between the ages of 10 and 20 and 45 and 70 years and has To determine the clinical, biochemical, and histologia relatively benign course at least in adults, 3 whereas LKMcal features, and outcome of childhood autoimmune 1 positive patients have a more severe disease with progreshepatitis (AIH), we reviewed the medical records of 52 sion to cirrhosis despite immunosuppressive treatment. gest that ANA/SMA positive AIH has two peaks of incidence median age: 10.5, range 2.3-14.9 years). At presentation, four children were positive for ANA alone (titer range: 1:80-1:320), 10 for SMA alone (titer range: 1:40-1:2560) and 14 for both ANA (titer range: 1:20-1:5120) and SMA (titer range: 1:40-1:1280). In two paAbbreviations: AIH, autoimmune hepatitis; ANA/SMA, nuclear and/or smooth muscle

Susceptibility to autoimmune hepatitis in white patients is associated with the human leukocyte antigen class II antigens DR3 and DR4. To analyze the molecular basis of these associations, we used oligonucleotide probes to determine the DRB, DQA and DQB hypervariable nucleotide sequences in 119 patients with autoimmune hepatitis and 177 matched controls. DRB3*0101, which encodes DR52a, predisposed patients most strongly to the disease. It was present in 58% of patients and 25% of controls (corrected P < 0.000005), whereas DQA1*0101 and 0102 conferred protection in males only. The DR4 subtype, DRB1*0401, was raised in the DRB3*0101-negative patients; 81% possessed either DRB3*0101 or DRB1*0401, compared with 42% of controls (corrected P < 0.0000001). These alleles encode the amino acid sequence Leu-Leu-Glu-Gln-Lys-Arg at positions 67 to 72 of the DR beta polypeptide, which was present in 94% of patients and 64% of controls (corrected P < 0.000001) and in all patients who tested positive for autoantibodies to the hepatic asialoglycoprotein receptor. The patients with DRB1*0401 had less severe disease, relapsed less frequently and were first seen significantly later in life than those patients with DRB3*0101; and whereas a single copy of DRB1*0401 predisposed to autoimmune hepatitis, DRB3*0101-associated susceptibility had a dose-related effect. These data provide evidence that specific residues in the DR beta polypeptides predispose to autoimmune hepatitis in white patients and genes linked to DRB3*0101 and DRB1*0401 may determine two clinically distinct disease patterns.

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A total of 152 consecutive children with oesophageal varices have been endoscopically reviewed since 1979. In all, 108 of these children presented with variceal bleeding which was managed by injection sclerotherapy. Variceal obliteration was achieved in 33 (92 per cent) children with extrahepatic portal hypertension and 54 (75 per cent) with intrahepatic portal hypertension. Prophylactic injection sclerotherapy was used to obliterate large varices in 11 children with no history of haemorrhage. Bleeding episodes occurred in 38 (39 per cent) children before variceal obliteration was complete. However, the mortality rate from variceal bleeding was only 1 per cent. Complications were oesophageal ulceration (29 per cent) and stricture (16 per cent) which both resolved with conservative management. During a mean follow-up period of 2.9 years after sclerotherapy, recurrent oesophageal or gastric varices developed in 12 (12 per cent) cases, with rebleeding in 9 (9 per cent), but all responded successfully to a second course of treatment. These results are superior to contemporary surgical management and injection sclerotherapy should therefore currently be the primary treatment of choice for bleeding oesophageal varices in children.

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