RESUMO
OBJECTIVES: The transglutaminase (TG) antibody test is accurate in identifying celiac disease in symptomatic children. We sought to determine the positive predictive value of this test in asymptomatic children at genetic risk for celiac disease. STUDY DESIGN: Asymptomatic children with a genetic risk for celiac disease were studied to investigate the relationships between TG antibody titer, small bowel histology, growth, and clinical features. Small bowel biopsy histology was graded by using the system of Marsh. RESULTS: Of 30 children with a positive TG antibody test result, 21 (70%) had definite (Marsh score 2 or 3) and 4 (13%) had possible (Marsh score 1) biopsy evidence of celiac disease. TG antibody titer correlated with Marsh score (r = 0.569, P <.01). There was an inverse correlation between Marsh score and height z score (r = -0.361, P =. 05). CONCLUSIONS: In this group of asymptomatic children screened because of a genetic risk, TG antibodies have a positive predictive value of 70% to 83% for biopsy evidence of celiac disease and may identify children before clinical features of celiac disease develop.
Assuntos
Autoanticorpos/análise , Doença Celíaca/enzimologia , Doença Celíaca/genética , Predisposição Genética para Doença , Transglutaminases/imunologia , Adolescente , Doença Celíaca/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Intestino Delgado/enzimologia , Intestino Delgado/imunologia , Intestino Delgado/patologia , Masculino , Valor Preditivo dos Testes , Radioimunoensaio , Estatísticas não ParamétricasRESUMO
OBJECTIVE: To determine whether consumption of oats is safe in children with newly diagnosed celiac disease who are starting a gluten-free diet. STUDY DESIGN: We conducted a self-controlled, open-label, 6-month trial of a commercial oat breakfast cereal product. Primary outcome variables were small bowel histomorphology and anti-tissue transglutaminase IgA antibody titer. RESULTS: The 10 children who completed the study were 6.8 +/- 4.0 (mean +/- SD) years of age and 5 were male. Over 6.6 +/- 0.7 months, they consumed 24 grams of oat cereal per day, or 1.2 +/- 0.9 g/kg/d. Compared with start of study, at completion there was a significant decrease in biopsy score (P <.01), intra-epithelial lymphocyte count (P <.005), anti-tissue transglutaminase IgA antibody titer (P <.01), and number of symptoms (P <.01). CONCLUSIONS: We conclude that consumption of a commercially available oat cereal product for 6 months is safe for children with celiac disease beginning a gluten-free diet. Studies are needed to determine the long-term safety of including oat cereal in the gluten-free diet.
Assuntos
Avena , Doença Celíaca/dietoterapia , Doença Celíaca/diagnóstico , Doença Celíaca/imunologia , Doença Celíaca/patologia , Criança , Feminino , Glutens , Humanos , Imunoglobulina A/análise , Intestino Delgado/enzimologia , Intestino Delgado/imunologia , Intestino Delgado/patologia , Contagem de Linfócitos , Masculino , Radioimunoensaio , Estatísticas não Paramétricas , Transglutaminases/imunologiaRESUMO
OBJECTIVES: To determine the accuracy of anti-neutrophil cytoplasmic antibodies (ANCAs) and anti-Saccharomyces cerevisiae antibodies (ASCA) in distinguishing patients with inflammatory bowel disease from patients with other disorders, seen in a pediatric gastroenterology clinic setting, and in distinguishing ulcerative colitis (UC) from Crohn's disease (CD). STUDY DESIGN: Serum samples from 120 children with new or established diagnoses of UC (n = 25) or CD (n = 20) and control children (n = 74) were analyzed in blinded fashion for the presence of IgG ANCAs and IgA and IgG ASCA. RESULTS: The highest sensitivity for detecting inflammatory bowel disease, 71%, was achieved by using ANCAs and ASCA together. The best test for UC was ANCAs, which had a sensitivity of 80%. However, the ANCA pattern characteristic of UC, perinuclear ANCAs eliminated by DNAse, had a sensitivity of 60%. High-titer ANCAs were specific for UC, whereas ASCA were specific for CD. CONCLUSIONS: Testing for ANCAs and ASCA together did not achieve sensitivity necessary for population screening. However, ANCAs and ASCA may be helpful in evaluating children suspected of having inflammatory bowel disease and in distinguishing UC from CD.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Anticorpos Antifúngicos/sangue , Colite Ulcerativa/diagnóstico , Doença de Crohn/diagnóstico , Doenças Inflamatórias Intestinais/diagnóstico , Saccharomyces cerevisiae/imunologia , Adolescente , Criança , Colite Ulcerativa/sangue , Doença de Crohn/sangue , Diagnóstico Diferencial , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Doenças Inflamatórias Intestinais/sangue , Masculino , Sensibilidade e Especificidade , Testes Sorológicos/métodosRESUMO
OBJECTIVE: To determine the outcome, in index patients followed at an American Center, of syndromic paucity of interlobular bile ducts (sPILBD; Alagille syndrome), with onset of cholestasis in infancy. DESIGN: Cohort. SETTING: Regional referral center for infants and children with liver disease. RESULTS: During the past 10 years, 26 unrelated children with sPILBD were identified. Fifteen (58%) are alive without liver transplantation at a median age of 12.1 years. Three (11%) died, all before 2 years of age. Eight patients (31%) underwent liver transplantation at a median age of 6.5 years; all eight are alive a median 5.4 years after transplantation. The most common factors contributing to the decision for transplantation were bone fractures, pruritus, and severe xanthoma. The predicted probability of reaching 19 years of age without transplantation is about 50%; however, with transplantation, the predicted probability of long-term survival is 87%. Of 26 patients 4 (15%) have had significant central nervous system disease, and two of them have died of intracranial hemorrhage. Of the four patients who underwent cholecystoportostomy or portoenterostomy, three required liver transplantation. CONCLUSIONS: Children with sPILBD identified in infancy because of cholestasis have a 50% probability of long-term survival without liver transplantation, a worse prognosis than other follow-up studies have reported. In selected patients, liver transplantation provides the opportunity for long-term survival with improved quality of life. Patients with sPILBD are at risk of having intracranial hemorrhage.