Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children.

Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children.

Unexpected relevant role of gene mosaicism in patients with primary immunodeficiency diseases.

Background: Postzygotic de novo mutations lead to the phenomenon of gene mosaicism. The 3 main types are called somatic, gonadal, and gonosomal mosaicism, which differ in terms of the body distribution of postzygotic mutations. Mosaicism has been reported occasionally in patients with primary immunodeficiency diseases (PIDs) since the early 1990s, but its real involvement has not been systematically addressed.

Growth and Endocrine Function in Tunisian Thalassemia Major Patients.

β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients.

[Effectiveness and acceptance of hydroxyurea in the treatment of severe forms of sickle cell disease: a prospective study of 65 cases].

Background: Sickle cell disease is a serious illness by its complications. For the severe forms, three therapeutic options are actually allowed: transfusion therapy, hydroxyurea and bone marrow transplantation. aim: To evaluate the contribution of hydroxyurea in the management of severe forms of sickle cell disease.