Improved childhood management strategies have decreased the early life morbidity and mortality of severe developmental lung disorders, such that an increasing number of individuals ultimately transition care to adult pulmonologists for management. Alternatively, individuals with milder malformations may present in adulthood or may have an increased risk for the development of more common adult pulmonary comorbidities (eg, COPD, pulmonary hypertension). As such, there is a critical need for adult pulmonologists to understand how developmental lung disorders may impact respiratory morbidity over the lifespan.

The immediate postoperative period after heart surgery poses a substantial risk of life-threatening complications, notably acute pulmonary and cardiac failure. Use of noninvasive ventilation (NIV) may reduce the incidence of pulmonary or heart failure, or both.

Balanced crystalloid solutions may improve clinical outcomes compared with saline for some critically ill adults, but it is unclear whether differences in composition between balanced crystalloid solutions affect outcomes.

Continuous glucose monitors (CGMs) could potentially improve management of hyperglycemia compared with standard point-of-care glucose monitoring for critically ill patients. However, there is limited evidence to support routine use of CGMs in the ICU.

Previous clinical data suggest that the presence of a pleural effusion is associated with poor survival. However, these studies were limited by either a small sample size or lack of an adequate control group.

Preserved ratio impaired spirometry (PRISm) findings are heterogeneous and include restrictive lung disease. Interstitial lung abnormalities (ILAs) may represent early interstitial lung disease. The relationship between PRISm and ILAs is not well understood.

A 21-year-old man with a history of glucose-6-phosphate dehydrogenase deficiency experienced an acute onset of atraumatic left upper abdominal and flank pain. An initial contrast-enhanced CT scan of the chest, abdomen, and pelvis revealed a consolidation in the left lower lobe, a small pleural effusion, and enlarged mesenteric lymph nodes. The patient was prescribed nonsteroidal antiinflammatory drugs for a presumed viral syndrome and was discharged. He returned the next day with increased pain, new onset of shortness of breath, and a fever of 38.6 °C. A diagnosis of community-acquired pneumonia was made, and doxycycline was prescribed. Twenty-four hours later, he presented again with severe pain, worsening dyspnea, and hypoxemia. A CT angiogram of the chest showed persistent consolidation in the left lower lobe and a now large left pleural effusion. He was admitted to the internal medicine service for the management of pneumonia and presumed para-pneumonic effusion.

A 40-year-old man with chronic myeloid leukemia presented to the hospital with recurrent dyspnea and hypoxemic respiratory failure. He presented from his outpatient transplant infectious diseases appointment with dyspnea, cough, worsening hypoxemia, acute kidney injury, and somnolence after discharge from the hospital 2 weeks prior with a similar presentation. During the previous hospital stay, he underwent bronchoscopy and alveolar lavage with negative infectious workup. He was prescribed caspofungin, amphotericin, and continued posaconazole for prior probable invasive fungal infection (elevated blood BD-glucan and pulmonary nodules). Antibiotics included previous meropenem for esophageal nocardiosis, cefepime and azithromycin during admission, and now ceftriaxone for nocardiosis in the outpatient setting for convenience of home infusion. He was prescribed diuretics for presumed volume overload. Despite home diuretics, antimicrobials, and supplemental oxygen, he re-presented with worsening symptoms.

A 56-year-old woman was admitted to our hospital, presenting with a history of recurrent fevers persisting for over 2 months. The febrile episode was self-limiting, accompanied by cough without significant expectoration, with the fever predominantly manifesting during the afternoon and evening hours. Furthermore, there were no concurrent symptoms indicative of chest tightness, wheezing, dyspnea, or hemoptysis. A CT scan in a local hospital demonstrated multifocal lymphadenopathy in the mediastinum and bilateral supraclavicular regions. The patient had neither a history of tobacco usage nor of alcohol consumption. Furthermore, there was no familial history of oncologic conditions. The patient's medical records revealed no evidence of hypertension, diabetes mellitus, coronary artery disease, infectious diseases, or immunologic disorders.

Pulmonary arterial hypertension (PAH) frequently is associated with an imbalance in antiproliferative bone morphogenic protein-2 receptor signaling and proproliferative type-II activin receptor signaling, favoring the latter. Sotatercept is an activin ligand trap that reduces the dominant detrimental activin signaling and provides clinical benefit. We report a patient with heritable PAH in whom sotatercept had neither positive nor negative effects; we relate that fact to his PAH being caused by a previously unreported variant of unknown significance (c.1276T>C, p.[Cys426Arg]) in the GDF2 gene. GDF2 encodes bone morphogenic protein type-9, the presence of which is required for proper functioning of the pulmonary microvasculature. Low levels of functionally active bone morphogenic protein type-9 contribute to PAH. As we enter an era of precision medicine for patients with PAH with increasingly costly therapies, genetic screening may direct appropriate therapy and limit the use of expensive but likely ineffective therapies.

High-flow nasal cannula (HFNC) is a first-line therapy for patients with acute respiratory failure. Despite increased HFNC utilization over recent years-accelerated in part by the COVID-19 pandemic-high-quality evidence to guide HFNC discontinuation is lacking. Decisions about when and how quickly to reduce flow rate, Fio2, or both simultaneously are frequently left to clinicians' discretion without clear guidance on an optimal approach. Failure to de-escalate HFNC support when clinically appropriate has many potential consequences, such as prolongation of ICU/hospital length of stay, increased health care costs, and reduced availability of limited hospital resources. With the goal of improving care efficiency and resource utilization among hospitalized patients with acute respiratory failure, we propose a standardized approach for HFNC discontinuation focused on "liberation" (similar to spontaneous breathing trials for patients undergoing mechanical ventilation), using a stepwise approach guided by physiology.

