Pseudohypoparathyroidism natural history, complications and prognosis
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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 lowered sexual drive and lowered sexual development, lowered energy levels, and increased weight.
Complications
Complications that can develop as a result of pseudohypoparathyroidism are
- Seizures (children).
- Hypothyroidism due to associated resistance to thyrotropin.
- Gonadotropin or GHRH resistance.
- Patients may develop hypocalcemia resulting in[1]
- Paresthesias
- Muscular cramping
- Tetany
- Carpopedal spasm
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 lowered sexual drive and lowered sexual development, lowered energy levels, and increased weight.
- Subcutaneous calcification in neonatal period.[2]
- Reproductive dysfunction is seen in patients with pseudohypoparathyroidism 1a. Women may experience delayed puberty, oligomenorrhea, and infertility.In men decreased Fertility may present with maturation arrest of Testes and cryptorchidism.
- Pseudohypoparathyroidism type 1b patients are at risk of developing tertiary hyperparathyroidism and hyperparathyroid bone disease. [3]
- Osteopenia and rickets in peudohypoparathyroidism-1a is associated with variable osteoclast responsiveness to parathyroid hormone.[4]
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.