Abstract:
BACKGROUND:Progressive familial intrahepatic cholestasis, which constitutes a heterogeneous group of imperfectly delineated syndromes and appears to be inherited as an autosomal recessive condition, has not been hitherto reported from the Middle East, in spite of the high rate of consanguineous marriage in this region. METHODS:Sixteen affected children from six Israeli Arab families were evaluated over 30 years. All were born to consanguineous parents. RESULTS:Jaundice appeared during the first 3 weeks of life in 15 babies. When first referred, 10 had hepatomegaly and nine had splenomegaly. A progression toward cirrhosis was the rule. Serum levels of conjugated bilirubin, liver enzymes, and alkaline phosphatase were raised; gamma-glutamyl transpeptidase levels were normal in all three infants in whom it was examined, but elevated in two siblings of another family at ages 2 and 3 years. No abnormal bile acids were detected in the serum and urine of patients. Histologic examination of the liver showed giant-cell transformation, paucity of intrahepatic bile ducts, cholestasis, fibrosis, or cirrhosis. The pattern of liver pathology differed at times among affected members within the same family. Therapeutic trials with phenobarbital, cholestyramine, or ursodeoxycholic acid were ineffective. Survival of the patients was from 5 to 18 months in four families; in the other two families, three children received liver transplants, and one is awaiting liver transplantation. CONCLUSIONS:Progressive familial intrahepatic cholestasis should be included in the differential diagnosis of infants with cholestatic jaundice of unknown etiology, especially those born to consanguineous Arab parents.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Naveh Y,Bassan L,Rosenthal E,Berkowitz D,Jaffe M,Mandel H,Berant Mdoi
10.1097/00005176-199705000-00011subject
Has Abstractpub_date
1997-05-01 00:00:00pages
548-54issue
5eissn
0277-2116issn
1536-4801journal_volume
24pub_type
杂志文章abstract::This study was designed to establish whether the starting time of breast-feeding (BF) may have affected gut closure for macromolecules during the earliest postnatal period. An increase of serum IgA concentrations was taken as the index of gut permeability to macromolecules. In 14 neonates, BF started 1-6 h after birth...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198411000-00011
更新日期:1984-11-01 00:00:00
abstract:OBJECTIVES:To assess and compare the long-term therapeutic response to lamivudine compared with interferon-alpha (IFN-alpha) in children with chronic hepatitis B. METHODS:A total of 40 children (27 male; age, 1.3-18 y, mean, 7.7 y) with chronic hepatitis B who received lamivudine for at least 12 months were followed f...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000243439.47334.4e
更新日期:2007-01-01 00:00:00
abstract::The levels of secretory IgA and of IgM and IgG in colostrums and milks obtained at various times postpartum were measured by radial immunodiffusion. Immunoglobulin A was the dominant immunoglobulin in all samples, and concentrations in colostrum ranged from 1.5 to 83.7 g/L (mean 32 g/L). Immunoglobulin M and IgG had m...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198201030-00018
更新日期:1982-01-01 00:00:00
abstract:BACKGROUND:Findings in studies in rodents have suggested that epithelial growth of the small intestine is dependent on activation of the immune system. The purpose of this study was to compare changes of postnatal epithelial growth with immunologic activity in humans. METHODS:Duodenal biopsies were obtained by endosco...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199805000-00004
更新日期:1998-05-01 00:00:00
abstract:BACKGROUND AND AIMS:The aim of the present study was to evaluate proinflammatory and anti-inflammatory cytokine levels in children with irritable bowel syndrome (IBS). PATIENTS AND METHODS:Thirty-five children with IBS (17 diarrhea-predominant, 7 constipation-predominant, and 11 mixed type) and 25 healthy children as ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181fd9816
更新日期:2011-04-01 00:00:00
abstract::Eosinophilic gastroenteropathy (EG) is an uncommon, idiopathic disease in children that is characterized by eosinophilic inflammation of the intestine. Over a 7-year period 17 infants and children with EG were evaluated; the retrospective review of their clinical data constitutes the basis of this report. A scale deve...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198805000-00011
更新日期:1988-05-01 00:00:00
abstract::The impact of human recombinant growth hormone (GH) after massive small bowel resection was studied in 38 weaning female Wistar rats (65 +/- 5 days old; 193 +/- 26 g). Animals underwent a 80% small bowel resection, leaving in place similar lengths of jejunum and ileum. Animals were assigned to four groups: group A (n ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199405000-00007
