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https://www.readbyqxmd.com/read/28432469/renal-findings-in-patients-with-mulibrey-nanism
#1
Johanna Sivunen, Susann Karlberg, Jouko Lohi, Niklas Karlberg, Marita Lipsanen-Nyman, Hannu Jalanko
BACKGROUND: Mulibrey nanism (MUL) is a rare inherited disease caused by genetic defects affecting peroxisomal TRIM37 protein. MUL affects multiple organs, leading to growth retardation and early onset type 2 diabetes. We aimed to characterize the structure and function of kidneys and the urinary tract in a large cohort of Finnish MUL patients. METHODS: Ultrasound, magnetic resonance imaging (MRI), and autopsy findings of the kidneys and urinary tract from 101 MUL patients were retrospectively analyzed...
April 22, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28428929/treatment-of-posttubercular-syringomyelia-not-responsive-to-antitubercular-therapy-case-report-and-review-of-literature
#2
Giuseppe Canova, Alessandro Boaro, Enrico Giordan, Pierluigi Longatti
Posttubercular adhesive arachnoiditis is a rare, late complication of tubercular meningitis. Syringomyelia can develop as a consequence of intramedullary cystic lesions and cerebrospinal fluid (CSF) flow disturbance around the spinal cord, even after successful chemotherapy. We reviewed the literature related to posttubercular syringomyelia treatment and suggest a new combined surgical approach. A 25-year-old Nigerian male patient presented with legs numbness, urinary disturbance, and legs weakness. Spinal magnetic resonance revealed a T5-T7 syringomyelia, secondary to adhesive spinal arachnoiditis related to a history of tuberculous meningitis...
April 2017: Journal of Neurological Surgery Reports
https://www.readbyqxmd.com/read/28427128/-the-application-value-of-contrast-enhanced-ultrasound-in-the-surgery-of-renal-masses
#3
M H Liu, J H Shi, Q M H Wangqi, Y Q Lin, Z X Sun
Objective: To investigate the clinical value of contrast-enhanced ultrasound in the diagnosis of renal masses. Methods: A total of 50 patients with renal masses who underwent partial nephrectomy were analyzed from January 2014 to October 2016 in the Department of Urology of China-Japan Union Hospital of Jilin University.In the intraoperative contrast-enhanced ultrasound, the characteristics of ultrasound imaging were recorded including location, size, boundary, echo, real-time internal and peripheral blood and renal pelvis distance, artery, guidance on the selection of surgical methods, intraoperative real-time estimation of the extent of tumor resection...
April 18, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28425757/the-prevalence-and-characteristics-of-a-subcortical-cystic-lesion-at-the-subspinous-region-of-the-knee
#4
Ji Ye Son, Young Cheol Yoon, Wook Jin, Jang Gyu Cha
Background Although we observed that subcortical cysts near the attachment of the meniscus or cruciate ligaments are frequently seen with a tear or degeneration of the adjacent meniscus and/or cruciate ligament, there is no large study describing the prevalence, distribution, and associated findings of these cysts. Purpose To evaluate the prevalence and distribution of subcortical cysts near the attachments of the meniscus or cruciate ligaments and evaluate pathologies of the meniscus or cruciate ligaments using magnetic resonance imaging (MRI)...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#5
Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E Kleiner, David Herion, T Jake Liang, Jay H Hoofnagle, Milica Chernick, Theo Heller
BACKGROUND & AIMS: Cystic fibrosis liver disease (CFLD), a leading cause of death in cystic fibrosis (CF), is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. METHODS: A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with a newly proposed CFLD criteria...
April 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28421458/surgical-management-of-pancreatic-cysts-a-shifting-paradigm-toward-selective-resection
#6
REVIEW
Jon M Gerry, George A Poultsides
Due to the widespread use of high-quality cross-sectional imaging, pancreatic cystic neoplasms are being diagnosed with increasing frequency. Clinicians are therefore asked to counsel a growing number of patients with pancreatic cysts diagnosed incidentally at an early, asymptomatic stage. Over the last two decades, accumulating knowledge on the biologic behavior of these neoplasms along with improved diagnostics through imaging and endoscopic cyst fluid analysis have allowed for a selective therapeutic approach toward these neoplasms...
April 18, 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28419866/o-aminobenzoyl-s-nitrosoglutathione-a-fluorogenic-cell-permeable-pseudo-substrate-for-s-nitrosoglutathione-reductase
#7
Bei Lei Sun, Lisa Palmer, Shagufta Rehman Alam, Itunuoluwa Adekoya, Kathleen Brown-Steinke, Ammasi Periasamy, Bulent Mutus
S-nitrosoglutathione reductase (GSNOR) is a multifunctional enzyme. It can catalyze NADH-dependent reduction of S-nitrosoglutathione (GSNO); as well as NAD(+)-dependent oxidation of hydroxymethylglutathione (HMGSH; an adduct formed by the spontaneous reaction between formaldehyde and glutathione). While initially recognized as the enzyme that is involved in formaldehyde detoxification, increasing amount of research evidence has shown that GSNOR also plays a significant role in nitric oxide mediated signaling through its modulation of protein S-nitrosothiol abundance via transnitrosation reactions with GSNO...
