胆道閉鎖症におけるductal plate malformationと肝線維化の関係  [in Japanese] Severity of Liver Fibrosis in Biliary Atresia With Ductal Plate Malformation  [in Japanese]

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Author(s)

    • 大林 樹真 Obayashi Juma
    • 聖マリアンナ医科大学外科学小児外科 Division of Pediatric Surgery, St. Marianna University School of Medicine
    • 小池 淳樹 Koike Junki
    • 聖マリアンナ医科大学病理学 Department of Pathology, St. Marianna University School of Medicine
    • 田中 邦英 Tanaka Kunihide
    • 聖マリアンナ医科大学外科学小児外科 Division of Pediatric Surgery, St. Marianna University School of Medicine
    • 長江 秀樹 Nagae Hideki
    • 聖マリアンナ医科大学外科学小児外科 Division of Pediatric Surgery, St. Marianna University School of Medicine

Abstract

<p>【目的】Ductal plate malformation(DPM)が原因とされる胆道閉鎖症(BA)は,早期発症と関連があり自己肝生存率に影響を与えている可能性がある.初回手術時の胆道閉鎖症の肝線維化とDPMの関連を検討した.</p><p>【方法】1976年から2017年に当院で葛西手術を施行し,同時に肝生検が行われたBA 32例を検討した.DPMを認めた群をA群,認めなかった群をB群とした.初回手術日齢,portal-portal bridging(P-P)の程度,portal-central vein bridging(P-C)の有無,黄疸消失率(総ビリルビン<2.0 mg/dl)および黄疸消失に至るまでの日数を評価した.P-P<50%をGrade 1,>50%をGrade 2,偽小葉化がみられるのをGrade 3とした.統計学的解析はχ二乗検定およびStudent's t-testを行い,有意差をp<0.05とした.</p><p>【結果】A群が9例,B群が23例だった.両群の生存率に差は見られなかった.初回手術日齢はA群67.3±17.8日,B群89.0±29.7日だった(p=0.050).P-PはA群ではGrade 1が2例,Grade 2が3例,Grade 3が4例で,B群ではそれぞれ7例,12例,4例だった(p=0.281).P-CはA群7例,B群8例で認めた(p=0.028).黄疸消失率,黄疸消失までの日数に差を認めなかった(p=0.599,p=0.252).</p><p>【結論】DPMを認めるBAは初回手術日齢が早いものの肝硬変像であるP-Cを認め,早期発症の可能性がある.</p>

<p><i>Purpose</i>: Several investigators have reported that ductal plate malformation (DPM) in the liver is characteristic of persistent embryonal ductular strictures and the embryonic subtype of biliary atresia (BA). We investigated the relationship between liver fibrosis and DPM in BA.</p><p><i>Methods</i>: Thirty-two BA patients underwent Kasai operation from 1976 to 2017. We compared two groups: Group A had DPM and Group B did not. Biopsies obtained during the initial Kasai procedure were histopathologically analyzed in terms of the degree of portal-portal (P-P) bridging and the existence of portal-central vein (P-C) bridging. The degree of P-P bridging was defined as follows: Grade 1, P-P <50%; Grade 2, >50%; Grade 3, nodular architecture. We also evaluated the age at the time of Kasai operation, the number of patients who became jaundice-free (JF; total bilirubin <2.0 mg/dl), and the time taken to become JF. Statistical comparisons of multiple data were carried out using the chi-square test and Student's t-test. Regression analysis was carried out with the significance level set at p <0.05.</p><p><i>Results</i>: There were nine patients in Group A and 23 patients in Group B. There was no significant difference in P-P bridging (p = 0.281). Seven patients had P-C bridging in Group A and eight in Group B (p = 0.028). The ages at the time of Kasai operation were 67.3 ± 17.8 days in Group A and 89.0 ± 29.7 days in Group B (p = 0.050). There were no significant differences in the number of JF patients (p = 0.599) and the time taken to become JF (p = 0.252).</p><p><i>Conclusions</i>: DPM tended to be associated with early age of operation and having P-C bridging.</p>

Journal

  • Journal of the Japanese Society of Pediatric Surgeons

    Journal of the Japanese Society of Pediatric Surgeons 55(6), 1061-1065, 2019

    The Japanese Society of Pediatric Surgeons

Codes

  • NII Article ID (NAID)
    130007731521
  • NII NACSIS-CAT ID (NCID)
    AN00192281
  • Text Lang
    JPN
  • ISSN
    0288-609X
  • NDL Article ID
    030066735
  • NDL Call No.
    Z19-244
  • Data Source
    NDL  J-STAGE 
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