Question of the Week

Question :

Posted On : 14 Sep 2006

13 year old girl presented with complaints of facial puffiness since 3wks,abdominal distension for 2wks,weakness and body pain lasting for 6-7 months. Yhere is no history of hematuria or polyuria. There is no h,o breathlesness, chestpain, cough. There is no h,o jaundice, bleeding manifestations, abdominal pain, diarrhoea, vomiting. There is no h,o tuberculosis in past.

On examination, There is severe pallor, no lymphadenopathy, no icteruus, anasarca. There is tense ascites and no other significant findings.

Investigations revealed:
Hb 5gm Percent
Total count: 12800 {p35, lympho65, eosino0, monocyte0}
Esr 70 mm
urine: alb plus plus plus plus , pus cells in full field and rbc absent
Blood sugar: 87 mg Percent
S.bili 0.9 mg Percent {Direct 0.2 mg Percent}
AST 34 IU,L ALT 35 IU,L
ALK PHOS 133 IU,L
S.PROTEIN total 2.0 gm Percent alb 0.8 gm Percent
Urine culture was sterlie.
Blood culture was sterile.
Periphearl smear: showed rbc microcytic,hypochromic,target cells. Platelets 1.7 lakhs,
Retic count 17 Percent
SICKLING TEST was positive.
USG ABDOMEN reveals normal liver texture, gross ascites and renal parenchyma disease grade 1.
Chest XRAY shows bilateral pleural effusion and pericardial effusion.
Pt was started with antibiotis and oral prednisolone. fresh FFP was transfused for 7 days. Blood transfusion was given for 3 units. even after 3 weeks of steroid, urine alb remains plus plus plus plus and ascites remained same. Diagnostic tap revealed. prot. 0.3 gm Percent Glucose 40 mg Percent cells 5,800,mm3 lymphocytes 100 Percent.
ADA 1.4 u,L
Repeated urine cultures sterile. PUS cells remains full field.
HIV SCREENING neg.

How to procede with the case. There are features of haemolytic anemia{SICKLE CELL},nephrotic syndrome and sterile pyuria. can we proceed with starting ATT drugs or go for UROGRAMS OR GO FOR RENAL BIOPSY TO FIND OUT RENAL PATHOLOGY?

Expert Answer :

No expert answer available.

Answer Discussion :

pediatriconcall

What are the serum creatinine and BUN? Apart from nephrotic syndrome and peritonitis, rule out hemolytic uremic syndrome. The ascitic tap is suggestive of inflammation. Do a cytospin for malignant cells. For positive sickling test, do HbEPP to rule out sickle cell anemia. Since the child has been transfused, the HbePP cannot be done at the present and one may need to do parents HbEPP to see if they have the thal trait.

18 years ago

S.D.Jambhekar

please do serum cholesterol level.It should be high, I expect.This seems to be a case of nephrotic syndrome with sever anaemia.with history of bone-body pains for long time, one would like to rule out leukemia in this child.a bone marrow is a must.once leukemia is ruled out standard treatment of nephrotic syndrome i.e. 2MKD devided in tds for 6weeks needs tobe given before making a dignosis of SRNS.if proved at the end of 6 weeks to be SRNS A renal biopsy need to be done. most likely it may turn out to be non-minimal change NS, being onset in pre-pubertal period. high protein diet is a must for this child with sever hypo proteinemia.think twice before you use diuretic in this patient.

18 years ago

BHARAT