116,509 results match your criteria Idiopathic Thrombocytopenic Purpura
Sleep Med 2018 Nov 19;54:116-120. Epub 2018 Nov 19.
Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. Electronic address:
Background: This study investigated the protective effect of rapid eye movement (REM) sleep without atonia against obstructive sleep apnea (OSA) in patients with idiopathic REM sleep behavior disorder (RBD).
Methods: In this case-control study, patients with idiopathic RBD and OSA (RBD-OSA) were consecutively enrolled and OSA controls without RBD were matched for age, sex, and apnea-hypopnea index (AHI). Clinical and polysomnographic characteristics were compared between RBD-OSA patients and OSA controls. Read More
Gait Posture 2018 Dec 6;68:300-304. Epub 2018 Dec 6.
School of Physiotherapy, Royal College of Surgeons in Ireland, 123 St Stephen's Green, Dublin 2, Ireland.
Background: Toe-walking is a normal variant in children up to 3 years of age but beyond this a diagnosis of idiopathic toe-walking (ITW) must be considered. ITW is an umbrella term that covers all cases of toe-walking without any diagnosed underlying medical condition and before assigning these diagnosis potential differential diagnoses such as cerebral palsy, peripheral neuropathy, spinal dysraphism and myopathy must be ruled out. Gait laboratory assessment (GLA) is thought to be useful in the evaluation of ITW, and kinematic, kinetic and electromyography features associated with ITW have been described. Read More
J Allergy Clin Immunol Pract 2018 Dec 12. Epub 2018 Dec 12.
Division of Allergy & Immunology, Children's Hospital of Philadelphia, Philadelphia, PA; Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA.
Clinical Implications: Pectin is a structural heteropolysaccharide previously associated with occupational asthma and only rare accounts of allergy. Few case reports, however, suggest that pectin may contribute to atypical or idiopathic food reactions, particularly in patients with IgE-mediated allergy to tree nuts. Read More
Urology 2018 Dec 12. Epub 2018 Dec 12.
Central Texas Veterans Healthcare System, 1901 Veterans Memorial Dr, Temple, TX.
Objectives: To determine if recurrence rates and complication rates differ between three different techniques for treatment of idiopathic hydrocele: Jaboulay bottleneck, Hydrocelectomy, or Lord's technique.
Methods: All surgeries for idiopathic hydrocele in the health system were reviewed from 2000 - 2011. Recurrence rate, complication rate, and other surgical data were collected and analyzed. Read More
Cerebellum 2018 Dec 14. Epub 2018 Dec 14.
Department of Neurology, Ludwig-Maximilians University, Marchioninistrasse 15, 81377, Munich, Germany.
The differential diagnosis of vertigo or dizziness as a result of cerebellar disorders can be difficult as many patients with a cerebellar pathology do not present with the full spectrum of cerebellar signs. The main goal of this study was to describe the typical clinical features of these patients with vertigo or dizziness of a cerebellar origin. We reviewed the medical records of 5400 patients with vertigo and dizziness from our tertiary outpatient clinic for vertigo and balance disorders. Read More
Pediatr Nephrol 2018 Dec 14. Epub 2018 Dec 14.
Department of Pediatric Nephrology, Hôpital Robert-Debré, APHP, 48 Bd Serurier, 75019, Paris, France.
Background: The optimal therapeutic regimen for children at onset of idiopathic nephrotic syndrome (INS) is still under debate. A better knowledge of the disease's course is necessary to design more appropriate and/or personalized treatment protocols.
Methods: We report the 5-year outcome of patients included from December 2007 to May 2010 in the prospective multicentric and multiethnic population-based NEPHROVIR study. Read More
Rheumatol Int 2018 Dec 14. Epub 2018 Dec 14.
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Juvenile dermatomyositis (JDM) is the most common childhood idiopathic inflammatory myopathy (IIM). It is characterized by the classic skin rash in the form of Gottron papules and heliotrope rash, and symmetric proximal muscle weakness. Renal involvement in JDM is rare which includes acute kidney injury and glomerulonephritis. Read More
Ann Rheum Dis 2018 Dec 14. Epub 2018 Dec 14.
