X linked hypophosphataemia: treatment, height gain, and nephrocalcinosis
Article Abstract:
Familial hypophosphatemia, an inherited disease characterized by low blood phosphate levels, is a vitamin D-resistant type of rickets. Features include abnormal bone structure related to altered calcium metabolism, increased urinary phosphate levels, normal blood calcium levels, and growth retardation. Hypophosphatemia is caused by poor kidney and intestinal handling of phosphate and low blood levels of a vitamin D metabolite (1,25-DHVD). Usually, the disorder is treated with high doses of vitamin D or 1,25-DHVD and phosphate supplements, but the benefit of this therapy has recently been questioned. Serious adverse effects associated with the therapy, including loss of bone mass and kidney calcium deposits, have been reported. The effect of vitamin D and phosphate treatment on 18 patients with familial hypophosphatemia was evaluated and compared with 16 untreated adult hypophosphatemic relatives. The average patient age at start of treatment was 5.8 years and the average length of treatment was 8.4 years. Although there was no difference in the final heights between treated and untreated patients, the height scores of the treated group increased significantly during treatment. It is possible that untreated family members had milder disease. Six of the treated patients had kidney calcification detectable by ultrasound imaging, but no functional signs of kidney damage; these patients had received significantly higher doses of oral phosphates. They also had higher urinary phosphate output and more frequent episodes of hypercalciuria, high urinary calcium. Kidney calcification probably occurred because in the presence of continuous high urinary phosphate, an episode of hypercalciuria will cause calcium precipitation. A second salt-calcium interaction is also possible. The results suggest that continued treatment of patients with hypophosphatemia with vitamin D and lower doses of phosphate is beneficial. These patients should maintain extensive fluid intake to enhance urinary dilution and prevent calcification. However, further research on the side effects and growth effects of the therapy is needed. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Archives of Disease in Childhood
Subject: Health
ISSN: 0003-9888
Year: 1990
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Cisplatin-based chemotherapy in a renal transplant recipient
Article Abstract:
Each year about 7,000 people in the US receive transplanted kidneys. Most likely because of the immunosuppression required for organ transplantation, kidney transplant recipients are about 100 times more likely to develop cancer than individuals of the same age in the general population. However, it is uncertain whether the conventional chemotherapeutic treatments are safe for kidney transplant recipients. Cisplatin is one of the most effective and most widely used drugs for treating solid tumors, but kidney toxicity is one of the most significant toxic side effects of this drug. The authors report the case of a 33-year-old male transplant recipient who was successfully treated with cisplatin chemotherapy. The patient developed a testicular mass two and one half years after receiving a transplanted cadaveric kidney. The left testis was removed, and pathological examination revealed it to contain a seminoma, a cancer of the germ cells. Enlarged lymph nodes were observed on CT scan, indicating that the cancerous cells had begun to spread. The patient was treated with etoposide and cisplatin. Creatinine in the blood serum was measured to monitor kidney function during the patient's chemotherapy, but little change was noted. The major side effect of chemotherapy in this patient was a depression of neutrophils and platelets. CT scan documented the regression of the affected lymph nodes, but the patient developed recurrences affecting the chest and the pelvis, indicating that chemotherapy was not completely effective. Radiation therapy was used, and the patient is currently alive without evidence of disease three years after the initial diagnosis. This case illustrates that kidney transplant recipients may be given cisplatin chemotherapy if it is required, and that the standard precautions given to any patient receiving cisplatin are appropriate. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Cancer
Subject: Health
ISSN: 0008-543X
Year: 1991
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