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Cholestasis in children

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https://www.readbyqxmd.com/read/29152259/prevalence-of-malnutrition-and-risk-of-undernutrition-in-hospitalised-children-with-liver-disease
#1
Ronghua Yu, Yizhong Wang, Yongmei Xiao, Lili Mo, Aishu Liu, Dan Li, Ting Ge, Guangjun Yu, Ting Zhang
Nutritional status of 380 hospitalised children aged from 1 month to 5 years with liver disease was evaluated in a single paediatric centre. The total prevalence of stunting (height-for-age Z (HAZ) < -2), underweight (weight-for-age Z (WAZ) < -2) and wasting (weight-for-height Z < -2) was 9·8, 9·0 and 7·9 %, respectively. The overall nutritional risk (-2 ≤ Z < -1) of stunting, underweight and wasting was 11·8, 12·9 and 12·6 %. The prevalence of undernutrition was significantly higher in children with cholestasis than children without cholestasis (stunting, 17·5 %/4·4 %, P < 0·001, and underweight, 14·9 %/4·9 %, P < 0·001)...
2017: Journal of Nutritional Science
https://www.readbyqxmd.com/read/29144045/the-triad-of-pruritus-xanthomas-and-cholestasis-two-cases-and-a-brief-review-of-the-literature
#2
Hadley J Pearson, Joy L Mosser, Stephanie K Jacks
When encountered in children, xanthomas are most commonly associated with a group of disorders known as familial hyperlipidemias. Aside from cosmetic concerns, these xanthomas are typically asymptomatic, but when generalized pruritus is a prominent associated symptom, clinicians should consider a different set of diagnoses that includes cholestasis of the liver. In this article we present two illustrative cases of children with cholestatic disease, pruritus, and xanthomas and discuss other disorders that may include this triad...
November 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29120457/management-of-neonatal-spontaneous-intestinal-perforation-by-peritoneal-needle-aspiration
#3
M Gébus, J-L Michel, S Samperiz, L Harper, J-L Alessandri, D Ramful
OBJECTIVE: To describe conservative management of spontaneous intestinal perforation (SIP) in preterm infants using peritoneal needle aspiration (PNA). STUDY DESIGN: Monocentric retrospective review of SIP cases treated primarily by PNA between 1999 and 2015 (n=31). RESULTS: Mean gestational age was 29.2±2.4 weeks and birthweight 1149±428 g. SIP occurred at 3.7±2.2 days of life. PNA achieved definitive treatment in 18 patients (60%) with a mean of 1...
November 9, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#4
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next Generation Sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29031874/an-update-on-the-physiopathology-and-therapeutic-management-of-cholestatic-pruritus-in-children
#5
A Thébaut, D Debray, E Gonzales
Pruritus is a disabling symptom accompanying chronic cholestasis. In extreme cases, the refractory nature of pruritus can result in a need for invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway (similar to that associated with pain) regulated by several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adults, there is no consensus in children, in light of the difficulty of conducting controlled clinical studies...
October 11, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29020816/predictors-of-adverse-perinatal-outcomes-in-intrahepatic-cholestasis-of-pregnancy-with-dichorionic-diamniotic-twin-pregnancies
#6
Youwen Mei, Lan Gao, Yonghong Lin, Dan Luo, Xine Zhou, Li He
OBJECTIVE: The aim of our study was to investigate the predictors of adverse perinatal outcomes in intrahepatic cholestasis of pregnancy (ICP) with dichorionic diamniotic (DCDA) twin pregnancies. METHODS: This study was a retrospective study of women diagnosed with ICP and DCDA twin pregnancies in Chengdu's women and children's central hospital. These patients were subdivided into mild and severe ICP groups according to total bile acid (TBA) level. The clinical characteristics and perinatal outcomes were collected and compared between the two groups...
October 11, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28940454/choledochal-cyst-with-17q12-chromosomal-duplication
#7
Radana Kotalova, Petra Dusatkova, Jana Drabova, Lenka Elblova, Tomas Dedic, Ondrej Cinek, Jan Lebl, Stepanka Pruhova
The 17q12 chromosomal region carries the HNF1B gene, mutations of which cause various conditions. When searching for HNF1B/17q12 rearrangements among children with biliary atresia and/or choledochal cysts, we identified a male proband carrying a 17q12 duplication spanning 1698 kb that included 24 genes from TBC1D3C to HNF1B. The boy presented with cholestatic jaundice at the age of 2 weeks due to a choledochal cyst sized 15 ×12 mm (type Ia according to the Todani classification). He underwent a shunt surgery consisting of a hepaticojejunostomy using Roux-en-Y loop at the age of 2 months, which led to a permanent relief of cholestasis...
