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Chromogenic in situ hybridization (CISH) and RNA-sequencing of the tumor were positive for FGF23 (CISH) and the transcriptional marker FN1-FGFR1, a novel fusion gene between fibronectin (FN1) and Fibroblast Growth Factor Receptor 1 (FGFR1), previously determined to be present in the majority of TIO-associated tumors. This case demonstrates the notion that rare and diagnostically challenging disorders like TIO can be undiagnosed and/or misdiagnosed for many years, even by experienced clinicians and routine lab testing. It also underscores the power of novel tools available to clinicians such as gene panels, CISH, and RNA sequencing, and their ability to characterize TIO and its related tumors in the context of several phenotypically similar diseases.Severely injured patients are beleaguered by complications during convalescence, such as dysregulated biomineralization. Paradoxically, severely injured patients experience the loss of bone (osteoporosis), resulting in diminished skeletal integrity and increased risk of fragility fractures; yet they also accrue mineralization in soft tissues, resulting in complications such as heterotopic ossification (HO). The pathophysiology leading to dysregulated biomineralization in severely injured patients is not well defined. It has been postulated that these pathologies are linked, such that mineralization is "transferred" from the bone to soft tissue compartments. The goal of this study was to determine if severe injury-induced osteoporosis and soft tissue calcification are temporally coincident following injury. Using a murine model of combined burn and skeletal muscle injury to model severe injury, it was determined that mice developed significant progressive bone loss, detectable as early as 3 days post injury, and marked soft tissue mineralization by 7 days after injury. The observed temporal concordance between the development of severe injury-induced osteoporosis and soft tissue mineralization indicates the plausibility that these complications share a common pathophysiology, though further experiments are required.
Assessment of liver fibrosis is essential for the management of liver disease. Although liver biopsy is the gold-standard modality for the diagnosis of liver fibrosis, it has some limitations. Thus, other methods are required to overcome the disadvantages of a liver biopsy. T1ρ magnetic resonance imaging (MRI) values are potential biomarkers for liver cirrhosis. This study aimed to assess the relationship between T1ρ MRI values and liver fibrosis severity by measuring the correlation between T1ρ values and shear wave elastography (SWE) values, which are routinely used for the diagnosis of liver fibrosis.
T1ρ imaging and SWE values were obtained from four healthy volunteers and 16 patients with chronic liver disease. The regions of interest on MR images were drawn and matched with those of the right liver lobe on SWE images.
The mean T1ρ values of the right liver lobe correlated positively with the mean SWE values (Pearson's correlation coefficient 0.783; p < 0.0001; 95 % confidence interval 0.623-0.880).
The mean T1ρ values of the right liver lobe may be correlated with the severity of liver fibrosis.
The mean T1ρ values of the right liver lobe may be correlated with the severity of liver fibrosis.
To assess the reproducibility of different epicardial fat measurement and their association with other adiposity measurements in HIV-infected and non-HIV-infected patients.
In this cross-sectional study, 167 HIV-infected and 58 non-HIV-infected consecutive participants (200 males; mean age 56 years) with low/intermediate cardiovascular risk were recruited between 2012 and 2017 from a large prospective cohort and underwent non-contrast cardiac CT. Two independent observers measured epicardial fat volume, area and thickness in all participants. For intra-observer agreement, one observer did a second assessment in a subset of 40 patients. Agreement was assessed with the intraclass correlation coefficient (ICC). Pearson's correlation was estimated to assess the association between epicardial fat, body-mass index (BMI) and dual-energy x-ray absorptiometry (DEXA) derived percentage of body fat.
Inter-observer agreement was excellent for epicardial fat volume (ICC 0.75) and area (ICC 0.95) and good for epicarddered over other CT assessment methods when quantifying epicardial fat in HIV patients.Pediatric obesity is a major health concern today, which pre-disposes individuals to metabolic syndrome (MS), and the risk of premature cardiovascular disease (CVD). Use of carotid intima media thickness (CIMT) is recognized as non-invasive way to assess vascular health. The objective of this study was to determine which MBS risk factors has an influence on increasing one's risk of an increased CIMT in children. In southern Maine 189 children (age 10.52 ± .52 years) had their MBS risk factors and CIMT assessed. Based on CIMT, children were divided into quartiles and compared to MBS risk factors. Children in the highest quartile for CIMT had the highest waist circumference (P less then .05) compared to all other groups, using a one-way analysis of variance. No other MBS risk factors had an influence on CIMT. It appears early identification of children with an elevated WC may be beneficial in identifying children at risk of premature CVD.Background Cystic Fibrosis (CF) is a chronic disease associated with low sodium status. The patients are usually treated with oral sodium chloride to control the side effects of low sodium status. Therefore, the fractional excretion of sodium (FENa) was assessed in patients with cystic fibrosis (CF) treated with oral sodium chloride (NaCl). Methods This was a prospective cross-sectional study that was conducted on forty children with cystic fibrosis who were under treatment with oral NaCl and were referred to Imam Hossein Hospital-Isfahan-Iran between 2017 to 2019. The patients were under treated with 2-4 mEq/kg per day oral NaCl and urinary and plasma sodium and creatinine, as well as FENa, were assessed after three months of taking NaCl. Also the patients were compared in terms of efficacy of treatment based on sodium level (between 135 and 145 mmol/L) and acceptable FENa level (between 0.5% and 1.5%). The sensitivity and specificity of FeNa and plasma sodium were assessed with ROC curve test. Results Plasma sodium was normal in 65% of treated patients, and FENa was also normal range in 47.5% of treated patients. The treatment also was desirable for 35% of the patients. The sensitivity and specificity of FeNa were 42.9% and 57.7%, respectively, and the sensitivity and specificity of plasma sodium were 85.7% and 26.9%, respectively. Conclusion Using of plasma sodium had higher sensitivity than FeNa and FeNa had higher specificity than plasma sodium to follow up of patients with CF.
To establish face, content and construct validity of an innovative fruit-tissue (apple) based transurethral resection of prostate (TURP) training model devised at our institute.
Six consultants, three fellows and 16 residents performed TURP on the new fruit-tissue (apple) based TURP model for ten minutes after watching a demo-video. The procedure was videographed. At the end, participants answered a set of questionnaires regarding their experience. The video and the apple both were examined by blinded separate assessors based on pre-decided parameters. Statistical analysis was done to find out the face, content and construct validity of the training model.
Participants were divided into two groups, expert and novice, based TURP surgery experience. The model was positively rated (lowest median value 4) by all novice for its 'realism' and 'acceptability'. The expert group also felt that the model reproduced real TURP experience (lowest median value 4). Thus, both face and content validity were established. The expert group resected more tissue (18.3±2.5 gm vs 10.3±3.4 gm; P<0.001) with less irrigation fluid (1566.6±187.0 ml vs 2112.5±344.2 ml; P<0.001) removing more chips (39.8±6.2 vs 25.6±3.0; P<0.001) and orientated themselves faster (63.3±12.2 sec vs 90.3±12.7 sec; P<0.001). The assessors' subjective evaluation of videos and apples both favored the experts. The model can differentiate 'expert' from 'novice', thus establishing 'construct validity'.
The new fruit-tissue (apple) based TURP model is a realistic and acceptable TRUP training model with proven face, content and construct validity.
The new fruit-tissue (apple) based TURP model is a realistic and acceptable TRUP training model with proven face, content and construct validity.
To report 2 children with acute hepatic myelopathy after hepatitis A infection who recovered completely after living donor liver transplantation.
All the children admitted into liver intensive care unit (LICU) from November 1st 2018 to 31st October 2019, were evaluated for the neurological features. The data was collected from the admission register of the LICU unit in children below 15 years age. Medical records of these children were reviewed and data collected. Established clinical criteria were used to categorize the various grades of hepatic encephalopathy/myelopathy.
37 children were seen over 1-year period between 6 months to 15 years age. There were 24 male(64.9%) and 13 females. Acute liver failure was seen in 19 (51.3%) and acute on chronic liver failure in 18 (48.7%). There were 10 cases of hepatitis A in acute liver failure group,10 of 19 cases (52.6%), while Wilson's disease and undetermined etiology group formed the chronic group. 2 cases of hepatic myelopathy were seen in acute liver failure following hepatitis A infection. Both these children underwent live liver donor transplantation and recovered completely. Further in hepatitis A group,3 children had spontaneous recovery, 4 died and 1 child was discharged with end of life care. Overall out of all 37 children with liver failure,20 (54%) were discharged, 6 (16.2%) were advised end of life care and 11 (29.8%) died.
Two cases (10.5%) of reversible hepatic myelopathy were seen in acute liver failure group of 19 cases. 18 out of 37 (48.6%) children had residual neurological features at discharge time.
Two cases (10.5%) of reversible hepatic myelopathy were seen in acute liver failure group of 19 cases. 18 out of 37 (48.6%) children had residual neurological features at discharge time.Obesity is a major risk factor for nonalcoholic steatohepatitis (NASH). Although weight loss has been shown to reverse histologic features of NASH, lifestyle intervention alone is often challenging and unfeasible. see more In this case report, we discuss the effects of intragastric balloon (IGB) therapy on steatosis, fibrosis, and portal pressures. We also demonstrate that improvement in histologic features persist at least 6 months after IGB removal. Although there are little data thus far to support IGB therapy in the treatment of NASH, our case provides evidence of the potential benefit of IGB on improving metabolic parameters and markers of liver fibrosis.Pancreatic fistulas are rare complications of chronic pancreatitis, typically caused when disruption of the pancreatic duct causes leakage of pancreatic fluid that erodes through neighboring organs and structures. Pancreatic fistulas to the pericardium and pleural spaces are extremely rare, and cases of multiple fistulas tracking from the pancreas have not been reported before. Management of these fluid collections is challenging with no consensus described in the current literature. We report a case of a patient with concurrent pancreaticopericardial and pancreaticopleural fistulas who improved with endoscopic management.
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