Bleyer AJ, de la Torre E, Igwemezie B, White W
Description of a distinct variant of calciphylaxis
Am Soc Nephrol
J Am Soc Nephrol (abstract)
(Sep) 7:1787 1996
Calciphylaxis is a serious, usually fatal disorder which appears to be
increasing in frequency. Since few physicians see more than a case or
two a year it is difficult to develop any clear picture or strategy
about this entity. Originally thought to be a consequence of severe
hyperparathyroidism with a very high calcium x phosphate product, we are
now seeing more and more cases with low PTH levels. Some cases are even
observed following transplantation. Clearly, more information is needed
about this terrible disorder and some kind of cooperative study should
be designed to assess etiology and begin to develop treatment.
The authors describe 6 cases with relatively low PTH levels (as they
point out, the patients' PTH levels were actually in the suggested
ideal range for dialysis patients). The patients were quite obese and
Caucasian and had central ulcers (on the proximal thighs or pannus).
They had acceptable calcium and phosphate levels. They tended to be a
bit younger and had been on dialysis a shorter duration than the rest of
the patients in their program. Parathyroidectomy did not alter the
outcome. Three patients have died and the rest continue with their
lesions. The authors suggest that obesity may be a major factor in
these patients' central calciphylaxis and that this may be somewhat
different than the usual form appearing more distally.
Comment: This is an interesting finding which should be pursued.
One question
that must be asked is how obesity might be involved. It would be
important to know whether they had microvascular disease, possibly due
in part to hyperlipidemia. A point they observed but did not comment on
is the failure of parathyroidectomy. It is extremely unlikely that
patients with PTH levels below 400 would have any benefit from this
surgery and it should not be done in calciphylaxis patients who have
such low values.
(Donald Sherrard, M.D., University of Washington)
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