Document Detail


Psychosocial functioning and quality of life in children and families affected by AEC syndrome.
MedLine Citation:
PMID:  19504609     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome, also known as Hay-Wells syndrome, is a rare genetic condition that results in abnormalities of the skin, hair, nails, and teeth and requires frequent self-management and medical care. We sought to describe the psychological adjustment and quality of life in children and adolescents with AEC syndrome, as well as the impact of the child's illness on their families. The sample included 18 children and adolescents with AEC syndrome and their parents who attended the International Research Symposium on AEC syndrome. Parents completed standardized self-report questionnaires about child and family functioning and participated in a semi-structured interview about the child's cognitive and social functioning and the impact of AEC syndrome on the child and family. Children completed standardized self-report questionnaires of psychosocial functioning and quality of life. Overall, results reflected a range of functioning across children and families, with some families reporting few ill effects of the condition and others describing reduced quality of life and negative impact on child and family. Identifying the domains that may be impacted should help clinicians better screen for problems in functioning of children affected by AEC syndrome and their families.
Authors:
Mariella M Lane; William T Dalton; Sandra A Sherman; Alanna F Bree; Danita I Czyzewski
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-26     Completed Date:  2010-01-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1926-34     Citation Subset:  IM    
Copyright Information:
(c) 2009 Wiley-Liss, Inc.
Affiliation:
Menninger Department of Psychiatry and Behavioral Sciences, Baylor College of Medicine, Psychiatry and Psychology Service, Texas Children's Hospital, 6621 Fannin St., Houston, TX 77030-2399, USA. mmlane@texaschildrenshospital.org
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics,  psychology*
Adolescent
Adult
Child
Child, Preschool
Cleft Lip / genetics,  psychology*
Cleft Palate / genetics,  psychology*
Ectodermal Dysplasia / genetics,  psychology*
Eyelids / abnormalities
Female
Humans
Infant
Interviews as Topic
Male
Parents
Psychology
Quality of Life*
Questionnaires
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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