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Jcem3307.Pdf SPECIAL FEATURE Editorial Hypoparathyroidism: Is It Time for Replacement Therapy? Mara J. Horwitz and Andrew F. Stewart Division of Endocrinology and Metabolism, The University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213 ndocrine diseases caused by hormone deficiencies are com- iopathic, and autosomal dominant hypoparathyroidism are Downloaded from https://academic.oup.com/jcem/article/93/9/3307/2596495 by guest on 01 October 2021 E monly treated by replacement of the deficient hormones. the most common varieties. For example, Addison’s disease is treated with oral glucocorti- Why does PTH deficiency cause hypocalcemia? Although coids, autoimmune hypothyroidism with oral thyroxine, and bone turnover is dramatically reduced in hypoparathyroidism, it premature ovarian failure with oral estrogen and progesterone. does not play a major role in causing hypocalcemia in hypopar- The availability of orally deliverable drugs makes this straight- athyroidism. Instead, the hypocalcemia in hypoparathyroidism forward. However, when the deficient hormone in question is a results principally from two other mechanisms (5–9). First, the protein, oral treatment presents problems because proteins are loss of PTH results in a failure of renal 1,25 dihydroxyvitamin D generally degraded by proteolytic digestive enzymes when de- [1,25(OH)2D] production, with a resultant reduction in the abil- livered orally. This obstacle commonly is circumvented by sc ity to absorb dietary calcium. Second, PTH is a potent antical- injection of the peptide hormone in question (i.e. GH, insulin, ciuric agent in the distal convoluted tubule. Therefore, its loss 1-desamino-␤-D-arginine vasopressin). So what is different results in striking increases in renal calcium excretion. As an about hypoparathyroidism, which also results from deficiency of illustration, the extracellular fluid compartment contains ap- PTH, a protein hormone? Why is this disorder not routinely proximately 1,000 mg calcium; the glomerulus filters 10,000 treated by replacement of PTH? This is the subject of a series of mg/d, and the distal tubule reabsorbs 1,000 mg/d. PTH, through reports by Winer et al. (1–4), the most recent of which is included its anticalciuric actions on the distal convoluted tubule, can re- in this month’s Journal (4). duce renal calcium excretion to essentially zero. These numbers Hypoparathyroidism results from deficiency of PTH (5–9). quantify the critical role of the kidney, and the potent antical- This may be a congenital abnormality resulting in failure of ciuric effect of PTH. Thus, in pathophysiological mechanistic the parathyroid glands to form, as exemplified by the Di- terms, the loss of PTH causes hypocalcemia through an indirect George syndrome, or may result from inactivating mutations failure [through 1,25(OH)2D] of intestinal calcium absorption, in the parathyroid-specific transcription factor, glial-cell and a direct failure to prevent renal calcium losses. missing B. It may result from mutations in the PTH gene itself. The standard treatment of hypoparathyroidism attempts to A functional, congenital form of hypoparathyroidism results correct hypocalcemia with oral vitamin D analogs and calcium from activating mutations in the calcium-sensing receptor that rather than with PTH replacement (5, 6, 8, 9). Large doses of oral tricks the parathyroid gland into believing that the host is calcium are given to augment intestinal calcium absorption. This hypercalcemic and, therefore, induces suppression of PTH is accompanied by large doses of vitamin D [to mimic the intes- secretion (5, 6, 8, 9). This syndrome is called autosomal dom- tinal effects of the missing 1,25(OH)2D] or physiological doses inant hypoparathyroidism. Hypoparathyroidism may also be of 1,25(OH)2D itself. The combined goal is to forcibly drive an intentional or unintentional result of thyroid, parathyroid, calcium transport across the intestinal epithelium in quantities and laryngeal surgery. It may also result from autoimmune sufficient to overwhelm the ability of the kidney to clear this attack on the parathyroid glands as a part of the type 1 intestinally derived calcium load, forcing the serum calcium to polyglandular failure syndrome, or from the accumulation of increase. copper (Wilson’s disease), or iron (hemochromatosis) in para- Although this is standard therapy, it is a slippery slope. Un- thyroid cells, and/or their destruction by metastatic cancer, or dertreatment or missed doses may result in persistent muscle invading sarcoid granulomas. Postsurgical, autoimmune/id- cramps, paresthesias, and seizures. A much more worrisome 0021-972X/08/$15.00/0 Abbreviation: 1,25(OH)2D, 1,25 dihydroxyvitamin D. Printed in U.S.A. Copyright © 2008 by The Endocrine