BackgroundInternational guideline-recommended on-demand treatments for hereditary angioedema (HAE) include: C1-esterase inhibitor (plasma-derived or recombinant), or bradykinin-receptor antagonists. In most low- and middle-income countries (LMIC) these products are not registered or are unaffordable. Solvent-detergent, fresh or freeze-dried plasma therapy is thus the only available on-demand treatment in these settings; but published data on efficacy and safety are limited. This study evaluated the efficacy and safety of on-demand plasma treatment of acute HAE in two LMICs.MethodsA retrospective folder or patient registry review of acute swelling episodes necessitating emergency room attendance amongst known HAE patients was conducted at treatment centers in South Africa and Iran. Data collected included the site of angioedema, timing and amount of fresh frozen plasma (FFP) administered, time-to-resolution, hospital length of stay and adverse events.ResultsThere were 176 acute swelling episodes amongst 43 HAE patients; 98 were treated with FFP. The face, upper airway, and abdomen were involved in 15.3% (15/98), 53.1% (52/98) and 29.6% (29/98) of episodes treated with FFP respectively. Median (interquartile range ([IQR]) of FFP administered was 400 (280–560) mLs. In all episodes except two, FFP led to resolution, with median (IQR) hours to resolution 4 (2–12). Five transfusion reactions occurred, with one case of anaphylaxis and no deaths; giving an adverse reaction rate of 5%. Differences between South Africa and Iran included: (1) proportion of HAE type II(2) median (IQR) hours to FFP administration and hospitalization, (3) number of intubations after FFP infusion. Healthcare cost for FFP treatment was USD369- 791 in South Africa and USD275-550 in Iran, largely influenced by hospital length of stay.ConclusionsPlasma (fresh-frozen) remains the only available effective on-demand treatment for acute HAE in many countries. FFP is effective and safe, but time-to-resolution is slower and adverse events are more frequent than published data on targeted therapies. Overall healthcare cost of FFP approaches that of targeted therapies – now available through global access programs – when hospitalization is prolonged.

Background and Objective: Asthma is a major health problem and a high-burden disease in the world. Unfortunately the prevalence of asthma in adult populations in Iran is not determined precisely. The aim of this study was to assess the prevalence of asthma symptoms and it's relation with ages, gender and smoking behaviour in city of Tehran. Methods: A telephone interview survey was conducted among 7150 randomly selected telephone numbers in city of Tehran. A questionnaire adapted from The European Community Respiratory Health Survey (ECRHS) with additional two questions about smoking behaviour and hookah. Results: From 7150 eligible telephone numbers dialled there were 5500 responses (76.92%). The study populations included 3412 female (62%), 2088 men (38%) and the mean age of responder were 31.15 ± 7.33. Their smoking behaviour were; 71.6% non smoker, 11.9% active smoker, 16.5% passive smoker and 7.8% used hookah. The prevalence of "wheezing in the last 12 months", "dyspnea with wheezing" and "wheezing and two nocturnal symptoms" were 24%, 10.8% and 5% respectively. Prevalence of asthma symptoms was significantly greater in smokers than non-smokers groups. Conclusion: In this study by using symptom based questionnaire (ECRHS), we concluded the prevalence of asthma in Tehran adult population was between 5% to 10%. Also this study may made cross-national comparison of our findings with other ECRHS studies possible; rates of "wheezing in the last 12 months", "dyspnea with wheezing" and "nocturnal dyspnea" were higher than the European Community Respiratory Health Survey median. Role of air pollution, smoking and other risk factors for asthma should be considered.

Key Clinical Message DOCK8 deficiency is a rare autosomal recessive combined immunodeficiency with high IgE level, eosinophilia, severe eczema, extensive cutaneous viral, and respiratory bacterial infections, mostly in populations with higher prevalence of consanguinity. Molecular diagnosis of this gene is a useful approach for early diagnosis and timely HSCT due to deleterious consequences.

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Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase–positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X–linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow–up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results.