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https://www.readbyqxmd.com/read/28933340/renal-histology-and-mri-findings-in-a-%C3%A2-37-year-old-japanese-patient-with-autosomal-recessive-polycystic-kidney-disease%C3%A2
#1
Yusuke Ito, Akinari Sekine, Daisuke Takada, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Takeshi Fujii, Kenichi Ohashi, Hiroaki Kikuchi, Shintaro Mandai, Motoko Chiga, Takayasu Mori, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
A 37-year-old Japanese man with a serum creatinine level of 2.5 mg/dL and hepatomegaly was admitted to our hospital for investigation of renal failure. Magnetic resonance imaging (MRI) showed hepatomegaly with small cystic lesions that had high signal intensity on T2-weighted images. There was no splenomegaly, and the kidneys were nearly normal in size with a few small cystic lesions. Renal biopsy revealed that interstitial fibrosis and tubular atrophy affected 60% of the cortex. There was cystic tubular dilation, mainly affecting the distal loop of Henle and distal tubules, since immunohistochemical staining of the dilated tubules was positive for cytokeratin 7 and Tamm-Horsfall protein but was negative for aquaporin 3 and CD10...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28923625/genitourinary-malignancies-in-transplant-or-dialysis-patients-the-frequency-of-two-newly-described-2016-world-health-organization-histopathologic-types
#2
A Billis, L L L Freitas, L B E Costa, I S Barreto, M A Asato, K S Araujo, D M Losada, A P Herculiani, G V B S Tabosa, B C Zaidan, G L P Oliveira, L Q A Bastos, R M Rocha
BACKGROUND: The aim of this study was to revise the histopathologic types of neoplasias in the genitourinary tract and determine the frequency of 2 new entities included in the 2016 book of World Health Organization classification of renal tumors. It is not established so far whether these 2 recently described tumors are the most frequent in association with end-stage kidney disease. METHODS: In a retrospective analysis, we revised the histopathologic type of 37 genitourinary tumors from 21 patients in dialysis and/or submitted to renal transplantation from 2003 to 2016 aiming to find the frequency of acquired cystic disease-associated renal cell carcinoma and clear cell papillary (tubulopapillary) renal cell carcinoma...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28905347/-mixed-epithelial-and-stromal-tumour-of-the-kidney-a-case-report
#3
G Brüwer, C M Naumann, M V Petersdorff, K Jünemann, D Osmonov
Mixed epithelial and stromal tumours of the kidney are rare. Histologically, they are characterised by a complex of epithelium and stroma with cystic and solid areas. They usually occur in perimenopausal women receiving hormone replacement with oestrogen. Typical symptoms are haematuria, flank pain and a palpable mass, with more and more authors reporting incidentally diagnosed tumours. This also applies to our case report. We are reporting the first published case in Germany: a 60-year-old female with a mixed epithelial and stromal tumour of the right kidney...
September 13, 2017: Aktuelle Urologie
https://www.readbyqxmd.com/read/28903517/association-of-vancomycin-trough-concentration-with-response-to-treatment-for-acute-pulmonary-exacerbation-of-cystic-fibrosis
#4
Nicholas M Fusco, Richard Francisconi, Calvin J Meaney, Desiree Duman, Carla A Frederick, William A Prescott
Background: Our goal was to determine the relationship between serum vancomycin trough concentrations (VTCs) and changes in pulmonary function among individuals with an acute pulmonary exacerbation (APE) of cystic fibrosis (CF). Methods: We included subjects who were ≥6 years of age, were hospitalized for an APE of CF between May 1, 2012, and April 30, 2014, were administered vancomycin for ≥48 hours, and had a history of airway infection with methicillin-resistant Staphylococcus aureus...
September 1, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28889233/adenoid-cystic-carcinoma-of-the-cavernous-sinus-otolaryngological-sequelae-of-therapy-case-report
#5
Edyta Dzięciołowska-Baran, Aleksandra Gawlikowska-Sroka
A 60-year-old woman, otherwise in a good health condition, was first admitted to the hospital with a year-long tingling sensations of the right half of the face, which gradually turned into severe neuralgia corresponding to maxillary (V2) and mandibular (V3) branches of the right trigeminal nerve. MRI scans of the head revealed an unusual hyperplastic or inflammatory changes along the brain base, cavernous sinus extending toward the pterygopalatine fossa, and lateral pterygoid on the right side. Meningioma was suspected and neuralgia was treated conservatively...
September 10, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28877884/aberrant-smad3-phosphoisoforms-in-cyst-lining-epithelial-cells-in-the-cpk-mouse-a-model-of-autosomal-recessive-polycystic-kidney-disease
#6
Taketsugu Hama, Koichi Nakanishi, Masashi Sato, Hironobu Mukaiyama, Hiroko Togawa, Yuko Shima, Masayasu Miyajima, Kandai Nozu, Shizuko Nagao, Hisahide Takahashi, Mayumi Sako, Kazumoto Iijima, Norishige Yoshikawa, Hiroyuki Suzuki
Cystic epithelia acquire mesenchymal-like features in polycystic kidney disease (PKD). In this phenotypic alteration, it is well known that transforming growth factor (TGF)-β/Smad3 signaling is involved, however, there is emerging new data on Smad3 phosphoisoforms: Smad3 phosphorylated at linker regions (pSmad3L) and COOH terminal regions (pSmad3C) and both (pSmad3L/C). pSmad3L/C has a pathological role in colorectal cancer. Mesenchymal phenotype-specific cell responses in TGF-β/Smad3 pathway are implicated in carcinomas...
September 6, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28877071/uncommon-localization-of-extrarenal-xp11-2-translocation-associated-renal-cell-carcinoma-rcc-case-report
#7
Jaudah Ahmed Al-Maghrabi, Mohamad Nidal Khabaz
The World Health Organization has recognized Xp11.2 translocation-associated renal cell carcinoma (RCC) as a distinct neoplasm that arises within the kidney. Although many reports of extrarenal carcinoma may be found in the literature, to the best of our knowledge, Xp11 translocation-associated RCC with intact kidneys has not been documented. This report describes a multilobulated right retroperitoneal soft tissue mass (7.9×5.3×12.6 cm) of a 37-year-old man complaining of abdominal pain in the right side...
September 4, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28862701/deletion-of-nedd4-2-results-in-progressive-kidney-disease-in-mice
#8
Tanya L Henshall, Jantina A Manning, Omri S Alfassy, Pranay Goel, Natasha A Boase, Hiroshi Kawabe, Sharad Kumar
NEDD4-2 (NEDD4L), a ubiquitin protein ligase of the Nedd4 family, is a key regulator of cell surface expression and activity of the amiloride-sensitive epithelial Na(+) channel (ENaC). While hypomorphic alleles of Nedd4-2 in mice show salt-sensitive hypertension, complete knockout results in pulmonary distress and perinatal lethality due to increased cell surface levels of ENaC. We now show that Nedd4-2 deficiency in mice also results in an unexpected progressive kidney injury phenotype associated with elevated ENaC and Na(+)Cl(-) cotransporter expression, increased Na(+) reabsorption, hypertension and markedly reduced levels of aldosterone...
September 1, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28856401/applications-of-contrast-enhanced-ultrasound-in-the-kidney
#9
REVIEW
Brittany Kazmierski, Corinne Deurdulian, Hisham Tchelepi, Edward G Grant
Incidental discovery of renal lesions on cross-sectional imaging studies performed for other indications is not uncommon. With the increased reliance on medical imaging, the number of incidentally detected renal lesions has also grown over time. While simple cysts account for the majority of these lesions, the presence of complex features within a cystic lesion, such as septations and solid components, can present a confusing picture. Solid lesions, too, can be indeterminate, and distinguishing between benign solid masses (like lipid-poor angiomyolipomas and oncocytomas) and renal cell carcinoma affects patient management and can prevent unnecessary interventions...
