title: Recovery of chylous pulmonary congestion in tuberous sclerosis complex-associated lymphangioleiomyomatosis.
abstract: Chyloptysis and chylous pulmonary congestion are extremely rare complications of lymphangioleiomyomatosis (LAM). We report a case of a 50-year-old woman with tuberous sclerosis complex-associated LAM, who presented with expectorating milky-white bronchial casts. She was diagnosed with chyloptysis and chylous pulmonary congestion by sputum analysis. Her symptoms and lung infiltration were improved by oral sirolimus therapy; moreover, serum Krebs von den Lungen-6 (KL-6) levels paralleled the symptoms and lung infiltration of these complications. We suggest that serum KL-6 may be a useful monitoring biomarker of chyloptysis and chylous pulmonary congestion in LAM.
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, National Hospital Organization, Kokura Medical Center, Kitakyushu, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kinoshita
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, National Hospital Organization, Kokura Medical Center, Kitakyushu, Japan.
authors.forename: Atsuhiko
authors.initials: A
authors.lastname: Sakamoto
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, National Hospital Organization, Kokura Medical Center, Kitakyushu, Japan.
authors.forename: Kouko
authors.initials: K
authors.lastname: Hidaka
mesh_terms: D015415:Biomarkers / Q000097:blood; D002913:Chyle; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D018192:Lymphangioleiomyomatosis / Q000150:complications*; D008875:Middle Aged; D018396:Mucin-1 / Q000097:blood; D020123:Sirolimus / Q000627:therapeutic use; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications*
chemical_list: D015415:Biomarkers; C060192:MUC1 protein, human; D018396:Mucin-1; D020123:Sirolimus