When fluorescein angioscopy or angiography is required in an infant, it is usually performed in the operating theater or neonatal unit. We report a case of an infant with incontinentia pigmenti in whom we were able to acquire angiographic information in an office setting by using an ultra-widefield non-contact system with oral fluorescein.
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Prevalence and clinical characteristics of incontinentia pigmenti: a nationwide population-based study.
Orphanet J Rare Dis
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Department of Dermatology, Aarhus University Hospital, Palle Juul-Jensens Boulevard 67, Aarhus N, 8200, Denmark.
Article Synopsis
- Incontinentia pigmenti (IP) is a rare X-linked dominant disorder linked to the IKBKG gene, and this study highlights the need for more research on its epidemiology in Denmark.
- A nationwide study identified 75 patients with IP, predominantly females, and estimated the birth prevalence at 2.37 per 100,000 live births, which is significantly higher than previous estimates.
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Incontinentia pigmenti underlies thymic dysplasia, autoantibodies to type I IFNs, and viral diseases.
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Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Inserm U1163, Necker Hospital for Sick Children, Paris, France.
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- * A study examined 131 female patients with X-linked dominant incontinentia pigmenti (IP), finding that 36% produced autoantibodies against IFN-α and/or IFN-ω, significantly higher than age-matched controls.
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Intravenous fluorescein overdose in a child undergoing fluorescein angiography.
J AAPOS
October 2024
Department of Ophthalmology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. Electronic address:
Fluorescein angiography is a fluorescent dye-based imaging procedure, most commonly indicated in the pediatric setting to evaluate peripheral retinal vascular lesions. Fluorescein dye is organic, water soluble, and largely excreted renally, with a reassuring safety profile at therapeutic doses. While toxicity with intrathecal overdose has been reported, the effect of intravenous exposure to supratherapeutic levels has not been previously documented in the literature.
View Article and Find Full Text PDFA case report of neonatal incontinentia pigmenti complicated by severe cerebrovascular lesions in one of the male monozygotic twins.
Front Pediatr
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Department of Neonatology, Shenzhen Baoan Women's and Children's Hospital, Shenzhen, China.
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Central nervous system anomalies in 41 Chinese children incontinentia pigmenti.
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Department of Emergency, Xi'an Children's Hospital (Xi'an Jiaotong University Affiliated Children's Hospital), No.69, Xiju Yuan Lane, Lianhu District, Xi'an, 86-710003, Shaanxi, People's Republic of China.
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