Department of Microbiology and Immunology, KUL - University of Leuven, Leuven, Belgium
Objectives: is a key immunological transcription factor, with knockout mice studies identifying functional roles in multiple immune cell types. Despite the importance of NFIL3, little is known about its function in humans.
Methods: Here, we characterised a kindred of two monozygotic twin girls with juvenile idiopathic arthritis at the genetic and immunological level, using whole exome sequencing, single cell sequencing and flow cytometry. Read More
Arch Iran Med 2018 Nov 1;21(11):530-535. Epub 2018 Nov 1.
Department of Microbiology, Immunology, and Biochemistry, University of Tennessee Health Science Center, Memphis, TN, USA.
Background: Discovering the association between genetic variations of metabolizing enzymes with idiopathic diseases such as ulcerative colitis (UC) may not only be an auxiliary agent in diagnosis but also could be an effective pharmacotherapy for inflammatory bowel disease (IBD). The aim of the present case-control study was to determine the association of cytochrome P450 2D6 (CYP2D6 *4), N-acteyltransferase-2 (NAT2*7) and multidrug resistance 1 (MDR1) 3435 C/T genotypes with UC susceptibility and thiopurine methyltransferase (TPMT) enzyme activity.
Methods: TPMT activity was measured by high performance liquid chromatography (HPLC) and genotypes for the 3 mentioned polymorphisms were determined in 215 unrelated UC patients and 212 unrelated healthy controls by polymerase chain reactionrestriction fragment length polymorphism (PCR-RFLP) in a Kurdish population from Iran. Read More
Hum Reprod 2018 Dec 14. Epub 2018 Dec 14.
Laboratory of Seminology - Sperm Bank 'Loredana Gandini' Department of Experimental Medicine, 'Sapienza' University of Rome, Italy.
Study Question: Is ageing associated with a decline in semen quality and molecular changes to human sperm?
Summary Answer: Semen quality declines with advancing age and characteristic molecular changes take place during the ageing process, including increased sperm DNA damage, altered sperm protamination and altered seminal plasma miRNA profile.
What Is Known Already: During ageing, the reproductive system is exposed to physiological changes and potentially damaging factors that may impair testicular function. Reactive oxygen species (ROS) can induce errors during DNA replication, transcription or post-transcriptional events (fragmentation, chromatin condensation abnormalities and protamine expression defects). Read More
Behav Brain Res 2018 Dec 11. Epub 2018 Dec 11.
University of Saskatchewan, Department of Physiology, Saskatoon, SK, Canada.
Abnormalities in social behavior are a co-morbid symptom of idiopathic generalized epilepsies such as childhood absence epilepsy. The Genetic Absence Epilepsy Rats from Strasbourg (GAERS) model is a spontaneously occurring absence epilepsy phenotype closely correlated to that of human absence epilepsies. Similar to the human conditions, GAERS display social abnormalities. Read More
Life Sci 2018 Dec 11. Epub 2018 Dec 11.
Department of Biophysics, All India Institute of Medical Sciences, New Delhi 110029, India. Electronic address:
Aims: Idiopathic nature of male infertility disorder needs to be investigated by different horizons of molecular biology for its treatment and to device male contraceptive. Further, it can also aid in advancement of assisted reproductive technology (ART), as nowadays the failure and disquiets of ART are consistent. Herein, we have attempted to find out proteins responsible for male infertility by comparing proteome profile of sperms collected from normal control and asthenozoospermic (AS) males. Read More
Am J Med 2018 Dec 11. Epub 2018 Dec 11.
University of South Carolina College of Pharmacy, Department of Clinical Pharmacy and Outcomes Sciences, Columbia, SC. Electronic address:
Chronic idiopathic constipation and irritable bowel syndrome with constipation are commonly encountered in ambulatory patients, but limited options exist for patients with persistent or severe symptoms following treatment with non-prescription products. Plecanatide (Trulance, Synergy Pharmaceuticals) is a 16-amino acid peptide analogue of uroguanylin that stimulates guanylate cyclase-C receptors to increase chloride and bicarbonate secretion into the intestine and prevents the absorption of sodium ions, thereby increasing the secretion of water into the lumen. The influx of additional fluid accelerates intestinal transit, softens the stool and facilitates easier defecation. Read More
PLoS One 2018 14;13(12):e0205810. Epub 2018 Dec 14.
Fondation Ophtalmologique Adolphe de Rothschild and Université Paris Descartes, Paris, France.
Objective: To compare the serum inhibin B, anti-Müllerian hormone (AMH) and leptin concentrations in girls with idiopathic central precocious puberty (CPP) to their concomitant characteristics and evaluate the capacity of each of these hormones to predict the age at first menstruation in those who were untreated and who completed their puberty.
Methods: This single-center study included 94 girls selected from a cohort of 493 girls seen between 1981 and 2012 and diagnosed with idiopathic CPP for whom a remaining serum sample collected at the initial evaluation was available. Of these 25 were untreated and completed their puberty. Read More
Retina 2018 Dec 6. Epub 2018 Dec 6.
Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota.
Purpose: To report visual and anatomical outcomes after the repair of chronic idiopathic macular holes (MHs) with no face-down positioning.
Methods: We conducted a retrospective review of chronic MH cases of greater than 1-year duration that were repaired through pars plana vitrectomy with broad internal limiting membrane peeling and no face-down positioning between March 2009 and December 2017. There were 18 eyes of 18 patients that met inclusion criteria. Read More
Isr Med Assoc J 2018 Dec;20(12):770-772
Department of Internal Medicine F, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
Background: Erythema nodosum (EN) is the most common type of panniculitis, commonly secondary to infectious diseases.
Objectives: To elucidate the causative factors and the clinical presentation of patients with EN (2004-2014) and to compare their data to those reported in a previous study.
Methods: A retrospective study was conducted of all patients diagnosed with EN who were hospitalized at Soroka University Medical Center (2004-2014). Read More
Isr Med Assoc J 2018 Dec;20(12):749-753
Heart Institute, Kaplan Medical Center, Rehovot, Israel.
Background: Early identification of patients with a likelihood of cardiac improvement has important implications for management strategies.
Objectives: To evaluate whether tissue Doppler imaging (TDI) and two-dimensional (2D) strain measures may predict left ventricular (LV) improvement in patients with recent onset dilated cardiomyopathy (ROCM).
Methods: Clinical and comprehensive echo were performed at baseline and at 6 months. Read More
Korean J Ophthalmol 2018 Dec;32(6):517-518
Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea.
Korean J Ophthalmol 2018 Dec;32(6):506-516
Department of Ophthalmology, Chungnam National University College of Medicine, Daejeon, Korea.
Purpose: We measured the thicknesses of the ganglion cell and inner plexiform layer (GCIPL), the macula, and the retinal nerve fiber layer (RNFL) using spectral-domain optical coherence tomography in patients with idiopathic macula holes to analyze the repeatability of these measurements and compare them with those of the fellow eye.
Methods: We evaluated 85 patients who visited our retinal clinic. The patients were divided into two groups according to their macular hole size: group A had a size of <400 μm, while group B had a size of ≥400 μm. Read More
Aging Cell 2018 Dec 12:e12858. Epub 2018 Dec 12.
Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China.
Idiopathic pulmonary fibrosis (IPF) is an aging-associated disease with poor prognosis. Currently, there are no effective drugs for preventing the disease process. The mechanisms underlying the role of alveolar epithelial cell (AEC) senescence in the pathogenesis of IPF remain poorly understood. Read More
Pediatr Int 2018 Dec 11. Epub 2018 Dec 11.
University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
Curr Opin Neurol 2018 Dec 12. Epub 2018 Dec 12.
Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston.
Purpose Of Review: To review the most relevant developments in the understanding of headache in idiopathic intracranial hypertension (IIH).
Recent Findings: The phenotype of the typical IIH headache is diverging from the historical thinking of a raised intracranial pressure headache, with the majority being classified as having migraine. A larger proportion of those with IIH have a past medical history of migraine, compared with the general population, highlighting the importance of re-examining those who have a change or escalation in their headache. Read More
Eur Cytokine Netw 2018 Sep;29(3):73-82
Unit of Reproductive Endocrinology, First Department of Obstetrics and Gynecology, Medical School, Aristotle University of Thessaloniki, Greece. "Papageorgiou General Hospital", Ring Road-Nea Efkarpia, 56403 Thessaloniki, Greece.
Many male infertility cases have no apparent cause, being characterized as idiopathic. Both inflammation and obesity have long been associated with infertility. On one hand, inflammation, such as orchitis and male accessory gland infections (MAGIs), are regulated by inflammatory cytokines. Read More
Pediatr Rheumatol Online J 2018 Dec 14;16(1):79. Epub 2018 Dec 14.
Department of Rheumatology, Ajou University of medical school, 164 Worldcup-ro, Yeongtong-gu, Suwon, 16499, Republic of Korea.
Tocilizumab, an anti-interleukin-6 (IL-6) agent, is indicated as a treatment for several autoimmune or inflammatory diseases, including rheumatoid arthritis and juvenile idiopathic arthritis (JIA). IL-6 plays roles in both immune system dysregulation and inflammation, and thus efforts to extend the utility of tocilizumab in patients with autoinflammatory conditions are ongoing. Here, we survey the literature on the off-label use of tocilizumab in patients with juvenile-onset rheumatic diseases including juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis (DM), vasculitis, juvenile scleroderma, and other autoinflammatory diseases. Read More
BMC Nephrol 2018 Dec 14;19(1):358. Epub 2018 Dec 14.
Nephrology department, The Xinjiang Uygur Autonomous Region People's Hospital, 91 Tianchi Road, Urumqi, Xinjiang, China.
Background: IgA nephropathy (IgAN) is one of the most common forms of idiopathic glomerular diseases and might lead to end-stage kidney disease. Accurate and non-invasive biomarkers for early diagnosis are required for early intervention and consequent therapy for IgAN patients. Because variance in the disease incidence and predisposing genes of IgAN has been detected among different ethnicities, the ethnicity factor should be considered in IgAN biomarker discovery. Read More
J Cardiol Cases 2014 Jan 13;9(1):35-39. Epub 2013 Dec 13.
Division of Cardiology, Fujita Health University School of Medicine, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan.
A 70-year-old woman with back pain and breathlessness was referred to our hospital for suspected myocardial infarction. Coronary angiogram was normal and endomyocardial biopsy showed inflammatory cell infiltrates consisting of eosinophils and multinucleated giant cells. The clinical course was hemodynamically fulminant, but steroid therapy improved the cardiac function. Read More
J Cardiol Cases 2010 Aug 21;2(1):e52-e54. Epub 2010 Mar 21.
Department of Cardiology, Clinical Hospital Dubrava, Zagreb, Croatia.
We report two cases of idiopathic atrial dilatation in two adult siblings, a brother and a sister. The first patient was a 36-year-old man who was referred to our institution for evaluation of atrial fibrillation and syncopes. Transthoracic echocardiography revealed an enlarged right atrium accompanied by a severe tricuspid regurgitation associated with annular dilatation. Read More
J Cardiol Cases 2016 Jul 5;14(1):8-10. Epub 2016 Apr 5.
Cardiology Department, Hospital Universitario de La Princesa, Madrid, Spain.
A 57-year-old woman presented with a large idiopathic pericardial effusion with subsequent cardiac tamponade, in whom the intrapericardial pressure measured by pericardiocentesis was not elevated. The patient did not present with the classical clinical features of cardiac tamponade, requiring echocardiographic follow-up to make accurate diagnosis and treatment. This entity called low-pressure cardiac tamponade has been diagnosed anecdotally being an insidious condition that requires a high degree of suspicion. Read More
J Cardiol Cases 2016 Jul 7;14(1):4-7. Epub 2016 Apr 7.
Division of Cardiology, Northwestern Memorial Hospital, Chicago, IL, USA.
Myocarditis can be idiopathic or arise in response to numerous systemic insults. Myocarditis occurring in the setting of an exacerbation of inflammatory bowel disease is a rare extra-intestinal manifestation of both ulcerative and Crohn's-related colitis. Here, we present a unique case of a 56-year-old female patient presenting with an acute Crohn's colitis flare that was eventually complicated by myocarditis. Read More
J Cardiol Cases 2016 Apr 7;13(4):101-104. Epub 2016 Feb 7.
Lokmanya Tilak Municipal Medical College & General Hospitals, Sion, Mumbai, India.
We report a rare clinical presentation of incessant idiopathic fascicular ventricular tachycardia (FVT), presenting as multi-organ dysfunction (MOD) syndrome with cardiogenic shock. Our patient was a 19-year-old male who presented with slowly progressive dyspnea from New York Heart Association (NYHA) II to NYHA IV at the time of presentation, palpitations, and dilated cardiomyopathy due to drug-refractory FVT. The patient was in cardiogenic shock with raised central venous pressures and required inotropic support for maintaining systolic blood pressure above 90 mmHg. Read More
J Cardiol Cases 2015 Oct 6;12(4):101-103. Epub 2015 Jun 6.
Cardiology Clinic, Türkiye Yüksek İhtisas Training and Research Hospital, 06100, Ankara, Turkey.
There have been rare case series with familial clustering of peripartum cardiomyopathy (PPCMP). Due to the concomitant occurrence of PPCMP and idiopathic dilated cardiomyopathy, it has been suggested that genetics might play a role in the pathogenesis of PPCMP. Herein, we report four cases of PPCMP in the same family, who showed full recovery of left ventricular function within a short period. Read More
J Cardiol Cases 2014 Aug 11;10(2):58-61. Epub 2014 Jun 11.
Department of Cardiology and Hematology, Fukushima Medical University, Fukushima, Japan.
A 60-year-old woman with a 6-year history of numbness in her hands was admitted to hospital with dyspnea. Laboratory findings showed the elevation of creatine kinase (creatine kinase MB isoenzyme was less than 4 IU/l). Chest X-ray revealed cardiomegaly and pulmonary edema. Read More
J Patient Rep Outcomes 2018 Dec 13;2(1):61. Epub 2018 Dec 13.
Temple University Hospital, 3401 N Broad Street, #1003, Philadelphia, PA, 19140, USA.
Background: The American Neurogastroenterology and Motility Society Gastroparesis Cardinal Symptom Index-Daily Diary (ANMS GCSI-DD) was developed to meet Food and Drug Administration (FDA) recommendations for patient-reported outcome (PRO) endpoints in gastroparesis studies, including therapeutic trials. The current version of the ANMS GCSI-DD contains five items pertaining to nausea, early satiety, post-prandial fullness, upper abdominal pain, and vomiting. The specific aims of this study were to determine if the appropriate symptoms are included in the ANMS GCSI-DD and to assess the content validity in patients with idiopathic (IG) and diabetic gastroparesis (DG). Read More
Paediatr Drugs 2018 Dec 13. Epub 2018 Dec 13.
Division of Rheumatology, Children's Mercy Kansas City, 2401 Gillham Road, Kansas City, MO, 64108, USA.
Background: Arthropathy of Down syndrome (DA) is largely under-recognized, with an average 2-year delay in diagnosis. Most patients present with polyarthritis, and treatment has historically been challenging.
Objectives: Our objective was to investigate the clinical features and treatment of DA in the largest cohort reported to date. Read More
J Vasc Surg Cases Innov Tech 2018 Dec 4;4(4):292-295. Epub 2018 Dec 4.
Division of Vascular Surgery, Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah.
Radial artery aneurysms are exceedingly rare, with only a few reported cases of surgical revascularization. We describe a 25-year-old man who presented with severe ischemia of the right hand secondary to an idiopathic true radial artery aneurysm at the anatomic snuff box. The patient had embolic occlusions in his hand and fingers that were treated with catheter-directed thrombolysis. Read More
J Spine Surg 2018 Sep;4(3):575-582
Department of Spinal Surgery, Royal Orthopaedic Hospital, Birmingham, UK.
Background: To assess the reliability of the indicators for performing magnetic resonance imaging in patients with scoliosis and assess the incidence of neural axis anomalies in a population with scoliosis referred to a specialist centre.
Methods: A retrospective review of magnetic resonance imaging (MRI) reports of all patients under the age of 18 who underwent a pre-operative MRI for investigation of their scoliosis between 2009 and 2014 at a single institution was performed.
Results: There were 851 patients who underwent an MRI scan of their whole spine with a mean age of 14. Read More
Sci Adv 2018 Dec 12;4(12):eaav1781. Epub 2018 Dec 12.
