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JOURNAL ARTICLE
Wendy Glaberson, Wacharee Seeherunvong, Jeffrey J Gaynor, Chryso Katsoufis, Marissa Defreitas, Yong Bao, Michael Freundlich, Gaetano Ciancio, Carolyn Abitbol, Jayanthi Chandar
INTRODUCTION: Hyperparathyroidism (HPT) can contribute to metabolic bone disease following kidney transplantation. We evaluated post-transplant trends in intact parathyroid hormone (iPTH) and determined predictors of HPT in pediatric kidney transplant (KTx) recipients. METHODS: In this single-center study, retrospective data were collected on 88 children from 2013 to 2019. Data collected included dialysis vintage, biochemical parameters, post-transplant trends in iPTH, 25(OH)Vitamin D levels and estimated glomerular filtration rate (eGFR ml/min/1...
March 2024: Clinical Transplantation
#22
JOURNAL ARTICLE
Simon Hsu, Nisha Bansal, Michelle Denburg, Charles Ginsberg, Andrew N Hoofnagle, Tamara Isakova, Joachim H Ix, Cassianne Robinson-Cohen, Myles Wolf, Bryan R Kestenbaum, Ian H de Boer, Leila R Zelnick
Fracture risk is high in chronic kidney disease (CKD) and underlying pathophysiology and risk factors may differ from the general population. In a cohort study of 3939 participants in the Chronic Renal Insufficiency Cohort (CRIC), we used Cox regression to test associations of putative risk factors with the composite of first hip or vertebral fracture assessed using hospital discharge codes. Mean age was 58 years, 45% were female, 42% were Black, and 13% were Hispanic. There were 82 hip and 24 vertebral fractures over a mean (SD) 11...
February 4, 2024: Journal of Bone and Mineral Research
#23
JOURNAL ARTICLE
Hui-Yi Li, Yu-Xuan Gao, Jun-Cheng Wu, Jing-Ze Li, Seng-Wang Fu, Ming-Yi Xu
AIM: To understand how liver sinusoidal endothelial cells (LSECs) respond to nonalcoholic steatohepatitis (NASH). METHODS: We profiled single-LSEC from livers of control and MCD-fed mice. The functions of C-Kit+ -LSECs were determined using coculture and bone marrow transplantation (BMT) methods. RESULTS: Three special clusters of single-LSEC were differentiated. C-Kit+ -LSECs of cluster 0, Msr1+ -LSECs of cluster 1 and Bmp4+ Selp+ -VECs of cluster 2 were revealed, and these cells with diverse ectopic expressions of genes participated in regulation of endothelial, fibrosis and lipid metabolism in NASH...
March 9, 2024: Cell & Bioscience
#24
JOURNAL ARTICLE
Rainer Büscher, Lars Pape, Anja K Büscher
BACKGROUND: Primary hyperoxaluria type 1 is characterized by hepatic oxalate overproduction, leading to nephrocalcinosis, kidney stones, kidney failure and systemic oxalosis, including oxalate osteopathy. Combined liver-kidney transplantation (CLKT) and kidney after liver transplantation (KALT) were established therapeutic options to stop the devastating consequences of oxalate bone disease. METHODS: We describe a retrospective cohort of 10 children with PH1who were referred to our hospital from different countries for combined transplantation...
2024: Frontiers in Pediatrics
#25
REVIEW
Aleksandra Kukla, Yogish C Kudva, Pavel Navratil, Sukhdeep S Sahi, Roberto P Benzo, David C Fipps, Avery E Erickson, Rachael R Majorowicz, Matthew M Clark, Carrie A Schinstock, Pankaj Shah, Meera Shah, Tayyab S Diwan
Bariatric surgery is increasingly recognized as a safe and effective treatment for obesity in patients with chronic kidney disease (CKD), including stages 4, 5, and 5D (on dialysis). Among the available surgical methods, sleeve gastrectomy (SG) is the most commonly performed weight loss procedure and is mainly done to facilitate kidney transplantation (KT). However, many KT candidates treated with SG remain on the transplant waiting list for months to years, with some never receiving a transplant. Therefore, appropriate candidates for SG must be selected, and post-SG management should address the unique needs of this population, with a focus on sustaining the metabolic benefits of surgery while minimizing potential side effects related to rapid weight loss which may inadvertently lead to muscle and bone catabolism...
