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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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Aneurysms of the renal arteries are rare, probably under-diagnosed, with an incidence estimated at 0.01%. A size exceeding 20mm and the presence of symptoms such as hypertension represent a therapeutic indication for most authors. Endovascular techniques are used more and more to treat proximal lesions and auto-transplantation is the technique of reference for intra-parenchymatous lesions. However, most hilar, non-intra-parenchymatous lesions can be treated in situ without cooling and with a reasonable clamping time.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Tags: Abstracts Presented to the French Vascular Surgery Society Source Type: research
The objective of this work was to study the mid-term results of the angioplast y of the renal artery on transplanted kidneys.
Source: Annals of Vascular Surgery - Category: Surgery Authors: Tags: Abstracts Presented to the French Vascular Surgery Society Source Type: research
We describe a 61-year-old African American diabetic gentleman who presented with nodules in a linear distribution on the flank. Histopathologic examination of a biopsied nodule revealed a pandermal sheet-like infiltrate of plasma cells and histiocytes, some demonstrating elastophagocytosis and emperipolesis. The lesional histiocytes were S100 and CD68 positive and CD1a negative—findings consistent with a diagnosis of CRDD. Additional laboratory work-up performed 12 weeks after the biopsy was taken revealed an elevated serum κ light chain concentration of 37.26 mg/L (reference range: 3.30–19.40 mg/L), whic...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Extraordinary Case Report Source Type: research
Conclusion: This case may illustrate a resemblance in the renal glomerulus basement membrane and retinal pigment epithelium–Bruch membrane complex, because the authors observed deposits of excess monoclonal kappa chains manifesting as extracellular, proteinaceous aggregates on the basement membrane of the glomerulus, and striking, globular subretinal deposits that overlay a thickened retinal pigment epithelium–Bruch membrane complex. The ocular lesions' refractoriness to intravitreal treatments could be attributed to the fact that they represent proteinaceous aggregates similar to those documented in the glomer...
Source: Retinal Cases and Brief Reports - Category: Opthalmology Tags: Case Report Source Type: research
Objectives: We aimed to explore the sexual and reproductive health attitudes, beliefs and behaviors of African Americans with sickle cell disease and understand the interplay of the various factors that influence their attitudes, beliefs and behavior.
Source: Contraception - Category: OBGYN Authors: Source Type: research
Abstract: Screening for iron deficiency anemia (IDA) in all pregnant women is recommended. IDA is a prevalent cause of nutritional deficiency anemia, and oral iron is the first line of treatment. Other treatments include parenteral iron or blood transfusion(s). Untreated IDA in pregnancy can result in complications for the mother and fetus.
Source: The Nurse Practitioner - Category: Nursing Tags: Feature: IRON DEFICIENCY ANEMIA Source Type: research
Source: American Journal of Hematology - Category: Hematology Authors: Tags: Correspondence Source Type: research
Authors: Wang L, Yu X, Liu JP Abstract Telomeres at the ends of chromosomes safeguard genome integrity and stability in human nucleated cells. However, telomere repeats shed off during cell proliferation and other stress responses. Our recent studies show that telomere attrition induces not only epithelial stem cell senescence but also low-grade inflammation in the lungs. The senescence-associated low-grade inflammation (SALI) is characteristic of alveolar stem cell replicative senescence, increased proinflammatory and anti-inflammatory cytokines, infiltrated immune cells, and spillover effects. To date, the mechan...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Continuous-flow ventricular assist devices (CF-VAD) have been widely used in end-stage heart failure in adult patients for both bridge to transplantation and destination therapy. Technology improvement has enabled miniaturization of the CF-VAD, which may greatly benefit the pediatric patients with sufficient body size in advanced heart failure. There are still, however, challenging situations for CF-VAD implantation, such as in single ventricle physiology, anatomically abnormal heart, small children and infants.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Source Type: research
Background: Severe heart failure (HF) patients often have renal dysfunction because of low cardiac output. Methods and Results: 39 patients underwent a centrifugal-type, continuous-flow left ventricular assist device (LVAD) (EVAHEART) implantation as a bridge to cardiac transplantation at our institute between 2005 and 2015. We investigated the changes in renal function over 2 years among 36 patients with supported by EVAHEART over 6 months. The median estimated glomerular filtration rate (eGFR) at implantation was 62  ± 32 mL/min/1.73 m2 and 47% of the patients showed eGFR 
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Source Type: research
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