Failure Of Regenerative Cell-based Therapy To Stimulate Coronary Collateral Growth In A Rat Model Of Metabolic Syndrome
Arteriogenesis; Cardiovascular System & Cardiology; Collateral circulation; Metabolic; oxidative stress; Stem cell therapy; syndrome
Logan S; Chilian W M; Ohanyan V A; Stevanov K; Enrick M; Kolz C L; Yin L Y
Circulation
2012
2012-11
Journal Article or Conference Abstract Publication
n/a
Impaired Coupling of Myocardial Blood Flow to Cardiac Work Leads to Cardiac Dysfunction
Cardiovascular; Cardiovascular System & Cardiology; Coronary vessels; Ion channels; Metabolic; Redox; syndrome
Ohanyan V A; Yin L Y; Pung Y F; Enrick M; Hakobyan T A; Kolz C L; Logan S; Bratz I; Chilian W M
Circulation
2011
2011-11
Journal Article
n/a
Membranous glomerulonephritis: treatment response and outcome in children
childhood; chlorambucil; clinical course; cyclophosphamide; cyclophosphamide; glomerular-filtration-rate; glomerulonephropathy; glomerulosclerosis; Membranous; Membranous glomerulonephritis; methylprednisolone; nephropathy; nephrotic; Nephrotic syndrome; Pediatrics; syndrome; systemic lupus erythematosus; Urology & Nephrology
The aim of this study was to characterize clinical features, treatment response, and outcome of idiopathic membranous glomerulonephritis (MGN) in a single-center cohort of children. A retrospective review of biopsy-proven idiopathic MGN in 12 children (mean age 11.9 years) was undertaken. Presentation was nephrotic syndrome (NS) (75%), hematuria/proteinuria (17%), and asymptomatic proteinuria (8%). Ten patients (83%) with NS and nephrotic range proteinuria (NRP) were treated with prednisone, and two patients with non-NRP were not treated with immunosuppressive medications. Steroid response in the treated patients was complete (10%), partial (40%), and absent (50%), respectively. Oral cyclophosphamide was used in seven patients of whom five were steroid resistant, one was steroid dependent, and one was partially responsive. At the mean follow up of 27 months, outcome parameters included an estimated glomerular filtration rate of 128 cc/min per 1.73 m(2), albumin of 4.2 gm/dL, and a urine protein/creatinine ratio of 0.87 [median 0.16 (range 0.02-6.52)]. Remission was complete in 75% of the patients and partial in 17%. One patient (8%) with chronic kidney disease (stage 2) was unresponsive to therapy. Complete remission was significantly associated with the absence of chronic histological changes (p=0.03). In conclusion, children with NS and/or NRP associated with MGN appear to have a good prognosis when treated with a combination of corticosteroids and cyclophosphamide.
Valentini R P; Mattoo T K; Kapur G; Imam A
Pediatric Nephrology
2009
2009-02
Journal Article
<a href="http://doi.org/10.1007/s00467-008-1005-9" target="_blank" rel="noreferrer noopener">10.1007/s00467-008-1005-9</a>
Understanding Bile Acid Signaling in Diabetes: From Pathophysiology to Therapeutic Targets.
BILE acids; Bile acids and salts; cholesterol 7-alpha-hydroxylase; Cytoplasmic and Nuclear; Endocrinology & Metabolism; FARNESOID X receptor; farnesoid-x-receptor; FATTY liver; fatty liver-disease; G protein coupled receptors; G-protein-coupled; Gastrointestinal microbiome; growth-factor 19; gut microbiota; hepatic steatosis; improves insulin sensitivity; liver disease; metabolic; Non-alcoholic fatty; Non-alcoholic Fatty Liver Disease; nuclear; receptor; Receptors; serum fgf21 levels; syndrome
Diabetes and obesity have reached an epidemic status worldwide. Diabetes increases the risk for cardiovascular disease and nonalcoholic fatty liver disease. Primary bile acids are synthesized in hepatocytes and are transformed to secondary bile acids in the intestine by gut bacteria. Bile acids are nutrient sensors and metabolic integrators that regulate lipid, glucose, and energy homeostasis by activating nuclear farnesoid X receptor and membrane Takeda G protein-coupled receptor 5. Bile acids control gut bacteria overgrowth, species population, and protect the integrity of the intestinal barrier. Gut bacteria, in turn, control circulating bile acid composition and pool size. Dysregulation of bile acid homeostasis and dysbiosis causes diabetes and obesity. Targeting bile acid signaling and the gut microbiome have therapeutic potential for treating diabetes, obesity, and non-alcoholic fatty liver disease. [ABSTRACT FROM AUTHOR]
Ferrell Jessica M; Chiang John Y L
Diabetes & Metabolism Journal
2019
2019-06
<a href="http://doi.org/10.4093/dmj.2019.0043" target="_blank" rel="noreferrer noopener">10.4093/dmj.2019.0043</a>
Q & A. Hyperostosis associated with hip surgery?
Syndrome; Hip; Arthroplasty; Replacement; Hyperostosis
Rothschild B M
Journal of Musculoskeletal Medicine
2004
2004-05
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Human quadrupeds, primate quadrupedalism, and Uner Tan Syndrome.
Adult; Female; Humans; Male; Animals; Child; Infant; Gait/*physiology; Syndrome; *Primates; Posture/physiology; Walking/*physiology; Molecular; Evolution
Since 2005, an extensive literature documents individuals from several families afflicted with "Uner Tan Syndrome (UTS)," a condition that in its most extreme form is characterized by cerebellar hypoplasia, loss of balance and coordination, impaired cognitive abilities, and habitual quadrupedal gait on hands and feet. Some researchers have interpreted habitual use of quadrupedalism by these individuals from an evolutionary perspective, suggesting that it represents an atavistic expression of our quadrupedal primate ancestry or "devolution." In support of this idea, individuals with "UTS" are said to use diagonal sequence quadrupedalism, a type of quadrupedal gait that distinguishes primates from most other mammals. Although the use of primate-like quadrupedal gait in humans would not be sufficient to support the conclusion of evolutionary "reversal," no quantitative gait analyses were presented to support this claim. Using standard gait analysis of 518 quadrupedal strides from video sequences of individuals with "UTS", we found that these humans almost exclusively used lateral sequence-not diagonal sequence-quadrupedal gaits. The quadrupedal gait of these individuals has therefore been erroneously described as primate-like, further weakening the "devolution" hypothesis. In fact, the quadrupedalism exhibited by individuals with UTS resembles that of healthy adult humans asked to walk quadrupedally in an experimental setting. We conclude that quadrupedalism in healthy adults or those with a physical disability can be explained using biomechanical principles rather than evolutionary assumptions.
Shapiro Liza J; Cole Whitney G; Young Jesse W; Raichlen David A; Robinson Scott R; Adolph Karen E
PloS one
2014
2014
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1371/journal.pone.0101758" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0101758</a>
An unusual dermatitis with annular lesions.
Atopic/*pathology; Biopsy; Child; Congenital/*pathology; Dermatitis; Diagnosis; Differential; Hair/*abnormalities/*pathology; Humans; Ichthyosiform Erythroderma; Male; Psoriasis/pathology; Scalp/pathology; Syndrome
Cernik Christina; Trevino Julian; Janik Matthew
Pediatric dermatology
2008
2008-04
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1111/j.1525-1470.2008.00622.x" target="_blank" rel="noreferrer noopener">10.1111/j.1525-1470.2008.00622.x</a>
Precordial Catch Syndrome in Elite Swimmers With Asthma.
Adolescence; Adolescent; Asthma – Complications; Asthma/*complications; Chest Pain – Etiology; Chest Pain/*etiology; Emergency Service; Hospital; Humans; Male; Swimming; Syndrome; Thoracic Wall; Thorax
Precordial catch syndrome is a benign cause of chest pain in children and adolescents that remains underrecognized. Because of distinctive symptoms, precordial catch syndrome is not necessarily a diagnosis of exclusion. However, a detailed history eliciting diagnostic features is important, along with a physical examination excluding other pathologic disorders. We present the cases of 2 elite swimmers with asthma who had acute episodes of precordial catch syndrome, one associated with an acute asthma exacerbation and one not, while swimming during competitive swim meets that required rescue efforts for both and eventual evaluation in the emergency department.
Hayes Don Jr; Younger Bradley R; Mansour Heidi M; Strawbridge Heather
Pediatric emergency care
2016
2016-02
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1097/PEC.0000000000000715" target="_blank" rel="noreferrer noopener">10.1097/PEC.0000000000000715</a>
Exploring the scope of post-intensive care syndrome therapy and care: engagement of non-critical care providers and survivors in a second stakeholders meeting.
*Health Status; *Intensive Care Units; Awareness; Continuity of Patient Care/*organization & administration; Critical Illness/*psychology; Health Education; Humans; Mental Health; Survivors/*psychology; Syndrome; United States
BACKGROUND: Increasing numbers of survivors of critical illness are at risk for physical, cognitive, and/or mental health impairments that may persist for months or years after hospital discharge. The post-intensive care syndrome framework encompassing these multidimensional morbidities was developed at the 2010 Society of Critical Care Medicine conference on improving long-term outcomes after critical illness for survivors and their families. OBJECTIVES: To report on engagement with non-critical care providers and survivors during the 2012 Society of Critical Care Medicine post-intensive care syndrome stakeholder conference. Task groups developed strategies and resources required for raising awareness and education, understanding and addressing barriers to clinical practice, and identifying research gaps and resources, aimed at improving patient and family outcomes. PARTICIPANTS: Representatives from 21 professional associations or health systems involved in the provision of both critical care and rehabilitation of ICU survivors in the United States and ICU survivors and family members. DESIGN: Stakeholder consensus meeting. Researchers presented summaries on morbidities for survivors and their families, whereas survivors presented their own experiences. MEETING OUTCOMES: Future steps were planned regarding 1) recognizing, preventing, and treating post-intensive care syndrome, 2) building strategies for institutional capacity to support and partner with survivors and families, and 3) understanding and addressing barriers to practice. There was recognition of the need for systematic and frequent assessment for post-intensive care syndrome across the continuum of care, including explicit "functional reconciliation" (assessing gaps between a patient's pre-ICU and current functional ability at all intra- and interinstitutional transitions of care). Future post-intensive care syndrome research topic areas were identified across the continuum of recovery: characterization of at-risk patients (including recognizing risk factors, mechanisms of injury, and optimal screening instruments), prevention and treatment interventions, and outcomes research for patients and families. CONCLUSIONS: Raising awareness of post-intensive care syndrome for the public and both critical care and non-critical care clinicians will inform a more coordinated approach to treatment and support during recovery after critical illness. Continued conceptual development and engagement with additional stakeholders is required.
