Interleukin-1 Receptor Antagonist Gene Polymorphisms in Egyptian Children and Adolescents With Primary Immune Thrombocytopenia: Association With Disease Susceptibility, Response to Therapy, and Outcome.

Immune thrombocytopenia (ITP) is one of the most common hematologic disorders with poorly predictable clinical course and outcome. We studied the distribution of interleukin 1 receptor antagonist (IL-1Ra) gene polymorphism (intron-2) among children and adolescents with ITP and correlated IL-1Ra gene polymorphism to disease susceptibility, response to therapy, and outcome. Sixty children with ITP (mean age: 9.2±4.5 y) and 100 healthy controls (mean age: 8.83±4.05 y) were enrolled. The frequencies of the allele A2 and genotype A1A2 were significantly higher in patients compared with controls ( P <0.0001, P =0.0008, respectively). Allele A2 conferred 3.1 times increased relative risk for disease development. Allele A2 and genotypes A1A2 and A2A2 were significantly more frequent among remitted patients ( P =0.028 and 0.024, respectively). There was no significant difference between different genotypes and alleles regarding bleeding score ( P >0.05). Patients with polymorphic allele A2 (A1A2/A2A2) showed significantly better response to steroids than those with homozygous wild allele A1 ( P =0.028). IL-1Ra polymorphism might contribute to the susceptibility to ITP in Egyptian children. The presence of A2 polymorphic allele of IL-1Ra gene was found to be associated with better disease outcome and response to steroids than those with homozygous wild allele.

Analysis of Causes of Hospitalization Among Children with Sickle Cell Disease in a Group of Private Hospitals in Jeddah, Saudi Arabia.

PURPOSE: Sickle cell anemia (SCA) is a chronic hematologic condition that requires frequent hospitalization representing a significant economic burden on the health services. The aim of this study was to explore the causes and underlying factors of hospitalization among children with SCA, as well as the factors affecting their length of hospital stay. PATIENTS AND METHODS: This retrospective study included children and adolescents less than 16 years old who were admitted in a group of private hospitals in Jeddah, Saudi Arabia, during the period from January 2018 to December 2019. RESULTS: A total of 94 patients were included in this study, 59.6% were males, with a mean age 7.29 ± 3.82 years. The majority of the patients (91.5%) had sickle cell disease. The most common cause of hospital admission was vaso-occlusive crises (VOC) (64.9%) followed by infection (24.5%), acute chest syndrome (ACS) (18.1%), and acute hemolytic crisis (12.8%). We found no significant difference between gender and different causes of admissions (p > 0.05). While in relation to age group, limb pain and back pain were found to be significantly more frequent among children ≥ 7 years old (p = 0.03,0.04), while infections were significantly more frequent among children < 7 years old (p = 0.003). We analyzed the length of hospital stay and different factors, and we found that the mean length of hospital stay was significantly higher among children who were admitted with infections (p = 0.01) and ACS (p < 0.001) and among children who are non-compliant on hydroxyurea (p = 0.04). CONCLUSION: The most common cause of hospitalization among children with SCD in Jeddah, Saudi Arabia, was VOC followed by infection, ACS and acute anemia. The length of hospital stay was more prolonged among children with infection and ACS, as well as children who were non-compliant to hydroxyurea.

Analysis of the most common causes of blood donor deferral in northern Jeddah: a single-center study.

PURPOSE: We aimed to conduct a retrospective study in order to statistically analyze the commonest causes for blood donor rejection in northern Jeddah, Kingdom of Saudi Arabia according to the American Association of Blood Banks. This will help in developing better strategies to minimize the loss of treasured blood donors. SUBJECTS AND METHODS: A sample of 500 rejected donors was randomly selected from a single blood bank between October 2016 to May 2017. The evaluation of blood donors was according to the personal history questionnaire and a medical examination done before the blood donation proceeded. RESULTS: The causes of deferral were categorized into three main categories: personal factors, medical examination and medical history. The most common personal cause of deferral was lack of sleep (29 [5.80%]); however, the most common medical examination cause of deferral was low blood pressure (68 [13.60%]). Concerning the medical history, the commonest cause was cupping (58 [11.6%]). CONCLUSION: Low blood pressure (13.6%), cupping (11.6%) and less hours of sleep in the night prior to donation (5.8%) were the major causes of rejection in this study. Similarities and variations between the commonest causes of blood donor rejection may be due to the differences and similarities in the geographic area and in the cultural, educational and socioeconomic factors.

Serum Sclerostin Level and Bone Mineral Density in Pediatric Hemophilic Arthropathy.

OBJECTIVE: To assess serum sclerostin levels in relation to severity of arthropathy and bone mineral density (BMD) in children with hemophilic arthropathy. METHODS: This cross-sectional study included 40 male children suffering from Hemophilia A, and 10 matched healthy controls. Assessment of factor VIII deficiency degree, frequency of bleeding, type of treatment, body mass index (BMI), disease severity using the Hemophilia Joint Health Score (HJHS) and lumbar spine (LS) Z score for bone mineral density (BMD) using dual-energy X-ray absorbiometry was done. Serum sclerostin levels were measured for all patients and controls. RESULTS: Significant difference of serum sclerostin levels between the patient and control groups with Mean ± SD (0.09 ± 0.07 ng/ml) and (0.04 ± 0.01 ng/ml) (P value = 0.028) respectively was found. Significant positive correlations between serum sclerostin levels and the patients' age, and HJHS (P value <0.05) were found, while it had negative correlation with DEXA Z score, not reaching a significant value. LS-BMD-Z score levels ranged from (-4.5 to 1.2), with 15 patients with low BMD Z score (less than -2) representing 37.5% of total patients. CONCLUSIONS: Serum sclerostin levels are elevated in hemophilic children denoting bone metabolism affection and correlates with increased age, and HJHS. Increased levels of serum sclerostin may identify hemophilic patients at high risk for developing osteoporosis.