This study has been important in establishing synthetic human parathyroid hormone 1-34 (PTH)
as a beneficial treatment for hypoparathyroidism, superior to conventional therapy with
calcium and calcitriol. Providing synthetic human parathyroid hormone 1-34 (PTH) to patients
who are unresponsive to conventional therapy has enabled severe cases of hypoparathyroidism
to be managed effectively with the investigational drug, PTH. The primary goals of this
study are to (1) provide long-term PTH therapy to patients who do not respond to
conventional therapy; (2) understand the long-term effect of therapeutic PTH replacement on
kidney function and bone mineral density; (3) study and track linear growth and bone accrual
in children with hypoparathyroidism. (4) determine if subjects reach a normal level of peak
bone mass and if the timing of this is comparable to normal age-matched healthy controls.
Vitamin D and its analogs, the conventional treatment for hypoparathyroidism, are associated
with chronic hypercalciuria due to their lack of calcium-retaining effect in the kidney.
This side effect usually occurs even while maintaining the serum calcium in the normal range
and may lead to calcium deposition in the kidney (nephrocalcinosis) and renal insufficiency.
This study examines the long-term effects of subcutaneous parathyroid hormone (PTH) therapy
on calcium metabolism, bone, and renal function. Our previous short-term pilot study
comparing subcutaneous PTH with calcitriol demonstrated a significant decrease in urinary
calcium excretion during PTH therapy. Based upon these results, we hypothesized that
treatment with PTH is more physiologic and provides improved long-term metabolic control.
Additionally, treatment with PTH may avoid the adverse side effects on the kidney that are
associated with conventional therapy. Patients initially come to the Clinical Center for a
two week inpatient evaluation. Subsequent follow-up will occur semiannually on an outpatient
- INCLUSION CRITERIA:
This study will include patients (ages 4-70) with biochemically confirmed
Women who are pregnant will be excluded.