Assessment of thyroid function in children aged 1-13 years with beta-thalassemia major

Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life. Thyroid function and iron load status were evaluated in 90 children with a mean age of 7.17 +/- 3.78 years with beta-thalassemia major by measuring serum free thyroxin [FT4], serum free triiodothyronine [FT3], total thyroxin [T3], serum total triiodothyronine [T4], thyroid-stimulating hormone [TSH] and ferritin levels from serum of patients admitted to the Pediatric Department, Faculty of Medicine University of Dicle between March 2005 and July 2009. A control group formed from an age-sex matched healthy children with a mean age of 6.98 +/- 3.66 years was also included. A standard thyrotropin releasing hormone test was applied to 3 patients who had high TSH levels and were classified as subclinical primer hypothyroidism. The study was designed according to the Declaration of Helsinki and informed consent was obtained from the parents of all participants. All thyroid parameters in patients were in the normal ranges compared with the controls except three of them which had high TSH levels. Serum ferritin level [2703 +/- 1649 ng/mL] in patients was significantly higher than in controls [81.5 +/- 15.5 ng/mL]. The work implies that hypothyroidism could be even seen in the first decade of life in patients with beta-thalassemia major in spite of improved hematological cares

Intracranial myeloid metaplasia in idiopathic myelofibrosis

Extramedullary hematopoiesis is a common finding in idiopathic myelofibrosis and is generally found in the liver, spleen and lymph nodes, but meningeal extramedullary hematopoiesis is very rare. Some diseases may be causes of intracranial masses and diagnosis is difficult. We present a case diagnosed as intracranial and meningeal extramedullary hematopoiesis with idiopathic myelofibrosis inducing serious headache

Aase-Smith syndrome type II

Aase-Smith syndrome type II is rare in childhood and there are few reported cases. Here, we report an 8-month-old boy with congenital red cell aplasia and triphalangeal thumbs. In addition to thumb anomalies, he presented with growth failure, hypertelorism and novel osseous radiologic abnormalities, large fontanelles and micrognathia as extraordinary. Some clinical symptoms had complete clinical remission with deflazacort treatment