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https://www.readbyqxmd.com/read/28801223/limited-benefits-of-presymptomatic-cord-blood-transplantation-in-neurovisceral-acid-sphingomyelinase-deficiency-asmd-intermediate-type
#1
Oriane Mercati, Samia Pichard, Marie Ouachée, Roseline Froissart, Odile Fenneteau, Bastien Roche, Monique Elmaleh-Bergès, Yves Bertrand, Hélène Ogier de Baulny, Marie T Vanier, Manuel Schiff
Acid sphingomyelinase (ASM) deficient Niemann-Pick disease is a lysosomal storage disorder resulting from mutations in the SMPD1 gene. The clinical spectrum distinguishes a severe infantile neurological form (type A), a non-neurological visceral form (type B) and a rare intermediate neurovisceral form. We report the first case of presymptomatic cord blood transplantation in a child with the intermediate type of ASM deficiency due to a homozygous Tyr369Cys mutation, whose affected elder brother had developed neurodevelopmental delay from 19 months of age, and had died from severe visceral complications at the age of 3...
July 29, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28801180/obesity-as-predictor-of-postoperative-outcomes-in-liver-transplant-candidates-review-of-the-literature-and-future-perspectives
#2
REVIEW
Michele Barone, Maria Teresa Viggiani, Alfonso W Avolio, Andrea Iannone, Maria Rendina, Alfredo Di Leo
BACKGROUND: Current American and European guidelines consider a pre-transplant BMI ≥40kg/m(2) as a relative contraindication for liver transplantation but this recommendation is graded as uncertain and requires further research. Moreover, conflicting results are reported on the predictive value of BMI 30-39.9kg/m(2) on post-transplant complication and mortality risk. AIM: This study analyzed the data of the literature on the effect of all three BMI classes of obesity on postoperative outcomes in liver transplantation...
July 22, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28780238/tcr%C3%AE-%C3%AE-and-cd19-cell-depleted-haploidentical-and-mismatched-hematopoietic-stem-cell-transplantation-in-primary-immune-deficiency
#3
Ravi M Shah, Reem Elfeky, Zohreh Nademi, Waseem Qasim, Persis Amrolia, Robert Chiesa, Kanchan Rao, Giovanna Lucchini, Juliana M F Silva, Austen Worth, Dawn Barge, David Ryan, Jane Conn, Andrew J Cant, Roderick Skinner, Intan Juliana Abd Hamid, Terence Flood, Mario Abinun, Sophie Hambleton, Andrew R Gennery, Paul Veys, Mary Slatter
BACKGROUND: Allogeneic hematopoietic stem cell transplantation (HSCT) is used as a therapeutic approach for primary immunodeficiencies (PIDs). The best outcomes have been achieved using HLA-matched donors, but when a matched donor is not available, a haploidentical or mismatched unrelated donor (mMUD) can be useful. Various strategies are used to mitigate the risk of graft versus host disease (GvHD) and rejection associated with such transplants. OBJECTIVE: We sought to evaluate the outcomes of haploidentical or mMUD HSCT after depleting GvHD causing TCRαβCD3+ cells from the graft...
August 2, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28760942/perivascular-adipose-tissue-angiotensin-ii-type-1-receptor-promotes-vascular-inflammation-and-aneurysm-formation
#4
Tomoki Sakaue, Jun Suzuki, Mika Hamaguchi, Chika Suehiro, Akiko Tanino, Tomoaki Nagao, Teruyoshi Uetani, Jun Aono, Hirotomo Nakaoka, Mie Kurata, Tomohisa Sakaue, Takafumi Okura, Takumi Yasugi, Hironori Izutani, Jitsuo Higaki, Shuntaro Ikeda
Perivascular adipose tissue exhibits characteristics of active local inflammation, which contributes to the development of atherosclerotic disease as a complication of obesity/metabolic syndrome. However, the precise role of perivascular adipose tissue in the progression of abdominal aortic aneurysm remains unclear. To test the hypothesis that genetic deletion of angiotensin II type 1a (AT1a) receptor in perivascular visceral adipose tissue (VAT) can attenuate aortic aneurysm formation in apolipoprotein E-deficient (ApoE(-/-)) mice, we performed adipose tissue transplantation experiments by using an angiotensin II-induced aneurysm murine model, in which we transplanted VAT from ApoE(-/-) or ApoE(-/-) AT1a(-/-) donor mice onto the abdominal aorta of ApoE(-/-) recipient mice...
