The prevalence of congenital hearing loss in neonates with Down syndrome.

OBJECTIVE: To determine the prevalence of hearing loss in newborns with Down syndrome. STUDY DESIGN: We performed a cross-sectional, retrospective chart review of all infants with Down syndrome born at a university-affiliated hospital (n = 77) or transferred in to the associated pediatric hospital (n = 32) following birth at an outlying hospital between 1995 and 2010. We determined the rate of failure of newborn hearing screens, the proportion of infants lost to follow-up, and the rate of confirmed hearing loss, as well as the associations of risk factors for hearing loss with confirmed hearing loss. RESULTS: Of the 109 patients with hearing screening data, 28 failed their newborn hearing screen. Twenty-seven infants were referred for audiologic evaluation, and 19 completed the evaluation. Fifteen of these 19 infants (79%) had confirmed hearing loss. The prevalence of congenital hearing loss in this sample of neonates with Down syndrome was 15%. Exposure to mechanical ventilation was the sole known risk factor associated with hearing loss. In this study, the loss to follow-up rate for infants with positive hearing screens was 32%. CONCLUSION: Newborns with Down syndrome have a higher prevalence of congenital hearing loss compared with the total neonatal population (15% vs 0.25%). Continued monitoring of hearing is needed in children with Down syndrome.

Progress toward sodium reduction in the United States / Progresos hacia la reducción del sodio en los Estados Unidos

The average adult in the United States of America consumes well above the recommended daily limit of sodium. Average sodium intake is about 3 463 mg/day, as compared to the 2010 dietary guidelines for Americans recommendation of < 2 300 mg/day. A further reduction to 1 500 mg/day is advised for people 51 years or older; African Americans; and people with high blood pressure, diabetes, or chronic kidney disease. In the United States of America, the problem of excess sodium intake is related to the food supply. Most sodium consumed comes from packaged, processed, and restaurant foods and therefore is in the product at the time of purchase. This paper describes sodium reduction policies and programs in the United States at the federal, state, and local levels; efforts to monitor the health impact of sodium reduction; ways to assess consumer knowledge, attitudes, and behavior; and how these activities depend on and inform global efforts to reduce sodium intake. Reducing excess sodium intake is a public health opportunity that can save lives and health care dollars in the United States and globally. Future efforts, including sharing successes achieved and barriers identified in the United States and globally, may quicken and enhance progress.

El adulto medio de los Estados Unidos consume una cantidad de sodio muy por encima del límite diario recomendado. La ingesta promedio de sodio es aproximadamente de 3 463 mg/día, en contraste con la recomendación de las Directrices alimentarias del 2010 para estadounidenses que es de menos de 2 300 mg/día. A las personas de 51 años o mayores, los afroestadounidenses, los hipertensos, los diabéticos o los que padecen una nefropatía crónica, se les recomienda una reducción adicional hasta 1 500 mg/día. En los Estados Unidos, el problema de la ingesta excesiva de sodio está relacionado con el suministro en los alimentos. La mayor parte del sodio consumido proviene de los alimentos envasados, procesados y que se sirven en restaurantes y, por consiguiente, ya está en el producto en el momento de la compra. Este artículo describe las políticas y los programas de reducción del sodio en los Estados Unidos a escalas federal, estatal y local; las iniciativas para vigilar la repercusión de la reducción del sodio en la salud; los procedimientos para evaluar los conocimientos, las actitudes y el comportamiento de los consumidores; y cómo estas actividades dependen de las iniciativas a escala mundial para reducir la ingesta de sodio y les proporcionan información. La reducción de la ingesta excesiva de sodio constituye una oportunidad de salud pública que puede salvar vidas y ahorrar dinero destinado a la atención de salud en Estados Unidos y a escala mundial. Las iniciativas futuras, entre ellas el intercambio de información sobre los éxitos logrados y los obstáculos encontrados en los Estados Unidos y a escala mundial, pueden acelerar y estimular el progreso.

Measuring disease-specific quality of life in rare populations: a practical approach to cross-cultural translation.

BACKGROUND: Disease-specific quality of life (QoL) measures have enhanced the capacity of outcome measures to evaluate subtle changes and differences between groups. However, when the specific disease is rare, the cohort of patients is small and international collaboration is often necessary to accomplish meaningful research. As many of the QoL measures have been developed in North American English, they require translation to ensure their usefulness in a multi-cultural and/or international society. Published guidelines provide formal methods to achieve cross-culturally comparable versions of a QoL tool. However, these guidelines describe a rigorous process that is not always feasible, particularly in rare disease groups. The objective of this manuscript is to describe the process that was developed to achieve accurate cross-cultural translations of a disease-specific QoL measure, to overcome the challenges of a small sample size, i.e. children with a rare disorder. PROCEDURE: A measurement study was conducted in the United Kingdom (UK), France, Germany and Uruguay, during which the validated measure was translated into the languages of the respective countries. RESULTS: This is a report of a modified, child-centric, cross-cultural translation and adaptation process in which culturally appropriate and methodologically valid translations of a disease-specific QoL measure, the Kids' ITP Tools (KIT), were performed in children with immune thrombocytopenic purpura (ITP). The KIT was translated from North American English into UK English, French, German, and Spanish. CONCLUSION: This study was a successful international collaboration. The modified process through which culturally appropriate and methodologically valid translations of QoL measures may be achieved in a pediatric population with a relatively rare disorder is reported.

Validity, reliability, and responsiveness of a new measure of health-related quality of life in children with immune thrombocytopenic purpura: the Kids' ITP Tools.

OBJECTIVE: To refine the disease-specific health-related quality of life measure in immune (idiopathic) thrombocytopenic purpura (ITP) and to determine its validity, reliability, and responsiveness to change. STUDY DESIGN: The initial phase involved cognitive debriefing of 12 families, on the basis of which the measure was modified and then named Kids' ITP Tools (KIT). The measure was administered on 2 occasions with the Pediatric Quality of Life Inventory (PedsQL) to 41 patients with acute ITP and 49 patients with chronic ITP, 2 to 18 years old, and their parents (proxy-respondents) at 6 North American centers. RESULTS: Patients with acute ITP had lower scores when compared with patients with chronic ITP (child 64 versus 76, proxy 69 versus 77). The KIT moderately correlated with the PedsQL. Child versus proxy KIT scores showed moderate correlation, and the KIT was superior to the PedsQL. Test-retest reliability was substantial in the child report, but only moderate for the proxy report, similar to the PedsQL. The KIT showed a mean score change of 13 in the child and 15 in the proxy, which was greater than the PedsQL child's change of 7 and proxy change of 5. CONCLUSION: The KIT is valid, with good distinction between acute and chronic ITP and a moderate correlation with the PedsQL. The KIT demonstrated reliability comparable with that of the PedsQL, yet it was more responsive to change. Therefore the KIT can be used as an outcome measure in future clinical trials of childhood ITP.