Biomaterial Database

Bronchiectasis and spontaneous pneumothorax in Marfan's syndrome. 1977

P C Teoh

A Chinese girl with Marfan's syndrome had spontaneous pneumothorax and bronchiectasis. Spontaneous pneumothorax is not an infrequent pulmonary manifestation of Marfan's syndrome, but bronchiectasis is rarely reported to occur. So far, it has not been reported that both bronchiectasis and spontaneous pneumothorax can occur in the same patient with Marfan's syndrome.

UI	MeSH Term	Description	Entries
D008382	Marfan Syndrome	An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE; AORTIC ANEURYSM; and AORTIC DISSECTION. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2.	Marfan Like Connective Tissue Disorder,Marfan Syndrome Type 1,Marfan Syndrome Type 2,Marfan Syndrome, Type II,Marfan Syndrome, Type I,Marfan's Syndrome,Marfans Syndrome
D011030	Pneumothorax	An accumulation of air or gas in the PLEURAL CAVITY, which may occur spontaneously or as a result of trauma or a pathological process. The gas may also be introduced deliberately during PNEUMOTHORAX, ARTIFICIAL.	Pneumothorax, Primary Spontaneous,Pressure Pneumothorax,Primary Spontaneous Pneumothorax,Spontaneous Pneumothorax,Tension Pneumothorax,Pneumothorax, Pressure,Pneumothorax, Spontaneous,Pneumothorax, Tension,Spontaneous Pneumothorax, Primary
D001987	Bronchiectasis	Persistent abnormal dilatation of the bronchi.	Cylindrical Bronchiectasis,Cystic Bronchiectasis,Saccular Bronchiectasis,Varicose Bronchiectasis,Bronchiectases,Bronchiectasis, Cylindrical,Bronchiectasis, Cystic,Bronchiectasis, Saccular,Bronchiectasis, Varicose,Cylindrical Bronchiectases,Cystic Bronchiectases,Saccular Bronchiectases,Varicose Bronchiectases
D001995	Bronchography	Radiography of the bronchial tree after injection of a contrast medium.	Bronchographies
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths