keyword
MENU ▼
Read by QxMD icon Read
search

LAL treatment in adults

keyword
https://www.readbyqxmd.com/read/27878737/sebelipase-alfa-a-review-in-lysosomal-acid-lipase-deficiency
#1
James E Frampton
Sebelipase alfa (Kanuma(®), Kanuma™), the first commercially available recombinant human lysosomal acid lipase (LAL), is approved in various countries worldwide, including those of the EU, the USA and Japan, as a long-term enzyme replacement therapy for patients diagnosed with LAL deficiency (LAL-D), an ultra-rare, autosomal recessive, progressive metabolic liver disease. In an ongoing study in nine infants presenting with early-onset LAL-D (Wolman disease), open-label treatment with sebelipase alfa significantly improved 1-year survival compared with historical controls...
November 23, 2016: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/27799810/novel-treatment-options-for-lysosomal-acid-lipase-deficiency-critical-appraisal-of-sebelipase-alfa
#2
REVIEW
Kim Su, Emma Donaldson, Reena Sharma
Lysosomal acid lipase deficiency (LAL-D) is a rare disorder of cholesterol metabolism with an autosomal recessive mode of inheritance. The absence or deficiency of the LAL enzyme gives rise to pathological accumulation of cholesterol esters in various tissues. A severe LAL-D phenotype manifesting in infancy is associated with adrenal calcification and liver and gastrointestinal involvement with characteristic early mortality. LAL-D presenting in childhood and adulthood is associated with hepatomegaly, liver fibrosis, cirrhosis, and premature atherosclerosis...
2016: Application of Clinical Genetics
https://www.readbyqxmd.com/read/27733791/protective-and-ameliorative-effect-of-sea-buckthorn-leaf-extract-supplementation-on-lead-induced-hemato-biochemical-alterations-in-wistar-rats
#3
Rizwana Zargar, Pratiksha Raghuwanshi, Ankur Rastogi, Aditi Lal Koul, Pallavi Khajuria, Aafreen Wahid Ganai, Sumeet Kour
AIM: To evaluate the protective and ameliorative effect of aqueous sea buckthorn leaf extract (SLE) on hemato-biochemical profile in lead intoxicated Wistar rats. MATERIALS AND METHODS: An experiment was conducted for 60 days. 36 adult male Wistar rats with a mean body weight of 177.8±12.6 g were divided into five groups and were subjected to various daily oral treatment regimens. Group I served as a negative control receiving only feed and water, Group II (positive control for lead) received lead acetate at 250 ppm in drinking water, and Group III (positive control for SLE) received SLE at 100 mg/kg b...
September 2016: Veterinary World
https://www.readbyqxmd.com/read/27437275/self-mutilating-behaviour-in-severe-meningococcal-infection-an-interesting-association
#4
Anju Dinkar, Jitendra Singh, Virendra Atam, Krishna Kumar Sahani, Munna Lal Patel
Neisseria meningitidis most commonly manifests as asymptomatic colonization in the nasopharynx of healthy adolescents and adults. It may rarely present as invasive disease which may be either bacterial meningitis or meningococcal septicaemia. Hereby we report a case presented with fever and rashes, irritability followed by self mutilating behaviour who was diagnosed as a case of invasive meningococcal infection. He responded well to treatment with intravenous ceftriaxone and self mutilating behaviour was subsided completely after treatment...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27293348/atypical-magnetic-resonance-imaging-features-in-subacute-sclerosing-panencephalitis
#5
Biplab Das, Manoj Kumar Goyal, Manish Modi, Sahil Mehta, Sudheer Chakravarthi, Vivek Lal, Sameer Vyas
OBJECTIVES: Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. METHODS AND RESULTS: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported...
April 2016: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/27124110/assessment-and-treatment-of-pain-in-thalassemia
#6
Ashutosh Lal
Pain is a subjective symptom whose prevalence can be grossly underestimated. The high proportion of adults with thalassemia who experience chronic pain is evident from recent surveys. However, pain has not received enough attention in the overall management of thalassemia. The association of pain with the type and treatment of thalassemia or with its comorbidities is unclear. Abnormal spine imaging is seen in patients reporting pain, although the role of osteopenia has not been established. Pain becomes more frequent with age...
