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Multisystem organ failure

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https://www.readbyqxmd.com/read/28127473/hemadsorption-with-adult-cytosorb%C3%A2-in-a-low-weight-pediatric-case
#1
Catalin Gabriel Cirstoveanu, Ileana Barascu, Samantha Mc Kenzie Stancu
Cytokine adsorber (CytoSorb) has been used successfully as adjunctive treatment for adult patients with elevated cytokine levels in the setting with severe sepsis and septic shock and to reduce blood myoglobin, unconjugated bilirubin, and conjugated bilirubin. In this article we present the case of a nine-month-old male infant who was admitted to the NICU due to sepsis after cardiac surgery, Fallot tetralogy, and multisystem organ failure (MSOF) including liver failure and renal failure which was successfully treated by a combination of continuous hemodiafiltration (HDF) and hemadsorption with CytoSorb...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28098544/fatal-emmonsia-sp-infection-and-fungemia-after-orthotopic-liver-transplantation
#2
Shanthi Kappagoda, Jason Y Adams, Robert Luo, Niaz Banaei, Waldo Concepcion, Dora Y Ho
We report a fatal case of disseminated Emmonsia sp. infection in a 55-year-old man who received an orthotopic liver transplant. The patient had pneumonia and fungemia, and multisystem organ failure developed. As human habitats and the number of immunocompromised patients increase, physicians must be aware of this emerging fungal infection.
February 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28041799/eif2b-related-multisystem-disorder-in-two-sisters-with-atypical-presentations
#3
Jin Sook Lee, Sangmoon Lee, Murim Choi, Byung Chan Lim, Jieun Choi, Ki Joong Kim, Jung-Eun Cheon, In-One Kim, Jong-Hee Chae
BACKGROUND: Vanishing white matter disease (VWM) is a chronic progressive leukoencephalopathy that is characterized by cerebellar ataxia and spasticity, together with cystic degeneration of the cerebral white matter as evidenced by brain magnetic resonance imaging (MRI). Here, we report two sisters with EIF2B2 variants, who presented with delayed development and failure to thrive before 1 year of age, developed cataracts, and showed diffuse leukoencephalopathy. CASE PRESENTATION: The index case had a history of hepatomegaly and intermittent vomiting after upper respiratory infection at 11 months of age...
July 18, 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/27932175/first-attempt-of-sequential-living-donor-liver-and-hematopoietic-stem-cell-transplantation-in-a-child-with-advanced-hepatocellular-carcinoma-case-report
#4
J A Picoraro, N Ovchinsky, M Martinez, S J Lobritto, P Satwani, R Ramphal, M S Cairo, T Kato
Effective therapeutic options for advanced hepatocellular carcinoma are limited. Hematopoietic stem cell transplantation may offer a graft-versus-tumor effect. Combined liver and hematopoietic stem cell transplantation from the same donor with preparatory conditioning may promote tolerogenicity to the liver allograft and offers the potential for immunosuppression withdrawal. We report our experience with the use of this approach in a pediatric patient with invasive hepatocellular carcinoma and pulmonary metastases who underwent a living-donor liver transplantation followed by reduced-toxicity myeloablative conditioning and hematopoietic stem cell transplant from the same parental donor...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27927857/novel-clinical-and-pathologic-findings-in-a-heartland-virus-associated-death
#5
Mary-Margaret A Fill, Margaret L Compton, Edward C McDonald, Abelardo C Moncayo, John R Dunn, William Schaffner, Julu Bhatnagar, Sherif R Zaki, Timothy F Jones, Wun-Ju Shieh
We describe an investigation into a Heartland Virus (HRTV)-associated death in Tennessee with novel clinical and pathologic findings. HRTV can cause rapidly fatal, widely disseminated infection with multisystem organ failure in patients without substantial comorbidities. We identified viral antigen in multiple organ tissues where it was not detected previously.
