title: Null allele alpha-1 antitrypsin deficiency: case report of the total pleural covering technique for disease-associated pneumothorax.
abstract: A 44-year-old woman, who had been diagnosed as having null type alpha-1 antitrypsin (AAT) deficiency, was admitted with right recurrent pneumothorax. She had undergone right visceral pleural strengthening and pleural adhesion-preventing surgery, the total pleural covering technique (TPC), with oxidized regenerative cellulose (ORC). Three years after the operation, she developed left recurrent pneumothorax and underwent left TPC. AAT deficiency is a rare inherited disease in Japan that causes early-onset emphysema and secondary pneumothorax. In addition, null/null phenotype has the highest risk of emphysema and may require lung transplantation. For future lung transplantation, pleural adhesion should be prevented. However, pleurodesis and surgery for pneumothorax generally cause adhesion. TPC with ORC is a surgical treatment for pneumothorax that is done to strengthen visceral pleura and prevent pleural adhesions. The patient has had no recurrence of pneumothorax 3.5 years after right TPC and 6 months after left TPC.
authors.affiliation: Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kusu
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Nakagiri
authors.forename: Masato
authors.initials: M
authors.lastname: Minami
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Shintani
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Kadota
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Inoue
authors.forename: Noriyoshi
authors.initials: N
authors.lastname: Sawabata
authors.forename: Meinoshin
authors.initials: M
authors.lastname: Okumura
mesh_terms: D000328:Adult; D002483:Cellulose, Oxidized; D004867:Equipment Design; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D010641:Phenotype; D010995:Pleural Diseases / Q000209:etiology / Q000517:prevention & control; D011030:Pneumothorax / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D012008:Recurrence; D013526:Surgical Mesh; D020775:Thoracic Surgery, Video-Assisted / Q000009:adverse effects / Q000295:instrumentation; D000267:Tissue Adhesions; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D000515:alpha 1-Antitrypsin / Q000235:genetics*; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis / Q000235:genetics*
chemical_list: D002483:Cellulose, Oxidized; C043392:SERPINA1 protein, human; D000515:alpha 1-Antitrypsin