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Primary hyperaldosteronism-seizures-neurological abnormalities syndrome

ORPHA:369929DiseaseNot applicableInfancy, Neonatal

Ассоциированные гены (1)

CACNA1D
calcium voltage-gated channel subunit alpha1 D
Disease-causing germline mutation(s) (gain of function) in
OMIM: 114206

Фенотипы (30)

Облигатный (100%)3
HP:0000822Hypertension
HP:0000859Hyperaldosteronism
HP:0040084Abnormal circulating renin
Очень частый (80–99%)5
HP:0001250Seizure
HP:0001263Global developmental delay
HP:0001714Ventricular hypertrophy
HP:0002900Hypokalemia
HP:0100021Cerebral palsy
Частый (30–79%)13
HP:0001258Spastic paraplegia
HP:0001959Polydipsia
HP:0002069Bilateral tonic-clonic seizure
HP:0002092Pulmonary arterial hypertension
HP:0002305Athetosis
HP:0002384Focal impaired awareness seizure
HP:0010864Intellectual disability, severe
HP:0011166Focal myoclonic seizure
HP:0011410Caesarian section
HP:0011706Second degree atrioventricular block
HP:0100285EMG: impaired neuromuscular transmission
HP:0100704Cerebral visual impairment
HP:0200114Metabolic alkalosis
Периодический (5–29%)7
HP:0000360Tinnitus
HP:0000421Epistaxis
HP:0000787Nephrolithiasis
HP:0001629Ventricular septal defect
HP:0002018Nausea
HP:0002170Intracranial hemorrhage
HP:0002315Headache
Очень редкий (1–4%)1
HP:0008221Adrenal hyperplasia
Исключён (0%)1
HP:0011739Dexamethasone-suppresible primary hyperaldosteronism

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы