Raras
Buscar doenças, sintomas, genes...
Hipocalcemia autossômica dominante
ORPHA:428CID-10 · E20.8CID-11 · 5A50.0YDOENÇA RARA

A hipocalcemia autossômica dominante (AD hipocalcemia) é um distúrbio que afeta a forma como o cálcio é regulado no corpo. Ela se manifesta por diferentes níveis de cálcio baixo no sangue (hipocalcemia), junto com taxas anormalmente baixas do hormônio da paratireoide (PTH). Apesar disso, o nível de cálcio na urina (calciúria) permanece normal ou elevado.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A hipocalcemia autossômica dominante (AD hipocalcemia) é um distúrbio que afeta a forma como o cálcio é regulado no corpo. Ela se manifesta por diferentes níveis de cálcio baixo no sangue (hipocalcemia), junto com taxas anormalmente baixas do hormônio da paratireoide (PTH). Apesar disso, o nível de cálcio na urina (calciúria) permanece normal ou elevado.

Pesquisas ativas
3 ensaios
9 total registrados no ClinicalTrials.gov
Publicações científicas
158 artigos
Último publicado: 2026 Apr 8

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.161
Denmark
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: E20.8
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
5 sintomas
🧠
Neurológico
4 sintomas
💪
Músculos
4 sintomas
🦴
Ossos e articulações
3 sintomas
🫘
Rins
3 sintomas
📏
Crescimento
3 sintomas

+ 16 sintomas em outras categorias

Características mais comuns

90%prev.
Hipocalcemia
Muito frequente (99-80%)
90%prev.
Depressão
Muito frequente (99-80%)
90%prev.
Hipercalciúria
Muito frequente (99-80%)
90%prev.
Mioclonia cortical
Muito frequente (99-80%)
90%prev.
Comportamento atípico
Muito frequente (99-80%)
90%prev.
Ansiedade
Muito frequente (99-80%)
44sintomas
Muito frequente (11)
Frequente (12)
Ocasional (7)
Sem dados (14)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 44 características clínicas mais associadas, ordenadas por frequência.

HipocalcemiaHypocalcemia
Muito frequente (99-80%)90%
DepressãoDepression
Muito frequente (99-80%)90%
HipercalciúriaHypercalciuria
Muito frequente (99-80%)90%
Mioclonia corticalCortical myoclonus
Muito frequente (99-80%)90%
Comportamento atípicoAtypical behavior
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico158PubMed
Últimos 10 anos88publicações
Pico202512 papers
Linha do tempo
2026Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

2 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant.

CASRExtracellular calcium-sensing receptorDisease-causing germline mutation(s) (gain of function) inAltamente restrito
FUNÇÃO

G-protein-coupled receptor that senses changes in the extracellular concentration of calcium ions and plays a key role in maintaining calcium homeostasis (PubMed:17555508, PubMed:19789209, PubMed:21566075, PubMed:22114145, PubMed:22789683, PubMed:23966241, PubMed:25104082, PubMed:25292184, PubMed:25766501, PubMed:26386835, PubMed:32817431, PubMed:33603117, PubMed:34194040, PubMed:34467854, PubMed:7759551, PubMed:8636323, PubMed:8702647, PubMed:8878438). Senses fluctuations in the circulating cal

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (3)
G alpha (i) signalling eventsClass C/3 (Metabotropic glutamate/pheromone receptors)G alpha (q) signalling events
MECANISMO DE DOENÇA

Hypocalciuric hypercalcemia, familial 1

A form of hypocalciuric hypercalcemia, a disorder of mineral homeostasis that is transmitted as an autosomal dominant trait with a high degree of penetrance. It is characterized biochemically by lifelong elevation of serum calcium concentrations and is associated with inappropriately low urinary calcium excretion and a normal or mildly elevated circulating parathyroid hormone level. Hypermagnesemia is typically present. Affected individuals are usually asymptomatic and the disorder is considered benign. However, chondrocalcinosis and pancreatitis occur in some adults.

