Lymphangioleiomyomatosis

GPTKB entity

Statements (40)
Predicate Object
gptkbp:instanceOf gptkb:disease
gptkbp:abbreviation gptkb:LAM
gptkbp:affects lungs
lymphatic system
women of childbearing age
gptkbp:associatedWith gptkb:tuberous_sclerosis_complex
gptkb:spontaneous_pneumothorax
chylous effusions
gptkbp:cause cystic lung destruction
progressive respiratory failure
gptkbp:characterizedBy abnormal growth of smooth muscle-like cells
gptkbp:complication respiratory failure
pleural effusion
renal angiomyolipoma
gptkbp:diagnosedBy lung biopsy
high-resolution CT scan
gptkbp:firstDescribed 1918
gptkbp:geneticStudies gptkb:TSC1_gene
gptkb:TSC2_gene
gptkbp:hasOrphanetID ORPHA:635
https://www.w3.org/2000/01/rdf-schema#label Lymphangioleiomyomatosis
gptkbp:ICD-10_code J84.81
gptkbp:MeSH_ID D008210
gptkbp:OMIM 606690
gptkbp:prevalence rare
gptkbp:progression slowly progressive
gptkbp:researchOrganization gptkb:European_LAM_Federation
gptkb:LAM_Foundation
gptkbp:riskFactor female sex
reproductive age
gptkbp:subspecies TSC-associated LAM
sporadic LAM
gptkbp:symptom fatigue
cough
shortness of breath
chest pain
gptkbp:treatment gptkb:sirolimus
lung transplantation
gptkbp:bfsParent gptkb:LAM
gptkbp:bfsLayer 6