摘要:

ObjectivesGlycogen storage disease type Ia (GSD-Ia), a congenital deficiency of hepatic glucose-6-phosphatase activity, is often associated with hyperproteinemia. To document the mechanism of hyperproteinemia, the proteins of the hemostatic system were analyzed according to their site of synthesis: hepatocyte, endothelial cell, or both. The role of inflammation was investigated by the measurement of tumor necrosis factor alpha (TNF-&agr;) and interleukin-6 (IL-6) levels in plasma.MethodsTwenty-seven patients with GSD-Ia were evaluated, as were 14 patients with other types of GSD and 30 healthy control subjects. Of the 41 patients with GSD, 15 also had hepatic adenoma (14 patients with GSD-Ia and 1 with GSD type III).ResultsIn patients with GSD-Ia, there was a two-fold increase in all hepatocyte-synthesized proteins (i.e., factor VII, protein C, C4b binding protein) compared with control subjects and patients with other types of GSD. The proteins with mixed endothelial and hepatocyte origin (i.e., antithrombin and protein S) also were significantly increased but to a lesser extent. In contrast, the mean concentration of von Willebrand factor, which is exclusively synthesized in endothelial cells, was normal, as was the concentration of TNF-&agr; and IL-6.ConclusionsThese results suggest that the hyperproteinemia of GSD-Ia (including hemostatic proteins) is attributable to hepatocyte dysfunction and not related to an inflammatory process.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundTo compare bone mineral density (BMD) in children and adolescents with celiac disease (CD) and control subjects and to evaluate diet adequacy and calcium metabolism in patients with CD.MethodsThirty patients with asymptomatic CD (17 children, 13 adolescents), on a gluten-free diet, and 23 healthy subjects were studied. BMD of the lumbar spine (dual energy x-ray absorptiometry) was performed on all patients and control subjects. In patients, food diaries for nine nonconsecutive days were obtained and analyzed. In patients, laboratory tests pertaining to calcium balance were obtained.ResultsThe mean weight and height of the adolescents with CD were lower than those of control subjects (weight: 45.8 ± 10.5 kgv55.3 ± 10.5 kg,P= 0.037; height: 153.0 ± 11.0 cmv167 ± 12.0 cm,P= 0.007). The mean BMD in adolescents with CD was significantly lower than that of the control subjects (0.917 ± 0.116 g/cm2v1.060 ± 0.158 g/cm2,P= 0.015), whereas no significant difference was found between children with CD and control subjects (P= 0.595). A multiple-regression model shows that increases in BMD relative to height were lower in adolescents with CD than in control subjects. The proportion of adolescents who had started a gluten-free diet after 2 years of age was higher than that of children with CD (P< 0.001). High percentages of magnesium, calcium, and phosphorous deficiencies were present in CD patients' diets. The serum levels of ionized and total calcium and parathormone were normal.ConclusionsThe BMD of adolescents with CD was lower than that of the control subjects, whereas no difference was found between the BMD of children with CD and that of control subjects.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundLittle is known about the enteric protein loss in patients after a modified Fontan operation before the appearance of overt symptoms or signs of protein-losing enteropathy (PLE). The authors examined the possibility of using fecal &agr;1-antitrypsin concentration measurements for the early detection of postoperative PLE and in longer term postoperative monitoring of these patients.MethodsThe authors compared fecal &agr;1-antitrypsin concentrations in stool samples from 12 children 12.0 to 43.7 months after modified Fontan operations with those of 12 age-matched control subjects and examined the relationship between the &agr;1-antitrypsin levels and time since operation. The authors also compared fecal &agr;1-antitrypsin concentrations of stools from the same patients obtained at two different time points after surgery with intervals between samples ranging from 14.7 to 19.8 months.ResultsNo significant differences in serum total protein and albumin levels were observed between patients after the modified Fontan operation and control subjects. The fecal concentrations of &agr;1-antitrypsin in patients after the Fontan operation were significantly (P< 0.01) higher than those in control subjects. There was no significant correlation between fecal &agr;1-antitrypsin concentrations and time elapsed after the Fontan operation. The fecal &agr;1-antitrypsin concentration increased significantly (P< 0.01) over periods of 14.7 to 19.8 months after the first measurement.ConclusionThe results show that enteric protein loss begins before the appearance of hypoproteinemia in patients after a modified Fontan operation, and that the measurement of fecal &agr;1-antitrypsin concentrations in random stool samples is useful as an early indicator. To watch for the development of PLE after Fontan operation, it may be important to perform longitudinal follow-up examinations of enteric protein loss by measuring fecal &agr;1-antitrypsin concentrations early in the postoperative period.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Backgroundn-3 Polyunsaturated fatty acids (PUFAs) have been suggested as a treatment for ulcerative colitis (UC). However, the efficacy of n-3 PUFAs against UC has not been examined in children. Therefore, the authors investigated the effects of eicosapentaenoic acid (EPA) on fatty acid composition and leukotriene (LT) production in children with UC.MethodsFor 2 months the authors administered highly purified EPA ethyl ester (EPA-E) (1.8 g/d) to children with UC in remission. Colonic mucosal histology, fatty acid composition of erythrocyte membrane phospholipids, and LTB4production by leukocytes and colonic mucosa were measured before and 2 months after the initiation of EPA-E treatment.ResultsNo patients relapsed during the study period, and no significant differences were detected in laboratory findings obtained before and 2 months after the initiation of EPA-E ingestion. There were no significant differences in mucosal histologic scores before and 2 months after EPA-E treatment. The EPA levels in erythrocyte membranes 2 months after the initiation of EPA-E treatment were significantly higher than before treatment, but the other fatty acids showed no significant changes. LTB4production by leukocytes and rectal mucosa after 2 months of EPA-E treatment was significantly lower than before treatment.ConclusionEPA-E treatment increased the levels of EPA in erythrocytes and decreased LTB4levels produced by leukocytes and colonic mucosa. To assess the concomitant clinical changes, we should examine the long-term effects of EPA-E ingestion on the maintenance of remission in children with UC.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectivesThromboembolism is a significant cause of morbidity and mortality in patients with inflammatory bowel disease (IBD). Plasma total homocysteine (tHcy) is a risk factor for vascular disease and has been implicated as a mediator of thromboembolic events in adults with IBD. The authors studied the link between tHcy and IBD in children, in whom associations may be clearer, and investigated associations with plasma von Willebrand factor antigen, a marker of vascular damage.MethodsThis cross-sectional study included 43 patients with IBD (27 Crohn disease, 9 ulcerative colitis, and 7 indeterminate colitis) and 46 control subjects from a pediatric gastroenterology clinic. Plasma tHcy, plasma 5-methyl tetrahydrofolate, red cell folate, plasma vitamin B12, plasma von Willebrand factor antigen, and methylene tetrahydrofolate reductase (MTHFR) genotype (for the C677T mutation) were measured.ResultsPlasma tHcy concentrations were higher in children with IBD than in control subjects, when corrected for age (P< 0.05), and plasma tHcy was negatively correlated with plasma 5 methyl tetrahydrofolate (P< 0.0005). Plasma 5 methyl tetrahydrofolate and age were the main predictors of plasma tHcy. Neither MTHFR genotype nor von Willebrand factor showed any association with any other measure, and there were no differences between children with IBD and control subjects.ConclusionsElevated plasma tHcy is a consequence of IBD in children, probably mediated by poor folate status associated with diet or the pathophysiology of the disease.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectivesTo determine the incidence, etiology and outcome of pancreatitis at a regional children's hospital.MethodsChart review of all patients with pancreatitis seen during a 6 year period at the Children's Hospital of Wisconsin. The diagnosis of pancreatitis required either a serum amylase or lipase >3 times normal or radiographic evidence of pancreatitis.ResultsTwo hundred fourteen episodes of pancreatitis in 180 patients were documented. The most common etiologies were systemic disease (14%), trauma (14%), drug induced (12%), biliary tract disease (12%), infectious (8%), and idiopathic (8%), which made up 68% of the total cases. Eleven patients died, all from underlying systemic illnesses. The serum amylase and lipase were elevated in 82% and 83% of patients respectively.ConclusionsPancreatitis is more common in children than previously thought. Upon careful assessment fewer cases were found to be idiopathic than in previous series. The outcome of pancreatitis depends on co-morbid conditions.