Pseudohypoparathyroidism natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
Patients with type Ia pseudohypoparathyroidism have an increased rate of other endocrine abnormalities (such as [[hypothyroidism (patient information)|hypothyroidism]] and [[hypogonadism (patient information)|hypogonadism]]). Complications of [[hypocalcemia]] associated with pseudohypoparathyroidism may include [[seizures]] and other endocrine problems, leading to decreased sexual drive and delayed sexual development, lowered energy levels, and increased weight.
Patients with type Ia pseudohypoparathyroidism have an increased rate of other endocrine abnormalities (such as [[hypothyroidism (patient information)|hypothyroidism]] and [[hypogonadism (patient information)|hypogonadism]]). Complications of [[hypocalcemia]] associated with pseudohypoparathyroidism may include [[seizures]] and other endocrine problems, leading to decreased sexual drive and delayed sexual development, fatigue, and increased weight.


==Natural History==
==Natural History==

Revision as of 14:06, 2 October 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]


Overview

Patients with type Ia pseudohypoparathyroidism have an increased rate of other endocrine abnormalities (such as hypothyroidism and hypogonadism). Complications of hypocalcemia associated with pseudohypoparathyroidism may include seizures and other endocrine problems, leading to decreased sexual drive and delayed sexual development, fatigue, and increased weight.

Natural History

Pseudohypoparathyroidism type 1b patients at an increased risk of developing hyperparathyroidism and hyperparathyroid bone disease as a result of long term elevation in parathyroid hormone.

Complications

Complications that can develop as a result of pseudohypoparathyroidism are

  • Complications of hypocalcemia associated with pseudohypoparathyroidism may include seizures and other endocrine problems, leading to lowered sexual drive and lowered sexual development, lowered energy levels, and increased weight.
  • Subcutaneous calcification in neonatal period.[2]

Prognosis

Insufficient data is available to determine the long term outcomes of pseudohypoparathyroidism. In some patients calcium homeostasis adapts to parathyroid hormone resistance resulting in resolution of hypocalcemia while others who do not adapt to parathyroid hormone resistance are managed with lifelong calcium supplementation. Long term levothyroxine is used in patients with associated hypothyroidism.

References

  1. Shalitin S, Davidovits M, Lazar L, Weintrob N (2008). "Clinical heterogeneity of pseudohypoparathyroidism: from hyper- to hypocalcemia". Horm. Res. 70 (3): 137–44. doi:10.1159/000137658. PMID 18663313.
  2. Adachi M, Muroya K, Asakura Y, Kondoh Y, Ishihara J, Hasegawa T (2009). "Ectopic calcification as discernible manifestation in neonates with pseudohypoparathyroidism type 1a". Int J Endocrinol. 2009: 931057. doi:10.1155/2009/931057. PMC 2778176. PMID 20011056.
  3. Neary NM, El-Maouche D, Hopkins R, Libutti SK, Moses AM, Weinstein LS (2012). "Development and treatment of tertiary hyperparathyroidism in patients with pseudohypoparathyroidism type 1B". J. Clin. Endocrinol. Metab. 97 (9): 3025–30. doi:10.1210/jc.2012-1655. PMC 3431579. PMID 22736772.
  4. Balavoine AS, Ladsous M, Velayoudom FL, Vlaeminck V, Cardot-Bauters C, d'Herbomez M, Wemeau JL (2008). "Hypothyroidism in patients with pseudohypoparathyroidism type Ia: clinical evidence of resistance to TSH and TRH". Eur. J. Endocrinol. 159 (4): 431–7. doi:10.1530/EJE-08-0111. PMID 18805917.


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