OBJECTIVETo recognize the clinical features of the bronchiolitis obliterans.

METHODClinical manifestation, chest X-ray, computed tomography (CT) and pulmonary function of 4 cases with bronchiolitis obliterans were retrospectively analyzed.

RESULTTwo cases were after Stevens-Johnson syndrome (SJS), the other 2 were after severe pneumonia, including one suffered from adenovirus pneumonia. Cough, tachypnea and wheezing persisted in all the 4 patients. The symptoms lasted for at least 6 weeks, in one case for over one year. Crackles and wheezing were present in all the 4 cases. Hyperinflation was seen in chest radiographs in all cases. On pulmonary CT/high-resolution CT (HRCT), patchy opacity and bronchial wall thickening were seen in each patient. Areas of air trapping were seen in three cases. Bronchiectasis was seen in 2 cases, atelectasis and mosaic perfusion were seen respectively in one case. PO(2) was low in all the four cases. Wheezing was not responsive to beta(2) agonist and other bronchodilating therapy. Prednisone was used at a dose of 1 mg/(kg.d) in 3 cases. Two cases were followed up for 3 months. The clinical condition of one case was improved, whose wheezing and bronchiolar constriction disappeared, cough and dyspnea were also relieved. However, the condition of one patient was not improved, although the wheezing disappeared. The HRCT of these two cases showed no improvement.

CONCLUSIONClinical symptoms of BO were cough, tachypnea, and wheezing after acute lung injury. Crackles and wheezing were the most common signs in the BO. Chest radiographs showed hyperinflation. Pulmonary CT showed bronchial wall thickening, bronchiectasis, atelectasis, and mosaic perfusion. Pulmonary function tests suggested obstruction of small airway.

Bronchiolitis Obliterans ; etiology ; pathology ; physiopathology ; Child ; Child, Preschool ; Humans ; Infant ; Male ; Pneumonia ; complications ; Pneumonia, Viral ; complications ; Prognosis ; Respiratory Function Tests ; Stevens-Johnson Syndrome ; complications ; Tomography, X-Ray Computed

OBJECTIVE: Critically ill patients with solid tumors complicated with paraneoplastic pemphigus are usually treated in intensive care units (ICU) for perioperative management after surgical treatment. In this study, the clinical characteristics and predictors of long-term prognosis of these critically ill patients were analyzed. METHODS: the clinical and laboratory data of 63 patients with solid tumors complicated with paraneoplastic pemphigus admitted to ICU from 2005 to 2020 were retrospectively analyzed, and the survival status of the patients were followed up. RESULTS: Among the 63 patients, 79.4% had Castleman disease as the primary tumor, and 20.6% with other pathological types; 69.8% had severe-extensive skin lesions, and 30.2% had other skin lesions; the patients with bronchiolitis obliterans accounted for 44.4%, and 55.6% were not merged. Postoperative fungal infection occurred in 23.8% of the patients, and 76.2% without fungal infection. The median follow-up time was 95 months, and 25 patients died during the study period. The 1-year, 3-year and 5-year survival rates were 74.6% (95%CI 63.8%-85.4%), 67.4% (95%CI 55.6%-79.2%) and 55.1% (95%CI 47.9%-62.3%), respectively. The log-rank univariate analysis showed that the patients had age>40 years (P=0.042), preoperative weight loss>5 kg (P=0.002), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.002), and perioperative fungal infection (P < 0.001) had increased mortality. Cox univariate analysis showed that preoperative weight loss >5 kg (P=0.005), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.009), preoperative bacterial pulmonary infection (P=0.007), prolonged surgical time (P=0.048), postoperative oxygenation index (P=0.012) and low albumin (P=0.010) and hemoglobin concentration (P=0.035) in ICU, acute physiology and chronic health evaluation (APACHE Ⅱ) score (P=0.001); sequential organ failure assessment (SOFA) score (P=0.010), and postoperative fungal infection (P < 0.001) were risk factors for long-term survival. Cox regression model for multivariate analysis showed that preoperative weight loss > 5 kg (HR 4.44; 95%CI 1.47-13.38; P=0.008), and preoperative albumin < 30 g/L (HR 4.38; 95%CI 1.72-11.12; P=0.002), bronchiolitis obliterans (HR 2.69; 95%CI 1.12-6.50; P=0.027), and postoperative fungal infection (HR 4.85; 95%CI 2.01-11.72; P < 0.001) were independent risk factors for postoperative mortality. CONCLUSION The 5-year survival rate of critically ill patients undergoing surgery for paraneoplastic pemphigus combined with solid tumors is approximately 55.1%, with preoperative weight loss > 5 kg, albumin < 30 g/L, bronchiolitis obliterans and postoperative fungal infection were associated with an increased risk of near- and long-term postoperative mortality.
Adult ; Albumins/therapeutic use* ; Bronchiolitis Obliterans/pathology* ; Critical Illness ; Hemoglobins ; Humans ; Neoplasms/complications* ; Paraneoplastic Syndromes/pathology* ; Pemphigus/drug therapy* ; Retrospective Studies ; Weight Loss

A case of inflammatory pseudotumor of the lung occurring in a six-year-old boy is reported with clinicopathologic findings, including its ultrastructure. The patient had had frequent upper respiratory tract infections, and one and half year before the discovery of the lung mass, he suffered from pneumonia of the right lung, which was serologically proven to be a mycoplasma pneumoniae infection. Exploratory thoracotomy revealed a large mediastinal mass that was removed together with the right middle and lower lobes of the lung. The mass arose from the lung with an endobronchial element. Microscopically, the mass was composed of a variety of inflammatory and mesenchymal cells, including plasma cells, histiocytes, lymphocytes, and fibroblast-like spindle cells. Ultrastructurally, the spindle-shaped mesenchymal cells were either fibroblasts or myofibroblasts. At the time of diagnosis of the inflammatory pseudotumor of the lung, the serum titer of antimycoplasma antibody rose again, and the lung parenchyma adjacent to the mass showed interstitial pneumonia with features of bronchiolitis obliterans. The present case suggests that the inflammatory pseudotumor of the lung could be a postinflammatory lesion associated with mycoplasma pneumoniae infection.
Antibodies, Bacterial/blood ; Bronchiolitis Obliterans/complications ; Child ; Diagnosis, Differential ; Fibroma/etiology/*pathology/surgery ; Humans ; Lung Neoplasms/etiology/*pathology/surgery ; Male ; Mycoplasma pneumoniae/immunology/*pathogenicity ; Pneumonia, Mycoplasma/complications/microbiology/*pathology ; Tomography, X-Ray Computed

BACKGROUND: Heart-lung transplantation (HLT) has provided hope to patients with end-stage lung disease and irreversible heart dysfunction. We reviewed the clinical outcomes of 10 patients who underwent heart-lung transplantation at Asan Medical Center. METHODS: Between July 2010 and August 2014, a total of 11 patients underwent HLT at Asan Medical Center. After excluding one patient who underwent concomitant liver transplantation, 10 patients were enrolled in our study. We reviewed the demographics of the donors and the recipients' baseline information, survival rate, cause of death, and postoperative complications. All patients underwent follow-up, with a mean duration of 26.1±16.7 months. RESULTS: Early death occurred in two patients (20%) due to septic shock. Late death occurred in three patients (38%) due to bronchiolitis obliterans (n=2) and septic shock (n=1), although these patients survived for 22, 28, and 42 months, respectively. The actuarial survival rates at one year, two years, and three years after HLT were 80%, 67%, and 53%, respectively. CONCLUSION: HLT is a procedure that is rarely performed in Korea, even in medical centers with large heart and lung transplant programs. In order to achieve acceptable clinical outcomes, it is critical to carefully choose the donor and the recipient and to be certain that all aspects of the transplant procedure are planned in advance with the greatest care.
Bronchiolitis Obliterans ; Cause of Death ; Chungcheongnam-do ; Demography ; Follow-Up Studies ; Heart ; Heart-Lung Transplantation* ; Hope ; Humans ; Korea ; Liver Transplantation ; Lung ; Lung Diseases ; Mortality ; Postoperative Complications ; Shock, Septic ; Survival Rate ; Tissue Donors

