Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0028961 (oliguria)
 1,847 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A successful live related renal transplant in a 29-year-old male patient with Type 1 primary hyperoxaluria, who remains well 32 months postoperatively, is described. The plasma oxalate and exchangeable oxalate pool before transplantation were 160 mumol/1 and 4429 mumol respectively. Since the transplant these have been greatly reduced although they remain elevated above the normal by a factor of 2. Pyridoxine therapy and the avoidance of oxalate-rich foods have been effective in maintaining these reduced levels and the 24-hr urinary oxalate excretion has also been maintained close to normal levels on this regime. After review of the previously reported transplants in patients with well documented primary hyperoxaluria and from the experience with this patient, the following guidelines for successful renal transplantation in primary hyperoxaluria are suggested: transplants should only be carried out in those who have shown a response to adequate pyrodoxine therapy; frequent haemodialysis pre-operatively and during periods of oliguria postoperatively is necessary; oxalate-rich foods should be avoided and a high fluid intake should be maintained after transplantation. If these guidelines are followed there is no contra-indicatin to live related renal transplants in primary hyperoxaluric patients.
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PMID:Successful renal transplantation in primary hyperoxaluria. 700 21

A 63-year-old woman who had received hemodialysis therapy since she fell acute on chronic renal failure 4 years ago presented with multiple joint pain. Nephrocalcinosis was not detected by abdominal X-ray when hemodialysis therapy was initiated. Laboratory testing showed azotemia, anemia, hypoproteinemia and mild liver dysfunction but no liver cirrhosis. Biopsied bone tissue demonstrated numerous calcium oxalate crystal depositions. Laparoscopy revealed black liver in macroscopic view. Histological studies showed numerous lipofuscin-like dark brown granules were deposited in hepatocytes. The activity of alanine : glyoxylate aminotransferase (AGT) was less than 0.1 U/g in biopsied patient's liver tissue. Generally, clinical symptoms demonstrated by Japanese primary hyperoxaluria type I (PH-I) patients are milder than those of European patients. Some PH-I patients may successfully avoid urinary tract calcification unless they fall into oliguria by some other causes. The lipofuscin granules are most likely the source of the dark color. Massive deposition of the lipofuscin granules indicated that the duration of the liver metabolic abnormality had lasted for long time. Thus, black liver may be related to a mild form of PH-I.
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PMID:A case of late onset primary hyperoxaluria type I (PH-I) presented with black liver. 977 23

The infant (a girl aged 6 months) was admitted to the hospital because of oliguria and acute renal dysfunction. The laboratory examination results showed serious metabolic acidosis and increased blood urea nitrogen and serum creatinine levels. The patient continued to be anuric after 10 days of treatment with continuous renal replacement therapy (CRRT). she died a day later. The family history showed that the patient's sister died of acute renal failure 6 months after birth. The genomic sequencing results showed AGXT mutation in the patient and confirmed the diagnosis of primary hyperoxaluria type 1 (PH1). Her parents were heterozygous carriers. PH1 should be considered when the children have abnormal renal function or recurrent renal calculi or have a family history of these symptoms. AGXT gene analysis is an important method for PH1 diagnosis.
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PMID:[Oliguria and acute renal dysfunction in a six-month-old infant]. 2820 21