The best donors for hematopoietic SCT (HSCT) are fully-matched siblings. In patients without fully-matched siblings, HLA registries or cord blood banks are alternative strategies with some restrictions. Owing to the high rate of consanguineous marriage in our country, between 2006 and 2013, extended family searches were undertaken in Hematology-Oncology Research Center and Stem Cell Transplantation (HORCSCT), Tehran, Iran, in 523 HSCT candidates with parental consanguinity and no available HLA identical sibling. Fully-matched other-relative donors were found for 109 cases. We retrospectively studied the HSCT outcome in these patients. Median time to neutrophil engraftment was 13 days (range: 9-31days). In 83 patients, full chimerism and in 17 patients, mixed chimerism was achieved. Acute GvHD (aGvHD) grade II-IV appeared in 36 patients (33%). The frequency of aGvHD development in various familial subgroups was NS. Five patients expired before day+100. In the surviving 104 cases, chronic GvHD developed in 20 patients (19.2%). The distantly related subgroup had significantly a higher rate of cGvHD (P = 0.04). The 2-year OS and disease-free survival (DFS) were 76.7 ± 4.5% and 71.7 ± 4.7%, respectively. No significant difference in OS (P = 0.30) and DFS (P = 0.80) was unraveled between various familial relationships. Our considerable rate of fully-matched non-sibling family members and the favorable outcome support the rationale for extended family search in regions where consanguineous marriage is widely practiced.
Abstract- Cystic fibrosis (CF), as a fatal genetic condition, is associated with high morbidity and mortality rates. In Iran, limited studies exist on this disease. This study aimed to compare the demographic, clinical and paraclinical data of CF patients. This cross-sectional study was conducted in 2014-2015 on 174 CF patients referred to the Tehran Children Medical Center hospital, which is the main referral center for CF. For each patient, the forced expiratory volume in one second (FEV1) was measured, and the comparative demographic, clinical, and laboratory data of patients were recorded. Overall, 59% of studied patients were boys (n=102) and 41% were girls (n=72). The mean patient age (and standard deviations) was 7.1±5.7 years, with a range of 10 days to 28 years. In 67% of cases, the disease was diagnosed before their first birthday. The patients in this study were classified based on the FEV1 into mild (62%), moderate (33%) and severe (5%), indicating the degree of pulmonary complications. Cultures of respiratory secretions were positive for Pseudomonas aeruginosa and Staphylococcus aureus, in 23% and 16% of cases, respectively. In total, 61% of patients (n=83) were assigned to receive oral azithromycin for prophylaxis. Gastroesophageal reflux (reflux) was the most common gastrointestinal complication (35%). Regarding the complex nature of CF and the necessity of constant monitoring of patients during the life-span, the comparative demographic, clinical and laboratory analysis of patients and registering and standardization of patients’ data can be a major step in the better understanding of the disease, and thereby increasing the quality of life and life expectancy in the affected population.
Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therapeutic modality in the evaluation of respiratory disorders in pediatric population. Objectives:The study aims to highlight common indications and risk of complications associated with bronchoscopy in our population.Methods: This retrospective evaluation was performed in all patients that underwent flexible bronchoscopy at Children's Medical Center (affiliated to Tehran University of Medical Sciences) between April 2011 and September 2016.Results: Of 800 bronchoscopies, 574 (71.7%) were performed for diagnostic and 226 (28.2%) for therapeutic purposes. Major indications included radiographic abnormalities (30%), a foreign body or suspected foreign body (28.5% of all FFBs) and stridor or wheezing (25% of all FBB). The incidence of major complications associated with FFBs was 0.87%. The most frequent complication was pneumothorax, followed by lung hemorrhage, and respiratory failure. Conclusions:Our findings support early intervention and utilization of bronchoscopy in the pediatric population with variable respiratory complaints.
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