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https://www.readbyqxmd.com/read/27921039/clinical-manifestation-and-management-of-adpkd-in-western-countries
#1
REVIEW
Claudia Sommerer, Martin Zeier
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease in Western countries. The prevalence is between 2.4/10,000 and 3.9/10,000. ADPKD represents a systemic disease resulting in deterioration in renal function. Until now, mutations in two genes (PKD1 and PKD2) have been identified. Recently, the European Medicines Agency (EMA) approved the use of the vasopressin V2 receptor antagonist tolvaptan to slow the progression of cyst development and renal insufficiency connected with ADPKD in adult patients with chronic kidney disease stages 1-3 at initiation of treatment with evidence of rapidly progressing disease...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27921038/the-clinical-manifestation-and-management-of-autosomal-dominant-polycystic-kidney-disease-in-china
#2
REVIEW
Cheng Xue, Chen-Chen Zhou, Ming Wu, Chang-Lin Mei
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic hereditary kidney disease characterized by progressive enlargement of renal cysts. The incidence is 1-2‰ worldwide. Mutations in two genes (PKD1 and PKD2) cause ADPKD. Currently, there is no pharmaceutical treatment available for ADPKD patients in China. Summary: This review focused on advances in clinical manifestation, gene diagnosis, risk factors, and management of ADPKD in China. There is an age-dependent increase in total kidney volume (TKV) and decrease in renal function in Chinese ADPKD patients...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920860/a-long-standing-external-iliac-artery-pseudoaneurysm-in-a-patient-with-a-history-of-a-failed-kidney-transplant
#3
Satyam Veean, Neil Patel, Daniel Lee, Hoang Vo, Eran Rotem
This is a case of a 66-year-old African American male with history of end stage renal disease due to polycystic disease and failed right kidney transplant. He presents with asymptomatic hematuria, and diagnostic angiography was performed which showed incidental anastomotic site pseudoaneurysm. Our patient had an unusual presentation of a pseudoaneurysm. Pseudoaneurysms associated with failed renal transplants are typically detected within weeks after transplantation and along with failure of the transplant...
December 2016: Radiology case reports
https://www.readbyqxmd.com/read/27916027/-current-status-of-lung-transplantation-in-china-problems-and-perspectives
#4
X A Wang, G N Jiang
Despite rapid progress, clinical lung transplantation in China still lags far behind. A great challenge remains in donor lung utilization and perioperative medicine. It's really abnormal that we are so backward in lung transplantation when we have come up with the advanced world levels in thoracic surgery, pulmonology and critical care medicine. Our shortcomings were analyzed by comparing lung transplantation in China and in the advanced countries. The first problem is multidisciplinary teamwork. In the United States, a lung transplant team includes physician specialized in lung transplantation, thoracic surgeons, nurses, respiratory therapists and other specialists possibly needed...
December 1, 2016: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/27915038/intrauterine-administration-of-hcg-activated-autologous-human-peripheral-blood-mononuclear-cells-pbmc-promotes-live-birth-rates-in-frozen-thawed-embryo-transfer-cycles-of-patients-with-repeated-implantation-failure
#5
Saijiao Li, Jing Wang, Yan Cheng, Danni Zhou, Tailang Yin, Wangming Xu, Nan Yu, Jing Yang
Recurrent implantation failure refers to unsuccessful implantation after repeated transfers of morphologically good quality embryos into a normal uterus. Recently, accumulating evidence has suggested that local immune cells at the implantation site have actively contributed to embryo implantation. Our aim was to study the effects of intrauterine administration of hCG-activated autologous human PBMC on clinical pregnancy, implantation rates and live birth rate of patients who received frozen/thawed embryo transfer...
November 27, 2016: Journal of Reproductive Immunology
https://www.readbyqxmd.com/read/27914026/limbal-stem-cell-and-oral-mucosal-epithelial-transplantation-from-ex-vivo-cultivation-in-lscd-induced-rabbits-histology-and-immunologic-study-of-the-transplant-epithelial-sheet
#6
Napaporn Tananuvat, Kanokkan Bumroongkit, Chainarong Tocharusa, Umnat Mevatee, Aphisek Kongkaew, Somsanguan Ausayakhun
PURPOSE: To evaluate the results of cultivated limbal epithelial and oral mucosal epithelial transplantation (CLET and COMET) in limbal stem cell deficiency (LSCD)-induced rabbit model. MATERIALS AND METHODS: Six New Zealand white rabbits were divided into two groups of three rabbits each. Limbal tissue was harvested from the first group, and oral mucosal biopsy was obtained from the second group. The tissues were cultured using an explant technique with amniotic membrane as a substrate and co-culture with the 3T3 fibroblast and air-lifting method...
December 2, 2016: International Ophthalmology
https://www.readbyqxmd.com/read/27913602/clonal-conversion-of-b-lymphoid-leukemia-reveals-cross-lineage-transfer-of-malignant-states
#7
Rajesh Somasundaram, Josefine Åhsberg, Kazuki Okuyama, Jonas Ungerbäck, Henrik Lilljebjörn, Thoas Fioretos, Tobias Strid, Mikael Sigvardsson
Even though leukemia is considered to be confined to one specific hematopoietic cell type, cases of acute leukemia of ambiguous lineage and patients relapsing in phenotypically altered disease suggest that a malignant state may be transferred between lineages. Because B-cell leukemia is associated with mutations in transcription factors of importance for stable preservation of lineage identity, we here investigated the potential lineage plasticity of leukemic cells. We report that primary pro-B leukemia cells from mice carrying heterozygous mutations in either or both the Pax5 and Ebf1 genes, commonly mutated in human leukemia, can be converted into T lineage leukemia cells...
December 2, 2016: Genes & Development
https://www.readbyqxmd.com/read/27913517/treatment-of-low-risk-myelodysplastic-syndromes
#8
Valeria Santini
The majority of myelodysplastic syndrome (MDS) patients belong to the International Prognostic Scoring System (IPSS) and IPSS-revised (IPSS-R) lower-risk categories. Their precise diagnostics and prognostic stratification is often a challenge, but may ensure the optimization of therapy. The availability of diverse treatment options has significantly improved the quality of life and survival of this group of patients. Anemia is the most relevant cytopenia in terms of frequency and symptoms in lower-risk MDS, and may be treated successfully with erythropoietic stimulating agents, provided a careful selection is performed on the basis of IPSS-R, endogenous erythropoietin levels, and transfusion independence...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913503/diffuse-large-b-cell-lymphoma-r-chop-failure-what-to-do
#9
Bertrand Coiffier, Clémentine Sarkozy
Although rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) is the standard treatment for patients with diffuse large B-cell lymphoma (DLBCL), ∼30% to 50% of patients are not cured by this treatment, depending on disease stage or prognostic index. Among patients for whom R-CHOP therapy fails, 20% suffer from primary refractory disease (progress during or right after treatment) whereas 30% relapse after achieving complete remission (CR). Currently, there is no good definition enabling us to identify these 2 groups upon diagnosis...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913466/nontransplant-therapy-for-bone-marrow-failure
#10
Danielle M Townsley, Thomas Winkler
Nontransplant therapeutic options for acquired and constitutional aplastic anemia have significantly expanded during the last 5 years. In the future, transplant may be required less frequently. That trilineage hematologic responses could be achieved with the single agent eltrombopag in refractory aplastic anemia promotes new interest in growth factors after years of failed trials using other growth factor agents. Preliminary results adding eltrombopag to immunosuppressive therapy are promising, but long-term follow-up data evaluating clonal evolution rates are required before promoting its standard use in treatment-naive disease...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27911463/vascular-access-types-in-patients-starting-hemodialysis-after-failed-kidney-transplant-does-close-nephrology-follow-up-matter
#11
Naveed Ul Haq, Mohamed Said Abdelsalam, Mohammed Mahdi Althaf, Abdulrahman Ali Khormi, Hassan Al Harbi, Bader Alshamsan, Mamdouh Nasser Albaqumi, Dieter Clemens Broering, Lutfi Alkorbi, Hassan Ali Aleid
BACKGROUND: Native arteriovenous fistulae (AVFs) are preferred while central venous catheters (CVCs) are least suitable vascular access (VA) in patients requiring hemodialysis (HD). Unfortunately, around 80% of patients start HD with CVCs. Late referral to nephrologist is thought to be a factor responsible for this. We retrospectively analyzed the types of VA at HD initiation in renal transplant recipients followed by nephrologists with failed transplant. If early referral to nephrologist improves AVF use, these patients should have higher prevalence of AVF at HD initiation...
