Search results for "PHENOTYPE CORRELATIONS"

showing 2 items of 12 documents

2q13 microdeletion syndrome: Report on a newborn with additional features expanding the phenotype

2021

In this paper we describe an additional newborn patient with craniofacial dysmorphisms, congenital heart disease, hypotonia and a 2q13 deletion of 1.7 Mb, whose clinical and genomic findings are consistent with the diagnosis of 2q13 microdeletion syndrome.

Pediatricsmedicine.medical_specialtyMedicine (General)Heart diseaseCNVgenotype-phenotype correlationR5-920newborngenotype‐phenotype correlationsfollow-upmedicineCraniofacialGenotype-Phenotype Correlationschromosome 2newborn.GeneticsCNVsfollow‐upbusiness.industryRGeneral MedicineMicrodeletion syndromemedicine.diseasePhenotypeHypotoniaMedicinemedicine.symptombusinessClinical Case Reports
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WWOX-related encephalopathies: delineation of the phenotypical spectrum and emerging genotype-phenotype correlation

2014

International audience; BACKGROUND:Homozygous mutations in WWOX were reported in eight individuals of two families with autosomal recessive spinocerebellar ataxia type 12 and in two siblings with infantile epileptic encephalopathy (IEE), including one who deceased prior to DNA sampling.METHODS:By combining array comparative genomic hybridisation, targeted Sanger sequencing and next generation sequencing, we identified five further patients from four families with IEE due to biallelic alterations of WWOX.RESULTS:We identified eight deleterious WWOX alleles consisting in four deletions, a four base-pair frameshifting deletion, one missense and two nonsense mutations. Genotype-phenotype correl…

WWOXMicrocephaly[SDV]Life Sciences [q-bio]Nonsense mutationMutation MissenseBiology03 medical and health sciences0302 clinical medicineGeneticsmedicineHumansSpinocerebellar AtaxiasMissense mutationAlleleGenetics (clinical)infantile030304 developmental biologyGeneticsComparative Genomic Hybridization0303 health sciences[ SDV ] Life Sciences [q-bio]Tumor Suppressor ProteinsChromosomal fragile siteHigh-Throughput Nucleotide Sequencinggenotype/phenotype correlationsmedicine.diseaseNull allele3. Good healthPhenotypeWW Domain-Containing OxidoreductaseCodon Nonsenseintellectual disabilitySpinocerebellar ataxiaOxidoreductasesSpasms Infantilehigh throughput data mining030217 neurology & neurosurgeryJournal of Medical Genetics
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