Psychotropic treatments in Prader-Willi syndrome: a critical review of published literature.

Prader-Willi syndrome (PWS) is a rare genetic syndrome. The phenotype includes moderate to intellectual disability, dysmorphia, obesity, and behavioral disturbances (e.g., hetero and self-injurious behaviors, hyperphagia, psychosis). Psychotropic medications are widely prescribed in PWS for symptomatic control. We conducted a systematic review of published literature to examine psychotropic medications used in PWS. MEDLINE was searched to identify articles published between January 1967 and December 2014 using key words related to pharmacological treatments and PWS. Articles with original data were included based on a standardized four-step selection process. The identification of studies led to 241 records. All selected articles were evaluated for case descriptions (PWS and behavioral signs) and treatment (type, titration, efficiency, and side effects). Overall, 102 patients were included in these studies. Treatment involved risperidone (three reports, n = 11 patients), fluoxetine (five/n = 6), naltrexone (two/n = 2), topiramate (two/n = 16), fluvoxamine (one/n = 1), mazindol (one/n = 2), N-acetyl cysteine (one/n = 35), rimonabant (one/n = 15), and fenfluramine (one/n = 15). CONCLUSION: We identified promising treatment effects with topiramate for self-injury and impulsive/aggressive behaviors, risperidone for psychotic symptoms associated with uniparental disomy (UPD), and N-acetyl cysteine for skin picking. The pharmacological approach of behavioral impairment in PWS has been poorly investigated to date. Further randomized controlled studies are warranted. WHAT IS KNOWN: Behavioral disturbances in Prader-Willi syndrome including aggressive reactions, skin picking, and hyperphagia might be very difficult to manage. Antipsychotic drugs are widely prescribed, but weight gain and increased appetite are their major side effects. WHAT IS NEW: Topiramate might be efficient for self-injury and impulsive/aggressive behaviors, N-acetyl cysteine is apromising treatment for skin picking and Antidepressants are indicated for OCD symptoms. Risperidone is indicated in case of psychotic symptoms mainly associated with uniparental disomy.

[Transplant coordination and logistics of intra and extra-hospital cadaver donor tissue. "The Pamplona Model". Sequence of tasks performed from 1992-2006]. / Coordinación y logística del trasplante de tejidos de donantes de cadáver intra y extra-hospitalarios. "Modelo Pamplona". Cadena de actuación 1992-2006.

INTRODUCTION: Tissue and organ donations are the only option for many patients. Cerebral death (CD) facilitates this approach. However, hospitals that do not provide CD donors have to adapt in order to obtain donors, referred to as tissue donors (TD), who have died from cardiac arrest. OBJECTIVE: Is this paper it descripte the model for coordination and donation of intra and extra-hospital TD in the Autonomous Community of Navarra. It creats a program for detection, donation and extractions called the Pamplona Model, from 1992-2006. MATERIAL AND METHODS: In 1990, a transplant team was created by an Intensive Medicine Physician of HVC, INML and SOS-Navarra. In 1996, VCH Transplant Coordination is defined as a reference centre for the Tissue Transplant Programme in the Autonomous Community of Navarra. RESULTS: Consensus protocols for "intra and extra-hospital detection" of persons having died from cardiac arrest are developed: - Alerts from NHS-O hospitals, SOS-Navarra; judges and INML forensic pathologists. - Criteria for selection, search and contacts with relatives. - Alert serology, extraction and transport teams. - Logistics and distribution of tissue. - Agreed incentives: Economic, administrative and relevant regulations. CONCLUSIONS: The Pamplona Model, with the Virgen Del Camino hospital has made important contributions and is unique in the world. Intra and extra-hospital coordination of cadaver donor from a referred hospital, it is a scientific and organizational advance to have in it counts for the creation of extraction and transplant tissues teams.

Coordinación y logística del trasplante de tejidos de donantes de cadáver intra y extra-hospitalarios. “Modelo Pamplona”. Cadena de actuación 1992-2006 / Transplant coordination and logistics of intra and extra-hospital cadaver donor tissue. “The Pamplona Model”. Sequence of tasks performed from 1992-2006

Fundamento. La donación de tejidos y órganos constituye la única alternativa para muchos pacientes. La muerte cerebral (MC) facilita esta actitud. Aquellos hospitales no-generadores de donantes con MC, tienen que generar donantes a partir de fallecidos por parada cardiorrespiratoria, llamados donantes de tejidos (DT). En este trabajo se expone el modelo de coordinación y donación de tejidos de DT intra y extra-hospitalarios en la Comunidad Foral de Navarra. Se crea un Programa de detección, donación, extracción, denominado Modelo Pamplona, desde 1992-2006. Material y métodos. A partir de 1990 se organiza el Equipo de Trasplantes formado por un médico de la Unidad de Cuidados Intensivos del hospital Virgen del Camino, Instituto Navarro de Medicina Legal (INML) y SOS-Navarra. En 1996 la Coordinación de Trasplantes del HVC es definido como centro de referencia para el Programa de Trasplantes de Tejidos de la Comunidad de Navarra. Resultados. Se desarrollan protocolos consensuados para la detección intra y extrahospitalaria de los fallecidos en parada cardiorrespiratoria: – Alarma desde hospitales del SNS-O, SOS-Navarra, jueces y forenses del INML. – Criterios de selección, búsqueda y contacto con familiares. – Aviso a equipos de serología, extracción y transporte. – Logística y reparto de los tejidos. – Incentivos pactados: económicos, administrativos y normativa al respecto. Conclusiones. El modelo Pamplona, desde el hospital Virgen del Camino resulta positivo y es único en el mundo. La coordinación intra y extrahospitalaria de trasplantes de donantes cadáver desde un hospital de referencia, es un avance científico y organizativo a tener en cuenta para la creación de equipos de extracción y trasplante de tejidos

Introduction. Tissue and organ donations are the only option for many patients. Cerebral death (CD) facilitates this approach. However, hospitals that do not provide CD donors have to adapt in order to obtain donors, referred to as tissue donors (TD), who have died from cardiac arrest. Objective. Is this paper it descripte the model for coordination and donation of intra and extra-hospital TD in the Autonomous Community of Navarra. It creats a program for detection, donation and extractions called the Pamplona Model, from 1992-2006. Material and methods. In 1990, a transplant team was created by an Intensive Medicine Physician of HVC, INML and SOS-Navarra. In 1996, VCH Transplant Coordination is defined as a reference centre for the Tissue Transplant Programme in the Autonomous Community of Navarra. Results. Consensus protocols for “intra and extra-hospital detection” of persons having died from cardiac arrest are developed: – Alerts from NHS-O hospitals, SOS-Navarra; judges and INML forensic pathologists. – Criteria for selection, search and contacts with relatives. – Alert serology, extraction and transport teams. – Logistics and distribution of tissue. – Agreed incentives: Economic, administrative and relevant regulations. Conclusions. The Pamplona Model, with the Virgen Del Camino hospital has made important contributions and is unique in the world. Intra and extra-hospital coordination of cadaver donor from a referred hospital, it is a scientific and organizational advance to have in it counts for the creation of extraction and transplant tissues teams