OUTRAS DOENÇAS (6)
autosomal dominant hypocalcemia 1familial hypocalciuric hypercalcemia 1neonatal severe primary hyperparathyroidismautosomal dominant hypocalcemia
HGNC:1514UniProt:P41180
GNA11Guanine nucleotide-binding protein subunit alpha-11Disease-causing germline mutation(s) (gain of function) inAltamente restrito
FUNÇÃO

Guanine nucleotide-binding proteins (G proteins) function as transducers downstream of G protein-coupled receptors (GPCRs) in numerous signaling cascades (PubMed:31073061). The alpha chain contains the guanine nucleotide binding site and alternates between an active, GTP-bound state and an inactive, GDP-bound state (PubMed:31073061). Signaling by an activated GPCR promotes GDP release and GTP binding (PubMed:31073061). The alpha subunit has a low GTPase activity that converts bound GTP to GDP, t

LOCALIZAÇÃO

Cell membraneCytoplasm

VIAS BIOLÓGICAS (10)
PLC beta mediated eventsG-protein activationADP signalling through P2Y purinoceptor 1G alpha (q) signalling eventsThrombin signalling through proteinase activated receptors (PARs)
MECANISMO DE DOENÇA

Hypocalciuric hypercalcemia, familial 2

A form of hypocalciuric hypercalcemia, a disorder of mineral homeostasis that is transmitted as an autosomal dominant trait with a high degree of penetrance. It is characterized biochemically by lifelong elevation of serum calcium concentrations and is associated with inappropriately low urinary calcium excretion and a normal or mildly elevated circulating parathyroid hormone level. Hypermagnesemia is typically present. Affected individuals are usually asymptomatic and the disorder is considered benign. However, chondrocalcinosis and pancreatitis occur in some adults.

EXPRESSÃO TECIDUAL(Ubíquo)
Cerebelo
153.0 TPM
Cérebro - Hemisfério cerebelar
140.5 TPM
Testículo
104.7 TPM
Esôfago - Muscular
97.3 TPM
Fallopian Tube
94.3 TPM
OUTRAS DOENÇAS (8)
autosomal dominant hypocalcemia 2familial hypocalciuric hypercalcemia 2anastomosing haemangiomauveal melanoma
HGNC:4379UniProt:P29992

Variantes genéticas (ClinVar)

584 variantes patogênicas registradas no ClinVar.

🧬 GNA11: NM_002067.5(GNA11):c.548G>C (p.Arg183Pro) ()
🧬 GNA11: NM_002067.5(GNA11):c.535G>A (p.Val179Met) ()
🧬 GNA11: NM_002067.5(GNA11):c.980A>G (p.His327Arg) ()
🧬 GNA11: NM_002067.5(GNA11):c.548G>A (p.Arg183His) ()
🧬 GNA11: NM_002067.5(GNA11):c.542G>C (p.Arg181Pro) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 2,413 variantes classificadas pelo ClinVar.

362
1810
241
Patogênica (15.0%)
VUS (75.0%)
Benigna (10.0%)
VARIANTES MAIS SIGNIFICATIVAS
CASR: NM_000388.4(CASR):c.260T>C (p.Leu87Pro) [Conflicting classifications of pathogenicity]
CASR: NM_000388.4(CASR):c.286del (p.Arg96fs) [Pathogenic]
CASR: NM_000388.4(CASR):c.2226G>A (p.Trp742Ter) [Pathogenic]
CASR: NM_000388.4(CASR):c.3233C>G (p.Ser1078Ter) [Uncertain significance]
CASR: NM_000388.4(CASR):c.1735G>T (p.Ala579Ser) [Uncertain significance]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 32
2Fase 24
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 8 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Hipocalcemia autossômica dominante

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

9 ensaios clínicos encontrados, 3 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
89 papers (10 anos)
#1

