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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fourteen patients with ileal dysfunction due to resection or bypass were encountered over an 18-month period. Symptoms had been present for a mean period of 1.8 years. Diarrhoea was a universal symptom, and varied from mild to incapacitating. Weight loss, due in part to malabsorption and in part to the patients' fear of eating, occurred in 10 of 14 patients. The chief metabolic abnormalities were steatorrhoea and hypokalaemia. Vitamin B12 deficiency, folate deficiency, anaemia, hypoalbuminaemia, hypocalcaemia, hypomagnesaemia, hyperoxaluria, and an abnormal prothrombin ratio were less frequently seen. Treatment with cholestyramine and/or long-chain fat restriction effectively reduced diarrhoea in every case, and this was supplemented by replacement of specific deficiencies. There was little added benefit from non-specific antidiarrhoeal agents. It was found that the major symptoms of ileal dysfunction are readily treated, but that attention should also be given to a number of nutritional deficiencies.
Med J Aust 1978 Dec 16
PMID:Consequences of ileal dysfunction: an approach to management. 10 34

To investigate the possibility of measuring urinary oxalate output instead of faecal fat excretion as an outpatient screening test for steatorrhoea, we determined 24 hour urinary oxalate and five day faecal fat excretion before and during an oral load of sodium oxalate 600 mg daily (oxalate 4.44 mmol), in 32 patients with suspected malabsorption on a diet containing oxalate 30 mg (0.33 mmol), fat 50 g (180 mmol), and calcium 1 g (25 mmol). Nineteen patients proved to have steatorrhoea (mean faecal fat 62 mmol/24 h, range 19--186 mmol) of varying aetiologies. On the diet alone, urinary oxalate was raised in only nine of these patients (mean 0.25 mmol/24 h, range 0.08--0.59 mmol) (normal less than 0.20). By contrast, when the diet was supplemented with oral sodium oxalate, all 19 patients with steatorrhoea had hyperoxaluria (mean 0.91 mmol/24 h, range 0.46--1.44 mmol) (normal less than 0.44). There was a significant positive linear relationship between urinary oxalate and faecal fat when the 32 patients were on the high oxalate intake (r = 0.73, P less than 0.001), but not when they were on the low oxalate intake. Mean percentage absorption of orally administered oxalate was 5.8 +/- 0.99% (+/- 1 SD) in normal subjects and 14.7 +/- 6.0% (P less than 0.002) in patients with steatorrhoea. Measurement of urinary oxalate output during oral sodium oxalate loading appears to be a reliable and convenient screening test for steatorrhoea.
Gut 1979 Dec
PMID:Oxalate loading test: a screening test for steatorrhoea. 52 84

Absorptive hypercalciuria was treated in 27 patients with cellulose phosphate. In all patients urinary calcium decreased and stone formation virtually ceased. The most striking side effect was an excessive hyperoxaluria, necessitating withdrawal of the drug in 8 patients. Succinate decreased the hyperoxaluria in 14 of 19 patients. All patients had mild hypercalciuria and hypermagnesiuria. This study was done to determine the therapeutic value and the side effects in the treatment of absorptive hypercalciuria with sodium cellulose phosphate and of hyperoxaluria with succinate.
J Urol 1978 Dec
PMID:Calcium oxalate stone disease: effects and side effects of cellulose phosphate and succinate in long-term treatment of absorptive hypercalciuria or hyperoxaluria. 73 12

In a child with renal failure and oliguria due to hyperoxaluria myelophthisis developed as a result of extensive bone-marrow replacement with calcium oxalate crystals and an accompanying fibrous proliferations. The histopathology associated with this metabolic disorder was demonstrated in posterior iliac crest bone-marrow trephine biopsies, renal biopsies, and nephrectomy specimens. Crystals were demonstrated in biopsy specimens of transplanted kidneys within six weeks following renal transplantation.
Am J Clin Pathol 1976 Dec
PMID:Oxalosis. An unusual cause of myelophthisis in childhood. 99 70

A woman had suffered from vulvar vestibulitis (vulvodynia) for four years. Pain from the disorder had disrupted her ability to function at work and home as well as sexually. An initial full range of treatments, including multiple operations, had produced no relief. Examination of the urine for evidence of excess oxalate, which has been shown to cause epithelial reactions similar to those found in vulvodynia, showed periodic hyperoxaluria and pH elevations related to the symptoms. Calcium citrate was given to modify the oxalate crystalluria. The symptoms were significantly reduced in three months, and the patient was pain free after one year. She was able to resume normal work, family, sexual and recreational activities. Withdrawal of the calcium citrate resulted in a return of the symptoms; reinstitution alleviated them. These findings suggest that further study of individualized metabolic factors that may underlie vulvodynia is warranted.
J Reprod Med 1991 Dec
PMID:Calcium citrate for vulvar vestibulitis. A case report. 181

During the past several years there has been increasing interest in refunctionalizing patients who have undergone radical extirpative surgery for pelvic malignancies and patients with dysfunctional bladders. To accomplish this, intestinal segments have been successfully employed in a variety of configurations. Independent of their optimal urosurgical implementation these procedures are not without potential complications, a significant portion of which involve metabolic derangements. Besides first follow-up results of patients with bladder substitution or continent urinary diversion, analysis of experimental investigations and functionally comparable clinical conditions enables an insight into potential following physiopathological interrelationships. These concern, besides the problem of chronic metabolic acidosis, disorders of bile acid and vitamin B12 metabolism as well as the potential induction of a secondary hyperoxaluria with subsequent oxalate concrement diathesis. Furthermore, there may be a malabsorption of calcium and vitamin D with development of intestinal osteopathy due to the reduction of absorptive surface. Apart from these problems of enteral loss and deficiency manifestations, several case reports and investigations suggest that bone demineralization can occur as a consequence of chronic metabolic acidosis and patients are at risk of skeletal demineralization. The pathogenesis of this association has yet to be clarified. These physiopathological interrelationships must be considered in medical attendance of patients with intestinal substitute bladders and continent supravesical pouch systems over many years. As these procedures become more popular, it becomes important to identify any metabolic changes that may occur as their consequence.
Prog Urol 1991 Dec
PMID:[Bladder replacement and continent diversion: what about metabolic complications?]. 184 45

No information exists in the literature about the optimal time for metabolic evaluation of stone patients in relation to extracorporeal shock wave lithotripsy (ESWL) treatment. It is uncertain whether the presence of a stone, ESWL treatment itself or subsequent colic episodes influence the urinary risk factors. A prospective study was performed to determine the optimal period for metabolic evaluation. Two 24-hour urine samples were collected directly before, and 1 week, 1 month and 3 months after therapy in an outpatient setting and tested for total volume, calcium, uric acid, oxalate, citrate and creatinine levels. A total of 66 patients was available for evaluation. Comparison of the 4 subsequent collecting periods showed no statistically significant differences in the excretion values. Also, in subgroups of patients with colic (16%), on a calcium oxalate restricted diet (12%) and with repeated treatments within 3 months (33%) no differences were noted. This means that the presence of a stone, treatment itself or subsequent colic episodes have no adverse effect on the urinary risk factors. For practical reasons metabolic evaluation directly before ESWL treatment seems most attractive. In the pre-ESWL samples hypercalciuria (greater than 7.5 mmol./24 hours), hyperuricosuria (greater than 6 mmol./24 hours), hyperoxaluria (greater than 0.5 mmol./24 hours) and hypocitraturia (less than 2 mmol./24 hours) were found in 31%, 12%, 18% and 27%, respectively, of the patients. It is concluded that metabolic evaluation before ESWL is practical, applicable and reliable.
J Urol 1991 Dec
PMID:Metabolic evaluation in stone patients in relation to extracorporeal shock wave lithotripsy treatment. 194 22

In approximately one-third of primary hyperoxaluria type 1 patients, disease is associated with a unique protein sorting defect in which hepatic L-alanine:glyoxylate aminotransferase (AGT; EC 2.6.1.44), which is normally peroxisomal, is mistargeted to mitochondria. In all such patients analyzed to date, the gene encoding the aberrantly targeted AGT carries three point mutations, each of which specifies an amino acid substitution. In this paper we show that one of these substitutions, a proline-to-leucine at residue 11, is necessary and sufficient for the generation of a mitochondrial targeting sequence in the AGT protein. AGT with this substitution appears to interact specifically with the mitochondrial protein import machinery, via a discrete N-terminal domain of the AGT protein. The N-terminal 19 amino acids of AGT with this substitution are sufficient to direct mouse cytosolic dihydrofolate reductase to mitochondria, and a synthetic peptide corresponding to this same 19-amino acid region reversibly inhibits mitochondrial protein import, not only of AGT but also of ornithine transcarbamoylase, a genuine cytoplasmically synthesized mitochondrial protein. We have extended these studies to analyze a region of normal human AGT cDNA directly upstream of the coding region. This sequence appears to correspond to an ancestral mitochondrial targeting sequence deleted from the human coding region by point mutation at the initiation codon. We show that reestablishment of this initiation codon produces an active mitochondrial targeting sequence that is different to that found in the hyperoxaluria patients. These results are discussed with reference to the AGT targeting defect in primary hyperoxaluria and also in relation to the highly unusual species specificity of subcellular distribution of AGT among mammals.
Proc Natl Acad Sci U S A 1991 Dec 01
PMID:Mistargeting of peroxisomal L-alanine:glyoxylate aminotransferase to mitochondria in primary hyperoxaluria patients depends upon activation of a cryptic mitochondrial targeting sequence by a point mutation. 196 59

Nephrolithiasis is a heterogeneous disorder, with varying chemical composition and pathophysiologic background. Although kidney stones are generally composed of calcium oxalate or calcium phosphate, they may also consist of uric acid, magnesium-ammonium phosphate, or cystine. Stones develop from a wide variety of metabolic or environmental disturbances, including varying forms of hypercalciuria, hypocitraturia, undue urinary acidity, hyperuricosuria, hyperoxaluria, infection with urease-producing organisms, and cystinuria. The cause of stone formation may be ascertained in most patients using the reliable diagnostic protocols that are available for the identification of these disturbances. Effective medical treatments, capable of correcting underlying derangements, have been formulated. They include sodium cellulose phosphate, thiazide, and orthophosphate for hypercalciuric nephrolithiasis; potassium citrate for hypocitraturic calcium nephrolithiasis; acetohydroxamic acid for infection stones; and D-penicillamine and alpha-mercaptopropionylglycine for cystinuria. Using these treatments, new stone formation can now be prevented in most patients.
Am J Kidney Dis 1991 Dec
PMID:Etiology and treatment of urolithiasis. 196 46

Oxalate is a major component of renal stones and an important determinant of calcium oxalate solubility in urine. Although the well-defined hyperoxaluric states are relatively uncommon, a significant number of patients with calcium oxalate stones have some degree of hyperoxaluria. For these reasons an understanding of both the causes of hyperoxaluria and methods of controlling oxalate synthesis and excretion is important. This review focuses on methods for the measurement of oxalate, the metabolic pathways of oxalate synthesis, the transport and excretion of oxalate, and the hyperoxaluric syndromes.
Endocrinol Metab Clin North Am 1990 Dec
PMID:The hyperoxaluric syndromes. 208 15


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