Abstract:
OBJECTIVES:The response of serum transaminase levels to penicillamine and zinc treatment in Wilson's disease is poorly understood. The aim of this multicenter retrospective study was to evaluate transaminase levels after penicillamine and zinc treatment in children with Wilson's disease. PATIENTS AND METHODS:One hundred and nine patients with Wilson's disease (median age at diagnosis, 7.2 years; range, 1 to 18 years), treated for at least 12 months and observed in the last 20 years at 11 Paediatric Departments were studied. Clinical, laboratory and histologic features at diagnosis and initial treatment were recorded. Efficacy parameters were normalization of serum transaminase level and improved clinical and/or laboratory signs. One hundred and two patients had clinical or laboratory signs of liver disease. RESULTS:Fifty-six of 87 patients (64%) given penicillamine normalized serum alanine aminotransferase (ALT) levels within a median of 17 months (range, 2 to 96 months). Of the 29 patients with persistent hyper-ALT, 17 (59%) switched to zinc; only four of these normalized ALT on zinc within a median period of 38 months (range, 7 to 48 months). Eleven (50%) of the 22 patients given zinc alone normalized ALT within a median period of 6 months (range, 1 to 36 months). Of the 11 patients with persistent hyper-ALT, five switched to penicillamine. Three of the five normalized ALT within a median period of 6 months (range, 6 to 9 months). Overall, in penicillamine-treated and zinc-treated patients with persistent hypertransaminasemia, ALT decreased from a basal median of 236 IU/L (range, 54 to 640 IU/L) to a median of 78 (range, 46 to 960 IU/L) at the end of follow-up (P = 0.0245). Poor compliance was suspected in only 10% of cases. No predictive factor of persistent hypertransaminasemia was identified. Liver disease did not worsen in any patient during the study. CONCLUSIONS:Although the efficacy of penicillamine and zinc is well documented, it is notable that a subset of children with Wilson's disease-related liver disease (36%) had hypertransaminasemia despite appropriate treatment with penicillamine or zinc.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Iorio R,D'Ambrosi M,Marcellini M,Barbera C,Maggiore G,Zancan L,Giacchino R,Vajro P,Marazzi MG,Francavilla R,Michielutti F,Resti M,Frediani T,Pastore M,Mazzarella G,Fusco G,Cirillo F,Vegnente A,Hepatology Committee ofdoi
10.1097/00005176-200410000-00006subject
Has Abstractpub_date
2004-10-01 00:00:00pages
331-6issue
4eissn
0277-2116issn
1536-4801pii
00005176-200410000-00006journal_volume
39pub_type
杂志文章abstract:BACKGROUND:Undernutrition is common in patients with cystic fibrosis (CF). Nutritional rehabilitation has been shown to improve linear growth, pulmonary function, well-being, and resistance to infection in this population. The purpose of this study was to determine whether the administration of megestrol acetate (MA) i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-200009000-00012
更新日期:2000-09-01 00:00:00
abstract:OBJECTIVES:Enterotoxigenic Escherichia coli (ETEC) infection is a major cause of dehydrating diarrhoea in infants and early-weaned piglets living under subhygienic conditions. We studied the effect of different tea types and subfractions on the intestinal fluid and electrolyte losses involved in ETEC diarrhoea. MATERI...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000239992.12646.df
更新日期:2006-10-01 00:00:00
abstract:OBJECTIVES:Functional dyspepsia (FD) is a functional abdominal pain disorder. There is paucity of data on the economic impact of FD in children. Primary aim of our study was to estimate annual evaluation cost ("diagnosis and visit" cost) and secondary aim was to identify potential prognostic factors of FD in children. ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002599
更新日期:2020-04-01 00:00:00
abstract::Paediatric autoimmune liver disease is characterized by inflammatory liver histology, circulating autoantibodies, and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis, and de novo AIH after...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001801
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Although the course of ulcerative proctitis in adults has been well described, little data are available concerning its clinical behavior in children and adolescents. This study sought to characterize the presentation, response to therapy, and long-term course of ulcerative proctitis in the pediatric populat...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/00005176-199708000-00004
更新日期:1997-08-01 00:00:00
abstract:BACKGROUND:Subacute nonsuppurative cholangitis (cholangitis lenta) is an uncommon yet important histological finding in liver biopsies from pediatric liver transplant recipients. The histopathological features include proliferation of bile ductules at the edges of portal tracts, inspissated bile within dilated bile duc...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318074c5d0
更新日期:2007-08-01 00:00:00
abstract::This study was designed to determine the cardiac, respiratory, and arousal responses to an esophageal acid infusion test in near-term infants free from neurological, gastroesophageal, and cardiopulmonary disease at time of testing during active sleep. Eight infants (gestational age 28-37.5 weeks, postconceptional age ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199208000-00006
更新日期:1992-08-01 00:00:00
abstract:OBJECTIVE:The high prevalence of celiac disease (CD) prompted us to evaluate a new, noninvasive disease screening strategy. The aim was to identify CD in 6- to 8-year-old children for a timely diagnosis, start gluten-free diet (GFD) in compliant subjects, achieve the growth target, and prevent CD complications. METHOD...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181e6f2d0
更新日期:2011-01-01 00:00:00
abstract:OBJECTIVES:The prevalence of duodenal ulcer (DU) in adult patients with portal hypertension is higher than in patients without portal hypertension. This study investigates the prevalence and characteristics of DU in children with portal hypertension. PATIENTS AND METHODS:From January 1997 to December 2001, 80 children...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200408000-00006
更新日期:2004-08-01 00:00:00
abstract:OBJECTIVES:Gender equality in the workplace has not been described in pediatric gastroenterology. METHODS:An electronic survey that explored perceptions of career parity, work-life balance, and workplace harassment was sent to all members of the North American Society for Pediatric Gastroenterology, Hepatology, and Nu...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000637
更新日期:2015-04-01 00:00:00
abstract:OBJECTIVES:Pediatric high-resolution manometry (HRM) and 24-hour pH-impedance with/without ambulatory manometry (pH-MII+/-mano) tests are generally performed using adult-derived protocols. We aimed to assess the feasibility of these protocols in children, the occurrence of patient-related imperfections and their influe...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000003000
更新日期:2021-02-01 00:00:00
abstract:BACKGROUND:Although perianal complications of Crohn disease (CD) are commonly encountered in clinical practice, the epidemiology of perianal CD among populations of children is poorly understood. We sought to characterize the prevalence of perianal disease in a large and diverse population of pediatric patients with CD...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,多中心研究
doi:10.1097/MPG.0000000000001447
更新日期:2017-05-01 00:00:00
abstract:OBJECTIVES:: Biliary atresia (BA) is a leading cause of end-stage paediatric liver disease. Standard BA treatment is sequential surgery with an initial Kasai procedure (KP) followed by liver transplant (LT) for patients who progress to liver failure. A key determinant for the post-KP patient survival with their native ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181d67e5e
更新日期:2010-07-01 00:00:00
abstract:OBJECTIVES:Little is known about the practice of introducing complementary feeding across Europe. We aim at describing times of solid introduction in healthy infants in 5 European countries. MATERIALS AND METHODS:Between October 2002 and June 2004, 1678 healthy term infants were either breast-fed (BF) for at least 4 m...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31819f1ddc
更新日期:2010-01-01 00:00:00
abstract:OBJECTIVES:Congenital portosystemic shunts (CPSSs) are rare but increasingly recognized as a cause of important multisystem morbidity. We present new cases and a systematic literature review and propose an algorithm for the identification and care of affected patients. METHODS:We reviewed the charts of consecutive pat...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e31828b3750
更新日期:2013-06-01 00:00:00
abstract:ABSTRACT:Transgender and gender non-conforming (TGNC) individuals have a different gender identity than the sex they were assigned at birth. Despite an increase in provider awareness of TGNC health over the past decade, no original research or societal guidelines exist on TGNC patients with Inflammatory Bowel Disease (...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000003027
更新日期:2020-12-23 00:00:00
abstract:OBJECTIVES:Osteoporosis is the most common manifestation of untreated celiac disease (CD). Bone quantitative ultrasound (QUS) has recently emerged as a new modality for bone status assessment. We evaluated bone status in children with CD using dual-energy x-ray absorptiometry and quantitative ultrasound. METHODS:This ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200411000-00011
更新日期:2004-11-01 00:00:00
abstract:OBJECTIVES:Low γ-glutamyl transpeptidase (GGT) level is an important marker for progressive familial intrahepatic cholestasis, yet the cutoff level and clinical application is not well defined. This study aimed to evaluate the role of GGT as a screening marker among diverse etiologies of infantile cholestasis. METHODS...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000538
更新日期:2014-12-01 00:00:00
