Removal of lung lavage fluid during whole-lung lavage using biphasic cuirass ventilation chest percussion in a patient with autoimmune pulmonary alveolar proteinosis.

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare lung disease. Although recombinant human granulocyte macrophage colony-stimulating factor (GM-CSF) therapy has emerged as a new therapeutic modality, whole-lung lavage (WLL) with manual chest percussion has been a standard therapy in advanced cases. The application of biphasic cuirass ventilation (BCV) instead of chest percussion has rarely been reported. We describe the case of a patient with advanced PAP who recovered well in both lungs, without complication, after we performed WLL with BCV under anesthetic mechanical ventilation. Both radiographical chest findings and clinical symptoms were improved, and oxygen therapy was finally withdrawn. This case illustrates that BCV can enhance the effective removal of lavage fluid and is an alternative to manual percussion.

Haemoptysis and bronchial congestion due to pulmonary vein stenosis after maze procedure.

Pulmonary vein stenosis (PVS) is a rare disease that can cause haemoptysis. Acquired PVS is indicated as a complication associated with cardiac catheter intervention; however, the maze procedure has not been reported to induce PVS. Here, we describe the diagnosis and treatment strategy for the first case of PVS with haemoptysis due to the maze procedure. A 56-year-old man who underwent the maze procedure seven years previously was referred for repeated haemoptysis. Contrast-enhanced computed tomography (CT) revealed complete occlusion of the left superior pulmonary vein. Bronchoscopy revealed localized bronchial congestion and varices. He was diagnosed with PVS due to the maze procedure, and he underwent catheter-balloon angioplasty. After treatment, haemoptysis disappeared and bronchial congestion and varices improved. History of cardiac ablation (surgical or catheter intervention) and localized bronchial congestion findings might facilitate the accurate diagnosis of PVS with haemoptysis. Catheter-balloon angioplasty is a minimally invasive treatment for PVS.

A case of solitary paraaortic lymph node recurrence of lung squamous cell carcinoma after resection.

Solitary abdominal paraaortic lymph node recurrence after radical lung cancer surgery is very rare. Here, we report a case of a solitary abdominal paraaortic lymph node recurrence of lung squamous cell carcinoma (SCC). A 63-year-old man was diagnosed with lung SCC (cT1cN0M0 stage IA3), underwent a video-assisted right lower lobectomy (ND2a-1), and the pathological findings showed SCC (pT1cN0M0 stage IA3). The EGFR mutation and ALK translocation statuses of SCC were negative, and adjuvant therapy was not performed. Follow-up positron emission tomography - computed tomography (PET/CT) showed a solitary fluorodeoxyglucose (FDG)-concentrated region in the swollen paraaortic lymph node. A paraaortic lymph node biopsy was performed by open laparotomy, to determine the precise diagnosis and identify the genetic status. Pathological findings revealed that the paraaortic lymph node contained poorly differentiated SCC, which was thought to metastasize from the lung cancer. The genetic status of the lymph node recurrence revealed a lack of EGFR mutations, ALK translocations, and ROS1 mutations, while the tumor proportion score (TPS) of PD-L1 was 55%, and we therefore administered pembrolizumab, an immune checkpoint inhibitor. Biopsies are very important for achieving precise diagnoses and determining the genetic statuses of tumors, since molecular-targeting drugs and immune checkpoint inhibitors are available. J. Med. Invest. 65:283-285, August, 2018.

Expression and characterization of Rab38, a new member of the Rab small G protein family.

