Via biliar y páncreas

OBJECTIVES: The aim of this study was to determine if comprehensive genetic testing was useful to identify genetic variants that discriminate chronic pancreatitis (CP) from acute recurrent pancreatitis (ARP) in a pediatric population. METHODS: We conducted a retrospective review of 50 patients enrolled in our institutional pancreatitis registry between April 2013 and January 2015. Genetic analysis of PRSS1, CFTR, SPINK1, and CTRC classified variants as mutations or variants of unknown clinical significance and the minor allele frequency of variants in our cohort was obtained...

OBJECTIVES: To determine whether recommendations for treatment of acute pancreatitis (AP) in adults impact the outcomes of pediatric AP. STUDY DESIGN: Adult guidelines regarding early management of AP were implemented through an admission order set at Cincinnati Children's Hospital Medical Center at the beginning of the year 2014. Recommendations included administering high rates of intravenous fluid (IVF) within 24 hours of admission and enteral nutrition within 48 hours of admission...

BACKGROUND/OBJECTIVES: Approximately 15-20% of pediatric patients with acute pancreatitis (AP) develop severe disease. Severity scoring tools were developed for adult patients, but have limitations when applied in children. We aimed to identify early predictors of severe acute pancreatitis (SAP) on hospital admission for early risk stratification of patients. METHODS: Retrospective review of AP admissions was conducted. The derivation cohort included cases at Cincinnati Children's Hospital Medical Center (CCHMC) between 2009 and 2013...

Acute pancreatitis is an emerging problem in pediatrics, with an incidence that is rising in the last 2 decades. Data regarding the optimal management and physician practice patterns are lacking. We present a literature review and updates on the management of pediatric pancreatitis. Prospective multicenter studies defining optimal management of pediatric pancreatitis are needed to guide care and improve outcomes for this patient population.

BACKGROUND: Biliary pancreatitis (BP) is common in adults and children. Current standard of care is to perform a cholecystectomy (CCE) to decrease the recurrence risk of pancreatitis. Controversy exists as to the timing of surgery, early versus delayed surgical intervention. Adult literature suggests a greater benefit of early CCE. Comparatively, there is limited pediatric literature as to the optimal timing of a CCE in children. We report a retrospective case series of children with BP who underwent early versus late CCE...

Nutrition is an integral part of acute pancreatitis (AP) management and is not adequately studied in pediatrics. The goal from the present study was to evaluate the effect of nutrition and fat content on the length of stay and pain severity in patients with AP. This is a retrospective review of our nutrition database between May 1, 2014 and December 1, 2014. Pain levels were similar between patients who were allowed to feed and patients kept nill per os. Higher fat intake grams per kilogram per day was associated with significantly lower pain scores...

IMPORTANCE: Pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) are poorly understood. OBJECTIVE: To characterize and identify risk factors associated with ARP and CP in childhood. DESIGN, SETTING, AND PARTICIPANTS: A multinational cross-sectional study of children with ARP or CP at the time of enrollment to the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) study at participant institutions of the INSPPIRE Consortium...

BACKGROUND: Approximately 10% of patients with acute pancreatitis (AP) progress to chronic pancreatitis. Little is known about the factors that affect recurrence of pancreatitis after an initial episode. We retrospectively investigated patients with AP, focusing on their outcomes and the predictors for disease progression. METHODS: Between July 2003 and June 2015, we retrospectively enrolled first-time AP patients with medical records on disease etiology, severity (according to the Atlanta classifications), and recurrence of AP...

OBJECTIVES: To prospectively assess the value of serum total bilirubin (TB) within 3 months of hepatoportoenterostomy (HPE) in infants with biliary atresia as a biomarker predictive of clinical sequelae of liver disease in the first 2 years of life. STUDY DESIGN: Infants with biliary atresia undergoing HPE between June 2004 and January 2011 were enrolled in a prospective, multicenter study. Complications were monitored until 2 years of age or the earliest of liver transplantation (LT), death, or study withdrawal...

BACKGROUND: The etiologies of conjugated hyperbilirubinemia in infancy are diverse. OBJECTIVE: Determine the prevalence rates of the specific etiologies of conjugated hyperbilirubinemia in infancy. DATA SOURCES: EMBASE and Pubmed were searched electronically and the bibliographies of selected studies were search manually. The search was conducted independently by two authors. STUDY SELECTION: (1) prospective or retrospective case series or cohort study with 10 or more subjects; (2) consecutive infants who presented with conjugated hyperbilirubinemia; (3) subjects underwent appropriate diagnostic work-up for conjugated hyperbilirubinemia; (4) no specific diagnoses were excluded in the studied cohort...