July 31, 2017: Hypertension
https://www.readbyqxmd.com/read/28755968/surgical-and-medical-approach-to-patients-requiring-total-small-bowel-resection-managing-the-no-gut-syndrome
#5
Ruy J Cruz, Laurie Butera, Kristine Poloyac, Jenee McGurgan, William Stein, David Binion, Abhinav Humar
BACKGROUND: Total resection of the jejunum and ileum, a rarely performed procedure, is indicated after mesenteric vascular events, trauma, or resection of abdominal neoplasms. We describe our recent experience with the operative and medical management of patients with "no gut syndrome." METHODS: We retrospectively reviewed 341 adult patients who were referred to our center between January 2013 and December 2016. RESULTS: Thirteen patients with a mean age of 42...
July 26, 2017: Surgery
https://www.readbyqxmd.com/read/28723751/early-laparotomy-and-timely-reconstruction-for-patients-with-abdominal-electrical-injury-five-case-reports-and-literature-review
#6
REVIEW
Pi-Hong Zhang, Zan Liu, Li-Cheng Ren, Ji-Zhang Zeng, Geng-Wen Huang, Mu-Zhang Xiao, Jie Zhou, Peng-Fei Liang, Ming-Hua Zhang, Xiao-Yuan Huang
INTRODUCTION: High-tension electricity can cause devastating injuries that may result in abdominal wall loss, visceral damage, and sometimes major threat to life. The visceral organ may be exposed after debridement and require flap cover, but the tensile strength of abdominal wall may be lack even if flap transplanted. METHODS: From April 2007 through May 2015, 5 patients with severe abdominal electrical injury were treated at our hospital. Exploratory laparotomy was performed based on their clinical manifestations and debridement findings of abdominal wall at early stage, and decision regarding technique for reconstruction of abdominal wall was based on an assessment of the location and extent of the defect...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28666508/disseminated-kaposi-s-sarcoma-in-an-immunosuppressed-patient-after-long-term-inhaled-and-systemic-steroid-therapy
#7
Shiyam Kumar, Ikram A Burney, P A M Saparamadu, Chandu de Silva
Kaposi's sarcoma is associated with immunosuppressant treatment administered to patients with organ transplant and other immune related disorders. Several case reports have reported association of Kaposi's sarcoma and steroids. Almost all patients in those case reports had localised disease to skin and they responded to tapering doses or discontinuation of steroids. Here we present a case of an elderly asthmatic man who was using self-medication with systemic and inhaled steroids for about two decades. He presented with widespread skin lesions and anemia...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28665532/characterization-of-body-composition-and-definition-of-sarcopenia-in-patients-with-alcoholic-liver-cirrhosis-a-computed-tomography-based-study
#8
Jaya Benjamin, Varsha Shasthry, Chetan Ramesh Kalal, Lovkesh Anand, Ankit Bhardwaj, Vanshja Pandit, Ankur Arora, Sasidharan Rajesh, Viniyendra Pamecha, Vikas Jain, Guresh Kumar, Anthony Lauria, Puneet Puri, Yogendra Kumar Joshi, Shiv Kumar Sarin
Alterations in body composition (BC) as loss of fat and muscle mass (sarcopenia) are associated with poor outcome in alcoholic liver cirrhosis (ALC). Prevalence of sarcopenia depends upon the method of assessment. Computed Tomography (CT) is a gold standard tool for assessing BC AIM: To characterize BC and define sarcopenia in ALC patients using CT METHODS: Single slice CT images at L3 vertebrae of healthy controls (HC) - organ transplant donors and ALC patients were analysed to give cross-sectional area of five skeletal muscles normalized for height -skeletal muscle index (SMI; cm(2) /m(2) ), area of subcutaneous (SAT;cm(2) ) and visceral adipose tissue (VAT;cm(2) )...
