introduction nonclassical congenital adrenal hyperplasia (cah) is characterized by sufficient cortisol and aldosterone production at the cost of androgen overproduction. hydrocortisone or dexamethasone in supraphysiological doses are current treatment; however, their downside is suppression of endogenous cortisol production resulting in corticosteroid dependency. we aimed to treat children with...

Five preterm, very low birthweight infants with severe hypotension and adrenocortical insuYciency are described. The profound hypotension was resistant to volume expansion and inotrope treatment, but responded promptly to corticosteroid treatment. A human corticotrophin releasing hormone (hCRH) test performed before corticosteroid treatment showed adequate pituitary response, and the endocrine ...

Eight non-splenectomized patients with corticosteroid-refractory idiopathic thrombocytopenic purpura (ITP) were treated with low-dose vincristine (1 mg/week up to a total dose of 4 mg). Complete remission was achieved in 2 cases and partial remission in 3. Bleeding stopped in one patient who failed to remit. No statistical relationship was found between the response to vincristine and the durat...

autoimmune lymphoproliferative syndrome (alps) is a rare, inherited disorder of immune dysregulation secondary to defective lymphocyte apoptosis. this leads to uninhibited proliferation of lymphoid tissue manifesting with lymphadenopathy, hepatosplenomegaly, autoimmune cytopenia, and an increased risk of lymphoid malignancy. we report a 2 year old boy with fever, generalized lymphadenopathy, he...