CHARGE Syndrome

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CHARGE syndrome, a term describing infants and young children born with severe abnormalities ( British Medical Journal, Vol. 304, No. 6840), occurs in one of about nine to ten thousand births and involves both physical and medical disabilities that differ between different children. In a majority of cases, CHARGE syndrome may not be found in any previous family medical history. Breathing problems and heart defects are examples of some life-threatening birth defects that are associated with CHARGE syndrome. Multiple surgeries and treatments are conducted after staying in hospitals for months; life at home may be hard for the patients if they encounter health issues such as difficulty breathing and swallowing. Delayed development as well as …show more content…

CHD7, a gene that has the role of turning other genes “on and off”, is the most common gene mutation that causes CHARGE, but other genes may also be mutated and have similar effects (CHARGE Syndrome Foundation, 1993-2015). CHD7, after further evaluations and genetic studies, was established as a vertebrate development regulator (Karim Bouazoune and Robert E. Kingston, 2012). After previous studies, results concluded that CHD7 functions as a regulator for both nucleoplasmic and nucleolar genes, paving a path into the investigation of pathogenesis in CHARGE syndrome (Chapter 1, Paragraph 3). Due to the birth defects caused by the gene mutation of CHD7, the name of this condition was given as the following: “Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and development, Genital and urinary abnormalities, and Ear abnormalities and deafness” (CHARGE Syndrome Foundation, …show more content…

Studies that were aimed to find the relationship between specific phenotypic and genotypic traits were not conducted by an otolaryngologist’s position, causing a lack of a detailed analysis of the senses tested. The goal of these studies was to make a connection between the genotype and phenotype while respecting the radiologic, audiometric, vestibular, gustatory, and olfactory parameters. A careful and close observation of the CHARGE phenotype and CHD7 will result in a better prediction of the function and deficits of a newly diagnosed patient. The goal of this study was to correlate genotypic information with phenotypes and the following: assessment of temporal bones and cranial nerves by radiology; audiometric data; and vestibular, olfactory, and gustatory testing and evaluation (Assessment of the Sensory Deficits in Individuals with CHARGE Syndrome and Correlation with Genotypic Data,

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