Panacinar emphysema complicating idiopathic pulmonary hemosiderosis: a case report.

Idiopathic pulmonary hemosiderosis (IPH) is a rare entity with no known underlying etiology. It can be complicated by lung fibrosis. Emphysema is rarely reported as a consequence of IPH. We present a case of a 30-year-old female who presented with recurrent hemoptysis and shortness of breath. Radiographs revealed advanced emphysematous changes of the lower lobes. The diagnosis of IPH was established with an open lung biopsy. She was treated with systemic steroids, underwent bullectomy and was subsequently maintained on inhaled steroids.

Ergonomics of bronchoscopy: good advice or a pain in the neck?

Interventional pulmonologists require a unique set of skills including precise motor abilities and physical endurance, but surprisingly the application of ergonomic principles in the field of bronchoscopy remains limited. This is particularly intriguing when considering the significant impact that poor ergonomics can have on diagnostic aptitude, income potential and overall health. It is therefore imperative to provide comprehensive education to physicians regarding the significance of ergonomics in their work, especially considering the introduction of advanced diagnostic and therapeutic procedures. By implementing simple yet effective measures (e.g. maintaining neutral positions of the wrist, neck and shoulder; adjusting the height of tables and monitors; incorporating scheduled breaks; and engaging in regular exercises), the risk of injuries can be substantially reduced. Moreover, objective tools are readily available to assess ergonomic postures and estimate the likelihood of work-related musculoskeletal injuries. This review aims to evaluate the current literature on the impact of procedure-related musculoskeletal pain on practising pulmonologists and identify modifiable factors for future research.

Airway Injury Caused by Aspiration of Iron Sulfate Pills: A Series of 11 Cases.

It is not widely recognized that iron (ferrous sulfate) pill aspiration causes airway damage. Clinical diagnosis is challenging because patients are often unaware that they have aspirated a pill. The literature on this entity consists mainly of case reports. The aim of this study is to describe the clinical and pathologic features of iron pill aspiration in a series of 11 patients. A retrospective review of our pathology archives was performed to identify cases of iron pill aspiration (2013-2023). All available histologic and cytologic material was rereviewed. Clinical information was collected from the electronic medical record, and imaging studies were rereviewed. Eighteen endobronchial biopsies were identified from 11 patients (7 women and 4 men; mean age, 70 years; range, 44-82 years). Eight patients had corresponding cytology (20 specimens). Medication history was available in 9 of 11 patients, all of whom were taking iron sulfate pills. Two patients reported possible aspiration episodes; 4 had risk factors for aspiration. The diagnosis of iron pill aspiration was suspected prior to biopsy in only 1 case. Histologically, iron pill particles were yellow, golden brown, or gray, were elongated and crystal or fiber like, and stained strongly with an iron stain. Common histologic findings included mucosal ulceration, acute and/or chronic inflammation, fibrosis, and squamous metaplasia. Iron pill particles were also identified in 11 cytology specimens from 6 patients. On Papanicolaou staining, iron pill particles were yellow to golden, fiber like, refractile, and crystalline. Reactive epithelial cells, squamous metaplasia, and acute inflammation were common. The combination of iron pill intake and discolored mucosa on bronchoscopy is a potential clue to the diagnosis of iron pill aspiration. Pathologists should familiarize themselves with the appearance of iron pill particles in endobronchial biopsies and cytology specimens from the respiratory tract as this diagnosis is seldom suspected on clinical grounds, and most patients lack a history of aspiration.

Innovation in rigid bronchoscopy-past, present, and future.

German laryngologist Gustav Killian performed the first "Direkte Bronchoskopie" using a rigid bronchoscope to extract a foreign airway body from the right main bronchus over a hundred years ago, transforming the practice of respiratory medicine. The procedure instantaneously became popular throughout the world. Chevalier Jackson Sr from the United States further advanced the instrument, technique, safety, and application. In the 1960s, Professors Harold H. Hopkins and N.S. Kapany introduced optical rods as well as fiberoptics that led Karl Storz to develop the cold light system improving endoluminal illumination, achievements that ushered in the modern era of flexible endoscopy. Several diagnostic and therapeutic procedures became possible such as transbronchial needle biopsy, transbronchial lung biopsy, airway electrosurgery, or cryotherapy. Dr. Jean-François Dumon from France advanced the use of Nd-YAG laser in the endobronchial tree and created the dedicated Dumon silicone stent introducing the whole new field of interventional pulmonology (IP). This major milestone revitalized interest in rigid bronchoscopy (RB). Now, advancements are being made in stenting, instrumentation, and education. RB robotic technology advancements are currently anticipated and can potentially revolutionize the practice of pulmonary medicine. In this review, we describe some of the most substantial advances related to RB from its beginning to the modern era.

Forbearance with endobronchial stenting: cognisance before conviction.

Endobronchial stenting is an important aspect of the practice of interventional pulmonology. The most common indication for stenting is the management of clinically significant airway stenosis. The list of endobronchial stents available on the market continues to grow. More recently, patient-specific 3D-printed airway stents have been approved for use. Airway stenting should be considered only when all other options have been exhausted. Due to the environment of the airways and the stent-airway wall interactions, stent-related complications are common. Although stents can be placed in various clinical scenarios, they should only be placed in scenarios with proven clinical benefit. The unwarranted placement of a stent can expose the patient to complications with little or no clinical benefit. This article reviews and outlines the key principles of endobronchial stenting and important clinical scenarios in which stenting should be avoided.

Lung Transplant After a Heart Transplant: A Case Report.

A heart-lung transplant is considered in patients with end-stage heart and lung disease. However, there is no report of a patient receiving a staged heart transplant followed by a lung transplant. Our case report describes a successful left single lung transplant for idiopathic pulmonary fibrosis 6 years after a heart transplant. This case illustrates that this approach can avoid significantly increased wait time until transplant, and it shows that early interstitial lung disease may not be a contraindication for the heart transplant.

Lung Imaging in COPD Part 2: Emerging Concepts.

The diagnosis, prognostication, and differentiation of phenotypes of COPD can be facilitated by CT scan imaging of the chest. CT scan imaging of the chest is a prerequisite for lung volume reduction surgery and lung transplantation. Quantitative analysis can be used to evaluate extent of disease progression. Evolving imaging techniques include micro-CT scan, ultra-high-resolution and photon-counting CT scan imaging, and MRI. Potential advantages of these newer techniques include improved resolution, prediction of reversibility, and obviation of radiation exposure. This article discusses important emerging techniques in imaging patients with COPD. The clinical usefulness of these emerging techniques as they stand today are tabulated for the benefit of the practicing pulmonologist.

Infection control in the bronchoscopy suite: effective reprocessing and disinfection of reusable bronchoscopes.

PURPOSE OF REVIEW: With advancements in technology, flexible bronchoscopes have become thinner in diameter and in need of more thorough reprocessing to prevent infection transmission than ever before. Many experienced bronchoscopists are not aware of the critical steps involved in effective bronchoscope reprocessing and we hope to bridge this gap by describing this process in detail. RECENT FINDINGS: Bronchoscope reprocessing includes several distinct steps (precleaning, leak testing, manual cleaning, visual inspection, terminal reprocessing, rinsing and drying). Each step is comprehensive and needs to be carried out systematically by trained personnel. Failure of any step can lead to serious downstream events such as outbreaks and pseudo-outbreaks. Some experts now recommend sterilization when feasible, although high-level disinfection remains the minimum standard. We also will review some literature on the utility of borescopes, automated endoscope reprocessors and disposable bronchoscopes. SUMMARY: Our article will focus on the most recent recommendations for effective reprocessing and disinfection of reusable bronchoscopes.

Acute Dyspnea After Lung Transplantation: A Twisted Tale.

CASE PRESENTATION: A 67-year-old woman developed sudden-onset severe dyspnea 24 h after a bilateral sequential lung transplant for COPD. She had an uneventful surgery performed on cardiopulmonary bypass support. She required two units of packed RBCs as well as fresh frozen plasma during the surgery. She was successfully extubated within 12 h of surgery. Her primary graft dysfunction score was grade 2, 1, and 1 at 0, 12, and 24 h, respectively. Approximately 24 h after the procedure, she complained of increasing shortness of breath without fever, chills, cough, or pleuritic chest pain.

