Form Of Fibrosis Research Articles

Effect of Home-Based Pulmonary Rehabilitation on Pulmonary Fibrosis.

Pulmonary fibrosis is a chronic, progressive lung condition that involves lung tissue scarring and thickening. The effects of home-based pulmonary rehabilitation (PR) in post-covid pulmonary fibrosis (PCPF) and other forms of fibrosis together have not been evaluated. This study aims to evaluate the effectiveness of home-based pulmonary rehabilitation on pulmonary function, functional capacity, and health-related quality of life in people with pulmonary fibrosis (post-COVID pulmonary fibrosis, pulmonary fibrosis secondary to pulmonary tuberculosis (TB), pulmonary fibrosis secondary to interstitial lung disease(ILD), pulmonary fibrosis secondary to bronchiectasis). A single-group pretest-posttest experimental study was performed after recruiting 98 pulmonary fibrosis subjects from K.M.C hospitals. After being screened for the inclusion and exclusion criteria, 45 subjects were analyzed, and 6 subjects were lost to follow-up. A home-based pulmonary rehabilitation program was carried out for 8 weeks (warm-up, stretching exercises, aerobic exercise, strength training for upper limb and lower limb, breathing exercises mainly involved; others: energy saving techniques, controlled coughing techniques, dyspnea relieving positions). The program was supervised via weekly phone calls. Pulmonary function (Pulmonary function test), exercise capacity (6-minute walk test), dyspnea (modified Borg scale), and health-related quality of life (SF-36) were evaluated before and after the intervention. During the enrollment and after the 6-minute walk test, saturation of peripheral oxygen (SPO2) level was also evaluated pre-intervention and after the 8-weeks program. Pulmonary function [FVC(L) t = -12.52, p<0.05; FEV1(L) t = -2.56, p<0.05; FEV1/FVC t = 7.98, p<0.05 and DLCO (ml/min/mmHg) t = -5.13, p<0.05], 6MWD [MD 88.66; p<0.05] and HRQOL measured by SF-36 scores (p<0.05) were improved significantly. Both the baseline SPO2 level before the 6MWT [MD 1.07, p<0.05] and the SPO2 level after the 6MWT [MD 1.16, p<0.05] showed a significant improvement. The rating of perceived exertion(dyspnea) [MD 1.30, p<0.05] was reduced significantly after the 8-week program. Our study shows that home-based pulmonary rehabilitation is an effective option for improving lung function and physical functional capacity by reducing dyspnea perception and improving the saturation of peripheral oxygen (SPO2) level, and enhancing the quality of life in people with pulmonary fibrosis.

Procollagen-lysine 2-oxoglutarate 5-dioxygenase 2 promotes collagen cross-linking and ECM stiffening to induce liver fibrosis

Procollagen-lysine 2-oxoglutarate 5-dioxygenase 2 (Plod2) is a key collagen lysyl hydroxylase mediating the formation of collagen fiber and stabilized collagen cross-links, and has been identified in several forms of fibrosis. However, the potential role and regulatory mechanism of Plod2 in liver fibrosis remain unclear yet. Mouse liver fibrosis models were induced by injecting carbon tetrachloride (CCl4) intraperitoneally. The morphology and alignment of collagen was observed under transmission and scanning electron microscopy, and extracellular matrix (ECM) stiffness was measured by atomic force microscopy. Large amounts of densely packed fibrillar collagen fibers produced by myofibroblasts (MFs) were deposited in fibrotic liver of mice reaching very large diameters in the cross section, accompanied with ECM stiffening, which was positively correlated with collagen-crosslinking. The expression of Plod2 was dynamically up-regulated in fibrotic liver of mouse and human. In MFs transfection of Plod2 siRNA made collagen fibers more orderly and linear aligned which can be easily degraded and protected from ECM stiffness. Administration of Plod2 siRNA preventatively or therapeutically in CCl4 mice reduced the average size of collagen bundles in transverse section, increased collagen solubility, decreases the levels of crosslinking products hydroxylysylpyridinoline and lysylpyridinoline, prevented ECM stiffening and alleviated liver fibrosis. Altogether, Plod2 mediates the formation of stabilized profibrotic collagen cross-links in MFs, leading to the alteration of collagen solubility and ECM stiffness, and eventually aggravates liver fibrosis, which provide potential target for the treatment of liver disease.

