BACKGROUND Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity. DESIGN AND METHODS This was a cross-sectional study of 168 patients with β thalassemia intermedia t...

To cite: Singh A, Varma S. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2014-207637 DESCRIPTION A 19-year-old man presented with dysmorphic facies, progressive fatigability and exertional breathlessness from the age of 15 years. On examination the patient had classical ‘Chipmunk facies’ (figure 1), short stature, delayed puberty and mild splenomegaly. X-ray of...

Hydroxyurea (HU) has been successfully used in patients with β-thalassemia intermedia (β-TI). We aimed to evaluate the effect of the long-term use of HU on thyroid function in patients with β-TI. Seventy-five patients with β-TI aged≥11 years and taking HU were randomly selected during 2010 in southern Iran. Thirty-one patients with β-TI without HU were considered as a control group. Serum level...

Advances in treatment of thalassemia have led to the aging of thalassemic patients, and consequently concern about successful reproductive outcome is augmented. Although women with thalassemia intermedia only were considered competent of achieving pregnancy, case series reveal the willingness of both thalassemia major and thalassemia intermedia women to have a family. Pregnancy in general is ch...

background and purpose: patients with non-transfusion-dependent beta thalassemia major (ntdtm) could reach old age, marry and have children with appropriate care. this study aimed to review the marital status and maternal-fetal outcomes of ntdtm patients at thalassemia research center (trc) of sari, iran. methods: in this study, medical records of patients with β-thalassemia major were reviewed...

INTRODUCTION Iron overload is a major problem in patients with b-thalassemia major, and it has many structural and metabolic consequences. In this study, we aimed to consider the prevalence of endocrine abnormalities in patients with β-thalassemia major and thalassemia intermedia. MATERIALS AND METHODS We ordered following tests for consideration endocrine abnormalities: fasting plasma glucos...

We describe a case of a young male without stroke risk factors who presented with a sudden onset of left-sided weakness, left hand numbness, and left eye blurriness. CT scan of the head without contrast and diffusion-weighted MRI of the brain with contrast revealed an ischemic stroke in the right middle cerebral artery distribution. Transesophageal echocardiography (TEE) revealed a mobile pedun...

Moyamoya disease is a cerebrovascular disorder with unknown cause characterized by the occlusion of the bilateral internal carotid arteries (ICA) and proximal segments of ICA.1,2 On the other hand, moyamoya syndrome (MMS) is a rare form of this condition with underlying several pathologies including hematologic disorders, congenital syndromes, vascular malformations or vasculitis after irradiat...