oxalates and Chronic-Kidney-Disease-Mineral-and-Bone-Disorder

The purpose of this article is to report the experience of three centers with combined hepatic and renal transplantation for pyridoxine-resistant primary hyperoxaluria type I (alanine:glyoxylate aminotransferase [EC 2.6.1.44] deficiency), with particular emphasis on the selection criteria and timing of the operation. Nine patients with this inherited disease were treated by combined hepatic and renal transplantation. The former replaces the enzyme-deficient organ while the latter replaces the functionally affected organ.. One patient with gross systemic oxalosis died in the immediate postoperative period and another died 8 weeks postoperatively of a generalized cytomegalovirus infection, having shown evidence of biochemical correction. One patient with particularly severe osteodystrophy at the time of the operation died 14 months postoperatively from renal failure due to progressive calcium oxalate nephrocalcinosis involving the transplanted kidney, plus thromboembolic disease. He also had very extensive systemic oxalosis. An additional patient with severe osteodystrophy died 9 months postoperatively. One patient developed hyper-rejection of the kidney and died later of gastrointestinal hemorrhage. The four long-term survivors (22 to 38 months) have remained asymptomatic from the standpoint of their renal disease, with resolution of any manifestations of systemic oxalosis that they may have had. They are either employed or continuing their education.. A prolonged period of end-stage renal failure treated by dialysis regimens that are suitable for non-hyperoxaluric renal failure and extensive systemic oxalosis, particularly oxalotic osteodystrophy, are poor prognostic features. We propose that hepatic transplantation should be considered as definitive treatment before end-stage renal failure develops. This should be supplemented by renal transplantation with vigorous pre- and perioperative hemodialysis to deplete the body stores of oxalate. Although some authorities would reserve hepatic transplantation for patients in whom renal transplantation has failed, we suggest that combined liver and kidney transplantation is appropriate in patients who have never had a renal graft. Furthermore, the time has come to consider hepatic transplantation before any irreversible renal damage has occurred in these patients.

Topics: Adolescent; Adult; Chronic Kidney Disease-Mineral and Bone Disorder; Contraindications; Female; Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Kidney Transplantation; Liver Transplantation; Male; Oxalates; Renal Dialysis

Rosette-like arrays of highly birefringent calcium oxalate crystals are commonly seen in the marrow space of bone biopsy specimens taken from patients with primary hyperoxaluria, particularly if complicated by renal failure. Similar deposits have been described in chronic hemodialysis patients with secondary forms of oxalosis. Large multinucleated histiocytes may be seen surrounding these crystal deposits. Many of these cells are histologically indistinguishable from osteoclasts. We present a patient in whom this histiocytic reaction appeared to be of sufficient magnitude to stimulate bone resorption and to cause severe osteodystrophy. This observation, and those of other investigators reviewed in the discussion, suggest that oxalate deposition within bone may contribute to the pathogenesis of uremic osteodystrophy in chronic renal failure patients with primary or secondary types of oxalosis.

Topics: Adult; Bone Resorption; Chronic Kidney Disease-Mineral and Bone Disorder; Humans; Male; Oxalates; Renal Dialysis

Primary oxalosis is a rare congenital disorder. The excessive oxalate biosynthesis induces deposits in many organs, particularly in kidney and bone. The late onset of primary oxalosis is reported in a 50-year-old man. His chronic renal failure was treated by maintenance hemodialysis for 3 years. He then developed a diffuse bone disease with osteosclerosis and roentgenographic features of hyperparathyroidism. A parathyroidectomy was performed, with debatable improvement of bone lesions. Laboratory results and histologic and histomorphometric studies before and after parathyroidectomy suggest a double histopathogenetic mechanism for this bone disease: renal osteodystrophy and massive bone oxalate deposits. Such deposits may induce both a heterogeneous osteosclerosis with dense metaphyseal bands and histologic bone lesions similar to those of hyperparathyroidism. The crystalline deposits induce in the bone tissue a granulomatous macrophagic reaction. These macrophages are unable to phagocytize the crystals and may be involved in active bone resorption. Bone lesions of oxalosis occur in patients with chronic renal failure, and hyperparathyroidism has a worsening role.

Topics: Bone and Bones; Chronic Kidney Disease-Mineral and Bone Disorder; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Hyperparathyroidism, Secondary; Kidney Failure, Chronic; Macrophages; Male; Middle Aged; Oxalates

We performed a bone biopsy on a patient who had been receiving hemodialysis for 23 years. The bone had oxalate deposition. Previous bone biopsies had shown osteitis fibrosis without oxalate deposition. The osteoid area was increased (11% of bone area), as was the fibrosis (6.1% of tissue area) and aluminum deposition (38% of surface). Bone formation rate was normal (259 mu 2/mm2/d). We examined bone biopsies of 22 patients who had been receiving hemodialysis for over 10 years, and none had oxalate deposits. We discovered that our patient had been ingesting 2.6 g/d of vitamin C. The bone oxalate deposition may have been caused by the ingestion of high doses of vitamin C.

Topics: Ascorbic Acid; Biopsy; Bone and Bones; Chronic Kidney Disease-Mineral and Bone Disorder; Humans; Male; Middle Aged; Oxalates; Renal Dialysis; Self Medication; Time Factors

Topics: Aldehyde-Ketone Transferases; Calcinosis; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chronic Kidney Disease-Mineral and Bone Disorder; Female; Glyoxylates; Humans; Ketoglutaric Acids; Kidney Calculi; Kidney Failure, Chronic; Male; Oxalates; Oxo-Acid-Lyases

Topics: Child; Child, Preschool; Chronic Kidney Disease-Mineral and Bone Disorder; Female; Humans; Infant; Kidney Calculi; Male; Metabolic Diseases; Nephrocalcinosis; Oxalates; Radiography

Topics: Adolescent; Child; Child, Preschool; Chronic Kidney Disease-Mineral and Bone Disorder; Female; Humans; Humerus; Hyperparathyroidism, Secondary; Infant; Kidney Failure, Chronic; Osteosclerosis; Oxalates; Radiography; Skull