Polyendocrine syndrome type 3C in a family from Pakistan.

In type 3 polyendocrine syndrome (PAS3), autoimmune thyroiditis occurs with other organ-specific autoimmune disease, but not with autoimmune adrenalitis. In this report we described a family from Pakistan in which mother and three daughters were affected by a PAS3. We studied a family from Pakistan: Father MMu age 44, mother KN aged 44, three daughters MM age 20, MH age 16 and MA age 14 and a son MU age 18. These subjects were tested for thyroids function, metabolic function, adrenal function, autoimmune disease. In this family the four females were shown hypothyroidism with presence of anti thyroid autoantibodies (AA) and high TSH serum concentration in association with the presence of anti transglutaminase AA. Moreover KN, MM and MH were positive for anti nuclear AA (granular pattern) and for antibodies against Saccaromyces cerevisiae. MM was positive for AA against nuclear extractable antigens (SSA and SSB) too. No diabetes or pernicious anemia were observed. Adrenal and Pituitary function were normal. PAS 3C is an uncommon disease. In this family from Pakistan we observed a PAS3C in the four female members: mother and three daughters while father and son were unaffected.

Rehabilitation Programs for Cystic Fibrosis - View from a CF Center.

BACKGROUND: Rehabilitation programs are comprehensive interventions which effectively improve the health status and reduce costs in chronic respiratory illnesses. Because patients with cystic fibrosis have been discouraged to participate for concerns of microbial cross infection, the efficacy of systematic rehabilitation is unknown for this group. METHODS: We retrospectively studied 142 cystic fibrosis patients aged 2-46 years who participated in rehabilitation programs taking place in Germany/Switzerland and in Israel, focusing on changes in lung function and weight. RESULTS: During 172 stays in 97 patients in Israel and 68 stays in 45 patients rehabilitating in Germany/Switzerland, overall lung function and weight improved. Outcome did not differ between Israel and German/Swiss sites. Interestingly, lung function improved during the initial phase of the stay, whereas weight gain was sustained throughout. The study uncovered gaps in reporting sufficient individual outcome information back to the admitting centre. CONCLUSIONS: Rehabilitation programs specified for cystic fibrosis patients need to be assessed prospectively to optimize treatment of this life limiting condition.

Plasma adiponectin levels in primary biliary cirrhosis: a novel perspective for link between hypercholesterolemia and protection against atherosclerosis.

INTRODUCTION: Hypercholesterolemia is a common finding in primary biliary cirrhosis (PBC), but the risk of cardiovascular events in PBC patients is not increased in respect to the general population. High serum adiponectin levels appear to play a protective role in the development of either metabolic syndrome or cardiovascular disease. AIM: To investigate factors potentially preventing atherosclerosis in PBC patients. METHODS: Circulating levels of adiponectin, resistin, leptin, and tumor necrosis factor-alpha (TNF-alpha) were measured in 137 consecutive PBC patients (125 women, 12 men; mean age 61.6 +/- 12.3 yr), 137 sex- and age-matched healthy controls, and 30 female patients with nonalcoholic steatohepatitis (NASH) and associated metabolic syndrome. RESULTS: The body mass index (BMI) was comparable in the three groups, whereas total cholesterol was significantly higher in both PBC and NASH cases than in controls (221.6 +/- 50.5 mg/dL in PBC vs 221.7 +/- 39.7 mg/dL in NASH vs 209.8 +/- 39.2 mg/dL in controls, P < 0.05). Serum concentrations of adiponectin, resistin, and leptin were significantly higher in PBC patients than in either NASH cases or controls (P < 0.05). Among the PBC patients, only adiponectin correlated positively with histological progression of the disease (P= 0.001) and negatively with BMI (P= 0.01). Logistic regression analysis revealed that adiponectin correlated independently with age, BMI, Mayo score, and gamma-glutamyl transpeptidase. CONCLUSIONS: The high adiponectin concentrations observed in PBC patients should be regarded as a possible protective factor against atherogenesis. The search for further protective factors should be encouraged.

An outbreak of febrile gastroenteritis associated with corn contaminated by Listeria monocytogenes.

BACKGROUND: On May 21, 1997, numerous cases of febrile gastrointestinal illness were reported among the students and staff of two primary schools in northern Italy, all of whom had eaten at cafeterias served by the same caterer. METHODS: We interviewed people who ate at the cafeterias about symptoms and foods consumed on May 20. There were no samples of foods left at the cafeterias, but we tested routine samples taken on May 20 by the caterer and environmental specimens at the catering plant. The hospitalized patients were tested for common enteropathogens and toxins. RESULTS: Of the 2189 persons interviewed (82 percent of those exposed), 1566 (72 percent) reported symptoms; of these, 292 (19 percent) were hospitalized. Among samples obtained from hospitalized patients, all but two of the stool specimens and all blood specimens were negative for common enteropathogens. Listeria monocytogenes was isolated from one blood specimen and from 123 of the 141 stool specimens. Consumption of a cold salad of corn and tuna was associated with the development of symptoms (relative risk, 6.19; 95 percent confidence interval, 4.81 to 7.98; P<0.001). L. monocytogenes was isolated from the caterer's sample of the salad and from environmental specimens collected from the catering plant. All listeria isolates were serotype 4b and were found to be identical on DNA analysis. Experimental contamination of sterile samples of the implicated foods showed that L. monocytogenes grew on corn when kept for at least 10 hours at 25 degrees C. CONCLUSIONS: Food-borne infection with L. monocytogenes can cause febrile illness with gastroenteritis in immunocompetent persons.

[The use of nefopam in the prophylaxis and treatment of postoperative shivering]. / Impiego del nefopam nella profilassi e nella terapia del brivido postoperatorio.

Chlorhydrate nefopam was used in the prophylaxis and treatment of postoperative shivering in 54 patients undergoing general anesthesia for radical cystectomy with trans-intestinal anastomosis. Postoperative shivering was not observed in any of the patients treated with nefopam before coming round, whereas it occurred in 55% of patients treated with placebo. Chlorhydrate nefopam subsequently stopped shivering in all these patients. The main side effects observed took the form of delayed awakening in 11% of patients receiving prophylactic treatment and somnolence lasting 5-10 minutes in all other patients.