Use and Effectiveness of Sleep Medications by Parent Report in Individuals with Williams Syndrome

Martens, Marilee A.; Seyfer, Daisha L.; Andridge, Rebecca; Coury, Daniel L.

doi:10.1097/dbp.0000000000000503

Cited by 10 publications

(4 citation statements)

References 41 publications

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“…<1 in female [84] Because of the apparent normal developmental course in early childhood, diagnosis may be delayed [116] Developmental Sleep problems are common in Rett's syndrome but there is some variation with age and mutation type [116] Impaired secretion of melatonin [117] Exogenous melatonin improved the sleep-wake cycle and sleep onset. The effect was maintained over 2 years without any adverse effects [117,118] The WBS group had shallower drops in cortisol and less pronounced increase in melatonin at bedtime compared to the control group [129,130] Melatonin was the most frequently reported medication taken for sleep problems in WBS, with 91% of parents reporting benefits for their child with WBS, and very few, if any, side effects [131,132]…”

Section: Relationships Between Melatonin and Neurodevelopmental Disor...mentioning

confidence: 99%

Melatonin in Neurodevelopmental Disorders: A Critical Literature Review

Feybesse,

Chokron,

Tordjman

2023

Antioxidants

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The article presents a review of the relationships between melatonin and neurodevelopmental disorders. First, the antioxidant properties of melatonin and its physiological effects are considered to understand better the role of melatonin in typical and atypical neurodevelopment. Then, several neurodevelopmental disorders occurring during infancy, such as autism spectrum disorder or neurogenetic disorders associated with autism (including Smith–Magenis syndrome, Angelman syndrome, Rett’s syndrome, Tuberous sclerosis, or Williams–Beuren syndrome) and neurodevelopmental disorders occurring later in adulthood like bipolar disorder and schizophrenia, are discussed with regard to impaired melatonin production and circadian rhythms, in particular, sleep–wake rhythms. This article addresses the issue of overlapping symptoms that are commonly observed within these different mental conditions and debates the role of abnormal melatonin production and altered circadian rhythms in the pathophysiology and behavioral expression of these neurodevelopmental disorders.

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Section: Relationships Between Melatonin and Neurodevelopmental Disor...mentioning

confidence: 99%

Melatonin in Neurodevelopmental Disorders: A Critical Literature Review

Feybesse,

Chokron,

Tordjman

2023

Antioxidants

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“…Crianças em idade escolar podem sofrer com distúrbios do sono do estágio II e sono REM. Isso pode ser ocasionado pela ansiedade que apresentam e por possíveis anormalidades endócrinas, as quais resultam em produção reduzida de melatonina e aumentada de cortisol durante a noite, sugerindo disfunção pineal ou circadiana (ANNAZ et al, 2011;MARTENS et al, 2017).…”

Section: Desenvolvimentounclassified

Correlações entre o fenótipo na síndrome de Williams e os genes deletados / Correlations between the phenotype in Williams syndrome and the deleted genes

Medeiros

Praxedes

2021

BJHR

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RESUMOA síndrome de Williams-Beuren (SWB) apresenta padrão de herança autossômico dominante, decorrente de uma microdeleção no braço longo do cromossomo 7, na região 7q11.23, que engloba de 26 a 43 genes. Os principais sinais da síndrome são características faciais dismórficas, alterações cognitivas, constipação, estenose aórtica e da artéria pulmonar. O diagnóstico genético é confirmado pela hibridização fluorescente in situ (FISH), análise cromossômica por microarray (CMA) ou por hibridização genômica comparativa (CGH-array). O objetivo desta pesquisa foi realizar uma revisão bibliográfica, para descrever as associações já registradas entre o quadro fenotípico apresentado pelos portadores da síndrome e a exclusão de genes decorrente da microdeleção. Para a revisão sistemática foram pesquisados e analisados artigos científicos obtidos a partir de busca nas bases de dados PubMed, LILACS e Bireme, além de livros e sites específicos. Aproximadamente 96% dos indivíduos com SWB apresentam exclusão do gene da elastina que codifica uma proteína presente no tecido conjuntivo, justificando os comprometimentos cardiovasculares, elasticidade da pele, voz rouca, frouxidão articular, clinodactilia, hérnia inguinal, envelhecimento prematuro, diverticulose vesical e de cólon, características faciais dismórficas (abundância de tecido adiposo periorbitário, bochechas e lábios proeminentes e má oclusão dentária) e perda auditiva. Outros genes deletados relacionados ao sistema cardiovascular são BCL7B, TBL2, MLXIPL, EIF4H, LAT2 e NCF1. Em relação às alterações de comunicação e comportamento verificada nos pacientes, sugere-se que estejam relacionadas à deleção dos genes: GTF2I, NFXL1, BAZ1B, CLIP2, DNAJC30, GTF2I, GTF2IRD1 e GTF2IRD2. Quanto à hiper sociabilidade, atenção focada, estímulos emocionais, sugerese uma relação com a ocitocina e vasopressina, devido a uma desregulação do sistema

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“…Sleep disorders are common in individuals with Williams syndrome and may have a significant negative impact on attention, learning and behavior. Parents often use medications such as melatonin to help their children with Williams syndrome sleep better . The neurodevelopmental profile of patients with Williams syndrome is characterized by high sociability and a variety of retained and compromised linguistic and cognitive abilities.…”

Section: Medical Interventional and Surgical Managementmentioning

confidence: 99%

Williams syndrome

Twite

Stenquist

Ing

2019

Pediatric Anesthesia

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Summary Williams syndrome affects approximately one in 10 000 people and is caused by the deletion of genes on chromosome 7q11.23 which code for elastin. The phenotypic appearance of people with Williams syndrome is well characterized, but there continues to be new genetic and therapeutic discoveries. Patients with Williams syndrome have increased morbidity and mortality under sedation and anesthesia, largely as a result of cardiovascular abnormalities. This review article focuses on new information about Williams syndrome and outlines a structured approach to patients with Williams syndrome in the perioperative period.

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Use and Effectiveness of Sleep Medications by Parent Report in Individuals with Williams Syndrome

Cited by 10 publications

References 41 publications

Melatonin in Neurodevelopmental Disorders: A Critical Literature Review

Melatonin in Neurodevelopmental Disorders: A Critical Literature Review

Correlações entre o fenótipo na síndrome de Williams e os genes deletados / Correlations between the phenotype in Williams syndrome and the deleted genes

Williams syndrome

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