caa a22 4500 465753183 CHVBK 20180323111839.0 cr unu---uuuuu 170327e19900501xx s 000 0 eng 10.1007/BF01678985 doi (NATIONALLICENCE)springer-10.1007/BF01678985 Pathology of hepatic peroxisomes and mitochondria in patients with peroxisomal disorders [Elektronische Daten] [J. Hughes, A. Poulos, E. Robertson, C. Chow, L. Sheffield, J. Christodoulou, R. Carter] Summary: 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. Springer-Verlag, 1990 Peroxisomes nationallicence Peroxisomal disorders nationallicence Neonatal adrenoleukodystrophy nationallicence Infantile Refsum's disease nationallicence Pseudo-Zellweger disease nationallicence Mitochondria nationallicence Hughes J. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut Poulos A. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut Robertson E. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut Chow C. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut Sheffield L. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut Christodoulou J. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut Carter R. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut Virchows Archiv A Springer-Verlag 416/3(1990-05-01), 255-264 0174-7398 416:3<255 1990 416 428 https://doi.org/10.1007/BF01678985 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01678985 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Hughes J. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut NATIONALLICENCE 700 1- Poulos A. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut NATIONALLICENCE 700 1- Robertson E. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut NATIONALLICENCE 700 1- Chow C. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut NATIONALLICENCE 700 1- Sheffield L. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut NATIONALLICENCE 700 1- Christodoulou J. Department of Anatomical Pathology and Murdoch Institute, Royal Children's Hospital, Melbourne, Victoria, Australia aut NATIONALLICENCE 700 1- Carter R. Departments of Histopathology and Chemical Pathology, Adelaide Children's Hospital, North Adelaide, South Australia aut NATIONALLICENCE 773 0- Virchows Archiv A Springer-Verlag 416/3(1990-05-01), 255-264 0174-7398 416:3<255 1990 416 428 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer