Covert food stealing is common among individuals with Prader-Willi syndrome. We found that verbal reprimands, delivered contingent upon eating prohibited foods, were sufficient to decrease the food stealing of a girl with Prader-Willi syndrome. Warning stimuli were then used to help her discriminate between permitted and prohibited foods during sessions in which food stealing was not directly o...

Prader-Willi syndrome is a neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Maladaptive behaviours, cognitive impairment, and impediments in speech and language seriously affect the early development and long-term functioning of individuals affected by the illness. We present a case of a 9-year-old child with Prader-Wil...

A five-year-old boy presented with progressive weight gain with effort intolerance and nocturnal symptoms suggesting obstructive sleep apnoea. A clinical diagnosis of Prader-Willi Syndrome was made. As the initial radiography and computed tomography suggested a foreign body, bronchoscopy was done under general anaesthesia and impacted peanuts were removed from the left main bronchus. His sympto...

Prader-Willi syndrome (PWS) is caused by the loss of RNA expression from an imprinted region on chromosome 15 that includes SNRPN, SNORD115, and SNORD116. Currently, there are no mouse models that faithfully reflect the human phenotype and investigations rely on human post-mortem material. During molecular characterization of tissue deposited in a public brain bank from a patient diagnosed with...

INTRODUCTION Prader-Willi syndrome is a complex genetic disease caused by lack of expression of paternally inherited genes on chromosome 15q11-q13. The prevalence of Prader-Willi syndrome is estimated to be one in 10,000 to 25,000. However, descriptions of the oral and dental phenotype are rare. CASE PRESENTATION We describe the clinical presentation and periodontal findings in a 20-year-old ...

Since the original description by Prader, Labhart, and Willi in 1956,' there have been over 700 case reports of the Prader-Willi syndrome and by the end of 1991 the PraderWilli Association were aware of 1595 affected individuals in North America.2 Estimates of prevalence vary: one group has reported a consensus figure of one in 10 000 births,3 but with modem techniques for laboratory diagnosis ...

OBJECTIVE Prader-Willi Syndrome is a common etiology of syndromic obesity that is typically caused by either a paternal microdeletion of a region in chromosome 15 (microdeletions) or a maternal uniparental disomy of this chromosome. The purpose of this study was to describe the most significant clinical features of 35 Brazilian patients with molecularly confirmed Prader-Willi syndrome and to de...

Since the original description by Prader, Labhart, and Willi in 1956,' there have been over 700 case reports of the Prader-Willi syndrome and by the end of 1991 the PraderWilli Association were aware of 1595 affected individuals in North America.2 Estimates of prevalence vary: one group has reported a consensus figure of one in 10 000 births,3 but with modem techniques for laboratory diagnosis ...

Since the original description by Prader, Labhart, and Willi in 1956,' there have been over 700 case reports of the Prader-Willi syndrome and by the end of 1991 the PraderWilli Association were aware of 1595 affected individuals in North America.2 Estimates of prevalence vary: one group has reported a consensus figure of one in 10 000 births,3 but with modem techniques for laboratory diagnosis ...

Monosomy 1p36 is the most common subtelomeric microdeletion syndrome with an incidence rate estimated to be 1 in 5000 births. A hypothesis of a similarity between patients with 1p36 deletion and those with Prader-Willi syndrome and the existence of two different phenotypes for 1p36 microdeletion has been suggested. The main objective of the present study was to determine the existence of 1p36 m...