Küçükaydín M, Patíroğlu T E, Okur H, Içer M
Department of Pediatric Surgery and Pathology, School of Medicine, Kayseri, Turkey.
Eur J Pediatr Surg. 1992 Oct;2(5):295-7. doi: 10.1055/s-2008-1063463.
This is a case report of juvenile gastrointestinal polyposis involving the gastrointestinal system from the stomach to the rectum. Only few cases have been reported and extra-intestinal manifestations of this syndrome include macrocephaly, hepatosplenomegaly, hypotonia, clubbing of fingers, anemia and protein-losing enteropathy. The disease usually has a poor prognosis, and the children rarely live more than 2 years.
这是一例累及从胃到直肠的整个胃肠道系统的青少年胃肠道息肉病的病例报告。仅有少数病例被报道,该综合征的肠外表现包括巨头畸形、肝脾肿大、肌张力减退、杵状指、贫血和蛋白丢失性肠病。这种疾病通常预后较差,患儿很少能活超过2年。