce & brkdown factors integral to fibrinolysis eg plasminogen & plasminogen activator clears activated clotting factors fr circ Albumin principal serum protein - synthesized only in rough endoplasmic reticulum of hepatocytes at 150 mg/k/d - half life: 20 d - maintains colloid osmotic pressure - bind/carrier of bilirubin, Ca, other drugs - in pts w/ ascites: may be dec due to inc in the distribution vol rather than dec synthesis - often sign of chronic rather than acute liver dse (since long half life) Other nonhepatic causes of low albumin poor nutrition, nephrotic (urine loss), protein losing enteropathies (fr gut), inc degradation rate (poorly understood) Page 13 /epcapul

12-18 SERUM ALBUMIN LEVELS g/dL 1-3mos 3.4 4-6mos 3.46 7-12 mo 3.62 13-24 mo 3.63 25-36mo 4.11 3-8yr 4 9-16 yr 4.25 +1 SD 0.72 0.36 0.6 0.8 0.78 0.65 0.7 M F 18-49 M F 14.5 14 15.5 14 13 12 13.5 12

serum albumin and PT are most impt parameters need liver transplant HEPATOPULMONARY SYNDROME 1. Hypoxemia 2. Intrapulmonic right to left shunting of blood 3. Liver disease Patient with chronic liver disease with history of shortness of breath or exercise inteolerance and clinical examination findings of cyanosis (particularly of the lips & fingers), digital clubbing, and O2 sats <96%, particularly in the upright position Tx: Liver transplantation ENDOSCOPIC ESOPHAGEAL VARICEAL LIGATION - mucosal and submucosal tissue are ensnared strangulation sloughing fibrosis obliteration of sub/mucosal vascular channels - < complication then sclero eg esophageal stricture, pneumonia, bact.peritonitis - fever treatment sessions IRON - absorption in prox small intestine - ferrous>ferric absorbed - Increases absorption: Gastric acid, some sugars, aa, Bile - Decreased absorption: Oxalate, phosphates - Stimulate inc absorption: 1. iron def, 2. hypoxia, 3. erythropoiesis HEMORRHOIDS Daflon micronized purified flavonoid fraction chronic conditions & venous insufficiency: 2 tabs/day acute hemorrhoidal attacks: 3tabs BID x 4 d, 2 tabs BID x 3 days Antibiotics in Gut Obstruction (rationale) Blood flow to the obstructed bowl decreases as the bowel dilates Blood flow is shifted away from the mucosa with loss of mucosal integrity Bacteria proliferates in the stagnant bowel with a predominance of coliforms and anaerobes Rapid proliferation of bacteria coupled with loss of mucosal integrity allows bacterial translocation across the bowel wall potentially resulting in endotoxinemia, bacteremia and sepsis Bowel gas Air is usually demonstrable radiographically in the stomach of a normal infant immediately after birth Within 1 hour, air may reach the proximal portion of the small intestine and segments of the colon Air may become visible in the distal parts of the colon as early as the 3rd hour or as late as 18 hours

MCV measures the average volume of a red blood cell categorizes red blood cells by size. Formula (2-10 yrs old) Lower limit: 70 fL + age in years Upper limit: 84 fL + ( age in yrs x 0.6 ), until upper limit of 96 is reached Whats the MCV range? Give LL and UL of a 7 years old. Answer: LL: 77 fL; UL: 88.2 fL RETICULOCYTE COUNT Measures erythrocyte production Expressed as % of circulating rbcs Take up reticulin stain (supravital): bec of inc RNA N = 0.5 % to 1.5 % or = .005 to .015 Reticulocyte index Anemic patient --> increased retic so have to correct: retic observed x px Hct / 0.45 Example: Hb 50 Hct 0.15 Retic count=.045= 4.5 % Corrected retic = 4.5% x .15/.45 = 1.5 % ( N = 0.5-1.5%) Absolute Retic Count More accurate Compute as ff: RBC (in n x 1012 ) x # retic/1000 rbc x 1000 Normal = 40,000 100,000/uL Example: Compute for absolute retic count : Hb 90 RBC 3 x 1012 /L Retic .015 Answer: 45,000 retics / uL IRON DEFICIENCY ANEMIA - microcytic, hypochromic, increased RDW Therapy: daily total dose of 4-6mg/kg of elemental iron in 3 divided doses Response to therapy Time after Iron administration Response 12-24hr Replacement of intracellular iron enzyme; subjective improvement, decreased irritability, increased appetite 24-48 hrs Initial bone marrow response; erythroid heperplasia 48-72 hrs Reticulocytosis, peaking at 5-7 days 4-30 days Increase in hemoglobin levels 1-3 months Repletion of stores

HEMATOLOGY and ONCOLOGY

ANEMIA Measured Hgb > 2 SD below the mean for age Age Mean 1 mo 14 2 mo 3-6 mo .5-2 y 2-6 y 6-12 y Pedia Notes 11.5 11.5 12 12.5 13.5 APLASTIC ANEMIA Severe ANC 500-1000 Very Severe ANC 200-500 BLEEDING Get Urinalysis with RBC, if RBC<5, may give Tranexamic Acid PO 25mg/kg max 1500/day; IV 15mg/kg max 500/day.

-2SD 10 9 9.5 10.5 11.5 11.5

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NEUTROPENIA Neutropenia- decrease in the absolute neutrophil count (ANC) ANC= WBC x (neutrophils and bands) Neutropenia < 1000/mm3 infants between 2 weeks and 1 year < 1500/mm3 beyond 1 year of age Severe Neutropenia: ANC less than 500/mm3 Moderate Neutropenia: ANC 500-1000/mm3 Mild Neutropenia:ANC 1000-15000/mm3 Transient- < 8weeks Chronic->8 weeks Clinical Features high fever, chills, severe prostration, and irritability extensive necrotic and ulcerative lesions: oropharyngeal and nasal tissues , skin, gastrointestinal tract , vagina and uterus Gram-negative septicemia ANC <1000/m3: stomatitis, gingivitis and cellulites ANC < 500/M3: perirectal abscess, pneumonia and sepsis Granulocyte colony-stimulating factor (G-CSF) -produces sustained neutrophil recovery in patients with severe chronic neutropenia -reduces the incidence and severity of infection and improves the quality of life -dose: 5ug/kg/day -response in 7 to 10 days HYPERLEUKOCYTOSIS - a total white cell count greater than 100,000/mm3 - 9-13% Acute Lymphocyte Leukemia and 5-22% Acute myeloid LEUKEMIA - occurs in almost all children with chronic myeloid leukemia - leads to increased blood viscosity and emboli - Hemorrhage and leukostasis leading to intracranial hemorrhage or thrombosis, pulmonary hemorrhage and leukostasis are more prevalent in AML than ALL - myeloblasts are larger than lymphoblasts and are more easily trapped in the microcirculation - Tumor lysis syndrome and metabolic abnormalities occur almost exclusively in ALL - lymphoblasts are more sensitive than myeloblasts to chemotherapy Clinical Features: CNS- blurred vision, confusion, delirium, and papilledema, CT scan hemorrhage or leukemic plaques Pulmonary- tachypnea, dypnea, hypoxia, CXR pneumonitis or leukemic emboli Genitourinary- oliguria, anuria, priapism TUMOR LYSIS SYNDROME -results from extremely rapid proliferation, accompanied by significant cell death and release of intracellular release of ions. >Hyperuricemia >Hyperkalemia >Hyperphosphatemia >Hypocalcemia >Hypercalcemia >Renal failure Hydration -Should be given at the rate of 3000mL/m2/day to maintain urine output of >100mL/m2/hr or >5mL/kg/hr Alkalinization of urine -Increase solubility of urates -maintain urine pH 6.5 to 7.5 -maintain urine specific gravity <1.010, monitor Q12 Uric acid reduction 300mg/m2/day TID or 200mg/m2/day IV Preparation: 100mg/tab, 300mg/tab Monitor electrolytes -monitor serum Na, K, Cl, Ca, uric acid , phosphorus, BUN, Crea every 6 hours Hyperphosphatemia -Aluminum hydroxide 150mg/kg/day every 4-6hours -Preparation: Alutab 600mg/tab

INFECTIOUS DISEASES
SIRS (Systemic Inflammatory Response Syndrome) The presence of at least 2 of the following 4 criteria, 1 of which must be abnormal temperature or leukocyte count; -core temperature of >38.5C or <36C -Tachycardia, defined as a mean heart rate >2 SD above normal for age in the absence of external stimulus, long-term drug or painful stimulus, or otherwise unexplained persistent elevations over 0.5-4 hours period OR for children <1 year old: bradycardia, defined as a mean heart rate <10th percentile for age in the absence of external vagal stimulus, betablockers, or congenital heart disease; or otherwise unexplained persistent depression over 0.5hr period - Mean respiratory rate >2 SD above normal for age or mechanical ventilation for an acute process not related to underlying neuromuscular disease or the receipt of general anesthesia - Leukocyte count elevated or depressed for age ( not secondary to chemotherapy induced leukopenia) or 10% immature neutrophils INFECTION Suspected or proven (by positive culture; tissue stain or PCR test) caused bu any pathogen OR a clinical syndrome associated with a high probability of infection. Evidence of infection include positive findings on clinical exam, imaging or laboratory tests (e.g. white blood cells in a normally sterile body fluid, perforated viscus, chest radiograph consistent with pneumonia, petechial, purpuric rash or purpura fulminans SEPSIS - SIRS in the presence of suspected or proven infection SEVERE SEPSIS - Sepsis plus 1 of the following: cardiovascular organ dysfunction OR 2 or more other organ dysfunctions TETANUS Etiology: Clostridium tetani Clinical Criteria for diagnosis of Tetanus 1. An illness characterized by the acute onset of hypertonia andor painful muscle contractions (usually of the jaw and neck) and generalized muscle spasms; 2. No history of contact with strychnine 3. Subsequent disease course consistent with tetanus Wound classification for tetanus prophylaxis Clinical features Tetanus prone Nontetanus prone Age of wound >6 hours 6 hours Configuration Stellate, avulsion Linear Depth >1cm 1 cm Mechanism of injury Missile, crush, burn, Sharp surface (glass, frostbite knife) Dentalized Present Absent conataminants (dirt) Present Absent Immunization Schedule History of Non-tetanus prone wound Tetanus prone wound (all tetanus (clean minor wound) other wounds) immunization Td1 TIG Td TIG Unknown or Yes No Yes Yes < 3 doses 3 or more No2 No No3 No doses Td Tetanus and diphtheria toxoid absorbed (adult) TIG Tetanus immune globulin 1 Yes if wound >24 hours old For children <7 years, DPT (DT if pertussis vaccine contraindicated) For persons >7 years Td preferred to tetanus toxoid alone 2 Yes if >10 years since last booster 3 Yes if > 5 years since last booster Neonatal tetanus suggested system of scoring to assess prognosis at time of admission and subsequently The severity of the disease is inversely proportionate to the score: 0 recovery improbable; 15 recovery Reassessment of score should be done 24 hourly An unchanged or lower score at subsequent assessment signified ineffective management or complications and calls for modification of treatment Page 15 /epcapul

Pedia Notes

Score 0 1 2 3 Age of onset 1-4 5-8 9-12 >12 of sx in days (incubation period) Interval <24 24-48 >48 No between first spontaneous symptom spasms and fisrt spasm in hours (onset interval) Spasms: Persistent >2 <2 Transient or duration in prolonged on minutes stimulation Temperature >3 >2-<3 >1-<2 Normal 1 C variation from normal Pneumonia Definite Definite Suspected Nil and/or widespread limited milk atelectasis Abletts Criteria for Classification of Severity of Tetanus Grade I Mild or no respiratory involvement and dysphagia Grade II Moderate respiratory involvement and trismus Grade IIIA Severe respiratory involvement, generalized rigidity and major spasms with no autonomic involvement Grade IIIB Severe manifestations as above with autonomic dysfunction Treatment of Tetanus 1. Immunization Passive immunization (TIG) preferably 3,000-6,000 u IM although experts claim 500 u is just as effective Alternate drug:Tetanus antitoxin 500u/kg body weight or 5,000 u newborn, 10,000 u children, 20,000 u adults; intravenously and the next intramuscularly Active immunization Tetanus toxoid. First dose admission; second dose discharge; third dose 6 months later 2. Antibiotics Metronidazole 30mkd Q6 X 10-14 days oral or iv Pen G: Neonate 100,000 u/kg/day Q8 Children 200,000 u/kg/day 4-6 doses Adults 1Mu IV Q6 X 15 days 3. Control of muscular spasms Prognosis: Serious case fatality rate: 44-55%; Neonatal tetanus 60%

Proper location of compression (thumb or two-finger techniques): between the xyphoid and line drawn between the nipples or lower third of the sternum Recommended dosage for Neonates Epinephrine: 0.1 to 0.3mL/kg of 1:10,000 (equal to 0.01 to 0.03 mg/kg) Sodium bicarbonate: 2mcg/kg (4mL/kg of 2.4% solution (slowly, no faster than a rate of 1 meq/kg/min) Naloxone hydrochloride: concentration 1mg/mL solution. Dose 0.1mg/kg Magnesium sulfate drip Loading dose: 200mg/kg/dose Maintainance dose: 30-50mg/kg/hr MgSO4 250mg/mL Example: Wt 3.1kg, prepare for 8 hours Order: Start MgSO4 drip as follows: 1. Loading dose of 620mg or 2.5mL + D5W to make 5cc to run for 30 min immediately followed by 2. Maintenance dose of 745mg or 3mL + D5W to make 8 cc to run at 1cc/hr NaHCO3: 75-154meqs.L D10NaHCO3K2 D10W 293cc NaHCO3 54cc KCl 3cc 350cc @ 14.5cc/hr TFI: 112; Wt 3.1 FiO2 CA Computations for O2 hood or CPC setup Formula for Hood: Compressed air = 100 desired FiO2 X 10 79 Oxygen = 10 Compressed air Formula for CPAP Compressed air = 100 desired FiO2 X desired PEEP 79 Oxygen = Desired PEEP Compressed Air Umbilical Artery Catheterization High thoracic vertebrae 6 and 9 Low should be below the 3rd lumbar vertebra Low UA: BW + 7; High UA: 3 x BW + 9 Umbilical Vein Catheterization Catherter tip should be at the junction of the inferior vena cava and right atrium projecting just above the diaphragm UV: High UA/2 + 1 Corrected age: actual in wks + 40-AOG JAUNDICE Physiologic Jaundice: >24hrs, <14d, B2 < 1, <0.5mg/dl/hr inc Exchange level: wt x 10 Photo level: 75% of exchange Apt Test Mix equal parts of the bloody material with master and centrifuge it. Add 1 part of 0.25 mol sodium hydroxide to 5 parts of the pink supernatant. If the fluid remains pink: fetal in origin, hemoglobin F. Turn from pink to yellow brown: maternal blood is hydrolyzed, hemoglobin A. Breastfeeding Jaundice - exaggeration of physiologic jaundice of the newborn as a result of inadequate breastmilk intake or insufficient breastfeeding frequency starvation jaundice - free fatty acids inhibition of glucuronyl transferase activity unconjugated bilirubin Management: increase breastfeeding frequency; breastfeeding should not be discontinued Breastmilk Jaundice - results from the presence of a yet unidentified factor which further increases absorption of unconjugated bilirubin in newborn Management: discontinue breastfeeding for 24-48 hours Jaundice disappears: breastmilk induced Jaundice persists: pathologic jaundice further diagnosis