Refractory Hypocalcemia from Combined Autosomal Dominant Hypocalcemia Type 2 and Postsurgical Hypoparathyroidism.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research2026 Feb 27

A 49-yr-old female with a history of chronic hypocalcemia, diagnosed twenty years earlier, had serum calcium concentrations that ranged between 6.4 and 8.5 mg/dL (nl, 8.6-10.3 mg/dL) with inappropriately low to low-normal parathyroid hormone (PTH) concentrations. She did not report symptoms of hypocalcemia such as paresthesias, muscle cramping, or tetany. More recently, a thyroid nodule was discovered, and the patient subsequently underwent total thyroidectomy for papillary thyroid cancer. Parathyroid autotransplantation was not performed since parathyroid glands were not identified during the surgery. After total thyroidectomy, the patient developed a more severe symptomatic, refractory hypocalcemia with undetectable PTH concentrations. Postoperative pathologic examination identified a single hypercellular parathyroid gland. Despite medical treatment, including frequent and regular calcium gluconate infusions, serum calcium concentrations remained persistently low. A genetic evaluation identified a pathogenic GNA11 variant (c.178C > T, p.Arg60Cys), consistent with autosomal dominant hypocalcemia type 2 (ADH2). This case expands the understanding of ADH2 and raises important questions about optimal treatment strategies in patients with coexisting genetic and postsurgical hypoparathyroidism.

#2

Autosomal dominant hypocalcemia type 1: Status quo of tailored management and future perspectives.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research2026 Feb 24

A 28-d-old female infant presented with clonic seizures secondary to hypocalcemia (calcium [Ca], 5.7 mg/dL) and hypoparathyroidism (intact parathyroid hormone [PTH], 7 pg/mL). Despite the initiation of oral alfacalcidol therapy, she experienced recurrent episodes of generalized convulsions or focal tetany, despite calcium lactate administration during febrile episodes. Her younger sister exhibited frequent irritability until day 38 of life due to hypocalcemia (Ca, 7.7 mg/dL) and hypoparathyroidism (intact PTH, 5 pg/mL). She experienced three febrile generalized seizures during infancy despite oral alfacalcidol treatment. Both sisters developed nephrocalcinosis despite oral hydrochlorothiazide treatment to reduce hypercalciuria. Genetic testing identified a pathogenic variant, c.2504C > A (p.Ala835Asp), in the calcium-sensing receptor (CASR) gene in both sisters. Their father carried the same variant but remained asymptomatic. This finding led to a diagnosis of autosomal dominant hypocalcemia type 1 (ADH1). Optimal active vitamin D treatment in ADH1 remains challenging because of difficulty maintaining stable serum Ca levels amid fluctuating physiological demands, as well as persistent hypercalciuria resulting from combined PTH deficiency and CaSR activation. Emerging therapies, including calcilytics, may help address these limitations.

#3

An interesting case of coexistence of autosomal dominant hypocalcemia 1 with Bartter syndrome and chronic myelogenous leukemia.

Hormones (Athens, Greece)2026 Mar

Autosomal dominant hypocalcemia type 1 (ADH1) is a rare form of hypoparathyroidism caused by heterozygous, inherited, or de novo activating mutations in the calcium-sensing receptor gene (CASR). A 29-year-old man was referred to the outpatient department for poorly controlled hypoparathyroidism with hypocalcemia, hypomagnesemia, mild hypokalemia, excessive hypercalciuria, and a worsening eGFR under conventional therapy. He was diagnosed with hypoparathyroidism on the second day of birth. He remained inadequately controlled even after the initiation of replacement therapy with rhPTH (1-84). He was also diagnosed with chronic myelogenous leukemia (CML) at the age of 28 years and was treated with tyrosine kinase inhibitor (TKI), which further worsened the control of hypoparathyroidism. Genetic analysis was performed at the age of 31 years and revealed a change c.2486 A > G in exon 7 of the CaSR gene. This case highlights the importance of characterizing the cause of non-surgical hypoparathyroidism, including ADH1, in the differential diagnosis. ADH1 may coexist with Bartter syndrome type V, making the patient's management more challenging. To our knowledge, this is the second case in the literature with the coexistence of two rare diseases, ADH1 and CML.In CML patients treated with TKIs, serum calcium levels should be monitored and, in the case of severe hypocalcemia accompanied by low or inappropriately normal PTH, the possible existence of ADH1 may need to be investigated.

