Pediatrics II

A Case presentation
Pangilinan, Juneve
Pascua, Krinzel
Perez, William
Pescante, Ma Nina

GENERAL DATA
Patient: D.D
Age: 6 y/o
Filipino
Male
Currently lives in San Andres Bukid, Manila
Birth date: February 7, 2008
3rd time admission at Ospital ng Maynila Medical

Center on June 23, 2014 at around 9:30 pm

Informant: Father
Reliability: 90%

CHIEF COMPLAINT
Difficulty opening his eyes

HISTORY OF PRESENT ILLNESS

16 MONTHS PTA
Patient manifested with periorbital edema in the morning.
Swelling progressed to his face then to his abdomen and

lower extremities.
Edema was described to be nonpitting which lasted for 4
days.
Tea-colored urine was also noted as well as a remarkable
increase in the patients weight from 30 kg to 40 kg.
No medication was taken but patient sought consult to
OMMC and was admitted for the first time

16 MONTHS PTA
During admission, patients highest BP was 120/90 mmHg

compared to his normal BP of 90/60 mmHg.

Patient was given with IV dextrose and albumin with
unrecalled dosage and swelling subsided.
It took 4 days before edema was noted to have completely
resolved thus the patient was discharged.

16 MONTHS PTA
Patient was prescribed with the ff medication:
prednisone 5 mL syrup once every other day

isoniazid (unrecalled dosage)

Patient was compliant in taking the medications.

They were advised that the patient should avoid salty foods,

chocolates and juice.

They were advised that the patient should avoid sick people

 Patient was apparently well after 1st admission.

Patient had a regular consult every 2 weeks at OMMC OPD.

Urine dipstick test was done every checkup and level of

albumin was regularly monitored.

 Father of the patient noticed that patient easily contracts

respiratory infections almost every 3 months.

Patient usually manifests with undocumented fever, clear
nasal discharge and cough which last for a week.
Patient was given amoxicillin (250mg/5mL) syrup for 7 days
every time he was suffering from respiratory infections.
Patient was compliant in taking the medication and
symptoms usually resolved after treatment.

6 MONTHS PTA
Patient manifested again with facial edema and abdominal

swelling of the same quality and pattern which prompted

second admission at OMMC
Same medications were given and patient stayed in the
hospital for a week.
Patient continued taking his medication after discharge and
was able to return to school after hospital admission.
Patient was apparently well after the 2nd admission until 1
month PTA

1 MONTH PTA
Patient missed a checkup because he was in the province.
On his next check up, patient had urine dipstick test

Results showed cloudy urine and +4 albumin.

In addition to his maintenace drug (prednisone), patient was

prescribed with enalapril with unrecalled dosage.

Patient was compliant together with other medication.
No other symptoms were reported until 1 week PTA

1 WEEK PTA
Patient woke up with periorbital and facial edema but

dismissed it as taba sa mukha

It was nonpitting without accompanying redness, tenderness,
itchiness or any discomfort.
Tea-colored urine described as iced tea in color was also
noted.
No other accompanying symptoms were noted like fever,
dysuria or flank pain.
No other medication was taken aside from prednisone and
enalapril.
Swelling did not resolve.

3 DAYS PTA
Presence of periorbital and facial edema persists.
Abdominal swelling and bilateral pitting sacral edema were

also noted.
Same urine color was noted but no changes in urine
frequency, volume and urgency were observed.
No other medications were taken. Consult was not done.

2 HOURS PTA
Swelling of the abdomen was still apparent and swelling on

his legs below the knee were noted.

Presence of periorbital and facial edema persists but patient
complained of difficulty opening his eyes so they sought
consult to OMMC hence the admission.