March 2016: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/27103984/reversal-of-intestinal-failure-associated-liver-disease-ifald-emphasis-on-its-multifactorial-nature
#7
Christian Hvas, Kamelia Kodjabashia, Emma Nixon, Stephen Hayes, Kirstine Farrer, Arun Abraham, Simon Lal
Patients with intestinal failure (IF) and home parenteral nutrition commonly develop abnormal liver function tests. The presentations of IF-associated liver disease (IFALD) range from mild cholestasis or steatosis to cirrhosis and decompensated liver disease. We describe the reversal of IFALD in an adult patient with IF secondary to severe Crohn's disease and multiple small bowel resections. The patient developed liver dysfunction and pathology consistent with IFALD. Multiple causal factors were implicated, including nutrition-related factors, catheter sepsis and the use of hepatotoxic medications...
April 2016: Frontline Gastroenterology
https://www.readbyqxmd.com/read/26976650/loss-of-adult-cardiac-myocyte-gsk-3-leads-to-mitotic-catastrophe-resulting-in-fatal-dilated-cardiomyopathy
#8
Jibin Zhou, Firdos Ahmad, Shan Parikh, Nichole E Hoffman, Sudarsan Rajan, Vipin K Verma, Jianliang Song, Ancai Yuan, Santhanam Shanmughapriya, Yuanjun Guo, Erhe Gao, Walter Koch, James R Woodgett, Muniswamy Madesh, Raj Kishore, Hind Lal, Thomas Force
RATIONALE: Cardiac myocyte-specific deletion of either glycogen synthase kinase (GSK)-3α and GSK-3β leads to cardiac protection after myocardial infarction, suggesting that deletion of both isoforms may provide synergistic protection. This is an important consideration because of the fact that all GSK-3-targeted drugs, including the drugs already in clinical trial target both isoforms of GSK-3, and none are isoform specific. OBJECTIVE: To identify the consequences of combined deletion of cardiac myocyte GSK-3α and GSK-3β in heart function...
April 15, 2016: Circulation Research
https://www.readbyqxmd.com/read/26944585/espen-guidelines-on-chronic-intestinal-failure-in-adults
#9
Loris Pironi, Jann Arends, Federico Bozzetti, Cristina Cuerda, Lyn Gillanders, Palle Bekker Jeppesen, Francisca Joly, Darlene Kelly, Simon Lal, Michael Staun, Kinga Szczepanek, André Van Gossum, Geert Wanten, Stéphane Michel Schneider
BACKGROUND & AIMS: Chronic Intestinal Failure (CIF) is the long-lasting reduction of gut function, below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth. CIF is the rarest organ failure. Home parenteral nutrition (HPN) is the primary treatment for CIF. No guidelines (GLs) have been developed that address the global management of CIF. These GLs have been devised to generate comprehensive recommendations for safe and effective management of adult patients with CIF...
April 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/26851681/theophylline-a-methylxanthine-drug-induces-osteopenia-and-alters-calciotropic-hormones-and-prophylactic-vitamin-d-treatment-protects-against-these-changes-in-rats
#10
Subhashis Pal, Kainat Khan, Shyamsundar Pal China, Monika Mittal, Konica Porwal, Richa Shrivastava, Isha Taneja, Zakir Hossain, Dhanaraju Mandalapu, Jiaur R Gayen, Muhammad Wahajuddin, Vishnu Lal Sharma, Arun K Trivedi, Sabyasachi Sanyal, Smrati Bhadauria, Madan M Godbole, Sushil K Gupta, Naibedya Chattopadhyay
The drug, theophylline is frequently used as an additive to medications for people suffering from chronic obstructive pulmonary diseases (COPD). We studied the effect of theophylline in bone cells, skeleton and parameters related to systemic calcium homeostasis. Theophylline induced osteoblast apoptosis by increasing reactive oxygen species production that was caused by increased cAMP production. Bone marrow levels of theophylline were higher than its serum levels, indicating skeletal accumulation of this drug...
March 15, 2016: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/26673907/pla2r-antibodies-glomerular-pla2r-deposits-and-variations-in-pla2r1-and-hla-dqa1-genes-in-primary-membranous-nephropathy-in-south-asians
#11
Raja Ramachandran, Vinod Kumar, Ashwani Kumar, Ashok Kumar Yadav, Ritambhra Nada, Harsha Kumar, Vivek Kumar, Manish Rathi, Harbir Singh Kohli, Krishan Lal Gupta, Vinay Sakhuja, Vivekanand Jha
BACKGROUND: Antibodies to M-type phospholipase A2 receptor (PLA2R) correlate with clinical activity of primary membranous nephropathy (PMN). Risk alleles in PLA2R1 and HLA-DQA1 genes are associated with PMN. Whether these alleles are associated with the development of anti-PLA2R is unknown. In this prospective study we evaluated anti-PLA2R, enhanced glomerular staining for PLA2R and variations in PLA2R1 and HLA-DQA1 genes in Indian patients with PMN and examined their association with response to treatment...
September 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/26591214/-prognostication-of-limited-accumulations-liquid-infection-by-severe-acute-pancreatitis
#12
V D Sheiko, A G Oganezyan
The results of examination and treatment of 53 patients on limited accumulations of liquid (LAL) for severe acute pancreatitis (SAP) were analysed. In 62.5% of patients on acute aseptic LAL celebrated parapancreatyc liquid accumulation were determinened. Most (94.6%) patients infected by LAL revealed heterogeneity of their structure according ultrasonography, in 81.1%--secvestral mass in their cavity. Systemic inflammatory response syndrome (SIRS) observed both aseptic and infected LAL. Prognostically important criteria LAL infection in patients on SAP is the heterogeneity of echostructure in absence of a downward trend...
July 2015: Klinichna Khirurhiia
https://www.readbyqxmd.com/read/26543922/preferences-of-young-adults-with-first-episode-psychosis-for-receiving-specialized-mental-health-services-using-technology-a-survey-study
#13
Shalini Lal, Jennifer Dell'Elce, Natasha Tucci, Rebecca Fuhrer, Robyn Tamblyn, Ashok Malla
BACKGROUND: Despite the potential and interest of using technology for delivering specialized psychiatric services to young adults, surprisingly limited attention has been paid to systematically assess their perspectives in this regard. For example, limited knowledge exists on the extent to which young people receiving specialized services for a first-episode psychosis (FEP) are receptive to using new technologies as part of mental health care, and to which types of technology-enabled mental health interventions they are amenable to...
April 2015: JMIR Mental Health
https://www.readbyqxmd.com/read/26454555/service-engagement-in-first-episode-psychosis-current-issues-and-future-directions
#14
REVIEW
Shalini Lal, Ashok Malla
It has been reported that up to 50% of patients receiving mental health services disengage from treatment, with adolescents and young adults being particularly at high risk. Even in the context of specialized services in youth mental health, such as early intervention programs for psychosis, disengagement rates remain high. There is a need for extensive and innovative efforts to address the issue of service disengagement in first-episode psychosis (FEP). A multi-dimensional understanding of the phenomenon of engagement can help to inform the development of strategies to address this important clinical issue...
August 2015: Canadian Journal of Psychiatry. Revue Canadienne de Psychiatrie
https://www.readbyqxmd.com/read/26439212/osteomyelitis-in-adult-patients-on-long-term-parenteral-nutrition-2745-patient-years-of-experience-in-a-national-referral-centre
#15
P Allan, P Stevens, P Chadwick, A Teubner, A Abraham, G Carlson, S Lal
BACKGROUND & AIMS: Osteomyelitis (OM) is a rare complication of catheter related sepsis after central venous catheter (CVC) use. The prevalence, characteristics and diagnosis of OM in patients with intestinal failure (IF) receiving long term parenteral nutrition (PN) through CVCs have not previously been described. METHODS: This was a retrospective study from a prospectively maintained database of patients referred to a National IF centre. Age, IF aetiology, past medical history, time on PN, OM site and organism(s) cultured were recorded...
October 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/26429175/histological-differences-between-laser-assisted-and-suction-assisted-lipoplasty-aspirates-a-comparative-study
#16
COMPARATIVE STUDY
Cristian Radu Jecan, Alexandru Daniel Hernic, Elena Cristina Tianu, Ioan Petre Florescu, Ioan Lascăr
INTRODUCTION: The authors aimed to assess the histological differences between the traditional suction-assisted lipoplasty (SAL) and the more recently developed laser-assisted lipoplasty (LAL) aspirates, in a 20-case comparative study. PATIENTS AND METHODS: Between March of 2011 and March of 2012, we operated on 20 healthy female patients seeking body contouring procedures of the abdomen, flanks and outer thighs, all having good to moderate skin tone and moderate to heavy adipose deposits and no previous treatment of the interested areas...