September 22, 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/28937538/severe-neonatal-cholestasis-in-cerebrotendinous-xanthomatosis-genetics-immunostaining-mass-spectrometry
#8
Jing-Yu Gong, Kenneth D R Setchell, Jing Zhao, Wujuan Zhang, Brian Wolfe, Yi Lu, Karolin Lackner, A S Knisely, Neng-Li Wang, Chen-Zhi Hao, Mei-Hong Zhang, Jian-She Wang
OBJECTIVES: Cerebrotendinous xanthomatosis (CTX) is caused by defects in sterol 27-hydroxylase (CYP27A1, encoded by CYP27A1), a key enzyme in the bile acid synthesis pathway. CTX usually presents as neurologic disease in adults or older children. The rare reports of CTX manifest as neonatal cholestasis assess the cholestasis as transient, with patient survival. Our experience differs. METHODS: Homozygous or compound heterozygous CYP27A1 mutations were detected in 8 neonatal cholestasis patients by whole exome sequencing, panel sequencing, or Sanger sequencing...
November 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#9
Abdulrahman Al-Hussaini, Kenneth D R Setchell, Bader AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASD) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. METHODS: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis...
September 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28809725/clinical-characteristics-and-complications-of-pediatric-liver-biopsy-a-single-centre-experience
#10
Patricia Almeida, Richard A Schreiber, Jennifer Liang, Quais Mujawar, Orlee R Guttman
INTRODUCTION: Percutaneous liver biopsy (LB) is the gold standard method for evaluation and management of patients with liver disease. The purpose of this study was to characterize pediatric patients undergoing LB at British Columbia Children's Hospital, and to determine the rate and timing of complications following the procedure. MATERIAL AND METHODS: The medical records of all pediatric patients who underwent LB during a six-year retrospective study were reviewed to collect demographic and procedure-related data...
September 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28763485/cxcr2-signaling-and-the-microbiome-suppress-inflammation-bile-duct-injury-and-the-phenotype-of-experimental-biliary-atresia
#11
Junbae Jee, Reena Mourya, Pranavkumar Shivakumar, Lin Fei, Michael Wagner, Jorge A Bezerra
Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dependent bile duct injury and obstruction. Adult wild-type (WT) and Cxcr2-/- mice were fed a diet supplemented with sulfamethoxazole/trimethoprim (SMZ/TMP) during pregnancy and lactation, and their pups were injected intraperitoneally with rhesus rotavirus (RRV) within 24 hours of life to induce experimental biliary atresia...
2017: PloS One
https://www.readbyqxmd.com/read/28745070/management-options-for-cholestatic-liver-disease-in-children
#12
Andrea Catzola, Pietro Vajro
Due to a peculiar age-dependent increased susceptibility, neonatal cholestasis affects the liver of approximately 1 in every 2500 term infants. A high index of suspicion is the key to an early diagnosis, and to implement timely, often life-saving treatments. Even when specific treatment is not available or curative, prompt medical management and optimization of nutrition are of paramount importance to survival and avoidance of complications. Areas covered: The present article will prominently focus on a series of newer diagnostic and therapeutic options of cholestasis in neonates and infants blended with consolidated established paradigms...
November 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28733223/sequencing-of-fic1-bsep-and-mdr3-in-a-large-cohort-of-patients-with-cholestasis-revealed-a-high-number-of-different-genetic-variants
#13
Carola Dröge, Michele Bonus, Ulrich Baumann, Caroline Klindt, Elke Lainka, Simone Kathemann, Florian Brinkert, Enke Grabhorn, Eva-Doreen Pfister, Daniel Wenning, Alexander Fichtner, Daniel N Gotthardt, Karl Heinz Weiss, Patrick McKiernan, Ratna Dua Puri, I C Verma, Stefanie Kluge, Holger Gohlke, Lutz Schmitt, Ralf Kubitz, Dieter Häussinger, Verena Keitel
BACKGROUND & AIMS: The bile salt export pump (BSEP, ABCB11), multidrug resistance protein 3 (MDR3, ABCB4) and the ATPase familial intrahepatic cholestasis 1 (FIC1, ATP8B1) mediate bile formation. This study aimed to determine the contribution of mutations and common variants in the FIC1, BSEP and MDR3 genes to cholestatic disorders of differing disease onset and severity. METHODS: Coding exons with flanking intron regions of ATP8B1, ABCB11, and ABCB4 were sequenced in cholestatic patients with assumed genetic cause...
December 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#14
Anita M Nucci, Kipp Ellsworth, Austin Michalski, Emily Nagel, Jackie Wessel
BACKGROUND: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized. METHODS: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section...
July 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28712532/influence-of-different-intravenous-lipid-emulsions-on-growth-development-and-laboratory-and-clinical-outcomes-in-hospitalised-paediatric-patients-a-systematic-review
#15
REVIEW
Roy-Rosshan Edward, Jacqueline K Innes, Luise V Marino, Philip C Calder
BACKGROUND & AIMS: Fats in the form of lipid emulsions (LEs) are an integral part of intravenous nutrition. The fatty acid composition of different LEs varies. The exact composition of a LE may influence cell and tissue function and clinical outcome. Currently, it is not clear which LE might be best for paediatric patients. We conducted a systematic review of the effects of different intravenous LEs in hospitalised paediatric patients. METHODS: Randomised controlled trials published in a peer reviewed journal, written in the English language, and comparing two or more different intravenous LEs in hospitalised paediatric patients were included...
July 8, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28710680/development-of-a-novel-tool-to-assess-the-impact-of-itching-in-pediatric-cholestasis
#16
Binita M Kamath, Linda Abetz-Webb, Ciara Kennedy, Bonnie Hepburn, Martha Gauthier, Nathan Johnson, Sharon Medendorp, Alejandro Dorenbaum, Lora Todorova, Benjamin L Shneider
OBJECTIVES: The aim was to develop a clinical outcome assessment (COA) for itching in children with cholestatic pruritus. METHODS: This prospective study aimed to enroll patients aged 4-30 years with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis type 1 and caregivers of patients aged 5 months to 14 years. Eligible patients experienced itching during ≥3 of the 7 days before enrollment and had not undergone liver transplant or surgical interruption of the enterohepatic circulation...
July 14, 2017: Patient
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#17
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28689603/small-bowel-dilation-in-children-with-short-bowel-syndrome-is-associated-with-mucosal-damage-bowel-derived-bloodstream-infections-and-hepatic-injury
#18
COMPARATIVE STUDY
Maria Hukkinen, Annika Mutanen, Mikko P Pakarinen
BACKGROUND: Liver disease occurs frequently in short bowel syndrome. Whether small bowel dilation in short bowel syndrome could influence the risk of liver injury through increased bacterial translocation remains unknown. Our aim was to analyze associations between small bowel dilation, mucosal damage, bloodstream infections, and liver injury in short bowel syndrome patients. METHODS: Among short bowel syndrome children (n = 50), maximal small bowel diameter was measured in contrast series and expressed as the ratio to the height of the fifth lumbar vertebra (small bowel diameter ratio), and correlated retrospectively to fecal calprotectin and plasma citrulline-respective markers of mucosal inflammation and mass-bloodstream infections, liver biochemistry, and liver histology...
September 2017: Surgery
https://www.readbyqxmd.com/read/28688656/notch-signaling-promotes-ductular-reactions-in-biliary-atresia
#19
Jessica A Zagory, William Dietz, Alex Park, Michael Fenlon, Jiabo Xu, Sarah Utley, Nirmala Mavila, Kasper S Wang
BACKGROUND: Biliary atresia (BA) is a congenital, progressive, fibro-obliterative disease of the extrahepatic biliary tree and the most common cause of end-stage liver disease in children. BA is characterized by extensive intrahepatic proliferating ductular reactions that may contribute to biliary fibrosis. Lineage tracing during experimental cholestasis indicates that cells within ductular reactions derive from PROM1-expressing hepatic progenitor cells. Given the role of Notch signaling in normal biliary development, we hypothesize that activated Notch signaling promotes the formation of ductular reactions in BA...
July 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28685316/-liver-biopsy-in-children-and-adolescents-preliminary-morphological-examinations-in-diffuse-liver-disease
#20
REVIEW
C Lackner, A S Knisely
BACKGROUND: Frequencies of types of liver disease differ between adults and nonadults (NA). OBJECTIVES: The particular problems encountered in interpreting liver-biopsy findings in NA often require referral in consultation.  To permit this efficiently, we recommend specific approaches to light microscopy, with special stains and immunohistochemistry, as well as to ultrastructural study. METHODS: Prosection and the choice of special stains are described, and are discussed in relation to clinical questions...
July 2017: Der Pathologe
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