Saudi Med J
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Anonychia refers to the absence of nail plates owing to an autosomal dominant or recessive inheritance. Congenital anonychia is a rare condition that may be associated with other ectodermal or mesodermal malformations like epidermolysis bullosa, (deafness, onychodystrophy, osteodystrophy, and mental retardation) syndrome and Iso-Kikuchi syndrome. Here, we report 3 cases with anonychia congenita appearing in different generations of a single family in Kingdom of Saudi Arabia.
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To investigate the prevalence and significance of different endocrinopathies in children and adolescents with transfusion-dependent thalassemia and sickle-cell anemia. ⋯ This is a descriptive, retrospective study between January 2010 and July 2018 in King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Data was collected through reviewing electronic hospital medical records then filling out data collection sheets and was interpreted through the IBM SPSS Statistics for Windows version 20.0 (IBM Corp, Armonk, NY, USA). Results: The total sample size was 119 patients, gender equality was almost achieved with 55.5% being male and 45.5% being female. The most common endocrinopathies were identified in the following order of short stature (39.5%), diabetes mellitus (29.4%), hypogonadism (12.6%), osteopenia (12.6%), osteoporosis (9.2%), hypothyroidism (9.2%), hypocortisolism (3.4%), and hypoparathyroidism (2.5%). All of which were statistically significant in their relationship to hemoglobinopathies with the exception of osteopenia and osteoporosis. Hypogonadism and hypocortisolism were found to be statistically significant in their relationship to a positive history of splenectomy at p=0.026 and p=0.012. Short stature was found to be statistically significant in its relationship to the male gender with a p=0.001. Conclusion: Endocrinopathy is a frequent complication of hemoglobinopathies, for which the most common were found to be short stature, diabetes mellitus, and low bone mineral density.
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To evaluate the cardiovascular disease (CVD) as demonstrated by carotid intima-media thickness (cIMT) and the cluster risk factors of CVD including traditional and non-traditional, urinary functions, iron buildup, and hemorheology in rheumatoid arthritis (RA) patients of Gulf Cooperative Council (GCC) countries. ⋯ Carotid intima-media thickness was obtained from 216 RA patients, free of atherosclerotic diseases. The correlation between cIMT and the possible CVD risk factors was carried out using regression analysis. Results: The mean cIMT was observed as 0.58±0.11 mm. Mean age was 48±13 years. Univariate analysis revealed a positive association (p less than 0.05) between cIMT and age, body mass index, systolic blood pressure (SBp), and diastolic blood pressure, c-reactive protein (CRP), triglycerides (TG), low-density lipoprotein (LDL), erythrocyte sedimentation rate (ESR), hemoglobin (Hb), hematocrit (Hct), mean cell volume, platelet, monocytes, eosinophils, ferritin, creatinine, and uric acid. Negative relationship was observed between cIMT and glomerular filtration rate (GFR), transferrin, and high-density lipoprotein. Multiple linear regression analysis exhibited a positive association between cIMT and the age, LDL, eosinophil, SBp, and the ESR, whereas, negative connection with the GFR and transferrin. Conclusion: In this study, we found that the eosinophils, and low transferrin, are the potential candidates for the CVD risk factors in RA patients. Fasting blood glucose level was also observed to be a significant risk factor in diabetic as well as non-diabetic RA. The remaining CVD risk factors in RA patients of GCC countries including older age, high SBp, ESR, LDL, and low GFR were similar to the international population.
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[No Abstract Available].
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To determine demographic features of patients provided intravenous immunoglobulin (IVIG) excluding replacement therapy in the inpatient services and to evaluate indications for IVIG, and side effects related to therapy. Methods: Patients who received IVIG therapy between January 2016 and August 2018 were retrospectively identified. The demographic features, diagnosis, IVIG dose, number of days they underwent IVIG therapy, and whether or not they experienced IVIG-related side effects were recorded. Results: A total of 186 patients were included in this study, and of these, 89 (47.8%) were females. The median age of all of the patients was 48.5 months old (range 13-120 months). ⋯ Side effects were seen in 15 (3.81%) of the 394 IVIG infusions, 2 of which were acute kidney failure and nausea/vomiting as delayed onset side effects. The rapid onset side effects included fever (n=5), headache (n=3), rash and redness (n=2), and pain in the infusion area, hypotension, and hypertension (n=1). Conclusion: Intravenous immunoglobulin preparations are used for the treatment of many diseases due to their immunoregulatory effects. In recent years, the use of IVIGs without FDA approval has been increasing.