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Chorioretinal lesions in patients and carriers of chronic granulomatous disease.
Goldblatt, D; Butcher, J; Thrasher, A J; Russell-Eggitt, I.
Afiliação
  • Goldblatt D; Immunobiology Unit and Molecular Immunology Units, Institute of Child Health, The Great Ormond Street Children's Hospital, London, United Kingdom.
J Pediatr ; 134(6): 780-3, 1999 Jun.
Article em En | MEDLINE | ID: mdl-10356152
OBJECTIVE: To investigate the frequency of retinal lesions in patients with chronic granulomatous disease (CGD) and to seek such lesions in carriers. STUDY DESIGN: Seventy-four individuals from 33 families were recruited; 38 had CGD (30 X-linked and 8 autosomal recessive inheritance). All participants (including 33 control subjects) underwent measurement of visual acuity, anterior segment examination by slit lamp, and dilated funduscopy. RESULTS: Nine of 38 (23.7%) of the affected children had chorioretinal lesions compared with 0 of 33 control subjects. All 9 were known to have X-linked CGD and absent gp91(phox). The "typical" retinal abnormality consisted of "punched out" chorioretinal lesions associated with pigment clumping lying along major retinal vessels. Unexpectedly, 3 XL-CGD asymptomatic carriers also had typical chorioretinal lesions. CONCLUSION: Retinal lesions are relatively common in patients with XL-CGD and may interfere with vision and thus should be sought in such patients.
Assuntos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Retina / Corioide / NADPH Oxidases / Doença Granulomatosa Crônica Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged / Newborn Idioma: En Revista: J Pediatr Ano de publicação: 1999 Tipo de documento: Article País de afiliação: Reino Unido País de publicação: Estados Unidos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Retina / Corioide / NADPH Oxidases / Doença Granulomatosa Crônica Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male / Middle aged / Newborn Idioma: En Revista: J Pediatr Ano de publicação: 1999 Tipo de documento: Article País de afiliação: Reino Unido País de publicação: Estados Unidos