REVIEW OF SYSTEMS
Constitutional

(-) loss of appetite

Skin and nails

(-) rashes (-) changes in skin color

Head

(-) headache (-) lightheadedness (-)dizziness (-) syncope

Eyes

(-) eye pain (-) blurred vision (-) discharge

Ears

(-) discharge (-) hearing problem

Nose and sinuses

(-) tenderness (-) epistaxis

Mouth and throat

(+) sore throat (-) dysphagia

Respiratory

(-) dyspnea (-)orthopnea (-) PND

Cardiovascular

(-) chest pain (-) palpitations

Gastrointestinal

(+) vomiting (+) diarrhea (-)hematemesis (-) hematochezia

REVIEW OF SYSTEMS
Genitourinary

(-) flank pain

Endocrine

(-) excessive sweating

Nervous/Behavioral

(-) paresthesia (-) numbness

Musculoskeletal

(-) muscle pain

Hematologic

(-) pallor (-) bruising (-) overt bleeding

PAST MEDICAL HISTORY

(-) allergies in food and medication
(+) measles and chickenpox

Immunization History
Immunizations taken:
1 dose BCG
3 doses DPT
3 doses OPV
3 doses hepatitis B
1 dose measles.

PERSONAL HISTORY
Feeding History
Patient has good appetite, eats regularly and does not

skip meals
Eats 3 full meals a day with snacks in between
MEAL: 1 cup of rice, fried egg white, 1 serving of meat (fried chicken

without skin, fish or pork)

SNACKS: sandwiches/ biscuits and mineral water
Seldom eats leafy and non-leafy vegetables

Preference for fruits, milk, and sweet foods

3-4 glasses of mineral water a day

Does not take vitamins nor food supplements.

PERSONAL HISTORY
Developmental/Behavioral History
first of two children in the family
Grade 1; goes to school regularly however misses

his Monday classes when he is scheduled for checkup every 2 weeks

TANNER STAGE 1
No pubic hair, preadolescent penis and testis, 3 cm

PERSONAL HISTORY
Family History
Father (39), works at an upholstery cleaning

service and Mother (36), housewife, both with no

known illness
Patient had a 3-mo old sibling with no known
illnesses.
Reported history of HPN on fathers side and DM
on mothers side. No family history of asthma,
kidney disease, cardiac disease, blood disorders and
cancer.

PERSONAL HISTORY
Socioeconomic History
lives with parents, sister and aunt (mothers side) in one

floor of a multi-storey residence in San Andres, Bukid,

Manila for 6years.
Father and aunt work for their living
Environmental History

Patient often stays at home, otherwise plays with friends at

school and neighbourhood.

No exposure to cigarette smoke at home.
Drinking water is mineral type
Regular medications: prednisone and enalapril; amoxicillin
and cough syrups

PHYSICAL EXAM

GENERAL SURVEY
Lying on bed
With oxygen mask
Regulated at 3L per minute

Conscious
Coherent

Slightly irritable but cooperative

Vital Signs
PR: 154 bpm
RR: 20 breaths per min
Regular rhythm and depth

BP: 100/60
Over right brachial artery, supine

Temp: 37.7C, axillary

Anthropometric
Weight: 24.9 kg
Height: 108 cm
BMI: 21.35
Abdominal circumference: 75 cm

Anthropometric

SKIN
Moist and warm
No jaundice or cyanosis
Good skin turgor
No suspicious nevi, rash, petechiae,
eccyhmoses
No clubbing on fingernails

HEAD
Normocephalic without overt lesion or

mass
Symmetric facial features, movements and
expressions
Hair is coarse in texture
Equally distributed w/o areas of hair loss,
lice or any lesion, redness and scaling
With facial edema

EYES
With periorbital edema
Symmetric eyelids without swelling,

ptosis, lesions
No excessive tearing and dryness
Pink palpebral conjunctivae without
discharge
Anicteric sclerae
Visual acuity 20/20 on the right eye,
20/20 left eye

EAR
Symmetric without swelling, redness, or

discharge
Non tender
Intact tympanic membrane on both sides
Heard sound on whisper test at 2ft. on left
and right ears
Weber and Rinne test not done

NOSE AND SINUSES

Symmetric without any deformities,
obstruction, lesions, exudates or
discharge.
Nasal mucosa was pink
Septum in the midline
No frontal and maxillary sinus
tenderness

MOUTH AND THROAT

No circumoral pallor
Lips were pink and moist without any sores or

lesions
Gums without swelling or ulceration
Oral mucosa was pinkish without ulcers, white
patches or nodule
Impacted first molar of all quadrants of the mouth
Tongue is pinkish, moist and in the midline without
lesions.
Uvula in the midline
Tonsils are pink, Grade 1

NECK
Trachea was in the midline
Nonpalpable and nontender cervical lymph nodes
Thyroid gland not enlarged

Carotid pulsations and jugular vein pulsation not

prominent

THORAX AND LUNGS

Symmetric chest without deformities
Transverse diameter > anteroposterior diameter
No retractions and respiratory lag

No use of accessory respiratory muscles

Equal chest expansion
Tactile fremitus not performed
Bronchovesicular breath sounds were heard on

majority of intercostal spaces on both lung fields

No adventitious breath sounds

CARDIOVASCULAR SYSTEM
Adynamic precordium
PMI at 4th ICS left midclavicular line
No heaves, lifts, or thrills
S1 louder than S2 at the apex
S2 was louder than S1 at the base
No murmurs

ABDOMEN
Protuberant
No scars, lesions, engorged blood vessels
No bulging of flanks
Skin on the abdomen pitted when the diaphragm of

the stethoscope was placed over it

No bruits on the epigastric area and hypogastric
area
Normoactive bowel sounds at 40/min
(-) fluid wave
No mass or tenderness

Genitourinary
(+) scrotal swelling
Tea-colored urine

EXTREMITIES AND
PERIPHERAL VASCULAR
Bilateral pitting edema
level below the knee
Feet
Face

Edema grade 2+
lasts for 37-40 seconds

No varicosities, deformities, visible

joint swelling and redness

EXTREMITIES AND
PERIPHERAL VASCULAR
Capillary refill time: <1 sec.

NEUROLOGIC
Conscious
Coherent
Slightly irritable
Cooperative
Clear speech
Folstein MMSE was not done

NEURO: CN Exam

MOTOR EXAM, TESTS FOR

COORDINATION, REFLEXES,
SENSORY EXAMINATION
Not done

SALIENT FEATURES
CC: Difficulty opening the eyes
6 years old male
Facial swelling
Periorbital swelling
Scrotal swelling
Tea-colored urine
Grade 2+ bipedal edema below the knee
Cloudy urine
+4 albumin in the urine
Irritability

APPROACH TO
DIAGNOSIS
The presenting manifestation of the patient
is EDEMA. Approach to diagnosis will be
based on the presenting manifestation that
points to a GROUP OF DISEASE OR
DISORDERS.

Trauma

Localized

Venous/ Lymphatic
Obstruction

Infection

Edema

Cardiovascular
Diseases

Congestive Heart
Failure

Infectious
Generalized

no cardiomegaly, no
dyspnea, no jugular
vein distention, no
chest pain

absence of infectious
agent

Renal Diseases

Non Infectious

Liver Diseases

Hepatic Failure

history of albuminuria

No jaundice, anicteric
sclera

Nephrotic
Syndrome
ProteinLosing
Enteropathy
Acute
Glomerulone
phritis

DIFFERENTIAL
DIAGNOSIS

DIFFERENTIAL DIAGNOSIS
Patients Signs &
Symptoms

Edema
Proteinuria
Hypertension
(once)
Tea-colored
urine
Cloudy urine
Weight gain
Others

1. Idiopathic
Nephrotic Syndrome
(INS)
(+)
(+)
rare

2. Acute
Glomerulonephritis
(AGN)
(+)
(+)
prominent

3. Protein-losing
Nephropathy
(PLN)
(+)
(+)
prominent

(+) ~20% presents

with microhematuria
(+)
(+)

(+) usually gross

hematuria
(+)
(-)

(+)

Decision

Most common in
males aged 2-6
years old
Usually presents with
cloudy urine which
was seen in the
patient

Cannot be ruled out

Patient did not present

with the following
characteristic symptoms
of AGN:
o Dyspnea
o Oliguria
o Headache
o Flank pain

Ruled out

(+)
(-)
Patient did not present
with the following
characteristic symptoms
of PLN:
o Abdominal pain
o Signs of liver
disease (ie.
jaundice,
splenomegaly etc.)

Ruled out

DIAGNOSTIC
WORK-UP

URINALYSIS
First test used in the diagnosis of nephrotic

syndrome
Proteinuria will be apparent by 3+ or 4+
readings on the dipstick, or by semiquantitative testing by sulfosalicylic acid
A 3+ reading represents 300 mg/dL of
urinary protein or more, which is 3 g/L or
more and thus in the nephrotic range
To check & monitor for severity of proteinuria
& to be able to adjust treatment

URINE PROTEIN MEASUREMENT

Measured by a timed collection done over a
24-hour period (starting at 7am and
finishing the next day at the same time) or a
single spot collection
In healthy individuals, there are no more
than 150 mg of total protein in a 24-hour
urine collection.

URINE PROTEIN MEASUREMENT

When the ratio of urine protein to urine
creatinine is greater than 2 g/g, this
corresponds to 3 g of urine protein per day
or more
The exact type of urine protein is of
potential interest and can be tested by urine
protein electrophoresis
This differentiates nephrotic syndrome from
other protein-secreting diseases other than
albumin

SEROLOGIC TESTS
Serum albumin level is classically low in nephrotic

syndrome, being below its normal range of 3.5-4.5 g/dL

In a study, frequency of focal and segmental

glomerulosclerosis increased with elevations in serum

albumin
from 26% in patients with serum albumin < 30 g/L to

74% in patients with serum albumin of 35 g/L or higher

Serum cholesterol and triglyceride are usually elevated.

Checking this will allow adjustment in treatment.

SEROLOGIC TESTS
Serum creatinine will be normal ranged in

uncomplicated nephrotic syndrome but it

may be increased due to diminished renal
perfusion and may indicate atypical
nephrotic syndrome that may warrant
referral to pediatric nephrologist.
Serum electrolyte balance should be
monitored.

Management

NONPHARMACOLOGIC
MANAGEMENT
Diet should provide adequate energy (caloric intake) and

adequate protein (1-2 g/kg/d)

SALT/FLUID RESTRICTION

Sodium intake should be reduced by the initiation of a

low-sodium diet and may be normalized when the child

enter remission.
1 to 3 years
2g salt per day (0.8g sodium)
4 to 6 years
3g salt per day (1.2g sodium)
7 to 10 years
5g salt per day (2g sodium)
11 and over
6g salt per day 2.4g sodium)
www.clinicalguidelines.scot.nhs.uk

NONPHARMACOLOGIC
MANAGEMENT
Fluid restriction maybe necessary if the child is hyponatremic.
A gentle fluid restriction is also usually beneficial to minimize

edema. Suggested fluid intake: (Christian, 2013)

<5 yrs = 750 ml/day

>5 yrs = 1 litre/day

A swollen scrotum may be elevated with pillows to enhance

the removal of fluid by gravity.

NONPHARMACOLOGIC
MANAGEMENT
Close monitoring of volume status, serum electrolyte

balance and renal function is necessary.

Daily weight monitoring and daily urine dipstick.
There are no activity restrictions for patients with Nephrotic
Syndrome. Ongoing activity rather than bedrest will reduce
the risk of blood clots.

PHARMACOLOGIC
MANAGEMENT
DIURETICS
Chlorothiazide: 10 mg/kg/dose IV every 12 hours

Metazolone: 0.1 mg/kg/dose PO BID

Furosemide: 1-2 mg/kg/dose IV every 12 hours (given 30

minutes later)
25% HUMAN ALBUMIN (with Furosemide)
2 indications:
Clinical hypovolemia ( Intravascular volume depletion)
Severe or symptomatic edema

0.5 g/kg/dose q 6-12 hours administered over 1-2 hours

Followed by Furosemide

When fluid restriction and parenteral diuretics are not

effective.