December 7, 2016: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/27887788/hospital-admission-volume-does-not-impact-the-in-hospital-mortality-of-acute-pancreatitis
#6
Ayesha Kamal, Amitasha Sinha, Susan M Hutfless, Elham Afghani, Mahya Faghih, Mouen A Khashab, Anne Marie Lennon, Dhiraj Yadav, Martin A Makary, Dana K Andersen, Anthony N Kalloo, Vikesh K Singh
BACKGROUND: Multiple factors influence mortality in Acute Pancreatitis (AP). METHODS: To evaluate the association of demographic, clinical, and hospital factors with the in-hospital mortality of AP using a population-based administrative database. The Maryland HSCRC database was queried for adult (≥18 years) admissions with primary diagnosis of AP between 1/94-12/10. Organ failure (OF), interventions, hospital characteristics and referral status were evaluated...
November 23, 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/27881386/ppar%C3%AE-augments-heart-function-and-cardiac-fatty-acid-oxidation-in-early-experimental-polymicrobial-sepsis
#7
Stephen Wade Standage, Brock G Bennion, Taft Olpin Knowles, Dolena R Ledee, Michael A Portman, John K McGuire, W Conrad Liles, Aaron K Olson
Children with sepsis and multisystem organ failure have downregulated leukocyte gene expression of PPARα, a nuclear hormone receptor transcription factor that regulates inflammation and lipid metabolism. Mouse models of sepsis have likewise demonstrated that the absence of PPARα is associated with decreased survival and organ injury, specifically of the heart. Using a clinically relevant mouse model of early sepsis, we found that heart function increases in wild type (WT) mice over the first 24 hours of sepsis, but that mice lacking PPARα (Ppara(-/-)) cannot sustain the elevated heart function necessary to compensate for sepsis pathophysiology...
November 23, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27880753/notes-from-the-field-community-based-prevention-of-rocky-mountain-spotted-fever-sonora-mexico-2016
#8
Anne Straily, Naomi Drexler, Denica Cruz-Loustaunau, Christopher D Paddock, Gerardo Alvarez-Hernandez
Rocky Mountain spotted fever (RMSF), a life-threatening tickborne zoonosis caused by Rickettsia rickettsii, is a reemerging disease in Mexico (1,2). R. rickettsii is an intracellular bacterium that infects vascular endothelium and can cause multisystem organ failure and death in the absence of timely administration of a tetracycline-class antibiotic, typically doxycycline. Epidemic RMSF, as described in parts of Arizona and Mexico, is associated with massive local infestations of the brown dog tick (Rhiphicephalus sanguineus sensu lato) on domestic dogs and in peridomestic settings that result in high rates of human exposure; for example, during 2003-2012, in Arizona the incidence of RMSF in the three most highly affected communities was 150 times the U...
November 25, 2016: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/27826760/myotonic-dystrophy-type-1-management-and-therapeutics
#9
REVIEW
Cheryl A Smith, Laurie Gutmann
Myotonic dystrophy (DM1) is the most common form of adult muscular dystrophy. It is a multisystem disorder with a complex pathophysiology. Although inheritance is autosomal dominant, disease variability is attributed to anticipation, a maternal expansion bias, variable penetrance, somatic mosaicism, and a multitude of aberrant pre-mRNA splicing events. Patient presentations range from asymptomatic or mild late onset adult to severe congenital forms. Multiple organ systems may be affected. Patients may experience early cataracts, myotonia, muscle weakness/atrophy, fatigue, excessive daytime sleepiness, central/obstructive apnea, respiratory failure, cardiac arrhythmia, insulin resistance, dysphagia, GI dysmotility, cognitive impairment, Cluster C personality traits, and/or mood disorders...
December 2016: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/27795844/hyaluronic-acid-in-vascular-and-immune-homeostasis-during-normal-pregnancy-and-preeclampsia
#10
M M Ziganshina, S V Pavlovich, N V Bovin, G T Sukhikh
Preeclampsia (PE) is a multisystem pathologic state that clinically manifests itself after the 20th week of pregnancy. It is characterized by high maternal and perinatal morbidity and mortality. According to modern concepts, the impairment of trophoblast invasion into maternal spiral arteries, leading to the development of ischemia in placenta, is considered to be the major pathogenetic factor of PE development. Ischemic lesions initiate the development of a systemic inflammatory response (SIR) and endothelial dysfunction, which is the main cause of the multiple organ failure in PE...
