Hypocalcaemia due to hypoparathyroidism in beta-thalassemia major: a study of a new case

To report a new case of hypoparathyroidism in a child with beta thalassemia major. We report a case of a 17-year-old Tunisian girl with transfusion-dependent thalassemia major presented with paresthesia and pubertal delay. Laboratory investigations showed hypocalcaemia and hyperphosphatemia. Parathyroid hormone level was low [2 ng/1, normal range: 12-72 ng/1] than expected for the degree of hypocalcaemia. Serum ferritin concentration was 1770ng/ml. The patient was started on oral daily calcium supplementation, Alfa calciferol and intensive iron chelation therapy. Follow-up after 6 and 12 months revealed normal Calcium and ECG showed QT intervalwithin normal range. Investigating calcium homeostasis at regular intervals and early management of any abnormality can preclude the occurrence of complications

[ outcome of 105 Tunisian pediatric patients with sickle cell disease]

Sickle cell disease is widespread in the world. It is a public health problem in Tunisia. In this paper, we will study the outcome of 105 Tunisians infants affected by this disease. It is a retrospective study over 8 years, enrolling 105 Tunisian infants with sickle cell disease. Their main age is 10 years 7 months [19 months-18 years]. They are distributed into 58 boys and 47 girls. Sixty two of them ate homozygotes S/S, and 43 are double heterozygotes [S/, thalassemics 41 cases, S/O arab=1 case, S/C=1 case]. The main acute complications were vaso occlusive crises in 85 patients. They essentially reach the limbs. One hundred and twenty one events acute anemia" were observed. They are distributed into 107 events splenic sequestration [884%] and 14 events erythroblastopenia [11.6%]. Eleven of our patients [10.4%] have presented 16 events ischemic cerebral vascular [strokes]. Twenty events acute chest syndrome were observed in 15 patients [14.3%]. Infectious complications were noted in 83 patients [79%]. The priapism was observed in only 4 boys out of 58. The chronic degenerative complications were as follows: Femur head necrosis in 9 cases, degenerative retinopathy in one case and ulcer leg in one case. Hydroxyurea was reserved to 30 patients with a severe form of the disease. All of them improve the clinical outcome as attested by the number of days of hospitalisation/patient/year, which drops from 30 days to 3 days only [p<0,001]. We not deplore any death. By its complications, sickle cell disease is a potentielly serious disease. Hydroxyurea is an efficient treatment for the most severe forms

[ cord blood bank in Tunisia: usefulness and feasibility]

The cord blood is a souce of hematopoietic stem cells. This have encouraged the creation of many cord blood banks around the world. By this prospective study during 2 years, we tryed to show the advantages of such sources of progenitors, and to evaluate our techniques of collection, freezing, biological and microbiological survey. For this aim, we tested 100 specimens of cord blood before and after defrosting. Our selection criteria were a volume >/= 60 ml, a CD 34+ cells count >/= 2. 10[6], bacteriological and serological negativity. This 100 specimens have an average volume of 95,22 ml, an average nuclear cells count of 10,95.10[6] and an average CD 34+ cells count of 2,96. 10[6]. The average cells viability after defrosting is 96,69%, and the average efficiency rate of 92,11%. The specimens selected according to our selection criterias were of 39%. Our results confirm the feasibility of a cord blood bank in the "Centre National de Transfusion Sanguine" in Tunis