Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I
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...
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| Format: | Article |
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Nature Portfolio
2022-10-01
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| Series: | Scientific Reports |
| Online Access: | https://doi.org/10.1038/s41598-022-19584-9 |
| _version_ | 1797996097164017664 |
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| author | Pia Recker Bodo Bernhard Beck Przemyslaw Sikora Heike Göbel Markus Josef Kemper Angel Nazco Cristina Martin-Higueras Bernd Hoppe |
| author_facet | Pia Recker Bodo Bernhard Beck Przemyslaw Sikora Heike Göbel Markus Josef Kemper Angel Nazco Cristina Martin-Higueras Bernd Hoppe |
| author_sort | Pia Recker |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T10:12:05Z |
| format | Article |
| id | doaj.art-af602ad563e54a95a037197be35b5916 |
| institution | Directory Open Access Journal |
| issn | 2045-2322 |
| language | English |
| last_indexed | 2024-04-11T10:12:05Z |
| publishDate | 2022-10-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Scientific Reports |
| spelling | doaj.art-af602ad563e54a95a037197be35b59162022-12-22T04:30:04ZengNature PortfolioScientific Reports2045-23222022-10-011211910.1038/s41598-022-19584-9Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type IPia Recker0Bodo Bernhard Beck1Przemyslaw Sikora2Heike Göbel3Markus Josef Kemper4Angel Nazco5Cristina Martin-Higueras6Bernd Hoppe7Department of Pediatric and Adolescent Medicine, Division of Pediatric Nephrology, University Hospital CologneInstitute of Human Genetics, University Hospital CologneDepartment of Pediatric Nephrology, Lublin Medical UniversityDepartment of Pathology, University Hospital CologneDepartment of Pediatric and Adolescent Medicine, Asklepios Klinik HamburgDepartment of Pathology, Nuestra Señora de Candelaria University HospitalDepartment of Basic Medical Sciences, Centre for Biomedical Research in Rare Diseases (CIBERER), Institute of Biomedical Technologies (ITB), University of La LagunaDepartment of Pediatric and Adolescent Medicine, Division of Pediatric Nephrology, University Hospital CologneAbstract 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.https://doi.org/10.1038/s41598-022-19584-9 |
| spellingShingle | Pia Recker Bodo Bernhard Beck Przemyslaw Sikora Heike Göbel Markus Josef Kemper Angel Nazco Cristina Martin-Higueras Bernd Hoppe Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I Scientific Reports |
| title | Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I |
| title_full | Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I |
| title_fullStr | Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I |
| title_full_unstemmed | Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I |
| title_short | Chronic liver disease and hepatic calcium-oxalate deposition in patients with primary hyperoxaluria type I |
| title_sort | chronic liver disease and hepatic calcium oxalate deposition in patients with primary hyperoxaluria type i |
| url | https://doi.org/10.1038/s41598-022-19584-9 |
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