Long-term complications of systemic oxalosis in children—a retrospective single-center cohort study.
Background: Systemic oxalosis is a severe complication seen in primary hyperoxaluria type I patients with kidney failure. Deposition of insoluble calcium oxalate crystals in multiple organs leads to significant morbidity and mortality. Methods: We describe a retrospective cohort of 11 patients with...
| Publicado en: | Pediatric Nephrology Vol. 36; no. 10; pp. 3123 - 3133 |
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| Autores principales: | , , , , , , , |
| Formato: | diagnostic images pictorial research tables/charts Journal Article |
| Publicado: |
Springer Nature
Oct2021
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=152503174&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 152503174 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Oct2021 vid: 36 iid: 10 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 152503174 149008633 152503174 152503174 10.1007/s00467-021-05002-1 152503174 ppf: 3123 ppct: 10 formats: fmt: @attributes: type: P tig: atl: Long-term complications of systemic oxalosis in children—a retrospective single-center cohort study. aug: au: Ben-Shalom, Efrat Cytter-Kuint, Ruth Rinat, Choni Becker-Cohen, Rachel Tzvi-Behr, Shimrit Goichberg, Jenny Peles, Vardit Frishberg, Yaacov affil: Institute of Pediatric Nephrology, Shaare Zedek Medical Center, 12 Shmuel Bait St., 9103102, Jerusalem, Israel sug: subj: Metabolic Diseases Complications Dialysis Kidney Physiology Oxalic Acids Metabolism Metabolic Diseases Therapy Human Retrospective Design Child Dialysis Centers Record Review Descriptive Statistics Male Female Bone Diseases Fractures Kidney Transplantation Liver Transplantation Child: 6-12 years Male Female ab: Background: Systemic oxalosis is a severe complication seen in primary hyperoxaluria type I patients with kidney failure. Deposition of insoluble calcium oxalate crystals in multiple organs leads to significant morbidity and mortality. Methods: We describe a retrospective cohort of 11 patients with systemic oxalosis treated at our dialysis unit from 1982 to 1998 (group 1) and 2007–2019 (group 2). Clinical and demographic data were collected from medical records. Imaging studies were only available for patients in group 2 (n = 5). Results: Median age at dialysis initiation was 6.1 months (IQR 4–21.6), 64% were male. Dialysis modality was mostly peritoneal dialysis in group 1 and daily hemodialysis in group 2. Bone disease was the first manifestation of systemic oxalosis, starting with the appearance of sclerotic bands (mean 166 days, range 1–235), followed by pathological fractures in long bones (mean 200.4 days, range 173–235 days). Advanced disease was characterized by vertebral fractures with resulting kyphosis, worsening splenomegaly, and adynamic bone disease. Two patients developed pulmonary hypertension, 4 and 8 months prior to their death. Four of 11 patients developed hypothyroidism 0–60 months after dialysis initiation. Only one patient survived after a successful liver–kidney transplantation. Four patients died after liver or liver–kidney transplantation. Conclusions: This is the first comprehensive description of the natural history of pediatric systemic oxalosis. We hope that our findings will provide basis for a quantitative severity score in future, larger studies. pubtype: Academic Journal doctype: diagnostic images pictorial research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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