Hypophosphatemic Rickets Medication
- Author: James CM Chan, MD; Chief Editor: Stephen Kemp, MD, PhD more...
Treatment options include calcitriol, GH, phosphates, and anticalciurics to promote healthy bone growth and diminish mineral loss associated with hypophosphatemic rickets. As previously stated, acute hypercalcemia (with resulting irritability, confusion, and potential seizures) can occur during treatment. Nephrocalcinosis, the long-term result of overaggressive therapy, may be more damaging.
Unless a concomitant GH deficiency is observed, administration of biosynthetic GH for growth promotion has not been approved, unless GH deficiency is observed. Only preliminary evidence of improved final height with GH therapy has been reported.
Standard protocol for treatment of familial hypophosphatemic rickets includes the use of 1,25-dihydroxy-vitamin D (calcitriol). The use of calcitriol in place of standard vitamin D obviates near-toxic dosage of the latter, avoids fat storage of parent vitamin D, and diminishes the danger of hypercalcemia.
Calcitriol increases calcium levels by promoting calcium absorption in the intestines and retention in kidneys.
Massive urinary phosphate loss is a problem intrinsic to the disorder, and the phosphate must be replaced.
This is a neutralized, buffered, oral phosphate-replacement solution containing 250 mg phosphorus, 280 mg potassium, 160 mg sodium . It is a combination of sodium and potassium phosphate.
Thiazides are anticalciuric, an effect that can assist in counteracting the tendency for bone calcium loss.
Hydrochlorothiazide is a well-known diuretic with antihypertensive action. It inhibits reabsorption of sodium in distal tubules, causing increased excretion of sodium and water, as well as of potassium and hydrogen ions. Hydrochlorothiazide is not metabolized and is rapidly excreted in the urine.
Hypokalemia is a hazard when kaliuretic-effect thiazides are used; this danger that can be counteracted with the use of a second diuretic. Amiloride has a well-characterized antikaliuretic effect. Often used together with thiazides for its synergistic antihypertensive effects, amiloride has the benefit of decreasing potassium loss. Thus, it is a useful adjunct in the treatment of patients with familial X-linked hypophosphatemia with thiazides, in whom hypokalemia is a risk.
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