June 30, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28660464/respiratory-tract-infection-caused-by-fonsecaea-monophora-after-kidney-transplantation
#9
Isabella Barbosa Cleinman, Sarah Santos Gonçalves, Marcio Nucci, Danielle Carvalho Quintella, Márcia Halpern, Tiyomi Akiti, Glória Barreiros, Arnaldo Lopes Colombo, Guilherme Santoro-Lopes
Fonsecaea spp. are melanized fungi which cause most cases of chromoblastomycosis. The taxonomy of this genus has been revised, now encompassing four species, with different pathogenic potential: F. pedrosoi, F. nubica, F. pugnacius, and F. monophora. The latter two species present wider clinical spectrum and have been associated with cases of visceral infection, most often affecting the brain. To our knowledge, this is the first report of proven case of F. monophora respiratory tract infection. A Brazilian 57-year-old-female patient underwent kidney transplantation on January 12, 2013...
June 28, 2017: Mycopathologia
https://www.readbyqxmd.com/read/28647177/infectious-diseases-in-solid-organ-transplant-recipients-analysis-of-autopsied-cases-in-japan
#10
Yuhko Suzuki, Akira Kenjo, Tomiteru Togano, Natsuo Yamamoto, Hitoshi Ohto, Hikaru Kume
BACKGROUND: With the improvements in immunosuppressive agents and graft survival, infections such as mycoses have become major complications after solid organ transplantation (SOT). METHODS: Our group has continuously updated an epidemiological database of visceral mycoses (VM) among autopsy cases in Japan since 1989. Data on infectious agents and clinical information were complied using similar procedures. RESULTS: Among the all autopsied cases studied, 356 undergone SOT...
August 2017: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/28640481/using-analytic-morphomics-to-describe-body-composition-associated-with-post-kidney-transplantation-diabetes-mellitus
#11
David C Cron, Kelly A Noon, Devan R Cote, Michael N Terjimanian, Joshua J Augustine, Stewart C Wang, Michael J Englesbe, Kenneth J Woodside
BACKGROUND: Better risk assessment tools are needed to predict post-transplantation diabetes mellitus (PTDM). Using analytic morphomic measurements from computed tomography (CT) scans, we aimed to identify specific measures of body composition associated with PTDM. METHODS: We retrospectively reviewed 99 non-diabetic kidney transplant recipients who received pre-transplant CT scans at a single institution between 1/2005 and 5/2014. Analytic morphomic techniques were used to measure abdominal adiposity, abdominal size, and psoas muscle area and density, standardized by gender...
June 22, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28628447/environmental-factors-as-key-determinants-for-visceral-leishmaniasis-in-solid-organ-transplant-recipients-madrid-spain
#12
Nerea Carrasco-Antón, Francisco López-Medrano, Mario Fernández-Ruiz, Eugenia Carrillo, Javier Moreno, Ana García-Reyne, Ana Pérez-Ayala, María Luisa Rodríguez-Ferrero, Carlos Lumbreras, Rafael San-Juan, Jorge Alvar, José María Aguado
During a visceral leishmaniasis outbreak in an area of Madrid, Spain, the incidence of disease among solid organ transplant recipients was 10.3% (7/68). Being a black person from sub-Saharan Africa, undergoing transplantation during the outbreak, and residing <1,000 m from the epidemic focus were risk factors for posttransplant visceral leishmaniasis.
July 2017: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/28624575/intestinal-fungal-dysbiosis-associates-with-visceral-hypersensitivity-in-patients-with-irritable-bowel-syndrome-and-rats
#13
Sara Botschuijver, Guus Roeselers, Evgeni Levin, Daisy M Jonkers, Olaf Welting, Sigrid Em Heinsbroek, Heleen H de Weerd, Teun Boekhout, Matteo Fornai, Ad A Masclee, Frank H J Schuren, Wouter J de Jonge, Jurgen Seppen, Rene M van den Wijngaard
BACKGROUND & AIMS: Visceral hypersensitivity is one feature of irritable bowel syndrome (IBS). Bacterial dysbiosis might be involved in activation of nociceptive sensory pathways, but there have been few studies of the role of the mycobiome (the fungal microbiome) in development of IBS. We analyzed intestinal mycobiomes of patients with IBS and a rat model of visceral hypersensitivity. METHODS: We used internal transcribed spacer 1-based metabarcoding to compare fecal mycobiomes of 18 healthy volunteers with those of 39 patients with IBS (with visceral hypersensitivity or normal levels of sensitivity)...
June 14, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28604513/severe-sarcopenia-and-increased-fat-stores-in-pediatric-patients-with-liver-kidney-or-intestine-failure
#14
Richard S Mangus, Weston J Bush, Chandrashekhar A Kubal, Christina Miller
OBJECTIVES: Malnutrition and wasting predict clinical outcomes in children with severe chronic illness. Objectively calculated malnutrition in children with end-stage organ failure has not been well studied. This analysis compares children with kidney, liver or intestine failure to healthy controls to quantitate the disparity in muscle and fat stores. METHODS: Children younger than age 19 with end stage liver, kidney or intestine failure and with pre-transplant computed tomography (CT) imaging were selected from the transplant database...
June 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28560469/gene-therapy-for-lysosomal-storage-disorders-recent-advances-for-metachromatic-leukodystrophy-and-mucopolysaccaridosis-i
#15
REVIEW
Rachele Penati, Francesca Fumagalli, Valeria Calbi, Maria Ester Bernardo, Alessandro Aiuti
Lysosomal storage diseases (LSDs) are rare inherited metabolic disorders characterized by a dysfunction in lysosomes, leading to waste material accumulation and severe organ damage. Enzyme replacement therapy (ERT) and haematopoietic stem cell transplant (HSCT) have been exploited as potential treatments for LSDs but pre-clinical and clinical studies have shown in some cases limited efficacy. Intravenous ERT is able to control the damage of visceral organs but cannot prevent nervous impairment. Depending on the disease type, HSCT has important limitations when performed for early variants, unless treatment occurs before disease onset...
July 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28544183/clinical-outcomes-of-intestinal-transplant-recipients-colonized-with-multidrug-resistant-organisms-a-retrospective-study
#16
Jacques Simkins, Michele I Morris, Jose F Camargo, Rodrigo Vianna, Thiago Beduschi, Lilian M Abbo
Rates of multidrug-resistant organisms (MDRO) colonization among intestinal transplant (ITx) recipients have not been reported. Colonization rates with vancomycin-resistant Enterococcus (VRE), carbapenem-resistant Gram-negative bacteria (CR-GNB), and methicillin-resistant Staphylococcus aureus (MRSA) were obtained retrospectively in adults undergoing ITx (isolated or multivisceral) from 1/2009 to 12/2015. We assessed for VRE, CR-GNB, and MRSA bacteremia during the first year post-transplant for patients colonized with VRE, CR-GNB, and MRSA, respectively, and for those who were not colonized...
September 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28538578/total-abdominal-wall-transplantation-an-anatomical-study-and-classification-system
#17
David Light, Neilendu Kundu, Risal Djohan, Cristiano Quintini, Namita Gandhi, Brian R Gastman, Richard Drake, Maria Siemionow, James E Zins
BACKGROUND: Candidates for multivisceral transplantation present with complex defects often beyond traditional reconstructive options. In this study, the authors describe a dissection technique for a total abdominal wall vascularized composite flap. In addition, the authors suggest a classification system for complex abdominal wall defects. METHODS: Forty fresh, cadaveric hemiabdomens were dissected, with care taken to preserve the iliofemoral, deep circumflex iliac, superficial circumflex iliac, deep inferior epigastric, and superficial inferior epigastric arteries and corresponding veins...
June 2017: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#18
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28489801/a-care-compliant-case-report-lung-transplantation-for-a-chinese-young-man-with-idiopathic-pleuroparenchymal-fibroelastosis
#19
Hui Huang, Ruie Feng, Shan Li, Bo Wu, Kai Xu, Zuojun Xu, Jingyu Chen
RATIONAL: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been less thoroughly studied. There are no effective medications for patients with iPPFE. PATIENT CONCERNS: A 34-year-old man with no asbestos or cigarette exposure was admitted to our ward due to worsening cough and exertional dyspnea for 10 years...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28457400/prostate-cancer-in-renal-transplant-recipients-diagnosis-and-treatment
#20
J A Carvalho, P Nunes, P J Dinis, H Antunes, B Parada, L Marconi, P Moreira, A Roseiro, C Bastos, F Rolo, V Dias, A Figueiredo
BACKGROUND: We currently know that prostate cancer (Pca) risk is reduced in patients undergoing kidney transplantation. However, its impact and treatment are not widely studied. METHODS: This was a retrospective study of male patients submitted to kidney transplantation in our center from 1980 to 2016 evaluating incidence, treatment, and follow-up of Pca in our population. RESULTS: In 1805 patients undergoing kidney transplantation, 20 men were diagnosed with Pca, leading to an incidence of 1...
May 2017: Transplantation Proceedings
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