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Idiopathic increased hypertension

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https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#1
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27903844/therapeutic-approaches-to-pediatric-pseudotumor-cerebri-new-insights-from-literature-data
#2
Giovanna Vitaliti, Piero Pavone, Nassim Matin, Omidreza Tabatabaie, Salvatore Cocuzza, Michele Vecchio, Luigi Maiolino, Paola Di Mauro, Angelo Conti, Riccardo Lubrano, Agostino Serra, Raffaele Falsaperla
The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards...
November 30, 2016: International Journal of Immunopathology and Pharmacology
https://www.readbyqxmd.com/read/27890184/imaging-of-cerebrospinal-fluid-rhinorrhea-and-otorrhea
#3
REVIEW
Mahati Reddy, Kristen Baugnon
As the obesity epidemic grows in the United States, rhinorrhea and otorrhea from spontaneous cerebrospinal fluid (CSF) leaks secondary to untreated idiopathic intracranial hypertension are increasing in prevalence. CSF rhinorrhea and otorrhea should also be carefully evaluated in posttraumatic and postsurgical settings, because untreated CSF leaks often have serious consequences. The work-up, diagnosis, and characterization of a CSF leak can be complex, often requiring a multimodality approach to optimize surgical planning...
January 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/27886896/venous-sinus-stenting-for-idiopathic-intracranial-hypertension-where-are-we-now
#4
REVIEW
Marc J Dinkin, Athos Patsalides
Idiopathic intracranial hypertension causes headache, papilledema and visual field loss, typically in obese women of childbearing age. Its anatomical underpinnings remain unclear, but a stenosis at the junction of the transverse and sigmoid sinuses has been recognized in the majority of patients through venography. The stenosis may result from intrinsic dural sinus anatomy or extrinsic compression by increased intracranial pressure, but in either case, its stenting has been shown to lead to an improvement in symptoms of intracranial hypertension and papilledema in multiple retrospective, non-controlled studies...
February 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/27884593/idiopathic-interstitial-pneumonia-associated-pulmonary-hypertension-a-target-for-therapy
#5
Steven D Nathan, Jürgen Behr, Vincent Cottin, Harold R Collard, Marius M Hoeper, Fernando J Martinez, Athol U Wells
Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined...
November 5, 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27881557/plasma-metabolomics-implicate-modified-transfer-rnas-and-altered-bioenergetics-in-the-outcome-of-pulmonary-arterial-hypertension
#6
Christopher J Rhodes, Pavandeep Ghataorhe, John Wharton, Kevin C Rue-Albrecht, Charaka Hadinnapola, Geoffrey Watson, Marta Bleda, Matthias Haimel, Gerry Coghlan, Paul A Corris, Luke S Howard, David G Kiely, Andrew J Peacock, Joanna Pepke-Zaba, Mark Toshner, Stephen J Wort, J Simon R Gibbs, Allan Lawrie, Stefan Gräf, Nicholas W Morrell, Martin R Wilkins
BACKGROUND: -Pulmonary arterial hypertension (PAH) is a heterogeneous disorder with high mortality. METHODS: -We conducted a comprehensive study of plasma metabolites using ultra-performance liquid chromatography mass-spectrometry to (1) identify patients at high risk of early death, (2) identify patients who respond well to treatment and (3) provide novel molecular insights into disease pathogenesis. RESULTS: -53 circulating metabolites distinguished well-phenotyped patients with idiopathic or heritable PAH (n=365) from healthy controls (n=121) following correction for multiple testing (p<7...
November 21, 2016: Circulation
https://www.readbyqxmd.com/read/27867361/calcium-sensing-receptor-regulates-cytosolic-ca-2-and-plays-a-major-role-in-the-development-of-pulmonary-hypertension
#7
REVIEW
Kimberly A Smith, Ramon J Ayon, Haiyang Tang, Ayako Makino, Jason X-J Yuan
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by elevated pulmonary vascular resistance (PVR) leading to right heart failure and premature death. The increased PVR results in part from pulmonary vascular remodeling and sustained pulmonary vasoconstriction. Excessive pulmonary vascular remodeling stems from increased pulmonary arterial smooth muscle cell (PASMC) proliferation and decreased PASMC apoptosis. A rise in cytosolic free Ca(2+) concentration ([Ca(2+)]cyt) in PASMC is a major trigger for pulmonary vasoconstriction and a key stimulus for PASMC proliferation and migration, both contributing to the development of pulmonary vascular remodeling...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27851839/diagnosis-treatment-and-clinical-management-of-pulmonary-arterial-hypertension-in-the-contemporary-era-a-review
#8
Bradley A Maron, Nazzareno Galiè
Importance: Pulmonary arterial hypertension (PAH) is characterized by severe remodeling of the distal pulmonary arteries, increased pulmonary vascular resistance, and right ventricular dysfunction that promotes heart failure. Once regarded as largely untreatable, evidence-based decision making now guides clinical management of PAH and improves outcomes. However, misconceptions regarding the approach to PAH in the modern era are common and associated with substandard clinical care. Observations: The clinical profile of PAH has changed substantially since its original description...
