16 research outputs found
Guideline for sustainable wetland management and utilization: key cornerstones
This research report is based on the project on Wetlands, Livelihoods and Environmental Security and
the Sustainable management of Inland Wetlands in Southern Africa: a livelihoods and ecosystem
approach projects. The projects were supported by the CGIAR´s Challenge Programme on Water and
Food (CPWF) and the Global Environment facility with matching funds from International Water
Management Institute (IWMI), Eduardo Mondlane (UEM), FSP and the University of Zimbabwe
(UZ) The authors acknowledge input by the entire project team. The support of IWMI, UEM, and the
UZ, and Institute de Recherche pour le Development (IRD) during project implementation is
gratefully acknowledged. The authors thank the input of CPWF Theme 3 leadership and the Limpopo
Basin Coordinator during project implementation
Manifestaciones neurológicas en pacientes pediátricos con fenilcetonuria. Revisión bibliográfica
La fenilcetonuria (PKU) es una enfermedad
congénita de herencia autosómica
recesiva, en la que existe el déficit
de la enzima fenilalanina hidroxilasa,
que produce lesión del sistema nervioso
central (SNC). La incidencia mundial
es de 1:20.000 recién nacidos vivos.
(1,2) El tratamiento es a base de dieta
con restricción de fenilalanina que
debe mantenerse para toda la vida
para prevenir el deterioro intelectual.
(3,4)
OBJETIVO: Identificar manifestaciones
neurológicas de pacientes pediátricos
con PKU.
METODOLOGIA: Revisión bibliográfica
de la literatura acerca de las manifestaciones
neurológicas para PKU
mediante la búsqueda electrónica en
varias bases de datos (MEDLINE, EMBASE,
COCHRANE, LILACS, PubMed) con
lÃmites en inglés y español de enero de
1970 a 2012.
RESULTADOS: Se identifica un estudio
transversal, un caso clÃnico, tres revisiones
narrativas en poblaciones pediátricas
y un caso clÃnico combinado niños
y adultos. La discapacidad intelectual
es la principal manifestación neurológica,
el criterio diagnóstico primordial
fue el valor sérico de fenilalanina. Existen
29 clasificaciones distintas sobre el
punto de corte para hiperfenilalaninemia
y 23 para fenilcetonuria.Phenylketonuria (PKU) is a congenital
autosomal recessive disease in which
there is a shortage of the enzyme
phenylalanine hydroxylase, which produces
an injury to the central nervous
system (CNS). The global incidence is
about 1:20,000 live newborns. (1.2) The
treatment is based on phenylalanine
restricted diet which should be maintained
for lifelong to prevent intellectual
impairment.
OBJECTIVE: To identify neurological manifestations
of pediatric patients with
PKU.
METHODOLOGY: A review of the literature
on the neurological manifestations
for PKU by searching several electronic
databases (MEDLINE , EMBASE, Cochrane
, LILACS , PubMed) with English and
Spanish limits from January 1970-2012.
RESULTS: One cross-sectional study, one
clinical case, three narrative reviews in
pediatric populations and one clinical
case combine children and adults. Intellectual
disability is the main neurological
manifestation; the primary diagnostic
criterion was the serum level of
phenylalanine. There are 29 different
classifications on the cutoff to hyperphenylalaninemia
and 23 for phenylketonuria.Cuenc