A 4 Year Old Girl Is Admitted In Pediatric Ward With The Chief Complaint Of Generalized Body Swelling

A 4 year old girl was admitted to the pediatric ward with the chief complains of generalized body swelling. He mother gives the history that she noticed the swelling first appearing on the face about 5 days ago which then gradually progressed to involve the entire body. The patient has decreased appetite and has lost interest in playing with her toys. Mother also mentions that the patient has only passed urine once yesterday and it was very small in quantity.Urine was dark colored but she did not see any blood in urine.  Before the onset of theses symptoms , the child was in good health except for a minor upper respiratory infection about a week ago. Before the illness the child was taking regular food cooked at home that included boiled eggs, vegetables and sometimes meat. She was immunized and her development was appropriate for her age.
On Examination she had a fever of 101 F. other vitals were within normal limits.

On general physical examination patient had a periorbital edema, puffy face, pedal edema and looked lethargic. There was no jaundice but she looked pale.
On abdominal examination she had ascites.
On auscultation of the chest there were some basal crepitations.

List the Differential Diagnosis of the above case:


1. Nephrotic Syndrome:
 Edema starts in face, arms and then descends. Periorbital edema is classical.
 Edema is usually noticed in morning; pitting edema
 History of previous renal disease may or may not be elicited
 Presence of proteinuria, hypoproteinemia and hypercholesterolemia on lab results will confirm the diagnosis.

2. Acute Nephritic Syndrome

3. Congestive Cardiac Failure.

4. Cirrhosis of Liver.

5. Anemia with Hypoproteinemia.

6. Myxedema.

What Investigations will you order for this patient?

1. Urine—for Proteinuria  by dipstick
2. Urine (24 hours)—Albumin > 1 gm/m2 (nephrotic syndrome)
3.  Urine (24 hours)—Urine Protein/Urine Creatinine ratio: Best performed on first morning voided urine sample. Ratios < 0.5 in children younger than 2 years of age and < 0.2in children more than 2 years of age sugges tnormal protein excretion. A ratio > 3 suggests nephrotic range proteinuria.
4.  Serum cholesterol and LDL—Elevated
5.  Serum albumin— < 3 gm/dl (Normal level is 3.5-5.5 gm/dL)
6.  Serum globulin—Normal, although α2 and β globulin are elevated
7.  Urine culture  to rule out Urinary tract infection
8.  Kidney function test—is usually normal but may be deranged in renal failure
9. Serum electrolytes
10. Complete blood count and ESR
11. Mantoux test—To rule out tuberculosis before starting steroids as steroids can flare up Tuberculosis
12.  Chest X-ray —To look for pleural effusion or pulmonary tuberculosis
13.  Ultrasound  abdomen—For kidney size or any organicpathology
14.  C3 levels— Serum C3 levels is determined in all patients of suspected acute glomerulonephritis because a low level narrows a differential diagnosis to certain forms of
glomerulonephritis like post-streptococcal, lupus, membranoproliferative glomerulonephritis
and chronic infection.
15. A kidney biopsy may be needed if symptoms do not improve with treatment.

Final Diagnosis: The patient is most probably suffering from minimal change nephrotic syndrome.

 How will you treat this patient?
1. Steroids: Oral prednisolone is started in a dosage of 2 mg/ kg/day (60 mg/m2/day). The total daily dose is usually split into two or three doses and given daily for 6 weeks.
For maintenance therapy prednisolone is given at ~1.5 mg/kg/day (40 mg/m2/day) given on alternate days, as a single morning dose, for a 6-week period after which it is discontinued.
2. An antacid preparation may be given with prednisolone to reduce gastric irritation.
3, A protein rich diet with no added salt is given.
4. In cases of severe edema fluid intake should be restricted.
5. diuretics may be needed if patient develops respiratory distress.

Some Important Definitions Related to Nephrotic Syndrome in Children:

1. Nephrotic syndrome: Clinical state characterized by proteinuria, hypoalbuminemia, edema and Hypercholesterolemia.

2. Hypoalbuminemia:
 Serum albumin < 2.5 gm%
 Edema appears when serum albumin < 2 gm%
 With serum albumin < 1.5 gm%: Pleural and pericardial effusion appear.

3. Hypercholestrolemia: Serum cholesterol > 250 mg%

4. Remission: Urine albumin nil or trace (or proteinuria < 4 mg/m2/hour) for 3 consecutive days.

5. Relapse: Urine albumin 3+ or 4+ (or proteinuria > 40 mg/m2/hour) for 3 consecutive days, having
been in remission previously.

6. Frequent relapses: Two or more relapses in six months of initial response, or more than three
relapses in any twelve months.

7. Steroid dependence: Two consecutive relapses when on alternate day steroids or within 14 days
of its discontinuation.

8. Steroid resistance: Absence of remission despite therapy with 4 weeks of daily prednisolone in a
dose of 2 mg/kg per day.