November 16, 2016: JAMA Cardiology
https://www.readbyqxmd.com/read/27817818/clinical-usefulness-of-end-tidal-co2-profiles-during-incremental-exercise-in-patients-with-chronic-thromboembolic-pulmonary-hypertension
#9
R P Ramos, E V M Ferreira, F M Valois, A Cepeda, C M S Messina, R K Oliveira, A T V Araújo, C A Teles, J A Neder, L E Nery, J S Ota-Arakaki
INTRODUCTION: Great ventilation to carbon dioxide output (ΔV˙E/ΔV˙CO2) and reduced end-tidal partial pressures for CO2 (PetCO2) during incremental exercise are hallmarks of chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary arterial hypertension (IPAH). However, CTEPH is more likely to involve proximal arteries, which may lead to poorer right ventricle-pulmonary vascular coupling and worse gas exchange abnormalities. Therefore, abnormal PetCO2 profiles during exercise may be more prominent in patients with CTEPH and could be helpful to indicate disease severity...
November 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27816994/bmprii-influences-the-response-of-pulmonary-microvascular-endothelial-cells-to-inflammatory-mediators
#10
Leanda Vengethasamy, Aurélie Hautefort, Birger Tielemans, Catharina Belge, Frédéric Perros, Stijn Verleden, Elie Fadel, Dirk Van Raemdonck, Marion Delcroix, Rozenn Quarck
Mutations in the bone morphogenetic protein receptor (BMPR2) gene have been observed in 70 % of patients with heritable pulmonary arterial hypertension (HPAH) and in 11-40 % with idiopathic PAH (IPAH). However, carriers of a BMPR2 mutation have only 20 % risk of developing PAH. Since inflammatory mediators are increased and predict survival in PAH, they could act as a second hit inducing the development of pulmonary hypertension in BMPR2 mutation carriers. Our specific aim was to determine whether inflammatory mediators could contribute to pulmonary vascular cell dysfunction in PAH patients with and without a BMPR2 mutation...
November 6, 2016: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/27800456/successful-lung-transplant-after-prolonged-extracorporeal-membrane-oxygenation-ecmo-in-a-child-with-pulmonary-hypertension-a-case-report
#11
Cecile Tissot, Walid Habre, Paola Soccal, Maja Isabel Hug, Dominique Bettex, Michel Pellegrini, Yacine Aggoun, Anne Mornand, Afksendyios Kalangos, Peter Rimensberger, Maurice Beghetti
INTRODUCTION: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential contraindication to, lung transplantation. However, only a few pediatric cases have been described thus far. CASE PRESENTATION: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter. ECMO was used as a bridge to lung transplantation. However, after prolonged resuscitation, he developed medullary ischemia and medullary syndrome...
August 2016: Research in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27796553/cerebrospinal-fluid-csf-shunting-and-ventriculocisternostomy-etv-in-400-pediatric-patients-shifts-in-understanding-diagnostics-case-mix-and-surgical-management-during-half-a-century
#12
A Henriette Paulsen, Bernt J Due-Tønnessen, Tryggve Lundar, Karl-Fredrik Lindegaard
OBJECTIVE: To characterize shifts from the 1960s to the first decade in the 21st century as to diagnostics, case-mix, and surgical management of pediatric patients undergoing permanent CSF diversion procedures. METHODS: One hundred and thirty-four patients below 15 years of age were the first time treated with CSF shunt or ETV for hydrocephalus or idiopathic intracranial hypertension (IIH) in 2009-2013. This represents our current practice. Our previously reported cohorts of shunted children 1967-1970 (n = 128) and 1985-1988 (n = 138) served as backgrounds for comparison...
October 29, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27770446/the-role-of-genetics-in-pulmonary-arterial-hypertension
#13
REVIEW
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically, it has been associated with a high mortality rate, although, over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
October 22, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27748297/association-of-organochlorine-pesticides-with-the-mrna-expression-of-tumour-necrosis-factor-alpha-tnf-%C3%AE-cyclooxygenase-2-cox-2-genes-in-idiopathic-preterm-birth
#14
Vipin Tyagi, M D Mustafa, Tusha Sharma, B D Banerjee, Rafat S Ahmed, A K Tripathi, Kiran Guleria
BACKGROUND & OBJECTIVES: Preterm birth (PTB) is an important cause of prenatal death, neonatal morbidity and mortality and adult illness. Increased inflammation occurs in normal parturition, and inflammatory cytokines and oxidative stress are found to be higher in PTB cases. The present study was planned to investigate the association of organochlorine pesticides (OCPs) with mRNA expression of inflammatory pathway genes such as tumour necrosis factor-alpha (TNF-α) and cyclooxygenase-2 (COX-2) in preterm delivery (PTD) cases...
