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A Journal on Surgery

Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 0,877

Frequency: Bi-Monthly

ISSN 0026-4733

Online ISSN 1827-1626


Minerva Chirurgica 2008 June;63(3):241-7


Alpha-1-antitrypsin deficiency (Siiyama) as indication for lung transplantation: proper timing for surgical intervention

Shindo G. 1, Endo T. 1, Onda M. 1, Hiroi M. 2, Tsutae W. 3

1 Cardiothoracic Department Meditopia Numazu Medical Clinic Numazu City, Japan
2 Department of Internal Medicine Ohmori Hospital Medical Center Toho University, Tokyo, Japan
3 Department of Molecular Biology Kishimoto Medical Insutitute, Sapporo, Japan

The authors report a new familial case of alpha-1- antitrypsin (AAT) deficiency with severe pulmonary emphysema and hemoptysis. A severely reduced serum AAT level of the proband, a 56-year-old farmer’s wife and her sister were observed. Mutation analysis of the AAT gene was performed using allele-specific polymerase chain reaction (PCR) analysis followed by direct sequencing. The proband and her younger sister proved to be homozygous for PISiiyama. Although home oxygen therapy was induced in addition to previous medications including bronchodilators and cardiovascular conditioning, the proband’s rate of decline of forced expiratory volume at one second (FEV1) was progressing. Lung transplantation was therefore advisable for the patient. Clinical analysis on Japanese cases reported in the literature shows that the rate of decline of FEV1 is one of the most convenient prognostic factors to find proper timing for surgical intervention. Lung transplantation is one of the best reliable current therapies to improve quality of life of severely impaired patients.

language: English


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