{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T20:54:09Z","timestamp":1780606449756,"version":"3.54.1"},"reference-count":24,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1987,10,6]],"date-time":"1987-10-06T00:00:00Z","timestamp":560476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1988,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Polarizing material has been reported in the liver of children with infantile Refsum's disease (IRD) and was absent in two patients with the cerebro\u2010hepato\u2010renal syndrome of Zellweger (CHRS). We examined in polarized light 15 liver biopsy and autopsy samples from six other patients with the cerebro\u2010hepato\u2010renal syndrome of Zellweger, two with the rhizomelic form of chondrodysplasia punctata (rCDP) and two with X\u2010linked adrenoleukodystrophy (ALD), all conditions with deficient peroxisomes. Two types of birefringent inclusions were found in CHRS only: the first is transparent in bright field, the second appears as brown granules or rods, similar to lipofuscins. As in IRD large PAS\u2010positive macrophage\u2010like cells contain the transparent type. Electron microscopical investigation of these cells shows trilaminar structures within membrane\u2010bound organelles. The two types were also seen in kidney and brown adipose tissue, the first type in pancreas, the second type in adrenal gland; no such was observed in myocardium or in thyroid gland (CHRS). No birefringent inclusions were present in rCDP and ALD. The nature of the inclusions is still unclear. An accumulation of the transparent polarizing material with increasing age of the patients is most likely.<\/jats:p>","DOI":"10.1007\/bf01800426","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:01:24Z","timestamp":1119373284000},"page":"372-386","source":"Crossref","is-referenced-by-count":16,"title":["Polarizing inclusions in some organs of children with congenital peroxisomal diseases (Zellweger's, Refsum's, chondrodysplasia punctata (rhizomelic form), X\u2010linked adrenoleukodystrophy)"],"prefix":"10.1002","volume":"11","author":[{"given":"I.","family":"Kerckaert","sequence":"first","affiliation":[{"name":"Menselijke Anatomie Vrije Universiteit Brussel  Laarbeeklaan 103 Brussels B\u20101090 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K. P.","family":"Dingemans","sequence":"additional","affiliation":[{"name":"Pathologie, Academisch Medisch Centrum  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. S. A.","family":"Heymans","sequence":"additional","affiliation":[{"name":"Pediatrie, Academisch Ziekenhuis  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Vamecq","sequence":"additional","affiliation":[{"name":"Laboratoire de Chimie Physiologique, I.C.P. Universit\u00e9 Catholique de Louvain  Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Roels","sequence":"additional","affiliation":[{"name":"Menselijke Anatomie Vrije Universiteit Brussel  Laarbeeklaan 103 Brussels B\u20101090 Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1987,10,6]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441774"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)38153-0"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02533618"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(81)90151-9"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00443065"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442582"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799743"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/24.6.59773"},{"issue":"14","key":"e_1_2_1_10_1","first-page":"22","article-title":"Polarizing inclusions in the liver of children with congenital peroxisomal diseases (syndrome of Zellweger; infantile Refsum's disease; chondrodysplasia punctata)","volume":"41","author":"Kerckaert I.","year":"1986","journal-title":"Eur. J. Cell. Biol."},{"key":"e_1_2_1_11_1","first-page":"1911","article-title":"Polarizing inclusions in the liver of children with congenital peroxisomal diseases (syndrome of Zellweger; infantile Refsum's disease; chondrodysplasia punctata)","volume":"16","author":"Kerckaert I.","year":"1986","journal-title":"Hepat. Rapid Lit. Rev."},{"key":"e_1_2_1_12_1","first-page":"486","volume-title":"Histochemie et Cytochemie Animales \u2014 Vol II","author":"Lison H.","year":"1960"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.3109\/01913128309141833"},{"key":"e_1_2_1_14_1","first-page":"264","volume-title":"Histochemistry: Theoretical and Applied","author":"Pearse A. G. E.","year":"1968"},{"key":"e_1_2_1_15_1","first-page":"294A","article-title":"Absence of hepatic peroxisomes in neonatal onset adrenoleukodystrophy","volume":"17","author":"Partin J. S.","year":"1983","journal-title":"Pediatr. Res."},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.59.3.222"},{"key":"e_1_2_1_17_1","first-page":"860","article-title":"Biochemical evidence for clinically diagnosed adrenoleukodystrophy in two brothers","volume":"65","author":"Pretorius P. J.","year":"1984","journal-title":"S.A. Med. J."},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320250210"},{"key":"e_1_2_1_19_1","doi-asserted-by":"crossref","unstructured":"Roels F. Pauwels M. Poll\u2010The B. T. Scotto J. Ogier H. Aubourg P.andSaudubray J. M.Hepatic peroxisomes in adrenoleukodystrophy and related syndromes: Cytochemical and morphometric data.Virchows Archiv A(1988) (in press).","DOI":"10.1007\/BF00783019"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(84)91430-X"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441734"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799998"},{"key":"e_1_2_1_23_1","first-page":"131","volume-title":"Statistical Methods","author":"Snedecor G. W.","year":"1967"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5320(69)90033-1"},{"key":"e_1_2_1_25_1","first-page":"524","article-title":"Multiple peroxisomal enzymatic deficiency disorders: a comparative biochemical and morphologic study of Zellweger cerebro\u2010hepato\u2010renal syndrome and neonatal adrenoleukodystrophy","volume":"125","author":"Vamecq J.","year":"1986","journal-title":"Am. J. Pathol."}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01800426","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01800426","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T08:56:27Z","timestamp":1694854587000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF01800426"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1987,10,6]]},"references-count":24,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1988,12]]}},"alternative-id":["10.1007\/BF01800426"],"URL":"https:\/\/doi.org\/10.1007\/bf01800426","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1987,10,6]]}}}