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Interstitial Diseases

Erina Sasaki, Angela T Byrne, Ethna Phelan, Desmond W Cox, William Reardon
The filamin A gene (FLNA) on Xq28 encodes the filamin A protein. Mutation in FLNA causes a wide spectrum of disease including skeletal dysplasia, neuronal migration abnormality, cardiovascular malformation, intellectual disability and intestinal obstruction. Recently, childhood-onset interstitial lung disease associated with a range of FLNA mutations has been recognised and reported. We document our personal experience of this emerging disorder and compile a comprehensive overview of clinical features and molecular changes in all identifiable published cases...
December 13, 2018: European Journal of Pediatrics
Atsuko Y Higashi, Bruce J Aronow, Gregory R Dressler
BACKGROUND: Renal interstitial fibrosis results from activation and proliferation of fibroblasts to myofibroblasts, secretion and accumulation of extracellular matrix, and displacement of normal renal tubules. In contrast to chronic renal disease, acute injury may be repaired, a process that includes a decrease in the number of myofibroblasts in the interstitium and degradation of the accumulated extracellular matrix, leaving little evidence of prior injury. METHODS: To investigate whether activated fibroblasts demonstrate changes in gene expression that correspond with regression after acute injury but are not observed in chronic models of fibrosis, we used microarrays to analyze gene expression patterns among fibroblast populations at different stages of injury or repair...
December 13, 2018: Journal of the American Society of Nephrology: JASN
Sun Ah Nam, Wan-Young Kim, Jin-Won Kim, Min Gyu Kang, Sang Hee Park, Myung-Shik Lee, Hyung Wook Kim, Chul Woo Yang, Jin Kim, Yong Kyun Kim
Renal fibrosis is the final common pathway of various renal injuries and it leads to chronic kidney disease. Recent studies reported that FOXD1-lineage pericyte plays a critical role in tubulointerstitial fibrosis (TIF). However the regulatory mechanisms remain unclear. Autophagy is a cellular process of degradation of damaged cytoplasmic components that regulates cell death and proliferation. To investigate the role of autophagy in FOXD1-lineage pericytes on renal TIF, we generated the FOXD1-lineage stromal cell-specific Atg7 deletion (Atg7△FOXD1 ) mice...
December 10, 2018: Biochemical and Biophysical Research Communications
Philipp Gauckler, Jae Il Shin, Gert Mayer, Andreas Kronbichler
Peripheral blood eosinophilia (PBE), defined as 500 eosinophils or above per microliter (µL) blood, is a condition that is not uncommon but often neglected in the management of patients with chronic kidney disease (CKD), acute kidney injury (AKI), or patients on renal replacement therapy (RRT). The nature of PBE in the context of kidney diseases is predominantly secondary or reactive and has to be distinguished from primary eosinophilic disorders. Nonetheless, the finding of persistent PBE can be a useful clue for the differential diagnosis of underdiagnosed entities and overlapping syndromes, such as eosinophilic granulomatosis with polyangiitis (EGPA), IgG4-related disease (IgG4-RD), acute interstitial nephritis (AIN), or the hypereosinophilic syndrome (HES)...
December 8, 2018: Journal of Clinical Medicine
Samuel P Trethewey, Gareth I Walters
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by a progressive and irreversible decline in lung function, which is associated with poor long-term survival. The pathogenesis of IPF is incompletely understood. An accumulating body of evidence, obtained over the past three decades, suggests that occupational and environmental exposures may play a role in the development of IPF. This narrative literature review aims to summarise current understanding and the areas of ongoing research into the role of occupational and environmental exposures in the pathogenesis of IPF...
December 10, 2018: Medicina
Kakuhiro Yamaguchi, Hiroshi Iwamoto, Shinjiro Sakamoto, Yasushi Horimasu, Takeshi Masuda, Shintaro Miyamoto, Taku Nakashima, Shinichiro Ohshimo, Kazunori Fujitaka, Hironobu Hamada, Nobuoki Kohno, Noboru Hattori
The aim of this study was to evaluate whether serial measurements of serum Krebs von den Lungen-6 (KL-6) could be used to monitor disease activity and to detect recurrence in patients with interstitial pneumonia (IP) with anti-aminoacyl-tRNA synthetase antibodies (ARS-IP).This retrospective cohort study included 44 patients with ARS-IP. Thirty-six patients had serial data of blood tests and pulmonary function tests. Baseline and longitudinal analyses were performed to investigate whether lung function parameters were associated with serum biomarkers (KL-6, lactate dehydrogenase [LDH], and C-reactive protein [CRP]) using Pearson correlation coefficient...
December 2018: Medicine (Baltimore)
F Vivero, F Campins, D Lancellotti, P Malfante, S Babini, J Sebastiani, V Basso, A Gaser, J Enghelmayer, E Gandara
Information about the prognosis and natural history of autoimmune interstitial lung diseases (Ai-ILD) is limited. The aim of the study was to evaluate the characteristics of patients diagnosed with Ai-ILD in Latin-America. We conducted an ambispective multicenter cohort study in 25 centers of Argentina, Colombia, and Uruguay between January 2015 and April 2018. Participants were included in the study if they had diagnosis of Ai-ILD performed by a multidisciplinary team. Patients were classified into the following sub-groups: connective tissue disease-associated ILD (ILD-CTD), interstitial pneumonia with autoimmune features (IPAF), and positive antineutrophils cytoplasmatic antibodies associated ILD (ILD-ANCA)...
