Gender differences in association between expiratory dynamic airway collapse and severity of obstructive sleep apnea.

OBJECTIVES: Repetitive unbalances and tensions generated by inspiratory efforts against an obstructive upper airway during sleep predispose the development of expiratory central airway collapse. In addition, structures of the upper airway between men and women have differences and could be the reasons for differences in obstructive sleep apnea (OSA) prevalence between genders. The present study aimed to evaluate the association between parameters of expiratory dynamic tracheal collapse measured using chest multidetector CT and objectively measured OSA severity between men and women. MATERIALS AND METHODS: A total of 901 participants who underwent chest CT and overnight in-home polysomnography from the Korean Genome and Epidemiology Study were cross-sectionally analyzed (women: 46.2%). The participants were divided into three groups based on OSA severity by apnea-hypopnea index (AHI). Multivariate linear regression analysis was performed to determine the effects of central airway collapse after adjustment for cardiovascular-related covariates. RESULTS: In a multivariate analysis, percentages of expiratory lumen structure reductions involving area, diameter, and perimeter were associated with AHI (all p values < 0.05) and with OSA severity (moderate-to-severe OSA than no OSA: ß = 3.30%, p = 0.03; ß = 2.05%, p = 0.02; ß = 1.97%, p = 0.02, respectively) in women, whereas men had only a greater percentage of expiratory wall thickness reduction in moderate-to-severe OSA than no OSA (ß = 0.72%, p = 0.003). In addition, women with both mild OSA and moderate-to-severe OSA had higher expiratory tracheal collapse than men without OSA, and a moderate effect of sex was observed (p for interaction = 0.007). CONCLUSION: The expiratory dynamic tracheal collapse was independently associated with severity of OSA in women than in men. CLINICAL RELEVANCE STATEMENT: Differences of pharyngeal structures and inherent features of airways by genders may affect the dissimilarities in vulnerability to sleep apnea between men and women. KEY POINTS: • The expiratory dynamic tracheal collapse was independently associated with severity of OSA in women than in men. • Women with over mild OSA had higher expiratory tracheal collapse than men without OSA, and moderate effect of sex was observed. • Structural differences of airway may affect differences in susceptibility of sleep apnea between genders.

Association of pericardial adipose tissue with left ventricular structure and function: a region-specific effect?

BACKGROUND: The independent role of pericardial adipose tissue (PAT) as an ectopic fat associated with cardiovascular disease (CVD) remains controversial. This study aimed to determine whether PAT is associated with left ventricular (LV) structure and function independent of other markers of general obesity. METHODS: We studied 2471 participants (50.9 % women) without known CVD from the Korean Genome Epidemiology Study, who underwent 2D-echocardiography with tissue Doppler imaging (TDI) and computed tomography measurement for PAT. RESULTS: Study participants with more PAT were more likely to be men and had higher cardiometabolic indices, including blood pressure, glucose, and cholesterol levels (all P < 0.001). Greater pericardial fat levels across quartiles of PAT were associated with increased LV mass index and left atrial volume index (all P < 0.001) and decreased systolic (P = 0.015) and early diastolic (P < 0.001) TDI velocities, except for LV ejection fraction. These associations remained after a multivariable-adjusted model for traditional CV risk factors and persisted even after additional adjustment for general adiposity measures, such as waist circumference and body mass index. PAT was also the only obesity index independently associated with systolic TDI velocity (P < 0.001). CONCLUSIONS: PAT was associated with subclinical LV structural and functional deterioration, and these associations were independent of and stronger than with general and abdominal obesity measures.

Nitrates administered by spray versus tablet: comparison of coronary vasodilation on CT angiography.

