Pseudohypoparathyroidism medical therapy: Difference between revisions

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==Medical Therapy==
==Medical Therapy==


*The mainstay of treatment for pseudohypoparathyroidism is oral [[calcium]] and 1alpha-hydroxylated vitamin D metabolites, such as [[calcitriol]]. <ref name="pmid26943720">{{cite journal |vauthors=Clarke BL, Brown EM, Collins MT, Jüppner H, Lakatos P, Levine MA, Mannstadt MM, Bilezikian JP, Romanischen AF, Thakker RV |title=Epidemiology and Diagnosis of Hypoparathyroidism |journal=J. Clin. Endocrinol. Metab. |volume=101 |issue=6 |pages=2284–99 |year=2016 |pmid=26943720 |pmc=5393595 |doi=10.1210/jc.2015-3908 |url=}}</ref>
*The mainstay of treatment for pseudohypoparathyroidism is oral [[calcium]] and 1alpha-hydroxylated vitamin D metabolites, such as [[calcitriol]] <ref name="pmid26943720">{{cite journal |vauthors=Clarke BL, Brown EM, Collins MT, Jüppner H, Lakatos P, Levine MA, Mannstadt MM, Bilezikian JP, Romanischen AF, Thakker RV |title=Epidemiology and Diagnosis of Hypoparathyroidism |journal=J. Clin. Endocrinol. Metab. |volume=101 |issue=6 |pages=2284–99 |year=2016 |pmid=26943720 |pmc=5393595 |doi=10.1210/jc.2015-3908 |url=}}</ref>
*Other forms of Vitamin D cannot be used as [[parathyroid]] hormone resistance in the [[proximal tubule]] decreases the production of 1,25(OH)2 vitamin D from 25-hydroxyvitamin D.Intravenous calcium is recommended for all patients who develop severe symptomatic [[hypocalcemia]].
*Other forms of [[Vitamin D]] cannot be used as [[parathyroid]] hormone resistance in the [[proximal tubule]] decreases the production of 1,25(OH)2 vitamin D from 25-hydroxyvitamin D.Intravenous [[calcium]] is recommended for all patients who develop severe symptomatic [[hypocalcemia]]
*The majority of cases of pseudohypoparathyroidism are self-limited and require only supportive care.
*The majority of cases of pseudohypoparathyroidism are self-limited and require only supportive care
* The goal of therapy is to:
* The goal of therapy is to:
** Serum [[calcium]] within the normal range to prevent [[hypercalciuria]]  
** Serum [[calcium]] within the normal range to prevent [[hypercalciuria]]  
** Maintain [[parathyroid]] hormone levels within reference range which helps prevent bone remodeling and [[hyperparathyroid]] bone disease
** Maintain [[parathyroid]] hormone levels within reference range which helps prevent bone remodeling and [[hyperparathyroid]] bone disease
**Intravenous calcium is recommended for all patients who develop severe symptomatic [[hypocalcemia]].
**Intravenous [[calcium]] is recommended for all patients who develop severe symptomatic [[hypocalcemia]]  
*'''Adult'''
*'''Adult'''
**Preferred regimen (1):elemental [[calcium]] (either [[calcium chloride]] or [[calcium gluconate]]) 100 mg of  over 10 to 20 minute
**Preferred regimen (1):elemental [[calcium]] (either [[calcium chloride]] or [[calcium gluconate]]) 100 mg of  over 10 to 20 minute
**An additional dose of 100 mg/hr of [[elemental calcium]] can be infused if symptoms do not resolve, with close monitoring of [[calcium]] levels
**An additional dose of 100 mg/hr of [[elemental calcium]] can be infused if symptoms do not resolve, with close monitoring of [[calcium]] levels
**Cardiac monitoring may help to guide therapy as rapid infusion of [[calcium]] can cause cardiac conduction defects;
**[[Cardiac]] monitoring may help to guide therapy as rapid infusion of [[calcium]] can cause [[cardiac]] conduction defects
**Preferred regimen (2):[[Parenteral|Parentera]]<nowiki/>l formulations available are [[calcium chloride]] and [[calcium gluconate|calcium]] [[calcium gluconate|gluconate]] 100 mg of  over 10 to 20 minute
**Preferred regimen (2):[[Parenteral|Parentera]]<nowiki/>l formulations available are [[calcium chloride]] and [[calcium gluconate|calcium]] [[calcium gluconate|gluconate]] 100 mg of  over 10 to 20 minute
*** <nowiki/>10% [[calcium chloride]] a 10-mL ampule contains 360 mg of [[elemental calcium]]
*** <nowiki/>10% [[calcium chloride]] a 10-mL ampule contains 360 mg of [[elemental calcium]]
*** 10% [[calcium gluconate]] 10-mL ampule contains 93 mg of elemental [[calcium]].
*** 10% [[calcium gluconate]] 10-mL ampule contains 93 mg of elemental [[calcium]]
*'''''For [[neonates]], [[infants]], and [[children]]'''''
*'''''For [[neonates]], [[infants]], and [[children]]'''''
**** Preferred regimen (1):10% [[calcium gluconate]]   0.5-1 mL/kg of administered over 5 minutes.
**Preferred regimen (1):10% [[calcium gluconate]]   0.5-1 mL/kg of administered over 5 minutes
{| class="wikitable"
{| class="wikitable"
! colspan="2" |Adverse drug reactions
! colspan="2" |Adverse drug reactions
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|[[Calcium chloride]]
|[[Calcium chloride]]
|
|
* Rapid I.V. injection may cause the patient to complain of tingling sensations, a calcium taste, a sense of oppression or “heat wave.”
* Rapid [[Intravenous injection]] may cause the patient to complain of [[tingling]] sensations, a [[calcium]] taste