August 30, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28838432/autosomal-dominant-polycystic-kidney-disease-transplant-recipients-after-kidney-transplantation-a-single-center-experience
#10
L Illesy, D Á Kovács, R P Szabó, A B L Asztalos, B Nemes
Kidney transplantation is indicated for end-stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) causes structural degeneration of the kidney and eventually becomes end-stage renal disease. ADPKD patients usually have several renal and nonrenal complications. We analyzed our kidney transplantation activities between 1991 and 2010 regarding ADPKD. We followed up with patients to December 31, 2016. Data were collected as patient and graft survival rates, the prevalence of polycystic manifestation of the gastrointestinal tract and other organs, and the attendance of urinary tract infection...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28826940/distinct-oxylipin-alterations-in-diverse-models-of-cystic-kidney-diseases
#11
Md Monirujjaman, Jessay G Devassy, Tamio Yamaguchi, Nikhil Sidhu, Masanori Kugita, Melissa Gabbs, Shizuko Nagao, Jing Zhou, Amir Ravandi, Harold M Aukema
Cystic kidney diseases are characterized by multiple renal cysts and are the leading cause of inherited renal disease. Oxylipins are bioactive lipids derived from fatty acids formed via cyclooxygenase, lipoxygenase and cytochrome P450 activity, and are important regulators of renal health and disease. Oxylipins are altered in nephronophthisis, a type of cystic kidney disease. To further investigate and to determine whether other cystic renal diseases share these abnormalities, a targeted lipidomic analysis of renal oxylipins was performed in orthologous models of autosomal dominant polycystic kidney disease 1 (Mx1Cre(+)Pkd1(flox/flox) mouse) and 2 (Pkd2(ws25/-) mouse), autosomal recessive polycystic kidney disease (PCK rat) and nephronophthisis (jck/jck mouse)...
August 18, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28826877/cystic-diseases-of-childhood-a-review
#12
REVIEW
Shivani Kwatra, Vinod Krishnappa, Christiane Mhanna, Taryn Murray, Robert Novak, Sidharth Kumar Sethi, Deepak Kumar, Rupesh Raina
Renal cystic lesions are considered the most common abnormality associated with the kidneys. Most renal cysts are usually uncomplicated simple cysts that are not life-threatening; however, fatal renal cystic diseases can develop from these space-occupying lesions. Although renal cystic diseases are similar in presentation, they possess distinct features, variable prognoses, and complications later in life. Early identification and effective management of these respected diseases has led to longer survival rates and better quality of life...
August 4, 2017: Urology
https://www.readbyqxmd.com/read/28817404/primary-renal-sarcomas-with-bcor-ccnb3-gene-fusion-a-report-of-2-cases-showing-histologic-overlap-with-clear-cell-sarcoma-of-kidney-suggesting-further-link-between-bcor-related-sarcomas-of-the-kidney-and-soft-tissues
#13
Pedram Argani, Yu-Chien Kao, Lei Zhang, Carlos Bacchi, Andres Matoso, Rita Alaggio, Jonathan I Epstein, Cristina R Antonescu
We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Both neoplasms demonstrated immunoreactivity for BCOR, cyclin D1, TLE1, and SATB2 in the spindle neoplastic cells and negativity in the prominent capillary vasculature...
