IgA deficiency: clinical correlates with IgG subclass and mannan-binding lectin deficiencies

OBJECTIVE: To characterize an IgA deficient population in terms of the incidence of IgG subclass and mannose-binding lectin (MBL) deficiencies and the type and severity of infections and other associated disorders. BACKGROUND: Selective IgA deficiency is probably the commonest of the primary immunodeficiency disorders and although it may lead to an increased risk for respiratory and gastrointestinal infections and associated to various autoimmune diseases, it may also be asymptomatic. Several studies have suggested the need of a concomitant defect in order for manifestation of its symptoms. METHODS: A total of 27 patients fulfilling the diagnostic criteria of selective IgA deficiency were evaluated for IgG subclass and MBL deficiencies after a thorough medical history, physical examination and pertinent evaluation for concomitant medical conditions. RESULTS: The overall incidence of IgG subclass deficiency found in the IgA deficient group was 18.5. MBL deficiency was found to be 3.7. These frequencies may have been influenced by the age group evaluated and the size of the population studied. Severe infections were more common in patients with combined deficiencies, either IgA and any of the IgG subclasses or IgA and MBL deficiency. Atopy was widely represented in the patients studied. CONCLUSIONS: The observed relationship between combined deficiencies Ig A, IgG subclasses and MBL and the increased representation of severe infections needs to be corroborated in a larger sample of patients with an inclusion of pediatric patients.

Composicion quimica de calculos biliares en Santa Cruz - Bolivia / Chemical composition of gallstones in Santa Cruz - Bolivia

The knowledge of the gallstones chemical composition is very important from view point of lithogenesis and possible medical therapy of dissolution. The composition (chemical) of gallstones in 30 consecutive colecistectomized patientes were studied in the 2nd. Departament of Surgery of Hospital San Juan de Dios, Santa Cruz, Bolivia (altitude 1900 m). All gallbladder stones wee washed, counted, measured and dried before sending them to Japan to be processed. The composition of gallstones was analyzed by infrared absorption spectroscopy. Cholesterolemia, age, sex and number of stones were analyzed in cases of pure cholesterol stones. The incidence of pure cholesterol stones was 56,6%, statistically similar to be reported by other authors, but very low if compared with the valley area of Bolivia (Cochabamba altitude 2300m) where hypothyroidism is frequent. Hypercholesterolemia had little relation with the pure cholesterol gallstones. There was no difference in the chemical composition and the size of the stones, but while their number was small, cholesterol stones were more frequent. The low incidence of pure cholesterol stones may show a bad nutritional condition of our population (poor fat and protein content) and this results is important in order in using the new advance: lithodissolvent drugs