OBJECTIVES: To compare the coronary vasodilation effects of spray with those induced by tablet administration in coronary CT angiography (CCTA). METHODS: A total of 2024 patients who underwent CCTA were identified for this retrospective study, including 828 patients with spray (spray group) and 1169 with tablets (tablet group). Of these, 93 patients underwent CCTA at least twice using both spray and tablets. The number of measurable segments and diameters of all 18 segments was measured. The number of measurable segments was compared between groups. RESULTS: No statistically significant differences were evident between these two groups in terms of clinical characteristics. All coronary segments except the ramus intermedius (RI) and left posterior descending artery (L-PDA) were significantly larger in the spray group than in the tablet group (all p < 0.001). In peripheral and branch vessels, as well as in central and main coronary arteries, the diameters were significantly larger in the spray group than in the tablet group (all p < 0.001). Although not always statistically significant, all coronary segments tended to be more measurable on CCTA with spray than with tablet. In the subgroup that underwent CCTA twice using both spray and tablets, all coronary segments except the RI, obtuse marginal artery 2 (OM2), and L-PDA were significantly larger in the spray group than in the tablet group (all p < 0.05). CONCLUSION: Lingual isosorbide dinitrate (ISDN) spray was more efficacious than sublingual nitroglycerin (NTG) tablets in coronary vasodilation for CCTA. Therefore, lingual ISDN spray should be preferred over sublingual NTG tablets for CCTA. KEY POINTS: • Lingual ISDN spray was more efficacious than sublingual NTG tablet for coronary vasodilation in coronary CT angiography, even in elderly patients. • The diameters of all coronary segments except RI and L-PDA were significantly larger, and there were significantly more coronary segments greater than 1.5 mm, except RI and L-PDA, in the spray group than in the tablet group in the whole study group. • Even in peripheral and branch vessels, the diameters of coronary arteries were significantly larger in the spray group than in the tablet group, and they were also larger in elderly patients.

Paravertebral Muscles as Indexes of Sarcopenia and Sarcopenic Obesity: Comparison With Imaging and Muscle Function Indexes and Impact on Cardiovascular and Metabolic Disorders.

OBJECTIVE. The objectives of this study were to propose the use of the cross-sectional area of paravertebral muscle (PMA) and the ratio of the PMA to the cross-sectional area of visceral fat (PVR) as new indexes of sarcopenia or sarcopenic obesity through comparison with existing indexes and to show the clinical associations of PMA and PVR with hypertension and diabetes. SUBJECTS AND METHODS. A total of 1270 participants (608 men and 662 women; mean [± SD] age, 63.57 ± 6.94 years) were recruited from a community-based population of elderly individuals. PMA and PVR were measured on single-slice abdominal CT images. Pearson correlation was used to evaluate the correlation of PMA and PVR with widely used imaging and muscle function indexes of sarcopenia and sarcopenic obesity. Tertile categories of PMA and PVR were evaluated to investigate associations with risks for hypertension and diabetes in men and women, by use of separate multivariable logistic regression models. RESULTS. PMA was correlated with the cross-sectional area of thigh muscle on CT, appendicular skeletal muscle mass (ASM) on dual-energy x-ray absorptiometry, height-adjusted ASM (calculated as ASM divided by the height in meters squared), and body mass index (BMI)-adjusted ASM (calculated as ASM divided by BMI) (p < .01). PMA was also correlated with hand grip strength and gait speeds (p < .01). PVR was correlated with height-adjusted ASM and BMI-adjusted ASM (p < .01). A high PVR significantly decreased the odds ratios for hypertension and diabetes in the unadjusted model and the model adjusted for age, smoking, and drinking status. The ratio of the cross-sectional area of thigh muscle to the cross-sectional area of visceral fat and the BMI-adjusted ASM produced results similar to those of PVR in terms of the odds ratios for hypertension and diabetes. CONCLUSION. Single-slice abdominal CT can supply PMA and visceral fat information together. PMA and PVR were found to be reliable indexes of sarcopenia and sarcopenic obesity. A high PVR was associated with low risks for hypertension and diabetes.

