The majority of connective tissue diseases (CTDs) are multisystem disorders that are often heterogeneous in their presentation and do not have a single laboratory, histologic, or radiologic feature that is defined as the gold standard to support a specific diagnosis. Given this challenging situation, the diagnosis of CTD is a process that requires the synthesis of multidisciplinary data which may include patient clinical symptoms, serologic evaluation, laboratory testing, and imaging. Pulmonary manifestations of connective tissue disease include interstitial lung disease as well as multicompartmental manifestations. These CT imaging patterns and features of specific diseases will be discussed in this article.

UNASSIGNED: Exercise-induced pulmonary hemorrhage (EIPH) is one of the most commonly diagnosed disorders in racehorses. Many EIPH risk factors such as breed, age, high or low environmental temperature, and distance of the race have been studied in racehorses.
UNASSIGNED: The aim of this study was to study the relationship between EIPH and the presence of jugular vein thrombose in racehorses.
UNASSIGNED: Forty-eight thoroughbred racehorses randomly selected from animals with exercise intolerance due to respiratory disorders were enrolled in the present study. Clinical and tracheobronchoscopy examinations were done for EIPH grading. In addition, both jugular veins were examined using ultrasonography for vein thrombosis.
UNASSIGNED: It was noted during endoscopy that many cases suffered from laryngeal paralysis, and we were not able to assess the degree of laryngeal paralysis under sedation. About 40% of horses with exercise intolerance suffered from EIPH of varying degrees. Most cases of jugular vein thrombosis were of the chronic type, as local heat and pain were not observed. About 42% of the exercise-intolerant horses had jugular vein thrombose with most jugular vein thrombosis on the left side. Combined jugular veins thrombose and EIPH were found in about 25% of exercise intolerance horses, while 17% showed jugular vein thrombose without EIPH, and 41% showed no EIPH with the absence of jugular vein thrombose.
UNASSIGNED: The present study revealed that jugular vein thrombosis may cause disorders-associated damage to the vessels and anatomical structures close to it, such as the trachea causing EIPH.

In this paper, we report the case of a boy in early childhood who presented with iron-deficiency anaemia, initially thought to be nutritional, who had a subsequent diagnosis of idiopathic pulmonary haemosiderosis (IPH). This is a slowly progressive and life-threatening disorder and is of paramount importance that this is identified early and treated appropriately. His first chest CT was not typical for IPH, and this appearance should be highlighted (small cystic changes alone initially). He also had focal disease, which allowed us to make the diagnosis using CT-guided biopsy. During his treatment, he experienced an uncommon side effect to a commonly prescribed medication (bradycardia with methylprednisolone). Since starting azathioprine as a steroid-sparing agent, he has been doing well.

In recent years, lung US has evolved from a marginal tool to an integral component of diagnostic chest imaging. Contrast-enhanced US (CEUS) can improve routine gray-scale imaging of the lung and chest, particularly in diagnosis of peripheral lung diseases (PLDs). Although an underused tool in many centers, and despite inherent limitations in evaluation of central lung disease caused by high acoustic impedance between air and soft tissues, lung CEUS has emerged as a valuable tool in diagnosis of PLDs. Owing to the dual arterial supply to the lungs via pulmonary and bronchial (systemic) arteries, different enhancement patterns can be observed at lung CEUS, thereby enabling accurate differential diagnoses in various PLDs. Lung CEUS also assists in identifying patients who may benefit from complementary diagnostic tests, including image-guided percutaneous biopsy. Moreover, lung CEUS-guided percutaneous biopsy has shown feasibility in accessible subpleural lesions, enabling higher histopathologic performance without significantly increasing either imaging time or expenses compared with conventional US. The authors discuss the technique of and basic normal and pathologic findings at conventional lung US, followed by a more detailed discussion of lung CEUS applications, emphasizing specific aspects of pulmonary physiology, basic concepts in lung US enhancement, and the most commonly encountered enhancement patterns of different PLDs. Finally, they discuss the benefits of lung CEUS in planning and guidance of US-guided lung biopsy. ©RSNA, 2024 Supplemental material is available for this article.