#4

Clinical characteristics of chinese patients with autosomal dominant hypocalcemia type 1: a single-center study.

Journal of endocrinological investigation2026 Mar 09
#5

Long-acting parathyroid hormone receptor agonist rectifies hypocalcemia in autosomal dominant hypocalcemia type 1 mice.

The Journal of clinical investigation2026 Feb 19

Publicações recentes

Ver todas no PubMed

📚 EuropePMC77 artigos no totalmostrando 88

2026

Clinical characteristics of chinese patients with autosomal dominant hypocalcemia type 1: a single-center study.

Journal of endocrinological investigation
2026

Refractory Hypocalcemia from Combined Autosomal Dominant Hypocalcemia Type 2 and Postsurgical Hypoparathyroidism.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2026

Autosomal dominant hypocalcemia type 1: Status quo of tailored management and future perspectives.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2026

Long-acting parathyroid hormone receptor agonist rectifies hypocalcemia in autosomal dominant hypocalcemia type 1 mice.

The Journal of clinical investigation
2025

Novel Variants and Clinical Heterogeneity in Pediatric Calcium Metabolism Disorders Identified Through High-Yield Tiered Genetic Testing in a Taiwanese Cohort.

Medicina (Kaunas, Lithuania)
2025

A novel chemoreactive calcilytic for the potential treatment of autosomal dominant hypocalcemia.

Acta pharmaceutica Sinica. B
2026

An interesting case of coexistence of autosomal dominant hypocalcemia 1 with Bartter syndrome and chronic myelogenous leukemia.

Hormones (Athens, Greece)
2025

Chronic Hypoparathyroidism-Current and Emerging Therapies.

Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists
2025

A calcium-sensing receptor allelic series and underdiagnosis of genetically driven hypocalcemia.

American journal of human genetics
2025

Seizures due to Autosomal Dominant Hypocalcemia-Type 1.

Indian journal of pediatrics
2025

Characterization of quinazolinone calcilytic therapy for autosomal dominant hypocalcemia type 1 (ADH1).

The Journal of biological chemistry
2025

An activating calcium-sensing receptor variant with biased signaling reveals a critical residue for Gα11 coupling.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2025

Management of autosomal dominant hypocalcemia type 1: Literature review and clinical practice recommendations.

Journal of endocrinological investigation
2024

A narrative review of monogenic disorders causing nephrolithiasis and chronic kidney disease.

Nephrology (Carlton, Vic.)
2025

CASRdb: A Publicly Accessible Comprehensive Database for Disease-Associated Calcium-Sensing Receptor Variants.

The Journal of clinical endocrinology and metabolism
2025

Heterogeneous Origins of Calcium Homeostasis Disorders Arising From 5 Heterozygous Calcium-Sensing Receptor Variants.

The Journal of clinical endocrinology and metabolism
2025

Hypoparathyroidism: an update on new therapeutic approaches.

Endocrine
2025

Monitoring Calcium-Sensing Receptor (CaSR)-Induced Intracellular Calcium Flux Using an Indo-1 Flow Cytometry Assay.

Methods in molecular biology (Clifton, N.J.)
2024

Molecular regulation of calcium-sensing receptor (CaSR)-mediated signaling.

Chronic diseases and translational medicine
2024

New insights into renal calcium-sensing receptor activation.

Current opinion in nephrology and hypertension
2024

Treatment of Hypoparathyroidism by Re-Establishing the Effects of Parathyroid Hormone.