Elliott Doug; Davidson Judy E; Harvey Maurene A; Bemis-Dougherty Anita; Hopkins Ramona O; Iwashyna Theodore J; Wagner Jason; Weinert Craig; Wunsch Hannah; Bienvenu O Joseph; Black Gary; Brady Susan; Brodsky Martin B; Deutschman Cliff; Doepp Diana; Flatley Carl; Fosnight Sue; Gittler Michelle; Gomez Belkys Teresa; Hyzy Robert; Louis Deborah; Mandel Ruth; Maxwell Carol; Muldoon Sean R; Perme Christiane S; Reilly Cynthia; Robinson Marla R; Rubin Eileen; Schmidt David M; Schuller Jessica; Scruth Elizabeth; Siegal Eric; Spill Gayle R; Sprenger Sharon; Straumanis John P; Sutton Pat; Swoboda Sandy M; Twaddle Martha L; Needham Dale M
Critical care medicine
2014
2014-12
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1097/CCM.0000000000000525" target="_blank" rel="noreferrer noopener">10.1097/CCM.0000000000000525</a>
Exome sequence identified a c.320A \textgreater G ALG13 variant in a female with infantile epileptic encephalopathy with normal glycosylation and random X inactivation: Review of the literature.
*Mutation; *X Chromosome Inactivation; ALG13; CDG Is; Child; Congenital disorder of glycosylation; Congenital Disorders of Glycosylation/diagnosis/*genetics; Exome; Female; Glycosylation; Heterozygote; Humans; Infant; Infantile/diagnosis/*genetics; Mental Retardation; Missense; N-Acetylglucosaminyltransferases/*genetics; Post-Translational; Preschool; Protein Processing; Spasms; Syndrome; Transferrin/metabolism; X-inactivation; X-Linked/diagnosis/*genetics
Congenital Disorders of Glycosylation (CDG) are new and rapidly expanding neurometabolic disorders with multisystem involvements, broad phenotypic manifestations, and variable severity. The majority results from a defect of one of the steps involved with protein or lipid N-glycosylation pathway. Almost all are inherited in autosomal recessive patterns with a few exceptions such as the
Hamici Sana; Bastaki Fatma; Khalifa Mohamed
European journal of medical genetics
2017
2017-10
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.ejmg.2017.07.014" target="_blank" rel="noreferrer noopener">10.1016/j.ejmg.2017.07.014</a>
Pathophysiology of empty nose syndrome.
Brain – Physiopathology; Brain/physiopathology; Computer Simulation; Dyspnea – Physiopathology; Dyspnea/physiopathology; Empty nose syndrome; Humans; Nasal Mucosa – Innervation; Nasal Mucosa/*innervation; Nasal Obstruction – Physiopathology; Nasal Obstruction/*physiopathology; nasal sensation; Neural Pathways – Physiopathology; Olfactory Pathways/physiopathology; Postoperative Complications – Physiopathology; Postoperative Complications/*physiopathology; Pulmonary Ventilation/*physiology; Respiratory Airflow – Physiology; Sensory Receptor Cells – Physiology; Sensory Receptor Cells/*physiology; Syndrome; Thermoreceptors – Physiopathology; Thermoreceptors/physiopathology; Tomography; Trigeminal Nerve – Physiopathology; Trigeminal Nerve/physiopathology; turbinate surgery; Turbinates – Physiopathology; Turbinates – Surgery; Turbinates/*physiopathology/*surgery; Wound Healing – Physiology; Wound Healing/*physiology; X-Ray Computed
OBJECTIVES/HYPOTHESIS: To review current knowledge on nasal airflow sensation in relation to empty nose syndrome (ENS). STUDY DESIGN: PubMed searches. METHODS: Current literature pertaining to measurement of nasal patency, mechanism of sensory perception of nasal airflow, and ENS. RESULTS: A reliance on pure anatomical analysis of the anatomy in ENS falls short of explaining the disorder. Our understanding of subjective nasal sensation has advanced, as has our understanding of the flow of air through the nose. Neural healing following a surgical insult may not result in a return to a normal physiologic state. Aberrations in neurosensory systems from improper healing may play a major role in the abnormal sensations ENS patients experience. CONCLUSIONS: An evidence-based hypothesis for the development and symptoms of ENS is offered.
Sozansky Jeanie; Houser Steven M
The Laryngoscope
2015
2015-01
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/lary.24813" target="_blank" rel="noreferrer noopener">10.1002/lary.24813</a>