BACKGROUND: The PI*S variant is one of the most prevalent mutations within alpha-1 antitrypsin deficiency (AATD). The risk of developing AATD-related lung disease in individuals with the PI*SS genotype is poorly defined despite its substantial prevalence. Our study aimed to characterize this genotype and its risk for lung disease and compare it with the PI*ZZ and PI*SZ genotypes using data from the European Alpha-1 antitrypsin Deficiency Research Collaboration international registry.
METHODS: Demographic, clinical, functional, and quality of life (QoL) parameters were assessed to compare the PI*SS characteristics with the PI*SZ and PI*ZZ controls. A propensity score with 1:3 nearest-neighbour matching was performed for the most important confounding variables.
RESULTS: The study included 1007 individuals, with PI*SS (n = 56; 5.6%), PI*ZZ (n = 578; 57.4%) and PI*SZ (n = 373; 37.0%). The PI*SS population consisted of 58.9% men, with a mean age of 59.2 years and a mean FEV1(% predicted) of 83.4%. Compared to PI*ZZ individuals they had less frequent lung disease (71.4% vs. 82.2%, p = 0.037), COPD (41.4% vs. 60%, p = 0.002), and emphysema (23.2% vs. 51.9%, p < 0.001) and better preserved lung function, fewer exacerbations, lower level of dyspnoea, and better QoL. In contrast, no significant differences were found in the prevalence of lung diseases between PI*SS and PI*SZ, or lung function parameters, exacerbations, dyspnoea, or QoL.
CONCLUSIONS: We found that, as expected, the risk of lung disease associated with the PI*SS genotype is significantly lower compared with PI*ZZ, but does not differ from that observed in PI*SZ individuals, despite having higher serum AAT levels.
BACKGROUND: www.
RESULTS: gov (ID: NCT04180319).

BACKGROUND: Chronic lung disease is a major cause of morbidity in African children with HIV infection; however, the microbial determinants of HIV-associated chronic lung disease (HCLD) remain poorly understood. We conducted a case-control study to investigate the prevalence and densities of respiratory microbes among pneumococcal conjugate vaccine (PCV)-naive children with (HCLD +) and without HCLD (HCLD-) established on antiretroviral treatment (ART).
METHODS: Nasopharyngeal swabs collected from HCLD + (defined as forced-expiratory-volume/second < -1.0 without reversibility postbronchodilation) and age-, site-, and duration-of-ART-matched HCLD- participants aged between 6-19 years enrolled in Zimbabwe and Malawi (BREATHE trial-NCT02426112) were tested for 94 pneumococcal serotypes together with twelve bacteria, including Streptococcus pneumoniae (SP), Staphylococcus aureus (SA), Haemophilus influenzae (HI), Moraxella catarrhalis (MC), and eight viruses, including human rhinovirus (HRV), respiratory syncytial virus A or B, and human metapneumovirus, using nanofluidic qPCR (Standard BioTools formerly known as Fluidigm). Fisher\'s exact test and logistic regression analysis were used for between-group comparisons and risk factors associated with common respiratory microbes, respectively.
RESULTS: A total of 345 participants (287 HCLD + , 58 HCLD-; median age, 15.5 years [IQR = 12.8-18], females, 52%) were included in the final analysis. The prevalence of SP (40%[116/287] vs. 21%[12/58], p = 0.005) and HRV (7%[21/287] vs. 0%[0/58], p = 0.032) were higher in HCLD + participants compared to HCLD- participants. Of the participants positive for SP (116 HCLD + & 12 HCLD-), 66% [85/128] had non-PCV-13 serotypes detected. Overall, PCV-13 serotypes (4, 19A, 19F: 16% [7/43] each) and NVT 13 and 21 (9% [8/85] each) predominated. The densities of HI (2 × 104 genomic equivalents [GE/ml] vs. 3 × 102 GE/ml, p = 0.006) and MC (1 × 104 GE/ml vs. 1 × 103 GE/ml, p = 0.031) were higher in HCLD + compared to HCLD-. Bacterial codetection (≥ any 2 bacteria) was higher in the HCLD + group (36% [114/287] vs. (19% [11/58]), (p = 0.014), with SP and HI codetection (HCLD + : 30% [86/287] vs. HCLD-: 12% [7/58], p = 0.005) predominating. Viruses (predominantly HRV) were detected only in HCLD + participants. Lastly, participants with a history of previous tuberculosis treatment were more likely to carry SP (adjusted odds ratio (aOR): 1.9 [1.1 -3.2], p = 0.021) or HI (aOR: 2.0 [1.2 - 3.3], p = 0.011), while those who used ART for ≥ 2 years were less likely to carry HI (aOR: 0.3 [0.1 - 0.8], p = 0.005) and MC (aOR: 0.4 [0.1 - 0.9], p = 0.039).
CONCLUSIONS: Children with HCLD + were more likely to be colonized by SP and HRV and had higher HI and MC bacterial loads in their nasopharynx. The role of SP, HI, and HRV in the pathogenesis of CLD, including how they influence the risk of acute exacerbations, should be studied further.
BACKGROUND: The BREATHE trial (ClinicalTrials.gov Identifier: NCT02426112 , registered date: 24 April 2015).

