Child nephrology and urology最新文献

A 1.5-year-old Saudi girl with hemolytic uremic syndrome is described. She developed hyperglycemia in the acute stage which required insulin therapy. After a short remission, she developed permanent insulin-dependent diabetes mellitus. Review of the literature of the occurrence and the pathophysiology of this phenomenon is presented.

We report on a 7-week-old infant with idiopathic hypercalcemia, hypercalciuria and nephrocalcinosis. At the time of admission, serum concentrations of parathyroid hormone and 1,25(OH)2D3 were found to be inadequately high, and those of calcitonin and 24,25(OH)2D3 too low, relative to the hypercalcemia. Treatment with calcitonin normalized serum calcium concentrations within 4 days, and a 3-week course of thiazides combined with a decreased dietary calcium:phosphorus ratio corrected the hypercalciuria. A repeat profile of the calcium-regulating hormones done at the age of 5.5 months was normal. Based on the clinical course and the hormonal profiles, we hypothesize that the idiopathic infantile hypercalcemia in this patient could have resulted from a generalized maturational delay of calcium homeostasis. Treatment with calcitonin, therefore, seems to be the most appropriate way to control the hypercalcemia.

The fourth component of complement (C4), especially B isotype, has been said to be deficient in the IgA nephropathy and Henoch-Schönlein nephritis. However, the association between these diseases and C4 deficiency was questioned recently, and the usual C4 allotyping method is unable to discriminate the C4 deficiency from the C4 duplication. So by combining the DNA restriction fragment length polymorphism with the usual C4 allotyping, we tried to determine whether the deficiency of C4 can be demonstrated in the DNA level. We found that the frequency of C4 gene deletion was increased, although the frequency of null phenotype was not different from the control. From these results we can say that C4 gene deletion is a genetic risk factor in these diseases, at least in the Japanese population.

Results of renal transplantation in younger children have not been very encouraging in the past. We therefore studied the effect of newer immunosuppressive regimens on the outcome of renal transplantation of 5 children aged 2.9 +/- 1.3 years (range 1.6-5.0), and compared it to 10 children of an older pediatric patient group aged 11.4 +/- 4.4 years (range 6.0-18.5). All patients with the exception of 1 underwent dialysis. The percentage of cadaveric and live-related transplants was similar in both groups. Recipients of a cadaveric transplant had at least 3 blood transfusions; recipients of live-related transplants had donor-specific transfusions with azathioprine. Posttransplantation immunosuppression consisted of prednisone and azathioprine; recipients of cadaveric transplants received also ciclosporin. Rejection episodes and side effects (hypertension, hirsutism) were comparable in both groups. In the younger patient group, 1 patient died of a congenital lung abnormality but had a functioning graft. In the older patient group, 1 patient lost his graft 16 months posttransplantation due to reduction of his immunosuppressives, necessitated by a severe CMV infection. Growth and development improved in the younger patient group, but was stable in older patients. Renal transplantation is a suitable option in younger pediatric patients. Graft survival rates are comparable to those of older patients.