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https://www.readbyqxmd.com/read/28625944/intractable-ictal-vomiting-a-new-form-of-reflex-epilepsy
#1
Sachin Sureshbabu, Dinesh Nayak, Vikash Aggarwal, Sudhir Peter, Laxmi Khanna, Gaurav Mittal
In this description of the clinical course of a young female with persistent and protracted vomiting along with occasional loss of consciousness and subtle motor manifestations, the differential diagnosis is debated. The epileptic origin of her symptoms was substantiated by the presence of interictal epileptiform discharges and dramatic response to valproate monotherapy. Possible lobar localizations are discussed with the support of existing literature on this rare ictal manifestation [Published with video sequence on www...
June 19, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/28597604/lesinurad-a-selective-uric-acid-reabsorption-inhibitor-in-combination-with-febuxostat-in-patients-with-tophaceous-gout-findings-of-a-phase-iii-clinical-trial
#2
Nicola Dalbeth, Graeme Jones, Robert Terkeltaub, Dinesh Khanna, Jeff Kopicko, Nihar Bhakta, Scott Adler, Maple Fung, Chris Storgard, Scott Baumgartner, Fernando Perez-Ruiz
OBJECTIVE: To investigate the efficacy and safety of lesinurad in combination with febuxostat in a 12-month phase III trial in patients with tophaceous gout. METHODS: Patients with serum urate (UA) ≥8.0 mg/dl (≥6.0 mg/dl with urate-lowering therapy) and ≥1 measurable target tophus were given febuxostat 80 mg/day for 3 weeks before randomization to receive lesinurad (200 or 400 mg daily) or placebo in addition to the febuxostat. The primary end point was the proportion of patients achieving a serum UA level of <5...
June 9, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28569193/health-care-utilization-in-patients-with-gout-a-prospective-multicenter-cohort-study
#3
Jasvinder A Singh, Aseem Bharat, Dinesh Khanna, Cleopatra Aquino-Beaton, Jay E Persselin, Erin Duffy, David Elashoff, Puja P Khanna
BACKGROUND: All published studies of health care utilization in gout have been cross-sectional to date, and most used a patient-reported diagnosis of gout. Our objective was to assess health care utilization and its predictors in patients with physician-confirmed gout in a prospective cohort study. METHODS: In a multi-center prospective cohort study of U.S. veterans with rheumatologist-confirmed gout (N = 186; two centers), we assessed patient self-reported overall and gout-specific health care utilization with the Gout Assessment Questionnaire (GAQ) every 3-months for a 9-month period...
May 31, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28544580/efficacy-of-mycophenolate-mofetil-and-oral-cyclophosphamide-on-skin-thickness-post-hoc-analyses-from-the-scleroderma-lung-study-i-and-ii
#4
Rajaie Namas, Donald P Tashkin, Daniel E Furst, Holly Wilhalme, Chi-Hong Tseng, Michael D Roth, Suzanne Kafaja, Elizabeth Volkmann, Philip J Clements, Dinesh Khanna
OBJECTIVES: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II. METHODS: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28516167/standardization-of-the-modified-rodnan-skin-score-for-use-in-clinical-trials-of-systemic-sclerosis
#5
Dinesh Khanna, Daniel E Furst, Philip J Clements, Yannick Allanore, Murray Baron, Lazlo Czirjak, Oliver Distler, Ivan Foeldvari, Masataka Kuwana, Marco Matucci-Cerinic, Maureen Mayes, Thomas Medsger, Peter A Merkel, Janet E Pope, James R Seibold, Virginia Steen, Wendy Stevens, Christopher P Denton
The modified Rodnan skin score (mRSS) is a measure of skin thickness and is used as a primary or secondary outcome measure in clinical trials of systemic sclerosis (scleroderma). This state-of-art review provides a historical perspective of the development of the mRSS, summarizes the performance of mRSS as an outcome measure, provides guidance on assessing mRSS, and makes recommendations for incorporation of the mRSS into clinical trials.
January 2017: Journal of Scleroderma and related Disorders
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#6
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28431140/performance-of-the-patient-reported-outcomes-measurement-information-system-29-in-scleroderma-a-scleroderma-patient-centered-intervention-network-cohort-study
#7
Linda Kwakkenbos, Brett D Thombs, Dinesh Khanna, Marie-Eve Carrier, Murray Baron, Daniel E Furst, Karen Gottesman, Frank van den Hoogen, Vanessa L Malcarne, Maureen D Mayes, Luc Mouthon, Warren R Nielson, Serge Poiraudeau, Robert Riggs, Maureen Sauvé, Fredrick Wigley, Marie Hudson, Susan J Bartlett
Objective: The Patient-Reported Outcomes Measurement Information System (PROMIS)-29 assesses seven health-related quality of life domains plus pain intensity. The objective was to examine PROMIS-29v2 validity and explore clinical associations in patients with SSc. Methods: English-speaking SSc patients in the Scleroderma Patient-centered Intervention Network Cohort from 26 sites in Canada, the USA and the UK completed the PROMIS-29v2 between July 2014 and November 2015...
