Novel clinical scoring system to identify patients with pneumothorax with suspicion for Birt-Hogg-Dubé syndrome.

BACKGROUND AND OBJECTIVE: Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary disease that presents with multiple lung cysts and pneumothorax (PTX). Although some reports propose that findings from chest computed tomography enable one to distinguish BHDS from primary spontaneous pneumothorax (PSP), it is still unclear whether clinical features are useful for identifying patients with suspicion of BHDS from those with PTX. METHODS: We retrospectively reviewed the medical records of patients with PTX who underwent video-assisted thoracoscopic surgery at Nissan Tamagawa Hospital from January 2012 to December 2015. RESULTS: We identified a total of 1141 patients with PTX, including 54 with BHDS and 517 with PSP. Among them, logistic regression analysis segregated five features that were significantly associated with BHDS: familial history of PTX, past history of bilateral PTX, age at the first episode of PTX (≥25 years old (y.o.)), body mass index (≥18.5) and gender (female). We assigned scores of 3, 3, 2, 2 and 1 to the five features, respectively, to establish a system with a calculated score from 0 to 11. The cut-off value of a calculated score ≥ 4 yielded the highest sensitivity of 93% and specificity of 86%. Receiver operating characteristic (ROC) analysis showed the area under the curve reflecting an accuracy of this diagnostic test as 0.953. CONCLUSION: BHDS has several clinical features distinct from PSP. Our scoring system consists of only five clinical variables that are easily evaluated and efficiently separate BHDS patients from those who have PTX without relying on an imaging study. Further prospective study is needed to confirm our findings.

Ex vivo supplementation of TGF-beta1 enhances the fibrous tissue regeneration effect of synovium-derived fibroblast transplantation in a tendon defect: a biomechanical study.

The present study was conducted to test a hypothesis that the ex vivo supplementation of TGF-beta1 into medium will significantly improve the mechanical properties of the fibrous tissue regenerated in the patellar tendon defect after transplantation of cultured autologous synovium-derived fibroblasts. Thirty rabbits were divided into the following three groups. In Group A, we applied phosphate buffered saline of 0.1 ml to the defect created in the patellar tendon. In Group B, we transplanted autologous fibroblasts, which had been cultured into the tendon defect. In Group C, we transplanted autologous fibroblasts, which had been cultured with supplementation of TGF-beta1, into the tendon defect. Animals were killed at 6 weeks, and the regenerated tissue was examined for biomechanics and histology. The tangent modulus and the tensile strength of Group C were significantly higher than that of Group B, while the tensile strength of Group C was significantly lower than that of Group A. Histologically, vascular formation was abundantly found in the regenerated tissue of Groups B and C as compared to the regenerated tissues in Group A. The present study showed that transplantation of cultured autologous synovium-derived fibroblasts enhanced vascular formation in the fibrous tissue regenerated in the patellar tendon defect, while cell transplantation deteriorated the mechanical properties of the regenerated fibrous tissue. However, the ex vivo supplementation of TGF-beta1 into the medium significantly decreased mechanical deterioration of the fibrous tissue regenerated in the tendon defect after transplantation of cultured autologous synovium-derived fibroblasts.