PATHOLOGY OF HEPATIC PEROXISOMES AND MITOCHONDRIA IN PATIENTS WITH PEROXISOMAL DISORDERS

被引：44

作者：

HUGHES, JL

POULOS, A

ROBERTSON, E

CHOW, CW

SHEFFIELD, LJ

CHRISTODOULOU, J

CARTER, RF

机构：

[1] ADELAIDE CHILDRENS HOSP INC,DEPT CHEM PATHOL,ADELAIDE,SA 5006,AUSTRALIA

[2] ROYAL CHILDRENS HOSP,DEPT ANAT PATHOL,PARKVILLE,VIC 3052,AUSTRALIA

[3] ROYAL CHILDRENS HOSP,MURDOCH INST,PARKVILLE,VIC 3052,AUSTRALIA

来源：

VIRCHOWS ARCHIV A-PATHOLOGICAL ANATOMY AND HISTOPATHOLOGY | 1990年 / 416卷 / 03期

关键词：

Infantile Refsum's disease; Mitochondria; Neonatal adrenoleukodystrophy; Peroxisomal disorders; Peroxisomes; Pseudo-Zellweger disease;

D O I：

10.1007/BF01678985

中图分类号：

R602 [外科病理学、解剖学]; R32 [人体形态学];

学科分类号：

100101 ;

摘要：

The morphology of hepatic peroxisomes in five patients with metabolic disorders believed to be due to inherited defects of peroxisomal function or biogenesis is described. Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). In the patients with IRD and NALD hepatic peroxisomes were significantly reduced in size and number and contained electron dense centres. In the liver of the patients with PZS the peroxisomes were enlarged. Morphologically abnormal peroxisomes were also detected in autopsy tissue from one boy with PZS using electron microscopy. Lamellar-lipid inclusions and mitochondria with crystalline inclusions and/or abnormal cristae are also described in two patients, one with IRD, the other with NALD. © 1990 Springer-Verlag.

引用

页码：255 / 264

页数：10

共 38 条

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