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https://www.readbyqxmd.com/read/28523321/autoimmune-hepatitis-with-sclerosing-cholangitis-in-a-patient-with-thiopurine-methyltransferase-deficiency-case-presentation
#1
Sorin Claudiu Man, Cristina Nicoleta Schnell, Valentina Sas, Anca Dana Buzoianu, Dan Gheban
The association between two autoimmune diseases is known in the literature as overlap syndrome. We present the case of an 18-year-old boy, diagnosed at the age of 13 with an overlap syndrome between type I autoimmune hepatitis and sclerosing cholangitis. The response to immunosuppressant therapy was hampered by azathioprine-induced toxicity causing severe pancytopenia, as a result of thiopurine methyltransferase enzyme genetic deficiency. Treatment was replaced by mycophenolate mofetil. Although the relapse rate was reduced, the disease progressed to cirrhosis...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28523127/high-intensity-focused-ultrasound-hifu-ablation-of-benign-thyroid-nodules-a-systematic-review
#2
REVIEW
Brian Hung-Hin Lang, Arnold L H Wu
BACKGROUND: With an increasing number of imaging studies being done nowadays, the number of incidentally discovered thyroid nodules is expected to rise. Although many of these nodules are small and benign in nature, some do grow and may cause pressure and/or thyrotoxic symptoms. Surgical resection has traditionally been recommended for symptomatic nodules but is associated with risk of hypothyroidism, bleeding, infection, and nerve damage. High intensity focused ultrasound (HIFU) is one of the non-surgical thermal ablation techniques that may serve as an alternative in the treatment of benign thyroid nodules...
2017: Journal of Therapeutic Ultrasound
https://www.readbyqxmd.com/read/28518132/mind-the-gap-can-videolaryngoscopy-bridge-the-competency-gap-in-neonatal-endotracheal-intubation-among-pediatric-trainees-a-randomized-controlled-study
#3
S Parmekar, J L Arnold, C Anselmo, M Pammi, J Hagan, C J Fernandes, K Lingappan
BACKGROUND: To study the impact of videolaryngoscopy (VL) on intubation success among pediatric trainees compared with direct laryngoscopy (DL). METHODS: One hundred pediatric residents were enrolled in a randomized, crossover, simulation study comparing VL to DL. Following a didactic session on neonatal intubation, residents intubated a standard neonatal mannequin. Three Neonatal Resuscitation Program (NRP) scenarios were then conducted, followed by a mannequin intubation with the alternate device...
May 18, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28516798/complementary-and-alternative-medicine-use-in-uninsured-children-in-texas
#4
Sanghamitra M Misra, Danielle Guffey, Isabel Roth, Angelo P Giardino
Use of complementary and alternative medicine (CAM) among US children is 12% according to the 2012 National Health Interview Study. Certain pediatric populations have higher CAM use. We studied an uninsured population because limited access to care likely results in higher CAM use. We surveyed 250 uninsured patients in a free pediatric mobile clinic program. In the largely Hispanic population, rate of CAM use in the preceding 12 months was 45% among children and 59% among parents. Ninety-one percent of children who used CAM had parents who used CAM while only 32% of parents used CAM for themselves but did not use CAM for their children ( P < ...
May 1, 2017: Clinical Pediatrics
https://www.readbyqxmd.com/read/28515113/force-sensing-catheters-during-pediatric-radiofrequency-ablation-the-federation-study
#5
Aarti S Dalal, Hoang H Nguyen, Tammy Bowman, George F Van Hare, Jennifer N Avari Silva
BACKGROUND: Based on data from studies of atrial fibrillation ablations, optimal parameters for the TactiCath (TC; St. Jude Medical, Inc) force-sensing ablation catheter are a contact force of 20 g and a force-time integral of 400 g·s for the creation of transmural lesions. We aimed to evaluate TC in pediatric and congenital heart disease patients undergoing ablation. METHODS AND RESULTS: Comprehensive chart and case reviews were performed from June 2015 to March 2016...
May 17, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28510776/patient-characteristics-and-surgical-approach-impacting-simultaneous-to-alternate-prism-cover-test-disparity-after-exotropia-surgery-a-quantitative-look-at-the-difference-in-motor-outcomes
#6
Pavlina S Kemp, Yoon-Hee Chang, Patrice Melvin, Linda R Dagi
PURPOSE: To investigate the relationship between simultaneous prism and cover test (SPCT) and alternate prism and cover test (APCT) outcomes after exotropia surgery, and to identify characteristics associated with significant disparity between them. METHODS: Review of sensorimotor outcomes 2 to 6 months after exotropia surgery identified patients with alignment documented by both SPCT and APCT at the same examination. Two hundred seventy-four and 319 patients had both measurements recorded at distance and near, respectively...
May 15, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28510769/comparison-of-objective-and-subjective-techniques-of-strabismus-measurement-in-adults-with-normal-retinal-correspondence
#7
Derek P Bitner, Ore-Ofe O Adesina, Kai Ding, Bradley K Farris, R Michael Siatkowski
PURPOSE: Accurate measurement of strabismus is vital to proper diagnosis and treatment. Objective and subjective measurement techniques can be used. The authors hypothesized that subjective measurement techniques would measure larger deviations than objective ones. METHODS: Adults with strabismus, visual acuity greater than 20/50 in each eye, and normal retinal correspondence were measured in primary gaze at distance and near using the alternate prism and cover test and the red glass test...
May 15, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28510691/nf-%C3%AE%C2%BAb-upregulation-through-epigenetic-silencing-of-ldoc1-drives-tumor-biology-and-specific-immunophenotype-in-group-a-ependymoma
#8
Andrea M Griesinger, Davis A Witt, Sydney T Grob, Sabrina R Georgio Westover, Andrew M Donson, Bridget Sanford, Jean M Mulcahy Levy, Randall Wong, Daniel C Moreira, John A DeSisto, Ilango Balakrishnan, Lindsey M Hoffman, Michael H Handler, Kenneth L Jones, Rajeev Vibhakar, Sujatha Venkataraman, Nicholas K Foreman
Background.: Inflammation has been identified as a hallmark of high-risk Group A (GpA) ependymoma (EPN). Chronic interleukin (IL)-6 secretion from GpA tumors drives an immune suppressive phenotype by polarizing infiltrating monocytes. This study determines the mechanism by which IL-6 is dysregulated in GpA EPN. Methods.: Twenty pediatric GpA and 21 pediatric Group B (GpB) EPN had gene set enrichment analysis for MSigDB Hallmark gene sets performed. Protein and RNA from patients and cell lines were used to validate transcriptomic findings...
May 16, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28509677/stool-cytomegalovirus-polymerase-chain-reaction-for-the-diagnosis-of-cytomegalovirus-related-gastrointestinal-disease
#9
Noparat Prachasitthisak, Pornthep Tanpowpong, Chatmanee Lertudomphonwanit, Suporn Treepongkaruna, Sophida Boonsathorn, Napat Angkathunyakul, Pattana Sornmayura, Wasun Chantratita
OBJECTIVES: The diagnosis of cytomegalovirus-related gastrointestinal disease (CMV-GI disease) still requires histopathology, but biopsy is considered invasive. Stool CMV PCR has been reported in adults as an alternative method to diagnose this condition; hence, the results between studies are discrepant. Moreover, no pediatric studies on stool CMV real-time PCR in CMV-GI disease have been carried out. Here, we evaluate the value of stool CMV real-time PCR in detecting CMV-GI disease among immunocompromised children...
May 15, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28506490/early-range-of-motion-exercise-in-pediatric-patients-with-olecranon-fractures-treated-with-tension-band-suture-with-double-loops-and-double-knots
#10
Tae Hun Kim, Doo-Hyung Lee, Kyeong Jin Han, Wan Sun Choi, Jae Ho Cho
BACKGROUND: Pediatric patients with olecranon fractures are uncommon. The tension band suture technique was introduced to reduce the burden of implant removal and other complications. However, to our knowledge, early range of motion (ROM) exercise has not been introduced in this population of patients. Double Vicryl loops and knots with 2 cross-pins are used to maintain the benefits of the tension band suture technique and to enhance fixation tensile strength. We believe that early ROM exercises could be achieved without nonunion or fixation failure...