Effective bidirectional communication is crucial during end-of-life decision-making, which requires clear understanding between clinicians and patients and their family members about treatment options, preferences, and goals of care. For those who have a non-English language preference or who have difficulty speaking, reading, writing, and understanding English, interpreters are essential. However, patients who speak rarer languages, known as languages of lesser diffusion (LLDs), such as Karen, spoken in Thailand and Myanmar, face unique challenges because of limited interpretation resources. In this work, we discuss the case of a Karen-speaking patient admitted to the ICU who lacked decision-making capacity, requiring the involvement of family members who also spoke Karen for a code status discussion. Despite efforts to find an interpreter, no Karen interpreter was available initially, complicating the communication and decision-making about changing the code status to do not resuscitate/do not intubate. A remote Karen interpreter was identified later, allowing for effective communication and clinician assurance that the family did understand the implications of their decision and had made it voluntarily. End-of-life decision-making is complex and challenging, requiring culturally sensitive communication. Patients who speak LLDs face unique difficulties in these discussions compared with those who speak more common languages because of the lack of interpretation resources. The purpose of this case report is to draw attention to these specific challenges and explore ethical concerns when engaging in decision-making conversations with patients and families who speak an LLD.

Pediatric pulmonary hypertension (PH) is a severe incurable disease with a poor prognosis. In pediatric PH, trial design is hampered by the absence of age-appropriate trial end points. This study evaluated physical activity (PA) measured by hip-anchored accelerometry as a potential trial end point in pediatric PH.

The cuff leak test (CLT) is an important tool to assess the risk of upper airway obstruction after extubation.

There is an ongoing debate about whether clearance of Mycobacterium tuberculosis infection occurs and at what magnitude. Recent studies quantifying "uncertainty zones" of interferon-gamma release assays (IGRAs) provide a more stringent estimate of reversion.

Patient-reported outcomes should be considered alongside clinical assessments to guide therapy for COPD.

Millions of people around the world survive critical illness each year only to realize that they and their loved ones are grappling with a new "normal" after hospital discharge for which their medical team may not have adequately prepared them. Up to one-half of all ICU survivors suffer from new or worsening impairments in physical, cognitive, and psychological domains of health that are often not realized until they attempt to re-enter their previous lives. These devastating long-term sequelae of critical illness, collectively described as post-intensive care syndrome (PICS), can carry enormous consequences for an ICU survivor's ability to care for their family, return to work, and regain their previous quality of life for months to years after their inciting illness. Despite mounting research on PICS and survivorship, a knowledge gap exists whereby ICU team members may not always be aware of PICS and may not counsel their patients on the challenges awaiting them after discharge. Understanding how best to communicate these challenges to patients and families is crucial in preparing for survivorship beyond the ICU. In this review, we summarize PICS and possible recovery trajectories of ICU survivors. We then discuss communication strategies, emphasizing the role of empathy. Finally, we provide a suggested framework to handle these crucial conversations. We aim to equip clinicians with the knowledge and framework to care for a patient who has survived critical illness but now faces the possibility of struggles inadequately addressed by our health care system.

In 2019, Korea initiated the world's first national low-dose CT imaging lung cancer screening (LCS) program, adapting the Lung CT Screening Reporting and Data System (Lung-RADS) to counteract the high false-positive rates driven by prevalent TB.

There is limited knowledge about long-term mortality, care pathways, and health-related quality of life (HrQoL) among patients in the ICU receiving prolonged mechanical ventilation (PMV).

Alpha-1-antitrypsin (A1AT) deficiency is a common hereditary disorder associated with increased risk of developing chronic obstructive pulmonary disease (COPD). Many individuals with severe A1AT deficiency go undiagnosed, or are diagnosed late, and fail to benefit from disease-specific counseling and modifying care. Since the 2012 Canadian Thoracic Society (CTS) A1AT deficiency clinical practice guideline, new approaches to optimal diagnosis using modern genetic testing and studies of A1AT augmentation therapy have been published. We performed a systematic review and meta-analysis, which along with expert clinical input, informed recommendations. We conditionally recommend testing for A1AT deficiency in all individuals with COPD at the time of diagnosis, individuals with adult-onset asthma with persistent airway obstruction, and individuals with unexplained bronchiectasis. We suggest genetic testing with DNA sequencing of SERPINA1 gene as the initial test for individuals with high clinical suspicion for A1AT deficiency, and initial measurement of serum A1AT levels in individuals with moderate clinical suspicion of A1AT deficiency, followed by genetic testing with DNA sequencing of SERPINA1 gene if A1AT level is <23 μmol/L (<1.2 g/L). Following identification of an abnormal gene for A1AT in individuals, whether heterozygote or homozygote, we suggest first-degree relatives be provided genetic counseling and offered testing for A1AT deficiency. The panel conditionally recommends A1AT augmentation therapy to patients who do not smoke or who formerly smoked with COPD (forced expiratory volume in 1 s [FEV1] < 80% predicted; associated with emphysema), with documented deficiency genotypes and severely reduced A1AT level (< 11 μmol/L or < 0.57 g/L) in addition to receiving optimal pharmacological and nonpharmacological therapies for COPD.