The role of long noncoding RNA MEG3 in fibrosis diseases.

Fibrosis is a prevalent pathological condition observed in various organs and tissues. It primarily arises from the excessive and abnormal accumulation of the extracellular matrix, resulting in the structural and functional impairment of tissues and organs, which can culminate in death. Many forms of fibrosis, including liver, cardiac, pulmonary, and renal fibrosis, are considered irreversible. Maternally expressed gene 3 (MEG3) is an imprinted RNA gene. Historically, the downregulation of MEG3 has been linked to tumor pathogenesis. However, recent studies indicate an emerging association of MEG3 with fibrotic diseases. In this review, we delve into the current understanding of MEG3's role in fibrosis, aiming to shed light on the molecular mechanisms of fibrosis and the potential of MEG3 as a novel therapeutic target.

Pleuro-Parenchymal Fibroelastosis (PPFE)-A Case Report

Pleuro-parenchymal fibroelastosis (PPFE) is a rare form of interstitial lung disease with pleural and parenchymal components in the form of fibrosis and elastosis. This is often missed due to its rarity and nonspecific symptoms. Most of the cases were attributed to trivial respiratory infections in the past. Here we present a case of an elderly female who is symptomatic after COVID-19 infection and has characteristic radiological patterns in high-resolution computed tomogram (HRCT) to suggest a diagnosis of PPFE.

Histological Reaction in the Round Window Membrane after Cochlear Implant Insertion in Nonhuman Primates

Introduction: The primary objective of this article was to determine if any histological alterations occur in the round window (RW) and adjacent anatomical structures of an animal model with normal hearing when a cochlear implant (CI) electrode array is inserted. Furthermore, this article aims to relate these histological alterations to the auditory changes generated. Methods: Cochlear implantation was performed, following the principles of minimally traumatic surgery (MTS), in 15 ears of nonhuman primates (Macaca fascicularis) (Mf) with normal hearing. Auditory brainstem-evoked potentials (ABR) using clicks and tones were used prior to surgery and during a 6-month follow-up period. Histological evaluation was carried out, analyzing the position of the electrode array with respect to the round window membrane (RWM), its percentage of occupation and integrity, the presence of intracochlear damage, and the tissue reaction provoked, the latter of which was quantified in the temporal bones. Results: Surgery was performed on all 15 ears without relevant incidences. Regarding histology, the electrode array in the RW of all ears presented a lateral position with respect to the modiolus. No lesions affecting the integrity of the RW were observed. The mean value of the array’s occupation in the RW was 25%. Tissue reaction, in the form of fibrosis, was observed in all ears and more intensely in the trans-RWM and post-RWM areas. In all ears, the electrodes remained on the scala tympani. No profound hearing impairment was recorded in any ear, being the mean loss of 25.4 dB when comparing presurgical thresholds with those collected 6 months after implantation in ABR click and 24.4 dB in ABR tone burst. Conclusions: The animal model and Hybrid L-14 (HL14) electrode array were optimal for implementing a surgical technique similar to that routinely performed on humans. Mild histological alterations were observed in the round window membrane and adjacent anatomical structures from the insertion of a cochlear implant electrode array. Following the minimally invasive technique, levels of hearing preservation were satisfactory, reaching a pre-post difference of 25.4 dB in the ABR click and 24.4 dB for a high-frequency tone burst. Complete hearing impairment was not observed in either ear. Correlation between the severity of histological alterations and hearing changes recorded in the ABR studies was observed.

Fibrosis in burns: an overview of mechanisms and therapies.

