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https://www.readbyqxmd.com/read/29643536/-genetic-diagnosis-of-caroli-syndrome-with-autosomal-recessive-polycystic-kidney-disease-a-case-report-and-literature-review
#1
X Y Yang, L P Zhu, X Q Liu, C Y Zhang, Y Yao, Y Wu
This case report is about one genetically specified diagnosed infant case of Caroli syndrome with autosomal recessive polycystic kidney disease (ARPKD) in China. The patient in this case report was an eight-month infant boy with an atypical onset and the main clinical manifestation was non-symptomatic enlargement of the liver and kidneys. The imaging study demonstrated a diffused cystic dilatation of intrahepatic bile ducts as well as polycystic changes in bilateral kidneys. The basic blood biochemical tests indicated a normal hepatorenal function...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29543508/primary-cilia-disruption-differentially-affects-the-infiltrating-and-resident-macrophage-compartment-in-the-liver
#2
Kurt A Zimmerman, Cheng Jack Song, Nancy Gonzalez-Mize, Zhang Li, Bradley K Yoder
Hepatorenal fibrocystic disease (HRFCD) is characterized by cysts in the kidney and liver with associated fibrosis and is the result of defects in proteins required for cilia function or assembly. Previous reports indicate that macrophages, mainly M2-like macrophages, contribute to HRFCD, although the origin (yolk-sac derived resident macrophages vs bone-marrow derived infiltrating macrophages) and contribution of these cells to the observed phenotypes is unknown. Herein, we utilize a congenital model of cilia dysfunction (IFT88Orpk ) to study the importance of macrophages in HRFCD...
March 15, 2018: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/29432428/plac1-as-a-serum-biomarker-for-breast-cancer
#3
Hongyan Yuan, Vincent Chen, Marc Boisvert, Claudine Isaacs, Robert I Glazer
Placental-specific protein 1 (PLAC1) is an X-linked trophoblast gene that is re-expressed in several malignancies, including breast cancer, and is therefore a potential biomarker to follow disease onset and progression. Sera from 117 preoperative/pretreatment breast cancer patients and 51 control subjects, including those with fibrocystic disease, were analyzed for the presence of PLAC1 protein as well as its expression by IHC in tumor biopsies in a subset of subjects. Serum PLAC1 levels exceeded the mean plus one standard deviation (mean+SD) of the level in control subjects in 67% of subjects with ductal carcinoma in situ (DCIS), 67% with HER2+ tumors, 73% with triple-negative cancer and 73% with ER+/PR+ tumors...
2018: PloS One
https://www.readbyqxmd.com/read/29400375/breast-cystic-lesions-not-so-simple-after-all-an-ultrasonographic-tactical-approach
#4
Anca Ileana Ciurea, Cătălin George Iacoban, Hanelore Argentina Herţa, Cristiana Augusta Ciortea
A wide spectrum of breast diseases breast diseases have or exhibit at some point in their natural history a complex cystic aspect. Through the lens of B-mode and Doppler ultrasound with sonoelastographic augmentation, conditions ranging from the utterly benign fibrocystic change to the most intricate encysted or cystically degenerated cancerous lesions, are covered in detail in this review. The aim of this paper is to familiarize the practitioner with the ultrasonographic aspects of complex breast cystic masses and to implement a gradual, strategic approach in the diagnostic algorithm...
February 4, 2018: Medical Ultrasonography
https://www.readbyqxmd.com/read/29323060/spectrum-of-hepatobiliary-cystic-lesions-a-7-year-experience-at-a-tertiary-care-referral-center-in-north-india-and-review-of-literature
#5
Prasenjit Das, Pragya Sharma, Tripti Nakra, Shouriyo Ghosh, Rajni Yadav, Brijnandan Gupta, Gaurav Khanna, K S Madhusudhan, Rajesh Panwar, M K Anoop, Ragini Kilambi, Anand N Singh, Nihar R Dash, Sujoy Pal, Siddhartha Datta Gupta
CONTEXT: Cysts arising from the hepatobiliary tree are a group of heterogeneous lesions with regard to pathogenesis, clinical presentation, and radiological finding. They can be intrahepatic or extrahepatic, developmental, secondary to infective/inflammatory etiologies, as well as neoplastic. This study was conducted to determine the spectrum of hepatobiliary cysts in surgically intervened cases, with regard to their prevalence, histological spectrum, and clinicoradiological correlation, wherever possible...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29290072/operations-for-suspected-neoplasms-in-a-resource-limited-setting-experience-and-challenges-in-the-eastern-democratic-of-congo
#6
Luc Malemo Kalisya, Jacques Fadhili Bake, Richard Bigabwa, David H Rothstein, Sarah B Cairo
INTRODUCTION: Surgery is an essential component of a functional health system, with surgical conditions accounting for nearly 11-15% of world disability. While communicable diseases continue to burden low- and low-middle-income countries, non-communicable diseases, such as cancer, are an important cause of morbidity and mortality worldwide. Preliminary data on malignancies in low- and middle-income countries, specifically in Africa, suggest a higher mortality compared to other regions of the world, a difference partially explained by limited availability of screening and early detection systems as well as poorer access to treatment...
December 31, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/29286226/almost-complete-lack-of-human-cytomegalovirus-and-human-papillomaviruses-genome-in-benign-and-malignant-breast-lesions-in-shiraz-southwest-of-iran
#7
Sahar Bakhtiyrizadeh, Seyed Younes Hosseini, Ramin Yaghobi, Aliakbar Safaei, Jamal Sarvari
Breast cancer ranks as the most common cancer among women worldwide. There have been controversial reports regarding contributions of human papillomaviruses (HPVs) and human cytomegalovirus (HCMV) to its development. The aim of this study was to determine the frequency of HPV and HCMV positivity in benign and malignant breast tumors. Materials and Methods: Formalin fixed paraffin-embedded tissue specimens of 150 breast cancers (invasive ductal and lobular carcinomas) and 150 non-malignant breast lesions (fibroadenomas, fibrocystic disease and adenosis) were examined...
December 29, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/29124658/atypical-hrct-manifestations-of-pulmonary-sarcoidosis
#8
Diletta Cozzi, Elena Bargagli, Alessandro Giuseppe Calabrò, Elena Torricelli, Federico Giannelli, Edoardo Cavigli, Vittorio Miele
PURPOSE: To present our experience of cases of pulmonary sarcoidosis with atypical HRCT patterns found during 2016 focusing on the differential diagnosis to contribute to the difficult role of the radiologist in the disease identification and to help the clinicians to reach the diagnosis. MATERIALS AND METHODS: The HRCT examinations of 47 patients with sarcoidosis were studied retrospectively. All patients had a histopathological confirm of the disease. 29 (61.7%) show a typical pulmonary pattern and 18 (38...
November 9, 2017: La Radiologia Medica
https://www.readbyqxmd.com/read/29090365/cd56-immune-cell-infiltration-and-mica-are-decreased-in-breast-lobules-with-fibrocystic-changes
#9
Daniel Kerekes, Daniel W Visscher, Tanya L Hoskin, Derek C Radisky, Rushin D Brahmbhatt, Alvaro Pena, Marlene H Frost, Muhammad Arshad, Melody Stallings-Mann, Stacey J Winham, Linda Murphy, Lori Denison, Jodi M Carter, Keith L Knutson, Amy C Degnim
PURPOSE: While the role of natural killer (NK) cells in breast cancer therapy has been investigated, little information is known about NK cell function and presence in nonmalignant and premalignant breast tissue. Here, we investigate and quantify NK cell marker CD56 and activating ligand MICA in breast tissue with benign breast disease. METHODS: Serial tissue sections from 88 subjects, 44 with benign breast disease (BBD) who remained cancer-free, and 44 with BBD who later developed cancer, were stained with H&E, anti-MICA, and anti-CD56...
November 1, 2017: Breast Cancer Research and Treatment
https://www.readbyqxmd.com/read/29082377/sub-classification-of-breast-masses-by-fine-needle-aspiration-cytology
#10
C I Madubogwu, C O Ukah, Snc Anyanwu, G U Chianakwana, I V Onyiaorah, Dcd Anyiam
OBJECTIVE: The aim of this study is to evaluate the accuracy of sub-classification of breast diseases using Fine Needle Aspiration cytology (FNAC). MATERIALS AND METHODS: A one-year prospective study of 180 consecutive patients with palpable breast lesions who underwent FNAC and subsequently open surgical biopsy for histological confirmation. FNAC was used to sub-classify breast lesions and then correlated with histological diagnosis. RESULTS: A total of 180 patients were enrolled into the study but only 110 patients with histology report were used for test validity...
