Epistaxis is a common debilitating manifestation in hereditary hemorrhagic telangiectasia (HHT), due to mucocutaneous telangiectases. The epistaxis can be difficult to control despite available treatments. Dysregulated angiogenesis has been shown to be associated with telangiectases formation. Topical propranolol has demonstrated antiangiogenic properties. We performed a two-phase study, i.e., a double-blind placebo-controlled phase, followed by an open-label phase. The aim of the study was assessment of safety and efficacy of nasal propranolol gel in HHT-related epistaxis. Twenty participants with moderate-severe HHT-related epistaxis were randomized to eight weeks of propranolol gel 1.5%, or placebo 0.5 cc, applied to each nostril twice daily; and continued propranolol for eight weeks in an open-label study. For the propranolol group, the epistaxis severity score (ESS) improved significantly (−2.03 ± 1.7 as compared with −0.35 ± 0.68 for the placebo group, p = 0.009); hemoglobin levels improved significantly (10.5 ± 2.6 to 11.4 ± 2.02 g/dL, p = 0.009); and intravenous iron and blood transfusion requirement decreased. The change in nasal endoscopy findings was not significant. During the open-label period, the ESS score improved significantly in the former placebo group (−1.99 ± 1.41, p = 0.005). The most common adverse event was nasal mucosa burning sensation. No cardiovascular events were reported. Our results suggest that topical propranolol gel is safe and effective in HHT-related epistaxis.
Although circumcision is commonly believed to protect against urinary tract infection (UTI), it is not unusual in neonates in Israel, where almost all male infants are circumcised. The aim of the study was to evaluate the burden of neonatal UTI in Israel and its relationship to circumcision. Medical records of neonates (< or =2 months old) hospitalised with UTI were reviewed and demographic and clinical data were collected. The second part of the study consisting of a telephone survey to assess timing and details concerning the circumcision, included two groups: a study group consisting of parents of male infants, aged 8-30 days, hospitalised with UTI, and a control group consisting of healthy neonates. 162 neonates (108 males, 54 females) were hospitalised with UTI. Mean age at admission was significantly lower in males (27.5 vs 37.7 days, p = 0.0002). The incidence of UTI in males peaked at 2-4 weeks of age, that is, the period immediately following circumcision. In females, the incidence tended to rise with age. Accordingly, male predominance disappeared at 7 weeks and the male-to-female ratio reversed. In the second part of the study, 111 males (< or =1 month old) were included: 48 post-UTI and 63 as a control group. While evaluating the impact of circumcision technique, we found that UTI occurred in six of the 24 infants circumcised by a physician (25%), and in 42 of the 87 infants (48%) circumcised by a religious authority; the calculated odds ratio for contracting UTI was 2.8 (95% CI 1 to 9.4). There was a higher preponderance of UTI among male neonates. Its incidence peaked during the early post-circumcision period, as opposed to the age-related rise in females. UTI seems to occur more frequently after traditional circumcision than after physician-performed circumcision. We speculate that changes in the haemostasis technique or shortening the duration of the shaft wrapping might decrease the rate of infection after Jewish ritual circumcision.
Background: Inhaled bronchodilators are frequently used among patients with primary ciliary dyskinesia (PCD), although neither the effectiveness nor the prevalence of their use is known, due to the paucity of relevant studies. Methods: This is a retrospective analysis of pre- and post-bronchodilator spirometry results, of patients with PCD from two centers. Correlations were examined of bronchodilator response, with asthma and atopy markers. Results: Of 115 patients, 46 (40%) completed spirometry pre- and post-bronchodilation. Of these, 26 (56.5%) demonstrated reversible airway obstruction (increase in %FEV1 predicted ≥ 10%). Obstruction reversibility was not found to be associated with a family history of asthma, blood eosinophil level, elevated IgE, or atopy symptoms. Of the 46 patients who completed bronchodilator spirometry, 29 (63%) were regularly using bronchodilators and inhaled corticosteroids. Conclusions: More than half of patients with PCD presented with reversible airway obstruction, without any correlation to markers of personal or familial atopy. Inhaled bronchodilators and corticosteroid therapies are commonly used for treating PCD. Evaluating bronchodilator response should be considered, and its effectiveness should be further studied.