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TRAPS syndrome
1 OMIM reference -
1 associated gene
88 connected diseases
37 signs/symptoms
Disease Type of connection
Intermittent hydrarthrosis
Autoimmune lymphoproliferative syndrome
Autosomal dominant nonsyndromic sensorineural deafness type DFNA
Fanconi anemia
Juvenile myelomonocytic leukemia
Noonan syndrome
FADD-related immunodeficiency
Oculootodental syndrome
Budd-Chiari syndrome
Essential thrombocythemia
Familial thrombocytosis
Myelofibrosis with myeloid metaplasia
Polycythemia vera
Anhidrotic ectodermal dysplasia - immunodeficiency - osteopetrosis - lymphedema
Chronic mucocutaneous candidiasis
Hypohidrotic ectodermal dysplasia with immunodeficiency
Incontinentia pigmenti
MALT lymphoma
Mendelian susceptibility to mycobacterial diseases due to partial STAT1 deficiency
Susceptibility to viral and mycobacterial infections
X-linked mendelian susceptibility to mycobacterial diseases due to IKBKG deficiency
Behavioral variant of frontotemporal dementia
CLN11 disease
Progressive non-fluent aphasia
Semantic dementia
Acrokeratosis verruciformis of Hopf
Acute fatty liver of pregnancy
Autosomal recessive nonsyndromic intellectual deficit
Autosomal recessive nonsyndromic sensorineural deafness type DFNB
Baraitser-Winter syndrome
Cardiomyopathy - hypotonia - lactic acidosis
Catecholaminergic polymorphic ventricular tachycardia
Congenital fibrosis of extraocular muscles
DDOST-CDG
Darier disease
Early-onset spastic ataxia-neuropathy syndrome
Hypomyelination with atrophy of basal ganglia and cerebellum
Inflammatory myofibroblastic tumor
Isolated ATP synthase deficiency
Kostmann syndrome
Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
Mitochondrial trifunctional protein deficiency
Polymicrogyria due to TUBB2B mutation
Primary dystonia, DYT4 type
Progressive sensorineural hearing loss - hypertrophic cardiomyopathy
SSR4-CDG
Severe combined immunodeficiency due to DNA-PKcs deficiency
Spinocerebellar ataxia type 28
Translocation renal cell carcinoma
LEOPARD syndrome
Metachondromatosis
Autoimmune lymphoproliferative syndrome with recurrent infections
Autosomal recessive systemic lupus erythematosus
Common variable immunodeficiency
Periventricular nodular heterotopia
Amyotrophic lateral sclerosis
Autosomal agammaglobulinemia
Autosomal dominant medullary cystic kidney disease with or without hyperuricemia
CLOVE syndrome
Congenital glaucoma
Cowden syndrome
Glycogen storage disease due to hepatic glycogen synthase deficiency
Hemimegalencephaly
Hereditary gingival fibromatosis
Hereditary nonpolyposis colon cancer
Hereditary thrombophilia due to congenital histidine-rich (poly-L) glycoprotein deficiency
Juvenile glaucoma
Macrodactyly of fingers, unilateral
Megalencephaly-capillary malformation-polymicrogyria syndrome
Noonan syndrome-like disorder with juvenile myelomonocytic leukemia
SHORT syndrome
Segmental progressive overgrowth syndrome with fibroadipose hyperplasia
Herpetic encephalitis
Distal 22q11.2 microdeletion syndrome
Familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis
Juvenile rheumatoid factor-negative polyarthritis
Oligoarticular juvenile arthritis
Squamous cell carcinoma of head and neck
Early-onset progressive neurodegeneration - blindness - ataxia - spasticity
Microcephaly-capillary malformation syndrome
Neonatal inflammatory skin and bowel disease
Niemann-Pick disease type A
Niemann-Pick disease type B
Young adult-onset Parkinsonism
Berardinelli-Seip congenital lipodystrophy
Diffuse cutaneous systemic sclerosis
Heritable pulmonary arterial hypertension
Limited cutaneous systemic sclerosis
Synonym(s):
- Autosomal dominant periodic fever
- Familial hibernian fever
- TNF receptor 1 associated periodic syndrome
- Tumor necrosis factor receptor 1 associated periodic syndrome

Classification (Orphanet):
- Rare genetic disease
- Rare immune disease
- Rare skin disease
- Rare systemic or rheumatologic disease

Classification (ICD10):
- Endocrine, nutritional and metabolic diseases -

Epidemiological data:
Class of prevalence: unknown
Average age onset: childhood
Average age of death: -
Type of inheritance: autosomal dominant
External references:
1 OMIM reference -
No MeSH references

Gene symbol UniProt reference OMIM reference
TNFRSF1A P19438191190
Very frequent
- Acute abdominal pain / colic
- Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP
- Cutaneous rash
- Erysipela
- Fever / chilling
- Macules
- Malabsorption / chronic diarrhea / steatorrhea
- Myalgia / muscular pain
- Pericardium anomalies / pericarditis / absence / cysts / pericardial effusion

Frequent
- Abnormal pleura / hydrothorax / pleuresia / pleural effusion / chylothorax
- Arthritis / synovitis / synovial proliferation
- Constipation
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Hyperleukocytosis / leukocytosis
- Intestinal obstruction / ileus
- Lymphadenopathy / polyadenopathies
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Splenomegaly
- Testes inflammation / orchitis

Occasional
- Articular / joint pain / arthralgia
- Cellulitis / panniculitis / pseudocellulitis / inflammation of subcutaneous tissue
- Chronic uveitis / blepharitis / episcleritis / scleritis / conjonctivitis / keratitis
- Cranial nerves palsy
- Dizziness
- Ecchymoses
- Facial pain / cephalalgia / migraine
- Fasciitis
- Follicular / erythematous / edematous papules / milium
- Myocardium anomalies / myocarditis
- Myositis
- Paresthesia / dysesthesia / hypoesthesia / anesthesia / numbness
- Peritonitis / peritoneal abscess
- Pharyngitis
- Psychic / behavioural troubles
- Sacroiliac joints anomalies
- Thoracic / chest pain
- Vascularitis / vasculitides / arteritis