abstract::Studies of gastric emptying in infancy indicate that a receptor system in the duodenum regulating emptying exists operating in a manner qualitatively similar to the adult. Further investigation is needed to describe this system in more detail and evaluate how other factors including feeding volume, nutrient digestion,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198300201-00019
更新日期:1983-01-01 00:00:00
abstract:OBJECTIVES:Liver nodularity occurs across the spectrum of cystic fibrosis liver disease (CFLD), from regenerative nodules to cirrhosis, and can occur without liver enzyme abnormalities. Our aims were to determine if incorporating abdominal ultrasound (US) with annual laboratory testing improves the detection of CFLD an...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002412
更新日期:2019-10-01 00:00:00
abstract::Enzymatic in vitro hydrolysis was evaluated as a possible treatment to abolish the allergenicity of whey proteins in view of their use in infant formulas. Guinea pigs without prior immunological contact (including fetal life) with cow's milk were fed various preparations of cow's milk proteins. Oral exposure to milk o...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1985-06-01 00:00:00
abstract::Eighty-eight consecutive children with inflammatory bowel disease were studied, and upper gastrointestinal endoscopy was performed in 80 of them as one of the initial investigations before commencing medical or nutritional treatment. Forty-one children were found to have Crohn's disease and 47, ulcerative colitis. Upp...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199408000-00007
更新日期:1994-08-01 00:00:00
abstract:BACKGROUND:Reduced osmolarity oral rehydration solution (ORS) improved small bowel absorption of fluid and electrolytes in segmental perfusion in experimental animals; this was borne out in clinical practice. Adding amylase-resistant starch (RS) to ORS is expected to increase colonic fluid absorption. This study used c...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000239998.43141.b2
更新日期:2006-11-01 00:00:00
abstract:BACKGROUND:To evaluate the effect of a therapeutic regimen of 7 days versus 14 days on the clinical manifestations of Helicobacter pylori gastritis in children. METHODS:Ninety children (age 2-19 years) who had abdominal pain and/or recurrent vomiting were determined to have H. pylori gastritis by endoscopy, histology,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00005176-200002000-00018
更新日期:2000-02-01 00:00:00
abstract::The effect of whole protein and L-amino acid ingestion on plasma amino acid concentrations was investigated in 10 men. Each subject ingested equivalent amounts of amino acids as cottage cheese (session 1), an L-amino acid mixture (session 2), and cottage cheese and L-amino acids (session 3). Postprandial changes from ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199302000-00008
更新日期:1993-02-01 00:00:00
abstract::Milk was collected from eight mothers. Total lipid and free fatty acids were determined on collection. The samples were divided into aliquots and stored at 25, 4, or -11 degrees C for 6, 12, 24, or 48 h. Additional samples were stored at -11 or -70 degrees C for 1, 2, 4, 6, or 8 weeks. The effect of temperature, lengt...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198709000-00019
更新日期:1987-09-01 00:00:00
abstract:BACKGROUND:Chronic constipation is one of the most common disorders in Western countries and despite numerous clinical, pathophysiologic, and epidemiologic studies its cause is still unclear. Several hypotheses have been proposed and according to experimental studies and clinical observation, fiber intake could play a ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199902000-00015
更新日期:1999-02-01 00:00:00
abstract:BACKGROUND:The Pediatric Ulcerative Colitis Activity Index (PUCAI) is a noninvasive clinician-based index, which reflects disease severity in pediatric ulcerative colitis (UC) when no endoscopy is performed. Here, we aimed to explore signs and symptoms important to children with UC and their caregivers as the first sta...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1097/MPG.0000000000001259
更新日期:2017-03-01 00:00:00
abstract:OBJECTIVES:Diagnostic and therapeutic benefits of double-balloon enteroscopy (DBE) have been documented in adults, with few data available on pediatric patients. We evaluated the diagnostic and therapeutic utility of DBE in children. METHODS:A prospective assessment of 113 DBE procedures in 58 consecutive children you...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000192
更新日期:2014-02-01 00:00:00
abstract:BACKGROUND:There is an inverse relationship between gestational age, birth weight and the time of first neonatal bowel movement. The authors hypothesized that delayed passage of meconium might result from a delayed maturation of the recto-anal inhibitory reflex (RAIR) in premature infants. OBJECTIVE:To evaluate whethe...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000150420.00161.b9
更新日期:2005-04-01 00:00:00