Rab38 is a new member of the Rab small G protein family that regulates intracellular vesicle trafficking. Rab38 is expressed in melanocytes and it has been clarified that a point mutation in the postulated GTP-binding domain of Rab38 is the gene responsible for oculocutaneous albinism in chocolate mice. However, basic information regarding recombinant protein production, intracellular location, and tissue-specific expression pattern has not yet been reported. We produced recombinant Rab38 using a baculovirus/insect cell-protein expression system. A combination of Triton X-114 phase separation and nickel-affinity chromatography yielded exclusively prenylated Rab38 that bound [alpha-32P]-GTP. The mRNA and the native protein were expressed in a tissue-specific manner, e.g., in the lung, skin, stomach, liver, and kidney. Freshly isolated rat alveolar type II cells were highly positive for the mRNA signal, but the signal was rapidly lost over time. Immunofluorescence staining demonstrated that expressed GST-tagged Rab38 was mainly co-localized with endoplasmic reticulum-resident protein and also partly with intermittent vesicles between the endoplasmic reticulum and the Golgi complex. These results indicate that Rab38 is expressed non-ubiquitously in specific tissues and regulates early vesicle transport relating to the endoplasmic reticulum, and hence suggest that Rab38 abnormality may cause multiple organ diseases as well as oculocutaneous albinism.

Video-assisted thoracoscopic wedge resection for pulmonary sequestration.

A 60-year-old woman underwent a video-assisted thoracoscopic wedge resection of intralobar pulmonary sequestration instead of a lobotomy because the lesion was localized in the right basal segment. Preoperative 3-dimensional computed tomography was useful for identifying an aberrant artery arising from the thoracic aorta and distributing to the lesion. A successful outcome more than 4 years after the surgery indicates that a wedge resection under video-assisted thoracoscopy may prove to be a therapeutic option for localized pulmonary sequestration.

H(2)O(2) inhibits alveolar epithelial wound repair in vitro by induction of apoptosis.

Reactive oxygen species (ROS) are released into the alveolar space and contribute to alveolar epithelial damage in patients with acute lung injury. However, the role of ROS in alveolar repair is not known. We studied the effect of ROS in our in vitro wound healing model using either human A549 alveolar epithelial cells or primary distal lung epithelial cells. We found that H(2)O(2) inhibited alveolar epithelial repair in a concentration-dependent manner. At similar concentrations, H(2)O(2) also induced apoptosis, an effect seen particularly at the edge of the wound, leading us to hypothesize that apoptosis contributes to H(2)O(2)-induced inhibition of wound repair. To learn the role of apoptosis, we blocked caspases with the pan-caspase inhibitor N-benzyloxycarbonyl-Val-Ala-Asp (zVAD). In the presence of H(2)O(2), zVAD inhibited apoptosis, particularly at the wound edge and, most importantly, maintained alveolar epithelial wound repair. In H(2)O(2)-exposed cells, zVAD also maintained cell viability as judged by improved cell spreading and/or migration at the wound edge and by a more normal mitochondrial potential difference compared with cells not treated with zVAD. In conclusion, H(2)O(2) inhibits alveolar epithelial wound repair in large part by induction of apoptosis. Inhibition of apoptosis can maintain wound repair and cell viability in the face of ROS. Inhibiting apoptosis may be a promising new approach to improve repair of the alveolar epithelium in patients with acute lung injury.

Keratinocyte growth factor can enhance alveolar epithelial repair by nonmitogenic mechanisms.

Pretreatment with keratinocyte growth factor (KGF) ameliorates experimentally induced acute lung injury in rats. Although alveolar epithelial type II cell hyperplasia probably contributes, the mechanisms underlying KGF's protective effect remain incompletely described. Therefore, we tested the hypothesis that KGF given to rats in vivo would enhance alveolar epithelial repair in vitro by nonproliferative mechanisms. After intratracheal instillation (48 h) of KGF (5 mg/kg), alveolar epithelial type II cells were isolated for in vitro alveolar epithelial repair studies. KGF-treated cells had markedly increased epithelial repair (96 +/- 22%) compared with control cells (P < 0.001). KGF-treated cells had increased cell spreading and migration at the wound edge but no increase in in vitro proliferation compared with control cells. KGF-treated cells were more adherent to extracellular matrix proteins and polystyrene. Inhibition of the epidermal growth factor (EGF) receptor with tyrosine kinase inhibitors abolished the KGF effect on epithelial repair. In conclusion, in vivo administration of KGF augments the epithelial repair rate of alveolar epithelial cells by altering cell adherence, spreading, and migration and through stimulation of the EGF receptor.