PHARMACOLOGIC
MANAGEMENT
STEROID THERAPY (INITIAL THERAPY)
Prednisone (12-week initial course):
60 mg/m2/ day [2 mg/kg/day] (minimum daily

dose, 80 mg divided into 2-3 doses) for 4-6

consecutive weeks
After initial 6-week course, Prednisone dose should be
tapered to 40 mg/m2/day [1.5 mg/kg] given every
other day as single morning dose for 6 weeks
The alternate-day dose is then slowly tapered and
discontinued over the next 2-3 months
Reduce dose by 5-10mg/m2 each week

PHARMACOLOGIC
MANAGEMENT
RESPONSE TO TREATMENT
Most respond to steroids within 2-4 weeks

Children who continue to have proteinuria (2+ or

greater) after 8 weeks of steroid therapy are

considered RESISTANT and a diagnostic renal
biopsy should be performed.

PHARMACOLOGIC
MANAGEMENT
TREATMENT FOR INFREQUENT RELAPSES
steroids are resumed, although for a shorter duration

than treatment during initial presentation

Daily divided-dose prednisone (60 mg/m2/ day [2
mg/kg/day]) until the child enters remission (urine
trace or negative for protein for 3 consecutive days).
After remission, prednisone (1.5 mg/kg [40
mg/m2]), given as a single dose on alternate days for 4
weeks
Urine checked 2x weekly, then weekly after the
episode

PHARMACOLOGIC
MANAGEMENT
STEROID-DEPENDENT NEPHROTIC

SYNDROME THERAPY

CYCLOPHOSPHAMIDE
2-3 mg/kg/24 hrs given as a single dose for a total

of 8-12 weeks
Prolongs the duration of remission and reduces the
number of relapses in children with frequently
relapsing and steroid-dependent nephrotic
syndrome.

PHARMACOLOGIC
MANAGEMENT
METHYLPREDNISONE
30 mg/kg bolus (maximum 1g)
First 6 doses given every other day followed by a tapering

regimen for periods up to 18 mos

Given for children with Complicated Nephrotic
Syndrome.

CYCLOSPORINE
36 mg/kg/24 hr divided q 12 hr
TACROLIMUS
0.15 mg/kg/24 hr divided q 12 hr

PHARMACOLOGIC
MANAGEMENT
ANTIHYPERTENSIVE THERAPY
Only when hypertension is present and particularly if

it persists
ACE Inhibitors
ARBs
Ca channel blockers
Beta blockers

REFERENCES
Fauci AS, Kasper DL, Longo DL, Braunwald E, Hauser SL, Jameson J.

(2008). Harrison's Principles of Internal Medicine. Philadelphia: McGraw

Hill Companies.
Gipson DS, Massengill SF,Yao L, Nagaraj S, Smoyer WE, Mahan JD,
Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA.
(2009). Management of Childhood Onset Nephrotic Syndrome.
Pediatrics 124: 747-757

Kliegman, R. (2007). Nelson textbook of pediatrics.18th ed. Philadelphia,

PA: Saunders Elsevier.

Lane JC, Langman CB, Finberg L. (2014). Pediatric Nephrotic
Syndrome Treatment & Management. Retrived on 6 July, 2014 from
http://emedicine.medscape.com/article/982920-treatment#showall

REFERENCES
Cohen, Eric P. and Vecihi Batuman. 2014. Nephrotic

Syndrome Diagnostic Workup. Accessed on 29 June, 2014

from http://emedicine.medscape.com/article/244631workup.
Fauci, A. S., Kasper, D. L., Longo, D. L., Braunwald, E.,
Hauser, S. L., Jameson, J., et al. (2008). Harrison's Principles of
Internal Medicine. Philadelphia: McGraw Hill Companies.
Kliegman, R. 2007. Nelson textbook of pediatrics. 18th ed.
Philadelphia, PA: Saunders Elsevier. p. 3564-3569.