Society doi: 10.1210/jc.2008-1216 Received June 4, 2008. Accepted July 15, 2008. For article see page 3389 J Clin Endocrinol Metab, September 2008, 93(9):3307–3309 jcem.endojournals.org 3307 3308 Horwitz and Stewart Hypoparathyroidism J Clin Endocrinol Metab, September 2008, 93(9):3307–3309 problem is overtreatment, which results in hypercalciuria, hy- the circulation (10); it seems unlikely that a single daily dose percalcemia, nephrolithiasis, nephrocalcinosis, and renal failure, would correct the mineral metabolic abnormalities 24 h/d. a regrettably common and severe adverse outcome in subjects This landscape has changed with the widespread availability with hypoparathyroidism (1–9). On reflection, this is not sur- since 2002 of injectable PTH(1–34) for the treatment of osteo- prising. First, this therapeutic model is a tightrope walk, attempt- porosis (11). Because PTH is now a drug, is available in a con- ing to precisely balance forced intestinal calcium overabsorption veniently used pen, and because the pain of injection is minimal, with unavoidable, indeed intentional, renal calcium overexcre- it is possible and reasonable to ask whether PTH might be as tion. However, this is not a steady state. If the patient decides to effective as standard vitamin D and calcium therapy. One obvi- ingest extra dietary calcium or if the subject becomes dehydrated, ous advantage might be that PTH, with its anticalciuric efficacy, doses of calcium and vitamin D that had permitted a steady state might permit correction of serum calcium without inducing hy- of serum calcium with a normal glomerular filtration rate now percalciuria, nephrocalcinosis, or nephrolithiasis, an outcome cause mild to severe hypercalcemia. Thiazide diuretics, which that would be particularly attractive in patients with autosomal Downloaded from https://academic.oup.com/jcem/article/93/9/3307/2596495 by guest on 01 October 2021 stimulate calcium reabsorption at the distal convoluted tubule dominant hypoparathyroidism. and are, therefore, also anticalciuric, are commonly used as an Collectively, the prior studies of the National Institutes of addendum to therapy in hypoparathyroidism for these reasons. Health group (1–3) highlight the precarious renal status of these However, they simply shift the tightrope to a new location, with- patients treated conventionally: 80% had reductions of glomer- out changing its fragility. ular filtration rate after years of conventional treatment with Another reason for poor outcomes in hypoparathyroidism is vitamin D and calcium, and 40% had nephrocalcinosis. Winer that the therapeutic goal is often mistakenly believed to be et al. (1–3) have shown that PTH can be effectively and safely achievement of a normal serum calcium, in the 9.0–10.5 mg/dl administered to adults with several different types of hypopar- (2.25–2.63 mM) range. However, if the serum calcium is driven athyroidism, and that this treatment compares very favorably to up to this degree by large doses of calcium and vitamin D, in the conventional therapy with calcium and vitamin D analogs. The absence of PTH, massive and chronic hypercalciuria will occur, serum calcium concentration can be maintained at slightly but and this may lead to nephrolithiasis, nephrocalcinosis, and renal statistically significantly higher levels, yet the PTH therapy re- failure. Thus, the therapeutic goal is not to achieve eucalcemia sults in lower urine calcium excretion than with conventional but, instead, to provide the minimal amount of calcium and therapy, as one might have hoped with the use of PTH. This is vitamin D required to achieve symptom relief without causing particularly apparent in the group with autosomal dominant hypercalciuria. This means that routine 24-h urinary calcium hypoparathyroidism. Given the brief half-life of PTH(1–34) measurements are de rigueur in the management of these patients (11), it is not surprising that in most of the studies, administra- and that the serum calcium target should be in the range of tion of PTH every 12 h results in superior (more stable) serum 8.0–9.0 mg/dl (2.00–2.25 mM), while maintaining a normal calcium profiles than once-daily treatment. The dose of PTH in 24-h urine calcium excretion. There is one subset of patients with these studies (25–75 ␮g/d) is slightly higher than that used ther- hypoparathyroidism who are particularly prone to hypercalci- apeutically for the treatment of osteoporosis. This may reflect the uria: those with autosomal dominant hypoparathyroidism (5, 6, lack of endogenous PTH in these subjects, the requirement of 8, 9). This is because at baseline, before treatment, the consti- multiple dosing, or other factors. As anticipated, bone formation tutively active calcium receptor in the kidney activates
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