先天性胆道拡張症の母子発生例  [in Japanese] CONGENITAL BILIARYDILATATION OCCURRED IN A MOTHER AND HER CHILD  [in Japanese]

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Abstract

先天性胆道拡張症(以下,本症)の家族内発生は稀である.最近われわれは本症の母子発生例を経験したので報告する.症例1: 48歳女性, (昭和57年当時),症例2の母親. 33歳時,本症の診断にて嚢胞・十二指腸吻合術を受けているが根治術を目的として入院,型のごとく嚢胞切除,総肝管空腸吻合術を行った.症例2: 34歳女性,症例1の長女.腹痛を主訴に来院,精査の結果Ic (p) IIfmの本症と診断,嚢胞切除,総肝管空腸吻合術を施行した.両症例とも嚢胞壁に悪性所見は認めなかった. <br> 本邦における本症の家族内発生例は16例8家系をかぞえるにすぎず,その発生頻度より遺伝傾向は極めて薄いものと推測される.一方,一卵性双生児での本症発生例は7例みられ,うち6例はその1方児にのみに発症していた.この点からも遺伝性に関しては否定的であった.しかしその遺伝性を結論づけるには報告例数があまりに乏しく現時点では更なる症例数の集積に努力が払われるべきである.

Familial occurrence of congenital biliary dilatation is rare. Recently we experienced the disease occurred in a mother and her child.<br> Case 1: A 48-year-old woman who is a mother of Case 2 was admitted to the hospital for a radical operation in 1982. There was a previous history of undergoing a cystoduodenostomy with a diagnosis of congenital biliary dilatation. Excision of the cyst and choledochojejunostomy were typically performed.<br> Case 2: A 34-year-old woman who is Case 1's eldest daughter was seen at the hospital because of an abdominal pain. After close examination, she was diagnosed as the disease of Ic (p) IIfm and underwent an excision of the cyst and choledochojejunostomy. No malignant findings were noted in the cystic wall in both cases.<br> Familial occurrences of the disease have counted up only 16 cases of 8 families in Japan so far. It is inferred that the disease is hardly inherited according to its frequency. On the other hand, there are seven cases of the disease in monozygotic twins and six out of seven patients are from either of twins. In terms of this point, it appears that a possibility of the heredity can be ruled out in the disease. However, we have not enough number of cases to come to any conclusion for this heredity problem.<br> We should make every efforts to accumulate such cases at present.

Journal

  • The journal of the Japanese Practical Surgeon Society

    The journal of the Japanese Practical Surgeon Society 57(9), 2254-2258, 1996-09-25

    Japan Surgical Association

References:  20

Cited by:  1

Codes

  • NII Article ID (NAID)
    10008524282
  • NII NACSIS-CAT ID (NCID)
    AN00198696
  • Text Lang
    JPN
  • Article Type
    Journal Article
  • ISSN
    03869776
  • Data Source
    CJP  CJPref  J-STAGE 
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