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Titolo:
Increased susceptibility of a carrier of X-linked chronic granulomatous disease (CGD) to Aspergillus fumigatus infection associated with age-related skewing of lyonization
Autore:
Rosen-Wolff, A; Soldan, W; Heyne, K; Bickhardt, J; Gahr, M; Roesler, J;
Indirizzi:
Univ Hosp Carl Gustav Carus, Dept Pediat, D-01037 Dresden, Germany Univ Hosp Carl Gustav Carus Dresden Germany D-01037 037 Dresden, Germany
Titolo Testata:
ANNALS OF HEMATOLOGY
fascicolo: 2, volume: 80, anno: 2001,
pagine: 113 - 115
SICI:
0939-5555(200102)80:2<113:ISOACO>2.0.ZU;2-H
Fonte:
ISI
Lingua:
ENG
Keywords:
chronic granulomatous disease (CGD); lyonization; acquired skewing; neutrophils; aspergillus;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
6
Recensione:
Indirizzi per estratti:
Indirizzo: Roesler, J Univ Hosp Carl Gustav Carus, Dept Pediat, Fetscherstr 74, D-01037 Dresden,Germany Univ Hosp Carl Gustav Carus Fetscherstr 74 Dresden Germany D-01037
Citazione:
A. Rosen-Wolff et al., "Increased susceptibility of a carrier of X-linked chronic granulomatous disease (CGD) to Aspergillus fumigatus infection associated with age-related skewing of lyonization", ANN HEMATOL, 80(2), 2001, pp. 113-115

Abstract

Chronic granulomatous disease (CGD) is an inherited disorder characterizedby the inability of phagocytes to generate normal amounts of superoxide (O-2(-)), leaving patients susceptible to life-threatening infections. It waspreviously assumed that once carriers of the X-linked form of CGD were found to have 30% or more of functionally normal neutrophils, they would be free of risk for infection because the lyonization ratio was believed to be constant. Our report strongly contradicts this assumption. A 45-year-old X-CGD carrier had approximately 40% of normal neutrophils in her peripheral blood at age 21 years. Recently, she contracted a life-threatening pulmonary infection with Aspergillus fumigatus. After recovery, the ratio of normal-to-nonfunctional neutrophils was re-evaluated. She was found to have only 6-8% of normal neutrophils, suggesting that a striking decrease in the numberof normal cells over the past 25 years was the reason for an increased susceptibility to Aspergillus infection. We conclude that age-related acquiredskewing of the lyonization ratio can result in an increased susceptibilityto life-threatening infections in X-CGD carriers.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/11/20 alle ore 06:32:53