Airway complications following lung transplantation.

Airway complications post lung transplant account for significant morbidity (between 2 and 18%) and mortality (2 to 4%). The commonly encountered airway complications include necrosis and dehiscence, exophytic granulation tissue, bronchial stenosis, tracheo-broncho-malacia, bronchial fistulae, and airway infections. With growing experience in surveillance bronchoscopy post lung transplant and availability of advanced endobronchial interventional therapies, better management of lung transplant recipients is now possible. In this article, we review the various contributing factors, clinical manifestations, diagnostic modalities, and treatment options for post lung transplant airway complications.

Twenty years of the Fabry Outcome Survey (FOS): insights, achievements, and lessons learned from a global patient registry.

BACKGROUND: Patient registries provide long-term, real-world evidence that aids the understanding of the natural history and progression of disease, and the effects of treatment on large patient populations with rare diseases. The year 2021 marks the 20th anniversary of the Fabry Outcome Survey (FOS), an international, multicenter, observational registry (NCT03289065). The primary aims of FOS are to broaden the understanding of Fabry disease (FD), an X-linked lysosomal storage disorder, and to improve the clinical management of affected patients. Here, we review the history of FOS and the analyses and publications disseminated from the registry, and we discuss the contributions FOS studies have made in understanding FD. RESULTS: FOS was initiated in April 2001 and, as of January 2021, 4484 patients with a confirmed diagnosis and patient informed consent have been enrolled from 144 centers across 26 countries. Data from FOS have been published in nearly 60 manuscripts on a wide variety of topics relevant to FD. Analyses of FOS data have investigated the long-term effectiveness and safety of enzyme replacement therapy (ERT) with agalsidase alfa and its effects on morbidity and mortality, as well as the benefits of prompt and early treatment with agalsidase alfa on the progression of cardiomyopathy and the decline in renal function associated with FD. Based on analyses of FOS data, ERT with agalsidase alfa has also been shown to improve additional signs and symptoms of FD experienced by patients. FOS data analyses have provided a better understanding of the natural history of FD and the specific populations of women, children, and the elderly, and have provided practical tools for the study of FD. FOS has also provided methodology and criteria for assessing disease severity which contributed to the continuous development of medical practice in FD and has largely improved our understanding of the challenges and needs of long-term data collection in rare diseases, aiding in future rare disease real-world evidence studies. CONCLUSION: FOS over the last 20 years has substantially increased the scientific knowledge around improved patient management of FD and continues to expand our understanding of this rare disease.

Prospective cohort of cryobiopsy in interstitial lung diseases: a single center experience.

RATIONALE: Transbronchial cryobiopsy has been increasingly used to diagnose interstitial lung diseases. However, there is uncertainty regarding its accuracy and risks, mainly due to a paucity of prospective or randomized trials comparing cryobiopsy to surgical biopsy. OBJECTIVES: To evaluate the diagnostic yield and complications of cryobiopsy in patients selected by multidisciplinary discussion. METHODS: This was a prospective cohort from 2017 to 2019. We included consecutive patients with suspected interstitial lung diseases being considered for lung biopsy presented at our multidisciplinary meeting. MEASUREMENTS AND MAIN RESULTS: Of 112 patients, we recommended no biopsy in 31, transbronchial forceps biopsy in 16, cryobiopsy in 54 and surgical biopsy in 11. By the end of the study, 34 patients had had cryobiopsy and 24 patients, surgical biopsy. Overall pathologic and multidisciplinary diagnostic yield of cryobiopsy was 47.1% and 61.8%, respectively. The yield increased over time for both pathologic (year 1: 28.6%, year 2: 54.5%, year 3: 66.7%, p = 0.161) and multidisciplinary (year 1: 50%, year 2: 63.6%, year 3: 77.8%, p = 0.412) diagnosis. Overall rate of grade 4 bleeding after cryobiopsy was 11.8%. Cryobiopsy required less chest tube placement (11.8% vs 100%, p < 0.001) and less hospitalizations compared to surgical biopsy (26.5% vs 95.7%, p < 0.001), but hospitalized patients had a longer median hospital stay (2 days vs 1 day, p = 0.004). CONCLUSIONS: Diagnostic yield of cryobiopsy increased over time but the overall grade 4 bleeding rate was 11.8%.

