Prevalence of endocrine disorders and their associated factors in transfusion-dependent thalassemia patients: a historical cohort study in Southern Iran.

PURPOSE: Transfusion-dependent beta-thalassemia (TDT) patients suffer from various endocrinopathies. The main contributing factor associated with these complications is iron overload, secondary to frequent blood transfusions. To improve patients' quality of life, we evaluated the prevalence of endocrine disorders while considering the associated factors for further assessment. METHODS: Seven hundred thirteen transfusion-dependent thalassemia patients with age range 10-62 years were enrolled in this study. Serum calcium, phosphorous, fast blood sugar, ferritin, 25-OH vitamin D, free thyroxin, thyroid-stimulating hormone and parathyroid hormone were assessed. Bone mineral density was measured by dual-energy X-ray absorptiometry. RESULTS: In total, 86.8% of the TDT patients suffered from at least one endocrinopathy. The prevalence of endocrinopathies in descending order of frequency was low bone mass (72.6%), hypogonadism (44.5%), diabetes mellitus (15.9%), hypoparathyroidism (13.2%), and hypothyroidism (10.7%). Age, body mass index and splenectomy were significantly associated with most of the endocrine disorders. CONCLUSION: Endocrine complications are frequently observed in TDT patients. Splenectomy is a major risk factor and should be generally avoided unless it is highly indicated. Periodic surveillance of endocrine function and proper management of iron overload are advised.

Prevalence and Incidence of Non-neutralizing Antibodies in Congenital Hemophilia A- A Systematic Review and Meta-Analysis.

Objectives: In hemophilia A the presence of non-neutralizing antibodies (NNAs) against Factor VIII (FVIII) may predict the development of neutralizing antibodies (inhibitors) and accelerate the clearance of administrated FVIII concentrates. This systematic review aimed to assess: (1) the prevalence and incidence of NNAs in patients with congenital hemophilia without inhibitors and (2) the association between NNAs and patient and treatment characteristics. Methods: We conducted a search in MEDLINE, Embase, Web of Science and the Cochrane database. We included cross-sectional and longitudinal studies reporting on NNAs in patients with hemophilia A and B, who were inhibitor-negative at the start of the observation period. Data were extracted on: hemophilia type and severity, patient and treatment characteristics, NNA prevalence and incidence, NNA assays and inhibitor development. Two independent reviewers performed study selection, data extraction and risk of bias assessment, using adapted criteria of the Joanna Briggs Institute. Studies were classified as high-quality when ≥5/9 criteria were met. NNA assays were classified as high-quality when both quality criteria were met: (1) use of positive controls and (2) competition with FVIII to establish FVIII-specificity. We reported NNA prevalence and incidence for each study. The pooled NNA prevalence was assessed for well-designed studies in previously treated patients, employing high-quality NNA assays. Results: We included data from 2,723 inhibitor-negative patients with hemophilia A, derived from 28 studies. Most studies were cross-sectional (19/28) and none reported on NNAs in hemophilia B. Study design was of high quality in 16/28 studies and the NNA assay quality was high in 9/28 studies. Various NNA assays were used, predominantly ELISA (18/28) with different cut-off values. We found a large variety in NNA prevalence (Range, 0-100%). The pooled NNA prevalence in high-quality studies was 25% (95% CI, 16-38%). The incidence of new NNA development was reported in one study (0.01 NNA per person-exposure day). Conclusion: This systematic review identified studies that were heterogeneous in study design, patient population and NNA assay type, with NNA prevalence ranging from 0 to 100% in inhibitor-negative patients with hemophilia A. The pooled NNA prevalence was 25% in high-quality studies including only previously treated patients and performing high-quality NNA assays.

Evaluation of Sexual Life and Marital Satisfaction in Patients with Anogenital Wart. / Evaluación de la vida sexual y de la satisfacción matrimonial en pacientes con verruga anogenital.

Anogenital wart (AGW) resulting from human papilloma virus (HPV) is one of the most common sexually transmitted diseases worldwide. Herein, we aimed to evaluate the effects of AGW on patients'marital satisfaction and sexual function. This cross-sectional study was consisted of 74 consecutive patients including 37 patients with AGW and 37 healthy controls. ENRICH Marital Satisfaction Scale and Arizona Sexual Experience Scale were completed and analyzed for all patients. ENRICH scale items were not significantly different between the two groups. Sexual function was found to be lower in female than male patients (P<.001). According to our findings marital satisfaction was not affected by AGW. However, a history of previous treatment for HPV decreased the marital satisfaction. In addition, AGW had more adverse effects on females' sexual function compared to males. Our findings suggest that the psychiatric treatments should be considered when managing an AGW patient.

Successful Management of Vinblastin Overdose with Exchange Transfusion: A Case Report.

Accidental overdose of chemotherapy drugs including vinblastin (VBL) have been reported in the literature. As VBL overdose is potentially fatal, we decided to introduce a 2-year-old girl affected by langerhans' cell histiocytosis who was accidentally injected 10-times the prescribed dose of VBL (16 mg), and was saved with whole blood double exchange transfusion at 8 and 20 hrs after the accidental injection. The earliest manifestations were irritability and sinus tachycardia which alleviated after starting digoxin and at the end of the 2(nd) exchange transfusion. Other reported adverse effects were myelosuppression, weakness of extremities, diminished deep tendon reflexes and ileus which resolved at the time of discharge from hospital on day 13 of admission. It is speculated that exchange transfusion is an effective modality in reducing the serious adverse effects of VBL overdose.

Maternal and prenatal risk factors for childhood leukemia in southern of iran.

BACKGROUND: The causes of childhood leukemia as the most common malignancy in children are vastly unknown.The aim of this study is to evaluate the relationship between maternal birth characteristics with environmental exposures in childhood leukemia. METHODS: This is a case-control study which consists of children younger than 18 years old suffering from leukemia who reside at Fars Province of Iran. Patients were individually matched with variables such as age, sex and residence region. In order to evaluate the relationships between each variable and the risk of leukemia, odds ratio(OR) and 95% confidence interval (CI) were estimated using conditional logistic regression. RESULTS: Statistically, the association between risk of childhood leukemia with birth order (OR=6.177, 95%CI:2.551-14.957), pet ownership (OR=2.565, 95%CI: 1.352-4.868) and history of leukemia in first and second degree relatives (OR=2.667, 95%CI: 1.043-6.815) was significant. However, there was no significant association between daycare attendance, history of miscarriage, number of siblings and history of mother's diagnostic radiology tests with risk of childhood leukemia. CONCLUSION: Although no definite etiologic factor for acute childhood leukemia has been clearly defined, the contribution of environmental risk factors in the context of genetic predisposition are strongly elucidated.