Chronic Granulomatous Disease (CGD) and Complete Myeloperoxidase Deficiency Both Yield Strongly Reduced Dihydrorhodamine 123 Test Signals but Can Be Easily Discerned in Routine Testing for CGD

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Chronic granulomatous disease (CGD) and complete myeloperoxidase deficiency both yield strongly reduced dihydrorhodamine 123 test signals but can be easily discerned in routine testing for CGD.

BACKGROUND The flow cytometric dihydrorhodamine 123 (DHR) assay is used as a screening test for chronic granulomatous disease (CGD), but complete myeloperoxidase (MPO) deficiency can also lead to a strongly decreased DHR signal. Our aim was to devise simple laboratory methods to differentiate MPO deficiency (false positive for CGD) and NADPH oxidase abnormalities (true CGD). METHODS We measur...

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Chronic granulomatous disease (CGD) presenting as quotidian fever

Clinical case The patient presented with 7 days of spiking fever not responding to oral antibiotics, arthralgias and a maculous rash. Laboratory results showed leukocytosis, thrombocytosis, CrP of 240 mg/l, ESR 100 mm. The symptoms persisted under intravenous antibiotic treatment. Focus work-up gave no evidence of an infectious origin, malignancy, IBD, vasculitis, connective tissue disease or p...

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Chronic Granulomatous Disease; fundamental stages in our understanding of CGD

It has been 50 years since chronic granulomatous disease was first reported as a disease which fatally affected the ability of children to survive infections. Various milestone discoveries from the insufficient ability of patients' leucocytes to destroy microbial particles to the underlying genetic predispositions through which the disease is inherited have had important consequences. Longterm ...

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Chronic granulomatous disease (CGD): an immunodeficiency diagnosed in pediatric ICU

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 ...

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X-linked chronic granulomatous disease in a male child with an X-CGD carrier, Klinefelter brother.

BACKGROUND Chronic granulomatous disease (CGD) is a rare primary immunodeficiency (PID) caused by a dysfunctional respiratory burst enzyme NADPH-oxidase. The concurrence of Klinefelter's Syndrome (KS) and CGD would be extremely rare. OBJECTIVE We describe the study of a family where the youngest male child had X-linked CGD (X-CGD) while his older brother was both an X-CGD carrier and a Klinef...

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ژورنال

عنوان ژورنال: Clinical Chemistry

سال: 2007

ISSN: 0009-9147,1530-8561

DOI: 10.1373/clinchem.2006.083444