Abstract:
BACKGROUND:Shwachman's syndrome is the second most common cause of inherited/congenital pancreatic insufficiency after cystic fibrosis. The main associated features are usually cyclic neutropenia, metaphyseal dysostosis, and growth retardation. Other organs or functions may be involved in this syndrome, showing a wide range of abnormalities and symptoms. There are reports of Shwachman's syndrome in childhood, but little is known about the long-term clinical course of these patients. This article reports on the pathomorphosis and long-term follow-up of 13 patients with Shwachman's syndrome diagnosed in infancy focusing, in particular, on modifications of the exocrine pancreatic function over time. METHODS:Exocrine pancreatic function was evaluated by duodenal intubation followed by a pancreatic stimulation test. Nutritional, biochemical, hematologic, radiologic, and psychological evaluations were performed at various intervals. Six patients were included in long-term follow-up evaluation. RESULTS:At diagnosis, growth retardation was present in all patients, and all subjects showed pancreatic insufficiency. Hematologic features (intermittent neutropenia, anemia and thrombocytopenia), respiratory infection during the first years of life, and skeletal abnormalities were also frequently observed. Other associated features at diagnosis included hepatic involvement and occasional renal dysfunction. In the six patients followed up, a significant growth improvement was observed. In five of them the pancreatic stimulation test showed values of lipase within reference range outputs, whereas fat balance or fecal fat losses were normal in all but one subject. Of seven subjects assessed by psychological evaluation, IQ test results were markedly abnormal in one and bordered on abnormality in the others. CONCLUSIONS:The present data on Shwachman's syndrome diagnosed in infancy underline the possibility of improvement or normalization of exocrine pancreatic function with age, suggesting the need for periodic checks on pancreatic activity in these subjects. It also indicates the possibility of diagnosis of this syndrome in the absence of pancreatic insufficiency; decreasing frequency of infections over time; and the usefulness of early neuropsychological evaluation.
journal_name
J Pediatr Gastroenterol Nutrjournal_title
Journal of pediatric gastroenterology and nutritionauthors
Cipolli M,D'Orazio C,Delmarco A,Marchesini C,Miano A,Mastella Gdoi
10.1097/00005176-199909000-00006subject
Has Abstractpub_date
1999-09-01 00:00:00pages
265-72issue
3eissn
0277-2116issn
1536-4801journal_volume
29pub_type
杂志文章abstract:OBJECTIVES:Button battery ingestions are potentially life threatening for children. Catastrophic and fatal injuries can occur when the battery becomes lodged in the esophagus, where battery-induced injury can extend beyond the esophagus to the trachea or aorta. Increased production of larger, more powerful button batte...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3181f98916
更新日期:2011-05-01 00:00:00
abstract:OBJECTIVES:Gastroesophageal reflux and aspiration can occur in premature infants who are supported with mechanical ventilation. The relation between physical positioning and gastric aspiration in ventilated infants has not been studied. Pepsin measured in tracheal aspirate (TA) emerged as a specific marker for aspirati...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000000601
更新日期:2015-03-01 00:00:00
abstract:OBJECTIVE:The outcomes of fundoplication for gastroesophageal reflux disease are suboptimal in many children, and alternatives are clearly needed. Dextranomer hyaluronic acid (DxHA) copolymer, an agent with proven efficacy in vesicoureteral reflux, was studied with respect to its effects on the gastroesophageal junctio...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000000259
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Many mathematical equations based on height have been developed to estimate the esophageal length (EL) in children. The aim of this study was to confirm whether the preexisting and most frequently used equation by Strobel et al is accurate in calculating the EL in our pediatric population. Our...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182952e50
更新日期:2013-08-01 00:00:00
abstract:BACKGROUND:It has been shown that gum arabic, a soluble fiber, enhances water, electrolyte, and glucose absorption from oral rehydration solutions in jejunal perfusion of healthy rats and in animals with theophylline-induced secretion or chronic osmotic-secretory diarrhea. This report concerns a study of the effectiven...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199910000-00008
更新日期:1999-10-01 00:00:00