Giant Cell Interstitial Pneumonia In Native, Transplanted And Re-Transplanted Lungs 8 Years Apart Without Known Hard Metal Exposure.

Pneumoconioses are a group of non-neoplastic pulmonary disorders caused by inhaled inorganic particles. Well-described pneumoconioses include asbestosis, silicosis, coal worker's pneumoconiosis, chronic beryllium disease, and hard metal lung disease. Giant cell interstitial pneumonia (GIP) is a distinctive and rare pneumoconiosis most frequently found in workers exposed to hard metals, primarily cobalt and tungsten carbide. The pathologic picture is considered virtually pathognomonic for hard metal lung disease, although this dogma has been questioned by a few reports of giant cell interstitial pneumonia in patients without apparent hard metal exposure. Giant cell interstitial pneumonia is even rarer in lung transplant recipients. Here, we present a patient without known hard metal exposure who was found to have persistent giant cell interstitial pneumonia in native, transplanted and re-transplanted lungs 8 years apart.

Early postoperative complications in lung transplant recipients.

Lung transplantation has become an established therapy for end-stage lung diseases. Early postoperative complications can impact immediate, mid-term, and long-term outcomes. Appropriate management, prevention, and early detection of these early postoperative complications can improve the overall transplant course. In this review, we highlight the incidence, detection, and management of these early postoperative complications in lung transplant recipients.

A Blueprint for Success: A Multidisciplinary Approach to Clinical Operations Within a Bronchoscopy Suite.

Building an efficient facility for advanced bronchoscopic procedures involves many considerations. This review places particular emphasis on anesthesiology services, based on experience at a tertiary/quaternary care referral academic medical center. Topics include equipment requirements, applicable clinical standards, and multidisciplinary collaboration. Patient flow arrangements for both outpatients and inpatients, from preoperative care to discharge/disposition, are highlighted. The importance of effective business planning, personnel training, leadership, communication, team building, quality of care, and patient safety are also discussed.

In-depth phenotyping for clinical stratification of Gaucher disease.

BACKGROUND: The Gaucher Investigative Therapy Evaluation is a national clinical cohort of 250 patients aged 5-87 years with Gaucher disease in the United Kingdom-an ultra-rare genetic disorder. To inform clinical decision-making and improve pathophysiological understanding, we characterized the course of Gaucher disease and explored the influence of costly innovative medication and other interventions. Retrospective and prospective clinical, laboratory and radiological information including molecular analysis of the GBA1 gene and comprising > 2500 variables were collected systematically into a relational database with banking of collated biological samples in a central bioresource. Data for deep phenotyping and life-quality evaluation, including skeletal, visceral, haematological and neurological manifestations were recorded for a median of 17.3 years; the skeletal and neurological manifestations are the main focus of this study. RESULTS: At baseline, 223 of the 250 patients were classified as type 1 Gaucher disease. Skeletal manifestations occurred in most patients in the cohort (131 of 201 specifically reported bone pain). Symptomatic osteonecrosis and fragility fractures occurred respectively in 76 and 37 of all 250 patients and the first osseous events occurred significantly earlier in those with neuronopathic disease. Intensive phenotyping in a subgroup of 40 patients originally considered to have only systemic features, revealed neurological involvement in 18: two had Parkinson disease and 16 had clinical signs compatible with neuronopathic Gaucher disease-indicating a greater than expected prevalence of neurological features. Analysis of longitudinal real-world data enabled Gaucher disease to be stratified with respect to advanced therapies and splenectomy. Splenectomy was associated with an increased hazard of fragility fractures, in addition to osteonecrosis and orthopaedic surgery; there were marked gender differences in fracture risk over time since splenectomy. Skeletal disease was a heavy burden of illness, especially where access to specific therapy was delayed and in patients requiring orthopaedic surgery. CONCLUSION: Gaucher disease has been explored using real-world data obtained in an era of therapeutic transformation. Introduction of advanced therapies and repeated longitudinal measures enabled this heterogeneous condition to be stratified into obvious clinical endotypes. The study reveals diverse and changing phenotypic manifestations with systemic, skeletal and neurological disease as inter-related sources of disability.

