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Multisystem organ failure

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https://www.readbyqxmd.com/read/29344295/peptide-receptor-radionuclide-therapy-as-a-new-tool-in-treatment-refractory-sarcoidosis-initial-experience-in-two-patients
#1
Constantin Lapa, Malte Kircher, Heribert Hänscheid, Andreas Schirbel, Götz Ulrich Grigoleit, Erdwine Klinker, Markus Böck, Samuel Samnick, Theo Pelzer, Andreas K Buck
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that can involve virtually all organ systems. Whereas most patients present without symptoms, progressive and disabling organ failure can occur in up to 10% of subjects. Somatostatin receptor (SSTR)-directed peptide receptor radionuclide therapy (PRRT) has recently received market authorization for treatment of SSTR-positive neuroendocrine tumors. Methods: We describe the first case series comprising two patients with refractory multi-organ involvement of sarcoidosis who received 4 cycles of PRRT...
2018: Theranostics
https://www.readbyqxmd.com/read/29296722/proliferation-through-activation-hemophagocytic-lymphohistiocytosis-in-hematologic-malignancy
#2
REVIEW
Eric J Vick, Kruti Patel, Philippe Prouet, Mike G Martin
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes...
May 9, 2017: Blood Advances
https://www.readbyqxmd.com/read/29290244/surgical-transfer-decision-making-how-regional-resources-are-allocated-in-a-regional-transfer-network
#3
Kristy Kummerow Broman, Michael J Ward, Benjamin K Poulose, Margaret L Schwarze
BACKGROUND: Tertiary care centers often operate above capacity, limiting access to emergency surgical care for patients at nontertiary facilities. For nontraumatic surgical emergencies there are no guidelines to inform patient selection for transfer to another facility. Such decisions may be particularly difficult for gravely ill patients when the benefits of transfer are uncertain. METHODS: To characterize surgeons' decision-making strategies for transfer, a qualitative analysis of semistructured interviews was conducted with 16 general surgeons who refer and accept patients within a regional transfer network...
January 2018: Joint Commission Journal on Quality and Patient Safety
https://www.readbyqxmd.com/read/29245301/copper-associated-hepatitis-in-a-patient-with-chronic-myeloid-leukemia-following-hematopoietic-stem-cell-transplantation-a-case-report
#4
Ching-Fen Lee, Chi-Hua Chen, Yu-Chuan Wen, Tsung-Yen Chang, Ming-Wei Lai, Tang-Her Jaing
RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29240626/first-report-of-biventricular-percutaneous-impella-ventricular-assist-device-use-in-pediatric-patients
#5
Javier J Lasa, Daniel A Castellanos, Susan W Denfield, William J Dreyer, Sebastian C Tume, Henri Justino, Athar M Qureshi
There is a growing interest in the use of percutaneously delivered ventricular assist devices (PVAD) in the pediatric patient population. A 16 year old female and 18 year old male both status post heart transplantation presented with evidence of graft rejection and acute severe global systolic and diastolic heart failure necessitating hemodynamic catheterization and inotropic support. Both underwent percutaneous Impella CP LVAD (Abiomed, Danvers, MA) placement with close monitoring of right ventricular function...
December 11, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29237671/painless-aortic-dissection-presenting-with-multisystem-organ-failure-and-owl-s-eyes-sign-of-the-spinal-cord
#6
Raymond Lam, Sara L Swenson, Thomas J Nuckton, Merrill Nisam
No abstract text is available yet for this article.
December 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29200153/dengue-associated-hemophagocytic-lymphohistiocytosis-a-rare-complication-of-a-common-infection-in-singapore
#7
Kai-Qian Kam, Shui Yen Soh, Rajat Bhattacharyya
Hemophagocytic lymphohistiocytosis (HLH) can progress rapidly, often leading to multisystem organ failure and death. Prompt recognition of the syndrome and institution of appropriate treatment are crucial steps in improving the outcome. Dengue virus infection is not commonly known to be associated with secondary HLH. We present a case of a child with dengue fever who subsequently developed classical features of HLH. He was treated successfully with 4 weeks of steroid monotherapy instead of the multidrug therapy proposed in the HLH 2004 protocol...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29194478/male-neonate-with-legionellosis
#8
Adam M Szewc, Steve Taylor, Gary D Cage, Daphne de Mello
Legionellaceae are composed of a single genus, Legionella, which is currently comprised of over 52 species. L. pneumophila, L. micdadei, L. longbeachae, and L. dumoffi are clinically considered the most important, with L. pneumophila causing >90%1,7 of cases of legionnaires disease (LD). Since LD's recognition in 1976 following an outbreak of pneumonia involving delegates of an American Legion convention in Philadelphia, there has been increased awareness and importance of the isolation, detection, and reporting of these cases...
November 28, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29181841/epidural-therapy-for-the-treatment-of-severe-pre-eclampsia-in-non-labouring-women
#9
REVIEW
Amita Ray, Sujoy Ray
BACKGROUND: Pre-eclampsia is a pregnancy-specific multi-organ disorder, which is characterised by hypertension and multisystem organ involvement and which has significant maternal and fetal morbidity and mortality. Failure of the placental vascular remodelling and reduced uteroplacental flow form the etiopathological basis of pre-eclampsia. There are several established therapies for pre-eclampsia including antihypertensives and anticonvulsants. Most of these therapies aim at controlling the blood pressure or preventing complications of elevated blood pressure, or both...
November 28, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29159459/epg5-related-vici-syndrome-a-primary-defect-of-autophagic-regulation-with-an-emerging-phenotype-overlapping-with-mitochondrial-disorders
#10
Shanti Balasubramaniam, Lisa G Riley, Anand Vasudevan, Mark J Cowley, Velimir Gayevskiy, Carolyn M Sue, Caitlin Edwards, Edward Edkins, Reimar Junckerstorff, C Kiraly-Borri, P Rowe, J Christodoulou
Vici syndrome is a rare, under-recognised, relentlessly progressive congenital multisystem disorder characterised by five principal features of callosal agenesis, cataracts, cardiomyopathy, combined immunodeficiency and oculocutaneous hypopigmentation. In addition, three equally consistent features (profound developmental delay, progressive failure to thrive and acquired microcephaly) are highly supportive of the diagnosis. Since its recognition as a distinct entity in 1988, an extended phenotype with sensorineural hearing loss, skeletal myopathy and variable involvement of virtually any organ system, including the lungs, thyroid, liver and kidneys, have been described...
November 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29129424/laparoscopic-ligation-of-cisterna-chyli-for-refractory-chylothorax-a-case-series-and-review-of-the-literature
#11
Ilitch Diaz-Gutierrez, Madhuri Vasudev Rao, Rafael Santiago Andrade
OBJECTIVES: We describe an alternative surgical technique for the treatment of chylothorax in patients who have had failure of or are not candidates for transthoracic ligation or embolization by interventional radiology. METHODS: We describe our experience with laparoscopic ligation of the cisterna chyli in 3 such patients and compare our results with published literature. We used a 5-port approach as for foregut surgery. We retracted the liver, transected the gastrohepatic ligament, and retracted the stomach to the left...
September 30, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29115094/the-first-successful-lung-transplantation-in-a-korean-child-with-cystic-fibrosis
#12
Soo Ran Noh, Eun Lee, Jisun Yoon, Sungsu Jung, Song I Yang, Jinho Yu, Soo Jong Hong
Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the case of a 12-year-old girl with respiratory failure due to CF who underwent lung transplantation. She had been diagnosed with CF 8 years previously after being treated for recurrent Pseudomonas aeruginosa pneumonia and malnutrition based on sweat chloride concentrations and the CFTR protein gene mutation test...
December 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29098097/early-renal-involvement-in-a-girl-with-classic-fabry-disease
#13
Fernando Perretta, Norberto Antongiovanni, Sebastián Jaurretche
Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency or absence of the enzyme alpha galactosidase A; this defect leads to the systemic accumulation of globotriaosylceramide and its metabolites. Organic involvement in men is well known, but in women it is controversial, mainly due to the random X-chromosome inactivation in each of their cells (Lyon hypothesis). This would explain why women (heterozygotes) present a wide variability in the severity of their phenotype. The manifestations are multisystemic and begin in early childhood, reaching a severe compromise in adulthood...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29095423/association-of-statins-with-decreased-acute-pancreatitis-severity-a-propensity-score-analysis
#14
Peter J Lee, Kunjam Modha, Tiffany Chua, Amitabh Chak, Daniel Jang, Rocio Lopez, Amir Gougol, Georgios I Papachristou, Tyler Stevens
BACKGROUND: Statins possess anti-inflammatory properties and have a protective effect in certain inflammatory conditions; however, their effect on the natural history of pancreatitis is unknown. AIM: The aim of this study is to assess the effect of statin exposure on the severity of pancreatitis and incidence of organ failure using a propensity-matched approach. METHODS: A historical cohort study was conducted of adult patients with acute pancreatitis (AP) admitted in the Cleveland Clinic Health System between 2007 and 2014...
October 31, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28975999/combining-multiple-biomarkers-differentiates-between-active-sjia-sjia-mas-and-ebv-hlh
#15
L K Weaver
Cytokine storm syndromes are a clinically heterogeneous group of conditions resulting from a maladaptive host response to an inflammatory trigger. These syndromes lead to rapid progression of immune-mediated damage to healthy tissues resulting in life-threatening multi-system organ failure. Prompt recognition of disease and medical intervention to limit damage to healthy tissues is essential to prevent cytokine storm morbidity and mortality. However, the diagnosis of cytokine storm syndromes is challenging, given the clinical heterogeneity in disease presentations...
October 4, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28937083/infantile-cystinosis-from-dialysis-to-renal-transplantation
#16
Manel Jellouli, Meriem Ferjani, Kamel Abidi, Chokri Zarrouk, Jaouida Abdelmoula, Tahar Gargah
Cystinosis is an autosomal recessive, lysosomal storage disease characterised by the accumulation of the amino acid cystine in different organs and tissues. It is a multisystemic disease that can present with renal and extra-renal manifestations. In this report, we present the first case of transplanted nephropathic cystinosis in a Tunisian child. A 4-year-old Tunisian boy born to nonconsanguineous parents, was treated in our medical services in 1990 for cystinosis. Since the age of five months, he developed symptoms of severe weight loss, vomiting, dehydration, and polyuria...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28923988/contemporary-management-of-cardiogenic-shock-a-scientific-statement-from-the-american-heart-association
#17
REVIEW
Sean van Diepen, Jason N Katz, Nancy M Albert, Timothy D Henry, Alice K Jacobs, Navin K Kapur, Ahmet Kilic, Venu Menon, E Magnus Ohman, Nancy K Sweitzer, Holger Thiele, Jeffrey B Washam, Mauricio G Cohen
Cardiogenic shock is a high-acuity, potentially complex, and hemodynamically diverse state of end-organ hypoperfusion that is frequently associated with multisystem organ failure. Despite improving survival in recent years, patient morbidity and mortality remain high, and there are few evidence-based therapeutic interventions known to clearly improve patient outcomes. This scientific statement on cardiogenic shock summarizes the epidemiology, pathophysiology, causes, and outcomes of cardiogenic shock; reviews contemporary best medical, surgical, mechanical circulatory support, and palliative care practices; advocates for the development of regionalized systems of care; and outlines future research priorities...
October 17, 2017: Circulation
https://www.readbyqxmd.com/read/28923819/geriatric-anorexia-nervosa
#18
Moaweya Zayed, Joseph P Garry
Eating disorders are not commonly diagnosed in individuals aged >50 years, yet they are associated with significant psychiatric comorbidities and overall morbidity. Anorexia nervosa is the most common eating disorder among this age group, and women are affected most often. We present the fatal case of a 66-year old woman with severe malnutrition and newly diagnosed anorexia nervosa. Inpatient refeeding was unsuccessful, and she succumbed to multisystem organ failure. The timely recognition of eating disorders among older people is important for family physicians who care for patients across the life spectrum...
September 2017: Journal of the American Board of Family Medicine: JABFM
https://www.readbyqxmd.com/read/28905470/outcomes-of-berlin-heart-excor-%C3%A2-pediatric-ventricular-assist-device-support-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#19
Jennifer A Su, Jondavid Menteer
The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR(®) ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR(®) were identified...
September 14, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#20
Rabih Tabet, Julie Zaidan, Boutros Karam, Samer Saouma, Foad Ghavami
Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient...
June 29, 2017: Curēus
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