November 28, 2016: Journal of Vascular Access
https://www.readbyqxmd.com/read/27910963/daratumumab-monoclonal-antibody-therapy-to-treat-multiple-myeloma
#12
C Xia, M Ribeiro, S Scott, S Lonial
Daratumumab (Darzalex[TM]) is a human monoclonal antibody (MAb) that targets CD38; a surface protein highly expressed across multiple myeloma (MM) cells. Preclinical studies have shown daratumumab induces MM cell death through several mechanisms, including complement-dependent cytotoxicity (CDC) antibody-dependent cell-mediated cytotoxicity (ADCC), antibody-dependent cellular phagocytosis (ADCP), apoptosis upon secondary crosslinking and immunomodulatory effects via a decrease in immune suppressive cells. Daratumumab has a favorable toxicity profile and encouraging clinical activity as a single agent and in combination with lenalidomide in heavily pretreated, relapsed patients in whom other novel agents (such as bortezomib, thalidomide and lenalidomide) and stem cell transplant have already failed...
October 2016: Drugs of Today
https://www.readbyqxmd.com/read/27906831/bos-is-associated-with-increased-cytotoxic-pro-inflammatory-cd8-t-nkt-like-and-nk-cells-in-the-small-airways
#13
Greg Hodge, Sandra Hodge, Aeneas Yeo, Phan Nguyen, Emily Hopkins, Holmes-Liew Cl, Paul N Reynolds, Mark Holmes
BACKGROUND: Immunosuppression therapy following lung transplantation fails to prevent bronchiolitis obliterans syndrome (BOS) in many patients, primarily a disease of the small airways. We have reported that BOS is associated with a lack of suppression of cytotoxic mediators, and pro-inflammatory cytokines, in peripheral blood T, NKT-like (particularly CD8+) and NK cells. We also showed a loss of glucocorticoid receptor (GCR) in pro-inflammatory lymphocytes following transplant. It is unknown whether these pro-inflammatory lymphocytes target the small and/or large airways in BOS...
December 1, 2016: Transplantation
https://www.readbyqxmd.com/read/27906830/heterotopic-abdominal-rat-heart-transplantation-as-a-model-to-investigate-volume-dependency-of-myocardial-remodeling
#14
Kálmán Benke, Alex Ali Sayour, Csaba Mátyás, Bence Ágg, Balázs Tamás Németh, Attila Oláh, Mihály Ruppert, István Hartyánszky, Zoltán Szabolcs, Tamás Radovits, Béla Merkely, Gábor Szabó
Heterotopic abdominal rat heart transplantation has been extensively used to investigate ischemic/reperfusion injury, immunological consequences during heart transplantations and also to study remodeling of the myocardium due to volume unloading. We provide a unique review on the latter and present a summary of the experimental studies on rat heart transplantation to illustrate changes that occur to the myocardium due to volume unloading. We divided the literature based on whether normal or failing rat heart models were used...
December 1, 2016: Transplantation
https://www.readbyqxmd.com/read/27905003/new-agents-in-hsc-mobilization
#15
REVIEW
Mélanie J Domingues, Susan K Nilsson, Benjamin Cao
Mobilized peripheral blood (PB) is the most common source of hematopoietic stem cells (HSC) for autologous transplantation. Granulocyte colony stimulating factor (G-CSF) is the most commonly used mobilization agent, yet despite its widespread use, a considerable number of patients still fail to mobilize. Recently, a greater understanding of the interactions that regulate HSC homeostasis in the bone marrow (BM) microenvironment has enabled the development of new molecules that mobilize HSC through specific inhibition, modulation or perturbation of these interactions...
