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Cyclic neutropenia

ORPHA:2686DiseaseAutosomal dominantAll ages

Ассоциированные гены (1)

ELANE
elastase, neutrophil expressed
Disease-causing germline mutation(s) in
OMIM: 130130

Фенотипы (32)

Очень частый (80–99%)4
HP:0000246Sinusitis
HP:0002315Headache
HP:0002653Bone pain
HP:0040289Cyclic neutropenia
Частый (30–79%)12
HP:0000155Oral ulcer
HP:0000230Gingivitis
HP:0001581Recurrent skin infections
HP:0001954Recurrent fever
HP:0002716Lymphadenopathy
HP:0011110Recurrent tonsillitis
HP:0011947Respiratory tract infection
HP:0012378Fatigue
HP:0025289Cervical lymphadenopathy
HP:0025439Pharyngitis
HP:0030757Tooth abscess
HP:0032323Periodic fever
Периодический (5–29%)11
HP:0000388Otitis media
HP:0000704Periodontitis
HP:0001873Thrombocytopenia
HP:0001888Lymphopenia
HP:0002027Abdominal pain
HP:0006308Atrophy of alveolar ridges
HP:0006357Premature loss of permanent teeth
HP:0009789Perianal abscess
HP:0031690Opportunistic infection
HP:0031891Decreased eosinophil count
HP:0100658Cellulitis
Очень редкий (1–4%)5
HP:0002586Peritonitis
HP:0004387Enterocolitis
HP:0031864Bacteremia
HP:0032169Severe infection
HP:0100806Sepsis

Эпидемиология (1)

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