Aşırı Kilo Kaybının Nadir Bir Nedeni: Diensefalik Sendromlu Üç Olgu

Diensefalik sendrom, daha çok erken çocukluk döneminde görülen, yaygın kilo kaybı ve cilt altı yağ dokusu kaybı, aşırı hareketlilik, öfori ve aşırı uyanıklık durumu ile karakterize bir sendromdur. Genellikle hipotalamik-optik kiazmatik bölgenin yer kaplayıcı neoplastik oluşumlarına eşlik eder. Kitle boyutu ile kilo alımı arasında ters orantı mevcuttur. Bu olgu sunumunda kliniğimizde hipotalamik-optik kiazmatik gliom tanısı ile tedavi alan ve boy uzaması devam ettiği halde aşırı kilo kaybı olan ve tartıları -2 standart sapmanın altında izlenen 1 yaş altı 3 olgu, endokrinolojik incelemeleri ile birlikte literatür bilgileri ışığında değerlendirilerek sunulmuştur. Kilo alımında duraklamanın ender de olsa önemli nedenlerinden birinin diensefalik sendrom olabileceğine dikkat çekilmiştir

A Rare Reason of Emaciation: Three Cases of Diencephalic Syndrome

Diencephalic syndrome DS is a rarely-seen early childhood syndrome, characterized by diffuse emaciation, loss of subcutaneous fat tissue, hyperactivity, euphoria and hyperalertness. Usually the syndrome is accompanied by space-occupying neoplastic lesions in hypothalamic-optic chiasmatic region. Tumor size is inversely correlated with weight gain.In this case series, we aimed to present three infants with hypothalamic-optic chiasmatic gliomas treated with chemotherapy in our clinic. All three patients had normal linear growth but excessive weight loss. Diencephalic syndrome is further discussed and it is pointed out that excessive weight loss might be the rare but important outcome of this syndrome

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Acıbadem Üniversitesi Sağlık Bilimleri Dergisi-Cover
  • ISSN: 1309-470X
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 2010
  • Yayıncı: ACIBADEM MEHMET ALİ AYDINLAR ÜNİVERSİTESİ