Lymphangioleiomyomatosis (LAM) is a multi-system disease of women.  Metabolomics have been used to determine if metabolites downstream of mTOR and other LAM realted pathways were present or related to disease progression in a national cohort.

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Lymphangioleiomyomatosis (LAM) is a rare lung and lymphatic disease categorised by infiltration of smooth muscle type cells in the lungs and lymphatics leading to progressive respiratory impairment. The disease only effects women and is caused by a defect in one of the two proteins associated with tuberous sclerosis, tuberin and hamartin.

HDR UK

BioLAM code

BioLAM data.csv

given

family

LAM diagnosis (def, prob, poss)

Date of diagnosis

Date last FU no rapa

Date last FU  rapa treated

date last follow up

Current age (yrs)

Date enrolled

Alive, transplant, dead

Date of death / transplant

TSC (yes, no)

angiomyolipoma

menopause

rapamycin (date started)

FEV1 (% predicted)

DLCO (% predicted)

ΔFEV1

ΔDLCO

Δ interval (months)

Δ n obs.

ΔFEV1 rapamycin

ΔDLCO rapamycin

Δ interval (months) rapamycin

Δ n obs. rapamycin

6MW dist

post ex SaO2

reversibility (%)

VEGFD

Date diagnosed

Disease duration at baseline (months)

Disease duration last FU no rapa (months)

Disease duration last FU rapa treated (months)

Total FU duration (months)

absolute FEV1

2 cluster result

3 cluster result

Pres. Sx-SOB

Pres. Sx- PTX

Pres. Sx- OTHER RESP

Pres. Sx- AML

Pres. Sx- SCREENED

Pres. Sx- CHANCE

Ever had PTX

Ever had AML

Ever had LYMPHATIC DISEASE

Ever had TSC

UK BioLAM

UK BioLAM

Documentation

Coverage

Provenance

Origin

Temporal

Accessibility

Access

Format and Standards

Observations

Origin