Endocrinology and metabolism (Seoul, Korea)
2024

G protein-coupled receptor (GPCR) gene variants and human genetic disease.

Critical reviews in clinical laboratory sciences
2023

A hypercalcemic episode in an adolescent with autosomal dominant hypocalcemia.

Pediatrics international : official journal of the Japan Pediatric Society
2023

Efficacy and Safety of Encaleret in Autosomal Dominant Hypocalcemia Type 1.

The New England journal of medicine
2023

Case Report: Calcium sensing receptor gene gain of function mutations: a case series and report of 2 novel mutations.

Frontiers in endocrinology
2023

A pediatric case of autosomal dominant hypocalcemia type 2.

Journal of pediatric endocrinology &amp; metabolism : JPEM
2023

Autosomal Dominant Hypocalcemia Type 1 and Neonatal Focal Seizures.

Children (Basel, Switzerland)
2023

GNA11 Variants Identified in Patients with Hypercalcemia or Hypocalcemia.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2023

Temperature sensing by the calcium-sensing receptor.

Frontiers in physiology
2023

Novel Calcium-Sensing Receptor (CASR) Mutation in a Family with Autosomal Dominant Hypocalcemia Type 1 (ADH1): Genetic Study over Three Generations and Clinical Characteristics.

Hormone research in paediatrics
2023

The importance of molecular diagnosis in the management of autosomal dominant hypocalcemia type 1 (ADH1): Case report.

Annales d'endocrinologie
2022

Autosomal Dominant Hypocalcemia Type 1: A Systematic Review.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2022

Autosomal dominant hypocalcemia with a novel CASR mutation: a case study and literature review.

The Journal of international medical research
2022

The Youngest Infant to Be Diagnosed with Autosomal Dominant Hypocalcemia Type 2 Harboring a Novel Variant of GNA11: A Case Study and Literature Review.

Annals of clinical and laboratory science
2022

Autosomal Dominant Hypocalcemia Type 1 (ADH1) Associated With Myoclonus and Intracerebral Calcifications.

Journal of the Endocrine Society
2022

Differential parathyroid and kidney Ca2+-sensing receptor activation in autosomal dominant hypocalcemia 1.

EBioMedicine
2021

A variant in the CASR gene (c.368T>C) causing hypocalcemia refractory to standard medical therapy.

Endocrinology, diabetes &amp; metabolism case reports
2022

Recurrent hypocalcemic tetany presenting to the emergency room: Answers.

Pediatric nephrology (Berlin, Germany)
2021

PTH Infusion for Seizures in Autosomal Dominant Hypocalcemia Type 1.

The New England journal of medicine
2021

The role of calcium-sensing receptor signaling in regulating transepithelial calcium transport.

Experimental biology and medicine (Maywood, N.J.)
2021

Rare diseases caused by abnormal calcium sensing and signalling.

Endocrine
2021

Autosomal Dominant Hypocalcemia With Atypical Urine Findings Accompanied by Novel CaSR Gene Mutation and VitD Deficiency.

Journal of the Endocrine Society
2020

[Clinical and genetic characteristics of primary hypoparathyroidism in children].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2020

Recombinant human parathyroid hormone (1-84) is effective in CASR-associated hypoparathyroidism.

European journal of endocrinology
2020

Calcilytic NPSP795 Increases Plasma Calcium and PTH in an Autosomal Dominant Hypocalcemia Type 1 Mouse Model.

JBMR plus
2021

A cell function study on calcium regulation of a novel calcium-sensing receptor mutation (p.Tyr825Phe).

Annals of pediatric endocrinology &amp; metabolism
2020

A Novel Phenotype Associated with CaSR-Related Familial Brain Calcifications.

Movement disorders clinical practice
2020

Familial Hypocalciuric Hypercalcemia Type 1 and Autosomal-Dominant Hypocalcemia Type 1: Prevalence in a Large Healthcare Population.

