Therapeutic Effect of Sirolimus for Lymphangioleiomyomatosis Remaining in the Abdominopelvic Region After Lung Transplantation: A Case Report.

PURPOSE: Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM. CASE SUMMARY: The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time. CONCLUSION: The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.

Pulmonary function in patients with chronic rhinosinusitis and allergic rhinitis.

BACKGROUND: A close relationship between upper and lower respiratory tract diseases has been reported. However, little is known about pulmonary function in patients with upper respiratory tract diseases. METHODS: Pulmonary function was measured in: 68 patients with chronic rhinosinusitis without nasal polyps, 135 patients with chronic rhinosinusitis with nasal polyps, 89 patients with allergic rhinitis and 100 normal control subjects. The relationships between pulmonary function and clinical parameters were assessed. These parameters included radiographic severity of chronic rhinosinusitis, serum total immunoglobulin E levels, concentrations of cytokines in nasal secretions and exhaled nitric oxide levels. RESULTS: The pulmonary function of patients with chronic rhinosinusitis was significantly affected. The level of interleukin-5 in nasal secretions was significantly correlated with pulmonary function in patients with chronic rhinosinusitis. CONCLUSION: The findings indicated latent obstructive lung function changes in chronic rhinosinusitis patients. The cytokines in nasal secretions might be related to obstructive lung function changes in chronic rhinosinusitis.

Less maintenance immunosuppression in lung transplantation following hematopoietic stem cell transplantation from the same living donor.

Living-donor lobar lung transplantation (LDLLT) is one of the final options for saving patients with pulmonary complications after hematopoietic stem cell transplantation (HSCT). We retrospectively investigated 19 patients who had undergone LDLLT after HSCT in Japan. Eight patients underwent LDLLT after HSCT in which one of the donors was the same living donor as in HSCT (SD group), while 11 received LDLLT from relatives who were not the HSCT donors (non-SD group). In the SD group, three patients underwent single LDLLT. The 5-year survival rate was 100% and 58% in the SD and non-SD groups, respectively. In the SD group, postoperative immunosuppression was significantly lower than in the non-SD group. Two patients died of infection and one died of post-transplant lymphoproliferative disease (PTLD) in the non-SD group, while only one patient died of PTLD 7 years after LDLLT in the SD group. Hematologic malignancy relapsed in two patients in the non-SD group. For the three single LDLLTs in the SD group, immunosuppression was carefully tapered. In our study, LDLLT involving the same donor as for HSCT appeared to have advantages related to lower immunosuppression compared to LDLLT from relatives who were not the HSCT donors.

[Treatment strategy and outcomes of invading apical lung cancer].

Invading apical lung cancers are generally the non-small-cell lung cancers (NSCLCs) which involve the apex of the chest wall. These tumors should be classified into 2 types based on the main location of tumor because of the difference of involved surrounding structures ; (1) the superior sulcus tumor origi nally termed Pancoast tumor which involves posterior region of the apex and (2) the anterior apical tumor which involves anterior region of the apex. Previously, these NSCLCs were considered to be inoperable showing a dismal prognosis. With the development of combined modality therapies for locally advanced NSCLCs, the prognosis of invading apical NSCLCs has been improved, especially since intro duction of the neoadjuvant chemoradiotherapy. Surgical resection for invading apical NSCLCs is 1 of challenging procedures for thoracic surgeons. The point is the anatomical complication of the small apex surrounding vital structures. Several approaches have been developed such as the posterior Paul-son's approach or anterior Masaoka's approach. In particular, the approach from anterior chest has been modified or devised to achieve safe and complete resection of tumors invading anterior structures like subclavian vessels. In this article, we reviewed our 13 cases of invading apical NSCLCs, especially from the view point of surgical approach. Thoracic surgeons should understand the properties of each approach and master them for complete resection avoiding serious complications.

[Second chondrosarcoma of the rib four years after the initial operation; report of a case].

Chondrosarcoma of rib origin is rare accounting for about 2% of all chondrosarcomas. A 63-year-old female with an anterior chest wall tumor was referred to our institution for surgical treatment of a 2nd chondrosarcoma in the right 2nd rib 4 years after the initial surgery for its primary lesion. Computed tomography (CT) showed a low density mass, 36 mm in diameter, arising from the 2nd rib. An extended excision of the chest wall including the tumor was performed followed by the reconstruction of the chest wall with double Marlex Mesh. As she had already undergone the reconstruction of the chest wall for its primary lesion, this reconstruction was her 2nd one. Nevertheless, her respiratory condition was well preserved with no significant chest deformity. Wide excision and reconstruction could be performed for the 2nd arising chondrosarcoma of the rib even after the initial lesion was already widely removed and reconstructed.