* Injections of calcium chloride are accompanied by [[Vasodilation|peripheral vasodilation]] as well as a local “burning” sensation, and there may be a moderate fall in blood pressure.
* Injections of [[calcium chloride]] are accompanied by [[Vasodilation|peripheral vasodilation]] as well as a local “burning” sensation, and there may be a moderate fall in [[blood pressure]]
|-
|-
|[[Calcium gluconate]]
|[[Calcium gluconate]]
|
|
* Patients may complain of tingling sensations, a sense of oppression or heat waves and a calcium or chalky taste following the intravenous administration of calcium gluconate.
* Patients may complain of tingling sensations, a sense of heat waves and a [[calcium]] or chalky taste following the [[intravenous]] administration of [[calcium gluconate]]


* Rapid intravenous injection of calcium salts may cause vasodilation, decreased blood pressure, [[bradycardia]], [[cardiac arrhythmias]], [[syncope]] and [[cardiac arrest]]. Use in digitalized patients may precipitate arrhythmias.
* Rapid [[intravenous injection]] of [[calcium]] salts may cause [[vasodilation]], decreased [[blood pressure]], [[bradycardia]], [[cardiac arrhythmias]], [[syncope]] and [[cardiac arrest]]. Use in digitalized patients may precipitate [[arrhythmias]]


* Local necrosis and abscess formation may occur with intramuscular injection.
* Local [[necrosis]] and [[abscess]] formation may occur with [[intramuscular injection]]
|}
|}



Latest revision as of 19:27, 17 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

The mainstay of treatment for pseudohypoparathyroidism is oral calcium and 1alpha-hydroxylated vitamin D analogues, such as calcitriol. Other forms of Vitamin D cannot be used as parathyroid hormone resistance in the proximal tubule decreases the efficiency of production of 1,25(OH)2 vitamin D from 25-hydroxyvitamin D.Intravenous calcium is recommended for all patients who develop severe symptomatic hypocalcemia.

Medical Therapy

Adverse drug reactions
Calcium chloride
Calcium gluconate

References

  1. Clarke BL, Brown EM, Collins MT, Jüppner H, Lakatos P, Levine MA, Mannstadt MM, Bilezikian JP, Romanischen AF, Thakker RV (2016). "Epidemiology and Diagnosis of Hypoparathyroidism". J. Clin. Endocrinol. Metab. 101 (6): 2284–99. doi:10.1210/jc.2015-3908. PMC 5393595. PMID 26943720.


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