April 15, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28417852/nuclear-medicine-in-pediatric-nephro-urology-an-overview
#8
REVIEW
Iosif Mendichovszky, Bernardita Troncoso Solar, Naima Smeulders, Marina Easty, Lorenzo Biassoni
In the context of ante-natally diagnosed hydronephrosis, the vast majority of children with a dilated renal pelvis do not need any surgical treatment, as the dilatation resolves spontaneously with time. Slow drainage demonstrated at Tc-99m-mercaptoacetyltriglycine (MAG3) renography does not necessarily mean obstruction. Obstruction is defined as resistance to urinary outflow with urinary stasis at the level of the pelvic-ureteric junction (PUJ) which, if left untreated, will damage the kidney. Unfortunately this definition is retrospective and not clinically helpful...
May 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28416122/patients-with-a-resected-pancreatic-mucinous-cystic-neoplasm-have-a-better-prognosis-than-patients-with-an-intraductal-papillary-mucinous-neoplasm-a-large-single-institution-series
#9
James F Griffin, Andrew J Page, Georges J Samaha, Adrienne Christopher, Feriyl Bhaijee, Maryam K Pezhouh, Niek A Peters, Ralph H Hruban, Jin He, Martin A Makary, Anne Marie Lennon, John L Cameron, Christopher L Wolfgang, Matthew J Weiss
BACKGROUND/OBJECTIVES: Mucinous cystic neoplasms (MCNs) are rare pancreas tumors distinguished from intraductal papillary mucinous neoplasms (IPMNs) by the presence of ovarian-type stroma. Historical outcomes for MCNs vary due to previously ambiguous diagnostic criteria resulting in confusion with IPMNs. This study seeks to characterize and clarify the clinical features and long-term outcomes of MCNs versus IPMNs in the largest single-institution series of pathology-confirmed MCNs to date...
April 11, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28416105/do-clear-cell-papillary-renal-cell-carcinomas-have-malignant-potential-diolombi-ml-cheng-l-argani-p-epstein-ji-am-j-surg-pathol-december-2015-39-12-1621-1634
#10
Oleksandr N Kryvenko
There have been no recurrences or metastases of clear cell papillary renal cell carcinoma (CCPRCC) in 268 reported cases with follow-up in the English-language literature. We identified all our cases of CCPRCC (1990-2013), reviewing all cases that preceded the formal designation of the entity. Immunohistochemical stains were performed on 32 cases during their initial workup. In addition, stains for carbonic anhydrase IX and cytokeratin 7 were performed on 2 cases, one with atypical follow-up and the other with a more compact morphology, although not performed initially...
April 13, 2017: Urologic Oncology
https://www.readbyqxmd.com/read/28416007/disseminated-alveolar-echinococcosis-resembling-metastatic-malignancy-a-case-report
#11
Laura Caire Nail, Ezequiel Rodríguez Reimundes, Christelle Weibel Galluzzo, Dan Lebowitz, Yasmine Lucile Ibrahim, Johannes Alexander Lobrinus, François Chappuis
BACKGROUND: Alveolar echinococcosis is a potentially lethal zoonosis caused by larval forms of the tapeworm Echinococcus multilocularis. Humans are aberrant intermediate hosts who become infected by ingestion of egg-contaminated food or water or via physical contact with domestic or wild animals that carry the parasite in their small intestine. In humans, the disease usually affects the liver and can spread to other organs causing metastatic infiltration. In this report, we describe an advanced presentation of human alveolar echinococcosis mimicking metastatic malignancy...
April 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28415674/primary-sj%C3%A3-gren-s-syndrome-with-diffuse-cystic-lung-changes-developed-systemic-lupus-erythematosus-a-case-report-and-literature-review
#12
Xiao Liu, Hao Li, Yunhong Yin, Dedong Ma, Yiqing Qu
Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease that can occur as a unique existence (primary Sjögren's syndrome) or merge with other systemic diseases like systemic lupus erythematosus (SLE), rheumatoid arthritis or systemic sclerosis (secondary Sjögren's syndrome). Data on the two diseases occurrence order are inadequate. Primary Sjögren's syndrome (pSS) may relatively uncommonly lead to diffuse cystic lung changes. We represent a female who was diagnosed pSS with diffuse cystic lung alterations developed SLE two years later...
March 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28415140/imaging-modalities-for-characterising-focal-pancreatic-lesions
#13
REVIEW
Lawrence Mj Best, Vishal Rawji, Stephen P Pereira, Brian R Davidson, Kurinchi Selvan Gurusamy
BACKGROUND: Increasing numbers of incidental pancreatic lesions are being detected each year. Accurate characterisation of pancreatic lesions into benign, precancerous, and cancer masses is crucial in deciding whether to use treatment or surveillance. Distinguishing benign lesions from precancerous and cancerous lesions can prevent patients from undergoing unnecessary major surgery. Despite the importance of accurately classifying pancreatic lesions, there is no clear algorithm for management of focal pancreatic lesions...
April 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28413736/targeted-intraspinal-radiofrequency-ablation-for-lumbar-spinal-stenosis
#14
Robert E Jacobson, Michelle Granville, Jesse Hatgis DO
INTRODUCTION: By using a combination of magnetic resonance imaging (MRI) and computed tomography (CT) of the lumbar spine, it is possible to distinguish between spinal stenosis caused by bone compression and specific soft tissue epidural intraspinal lesions that cause localized spinal canal stenosis and neural compression. Examples include facet cysts and yellow ligament hypertrophy. Many of these patients are elderly with medical comorbidities that make open surgery problematic. MATERIALS & METHODS: This is a study of patients with predominantly soft tissue stenosis being treated with targeted intraspinal radiofrequency (RF) heat ablation...
March 10, 2017: Curēus
https://www.readbyqxmd.com/read/28413666/renal-cell-carcinoma-presenting-as-a-simple-renal-cyst-a-case-report
#15
Yanlan Yu, Liang Ma, Zhenghui Wang, Zhigen Zhang
Cases of renal cell carcinoma (RCC) presenting as a simple cyst are extremely rare. We herein report the case of a patient with RCC diagnosed as a simple renal cyst preoperatively. A 39-year-old female patient presented with abdominal pain for 3 months. Ultrasonography and contrast-enhanced computed tomography revealed a simple cyst in the left kidney. The patient underwent laparoscopic decortication of the renal cyst. Biochemical analysis of the cystic fluid revealed unusually low levels of potassium, sodium, calcium and glucose, and the histological examination of the floor of the cyst indicated malignancy...
April 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28413556/totally-cystic-intradural-schwannoma-in-thoracic-region
#16
Sushil Kumar, Raghavendra Gupta, Amit Handa, Rohan Sinha
Spinal schwannomas are benign intradural extramedullary tumors arising from spinal nerve root sheath. They are usually solid or heterogeneously solid. Totally cystic schwannomas are rare entities. Herein, we report a 60-year-old male presenting with backache radiating along the chest wall and weakness of both lower limbs. He had spastic paraparesis. Magnetic resonance imaging revealed a cystic mass in the thoracic region. At operation, the cystic mass was seen to be attached to D4 dorsal rootlets. It was excised in toto and histopathology confirmed it to be a schwannoma...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28413533/persistent-l5-lumbosacral-radiculopathy-caused-by-lumbosacral-trunk-schwannoma
#17
Guive Sharifi, Amin Jahanbakhshi
Schwannomais, usually, benign tumor of nerve sheath that occurs evenly along the spinal cord. Intra-pelvic schwannoma is very rare entity that may arise from lumbosacral nerve roots or from sciatic nerve. Radicular pain of the lower limb as a presenting symptom of pelvic schwannoma is extremely rare. In the current report, the patient is presented with a right sided L5 radicular pain typical of lumbar discopathy. Interestingly, a herniated lumbar disc was noted on lumbosacral magnetic resonance imaging (MRI)...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28408261/use-of-intraoperative-navigation-for-reconstruction-of-the-c1-lateral-mass-after-resection-of-aneurysmal-bone-cyst
#18
Jennifer Neva, Brandon W Smith, Jacob R Joseph, Paul Park
BACKGROUND: Aneurysmal bone cysts (ABC) are rare blood-filled cystic lesions that are found in the long bones and spine. Here, we present a case of an ABC found in the lateral mass and lamina of C1. Lesions in this area provide a surgical challenge due to its difficulty in access as well as its need for reconstruction. We describe a novel use of intraoperative navigation (ION) to assist in the placement of a C1 lateral mass titanium cage. CASE DESCRIPTION: An 18-year-old female presented with headaches and progressive neck pain...
April 10, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28405085/giant-cystic-umbilical-cord-associated-with-patent-urachus-and-intrauterine-fetal-demise
#19
Kira Brooks, Joseph Guileyardo, Don Le, Jesy Joykutty
True cystic structures within the umbilical cord are rare, and when they persist into the second and third trimester, they are often associated with an abnormal karyotype or other developmental abnormalities. Clinically significant pseudocysts resulting from massive edema of the umbilical cord have been associated with a congenitally patent urachus. We present a case of intrauterine fetal demise at 28 weeks' gestation in which cystic dilatation of the umbilical cord was diagnosed prenatally by ultrasound imaging...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28404017/dna-testing-of-pancreatic-cyst-fluid-is-it-ready-for-prime-time
#20
REVIEW
Aatur D Singhi, Marina N Nikiforova, Kevin McGrath
Pancreatic cysts are a clinical quandary in both diagnosis and management. Although many cysts, such as pseudocysts and serous cystadenomas, are benign and can be monitored clinically, mucinous cysts, such as intraductal papillary mucinous neoplasms and mucinous cystic neoplasms, have the potential to progress to pancreatic cancer. Considering the poor prognosis of pancreatic cancer, the detection of a pancreatic cyst can be a source of anxiety for both the patient and physician. This diagnosis in turn can lead to expensive, invasive, and even harmful surveillance and treatment options...
January 2017: Lancet. Gastroenterology & Hepatology
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