Frontal lobe like syndrome due to bee sting [Ari{dotless} sokmasi{dotless}na bagli{dotless} frontal lob sendromu]

A 34 year-old male beekeeper experienced flushing, breathing difficulty and loss of consciousness five minutes after he got stung by a honeybee. He was admitted to a local emergency room with hypotension and a phyliform pulse. After a 24-hour period of unresponsiveness, the patient gained consciousness and was discharged home. Two weeks later however, the patient presented with slowing of speech, personality and behavioral changes and difficulty in resolving personal needs such as dressing. Neuropsychological assessment revealed deficits in attention, concentration, executive function, recall memory, organization and coordination and slurred speech. Cranial MRI revealed marked symmetrical hyperintense lesions involving bilateral lentiform and caudate nuclei. Skin prick tests to hymenoptera species were negative. Specific IgE levels were also undetectable. The patient's baseline tryptase level was 49 ng/mL (normal <14.1 ng/ml). Bone marrow biopsy showed dense compact mast cell aggregates in CD117 (c-kit) and tryptase stained sections. Coexpression of CD25 and CD2 were identified on mast cells by flow cytometry. An activating somatic codon Asp816›Val KIT mutation was detected in mast cells but not in neutrophils. Based on these findings, diagnosis of systemic mastocytosis was made. This patient represents the first case in English literature who was diagnosed with sytemic mastocytosis and frontal lobe syndrome. Frontal lobe dysfunction may emerge with bilateral basal ganglia lesions. Mast cell degranulation has been documented to enhance vascular permeability and to regulate blood-brain barrier permeability. Besides probable hypoxic encephalopathy, the increased tendency for mast cells to undergo spontaneous degranulation can be explanatory for vasogenic edema in systemic mastocytosis

An important source for cat and house dust mite allergens: Day-care centers [Kedi ve ev tozu akan allerjenleri igin önemli bir kaynak: Anaokullan]

Objective: Exposure to indoor allergens during childhood has been associated with an increased risk of sensitization. There is no data about indoor allergen levels in day-care centers in Turkey. We hypothesized that day-care centers (DC) would be relevant sources of cat and mite allergens. Material and Methods: Fifty-seven dust samples were collected from 19 DCs in Izmir, their gardens, and classrooms of the primary schools where the DCs are located in. A questionnaire about characteristics of DCs was completed. Feld 1, Der p 1 and Der f 1 allergen levels were quantitated by enzyme-linked immunoassay. Results: Feld 1 was detected in all, and mite allergens in 94.7% of the samples. Levels exceeding sensitization threshold level for cat and mite allergens were present in 73.7%, and 21.1% of DCs, respectively. Feld 1 levels exceeding threshold level that might cause asthma exacerbation was detected in 21% of DCs. Feld 1 levels in DCs and their gardens were higher than the classrooms of the same school. Der f 1 levels were identical in DCs, gardens and classrooms. Der p 1 concentration was higher in DCs with air-conditioning, than DCs without a ventilation system. Although there was no difference for Feld 1 levels in DCs with or without carpeted floor, Feld 1 concentrations in DCs with carpet were significantly higher than in classrooms with no carpet. Conclusion: Day-care centers in Izmir are important sources of indoor allergens that could cause sensitization or even allergic symptoms in children and their staff. © 2012 by Türkiye Klinikleri

International Consensus on the Use of Genetics in the Management of Hereditary Angioedema

Hereditary angioedema (HAE) is becoming much more genetically complex than was initially considered. Thus, the role of HAE genetics is expanding beyond research laboratories, and the genotyping of subjects suffering from HAE has become diagnostically indispensable in clinical practice. The synthesis and interpretation of the clinical and biochemical analyses to facilitate appropriate genetic test selection has thus also become significantly more complex. With this in mind, an international multidisciplinary group of 14 experts in HAE genetics and disease management was convened in October 2018. The objective was to develop clear, actionable, evidence- and consensus-based statements aiming to facilitate the communication between physicians treating patients with HAE and clinical geneticists, and thus promote the effective use of genetics in the management of the disease. Eleven consensus statements were generated, encompassing considerations regarding the clinical indications for genotyping patients with angioedema, the methods of detection of HAE-causative variants, the variant pathogenicity curation, the genotyping of patients with HAE in the clinic, and genetic counseling. These statements are intended both to guide clinicians and to serve as a framework for future educational and further genetic testing developments as the field continues to evolve rapidly. © 2019 American Academy of Allergy, Asthma & Immunolog

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Background: The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1-INH-HAE. Methods: During an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting. Results: The symptoms of C1-INH-HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1-INH-HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1-INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1-INH-HAE family member should be screened for C1-INH deficiency. Pediatric patients should always carry a C1-INH-HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma-derived C1-INH, recombinant C1-INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center. Conclusions: The pediatric-focused international consensus for the diagnosis and management of C1-INH-HAE patients was created. © 2016 The Authors. Allergy Published by John Wiley & Sons Ltd