Partial Splenic Embolization: Successful treatment of hypersplenism, secondary to biliary cirrhosis and portal hypertension in cystic fibrosis

AbstractPartial Splenic Embolization (PSE) is a non-surgical treatment for hypersplenism. It has been reported only in a limited number of patients with Cystic Fibrosis (CF). We report a case of a female cystic fibrosis patient who developed hypersplenism at the age of 14 and underwent PSE. Long term results over a period of 14 years after the procedure are presented

Adiponectin and body composition in cystic fibrosis

AbstractThe aim of the study was to evaluate adiponectin (AD) serum concentrations in 43 stable CF patients and 27 healthy subjects and to correlate them with their nutritional status. Body Composition (Bioelectrical Impedance), visceral/subcutaneous adipose tissue (VAT-SAT) in CF patients (CT-scan at L4), insulin resistance (HOMA-IR) and AD serum concentrations (ELISA) were studied. CF patients and controls had comparable weight, height, %BF, %FFM, fasting glucose, insulin and insulin resistance. CF patients had significantly lower BMI-SDS. CF males had higher %FFM and total FFM and lower %BF and total BF than females (p<0.001). Serum AD was higher in CF patients than controls (11.53±5.37 vs. 9.07±4.41 μg/ml) and comparable between females and males. AD was lowest among young malnourished patients (8.06±1.85 μg/ml) and highest among young patients with normal nutrition (14.56±7.69 μg/ml). Patients with biliary cirrhosis had higher levels than patients with normal liver (10.52±5.49 vs. 14.04±4.52 μg/ml, p<0.05). AD correlated with %BF, %FFM, FFM (kg) (p<0.05).VAT was significantly increased in malnourished patients. AD was not affected by VAT. Conclusions: Adiponectin is higher in CF patients than healthy individuals. It is decreased in malnourished young patients and increased in patients with normal nutrition and in patients with liver disease. This may be attributed to the reduced BF and to the energy deficit inherent to the disease

Patients With Celiac Disease Have an Increased Risk for Pancreatitis

Background & Aims Patients with celiac disease have been reported to be at increased risk for pancreatitis and pancreatic insufficiency, but the risk might have been overestimated because of patient selection and limited numbers of patients for analysis. Furthermore, no distinction has been made between patients with gallstone-related and non–gallstone-related pancreatitis. We performed a nationwide study to determine the risk for any pancreatitis or subtype of pancreatitis among patients with biopsy-verified celiac disease. Methods We analyzed data from patients in Sweden with celiac disease (n = 28,908) who were identified on the basis of small intestinal biopsy records from 28 pathology departments (those with villous atrophy, Marsh 3). Biopsies were performed from 1969 to 2008, and biopsy report data were collected from 2006 to 2008. Patients with pancreatitis were identified on the basis of diagnostic codes in the Swedish Patient Register and records of pancreatic enzyme use in the Swedish Prescribed Drug Register. Data were matched with those from 143,746 individuals in the general population; Cox regression was used to estimate hazard ratios (HRs) for pancreatitis. Results We identified 406 patients with celiac disease who were later diagnosed with pancreatitis (and 143 with expected pancreatitis) (HR, 2.85; 95% confidence interval [CI], 2.53–3.21). The absolute risk of any pancreatitis among patients with celiac disease was 126/100,000 person-years, with an excess risk of 81/100,000 person-years. The HR for gallstone-related acute pancreatitis was 1.59 (95% CI, 1.06–2.40), for non–gallstone-related acute pancreatitis HR was 1.86 (95% CI, 1.52–2.26), for chronic pancreatitis HR was 3.33 (95% CI, 2.33–4.76), and for supplementation with pancreatic enzymes HR was 5.34 (95% CI, 2.99–9.53). The risk of any pancreatitis within 5 years of diagnosis was 2.76 (95% CI, 2.36–3.22). Conclusions Based on an analysis of medical records from Sweden, patients with celiac disease have an almost 3-fold increase in risk of developing pancreatitis, compared with the general population

Genetic Determinants and Epidemiology of Cystic Fibrosis–Related Diabetes: Results from a British cohort of children and adults

OBJECTIVE—Longer survival of patients with cystic fibrosis has increased the occurrence of cystic fibrosis–related diabetes (CFRD). In this study we documented the incidence of CFRD and evaluated the association between mutations responsible for cystic fibrosis and incident CFRD, while identifying potential risk factors