Nutritional selection pediatric liver disease

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BACKGROUND: Intestinal Failure, parenteral nutrition (PN) dependence, and subsequent liver disease are the most challenging and life-threatening complications of short bowel syndrome experienced by patients with total intestinal aganglionosis. Skipped Aganglionic Lengthening Transposition (SALT) showed to be a promising procedure to overcome such problems. We herein report the results of two patients who underwent SALT at the Umberto Bosio Center for Digestive Diseases. PATIENTS AND METHODS: Between November 2019 and July 2022, 2 patients with total intestinal aganglionosis underwent SALT as autologous intestinal lengthening procedure...

BACKGROUND: This review aims to identify the current indications and gaps in the management of fat-soluble vitamins in pediatric patients with cholestasis. METHODS: A comprehensive review of the literature using PubMed, Scopus, Web of Science and Embase was performed. Two authors independently identified the most relevant studies published over the past 20 years up to February 2022, including original papers, narrative reviews, observational studies, clinical trials, systematic reviews and meta-analyses...

Cystic fibrosis (CF) is one of the most common autosomal recessive genetic diseases in Caucasians, but CF patients in China are rare, and it was listed as the first batch of rare diseases in China in 2018. In recent years, CF has been gradually recognized in China, and the number of CF patients reported in China in the past 10 years is more than 2.5 times the total number in the previous 30 years, and the total number of CF patients is estimated to be more than 20 000. The research progress of CF gene modification has led to the innovation of CF treatment...

PURPOSE: This study aimed to evaluate the prevalence and risk factors of infant dyschezia as well as pediatrician awareness regarding this disease in Indonesia. METHODS: This is a two-part cross-sectional study, which was divided into study A and B. Study A: Parents whose infants were under 9 months old and attended well-baby clinics were recruited at two randomly selected primary health centers. Parents also provided information on the infant's previous medical history, and socio-demographic and family details...

BACKGROUND: We aimed to evaluate costs from transplant to discharge in children who had undergone intestine transplant. METHODS: We performed a cross-sectional observational study of pediatric intestine transplant recipients from 2004 through 2020, utilizing the Pediatric Health Information System database. Standardized costs were applied to all charges and converted to 2021 US dollars. We analyzed the association of cost from transplant to discharge with age, sex, race and ethnicity, length of stay, insurance type, transplant year, short bowel syndrome diagnosis, liver-containing graft, hospitalization status, and immunosuppressive regimen...

BACKGROUND: Complications of Crohn's disease (CD) often impair patients' quality of life. It is necessary to predict and prevent these complications (surgery, stricturing [B2]/penetrating [B3] disease behavior, perianal disease, growth retardation and hospitalization). Our study investigated previously suggested and additional predictors by analyzing data of the CEDATA-GPGE registry. METHODS: Pediatric patients (< 18 years) diagnosed with CD with follow up data in the registry were included in the study...

BACKGROUND: In research on pediatric chronic intestinal failure, heterogeneity in reported definitions and outcomes exists. This leads to a risk of reporting bias and impossibility of evidence synthesis. Also, reported outcomes should be relevant to both healthcare providers and patients and their parents. Therefore, the aim of this study is to create a core outcome set (COS) to be used in studies on pediatric chronic intestinal failure. METHODS: Candidate outcomes were selected from a recent systematic review...

The irritable bowel syndrome (IBS) is a chronic disorder of gut-brain interaction. IBS is still associated with areas of uncertainties, especially regarding the optimal diagnostic work-up and the more appropriate management. Experts from 7 Italian Societies conducted a Delphi consensus with literature summary and voting process on 27 statements. Recommendations and quality of evidence were evaluated using the grading of recommendations, assessment, development, and evaluation (GRADE) criteria. Consensus was defined as >80% agreement and reached for all statements...

PURPOSE: Golimumab (GLM) is an anti-tumor necrosis factor (TNF)-α antibody preparation known to be less immunogenic than infliximab (IFX) or adalimumab. Few reports on GLM in pediatric patients with ulcerative colitis (UC) are available. This study aimed to review the long-term durability and safety of GLM in a pediatric center. METHODS: The medical records of 17 pediatric patients (eight boys and nine girls) who received GLM at the National Center for Child Health and Development were retrospectively reviewed...

Purpose: Perinatal cytomegalovirus (CMV) infection can lead to biliary atresia (BA) in different entities. This study aimed to compare the clinical, hematological, biochemical, and histological features of infants with BA based on their CMV immunoglobulin M (IgM) status at presentation. Methods: This cross-sectional descriptive study was carried out between January 2019 and June 2020 at the Department of Pediatric Gastroenterology and Nutrition at the Bangabandhu Sheikh Mujib Medical University (BSMMU) in Dhaka...

In pediatric fatty liver disease (FLD) can develop into steatohepatitis, cirrhosis and hepatocellular carcinoma in adulthood. We assessed if early-life physical exercise reduced the effects of high fat diet (HF)-induced steatosis. Male HF-fed rats with access to a running wheel from weaning until day (D)60 (early exercise) or from D67-D120 (late exercise) were compared to control HF- or chow-fed groups with no wheel. At D63 and D120 liver histopathology (Kleiner score), type I collagen and plasma enzymes were assessed...

Purpose: The treatment and long term clinical outcomes of anorectal malformations (ARM) in children have always been the focus of pediatric surgeons. This study aimed at reporting our experience as far as long-term follow-up of ARM in children is concern. Methods: We enrolled patients treated between 1999 and 2019, and established selection criteria to choose appropriate subjects. A validated questionnaire was used to determine long-term quality of life outcomes...

PURPOSE: To compare the effectiveness, tolerability, acceptability, and safety of sodium picosulphate with magnesium citrate (PS/Mg) and polyethylene glycol (PEG) in children (≤18 years) preparing for colonoscopy. METHODS: Three electronic databases (MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials) were searched till July 2020. Only randomized controlled trials (RCTs) were included. At least two authors independently selected studies and performed risk of bias assessment and data extraction...

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. Primary-care physicians (PCPs) play a key role in identifying patients requiring specialist referral. In this study, we aim to determine PCPs' practice patterns for paediatric NAFLD, as knowledge gaps have been reported for adult NAFLD. METHODS: A survey was sent to 60 PCPs in the Eastern Ontario Network from July 2019 to January 2020. RESULTS: Thirty-seven (62%) PCPs responded to the survey...

BACKGROUND: Despite parenteral nutrition (PN) being life sustaining, one of the risk factors associated with its long-term use is intestinal failure-associated liver disease (IFALD), which increases the risk for morbidity and mortality. This review examines some of the risk factors associated with IFALD. METHODS: A literature review using the databases PubMed, EMBASE, and CINAHL between 2010 and 2020 was performed using search terms, including long-term total PN and liver failure, serum plant sterols and liver failure, and complications and PN...

This study estimated the direct and indirect socioeconomic costs of 238 diseases and 22 injuries from a social perspective in Korea from 2007 to 2015. The socioeconomic cost of each disease group was calculated based on the Korean Standard Disease Classification System. Direct costs were estimated using health insurance claims data provided by the National Health Insurance Service. The numbers of outpatients and inpatients with the main diagnostic codes for each disease were selected as a proxy indicator for estimating patients' medical use behavior by disease...

OBJECTIVES: Progressive Familial Intrahepatic Cholestasis, is an expanding group of autosomal recessive intrahepatic cholestatic disorders. Recently, Next Generation Sequencing allowed identifying new genes responsible for new specific disorders. Two biochemical phenotypes have been identified according to gamma-glutamyltransferase (GGT) activity. Mutations of the myosin 5B gene (MYO5B) are known to cause Microvillus Inclusion Disease. Recently, different mutations in MYO5B gene have been reported in patients with low-GGT cholestasis...

Inflammation plays an important role in the outcome of patients with cystic fibrosis (CF). It may develop due to cystic fibrosis transmembrane conductance regulator protein dysfunction, pancreatic insufficiency, or prolonged pulmonary infection. Fecal calprotectin (FC) has been used as a noninvasive method to detect inflammation. Therefore, the aim of the current meta-analysis was to investigate the relationship between FC and phenotype severity in patients with CF. In this study, searches were conducted in PubMed, Science Direct, Scopus, and Embase databases up to August 2021 using terms such as "cystic fibrosis," "intestine," "calprotectin," and "inflammation...

BACKGROUND: Total parenteral nutrition (TPN) dependence leads to development of intestinal failure-associated liver disease (IFALD). The spectrum of diseases ranges from cholestasis, steatosis, fibrosis, and cirrhosis that causes significant morbidity. Understanding the disease at molecular level helps us to develop therapeutic targets. We performed transcriptomic analysis on liver from rats with TPN administration, and we assessed the role of selected differentially expressed genes (DEGs), functional pathways, transcriptional factors, and their associations with pathological parameters of IFALD...