Anti-Inflammatory Agents ; therapeutic use ; Bronchi ; pathology ; Bronchiolitis Obliterans ; pathology ; Humans ; Lung ; pathology ; Male ; Methylprednisolone ; therapeutic use ; Middle Aged ; Pneumonia ; pathology ; Pulmonary Fibrosis ; complications ; drug therapy ; pathology ; Respiratory Distress Syndrome, Adult ; etiology

Objective: To investigate the early effect and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with a 10-day decitabine-containing conditioning regimen in the treatment of acute myeloid leukemia (AML) /myelodysplastic syndrome (MDS) . Methods: From April 2021 to May 2022, 31 AML/MDS patients who received allo-HSCT with a 10-day decitabine-containing conditioning regimen were analyzed. Results: AML (n=10), MDS-AML (n=6), CMML-AML (n=1), and MDS (n=14) were identified in 31 patients, 16 males, and 15 females, with a median age of 41 (20-55) yr. Neutrophils and platelets were successfully implanted in 31 patients (100%), with a median implantation duration of 12 (9-30) and 14 (9-42) days, respectively. During the preconditioning period, 16 patients (51.6%) developed oral mucositis, with 15 cases of Ⅰ/Ⅱ grade (48.4%) and one case of Ⅲ grade (3.2%). After transplantation, 13 patients (41.9%) developed CMV viremia, six patients (19.4%) developed hemorrhagic cystitis, and four patients (12.9%) developed a local infection. The median time of acute graft versus host disease (aGVHD) following transplantation was 33 (12-111) days. The cumulative incidence of aGVHD and Ⅲ/Ⅳ grade aGVHD was 41.9% (95% CI 26.9%-61.0%) and 22.9% (95% CI 13.5%-47.5%), respectively. There was no severe cGVHD, and mild and moderate chronic GVHD (cGVHD) incidence was 23.5% (95% CI 12.1%-43.6%). As of November 30, 2022, only one of the 31 patients had relapsed, with a 1-yr cumulative relapse rate (CIR) of 3.2% (95% CI 0.5%-20.7%). There was only one relapse patient death and no non-relapse deaths. The 1-yr overall survival (OS) and disease-free survival (DFS) rates were 92.9% (95% CI 80.3%-100%) and 96.8% (95% CI 90.8%-100%), respectively. Conclusions: A 10-day decitabine-containing conditioning regimen for allo-HSCT reduced relapse and was safe and feasible in treating AML/MDS.
Male ; Female ; Humans ; Decitabine ; Myelodysplastic Syndromes/therapy* ; Leukemia, Myeloid, Acute/complications* ; Disease-Free Survival ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Recurrence ; Chronic Disease ; Graft vs Host Disease/etiology* ; Transplantation Conditioning/adverse effects* ; Bronchiolitis Obliterans Syndrome ; Retrospective Studies

We report a case of bronchiolitis obliterans associated with Stevens-Johnson syndrome. A 59-year-old man presented with respiratory distress that gradually worsened over 3 months. He had been diagnosed with Stevens-Johnson syndrome 3 months before admission. He had no history of previous airway disease. On physical examination, expiratory breathing sounds were not audible, and a chest X-ray revealed a hyperinflated lung. A pulmonary function test indicated a severe obstructive pattern. Computed tomography scans of inspiratory and expiratory phases of respiration showed oligemia and air trapping, and both were more prominent on expiration view than on inspiration view. The pathogenesis of bronchiolitis obliterans associated with Stevens-Johnson syndrome is largely unknown.
Anti-Bacterial Agents/therapeutic use ; Bronchiolitis Obliterans/etiology/*radiography/therapy ; Bronchoscopy ; Dyspnea/*complications ; Fatal Outcome ; Humans ; Male ; Middle Aged ; Radiography, Thoracic ; Respiratory Distress Syndrome, Adult/*etiology/therapy ; Respiratory Function Tests ; Roxithromycin/therapeutic use ; Stevens-Johnson Syndrome/*complications/drug therapy ; Tomography, X-Ray Computed/methods ; Tracheostomy

OBJECTIVETo establish an animal model of obliterative bronchiolitis (OB) after lung transplantation and investigate the pathogenesis preliminarily.