American journal of human genetics
2020

Activating Mutations of the G-protein Subunit α 11 Interdomain Interface Cause Autosomal Dominant Hypocalcemia Type 2.

The Journal of clinical endocrinology and metabolism
2019

Intractable Generalized Epilepsy and Autosomal Dominant Hypocalcemia: A Case Report.

Child neurology open
2019

Genetic Screening in a Large Chinese Cohort of Childhood Onset Hypoparathyroidism by Next-Generation Sequencing Combined with TBX1-MLPA.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2019

Persistent Activation of Calcium-Sensing Receptor Suppresses Bone Turnover, Increases Microcracks, and Decreases Bone Strength.

JBMR plus
2019

Treatment of Autosomal Dominant Hypocalcemia Type 1 With the Calcilytic NPSP795 (SHP635).

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2018

Causes and pathophysiology of hypoparathyroidism.

Best practice &amp; research. Clinical endocrinology &amp; metabolism
2019

Bone Matrix Mineralization in Patients With Gain-of-Function Calcium-Sensing Receptor Mutations Is Distinctly Different From that in Postsurgical Hypoparathyroidism.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2018

Long-Term Parathyroid Hormone 1-34 Replacement Therapy in Children with Hypoparathyroidism.

The Journal of pediatrics
2018

New Directions in Treatment of Hypoparathyroidism.

Endocrinology and metabolism clinics of North America
2018

Medical Hypoparathyroidism.

Endocrinology and metabolism clinics of North America
2019

Advances in the treatment of hypoparathyroidism with PTH 1-34.

Bone
2018

De novo a novel variant of CaSR gene in a neonate with congenital hypoparathyroidism.

Annals of pediatric endocrinology &amp; metabolism
2018

Congenital chloride diarrhea needs to be distinguished from Bartter and Gitelman syndrome.

Journal of human genetics
2018

Homozygous Calcium-Sensing Receptor Polymorphism R544Q Presents as Hypocalcemic Hypoparathyroidism.

The Journal of clinical endocrinology and metabolism
2018

Identification and Functional Characterization of a Novel Mutation in the Human Calcium-Sensing Receptor That Co-Segregates With Autosomal-Dominant Hypocalcemia.

Frontiers in endocrinology
2018

A calcium-sensing receptor mutation causing hypocalcemia disrupts a transmembrane salt bridge to activate β-arrestin-biased signaling.

Science signaling
2017

Clinical characterization of a novel calcium sensing receptor genetic alteration in a Greek patient with autosomal dominant hypocalcemia type 1.

Hormones (Athens, Greece)
2017

[Calcilytic drugs:Feature and future.].

Clinical calcium
2017

[Disorders Caused by Mutations in Calcium-Sensing Receptor and Related Diseases.].

Clinical calcium
2017

Gα11 mutation in mice causes hypocalcemia rectifiable by calcilytic therapy.

JCI insight
2017

Knockin mouse with mutant Gα11 mimics human inherited hypocalcemia and is rescued by pharmacologic inhibitors.

JCI insight
2017

Diseases associated with calcium-sensing receptor.

Orphanet journal of rare diseases
2016

Autosomal Dominant Hypocalcemia (Hypoparathyroidism) Types 1 and 2.

Frontiers in physiology
2016

Natpara for the treatment of hypoparathyroidism.

Expert opinion on biological therapy
2017

Autosomal dominant hypocalcemia due to a truncation in the C-tail of the calcium-sensing receptor.

Molecular and cellular endocrinology
2016

Activating Calcium-Sensing Receptor Mutations: Prospects for Future Treatment with Calcilytics.

Trends in endocrinology and metabolism: TEM
2016

Impaired growth and intracranial calcifications in autosomal dominant hypocalcemia caused by a GNA11 mutation.

European journal of endocrinology
2016

Epidemiology and Health-Related Quality of Life in Hypoparathyroidism in Norway.