Relationship of obstructive sleep apnoea severity and subclinical systemic atherosclerosis.

Obstructive sleep apnoea (OSA) is a common form of sleep disordered breathing. Untreated OSA might accelerate atherosclerosis, potentially increasing the cardiovascular disease burden in patients. The present study aimed to evaluate the association between objectively measured OSA severity and the presence of subclinical systemic atherosclerosis using noninvasive measurements, including tomographic quantification of the calcium burden.A total of 2157 participants of the Korean Genome and Epidemiology Study, who were free of structural heart disease and underwent both in-home polysomnography and chest computed tomography, were cross-sectionally analysed. Participants were divided into three groups based on the severity of OSA: no OSA (apnoea-hypopnoea index (AHI) <5 events·h-1, n=1096), mild OSA (AHI 5- <15 events·h-1, n=700) and moderate-to-severe OSA (AHI ≥15 events·h-1, n=361). Calcium deposits in the thoracic aorta and coronary arteries were measured by the Agatston score.Participants with moderate-to-severe OSA were 1.6 times (95% CI 1.18-2.15 times; p=0.002) more likely to have ascending thoracic aorta calcification (≥100 units) than those without OSA, after adjustment for cardiovascular risk factors. In addition, the association between moderate-to-severe OSA and ascending thoracic aorta calcification of subjects with higher epicardial fat volume was slightly stronger than that in patients without OSA and in the lowest epicardial fat volume tertile (OR 2.11, 95% CI 1.30-3.43).Severity of OSA in the general population was independently associated with subclinical systemic atherosclerosis. These findings highlight the potential importance of severe OSA, especially in subjects with higher epicardial fat, as a possible predictive factor for systemic atherosclerosis and cardiovascular disease.

Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study.

OBJECTIVES: To determine whether sirolimus has beneficial effects on lymphangioleiomyomatosis (LAM) lung cysts in CT with long-term follow-up (FU) and to investigate whether CT is an appropriate imaging biomarker to monitor and evaluate LAM progression. METHODS: In this retrospective study, 73 female patients diagnosed with definite LAM between May 2001 and June 2018 were included. Among these, 39 (53.4%) were treated with sirolimus. Quantitative and qualitative CT scoring for lung cysts (CS) were performed and compared between time points (baseline vs. FU at starting sirolimus, baseline vs. last FU, and FU at starting sirolimus vs. last FU for patients treated with sirolimus; baseline vs. last FU for patients without sirolimus). The correlation between CS at each time point and pulmonary function tests (PFTs) at each time point in the patients treated with sirolimus was also investigated. The quantitative and qualitative analyses and PFT results were compared between time points. RESULTS: In both quantitative and qualitative analyses, CS significantly increased from baseline to FU after starting sirolimus, and from baseline to last FU (all p < 0.05), whereas there was no significant difference between scores at the start of sirolimus vs. last in the patients treated with sirolimus. After sirolimus treatment, diffusing capacity for carbon monoxide (DLCO) was significantly increased. There were significant correlations between CS at each time point and PFT (correlation coefficient [r], - 0.383-0.935; all p < 0.001). CONCLUSION: Patients with LAM benefited from sirolimus. CT could be a useful imaging biomarker for evaluating and monitoring lung cysts in LAM. KEY POINTS: • Qualitative analysis showed a total of 15.8% to 21.1% of patients had a reduced lung cyst volume after sirolimus treatment, and in quantitative analysis, there was no significant difference in lung cyst volume between CT at the start of sirolimus therapy and the last CT. • Pulmonary function was also improved or maintained after sirolimus treatment. • Chest CT could be a useful imaging biomarker for evaluating and monitoring lung cysts in patients with lymphangioleiomyomatosis.

The Optimal Energy Level of Virtual Monochromatic Images From Spectral CT for Reducing Beam-Hardening Artifacts Due to Contrast Media in the Thorax.

