Pediatric Hypoparathyroidism Treatment & Management

  • Author: Pisit (Duke) Pitukcheewanont, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Aug 05, 2015
 

Medical Care

 

Symptomatic hypocalcemia (eg, seizure, tetany, laryngospasm) in patients with hypoparathyroidism requires intravenous calcium and continuous monitoring for cardiac arrhythmias.

Oral calcium and active vitamin D (calcitriol) should be initiated as soon as possible (eg, when the patient is tolerating oral feeds).

Once serum calcium concentrations are in a safe range (>7.5 mg/dL), intravenous calcium can be stopped. Prolonged intravenous calcium therapy with existing high serum phosphate will increase further precipitation of calcium-phosphate compound. However, rebound hypocalcemia can occur and requires that a patient be monitored for therapeutic success on oral agents for at least 24 hours after intravenous calcium is withdrawn. In patients with hungry bone syndrome, prolonged intravenous calcium therapy may be needed.

The active form of vitamin D, 1,25-dihydroxyvitamin D (calcitriol), is preferred in the treatment of hypoparathyroidism because both the parathyroid hormone (PTH) deficiency/resistance and the hyperphosphatemia impair the activation of 25-hydroxyvitamin D by 1-alpha-hydroxylase.

General recent guidelines on chronic hypoparathyroidism by the European Society of Endocrinology are below:[5]

  • Consider a diagnosis of chronic hypoparathyroidism (HypoPT) in a patient with hypocalcemia and inappropriately low parathyroid hormone (PTH) levels.
  • Consider genetic testing and/or family screening in a patient with HypoPT of unknown etiology.
  • Treatment targeted to maintain serum calcium level (albumin adjusted total calcium or ionized calcium) in the lower part or slightly below the lower limit of the reference range (target range) is suggested, with patients being free of symptoms or signs of hypocalcemia.
  • Treat patients with chronic HypoPT with symptoms of hypocalcemia and/or an albumin adjusted serum calcium level <2.0 mmol/L (<8.0 mg/dL/ionized serum calcium levels [S-Ca 2] <1.00 mmol/L).
  • Offer treatment to asymptomatic patients with chronic HypoPT and an albumin adjusted calcium level between 2.0 mmol/L (8.0 mg/dL/S-Ca 2+ 1.00 mmol/L) and the lower limit of the reference range in order to assess whether this may improve their well-being.
  • Use activated vitamin D analogues plus calcium supplements in divided doses as the primary therapy.
  • If activated vitamin D analogues are not available, treat with calciferol (preferentially cholecalciferol).
  • Titrate activated vitamin D analogues or cholecalciferol in such a manner that patients are without symptoms of hypocalcaemia and serum calcium levels are maintained within the target range.
  • Provide vitamin D supplementations in a daily dose of 400–800 IU to patients treated with activated vitamin D analogues.
  • In a patient with hypercalciuria, consider a reduction in calcium intake, a sodium-restricted diet, and/or treatment with a thiazide diuretic.
  • In a patient with renal stones, evaluate renal stone risk factors and management according to relevant international guidelines.
  • In a patient with hyperphosphatemia and/or an elevated calcium-phosphate product, consider dietary interventions and/or adjustment of treatment with calcium and vitamin D analogues.
  • In a patient with hypomagnesemia, consider measures that may increase serum magnesium levels.
  • The routine use of replacement therapy with PTH or PTH analogues is not recommended.
Next

Diet

No special diet is required, but adequate calcium and vitamin D intake is recommended.

Previous
 
 
Contributor Information and Disclosures
Author

Pisit (Duke) Pitukcheewanont, MD Associate Professor of Clinical Pediatrics, University of Southern California, Keck School of Medicine, Childrens Hospital Los Angeles

Pisit (Duke) Pitukcheewanont, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, American Society for Bone and Mineral Research, Endocrine Society, Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, American College of Endocrinology

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

References
  1. Roth KS, Ward RJ, Chan JCM, Sarafoglou K. Disorders of calcium, phosphate, and bone metabolism. Sarafoglou K. Hoffman GF, Roth KS. Pediatric endocrinology and inborn errors of metabolism. 1st ed. New York, NY: McGraw Hill; 2009. 619-64.

  2. Baumber L, Tufarelli C, Patel S, King P, Johnson CA, Maher ER, et al. Identification of a novel mutation disrupting the DNA binding activity of GCM2 in autosomal recessive familial isolated hypoparathyroidism. J Med Genet. 2005 May. 42(5):443-8. [Medline]. [Full Text].

  3. Mannstadt M, Bertrand G, Muresan M, Weryha G, Leheup B, Pulusani SR, et al. Dominant-negative GCMB mutations cause an autosomal dominant form of hypoparathyroidism. J Clin Endocrinol Metab. 2008 Sep. 93(9):3568-76. [Medline]. [Full Text].

  4. Shiohara M, Shiozawa R, Kurata K, Matsuura H, Arai F, Yasuda T. Effect of parathyroid hormone administration in a patient with severe hypoparathyroidism caused by gain-of-function mutation of calcium-sensing receptor. Endocr J. 2006 Dec. 53(6):797-802. [Medline].

  5. Bollerslev J, Rejnmark L, Marcocci C, Shoback DM, Sitges-Serra A, van Biesen W, et al. European Society of Endocrinology Clinical Guideline: Treatment of chronic hypoparathyroidism in adults. Eur J Endocrinol. 2015 Aug. 173 (2):G1-G20. [Medline].

  6. Winer KK, Sinaii N, Peterson D, Sainz B Jr, Cutler GB Jr. Effects of once versus twice-daily parathyroid hormone 1-34 therapy in children with hypoparathyroidism. J Clin Endocrinol Metab. 2008 Sep. 93(9):3389-95. [Medline].

  7. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical variation of autoimmune polyendocrinopathy-candidiasis- ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med. 1990 Jun 28. 322(26):1829-36. [Medline].

  8. Arnold A, Horst SA, Gardella TJ, et al. Mutation of the signal peptide-encoding region of the preproparathyroid hormone gene in familial isolated hypoparathyroidism. J Clin Invest. 1990 Oct. 86(4):1084-7. [Medline]. [Full Text].

  9. Chattopadhyay N, Mithal A, Brown EM. The calcium-sensing receptor: a window into the physiology and pathophysiology of mineral ion metabolism [published erratum appears in Endocr Rev 1996 Oct;17(5):517]. Endocr Rev. 1996 Aug. 17(4):289-307. [Medline]. [Full Text].

  10. Chinnery PF, Turnbull DM. Mitochondrial medicine. QJM. 1997 Nov. 90(11):657-67. [Medline]. [Full Text].

  11. Farfel Z, Bourne HR, Iiri T. The expanding spectrum of G protein diseases. N Engl J Med. 1999 Apr 1. 340(13):1012-20. [Medline].

  12. Fischer JA, Egert F, Werder E, Born W. An inherited mutation associated with functional deficiency of the alpha-subunit of the guanine nucleotide-binding protein Gs in pseudo- and pseudopseudohypoparathyroidism. J Clin Endocrinol Metab. 1998 Mar. 83(3):935-8. [Medline]. [Full Text].

  13. Hasegawa T, Hasegawa Y, Aso T, et al. HDR syndrome (hypoparathyroidism, sensorineural deafness, renal dysplasia) associated with del(10)(p13). Am J Med Genet. 1997 Dec 31. 73(4):416-8. [Medline].

  14. Hayward BE, Moran V, Strain L, Bonthron DT. Bidirectional imprinting of a single gene: GNAS1 encodes maternally, paternally, and biallelically derived proteins. Proc Natl Acad Sci U S A. 1998 Dec 22. 95(26):15475-80. [Medline]. [Full Text].

  15. Juppner H, Schipani E, Bastepe M, et al. The gene responsible for pseudohypoparathyroidism type Ib is paternally imprinted and maps in four unrelated kindreds to chromosome 20q13.3. Proc Natl Acad Sci U S A. 1998 Sep 29. 95(20):11798-803. [Medline]. [Full Text].

  16. Levine MA. Pseudohypoparathyroidism: from bedside to bench and back. J Bone Miner Res. 1999 Aug. 14(8):1255-60. [Medline].

  17. Nakamoto JM, Sandstrom AT, Brickman AS, et al. Pseudohypoparathyroidism type Ia from maternal but not paternal transmission of a Gsalpha gene mutation. Am J Med Genet. 1998 May 26. 77(4):261-7. [Medline].

  18. Pearce SH, Williamson C, Kifor O, et al. A familial syndrome of hypocalcemia with hypercalciuria due to mutations in the calcium-sensing receptor. N Engl J Med. 1996 Oct 10. 335(15):1115-22. [Medline].

  19. Perheentupa J. Autoimmune polyendocrinopathy--candidiasis--ectodermal dystrophy (APECED). Horm Metab Res. 1996 Jul. 28(7):353-6. [Medline].

  20. Pollak MR, Brown EM, Estep HL, et al. Autosomal dominant hypocalcaemia caused by a Ca(2+)-sensing receptor gene mutation. Nat Genet. 1994 Nov. 8(3):303-7. [Medline].

  21. Sticht H, Hashemolhosseini S. A common structural mechanism underlying GCMB mutations that cause hypoparathyroidism. Med Hypotheses. 2006 May 11. [Medline].

  22. Sunthornthepvarakul T, Churesigaew S, Ngowngarmratana S. A novel mutation of the signal peptide of the preproparathyroid hormone gene associated with autosomal recessive familial isolated hypoparathyroidism. J Clin Endocrinol Metab. 1999 Oct. 84(10):3792-6. [Medline]. [Full Text].

  23. Yamamoto M, Akatsu T, Nagase T, Ogata E. Comparison of hypocalcemic hypercalciuria between patients with idiopathic hypoparathyroidism and those with gain-of-function mutations in the calcium-sensing receptor: is it possible to differentiate the two disorders?. J Clin Endocrinol Metab. 2000 Dec. 85(12):4583-91. [Medline]. [Full Text].

 
Previous
Next
 
Electrocardiogram (ECG) findings in severe hypocalcemia.
 
Medscape Consult
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.