Towards an individual screening strategy for first-degree relatives of celiac patients.

Celiac disease (CD) is known to be more prevalent in first-degree relatives of patients. In this retrospective cohort study of 609 relatives between 1994 and 2016, we investigated the effect of sex, HLA type, and age at time of index celiac diagnosis. Pearson's chi-square test and Kaplan-Meier survival analysis were used as statistical analyses. CD screening was carried out for 427 relatives (70%), resulting in a prevalence of 15%. HLA typing in 335 relatives showed HLA-DQ2/DQ8 positivity in 87.5%. In 63% of children and all parents, celiac disease was diagnosed at first screening. It was diagnosed significantly more often in females, HLA-DQ2 homozygosity, and children (all p < 0.05). In children aged 0-1 year at time of index diagnosis, celiac disease was diagnosed after consecutive screening in 58%, after 3.9 ± 2.5 (max 10) years (p < 0.001).Conclusion: Future screening policies for relatives of celiac patients should include retesting, especially in HLA-positive relatives younger than 10 years of age. In addition, one-time celiac-specific antibody testing alone could be sufficient to rule out the disease in adolescent siblings and parents of newly diagnosed celiac patients. What is Known: • Celiac disease is more prevalent in first-degree relatives of celiac patients (risk 3-12%). • HLA-DQ2 homozygous sisters/daughters are at highest risk (25%). What is New: • If younger than 10 years of age, repeated testing is necessary in HLA-DQ2/DQ8-positive first-degree relatives when celiac disease is diagnosed in a family. • One-time celiac-specific antibody testing alone could be sufficient to rule out the disease in adolescent siblings and parents of newly diagnosed celiac patients.

Intravenous midazolam sedation in pediatric diagnostic upper digestive endoscopy. A prospective study in a general hospital.

The positive role of benzodiazepines (Midazolam) in conscious sedation in pediatric patients is widely known. However, problems concerning the role of sedation in diagnostic upper endoscopy are a matter for debate as little is known about dosage and timing. We prospectively evaluated the efficacy, safety and optimal intravenous sedation dosage of midazolam in 257 consecutive patients, aged 2 months to 18 years old, who underwent upper endoscopy of the gastrointestinal tract. The initial midazolam dosage was 0.2 mg/kg Bw (Body weight) i.v. for 1 minute and, if necessary, another 0.1 mg/kg Bw was administered 5 minutes later. If sedation was sufficient, the procedure would be started 4-5 minutes later; if not, another 0.1 - 0.2 mg/kg Bw would be administered. All procedures were performed by a pediatrician together with a gastroenterologist. No serious complications occurred in any of the procedures. Oxygen saturation (OS) was maintained at over 90%, if necessary with blowby oxygen. Flumazenil was administered to 7 children (OS < 90%). Endoscopy could not be completed in 1 child. All endoscopies were completed within 10 minutes. No unexpected hospital admissions were necessary. The mean midazolam dosage was 0.4 mg/kg Bw in patients up to 6 years, for the over 6 years-olds the mean dosage was decreased to 0,2 mg/kg Bw. Particular attention was paid to the importance of informing patients before the procedure. Endoscopic diagnostic procedures can be performed safely and effectively in children with intravenous sedation in a well equipped pediatric endoscopy unit.