August 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#8
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
April 13, 2017: Lancet
https://www.readbyqxmd.com/read/28412704/structural-validity-of-the-rheumatology-attitudes-index-in-systemic-sclerosis-analysis-from-the-ucla-scleroderma-quality-of-life-study
#9
Shadi Gholizadeh, Sarah D Mills, Rina S Fox, Erin L Merz, Scott C Roesch, Philip J Clements, Suzanne Kafaja, Daniel E Furst, Dinesh Khanna, Vanessa L Malcarne
OBJECTIVE: To evaluate the structural validity of the Rheumatology Attitudes Index (RAI), a widely used measure of rheumatic disease-related helplessness in patients with systemic sclerosis (SSc). METHODS: Patients with physician-confirmed SSc from the University of California, Los Angeles (UCLA) Scleroderma Quality of Life Study (n = 208) received clinical examinations and completed self-report questionnaires. The structural validity of the RAI was examined through confirmatory and exploratory factor analysis (CFA/EFA)...
June 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28376288/mycophenolate-mofetil-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#10
COMPARATIVE STUDY
Elizabeth R Volkmann, Donald P Tashkin, Ning Li, Michael D Roth, Dinesh Khanna, Anna-Maria Hoffmann-Vold, Grace Kim, Jonathan Goldin, Philip J Clements, Daniel E Furst, Robert M Elashoff
OBJECTIVE: To compare mycophenolate mofetil (MMF) with placebo for the treatment of systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: We included participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II. SLS I randomized participants to receive either oral cyclophosphamide (CYC) or placebo for 1 year, while SLS II randomized participants to receive either MMF for 2 years or oral CYC for 1 year followed by 1 year of placebo...
July 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28298560/reliability-and-validity-of-the-tender-and-swollen-joint-counts-and-the-modified-rodnan-skin-score-in-early-diffuse-cutaneous-systemic-sclerosis-analysis-from-the-prospective-registry-of-early-systemic-sclerosis-cohort
#11
Jessica K Gordon, Gandikota Girish, Veronica J Berrocal, Meng Zhang, Christopher Hatzis, Shervin Assassi, Elana J Bernstein, Robyn T Domsic, Faye N Hant, Monique Hinchcliff, Elena Schiopu, Virginia D Steen, Tracy M Frech, Dinesh Khanna
OBJECTIVE: To determine the inter/intraobserver reliability of the tender and swollen joint counts (TJC, SJC) and the modified Rodnan Skin Score (mRSS) in diffuse cutaneous systemic sclerosis (dcSSc) and to assess content validity of the TJC/SJC. METHODS: Ten rheumatologists completed the SJC, TJC, and mRSS on 7 patients. Musculoskeletal ultrasound (MSUS) was performed. RESULTS: Interobserver and intraobserver reliability for the TJC was 0...
June 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28285914/pharmacokinetic-optimitzation-of-ccg-203971-novel-inhibitors-of-the-rho-mrtf-srf-transcriptional-pathway-as-potential-antifibrotic-therapeutics-for-systemic-scleroderma
#12
Kim M Hutchings, Erika M Lisabeth, Walajapet Rajeswaran, Michael W Wilson, Roderick J Sorenson, Phillip L Campbell, Jeffrey H Ruth, Asif Amin, Pei-Suen Tsou, Jeffrey R Leipprandt, Samuel R Olson, Bo Wen, Ting Zhao, Duxin Sun, Dinesh Khanna, David A Fox, Richard R Neubig, Scott D Larsen
We recently reported the development of a novel inhibitor of Rho-mediated gene transcription (1, CCG-203971) that is efficacious in multiple animal models of acute fibrosis, including scleroderma, when given intraperitoneally. The modest in vivo potency and poor pharmacokinetics (PK) of this lead, however, make it unsuitable for long term efficacy studies. We therefore undertook a systematic medicinal chemistry effort to improve both the metabolic stability and the solubility of 1, resulting in the identification of two analogs achieving over 10-fold increases in plasma exposures in mice...
April 15, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28251500/responsiveness-to-change-and-minimally-important-differences-of-the-patient-reported-outcomes-measurement-information-system-gastrointestinal-symptoms-scales
#13
Dinesh Khanna, Ron D Hays, Andrew B Shreiner, Gil Y Melmed, Lin Chang, Puja P Khanna, Roger Bolus, Cynthia Whitman, Sylvia H Paz, Tonya Hays, Steven P Reise, Brennan Spiegel
BACKGROUND: The NIH-sponsored Patient-Reported Outcomes Measurement Information System (PROMIS) Gastrointestinal (GI) Symptoms scales were developed to assess patients' GI symptoms in clinical settings. AIMS: To assess responsiveness to change and provide minimally important difference (MID) estimates for the PROMIS GI Symptoms scales. METHODS: A sample of 256 GI outpatients self-administered the eight PROMIS GI Symptoms scales (gastroesophageal reflux, disrupted swallowing, diarrhea, bowel incontinence/soilage, nausea and vomiting, constipation, belly pain, and gas/bloating/flatulence) at two visits...
May 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28119942/congenital-extrahepatic-portosystemic-shunt-in-a-child-with-caroli-s-syndrome
#14
Bikrant Bihari Lal, Ankur Arora, Vikrant Sood, Dinesh Rawat, Rajeev Khanna, Seema Alam
No abstract text is available yet for this article.
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28111909/profile-and-outcome-of-first-109-cases-of-pediatric-acute-liver-failure-at-a-specialized-pediatric-liver-unit-in-india
#15
Seema Alam, Rajeev Khanna, Vikrant Sood, Bikrant Bihari Lal, Dinesh Rawat
BACKGROUND AND AIMS: The outcome of Pediatric acute liver failure largely depends on age and etiology. The aim of this work was to study the etiological spectrum and outcome of the pediatric acute liver failure cases. METHODS: This prospective observational study included all children (< 18 years age) fulfilling pediatric acute liver failure study group definition. Etiological evaluation was done and predictive factors for poor outcome (death or liver transplantation) were analyzed...
January 23, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28045774/study-of-carnitine-acylcarnitine-and-amino-acid-profile-in-children-and-adults-with-acute-liver-failure
#16
Vikrant Sood, Dinesh Rawat, Rajeev Khanna, Shvetank Sharma, Prem K Gupta, Seema Alam, Shiv Kumar Sarin
OBJECTIVES: Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and amino acid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and amino acid profile in ALF. METHODS: A prospective study was performed including all patients with ALF, and detailed evaluation including metabolic testing was done...
June 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28038680/changes-in-plasma-cxcl4-levels-are-associated-with-improvements-in-lung-function-in-patients-receiving-immunosuppressive-therapy-for-systemic-sclerosis-related-interstitial-lung-disease
#17
Elizabeth R Volkmann, Donald P Tashkin, Michael D Roth, Philip J Clements, Dinesh Khanna, Daniel E Furst, Maureen Mayes, Julio Charles, Chi-Hong Tseng, Robert M Elashoff, Shervin Assassi
BACKGROUND: Increased circulatory levels of the chemokine CXCL4 have been associated with the presence of interstitial lung disease (ILD) in an observational study of patients with systemic sclerosis (SSc). The purpose of the present study was to evaluate the relationship between baseline CXCL4 level and extent of ILD in the context of a randomized controlled trial and to determine whether changes in CXCL4 levels in response to immunosuppression are associated with future progression of SSc-ILD...
December 30, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28028159/racial-differences-in-health-related-quality-of-life-and-functional-ability-in-patients-with-gout
#18
Jasvinder A Singh, Aseem Bharat, Dinesh Khanna, Cleopatra Aquino-Beaton, Jay E Persselin, Erin Duffy, David Elashoff, Puja P Khanna
OBJECTIVE: To compare the health-related quality of life (HRQOL) and the functional ability by race in patients with gout. METHODS: In a 9-month prospective cohort multicentre study, patients with gout self-reported race, dichotomized as Caucasian or African American (others excluded). We calculated HRQOL/function scores adjusted for age, study site and college education for Short Form-36 (SF-36; generic HRQOL), Gout Impact Scale (GIS; disease-specific HRQOL) and HAQ-disability index (HAQ-DI; functional ability)...
January 2017: Rheumatology
https://www.readbyqxmd.com/read/28018840/hand-impairment-in-systemic-sclerosis-various-manifestations-and-currently-available-treatment
#19
Amber Young, Rajaie Namas, Carole Dodge, Dinesh Khanna
Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life...
September 2016: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/28012804/improved-cough-and-cough-specific-quality%C3%A2-of-life-in-patients-treated-for-scleroderma-related-interstitial-lung-disease-results-of-scleroderma-lung-study-ii
#20
RANDOMIZED CONTROLLED TRIAL
Donald P Tashkin, Elizabeth R Volkmann, Chi-Hong Tseng, Michael D Roth, Dinesh Khanna, Daniel E Furst, Philip J Clements, Arthur Theodore, Suzanne Kafaja, Grace Hyun Kim, Jonathan Goldin, Edgar Ariolla, Robert M Elashoff
BACKGROUND: Cough is a common symptom of scleroderma-related interstitial lung disease (SSc-ILD), but its relationship to other characteristics of SSc-ILD, impact on cough-specific quality of life (QoL), and response to therapy for SSc-ILD have not been well studied. METHODS: We investigated frequent cough (FC) in patients with SSc-ILD (N = 142) enrolled in the Scleroderma Lung Study II, a randomized controlled trial comparing mycophenolate mofetil (MMF) and oral cyclophosphamide (CYC) as treatments for interstitial lung disease (ILD)...
April 2017: Chest
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