Autoimmune Hepatitis with Distal Renal Tubular Acidosis and Small Bowel Partial Malrotation

Abstract

Renal tubular acidosis (RTA) is not uncommon in patient with chronic autoimmune hepatitis (AIH), but usually remains latent. Here, we report a case of renal tubular acidosis RTA who presented with AIH. She was also diagnosed to have partial bowel malrotation. A 9-year-old girl, a case of distal RTA, presented with jaundice, abdominal distension and altered sensorium. She was diagnosed to be AIH, which was successfully treated with steroids and azathioprine. Coexistent midgut partial malrotation with volvulus was diagnosed during the treatment. She was treated successfully with anti-tuberculous treatment for cervical lymphadenitis. Autoimmune hepatitis should not be ruled out in each case of RTA presenting with jaundice.

How to cite this article: Modi TK, Parikh H, Sadalge A, Gupte A, Bhatt P, Shukla A. Autoimmune Hepatitis with Distal Renal Tubular Acidosis and Small Bowel Partial Malrotation. Euroasian J Hepato-Gastroenterol 2015;5(2):107-109.

Keywords: Autoimmune hepatitis; Malrotation; Renal tubular acidosis..

Fig. 1:

Liver biopsy showing dense portal and interface inflammation. (HE, 100x)

Fig. 2:

Closer view shows portal and interface inflammation with predominantly lymphocytes and plasma cells. Bile ductular proliferation is also noted. Hepatocytes show minimal steatosis (<10%). (HE, 400x)

Fig. 3:

Contrast enhanced computed tomography shows duodenojejunal flexure on right side of spine with jejunal loops (filled with iodinated contrast predominantly on right side (small arrow). There is evidence of twisting of mesenteric vessels surrounding small bowel (whirlpool sign); midgut volvulus (large arrow)

Fig. 4 :

Lymph node showing confluent epithelioid cell granulomas with Langhan’s giant cells and caseous necrosis (HE, 100×)