Acute primary purulent pericarditis in an adult patient with unknown X-linked agammaglobulinemia |
| |
| Affiliation: | 1. Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil;2. Núcleo Multidisciplinar de Pesquisa UFRJ – Xerém em Biologia (NUMPEX-BIO), Campus Duque de Caxias Professor Geraldo Cidade – Universidade Federal do Rio de Janeiro, Duque de Caxias, Rio de Janeiro, Brazil;3. Laboratório Interdisciplinar de Pesquisas Médicas, Instituto Oswaldo Cruz, Fundação Oswaldo Cruz, Brazil;4. Núcleo de Doenças Infecciosas/ Núcleo de Biotecnologia- Universidade Federal do Espírito Santo, Vitória, Espírito Santo, Brazil;5. Laboratório de Imunopatologia, Instituto Oswaldo Cruz, Fundação Oswaldo Cruz, Brazil;6. Faculdade de Medicina, Centro de Pesquisa em Tuberculose, Universidade Federal do Rio de Janeiro, Brazil;7. Instituto de Veterinária, Universidade Federal Rural do Rio de Janeiro, Seropédica, RJ, Brazil;1. University of Chile, Faculty of Physical and Mathematical Sciences, CeBiB, Chile;2. University of Chile, Faculty of Medicine, ICBM, Immunology Disciplinary Program, Chile;3. University of Chile, Faculty of Medicine, Anatomy and Developmental Biology Disciplinary Program, ICBM, Experimental and Molecular Embryology Laboratory, Chile;1. Department of Biomedical Sciences, Heritage College of Osteopathic Medicine, Ohio University, United States;2. Molecular and Cellular Biology Program, Ohio University, United States;3. Biomedical Engineering Program, Russ College of Engineering and Technology, Ohio University, United States;4. The Diabetes Institute at Ohio University, United States;1. Henan Institute of Medical and Pharmaceutical Sciences, Zhengzhou University, Zhengzhou, 450052, Henan, China;2. Academy of Medical Science, Zhengzhou University, Zhengzhou, 450001, Henan, China;3. Henan Key Laboratory of Tumor Epidemiology, Zhenghzou University, Zhengzhou, 450052, Henan, China;4. Department of Clinical Laboratory, Fuwai Central China Cardiovascular Hospital, Zhengzhou, 451464, Henan, China;5. Department of Clinical Laboratory, Zhengzhou Hospital of Traditional Chinese Medicine, Zhengzhou, 450000, Henan, China;1. Singapore Immunology Network (SIgN), A*STAR, Singapore;2. Department of Microbiology and Immunology, National University of Singapore (NUS), Singapore, Singapore;1. Department of Oral Biology, College of Dentistry, University of Florida, Gainesville, FL, USA;2. Department of Diagnosis and Surgery, UNESP-State University of Sao Paulo, School of Dentistry at Araraquara, Araraquara, SP, Brazil;3. Department of Physical Therapy, University of Florida Health Science Center, Gainesville, FL, USA;4. Department of Stomatology, School of Dentistry, Federal University of Goias (UFG), Goiania, GO, Brazil;5. Department of Foundational Sciences, College of Dental Medicine, East Carolina University, Greenville, NC, USA |
| |
| Abstract: | X-linked agammaglobulinemia (XLA) is a rare form of inherited immunodeficiency due to an impairment in B-lymphocyte differentiation and maturation. In the majority of cases XLA is diagnosed in childhood, particularly among males affected by recurrent infections and with a family history of immunodeficiency. Infections of respiratory tract, gastrointestinal apparatus, eyes, nose and ears are frequent in XLA patients; on the contrary, infections of myocardium, cardiac valves and pericardium are rarely described in XLA.A 34-year-old man with unknown XLA was hospitalized because of syncope, due to pericardial tamponade, caused by acute primary purulent pericarditis. Immediate pericardiocentesis was effective in improving hemodynamics, and empiric antibiotic therapy was successful in controlling the infection.Purulent pericarditis is a rare disease with high mortality rate: it is usually caused by hematogenous bacterial propagation, direct infection of pericardial space by chest wounds or thoracic surgery, or extension of infection from adjacent tissues. However, this patient had no recent local or systemic infections. Because of unusual clinical picture during hospitalization he underwent further clinical and laboratory evaluations, that showed low immunoglobulin levels. After exclusion of acquired immunodeficiency, genetic tests were performed: they detected deletion of exons 8-9-10 of Bruton Tyrosine Kinase gene on X chromosome, leading to the diagnosis of XLA.Acute purulent primary pericarditis may also occur in adult XLA patients as first clinical manifestation. According to this case report, a primary immunodeficiency syndrome should be considered in patients with atypical cardiac infections and no predisposing conditions, regardless of age. |
| |
| Keywords: | Purulent pericarditis X-linked agammaglobulinemia Cardiac infection Bruton tyrosine kinase gene |
| 本文献已被 ScienceDirect 等数据库收录！ |
|
