Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome.

BACKGROUND: STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. METHOD: Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. RESULT: Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. CONCLUSION: Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.

Experimental replacement of esophagus with a short segment of trachea.

BACKGROUND: Segmental resection of esophagus with primary anastomosis is prohibited because of the risk of dehiscence. We previously have shown that replacement of a segment of cervical esophagus with a tracheal segment of the same length could successfully be performed in a canine model. In this study, we sought to assess the feasibility of replacement of the esophageal defect with a shorter segment of trachea. METHODS: In five mongrel dogs weighting 20-30 kg, under general anesthesia and after a cervical incision, 8 cm of the cervical esophagus was resected and replaced by a 4-cm segment of the adjacent trachea. The animals were evaluated clinically for signs and symptoms of stenosis and dehiscence and then euthanized after 2 mo of follow-up. RESULTS: All dogs recovered from surgery and started regular diet on the seventh postoperative day. No clinical or endoscopic sign of stenosis or voice change was seen. Squamous metaplasia and atrophy of mucosal glands and cartilage were detected in the histopathologic examination of the replaced segments. CONCLUSIONS: Replacement of a cervical esophageal defect with a shorter segment of trachea can be performed successfully in dogs. This procedure can be potentially used for the treatment of cervical esophageal lesions in humans.

Surgical Interventions in Coronavirus Disease 2019 Pleural and Pulmonary Complications: A Case Series of the Tertiary Thoracic Surgery Center Experience.

Background: In most cases of pulmonary or pleural post-coronavirus disease 2019 (COVID-19) complications, surgical interventions are performed to treat these complications, but the method of the surgery and its outcome in these patients is not clearly defined. We present 40 patients with pulmonary and pleural complications after COVID-19 who required surgical intervention. Patients and Methods: In this case series, patients' data were prospectively collected from April to August 2022 at Masih Daneshvari Hospital. Inclusion criteria were patients with COVID-19 who were referred to the thoracic surgery department because of pleural effusion, pneumothorax, empyema, infected or non-infected pneumatocele, and lung cavity with suspected fungal infections. The required intervention for each patient was assessed. Results: Patients' mean age was 49.21 ± 11.5 (30-69 years). Nine patients (22.5%) were female. Pure pleural effusion was reported in five (12.5 %), pneumothorax in eight (20%), empyema in 29 (72.5%), and infected pneumatocele in two patients (5%). Twelve patients had bronchial fistulas that were clarified at the time of surgery that needed repair after resection. In 13 patients (32.5%) because of pleural effusion or pneumothorax, a chest tube was inserted and after two weeks lungs were fully expanded. All patients with pneumothorax were managed by chest tube initially but in the presence of continuous air leakage and non-expanding lungs surgical thoracotomy or video-assisted thoracoscopic surgery (VATS) were considered for correction. In 10 patients who required thoracotomy, the chest tube was necessary for more than one month. In most of the patients, there were small cystic lesions or peripheral bronchopleural fistula. In 17 (42.5%) cases of empyema, necrotic pneumonia was documented and eight patients (20%) had aspergillus infection in the pathology report and two patients had a pulmonary abscess. Conclusions: Pleural COVID-19 complications can be treated with conventional surgical methods such as chest tube insertion, and debridement of infected tissue with no mortality and further complications.

Reconstruction of Chest Wall by Cryopreserved Sternum Allograft After Resection of Sternal Hemangioma: A Case Report.

A 5-year-old girl was referred to our department for a mass of sternum that was previously biopsied and diagnosed as hemangioma. Chest X-ray and CT scan confirmed a large sternal mass. We resected the sternum completely and reconstructed a large anterior chest wall defect by a cryopreserved sternal allograft. In the follow-up of the patient, there was no instability of the chest wall and acceptable cosmetic results.

Evaluation of clinical status, diagnosis, treatment and radiological findings of pulmonary hydatid cyst: 5-years' experience at tertiary lung center.

BACKGROUND: Hydatidosis is one of the most critical worldwide parasitic zoonotic diseases. The lung is the second most common site of hydatidosis. This study aimed to evaluate the clinical status, diagnosis, treatment, and radiological findings of pulmonary hydatid cyst in patients referred to tertiary lung center. METHODS: From April 2014 to July 2019, patients referred to Masih Daneshvari University Medical Center with the impression of alveolar hydatidosis included. Demographic data of 304 patients were collected including clinical symptoms, laboratory studies, radiological findings, location of the lung involvement, and cyst characteristics. Also, surgical procedures, medical treatments, and post-operative complications were recorded. RESULTS: Pulmonary hydatidosis was confirmed for 234 patients. 55% of patients were males with the mean age of 45.1±16.6 years. The most common symptoms were cough (59.8%), dyspnea (31.1%), and hemoptysis (26%). Left lung, right lung, and bilateral involvement were reported in 40.1%, 55.1%, and 4.8% of cases, respectively. Cyst perforation (39.8%) was the most common intra-operative finding. Surgical interventions included thoracotomy, rigid bronchoscopy, cyst aspiration, and enucleation. The liver was the most concomitant organ involved due to pulmonary hydatidosis (16.6%). The most common postoperative complication was atelectasis, with the rate of 35.7%. 52.2% of patients were discharged within 10 days after surgery. No mortality was reported. CONCLUSION: Sometimes atypical findings in different imaging modalities make the hydatid cyst diagnosis challenging. Although lobe involvement more than 50% has the indication for lobectomy, we conserved lobes with about 70% involvement in our institution, and patients had no postoperative complications.

COVID-19 after Thoracotomy in Patients with Pulmonary Hydatidosis.

Hydatidosis is one of the most important parasitic and zoonotic endemic infections caused by the larvae of cestode Echinococcus granulosus. Co-infection of hydatid cyst with the coronavirus disease 2019 (COVID-19), which is caused by the severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2), has been previously reported. The mortality rate of hydatidosis is reported to be 2-4% and the liver and lungs are the two most commonly involved organs, respectively. In the present study, we have reported two recovered pulmonary hydatidosis patients who were infected with SARS-CoV-2 after thoracotomy in the hospital. In general, current cases suggest that patients with thoracic surgery are more likely to develop severe infection with severe acute respiratory syndrome coronavirus 2 (SARS-COV-2). The patients presented COVID-19 symptoms shortly after thoracotomy and their viral tests were confirmed with the positive result of SARS-CoV-2 RT-PCR. In conclusion, possible differential diagnoses should be considered in similar cases and adequate attention should be paid to intraoperative and postoperative care.

T790M and Acquired Resistance of Epidermal Growth Factor Receptor to Tyrosine Kinase Inhibitors in Patients with Lung Adenocarcinoma.

Background: Activating mutations in the epidermal growth factor receptor (EGFR) are initially responsive to tyrosine kinase inhibitors (TKIs), but responses to TKIs is not permanent and drug resistance eventually happens for almost all patients. Subsequent studies found different resistance mechanisms, among which (EGFR) T790M mutation is the most important mechanism of TKI treatment failure. Using cell-free DNA (cfDNA) is a new way for diagnosing resistance mutations in EGFR. The aim of present study is to determine cfDNA-identified recurrence mutation rate and their association with clinical outcome in lung Adenocarcinoma patients. Materials and Methods: Patients who were diagnosed with metastatic adenocarcinoma of the lung and acquired resistance to TKIs were enrolled. The incidence of T790M positivity, overall survival (OS) and median duration of TKI treatment before progression was calculated. Polymerase chain reaction (PCR) and sequencing were used to identify the T790M mutation in cfDNA. Results: The incidence of T790M mutations was higher in men, younger cases (<59 years), in patients with L858R primary mutation and never smokers although they were not significantly different (P-values= 041, 0.316, 0.316 and 0.158, respectively). There was significant longer OS in the Del19 subgroup than the L858R subgroup (p = 0.014). In multivariable analysis, significant longer OS was associated with younger age (<59 years) and primary EGFR mutation exon 19 (P-values= 0.028 and 0.050, respectively). Conclusion: T790M mutations frequency may differ by ethnicity, genetic factors and EGFR primary mutations. Detecting T790M mutations in plasma is considered as an indicator of treatment with third generation EGFR-TKIs.

Uncommon Complications of Lung Transplantation in a Referral Center.

