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https://www.readbyqxmd.com/read/28913922/a-clinically-meaningful-fetal-hemoglobin-threshold-for-children-with-sickle-cell-anemia-during-hydroxyurea-therapy
#1
Jeremie H Estepp, Matthew P Smeltzer, Guolian Kang, Chen Li, Winfred C Wang, Christina Abrams, Banu Aygun, Russell E Ware, Kerri Nottage, Jane S Hankins
Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. HUSTLE (NCT00305175) is a prospective observational study with a primary goal of describing the long-term clinical effects of hydroxyurea escalated to maximal tolerated dose (MTD) in children with SCA...
September 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28910912/-percutaneous-catheterization-for-extracorporeal-membrane-oxygenation-a-retrospective-case-series
#2
X Y Li, H Y He, X Tang, R Wang, C Y Zhang, S Q Wang, B Sun, Z H Tong
Objective: To review the experience of percutaneous catheterization for extracorporeal membrane oxygenation (ECMO) by respiratory intensivists at a single institution. Methods: A retrospective review of 87 patients undergoing percutaneous catheterization for ECMO in Department of Respiratory and Critical Care Medicine of Beijing Chaoyang Hospital from November 2009 to January 2017. Subject demographics, type of support, cannulation configuration, types of cannulas, use of imaging modalities, and complications were recorded and summarized...
September 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28905804/large-pancreatic-pseudocyst-penetrating-into-posterior-mediastinum
#3
Adam Roman Markowski, Elżbieta Brodalka, Katarzyna Guzinska-Ustymowicz, Konrad Zaręba, Dariusz Cepowicz, Bogusław Kędra
We report a rare case of a large mediastinal pancreatic pseudocyst compressing the left atrium and the esophagus and causing dyspnea, palpitations, and emesis. Chest radiograph was non-diagnostic, esophagogastroduodenoscopy showed diffuse extrinsic compression of the distal esophagus and gastric corpus, but a definitive diagnosis was confirmed by computed tomography. We decided to perform surgery due to the recurrence of the pancreatic pseudocyst, a history of unsuccessful radiologically guided external drainage a few years earlier, and a very large diameter of the pseudocyst causing acute cardio-pulmonary distress syndrome...
August 31, 2017: Polski Przeglad Chirurgiczny
https://www.readbyqxmd.com/read/28905364/a-clinical-risk-score-for-pulmonary-artery-thrombosis-during-acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease
#4
Anaïs Winchenne, Jérôme Cecchini, Jean-François Deux, Nicolas De Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Anoosha Habibi, Pablo Bartolucci, Giovanna Melica, Mehdi Khellaf, Marc Michel, Bernard Maitre, Armand Mekontso Dessap
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28904980/enterovirus-human-rhinovirus-a-rare-cause-of-acute-respiratory-distress-syndrome
#5
Parita Soni, Anand Rai, Nidhi Aggarwal, Stephan Kamholz, Taek Yoon, Yizhak Kupfer
A 22-year-old Asian woman presented with respiratory distress, cough, and wheezing for 1 week. Prior history included asthma and Turner syndrome. On presentation to the emergency department, the patient was hypotensive, tachycardic, tachypneic, with an oxyhemoglobin saturation in the mid 80% range while breathing ambient air. Chest radiograph revealed pulmonary vascular congestion and a left lower lobe infiltrate. Endotracheal intubation, mechanical ventilation, and vasopressors were initiated. Empiric therapy for community-acquired pneumonia was administered utilizing broad-spectrum intravenous antibiotics...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28904482/mathematics-of-ventilator-induced-lung-injury
#6
REVIEW
Ubaidur Rahaman
Ventilator-induced lung injury (VILI) results from mechanical disruption of blood-gas barrier and consequent edema and releases of inflammatory mediators. A transpulmonary pressure (PL) of 17 cmH2O increases baby lung volume to its anatomical limit, predisposing to VILI. Viscoelastic property of lung makes pulmonary mechanics time dependent so that stress (PL) increases with respiratory rate. Alveolar inhomogeneity in acute respiratory distress syndrome acts as a stress riser, multiplying global stress at regional level experienced by baby lung...
August 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28904478/how-useful-is-extravascular-lung-water-measurement-in-managing-lung-injury-in-intensive-care-unit
#7
Anirban Bhattacharjee, Debasis Pradhan, Prithwis Bhattacharyya, Samarjit Dey, Daniala Chhunthang, Akash Handique, Angkita Barman, Mohd Yunus
CONTEXT: The primary goal of septic shock management is optimization of organ perfusion, often at the risk of overloading the interstitium and causing pulmonary edema. The conventionally used end points of resuscitation do not generally include volumetric parameters such as extravascular lung water index (EVLWI) and pulmonary vascular permeability index (PVPI). AIMS: This study aimed to assess the prognostic value of EVLWI and PVPI by calculating their correlation with the severity of lung injury...
August 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28901858/expression-of-a-nitric-oxide-synthesizing-protein-in-arterial-endothelial-cells-in-response-to-different-anti-anginal-agents-used-in-acute-coronary-syndromes
#8
Sarbashri Bank, Pradipta Jana, G V Girish, Asru Sinha, Smarajit Maiti
Organic "nitro" compounds such as nitroglycerine, isosorbate nitrate are useful in the control of chest pain in acute coronary syndrome. But the mechanism of it in pain regulation remains speculative. Here, increase of NO production was investigated by the possible regulation of constitutive nitric oxide synthase (cNOS) function from goat arterial endothelial cells. This protein was purified and sequence wise characterized as protein disulfide isomerase (PDI) in response to different nitro compounds. The NO generating protein was isolated from arterial endothelial cells and prepared to homogeneity...
September 11, 2017: Protein and Peptide Letters
https://www.readbyqxmd.com/read/28900861/acute-kidney-injury-caused-by-decompression-illness-successfully-treated-with-hyperbaric-oxygen-therapy-and-temporary-dialysis
#9
Arata Hibi, Keisuke Kamiya, Takahisa Kasugai, Keisuke Kamiya, Satoru Kominato, Chiharu Ito, Toshiyuki Miura, Katsushi Koyama
A 52-year-old Japanese male professional diver was referred to our hospital for decompression illness (DCI). After 1 h of diving operation at 20 m below sea level, he complained of dyspnea, chest pain, and abdominal pain. He dove again, intending to ease the symptoms, but the symptoms were never relieved. He dove for a total of 4 h. No neurological abnormalities were observed. Computed tomography images revealed portal venous gas and mesenteric venous gas, in addition to bubbles in the femoral veins, pelvis, lumbar canal, intracranial sinuses, and joints...
September 12, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28892533/association-of-guideline-adherent-antibiotic-treatment-with-readmission-of-children-with-sickle-cell-disease-hospitalized-with-acute-chest-syndrome
#10
David G Bundy, Troy E Richardson, Matthew Hall, Jean L Raphael, David C Brousseau, Staci D Arnold, Ram V Kalpatthi, Angela M Ellison, Suzette O Oyeku, Samir S Shah
Importance: Acute chest syndrome (ACS) is a common, serious complication of sickle cell disease (SCD) and a leading cause of hospitalization and death in both children and adults with SCD. Little is known about the effectiveness of guideline-recommended antibiotic regimens for the care of children hospitalized with ACS. Objectives: To use a large, national database to describe patterns of antibiotic use for children with SCD hospitalized for ACS and to determine whether receipt of guideline-adherent antibiotics was associated with lower readmission rates...
September 11, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28889147/acute-coronary-syndrome-risk-prediction-of-rapid-emergency-medicine-scoring-system-in-acute-chest-pain-an-observational-study-of-patients-presenting-with-chest-pain-in-the-emergency-department-in-central-saudi-arabia
#11
Tahir Mehmood, Mohammad S Al Shehrani, Muhammad Ahmad
To assess the diagnostic validity of the rapid emergency medical score (REMS) for the risk stratification of acute coronary syndrome (ACS) from non-cardiogenic chest pain. Methods: An observational cross-sectional study was carried out among patients presenting with chest pain to the Emergency Department of Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia, for 6 months from January to June 2016. All patients, included through non-probability convenience sampling, were assessed using standard protocols for the physiological parameters of the REMS, and ACS was confirmed through electrocardiography, cardiac enzyme testing, and angiography (if needed)...
September 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28884028/spontaneous-coronary-artery-dissection-a-rare-manifestation-of-alport-syndrome
#12
Amornpol Anuwatworn, Prince Sethi, Kelly Steffen, Orvar Jonsson, Marian Petrasko
Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28882446/relationship-between-mixed-donor-recipient-chimerism-and-disease-recurrence-following-hematopoietic-cell-transplantation-for-sickle-cell-disease
#13
Allistair Abraham, Matthew Hsieh, Mary Eapen, Courtney Fitzhugh, Jeanette Carreras, Daniel Kessler, Gregory Guilcher, Naynesh Kamani, Mark C Walters, Jaap J Boelens, John Tisdale, Shalini Shenoy
Mixed donor chimerism following hematopoietic cell transplantation for sickle cell disease (SCD) can result in resolution of disease symptoms but symptoms recur when donor chimerism is critically low. The relationship between chimerism, hemoglobin S (HbS) level and symptomatic disease was correlated retrospectively in 95 patients who had chimerism reports available at day 100, 1 and 2 years after transplantation. Recurrent disease was defined as recurrence of vaso-occlusive crises, acute chest syndrome, or stroke, and/or HbS level >50%...
