摘要:

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; γ-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.

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Background:Literature information regarding clinical and histological correlates in celiac disease is limited. The present study was designed to assess the value of various clinical parameters in predicting the severity of small bowel histopathology.Methods:Small bowel biopsy specimens of 59 children with established celiac disease (ESPGAN criteria) were evaluated blindly. Morphology was evaluated based on a common histopathology score. The following clinical variables were evaluated: age at diagnosis, duration of symptoms, severity score of clinical symptoms, severity score of physical signs, and growth parameters (height and weightZscores). Multiple regression analysis was performed to evaluate the relative importance of each clinical parameter.Results:Only three clinical variables revealed a significant correlation with the histopathology score. The symptom severity score (t= 3.883,p= 0.0003) demonstrated a positive correlation. The two others, age at diagnosis (t= 3.076,p= 0.0032) and duration of symptoms (t= -2.987,p= 0.0041), revealed a negative correlation.Conclusions:We conclude that more severe clinical symptoms of a shorter duration, presented at a younger age, are better predictors of a more severe form of small bowel histopathology in children with celiac disease.

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Background:We carried out a study of the antibody pattern in 50 celiac children [34 females (F) and 16 males (M); F/M, 2.1], ages 7 months-15 years, compared with that in 25 control subjects (13 females and 12 males) of the same age.Methods:IgA and IgG antigliadin antibodies (AGA) were determined with an enzyme-linked immunosorbent assay technique. IgA anti-R1-reticulin antibodies (ARA) and IgA antiendomysium antibodies (EmA) were determined with the fluorescein isothiocyanate-conjugate-labeled anti-human immunoglobulin technique. To compare sensitivity and specificity, EmA were identified using monkey esophagus and human umbilical cord as substrates.Results:While AGA (IgA and IgG) showed a high sensitivity but a low specificity, ARA showed a high specificity but a low sensitivity. Data on EmA showed a high sensitivity and specificity with both tissue sections, with monkey esophagus being more sensitive (96%) and umbilical cord more specific (100%).Conclusions:Our results confirm the importance of celiac disease-related antibodies in identifying celiac children. Moreover, the easy availability of human umbilical cord indicates that it would be proper to use this tissue as substrate, instead of monkey esophagus, for EmA search in the future.

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Background:An improvement in screening for celiac disease has recently been described that uses human umbilical cord as a substitute for monkey esophagus to determine IgA endomysium antibodies in adults. As using monkey esophagus is ethically questionable for large-scale screening, we studied whether substitution of umbilical cord would be suitable for pediatric patients as well.Methods:Serum from 53 children with untreated celiac disease, 22 in remission and 13 on challenge, were screened for antigliadin IgA, antigliadin IgG, and IgA reticulin antibodies, in addition to IgA endomysium antibodies tested both on monkey esophagus and on human umbilical cord. Controls included 20 patients with cow-milk-sensitive enteropathy, 23 with inflammatory bowel disease, and 23 with diabetes mellitus, and 48 patients who were biopsied to exclude celiac disease either because of positive gliadin antibody test or disturbed growth.Results:Sensitivity (0.94) and specificity (1.0) were similar for umbilical cord and esophageal determinations in active celiac disease. Both substrates detected identical positive cases and neither gave false-positive results. In celiac patients on a gluten-free diet, endomysium antibodies with either substrate were positive in seven identical cases and negative in 15 of 22 cases. Correlations with reticulin antibodies were comparable with human umbilical cord and monkey esophagus (0.83 and 0.85, respectively; Spearman Correlation Scction Pair-Wise deletion).Conclusions:Human umbilical cord is an excellent substitute for monkey esophagus to determine endomysium antibodies in celiac diagnosis in children and adolescents.

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Background:Eosinophils contribute to the intestinal inflammatory infiltrate in Crohn's disease (CD). Eosinophilic infiltration occurs early in Crohn's recurrences, and a release of eosinophil cationic proteins has been observed in active CD. The proliferation, differentiation, and activation of eosinophils are highly dependent on the cytokine interleukin 5 (IL5). In the present study, we used in situ hybridization (ISH) to investigate the expression of the IL5 gene in intestinal specimens from patients with CD.Methods:We studied 14 intestinal samples from eight children who had undergone ileocolectomy for advanced CD. The samples were examined for the intensity of the inflammatory infiltrate. Normal pediatric intestine specimens served as controls. In situ hybridization was performed on frozen tissue using radiolabeled IL5 mRNA probes.Results:Positive signal with the IL5 antisense probe was observed within numerous cells infiltrating the specimens involved with CD. The number of IL5-expressing cells correlated with the histological grade of inflammation. Most of the labeled cells were eosinophils, characterized by their bilobed nuclei. Rare IL5-positive cells were detected in the control tissues. No positive signal was obtained with the IL5 sense probe.Conclusion:These results suggest that IL5 can be produced by eosinophils at the sites of inflammation in active CD and could be involved in the immune response by activating eosinophils, at least in part through an autocrine pathway, and perhaps by interacting with B and T cells.

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:0277-2116    

出版商:OVID    

年代:1997

数据来源: OVID