Background: Many efforts were made to determine the uncommon clinical complications after lung transplantation and treatment options to tackle them; however, many of these rare complications have not been mentioned in recent publications. Evaluating and recording adverse effects after organ transplantation can significantly prevent post-transplant mortality. This study aimed to examine rejection factors by examining individuals undergoing lung transplantation surgery. Materials and Methods: In a prospective longitudinal study, we followed up on complications of 60 lung recipients post lung-transplantation surgery for six years from 2010 to 2018. All complications were recorded in follow-up visits or hospital admissions during these years. Finally, the patients' information was categorized and evaluated by designing a questionnaire. Results: From a total of 60 transplant recipients, from 2010 to 2018, 58 patients were initially enrolled in our study, but two were lost to follow-up. Uncommon complications witnessed in the post-transplantation period included endogenous endophthalmitis, herpetic keratitis, duodenal strongyloidiasis, intestinal cryptosporidiosis, myocardial infarction, diaphragm dysfunction, Chylothorax, thyroid nodule, and necrotizing pancreatitis. Conclusion: Meticulous postoperative surveillance is crucial for managing lung transplant patients for early detection and treatment of common and uncommon complications. Therefore, it is necessary to establish procedures for assessing the patients' constancy until complete recovery.

Clinical whole-exome sequencing analysis reveals a novel missense COL11A1 mutation resulting in an 18-week Iranian male aborted fetus with Fibrochondrogenesis 1: A case report.

Fibrochondrogenesis 1, an autosomal recessive syndrome, is a rare disease that causes short-limbed skeletal dysplasia. Mutations in the gene encoding the α1 chain of type XI collagen (COL11A1) are seen to be the main cause of this disease. We present an 18-week Iranian male aborted fetus with Fibrochondrogenesis 1 from consanguineous parents. Whole-exome sequencing revealed a novel missense variant from G to A in exon 45 of 68 in the COL11A1 gene (NM_080629.2: c.3440G > A, [p.G1147E, g.103404625]). The mutation was confirmed by Sanger sequencing and further, MutationTaster predicted this variant to be disease-causing. Bioinformatic analysis suggests that this variant is highly conserved in both nucleotide and protein levels, suggesting that it has an important function in the proper role of COL11A1 protein. In silico analysis suggests that this mutation alters the COL11A1 protein structure through a Glycine to Glutamic acid substitution.

Thoracotomy for Extracting Grass Inflorescence Aspiration: a Case Report.

Following aspiration of grass inflorescence, it often migrates to the peripheral part of the airway due to its unique shape. It often causes complications like chronic pneumonia, recurrent asthmatic attacks, lung abscess, recurrent hemoptysis, and bronchiectasis that may necessitate surgical intervention. In this case, we have reported a 13-year-old boy with grass inflorescence aspiration. The bronchoscopic procedure failed to remove the foreign body, so he underwent thoracotomy.

Relapse of Lymphangioleiomyomatosis Five Years after Bilateral-Lung Transplantation.

Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease principally affecting women during childbearing years and eventually leading to progressive respiratory failure. Lung transplantation is a viable option for patients with end-stage disease. LAM-related complications remain common, but recurrence of LAM following allograft transplantation is rare. We present a 25-year-old woman who presented with progressive dyspnea five years after bilateral lung transplantation for end-stage LAM. Histological examination of transbronchial lung biopsy sample confirmed recurrent LAM. We changed cyclosporine to sirolimus and she is currently being considered for re-transplantation.

Primary Erlotinib Resistance in a Patient with Non-Small Cell Lung Cancer Carrying Simultaneous Compound EGFR L718A, Q849H, and L858R Mutations.

Nowadays, mutations in the epidermal growth factor receptor (EGFR) kinase domain are studied in targeted therapy of non-small cell lung cancer (NSCLC) with EGFR tyrosine kinase inhibitors including gefitinib and erlotinib. The present study reports a rare case of a patient harboring three simultaneous EGFR mutations (L718A, Q849H, and L858R). The development of erlotinib resistance was detected in the subsequent treatment. Using a computational approach, the current study investigated the conformational changes of wild-type and mutant EGFR's kinase domains in the interaction with erlotinib. Their binding modes with erlotinib were elucidated during molecular dynamics simulation, where higher fluctuations were detected in the mutated forms of the EGFR tyrosine kinase domain. Prediction of stability and functional effect of mutations revealed that amino acidic substitutions have decreased the protein stability as well as the binding affinity to erlotinib. These results may be useful for a recommendation of EGFR mutational analysis for patients with NSCLC carcinoma.

Postintubation Multisegmental Tracheal Stenosis: A 24-Year Experience.

BACKGROUND: Management of multisegmental tracheal stenosis is challenging. In this 24-year longitudinal single-center study, we present an algorithmic treatment approach. METHODS: A retrospective analysis of 2167 patients with postintubation tracheal stenosis indicated 83 (3.83%) patients with multisegmental tracheal stenosis. Patients were assigned to 4 management groups according to the length, location, and severity of stenoses; tracheal infection/mucositis; laryngeal function; symptoms; general condition; and comorbid diseases. Type 1 (n = 13): 1-stage resection of both strictures, Type 2 (n = 6): 2-stage resection of both strictures, Type 3 (n = 40): resection of 1 stricture and nonresectional management of the other, Type 4 (n = 24): nonresectional management of both strictures. Outcomes were categorized as Good, Acceptable, or Poor. Univariate analyses for factors predictive of recurrence and outcome were performed. RESULTS: Follow-ups were completed in 70 (84.34%) patients (median, 22.5 months). Outcome was assessed as Good in 56 (82.35%), Acceptable in 10 (14.71%), Poor in 2 (2.94%), and mortality in 2 (2.94%) patients. The median length of airway resection was 46, 67.5, and 40 mm in Types 1-3, respectively. Only 11 (13.25%) patients had no history of tracheostomy or tracheal surgery. By univariate analysis, a shorter intubation period was associated with Good outcome (P = .017). No factors predictive of recurrence or outcome were ascertained. CONCLUSIONS: Multisegmental tracheal stenosis, generally caused by performing an inappropriate tracheostomy, is an iatrogenic disease that can be prevented. Although resection of both strictures may be feasible and is associated with Good results, in the majority of cases, a combination of surgical resection and non-resectional methods are sufficient to achieve Good results.

Idiopathic Subglottic Tracheal Stenosis in Identical Twin Sisters.

INTRODUCTION: Idiopathic subglottic tracheal stenosis is a rare inflammatory disease of the trachea; most commonly affects females within the age range of 20-50 years. No etiologic factor has yet been identified for this rare tracheal disease and therefore it should be diagnosed after the exclusion of other inflammatory, traumatic, and autoimmune diseases of the trachea. The familial or genetic predisposition to this disease is still unknown although one published report in the literature showed some familial predisposition. CASE REPORT: A 41-year old woman presented with progressive dyspnea and stridor. The bronchoscopic evaluation revealed subglottic tracheal stenosis; however, there was no significant etiology of this disease after complete evaluations. Therefore, the idiopathic subglottic stenosis was the final diagnosis. After two years, her identical twin sister presented with the same signs and symptoms. There was also no etiology for her tracheal stenosis. The first patient was managed surgically through cricotracheal resection. However, the second sister didn't need surgical resection due to the mild to moderate tracheal stenosis. CONCLUSION: The obtained results of our cases along with the previously reported family cases can potentiate the hypothesis that there is some genetic predisposition to the development of this disease.

Abdominal Compartment Syndrome in Critically Ill Patients.

BACKGROUND: Abdominal compartment syndrome patients suffer severe obstacles such as kidney failure and shock. To evade further complications, identifying the abdominal compartment syndrome (ACS) and Intra-abdominal hypertension (IAH), in critically ill individuals and hospitalised in the intensive care unit (ICU) is obligated. AIM: The current study intended to study the abdominal compartment syndrome and the concomitant risk factors among hospitalised patients in ICU, by using the Intra-abdominal pressure test. MATERIAL AND METHODS: One hundred and twenty-five hospitalised patients at ICU entered the current survey. Abdominal pressure was measured by standard intravesical technique. The SPSS 21 analysed the preoperative and intraoperative factors such as demographic records and comorbidities. RESULTS: Seventy-three (58.4%) participants were males and 52 (41.6%) were women in the mean age of 55.1 ± 18.3 years. Eighty-nine patients (71.2%) showed normal intra-abdominal pressure since 31 patients (24.8%), and 5 patients (4%) developed IAH and ACS. The intra-abdominal pressure (IAP) applied to Glasgow Coma Scale (GCS), Acute Physiology, shock, Systemic Inflammatory Response Syndrome (SIRS), central venous oxygen saturation and Chronic Health Evaluation (APACHE II) score (P < 0.05). Patients with high IAP have shown a higher mortality frequency, compared to others (P < 0.05). CONCLUSION: Current findings showed a correlation between IAP hospitalised patients in ICU and shock, SIRS, APACHE II, central venous oxygen saturation and GCS. Intra-abdominal pressure test, as a valuable prognosis test for the abdominal compartment syndrome (ACS) and Intra-abdominal hypertension (IAH), may offer better results when added to the routine medical checkup of ICU patients.

ECMO-assisted resection of huge thoracic mass.