Scar development remains a common occurrence and a major healthcare challenge affecting the lives of millions of patients annually. Severe injuries to the skin, such as burns can lead to pathological wound healing patterns, often characterized by dermal fibrosis or excessive scarring, and chronic inflammation. The two most common forms of fibrotic diseases following burn trauma are hypertrophic scars (HSCs) and keloids, which severely impact the patient's quality of life. Although the cellular and molecular mechanisms are similar, HSC and keloids have several distinct differences. In this review, we discuss the different forms of fibrosis that occur postburn injury, emphasizing how the extent of burn influences scar development. Moreover, we highlight how a systemic response induced by a burn injury drives wound fibrosis, including both the role of the inflammatory response, as well as the fate of fibroblast during skin healing. Finally, we list potential therapeutics aimed at alleviating pathological scar formation. An understanding of the mechanisms of postburn fibrosis will allow us to effectively move studies from bench to bedside.

Gambaran Patologi Anatomi dan Histopatologi Organ Visceral Kambing Kacang (Capra aegagrus hircus) Yang Terinfeksi Sistiserkus

The demand for kacang goat (Capra aegagrus hircus)in Kupang City is very high in the local market, especially before the sacrificial festival. Although the goat population growth is high, there are several reasons that cause low productivity, namely parasitic infection.worms Taenia infect carnivores (as well as humans) as the definitive host and involve an intermediate host which will develop into a cysticercus. The purpose of this study was to determine the anatomical pathology and histopathology of the visceral organs of kacang goats infected with cysticercus. This study used 6 samples of visceral organs of kacang goats consisting of 4 liver organs, 1 rumen organ and 1 colon organ from 6 kacang goats infected with cysticercus, in Pasir Panjang Village, Kupang City. Macroscopic observations, tissue fixation in 10% formalin, tissue processing and HE staining were carried out. The results of the study on liver organs infected with cysticerci showed pathological changes in the form of fibrosis, while in the rumen and colon only cysticerci were formed. Histopathological changes in the liver organs of kacang goat infected with cysticercus in the form of mononuclear and polymorphonuclear inflammatory cell infiltration, necrosis, congestion, hemorrhage, hydropic degeneration, widening of sinusoids and fibrous tissue. Meanwhile, changes that occur in the rumen and colon organs are in the form of infiltration of mild inflammatory cells.

Systems level identification of a matrisome-associated macrophage polarisation state in multi-organ fibrosis.

Tissue fibrosis affects multiple organs and involves a master-regulatory role of macrophages which respond to an initial inflammatory insult common in all forms of fibrosis. The recently unravelled multi-organ heterogeneity of macrophages in healthy and fibrotic human disease suggests that macrophages expressing osteopontin (SPP1) associate with lung and liver fibrosis. However, the conservation of this SPP1+ macrophage population across different tissues and its specificity to fibrotic diseases with different etiologies remain unclear. Integrating 15 single-cell RNA-sequencing datasets to profile 235,930 tissue macrophages from healthy and fibrotic heart, lung, liver, kidney, skin, and endometrium, we extended the association of SPP1+ macrophages with fibrosis to all these tissues. We also identified a subpopulation expressing matrisome-associated genes (e.g., matrix metalloproteinases and their tissue inhibitors), functionally enriched for ECM remodelling and cell metabolism, representative of a matrisome-associated macrophage (MAM) polarisation state within SPP1+ macrophages. Importantly, the MAM polarisation state follows a differentiation trajectory from SPP1+ macrophages and is associated with a core set of regulon activity. SPP1+ macrophages without the MAM polarisation state (SPP1+MAM-) show a positive association with ageing lung in mice and humans. These results suggest an advanced and conserved polarisation state of SPP1+ macrophages in fibrotic tissues resulting from prolonged inflammatory cues within each tissue microenvironment.

