Neonatal Presentation of Prader-Willi Syndrome / 대한주산의학회잡지
Korean Journal of Perinatology
; : 95-100, 2012.
Article
ي Ko
| WPRIM
| ID: wpr-226171
المكتبة المسؤولة:
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.
Key words
النص الكامل:
1
الفهرس:
WPRIM
الموضوع الرئيسي:
Phenotype
/
Prader-Willi Syndrome
/
Prognosis
/
Infant, Premature
/
Growth Hormone
/
Intensive Care, Neonatal
/
Retrospective Studies
/
Oligohydramnios
/
Polyhydramnios
/
Gestational Age
نوع الدراسة:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Screening_studies
المحددات:
Female
/
Humans
/
Infant
/
Male
/
Newborn
/
Pregnancy
البلد/الأقليم حسب الموضوع:
Asia
اللغة:
Ko
مجلة:
Korean Journal of Perinatology
السنة:
2012
نوع:
Article