An ICET-A survey on occult and emerging endocrine complications in patients with ß-thalassemia major: Conclusions and recommendations.

In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries. The data reported by 15 countries are presented.The commonest endocrine complications registered in 3.114 TM adults are CH and GHD (4.6 % and 3.0 %, respectively), followed by latent hypocortisolism (1.2%). In 13 patients (0.41%) a cytological papillary or follicular thyroid carcinoma was diagnosed in 11 and 2 patients, respectively, and a lobectomy or thyroidectomy was carried out. Of 202 TM patients below the age of 18 years, the  reported endocrine complications were: GHD in 4.5%, latent hypocortisolism in 4.4% and central hypothyrodisim in 0.5%. Transition phase was an area of interest for many clinicians, especially as patients with complex chronic health conditions are responding to new treatments extending their lifespan beyond imagination.. In conclusion, our survey provides a better understanding of  physicians' current clinical practices and beliefs in the detection, prevention and treatment of some endocrine complications prevailing in adult TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are recommended.

Survival rate of childhood leukemia in shiraz, southern iran.

OBJECTIVE: Leukemia is the most prevalent type of cancer in children. The aim of this study was to estimate the 5-year survival rates of Acute Lymphoblastic Leukemia (ALL) and Acute Myeloblastic Leukemia (AML) as well as factors influencing them. METHODS: This is a nonrandomized retrospective study conducted on 280 patients with ALL and AML. They were all below 15 years old children admitted to Shahid Faghihi hospital, Shiraz, Iran from 2004 to 2008.The survival rates were estimated by applying the Kaplan-Meier method. In addition, the log rank test was used to estimate the statistical significance of differences in the survival probability. Cox regression model was applied to conduct multivariate analysis for adjusting confounding variable. All analyses were performed in SPSS statistical software (version 16). P-values less than 0.05 were considered as statistically significant. FINDINGS: The mean (± standard deviation) of the observation period was 28.2±16.1 months. In this period, 60 (24.7%) patients (47 ALL and 15 AML) passed away. The cumulative rate of survival in this study was 53.3±0.1 percent. This probability was 56.6±0.1% and 44.2±0.1% for ALL and AML patients, respectively, which indicates no statistically significant difference between them (P=0.8). According to Cox model, there was a significant relationship among the variables of platelet count and relapse with the survival rate. CONCLUSION: Platelet count was identified as a positive prognostic factor of the survival rate in ALL patients. However, on the base of our results and other studies, incidence of relapse and the number of relapses are significant factors of survival rates of leukemia.

Comparative evaluation of renal findings in Beta-thalassemia major and intermedia.

Thalassemia is a systematic disease in which the renal involvement has not yet been scrupulously studied. In a cross-sectional study, the renal findings of 50 cases of thalassemia intermedia (group 1) were compared to 58 patients with thalassemia major (group 2). Blood urea nitrogen, serum creatinine, uric acid, calcium, phosphorus, urinalysis, and ultrasonographical findings were evaluated. Mean age was 18 +/- 3.0 in group 1 and 17 +/- 3.5 years in group 2. The mean of serum ferritin levels was 871 +/- 81.8 ng/ml in group 1 vs. 3503 +/- 201 ng/ml in thalassemia major (p > 5) was observed among 19 children (17.6%); 17 of them were in group 1. In contrast, children with thalassemia major had significantly higher serum creatinine (0.89 +/- 0.18 vs. 0.59 +/- 0.37 mg/dl, p < 0.05) and blood urea nitrogen values (12.14 +/- 5.58 vs. 13.85 +/- 3.54 mg/dl, p < 0.05). We conclude that significant renal involvement is not a frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Microscopic hematuria in thalassemia intermedia might be related to either hypercalciuria or hyperuricosuria.