The Journal of clinical endocrinology and metabolism
2016

Allosteric Modulation of the Calcium-sensing Receptor Rectifies Signaling Abnormalities Associated with G-protein α-11 Mutations Causing Hypercalcemic and Hypocalcemic Disorders.

The Journal of biological chemistry
2016

Identification of a G-Protein Subunit-α11 Gain-of-Function Mutation, Val340Met, in a Family With Autosomal Dominant Hypocalcemia Type 2 (ADH2).

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2016

Novel calcium-sensing receptor cytoplasmic tail deletion mutation causing autosomal dominant hypocalcemia: molecular and clinical study.

European journal of endocrinology
2016

GENETICS IN ENDOCRINOLOGY: Gain and loss of function mutations of the calcium-sensing receptor and associated proteins: current treatment concepts.

European journal of endocrinology
2016

Pathogenesis of hypokalemia in autosomal dominant hypocalcemia type 1.

Clinical and experimental nephrology
2016

Calcimimetic and Calcilytic Drugs: Feats, Flops, and Futures.

Calcified tissue international
2015

14-3-3 Proteins Buffer Intracellular Calcium Sensing Receptors to Constrain Signaling.

PloS one
2015

[Bone and Nutrition. A prospect of calcium sensing receptor].

Clinical calcium
2015

The Calcilytic Agent NPS 2143 Rectifies Hypocalcemia in a Mouse Model With an Activating Calcium-Sensing Receptor (CaSR) Mutation: Relevance to Autosomal Dominant Hypocalcemia Type 1 (ADH1).

Endocrinology
2015

Calcilytic Ameliorates Abnormalities of Mutant Calcium-Sensing Receptor (CaSR) Knock-In Mice Mimicking Autosomal Dominant Hypocalcemia (ADH).

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
2015

Autosomal dominant hypocalcemia with Bartter syndrome due to a novel activating mutation of calcium sensing receptor, Y829C.

Korean journal of pediatrics
2015

Novel Mutation in the CASR Gene (p.Leu123Ser) in a Case of Autosomal Dominant Hypocalcemia.

Journal of pediatric genetics

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Hipocalcemia autossômica dominante.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Hipocalcemia autossômica dominante

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Refractory Hypocalcemia from Combined Autosomal Dominant Hypocalcemia Type 2 and Postsurgical Hypoparathyroidism.
    Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research· 2026· PMID 41757505mais citado
  2. Autosomal dominant hypocalcemia type 1: Status quo of tailored management and future perspectives.
    Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research· 2026· PMID 41732805mais citado
  3. An interesting case of coexistence of autosomal dominant hypocalcemia 1 with Bartter syndrome and chronic myelogenous leukemia.
    Hormones (Athens, Greece)· 2026· PMID 40760326mais citado
  4. Clinical characteristics of chinese patients with autosomal dominant hypocalcemia type 1: a single-center study.
    Journal of endocrinological investigation· 2026· PMID 41801666mais citado
  5. Long-acting parathyroid hormone receptor agonist rectifies hypocalcemia in autosomal dominant hypocalcemia type 1 mice.
    The Journal of clinical investigation· 2026· PMID 41712458mais citado
  6. Non-Classical Presentations of Rare Hereditary Hypoparathyroidism: A Case Series of CASR, GNA11 and GATA3 Mutations.
    Clin Endocrinol (Oxf)· 2026· PMID 41952051recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:428(Orphanet)
  2. MONDO:0018543(MONDO)
  3. GARD:2877(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q30989948(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Hipocalcemia autossômica dominante
Compêndio · Raras BR

Hipocalcemia autossômica dominante

ORPHA:428 · MONDO:0018543
Prevalência
Unknown
Herança
Autosomal dominant
CID-10
E20.8 · Outro hipoparatireoidismo
CID-11
Ensaios
3 ativos
Início
All ages
Prevalência
0.161 (Denmark)
MedGen
UMLS
C0342345
Repurposing
2 candidatos
calcitriolvitamin D receptor agonist
dihydrotachysterolvitamin analog
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades