Atlas One · Host Biology · Scale 07 — System

Birt-Hogg-Dubé Syndrome

Birt-Hogg-Dubé syndrome (BHD) is caused by germline FLCN mutations; fibrofolliculomas (skin), pulmonary cysts (pneumothorax risk 7×), bilateral multifocal chromophobe/hybrid oncocytic RCC (~30% lifetime); nephron-sparing surveillance surgery; mTOR inhibitors explored.

Also known as: BHD, Birt-Hogg-Dubé syndrome, Birt-Hogg-Dube, FLCN syndrome, BHD syndrome, chromophobe RCC hereditary, fibrofolliculoma syndrome, BHD RCC, BHD pneumothorax, BHD kidney cancer

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
birt-hogg-dube-syndrome

Birt-Hogg-Dubé Syndrome

Birt-Hogg-Dubé syndrome (BHD) is caused by germline FLCN mutations; fibrofolliculomas (skin), pulmonary cysts (pneumothorax risk 7×), bilateral multifocal chromophobe/hybrid oncocytic RCC (~30% lifetime); nephron-sparing surveillance surgery; mTOR inhibitors explored.

Entry Metadata

FieldValue
IDbirt-hogg-dube-syndrome
NameBirt-Hogg-Dubé Syndrome
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toFlcn connects-toMtor connects-toVhl connects-toRenal Cell Carcinoma connects-toLung connects-toSkin connects-toAmpk connects-toCowden Syndrome connects-toType Ii Pneumocyte connects-toKidney connects-toTuberous Sclerosis Complex connects-toHlrcc connects-toVhl Disease connects-toFibroblast connects-toRenal System connects-toRespiratory System connects-toPten connects-toAutophagy connects-toCollagen connects-toIntegumentary System connects-toColorectal Cancer connects-toThyroid Cancer connects-toMen1 Syndrome connects-toAdipocyte connects-toPancreas connects-toOxygen connects-toMet connects-toAkt connects-toHif 1Alpha connects-toMacrophage connects-toLarge Intestine connects-toNitrogen connects-toFibrosis connects-toVegf connects-toPhoton connects-toAlveolus connects-toThyroid connects-toElectron connects-toEye connects-toAdrenal Gland connects-toSmooth Muscle Cell connects-toEndothelial Cell connects-toStomach connects-toReproductive System connects-toBasal Cell Carcinoma connects-toErythrocyte connects-toMelanoma connects-toCyclin D1 connects-toPeutz Jeghers Syndrome connects-toCopd connects-toTgf Beta connects-toP53 connects-toNf Kb connects-toStat3 connects-toCkd connects-toAnemia Of Chronic Disease connects-toVenous Thromboembolism connects-toMajor Depressive Disorder connects-toAspergillus Fumigatus connects-toSepsis connects-toGeneralized Anxiety Disorder connects-toWound Healing connects-toDigestive System connects-toEndocrine System connects-toCardiovascular System connects-toMusculoskeletal System connects-toImmune System connects-toNeurofibromatosis Type 1 connects-toNeurofibromatosis Type 2 connects-toGorlin Syndrome connects-toTargeted Therapy connects-toDicer1 Syndrome connects-toCarney Complex connects-toLung Slice connects-toCancer Chemotherapy connects-toCheckpoint Inhibitors connects-toStk11 connects-toAtp connects-toLi Fraumeni Syndrome connects-toMarfan Syndrome connects-toCystic Fibrosis connects-toIntestinal Epithelium connects-toTsc1 Tsc2 connects-toYap1 connects-toPheochromocytoma Paraganglioma connects-toMyc connects-toNfe2L2 connects-toWnt Beta Catenin connects-toFoxo1 connects-toErk1 2 connects-toEgln1 connects-toCdh1 connects-toSdhb connects-toTert connects-toFh connects-toIgf 1 connects-toCaspase 3 connects-toPik3Ca connects-toNotch connects-toBcl 2 connects-toIdh1 connects-toPdgf connects-toKras connects-toEpas1 connects-toGalectin 3 connects-toJak1 2 connects-toStat1 connects-toSmad4 connects-toCgas Sting connects-toCdk4 6 connects-toPerforin connects-toGsk 3B connects-toMdm2 connects-toS100A8 A9 connects-toSrc Kinase connects-toDnmt3A connects-toCcr5 connects-toArid1A connects-toCxcl12 connects-toIl 1B connects-toIl 6 connects-toIl 33 connects-toIl 17A connects-toComplement C3 connects-toInsulin connects-toAxl Receptor connects-toMhc Class Ii connects-toIl 2 connects-toT Cytotoxic Cell connects-toXanthine Oxidase connects-toIl 10 connects-toNitric Oxide connects-toCholesterol connects-toIl 4 connects-toIl 13 connects-toIron connects-toLeptin connects-toAdiponectin connects-toResistin connects-toVhl Disease connects-toTuberous Sclerosis Complex connects-toFibroblast connects-toIfn Gamma connects-toIl 12 connects-toType I Interferon connects-toIl 5 connects-toIl 23 connects-toIge connects-toT Helper Cell connects-toMast Cell connects-toC5Ar1 connects-toComplement C5 connects-toFactor H connects-toTransferrin connects-toTslp connects-toBradykinin connects-toErythropoietin connects-toHistamine connects-toMelatonin connects-toC1 Esterase Inhibitor connects-toTestosterone connects-toSerotonin connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSelenium connects-toSodium connects-toPotassium connects-toMagnesium connects-toIodine connects-toCopper connects-toZinc connects-toCarbon connects-toChloride connects-toHydrogen connects-toPd 1 connects-toGlp 1 connects-toAngiotensin Ii connects-toRankl connects-toFibronectin connects-toActivin A connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin connects-toBdnf connects-toOsteopontin connects-toFgfr connects-toEpinephrine