更新日期:1994-05-01 00:00:00
abstract:OBJECTIVES:We aimed to identify early noninvasive predictors of clinical and endoscopic remission in children with Crohn disease (CD) under infliximab (IFX). METHODS:Prospective observational study conducted in children with moderate-to-severe CD starting IFX. All patients underwent weighted pediatric CD activity inde...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002262
更新日期:2019-06-01 00:00:00
abstract::We evaluated plasma PIVKA-II (protein induced by vitamin K absence or antagonist-II, acarboxy prothrombin) levels in three infants with hepatoblastoma as a tumor marker. PIVKA-II levels were highly elevated in all three patients. Vitamin K administration, performed in two patients, resulted in only moderate reduction ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198701000-00008
更新日期:1987-01-01 00:00:00
abstract::Constipation is a common problem in children worldwide. It can also be a chronic problem persisting for many months to years. Successful treatment of constipation requires long-term use of laxatives. Commonly used laxatives in children include milk of magnesia, lactulose, mineral oil, and polyethylene glycol. Compared...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/MPG.0b013e318296404a
更新日期:2013-08-01 00:00:00
abstract:OBJECTIVES:The aim of the present study was to determine whether serial gastric volumes and intragastric curding are repeatable within individual preterm infants when given sequential feeds of the same volume and composition. Infant and feeding characteristics that may influence differences in measured gastric volumes,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000397
更新日期:2014-08-01 00:00:00
abstract::In a 10-week randomised cross-sectional study we used an 88-item questionnaire to assess the quality of life in 16 children (ages 8-17 years) with Crohn's disease and their families. The questionnaire covered six domains of health-related quality of life, including disease and its treatment, social, emotional, family,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199612000-00003
更新日期:1996-12-01 00:00:00
abstract::Young infants with cystic fibrosis (CF) are at risk for developing symptomatic protein-calorie malnutrition (PCM) characterized by hypoalbuminemia, edema, and anemia. We reviewed the hospital charts of all infants less than 12 months of age referred to our CF clinic between 1979 and 1982 and found nine patients with P...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198605000-00010
更新日期:1986-05-01 00:00:00
abstract:ABSTRACT:Fructose is a highly abundant carbohydrate in western diet and may induce bowel symptoms in children as in adults. The main objective of this study is to describe the frequency of fructose malabsorption (FM) in symptomatic patients 18 years or younger undergoing fructose breath test in a single tertiary center...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002903
更新日期:2021-01-01 00:00:00
abstract::Nineteen patients with cystic fibrosis (CF) were studied to determine whether plasma fatty acids correlated with severity of their lung disease as assessed by pulmonary function testing. Results were compared with 19 normal subjects of similar age and sex. Linoleic acid content of all lipid fractions was significantly...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198605000-00013
更新日期:1986-05-01 00:00:00
abstract:BACKGROUND AND AIM:Patients with cystic fibrosis (CF) have low levels of n-3 long-chain polyunsaturated fatty acids (n-3 LCPUFA) in plasma or red blood cells (RBC), as also seen in other chronic and acute liver diseases. The differences may be more pronounced in CF transmembrane conductance regulator protein (CFTR)-reg...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e318249438c
更新日期:2012-07-01 00:00:00
abstract::Monitoring therapy in esophageal inflammatory disorders such as eosinophilic esophagitis and reflux esophagitis often requires frequent endoscopic evaluation. We recently reported the effective use of unsedated in-office transnasal esophagoscopy that significantly decreased costs and anesthetic exposure associated wit...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001631
更新日期:2018-01-01 00:00:00
abstract::We studied jejunal biopsy specimens of 13 IgA-deficient persons (IgAdp) and 12 controls. Four Ig-Adp had celiac disease, in the others the jejunal mucosa appeared normal. Monoclonal antibodies and the peroxidase technique were used to identify T lymphocytes, T-lymphocyte subsets, HLA-DR antigens, and IgE-containing ce...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198807000-00010
更新日期:1988-07-01 00:00:00