September 4, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28875364/age-and-sex-based-resource-utilisation-and-costs-in-patients-with-acute-chest-pain-undergoing-cardiac-ct-angiography-pooled-evidence-from-romicat-ii-and-acrin-pa-trials
#14
Fabian Bamberg, Thomas Mayrhofer, Maros Ferencik, Daniel O Bittner, Travis R Hallett, Sumbal Janjua, Christopher L Schlett, John T Nagurney, James E Udelson, Quynh A Truong, Pamela K Woodard, Judd E Hollander, Harold Litt, Udo Hoffmann
OBJECTIVES: To determine resource utilisation according to age and gender-specific subgroups in two large randomized diagnostic trials. METHODS: We pooled patient-specific data from ACRIN-PA 4005 and ROMICAT II that enrolled subjects with acute chest pain at 14 US sites. Subjects were randomized between a standard work-up and a pathway utilizing cardiac computed tomography angiography (CCTA) and followed for the occurrence of acute coronary syndrome (ACS) and resource utilisation during index hospitalisation and 1-month follow-up...
September 5, 2017: European Radiology
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#15
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28871571/acute-aortic-dissection-pathogenesis-risk-factors-and-diagnosis
#16
Joanna Gawinecka, Felix Schönrath, Arnold von Eckardstein
Acute aortic dissection is a rare but life-threatening condition with a lethality rate of 1 to 2% per hour after onset of symptoms in untreated patients. Therefore, its prompt and proper diagnosis is vital to increase a patient's chance of survival and to prevent grievous complications. Typical symptoms of acute aortic dissection include severe chest pain, hypotension or syncope and, hence, mimic acute myocardial infarction or pulmonary embolism. Advanced age, male gender, long-term history of arterial hypertension and the presence of aortic aneurysm confer the greatest population attributable risk...
September 5, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28870153/myocardial-infarction-during-anaphylaxis-in-a-young-healthy-male-with-normal-coronary-arteries-is-epinephrine-the-culprit
#17
W D Jayamali, H M M T B Herath, Aruna Kulathunga
BACKGROUND: Anaphylaxis is an acute, potentially fatal medical emergency. Myocardial injury or infarction in the setting of an anaphylaxis can be due the anaphylaxis itself, when it is known as Kounis syndrome or it can also be due to the effect of epinephrine treatment. Epinephrine is considered as the cornerstone in management of anaphylaxis. Myocardial infarction secondary to therapeutic doses of adrenaline is a rare occurrence and only a few cases have been reported in literature...
September 4, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28868627/hydroxyurea-prescription-availability-and-use-for-children-with-sickle-cell-disease-in-italy-results-of-a-national-multicenter-survey
#18
Raffaella Colombatti, Giovanni Palazzi, Nicoletta Masera, Lucia Dora Notarangelo, Elisa Bonetti, Piera Samperi, Angelica Barone, Silverio Perrotta, Elena Facchini, Maurizio Miano, Giovanni Carlo Del Vecchio, Maria Elena Guerzoni, Paola Corti, Federica Menzato, Simone Cesaro, Maddalena Casale, Paolo Rigano, Gian Luca Forni, Giovanna Russo, Laura Sainati
BACKGROUND: The number of patients with sickle cell disease (SCD) has increased in Italy in the past decade due to immigration. In spite of the established efficacy of hydroxyurea (HU) in childhood, population-based data regarding its prescription and effectiveness come mainly from studies performed in adults or outside Europe. POPULATION AND METHODS: The Hydroxyurea in SCD: A Large Nation-wide Cohort Study from Italy was a retrospective cohort study of adult and pediatric patients with SCD attending 32 centers...
September 4, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28868102/a-novel-electrocardiographic-sign-of-an-st-segment-elevation-myocardial-infarction-equivalent-de-winter-syndrome
#19
Mustafa Ugur Goktas, Ozgur Sogut, Mehmet Yigit, Onur Kaplan
Patients with de Winter syndrome, also termed anterior ST-segment elevation myocardial infarction (STEMI)-equivalent, represent 2% of all patients with acute anterior myocardial infarctions admitted to emergency departments (EDs). STEMI-equivalents do not present with classical electrocardiogram (ECG) changes but exhibit a critical stenosis of the left anterior descending (LAD) coronary artery. This is under-recognized by clinicians and is therefore associated with high morbidity and mortality. Here, we report a rare case of a novel, typical, STEMI-equivalent ECG pattern without obvious ST-segment elevation in a 34-year-old female who presented to our ED with substantial chest pain and a large, acute, transmural anterior myocardial infarction caused by acute occlusion of the LAD coronary artery...
August 2017: Cardiology Research
https://www.readbyqxmd.com/read/28865527/emergency-computed-tomography-angiogram-of-the-chest-abdomen-and-pelvis
#20
Kimberly Weatherspoon, Wayne Gilbertie, Tara Catanzano
In the setting of blunt trauma, the rapid assessment of internal injuries is essential to prevent potentially fatal outcomes. Computed tomography is a useful diagnostic tool for both screening and diagnosis. In addition to trauma, acute chest syndromes often warrant emergent computed tomographic angiography, looking for etiologies such as aortic aneurysms or complications of aortic aneurysms, or both, pulmonary emboli, as well as other acute vascular process like aortic dissection and Takayasu aortitis. With continued improvements in diagnostic imaging, computed tomographic angiography of the chest, abdominal and pelvis proves to be an effective modality to image the aorta and other major vascular structures...
August 2017: Seminars in Ultrasound, CT, and MR
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