Chronic granulomatous disease (CGD): an immunodeficiency diagnosed in pediatric ICU
نویسندگان
چکیده
Background This paper aims at showing a severe case of a patient with Chronic Granulomatous Disease (CGD), an immunodeficiency which is not so uncommon, affecting 1/ 250000 born alive, and which is due to phagocytic dysfunction. It is characterized by inability of phagocytic cells to produce hydrogen peroxide and other oxidants needed to eliminate microorganisms, in addition there is excessive accumulation of immune cells into aggregates, known as granulomas, at sites of infection or inflammation. The patient may experience fungal or bacterial infections affecting the skin, lungs (Aspergillus pneumonias), lymph nodes, liver (liver abscesses 90% caused by Staphylococcus), bones (osteomyelitis), and granulomas which may lead to obstruction of gastrointestinal and urinary tracts.
منابع مشابه
Pulmonary Aspergillosis in a Previously Healthy 13-Year-Old Boy
Chronic granulomatous disease (CGD) is a rare, polygenic primary immunodeficiency. In this case report, we describe a previously healthy 13-year-old boy who presented with multifocal pulmonary aspergillosis and was subsequently diagnosed with an autosomal recessive form of chronic granulomatous disease. CGD has a variable natural history and age of presentation and should be considered when inv...
متن کاملTwo Chronic Granulomatous Disease Diagnosed in Adult Age.
Chronic granulomatous disease (CGD) is a rare, inherited primary immunodeficiency that is usually diagnosed at adulthood and is presented with recurrent bacterial and fungal infections. In this case report, two adult cases of CGD have been presented. A 29-year-old woman was referred to our clinic with hypoxic respiratory failure, with a pre-diagnosis of multidrug resistant tuberculosis (TB). He...
متن کاملSuccessful Treatment of Fungal Osteomyelitis with Voriconazole in a Patient with Chronic Granulomatous Disease
BACKGROUND Chronic granulomatous disease (CGD) is an immunodeficiency affecting phagocytic leukocytes. Defective respiratory burst mechanism renders the affected patients to be susceptible to catalase positive microorganisms. With the great successes in antibacterial prophylaxis and therapy, fungal infections are a persistent problem. Invasive aspergillosis is the most important cause of mortal...
متن کاملChronic Granulomatous Disease, Case Report and Review of Literature
Chronic granulomatous disease (CGD) is a rare inherited disorder characterized by inability of phagocytes to generate oxygen radicals needed for intracellular killing of phagocytic microorganisms. We report a 2.5-year-old Iranian female with multiple liver abscesses. She was admitted in surgical ward because of abdominal pain and fever for one month duration that had no response to conventio...
متن کاملThe search for a genetic defect in Polish patients with chronic granulomatous disease.
INTRODUCTION Chronic granulomatous disease (CGD)is a rare inherited disorder in which phagocytic cells are unable to generate superoxide anions. Patients with CGD are predisposed to recurrent bacterial and fungal infections because the superoxide-generating NADPH oxidase activity is needed for efficient killing of microbes. Among the at least 5 subunits cre-ating a functional NADPH oxidase, a m...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 8 شماره
صفحات -
تاریخ انتشار 2015