Cases reported "Hyperoxaluria"

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11/22. Dental and jaw changes in primary hyperoxaluria.

    A case of teeth and jaw changes in primary hyperoxaluria is described. The patient, a 25-yr-old man, was treated by kidney transplantation twice, and finally by combined liver and kidney transplantation. The teeth and jaw changes consisted of deposition of oxalate crystals in the gingiva, in the pulp and in the vicinity of bone and dentin. The resorption of bone and dentin was extensive.
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12/22. Vitamin B6 resistant primary hyperoxaluria type I. Report of 5 cases.

    Primary hyperoxaluria type I (PH I) is characterized by an excessive endogenous production and excretion of oxalic and glycolic acid. prognosis of this "inborn error of metabolism" is not favorable due to calcium-oxalate depositions in kidney and other organs. Vitamin B6 administration and/or renal transplantation can greatly improve the prognosis, as reported in literature. In this article our experience with 5 patients with vitamin B6 resistant hyperoxaluria is reported. Symptomatology and progression of the primary disease are described. The results of treatment interfering with oxalate production and calcium-oxalate crystallization are given. Three patients underwent renal replacement therapy. In these, oxalosis developed during hemodialysis and progressed following transplantation; a disabling bone disease was the most severe complication. Outcome of transplantation was disappointing. In two out of three patients, there was recurrence of the primary disease in the graft. In only one of them long-term graft function was satisfying. However, even this good function could not prevent disabling symptoms of oxalosis. Therefore, evaluation of the results of transplantation should not only include data related to graft function and survival, but also the complications due to calcium-oxalate depositions in various organs. To prevent oxalosis, kidney transplantation should be performed before end stage renal disease is achieved in patients with vitamin B6 resistant PH I.
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13/22. Reversible arterial spasm in an adolescent with primary oxalosis.

    A 16-year-old girl with primary oxalosis type I presented with progressive claudication soon after being treated with chronic intermittent hemodialysis. Arterial insufficiency of the lower limbs was confirmed clinically (purple discoloration of the skin and absence of arterial pulses) and with Doppler sonography. The arteriogram showed diffuse and symmetric narrowing with smooth vessel walls. Treatment with sodium nitroprusside had a spectacular effect; nifedipine was less effective. Renal transplantation with the father's kidney resulted in a rapid, complete and sustained reversal of the ischemic features. magnesium withdrawal is assumed to be a pathogenic factor of the vascular spasm in this patient.
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14/22. urolithiasis and enteric hyperoxaluria in a child with steatorrhea.

    Malabsorptive states are frequently associated with increased urinary oxalate excretion. The authors describe a 10-year-old girl with steatorrhea, hyperoxaluria, and a renal calculus in a single functioning kidney. Successful management of steatorrhea corrected both the chronic diarrhea and hyperoxaluria. Enteric hyperoxaluria is a well-known etiology of calcium oxalate urolithiasis in adults. Pediatricians caring for children with malabsorptive conditions should be aware of the risk of urinary calculus formation as a result of increased dietary oxalate absorption.
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15/22. Primary bone oxalosis: the roles of oxalate deposits and renal osteodystrophy.

    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.
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16/22. Mild metabolic hyperoxaluria and its response to pyridoxine.

    Three cases of mild metabolic hyperoxaluria (with glycollaturia) are described. They showed different types of response to pyridoxine. One responded to low dose, one responded at first to low dose but became resistant, and the third showed temporary response to high dose. One case also had primary hyperparathyroidism and one had medullary sponge kidneys and hypercalciuria. It is important to measure urinary oxalate (and glycollate) in all cases of calcium oxalate urolithiasis.
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17/22. Rapid reversal of bone scan abnormalities in a patient with type 1 primary hyperoxaluria and oxalosis.

    Serial bone scanning was performed on a 23-year-old man with type 1 primary hyperoxaluria, renal failure, oxalosis, and cardiac failure. The initial bone scan (6/22/90) demonstrated diffuse increased uptake in the axial and peripheral skeleton, heart, and the soft tissues of the lower extremities. A combined liver and kidney transplant was successfully performed with subsequent resolution of the oxalosis, renal failure, and cardiac failure. A follow-up bone scan (9/24/90) demonstrated resolution of abnormal heart and soft tissue uptake, as well as decreased uptake in the long bones. This case demonstrates that bone scanning may be a useful tool in the management of patients with oxalosis. The scintigraphic findings closely paralleled the clinical response to treatment of the disease and suggested decreased total body stores of calcium oxalate.
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18/22. The infant with primary hyperoxaluria and oxalosis: from diagnosis to multiorgan transplantation.

    The care of an infant with primary hyperoxaluria and oxalosis is discussed. After an unheralded presentation, followed by 9 months of intensive treatment that included combined hemodialysis and peritoneal dialysis, the infant successfully underwent combined liver and kidney transplantation to definitely address both kidney failure and the underlying metabolic defect. Discussion of this approach, including ongoing input from the parents, addresses both the implications of undertaking the "best therapy" for this disease as well as the ethical dilemma passed by the decision whether to proceed or not to proceed with therapy.
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19/22. Consecutive successful pregnancies in a combined liver and kidney transplant recipient with type 1 primary hyperoxaluria.

    pregnancy is now a common, but high-risk event, in young women who have received transplants. Consequences to the fetus are known, but pregnancy may also interfere with graft function. We report the outcome of two successive and successful pregnancies in a 29-year-old woman with type 1 hyperoxaluria, who received a combined liver and kidney transplant. Two healthy children were born at 35 and 37 weeks of gestation, with low birth weight. liver function remained normal before, during, and after pregnancies up to 52 months after transplantation. Renal function was impaired before the first conception, worsened during both pregnancies, and returned to the previous level in both immediate postpartum periods. However, renal function has declined 17 months after the last delivery. This report shows the feasibility of successive pregnancies in multiple organ transplant recipients, but raises the question of long-term maternal kidney graft survival.
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20/22. Primary hyperoxaluria in an adult with renal failure, livedo reticularis, retinopathy, and peripheral neuropathy.

    We present the case of a young woman who developed renal failure of unknown cause, and after 2 months of maintenance hemodialysis developed livedo reticularis, retinopathy, and peripheral sensory neuropathy. The patient was subsequently shown to have primary oxalosis type I, a rare autosomal recessive error of metabolism characterized by accumulation of calcium oxalate crystals in the kidneys, eyes, skin, and other organs. Intravascular obstruction, caused by deposition of calcium oxalate crystals in cutaneous arterioles, is thought to be responsible for the ischemic livedo reticularis lesions observed in this patient. A method is described for measuring serum glycolate by isotope dilution gas chromatography-mass spectrometry (GC-MS). An approach to the diagnosis and management is also briefly mentioned.
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