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Pediatric systemic lupus erythematosus

ORPHA:93552DiseaseNot applicableAdolescent, Childhood, Infancy, Neonatal

Ассоциированные гены (4)

SAT1
spermidine/spermine N1-acetyltransferase 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 313020
STAT4
signal transducer and activator of transcription 4
Major susceptibility factor in
OMIM: 600558
SPP1
secreted phosphoprotein 1
Major susceptibility factor in
OMIM: 166490
IRAK1
interleukin 1 receptor associated kinase 1
Major susceptibility factor in
OMIM: 300283

Фенотипы (53)

Очень частый (80–99%)3
HP:0003565Elevated erythrocyte sedimentation rate
HP:0005421Decreased circulating complement C3 concentration
HP:0045042Decreased circulating complement C4 concentration
Частый (30–79%)21
HP:0000079Abnormality of the urinary system
HP:0000083Renal insufficiency
HP:0000093Proteinuria
HP:0000100Nephrotic syndrome
HP:0000123Nephritis
HP:0000790Hematuria
HP:0000951Abnormality of the skin
HP:0000969Edema
HP:0000988Skin rash
HP:0001698Pericardial effusion
HP:0001873Thrombocytopenia
HP:0001882Leukopenia
HP:0001888Lymphopenia
HP:0001937Microangiopathic hemolytic anemia
HP:0001945Fever
HP:0002202Pleural effusion
HP:0002716Lymphadenopathy
HP:0003493Antinuclear antibody positivity
HP:0003613Antiphospholipid antibody positivity
HP:0011024Abnormality of the gastrointestinal tract
HP:0025435Increased circulating lactate dehydrogenase concentration
Периодический (5–29%)21
HP:0000155Oral ulcer
HP:0000707Abnormality of the nervous system
HP:0000709Psychosis
HP:0001250Seizure
HP:0001324Muscle weakness
HP:0001369Arthritis
HP:0001541Ascites
HP:0002013Vomiting
HP:0002027Abdominal pain
HP:0002086Abnormality of the respiratory system
HP:0002094Dyspnea
HP:0002301Hemiplegia
HP:0002315Headache
HP:0002725Systemic lupus erythematosus
HP:0002829Arthralgia
HP:0003270Abdominal distention
HP:0004372Reduced consciousness/confusion
HP:0007417Discoid lupus rash
HP:0025300Malar rash
HP:0025343Lupus anticoagulant
HP:0040319Dark urine
Очень редкий (1–4%)8
HP:0001596Alopecia
HP:0002014Diarrhea
HP:0002463Language impairment
HP:0003453Antineutrophil antibody positivity
HP:0030880Raynaud phenomenon
HP:0100543Cognitive impairment
HP:0100614Myositis
HP:0100749Chest pain

Эпидемиология (2)

Point prevalence
1-9 / 100 000
United States
Annual incidence
1-9 / 1 000 000
United States

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы