Increased Primary Cilia in Idiopathic Pulmonary Fibrosis.

Primary cilia are solitary, non-motile, axonemal microtubule-based antenna-like organelles that project from the plasma membrane of most mammalian cells and are implicated in transducing hedgehog signals during development. It was recently proposed that aberrant SHH signaling may be implicated in the progression of idiopathic pulmonary fibrosis (IPF). However, the distribution and role of primary cilia in IPF remains unclear. Here, we clearly observed the primary cilia in alveolar epithelial cells, fibroblasts, and endothelial cells of human normal lung tissue. Then, we investigated the distribution of primary cilia in human IPF tissue samples using immunofluorescence. Tissues from six IPF cases showed an increase in the number of primary cilia in alveolar cells and fibroblasts. In addition, we observed an increase in ciliogenesis related genes such as IFT20 and IFT88 in IPF. Since major components of the SHH signaling pathway are known to be localized in primary cilia, we quantified the mRNA expression of the SHH signaling components using qRT-PCR in both IPF and control lung. mRNA levels of SHH, the coreceptor SMO, and the transcription factors GLI1 and GLI2 were upregulated in IPF compared with control. Furthermore, the nuclear localization of GLI1 was observed mainly in alveolar epithelia and fibroblasts. In addition, we showed that defective KIF3A-mediated ciliary loss in human type II alveolar epithelial cell lines leads to disruption of SHH signaling. These results indicate that a significant increase in the number of primary cilia in IPF contributes to the upregulation of SHH signals.

Off-pump open pulmonary embolectomy for patients with major pulmonary embolism.

Acute major pulmonary artery embolism (AMPE) requires rapid diagnosis and early intensive treatment to optimize patient outcomes. Most patients with AMPE and hemodynamic instability need open pulmonary embolectomy (OPE). We modified the technique of OPE to include a minimally invasive procedure without the use of cardiopulmonary bypass (CPB). From March 1988 to April 2006, we performed OPE on a total of 12 patients (21 sides) with AMPE. Seven patients (13 sides) underwent conventional OPE with CPB and 5 patients underwent off-pump OPE (OPPE), 4 (8 sides) with AMPE and 1 with catheter embolus with thrombosis. In patients who underwent conventional OPE, there was 1 hospital death in a patient with severe right ventricle dysfunction and 2 significant cases of airway bleeding. In patients who underwent OPPE, there was 1 case of minimal airway bleeding. Mean systolic pulmonary artery pressure in conventional OPE and OPPE patients, respectively, decreased from 50.3 +/- 14 mmHg and 35.4 +/- 6.6 mmHg pre-operatively to 41.7 +/- 20 and 28 +/- 3 mmHg postoperatively. During the long-term follow-up, there were 2 cancer-related deaths but no recurrence of PE. All surviving patients maintained functional class I (n = 10) or II (n = 1). Compared with conventional OPE, OPPE was effective for treating AMPE in our selected cases. Modification of conventional CPB and systemic full heparinization to minimal use of systemic heparinization without CPB may be helpful in treating selected patients with AMPE.

Endovascular treatment of ruptured infected aortic aneurysm with sepsis.

The generally accepted treatment for infected aortic aneurysms involves open surgical resection and debridement, with in situ or extra-anatomical bypass. Occasionally, endovascular management can be substituted for the standard operation dependent on the patient's condition. We report the case of an 81-year-old female with a ruptured infected aortic aneurysm and sepsis, successfully treated endovascularly. She had been on oral antibiotics for one year and is doing well 2 years after discharge.

Short-term and intermediate-term results after unclipping: what happened to primary hyperhidrosis and truncal reflex sweating after unclipping in patients who underwent endoscopic thoracic sympathetic clamping?

Endoscopic thoracic sympathetic clamping (ETC) is used to treat patients with primary hyperhidrosis because it offers the potential of a reversal operation (unclipping) when severe reflex sweating (RS) occurs. Although unclipping has been reported to be effective, the short-term or intermediate-term results after unclipping are unclear. From March 2002 to October 2006, 15 (12.9%) out of 116 patients with primary hyperhidrosis, who underwent ETC, had the endoclip(s) removed as a result of RS. Fourteen patients could be followed up for more than 6 months. The patients answered a telephone interview on the severity of RS, the recurrence of the primary site, and their level of satisfaction. There was no mortality or significant morbidity encountered. On the follow-up, 9 (64%) of the 14 patients who underwent unclipping reported symptomatic recovery from RS. Of these 9 patients with early unclipping (within 4 wk after ETC), only 7 (78%) were satisfied with the outcomes. This suggests that early unclipping does not always guarantee satisfactory recovery from RS. Because early unclipping does not guarantee a full recovery in all patients, special consideration in ETC is needed to determine when to remove the clamp and how strongly to apply the clamp to achieve better results.