Renal transplantation in a patient with methylmalonic acidaemia.

Renal insufficiency is frequently reported in mutase-deficient methylmalonic acidaemia. We present a case report of a patient with mut- methylmalonic acidaemia who developed chronic tubulointerstitial nephropathy during adolescence. At 24 years of age, she developed end-stage renal failure and underwent renal transplantation. Both plasma and urine methylmalonic acid levels decreased significantly with improved renal function following transplantation. Complications included cyclosporin toxicity and development of diabetes. Renal, metabolic, and clinical status remained improved at 3 years after the kidney transplant.

The feasibility of consortia for internal medicine graduate medical education: perspectives from a survey of residency directors.

PURPOSE: To assess the perspectives of internal medicine (IM) residency directors on issues that might determine the feasibility of consortia for IM graduate medical education (GME). METHOD: A self-administered questionnaire was mailed to all 413 U.S. IM program directors in June 1994. Of the 413 IM programs, 215 were located in community hospitals; 123 in university hospitals; and 75 in municipal, Veterans Administration, or military hospitals, or hospitals associated with multispecialty clinics ("other"). The questionnaire elicited responses concerning (1) perspectives on the quality of academic affiliations, (2) experience with formal institutional collaboration on GME issues and projection of consortium success, and (3) possible barriers to the success of consortia. Data were analyzed by comparing responses from the three program categories. RESULTS: In all, 330 (80%) program directors responded. Two-thirds reported ongoing academic affiliations. A larger percentage of university program directors considered these ties to be strong and advantageous than did their colleagues in community or other hospitals, who also considered their affiliations to be less equitable and less mutually trusting. Only 31% of community and university programs and 40% of other programs reported any prior experience with institutional collaboration on GME issues. A high percentage of those respondents considered these collaborative experiences to be successful and were optimistic about the projected success of consortia. Of seven possible barriers to consortium success, competition, governance, bureaucracy, and mistrust were most often perceived as major barriers. CONCLUSION: The data appear to indicate some optimism for the prospects of GME consortia, thereby supporting their feasibility for IM GME. Although many respondents perceived barriers to success, such perceptions were less common among program directors who had had direct experience with previous collaborative efforts. Nevertheless, these barriers may require attention if consortia are to succeed in achieving their many possible advantages.

Self-hemodialysis. The optimal mode of dialytic therapy.

Maintenance hemodialysis is presently the mainstay of treatment for the majority of patients with end-stage renal disease. There has been disagreement, however, as to what form the delivery of dialysis should take-self-dialysis, at home, or in-center, as opposed to in-center, limited-care dialysis. This review of the recent literature strongly supports self-dialysis as the optimal form of therapy, since the cost is less, and survival and rehabilation are better than with limited-care dialysis. We conclude that a greater effort should be expended to encourage and even direct patients toward this form of therapy.

Renal urate excretion in normal man.

The development of our knowledge of the intrarenal process involved in the control of urate excretion in normal man is summarized. Although there are many gaps in our current knowledge, and different interpretations may be given to the available data, current evidence seems to favor the existence of extensive tubular reabsorption of urate following its glomerular filtration. Subsequently, tubular secretion of urate and the reabsorption of an unknown amount of the secreted urate probably take place. For reasons discussed, it seems most likely that the regulation and control of urate excretion are accomplished through modulations in tubular secretion, post-secretory reabsorption, or both.

Intrinsic renal disease leading to abnormal urate excretion.

Since approximately two thirds of daily urate production is normally excreted by the kidney, intrinsic renal disease resulting in abnormalities of urate excretion may have a profound effect upon urate homeostasis. Alterations in the pattern of urate excretion encountered in chronic renal failure are reviewed in depth, with a description of adaptive mechanisms for urate excretion which develop in residual nephrons, as exemplified by the remaining normal kidney of transplant donors. In addition, abnormalities in urate excretion in the presence of a normal complement of nephrons are described. Diminished urate excretion per nephron appears to be responsible for hyperuricemia in some patients with gout, while a variety of tubular defects resulting in excessive renal urate excretion have been documented as the basis for some cases of hypouricemia.