November 30, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27903535/magnetic-resonance-imaging-of-cardiac-strain-pattern-following-transplantation-of-human-tissue-engineered-heart-muscles
#16
Xulei Qin, Johannes Riegler, Malte Tiburcy, Xin Zhao, Tony Chour, Babacar Ndoye, Michael Nguyen, Jackson Adams, Mohamed Ameen, Thomas S Denney, Phillip C Yang, Patricia Nguyen, Wolfram H Zimmermann, Joseph C Wu
BACKGROUND: The use of tissue engineering approaches in combination with exogenously produced cardiomyocytes offers the potential to restore contractile function after myocardial injury. However, current techniques assessing changes in global cardiac performance after such treatments are plagued by relatively low detection ability. Since the treatment is locally performed, this detection could be improved by myocardial strain imaging that measures regional contractility. METHODS AND RESULTS: Tissue engineered heart muscles (EHMs) were generated by casting human embryonic stem cell-derived cardiomyocytes with collagen in preformed molds...
November 2016: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27902998/autoimmune-cytopenias-diagnosis-management
#17
Christian P Nixon, Joseph D Sweeney
The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...
December 1, 2016: Rhode Island Medical Journal
https://www.readbyqxmd.com/read/27900974/disseminated-mycobacteria-chelonae-infection-in-a-kidney-pancreas-transplant-recipient-a-case-report-and-review-of-the-literature
#18
Shafi Malik, Ananda Ghosh, Shahid Husain
A 40-year-old male with a long-standing history of type 1 diabetes with end-stage renal failure underwent combined kidney-pancreas (KP) transplant from a standard criteria donor. Post-operative course was uncomplicated with good primary function of both transplant grafts. Induction was with thymoglobulin and maintenance immunosuppression was with tacrolimus, mycophenolate mofetil and prednisolone. Nine weeks post-transplant, the patient developed dysfunction of both grafts. Panel reactive antibody testing revealed that the patient had developed a de novo donor-specific antibody and considering an antibody-mediated rejection the patient was treated with intravenous pulse methyl prednisone 500 mg ×3 doses, IV immunoglobulin 2 mg/kg in two divided doses, and ATG 7 mg/kg (total dose of 700 mg)...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900108/high-dose-chemotherapy-with-autologous-peripheral-blood-stem-cell-transplantation-for-choriocarcinoma-a-case-report-and-literature-review
#19
Eiko Yamamoto, Kaoru Niimi, Kayo Fujikake, Tetsuya Nishida, Makoto Murata, Ayako Mitsuma, Yuichi Ando, Fumitaka Kikkawa
Choriocarcinoma is a malignant gestational trophoblastic neoplasia (GTN) and one of the curable types of gynecological cancer. However, 10% of choriocarcinoma patients have a poor prognosis, particularly when they have metastasis, apart from pulmonary metastasis, or do not go into remission by the second chemotherapeutic regimen. We herein present the case of a 36-year-old patient who had choriocarcinoma with metastases to the lungs, liver and kidneys. The 5th and 6th regimens with cisplatin for choriocarcinoma failed and the patient developed brain metastases...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27893415/pentraxin-3-plasma-levels-at-graft-versus-host-disease-onset-predict-disease-severity-and-response-to-therapy-in-children-given-haematopoietic-stem-cell-transplantation
#20
Erica Dander, Paola De Lorenzo, Barbara Bottazzi, Paola Quarello, Paola Vinci, Adriana Balduzzi, Francesca Masciocchi, Sonia Bonanomi, Claudia Cappuzzello, Giulia Prunotto, Fabio Pavan, Fabio Pasqualini, Marina Sironi, Ivan Cuccovillo, Roberto Leone, Giovanni Salvatori, Matteo Parma, Elisabetta Terruzzi, Fabio Pagni, Franco Locatelli, Alberto Mantovani, Franca Fagioli, Andrea Biondi, Cecilia Garlanda, Maria Grazia Valsecchi, Attilio Rovelli, Giovanna D'Amico
Acute Graft-versus-Host Disease (GvHD) remains a major complication of allogeneic haematopoietic stem cell transplantation, with a significant proportion of patients failing to respond to first-line systemic corticosteroids. Reliable biomarkers predicting disease severity and response to treatment are warranted to improve its management. Thus, we sought to determine whether pentraxin 3 (PTX3), an acute-phase protein produced locally at the site of inflammation, could represent a novel acute GvHD biomarker. Using a murine model of the disease, we found increased PTX3 plasma levels after irradiation and at GvHD onset...
November 21, 2016: Oncotarget
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