Diagnostic Dilemma

Jaundice and arthritis


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Case Report: - A 3½ years old boy born of non-consanguineous marriage presented with recurrent multiple joint pains and swelling with restriction of movement for 1½ year associated with fever. The joints commonly involved were small joints of the hand, wrists, ankles and cervical spine. He was on homeopathic treatment for the same for 1 year and aspirin (100 mg/kg/day) for last 14 days. On examination, he was febrile, had pallor, cervical lymphadenopathy and hepatomegaly. Joints were normal. Investigations showed:


·          Hemoglobin = 9.1 gm/dl


·          WBC = 7,600/cumm (75% polymorphs, 25% lymphocytes)


·          Platelet = 2,53,000/cumm


·          ESR = 55 mm at end of 1 hour


·          Renal function tests = Normal


·          SGPT = 571 IU/L


·          RA Factor = Negative


·          USG Abdomen = Hepatomegaly with echo poor hepatic echotexture with sludge in gall bladder with minimal ascitis


·          A repeat LFT next day showed


·          Bilirubin = 10.8 mg% (direct = 5.2 mg %)


·          SGOT = 3380 IU/L


·          SGPT = 880 IU/L


·          Total proteins = 6 gm% (Albumin = 2.5 gm %)


·          PT = 31.8 seconds, PTT = 70.1 seconds


·          HIV = Negative


 


The child on next day developed hypotension, bradycardia and severe respiratory distress. He was given IV Fluids, ionotropic support and IPPR but died within next 12 hours due to pulmonary edema. Echocardiography was normal



What is the cause of death?
Expert Opinion :
We did a bronchoalveolar lavage and that did not cause any TB on culture. CT chest shows destroyed left lower lobe. We are planning lobectomy of the diseased lung to prevent recurrent infections.
Answer Discussion :
D
Dr. Tapomay Banerjee
bubble
hemophagocytyic syndrome
17 years ago
D
Dr. Altaf Naseem
bubble
reye,s syndrome
17 years ago

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