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https://www.readbyqxmd.com/read/28425419/a-rare-bsep-mutation-associated-with-a-mild-form-of-progressive-familial-intrahepatic-cholestasis-type-2
#1
Orith Waisbourd-Zinman, Lea F Surrey, Anna E Schwartz, Pierre A Russo, Jessica Wen
Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28424902/physical-and-psychosocial-aspects-of-adolescent-and-young-adults-after-allogeneic-hematopoietic-stem-cell-transplantation-results-from-a-prospective-multicenter-trial
#2
Kristin Pulewka, Daniel Wolff, Philipp Y Herzberg, Hildegard Greinix, Pia Heussner, Friederike H A Mumm, Stephanie von Harsdorf, Kathrin Rieger, Philipp Hemmati, Andreas Hochhaus, Inken Hilgendorf
PURPOSE: Allogeneic hematopoietic stem-cell transplantation (alloHSCT) is physically and psychosocially demanding. Among transplant recipients, adolescent and young adults (AYA) represent a special group, as disease occurs early in life, resulting in the prospect of long survival time and high burden of alloHSCT sequelae. However, data focusing on AYA undergoing alloHSCT are rare. METHODS: Data resulting from a prospective multicenter trial initially focusing on graft-versus-host disease (GvHD) after alloHSCT were reused to analyse the differences between AYA and elderly patients...
April 19, 2017: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/28408026/the-aging-cardiovascular-system-understanding-it-at-the-cellular-and-clinical-levels
#3
REVIEW
Francesco Paneni, Candela Diaz Cañestro, Peter Libby, Thomas F Lüscher, Giovanni G Camici
Cardiovascular disease (CVD) presents a great burden for elderly patients, their caregivers, and health systems. Structural and functional alterations of vessels accumulate throughout life, culminating in increased risk of developing CVD. The growing elderly population worldwide highlights the need to understand how aging promotes CVD in order to develop new strategies to confront this challenge. This review provides examples of some major unresolved clinical problems encountered in daily cardiovascular practice as we care for elderly patients...
April 18, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28402945/a-prospective-open-label-multicenter-observational-study-to-evaluate-the-efficacy-and-safety-of-bortezomib-melphalan-prednisone-as-initial-treatment-for-autologous-stem-cell-transplantation-ineligible-patients-with-multiple-myeloma
#4
Min Kyoung Kim, Kihyun Kim, Chang-Ki Min, Jae-Yong Kwak, Sang-Byung Bae, Sung-Soo Yoon, Je-Jung Lee, Ki Hwan Kim, Seung-Hyun Nam, Yeung-Chul Mun, Hyo Jung Kim, Sung Hwa Bae, Ho-Jin Shin, Jung-Hee Lee, Joon Seong Park, Seong Hyun Jeong, Mark Hong Lee, Yang-Soo Kim, Ho Sup Lee, Keon Woo Park, Won-Sik Lee, Sang Min Lee, Jeong-Ok Lee, Myung Soo Hyun, Deog Yeon Jo, Sung-Nam Lim, Jae Hoon Lee, Do-Yeun Cho, Young Rok Do, Jeong-A Kim, Seong Kyu Park, Jin Seok Kim, Soo-Jeong Kim, Hawk Kim, Hyeon Gyu Yi, Joon Ho Moon, Chul Won Choi, Sung-Hyun Kim, Young-Don Joo, Hoon-Gu Kim, Byung Soo Kim, Moo-Rim Park, Moo-Kon Song, Su-Youn Kim
Bortezomib-melphalan-prednisone (VMP) showed superior efficacy versus MP as first-line treatment for transplantation-ineligible multiple myeloma (MM). This study investigated the efficacy of VMP for Korean patients with MM.Overall, 177 MM patients received 9 cycles of VMP in this prospective, multicenter, observational study. The primary endpoint was 2-year progression-free survival (PFS).Thirty-nine (22%) patients were aged ≥ 75 years and 83 (47.4%) patients had International Staging System stage III. A median of 5 cycles were delivered...
April 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28401745/hypocellular-acute-myeloid-leukemia-treated-with-bone-marrow-transplantation
#5
Dai Keino, Kensuke Kondoh, Ryo Ohyama, Mizuho Morimoto, Tetsuya Mori, Masafumi Ito, Akitoshi Kinoshita
Hypocellular acute myeloid leukemia (AML) mainly occurs in elderly patients, and is extremely rare in childhood. There is still no established treatment for hypocellular AML. We report the case of an 11-year-old boy with hypocellular AML who was treated successfully with allogenic bone marrow transplantation (allo-BMT). He presented with fever, pallor and pancytopenia. Bone marrow aspiration and biopsy confirmed a diagnosis of hypocellular AML. Although low-dose cytarabine induced reduction of blasts, it did not lead to complete remission...
April 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28396733/current-trends-in-european-renal-epidemiology
#6
James Heaf
The incidence of end-stage renal disease (ESRD) continues to vary substantially between the countries in Europe that contribute data to the ERA-EDTA Registry. Differences can be attributed to socioeconomic factors and prophylaxis programs for patients with chronic kidney disease (CKD) and may also express real differences in CKD incidence. Recently, age-adjusted ESRD incidence has begun to fall in many countries, probably related to improved prophylaxis. However, absolute rates may increase, partly due to socioeconomic advances in countries with a low gross domestic product and partly due to continuing increases in the proportion of elderly patients...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28383177/low-socioeconomic-status-adversely-effects-dialysis-survival-in-australia
#7
Rathika Krishnasamy, Nicholas A Gray
BACKGROUND: Low socioeconomic status (SES) is associated with increased incidence of end stage kidney disease and in the USA poorer dialysis survival. All Australians have access to a universal health care system. METHODS: The study included all non-indigenous adult Australians registered with the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) who commenced dialysis between 2003 and 2013. SES at dialysis start was classified into quartiles of advantaged through to disadvantaged using Australian Bureau of Statistics socio-economic indexes for areas...
April 6, 2017: Nephrology
https://www.readbyqxmd.com/read/28379169/intake-of-marine-derived-omega-3-polyunsaturated-fatty-acids-and-mortality-in-renal-transplant-recipients
#8
António W Gomes Neto, Camilo G Sotomayor Campos, Ilse G Pranger, Else van den Berg, Rijk O B Gans, Sabita S Soedamah-Muthu, Gerjan J Navis, Stephan J L Bakker
The effect of marine-derived omega-3 polyunsaturated fatty acids (n-3 PUFA) on long-term outcome in renal transplant recipients (RTR) remains unclear. We investigated whether marine-derived n-3 PUFA intake is associated with all-cause and cardiovascular (CV) mortality in RTR. Intake of eicosapentaenoic acid plus docosahexaenoic acid (EPA-DHA) was assessed using a validated Food Frequency Questionnaire. Cox regression analyses were performed to evaluate the associations of EPA-DHA intake with all-cause and CV mortality...
April 5, 2017: Nutrients
https://www.readbyqxmd.com/read/28377416/spectrum-of-immune-related-conditions-associated-with-risk-of-keratinocyte-cancers-among-elderly-adults-in-the-united-states
#9
Elizabeth L Yanik, Ruth M Pfeiffer, D Michael Freedman, Martin A Weinstock, Elizabeth K Cahoon, Sarah T Arron, Matthew Chaloux, M Kari Connolly, Priyadharsini Nagarajan, Eric A Engels
Elevated keratinocyte carcinoma (KC) risk is present with several immune-related conditions, e.g., solid organ transplantation and non-Hodgkin lymphoma. Because many immune-related conditions are rare, their relationships with KC have not been studied. We used Medicare claims to identify cutaneous squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) cases in 2012, and controls matched on sex and age. All subjects were aged 65-95 years, of white race, and had attended ≥1 dermatologist visit in 2010-2011...
April 4, 2017: Cancer Epidemiology, Biomarkers & Prevention
https://www.readbyqxmd.com/read/28361527/clinical-presentation-of-candidaemia-in-elderly-patients-experience-in-a-single-institution
#10
A Lerma, E Cantero, M Soriano, B Orden, E Muñez, A Ramos-Martinez
OBJECTIVE: To analyse the clinical presentation of candidaemia in elderly patients. METHODS: A comparison of clinical presentation of candidaemia cases was carried out in a Spanish tertiary hospital between January 2010 and September 2015. RESULTS: Forty-five cases (32%) corresponded to elderly patients (≥ 75 years) and 95 cases (68%) to non-elderly patients (16-74 years). A higher proportion of elderly patients presented solid tumour (51% versus 32%, p=0...
