Neonatal Presentation of Prader-Willi Syndrome / 대한주산의학회잡지
Korean Journal of Perinatology
; : 95-100, 2012.
Article
em Ko
| WPRIM
| ID: wpr-226171
Biblioteca responsável:
WPRO
ABSTRACT
PURPOSE:
In Korea, neonatal clinical features and feeding pattern are not reported systematically. So we report the neonatal presentation of Prader-Willi syndrome.METHODS:
This is a retrospective study of 14 neonate with Prader-Willi syndrome whose diagnosed in neonatal intensive care unit, Samsung Medical Center between January 1st 1997 and December 30th 2011.RESULTS:
The birth characteristics of patients, maternal age was 31.5+/-4.5 years and 5 (35.7%) were primiparous. Oligohydramnios were reported in 2 cases (14.2%), polyhydramnios were reported in 4 cases (28.5%). 5 pregnancies (35.7%) had complained that decreased fetal movements. Mean gestational age were 38.1+/-2.2 weeks and 12 patients (85%) were term infants, one patient (7.1%) was post term infant, the other patient was preterm infants (7.1%). The average weight at birth were 2509.0+/-442.5 grams. All 14 patients (100%) had hypotonia and a weak cry at birth. 9 patients (64.5%) had the characteristic facial dysplasia. In all, nine male patients (100%) had cryptorchidism. All patients had weak sucking power, so they started to eat via nasogastric tube at birth. The mean duration of use nasogastric tube were 3.2+/-2.0 months. The median age of diagnosis were 1.5 months. 7 cases (50%) were diagnosed within one month of life. Median time of starting GH treatment was 2.0 years. The mean interval of from diagnosis to treatment of growth hormone were 2.2+/-2.0 years.CONCLUSION:
Early diagnosis of Prader-Willi syndrome is a very important to improve long-term prognosis.Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Fenótipo
/
Síndrome de Prader-Willi
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Prognóstico
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Recém-Nascido Prematuro
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Hormônio do Crescimento
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Terapia Intensiva Neonatal
/
Estudos Retrospectivos
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Oligo-Hidrâmnio
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Poli-Hidrâmnios
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Idade Gestacional
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Screening_studies
Limite:
Female
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Humans
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Infant
/
Male
/
Newborn
/
Pregnancy
País/Região como assunto:
Asia
Idioma:
Ko
Revista:
Korean Journal of Perinatology
Ano de publicação:
2012
Tipo de documento:
Article