March 2024: Mayo Clinic Proceedings
#26
JOURNAL ARTICLE
Ying Ma, Yuancheng Bao, Han Wang, Huaizhou Jiang, Lei Zhou, Bo Yang, Xiaofeng Huang, Wenming Yang, Daojun Xie, Juan Zhang
BACKGROUND: We administered Bushen Huoxue Huazhuo Formula (BSHXHZF) and transplanted bone marrow mesenchymal stem cells (BMSCs) into mice with Wilson's disease (WD)-related liver fibrosis to evaluate the liver-protecting mechanism of this prescription. METHODS: Mice, randomly divided into different treatment groups, showed histopathological changes and degree of hepatocyte apoptosis. For hepatic hydroxyproline (Hyp) determination, transforming growth factor-β1 (TGF-β1) and bone morphogenetic protein-7 (BMP-7) mRNA and protein were measured...
March 1, 2024: Journal of Pharmacy and Pharmacology
#27
JOURNAL ARTICLE
Chunlai Xiao, Genqiang Bao, Guohui Zhang, Yulong Guan, Quan Wang, Hua Li
OBJECTIVE: To investigate the application of digital artery transposition in replanting severed fingers with vascular defects and its impact on nerve and joint function recovery. METHODS: 200 patients who received replantation of severed fingers were randomly divided into artery transposition group (n = 100) and vein transplantation group (n = 100). The digital artery transposition technique was used in the artery transposition group, and the autologous vein bridging technique was used in the vein transplantation group...
March 1, 2024: Journal of Musculoskeletal & Neuronal Interactions
#28
JOURNAL ARTICLE
Daniel Salevitz, Lucia Mirea, Gwen M Grimsby
INTRODUCTION: Hemorrhagic cystitis (HC) is a devastating complication of bone marrow (BMT) and stem cell transplant (SCT). Much of the literature has focused on exclusively adult patient populations, with limited evidence regarding risk factors for mortality and morbidity among pediatric HC patients. OBJECTIVE: To examine factors associated with all-cause mortality in children with HC after BMT/SCT. METHODS: The Pediatric Health Information System database was queried for patients with ICD-9/10 codes for hematopoietic transplant and gross hematuria, hematuria unspecified, or cystitis with hematuria...
February 15, 2024: Journal of Pediatric Urology
#29
JOURNAL ARTICLE
Novella Rapini, Maurizio Delvecchio, Mafalda Mucciolo, Rosario Ruta, Ivana Rabbone, Valentino Cherubini, Stefano Zucchini, Stefano Cianfarani, Elena Prandi, Riccardo Schiaffini, Carla Bizzarri, Barbara Piccini, Giulio Maltoni, Barbara Predieri, Nicola Minuto, Rossella Di Paola, Mara Giordano, Nadia Tinto, Valeria Grasso, Lucia Russo, Valentina Tiberi, Andrea Scaramuzza, Giulio Frontino, Maria Cristina Maggio, Gianluca Musolino, Elvira Piccinno, Davide Tinti, Paola Carrera, Enza Mozzillo, Marco Cappa, Dario Iafusco, Riccardo Bonfanti, Antonio Novelli, Fabrizio Barbetti
CONTEXT: In the last decade Sanger method of DNA sequencing has been replaced by next generation sequencing (NGS). NGS is valuable in conditions characterized by high genetic heterogeneity such as neonatal diabetes mellitus (NDM). OBJECTIVE: To compare results of genetic analysis of patients with NDM and congenital severe insulin resistance (c.SIR) identified in Italy in 2003-2012 (Sanger) versus 2013-2022 (NGS). METHODS: We reviewed clinical and genetic records of 104 cases with diabetes onset before 6 months of age (NDM+c...
February 26, 2024: Journal of Clinical Endocrinology and Metabolism
#30
JOURNAL ARTICLE
Mei Li, Ahmed A Hasan, Chang Chu, Johann-Georg Hocher, Yvonne Liu, Xiaoli Zhang, Xin Chen, Benito Yard, Bernhard K Krämer, Berthold Hocher
Sclerostin (SOST) is produced by osteocytes and is known as a negative regulator of bone homeostasis. Parathyroid hormone (PTH) regulates calcium, phosphate as well as vitamin D metabolism, and is a strong inhibitor of SOST synthesis in vitro and in vivo. PTH has two methionine amino acids (positions 8 and 18) which can be oxidized. PTH oxidized at Met18 (Met18(ox)-PTH) continues to be bioactive, whereas PTH oxidized at Met8 (Met8(ox)-PTH) or PTH oxidized at Met8 and Met18 (Met8, Met18(di-ox)-PTH) has minor bioactivity...
February 23, 2024: Pflügers Archiv: European Journal of Physiology
#31
Devprakash Choudhary, K S Lekshmon, Charanpreet Singh, Vanji N Subramani, Yogendra Singh, Suvradeep Mitra, Aravind Sekar, Manaswinee Malik, Naveen Bhagat, S P Shiva Kumar, Sunil Taneja, Vikas Gupta, Raja Ramachandran, Sarbpreet Singh, Ritambhra Nada, Deepesh Kenwar, Ajay K Duseja, T D Yadav, Pankaj Malhotra, Ashish Sharma
Organ transplantation is the primary therapy for organ failure caused by telomere biology disorder (TBD). We describe the first documented case of simultaneous liver and kidney transplantation (SLKTx) for TBD, although the diagnosis of TBD was reached only three months following SLKTx. The patient was born prematurely, displayed growth retardation, and developed chronic kidney and liver diseases. His pre-SLKTx autoimmune, metabolic, and viral assessments were negative, and persistent pancytopenia (bone marrow cellularity 70-80%) was attributed to renal disease-associated bone marrow changes...
2024: Journal of Clinical and Experimental Hepatology
#32
JOURNAL ARTICLE
Zeying Li, Shan Jin, Tong Xu, Hongzhi Chen, Wenping Cai, Jin Du, Qiu Jin, Sihui Zhuang, Yan Qi, Wenyi Gu, Lijuan Pang
Seipin deficiency is an important cause of type 2 Berardinelli-Seip congenital dyslipidemia (BSCL2). BSCL2 is a severe lipodystrophy syndrome with lack of adipose tissue, hepatic steatosis, insulin resistance, and normal or higher bone mineral density. Bone marrow mesenchymal stem cells (BMSCs) are believed to maintain bone and fat homeostasis by differentiating into osteoblasts and adipocytes. We aimed to explore the role of seipin in the osteogenic/adipogenic differentiation balance of BMSCs. Seipin loxP/loxP mice are used to explore metabolic disorders caused by seipin gene mutations...
February 22, 2024: Stem Cells and Development
#33
Muride Aktas, Hayriye Nur Oray Unlu, Murat Karatas, Gokalp Okut, Berna Eren, Adam Uslu, Erhan Tatar
Deviations of calcium, phosphate, parathyroid hormone, and vitamin D levels are the basis for the diagnosis of calcium-phosphate metabolism disorders. The plasma concentration of the biologically active form known as free calcium is regulated in a harmonious manner by its exchange in the bones and reabsorption by the kidneys. These steps take place under the control of parathyroid hormone and calcitriol. In the process of chronic kidney disease, the kidney cannot synthesize adequate calcitriol, and the resulting hypocalcemia and hyperphosphatemia cause the development of secondary hyperparathyroidism...
January 2024: Experimental and Clinical Transplantation
#34
JOURNAL ARTICLE
Fernanda Guedes Rodrigues, Willemijn Y Van Der Plas, Camilo German Sotomayor, Amarens Van Der Vaart, Daan Kremer, Robert A Pol, Schelto Kruijff, Ita Pfeferman Heilberg, Stephan J L Bakker, Martin H De Borst
The impact of pre-transplant parathyroid hormone (PTH) levels on early or long-term kidney function after kidney transplantation is subject of debate. We assessed whether severe hyperparathyroidism is associated with delayed graft function (DGF), death-censored graft failure (DCGF), or all-cause mortality. In this single-center cohort study, we studied the relationship between PTH and other parameters related to bone and mineral metabolism, including serum alkaline phosphatase (ALP) at time of transplantation with the subsequent risk of DGF, DCGF and all-cause mortality using multivariable logistic and Cox regression analyses...
2024: Transplant International
#35
JOURNAL ARTICLE
Chang Ma, Yihui Li, Mi Tian, Qiming Deng, Xiaoteng Qin, Hanlin Lu, Jiangang Gao, Min Chen, Lee S Weinstein, Mei Zhang, Peili Bu, Jianmin Yang, Yun Zhang, Cheng Zhang, Wencheng Zhang
BACKGROUND: Many cardiovascular pathologies are induced by signaling through G-protein-coupled receptors via Gsα (G protein stimulatory α subunit) proteins. However, the specific cellular mechanisms that are driven by Gsα and contribute to the development of atherosclerosis remain unclear. METHODS: High-throughput screening involving data from single-cell and bulk sequencing were used to explore the expression of Gsα in atherosclerosis. The differentially expression and activity of Gsα were analyzed by immunofluorescence and cAMP measurements...
March 29, 2024: Circulation Research
#36
JOURNAL ARTICLE
Christopher L Haga, Cori N Booker, Jacqueline Strivelli, Siddaraju V Boregowda, Donald G Phinney
BACKGROUND AIMS: Mesenchymal stem/stromal cells (MSCs) are defined as culture-expanded populations, and although these cells recapitulate many properties of bone marrow (BM) resident skeletal stem/progenitor cells, few studies have directly compared these populations to evaluate how culture adaptation and expansion impact critical quality attributes. METHODS: We analyzed by RNA sequencing Lin- SCA1+ MSCs enriched from BM by immunodepletion (ID) and after subsequent culture expansion (Ex) and Lin- LEPR+ MSCs sorted (S) directly from BM...
February 17, 2024: Cytotherapy
#37
JOURNAL ARTICLE
Maddalena Migliavacca, Federica Barzaghi, Claudia Fossati, Paola M V Rancoita, Michela Gabaldo, Francesca Dionisio, Stefania Giannelli, Federica Andrea Salerio, Francesca Ferrua, Francesca Tucci, Valeria Calbi, Vera Gallo, Salvatore Recupero, Giulia Consiglieri, Roberta Pajno, Maria Sambuco, Alessio Priolo, Chiara Ferri, Vittoria Garella, Ilaria Monti, Paolo Silvani, Silvia Darin, Miriam Casiraghi, Ambra Corti, Stefano Zancan, Margherita Levi, Daniela Cesana, Filippo Carlucci, Anna Pituch-Noworolska, Dalia AbdElaziz, Ulrich Baumann, Andrea Finocchi, Caterina Cancrini, Saverio Ladogana, Andrea Meinhardt, Isabelle Meyts, Davide Montin, Lucia Dora Notarangelo, Fulvio Porta, Marlène Pasquet, Carsten Speckmann, Polina Stepensky, Alberto Tommasini, Marco Rabusin, Zeynep Karakas, Miguel Galicchio, Lucia Leonardi, Marzia Duse, Sukru Nail Guner, Clelia Di Serio, Fabio Ciceri, Maria Ester Bernardo, Alessandro Aiuti, Maria Pia Cicalese
Adenosine deaminase (ADA) deficiency leads to severe combined immunodeficiency (SCID). Previous clinical trials showed that autologous CD34+ cell gene therapy (GT) following busulfan reduced-intensity conditioning is a promising therapeutic approach for ADA-SCID, but long-term data are warranted. Here we report an analysis on long-term safety and efficacy data of 43 patients with ADA-SCID who received retroviral ex vivo bone marrow-derived hematopoietic stem cell GT. Twenty-two individuals (median follow-up 15...
February 14, 2024: Nature Medicine
#38
REVIEW
Varun J Sharma, Aashima Singh, James Llewelyn Grant, Jaishankar Raman
Histopathology is the gold standard for diagnosing fibrosis, but its routine use is constrained by the need for additional stains, time, personnel and resources. Vibrational spectroscopy is a novel technique that offers an alternative atraumatic approach, with short scan times, while providing metabolic and morphological data. This review evaluates vibrational spectroscopy for the assessment of fibrosis, with a focus on point-of-care capabilities. OVID Medline, Embase and Cochrane databases were systematically searched using PRISMA guidelines for search terms including vibrational spectroscopy, human tissue and fibrosis...
January 18, 2024: Pathology
#39
JOURNAL ARTICLE
Flávia Maria Borges Vigil, Pedro Alves Soares Vaz de Castro, Ursula Gramiscelli Hasparyk, Victória Soares Bartolomei, Ana Cristina Simões E Silva
BACKGROUND: The impact of treatments, suppressing the immune system, persistent hyperparathyroidism, and other risk factors on mineral and bone disorder (MBD) after kidney transplantation is well-known. However, there is limited knowledge about their effect on bone metabolism biomarkers. This study aimed to investigate the influence of kidney transplant on these markers, comparing them to patients undergoing hemodialysis and healthy individuals. METHODS: In this cross-sectional study, three groups were included: kidney transplant patients (n = 57), hemodialysis patients (n = 26), and healthy controls (n = 31)...
February 7, 2024: Current Medicinal Chemistry
#40
JOURNAL ARTICLE
Qidong Huo, Tongpeng Yue, Wenxuan Li, Xinyue Wang, Yinping Dong, Deguan Li
PURPOSE: Damage to the hematopoietic system and functional inhibition are severe consequences of radiation exposure. In this study, we have investigated the effect of empagliflozin on radiation-induced hematopoietic damage, with the aim of providing new preventive approach to such injuries. METHODS AND MATERIALS: Mice were given 4 Gy total body irradiation (TBI) 1 h after the oral administration of empagliflozin, followed by the continuous administration of the same dose of empagliflozin for 6d, and then sacrificed on the 10th day after irradiation...
February 6, 2024: Life Sciences
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