abstract:BACKGROUND:An increased incidence of necrotizing enterocolitis (NEC) has been noted in infants who are born to mothers with chorioamnionitis. HYPOTHESIS:Our objective was to test the hypothesis that newborn rat pups born to mothers exposed to prenatal lipopolysaccharide during pregnancy would be more susceptible to in...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/mpg.0b013e31818936b8
更新日期:2009-03-01 00:00:00
abstract:BACKGROUND:It is important to identify patients with monogenic IBD as management may differ from classical IBD. In this position statement we formulate recommendations for the use of genomics in evaluating potential monogenic causes of IBD across age groups. METHODS:The consensus included paediatric IBD specialists fr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000003017
更新日期:2020-12-16 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::The effect of protein-calorie malnutrition during gestation on the brain amino acids of rat pups was studied following nutritional recovery during lactation. The brain amino acids of rat pups born to dam rats malnourished during gestation were studied after these rat pups received proper nutrition during lactation. Pr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198411000-00027
更新日期:1984-11-01 00:00:00
abstract::Zinc concentrations in plasma, hair, and urine from children and young adults with insulin-requiring or Type I diabetes mellitus were significantly correlated with height, weight, and age, as well as with indices of metabolic control, i.e., fasting serum glucose, percent glycosylated hemoglobin (HbA1), and 24-h urine ...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198409000-00018
更新日期:1984-09-01 00:00:00
abstract::A 3-year-old boy with transient protein loss due to hypertrophic gastropathy is described. Serologic evidence of concomitant Mycoplasma pneumoniae infection was present. Although sometimes associated with infections, protein-losing hypertrophic gastropathy has not been reported previously in association with Mycoplasm...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199202000-00021
更新日期:1992-02-01 00:00:00
abstract::Eighteen children and adolescents, of a total of 90 treated in our unit between 1982 and 1992, underwent colectomy for ulceractive colitis. Six (6.7%) of the 90 patients had short stature, i.e., a height below the 3rd centile, and 8 of the 18 who had surgery had a subnormal height velocity, i.e., below the 25th centil...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199507000-00014
更新日期:1995-07-01 00:00:00
abstract:OBJECTIVES:On the basis of studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease (CLD). Therefore, we investigated renal function in children with CLD. METHODS:In a retrospective study of 277 children with CLD, renal function was investigated as glomerular fi...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001862
更新日期:2018-04-01 00:00:00
abstract:OBJECTIVES:Endoscopic retrograde cholangiopancreatography is used to manage biliary pathology in pediatric patients. Plastic biliary stents have been used in this population for obstructive lesions and bile leaks; however, they are sometimes not effective due to migration, occlusion, or ineffective sealing. Fully cover...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000001723
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Health-related quality of life (HRQOL) describes the physical, social, and emotional attitudes and behavior of people in relation to health status. By the 1980s, HRQOL was noted to be impaired in chronic diseases but inflammatory bowel disease, had not been extensively described. METHODS:This review summari...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,评审
doi:10.1097/00005176-199904001-00003
更新日期:1999-04-01 00:00:00
abstract:BACKGROUND:Although the dissociative sedative ketamine is used commonly for pediatric procedural sedation in other settings, the safety of this agent in pediatric gastroenterology is not well-studied. A 5-year experience with ketamine sedation for pediatric gastroenterology procedures was reviewed to document the safet...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-200101000-00010
更新日期:2001-01-01 00:00:00
abstract::Vitamin K prophylaxis is recommended to prevent the hazard of hemorrhage caused by vitamin K deficiency in young infants. A single administration after birth seems inadequate to completely prevent late haemorrhagic disease in breast-fed infants. The preventive effect of a daily oral dose of 25 micrograms vitamin K1, w...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199304000-00014
更新日期:1993-04-01 00:00:00
