Extra-hepatic portal vein obstruction and type 1 diabetes in a child: a co-incidence or causal association?

Case report An eight year old boy presented with polyuria and polydipsia for 1 month. On examination, liver was not palpable but spleen was enlarged (6cm). Blood sugar was 460 mg/dl and type1 DM was confirmed. Ultrasonography showed portal vein cavernoma with normal liver and pancreas. Endoscopy revealed grade 2 esophageal varices. Thus diagnosis of EHPVO with portal hypertension was also confirmed. Other investigations including liver functions, hemogram, renal functions, serum amylase, lipid profile, thyroid function, Protein C, S and factor V Laiden estimation were normal. There was no history of abdominal infections like peritonitis, pancreatitis and umbilical sepsis or umbilical artery catheterization in neonatal period. C-peptide level was low (0.111 ng/ml) with weakly positive Islet cell antibodies, positive GAD 65 antibodies (> 2000 IU/ml). Tissue peroxidase and Tissue transglutaminase antibodies were negative. Patient is asymptomatic after endoscopic band ligation of esophageal varices and split-mix insulin regimen.