2015: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/26320116/tumor-microenvironment-tenascin-c-promotes-glioblastoma-invasion-and-negatively-regulates-tumor-proliferation
#17
Shuli Xia, Bachchu Lal, Brian Tung, Shervin Wang, C Rory Goodwin, John Laterra
BACKGROUND: Glioblastoma (GBM) is the most frequent and aggressive primary brain tumor in adults. Recent research on cancer stroma indicates that the brain microenvironment plays a substantial role in tumor malignancy and treatment responses to current antitumor therapy. In this work, we have investigated the effect of alterations in brain tumor extracellular matrix tenascin-C (TNC) on brain tumor growth patterns including proliferation and invasion. METHODS: Since intracranial xenografts from patient-derived GBM neurospheres form highly invasive tumors that recapitulate the invasive features demonstrated in human patients diagnosed with GBM, we studied TNC gain-of-function and loss-of function in these GBM neurospheres in vitro and in vivo...
April 2016: Neuro-oncology
https://www.readbyqxmd.com/read/26283035/a-phase-iii-study-of-belagenpumatucel-l-an-allogeneic-tumour-cell-vaccine-as-maintenance-therapy-for-non-small-cell-lung-cancer
#18
RANDOMIZED CONTROLLED TRIAL
G Giaccone, L A Bazhenova, J Nemunaitis, M Tan, E Juhász, R Ramlau, M M van den Heuvel, R Lal, G H Kloecker, K D Eaton, Q Chu, D J Dunlop, M Jain, E B Garon, C S Davis, E Carrier, S C Moses, D L Shawler, H Fakhrai
BACKGROUND: Treatment options after first-line chemotherapy are limited in non-small cell lung cancer (NSCLC). Belagenpumatucel-L is a therapeutic vaccine comprised of 4 transforming growth factor (TGF)-β2-antisense gene-modified, irradiated, allogeneic NSCLC cell lines that may be useful for maintenance after initial treatment. METHODS: Stage III/IV NSCLC patients who did not progress after platinum-based chemotherapy were randomised 1:1 to receive maintenance belagenpumatucel-L or placebo...
November 2015: European Journal of Cancer
https://www.readbyqxmd.com/read/26244512/the-burden-and-determinants-of-non-communicable-diseases-risk-factors-in-nepal-findings-from-a-nationwide-steps-survey
#19
Krishna Kumar Aryal, Suresh Mehata, Sushhama Neupane, Abhinav Vaidya, Meghnath Dhimal, Purushottam Dhakal, Sangeeta Rana, Chop Lal Bhusal, Guna Raj Lohani, Frank Herbert Paulin, Renu Madanlal Garg, Regina Guthold, Melanie Cowan, Leanne Margaret Riley, Khem Bahadur Karki
BACKGROUND: World Health Organization (WHO) estimates for deaths attributed to Non Communicable Diseases (NCDs) in Nepal have risen from 51% in 2010 to 60% in 2014. This study assessed the distribution and determinants of NCD risk factors among the Nepalese adult population. METHODS AND FINDINGS: A nationally representative cross-sectional survey was conducted from Jan to June 2013 on the prevalence of NCD risk factors using the WHO NCD STEPS instrument. A multistage cluster sampling method was used to randomly select the 4,200 respondents...
2015: PloS One
https://www.readbyqxmd.com/read/26231742/a-comparison-of-two-myringoplasty-techniques-in-nepalese-children-a-prospective-randomized-trial
#20
RANDOMIZED CONTROLLED TRIAL
Prashant Tripathi, Rajendra Prasad Guragain, Chop Lal Bhusal, Sureshwar Lal Karna, Johannes Borgstein
BACKGROUND: In children, the success of myringoplastywith temporalis fascia is lower compared to adults and cartilage as an alternative graft material has shown higher success rate. OBJECTIVE: To compare results of myringoplasty using tragal cartilage palisades with the use of temporalis fascia in children with large tympanic membrane perforations. MATERIALS AND METHODS: This is a prospective and randomized study conducted in children of age 6-14 years with large tympanic membrane perforation of more than two quadrants...
September 2015: International Journal of Pediatric Otorhinolaryngology
keyword
keyword
116518
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"