Clinical features of chronic granulomatous disease: a series of 26 patients from a single center
Chronic granulomatous disease is a genetically determined immunodeficiency disorder affecting phagocytic cells rendering them unable to kill certain bacteria and fungi. The present study is a single-center retrospective study that aimed to document the clinical course of 26 children, with a m...
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| Format: | Article |
| Language: | English |
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Hacettepe University Institute of Child Health
2010-12-01
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| Series: | The Turkish Journal of Pediatrics |
| Online Access: | https://turkjpediatr.org/article/view/5066 |
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| author | Tuba Turul-Ozgür Gülten Türkkani-Asal Ilhan Tezcan M Yavuz Köker Ayşe Metin Leman Yel Fügen Ersoy Ozden Sanal |
| author_facet | Tuba Turul-Ozgür Gülten Türkkani-Asal Ilhan Tezcan M Yavuz Köker Ayşe Metin Leman Yel Fügen Ersoy Ozden Sanal |
| author_sort | Tuba Turul-Ozgür |
| collection | DOAJ |
| description |
Chronic granulomatous disease is a genetically determined immunodeficiency disorder affecting phagocytic cells rendering them unable to kill certain bacteria and fungi. The present study is a single-center retrospective study that aimed to document the clinical course of 26 children, with a median age of 2.5 years, from 21 families diagnosed as chronic granulomatous disease from 1989-2008. A median delay of 39 months was observed between the onset of infections and age at diagnosis. Pneumonia was the most common initial manifestation of the disease followed by lymphadenitis, skin abscess and diarrhea. An AR inheritance was predominant in the study group. All patients received antibacterial and antifungal prophylaxis, resulting in a marked decrease in the incidence of infections. Overall mortality was 19.2%. These results showed that all features in our group (clinical, progression and outcome) were similar to the literature except for the predominance of autosomal recessive form.
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| format | Article |
| id | doaj-art-e3118174dfc542079ee5149974955fd2 |
| institution | OA Journals |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2010-12-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-e3118174dfc542079ee5149974955fd22025-08-20T02:01:57ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212010-12-01526Clinical features of chronic granulomatous disease: a series of 26 patients from a single centerTuba Turul-Ozgür0Gülten Türkkani-AsalIlhan TezcanM Yavuz KökerAyşe MetinLeman YelFügen ErsoyOzden SanalDivision of Immunology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. Chronic granulomatous disease is a genetically determined immunodeficiency disorder affecting phagocytic cells rendering them unable to kill certain bacteria and fungi. The present study is a single-center retrospective study that aimed to document the clinical course of 26 children, with a median age of 2.5 years, from 21 families diagnosed as chronic granulomatous disease from 1989-2008. A median delay of 39 months was observed between the onset of infections and age at diagnosis. Pneumonia was the most common initial manifestation of the disease followed by lymphadenitis, skin abscess and diarrhea. An AR inheritance was predominant in the study group. All patients received antibacterial and antifungal prophylaxis, resulting in a marked decrease in the incidence of infections. Overall mortality was 19.2%. These results showed that all features in our group (clinical, progression and outcome) were similar to the literature except for the predominance of autosomal recessive form. https://turkjpediatr.org/article/view/5066 |
| spellingShingle | Tuba Turul-Ozgür Gülten Türkkani-Asal Ilhan Tezcan M Yavuz Köker Ayşe Metin Leman Yel Fügen Ersoy Ozden Sanal Clinical features of chronic granulomatous disease: a series of 26 patients from a single center The Turkish Journal of Pediatrics |
| title | Clinical features of chronic granulomatous disease: a series of 26 patients from a single center |
| title_full | Clinical features of chronic granulomatous disease: a series of 26 patients from a single center |
| title_fullStr | Clinical features of chronic granulomatous disease: a series of 26 patients from a single center |
| title_full_unstemmed | Clinical features of chronic granulomatous disease: a series of 26 patients from a single center |
| title_short | Clinical features of chronic granulomatous disease: a series of 26 patients from a single center |
| title_sort | clinical features of chronic granulomatous disease a series of 26 patients from a single center |
| url | https://turkjpediatr.org/article/view/5066 |
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