暂无摘要。

UNASSIGNED: Automated machine learning (autoML) removes technical and technological barriers to building artificial intelligence models. We aimed to summarise the clinical applications of autoML, assess the capabilities of utilised platforms, evaluate the quality of the evidence trialling autoML, and gauge the performance of autoML platforms relative to conventionally developed models, as well as each other.
UNASSIGNED: This review adhered to a prospectively registered protocol (PROSPERO identifier CRD42022344427). The Cochrane Library, Embase, MEDLINE and Scopus were searched from inception to 11 July 2022. Two researchers screened abstracts and full texts, extracted data and conducted quality assessment. Disagreement was resolved through discussion and if required, arbitration by a third researcher.
UNASSIGNED: There were 26 distinct autoML platforms featured in 82 studies. Brain and lung disease were the most common fields of study of 22 specialties. AutoML exhibited variable performance: area under the receiver operator characteristic curve (AUCROC) 0.35-1.00, F1-score 0.16-0.99, area under the precision-recall curve (AUPRC) 0.51-1.00. AutoML exhibited the highest AUCROC in 75.6% trials; the highest F1-score in 42.3% trials; and the highest AUPRC in 83.3% trials. In autoML platform comparisons, AutoPrognosis and Amazon Rekognition performed strongest with unstructured and structured data, respectively. Quality of reporting was poor, with a median DECIDE-AI score of 14 of 27.
UNASSIGNED: A myriad of autoML platforms have been applied in a variety of clinical contexts. The performance of autoML compares well to bespoke computational and clinical benchmarks. Further work is required to improve the quality of validation studies. AutoML may facilitate a transition to data-centric development, and integration with large language models may enable AI to build itself to fulfil user-defined goals.

暂无摘要。

BACKGROUND: Pulmonary hamartomas are benign lung lesions. Histopathologically, pulmonary hamartoma is composed of varying amounts of mesenchymal elements, including chondroid tissue, mature adipose tissue, fibrous stroma, smooth muscle, and entrapped respiratory epithelium. Most pulmonary hamartoma cases are asymptomatic and found incidentally during imaging. They usually appear as well-circumscribed lesions with the largest dimension of less than 4 cm. Asymptomatic giant pulmonary hamartomas that more than 8 cm are rare.
METHODS: In the current case report, a 12.0 × 9.5 × 7.5 cm lung mass was incidentally noticed in a 59-year-old female during a heart disease workup. Grossly, the lesion was lobulated with pearly white to tan-white solid cut surface and small cystic areas. Microscopically, representative tumor sections demonstrate a chondromyxoid appearance with relatively hypocellular stroma and entrapped respiratory epithelium at the periphery. No significant atypia is noted. No mitosis is noted, and the proliferative index is very low (< 1%) per Ki-67 immunohistochemistry. Mature adipose tissue is easily identifiable in many areas. Histomorphology is consistent with pulmonary hamartoma. A sarcoma-targeted gene fusion panel was further applied to this case. Combined evaluation of microscopic examination and sarcoma-targeted gene fusion panel results excluded malignant sarcomatous transformation in this case. The mediastinal and hilar lymph nodes are histologically benign. After surgery, the patient had an uneventful postoperative period.
CONCLUSIONS: Giant pulmonary hamartoma is rare; our case is an example of a huge hamartoma in an asymptomatic patient. The size of this tumor is concerning. Thus, careful and comprehensive examination of the lesion is required for the correct diagnosis and to rule out co-existent malignancy.