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

[Thrombocytopenic idiopathic purpura: predictive factors for chronic disease]

The aim of this study is to determine factors predicting development of chronic thrombocytopenic idiopathic purpura. It was a retrospective study, regarding the cases of PTI diagnosed in "service de medicine infantile C H"pital d'Enfants de Tunis" during 11 years. Acomparison was done between two groups: the first including acute PTI and the second including chronic and recurrent PTI. Factors predicting development of chronic disease were searched by the Fisher test which was significant when p < 0, 05. 33 cases of PTI were diagnosed. The ratio sex was 1.06. The mean age was five years and a half. The disease onset was insidious in three cases; all of them have had a chronic course. An haemorrhagic personnel background was present in three cases. Counselling cause was always petechies and ecchymoses. The mean platelets rate was 14555, 5/mm3. Therapeutics abstinence was carried out in five cases. 20 infants received an initial corticotherapy. The immunoglobulins were prescribed in nine cases, witch six associated to Corticotherapy. Three modes of outcome were discerned: acute PTI [n=23], recurrent PTI [n=4] and chronic PTI [n=6]. Factors predicting development of chronic disease were: haemorrhagic personnel background, an insidious disease onset and the failure of an initial therapeutic abstinence. Acute PTI is the most common. The disease had a chronic or recurrent outcome in 1/3 of cases. The search after factors predicting chronic disease allows an early prognosis. Thus, a best management of the disease can be achieved

[Neonatal heart failure revealing a cardiac rhabdomyoma with tuberous sclerosis]

Cardiac rhabdomyoma is the most common cardiac tumour in childhood and is often associated to tuberous sclerosis [TS]. However, a neonatal cardiac failure was uncommonly reported as the first manifestation of tuberous sclerosis. We report a case of a cardiac tumour revealed by a heart failure in a newborn who developed later clinical manifestations of TS. We report a case of a seven-day-old girl, admitted for cardiac failure. Echocardiography discovered a voluminous single mass in the right ventricle that affected partially heart output consistent with rhabdomyoma. Clinical course improved with symptomatic treatment. Seven months later, she developed generalized seizures associated to depigmental macules and facial neuro-fibroms. Cerebral magnetic resonance imaging showed cerebral hamartoms. Subsequent eight years follow-up revealed a spontaneous regression of cardiac rhabdomyoma and no further cardiac manifestations in contrast to a slight debility and a partial control of epilepsy. A cardiac rhabdomyoma discovered in neonates is a good indicator of TS suggesting careful follow-up and management