METHODSTracheal segments (5 cartilaginous rings each) were transplanted from SD rats to SD rats (Group I) or to Wistar rats (Group II and III). Grafts were implanted into an abdominal cavity and wrapped in the omentum. Animals in Group I and II did not receive CsA, animals in Group III received CsA daily by gastro-tube at 10 mg.kg(-1).d(-1) from beginning to end. Grafts were harvested on day 3, 14, 28 after transplantation as representative time points for 3 phases of injury in the evolution of allograft airway obliteration, then examined histological changes and gene expression of T-helper 1-and T-helper 2-type cytokines [Th1: interleukin-2 (IL-2), interferon-gamma (IFN-gamma); Th2: interleukin-4 (IL-4), interleukin-10 (IL-10)] in grafts. At the same time, effects of CsA were observed on the above-mentioned indices.

RESULTSThere was no significant difference in histological changes on day 3 after transplantation among 3 groups (P > 0.05). Tracheas in Group I approached to normal morphology on day 14 after transplantation. Airway epithelium of Group II and III almost lost completely on day 14 after transplantation. There was no significant difference between Group II and Group III (P > 0.05), but there were significant differences between Group I and Group II or Group III. The cross-sectional area of the tracheal lumen was narrowed by approximately (5.0 +/- 1.2)%, (28.5 +/- 5.0)% and (19.4 +/- 2.9)% respectively on day 14 after transplantation in Group I, II and III, there were significant differences among 3 groups. On day 14 after transplantation, tracheas in Group I revealed few lymphocytic infiltration, but it showed dense lymphocytic infiltration in Group II. Tracheas in Group III have much more lymphocyte infiltration than that in Group I, but much less than that in Group II. There were significant differences among 3 groups, too (P < 0.01). The tracheal lumen revealed almost total luminal obstruction (94.8 +/- 3.6)% on day 28 after transplantation in Group II. The cross-sectional area of the tracheal lumen was narrowed by approximately (3.7 +/- 0.8)% and (36.6 +/- 7.6)% respectively in Group I and III on day 28. There were significant differences among 3 groups (P < 0.01). Compared with that on day 14, lymphocytic infiltration had decreased gradually on day 28 in Group II and III. There were significant differences among 3 groups all the same (P < 0.01). In Group II, expression of IL-2, IFN-gamma, IL-4, and IL-10 were much higher than that in Group I. Expression of Th1 cytokines was increased to a greater extent than that of Th2 cytokines in Group II compared with Group I. Allografts in Group III expressed significantly less IL-2 gene transcripts than that in Group II over all the points. There was no significant difference between Group II and III in IFN-gamma, IL-4, and IL-10 gene expression.

CONCLUSIONSCompared with isografts, allografts have more obvious changes, such as epithelial damage, fibroproliferation and lymphocytic infiltration. Th1 and Th2 lymphocyte subtypes contribute to the development of obliterative bronchiolitis in heterotopic trachea transplant model of rat, and changes of their cytokines gene expression may be involved in the pathogenesis. CsA could reduce the development of fibroproliferation and lymphocyte infiltration markedly, but it could not protect airway epithelium. CsA inhibits IL-2 gene transcripts, so it can reduce development of the pathologic lesion of obliterative bronchiolitis to a certain degree.

Abdominal Cavity ; surgery ; Animals ; Bronchiolitis Obliterans ; etiology ; pathology ; prevention & control ; Cyclosporine ; pharmacology ; Disease Models, Animal ; Gene Expression ; Immunosuppressive Agents ; pharmacology ; Interferon-gamma ; genetics ; Interleukin-10 ; genetics ; Interleukin-2 ; genetics ; Interleukin-4 ; genetics ; Lung Transplantation ; adverse effects ; methods ; Postoperative Complications ; etiology ; pathology ; prevention & control ; Rats ; Rats, Sprague-Dawley ; Rats, Wistar ; Reverse Transcriptase Polymerase Chain Reaction ; Trachea ; metabolism ; pathology ; transplantation ; Transplantation, Homologous