Is it really biliary atresia_?
Author: Pediatric Oncall
A 10 years old boy presented with ascites and joint stiffness for 1 month. He was operated at 4 months of age for biliary atresia and a Kasai surgery was done. There is a history of neonatal ichthyosis and now prolonged itching. He also had persistent jaundice post kasai surgery. He was born of third degree consanguineous marriage and milestones were normal. On examination, weight was 29.2 kg, height was 134.5 cm, he had clubbing with jaundice, hepatosplenomegaly, ascites, hypotrichosis, alopecia and dry scaly skin {Figure 1}. Other systems were normal. Investigations showed: bilirubin 3.3 mg, dl {direct 2.7 mg, dl}, SGOT = 124 IU, L, SGPT = 65 IU, L, Total proteins = 4.2 gm, dl, albumin = 2.1 gm, dl and alkaline phosphatase 418 IU, L. Prothrombin time {PT} was 19 sec and PTT was 36 sec. Ultrasound abdomen showed cirrhosis with splenomegaly and ascites and reversal of flow in portal vein. He had hypersplenism in form of hemoglobin of 8.8 gm, dl, white cell count of 4900 cells, cumm and platelets of 1,19,000, cumm. He was suspected to have NISCH syndrome and peripheral smear did not show vacuolated white cells but thyroid functions showed Free T3 of 3.93, Free T4 of 1.29, TSH of 7.555 and lipid profile was normal {cholesterol = 129 mg, dl, triglycerides = 108 mg, dl though HDL cholesterol was low 15 mg, dl}. He was advised genetic studies but was subsequently lost to follow up.
Answers of this discussion
Currently no answers was available for this question.
Disclaimer: The information given by is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.