December 10, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Marc Parrilla, Maria Cuartero, Sara Padrell Sánchez, Mina Rajabi, Niclas Roxhed, Frank Niklaus, Gaston A Crespo
A new analytical all-solid-state platform for intradermal potentiometric detection of potassium in interstitial fluid is presented here. Solid microneedles are modified with different coatings and polymeric membranes to prepare both the potassium-selective electrode and reference electrode needed for the potentiometric readout. These microneedle-based electrodes are then fixed in an epidermal patch suitable for insertion into the skin. The analytical performances observed for the potentiometric cell (Nernstian slope, limit of detection of 10-4...
December 13, 2018: Analytical Chemistry
Nobuyuki Ono, Keita Kai, Akihito Maruyama, Mariko Sakai, Yuri Sadanaga, Shuichi Koarada, Takuya Inoue, Yoshifumi Tada
Objectives: Based on the antibody profiles of inflammatory myositis patients, we investigated the type 1 IFN (T1-IFN) signature in serum and DM skin to determine the relationship between T1-IFN and vasculopathy in anti-melanoma differentiation-associated 5 gene (MDA5) antibody-positive DM patients. Methods: We examined 47 patients with new-onset inflammatory myositis. We divided them into three groups: the anti-MDA5 antibody-positive patients (MDA5 group, n = 16), the anti-aminoacyl-tRNA synthetase antibody-positive patients (aminoacyl-tRNA synthetase group, n = 12), and the double-negative patients (n = 19)...
December 11, 2018: Rheumatology
Andrew M Courtwright, Souheil El-Chemaly
Telomeres are repetitive nucleotide sequences that cap linear chromosomes, thereby limiting progressive chromosomal shortening during cell replication. In conjunction with environmental factors, common single nucleotide polymorphisms and rare and ultra-rare telomere-related mutations are associated with accelerated telomere shortening resulting in organ dysfunction, including interstitial lung disease (ILD). The most common telomere-related mutation-associated ILD is idiopathic pulmonary fibrosis (IPF). Up to one-third of individuals with familial IPF have shortened telomeres and/or carry a telomere-related mutation and one in ten individuals with sporadic IPF have telomere-related mutations...
December 12, 2018: Annals of the American Thoracic Society
Júlia Vízkeleti, Georgina Fröhlich, Nguyen Anhhong Nhung, Katalin Horváth, Tibor Major, Csaba Polgár
We present the early clinical results achieved with image-guided adaptive brachytherapy (IGABT) with combined intracavitary-interstitial (IC-IS) technique recently implemented in Hungary in the treatment of locally advanced cervical cancer (LACC). Twenty-one patients were treated with radio-chemotherapy (RCT) followed by combined IC-IS BT. At the end of the RCT we assessed the residual tumour with pelvic MRI. On CT images registered with the applicator in place we contoured the organs at risk and the high-risk clinical target volume, which included the whole cervix and the eventual residual tumour in the parametria...
December 12, 2018: Magyar Onkologia
Jung Nam An, Seung Hee Yang, Yong Chul Kim, Jin Ho Hwang, Jae Yoon Park, Dong Ki Kim, Jin Hyuk Kim, Dae Woo Kim, Dong Gu Hur, Yun Kyu Oh, Chun Soo Lim, Yon Su Kim, Jung Pyo Lee
Periostin plays a crucial role in fibrosis, and acute kidney injury results in a high risk of progression to chronic kidney disease. Therefore, we hypothesized that periostin was involved in the progression of acute kidney injury to kidney fibrosis. Unilateral ischemia-reperfusion injury (UIRI) was induced in 7- to 8-week-old male wild-type and periostin- null mice, and the animals were observed for 6 weeks. In vitro, human kidney-2 cells and primary cultured human tubular epithelial cells were incubated under hypoxic conditions (5% O2 , 5% CO2 , and 90% N2 ) for 5 days...
December 12, 2018: American Journal of Physiology. Renal Physiology
Shigekuni Hosogi, Masahiro Ohsawa, Ikuo Kato, Atsukazu Kuwahara, Toshio Inui, Akio Inui, Yoshinori Marunaka
Diabetes mellitus is a well-known common disease and one of the most serious social problems in the worldwide. Although various types of drugs are developed, the number of patients suffering from diabetes mellitus is still increasing. Ninjin'yoeito (NYT) is one of formulas used in Japanese traditional herbal medicines for improving various types of metabolic disorders. However, the effect of NYT on diabetes mellitus has not yet been investigated. In the present study, we tried to clarify the action of NYT on the serum glucose level in streptozotocin (STZ)-induced diabetic mice...
