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Hereditary angioedema type 1

ORPHA:100050Etiological subtypeAutosomal dominantAll ages

Ассоциированные гены (1)

SERPING1
serpin family G member 1
Disease-causing germline mutation(s) in
OMIM: 606860

Фенотипы (26)

Очень частый (80–99%)12
HP:0000282Facial edema
HP:0001025Urticaria
HP:0001939Abnormality of metabolism/homeostasis
HP:0002027Abdominal pain
HP:0003401Paresthesia
HP:0005225Intestinal edema
HP:0007514Edema of the dorsum of hands
HP:0011971Dermatographic urticaria
HP:0012027Laryngeal edema
HP:0012252Abnormal respiratory system morphology
HP:0025349Limbal edema
HP:0040315Tongue edema
Частый (30–79%)6
HP:0002013Vomiting
HP:0002014Diarrhea
HP:0002015Dysphagia
HP:0002018Nausea
HP:0002094Dyspnea
HP:0100755Abnormality of salivation
Периодический (5–29%)8
HP:0000172Abnormality of the uvula
HP:0001609Hoarse voice
HP:0002098Respiratory distress
HP:0002615Hypotension
HP:0005348Inspiratory stridor
HP:0005483Abnormal epiglottis morphology
HP:0011855Pharyngeal edema
HP:0100736Abnormality of the soft palate

Эпидемиология (2)

Point prevalence
1-9 / 100 000
Italy
Point prevalence
1-9 / 100 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы