A 3 years old girl with proteinuria and hepatosplenomegaly

Dr Ira Shah

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A 3 years old girl with proteinuria and hepatosplenomegaly

Dr Ira Shah.

Medical Sciences Department, Pediatric Oncall, Mumbai, India.

ADDRESS FOR CORRESPONDENCE
Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056.

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Clinical Problem
Case summary:

A 3-½ years old female child born of non-consanguineous marriage presented to a pediatrician with generalized edema and decreased urine output 1 month back. She was investigated and found to have proteinuria.

Investigations done were
Hb = 6.2 gm%
WBC = 13,800 cells/cumm [poly = 36%, lympho = 58%, eosino = 6%]
ESR = 55 mm at end of 1 hour
SGPT = 15
S. Creatinine = 1.2 mg
S. Bilirubin = 0.78 mg%
Total proteins = 6.8 gm/dl
S. Albumin = 3.5 gm/dl
Urine – Albumin 3+, RBC - Occasional

She was diagnosed as Nephrotic syndrome and started on Prednisolone (2.5 mg/kg/day) for 15 days and then shifted to alternate day (2 mg/kg/alternate day).

In view of the low hemoglobin, she was given a blood transfusion 4 days back following which she developed hematuria and petechiae over lower limbs. On investigation, her
Hb = 11.5 gm%
WBC = 10,800 cells/cumm [poly = 29%, lympho = 68%, eosino = 3%]
Platelet count = 35,000 cells/cumm
S. Creatinine = 0.2 mg%
Urine - Albumin 2+, RBC – plenty.

On presentation to us, she had pallor, petechiae over both lower limbs and a firm hepatosplenomegaly with ascitis. There was no puffiness over eyelids or pedal edema.

Her urine showed 2+ albumin with 40-41 RBCs / Hpf
Cholesterol = 105 mg/dl
Total proteins = 6.0 gm%
S. Albumin = 3.9 gm%
Globulin = 2.1 gm%
Creatinine = 0.5 mg/dl
BUN = 8 mg%

Is the diagnosis of nephrotic syndrome correct and will the previous treatment affect the present illness?

Discussion
Here is a situation, where the child was diagnosed as Nephrotic syndrome just on the basis of proteinuria and edema. However if one goes back to the basics, nephrotic syndrome consists of proteinuria, hypoalbuminemia, hyperlipidemia and edema. This child though has proteinuria does not have hypoalbuminemia and hyperlipidemia. Also, one has not established whether the proteinuria is in the nephrotic range or not by doing the 24 hours urinary albumin or the urine albumin/creatinine ratio. Hence, to proclaim this child as nephrotic syndrome in the 1st place would be erroneous.
This child has hepatosplenomegaly with petechiae and thrombocytopenia with recurring pallor. A diagnosis of leukemia may enter one’s mind. A hemogram on this child picked up 73% blasts on peripheral smear and USG Abdomen showed multiple retroperitoneal lymphnodes with metastasis in the liver and bone marrow aspiration was suggestive of acute lymphoblastic leukemia. Hence, the child was diagnosed as leukemia.

This child had received steroids for a month as part of treatment of nephrotic syndrome and had also received a blood transfusion. Both these agents can lead to a temporary remission of the leukemia. Thus, this diagnosis of leukemia in this child would have been missed leading to delayed diagnosis and poor response to subsequent chemotherapy. The initial clue to the leukemia would have been the hepatosplenomegaly in the child, which is not seen in a child with nephrotic syndrome.

Thus, to conclude – a diagnosis of nephrotic syndrome should be made in a child when it fulfils all the 4 criteria of proteinuria, hypoalbuminemia, hyperlipidemia and edema and one should learn a lesson of being extremely careful while starting steroids in a child and rule out other co-morbid conditions before starting steroids.

Compliance with ethical standards

Funding: None

Conflict of Interest: None

Cite this article as:
Shah I. A 3 years old girl with proteinuria and hepatosplenomegaly. Pediatr Oncall J. 2004;1.