OBJECTIVE: The purpose of this study is to determine the optimal energy level of virtual monochromatic images from spectral CT compared with conventional polychromatic images for reducing beam-hardening artifacts caused by contrast media in the thorax. MATERIALS AND METHODS: A total of 101 consecutive patients who underwent chest CT with contrast enhancement were retrospectively included in this study. The same contrast media and injection protocols were applied to the whole study population. Virtual monochromatic image datasets ranging from 70 to 200 keV and conventional polychromatic images were obtained. Readers' subjective image quality scores were recorded for conventional polychromatic and virtual monochromatic images obtained at 70, 100, 130, and 200 keV. Image noise, CT attenuation difference, contrast-to-noise ratio (CNR), and signal-to-noise ratio (SNR) were also obtained in each algorithm. Comparisons of parameters between algorithms were performed. RESULTS: The best subjective image quality score and significantly lower image noise were observed for 130-keV virtual monochromatic images compared with conventional polychromatic images (all p < 0.001). Also, CT attenuation differences were significantly lower for both 100- and 130-keV virtual monochromatic images than for conventional polychromatic images (all p < 0.001). Meanwhile, the lowest differences in CT attenuation were observed for 100-keV virtual monochromatic images compared with conventional polychromatic images. However, there were no significant differences in CT attenuation between 100- and 130-keV virtual monochromatic images. SNR was similar between 130-keV virtual monochromatic images and conventional polychromatic images, although both SNR and CNR decreased as the energy level increased. CONCLUSION: Virtual monochromatic imaging reduced beam-hardening artifacts and improved image quality, and optimal evaluation of chest CT was best achieved at 100 and 130 keV.

A comparison of digital tomosynthesis and chest radiography in evaluating airway lesions using computed tomography as a reference.

OBJECTIVES: To compare the diagnostic performance of digital tomosynthesis (DTS) and chest radiography for detecting airway abnormalities, using computed tomography (CT) as a reference. MATERIALS AND METHODS: We evaluated 161 data sets from 149 patients (91 with and 70 without airway abnormalities) who had undergone radiography, DTS, and CT to detect airway problems. Radiographs and DTS were evaluated to localize and score the severity of the airway abnormalities, and to score the image quality using CT as a reference. Receiver operating characteristics (ROC), McNemar's test, weighted kappa, and the paired t-test were used for statistical analysis. RESULTS: The sensitivity of DTS was higher (reader 1, 93.51 %; reader 2, 94.29 %) than chest radiography (68.83 %; 71.43 %) in detecting airway lesions. The diagnostic accuracy of DTS (90.91 %; 94.70 %) was also significantly better than that of radiography (78.03 %; 82.58 %, all p < 0.05). DTS image quality was significantly better than chest radiography (1.83, 2.74; p < 0.05) in the results of both readers. The inter-observer agreement with respect to DTS findings was moderate and superior when compared to radiography findings. CONCLUSIONS: DTS is a more accurate and sensitive modality than radiography for detecting airway lesions that are easily obscured by soft tissue structures in the mediastinum. KEY POINTS: • Digital tomosynthesis offers new diagnostic options for airway lesions. • Digital tomosynthesis is more sensitive and accurate than radiography for airway lesions. • Digital tomosynthesis shows better image quality than radiography. • Assessment of lesion severity, via tomosynthesis is comparable to computed tomography.

Comparison of chest radiography, chest digital tomosynthesis and low dose MDCT to detect small ground-glass opacity nodules: an anthropomorphic chest phantom study.

