Assessing the integrity of auditory processing and sensory memory in adults with cystinosis (CTNS gene mutations)

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CTNS mutations in publicly-available human cystinosis cell lines

Patient samples play an important role in the study of inherited metabolic disorders. Open-access biorepositories distribute such samples. Unfortunately, not all clinically-characterized samples come with reliable genotype information. During studies directed toward population frequency assessments of cystinosis, a rare heritable disorder, we sequenced the CTNS gene from 14 cystinosis-related s...

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Relation between Working Memory Capacity and Auditory Stream Segregation in Children with Auditory Processing Disorder

Background: This study assessed the relationship between working memory capacity and auditory stream segregation by using the concurrent minimum audible angle in children with a diagnosed auditory processing disorder (APD).Methods: The participants in this cross-sectional, comparative study were 20 typically developing children and 15 children with a diagnosed APD (age, 9–11 years) according to...

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First Report of CTNS Mutations in a Chinese Family with Infantile Cystinosis

Infantile cystinosis (IC) is a rare autosomal recessive disorder characterized by a defect in the lysosomal-membrane transport protein, cystinosin. It serves as a prototype for lysosomal transport disorders. To date, several CTNS mutations have been identified as the cause of the prototypic disease across different ethnic populations worldwide. However, in Asia, the CTNS mutation is very rarely...

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Objectives: This study investigated the efficacy of working memory training for improving working memory capacity and related auditory stream segregation in auditory processing disorders children. Methods: Fifteen subjects (9-11 years), clinically diagnosed with auditory processing disorder participated in this non-randomized case-controlled trial. Working memory abilities and auditory strea...

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ژورنال

عنوان ژورنال: Orphanet Journal of Rare Diseases

سال: 2021

ISSN: 1750-1172

DOI: 10.1186/s13023-021-01818-0