[Phrenic nerve paralysis following lung transplantation].

Phrenic nerve paralysis is a well-documented complication of cardiac operation, but there is less commonly reported after lung transplantation. A retrospective study of 49 lung transplantation was done at Okayama University Hospital. Phrenic nerve paralysis (unilateral in 3 patients and bilateral in 1) was found in 4 patients (8.2%). All of these paralyses were transiently recovered. The average length of ventilation, intensive care unit stay and hospitalization for recipients with phrenic nerve paralysis was not significantly longer than the other (no diaphragmatic paralysis) recipients, but there was a tendency to be longer. Diaphragmatic paralysis is most likely related to difficulty in detecting the phrenic nerve caused by adhesions, injury due to dissection, thermal injury by electrocartery, or local topical hypothermia using ice-slush. Therefore, it is important to take care of avoiding the injury of the nerve during the operation.

[Living-donor lobar lung transplantation for infectious lung diseases].

The rate of infection among lung transplant recipients is several times higher than that among recipients of other organs and is most likely related to the exposure of the allograft to the external environment. Meticulous peri-operative management is mandatory in performing living-donor lobar lung transplantation for patients with infectious lung diseases. All 5 patients with end-stage infectious lung diseases are currently alive for 17-104 months after receiving living-donor lobar lung transplantation at Okayama University Hospital.

Relation of interlobar collaterals to radiological heterogeneity in severe emphysema.

BACKGROUND: A study was undertaken to assess the prevalence of interlobar collateral ventilation in patients with severe emphysema to identify factors that may help to predict patients with significant collateral ventilation. METHODS: Between April 2002 and August 2003, ex vivo assessment of the lungs 17 consecutive patients with smoking related severe emphysema was performed. To assess collateral flow, all lobes of explanted specimens were selectively intubated using a wedged cuffed microlaryngeal intubation tube and then manually ventilated using a bagging circuit. Interlobar collateral ventilation was defined as the ability to easily inflate a non-intubated lobe at physiological pressures. Pre-transplant demographic characteristics, physiological data, radiological results, and explant histology were assessed for retrospective relationships with the degree of interlobar collateral ventilation in the explanted lung. RESULTS: A total of 23 lungs were evaluated, 15 of which (66%) had significant collateral interlobar airflow. There were no significant differences in any demographic, physiological, or pathological variables between patients with collateral ventilation and those with no collateral ventilation. However, there was a significant relationship between the presence of interlobar collateral ventilation and radiological scores (p<0.05). CONCLUSIONS: Interlobar collateral ventilation occurs to a much greater extent in patients with radiologically homogeneous emphysema than in those with heterogeneous emphysema. Heterogeneity of emphysema may predict patients with a significantly reduced risk of interlobar collateral ventilation.

Effects of inhaled nitric oxide in a canine living-donor lobar lung transplant model.

OBJECTIVE: In living-donor lobar lung transplantation, early severe graft dysfunction can occur if the size or amount of transplanted lung tissue is insufficient. The purpose of this study was to evaluate the effects of inhaled nitric oxide on early pulmonary function in a canine bilateral living-donor lobar lung transplant model. METHODS: Sixteen pairs of mongrel dogs with a donor/recipient weight ratio less than 1.2 were used. The donor lung bloc was extirpated after heparinization. Right middle, lower and cardiac lobes were implanted as a right lung of the recipient and left lower lobe was implanted as a left lung without cardiopulmonary bypass. In Group 1 (n = 9), nitric oxide gas was administered continuously at a concentration of 40 parts per million prior to reperfusion of the right lung throughout the 6-hour assessment period after transplantation. In Group 2 (n = 7), nitrogen gas was administered in the same manner as nitric oxide, for control. RESULTS: At the end of assessment, the survival rate was 89% (8/9) in Group 1 and 57% (4/7) in Group 2. The arterial oxygen tension in Group 1 was significantly higher than that in Group 2. The pulmonary arterial pressure and pulmonary vascular resistance index were significantly lower in Group 1 than in Group 2. The aortic pressure and cardiac index did not differ significantly between the two groups. The wet-to-dry weight ratio and myeloperoxidase activity were significantly lower in Group 1 than in Group 2. CONCLUSIONS: These data suggested that inhaled nitric oxide improved early pulmonary function in living-donor lobar lung transplantation by vasodilatating the pulmonary vasculature and inhibiting neutrophil activation.