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Myelodysplastic and myeloproliferative disorders of childhood.

Myelodysplastic and myeloproliferative disorders of childhood. Hematology Am Soc Hematol Educ Program. 2016 Dec 02;2016(1):598-604 Authors: Hasle H Abstract Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions. RCC is often hypoplastic and may respond to immunosuppressive therapy. In case of immunosuppressive therapy failure, hypercellular RCC, or RCC with monosomy 7, hematopoietic stem cell transplantation (HSCT) using reduced-intensity conditioning regimens is indicated. Almost all patients with refractory anemia with excess blasts are candidates for HSCT; children age 12 years or older have a higher risk of treatment-related death, and the conditioning regimens should be adjusted accordingly. Unraveling the genetics of JMML has demonstrated that JMML in patients with germ line PTPN11 and CBL mutations often regresses spontaneously, and therapy is seldom indicated. Conversely, patients with JM...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

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Authors: Stahlschmidt A, Novelo B, Alexi Freitas L, Cavalcante Passos S, Dussián-Sarria JA, Félix EA, Wajnberg Gamermann P, Caumo W, Cadore Stefani LP Abstract INTRODUCTION: Morbidity and mortality associated with urgent or emergency surgeries are high compared to elective procedures. Perioperative risk scores identify the non-elective character as an independent factor of complications and death. The present study aims to characterize the population undergoing non-elective surgeries at the Hospital de Clínicas de Porto Alegre and identify the clinical and surgical factors associated with death...
Source: Revista Brasileira de Anestesiologia - Category: Anesthesiology Tags: Rev Bras Anestesiol Source Type: research
Bantounas et  al. (Stem Cell Reports. 2018;10:766–779. https://doi.org/10.1016/j.stemcr.2018.01.008)
Source: Kidney International - Category: Urology & Nephrology Tags: Journal Club Source Type: research
Novel concepts employing autologous, ex  vivo expanded natural regulatory T cells (nTreg) for adoptive transfer has potential to prevent organ rejection after kidney transplantation. However, the impact of dialysis and maintenance immunosuppression on the nTreg phenotype and peripheral survival is not well understood, but essential when assessing patient eligibility. The current study investigates regulatory T-cells in dialysis and kidney transplanted patients and the feasibility of generating a clinically useful nTreg product from these patients.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Clinical Investigation Source Type: research
Veterinary and Comparative Oncology, EarlyView.
Source: Veterinary and Comparative Oncology - Category: Veterinary Research Authors: Source Type: research
Authors: Kocaba V, Damour O, Auxenfans C, Burillon C Abstract In France, endothelial dysfunction represents approximately one half of the indications for corneal transplants performed each year. However, the use of endothelial keratoplasty is limited by the technical difficulty of the procedure, a shortage of available grafts, and the potential for graft failure or rejection. These limitations are driving researchers to develop new, less invasive, and more effective therapies. Corneal endothelial cell therapy is being explored as a potential therapeutic measure, to avoid the uncertainty associated with grafting. Th...
Source: Journal Francais d Ophtalmologie - Category: Opthalmology Tags: J Fr Ophtalmol Source Type: research
Abstract Hypoxia plays a crucial role in cancer progression, and it has great significance for monitoring hypoxic level in biosystems. Cancer stem cells (CSCs) represent a small population of tumour cells that regard as the key to seed tumours. The survival of CSCs depend on the tumour microenvironment, which is distinct region has the hypoxic property. Therefore, the detection of the hypoxic CSC niche plays a pivotal role in the destructing the 'soil' of CSCs, and eliminating CSCs population. Numerous one-photon excited fluorescent probes have been developed to indicate the hypoxic status in tumours through the d...
Source: Analytica Chimica Acta - Category: Chemistry Authors: Tags: Anal Chim Acta Source Type: research
Abstract Bleeding and thrombosis represent common complications in myeloproliferative neoplasms (MPN) and significantly contribute to morbidity and mortality. Molecular markers, including CALR mutations, were considered not only as diagnostic markers, but also as risk factors for bleeding and thrombosis associated with MPN, especially for patients in remote primary hospitals. We sought to develop an easy-to-use assay for the rapid detection of CALR type 1 (CALR-1) and type 2 (CALR-2) mutations in Philadelphia chromosome-negative MPN patients. Peptide nucleic acid-locked nucleic acid (PNA-LNA) clamping loop-mediate...
Source: Analytica Chimica Acta - Category: Chemistry Authors: Tags: Anal Chim Acta Source Type: research
In this study, we used MS to compare the endogenous metabolite level in the human MSCs originating from the bone marrow, adipose tissue, periodontal ligaments, and salivary glands. Using an optimized metabolomics technique, we verified that human MSCs exhibit differences in the endogenous metabolite level depending on their source material, while the multivariate analysis showed that 5 lysophosphatidylcholines and 3 lysophosphatidylethanolamines can serve as markers for the discrimination between MSC sources and may be related to differences in their differentiation capacity. These results may significantly contribute to f...
Source: Analytica Chimica Acta - Category: Chemistry Authors: Tags: Anal Chim Acta Source Type: research
The amazing moment a British transplant patient becomes one of the first to receive a ‘warm and beating’ donor heart has been captured for the first time by television cameras.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
“Paying attention in a particular way: on purpose, in the present moment and nonjudgmentally.” That’s how Jon Kabat-Zinn, PhD, describes mindfulness. In the book Zen Mind, Beginner’s Mind, another thought leader in mindfulness, Shunryu Suzuki, says that, “In the beginner’s mind there are many possibilities, but in the expert’s there are few.” In my experience, I’ve found that cultivating a beginner’s mind opens doors and improves clinical diagnosis. Medical training has phases, and clinicians in different phases think differently. In medical school, students learn...
Source: Kevin, M.D. - Medical Weblog - Category: General Medicine Authors: Tags: Physician Primary Care Source Type: blogs
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