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundThe bacterial and host factors that influence the clinical outcomes of theHelicobacter pyloriinfection have not been fully identified. Cytotoxin-associated gene product (CagA), one of the virulence factors, has been associated with a more aggressive form of infection. The authors studied the relationship between CagA status and clinical outcome in Chilean children and adults withH. pyloriinfection.MethodsOne hundred eighty consecutive patients undergoing upper gastrointestinal endoscopic analysis were enrolled after informed consent was obtained. Rapid urease test and histologic analysis were used to detectH. pyloriinfection. IgA and IgG antibodies toH. pyloriwhole cell antigen preparation and IgG antibodies to CagA were measured by enzyme-linked immunosorbent assay (ELISA).ResultsH. pyloriinfection was detected in 42% of the patients by biopsy or urease test and in 38% and 20% of patients by IgG and IgA antibodies, respectively. The prevalence ofH. pylorieither by the invasive or the serologic tests was directly related to patient age. Among patients withH. pylori, there was no significant association between age and prevalence of CagA. Nearly 70% of the patients withH. pyloriand peptic ulcer disease had CagA-positive strains. In contrast, only 49% of the patients with chronic gastritis alone had CagA-positive strains (P< 0.05).ConclusionsIn Chile, patients infected withH. pylorihave a proportion of CagA-positive strains similar to that reported in developed countries. CagA prevalence was not significantly different in adults and children infected withH. pylori, suggesting that variations in clinical outcome may be related to host immune or environmental factors.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundCeliac disease is a multifactorial disorder of the proximal small intestine associated with a permanent intolerance to gluten. The HLA-DQ(&agr;1*0501, &bgr;1*02) heterodimer is strongly associated with this disease.Materials and MethodsThe authors studied a sample of 354 unrelated Caucasoid individuals: 118 patients with celiac disease and 236 control subjects. All patients and controls subjects were born in Gran Canaria (Canary Islands) at least two generations ago.The authors typed the HLA-DQA1 and DQB1 genes by DNA methods. The positive and negative predictive values of the test were studied.ResultsThe mean age at diagnosis was 25.4 months, with a statistically significant proportion of females (64.4%,P< 0.002). For DQB1 gene, the susceptibility allele found was DQB1*02 (relative risk [RR] = 7.60, confidence interval [CI]: 5.35–10.78), whereas for the DQA1 gene, the susceptibility alleles found were DQA1*0501 (RR = 2.99, CI: 2.16–4.14) and DQA1*0201 (RR = 1.88, CI: 1.25–2.82). The presence of the DQ(&agr;1*0501, &bgr;1*02) heterodimer was strongly associated with the disease (92.4% in the patients group vs. 21.6% in control subjects). HLA-DQ8 heterodimer was absent in the authors' patients. DQB1*02 homozygous subjects presented a higher relative risk for celiac disease. There was no correlation of DQB1*02 dosage with age at onset below 12 years of age or with gender distribution. Sensitivity, specificity, and the positive and negative predictive values of the test were 92.4%, 78.4%, 68.1%, and 95.4%, respectively.ConclusionsThe presence of the DQ2 (DQA1*0501/DQB1*02) heterodimer is strongly associated with celiac disease in the population studied by the authors. The value of this test derives from its ability to exclude disease when a negative result occurs.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectivesYoung children are thought to be a unique subset of pediatric patients with inflammatory bowel disease (IBD). The authors' objective was to evaluate the differences in initial clinical presentation of young and older children with IBD and to determine whether a positive family history of IBD is associated with the age of presentation.MethodsThe authors reviewed the records of all patients with new diagnoses of Crohn disease (CD) and ulcerative colitis (UC) who presented between July 1996 and July 1999. Initial evaluation included assessment of growth parameters and laboratory values (hemoglobin concentration, platelet count, erythrocyte sedimentation rate, and serum albumin). Inquiry regarding a family history of IBD was made in every patient.ResultsThere were 153 patients with new diagnoses (82 with CD and 71 with UC), with a mean age of 11.9 years (range, 16 months–18 years). The children with CD had a higher sedimentation rate and platelet count and a lower mean hemoglobin concentration and serum albumin at presentation than did children with UC. Body mass index (BMI) was significantly lower in patients with newly diagnosed CD than in those with UC. The only significant laboratory differences between patients younger than 11 years and those 11 years or older was a higher mean platelet count in patients with CD who were younger than 11 years. Of the younger patients with CD, 41.7% had a positive family history of IBD, which was significantly greater that that found in the older patients with CD.ConclusionsExcept for higher platelet counts, a lower BMI, and a higher frequency of positive family history in young children with CD, there were no significant differences in the presentation of young children with IBD compared with older children.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID