Low rate of production of apolipoproteins B100 and AI in 2 patients with Anderson disease (chylomicron retention disease).

OBJECTIVE: Anderson disease is a rare inherited lipid malabsorption syndrome associated with hypocholesterolemia and linked to SAR1B mutations. The aim of this article was to analyze the mechanisms responsible for the low plasma apolipoprotein Apo-B100 and Apo-AI in 2 patients with Anderson disease. METHODS AND RESULTS: A primed constant infusion of (13)C-leucine was administered for 14 hours to determine the kinetics of lipoproteins. In the 2 patients, total cholesterol (77 and 85 mg/dL versus 155±32 mg/dL), triglycerides (36 and 59 versus 82±24 mg/dL), Apo-B100 (48 and 43 versus 71±5 mg/dL), and Apo-AI (47 and 62 versus 130±7 mg/dL) were lower compared with 6 healthy individuals. Very-low-density lipoprotein-B100 production rate of the patients was lower (4.08 and 5.52 mg/kg/day versus 12.96±2.88 mg/kg/day) as was the fractional catabolic rate (5.04 and 4.32 day(-1) versus 12.24±3.84 day(-1)). No difference was observed in intermediate-density lipoprotein-B100 and LDL-B100 kinetic data. The production rate of high-density lipoprotein Apo-AI was lower in the patients (7.92 and 8.64 versus 11.96±1.92 mg/kg/day) and the fractional catabolic rate was higher (0.38 and 0.29 versus 0.22±0.01 day(-1)). CONCLUSIONS: The low plasma Apo-B100 and Apo-AI concentrations in the patients with Anderson disease were mainly related to low rates of production.

Predicting mortality in systemic Wegener's granulomatosis: a survival analysis based on 93 patients.

OBJECTIVE: To determine predictors of survival of patients with systemic Wegener's granulomatosis (WG). METHODS: We retrospectively studied 93 patients (median age 52 years, male/female ratio 1.7) with systemic WG. All subjects received cytotoxic drugs. Survival was evaluated as a function of the main clinical and laboratory parameters and 2 disease activity scores assessed at diagnosis. Statistical analyses used the multivariate Cox proportional hazards regression model. RESULTS: The mean followup was 4.5 years; 25 (27%) patients died. According to univariate analysis, a pejorative prognostic value was attributed to serum creatinine >160 micromole/liter (P < 0.001); age >52 years (P < 0.002); absence of ear, nose, and throat (ENT) involvement (P < 0.001); and hemoglobin < or =11.8 gm/dl (P = 0.02). Multivariate analysis retained age >52 years (hazard ratio [HR] = 3.40, P = 0.04) as an independent predictor of poor outcome, whereas the presence of ENT involvement was associated with a longer survival (HR = 0.31, P = 0.02). CONCLUSION: Our results suggest that an older age and the absence of ENT involvement at diagnosis independently predict an increased risk of mortality for WG patients. These findings could indicate that the prognosis of WG might be governed by the balance between the granulomatosis as opposed to the vasculitic disease process.

[Clostridium difficile infection in a Department of Internal Medicine. A consecutive series of 45 patients]. / L'infection à Clostridium difficile dans un service de Médecine Interne.

OBJECTIVES AND METHODS: A retrospective study of 45 patients with Clostridium difficile infection over a 4-year period in a department of Internal Medicine. RESULTS: Mean age was 79 years; sex-ratio (F/M)=1.5; 38% of the patients had neurological or severe psychiatric disorders; 20% had a neoplastic disease. Ninety-three percent of cases had received one or more antibiotics before onset of diarrhea, prescribed mainly for a pulmonary infection. Amoxicillin clavulanic acid and cephalosporins were the most frequently used treatments, respectively in 48% and 40% of cases. For 25 patients (56%) Clostridium difficile-associated diarrhea was considered as a nosocomial infection, and as community-acquired diarrhea in 20 cases (44%). Treatment included isolation of the patient as soon as bacteriological diagnosis was known and specific therapy was instituted by metronidazole or vancomycin for a mean of 18 days. The addition of Saccharomyces boulardii was used in of cases. The clinical course was rapidly favorable for 80% of patients. Five patients died with complications of severe colitis in 2 cases. Mean hospital stay was 49 days (annual mean of the department=10 days). CONCLUSION: Clostridium difficile diarrhea concerns above all elderly patients with one or more underlying pathologies. Amoxicillin clavulanic acid and third-generation cephalosporins are the most frequently prescribed antibiotics in these cases and have the highest correlation with this infectious complication. This medical problem requires greater knowledge as it causes significant morbidity and increases the risk of prolonged hospital stays.