Enayatollah Nemat Khorasani

Johanson–Blizzard syndrome (JBS) is a rare, sometimes fatal autosomal recessive multisystem congenital disorder featuring abnormal development of the pancreas, nose and scalp, with mental retardation, hearing loss and growth failure. It is sometimes described as a form of ectodermal dysplasia. The disorder is especially noted for causing profound developmental errors and exocrine dysfunction of the pancreas, and it is considered to be an inherited pancreatic disease. We report a ten years Irannian child with signs and symptoms suggestive of this syndrome (JBS). J Nepal Paediatr Soc 2015;35(3):287-289.

The prevalence of gastroesophageal reflux disease (GERD) is increasing in patients with asthma and the effect of proton pump inhibitor therapy on asthma outcome has shown variable results. The aim of this study was to determine the efficacy of omeprazole in the treatment of asthma and improvement of pulmonary function in adolescents with GERD. Thirty-six consecutive patients (range, 13–20 years old) with moderate persistent asthma and GERD were recruited on regular follow-up in Mashhad City. The case group included 18 patients who received oral omeprazole (20 mg twice a day for 6 weeks) and the control group included 18 patients who received placebo. A pulmonary function test was examined in two groups immediately before and 6 weeks after medication. The symptoms of GERD were significantly improved with omeprazole in the case group. After 6 weeks of study, the mean values of forced vital capacity, forced expiratory volume in 1 second, and peak expiratory flow rate were higher in patients treated with omeprazole (p < 0.0001). Treatment by omeprazole is effective for treatment of asthmatic patients with GERD.