Diagnostic Dilemma

Polyserositis


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Question

12 yrs male child came with swelling all over body including face, lower limb scrotum, abdomen since last four months with decreased urine output. Swelling reduced after taking some medication (?diuretics). There is history of fever on and off since last two months. There is no hematuria, burning micturation, diurnal variation of swelling, jaundice, breathlessness, palpitation or cyanosis. There is no contact with TB. On examination the child is averagely built and nourished (weight-26 kg; height-130 cm), vital parameters are stable and BP is 100/70mm of Hg. Skin is dark and there is conjunctival xerosis. There is no icterus, pedal edema and genitals are normal. There are no signs of liver failure; JVP is normal. On systemic examination abdomen is distended with smiling umbilicus, non tender hepatomegaly with span 16cm, firm, rounded margins and a just palpable spleen. Horse shoe shaped free fluid is present. On cardiovascular system heart sounds are muffled. On respiratory system, air entry is reduced in bilateral basal regions. CNS is normal. Investigations showed:


• Hemoglobin-13.4gm%
• WBC- 10,200 (54% polymorphs, 44% lymphocytes, 2% monocytes).
• Urine-Normal.
• Renal function Tests-Normal
• Bilirubin-2.2mg/dl (Direct-1.18mg/dl), SGOT-32.5 IU/L, SGPT-73 IU/L, alkaline phosphatase-220, Total Protein-8.2 gm/dl, Serum albumin-3.2 gm/dl
• HBsAg-negative
• Prothrombin Time-20.5 sec, INR-1.4.
• Chest X ray-mild cardiomegaly.
• USG abdomen-gross ascitis with bilateral pleural effusion(mild), mild hepatosplenomegaly,
• Echocardiography-Moderate pericardial effusion. with pericardium thickened with some exudative threads with MVP with mild MR and mild TR.
• Ascitis fluid-straw colour, protein-4gm,glucose-normal,cells-400 (40% neutrophils, 60% lymphocytes). Few gram positive cocci seen. AFB not seen. ADA-15(normal)
• LDH-normal



What is the diagnosis?
Expert Opinion :
An update on the case: his genetic markers were sent for wilsons disease which were negative. Thus, wilsons was ruled out in this child. This child could still be autoimmune liver disease as is on a close watch to check for the same.
Answer Discussion :
V
varada rajendra
bubble
budd-chiari syndrome.
17 years ago
D
Dr. Kavan Deshpande
bubble
budd charrie syndrome
17 years ago

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