NEONATOLOGY
NEONATAL RESUSCITATION PROGRAM Tube size (mm) inside Weight (kg) Gestational age diameter (weeks) 2.5 <1,000 <28 3.0 1,000-2,000 28-34 3.5 2,000-3,000 34-38 3.5-4.0 > 3,000 >38 Laryngoscope: Size 0 preterm, Size 1 term Weight (kg) Depth of insertion (cm from upper lip) <1 6 1 7 2 8 3 9 4 10 Adding 6 to the babys weight in kg will give you rough estimate of depth of ET Positve Pressure Ventilation breath two three: 40 to 60 breaths / minute PPV + Chest compressions one and two and three and breathe: 30 breaths: 90 compressions or 120 events Pedia Notes

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PHOTOTHERAPY Not for treatment of hyperbilirubinemia It only decreases the need for exchange transfusion Criteria to rule out physiologic jaundice Clinical jaundice <24 hours old TSB increases >5 mg/dl/day (85 mmol/L/day) TSB >12 mg/dl in FT, >15mg/dl in PT Jaundice >1week in FT, >2 weeks in PT DB >2 mg/dl or >20% of TSB To establish etiology of hyperbilirubinemia Baseline TB, DB, IB CBC with PC PBS, Coombs test,Reticulocyte count Mothers and babys blood type Skin color is not reliable Policy on Improvised Bilirubin Lights 10 fluorescent bulbs at 20 watts each Distance of 20 inches or 50cm from the patient Duration of use should not be more than 2000 hours Stop photo when: 130.7 (FT); 10.71.2 (PT) Prophylactic phototherapy Extensive bruisingin VLBW Diagnosis of hemolytic disease Reminders: Determine bilirubin levels every 8-12 hours Follow fluid balance carefully. Increase TFI if on phototherapy. Avoid if with liver disease or obstructive jaundice (DB >2mg/dl) because of risk of bronze baby syndrome Anticipate revound of 25% after phototherapy is discontinued Cover eyes & genitals with black cloth to protect from radiation Discontinue if patient becomes hyperthermic Potential Complications Impaired maternal-fetal bonding Retinal damage Diarrhea / ileus Dehydration Hyperthermia Skin rashes Bronze baby syndrome EXCHANGE TRANSFUSION Indications Correction of anemia Removal of sensitized RBCs Reduction of TSB Immune thrombocytopenia Equivocal efficacy: Treatment of sepsis, RDS, DIC Consider for the following conditions: Rh incompatibility ABO incompatibility with eigher bilirubin >20 mg/dl or lesser if clinical condition warrants or evidence of kernicterus at any level Hyperbilirubinemia due to other causes: VLBW infants, BW in kg X 10 exchange necessary Metabolic-toxic conditions: hyperammonemia in UCDs and drug overdose Techniques for exchange transfusion: a. Prepare fresh whole blood (mothers blood type if ABO incompatibility): should be cross-mathced with maternal blood if ABO/Rh incompatibility b. Place a UVC after aspirating gastric contents c. A two-volume exchange (DVET): 80-85% turnover. A onevolume exchange only 60%. d. Allow 1-2 minute per cycle; hour per volume, so 1 hour for DVET e. Pre exchange studies: CBC with PC< bilirubin f. Post exchange studies: CBC with PC, bilirubin, RBS, K, Ca taken 6-12 hours post exchange, blood CS is controversial g. A CVP of 5-8 cm H2O be maintained at all times h. Keep thermoregulated during procedure i. Resume feeds 4 hours after exchange Calculations Total blood volume (TBV) Term or >1kg: 80cc/kg Preterm or <1kg: 100cc/kg Volume for DVET = TBV X 2 Pedia Notes

Volume per aliquot: 5% of TBV cc per aliquot = TBV X 0.05 Number of exchange/cycles = Volume per aliquot / Volume for DVET Duration per cycle: 1-2 minutes Duration for DVET: max of 1 hour Total exchange vol=80 x wt x 2 Vol per exchange=80 x wt x 0.05 # of exchanges=total vol/ vol per exchange Wt increase: 30gm/d (1mo); 20gm/d (3-4mos) Breastfeeding Contraindications 1. Maternal infections a. HIV b. HTLV I, II (Human T Lymphotrophic virus) c. Active tuberculosis d. active herpes simplex 2. Very low birth weight infants (<1500g) 3. Maternal medications contraindicated in lactation a. antithyroid medications b. lithium c. cancer meds d. isoniazid e. recreational drugs f. phemindione 4. Consider alternatives to treatment. Consider timing of doses; temporarily expresses and discard milk if necessary. Residuals / Gastric aspirate A volume of >30% of the total formula given at the last feeding may be abnormal and requires extensive evaluation. A gastric aspirate of >1015mL is considered extensive. NECROTIZING ENTEROCOLITIS Risk Factors 5 Is Ischemia Immaturity Immunologic Infection Intake Stage Systemic Stage I NEC Nonspecific: suspect apnea, decreased HR, lethargy, temperature instability Stage IIA Mild Same NEC

Intestinal Gastric residuals; guiac + stools

Radiographic Nonspecific

Stage IIB Moderate NEC

Mild acidosis, APC Respiratory / Metabolic acidosis, assis vent for apnea, decreased BP, decreased UP, neutropenia, DIC Deteriorating VS and laboratory indices

Stage IIIA Advanced NEC

Prominent abdominal distention tenderness, (-) bowel sounds, gross blood in stools Abdominal wall edema, tenderness palpable mass Spreading edema, erythema, abdominal induration

Ileus, dilated bowel loops, focal areas of pneumatosis intestinalis

Extensive pneumatosis intestinalis Prominent ascites, persistent sentinel loops with no perforation

Stage IIIB

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OXYGENATION INDEX (OI) OI = (MAP X FiO2 X 100) / Postductal PaO2 MAP mean airway pressure OI > 15 signifies severe respiratory compromise 30-35 failure to respond to the existing mode of ventilator support >40 80% risk of death, ECMO Screening ROP 4-6 weeks chronologic age, 31-33 wks PCA 1500, AOG 28 weeks, unstable course Hearing wt>1.5, off vent/meds <1500, furosemide, low APGAR, craniofacial malformation, CNS infection, gentamycin, vancomycin, TORCH Pentoxyfylline Preparation: 300mg/15mL Therapeutic dose 6mg/mL Example 1.2 kg X = 6mg/kg X 1.2kg X 6 = 43.2 or ~ 44 X = 44mg (15mg/300mg) = 2.2 mL Order: Give 3.8 mL pNSS + 2.2 mL Pentoxifylline to make 6mL to run at 1cc/hr X 6 hrs OD for 6 days RESPIRATORY DISTRESS SYNDROME I / HYALINE DISEASE Bonsel Grading (Radiographic) Severity Grade Reticulogram Cardiothymic shadow Mild 1 Mild, hazy Clearly generalized defined 2 Moderate 3 Moderate / generalized Heavier and more confluent White out lung fields Still discernible Hazy, barely discernible Up to lung periphery MEMBRANE

Air Bronchogram Perihilar within CT shadow Just past CT borders Past 2/3 lung

Severe

Cardiac borders no longer visible

NEPHROLOGY
OSMOLALITY Osmolality = 2(Na) + BUN/18 + Glucose/2.8 nv: 220-320 nCVP: 5-10cm ARTERIAL BLOOD GAS Compute for the pH Compute for the expected bicarbonate when it is abnormal Primary Expected Change Disorder HCO3 pCO2 SBE Metabolic <22 (1.5 xHCO3) 5 + (82) acidosis Metabolic >26 (0.7xHCO3) + 5 alkalosis (212) Acute Respi [(pCO2-40) >45 or pH = =0 Acidosis 10] + 24 0.008 x (pCO240) Chronic Respi [(pCO2-40) 3] >45 or pH = 0.4 x (pCO2Acid +24 0.003 x (pCO2- 40) 40) Acute Respi [(40-pCO2) 5] <35 or pH = =0 Alkalosis +24 0.008 x (40pCO2) Chronic Respi [(40-pCO2) <35 or pH = 0.4 x (pCO2Alk 10] +24 0.017 x (40- 40) pCO2) pH <7.35 ACIDOSIS HCO3 <22 Pedia Notes

METABOLIC ACIDOSIS COMPENSATION Respiratory changes in paCO2 If actual paCO2 = expected paCO2 COMPENSATED METAB ACIDOSIS If actual paCO2 < expected paCO2 METAB ACIDOSIS WITH RESP ALKALOSIS If actual paCO2 > expected paCO2 METAB ACIDOSIS WITH RESP ACIDOSIS Metabolic Acidosis pH <7.35 HCO3 <22 Respiratory compensation? Calculate for expected paCO2 Compute anion gap ([Na+] + [K+]) ([Cl-] + [HCO3]) Normal = 16 2-4 mEq/L (if K+ included) = 12 2-4 mEq/L (without K+) Anion Gap = (Na+K) (Cl+HCO3) nv 8-16 (w/o K), 12-20 (w/ K) Ratio = actual AG nAG or 12/ nHCO3 or 24 actual HCO3 Ratio: <0.4 hyperchloremic acidosis; <1 loss of base (combined high and N AG); 1-2 simple high AG; >2 gain of base (concurrent metabolic alkalosis) Metabolic Alkalosis pH >7.45 HCO3 >26 Respiratory compensation? Calculate for expected paCO2 pH <7.35 ACIDOSIS paCO2 >45 RESPIRATORY ACIDOSIS Acute or Chronic? COMPENSATION pH RENAL changes in HCO3 Remember that this TAKES TIME First, Compute for expected pH If actual pH = 0.008 X PCO2 (expected pH in acute resp acidosis) UNCOMPENSATED ACUTE RESPIRATORY ACIDOSIS If actual pH = 0.003 X PCO2 (expected pH in chronic resp acidosis) COMPENSATED CHRONIC RESPIRATORY ACIDOSIS If actual pH >0.003 but <0.008 X pCO2 PARTIAL RENAL COMPENSATION (Partially compensated respiratory acidosis) If actual pH >0.008 X pCO2 Overlapping Metabolic derangement: RESP ACIDOSIS WITH METABOLIC ACIDOSIS OR RESP ACIDOSIS WITH METABOLIC ALKALOSIS If actual pH >0.008 X pCO2 - Overlapping Metab acidosis or alkalosis? So, Compute for expected HCO3 If actual HCO3 < expected HCO3 RESP ACIDOSIS WITH METABOLIC ACIDOSIS If actual HCO3 > expected HCO3 RESP ACIDOSIS WITH METABOLIC ALKALOSIS Respiratory Acidosis pH <7.35, pCO2 > 45 NOTE: pH and paCO2 move in opposite direction Compute for expected pH Acute resp acidosis, uncompensated Compensated chronic respiratory acidosis Partially compensated respiratory acidosis If actual pH > 0.008 X pCO2, then compute for expected HCO3 concomittant metab acidosis or metab alkalosis pH >7.45 ALKALOSIS paCO2 <35 RESPIRATORY ALKALOSIS Page 18 /epcapul

 Acute or Chronic? COMPENSATION RENAL changes in HCO3 Remember that this TAKES TIME First, Compute for expected pH If actual pH = 0.008 X PCO2 (expected pH in acute resp alkalosis) UNCOMPENSATED ACUTE RESPIRATORY ALKALOSIS If actual pH = 0.017 X PCO2 (expected pH in chronic resp alkalosis) COMPENSATED CHRONIC RESPIRATORY ACIDOSIS If actual pH >0.008 but <0.017 X pCO2 PARTIAL RENAL COMPENSATION (Partially compensated respiratory alkalosis) If actual pH >0.017 X pCO2 Overlapping Metabolic derangement: RESP ACIDOSIS WITH METABOLIC ACIDOSIS OR RESP ACIDOSIS WITH METABOLIC ALKALOSIS If actual pH >0.017 X pCO2 - Overlapping Metab acidosis or alkalosis? So, Compute for expected HCO3 If actual HCO3 < expected HCO3 RESP ACIDOSIS WITH METABOLIC ACIDOSIS If actual HCO3 > expected HCO3 RESP ACIDOSIS WITH METABOLIC ALKALOSIS Respiratory Alkalosis pH >7.46, pCO2 <35 NOTE: pH and paCO2 move in opposite direction Compute for expected pH Acute resp alkalosis, uncompensated Compensated chronic respiratory alkalosis Partially compensated respiratory acidosis If actual pH > 0.017 X pCO2, then compute for expected HCO3 concomittant metab acidosis or metab alkalosis Oxygenation Status At room air, sea level: PaO2 80-100 normal or acceptable PaO2 < 80 mild hypoxemia PaO2 < 60 moderate hypoxemia PaO2 < 40 severe hypoxemia On oxygen support: PaO2 80-100 corrected hypoxemia PaO2 > N overcorrected hypoxemia PaO2 < N uncorrected hypoxemia Computing for pHR Normal pCO2 = 40 and normal pH = 7.4 If actual pCO2 > 40: pHR = (40 Actual pCO2) x 0.05 + 7.4 10 If actual pCO2 < 40: pHR = (40 Actual pCO2) x 0.1 + 7.4 10 If pHR compared to actual pH is: < 0.03 purely respiratory > 0.03 compensated

pH < 7.35 part comp

pH 7.35 7.4 pH 7.4 7.45 pH > 7.45 comp metabolic acidosis comp respiratory respiratory alkalosis alkalosis

pCO2 metabolic < 35 acidosis

pCO2 metabolic 35-45 acidosis

normal

normal

metabolic alkalosis part comp metabolic alkalosis

comp

comp metabolic alkalosis

pCO2 respiratory respiratory > 45 acidosis acidosis

Estimated GFR = Ht (cm)x 0.5(children/adol girls) or 0.7 (Adol boys) Serum creatinine mg/dL Estimated GFR (mL/min/1.73m2)=kL/Pcr L (length/height, cm) Pcr- plasma creatinine k- constant k LBW during first year of life 0.33 Term AGA during first year of life 0.45 Children and Adolescent girls 0.55 Adolescent boys 0.70 Creatinine Clearance (mL/min/1.73m2) =Urine cr x Urine vol x 1.73 Plasma cr 1440 BSA Normal Values of GFR Age GFR (mean) mL/min/1.73m2 Neonates <34wk gestational age 2-8 days 11 4-28 days 20 30-90 days 50 Neonates >34wk gestational age 2-8 days 39 4-28 days 47 30-90 days 58 1-6 mo 7 6-12 mo 103 12-19 mo 127 2 yr-adult 127

Range mL/min/1.73m2

11-15 15-28 40-65

17-60 26-68 30-86 39-114 49-157 62-191 89-165

Laboratory Indices for Prerenal vs Intrinsic Acute Renal Failure Index Prerenal Intrinsic Renal Specific gravity >1.020 <1.010 Urine osmolality (mOsm) >500 <350 Urine Sodium(meq/L) <20 >40 FENa (%) <1 >2 Blood urea nitrogen >20 <20 / Creatinine FENa (%)= UNa x PCr x 100 PNa x Ucr RFI (Renal Failure Index) = urine Na/ (urine Cr/plasma Cr) Mean values of serum creatinine during the first weeks of life of term and very low weight neonates Serum creatinine (umol/L) Birthweight Postnatal period(day) (g) 1-2 8-9 15-16 22-23 1001-1500 95 64 49 35 1501-2000 90 58 50 30 2001-2500 83 47 38 30 Term 66 40 30 27

Pedia Notes

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Serum Creatinine in Children Age Serum Creatinine (yr) Umol/L mg/dL <2 35-40 0.4-0.5 2-8 40-60 0.5-0.7 9-18 50-80 0.6-0.9 MAP=1/3SBP+2/3 DBP; dec BP by 20=30% of MAP Age Systolic Diastolic Birth (12h, <1000gm) 35-59 16-36 Birth (12h, 3kg) 50-70 25-45 Neonate (96hrs) 60-90 20-60 Infant (6mos) 87-105 53-66 Toddler (2yrs) 95-105 53-66 School age (7yrs) 97-112 57-71 Adolescent (15yrs) 112-128 66-80 CHON Spillage=TP x 1000/ 24 x BSA; nUprot: 100mg/m2/d BUN/Crea x 247 uAG= (uNa + uK) uCl postive uAG: dec uNH4Cl excretion (dRTA); negative uAG: intact uNH4Cl excretion (diarrhea, non-distal nephron defect or high AG acidosis) SIADH CSW Central DI Serum Na Low Low High Urine output High High N or Urine Na High Very high Low Intravascular Low Low N or volume status Serum uric acid Low High N or Vasopressin High Low Low level Acute Glomerulonephritis Ssx: edema (facial or bipedal), hypertension, hematuria, oliguria Labs: urinalysis with RBC morphology (mild hematuria RBC 1-2 with dysmorphic RBC) C3 ASO CBC BUN, Crea NO ultrasound nonspecidifc Treatment: Furosemide (3) Q6 Continuous Furosemide drip 0.5 mg/kg/hr Rate = WT X 0.5 Preparation: 100mg Furosemide + 100cc D5W to make 1mg/mL Nephrotic Syndrome Ssx: Generalized edema, heavy proteinuria, hypoalbuminemia Diagnostics: Urinalysis Albumin 24 hour urine collection with urine protein and urine creatinine NO ultrasound Protein spillage: Significant proteinuria: 4-40mg/m2/hr Nephrotic range or heavy proteinuria: >40 Total protein spillage Management Diuretics Bumeanide 1mg/tab 1 tab BID to Q6 HCTX 25 or 50mg tab BID Antibiotics Penicillin G if with infection Target Group A beta-hemolytic Streptococcus Steroids Prednisone: Initiation: 60mg/m2/day Max of 60mg/day 20mg tabs 3 tabs max Pedia Notes

Check response in 7-10 dyas (half life of prednisone) May be given for 2-4 weeks; Maximum of 10 weeks If (-)n protein or repeat UA with decreased protein, may shift to maintenance Maintenance phase 40mg/m2 every other day after breakfast to counteract cortisol surge producing less side efects Given for 6-0 months Taper slowly every 2 weeks until with (-) protein Hydrocortisone IV Steroid dependent relapse while on alternate-day steroid therapy or within 28 days of completing a successful course of prednisone therapy Steroid resistant fail to respond to prednisone therapy within 8 weeks Frequent relapser respond well to prednisone therapy but relapse 4 times in 12 month period Urinary Tract Infection Inquire regarding manner of collection of urine sample for urinalysis Wee bag increased sensitivity: if urinalysis is negative then we are sure it is not UTI Midstream catch Clean catch Diagnostics: Ultrasound Dimercaptosuccinic acid scan (DMSA) check renal scarring. If there is no scarring, then it is not reflux. Voiding cystourethreogram (VCUG) Urodynamic studies Medications: Cefuroxime, Co-amoxiclav Duration: if culture (-), treat for 7 days; if culture (+) treat for 14 days Renal Failure Pediatric Modified RIFLE Criteria Criteria Estimated Creatinine Clearance Risk eCCl decrease by 25% Injury eCCl decrease by 50% Failure eCCl decrease by 75% or eCCl <35ml/min/1.73m2 Loss Persistent failure >4wk EndEnd-stage renal disease stage (persistent failure >3mo)

Urine Output <0.5ml/kg/hr for 8 hr <0.5ml/kg/hr for 16 hr <0.3ml/kg/hr for 24 hr or anuric for 12 hr

Renal Support Medications CaCO3 50-100mkd TID (Prep 500mg, 650mg) NaHCO3 1-3 meqs/kg/day (BID-QID) (Prep 325mg/tab, 450mg/tab = 7.7 meqs) FeSO4 3-6 mkd Erythropoietin 500mkdose (prep 2000 u, 4000 u) Cyclphosphamide Prehydration D5 0.3 NaCL : BSA X 3000mL to run in 1 hours Cyclophosphamide (500mg/BSA + 40-60% Mesna dilute in D5W to make 100mL to run in 1 hour Posthydration: FM X 6 hours (D5 0.3NaCl) Peritocat / Stiff Cath Insertion 1. Strict asepsis 2. Instill Lidocaine in 2 fingerbreaths midline below the umbilicus 3. Using IV needle gauge 16 (large bore), insert perpendicular/vertical once give is felt, withdraw needle slowly and continue insertion of IV cannula into peritoneum 4. Induce ascites using Eruopersol 1.5% until boardlike rigidity of abdomen is felt 5. Withdraw IV cannula and insert stiff cathe in a screwing motion into the peritoneum. Once with give withdraw needle and insert eigher to R or L of abdomen (measure depth of peritoneum catheter from umbilicus to symphysis pubis) 6. Once in place, connect extension tube and draw fluid. 7. Stabilize peritoneal catheter by suturing continuously at the 3, 6, 9, 12 oclock position and approximately below pericath marker and tie. 8. Clean with betadine Page 20 /epcapul

NEUROLOGY
GCS Eye opening Spont Speech Pain None Verbal Motor Oriented/Smiles 5 Obeys Confused/ 4 Localizes Consolable Withdraws Words/ 3 Flexion Inconsolable Extension Sounds/ Grunts 2 None None 1 nICP: Infants 5mmHg; Children 6-13; Adults 5-15mmHg upper limit: 20mmHg CPP=MAP-ICP; >50-70mmHg 4 3 2 1 6 5 4 3 2 1

Polymicrogyria Neuronal migration defect Excess of small and poorly developed cerebral gyri Most commonly sporadic, may be associated with Zellweger cerebrohepato-renal syndrome Severe mental retardation, seizures Schizencephaly Unilateral or bilateral gray matter lined cleft of the lateral cerebral wall, extending from the periventricular zone to the meninges Hydranencephaly Congenital absences of cerebral hemispheres which are replaced by large CSF filled cavities Brainstem and basal ganglia are present and there may be rudiments of frontal and occipital cortex Chiari Malformations Chiari I Elongated peglike cerebellar tonsils displaced in the upper cervical canal 20-40% with associated syrinx Chiari II (Arnold-Chiari) Vermis, pons, medulla and an elongated fourth ventricle are displaced inferiorly into the cervical canal Myelomeningocoele in nearly 100% Chiari III Hindbrain herniation into a low occipital or high cervical encephalocoele in combination with features of chiari II Chiari IV Associated with cerebellar hypoplasias and dysplasias Dandy Walker Malformation Large posterior fossa Fourth ventricle floor present, ventricle open dorsally to large posterior fossa cyst Hydrocephalus in 80% Vermian, cerebellar hemispheric hypoplasia Brainstem may be hypoplastic, compressed Porencephaly Defect in the cerebral mantle resulting in a cyst-like expansion of the lateral ventricle Usually unilateral Usually secondary to local damage to the cerebrum, either during late fetal life or early infantile life Status Epilepticus a neurologic emergency wherein the patient develops generalized or partial seizures lasting for 30 minutes or longer, or a series of seizures wherein the patient does not regain consciousness in between seizures Refractory Status Epilepticus status epilepticus of more than 60 minutes, where adequate dosages of benzodiazepines, Phenobarbital and Phenytoin fail to terminate the seizure Intractable Epilepsy at least 1 seizure per month for at least 12 months, refractory to maximal, tolerable doses of at least 2 first line anticonvulsants, with compromised quality of life. Simple Febrile Seizure seizure characterized as generalized (usually tonic-clonic), lasting for less that 15 minutes and which does not recur within the same febrile illness. Complex Febrile Seizure seizure with partial onset, prolonged duration (lasting >10 or >15 minutes, both have been used) and recurrent (more than 1 seizure in a single illness episode, generally in 24=hr period).

Cerebral Dominance Dominant Hemisphere handedness; perception of language and speech, writing Nondominant Hemisphere spatial perception; recognition of faces and music Lentiform nucleus glovus pallidus + putamen Corpus striatum caudate nucleus + lentiform nucleus Neostriatum caudate nucleus + putamen Aphasia Expressive aphasia- Brocas area; destructive lesions in the left inferior frontal gyrus; loss of ability to produce speech Receptive aphasia Wernickes area; destructive lesions restricted to Wernickes speech area; loss of ability to understand the spoken and written word Abnormal Respiratory Patterns Cheyne-Stokes breathing- forebrain damage Central neurogenic hyperventilation- hypothalamic-midbrain damage Apnea; cluster breathing- lower pons Ataxic breathing- medulla Pupillary size and reaction Anisocoria- uncal herniation Small, reactive- metabolic, diencephalic compression Pinpoint- pons Midposition, fixed- midbrain Large, fixed- tectal Posturing Decorticate rigidity- flexor response in the arms with extension of the legs Localization: cerebral hemisphere Decerebrate rigidity- abnormal extensor response in the arms and legs Localization: bilateral diencephalic and hemisphere damage; upper brainstem injury Can be seen in the ff. conditions: Massive head trauma and cerebral hemorrhage Rostro-caudal deterioration Posterior fossa or cerebellar lesions Severe metabolic disorders- hepatic coma Holoprosencephaly Failure to form the paired cerebral hemispheres Lateral ventricles are represented by a single midline cavity Lissencephaly Defect in migration of the cerebral neurons Cortical gyri fail to develop Surface of the cerebral hemispheres is smooth with absence of normal cortical layers microscopically. Severe mental retardation Pachygyria Defect in the migration of the cerebral neurons Few and broad gyri Associated with a four-layer cortex that underlies thickened gyri Pedia Notes

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Myaesthenia Gravis Grade I weakness restricted to extraocular muscles Grade IIa Generalized mild weakness Grade IIb Generalized moderate weakness Grade III Generalized severe weakness Grade IV Life threatening weakness of respiratory muscles Hepatic Encephalopathy I Changes in behavior, minimal change in level of consciousness, altered sleep (hypersomnia, insomnia), inversed sleep cycle in the newborn II Spatiotemporal disorientation, drowsiness, inappropriate behavior, obvious asterixis III Marked confusion, stuporous, repond or not to auditory stimuli, decerebrate posturing to pain, asterixis usually absent IV comatose, unresponsive to pain, decorticate posturing Subacute Sclerosing Panencephalitis (SSPE) Stage IA Behavioral, cognitive and personality change (decreased school performance, attention / hyperactivity, inappropriate socially, sleep disturbance. Walking Stage IB Myoclonic spasm a periodic, focal, independent ambulation. Same mental / behavioral symptoms as IA. Stage IIA Further mental-behavioral deterioration. Myoclonic spasms periodic, generalized and synchronous, frequent. Can walk independently but doesnt because of drop spells. Stage IIB Apraxia, agnosias, language difficulties. Motor signs spasticity, ataxia. Ambulatory with assistance. Stage IIIA Speaking less, visual difficulties, no ADLs. Sits up independently, may stand, no independent ambulation. Myolonic spasms frequent, multifocal, short inter-spasm intervals (3-5seconds); long duration (3-4seconds). May have seizures. Stage IIIB No spontaneous speech, poor verbal comprehension, may be blind. Myoclonic spasms same as in IIIA. Bedridden, dysphagia, may have to be fed by NG tube. No EEG delta background activity. PSWC (periodic slow wave complexes) often obscured in background. Movement disorder may appear (chorea-ballismus-athetosis). Stage IV No myoclonic spasms. EEG very low voltage background activity. No PSWC. Patient in neurovegetative state. Acetazolamide cerebral vasodilator; transiently worsen intracranial hypertension. Contraindicated in closed head injury

PULMONOLOGY
RR Lights Criteria (exudates) <2mos <60 PF LDH (>2/3 or 200) 2mos-1yr <50 PF Prot (>3g/dl) 1-5yrs <40 PF:Serum LDH (>0.6) 6-8yrs <30 PF:Serum Prot (>0.5) Expected PEFR Male=(ht in cm-100)x5+175 Female=(ht in cm-100)x5+170 PEFR var= actual/ exp x 100 FiO2 Estimates Nasal cannula = flow rate (lpm) x 4 +21 Funnel = 6lpm 80FiO2 Face mask = flow rate -1 x 10 Hood = 8lpm60FiO2; 10lpm70FiO2 PiO2=FiO2 in decimal x (760mmHg 47mmHg) PAO2=PiO2 (PACO2/0.8) A-a gradient=PAO2-PaO2 nv: 20-65mmHg on 100%O2; 5-20 on RA; >65 respiratory compromise MAP=PIP-PEEP x IT x RR/60 + PEEP; nv <8 OI= MAP x FiO2 x 100/ PaO2 OI>35 for 5-6hrs 1 criterion for ECMO O2 content (CAO2) in ml/dL= Hgb in g/dL x 1.34 x O2 Sat in decimal + PO2 x 0.003; nv 18-20 AV difference (AVDO2) = CAO2 CVO2; nv 5mL/ 100dL O2 extraction = CAO2 CVO2/ CAO2; nv 0.25 Shunt fraction = pAO2 CAO2/ pAO2 CVO2; nv <5% Pedia Notes

ET size: age/4+4; <1kg 2.5, 1-2kg 3, 2-3kg 3.5, 3-4kg 4 ET lt: size x 3 or wt + 6 or age/2+12 Drugs for RSI Adjunctive Sedative Paralytic Head injury/ Inc Lidocaine Thiopental/ Vecuronium ICP/ Status Ep Propofol/ + Normal BP Etomidate/ Midazolam Head injury/ Inc Lidocaine Etomidate/ low Vecuronium ICP/ Status Ep dose Thiopental/ + Hypotension Midazolam Normotensive Midaz/Etom/ Vecuronium Euvolemic Prop/Thiopental MILD Shock Atropine Ketamine/ low Vecuronium Hypotensive dose Midaz/ Hypovolemic Etomidate SEVERE Atropine Etom/ Ket/ None Vecuronium Shock Status Atropine Ketamine/ Midaz Vecuronium Asthmaticus MECHANICAL VENTILATION Initial Ventilator Settings Volume Control Ventilator o Tidal volume - around 10 cc/kg; Assess for chest rise & oxygenation o PEEP - depends on lung status; start around 5 cm H2O; Assess for oxygenation & hemodynamic parameters o FiO2 maintain adequate oxygenation o RR normal for age Pressure Limited Time Cycled Ventilator o PIP assess chest rise and oxygenation o PEEP assess oxygenation and hemodynamics o It maintain normal for age o FiO2 maintain adequate oxygenation o RR normal for age Revision of Ventilator Settings Oxygenation - Assessed via pO2 pO2 mean airway pressure Mean airway pressure PIP x It + PEEP x Et It + Et Area under pressure-time curve Increase pO2 Decrease pO2 Increase PIP or tidal volume Decrease PIP or tidal volume Increase PEEP Decrease PEEP Increase It Decrease It Increase FiO2 Decrease FiO2 Ventilation - Assessed via pCO2 pCO2 1/minute ventilation Minute ventilation = Frequency x tidal volume Volume control ventilator: Tidal volume is set Pressure control ventilator: Tidal volume is PIP PEEP Decrease pCO2 Increase pCO2 Decrease frequency Increase frequency Decrease tidal volume Increase tidal volume Decrease PIP Increase PIP Increase PEEP Decrease PEEP CONGENITAL CYSTIC ADENOMATOID MALFORMATION (CCAM) Type I Macrocystic; single or several large (>2cm), ciliated pseudostratified epithelium; good survival Type II Micrycystic; multiple small cysts, associated with other congenital anomalies; poor prognosis Type III Solid with bronchiole-like structures lined with cuboidal ciliated epithelium ACUTE RESPIRATORY DISTRESS SYNDROME American European Consensus Conference Acute onset Bilateral infiltrates on chest radiograph PCWP < 18 mmHg or absence of clinical evidence of left atrial hypertension PaO2:FiO2 < 200 (ALI if PaO2:FiO2 < 300) Page 22 /epcapul

Phases of ARDS Exudative Phase Histologic Diffuse alveolar features damage Neutrophils, macrophages and erythrocytes Hyaline membranes Protein-rich edema fluid in alveolar spaces Capillary injury Disruption of alveolar epithelium Clinical 3-7 days from features onset Accounts for 33% to 50% of deaths Arterial hypoxemia refractory to supplemental oxygen

RHEUMATOLOGY
FibrosingAlveolitis Phase Fibrosis Acute and chronic inflammatory cells Partial resolution of pulmonary edema Resolution Phase Degree of histologic resolution of fibrosis not well characterized SYSTEMIC LUPUS ERYTHEMATOSUS 1997 Revised Classification Criteria CRITERION DEFINITION Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions Photosensitivity Rash as a result of unusual reaction to sunlight (elicited by patient history or physician observation) Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physician Arthritis Non-erosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion Serositis Pleuritis:convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion OR Pericarditis:documented by ECG or rub or evidence of pericardial effusion Renal disorder Persistent proteinuria >0.5 g/day or >3-plus (+ + +) if quantitation not performed OR Cellular casts: may be red blood cell, hemoglobin, granular, tubular, or mixed Neurologic Seizures:in the absence of offending drugs or known disorder metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance) OR Psychosis:in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance) Hematologic Hemolytic anemia, with reticulocytosis disorder OR Leukopenia: <4,000/mm3 total on two or more occasions OR Lymphopenia: <1,500/mm3 on two or more occasions OR Thrombocytopenia: <100,000/mm Immunologic Anti-DNA antibody to native DNA in abnormal titer disorder OR Anti-Smith:presence of antibody to Smith nuclear antigen OR Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of lgG or lgM anticardiolipin antibodies;(2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 mo and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test (FTA-ABS), Standard methods should be used in testing for the presence of antiphospholipid. Antinuclear An abnormal titer of antinuclear antibody by antibody immunofluorescence or an equivalent assay at any time and in the absence of drugs known to be associated with drug-induced lupus syndrome
(From Hochberg MC: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725. Reprinted with permission of Wiley-Liss, Inc., a subsidiary of John Wiley & Sons, Inc.)

Radiologic features

CT findings

Bilateral infiltrates Maybe patchy or asymmetric May include pleural effusions Alveolar filling Consolidation Atelectasis Predominantly in dependent lung zones

Observed histologically as early as 5-7 days of onset Persistent hypoxemia Increased alveolar dead space Further decrease in pulmonary compliance Pulmonary hypertension Linear opacities consistent with evolving fibrosis Pneumothorax

Gradual resolution of hypoxemia and improved lung compliance

Radiographic abnormalities resolve completely

Diffuse interstitial opacities Bullae

Etiology Direct Lung Injury o Pneumonia o Aspiration of gastric contents o Pulmonary contusion o Fat emboli o Near-drowning o Inhalational injury o Reperfusion pulmonary edema Indirect Lung Injury o Sepsis o Severe trauma with shock and multiple transfusions o Cardiopulmonary bypass o Drug overdose o Acute pancreatitis o Transfusions with blood products (TRALI) General Measures Careful search for underlying cause Prevention or early treatment of nosocomial infection Adequate nutrition preferably enteral Prevention of GI bleeding Prevention of thromboembolism Specific Measures o To decrease ventilator-induced lung injury: Mechanical ventilation o To address surfactant deficiency and dysfunction: Surfactant therapy o To improve V/Q mismatch: Prone positioning o Inhaled nitric oxide and other vasodilators o To decrease pulmonary edema: Fluid and hemodynamic management; b-agonist (?) o To decrease inflammation: Glucocorticoids and other antiinflammatory agents Pedia Notes

Criteria for the Classification of Juvenile Rheumatoid Arthritis Age at onset: <16 yr Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in 1 joints Duration of disease: 6 wk Onset type defined by type of articular involvement in the 1st 6 mo after onset: Polyarthritis: 5 inflamed joints Oligoarthritis: 4 inflamed joints Systemic disease: arthritis with a characteristic intermittent fever Exclusion of other forms of juvenile arthritis
Modified from Cassidy JT, Levison JE, Bass JC, et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum 1986;29;174181.

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TOXICOLOGY
Yellow phosphorus most toxic ingredient in fire crackers like Watusi; classic syndrome of hepatotoxicity Mechanism of toxicity Liver steatosis, necrosis Renal tubules & myocardium are not spared. Vascular collapse and hepatorenal failure. Calcium is excreted as a result of phosphorus absorption explaining cardiac abnormalities. Toxicokinetics peak plasma level 2-3 hours. Fatal dose 1mg/kg, minimal toxic dose 0.3 mg.kg Manifestation of toxicity First stage 8-24 hours Nausea, vomiting, abdominal pain, diarrhea Hematemesis Extreme tirst Shock, seizure, coma Strong odor or garlic on breath, vomitus, & feces (smoking stool syndrome) Second stage 1-3 days, symptom-free, latent stage Third stage - Hepatic failure, jaundice, renal insufficiency, restlessness, delirium, toxic psychosis, coma; Mortality > 50% Laboratory: CBC, BT, LFT, Urinalysis, BUN, Crea, serum e, ABG, FOBT Therapeutics Calcium gluconate 10% Dextrose 50% NAD Phytomenadione Vitamin C

Milrinone 50mcg/k bolus x 15mins; 0.5-1mcg/k/min infusion Morphine 0.1-0.2mkdose q2-4 (max 15mg/dose) Nicardipine 1-3mcg/k/min infusion Nifedipine 0.25-0.5mkdose q4-6 (max 10mkdose or 3mkd) Nitroglycerin 1-5mcg/k/min (max 20mcg/kg/min) Omeprazole 0.6-0.7mkdose OD-BID Phenobarbital 20mkLD, 5mkdose q30min (max 30mkLD) Phenytoin 20mkLD; MD: 5mkd/q12-q8 Prednisone 2mkd/OD-BID (max 80mg); taper if >5-7days Procainamide for VTach 15mg/kg (do not give w/ amiodarone) Propofol 2mg/kg Propranolol for Tet 0.15-0.25mkdose SIV; may rpt in15mins Prostaglandin E1 LD 0.05-0.1mcg/k/min; MD 0.005-0.04mcg/k/min Sodium bicarbonate 0.3 x wt x base deficit; max concentration for infusion 0.5meqs/mL; max rate 1meq/k/hr Spironolactone 1-3mkd/OD-QID Terbutaline 2-10mcg/k LD; 0.1-0.4mcg/k/min infusion Thiamine for Wernickes enceph 100mg IV x 1 then OD Thiopental 2-4mg/kg Tramadol 1-2mkdose q4 (max 500mg/dose) Tranexamic acid 25mkdose TID Vecuronium 0.1mkdose q1 or 0.05-0.07 mg/kg/hr infusion Vancomycin Example 3kg Vancomycin (15mkdose or 60mkd) 500mg/vial + 10mL sterile water to make 50mg/mL stock solution, give 45mg or 0.9mL (50mg/mL stock solution) + 9mL NSS to make 5mg/mL solution. Infuse over 1 hour Q6. Monitor for increased/decreased BP, tachycardia. If these appear, stop infusion and give Diphenhydramine 1mg/kg/dose IV.

MEDICATIONS
Amphotericin B Amphotericin B (1mkd) 50mg/vial + 10mL sterile water to make a 5m/mL stock solution. Give 3mg or 0.6mL (5mg/mL stock solution) + 30mL D5W to make a 0.1 mg/mL solution. Infuse over 6 hours OD. Adenosine for SVT 0.1mg/kg (max 1st dose 6mg, 2nd 12mg) Albumin 0.5-1gm/k/dose x 30-120mins (max 6gm/k/day) Aminophylline 6mkLD x 20mins; MD 1-2mkdose q6-8 Amiodarone 5mkdose x 20-60min (VT), bolus (VF/Pulseless VT) Atropine 0.01-0.02mg/k (min0.1; max0.5mg); may rpt once Bumetanide 0.015mg-0.1mkdose (max: 10mg/day) Calcium gluc 100mkdose x 1hr (max 3gm); 200-500mkd/q6 Chloral hydrate 25-100mkdose Dexamethasone 1-2mkLD, MD 1-1.5mkd/q4-q6 (max 16mg/day) for cerebral edema; 0.5-2mkd/q6 for airway edema Dobutamine 2.5-20mcg/kg/min; rate= wt x dose/16.6 Dopamine 2-20mcg/kg/min; rate= wt x dose/13.3 Epinephrine 0.01ml/k SC (allergy/asthma); drip 0.1-1mcg/k/min; racemic 0.5ml/kg in 3mlNSS (max 2.5ml<4yo; 5ml>4yo) Etomidate -.2-0.4mg/kg Fentanyl 1-2mcg/kg (for BP&head injury) Furosemide 0.5-2mkdose (max: 6mkdose) Granisetron 10-20mcg/k/dose Hydralazine 0.1-0.2mkdose q4-6 (max 20mg/dose) Hydrocortisone 4-8mk LD (max 250mg); MD 8mkd/q6 (asthma), 15mkd/q12-OD (allergy) Ipratropium bromide 0.25-0.5mg/dose TID-QID Ketamine 1-4mg/kg Ketorolac 0.5mkdose IV q6 (max 30mg/dose) Labetalol 0.3-3mg/kg/hr infusion Lidocaine for wide complex tach 1-2mg/kg Mannitol 0.5gm/k or 2.5cc/k; 1gm/k or 5cc/k MgSO4 25-75mkdose x 20mins q4-6 (max 2gm) Midazolam 0.05-0.1mkdose; 1-5mcg/k/min Pedia Notes

REFERENCES: Bambo Notes Nelson Textbook of Pediatrics Pedia Lectures Cardio 2010 Lectures PICU Lectures /Pedia Notes EPCapul 5.0 phil4:13 Page 24 /epcapul