abstract:OBJECTIVES:The prevalence of ulcerative colitis (UC) differs by country, which is likely due to differences in genetic factors among ethnicities. Moreover, the prevalence of pediatric UC with a family history (FH) is 4.1% in Japanese patients; its clinical course begins at an early age and is more severe. Recently, a g...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001275
更新日期:2016-11-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Acute pancreatitis (AP) can be a rare extraintestinal manifestation of inflammatory bowel disease (IBD). There are only a few reports of AP presenting before the diagnosis of IBD. We aimed to characterize the demographic, clinical, and laboratory data of patients with IBD in whom AP preceded d...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182065cad
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:The IMPACT-III measures health-related quality of life (HRQOL) in pediatric patients with Inflammatory Bowel Disease (IBD). The original IMPACT domain structure was developed based on expert opinion and was not psychometrically validated. This study developed a new domain structure based on the current IMPAC...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002824
更新日期:2020-10-01 00:00:00
abstract:BACKGROUND:There are few studies of Helicobacter pylori reinfection in childhood. In the current study the reinfection rate of H. pylori and ulcer recurrence were investigated during a follow-up period of 12 months or more in children who had undergone eradication therapy. METHODS:Twenty-seven patients aged 5 to 16 ye...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199811000-00009
更新日期:1998-11-01 00:00:00
abstract::The development of alkaline phosphatase, maltase, and sucrase activities in the duodenum of the chick embryo was followed in organ culture in chemically defined medium; 14-day duodenum was used in most experiments, with comparisons being made with 18-day tissue. As has been previously shown for the other enzymes, sucr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1982-01-01 00:00:00
abstract:OBJECTIVES:Current pediatric guidelines allow noninvasive diagnosis of celiac disease in selected children. We investigated in a large cohort study whether the severity of villous atrophy at diagnosis is associated with clinical characteristics or long-term health outcomes, thus having a prognostic significance. METHO...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002675
更新日期:2020-07-01 00:00:00
abstract::Parasitic infection of the gut is highly prevalent in developing countries and contributes to the severe malnutrition associated with persistent diarrhoea in children. Using random faecal alpha 1-antitrypsin measurements as an index of protein-losing enteropathy, we examined children with and without parasitic infecti...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199211000-00006
更新日期:1992-11-01 00:00:00
abstract:OBJECTIVES:Failure-to-thrive is defined as an abnormally low weight and/or height for age. The term "nonorganic failure-to-thrive" (NOFT) has been used to describe "failure-to-thrive" without an obvious cause underlying the growth failure. The purpose of the present study was to compare sensory processing abilities bet...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000707
更新日期:2015-06-01 00:00:00
abstract:BACKGROUND:The use of protein hydrolysate preterm formulas is restricted because data on their nutritional adequacy are scarce. The authors evaluated the rate of growth and indices of protein metabolism in low-birth weight infants fed extensive and partial protein hydrolysate preterm formula followed for 12 weeks. MET...
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:10.1097/00005176-200103000-00013
更新日期:2001-03-01 00:00:00
abstract:OBJECTIVES:Little is known about the effect of diet on the oral microbiota of infants, although diet is known to affect the gut microbiota. The aims of the present study were to compare the oral microbiota in breast-fed and formula-fed infants, and investigate growth inhibition of streptococci by infant-isolated lactob...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31826f2bc6
更新日期:2013-02-01 00:00:00
abstract::The system of institutional review boards (IRBs) was established about 30 years ago for the protection of human subjects and as a mechanism for assuring an independent peer review of research activities. To keep pace with an evolving environment, a new model for human research oversight is needed that focuses less on ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200311001-00002
更新日期:2003-11-01 00:00:00
abstract:OBJECTIVES:In developed countries, small bowel histology in coeliac disease is a spectrum, ranging from normal with increased intraepithelial lymphocytes to the classic flat mucosa. In developing countries, mild to moderate enteropathies in children with chronic diarrhea and growth failure are assumed to be caused by t...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000172261.24115.29
更新日期:2005-08-01 00:00:00