Epidemiologic study, level V.

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AIM:To determine the merits of magnetic resonance cholangiopancreatography (MRCP) as the primary diagnostic test for choledochal cysts (CC's). METHODS:Between 2009 and 2012, patients who underwent MRCP for perioperative diagnosis were identified. Demographic information, clinical characteristics, and radiographic findings were recorded. MRCP results were compared with intraoperative findings. A PubMed search identified studies published between 1996-2012, employing MRCP as the primary preoperative imaging and comparing results with either endoscopic retrograde cholangiopancreatography (ERCP) or operative findings. Detection rates for CC's and abnormal pancreaticobiliary junction (APBJ) were calculated. In addition detection rates for clinically related biliary pathology like choledocholithiasis and cholangiocarcinomas in patients diagnosed with CC's were also evaluated. RESULTS:Eight patients were identified with CC's. Six patients out of them had type Ⅳ CC's, 1 had type I and 1 had a new variant of choledochal cyst with confluent dilatation of the common bile duct (CBD) and cystic duct. Seven patients had an APBJ and 3 of those had a long common-channel. Gallstones were found in 2 patients, 1 had a CBD stone, and 1 pancreatic-duct stone was also detected. In all cases, MRCP successfully identified the type of CC's, as well as APBJ with ductal stones. From analyzing the literature, we found that MRCP has 96%-100% detection rate for CC's. Additionally, we found that the range for sensitivity, specificity, and diagnostic accuracy was 53%-100%, 90%-100% and 56%-100% in diagnosing APBJ. MRCP's detection rate was 100% for choledocholithiasis and 87% for cholangiocarcinomas with concurrent CC's. CONCLUSION:After initial ultrasound and computed tomography scan, MRCP should be the next diagnostic test in both adult and pediatric patients. ERCP should be reserved for patients where therapeutic intervention is needed.© 2013 Baishideng. All rights reserved.Key words: Magnetic resonance cholangiopancreatography; Choledochal cyst; Abnormal pancreaticobiliary junction; Diagnostic test; Choledocholithiasis; Cholangiocarcinomas Core tip: Magnetic resonance cholangiopancreatography (MRCP) is used as primary diagnostic approach in various biliary pathologies. This is the first literature review of published studies discussing MRCP as a diagnostic modality for choledochal cysts. This review further outlines how recent imaging techniques have improved diagnostic accuracy of MRCP in diagnosing choledochal cysts and their associated anatomic variants. Advantages, disadvantages and contraindication for MRCP with respect to endoscopic retrograde cholangiopancreatography are also discussed.

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Summary. Congenital factor XIII (FXIII) deficiency is an extremely rare, potentially life-threatening bleeding disorder. Routine prophylactic management is recommended for individuals with clinically relevant FXIII deficiency. This prospective, multicentre, openlabel study evaluated the long-term efficacy and safety of prophylactic infusions of FXIII concentrate (human) 40 IU kg À1 in patients with congenital FXIII deficiency. FXIII concentrate (human) was administered every 4 weeks for 12 months. Dosing was adjusted to maintain trough FXIII activity levels of 5-20%. Logistical and ethical constraints precluded use of a placebo control group. Annualized incidence of spontaneous bleeding was compared with historical rates; safety was assessed as a secondary objective. Forty-one patients were enrolled and completed the study. The annualized rate for spontaneous bleeding episodes requiring FXIII treatment was 0.000 episodes per patient-year (95% CI: 0.000; 0.097). The study met its primary endpoint: the upper limit of the 95% CI was substantially below the historical rate of 2.5 bleeding episodes per patient-year. Five spontaneous bleeding episodes (involving three patients; none requiring FXIII treatment) and eight trauma-related bleeding episodes (two requiring FXIII treatment) occurred. Five patients had surgery during the study, only one of whom required FXIII treatment for post-surgical bleeding. Most patients (≥85%) had trough FXIII activity levels ≥10%. No patient discontinued treatment due to an adverse event. No adverse events related to thromboembolism or viral transmission were reported. Prophylactic treatment with FXIII concentrate (human) was well tolerated and prevented spontaneous bleeding episodes that were serious enough to require treatment with FXIII-containing product. Clinical trial registration: www.clinicaltrials.gov/ct2/show/NCT00885742.

Lack of restraint use in the pregnant population is associated with increased MD. Although not validated in the pregnant population, the ISS and RTS were associated with maternal mortality outcomes.