Steroid refractory type 2 autoimmune liver failure complicated by HSV induced leukoencephalopathy
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Abstract
Background: AIH type II is a rare cause of end-stage liver disease in children and requires aggressive management. Case: A 4-year-old boy developed progressive jaundice for 1 month, unresponsive to oral medicines. On day 15, he developed altered sensorium requiring hospitalization for 8 days. Lab investigations revealed serum ammonia 235mmol/L, INR-3.2, TSB-19.7 mg/dl, SGOT/SGPT-705/3260 U/L, Albumin 2.4 gm/dl, Hb 6.6 gm%, Anti HAV IgM positive. USG abdomen suggested altered liver echotexture, splenomegaly and ascites. Encephalopathy improved over next 3 days but coagulopathy persisted. Suspecting ACLF, he was referred for liver transplant. On examination child was lethargic with pallor, icterus, hepatosplenomegaly and ascites. Further investigation showed IgG-4458 mg/dl, DCT positive (4+), Ceruloplasmin -16.2 mg/dl, T.Bil-21.8 mg/dl, D-14.7 mg/dl, S.Albumin- 2.4 gm/dl, SGOT/PT- 898/355 U/L, ammonia-344 and normal G-6PD. Anti LKM and Anti LC-1 came positive. CT liver showed early cirrhotic changes without portosystemic collaterals. We started Injection methylprednisolone. After 48 hours, child developed altered sensorium (GCS=8). Blood culture showed E. coli (sensitive to meropenem). Inspite of upgraded antibiotic, stable LFT and INR (between 3-4), child remained in altered sensorium (GCS=8). MRI brain was done which showed leukoencephalopathy (? infective/toxic). HSV IgM came positive and Injection Acyclovir was started. Sensorium improved over next 24 hours. He was started on oral prednisolone after repeat blood culture showed no growth. Oral steroids were continued for 14 days nonetheless transaminases and INR did not show any significant improvement. Tab Mycophenolate Mofetil was added. Patient was closely monitored for any deterioration. Liver enzymes, bilirubin and INR started showing sustained improving trend after 5 days of MMF. His latest Investigations after three months of therapy were S.Albumin-3.5gm/dl, SGOT/SGPT-115/75 U/L, T.Bil-1.7mg/dl (D-1.0), PT/INR-17.2/1.6. Conclusion: In patient with chronic liver disease altered mental status is often attributed to HE, however other causes specially infection must be considered in unexplained scenario.
