Universität zu Köln

Recker, Pia, Beck, Bodo Bernhard, Sikora, Przemyslaw, Goebel, Heike ORCID: 0000-0002-0171-7025, Kemper, Markus Josef, Nazco, Angel, Martin-Higueras, Cristina ORCID: 0000-0003-1139-4642 and Hoppe, Bernd (2022). Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I. Sci Rep, 12 (1). BERLIN: NATURE PORTFOLIO. ISSN 2045-2322

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Abstract

Patients with primary hyperoxaluria type I (PH I) are prone to develop early kidney failure. Systemic deposition of calcium-oxalate (CaOx) crystals starts, when renal function declines and plasma oxalate increases. All tissue, but especially bone, heart and eyes are affected. However, liver involvement, as CaOx deposition or chronic hepatitis/fibrosis has never been reported. We examined liver specimen from 19 PH I patients (aged 1.5 to 52 years at sample collection), obtained by diagnostic biopsy (1), at autopsy (1), or transplantation (17). With polarization microscopy, birefringent CaOx crystals located in small arteries, but not within hepatocytes were found in 3/19 patients. Cirrhosis was seen in one, fibrosis in 10/19 patients, with porto-portal and nodular fibrosis (n = 1), with limitation to the portal field in 8 and/or to central areas in 5 patients. Unspecific hepatitis features were observed in 7 patients. Fiber proliferations were detectable in 10 cases and in one sample transformed Ito-cells (myofibroblasts) were found. Iron deposition, but also megakaryocytes as sign of extramedullary erythropoiesis were found in 9, or 3 patients, respectively. Overall, liver involvement in patients with PH I was more pronounced, as previously described. However, CaOx deposition was negligible in liver, although the oxalate concentration there must be highest.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Recker, Pia UNSPECIFIED UNSPECIFIED UNSPECIFIED Beck, Bodo Bernhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Sikora, Przemyslaw UNSPECIFIED UNSPECIFIED UNSPECIFIED Goebel, Heike UNSPECIFIED orcid.org/0000-0002-0171-7025 UNSPECIFIED Kemper, Markus Josef UNSPECIFIED UNSPECIFIED UNSPECIFIED Nazco, Angel UNSPECIFIED UNSPECIFIED UNSPECIFIED Martin-Higueras, Cristina UNSPECIFIED orcid.org/0000-0003-1139-4642 UNSPECIFIED Hoppe, Bernd UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-685672
DOI:	10.1038/s41598-022-19584-9
Journal or Publication Title:	Sci Rep
Volume:	12
Number:	1
Date:	2022
Publisher:	NATURE PORTFOLIO
Place of Publication:	BERLIN
ISSN:	2045-2322
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CHILDREN; TRANSPLANTATION; DIALYSIS; REGISTRY; PLASMA Multiple languages Multidisciplinary Sciences Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/68567