2018: Frontiers in Nutrition
Francisco J García-Hernández, María J Castillo-Palma, Carles Tolosa-Vilella, Alfredo Guillén-Del Castillo, Manuel Rubio-Rivas, Mayka Freire, José A Vargas-Hitos, José A Todolí-Parra, Mónica Rodríguez-Carballeira, Gerard Espinosa-Garriga, Dolores Colunga-Argüelles, Norberto Ortego-Centeno, Luis Trapiella-Martínez, María M Rodero-Roldán, Xavier Pla-Salas, Isabel Perales-Fraile, Isaac Pons-Martín Del Campo, Antonio J Chamorro, Rafael A Fernández-de la Puebla Giménez, Ana B Madroñero-Vuelta, Manuel Ruíz-Muñoz, Vicent Fonollosa-Pla, Carmen P Simeón-Aznar
INTRODUCTION: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors. METHOD: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified...
December 7, 2018: Clinical Rheumatology
Qiang Fu, Li Wang, Luling Li, Yifan Li, Ran Liu, Yi Zheng
BACKGROUND: Factors associated with progression and survivals in rheumatoid arthritis-associated interstitial lung disease (RA-ILD) have not been described in a large center China cohort. METHODS: Seven-hundred and ninety-one consecutive RA patients who completed lung HRCT were considered as potential participants in this retrospective cohort study. Determinants of ILD progression were identified through multivariable logistic analysis. Cox hazards analysis was used to determine significant variables associated with survival...
December 7, 2018: Clinical Rheumatology
Tamaki Kakuwa, Shinyu Izumi, Keita Sakamoto, Tomoyuki Suzuki, Motoyasu Iikura, Haruhito Sugiyama
Rheumatoid arthritis-related interstitial pneumonia with a usual interstitial pneumonia (RA-UIP) has a poor prognosis and a new treatment strategy is required. The antifibrotic agent nintedanib reduces the annual rate of decline in forced vital capacity (FVC) in idiopathic pulmonary fibrosis (IPF) patients. Recently, the potential efficacy of antifibrotic agents against chronic progressive fibrotic diseases including RA-UIP has been attracting attention. A 74-year-old man diagnosed with IPF on high-resolution computed tomography (HRCT)...
2019: Respiratory Medicine Case Reports
Christian Wong, Sonu Sahni, Muhammad Azaz I Cheema, Asma Iftikhar
Mycobacterium tuberculosis (MTB) infection is the ninth leading cause of death worldwide, with many individuals with undiagnosed active or latent disease. The presence of parenchymal lung disease, such as interstitial lung disease (ILD), has been suggested to increase the risk of pulmonary tuberculosis (TB). In the clinical setting of ILD, the diagnosis of an underlying MTB infection may be challenging due to the interstitial process and underlying fibrosis, which may mask the infection. An atypical presentation and misleading radiological patterns may delay the diagnosis of the underlying MTB infection...
October 1, 2018: Curēus
Rammah Abohtyra, Yossi Chait, Michael J Germain, Christopher V Hollot, Joseph Horowitz
OBJECTIVE: There are approximately 660,000 end-stage renal disease (ESRD) patients in the USA, with hemodialysis (HD) the primary form of treatment. High ultrafiltration rates (UFRs) are associated with intradialytic hypotension, a complication associated with adverse clinical outcomes including mortality. Individualized UFR profiles could reduce the incidence of intradialytic hypotension. METHODS: The patient's fluid dynamics during HD is described by a nonlinear model comprising intravascular and interstitial pools, whose parameters are given by the patient's estimated nominal parameter values with uncertainty ranges; the output measurement is hematocrit...
December 4, 2018: IEEE Transactions on Bio-medical Engineering
Swati Kumari, P K Ray, Rahul Singh, P A Desingu, Rajat Varshney, G Saikumar
The aim of the present study was to pathological and molecular investigation of porcine sapelovirus (PSV) in naturally infected Indian pigs of various age groups. Eight samples (16%) out of 49 necropsied animals were positive for PSV on the basis of pathological and molecular investigation. Major lesions of PSV positive cases were thickening and clouding of meninges, congestion in brain, severe to moderate congestion in lungs along with froathy exudates in trachea, thickening of intestinal mucosa, especially mucosal folds of ileum...
December 5, 2018: Microbial Pathogenesis
Vinícius Pereira de Carvalho, Mariana Lopes Grassi, Camila de Souza Palma, Helio Humberto Angotti Carrara, Vitor Marcel Faça, Francisco José Candido Dos Reis, Aline Poersch
Despite all the advances in understanding the mechanisms involved in ovarian cancer (OC) development, many aspects still need to be unraveled and understood. Tumor markers (TMs) are of special interest in this disease. Some aspects of clinical management of OC might be improved by the use of validated TMs, such as differentiating subtypes, defining the most appropriate treatment, monitoring the course of the disease, or predicting clinical outcome. The Food and Drug Administration (FDA) has approved a few TMs for OC: CA125 (cancer antigen 125; monitoring), HE4 (Human epididymis protein; monitoring), ROMA (Risk Of Malignancy Algorithm; HE4+CA125; prediction of malignancy) and OVA1 (Vermillion's first-generation Multivariate Index Assay [MIA]; prediction of malignancy)...
November 20, 2018: Translational Research: the Journal of Laboratory and Clinical Medicine
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