July 2016: Acta Naturae
https://www.readbyqxmd.com/read/27737399/acute-tubulointerstitial-nephritis-with-severe-renal-impairment-associated-with-multisystem-igg4-related-disease
#11
Rafael Coimbra Ferreira Beltrame, Maurício Friderichs, Bárbara Rayanne Fior, Pedro Guilherme Schaefer, Gustavo Gomes Thomé, Dirceu Reis da Silva, Elvino José Guardão Barros, Renato Seligman, Francisco Veríssimo Veronese
The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/27660567/using-the-minimally-invasive-impella-5-0-via-the-right-subclavian-artery-cutdown-for-acute-on-chronic-decompensated-heart-failure-as-a-bridge-to-decision
#12
Aditya Bansal, Jay K Bhama, Rajan Patel, Sapna Desai, Stacy A Mandras, Hamang Patel, Tyrone Collins, John P Reilly, Hector O Ventura, P Eugene Parrino
BACKGROUND: Outcomes of traditional mechanical support paradigms (extracorporeal membrane oxygenation, intraaortic balloon pump [IABP], and permanent left ventricular assist device [LVAD]) in acute decompensated heart failure have generally been suboptimal. Novel approaches, such as minimally invasive LVAD therapy (Impella 5.0 device), promise less invasive but equivalent hemodynamic support. However, it is yet unknown whether the outcomes with such devices support widespread acceptance of this new technology...
2016: Ochsner Journal
https://www.readbyqxmd.com/read/27651731/multisystem-failure-and-death-due-to-extensive-hemorrhaging-and-brain-herniation-subsequent-to-a-bite-by-an-unidentified-snake
#13
N D B Ehelepola, S M A N Samaranayake, B M L S Basnayake, C G K Amiyangoda, D M U C B Dhanapala, K L R Kalupahana
BACKGROUND: Snakebites cause considerable morbidity and mortality in tropical and subtropical countries even though existing treatment methods can prevent most deaths if presentation occurs early to hospitals. Envenomation by unidentified snakes is common in central Sri Lanka. Management of such patients is challenging especially if presentation is late. CASE PRESENTATION: Here, we report a case of a 52-year-old man from central Sri Lanka who presented late after being bitten by an unidentified snake...
2016: Tropical Medicine and Health
https://www.readbyqxmd.com/read/27630223/massive-hemorrhage-from-multiple-hepatic-artery-aneurysms
#14
S Lowell Kahn, Jonathan McClain, Jeffrey L Kaufman
A 66-year-old man, with an abnormal porta hepatis, consistent with tumor or inflammation, developed massive bleeding from one of numerous hepatic artery aneurysms, and coil embolization achieved control of bleeding. He died of subsequent multisystem organ failure, and the most likely diagnosis was either polyarteritis nodosa or segmental arterial mediolysis. Although the dual hepatic blood supply allows a degree of arterial embolization, this case demonstrates the risks associated with large territory hepatic arterial embolization in the presence of hemodynamic instability...
October 2016: Vascular and Endovascular Surgery
https://www.readbyqxmd.com/read/27574440/menkes-disease-what-a-multidisciplinary-approach-can-do
#15
REVIEW
Rahul Ojha, Asuri N Prasad
Disorders of copper homeostasis are currently recognized across the life span. Their recognition and links to human disease have spanned several decades, beginning with the recognition of a degenerative disorder in the offspring of sheep grazing in copper-deficient pastures, through to the description of infants suffering from a progressive neurodegenerative disorder characterized by epileptic seizures, developmental regression, failure to thrive, and an unusual hair quality (giving the condition its distinctive label of "kinky hair disease")...
2016: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/27563745/baclofen-in-the-therapeutic-of-sequele-of-traumatic-brain-injury-spasticity
#16
REVIEW
Adán Pérez-Arredondo, Eduardo Cázares-Ramírez, Paul Carrillo-Mora, Marina Martínez-Vargas, Noemí Cárdenas-Rodríguez, Elvia Coballase-Urrutia, Radamés Alemón-Medina, Aristides Sampieri, Luz Navarro, Liliana Carmona-Aparicio
Traumatic brain injury (TBI) is an alteration in brain function, caused by an external force, which may be a hit on the skull, rapid acceleration or deceleration, penetration of an object, or shock waves from an explosion. Traumatic brain injury is a major cause of morbidity and mortality worldwide, with a high prevalence rate in pediatric patients, in which treatment options are still limited, not available at present neuroprotective drugs. Although the therapeutic management of these patients is varied and dependent on the severity of the injury, general techniques of drug types are handled, as well as physical and surgical...
November 2016: Clinical Neuropharmacology
https://www.readbyqxmd.com/read/27500162/cardiac-restricted-expression-of-vcp-ter94-rnai-or-disease-alleles-perturbs-drosophila-heart-structure-and-impairs-function
#17
Meera C Viswanathan, Anna C Blice-Baum, Tzu-Kang Sang, Anthony Cammarato
Valosin-containing protein (VCP) is a highly conserved mechanoenzyme that helps maintain protein homeostasis in all cells and serves specialized functions in distinct cell types. In skeletal muscle, it is critical for myofibrillogenesis and atrophy. However, little is known about VCP's role(s) in the heart. Its functional diversity is determined by differential binding of distinct cofactors/adapters, which is likely disrupted during disease. VCP mutations cause multisystem proteinopathy (MSP), a pleiotropic degenerative disorder that involves inclusion body myopathy...
June 2016: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/27468829/ebola-virus-disease-and-critical-illness
#18
REVIEW
Aleksandra Leligdowicz, William A Fischer, Timothy M Uyeki, Thomas E Fletcher, Neill K J Adhikari, Gina Portella, Francois Lamontagne, Christophe Clement, Shevin T Jacob, Lewis Rubinson, Abel Vanderschuren, Jan Hajek, Srinivas Murthy, Mauricio Ferri, Ian Crozier, Elhadj Ibrahima, Marie-Claire Lamah, John S Schieffelin, David Brett-Major, Daniel G Bausch, Nikki Shindo, Adrienne K Chan, Tim O'Dempsey, Sharmistha Mishra, Michael Jacobs, Stuart Dickson, G Marshall Lyon, Robert A Fowler
As of 20 May 2016 there have been 28,646 cases and 11,323 deaths resulting from the West African Ebola virus disease (EVD) outbreak reported to the World Health Organization. There continue to be sporadic flare-ups of EVD cases in West Africa.EVD presentation is nonspecific and characterized initially by onset of fatigue, myalgias, arthralgias, headache, and fever; this is followed several days later by anorexia, nausea, vomiting, diarrhea, and abdominal pain. Anorexia and gastrointestinal losses lead to dehydration, electrolyte abnormalities, and metabolic acidosis, and, in some patients, acute kidney injury...
July 29, 2016: Critical Care: the Official Journal of the Critical Care Forum
https://www.readbyqxmd.com/read/27461025/the-use-of-dronedarone-for-recurrent-ventricular-tachycardia-a-case-report-and-review-of-the-literature
#19
Jacques Rizkallah, Vikas Kuriachan, L Brent Mitchell
BACKGROUND: Dronedarone is a benzofuran derivative resembling amiodarone that was intended to reduce the iodine-associated tissue deposition and organ toxicity seen with the latter. The utility of dronedarone for patients with ventricular arrhythmias has not been thoroughly evaluated. We present our experience with its use to treat refractory ventricular tachycardia storm and review the literature. CASE PRESENTATION: An 85 year-old gentleman with multiple medical comorbidities including ischemic and non-ischemic cardiomyopathy with severe biventricular systolic dysfunction presented with ventricular tachycardia storm...
July 27, 2016: BMC Research Notes
https://www.readbyqxmd.com/read/27414068/notes-from-the-field-meningococcal-disease-in-an-international-traveler-on-eculizumab-therapy-united-states-2015
#20
Allen O Applegate, Vanessa C Fong, Kara Tardivel, Susan A Lippold, Sheilah Zarate
On June 2, 2015, CDC was notified that a male airline passenger, aged 41 years, with a fever of 105.4°F, headache, nausea, photophobia, diarrhea, and vomiting, which began approximately 3 hours after departure, was arriving to San Francisco, California, on a flight from Frankfurt, Germany. His symptoms reportedly started with neck stiffness 1 day earlier. Upon arrival, the patient was immediately transported to a local hospital, where he was in septic shock, which was followed by multisystem organ failure...
July 15, 2016: MMWR. Morbidity and Mortality Weekly Report
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