Program in Developmental & Stem Cell Biology, The Hospital for Sick Children, 686 Bay Street, Toronto, Ontario M5G 0A4, Canada.
The etiopathogenesis of idiopathic scoliosis (IS), a highly prevalent spinal deformity that occurs in the absence of obvious congenital or physiological abnormalities, is poorly understood. Although recent zebrafish genetic studies have linked cilia motility and cerebrospinal fluid (CSF) flow defects with scoliosis progression, underlying mechanisms were not identified. Here, we use next-generation sequencing and conditional genetic methodologies to define the spatial and biological origins of spinal curve formation in mutant zebrafish, a faithful IS model. Read More
Exp Ther Med 2018 Dec 5;16(6):5224-5226. Epub 2018 Oct 5.
Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido 060-8638, Japan.
It is often difficult to correctly diagnose patients who present with dilation of the bile duct. Cholangiocarcinoma, primary sclerosing cholangitis (PSC) and immunoglobulin (Ig)G4-related sclerosing cholangitis must be considered as potential diagnoses for these cases. The current study presents a 73-year-old female patient who presented with a high fever and abdominal pain. Read More
Drugs Ther Perspect 2018 15;34(7):300-310. Epub 2018 Jun 15.
Springer, Private Bag 65901, Mairangi Bay 0754, Auckland, New Zealand.
Macrogol 4000, a biologically inert, non-absorbable osmotic laxative, is a highly effective and well-tolerated first-line option for the treatment of the symptoms of chronic idiopathic/functional constipation in children and adults. High-molecular-weight (HMW) macrogols ± electrolytes have generally similar efficacy profiles; however, the taste of macrogol 4000 is generally preferred over that of macrogol 3350 + electrolytes. Macrogol 4000 is more effective than lactulose in improving stool frequency and consistency, and is associated with less vomiting and flatulence. Read More
Drugs Ther Perspect 2018 31;34(12):543-553. Epub 2018 Oct 31.
Springer, Private Bag 65901, Mairangi Bay, 0754 Auckland, New Zealand.
The EU indication for anakinra has been extended to include Still's disease, a serious rare inflammatory disorder of unknown aetiology that comprises adult-onset Still's disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA). As activated interleukin-1 pathways are associated with the systemic manifestations of these disorders, targeted treatment with anakinra, an interleukin-1 inhibitor, has been investigated. Across clinical and real-world studies in patients with AOSD and SJIA, treatment with anakinra achieved clinical remission/response, provided rapid and sustained improvements in systemic and laboratory manifestations, and allowed the use of corticosteroid- and disease-modifying anti-rheumatic drugs (DMARD) to be reduced or discontinued. Read More
Egypt J Neurol Psychiatr Neurosurg 2018 21;54(1):37. Epub 2018 Nov 21.
Faculty of Medicine, Fayoum University, Fayoum City, 63611 Egypt.
Background: Carnitine deficiency is relatively common in epileptic patients. The risk factors reported include the combination of valproic acid with other antiepileptic drugs (AEDs), young age, multiple neurologic disabilities, non-ambulatory status, and being underweight.
Objectives: To study the level of carnitine deficiency and its associated risk factors among a group of children with idiopathic epilepsy treated with different AEDs. Read More
Eur Respir J 2018 Dec 13. Epub 2018 Dec 13.
Vanderbilt University Medical Center, Nashville, TN, USA.
Since 2000 there have been major advances in our understanding of the genetic and genomics of pulmonary arterial hypertension (PAH), although there remains much to discover. Based on existing knowledge, around 25-30% of patients diagnosed with idiopathic PAH have an underlying Mendelian genetic cause for their condition and should be classified as heritable PAH (HPAH). Here, we summarise the known genetic and genomic drivers of PAH, the insights these provide into pathobiology, and the opportunities afforded for development of novel therapeutic approaches. Read More
Eur Respir J 2018 Dec 13;52(6). Epub 2018 Dec 13.
National Reference Center for Rare Pulmonary Disease, Louis Pradel Hospital; UMR754, Claude Bernard University, Lyon, France.
