Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is a primary immune deficiency disorder of the phagocytes. In this disorder, phagocytic cells (polymorphonuclear leukocytes and monocytes) cannot produce active oxygen metabolites, and therefore, cannot destroy the ingested intracellular bacteria. Clinically, pati...
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2006-09-01
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| Series: | Libyan Journal of Medicine |
| Subjects: | |
| Online Access: | http://www.ljm.org.ly/articles/dec06/AOP060905.pdf |
| _version_ | 1818357301694693376 |
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| author | Ilka Schulze Salem Zaroog El-Hassan Dawi Abdulhamid Al-Tubuly Ahmed Al-Bousafy |
| author_facet | Ilka Schulze Salem Zaroog El-Hassan Dawi Abdulhamid Al-Tubuly Ahmed Al-Bousafy |
| author_sort | Ilka Schulze |
| collection | DOAJ |
| description | Chronic granulomatous disease (CGD) is a primary immune deficiency disorder of the phagocytes. In this disorder, phagocytic cells (polymorphonuclear leukocytes and monocytes) cannot produce active oxygen metabolites, and therefore, cannot destroy the ingested intracellular bacteria. Clinically, patients with CGD usually have recurrent bacterial and fungal infections causing abscess and granuloma formation in the skin, lymph nodes and visceral organs.In this report, we present a boy from Libya with a rare autosomal recessive trait of CGD (defect of p22-phox) who has chronic lung disease following multiple severe pneumonia attacks. The case we present suffered from bloody diarrhea since the third month of his life. He also had recurrent episodes of fever, and later, developed persistent cervical lymphadenitis and failure to gain weight. CGD is a very rare condition worldwide. It is also not recognized here in Libya, and usually not in the list of differential diagnosis for chronic pulmonary infections. We advise that pediatricians and general practitioners who treat chronic cases of lung diseases (with or without chronic diarrhea) should consider primary immunodeficiency disorders in the hope that early diagnosis and treatment may prevent chronic complications especially of the respiratory tract. Furthermore, we state that, to the best of our knowledge, this is the first documented case of CGD from Libya. |
| first_indexed | 2024-12-13T20:10:56Z |
| format | Article |
| id | doaj.art-52d49410580c48938b849304792f187e |
| institution | Directory Open Access Journal |
| issn | 1819-6357 |
| language | English |
| last_indexed | 2024-12-13T20:10:56Z |
| publishDate | 2006-09-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Libyan Journal of Medicine |
| spelling | doaj.art-52d49410580c48938b849304792f187e2022-12-21T23:32:55ZengTaylor & Francis GroupLibyan Journal of Medicine1819-63572006-09-0112AOP: 060905Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous DiseaseIlka SchulzeSalem ZaroogEl-Hassan DawiAbdulhamid Al-TubulyAhmed Al-BousafyChronic granulomatous disease (CGD) is a primary immune deficiency disorder of the phagocytes. In this disorder, phagocytic cells (polymorphonuclear leukocytes and monocytes) cannot produce active oxygen metabolites, and therefore, cannot destroy the ingested intracellular bacteria. Clinically, patients with CGD usually have recurrent bacterial and fungal infections causing abscess and granuloma formation in the skin, lymph nodes and visceral organs.In this report, we present a boy from Libya with a rare autosomal recessive trait of CGD (defect of p22-phox) who has chronic lung disease following multiple severe pneumonia attacks. The case we present suffered from bloody diarrhea since the third month of his life. He also had recurrent episodes of fever, and later, developed persistent cervical lymphadenitis and failure to gain weight. CGD is a very rare condition worldwide. It is also not recognized here in Libya, and usually not in the list of differential diagnosis for chronic pulmonary infections. We advise that pediatricians and general practitioners who treat chronic cases of lung diseases (with or without chronic diarrhea) should consider primary immunodeficiency disorders in the hope that early diagnosis and treatment may prevent chronic complications especially of the respiratory tract. Furthermore, we state that, to the best of our knowledge, this is the first documented case of CGD from Libya.http://www.ljm.org.ly/articles/dec06/AOP060905.pdfPhagocytesPrimary Immune deficiencyChronic Granulomatous DiseaseCGDP22-phox |
| spellingShingle | Ilka Schulze Salem Zaroog El-Hassan Dawi Abdulhamid Al-Tubuly Ahmed Al-Bousafy Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease Libyan Journal of Medicine Phagocytes Primary Immune deficiency Chronic Granulomatous Disease CGD P22-phox |
| title | Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease |
| title_full | Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease |
| title_fullStr | Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease |
| title_full_unstemmed | Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease |
| title_short | Libyan Boy with Autosomal Recessive Trait (P22-phox Defect) of Chronic Granulomatous Disease |
| title_sort | libyan boy with autosomal recessive trait p22 phox defect of chronic granulomatous disease |
| topic | Phagocytes Primary Immune deficiency Chronic Granulomatous Disease CGD P22-phox |
| url | http://www.ljm.org.ly/articles/dec06/AOP060905.pdf |
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