Some advanced thoracic malignancy cannot be resected safely by using of conventional ventilation, so some sort of cardiopulmonary support is needed for hemodynamic and ventilation management of the patient. Using extracorporeal membrane oxygenation (ECMO) in comparing with cardiopulmonary bypass has some advantages. Three patients with huge thoracic tumors with different ages experienced major surgery in our center by using ECMO in order to face major complications mainly due to the size of mass to achieve better hemostatic stabilities, lower bleeding, and injuries to main airways and secure oxygenation. This is the first case series in Iran, as our best knowledge that explains cases of huge chest mass which were operated perfectly by using ECMO and short ICU stay and interestingly no major complications.

Fatal Outcome of Ruptured Pulmonary Hydatid Cyst.

Most authors believe that the optimal treatment for pulmonary hydatid cyst is surgery. Albendazole has been used as a prophylactic measure for reducing recurrence rate but there are some controversies about this strategy. Some researchers have described the increased risk of spontaneous rupture of cysts following albendazole treatment. In this case report, we present a case of spontaneous rupture of pulmonary hydatid cyst with fatal outcome that may be the adverse cause of albendazole.

Reconstruction of Chest Wall by Cryopreserved Sternal Allograft after Resection of Aneurysmal Bone Cyst of Sternum.

A 20-year-old female was referred to our hospital due to deformity and bulging in anterior aspect of chest wall in sternal area. Chest X-ray and CT scan confirmed a large mass with destruction of sternum. Pathologic diagnosis after incisional biopsy was compatible with aneurysmal bone cyst. We resected sternum completely and reconstructed large anterior defect by a cryopreserved sternal allograft. In follow-up of patient there was no unstability of chest wall with good cosmetic result.

Right Ventricular Perforation with the Body of Swan-Ganz Catheter during Lung Transplantation by ECMO Support: A Case Report.

A 46-year-old woman with a 12-year history of lymphangiomyomatosis (LAM) was admitted for lung transplantation in January 2017. We decided to apply veno-arterial extracorporeal membrane oxygenation (ECMO) to manage arrhythmia and hypotension during lung transplantation, since it was not controllable with inotropic drugs. After transplanting the right (first) lung and at the time of left pneumonectomy, the body of the Swan-Ganz catheter was suddenly observed to be protruding from the right ventricular (RV) wall. The catheter was found folded at part of its body and ran out 0.5 cm from the RV. The protruding part of the catheter was inserted before the perforated part of the cardiac muscle was repaired in order to control the bleeding. ECMO was used throughout the rest of the procedure and the patient was transferred to the intensive care unit (ICU) in good condition before being weaned from the ventilator after 16 hours. It seems that gentle manipulation, concurrent use of transesophageal echocardiography (TEE), insertion of the appropriate length of the catheter into the heart chambers, and a softer material in the structure of the catheters would be helpful to prevent these kinds of potentially fatal complications.

Potential circulating miRNA signature for early detection of NSCLC.

Circulating microRNAs (c-miRNAs) are promising biomarkers for screening, early detection and prognosis of cancer. The purpose of this investigation was to identify a panel of c-miRNAs in plasma that could contribute to early detection of non-small cell lung cancer (NSCLC). We profiled the expression of 44 unique plasma miRNAs in training set of 34 NSCLC patients and 20 matched healthy individuals by miRCURY LNA™ Universal RT microRNA PCR Panel and calculated dysregulation fold changes using the 2-ΔΔCt equation. Selected plasma miRNAs were then validated by SYBR green q-RT PCR using an independent validation set of plasma samples from NSCLC patients (n: 72) and NC (n: 50). In the validation set, the receiver operating characteristic (ROC) curves were generated for four miRNAs. In the training set, 17 miRNAs were significantly up-regulated and nine were down-regulated in the plasma from NSCLC patients versus matched normal controls. Four miRNAs (miR-21, miR-328, miR-375 and miR-141) were selected for validating their diagnostic value in the testing set. ROC plot analysis showed that a high specificity (98%) and sensitivity (82.7%) in miR-141 in comparing early NSCLC patient and controls. So among these four plasma miRNAs only miR-141 could be promising biomarkers for early detection of NSCLC. In addition to, we found a significant positive correlation between stage and miR-21 expression level (95% CI: 0.687-0.863; p-value < 0.0001). Considering the accessibility and stability of circulating miRNAs, plasma miR-141 is a useful biomarker early detection of NSCLC as a supplement in future screening studies.