dc.contributor.author | Palanduz, Ayşe | |
dc.contributor.author | ABEL, Laurent | |
dc.contributor.author | CASANOVA, Jean-Laurent | |
dc.contributor.author | PLANCOULAINE, Sabine | |
dc.contributor.author | Canpolat, Nur | |
dc.contributor.author | AYDOGAN, Gonul | |
dc.contributor.author | CASSAR, Olivier | |
dc.contributor.author | SAHIN, Guerses | |
dc.contributor.author | ERTEM, A. Ulya | |
dc.contributor.author | TELHAN, Leyla | |
dc.contributor.author | JOUANGUY, Emmanuelle | |
dc.contributor.author | PICARD, Capucine | |
dc.contributor.author | GESSAIN, Antoine | |
dc.date.accessioned | 2021-03-04T11:26:22Z | |
dc.date.available | 2021-03-04T11:26:22Z | |
dc.date.issued | 2010 | |
dc.identifier.citation | SAHIN G., Palanduz A., AYDOGAN G., CASSAR O., ERTEM A. U. , TELHAN L., Canpolat N., JOUANGUY E., PICARD C., GESSAIN A., et al., "Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds", PEDIATRICS, cilt.125, sa.3, 2010 | |
dc.identifier.issn | 0031-4005 | |
dc.identifier.other | vv_1032021 | |
dc.identifier.other | av_72564576-dbb8-42c0-8440-5b5a0248d18a | |
dc.identifier.uri | http://hdl.handle.net/20.500.12627/78692 | |
dc.identifier.uri | https://doi.org/10.1542/peds.2009-2224 | |
dc.description.abstract | Infection by human herpesvirus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases having been reported since 1960. We recently reported 2 children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this article, we describe 3 unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at 2 years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly, and the patient died within 3 months despite treatment with vincristine. The other 2 children developed a milder form of KS at the age of 9 years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at the age of 14, 2 years after treatment. The second girl is currently stabilized with etoposide, which was begun 4 months ago. None of the 3 children had any relevant familial history or other clinical features. The occurrence of classic KS in 3 unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children. Pediatrics 2010; 125: e704-e708 | |
dc.language.iso | eng | |
dc.subject | Çocuk Sağlığı ve Hastalıkları | |
dc.subject | Dahili Tıp Bilimleri | |
dc.subject | Sağlık Bilimleri | |
dc.subject | Tıp | |
dc.subject | Klinik Tıp (MED) | |
dc.subject | Klinik Tıp | |
dc.subject | PEDİATRİ | |
dc.title | Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds | |
dc.type | Makale | |
dc.relation.journal | PEDIATRICS | |
dc.contributor.department | Bakirkoy Children''s & Maternity Hospital , , | |
dc.identifier.volume | 125 | |
dc.identifier.issue | 3 | |
dc.contributor.firstauthorID | 93917 | |