Thalassemia intermedia and extramedullary hematopoiesis

Excessive ineffective erythropoiesis in thalassemia intermedia may cause paravertebral pseudotumors of extramedullary hematopoiesis. Due to the proximity to the spinal canal, these paravertebral masses carry the risk of severe neurological damage. Treatment strategie include hypertransfusion, radiotherapy, and laminectomy.Hydroxyurea, stimulating fetal hemoglobin synthesis, may represent an alternative therapeutic approach. This presentation report a case of female known to have thalassemia intermedia who presented with abnormal gait with weakness of lower limbs. MRI study showed masses originate from thorax vertebra. Hypertransfusion therapy and splenectomy were followed by regular transfusion [baseline hemoglobin 10 g/dl] and chelation with desferrioxamine. With this treatment, clinical symptoms disappeared, paravertebral ematopoietic masses did not progress, Hydroxyurea therapy was initiated to reduce the required transfusion volume but suppressing concomitantly further expansion of extramedullary hematopoiesis, and finally leading to a reduction of transfusional iron load. Treatment was started with 4 mg/kg per day and stepwise increased to 20 mg/kg per day. Follow up to the patient done by clinical examination and imaging study to assess the vertebral masses

Rickets in children below 2 years

Although breast-feeding is highly appraised and widely practiced in Iraq, human milk supplies all necessary nutrients except a few including vitamin D. Despite abundance of sunshine, vitamin D deficiency rickets is not rare in Iraq. We carried out this study with an objective to determine presence, presentation and predisposing factors of rickets in pediatric patients attending Al Kadymia Teaching HospitAl Baghdad. This study was conducted in Department of pediatrics, AL Kahdymia Teaching Hospital over one year period from first of October 2008 to first of October 2009. Children from newborns to twenty fourth months of age presenting with signs and symptoms of rickets were included and information regarding signs, symptoms predisposing factors [crowded housing, isolated housing with deficient sun exposure, abundant sun but lack of awareness, malnutrition and antenatal factors] and investigations was recorded on a proforma. Diagnosis was based on clinical signs, radiological changes on x-ray of wrist joint and biochemical disturbances in serum levels of alkaline phosphatase, calcium and inorganic phosphorus. Sixty children with rickets reported during the study period. Overall, 40 infants [66.6%] were exclusively on breast feeding. The main clinical presentation was in the form of recurrent lower respiratory tract infection 30 infants [50%], recurrent diarrhea and delayed milestones 20 [33.3%] and seizure 6 patients [10%]. Skeletal changes on clinical examination were present in 30 [50%]. Radiological signs of rickets were present in 50 [83.3%]. Symptoms and signs reverted to normal in all cases after vitamin D supplementation. Nutritional rickets is still prevalent in Iraq, presenting with variable signs and symptoms, predisposing the childhood population to different illnesses and skeletal deformities. In the presence of abundant sunshine, lack of awareness of exposure to sun, may be the important predisposing factors for development of nutritional rickets