RESUMO
AIM: In this cross-sectional descriptive study, we aimed to determine the clinical characteristics of children admitted to a tertiary hospital with asthma exacerbations in a city in southern Turkey where aeroallergens are common and to determine how these characteristics affect the severity of exacerbations. METHODS: Data from a cross-sectional analysis of children with asthma exacerbations who were followed up at the Cukurova University Medical Faculty Pediatric Emergency Department (ED) and Pediatric Allergy & Immunology inpatient clinic were retrospectively analyzed. The study included 106 children who were diagnosed with asthma and did not have any additional comorbidities. In a comparative analysis, the clinical characteristics and laboratory parameters of children with mild/moderate and severe exacerbations were examined. RESULTS: While 81.1% of the patients had mild/moderate exacerbation, 18.8% had severe exacerbation. Additional atopic disease, Alternaria positivity in the skin prick test, the frequency of exacerbations in the previous year, Streptococcus pneumoniae infection, and the rate of noncompliance with treatment were significantly higher in children with severe asthma exacerbations. PEF, FEV1, and FEV1/FVC values were considerably lower in patients with severe exacerbations. CONCLUSIONS: Bacterial infections, presence of atopic disease, Alternaria exposure, low spirometric measures, number of exacerbations in the previous year, and low rate of treatment adherence may be relevant in predicting the severity of asthma exacerbations.
RESUMO
Griscelli syndrome is a rare inherited autosomal recessive syndrome that causes immunodeficiency. Hemophagocytic lymphohistiocytosis (HLH), which is characterized by a high mortality rate, may develop because of Griscelli syndrome type 2 (GS2). We aimed to share our experience with the diagnosis and treatment methods of patients who developed HLH secondary to GS2. Patients with GS2 who were diagnosed and treated for HLH between 2017 and 2022 at the Cukurova University Division of Pediatric Allergy & Immunology and Division of Pediatric Hematology were included in the study. Microscopic examination of the hair shaft and next-generation sequencing for molecular genetic testing of RAB27A helped in the diagnosis of GS2. The first clinical presentation of 8 patients was HLH. One patient presented with CNS involvement and two patients presented with recurrent fever. Over 5 years, GS2 was diagnosed in 15 patients, of whom 11 (73.3%) developed HLH. The HLH-2004 protocol was used to treat these patients. Hematopoietic stem cell transplantation (HSCT) was performed in five patients who were matched with suitable donors. While all patients who underwent HSCT were alive, three patients who could not undergo HSCT because no donor could be found died. Deletion of CAAGC at nucleotides 514_518 in GS2 patients is associated with CNS involvement and a poor prognosis. HLH may be the first sign of presentation in patients with GS2. Although further research is needed, regardless of the conditioning regimen utilized, early HSCT remains the primary therapy option for preventing GS2-induced mortality in HLH.