June 2016: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/27739559/bilateral-cavernous-sinus-thrombosis-in-a-patient-with-tacrolimus-associated-posttransplant-thrombotic-microangiopathy
#15
Anton M Kolomeyer, Ken K Nischal, Ellen Mitchell
PURPOSE: To report a case of bilateral cavernous sinus thrombosis (CST) in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy. METHODS: Case report. RESULTS: An 8-year-old boy with a medical history of orthotopic heart transplant, posttransplant lymphoproliferative disease, and recurrent infections was hospitalized for nausea, vomiting, and diarrhea. His ocular history included accommodative esotropia, hyperopia with astigmatism, Molluscum contagiosum lid lesions, and idiopathic intracranial hypertension...
October 13, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/27725386/molecular-mechanism-of-dihydropyridine-ca-2-channel-blockers-in-pulmonary-hypertension
#16
Aya Yamamura
 Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and fatal disease of unidentified pathogenesis. IPAH is pathologically characterized as sustained vasoconstriction and vascular remodeling of the pulmonary artery. In pulmonary arterial smooth muscle cells (PASMCs), an increase in cytosolic Ca(2+) concentration ([Ca(2+)]cyt) triggers vasoconstriction and stimulates cell proliferation leading to vascular remodeling. However, dihydropyridine-type voltage-dependent Ca(2+) channel blockers are only effective in very few patients with IPAH (<10%)...
2016: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://www.readbyqxmd.com/read/27711045/non-invasive-inhaled-nitric-oxide-in-the-treatment-of-hypoxemic-respiratory-failure-in-term-and-preterm-infants
#17
R Sahni, X Ameer, K Ohira-Kist, J-T Wung
OBJECTIVES: Inhaled nitric oxide (iNO) is effective in conjunction with tracheal intubation (TI) and mechanical ventilation (MV) for treating arterial pulmonary hypertension and hypoxemic respiratory failure (HRF) in near-term and term newborns. Non-invasive respiratory support with nasal continuous positive airway pressure (CPAP) is increasingly used to avoid morbidity associated with TI and MV, yet the effectiveness of iNO delivery via nasal CPAP remains unknown. To evaluate the effectiveness of iNO delivered via the bubble nasal CPAP system in term and preterm newborns with HRF...
October 6, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/27683614/sphingosine-1-phosphate-is-involved-in-the-occlusive-arteriopathy-of-pulmonary-arterial-hypertension
#18
Salina Gairhe, Sachindra R Joshi, Mrigendra M Bastola, Jared M McLendon, Masahiko Oka, Karen A Fagan, Ivan F McMurtry
Despite several advances in the pathobiology of pulmonary arterial hypertension (PAH), its pathogenesis is not completely understood. Current therapy improves symptoms but has disappointing effects on survival. Sphingosine-1-phosphate (S1P) is a lysophospholipid synthesized by sphingosine kinase 1 (SphK1) and SphK2. Considering the regulatory roles of S1P in several tissues leading to vasoconstriction, inflammation, proliferation, and fibrosis, we investigated whether S1P plays a role in the pathogenesis of PAH...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27683612/versican-accumulates-in-vascular-lesions-in-pulmonary-arterial-hypertension
#19
Ya-Ting Chang, Christina K Chan, Inger Eriksson, Pamela Y Johnson, Xiaofang Cao, Christian Westöö, Christian Norvik, Annika Andersson-Sjöland, Gunilla Westergren-Thorsson, Staffan Johansson, Ulf Hedin, Lena Kjellén, Thomas N Wight, Karin Tran-Lundmark
Pulmonary arterial hypertension (PAH) is a lethal condition for which there is no effective curative pharmacotherapy. PAH is characterized by vasoconstriction, wall thickening of pulmonary arteries, and increased vascular resistance. Versican is a chondroitin sulfate proteoglycan in the vascular extracellular matrix that accumulates following vascular injury and promotes smooth-muscle cell proliferation in systemic arteries. Here, we investigated whether versican may play a similar role in PAH. Paraffin-embedded lung sections from patients who underwent lung transplantation to treat PAH were used for immunohistochemistry...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27662346/distortion-product-otoacoustic-emissions-and-intracranial-pressure-during-csf-infusion-testing
#20
Michael A Williams, Jan Malm, Anders Eklund, Nicholas J Horton, Susan E Voss
BACKGROUND: A noninvasive method to monitor changes in intracranial pressure (ICP) is required for astronauts on long-duration spaceflight who are at risk of developing the Visual Impairment/Intracranial Pressure syndrome that has some, but not all of the features of idiopathic intracranial hypertension. We assessed the validity of distortion product otoacoustic emissions (DPOAEs) to detect changes in ICP. METHODS: Subjects were eight patients undergoing medically necessary diagnostic cerebrospinal fluid (CSF) infusion testing for hydrocephalus...
2016: Aerospace Medicine and Human Performance
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