- the case definition for the syndrome of idiopathic cd4+ t-hymphocytopenia (1cl) includes a heterogeneity of disorders. as yet there have been very few published reports of chiulren who meet the whojcdc criteria and it is unclear whether icl is an acquired or inherited disorder. chiulren referred to a pacdiatric immunodeficiency unit between 1991 and 1993 for investigation of immunodeficiency ...

In this case report we will describe a rare association between anhyrotic ectodermal dysplasia (AED) and immunodeficiency and autoimmunity [in our case: Idiopathic Thrombocytopenic Purpura (ITP) and Crohn disease]. AED is a rare congenital disorder characterized by sparse hair, abnormal teeth and anhidrosis due to lack of eccrine glands. The survey of 87 cases with (AED) revealed only one Irrit...

Background Primary ciliary dyskinesia (PCD) is a rare disorder (prevalence 1/20000), caused by congenital dysmotility of the respiratory cilia. Humoral immunodeficiency (HID) often presents in a similar way with recurrent ear, nose and sinopulmonary infections, not seldom evolving to chronic lung disease. Although isolated IgG subclass deficiencies and IgA deficiency are common conditions, Comm...

Nine patients with immunoglobulin deficiency and various haematological disorders are presented. In all patients, recurrent infections had antedated the onset of the haematological disorder but, in most, the possibility of primary immunodeficiency had not been considered until after the haematological diagnosis had been established. The recognition of immunodeficiency is important since such pa...

Blastomycosis-like pyoderma (BLP) is a rare cutaneous disorder that results from an exaggerated response to bacterial infection, most commonly Staphylococcus aureus. There are variety of reported predisposing factors, including diabetes, obesity, leukemia, primary immunodeficiency, and immunomodulatory therapy. Clinical histopathological findings BLP can be quite disparate, often resulting in d...

common variable immunodeficiency (cv id) is a heterogeneous primary immunodeficiency disorder, characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. to determine the spectrum of infectious complications in patients with common variable immunodeficiency (cv id), we reviewed the hospital records of 64 cvid patients, who were diagnosed in children&a...