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Tumor necrosis factor receptor 1 associated periodic syndrome

ORPHA:32960DiseaseAutosomal dominantAdolescent, Adult, Childhood, Infancy

Ассоциированные гены (1)

TNFRSF1A
TNF receptor superfamily member 1A
Disease-causing germline mutation(s) in
OMIM: 191190

Фенотипы (40)

Очень частый (80–99%)10
HP:0000988Skin rash
HP:0001055Erysipelas
HP:0001701Pericarditis
HP:0001954Recurrent fever
HP:0002014Diarrhea
HP:0002027Abdominal pain
HP:0003326Myalgia
HP:0003565Elevated erythrocyte sedimentation rate
HP:0011227Elevated circulating C-reactive protein concentration
HP:0012733Macule
Частый (30–79%)10
HP:0001369Arthritis
HP:0001744Splenomegaly
HP:0001974Leukocytosis
HP:0002013Vomiting
HP:0002019Constipation
HP:0002102Pleuritis
HP:0002716Lymphadenopathy
HP:0005214Intestinal obstruction
HP:0010783Erythema
HP:0100796Orchitis
Периодический (5–29%)20
HP:0000509Conjunctivitis
HP:0000554Uveitis
HP:0000708Atypical behavior
HP:0000978Bruising susceptibility
HP:0001034Hypermelanotic macule
HP:0001637Abnormal myocardium morphology
HP:0002076Migraine
HP:0002321Vertigo
HP:0002586Peritonitis
HP:0002633Vasculitis
HP:0002829Arthralgia
HP:0003401Paresthesia
HP:0006824Cranial nerve paralysis
HP:0100537Fasciitis
HP:0100539Periorbital edema
HP:0100614Myositis
HP:0100658Cellulitis
HP:0100749Chest pain
HP:0100776Recurrent pharyngitis
HP:0100781Abnormality of the sacroiliac joint

Эпидемиология (2)

Point prevalence
Unknown
Worldwide
Point prevalence
1-9 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы