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https://www.readbyqxmd.com/read/29905968/skeletal-consequences-of-nephropathic-cystinosis
#1
Pablo Florenzano, Carlos Ferreira, Galina Nesterova, Mary Scott Ramnitz, Sri Harsha Tella, Luis Fernandez de Castro, Sydney M Brown, Adom Whitaker, Renata C Pereira, Dorothy Bulas, Rachel I Gafni, Isidro B Salusky, William A Gahl, Michael T Collins
Nephropathic cystinosis is a rare lysosomal storage disorder. Patients present in the first year of life with renal Fanconi syndrome that evolves to progressive chronic kidney disease (CKD). Despite the multiple risk factors for bone disease, the frequency and severity of skeletal disorders in nephropathic cystinosis have not been described. We performed systematic bone and mineral evaluations of subjects with cystinosis seen at the NIH (N = 30), including history and physical examination, serum and urine biochemistries, DXA, vertebral fracture assessment, skeletal radiographs, and renal ultrasound...
June 15, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29899188/effects-of-the-calcineurin-inhibitors-cyclosporine-and-tacrolimus-on-bone-metabolism-in-rats
#2
Junkichi Kanda, Nobuo Izumo, Megumi Furukawa, Taketoshi Shimakura, Noriaki Yamamoto, Hideaki E Takahashi, Toshinari Asakura, Hiroyuki Wakabayashi
Immunosuppressive therapy is considered as one of the factors inducing to the onset of osteoporosis after organ transplantation. Chronic immunosuppressive therapy after transplantation is required for organ transplant patients, and it is important to prevent the occurrence of osteoporotic fractures to maintain the quality of life in patients. In this study, we examined the effects of cyclosporine and tacrolimus on bone metabolism in rats. Five-week-old male Wistar rats were treated orally with 15 mg/kg cyclosporine or 1...
2018: Biomedical Research
https://www.readbyqxmd.com/read/29880247/development-and-current-use-of-in-hematopoietic-stem-cell-transplantation-in-children-and-adolescents-in-poland-report-of-the-polish-pediatric-study-group-for-hematopoietic-stem-cell-transplantation-of-the-polish-society-for-pediatric-oncology-and-hematology
#3
REVIEW
Jacek Wachowiak, Alicja Chybicka, Jerzy R Kowalczyk, Mariusz Wysocki, Jolanta Goździk, Ewa Gorczyńska, Krzysztof Kałwak, Jan Styczyński, Katarzyna Drabko, Anna Pieczonka
The purpose of the survey was to evaluate the development and current use of hematopoietic stem cell transplantation (HSCT) in Poland between 1989-2016. The data for analysis (indication, number of performed HSCT, HSCT type, donor type, and stem cell source, year) have been collected annually using a standardized form. In Poland, between 1989-2016, the number of pediatric transplant beds grew from one to 40 and number and rate of transplants increased annually from 1/year (0.8/10 million) to 186/year (248/10 million)...
May 16, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29880053/indications-and-complications-of-inpatient-parenteral-nutrition-prescribed-to-children-in-a-large-tertiary-referral-hospital
#4
C Mantegazza, N Landy, G V Zuccotti, J Köglmeier
BACKGROUND: Parenteral Nutrition (PN) is prescribed to children with intestinal failure. Although life saving, complications are common. Recommendations for indications and constituents of PN are made in the 2005 guidelines by the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN). The aim of this study was to establish if the indications for prescribing PN in a tertiary children's hospital were appropriate, and to identify complications encountered. Data were compared to those published by the National Confidential Enquiry into patient outcome and death (NCEPOD) carried out in the United Kingdom in 2010...
June 8, 2018: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/29873153/a-vexing-case-of-bone-pain-in-a-renal-transplant-recipient-voriconazole-induced-periostitis
#5
Krishna Poinen, Marianna Leung, Alissa Jade Wright, David Landsberg
BACKGROUND: Immunosuppression increases the risk of opportunistic infections including fungal infections in solid organ transplant recipients. Voriconazole is used to treat invasive aspergillus infections but prolonged usage may rarely lead to periostitis. Increased plasma fluoride concentration leading to osteoblastic upregulation is thought to be the catalyst, and symptom reversal occurs with discontinuation of the offending agent. CASE: A renal transplant recipient who was on voriconazole for invasive aspergillosis developed diffuse debilitating symmetrical bone pain...
June 5, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29844930/macrophage-inducible-nitric-oxide-synthase-circulates-inflammation-and-promotes-lung-carcinogenesis
#6
Xin Wang, Zane Gray, Jami Willette-Brown, Feng Zhu, Gongping Shi, Qun Jiang, Na-Young Song, Liang Dong, Yinling Hu
Human lung squamous cell carcinoma (SCC) is highly associated with increased pulmonary macrophage infiltration. Previously, we showed that marked pulmonary infiltrating macrophages were required for spontaneous lung SCC development in a mouse model ( L-Ikkα KA/KA , KA/KA ) that resembles human lung SCC. Interestingly the lung SCC-associated macrophages specifically express elevated inducible nitric oxide synthase (NOS2). However, the role of macrophage NOS2 in lung carcinogenesis has not been explored. Here, we show that NOS2 ablation inhibits macrophage infiltration, fibrosis, and SCC development in the lungs of KA/KA mice...
2018: Cell Death Discovery
https://www.readbyqxmd.com/read/29800310/metabolic-risk-profile-in-kidney-transplant-candidates-and-recipients
#7
Giovanni Piotti, Ilaria Gandolfini, Alessandra Palmisano, Umberto Maggiore
Metabolic risk factors of cardiovascular disease such as abnormal glucose regulation, obesity and metabolic syndrome, dyslipidaemia, metabolic bone disease, hyperuricaemia and other less traditional abnormalities are common in both kidney transplant candidates and recipients. In kidney transplant candidates, the presence of these risk factors may impede patient access to transplantation by increasing the risk of developing comorbidities while on the waiting list, prolonging the time to wait-listing and, in some patients, eventually jeopardizing their suitability for kidney transplantation or increasing the risk of severe perioperative complications...
May 25, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29799319/the-role-of-nrf2-in-the-response-to-normal-tissue-radiation-injury
#8
Brent D Cameron, Konjeti R Sekhar, Maxwell Ofori, Michael L Freeman
The transcription factor Nrf2 is an important modulator of antioxidant and drug metabolism, carbohydrate and lipid metabolism, as well as heme and iron metabolism. Regulation of Nrf2 expression occurs transcriptionally and post-transcriptionally. Post-transcriptional regulation entails ubiquitination followed by proteasome-dependent degradation. Additionally, Nrf2-mediated gene expression is subject to negative regulation by ATF3, Bach1 and cMyc. Nrf2-mediated gene expression is an important regulator of a cell's response to radiation...
May 25, 2018: Radiation Research
https://www.readbyqxmd.com/read/29780418/biological-and-clinical-effects-of-calciprotein-particles-on-chronic-kidney-disease-mineral-and-bone-disorder
#9
REVIEW
Kenichi Akiyama, Takaaki Kimura, Kazuhiro Shiizaki
Calciprotein particles (CPPs) are a new biological marker of chronic kidney disease-mineral and bone disorder (CKD-MBD). CPPs consist of phosphate, calcium, and some proteins, with phosphate being the major contributor to the level and biological activity of CPPs. Recent studies have shown the physiological and pathological significance of CPPs, including contributions to bone and mineral metabolism, and to tissue and organ impairments such as cardiovascular damage and inflammatory responses. These actions are well known as important aspects of CKD-MBD...
2018: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29775762/aortic-vascular-calcification-is-inversely-associated-with-the-trabecular-bone-score-in-patients-receiving-dialysis
#10
Jasna Aleksova, Samantha Kurniawan, Mirna Vucak-Dzumhur, Peter Kerr, Peter R Ebeling, Frances Milat, Grahame J Elder
INTRODUCTION: Progressive chronic kidney disease (CKD) confers a marked increase in risk for vascular calcification, cardiovascular disease, fracture and mortality, with likely contributing factors including dysregulated bone metabolism and mineral homeostasis. In general population studies, increased vascular calcification is directly related to mortality and inversely related to bone mineral density (BMD) measured by dual-energy X-ray absorptiometry (DXA). In patients with CKD, abnormalities in turnover, mineralization and bone volume, reduce the ability of DXA to predict fracture...
May 15, 2018: Bone
https://www.readbyqxmd.com/read/29756133/biology-of-teeth-and-implants-host-factors-pathology-regeneration-and-the-role-of-stem-cells
#11
F-Michael Eggert, Liran Levin
In chronic periodontitis and peri-implantitis, cells of the innate and adaptive immune systems are involved directly in the lesions within the tissues of the patient. Absence of a periodontal ligament around implants does not prevent a biologic process similar to that of periodontitis from affecting osseointegration. Our first focus is on factors in the biology of individuals that are responsible for the susceptibility of such individuals to chronic periodontitis and to peri-implantitis. Genetic factors are of significant importance in susceptibility to these diseases...
2018: Quintessence International
https://www.readbyqxmd.com/read/29720911/association-between-thiopurine-s-methyltransferase-tpmt-genetic-variants-and-infection-in-pediatric-heart-transplant-recipients-treated-with-azathioprine-a-multi-institutional-analysis
#12
Dionna J Green, Son Q Duong, Gilbert J Burckart, Tristan Sissung, Douglas K Price, William D Figg, Maria M Brooks, Richard Chinnock, Charles Canter, Linda Addonizio, Daniel Bernstein, David C Naftel, Adriana Zeevi, James K Kirklin, Steven A Webber, Brian Feingold
OBJECTIVES: Bone marrow suppression is a common adverse effect of the immunosuppressive drug azathioprine. Polymorphisms in the gene encoding thiopurine S -methyltransferase (TPMT) can alter the metabolism of azathioprine, resulting in marrow toxicity and life-threatening infection. In a multicenter cohort of pediatric heart transplant (HT) recipients, we determined the frequency of TPMT genetic variation and assessed whether azathioprine-treated recipients with TPMT variants were at increased risk of infection...
March 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29705267/branched-chain-amino-acid-depletion-conditions-bone-marrow-for-hematopoietic-stem-cell-transplantation-avoiding-amino-acid-imbalance-associated-toxicity
#13
Adam C Wilkinson, Maiko Morita, Hiromitsu Nakauchia, Satoshi Yamazaki
Hematopoietic stem cells (HSCs) are used clinically in bone marrow (BM) transplantation due to their unique ability to reform the entire hematopoietic system. Recently, we reported that HSCs are highly sensitive to valine, one of the three branched-chain amino acids (BCAAs) in addition to isoleucine and leucine. Dietary depletion of valine could even be used as a conditioning regimen for HSC transplantation. Here, we report that HSCs are highly sensitive to the balance of BCAAs, with both proliferation and survival reduced by BCAA imbalance...
April 26, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29700855/long-term-extrapulmonary-comorbidities-after-lung-transplantation-in-cystic-fibrosis-update-of-specificities
#14
REVIEW
Sabine Jardel, Quitterie Reynaud, Isabelle Durieu
Lung transplantation (LT) is the standard therapeutic option for cystic fibrosis (CF) patients with end-stage lung disease. Both conditions lead to extrarespiratory complications, such as diabetes, renal insufficiency, bone disease, and cancer. The purpose of this study was to provide an update of the nonrespiratory comorbidities following LT in adult patients with CF and their specificities regarding their multisystemic underlying condition despite their younger age compared to other patients undergoing LT...
April 26, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29700120/protease-activated-receptor-2-plays-a-critical-role-in-vascular-inflammation-and-atherosclerosis-in-apolipoprotein-e-deficient-mice
#15
Tomoya Hara, Pham Tran Phuong, Daiju Fukuda, Koji Yamaguchi, Chie Murata, Sachiko Nishimoto, Shusuke Yagi, Kenya Kusunose, Hirotsugu Yamada, Takeshi Soeki, Tetsuzo Wakatsuki, Issei Imoto, Michio Shimabukuro, Masataka Sata
Background -The coagulation system is closely linked with vascular inflammation, although the underlying mechanisms are still obscure. Recent studies show that protease-activated receptor (PAR)-2, a major receptor of activated factor X (FXa), are expressed in both vascular cells and leukocytes, suggesting that PAR-2 may contribute to the pathogenesis of inflammatory diseases. Here we investigated the role of PAR-2 in vascular inflammation and atherogenesis. Methods -We generated apolipoprotein E-deficient ( ApoE-/- ) mice lacking systemic PAR-2 expression ( ApoE-/- PAR-2-/- )...
April 26, 2018: Circulation
https://www.readbyqxmd.com/read/29693762/growth-body-composition-and-bone-density-following-pediatric-liver-transplantation
#16
Amin Sheikh, Tim Cundy, Helen Maria Evans
Patients transplanted for cholestatic liver disease are often significantly fat-soluble vitamin deficient and malnourished pretransplant, with significant corticosteroid exposure post-transplant, with increasing evidence of obesity and metabolic syndrome post-LT. Our study aimed to assess growth, body composition, and BMD in patients post-pediatric LT. Body composition and bone densitometry scans were performed on 21 patients. Pre- and post-transplant anthropometric data were analyzed. Bone health was assessed using serum ALP, calcium, phosphate, and procollagen-1-N-peptide levels...
April 24, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29675567/human-urine-derived-stem-cells-play-a-novel-role-in-the-treatment-of-stz-induced-diabetic-mice
#17
Tianxue Zhao, Deng Luo, Yun Sun, Xin Niu, Yang Wang, Chen Wang, Weiping Jia
Human urine-derived stem cells (hUSCs) are a potential stem cell source for cell therapy. However, the effect of hUSCs on glucose metabolism regulation in type 1 diabetes was not clear. Therefore, the aim of the study was to evaluate whether hUSCs have protective effect on streptozotocin (STZ)-induced diabetes. hUSCs were extracted and cultivated with a special culture medium. Flow cytometry analysis was applied to detect cell surface markers. BALB/c male nude mice were either injected with high-dose STZ (HD-STZ) or multiple low-dose STZ (MLD-STZ)...
April 19, 2018: Journal of Molecular Histology
https://www.readbyqxmd.com/read/29666081/mantle-cell-lymphoma-presenting-as-bilateral-adrenal-huge-masses
#18
Abtin Doroudinia, Mehrdad Bakhshayesh Karam, Mostafa Ranjbar, Arash Nikmanesh, Payam Mehrian
We present a middle-aged woman complaining of weakness, lethargy and weight loss for 6 months. Positron emission tomography (PET)/CT scan revealed huge bilateral adrenal masses with intense 18F-fluorodeoxyglucose avidity. Biopsy and immunohistochemical staining were compatible with mantle cell lymphoma (MCL). The patient received six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) followed by four cycles of intrathechal methotrexate chemotherapy. Bone marrow aspiration biopsy was performed for initial staging and also before bone marrow transplantation...
April 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29619911/early-use-of-etanercept-for-graft-versus-host-disease-after-liver-transplant-the-importance-of-broad-spectrum-infective-prophylaxis
#19
Annalisa Boscolo, Eugenia Menin, Beatrice Zelaschi, Laura Albertoni, Giacomo Zanus, Fabio Baratto
Graft-versus-host-disease after orthotopic liver transplant is a rare and life-threatening complication. The diagnosis is challenging and usually confirmed by chimerism and skin biopsies. The most common cause of death is sepsis (60%), and broad-spectrum antibiotics and antifungal prophylaxis are strongly recommended. We present a case of a 61-year-old man with hepatocellular carcinoma and a previous history of metabolic and alcoholic cirrhosis who underwent orthotopic liver transplant. The immunosuppression regimen consisted of corticosteroids, calcineurin inhibitor, and mammalian target of rapamycin complex 1 inhibitor...
April 4, 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29605874/the-hematopoietic-stem-cell-diet
#20
REVIEW
Adam C Wilkinson, Satoshi Yamazaki
Hematopoietic stem cells (HSCs) are responsible for sustaining life-long blood formation or hematopoiesis and are also used clinically in a form of bone marrow transplantation, a curative cellular therapy for a range of hematological diseases. HSCs are maintained throughout adult life by a complex biological niche or microenvironment, which is thought to be composed of a range of cellular, molecular, and metabolic components. The metabolic components of the HSC niche have become of increasing interest over the past few years...
June 2018: International Journal of Hematology
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