August 16, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28816297/-perirenal-cystic-lymphangioma-in-an-adult-a-case-report-and-literature-review
#14
W He, Y C Hao, H Z Xia, R Z Ma, B Yang, J Lu
Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28815891/cystic-kidneys-in-fetal-walker-warburg-syndrome-with-pomt2-mutation-intrafamilial-phenotypic-variability-in-four-siblings-and-review-of-literature
#15
Marwa M Nabhan, Nour ElKhateeb, Daniela A Braun, Sungho Eun, Sahar N Saleem, Heon YungGee, Friedhelm Hildebrandt, Neveen A Soliman
Walker-Warburg syndrome (WWS) is a severe form of congenital muscular dystrophy secondary to α-dystroglycanopathy with muscle, brain, and eye abnormalities often leading to death in the first weeks of life. It is transmitted in an autosomal recessive pattern, and has been linked to at least 15 different genes; including protein O-mannosyltransferase 1 (POMT1), protein O-mannosyltransferase 2 (POMT2), protein O-mannose beta-1,2-N acetylglucosaminyltransferase (POMGNT1), fukutin (FKTN), isoprenoid synthase domain-containing protein (ISPD), and other genes...
August 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#16
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28807340/unusual-locations-of-hydatid-disease-a-10-year-experience-from-a-tertiary-reference-center-in-western-turkey
#17
Eylul Gun, Demet Etit, Dilara O Buyuktalanci, Fulya Cakalagaoglu
INTRODUCTION: Hydatid disease is an endemic parasitic infection caused by Echinococcus granulosus mostly seen in the Mediterranean countries. The most affected organ is the liver, however hydatidosis can be found anywhere in the human body. METHODS: The records of patients who were diagnosed with hydatid disease in our hospital from December 2005 to February 2016 were analyzed retrospectively. The cases were evaluated and recorded depending on their gender, age and the localization of the cysts...
August 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28797981/rhabdoid-meningioma-arising-concurrent-in-pulmonary-and-intracranial-with-a-rare-malignant-clinical-progression-case-report-and-literature-review
#18
Peng Zhao, Ning Li, Jinfeng Cao, Xiangtao Lin, Changhu Liang
BACKGROUND: Rhabdoid meningioma (RM) is an unusual variant of meningioma, classified as WHO grade III. Although its recurrence is common, extracranial metastasis is rare and usually misdiagnosed. The transfer mechanism and pathway are ambiguous; once it develops, there is a poor prognosis and no effective management. The present case is the first report on concurrent intracranial and pulmonary RM with rapid and widespread metastasis. We hope this report can be a helpful reference for clinicians and radiologists...
August 7, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28794366/regression-of-crizotinib-associated-complex-cystic-lesions-after-switching-to-alectinib
#19
Kageaki Taima, Hisashi Tanaka, Yoshihito Tanaka, Masamichi Itoga, Shingo Takanashi, Sadatomo Tasaka
Crizotinib, which is effective in patients with anaplastic lymphoma kinase (ALK) positive non-small cell lung cancer, is sometimes associated with the generation of complex renal cysts. A 56-year-old man with ALK positive adenocarcinoma received crizotinib. Ten months after the introduction of crizotinib, a cystic lesion developed from his right kidney to the iliopsoas muscle, accompanied by fever, anemia, and hypoproteinemia. After 17 months of treatment, crizotinib was switched to alectinib, followed by the recovery of hypoproteinemia and systemic inflammation...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28794066/insights-into-cellular-and-molecular-basis-for-urinary-tract-infection-in-autosomal-dominant-polycystic-kidney-disease
#20
Chao Gao, Long Zhang, Ye Zhang, Darren Paul Wallace, Reynold I Lopez-Soler, Paul J Higgins, Wenzheng Zhang
Urinary tract infection (UTI) is a broad term referring to an infection of the kidneys, ureters, bladder and/or urethra. Due to its prevalence, frequent recurrence and rising resistance to antibiotics, UTI has become a challenge in clinical practice. Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic disorder of the kidney and is characterized by the growth of fluid-filled cysts in both kidneys. Progressive cystic enlargement, inflammation and interstitial fibrosis result in nephron loss with subsequent decline in kidney function...
August 9, 2017: American Journal of Physiology. Renal Physiology
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