Defects of renal tubular homeostasis and cystogenesis in the knockout.

Loss of -gene function causes autosomal recessive polycystic kidney disease (ARPKD) characterized by bilateral severely enlarged kidneys and congenital liver fibrosis requiring kidney replacement therapy most frequently during childhood. Studies using renal tissue from ARPKD patients suggest cyst promotion by suppressed hippo activity and enhanced Src/STAT3-signaling. We address renal homeostasis in female -knockout mice, aged 3 to 9 months, and observe features in common with late-onset ARPKD.

Age Estimation and Gender Attribution in Typically Developing Children and Children With Dysarthria.

Purpose: The purposes of this study were (a) to investigate adult listeners' perceptions of age and gender in typically developing children and children with dysarthria and (b) to identify predictors of their estimates among auditory-perceptual parameters and an acoustic measure of vocal pitch (0). We aimed to evaluate the influence of dysarthria on the listeners' impressions of age and gender against the background of typical developmental processes.

Method: In a listening experiment, adult listeners completed age and gender estimates of 144 typically developing children (3-9 years of age) and 25 children with dysarthria (5-9 years of age).

Profiles of Dysarthria: Clinical Assessment and Treatment.

In recent decades, we have witnessed a wealth of theoretical work and proof-of-principle studies on dysarthria, including descriptions and classifications of dysarthric speech patterns, new and refined assessment methods, and innovative experimental intervention trials [...

Clinical Assessment of Communication-Related Speech Parameters in Dysarthria: The Impact of Perceptual Adaptation.

Purpose: In current clinical practice, intelligibility of dysarthric speech is commonly assessed by speech-language therapists (SLTs), in most cases by the therapist caring for the patient being diagnosed. Since SLTs are familiar with dysarthria in general and with the speech of the individual patient to be assessed in particular, they have an adaptation advantage in understanding the patient's utterances. We examined whether and how listeners' assessments of communication-related speech parameters vary as a function of their familiarity with dysarthria in general and with the diagnosed patients in particular.

Speech Naturalness in the Assessment of Childhood Dysarthria.

Purpose: This study investigated perceived speech naturalness estimated by adult listeners in typically developing children and children with dysarthria. We aimed to identify predictors of naturalness among auditory-perceptual parameters and to evaluate the concept of naturalness as a clinical marker of childhood dysarthria.

Method: In a listening experiment, naive adult listeners rated speech naturalness of 144 typically developing children (3-9 years old) and 28 children with neurological conditions (5-9 years old) on a visual analog scale.