New coronavirus infection as a risk-factor for the development of tuberculosis: a clinical observation

A new coronavirus infection is an acute viral disease with a primary lesion of the respiratory tract. Morphological changes in the lungs with a new coronavirus infection are characterized by damage to lymphocytes, endotheliocytes, macrophages, monocytes and diffuse lesions of the alveoli with local hemorrhages and accumulation of fluid and fibrin in their lumen and the development of residual changes in the form of fibrosis. The resulting changes in the target organ can lead to the activation of opportunistic infections, including tuberculosis. The clinical observation of the onset of a tuberculous process in a patient who had a new coronavirus infection with residual changes in the lung tissue in the form of fibrosis. Patient I., 30 years old, was admitted to a tuberculosis hospital for examination and treatment. The result of x-ray examination revealed signs of decay in the sixth segment of the lower lobe of the right lung. A positive sputum microscopy result for acid-fast mycobacteria was also obtained. Molecular genetic study of sputum confirmed the presence of Mycobacterium tuberculosis. From the anamnesis it is known that 5 months ago she suffered a new coronavirus infection, confirmed by a molecular genetic method, with a lesion of the sixth segment of the right lung. At the end of the treatment with COVID-19 were performed computed tomography of the chest organs – residual fibrous changes were noted in the sixth segment of the right lung after pneumonia. This clinical observation showed that the presence of residual fibrotic changes in the lungs after suffering a coronavirus disease in a patient is a risk factor for tuberculosis.

Features of lung impairment due to COVID-19 in patients of the first wave of the pandemic (literature review)

During the COVID-19 pandemic number of patients suffering from symptoms, decreased work ability and the deterioration in quality of life for several weeks and even months after the onset of the disease is increasing. The aim of the literature review is to search and study the existing literature data regarding the lung impairment features in COVID-19, as well as the morphological and pathophysiological prerequisites for the long-term respiratory disorders in patients who have had this disease. The most common complaints of patients after acute COVID-19 are fatigue, dyspnea, cough and chest discomfort. In these cases restrictive pattern in the pulmonary function test, a reduced gas transfer and persistent changes in computed tomography often in the form of fibrosis are observed, less often in the form of bullous-emphysematous changes, bronchiectasis and pleural effusion. The main pathophysiological mechanisms of acute lung impairment in COVID-19 are direct viral toxicity, impaired function of the immune system, hyperinflammatory response, endotheliopathy, microcirculatory disorders and increased blood clotting with small and large vessels thrombosis, which can lead to pneumonia or acute respiratory distress syndrome. After the acute phase of COVID-19 the pathology of the lung can be caused by an immunogenic and hyperinflammatory response, hypercoagulability and lead to pulmonary fibrosis. The possible mechanisms of the pulmonary fibrosis are thrombotic processes, hemosiderosis, necrotic capillaritis, angiogenesis, hypoxia, cytokine storm. Thus abnormal immune response, hyperinflammation, endothelial dysfunction and excessive coagulation not only cause pneumonia or acute respiratory distress syndrome in acute COVID-19, but also may persist for a long time and contribute to pulmonary fibrosis in some patients. The causes of a longer duration and increased severity of the lung impairment in some people compared to others are the subject of current investigations.

Post therapeutic changes in various malignancies

Background: Neo adjuvant Radiotherapy and chemotherapy can cause significant changes in the gross and microscopic appearance of malignant tissues and normal surrounding tissues. Since radiotherapy is now offered to a large number of cancer patients, pathologists are more frequently presented with such changes. Both epithelial and stromal changes can be present. Epithelial changes in the form of atrophy, apoptosis, necrosis, dysplasia and neoplasia, while stromal changes the form of fibrosis, exudates, necrosis, pattern of invasion and vessel wall thickness. These changes may interfere with recognition of microscopic residual tumor if present. With increasing use of radiation therapy in cancer treatment and longer survival of patients, assessment of radiation-associated changes in tissues and tumours has become an important issue in modern pathology.Materials and Methods: The present study was carried out on 50 patients. Type of samples includes cytological smears and histopathological specimens received in respective cytopathology and histopathology section of the pathology department after any chemotherapy or radiotherapy.Objective1.To study cyto-morphological changes after chemotherapy and/or radiotherapy. 2.To differentiate secondary malignancy from radiation damage.Results: The present study was carried out on 50 patients including 13 patients with lymph node metastasis,12 cases with head & neck region, 2 scrap smear, 9 anterior chest wall swelling, 3 cases of liver, 5 cases of lung,1 plural fluid and 5 cases of breast. The effects have been divided mainly into two, as pathological changes in tumor cells and changes in the stroma.Conclusion: It is concluded from the findings of present study that various nuclear abnormalities reveal a statistically significantly increases with increasing chemoradiation doses and time interval. Post neoadjuvant chemotherapy, specimens revealed nuclear enlargement, nuclear shrinkage, necrosis, vacuolation of nucleus, vacuolation of cytoplasm, dyscohesion, and shrinkage of tumor cells with nuclear changes of nonviability like karyorrhexis, karyolysis, and pyknosis. Stromal reactions manifested as fibrosis, elastosis, collagenization, hyalinization, microcalcification, and neovascularization. Areas of necrosis included both vascular and avascular pattern. The stroma also revealed fibrinoid necrosis and mucinous change. Hyalinization of the blood vessel wall was a common finding.

Senescent Cells Accumulate in Systemic Sclerosis Skin

Systemic sclerosis (SSc) is characterized by intractable fibrosis in the skin and internal organs. A unifying paradigm across all forms of fibrosis is the transformation of quiescent progenitor cells into activated myofibroblasts, but the pathways underlying the transformation and persistence of myofibroblasts in SSc remain poorly understood. SSc might be viewed as an accelerated aging phenotype, and patients with SSc prematurely display hallmarks of aging, including telomere attrition, genomic instability, epigenetic alterations, deregulated nutrient sensing, stem cell exhaustion, and mitochondrial dysfunction ( Luckhardt and Thannickal, 2015 Luckhardt T.R. Thannickal V.J. Systemic sclerosis-associated fibrosis: an accelerated aging phenotype?. Curr Opin Rheumatol. 2015; 27: 571-576 Crossref PubMed Scopus (31) Google Scholar ; Tsou et al., 2022 Tsou P.S. Shi B. Varga J. Role of cellular senescence in the pathogenesis of systemic sclerosis. Curr Opin Rheumatol. 2022; 34 (343‒50) Crossref PubMed Scopus (0) Google Scholar ). Cellular senescence is another hallmark of aging. Sustained tissue accumulation of senescent cells is detrimental to organ function owing in part to the senescence-associated secretory phenotype, characterized by secretion of cytokines/chemokines, growth factors, extracellular matrix modifiers, and other substances that promote chronic, sterile inflammation and fibrosis.

Replacement of Lost Substance P Reduces Fibrosis in the Diabetic Heart by Preventing Adverse Fibroblast and Macrophage Phenotype Changes.

Reduced levels of the sensory nerve neuropeptide substance P (SP) have been reported in the diabetic rat heart, the consequence being a loss of cardioprotection in response to ischemic post-conditioning. We considered whether this loss of SP also predisposes the heart to non-ischemic diabetic cardiomyopathy in the form of fibrosis and hypertrophy. We report that diabetic Leprdb/db mice have reduced serum SP and that administration of exogenous replacement SP ameliorated cardiac fibrosis. Cardiac hypertrophy did not occur in Leprdb/db mice. Cardiac fibroblasts exposed to high glucose converted to a myofibroblast phenotype and produced excess extracellular matrix proteins; this was prevented by the presence of SP in the culture media. Cardiac fibroblasts exposed to high glucose produced increased amounts of the receptor for advanced glycation end products, reactive oxygen species and inflammatory cytokines, all of which were prevented by SP. Cultured macrophages assumed an M1 pro-inflammatory phenotype in response to high glucose as indicated by increased TNF-α, CCL2, and IL-6. SP promoted a shift to the reparative M2 macrophage phenotype characterized by arginase-1 and IL-10. Leprdb/db mice showed increased left ventricular M1 phenotype macrophages and an increase in the M1/M2 ratio. Replacement SP in Leprdb/db mice restored a favorable M1 to M2 balance. Together these findings indicate that a loss of SP predisposes the diabetic heart to developing fibrosis. The anti-fibrotic actions of replacement SP involve direct effects on cardiac fibroblasts and macrophages to oppose adverse phenotype changes. This study identifies the potential of replacement SP to treat diabetic cardiomyopathy.

The dynamic organelle primary cilia: emerging roles in organ fibrosis.

Primary cilia, the antenna-like organelles on most mammalian cells, host key components of multiple morphogen signal transduction pathways. Mutations in genes responsible for primary cilia assembly and function generally result in pathological conditions known as ciliopathies, which underlie several diseases, including various forms of fibrosis. Primary cilia modulate cellular responses to extracellular cues, including TGF-β and morphogens, such as Hedgehog. Aberrant morphogen signaling is recognized as essential for the transition of mesenchymal progenitor cells to myofibroblasts, the key step in fibrosis. This article aims to provide a critical overview of recent developments and insights in primary cilia biology relevant to fibrosis. Several studies have highlighted the association of altered primary cilia with various forms of fibrosis. In a rather complex manner, the presence of primary cilia seems to be required for initiation of myofibroblast transition, whereas its loss promotes myofibroblast transition at a later stage. Recent evidence also suggested that noncanonical functions of ciliary transport proteins may influence, such cellular transitions independently of primary cilia. The possibility of opposing signaling regulations being topologically separated between primary cilia and plasma membrane could also be critical for fibrosis. Recent progress in the field suggests that primary cilia are critical mediators of the pathogenesis of fibrosis. Understanding the potential role of primary cilia in fibrosis and the underlying mechanisms may pave the way for entirely new approaches for fibrosis prevention and treatment of SSc.

Growth of Aspergillus and Mucor spp. in sputum culture of a post-COVID patient: fungal infection or colonization? A clinical challenge in COVID-era

Opportunistic infections, especially invasive fungal infections have emerged as an added health issue in this COVID-era. The dysregulated immune mechanisms in the pathophysiology of COVID-19, its treatment with corticosteroids and other immunomodulatory agents, invasive mechanical ventilation and other forms of ventilatory and oxygen delivery systems, prolonged hospital stay, injudicious use of antimicrobials – all have set the required stages for such infections. Aspergillus spp. and Mucorales are ubiquitous, environmental fungus; may colonize in airways and chronically damaged human lungs, without significant health effects and cause disease, especially in immunocompromized patients. We report case history of a middle aged Bangladeshi man with multiple comorbidities, who presented with fever and respiratory symptoms, four-months after recovery from COVID-19. He had Aspergillus and Mucors on sputum microscopy and cultures; but his response to antibiotics suggested these were colonozations in previously injured lungs by tuberculosis in the form of fibrosis and bronchiectasis. He is well and free of symptoms, two-months since discharge.&#x0D; BIRDEM Med J 2021; 11(3): 218-222

Fibrosis in Hypertrophic Cardiomyopathy Patients With and Without Sarcomere Gene Mutations

Patients with hypertrophic cardiomyopathy (HCM) and an identified sarcomere mutation have worse outcomes than those without though the underlying mechanism is incompletely understood. The presence of replacement fibrosis measured by late gadolinium enhancement (LGE) and diffuse fibrosis measured by extracellular volume (ECV) using cardiac magnetic resonance imaging (CMR) are associated with ventricular arrhythmias and cardiac mortality. We aimed to associate these two forms of fibrosis with identified sarcomere mutations. Three hundred and thirty-six (336) patients with HCM underwent CMR at a single quaternary referral centre between January 2012 and February 2017. Genetic testing was performed in 73 of these patients, yielding an identified sarcomeric mutation in 29 (G+), no mutation in 39 (G-), and a variant of unknown significance (VUS) in five. LGE was more prevalent in G+ compared to G- patients (86 vs. 56%, OR 4.3, p=0.01) and was more extensive (7.5±5.5% of left ventricular [LV] mass vs. 3.0±3.0%, p<0.001). Global ECV from myocardial segments excluding LGE was similar among both groups (26.9±2.9 vs. 25.6±2.8%, p=0.46). However, in G+ patients ECV was greater in the hypertrophied regions of the basal anteroseptum (30.2±7.0 vs. 26.8±3.6%, p=0.004) and basal inferoseptum (28.1±4.3 vs. 26.2±2.9%, p=0.005). Genotyped HCM patients with an identified sarcomere mutation have greater LGE and greater regional, but not global, ECV than HCM patients without an identified mutation. This difference in fibrosis may contribute to worse outcomes in patients with an identified HCM mutation.

CT Characteristics of Consolidation Type of Pulmonary Cryptococcosis in Immunocompetent Patients

Objective To analyze the CT characteristics of consolidation type of pulmonary cryptococcosis in immunocompetent patients,and thus improve the diagnosis of this disease. Methods A total of 20 cases with consolidation-type pulmonary cryptococcosis confirmed by pathological examinations were studied.Each patient underwent breath-hold multislice spiral CT,and 10 patients underwent contrast enhanced CT.The data including lesion number,lesion distribution,lesion density,performance of enhanced CT scan,accompanying signs,and prognosis were analyzed. Results The occurrence rates of single and multiple lesions were 80.0%(n=16)and 20.0%(n=4),respectively.In all the 16 multiple-lesion patients,the occurrence rate of unilateral lobar distribution was 56.0%(n=9).The 76 measurable lesions mainly presented subpleural distribution(71.1%,n=54)and lower pulmonary distribution(75.0%,n=57).A total of 39 lesions were detected in the 10 patients received contrast enhanced CT,in which 31 lesions(79.5%)showed homogeneous enhancement,34 lesions(87.2%)showed moderate enhancement,and all the lesions manifested angiogram sign.Consolidation lesions were accompanied by many CT signs,of which air bronchogram sign had the occurrence rate of 63.2%(n=48),including types Ⅲ(n =37)and Ⅳ(n=11).Other signs included halo signs(43/76,56.6%),vacuoles or cavities(9/76,11.8%),pleural thickening(14/20,70.0%),and pleural effusion(2/20,10.0%).After treatment,the lesions of 7 patients were basically absorbed and eventually existed in the form of fibrosis. Conclusions The lesions in the immunocompetent patients with consolidation type of pulmonary cryptococcosis usually occur in the lower lobe and close to the pleura,mainly presenting unilateral distribution.The CT angiogram signs,proximal air bronchogram signs,and halo signs are the main features of this disease,which contribute to the diagnosis.

ТРАНСПЛАНТАЦИЯ ЛЕГКИХ У ПОДРОСТКА С ЛЕГОЧНЫМ ФИБРОЗОМ В ИСХОДЕ ИНТЕРСТИЦИАЛЬНОЙ БОЛЕЗНИ ЛЕГКИХ (ГИПЕРСЕНСИТИВНОГО ПНЕВМОНИТА)

Interstitial lung disease (ILD) is a large group of diseases with a severe course and, as a rule, an unfavorable prognosis, since irreversible pulmonary fibrosis forms in the outcome of most ILD. Among ILD in children and adolescents, hypersensitivity pneumonitis (HP) is of significant clinical importance. The clinical observation presented in the article demonstrates the first successful experience in our country (2018) of transplanting both lungs to an adolescent with pulmonary fibrosis resulting from the HP chronic course. Lung transplantation has become the only way to prolong the patient's life.

P89 Role of inflammation as a risk factor for atrial fibrillation in rheumatic mitral stenosis and its correlation with atrial late gadolinium enhancement (LGE) on cardiac MRI

Abstract Funding Acknowledgements ICMR Aims Association of atrial fibrillation (AF) is a common cause of morbidity and mortality in patients with rheumatic heart disease (RHD). Association of Atrial Fibrillation with inflammation is well delineated in nonvalvular AF as compared to Rheumatic AF. This study evaluated the presence of atrial inflammation, in patients with AF and rheumatic mitral stenosis (MS), using cardiac MRI and its correlation with inflammatory markers. Methods and Results Twenty RHD patients with predominant mitral stenosis were recruited for the study. Ten patients had persistent AF, three had paroxysmal AF and seven were in sinus rhythm. Patients with AF had lower mitral valve areas. Cardiac MRI showed evidence of late gadolinium enhancement (LGE) in the atrial wall in 61.54% (8/13) of patients with AF as compared with only 20% (1/5; p &amp;lt; 0.05) of patients in sinus rhythm. Mean levels of IL-6 were also significantly higher in patients with AF (10.62 ± 5.92 pg/mL versus 4.37 ± 2.68 pg/mL; P= 0.017). IL6 with a cutoff of ≥ 6.5 pg/mL was associated with a sensitivity of 76.92 % and specificity of 71.43 % for prediction of AF. There was also significant association between high IL-6 levels and LGE on MRI (13.13 ± 5.75 pg/mL vs 5.29 ± 3.23pg/mL; P &amp;lt; 0.05) Conclusion Patients with MS who developed AF show evidence of atrial inflammatory sequelae in the form of fibrosis as suggested by LGE on cardiac MRI. Systemic inflammation in the form of elevated IL-6 levels correlated significantly with atrial fibrosis and AF. Table 1: MS patients with or without AF Clinical Features Rhythm P-Value NSR (n = 7) AF (n = 13) Mean Valve Area (cm2) 1.28 ± 0.35 0.83 ± 0.26 &amp;lt; 0.01 Mean Diastolic Gradient (mmHg) 6.29 ± 2.2 11.92 ± 5.65 &amp;lt;0.01 Wilkin Score 7 ± 0.816 8.46 ± 1.66 0.04 LVEF % (non indexed) 59.57 ± 1.13 58.77 ± 2.89 0.49 LA Volume with MRI (ml) 127.50 ± 51.32 284.33 ± 133.12 &amp;lt;0.01 Inflammatory Markers hs-CRP (mg/L) 5.59 ± 3.89 3.94 ± 3.47 0.34 IL6 (pg/mL) 4.37 ± 2.68 10.62 ± 5.92 0.017 sCD-40L (ng/mL) 3.72 ± 3.73 4.77 ± 2.80 0.48 Atrial LGE 0 (0%) 8 (61.5%) 0.04 Abstract P89 Figure. Image 1: Cardiac MRI showing LGE

Leukemia cutis: A study from a tertiary care hospital in North India

Background/Objectives: Only anecdotal case reports or small case series have attempted to study the histomorphology patterns of leukemic infiltration. This study explored the possible association of leukemia cutis with, temporal course of disease, subtypes of leukemia, blast count, total leukocyte count, lactate dehydrogenase (LDH) levels, and histopathological patterns. Materials and Methods: Clinical and laboratory data were retrieved from the hospital information system. Selected cases were reviewed by two pathologists. Data in terms of demographics, clinical presentation, bone marrow findings, immunophenotype, and temporal course were recorded where available. Results: Twenty-three diagnosed cases of leukemia cutis were reported during the study period. There was no gender predilection with a wide age range (8–82 years). No particular trend was noted in relation to total leukocyte or bone marrow blast count. Common skin manifestations included multiple erythematous papular eruptions, frequently involving the skin of the limbs. All cases showed a consistently high serum LDH level. 11/23 (48%) showed cutaneous manifestation of undiagnosed leukemia or indicated worsening in a diagnosed case. The histological patterns varied from perivascular to diffuse dermal interstitial infiltration. In addition, subtle changes were seen in the form of fibrosis, thrombosis, and epidermotropism in 12%–50% of cases. The judicious use of a panel of immunohistochemistry was vital to establish a diagnosis. Conclusions: Leukemia cutis is a diagnostic challenge on histopathology due to lack of any specific pattern in absence of a clinical suspicion. Its diagnosis may be an indicator of undiagnosed leukemia or worsening in an already known case of leukemia.