March 31, 2017: Revista Española de Quimioterapia: Publicación Oficial de la Sociedad Española de Quimioterapia
https://www.readbyqxmd.com/read/28340875/angioimmunoblastic-t-cell-lymphoma
#11
REVIEW
Alessandro Broccoli, Pier Luigi Zinzani
Angioimmunoblastic T-cell lymphoma is a follicular T-helper-derived neoplasm displaying a peculiar morphologic appearance and biological complexity. New mutations have been described that contribute to elucidating the underlying pathogenetic events. The disease behaves aggressively and typically affects elderly patients. The outcomes reported with anthracycline-containing regimens are poor; therefore autologous transplantation in first remission should be offered whenever possible. Newer approaches are urgently needed for relapsed and refractory patients...
April 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28331803/ivig-a-cure-to-severe-refractory-nap-1-clostridium-difficile-colitis-a-case-of-successful-treatment-of-severe-infection-which-failed-standard-therapy-including-fecal-microbiota-transplants-and-fidaxomicin
#12
Kelley Coffman, Xian Jie Cindy Chen, Charles Okamura, Eddie Louie
The mainstay treatment of Clostridium difficile infections (CDI) is antimicrobials with growing support for fecal microbiota transplants. We report the first case of an elderly man with severe refractory NAP-1 pseudomembranous CDI who failed all medical therapy and two fecal transplants with response only seen after administration of intravenous immunoglobulin.
2017: IDCases
https://www.readbyqxmd.com/read/28331061/an-intracellular-matrix-metalloproteinase-2-isoform-induces-tubular-regulated-necrosis-implications-for-acute-kidney-injury
#13
Carla S Ceron, Celine Baligand, Sunil K Joshi, Shaynah Wanga, Patrick M Cowley, Joy P Walker, Sang Heon Song, Rajeev Mahimkar, Anthony J Baker, Robert L Raffai, Zhen J Wang, David H Lovett
Acute kidney injury (AKI) causes severe morbidity, mortality, and chronic kidney disease (CKD). Mortality is particularly marked in the elderly and with pre-existing CKD. Oxidative stress is a common theme in models of AKI induced by ischemia/reperfusion (I/R) injury. We recently characterized an intracellular isoform of matrix metalloproteinase-2 (MMP-2) induced by oxidative stress-mediated activation of an alternate promoter in the first intron of the MMP-2 gene. This generates an N-terminal truncated MMP-2 isoform (NTT-MMP-2) that is intracellular and associated with mitochondria...
March 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28329396/heart-transplantation-in-selected-patients-aged-60-years-and-older-a-two-decade-retrospective-and-multicentre-analysis
#14
Christian Bosseau, Bernard Lelong, Sabine Pattier, Jean-Noël Trochu, Jean-Christian Roussel, Agnès Sirinelli, Michel Aupart, Céline Chabanne, Richard Dorent, Christelle Cantrelle, Philippe Mabo, Christophe Leclercq, Jean-Philippe Verhoye, Erwan Flécher
OBJECTIVES: This study analysed survival and long-term outcomes of heart transplantation in patients aged 60 years and older. We also analysed the impact of a national graft allocation priority [Super Emergency (SE)] and compared survival with younger patients in our centres and in France. METHODS: We performed a multicentre (University Hospitals in Nantes, Rennes and Tours), 2-decade retrospective study between 1 January 1994 and 31 December 2013. Elderly recipients were placed on the same list as younger patients; the use of marginal donors remained occasional...
May 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28324640/cytogenetics-and-comorbidity-predict-outcomes-in-older-myelodysplastic-syndrome-patients-after-allogenic-stem-cell-transplantation-using-reduced-intensity-conditioning
#15
Orhan Kemal Yucel, Rima M Saliba, Gabriella Rondon, Sairah Ahmed, Amin Alousi, Qaiser Bashir, Stefan O Ciurea, Uday Popat, Isa Khouri, David Marin, Katy Rezvani, Partow Kebriaei, Elizabeth J Shpall, Richard E Champlin, Betül Oran
BACKGROUND: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only treatment with a curative potential for myelodysplastic syndrome (MDS) patients. Allo-HSCT has substantial risks, particularly in the elderly, and its role for older MDS patients has yet to be defined. METHODS: We analyzed 88 MDS patients aged ≥ 60 years with allo-HSCT after reduced intensity conditioning regimens over the last decade. The study cohort had high risk features; 47 of 88 (53...
