Cases reported "Hyperoxaluria"

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1/22. Domino hepatic transplantation using the liver from a patient with primary hyperoxaluria.

    BACKGROUND: We report a case of domino liver transplantation using the liver harvested from a patient who underwent a combined liver and kidney transplantation for primary hyperoxaluria (PH). METHOD: A cadaveric liver transplantation was performed in a 19-year-old man with PH. In a second step, the PH liver harvested from the first patient was transplanted in a 69-year-old man with hepatitis c-related cirrhosis, not a candidate for a classic liver graft owing to multifocal hepatocellular carcinoma. RESULTS: At 8 months after transplantation, the domino recipient has normal hepatic function and no signs of tumoral recurrence, but he progressively developed hyperoxalemia, hyperoxaluria, and renal insufficiency. CONCLUSION: Regarding the favorable postoperative clinical evolution, domino liver transplantations using livers from PH patients may represent a new opportunity for marginal candidates for liver transplantation. However, the progressive renal insufficiency expected in such domino recipients should limit this procedure to selected cases.
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2/22. Novel mutation in the GRHPR gene in a Chinese patient with primary hyperoxaluria type 2 requiring renal transplantation from a living related donor.

    We identified a patient with primary hyperoxaluria type 2 (PH2) showing recurrent stone formation, nephrocalcinosis, end-stage renal failure, and rapid oxalate deposition after renal transplantation from a living related donor. Urinary organic acid analysis performed after renal transplantation confirmed the diagnosis of PH2. We analyzed the glyoxylate reductase/hydroxypyruvate reductase (GRHPR) gene of the patient. dna sequencing of all nine exons and exon-intron boundaries showed a novel homozygous mutation deleting the last two nucleotides of exon 8, ie, 862delTG. This deletion results in a frameshift and introduction of a premature stop codon at codon 310, ie, Ala310Stop. One of the patient's sisters is heterozygous for this mutation, and the other sister, who is the donor, does not have this mutation. The rapid deposition of oxalate in the transplanted kidney indicates that the kidney is not a major site of oxalate production. The more favorable long-term prognosis of PH2 needs to be reevaluated now that the molecular basis of PH2 has been established. dna-based diagnosis will facilitate carrier detection, prenatal diagnosis, genetic counseling, and selection of living related donors.
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3/22. endocytosis of calcium oxalate crystals and proliferation of renal tubular epithelial cells in a patient with type 1 primary hyperoxaluria.

    A patient with primary hyperoxaluria who received a liver-kidney transplant is presented. A postoperative renal biopsy showed apparent tubular cell endocytosis of calcium oxalate crystals and cell proliferation, indicating that renal epithelial cells do not perceive urinary crystals as inert. Such cellular responses to crystals may have a role in nephrolithiasis.
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4/22. erythropoietin resistance as a result of oxalosis in bone marrow.

    anemia is an important cause of morbidity in patients suffering from chronic renal failure, and erythropoietin is a milestone of anemia treatment. Various factors may cause erythropoietin resistance. Herein, we describe the case of 32-year-old man who presented with anemia and weakness. He developed progressive renal failure secondary to recurrent kidney stones. One year before admission, he developed anemia for which he had been treated with erythropoietin. However, the anemia persisted. Examination of bone marrow biopsy specimen showed that the marrow was extensively replaced with oxalate crystals and fibrous connective tissue with severe decrease of hematopoietic cells. To the best of our knowledge, our patient represents the first case in the literature describing the association between the oxalate deposition and EPO resistance.
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5/22. Amelioration of anemia after kidney transplantation in severe secondary oxalosis.

    INTRODUCTION: In small bowel disease such as M. Crohn, the intestinal absorption of oxalate is increased. Severe calcium oxalate deposition in multiple organs as consequence of enteric hyperoxaluria may lead to severe organ dysfunction and chronic renal failure. The management of hemodialyzed patients with short bowel syndrome may be associated with vascular access problems and oxalate infiltration of the bone marrow leading to pancytopenia. Although the risk of recurrence of the disease is very high after renal transplantation, it may be the ultimate therapeutic alternative in secondary hyperoxaluria. CASE: Here, we report a patient with enteric oxalosis due to Crohn's disease. He developed end-stage renal disease, erythropoietin-resistant anemia, oxalate infiltration of the bone marrow and severe vascular access problems. Following high-urgency kidney transplantation, daily hemodiafiltration of 3 hours was performed for 2 weeks to increase oxalate clearance. Despite tubular and interstitial deposition of oxalate in the renal transplant, the patient did not require further hemodialysis and the hematocrit levels normalized. DISCUSSION: Early treatment of hyperoxaluria due to short bowel syndrome is essential to prevent renal impairment. Declining renal function leads to a further increase in oxalate accumulation and consecutive oxalate deposition in the bone marrow or in the vascular wall. If alternative treatments such as special diet or daily hemodialysis are insufficient, kidney transplantation may be a therapeutic alternative in severe cases of enteric oxalosis despite a possible recurrence of the disease.
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6/22. Reversal of cardiac dysfunction secondary to type 1 primary hyperoxaluria after combined liver-kidney transplantation.

    A 23-year-old man with type 1 primary hyperoxaluria, renal failure, and oxalosis developed a severe cardiomyopathy while awaiting combined liver-kidney transplantation. This manifested as radiographic cardiomegaly, a dilated hypokinetic left ventricle with a decreased ejection fraction, ventricular arrhythmias, and cardiac uptake on bone scanning. On liver and kidney transplantation, these abnormalities markedly improved and/or reversed. The cardiac size almost normalized, the left ventricular ejection fraction increased from 20% to 34%, the ventricular arrhythmias resolved, and the cardiac uptake on bone scanning disappeared. This coincided with normalization of oxalate production and excretion. Severe cardiac involvement secondary to oxalosis in patients with primary hyperoxaluria may improve or reverse with combined liver-kidney transplantation.
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7/22. Clinical and histologic features of primary oxalosis.

    Primary oxalosis should be considered in patients with multisystem disease of the kidneys, heart, peripheral vasculature, and skin. Crystalline deposits can lead to nephrolithiasis with kidney failure, complete heart block, peripheral vasospasm, and livedo reticularis, as in our patient. Crystals were first observed in the myocardial biopsy specimen and then identified as calcium oxalate in skin from an area of livedo reticularis.
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8/22. recurrence of nephrocalcinosis after renal transplantation in an adult patient with primary hyperoxaluria type I.

    The medical history of a 42-year-old patient with primary hyperoxaluria type I is presented. Primary hyperoxaluria was suspected after renal transplantation, when oxalate deposits were found in a biopsy of the kidney graft. Diagnosis of type I hyperoxaluria was confirmed by the finding that significantly increased amounts of glycolic acid and oxalic acid were excreted. Treatment of the patient with 500 mg pyridoxine daily resulted in a decrease of the excretion of oxalate to normal values.
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9/22. Bilateral renal autotransplantation with pyelovesicostomy: a surgical treatment of refractory enteric hyperoxaluria.

    A 38-year-old man, with only 3 feet of small bowel remaining after multiple resections because of chronic inflammatory bowel disease, had severe symptomatic calcium oxalate nephroureterolithiasis. Because of the refractory symptoms, he was successfully treated with bilateral autotransplantation of the kidneys, totally bypassing the ureters. Anatomically effective urinary tract continuity was reestablished by means of bilateral pyelovesicostomies with concomitant rectus muscle vesicofixation to create direct stone-dumping channels into the urinary bladder. The patient is now completely without symptoms 18 months after surgery. A description and rationale for this surgical treatment is provided. Bilateral autotransplantation of the kidneys with direct drainage into the urinary bladder may be an attractive and viable therapeutic option in complicated patients with short-gut syndrome and severe refractory calcium oxalate nephroureterolithiasis.
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10/22. Late presentation and microcrystalline arthropathy in primary hyperoxaluria.

    A 66 year-old woman was referred in 1981 because of renal insufficiency and pronounced nephrocalcinosis. The urinary oxalate excretion was elevated. Secondary hyperoxaluria was excluded. End-stage renal disease necessitated hemodialysis from late in 1982 up to her death in 1986, at the age of 71 years. During the course of the disease, an aggressive arthropathy developed in the fingers. Classical signs of oxalosis were found: deposits of calcium oxalate crystals in bone tissue, the pancreas, myocardium, subcutaneous tissue and especially in the kidneys. This rare case documents the possible occurrence of late clinical presentation and long survival in primary oxalosis.
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