• Title/Summary/Keyword: Nesidioblastosis

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A Case of Surgical Treatment of Nesidioblastosis in Infancy (영아에서 발생한 췌도모세포증의 외과적 치료 1예)

  • Huh, Young-Soo;Chai, Sang-Chul;Nah, Mok-Chan;Kim, Mi-Jin
    • Advances in pediatric surgery
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    • v.1 no.2
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    • pp.195-199
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    • 1995
  • Nesidioblastosis in one of the causes of hyperinsulinemic hypoglysemia in infancy. The most important goal of treatment for persistent hypoglycemia is the prevention of permanent brain damage. The early surgical management is satisfactory to this goal in nesidioblastosis and maintains normal blood sugar level without administration of drugs or supplement of sugar postoperatively in many cases. We experienced a female infant of 3 months old who has suffered from persistent hypoglysemia due to hyperinsulinism and was suspected nesidioblastosis for its cause clinically. She underwent 95% distal pancreatectomy. The histologic findings of nesidioblastosis was confirmed postoperatively. No postoperative complication was occured and her blood sugar levels were maintained within normal range without medical treatment.

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Sudden Unexpected Death in Infancy (Analysis of 34 Cases Including 13 Autopsies) (영아 돌연사에 대한 고찰 (13 부검례를 포함한 34례 분석))

  • Moon, Yeo Ok;Choi, Hee Kyoung;Her, Jeoung-A;Shin, Woo Jong;Kim, Myoung-A;Lee, Seong Yong;Jang, Seong Hee;Dong, Eun Sil;Kim, Chong Jae;Ahn, Young Min;Chi, Je Geun
    • Clinical and Experimental Pediatrics
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    • v.45 no.9
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    • pp.1065-1074
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    • 2002
  • Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical history, death scene examination, autopsy findings, acylcarnitine and organic acid analysis. Results : Among the total 34 infants, 18 were male(52.9%) and 16 were female(47.1%). Thirty infants(88%) were below the six months of age. Winter was the most affected season(38.2%). Eighteen infants(52.9%) died between 6 and 12AM. The prone sleeping position was observed more frequently than the supine position at death; nine cases in the prone position, six cases in the supine position. The cause of death of 23 cases could not be found by only history and death scene examination. Autopsy was done in 13 cases. Seven cases of them were thought to be SIDS. In six cases, we explained the cause of death with autosy findings. They were an endocardial fibroelastosis, a nesidioblastosis, a subdural hematoma, a bronchopneumonia and two fatty changes of liver. Metabolic screening tests performed in three cases to rule out metabolic disorder since 2000 were all normal. Conclusion : We concluded that autopsy and metabolic screening test should be performed to find out the cause of death in sudden unexpected death in infancy.