J Am Coll Cardiol 2018 Dec;72(24):3177-3188
Université de Rennes1-Faculté de Médecine, Rennes, France; Service de Cardiologie, Centre Hospitalier Universitaire, Rennes, France; LTSI-INSERM U1099, Rennes, France.
Idiopathic or iatrogenic left bundle branch block (LBBB) is a unique model of electro-mechanical ventricular dyssynchrony with concordant changes in electrical activation sequence and mechanical ventricle synchronization. In chronic animal models, isolated LBBB induces structural remodeling with progressive left ventricular (LV) dysfunction. Most abnormalities can be reverted after cardiac resynchronization therapy (CRT). Read More
Arthritis Res Ther 2018 Dec 13;20(1):276. Epub 2018 Dec 13.
German Rheumatism Research Center, a Leibniz Institute, Charitéplatz 1, 10117, Berlin, Germany.
Objective: The objective was to evaluate the 25(OH) vitamin D (25(OH)D) status of patients with juvenile idiopathic arthritis (JIA) and determine whether the 25(OH)D level is associated with disease activity and the course of JIA.
Methods: Patients ≤ 16 years of age with recently diagnosed JIA (< 12 months) were enrolled in the inception cohort of patients with newly diagnosed JIA (ICON), an ongoing prospective observational, controlled multicenter study started in 2010. Clinical and laboratory parameters were ascertained quarterly during the first year and half-yearly thereafter. Read More
Arthritis Res Ther 2018 Dec 13;20(1):275. Epub 2018 Dec 13.
Department of Rheumatology, Hospital Clinic, University of Barcelona, Carrer Villarroel 170, 08036, Barcelona, Spain.
Background: Calprotectin is a biomarker of disease activity in rheumatoid arthritis (RA) and psoriatic arthritis (PsA) and predicts relapse in juvenile idiopathic arthritis. Higher drug trough serum levels are associated with a good response in patients treated with tumor necrosis factor inhibitors (TNFi). Power Doppler ultrasound synovitis is predictive of relapse and structural damage progression in patients in clinical remission. Read More
Orv Hetil 2018 Dec;159(50):2144-2149
Fogászati és Szájsebészeti Klinika, Pécsi Tudományegyetem, Klinikai Központ Pécs, Dischka Győző u. 5., 7621.
From an anatomic and functional point of view, the stomatognathic system and the upper cervical spine are closely connected. Together with the complex neuromuscular relationships, this generates an important field of cooperation between dentists and orthopedics. The aim of this case report was to demonstrate the improvements of temporomandibular joint (TMJ) function after orthopedic surgery in case of a patient with idiopathic scoliosis. Read More
Medicina (Kaunas) 2018 Dec 10;54(6). Epub 2018 Dec 10.
Birmingham Regional NHS Occupational Lung Disease Service, Birmingham Chest Clinic, Birmingham B33HX, UK.
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterised by a progressive and irreversible decline in lung function, which is associated with poor long-term survival. The pathogenesis of IPF is incompletely understood. An accumulating body of evidence, obtained over the past three decades, suggests that occupational and environmental exposures may play a role in the development of IPF. Read More
Medicine (Baltimore) 2018 Dec;97(49):e13492
Rationale: Inflammatory bowel disease (IBD) patients manifest symptoms of disturbed gut function, such as neural sensory-motor changes. Programmed cell death-ligand 1 (PD-L1), normally present in neural tissue, exists in close apposition to the mucosal immune system and intestinal epithelium, and a bi-directional communication is known to occur at these interfaces. Somatostatin has been shown to suppress the inflammatory reaction, and has been used in several clinical trials to treat inflammatory disorders, such rheumatoid arthritis. Read More
Medicine (Baltimore) 2018 Dec;97(49):e13365
Department of Ophthalmology, Fondation Ophtalmologique Adolphe de Rothschild.
Rationale: The association between intracranial hypertension (ICH) and ulcerative colitis (UC) is rare. We report the unusual case of a male patient with UC and ICH in whom both conditions resolved with mesalazine therapy.
Patient Concerns: A 48-year-old Caucasian man presented to our department in June 2016 for decreased vision, transient visual obscuration, pulsatile tinnitus and headaches of 7 months duration. Read More