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SUMMARY The frequency of HLA antigens was studied in 25 patients with primary sclerosing cholangitis and compared with a control group of 561 kidney donors. Fourteen patients also had ulcerative colitis. A significant increase in the frequency of HLA-B8 (60%) was found in the primary sclerosing cholangitis patients compared with controls (25%) (p<0-001). HLA-B8 was found in eight patients with ulcerative colitis. The frequency of HLA-B12 was significantly decreased (8%) compared with controls (30%) (p<0-02). Piecemeal necrosis was observed on liver histology in 66% of HLA-B8 positive and 50% of HLA-B8 negative patients. Low titres of serum autoantibodies were frequently found in the primary sclerosing cholangitis group but did not correspond to the presence of HLA-B8. Raised serum concentrations of IgM and IgG were not related to HLA-B8. This study has shown that in patients with primary sclerosing cholangitis there exists a disease susceptibility gene closely associated with the B locus of the major histocompatibility complex which may be modified by other factors such as ulcerative colitis. Patients with ulcerative colitis and HLA-B8 may be particularly liable to develop primary sclerosing cholangitis.Primary sclerosing cholangitis is an uncommon disease characterised by an intense inflammatory fibrosis usually involving the whole biliary system.'The aetiology of this disorder is unknown, although it is closely associated with ulcerative colitis which coexists in approximately two-thirds of patients with primary sclerosing cholangitis.'The associations between certain human leucocyte histocompatibility (HLA) antigens and an increased risk of certain diseases have recently been identified. HLA-B8 and HLA-DRW3, for example, have been closely associated with diseases involving immunological dysfunction such as autoimmune chronic active hepatitis.2 HLA-A3 has been closely associated with idiopathic haemochromatosis.3The purpose of the present study was to investigate possible associations between the HLA system and patients with primary sclerosing cholangitis, with and without coexisting ulcerative colitis. ' Address for correspondence: Dr R W Chapman, John Radcliffe Hospital, Headington, Oxford. Received for publication 13 April 1982 Methods PATIENTS Twenty-five consecutive patients (17 men and eight women) with primary sclerosing cholangitis who were admitted to the Royal Free Hospital for investigation were studied. The diagnosis was based on accepted criteria -namely, the demonstration of multiple strictures in the biliary system, the absence of previous biliary tract surgery, and the exclusion of bile duct cancer by at least two years' follow-up

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The relative roles of hepatic lipase and lipoprotein lipase in the pathogenesis of uremic lipid abnormalities were studied in 92 hemodialysis patients. Fasting serum cholesterol, triglyceride, and HDL-cholesterol concentrations were measured. Plasma lipoprotein electrophoretic patterns were determined in all patients. Hepatic lipase and lipoprotein lipase activities were selectively measured in post-heparin plasma in 59 patients. Hemodialysis patients had higher serum triglyceride and lower HDL-cholesterol concentrations than did their age and sex-matched control subjects. Both hepatic and lipoprotein lipase activities were reduced in hemodialysis patients. An inverse relation between lipoprotein lipase activities and serum triglyceride concentrations emerged. Lipoprotein lipase activities correlated with in vivo post-heparin fractional clearance rates of Intralipid. A positive correlation between lipoprotein lipase activities and HDL-cholesterol concentrations probably reflected impaired catabolism of triglyceride-rich lipoproteins being responsible for the low HDL-cholesterol concentrations. Hemodialysis patients (41.3%) had an abnormal lipoprotein (the 'mid-band'). While hepatic lipase activities did not correlate with any parameters of lipid metabolism, patients with 'low' hepatic lipase activities had a significantly higher prevalence of 'mid-bands' than did those with 'normal' activities. No evidence was developed to prove that the 'mid-band' lipoproteins were remnant particles.

It has recently been established that the rate of progression of chronic renal failure in man can be slowed by restricting dietary protein. Consequently, the short term and long term effects of a low protein diet on the course of different chronic nephropathies were studied in an attempt to delineate the factors that determine the response to such a diet. When a low protein diet was given for six months renal function improved significantly in nine patients with chronic tubulointerstitial nephritis (p <0 025); the diet had a marginally beneficial effect in 12 patients with chronic glomerulonephritis (p <0 05) and no effect in nine with hypertensive nephrosclerosis. The heterogenous functional response in the patients with chronic glomerulonephritis correlated closely with the effect of the diet on these patients' proteinuria (r=0 76, p <0 01). In a short term study (four weeks) of 12 patients with chronic renal failure changes in renal plasma flow were proportional to dietary protein intake. Renal vascular resistance fell during a high protein diet and increased when dietary protein was restricted. The changes in renal plasma flow during the low protein diet correlated well with the patients' long term functional response to the diet (r-0076, p <0 01).It is concluded that the response to a low protein diet in chronic renal failure is determined, firstly, by the nature of the underlying nephropathy, with maximal benefit being observed in non-glomerular disorders;