October 2017: European Journal of Breast Health
https://www.readbyqxmd.com/read/29052317/defective-ciliogenesis-in-inpp5e-related-joubert-syndrome
#11
Isabel Hardee, Ariane Soldatos, Mariska Davids, Thierry Vilboux, Camilo Toro, Karen L David, Carlos R Ferreira, Michele Nehrebecky, Joseph Snow, Audrey Thurm, Theo Heller, Ellen F Macnamara, Meral Gunay-Aygun, Wadih M Zein, William A Gahl, May Christine V Malicdan
Joubert syndrome is a neurodevelopmental disorder, characterized by malformation of the mid and hindbrain leading to the pathognomonic molar tooth appearance of the brainstem and cerebellum on axial MRI. Core clinical manifestations include hypotonia, tachypnea/apnea, ataxia, ocular motor apraxia, and developmental delay of varying degrees. In addition, a subset of patients has retinal dystrophy, chorioretinal colobomas, hepatorenal fibrocystic disease, and polydactyly. Joubert syndrome exhibits genetic heterogeneity, with mutations identified in more than 30 genes, including INPP5E, a gene encoding inositol polyphosphate 5-phosphatase E, which is important in the development and stability of the primary cilium...
December 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29048330/calibration-and-analysis-of-a-multimodal-micro-ct-and-structured-light-imaging-system-for-the-evaluation-of-excised-breast-tissue
#12
David M McClatchy, Elizabeth J Rizzo, Jeff Meganck, Josh Kempner, Jared Vicory, Wendy A Wells, Keith D Paulsen, Brian W Pogue
A multimodal micro-computed tomography (CT) and multi-spectral structured light imaging (SLI) system is introduced and systematically analyzed to test its feasibility to aid in margin delineation during breast conserving surgery (BCS). Phantom analysis of the micro-CT yielded a signal-to-noise ratio of 34, a contrast of 1.64, and a minimum detectable resolution of 240 μm for a 1.2 min scan. The SLI system, spanning wavelengths 490 nm to 800 nm and spatial frequencies up to 1.37 [Formula: see text], was evaluated with aqueous tissue simulating phantoms having variations in particle size distribution, scatter density, and blood volume fraction...
November 10, 2017: Physics in Medicine and Biology
https://www.readbyqxmd.com/read/28952822/recent-advances-in-the-molecular-diagnosis-of-polycystic-kidney-disease
#13
Carsten Bergmann
Polycystic kidney disease (PKD) is clinically and genetically heterogeneous and constitutes the most common heritable kidney disease. Most patients are affected by the autosomal dominant form (ADPKD) which generally is an adult-onset multisystem disorder. By contrast, the rarer recessive form ARPKD usually already manifests perinatally or in childhood. In some patients, however, ADPKD and ARPKD can phenotypically overlap with early manifestation in ADPKD and only late onset in ARPKD. Progressive fibrocystic renal changes are often accompanied by severe hepatobiliary changes or other extrarenal abnormalities...
November 16, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28846009/metabolic-response-to-omega-3-fatty-acids-and-vitamin-e-co-supplementation-in-patients-with-fibrocystic-breast-disease-a-randomized-double-blind-placebo-controlled-trial
#14
Seyyed Mehdi Mirhashemi, Mehdi Sahmani, Behnaz Salehi, Javad Zavar Reza, Mohsen Taghizadeh, Nushin Moussavi, Bita Badehnoosh, Zatollah Asemi
BACKGROUND: There is scarce data on the effects of omega-3 fatty acids and vitamin E co-supplementation on metabolic status in patients with fibrocystic breast disease (FBD). The current study was carried out to determine the effects of omega-3 fatty acids and vitamin E co-supplementation on metabolic status in patients with FBD. METHODS: A randomized clinical trial was conducted on 56 patients with FBD. Participants were randomly divided into two groups to receive either 1000 mg omega-3 fatty acids plus 400 mg vitamin E (n = 28) or placebo (n = 28) for 12 weeks...
August 2017: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/28649033/danazol-alters-mitochondria-metabolism-of-fibrocystic-breast-mcf10a-cells
#15
Zhazira Irgebay, Banu Yeszhan, Bhaswati Sen, Sultan Tuleukhanov, Ari D Brooks, Richard Sensenig, Zulfiya Orynbayeva
Fibrocystic Breast Disease (FBD) or Fibrocystic change (FC) affects about 60% of women at some time during their life. Although usually benign, it is often associated with pain and tenderness (mastalgia). The synthetic steroid danazol has been shown to be effective in reducing the pain associated with FBD, but the cellular and molecular mechanisms for its action have not been elucidated. We investigated the hypothesis that danazol acts by affecting energy metabolism. Effects of danazol on Mcf10A cells homeostasis, including mechanisms of oxidative phosphorylation, cytosolic calcium signaling and oxidative stress, were assessed by high-resolution respirometry and flow cytometry...
October 2017: Breast: Official Journal of the European Society of Mastology
https://www.readbyqxmd.com/read/28625504/mutations-in-armc9-which-encodes-a-basal-body-protein-cause-joubert-syndrome-in-humans-and-ciliopathy-phenotypes-in-zebrafish
#16
Julie C Van De Weghe, Tamara D S Rusterholz, Brooke Latour, Megan E Grout, Kimberly A Aldinger, Ranad Shaheen, Jennifer C Dempsey, Sateesh Maddirevula, Yong-Han H Cheng, Ian G Phelps, Matthias Gesemann, Himanshu Goel, Ohad S Birk, Talal Alanzi, Rifaat Rawashdeh, Arif O Khan, Michael J Bamshad, Deborah A Nickerson, Stephan C F Neuhauss, William B Dobyns, Fowzan S Alkuraya, Ronald Roepman, Ruxandra Bachmann-Gagescu, Dan Doherty
Joubert syndrome (JS) is a recessive neurodevelopmental disorder characterized by hypotonia, ataxia, abnormal eye movements, and variable cognitive impairment. It is defined by a distinctive brain malformation known as the "molar tooth sign" on axial MRI. Subsets of affected individuals have malformations such as coloboma, polydactyly, and encephalocele, as well as progressive retinal dystrophy, fibrocystic kidney disease, and liver fibrosis. More than 35 genes have been associated with JS, but in a subset of families the genetic cause remains unknown...
July 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28611971/diagnosis-and-management-of-hepatobiliary-complications-in-autosomal-recessive-polycystic-kidney-disease
#17
REVIEW
Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen
Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28598826/the-evaluation-of-the-effect-of-metformin-on-breast-fibrocystic-disease
#18
A Talaei, A Moradi, F Rafiei
No abstract text is available yet for this article.
June 2, 2017: Breast Disease
https://www.readbyqxmd.com/read/28515578/imaging-in-ductal-plate-malformations
#19
Binit Sureka, Archana Rastogi, Chhagan Bihari, Kishore G S Bharathy, Vikrant Sood, Seema Alam
Ductal plate malformations are a heterogenous group of congenital fibrocystic liver diseases resulting from insult to the ductal plate at various stages of embryogenesis. As a result various biliary malformations, cysts, hamartomas and congenital hepatic fibrosis may be seen. We present a radiological pictorial of ductal plate malformations, accurate diagnosis of which is important for clinical management.
January 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28473971/design-and-implementation-of-the-hepatorenal-fibrocystic-disease-core-center-clinical-database-a-centralized-resource-for-characterizing-autosomal-recessive-polycystic-kidney-disease-and-other-hepatorenal-fibrocystic-diseases
#20
Bakri Alzarka, Hiroki Morizono, John W Bollman, Dongkyu Kim, Lisa M Guay-Woodford
Autosomal recessive polycystic kidney disease (ARPKD) and other hepatorenal fibrocystic diseases (HRFD) are relatively rare recessive disorders that constitute an important set of childhood nephropathies. Little is known about fundamental pathogenesis, and advances toward clinical trials will require well-characterized patient cohorts and the development of predictive and prognostic biomarkers. Such studies in rare diseases require greater collaboration than the efforts in common diseases where large patient repositories can be built at a single site...
2017: Frontiers in Pediatrics
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