AIMS OF THE STUDY: Laparoscopic simulation has transformed skills acquisition for many procedures. However, realistic nonbiological simulators for complex reconstructive surgery are rare. Life-like tactile feedback is particularly difficult to reproduce. Technological innovations may contribute novel solutions to these shortages. We describe a hybrid model, harnessing 3D technology to simulate laparoscopic choledochal surgery for the first time. METHODS: Digital hepatic anatomy images and standard laparoscopic trainer dimensions were employed to create an entry level laparoscopic choledochal surgery model...

BACKGROUND: Hemobilia in children is a rare phenomenon which has been described mostly in the context of traumas. The descriptions of massive hemobilia in children after liver biopsy are a rarity in the scientific literature because there are only a few examples of it. Hemobilia rarely develops spontaneously. Generally, this is a complication after a blunt abdominal trauma or after medical (especially surgical) procedures. Correct diagnosis and treatment of hemobilia are essential, especially in the case of patients with severe - sometimes life-threatening - haemorrhage from biliary ducts...

BACKGROUND/PURPOSE: Surgical intervention that improves pancreatic ductal drainage is a reasonable treatment strategy for recurrent pancreatitis in children. METHODS: This study was approved by the Committee on Human Research (San Francisco, CA). A retrospective chart review was performed on children aged 0 to 17 years given the International Classification of Diseases, Ninth Revision coding diagnosis of chronic pancreatitis who underwent surgical intervention from 1981 to 2005...

PURPOSE: The aim of this study was to evaluate the role of longitudinal pancreaticojejunostomy (modified Puestow procedure) in the treatment of complicated hereditary pancreatitis (HP) in children. METHODS: The authors reviewed their experience with the modified Puestow procedure for complicated HP in patients less than 18 years of age at a single tertiary care facility between 1973 and 1998. Main study outcomes included surgical morbidity and mortality, pre- and postoperative pancreatic function, number of hospitalizations, and percentile ideal body weight (IBW)...

PURPOSE: To present our experience with the modified Puestow procedure in the management of children with chronic pancreatitis. METHODS: Retrospective chart review of patients treated between 2003 and 2012. RESULTS: Six patients underwent a modified Puestow procedure (lateral pancreaticojejunostomy) for the management of chronic pancreatitis, three females and three males. Four patients had hereditary pancreatitis (three with confirmed N34S mutation in the SPINK1 gene), one patient had chronic pancreatitis of unknown etiology, and one patient with annular pancreas developed obstructive chronic pancreatitis...

An interventional radiologist is frequently called to evaluate and treat biliary diseases in children; a tailored approach specific to this population is required. Imaging with an emphasis on minimizing ionizing radiation is used not only in the initial workup but also to guide interventions. The most common form of intervention generally consists of transhepatic biliary drainage to treat either biliary obstruction or bile leakage, a scenario frequently encountered after pediatric liver transplantation. Other pathologies referred for evaluation and management include biliary atresia and, rarely, symptomatic choledochal cysts...

This review is based upon an invited lecture for the 52nd Annual Meeting of the British Association of Paediatric Surgeons, July 2015. The aetiology of biliary atresia (BA) is at best obscure, but it is probable that a number of causes or pathophysiological mechanisms may be involved leading to the final common phenotype we recognise clinically. By way of illustration, similar conditions to human BA are described, including biliary agenesis, which is the normal state and peculiar final pattern of bile duct development in the jawless fish, the lamprey...

AIM OF THE STUDY: To report the outcomes of children who underwent Sengstaken-Blakemore tube (SBT) insertion for life-threatening haemetemesis. METHODS: Single institution retrospective review (1997-2012) of children managed with SBT insertion. Patient demographics, diagnosis and outcomes were noted. Data are expressed as median (range). MAIN RESULTS: 19 children [10 male, age 1 (0.4-16) yr] were identified; 18 had gastro-oesophageal varices and 1 aorto-oesophageal fistula...

BACKGROUND: We assessed the long-term outcomes of patients with biliary atresia with splenic malformation (BASM). METHODS: We retrospectively assessed outcomes of 255 patients who underwent the Kasai procedure (KP) at our hospital between 1972 and 2014. Clinical outcomes of 11 patients with BASM (group A: nine with polysplenia, two with asplenia) and 244 patients with isolated BA (group B) were compared. RESULTS: The incidence of early cholangitis and hepatopulmonary syndrome (HPS) was significantly higher in group A than in group B...

The use of adjuvant steroids following Kasai porteoenterostomy (KPE) for biliary atresia is controversial. The aim of this study was twofold: a systematic review of published literature and an update of the clinical Kings College Hospital series to look for evidence of an effect of age on the outcome in a group of BA infants treated with high-dose steroids. This clinical study included infants treated between January 2006 and June 2014 who underwent KPE by day 70 of life and who received high-dose steroids (oral prednisolone starting 5 mg/kg/day)...