Potasman I, Oliven A, Bassan H M
Harefuah. 1989 Mar 15;116(6):302-4.
Diffuse idiopathic pulmonary fibrosis (DIPF) carries a poor prognosis, with an average survival of 4 years. A 37-year-old man had been followed by our department for over 5 years for biopsy-proven DIPF. The disease progressed relentlessly, unresponsive to corticosteroid or colchicine treatment. Marked pulmonary hypertension and right heart failure developed. Arterial pO2 decreased to 30 mmHg and he became bedridden and totally dependent on oxygen inhalation. A heart-lung transplantation was successful in June 1987. The anti-rejection regimen included cyclosporine and azathioprine. Subsequently his functional capacity improved remarkably and pO2 reached 87 mmHg on ambient air. Regular weekly followup visits have included physical examination, chest X-ray, cyclosporine blood levels, blood counts and determination of renal function. During a year of followup there has been no rejection episode nor any other major complication. Although experience with lung transplantation for end-stage DIPF is still limited, it is gradually being accepted as the only successful therapeutic approach. The case presented is the first in Israel of successful heart-lung transplantation for DIPF.
弥漫性特发性肺纤维化（DIPF）预后较差，平均生存期为4年。一名37岁男性因经活检证实的DIPF在我院随访超过5年。疾病持续进展，对皮质类固醇或秋水仙碱治疗无反应。出现了明显的肺动脉高压和右心衰竭。动脉血氧分压降至30 mmHg，他卧床不起，完全依赖吸氧。1987年6月心肺移植成功。抗排斥方案包括环孢素和硫唑嘌呤。随后他的功能能力显著改善，在空气中的血氧分压达到87 mmHg。每周定期随访包括体格检查、胸部X线检查、环孢素血药浓度、血细胞计数和肾功能测定。在一年的随访中，没有发生排斥反应或任何其他重大并发症。尽管终末期DIPF肺移植的经验仍然有限，但它正逐渐被接受为唯一成功的治疗方法。所介绍的病例是以色列首例成功进行的DIPF心肺移植。
