Study Guide NU 647 Hematologic & Immunologic Dysfunction Chapter 49 Anemia -symptoms: fatigue, altered CBC, pale, head

ache, tachycardia, low blood pressure; murmur (due to increase in turbulence w/in heart; decreased peripheral resistance); cyanosis typically not present; Hgb below 10 or 11; iron deficiency- children drink lots of milk; -management: reverse anemia by treating underlying cause; transfusion; nutritional supplements; IV fluids, oxygen, bed rest Sickle Cell Anemia (Hgb replaced by Hgb S; primarily A.A.) -symptoms: rigid sickle shaped cells obstruct blood flow/vasoclusion/hypoxia; -sickle cell crisis- O2 demand (trauma, fever, infection, stress) leads to ischemia/infarction and splenic sequestration (blood pooling in spleen --> hypovolemia/shock); acute chest- presents like pneumonia; chest pain/wheezing; CVA (infarction) is sudden -management: hydration, oxygen (short term), pain management, rest, warm packs, electrolyte replacement; med: Hydroxyurea Thalassemia (inherited blood disorder of Hgb synthesis) -symptoms: severe anemia (<6), hepatosplenomegaly, FTT, pallor, headache, irritability, anorexia, epistaxis, bone pain, exercise intolerance; usually detected by 6 weeks old - Thalassemia- 4 types: Thalassemia minor: asymptomatic silent carrier Thalassemia trait: mild microcytic anemia Thalassemia intermediate: moderate-to-severe anemia plus splenomegaly Thalassemia major: Cooleys anemia; severe anemia requiring transfusions to survive -management: transfusions (side effect- too much iron- need chelation); spleneoctomy (d/t splenomegaly/ decreases abdominal pressure and RBC destruction)- increases risk of infection; still have Hgb deficiency Aplastic Anemia (pancytopenia- RBC, WBC, platelets; congenital or acquired) -symptoms: severe anemia and thrombocytopenia -management: immunosuppressive therapy, bone marrow transplant Idiopathic Thrombocytopenic Pupura (acquired; thrombocytopenia) -symptoms: pupura, petechiae, nosebleeds, bleeding from mouth/gums, blood in vomit, urine, or stool; -acute: children ages 2-6, follows viral infection (sudden onset, resolves in weeks to months) -chronic: adults, more girls than boys, sx last months to lifetime -management: steroids, IV gamma globulin, Anti-D antibody, no contact sports, no Motrin Hemophilia A- CF VIII (classic) Hemophilia B- CF IX (Christmas disease) Von Willebrands Disease (bleeding disorder- males and females) -bleeding/ecchymosis -ice, elevation, compression -DDAVPs Leukemia (overproduction of immature WBCs) -symptoms: anemia, infections, bleeding; bone and joint pain; spleen, liver, lymph gland infiltration/fibrosis

-ALL (75-80% of leukemias in children; too many lymphocytes; chromosomal abnormalities usually present) -AML (too many granulocytes; associated w/genetic disorders) -management: Chemo, vesicants, radiation e Bone Marrow Transplant- different types/management Hodgkins vs Non-Hodgkins H- typical progression (15-19 yo); spleen, liver, bone marrow, lungs; Reed-Sternberg cells; manifests as firm, non-tender, swollen lymph nodes; weight loss, fever, night sweats; Tx is radiation and chemo; good prognosis NH- random progression (<14 yo); 60% of pediatric lymphomas, diffuse involvement Blood Transfusions -indications -management: infuse over no more than 4 hours, use w/in 30 minutes of delivery, stay with pt for first 15 minutes; reactions: hemolytic, febrile, allergic, hypothermia, air embolism Genitourinary Dysfunctions Chapter 50 UTI -symptoms: incontinence in toilet trained child, strong/foul smelling urine, frequency/urgency, painful urination, thirst, blood in urine, poor appetite -management: antibiotics, increase fluid intake, ID contributing factors, preserve renal function, prevent systemic infection; assess for vesicoureteral reflux (by VCUG) Nephrotic Syndrome -children 2-7 yo, proteinuria, hypoalbuminemia, edema, high cholesterol -symptoms: fatigue/malaise, appetite, weight gain/edema/facial swelling/abdominal swelling, foamy urine, pale fingernail beds, dull hair, food intolerances/allergies -management: diet (low pro + Na), steroids, immunosuppressive therapy, diuretics, fluid restrictions, daily weights, measure abdominal girth, IV albumin; usually resolves w/puberty; social isolation issue (lack of energy, immunosuppression, edema/change in appearance & selfimage) Glomerulonephritis (inflammation of glomeruli; decreased ability to filter urine/decreased plasma filtration) -symptoms: often develops after bacterial/viral infection (strep?); peak onset 6-7 yo; hematuria, smoky/cola/tea-colored urine, proteinuria, edema (periorbital->lower extremities->ascites), oliguria, -management: fluid restriction, protein, salt, potassium, diuretics, dialysis, meds: bp, immunosuppressives, corticosteroids; daily weights, I & O, measure abdominal girth; most children recover completely (few chronic) Acute Renal Failure -Sudden onset, most often caused by dehydration, usually reversible -oliguria/hypotension -Azotemia, uremia, metabolic acidosis, high potassium -tx: IV fluids/electrolytes, monitor I &O, EKG monitoring Chronic Renal Failure -causes: renal/UT malformations, vesicoureteral reflux, chronic pyelonephritis/glomerulonephritis -waste product, H2O, sodium retention -Uremic breath, uremic frost on skin

-management: diet (low phosphorus), monitor for htn, infections, hyperkalemia, seizures; dialysis, transplant Dialysis different types -Peritoneal: preferred for kids, -Hemodialysis: for kids who can visit facility 3x/week for 4-6 hours; rapid; requires vascular access Cardiovascular Ch 48 S & S of Cardiac Dysfunction in Children -poor feeding, tachypnea, FTT, developmental delays (Maslows hierarchy, need O2 first) -prenatal history: preterm, IDM, IUGR -family history: frequent fetal loss, SIDS, sudden death as adult Fetal Circulation -Four Specialized Circulatory Paths shunt blood from liver and lungs Ductus Arteriosus Foramen Ovale Ductus Venous Placenta S & S Cardiac Tamponade Cardiac disorders with increased blood flow -high pressure left to low pressure right ( pulmonary blood flow systemic blood flow) -ASD, VSD, PDA Cardiac disorders with decreased blood flow -T of F, Tricuspid atresia Cardiac disorders with obstruction -stenosis (pulmonic, aortic, etc.) Common pediatric cardiac defects: Tetrology of Fallot o Pulmonary artery stenosis o Right ventricular hypertrophy o VSD o Displacement of aorta (overriding) Atrial Septal Defect Ventricular Septal Defect- more serious PFO Congestive Heart Failure -excess blood in cardiac area (tries to pump harder/faster)- becomes enlarged

Digitoxin -cardiac glycoside -longer lasting digoxin (excreted through kidneys); excreted through liver -a fib, a flutter, heart failure - force of contraction and CO + slows heart rate -Tx for Kawasakis (extensive inflammation of the small vessels and capillaries) Common Cardiac Meds -ACE Inhibitors (CHF): causes vasodilation (lowers vascular resistance/BP/afterload) -Beta blockers: BP, HR, vasodilation Lasix -Diuretic: removes excess fluid to decrease workload of heart and lower BP -give potassium supplementsfoods bananas, green leafy vegetables Cerebral Dysfunction Ch 51 ICP/Coma -S&S: irritability, high pitched cry, distended veins in head, bulging fontanels, lethargy/drowsiness, inability to pay attention, headache, vomiting (confused for GI issue?), seizures, diplopia; Late: LOC, pupil changes, decerebrate or decorticate, cheyne-stokes respirations -monitoring: GCS 8 or less or with respiratory assistance, deteriorating neurologic condition -management: positioning, quiet, suctioning (issues?), nutrition/hydration -SIADH? Concussion/head trauma -Primary (at time of trauma) Skull fracture Concussion- transient & reversible Contusions- visible bruising and tearing of cerebral tissue Coup: bruising at point of impact Contrecoup: bruising at a site far removed from point of impact Intracranial hematoma Subdural- worse in general (depends on exact placement and size) Epidural

Diffuse injury (important to detect, difficult to recover from) Seizures Brain Tumors -Most common solid tumors in children -60% are infratentorial Occur in posterior third of brain Primarily in cerebellum or brainstem -40% are supratentorial Occur in anterior two thirds of brain Mainly in cerebrum -Neuroblastoma- metastasis Rabies Meningitis (Acute inflammation of the meninges and CSF) -Bacterial: strep; e.coli; vaccine: Hib; spread through droplets -Viral: aseptic, treat symptoms, frequently associated with other diseases (measles, mumps, herpes, leukemia) Encephalitis -Inflammatory process of CNS with altered function of brain and spinal cord -mosquitoes & ticks- vectors Musculoskeletal Disorders Ch 54 Legg-Calve-Perthes -ball of the thighbone in the hip doesn't get enough blood, causing the bone to die collapse and become flat blood supply returns over several months, bringing in new bone cells that gradually replace the dead bone over 2 - 3 years -Usually only one hip is affected, although it can occur on both sides. Traction Osteomyelitis -bone infection Scoliosis Types of fractures Muscular Dystrophy -gradual degeneration of muscle fibers, progressive weakness, and wasting of skeletal muscles (increasing disability and loss of strength) -DMD: most common and severe; Respiratory/cardiac failure- respiratory muscles fail = death Guillain-Barr syndrome (GBS) -acquired -symmetric paralysis is part of differential diagnosis -death by respiratory failure Cerebral Palsy -A group of permanent disorders of the development of movement and postures, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain

-abnormal muscle tone and coordination -15-60% will have epilepsy -Athetoid/dyskinetic: Slow, wormlike movements of extremities, trunk, face, tongue Drooling, speech problems Lead to choreajerky involuntary movements worsen with emotional stress -Ataxic: Rapid repetitive movements; wide gait, unable to hold onto objects -Mixed/dystonic: Combination or spastic and athetosis Spinal Muscular Atrophy SMA-1 -Progressive weakness and wasting of skeletal muscle -floppy baby syndrome -Werdnig-Hoffmann disease -onset <2 months; death by 2 years SMA-2 -intermediate -life span 7 months to 7 years SMA-3 -Kugelberg-Welander Syndrome -Worsens over time- ALS type presentation; normal life span Hip Dysplasia Neuromuscular Disorders Ch 55 Latex allergies At risk: Spina Bifida Patients with urogenital anomalies Patients with multiple surgeries VATER association Preterm infants Ventriculoperintoneal shunt Cerebral palsy Multiple surgeries Quadriplegia Neural Tube Defects -Meningocele -Myelomeningocele Hematomas Juvenile Idiopathic Arthritis