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The HLA‐A and B phenotypes of 42 Greek children with CDH were determined and compared to those of 400 controls. A significant deviation of the frequency of the antigen HLA‐A1, (Pc < 0.003) was noted. It is suggested that immunogenetic factors are contributing to the development of the disease.

Correspondence 945dosage. In all patients, blood pressure, pulse pressure, and pulse rate remained essentially unchanged and thyroid function tests were confirmatory of drug compliance.The very nature of this trial demands that the results be interpreted with caution. Nonetheless, certain observations lead us to believe that therapy with T3 was the factor responsible for the remissions experienced by our patients. In the first place, every one of them described a substantial, if not dramatic, improvement in their condition. Furthermore, this improvement occurred during the winter months when attacks would be expected to be most frequent and severe. Indeed, one patient (No 1) spontaneously commented that 'this was the best winter she could remember'. Finally, there was coexistent biochemical evidence of strict compliance.Our A 62 year old woman was admitted to hospital because of fever and chills. Two weeks before admission she developed increasing fatigue, persistent sore throat with chills and fever reaching 39-4°C, night sweats, malaise, weight loss, pain in her left knee, and a morbilliform rash which in five days assumed an urticarial appearance. On admission, a painful tender left knee and oedematous dusky erythema on the periorbital region were noticed. Her temperature was 39C, pulse 95 beats/min, and the blood pressure 125/80-mmHg. The rest of the systematic examination was unremarkable. A tentative clinical diagnosis of dermatomyositis was made.Laboratory investigations showed erythrocyte sedimentation rate 100 mm/h, leucocytes 13-8x109/l with a shift to the left (total granulocytes 90% and lymphocytes 10%), and packed cell volume 40%. Alkaline phosphatase was more than 200 SIU (normal<75 SIU). Serum aspartate transaminase 126 U/l (normal<27 U/1), serum alanine transaminase 117 U/l (normal<30 U/l), lactic dehydrogenase 290 U/I (normal<290 U/1), and y-glutamyl transferase 224 U/I (normal<30 U/1). The following were normal or negative: renal function studies, bilirubin, hepatitis B surface antigen, heterophil agglutinins, creatine phosphokinase, aldolase, amylase, thyroid function tess, rheumatoid factor, antinuclear antibodies, antimitochondrial antibodies, smooth muscle antibodies, serum complement levels, cultures from throat, urine, and blood, tuberculin skin test, stool specimen, chest x rays, electrocardiogram, electromyogram (EMG), upper gastrointestinal study, intravenous pressure, ultrasonographic study, and the computed tomographic scan of the abdomen. A muscle biopsy showed typical changes of fragmented and degenerated muscle fibres in a background of fibrous tissue heavily infiltrated by leucocytes (Fig. 1).Three weeks later pyrexia continued and the patient developed jaundice with pruritus and ascites. Her condition deteriorated, she had a massive haematemesis, and died. The postmortem examination showed an anaplastic adenocarcinoma of the ampulla of Vater (diameter 1-5 cm). Liver histology showed acute cholestasis.