May 11, 2017: Journal of Shoulder and Elbow Surgery
https://www.readbyqxmd.com/read/28505015/virologic-response-to-first-line-efavirenz-or-nevirapine-based-antiretroviral-therapy-in-hiv-infected-african-children
#11
Adeodata Kekitiinwa, Alexander J Szubert, Moira Spyer, Richard Katuramu, Victor Musiime, Tawanda Mhute, Sabrina Bakeera-Kitaka, Oscar Senfuma, Ann Sarah Walker, Diana M Gibb
BACKGROUND: Poorer virologic response to nevirapine- versus efavirenz-based antiretroviral therapy (ART) has been reported in adult systematic reviews and pediatric studies. METHODS: We compared drug discontinuation and viral load (VL) response in ART-naïve Ugandan/Zimbabwean children ≥3 years of age initiating ART with clinician-chosen nevirapine versus efavirenz in the ARROW trial. Predictors of suppression <80, <400 and <1000 copies/mL at 36, 48 and 144 weeks were identified using multivariable logistic regression with backwards elimination (P = 0...
June 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28504355/home-high-flow-nasal-cannula-as-a-treatment-for-severe-tracheomalacia-a-pediatric-case-report
#12
Kevin Vézina, Sophie Laberge, The Thanh Diem Nguyen
Tracheomalacia refers to a softness of the tracheal cartilage that makes the airway more susceptible to collapse. In contrast to milder cases where conservative therapy is preferred, severe tracheomalacia is often a life threatening condition requiring more aggressive management. For children with this condition, a variety of treatment options are available. To our knowledge, this is the first report of home high-flow nasal cannula as an alternative therapy to continuous positive airway pressure (CPAP) and surgical procedures in a pediatric patient with severe extensive tracheomalacia...
May 15, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28501574/androgen-production-in-pediatric-adrenocortical-tumors-may-occur-via-both-the-classic-and-or-the-alternative-backdoor-pathway
#13
Nesa Marti, Jana Malikova, José A Galván, Maude Aebischer, Marco Janner, Zdenek Sumnik, Barbora Obermannova, Genevieve Escher, Aurel Perren, Christa E Flück
Children with adrenocortical tumors (ACTs) often present with virilization due to high tumoral androgen production, with dihydrotestosterone (DHT) as most potent androgen. Recent work revealed two pathways for DHT biosynthesis, the classic and the backdoor pathway. Usage of alternate routes for DHT production has been reported in castration-resistant prostate cancer, CAH and PCOS. To assess whether the backdoor pathway may contribute to the virilization of pediatric ACTs, we investigated seven children suffering from androgen producing tumors using steroid profiling and immunohistochemical expression studies...
May 10, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28498454/identification-of-setd2-nf1-fusion-gene-in-a-pediatric-spindle-cell-tumor-with-the-chromosomal-translocation-t-3-17-p21-q12
#14
Ioannis Panagopoulos, Ludmila Gorunova, Ingvild Lobmaier, Bodil Bjerkehagen, Sverre Heim
Spindle cell tumors are clinically heterogeneous but morphologically similar neoplasms. The term refers to the tumor cells' long and slender microscopic appearance. Distinct subgroups of spindle cell tumors are characterized by chromosomal translocations and also fusion genes. Other spindle cell tumors exist that have not yet been found to have characteristic, let alone pathognomonic, genetic or pathogenetic features. Continuous examination of spindle cell tumors is likely to reveal other subgroups that may, in the future, be seen to correspond to meaningful clinical differences and may even be therapeutically decisive...
May 4, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28497184/management-of-hydrocephalus-in-pediatric-metastatic-tumors-of-the-posterior-fossa-at-presentation
#15
Luc Le Fournier, Matthieu Delion, Maxime Esvan, Emilie De Carli, Céline Chappé, Philippe Mercier, Philippe Menei, Laurent Riffaud
PURPOSE: Presence of metastases in newly diagnosed pediatric posterior fossa tumors (PFT) is not a rare situation, but optimal treatment of associated hydrocephalus in these children has remained undetermined. METHODS: Twenty-nine children treated between January 2005 and December 2015 for a metastatic PFT associated with hydrocephalus constituted the study cohort. Patients were divided into three groups: ventriculoperitoneal shunt (VPS), endoscopic third ventriculostomy (ETV), and temporary ventricular drainage before or during tumor resection (PVD)...
May 11, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28492439/retrobulbar-block-in-pediatric-vitreoretinal-surgery-eliminates-the-need-for-intraoperative-fentanyl-and-postoperative-analgesia-a-randomized-controlled-study
#16
Lan Yao, Hong Zhao, Bailin Jiang, Yi Feng
BACKGROUND AND OBJECTIVES: Pediatric ophthalmologic surgery is traditionally accomplished by general anesthesia with opioids, but respiratory depression remains a major concern. Our study compared the efficacy of retrobulbar block with systemic fentanyl on pain, hemodynamic, and stress response in pediatric vitreoretinal surgery. METHODS: A prospective double-blind, randomized controlled study was performed comparing retrobulbar block with intravenously administered fentanyl in 28 children aged 1 to 6 years undergoing vitreoretinal surgery...
May 10, 2017: Regional Anesthesia and Pain Medicine
https://www.readbyqxmd.com/read/28492193/effectiveness-of-two-different-behavioral-modification-techniques-among-5-7-year-old-children-a-randomized-controlled-trial
#17
Aruna Prashanth Vishwakarma, Prashant Arjun Bondarde, Sudha Bhimangouda Patil, Arun Suresh Dodamani, Prashanth Yachrappa Vishwakarma, Shoeb A Mujawar
BACKGROUND: Dental fear is a common, essential, and inevitable emotion that appears as a response to the stressful situation, which raises children's anxiety level, resulting in reduced demand for pediatric dental care. AIMS: (1) To compare and evaluate the effectiveness of customized tell-play-do (TPD) technique with live modeling for behavior management of children. (2) To compare the behavioral modification techniques in managing the children during their dental visits...
April 2017: Journal of the Indian Society of Pedodontics and Preventive Dentistry
https://www.readbyqxmd.com/read/28490038/pediatric-sleep-disordered-breathing-an-update-on-diagnostic-testing
#18
Brian McGrath, Jerrold Lerman
PURPOSE OF REVIEW: Recent advances in diagnostic testing for obstructive sleep apnea in children have refined the standard tests while identifying several new tools that hold promise to radically change how we diagnose sleep apnea. RECENT FINDINGS: Studies have demonstrated that the polysomnogram may be modified to permit home assessment of sleep disturbed breathing in children to ensure more widespread access to the test. Alternately, questionnaires, nocturnal oximetry, and diagnostic urinary biomarkers have shown great promise as both sensitive and specific tools to diagnose sleep apnea in children as well as track the severity of the disease...
June 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28487619/one-step-minilaparotomy-assisted-transmesenteric-portal-vein-recanalization-combined-with-transjugular-intrahepatic-portosystemic-shunt-placement-a-novel-surgical-proposal-in-pediatrics
#19
Gloria Pelizzo, Pietro Quaretti, Lorenzo Paolo Moramarco, Riccardo Corti, Marcello Maestri, Giulio Iacob, Valeria Calcaterra
Transjugular intrahepatic portosystemic shunt (TIPS) placement is a standard procedure for the treatment of portal hypertension complications. When this conventional approach is not feasible, alternative procedures for systemic diversion of portal blood have been proposed. A one-step interventional approach, combining minilaparotomy-assisted transmesenteric (MAT) antegrade portal recanalization and TIPS, is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma (PC). A 16-year-old girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC...
April 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28483608/safety-and-effectiveness-of-clofazimine-for-primary-and-refractory-nontuberculous-mycobacterial-infection
#20
Stacey L Martiniano, Brandie D Wagner, Adrah Levin, Jerry A Nick, Scott D Sagel, Charles L Daley
BACKGROUND: Clofazimine is an antimicrobial agent that has activity in vitro against mycobacteria. Increasingly, it has been used for the treatment of nontuberculous mycobacteria (NTM), despite limited data supporting use in this setting. The objective of this study was to evaluate safety, tolerability, and clinical outcomes associated with clofazimine in patients with NTM infection. METHODS: Observational-cohort study of clofazimine used for pediatric and adult cystic fibrosis (CF) and non-CF patients with pulmonary and extrapulmonary NTM infection as part of a multidrug regimen from 2006-2014...
May 5, 2017: Chest
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