OBJECTIVES: The purpose of this study was to evaluate the diagnostic performance of chest radiography (CXR), chest digital tomosynthesis (DT) and low dose multidetector computed tomography (LDCT) for the detection of small pulmonary ground-glass opacity (GGO) nodules, using an anthropomorphic chest phantom. METHODS: Artificial pulmonary nodules were placed in a phantom and a total of 40 samples of different nodule settings underwent CXR, DT and LDCT. The images were randomly read by three experienced chest radiologists. Free-response receiver-operating characteristics (FROC) were used. RESULTS: The figures of merit for the FROC curves averaged for the three observers were 0.41, 0.37 and 0.76 for CXR, DT and LDCT, respectively. FROC analyses revealed significantly better performance of LDCT over CXR or DT for the detection of GGO nodules (P < 0.05). The difference in detectability between CXR and DT was not statistically significant (P = 0.73). CONCLUSION: The diagnostic performance of DT for the detection of pulmonary small GGO nodules was not significantly different from that of CXR, but LDCT performed significantly better than both CXR and DT. DT is not a suitable alternative to CT for small GGO nodule detection, and LDCT remains the method of choice for this purpose. KEY POINTS: For GGO nodule detection, DT was not significantly different from CXR. DT is not a suitable alternative to CT for GGO nodule detection. LDCT is the method of choice for GGO nodule detection.

Quantitative analysis of lungs and airways with CT in subjects with the chronic obstructive pulmonary disease (COPD) candidate FAM13A gene: case control study for CT quantification in COPD risk gene.

OBJECTIVE: To investigate the relationship between a chronic obstructive pulmonary disease (COPD) candidate gene, based on a genomewide association study, and computed tomographic (CT) quantitative analysis; and to find a phenotype in the COPD candidate FAM13A gene. MATERIALS AND METHODS: This study was conducted in subclinical male smokers between 2 groups with matched age and smoking status; 162 subjects (mean age, 58 years) with risk (CTGA, n = 85) and reference (TCAG, n = 77) diplotypes replicated through genomewide association study underwent chest CT for quantitative analysis of lungs and airways. We analyzed the measures in both the risk and reference groups using a 2-sample t test. RESULTS: Subjects with the risk CTGA diplotype had significantly higher total lung volume and emphysema index than the reference TCAG diplotype (P = 0.04). Mean lung density was significantly lower (P < 0.05) in the risk group. However, in the analysis of airways, wall area, luminal area, wall and lumen area ratio, and mean lung density on expiratory and inspiratory phases, no significant differences between the 2 groups were seen. CONCLUSIONS: There is a strong relationship between CT quantitative analysis and the COPD candidate gene. Furthermore, the CTGA diplotype was associated with emphysema among the phenotypes of COPD.

[Visualization of Borderline Coronary Artery Lesions by CT Angiography and Coronary Artery Disease Reporting and Data System]. / 관상동맥 질환 íŒë ê³¼ 자료 체계와 CT í˜ˆê´€ì¡°ì˜ìˆ ì—ì„œì˜ 경계성 관상동맥 병변.

Coronary artery disease (CAD) narrows vessel lumens at the sites of atherosclerosis, increasing the risk of myocardial ischemia or infarction. Early and accurate diagnosis of CAD is crucial to significantly improve prognosis and management. CT angiography (CTA) is a noninvasive imaging technique that enables assessment of vascular structure and stenosis with high resolution and contrast. Coronary CTA is useful in the diagnosis of CAD. Recently, the CAD-reporting and data system (CAD-RADS), a diagnostic classification system based on coronary CTA, has been developed to improve intervention efficacy in patients suspected of CAD. While the CAD-RAD is based on CTA, it includes borderline categories where interpreting the coronary artery status solely based on CTA findings may be challenging. This review introduces CTA findings that fall within the CAD-RADS categories that necessitate additional tests to decide to perform invasive coronary angiography and discusses appropriate management strategies.

Study of intrathyroid fat-containing lesions using CT imaging with literature review.

