摘要:

BackgroundCeliac disease (CD) is an autoimmune disorder caused by intolerance to ingested gluten that develops in genetically susceptible individuals. The contribution of human leukocyte antigen (HLA) genes to the genetic risk to CD has been known for a long time; however, non-HLA genetic factors are likely to be required for the development of the disease. Several studies have associated theCD28/CTLA4region on chromosome 2q33 with the disease in different populations. TheCTLA4gene encodes a receptor involved in the control of T-cell proliferation and mediates T-cell apoptosis.AimTo determine the contribution of two polymorphisms of theCTLA4to the disease: the A/G dimorphism at position +49 in exon 1 and the (AT)nmicrosatellite in the 3´ untranslated region.PatientsForty-one celiac families of Basque origin (43 patients with CD and 80 first-degree relatives).MethodsRestriction enzyme digestion of polymerase chain reaction amplified genomic DNA for the A/G dimorphism and polymerase chain reaction followed by high-resolution electrophoresis for the (AT)nmicrosatellite. For disease association studies, the Affected Family Based Controls approach was used.ResultsThe frequency of the A allele of 49 A/G polymorphism was 67.47% in the celiac allele group compared with 70.13% in the Affected Family Based Controls group. These differences were not significant. Analysis of the (AT)npolymorphism identified 17 different alleles, ranging from 262 to 312 bp in length, but no allele was significantly associated with the disease.ConclusionsOur results did not show any evidence of association of any of theCTLA4gene polymorphisms with the disease. This may result from population-specific differences in genetics and environmental susceptibility to CD.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectiveTo evaluate the efficacy and safety of propofol and meperidine plus midazolam for sedation during esophagogastroduodenoscopy (EGD) in children.MethodsData were collected prospectively and retrospectively from neurologically intact children (0.2–17.7 years of age) who underwent ambulatory diagnostic EGD during a 4-year period. Data were included from 155 consecutive patients receiving propofol with or without premedication with midazolam (PM group). One hundred five consecutive patients who received sedation with a midazolam plus meperidine combination served as a comparison (MM group). Outcome variables were: time required for induction of sedation, length of procedure, time for recovery, need for additional supportive measures, and need for physical restraint.ResultsThe onset of sedation was faster and the length of procedure and recovery were significantly shorter in the PM group as compared with the MM group (P< 0.01). Patients in the MM group required restraint more often than in the PM group. A higher dose of meperidine and midazolam was used in the prospective study. This led to deeper sedation but increased need for additional support.ConclusionsPropofol is safe and effective for facilitating EGD in children.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Dermatologic extraintestinal manifestations of Crohn disease may be refractory to treatment with corticosteroids and immunomodulators. The authors describe four children with Crohn disease with dermatologic manifestations: pyoderma gangrenosum, orofacial involvement, erythema nodosum, and idiopathic lymphedema. These dermatologic conditions were unresponsive to conventional therapy but had rapid and sustained response to the anti–TNF-&agr; antibody infliximab. No adverse reactions occurred. Infliximab should be considered for treating the extraintestinal dermatologic manifestations of Crohn disease in children.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The functional status and health-related quality of life (HRQOL) of children who survive liver transplantation (LT) have not been well documented. The purpose of this study was to determine the functional status and HRQOL in this population using a validated measure for children, the Child Health Questionnaire-Parent Form 50 (CHQ-PF50).MethodsThe CHQ-PF50 instrument was completed by the parents of 55 children who agreed to participate in a mailing survey. Subscale scores for the sample were compared with those of a published normal population (n = 391).ResultsStudy sample characteristics were: 87% Caucasian, 54.5% female, mean age at survey was 9.6 years (range, 5–17 years). Responding caregivers were 95% biologic parents and 93% female. Compared with the normal population, LT recipients had lower subscale scores for general health perceptions (P< 0.0005), emotional impact on parents (<0.0005) and disruption of family activities (0.0005). The mean physical summary score of the LT recipients was lower than that of the normal population 48.1 ± 12.1 (P= 0.005), but the mean psychosocial summary score was similar 48.8 ± 11.9 (P= 0.156). Within the LT population, the original diagnosis (biliary atresia vs. other), type of LT (living donor vs. cadaveric), age at LT, z score for height, and hospital days did not significantly influence any of the subscale scores.ConclusionsChildren who have survived LT have functional outcomes in the physical domain that are lower than those of normal children. Self-esteem and mental health in this group appeared normal. The parents in this sample experienced more emotional stress and disruption of family activities than did parents in a normal population.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectiveTo evaluate in premature infants a new parenteral lipid emulsion based on olive and soybean oils (ratio 4:1), with less polyunsaturated fatty acids (PUFA) and more &agr;-tocopherol than standard soybean oil emulsion.Study designPremature infants (gestational age, 28–<37 weeks) were randomized to receive one of the two emulsions within the first 72 hours of life. The triglyceride dose was increased to 2 g/kg/day within 3 days. Plasma phospholipid fatty acids, &agr;-tocopherol/lipid ratio, and urinary malondialdehyde (MDA) excretion were determined at baseline and after 7 days.ResultsOf 45 recruited infants, 33 completed the study per protocol (15 soybean oil, 18 olive oil emulsion). At study end, groups did not differ in plasma phospholipid arachidonic acid, total n-6 and n-3 metabolites, but the olive oil group showed higher values of the PUFA intermediates C18:3n-6 (0.19% ± 0.01% vs. 0.13% ± 0.02%,P< 0.05) and C20:3n-6 (2.92% ± 0.12% vs. 2.21% ± 0.17%,P= 0.005). The plasma &agr;-tocopherol/total lipd ratio was higher in the olive oil group (2.45 ± 0.27 &mgr;mol/mmol vs. 1.90 ± 0.08 &mgr;mol/mmol,P= 0.001), whereas urinary MDA excretion did not differ.ConclusionThe lower PUFA supply with the olive/soybean oil emulsion appears to enhance linoleic acid conversion. The reduced PUFA content, combined with a higher antioxidant intake in the olive oil group, results in an improved vitamin E status. The olive oil-based emulsion is a valuable alternative for parenteral feeding of preterm infants who are often exposed to oxidative stress, while their antioxidative defense is weak.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectivesBacterial colonization of the infant gut may have important influences on the development of gastrointestinal, respiratory, and allergic disease. Early diet is a major determinant of the gut microflora. It is very difficult to carry out studies in human infants that can investigate the interaction of diet, flora, and mucosa. In this study we have developed an infant human flora-associated (IHFA) rat model to allow such investigation.MethodsGerm-free infant rats were infected with fecal bacteria from exclusively breast-fed infants and were maintained on a modified infant formula for 8 weeks. The fecal and cecal contents were collected and compared with feces of breast-fed infants for bacterial populations, bacterial metabolites, and enzymes and for the ability to inhibit adhesion of pathogenic bacteria to human mucosal cells.ResultsThe IHFA cecum and feces were dominated by lactic acid bacteria, Bifidobacterium, and lactobacilli, which were representative of the infant feces. The fecal short-chain fatty acid profile was dominated by acetic and lactic acid in a similar manner to human infant feces. Other bacterial metabolites were similar to those of the human infant. Rat intestinal samples were able to inhibit the adhesion of pathogens to mucosal cells, but to a lesser extent than the human samples.ConclusionsThis IHFA infant model of the intestinal flora of the breast-fed infant is considered valid for studying the effect of diet on bacterial colonization and metabolism.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

ObjectiveChronic acalculous cholecystitis previously has been diagnosed by hepatobiliary scan stimulated with intravenous octapeptide of cholecystokinin. This compound may soon be unavailable in the United States. The goal of this study was to describe the characteristics of children with chronic acalculous cholecystitis diagnosed by hepatobiliary scintigraphy with oral Lipomul challenge, and to evaluate their clinical response to cholecystectomy.MethodsRetrospective chart review of patients with no gall stones detected by abdominal ultrasound or computed tomography with gallbladder ejection fraction (GBEF) <35% after Lipomul challenge who subsequently underwent cholecystectomy. Fifteen patients with a mean age of 14.9 ± 0.9 years were included. The mean duration of symptoms before evaluation was 8.2 ± 2.5 months.ResultsThe mean GBEF after Lipomul challenge was 16.7% ± 2.7%. All patients had abdominal pain. Ninety percent had right upper quadrant pain and 86% had typical biliary colic. Pain was precipitated by fatty meals in 73.3%. Histopathologic analysis of the gallbladder demonstrated chronic cholecystitis in 80% of cases. The mean postoperative follow-up was 20 ± 5 months. Six months after the surgery, nine patients (60%) were asymptomatic, five (33%) had marked improvement of symptoms, and one (6%) was unchanged. At the time of latest follow-up, symptoms had reappeared in two patients who had been asymptomatic at the 6-month visit (13%). Seven patients (46%) remained asymptomatic, five (33%) had marked improvement but continued to have some persistent symptoms, and one (6%) was unchanged.ConclusionsChronic acalculous cholecystitis may be responsible for right upper quadrant pain in children without gallstones. A GBEF <35% at 30 minutes after Lipomul challenge may be useful in identifying patients who could benefit from cholecystectomy. Lipomul may be a good alternative to cholecystokinin for gallbladder stimulation during scintigraphy.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundVaricella is a common childhood disease that can cause morbidity and mortality among immunosuppressed patients. There have been few previous studies monitoring the course of pediatric liver transplant patients with acute varicella. The aim of this study was to evaluate the treatment, outcomes, and complications of pediatric liver transplant patients admitted with acute varicella infection.MethodsA retrospective chart review was carried out based on discharge diagnoses of orthotopic liver transplant and varicella among pediatric patients (age range, birth–18 years) admitted to the UCLA Medical Center between 1985 and 2001.ResultsFive hundred fifty-six pediatric patients received liver transplantations between 1985 and 2001. Twenty-two of these patients were admitted to the UCLA Medical Center with varicella (11 females, 11 males). No patients were treated on an outpatient basis. Mean age of the patients was 6 years (range, 1–16 years). None of these patients received the varicella vaccine before hospitalization. On admission, 5 of 22 patients (23%) had received varicella zoster immunoglobulin within 96 hours of exposure. The mean length of hospitalization was 6 days (range, 2–11 days). All immunosuppression dosages were reduced during the admissions. None of the patients had been treated with high-dose corticosteroids for acute rejection before the onset of the varicella infection. Patients were treated until defervescence with intravenous acyclovir and until their varicella lesions crusted. Patients were discharged with oral acyclovir to complete a 10-day course (including the intravenous treatment). No patients had complications from the varicella infection. A complication of an elevated serum creatinine for one patient was noted with the intravenous acyclovir treatment. This patient had associated headache and nausea that resolved when the creatinine level returned to normal.ConclusionsThere were no complications or dissemination of varicella infection among our pediatric liver transplant patients. Further prospective randomized trials are required to evaluate the management of pediatric liver transplant patients infected with varicella.

ISSN:0277-2116    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundTransforming growth factor beta 1 (TGF&bgr;1) is a major fibrogenic cytokine, the expression of which is increased in the livers of children with cystic fibrosis liver disease (CFLD) and in the bronchoalveolar lavage fluid of patients with cystic fibrosis pulmonary disease (CFPD). The purpose of our study was to investigate the usefulness of plasma TGF&bgr;1 as a noninvasive marker of CFLD and CFPD, or both and to investigate the contribution of platelet-derived TGF&bgr;1 to plasma TGF&bgr;1 by correlating the latter with platelet factor 4 (PF4).MethodsThree groups of patients with cystic fibrosis were studied: 1) those with CFLD, 2) those with CF and no liver disease (CFNLD), and 3) those with CFPD. Controls were healthy adolescents and adults. Plasma TGF&bgr;1 was assayed using the R and D Quantikine quantitative sandwich enzyme immunoassay technique and PF4 (American Bioproducts ELISA kit).ResultsPlasma TGF&bgr;1 was markedly increased in CFPD (15 ± 3 ng/mL) versus healthy adults (1 ± 0 ng/mL;P< 0.004. The PF4 values followed a similar pattern: 105 ± 8 ng/mL in CFPD versus 12 ± 4 ng/mL (P< 0.0001). Plasma TGF&bgr;1 values in CFLD did not differ from CFNLD patients of comparable age nor from values for healthy adolescents. Plasma TGF&bgr;1 values were strongly correlated with values for PF4:r= 0.543,P< 0.0001.ConclusionsPlasma TGF&bgr;1 is not a useful marker of CFLD. The increased plasma TGF&bgr;1 and PF4 in CFPD patients are of interest both as possible noninvasive markers of pulmonary fibrosis and because the increased values suggest that platelet activation may play a pathogenetic role in CFPD.

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