Abstract:
OBJECTIVES:Wilson disease (WD) is a rare inborn error of copper metabolism with diverse manifestations. There has been no study of zinc (Zn), the copper's antagonist, in WD diagnosis and severity so far. Our aims were to evaluate serum Zn in WD and its correlation with the disease severity score (revised WD index). Although the ATP7B mutation analysis is highly accurate for WD diagnosis, it may not be readily available in a resource-limiting setting. We proposed a disease diagnostic score (Proposed WD diagnostic score) which incorporates serum Zn. METHODS:Medical records of WD and non-WD children seen at King's College Hospital from 2005 to 2015 were reviewed for the selected parameters using the Proposed WD diagnostic score. Available serum Zn data in WD children before disease diagnosis and the calculated severity score were statistically analyzed. Diagnostic values of the Proposed WD diagnostic score were evaluated. RESULTS:Serum Zn level was significantly lower in 8 WD-acute liver failure (ALF) (5.8 [4.1-8.3] μmol/L) compared to 18 WD-non-ALF (13.5 [6.1-22.2] μmol/L) and 9 ALF from indeterminate cause (9.8 [7.0-12.1] μmol/L) (P < 0.001). Serum Zn significantly correlated with the revised WD index (r = -0.554, P = 0.004). The Proposed WD diagnostic score that included serum Zn level as 1 of the parameters had sensitivity and specificity of 87% and 99.2%, respectively. CONCLUSIONS:Serum Zn is a novel parameter for diagnosis and correlates with severity of WD. The Proposed WD diagnostic score is useful while awaiting ATP7B mutation analysis.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Sintusek P,Kyrana E,Dhawan Adoi
10.1097/MPG.0000000000002007subject
Has Abstractpub_date
2018-09-01 00:00:00pages
377-382issue
3eissn
0277-2116issn
1536-4801journal_volume
67pub_type
杂志文章abstract:OBJECTIVES:Glucagon-like peptide 2 (GLP-2) is a 33 amino acid peptide hormone released from enteroendocrine L-cells following nutrient ingestion. It has been shown to exert trophic effects on the gut. We set out to measure GLP-2 concentrations in blood in children with diarrhoea and malnutrition. METHODS:GLP-2 levels ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002633
更新日期:2020-04-01 00:00:00
abstract:BACKGROUND:Results from the 13C mixed triglyceride (MTG) breath test correlate with duodenal lipase activity in adults. This noninvasive test is a potential screening and diagnostic tool for children with fat malabsorption. The aim of this study was to adapt the methodology of the MTG breath test to study test meals an...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200105000-00017
更新日期:2001-05-01 00:00:00
abstract:OBJECTIVE:The aim of the study is to analyze the impact of physical activity and eating behaviors on precursors of cardiovascular disease-including overweight/obesity, hypertension, low high-density lipoprotein, and impaired glucose tolerance-in pediatric liver transplant (LT) recipients and matched controls. METHOD:C...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002150
更新日期:2019-01-01 00:00:00
abstract:OBJECTIVES:Midgut volvulus with malrotation is typical in newborns. We present our experience to emphasize the importance of suspecting midgut volvulus as a cause of abdominal pain also beyond infancy, particularly in relation to malrotation, and the relevance of ultrasonographic (US) signs in its diagnosis. METHODS:A...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000505
更新日期:2014-12-01 00:00:00
abstract::To further evaluate the nature of malnutrition, define at-risk groups, and confirm the efficacy of nutritional supplementation on body composition in cystic fibrosis (CF), we have conducted longitudinal and cross-sectional studies of total body potassium (TBK) in 161 unselected CF subjects aged 1 month to 17 years. TB...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198908000-00012
更新日期:1989-08-01 00:00:00
abstract::Nonalcoholic fatty liver disease (NAFLD), defined as fat accumulation greater than 5% in hepatocytes, may progress to fibrosis or cirrhosis later in life. NAFLD prevalence in adolescents has increased significantly in direct relation with obesity prevalence. Fatty liver has become the most frequent indication for live...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002523
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVE:Esophageal strictures in children are serious and require specialized care. Peptic stricture is most common in our context, whereas caustic and congenital strictures are rare. The present study reports our experience in esophageal endoscopic dilation while specifying the causes of esophageal strictures as wel...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31824b16b2
更新日期:2012-06-01 00:00:00
abstract::Bowel habits were assessed in 400 healthy Thai children aged 1-72 months. The study was undertaken principally because information concerning the bowel habits of children living on a non-Western diet is lacking. Ninety-four percent of the children moved their bowels at least once a day. Mean frequency of bowel actions...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199504000-00013
更新日期:1995-04-01 00:00:00
abstract::To determine the frequency of gastric acid hypersecretion in infants with chronic malabsorption due to short bowel syndrome, acid secretory function was determined in 23 infants with malabsorption 2-22 months following small bowel resection and in a control group of 14 chronically ill, age- and weight-matched infants ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1986-03-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to describe the prevalence of childhood constipation, stool characteristics, and their relationship with toilet training and age of introduction of cow's milk. METHODS:A total of 4231 children born in 2004, from a birth cohort study, were assessed at 12, 24, and 48 months of...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318251482b
更新日期:2012-10-01 00:00:00
abstract:BACKGROUND:Abnormal gastroesophageal reflux after percutaneous endoscopic gastrostomy is a serious problem in neurologically impaired children. Protective fundoplication has been advocated. Whether esophageal pH monitoring before percutaneous endoscopic gastrostomy will predict later problems with gastroesophageal refl...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199803000-00006
更新日期:1998-03-01 00:00:00
abstract::A systematic review of literature was performed on the practice of mucous fistula refeeding in neonates with short bowel syndrome. No randomised controlled studies were identified. Case series reports of 30 infants showed improved weight gain (from -2.36 +/- 1.24 to 21.06 +/- 3.02 g/d) and reduced need for total paren...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/01.mpg.0000228111.21735.02
更新日期:2006-08-01 00:00:00
abstract:OBJECTIVES:Early amino acid administration has been recommended in preterm neonates to avoid protein catabolism. In the present work, blood urea nitrogen (BUN) was used to evaluate the quantity of protein intake in preterm infants. The aim of the study was to investigate the relation between protein intake and BUN duri...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181cd270f
更新日期:2010-08-01 00:00:00
abstract::Although bolus and continuous tube feedings are common, little is known about their effect on the developing small intestine. To compare their effect on small-intestinal growth and differentiation, six pairs of 3-day-old piglet littermates were randomized to receive similar volumes of sow milk replacer, either by bolu...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199404000-00017
更新日期:1994-04-01 00:00:00
abstract::Portal vein thrombosis (PVT) is a common cause of portal hypertension in children. Predisposing conditions for PVT are obscure in more than half of the cases. Variceal bleeding and splenomegaly are the most frequent initial manifestations. Radiologic imaging studies are the mainstay for diagnosis. Treatment includes p...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3182a1cd7f
更新日期:2013-10-01 00:00:00
abstract:OBJECTIVE:The aim of the study was to establish an apparent diffusion coefficient (ADC) cut-off value to classify active and non-active lesions in inflammatory bowel disease. METHODS:We reviewed 167 paediatric magnetic resonance enterographies executed for suspected inflammatory bowel disease by using a 1.5- and 3-T s...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002178
更新日期:2019-03-01 00:00:00
abstract:OBJECTIVE:The purpose of this study was to describe the clinical experience with the anti-tumor necrosis factor chimeric monoclonal antibody, infliximab, in pediatric patients with Crohn disease in The Netherlands. DESIGN:Descriptive. METHODS:Clinical response and adverse effects of infliximab were recorded for pedia...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200407000-00010
更新日期:2004-07-01 00:00:00
abstract:AIM:Anti-neutrophil cytoplasmic antibodies (ANCAs) detected by indirect immunofluorescence have been found in patients with inflammatory bowel disease (IBD). Nevertheless, specific antibodies against proteinase-3 (PR3) are rare in this context. METHODS:Sera from 30 consecutive pediatric patients with IBD were evaluate...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31829d4e9e
更新日期:2013-10-01 00:00:00
abstract:OBJECTIVE:The aetiology of biliary liver disease in children with intestinal failure treated with long-term parenteral nutrition (PN) is multifactorial. Risks include the lipid component of PN. The aim of the study was to compare prevalence and outcome of gallstones with different types of intravenous lipids. METHODS:...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000761
更新日期:2015-08-01 00:00:00
abstract:OBJECTIVES:We evaluated the response to infliximab in pediatric patients with ulcerative colitis (UC) and their long-term follow-up. We expanded our previous study of 14 patients and furthermore evaluated the success of weaning patients from infliximab. PATIENTS AND METHODS:We reviewed the charts of 27 pediatric patie...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31802e98d4
更新日期:2007-03-01 00:00:00
abstract::The incidence of inflammatory bowel disease (IBD) is steadily on the rise in Western and in developing countries paralleling the increase of westernized diets, characterized by high protein and fat as well as excessive sugar intake, with less vegetables and fiber. Furthermore, nutrition is involved in several aspects ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0000000000001785
更新日期:2018-03-01 00:00:00
abstract::The underlying causes of chronic diarrhea beginning early in life are increasingly well defined. Infectious and post-infectious enteropathies and food sensitive/allergic enteropathy account for the majority of cases. Recent attention has focused on characterizing defined entities, which cause protracted diarrhea in in...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-200401000-00007
更新日期:2004-01-01 00:00:00
abstract:OBJECTIVES:Children with coeliac disease (CD) have an increased number of chromosome aberrations in peripheral blood lymphocytes. Whether genetically determined or a secondary phenomenon in CD, chromosome abnormalities may be involved in the predisposition to cancer in CD patients. The aim of the study was to follow a ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200402000-00014
更新日期:2004-02-01 00:00:00
abstract:OBJECTIVES:The clinical presentation of colonic juvenile polyps with abdominal discomfort and occult rectal bleedings make them difficult to recognize. The aim of this study was to report the clinical features of colonic juvenile polyps in children referred to colonoscopy and evaluate fecal calprotectin (FCP) as a scre...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000893
更新日期:2016-01-01 00:00:00
abstract::The influence of diet and maternal parity on the fatty acid composition of mature breast milk have been studied in 23 rural Gambian mothers. The subjects' habitual diet was low in fat (16% total energy), groundnuts (peanuts) constituting the principal fat source. The high abundance of oleic and linoleic acids in groun...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198905000-00011
更新日期:1989-05-01 00:00:00
abstract::Inflammatory bowel diseases (IBD) are characterized by the invasion of leukocytes into the intestinal mucosa. However, a mixed inflammatory picture is observed that includes neutrophils, lymphocytes, monocytes, and eosinophils. To this day, the role of eosinophils in health and in disease remains unclear. Investigatio...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e3182128512
更新日期:2011-06-01 00:00:00
abstract::We investigated the mechanisms of gastroesophageal reflux (GER) and esophageal motility during endogenous esophageal acid exposure in 17 patients with reflux disease alone (age range 3-20 months) (group A) and in 10 patients with reflux disease complicated by esophagitis (age range 4-19 months) (group B), by simultane...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198811000-00006
更新日期:1988-11-01 00:00:00
abstract:OBJECTIVES:Hepatitis-associated aplastic anemia (HAAA) is a potentially life-threatening diagnosis without clear treatment guidelines. The goal of the study was to characterize the presentation, evaluation, histopathology, and outcomes of therapy in children with HAAA to guide future research and to develop standardize...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002940
更新日期:2021-02-01 00:00:00
abstract:OBJECTIVES:Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS:We performed a single-cen...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001493
更新日期:2017-03-01 00:00:00
abstract:OBJECTIVES:The present guidelines of the American Academy of Pediatrics recommend that osmolarity not exceed 450 mOsm/kg (or approximately an osmolarity of 400 mOsm/L) for breast milk or infant formulae, to minimize the risk factors for necrotizing enterocolitis. A commercial protein supplement has been developed to me...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182a208c7
更新日期:2013-10-01 00:00:00