INTRODUCTION: This study was conducted to estimate the incidence of intrathyroid fat-containing lesions based in the general population on CT imaging. The imaging characteristics of the lesions on CT were also analyzed. METHODS: This retrospective study included data for 1,205 patients who underwent neck CT at our institution from January 2012 to January 2013. Data concerning size, shape, homogeneity of attenuation and location of lesions, and the presence of a fatty band connecting to extrathyroid fat were evaluated. Pathologic specimens were reevaluated when available. A literature review was conducted based on 16 peer-reviewed articles describing intrathyroid fat-containing lesions. RESULTS: Intrathyroid fat-containing lesions were detected in 13 patients (1.2 %). The mean lesion size was 5.7 mm (range, 2.6-10.6 mm). Nodular shape (92 %), homogeneous attenuation (92 %), and the fatty band (77 %) were common CT features. Posterior (85 %), medial (85 %), and upper (92 %) aspects of the thyroid gland were most commonly involved. In four patients who underwent surgery, one had fat-containing nodular hyperplasia, one had ectopic thymic tissue within mature fat, and one had mature fat itself. In 16 previously reported studies, the 41 lesions presented two fat infiltration patterns, diffuse (n = 12) and localized (n = 29), and two lesions showed mature fat tissue itself. CONCLUSION: Intrathyroidal fat-containing lesions were found in 1.2 % of patients presenting for neck CT. Almost all of the lesions occurred in common locations and showed similar features on CT, including nodular shape, homogeneous attenuation, and a fatty band.

Spontaneously healed asymptomatic pulmonary tuberculosis: prevalence of airflow obstruction, and correlation between high-resolution CT findings and pulmonary function tests.

RATIONALE AND OBJECTIVES: We investigated the relationships between spontaneously healed asymptomatic pulmonary tuberculosis (SHAPTB), airflow obstruction (AFO), and high-resolution computed tomography (HRCT) findings. MATERIALS AND METHODS: We selected 82 participants with SHAPTB diagnosed by interferon-γ release assay and 8044 with normal chest radiographs (CXR). We applied a CT scoring system for the extent of tuberculous sequelae to correlate the HRCT findings with pulmonary function test. We compared the AFO prevalence between subjects with and without SHAPTB. RESULTS: The subjects with SHAPTB diagnosed by interferon-γ release assay had a significantly higher prevalence of AFO (13.4% [11/82]) than those with normal CXR (7.4% [595/8044]). The important HRCT findings that correlated with AFO were the number of lung segments with TB sequelae and the CT score for emphysema. CONCLUSION: The subjects with SHAPTB had a higher AFO prevalence than those with normal CXR, and the important HRCT findings correlated with AFO were the extent of tuberculous sequelae and emphysema.

Genome-Wide Association Study of Airway Wall Thickening in a Korean Chronic Obstructive Pulmonary Disease Cohort.

Airway wall thickening (AWT) plays an important pathophysiological role in airway diseases such as chronic obstructive pulmonary disease (COPD). There are only a few studies on the genetic components contributing to AWT in the Korean population. This study aimed to identify AWT-related single-nucleotide polymorphisms (SNPs) using a genome-wide association study (GWAS). We performed GWAS for AWT using the CODA and KUCOPD cohorts. Thereafter, a meta-analysis was performed. Airway wall thickness was measured using automatic segmentation software. The AWT at an internal perimeter of 10 mm (AWT-Pi10) was calculated by the square root of the theoretical airway wall area using the full-width-half-maximum method. We identified a significant SNP (rs11648772, p = 1.41 × 10-8) located in LINC02127, near SALL1. This gene is involved in the inhibition of epithelial-mesenchymal transition in glial cells, and it affects bronchial wall depression in COPD patients. Additionally, we identified other SNPs (rs11970854, p = 1.92 × 10-6; rs16920168, p = 5.29 × 10-6) involved in airway inflammation and proliferation and found that AWT is influenced by these genetic variants. Our study helps identify the genetic cause of COPD in an Asian population and provides a potential basis for treatment.

A Challenge for Emphysema Quantification Using a Deep Learning Algorithm With Low-dose Chest Computed Tomography.

PURPOSE: We aimed to identify clinically relevant deep learning algorithms for emphysema quantification using low-dose chest computed tomography (LDCT) through an invitation-based competition. MATERIALS AND METHODS: The Korean Society of Imaging Informatics in Medicine (KSIIM) organized a challenge for emphysema quantification between November 24, 2020 and January 26, 2021. Seven invited research teams participated in this challenge. In total, 558 pairs of computed tomography (CT) scans (468 pairs for the training set, and 90 pairs for the test set) from 9 hospitals were collected retrospectively or prospectively. CT acquisition followed the hospitals' protocols to reflect the real-world clinical setting. Using the training set, each team developed an algorithm that generated converted LDCT by changing the pixel values of LDCT to simulate those of standard-dose CT (SDCT). The agreement between SDCT and LDCT was evaluated using the intraclass correlation coefficient (ICC; 2-way random effects, absolute agreement, and single rater) for the percentage of low-attenuated area below -950 HU (LAA-950 HU), κ value for emphysema categorization (LAA-950 HU, <5%, 5% to 10%, and ≥10%) and cosine similarity of LAA-950 HU. RESULTS: The mean LAA-950 HU of the test set was 14.2%±10.5% for SDCT, 25.4%±10.2% for unconverted LDCT, and 12.9%±10.4%, 11.7%±10.8%, and 12.4%±10.5% for converted LDCT (top 3 teams). The agreement between the SDCT and converted LDCT of the first-place team was 0.94 (95% confidence interval: 0.90, 0.97) for ICC, 0.71 (95% confidence interval: 0.58, 0.84) for categorical agreement, and 0.97 (interquartile range: 0.94 to 0.99) for cosine similarity. CONCLUSIONS: Emphysema quantification with LDCT was feasible through deep learning-based CT conversion strategies.

A case of Fabry's disease with congenital agammaglobulinemia.

Fabry's disease is an X-linked lysosomal storage disorder caused by abnormalities in the α-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues. It manifests as progressive multiple organ dysfunctions caused by the deposition of Gb3. On the other hand, congenital agammaglobulinemia is usually caused by mutations in Bruton's tyrosine kinase (Btk) gene with X-linked dominence, suppresses B cell maturation, and causes recurrent pyogenic infections. In former reports, the distance between the loci in the Xq22 region of the human X chromosome was found to be about 69 kilobases. A 23-yr-old man diagnosed with congenital agammaglobulinemia at age 5, showed typical clinical and laboratory and histopathological findings of Fabry's disease. The genetic basis of this combination of the two syndromes was studied in this patient. Here, we report a case of Fabry's disease with congenital agammaglobulinemia.

The impacts of open-mouth breathing on upper airway space in obstructive sleep apnea: 3-D MDCT analysis.

Open-mouth breathing during sleep is a risk factor for obstructive sleep apnea (OSA) and is associated with increased disease severity and upper airway collapsibility. The aim of this study was to investigate the effect of open-mouth breathing on the upper airway space in patients with OSA using three-dimensional multi-detector computed tomography (3-D MDCT). The study design included a case-control study with planned data collection. The study was performed at a tertiary medical center. 3-D MDCT analysis was conducted on 52 patients with OSA under two experimental conditions: mouth closed and mouth open. Under these conditions, we measured the minimal cross-sectional area of the retropalatal and retroglossal regions (mXSA-RP, mXSA-RG), as well as the upper airway length (UAL), defined as the vertical dimension from hard palate to hyoid. We also computed the volume of the upper airway space by 3-D reconstruction of both conditions. When the mouth was open, mXSA-RP and mXSA-RG significantly decreased and the UAL significantly increased, irrespective of the severity of OSA. However, between the closed- and open-mouth states, there was no significant change in upper airway volume at any severity of OSA. Results suggest that the more elongated and narrow upper airway during open-mouth breathing may aggravate the collapsibility of the upper airway and, thus, negatively affect OSA severity.

[Diagnosis of Hypersensitivity Pneumonitis: 2020 Clinical Practice Guideline]. / 2020ë „ ê°œì • 진료 치침에 따른 ê³¼ë¯¼ì„±íë ´ì˜ 진단.

Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) characterized by an inhaled inciting antigen that leads to the inflammation of the lung parenchyma and small airway with immunologic reactions. Over the last decades, the most effective therapeutic option for HP has been limited to antigen avoidance. The differential diagnosis of HP from other ILDs is the beginning of treatment as well as diagnosis. However, the presence of several overlapping clinical and radiologic features makes differentiating HP from other ILDs particularly challenging. In 2020, a multidisciplinary committee of experts from the American Thoracic Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax suggested a new clinical practice guideline classifying HP into nonfibrotic and fibrotic phenotypes on the basis of chest high-resolution CT (HRCT) findings. Therefore, we introduced a new diagnostic algorithm based on chest HRCT in the clinical practice guideline for the diagnosis of HP.

Evaluation of polyhexamethylene guanidine-induced lung injuries by chest CT, pathologic examination, and RNA sequencing in a rat model.

Our aim was to correlate chest CT and pathologic findings of polyhexamethylene guanidine phosphate (PHMG)-induced lung injuries in a rat model, to determine whether PHMG exposure causes lung tumors, and to explore genetic alterations according to PHMG exposure under the guidance of CT. A PHMG solution was intratracheally administrated to 40 male rats. Chest CT was carried out in all rats and both lungs were collected for histopathologic evaluation. At 4- and 8-weeks post-instillation, one lobe of the right lung from 3 rats was subjected to RNA sequencing. At least one abnormal CT finding was found in all rats at all weeks. The major CT findings were inflammation, fibrosis, and tumors in the pathologic analysis, where significant changes were observed over time. The lung lesions remained persistent after 8 weeks of PHMG exposure. In the pathologic analysis, the extent/severity of inflammation did not show statistically significant changes over time, whereas the extent/severity of fibrosis increased continuously up to 6 weeks after PHMG exposure and then decreased significantly at 8 weeks. Bronchiolar-alveolar adenomas which have malignant potential were found in 50% of rats at 6 and 8 weeks after PHMG exposure. Also, several genes associated with lung cancer, acute lung injury, and pulmonary fibrosis were detected. Our study revealed that PHMG-induced lung injury and its changes according to the number of weeks after exposure were demonstrated using chest CT and pathologic evaluation. In addition, we showed that PHMG exposure caused lung tumors and genetic alterations according to PHMG exposure under the guidance of CT.

Evaluation of the effect of filtered ultrafine particulate matter on bleomycin-induced lung fibrosis in a rat model using computed tomography, histopathologic analysis, and RNA sequencing.

We aimed to investigate the effect of chronic particulate matter (PM) exposure on bleomycin-induced lung fibrosis in a rat model using chest CT, histopathologic evaluation, and RNA-sequencing. A bleomycin solution was intratracheally administrated to 20 male rats. For chronic PM exposure, after four weeks of bleomycin treatment to induce lung fibrosis, PM suspension (experimental group) or normal saline (control group) was intratracheally administrated for 10 weeks. Chest CT was carried out in all rats, and then both lungs were extracted for histopathologic evaluation. One lobe from three rats in each group underwent RNA sequencing, and one lobe from five rats in each group was evaluated by western blotting. Inflammation and fibrosis scores in both chest CT and pathologic analysis were significantly more aggravated in rats with chronic PM exposure than in the control group. Several genes associated with inflammation and immunity were also upregulated with chronic PM exposure. Our study revealed that chronic PM exposure in a bleomycin-induced lung fibrosis rat model aggravated pulmonary fibrosis and inflammation, proven by chest CT, pathologic analysis, and RNA sequencing.