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Apparent mineralocorticoid excess

ORPHA:320DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

HSD11B2
hydroxysteroid 11-beta dehydrogenase 2
Disease-causing germline mutation(s) (loss of function) in
OMIM: 614232

Фенотипы (17)

Очень частый (80–99%)3
HP:0000822Hypertension
HP:0002900Hypokalemia
HP:0003351Decreased circulating renin concentration
Частый (30–79%)8
HP:0000121Nephrocalcinosis
HP:0001508Failure to thrive
HP:0001959Polydipsia
HP:0001960Hypokalemic metabolic alkalosis
HP:0004319Decreased circulating aldosterone level
HP:0004322Short stature
HP:0011731Abnormality of circulating cortisol level
HP:0012603Abnormal urine sodium concentration
Периодический (5–29%)5
HP:0000083Renal insufficiency
HP:0001095Hypertensive retinopathy
HP:0001297Stroke
HP:0001511Intrauterine growth retardation
HP:0001712Left ventricular hypertrophy
Исключён (0%)1
HP:0012606Renal sodium wasting

Эпидемиология (1)

Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы