Daugherty C C, Yazigi N, Bove K, Balistreri W
Department of Pathology, University of Cincinnati College of Medicine, Children's Hospital Medical Center, Ohio, USA.
Pediatr Pathol Lab Med. 1996 Mar-Apr;16(2):263-74.
Elongated, enlarged mitochondria with crystalloid matrix arrays were discovered in periportal hepatocytes in 11 of 12 children (age 6 to 15 years) with portal hypertension, minimal alterations on light microscopy, and cavernous transformation of the portal vein. Eleven of the children were clinically well before onset of symptoms, one was anemic with megaloblastic bone marrow, and a second had undergone renal transplantation. Minimal findings by light microscopy included slight portal fibrosis (six cases), pericentral venular fibrosis (one case), mild, patchy sinusoidal sclerosis (one case), central venular and sinusoidal dilatation (two cases), and mild hepatocellular lipid accumulation (one case). Four were judged normal by routine histologic examination. Subtle depletion of periportal hepatocellular glycogen was present in six. In 10, subtle striation or granularity of periportal hepatocyte cytoplasm was visible with high-magnification light microscopy. Although similar mitochondria are seen sporadically in hepatocytes in diverse settings, enlarged mitochondria with crystalloid matrix inclusions have not been previously reported as a uniform feature in children with portal hypertension due to cavernous transformation of the portal vein and minimal other hepatic alteration. It is postulated that the mitochondria are adapting in response to an abnormal metabolic milieu created by hemodynamic alterations.
在12名门静脉高压患儿（年龄6至15岁）中，有11名患儿的门周肝细胞中发现了拉长、增大且具有晶体状基质排列的线粒体。这些患儿门静脉海绵样变，光镜下改变轻微。11名患儿在症状出现前临床状态良好，1名患儿患有巨幼细胞性贫血，另1名患儿接受过肾移植。光镜下的最小发现包括轻度门周纤维化（6例）、中央静脉周围纤维化（1例）、轻度斑片状窦周硬化（1例）、中央静脉和窦扩张（2例）以及轻度肝细胞脂质蓄积（1例）。4例经常规组织学检查判断为正常。6例存在门周肝细胞糖原的轻微减少。10例在高倍光镜下可见门周肝细胞胞质有细微条纹或颗粒状。尽管在不同情况下肝细胞中偶尔可见类似的线粒体，但此前尚未报道具有晶体状基质内含物的增大线粒体是门静脉海绵样变所致门静脉高压患儿且肝脏其他改变轻微时的一个一致特征。据推测，线粒体是在对血流动力学改变所产生的异常代谢环境做出适应性反应。