Safety of Laser and Thermal Therapy During Rigid Bronchoscopy Using Manual Hand Jet Ventilation.

INTRODUCTION: Thermal ablative therapies (laser, radiofrequency ablation, electrocautery, argon plasma coagulation) are often used during rigid bronchoscopy for the treatment of central airway obstructions (CAO). An airway fire is a feared complication that can occur during endobronchial thermal ablation. MATERIALS AND METHODS: This was a single-center, retrospective, observational study. A total of 175 patients were reviewed undergoing rigid bronchoscopy in the operating room and bronchoscopy suite requiring manual hand jet ventilation and thermal therapy between September 2014 and September 2018. The study objective was to determine the safety of manual hand jet ventilation during endobronchial thermal therapies with rigid bronchoscopy. RESULTS: Over a five-year period, 175 patients underwent endobronchial thermal therapy during rigid bronchoscopy with manual hand jet ventilation for the treatment CAOs. Immediately prior to thermal therapy activation, jet ventilation was paused. No incidences (0/175) of airway fires occurred despite immediate delivery of thermal energy following a jet ventilation hold. CONCLUSIONS: Results of our study show that performing thermal ablative therapy during rigid bronchoscopy with jet ventilation using a breath-hold technique is safe.

Recycling plastic: diagnosis and management of plastic bronchitis among adults.

Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even life-threatening airway obstruction. Plastic bronchitis in adulthood should not be overlooked, particularly in patients with chronic inflammatory lung diseases. This review presents current understanding of the presentation, aetiology, pathogenesis, pathology and management of plastic bronchitis in adults.

Predicting Lymph Node Metastasis in Non-small Cell Lung Cancer: Prospective External and Temporal Validation of the HAL and HOMER Models.

BACKGROUND: Two models, the Help with the Assessment of Adenopathy in Lung cancer (HAL) and Help with Oncologic Mediastinal Evaluation for Radiation (HOMER), were recently developed to estimate the probability of nodal disease in patients with non-small cell lung cancer (NSCLC) as determined by endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA). The objective of this study was to prospectively externally validate both models at multiple centers. RESEARCH QUESTION: Are the HAL and HOMER models valid across multiple centers? STUDY DESIGN AND METHODS: This multicenter prospective observational cohort study enrolled consecutive patients with PET-CT clinical-radiographic stages T1-3, N0-3, M0 NSCLC undergoing EBUS-TBNA staging. HOMER was used to predict the probability of N0 vs N1 vs N2 or N3 (N2|3) disease, and HAL was used to predict the probability of N2|3 (vs N0 or N1) disease. Model discrimination was assessed using the area under the receiver operating characteristics curve (ROC-AUC), and calibration was assessed using the Brier score, calibration plots, and the Hosmer-Lemeshow test. RESULTS: Thirteen centers enrolled 1,799 patients. HAL and HOMER demonstrated good discrimination: HAL ROC-AUC = 0.873 (95%CI, 0.856-0.891) and HOMER ROC-AUC = 0.837 (95%CI, 0.814-0.859) for predicting N1 disease or higher (N1|2|3) and 0.876 (95%CI, 0.855-0.897) for predicting N2|3 disease. Brier scores were 0.117 and 0.349, respectively. Calibration plots demonstrated good calibration for both models. For HAL, the difference between forecast and observed probability of N2|3 disease was +0.012; for HOMER, the difference for N1|2|3 was -0.018 and for N2|3 was +0.002. The Hosmer-Lemeshow test was significant for both models (P = .034 and .002), indicating a small but statistically significant calibration error. INTERPRETATION: HAL and HOMER demonstrated good discrimination and calibration in multiple centers. Although calibration error was present, the magnitude of the error is small, such that the models are informative.