March 21, 2017: Cancer
https://www.readbyqxmd.com/read/28324233/diagnosis-and-treatment-of-kaposi-sarcoma
#16
REVIEW
Johann W Schneider, Dirk P Dittmer
Kaposi sarcoma (KS) is the most common neoplasm of people living with HIV today. In Sub-Saharan Africa, KS is among the most common cancers in men, overall. Not only HIV-positive individuals present with KS; any immune compromised person infected with KS-associated herpesvirus (KSHV) or human herpesvirus 8 is at risk: the elderly, children in KSHV-endemic areas, and transplant recipients. KS diagnosis is based on detection of the viral protein latency-associated nuclear antigen (LANA) in the biopsy, but not all cases of KS are the same or will respond to the same therapy...
March 21, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28320767/comparing-outcomes-between-antibody-induction-therapies-in-kidney-transplantation
#17
Neel Koyawala, Jeffrey H Silber, Paul R Rosenbaum, Wei Wang, Alexander S Hill, Joseph G Reiter, Bijan A Niknam, Orit Even-Shoshan, Roy D Bloom, Deirdre Sawinski, Susanna Nazarian, Jennifer Trofe-Clark, Mary Ann Lim, Jesse D Schold, Peter P Reese
Kidney transplant recipients often receive antibody induction. Previous studies of induction therapy were often limited by short follow-up and/or absence of information about complications. After linking Organ Procurement and Transplantation Network data with Medicare claims, we compared outcomes between three induction therapies for kidney recipients. Using novel matching techniques developed on the basis of 15 clinical and demographic characteristics, we generated 1:1 pairs of alemtuzumab-rabbit antithymocyte globulin (rATG) (5330 pairs) and basiliximab-rATG (9378 pairs) recipients...
March 20, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28319943/femoral-neck-x-ray-absorptiometry-parameters-and-peripheral-quantitative-computer-tomography-tibial-cortical-density-predict-survival-in-dialysis-patients
#18
Natalie Yap, Phillip Wong, Stella McGinn, Maria-Liza Nery, Jean Doyle, Lynda Wells, Phillip Clifton-Bligh, Roderick J Clifton-Bligh
BACKGROUND: Low bone mineral density (BMD) is a known independent predictor of mortality in the general elderly population. However, studies in patients with end-stage renal disease (ESRD) are limited. The present study evaluated mortality during long-term follow-up in a population of patients having dialysis for ESRD, in whom BMD was also measured. METHODS: Fifty-eight patients with ESRD were recruited consecutively from a dialysis clinic and followed prospectively for 6 years...
March 21, 2017: Nephron
https://www.readbyqxmd.com/read/28293918/the-elderly-liver-transplant-recipients-anxiety-depression-fatigue-and-life-satisfaction
#19
Felix Krenzien, Nicco Krezdorn, Mehmet H Morgül, Georg Wiltberger, Georgi Atanasov, Hans-Michael Hau, Andreas Hinz, Moritz Schmelzle, Michael Bartels, Christian Benzing
Introduction Older patients are neglected in clinical trials and are likely to be excluded from liver transplantation (LT). The aim of this study was to assess fatigue, anxiety, depression, and health-related quality of life (HRQoL) in elderly LT recipients. Methods Questionnaires were mailed to patients who underwent LT between 1993 and 2013. Three groups were created: patients ≥ 70, 60 - 69, and < 60 years old. Mental-health status, life satisfaction, and fatigue were assessed using, respectively, the Hospital Anxiety and Depression Scale (HADS), Questions on Life Satisfaction (FLZ-M), and the Multidimensional Fatigue Inventory (MFI-20)...
March 14, 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/28286198/in-hospital-mortality-and-post-transplantation-complications-in-elderly-multiple-myeloma-patients-undergoing-autologous-hematopoietic-stem-cell-transplantation-a-population-based-study
#20
Larysa Sanchez, Michael Sylvester, Ricardo Parrondo, Veronica Mariotti, Jean Anderson Eloy, Victor T Chang
Autologous hematopoietic stem cell transplantation (auto-HSCT) has improved survival in patients with multiple myeloma (MM) and is increasingly used in elderly patients. The aim of this study was to characterize and compare in-hospital complications and mortality after auto-HSCT in younger (< age 65) versus elderly (> age 65) MM patients utilizing the Nationwide Inpatient Sample. Over a 3-year period (2008 to 2010), 2209 patients with MM were admitted to US hospitals for auto-HSCT. The median age was 59 years, with 1650 patients (74...
March 9, 2017: Biology of Blood and Marrow Transplantation
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