Complete deficiency of the complement C4A isotype is a known genetic risk factor for systemic lupus erythematosus (SLE). The disease phenotype of C4A-deficient patients has never been defined. Among ...200 patients with SLE from five centers, 18 (9%) with C4A deficiency were identified. These individuals were compared to those who were C4A replete with regard to a series of clinical and serologic features. The only significant differences between the two groups were in the presence of renal disease (C4A deficient, 11%; C4A replete, 46%; P < 0.006) and a decrease in the serum concentrations of C3 (C4A deficient, 11%; C4A replete, 35%; P < 0.04). There was also a trend for the C4A-deficient individuals to have milder disease. In light of the tendency for C4A-deficient individuals to have lower serum concentrations of C4, it is important that such patients not be subjected to overly aggressive efforts to "normalize" their C4 levels.
A prospective controlled study of the diagnostic accuracy of blind percutaneous liver biopsy in comparison to laparoscopy plus guided biopsy for the recognition or exclusion of cirrhosis has been ...performed. One hundred twenty-six patients with a clinical diagnosis of chronic, diffuse, well-compensated liver disease were randomized into two groups and submitted either to percutaneous blind liver biopsy (PB: 64 patients) or to laparoscopy with guided biopsy (LB: 62 patients), in order to assess the accuracy of either procedure in diagnosing cirrhosis. PB correctly recognized or ruled out cirrhosis in 52 patients (82%). Inconclusive results were mostly false negative, as demonstrated by the presence on endoscopy of esophageal varices or by subsequent LB. LB demonstrated presence or absence of cirrhosis in all patients. The difference between the rate of accurate results of the two procedures is statistically significant. It is concluded that in patients without esophageal varices, LB should be the investigation of choice for the assessment of liver structure since the presence of cirrhosis can be missed in up to 20% of cases by PB.
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