abstract:OBJECTIVES:The purpose of this study was to evaluate the effect of calcium and vitamin D2 supplementation on bone mineral density (BMD) in children with inflammatory bowel disease (IBD). PATIENTS AND METHODS:This was an open-label, prospective study conducted over a 12-month period. Seventy-two patients were divided i...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3180dca0cc
更新日期:2007-11-01 00:00:00
abstract::Both storage of breast milk for 3 days at 4 degrees C and the addition of breast milk fortifier containing lipid improved the delivery of lipids during continuous pump infusion. The percentage of the original lipid delivered after 8 h of pumping was 9, 25, 58, and 66% from the freshly collected milk, stored milk, fres...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198905000-00013
更新日期:1989-05-01 00:00:00
abstract:OBJECTIVES:In light of a paucity of data on the role of diet in colonoscopy preparation in paediatric population, the present study was designed to compare the effectiveness of clear liquid and low-fibre diets for breakfast and lunch on the day preceding colonoscopy in children. METHODS:This prospective, randomised tr...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章,随机对照试验
doi:10.1097/MPG.0000000000001832
更新日期:2018-05-01 00:00:00
abstract::During an 8-year prospective study of post-transfusion hepatitis conducted at the Thalassemic Center of Cagliari (Italy), including 135 newly diagnosed thalassemic children on long-term transfusion maintenance, 83 children (61%) developed non-A, non-B hepatitis (NANBH). Resolution of NANBH was observed in 17 (20%) cas...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-199305000-00020
更新日期:1993-05-01 00:00:00
abstract:OBJECTIVES:: Given dietary gluten exposure, growing children with celiac disease may experience malabsorption of nutrients, negatively affecting bone health. The purpose of this study was to determine the prevalence of low bone mass in children with celiac disease, according to the presence of symptoms at diagnosis. M...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31819ca18e
更新日期:2009-11-01 00:00:00
abstract::Thin frozen sections of 11 jejunal biopsies from 10 children at different stages of coeliac disease were stained by immunofluorescence technique using a panel of anti-HLA class I (A, B, C) and anti-HLA class II (DR and DQ) monoclonal antibodies. On the epithelium of flattened mucosa, in contrast with control sections,...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/00005176-198701000-00010
更新日期:1987-01-01 00:00:00
abstract:OBJECTIVES:Pediatric patients with inflammatory bowel diseases (IBD) require treatment, monitoring, and health maintenance services. We described patterns of primary, specialty, emergency department (ED) and urgent care delivery, and explored patient- and system-related variables that impact ED/urgent care utilization....
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0000000000002703
更新日期:2020-07-01 00:00:00
abstract:BACKGROUND:Childhood overweight has become a serious health problem among children and adolescents in the United States. No previous study, to our knowledge, has analyzed the effect of body mass index (BMI) on range of motion and carrying angle of the elbow joint in a healthy pediatric population. The primary objective...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e31812f568b
更新日期:2008-02-01 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: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::Hydrogen gas (H2) is a product of the fermentation of dietary carbohydrate (CHO) by bacteria in the lumen of the gastrointestinal tract in man. Thus, H2 is actually an exogenously produced gas, which either is passed as flatus, or diffuses into the body and is exhaled. In the adult, a fairly constant fraction is expir...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:
更新日期:1983-01-01 00:00:00
abstract:OBJECTIVES:The transfer of exogenous genetic material to cells within the gastrointestinal (GI) tract has many potential therapeutic applications. An attractive feature of the GI tract for gene transfer is its accessibility through the orogastric route. In this study, we evaluated the stability of recombinant adeno-ass...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/01.mpg.0000228118.59853.ba
更新日期:2006-08-01 00:00:00
abstract::This clinical report aims to review key self-management and adherence issues in pediatric inflammatory bowel disease (IBD) and to provide recommendations for health care providers regarding evidence-based assessment and treatment approaches to promote optimal self-management. Self-management difficulties in the form o...
journal_title:Journal of pediatric gastroenterology and nutrition
pub_type: 杂志文章
doi:10.1097/MPG.0b013e3182999b21
更新日期:2013-08-01 00:00:00