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Beckwith-Wiedemann Sendromu ve Uzamış Hipoglisemi

Beckwith-Wiedemann Syndrome and Prolonged Hypoglycemia

Journal Name:

  • Selçuk Tıp Dergisi

Publication Year:

  • 2010

Key Words:

Keywords (Original Language):

Author NameUniversity of Author
Abstract (2. Language): 
Macroglossia, prenatal or postnatal overgrowth, macrosomia, macroglossia, and abdominal wall defects (omphalocele, umbilical hernia, or diastasis recti) permit early recognition of BeckwithWiedemann syndrome. Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others. The frequency of hypoglycemia in this population is between 30% and 50%. The cause of hypoglycemia is unclear but supports a hyperinsulinemia as the major factor. The majority of infants with hypoglycemia will be asymptomatic and have resolution of the hypoglycemia within the first 3 days of life. Less than 5% will have hypoglycemia beyond the neonatal period. As conclusion, in patients with BWS levels of insulin and blood glucose must be monitorized beginning from first hours of life. Hypoglycemia may be prolonged in presence of hyperinsulinemia as like this present case.
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Abstract (Original Language): 
Beckwith-Wiedemann sendromu (BWS), makrosomi, makroglossi, karın duvar defektleri, hemihipertrofi ile karakterize prenatal veya postnatal bir aşırı büyüme sendromudur. Hastalığın komplikasyonları arasında wilms tümörü, rabdomyosarkom, nöroblastom gibi embriyonal kanserlerle ve hipoglisemi sayılmaktadır. BWS’lu bebeklerin yaklaşık %30’unda hipoglisemi rastlanmaktadır. Hipoglisemi nedeni tam olarak bilinmemekte ve hiperinsülinemi suçlanmaktadır. BWS’da hipoglisemi genellikle yaşamın ilk üç gününden sonra iyileşmesine rağmen vakaların %5’inde dirençli hipoglisemi devam etmektedir. Sonuç olarak BWS’lu bebeklerde kan şekeri düzeyleri ilk saatlerden itibaren düzenli izlenmeli ve insülin düzeyleri de kontrol edilmelidir. Hiperinsülinemi varlığında hipoglisemi problemi burada sunulan vakada olduğu gibi uzayabilir
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  • Turkish

REFERENCES

References: 

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