idiopathic cd4+ lymphocytopenia is a rare combined immunodeficiency disease, characterized by low cd4+ t-cell count and increased susceptibility to opportunistic infections, autoimmunity and malignancies after exclusion of secondary forms of cd4 lymphocytopenia. here we present a 13-year old boy who was referred to our center because of destructive ulceration of soft and hard palates with exten...

Immunodeficiency is a common thought among both patients and physicians when confronted with what is perceived as an excessive number, duration, or severity of infections. Because of this, the starting point for evaluating patients for suspected immunodeficiency is based on what constitutes ”too many infections.” It generally is agreed that children with normal immune systems may have an averag...

INTRODUCTION Disorders of the auditory and vestibular system are often associated with human immunodeficiency virus infection and acquired immunodeficiency syndrome. However, the extent and nature of these vestibular manifestations are unclear. OBJECTIVE To systematically review the current peer-reviewed literature on vestibular manifestations and pathology related to human immunodeficiency v...

schimke immuno-osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid-resistant nephrotic syndrome, immunodeficiency, and spondyloepiphy-seal dysplasia. mutations in swi/snf2 related, matrix associated, actin dependent regulator of chromatin, subfamily a-like 1 (smarcal1) gene are responsible for the disease. the present report describes, for the first tim...

9.5 Range of Application, Dosage 9.5.1 Indications for Subcutaneous or Intramuscular Injection of Normal Immunoglobulins 9.5.2 Indications for Intravenous Injection of Normal Immunoglobulins 9.5.2.1 Primary Immunodeficiency Diseases 9.5.2.2 Secondary Immunodeficiency Diseases 9.5.2.3 High-Dose ivIg Treatment in Certain Autoimmune Diseases and Diseases of Unknown Etiology 9.5.3 Licensed Indicati...

We performed a prospective study of discontinuation of secondary prophylaxis against cytomegalovirus (CMV) in 36 patients with acquired immunodeficiency syndrome and quiescent CMV retinitis after successful treatment with highly active antiretroviral therapy (HAART). No reactivation or progression of retinitis was observed in 35 patients with persistent response to HAART, findings that support ...

Good's syndrome is extremely rare and refers to an acquired B and T cell immunodeficiency in thymoma patients. The authors of this article present a case report of a 75-year-old, caucasian male patient previously subjected to examinations for secondary dementia and recurrent infections, which revealed paraneoplastic syndrome arose from thymoma. He underwent thymectomy, while his immunodeficienc...

9.5 Range of Application, Dosage 9.5.1 Indications for Subcutaneous or Intramuscular Injection of Normal Immunoglobulins 9.5.2 Indications for Intravenous Injection of Normal Immunoglobulins 9.5.2.1 Primary Immunodeficiency Diseases 9.5.2.2 Secondary Immunodeficiency Diseases 9.5.2.3 High-Dose ivIg Treatment in Certain Autoimmune Diseases and Diseases of Unknown Etiology 9.5.3 Licensed Indicati...