The Ultra-nanocrystalline diamond (UNCD) was synthesized by the detonation of a high explosive mixture in water confinement. The presence of a diamond phase was revealed by X-ray diffraction (XRD) and transmission electron microscopy (TEM). X-ray line broadening (XRLB) was used to evaluate the peak profiles of diamond nanoparticles and their corresponding average crystallite sizes. The micro st...

We report the discovery of vertically aligned nano-scale diamond tips grown on the polycrystalline diamond substrates in a CH4/N2/H2 mixed plasma. The best diamond quality occurs at the top part of the nanotip, which is single crystalline diamond. The quality of the diamond nanotip is justified by the diamond characteristic peak at 1332 cm 1 in the Raman spectrum, the discrete electron diffract...

(3094 articles) Hematopoiesis and Stem Cells • (1597 articles) Free Research Articles • (3667 articles) Clinical Trials and Observations • Articles on similar topics can be found in the following Blood collections http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found online at: http:...

Shwachman-Diamond syndrome is a rare recessive genetic disease caused by mutations in SBDS gene, at chromosome 7q11. Phenotypically, the syndrome is characterized by exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal dysplasia and variable cognitive impairments. Structural brain abnormalities (smaller head circumference and decreased brain volume) have also been reported. No c...

9 Conclusions: This study expanded the phenotypic features of SDS to include primary low-turnover osteoporosis and structural alterations in the brain. Pancreatic MRI showed characteristic changes in the SBDS mutation-positive patients while these were absent in the mutation-negative patients, suggesting that MRI can be used to differentiate patients harboring SBDS mutations from those without ...

The profound anemia of Diamond-Blackfan syndrome (DBS) is due to marrow red cell failure, but the pathogenesis is not understood. Studies by others indicated cell-mediated erythropoietic suppression in this condition. To explore this mechanism further, Ficoll-Hypaque--separated peripheral blood lymphocytes (PBL) from four anemic untreated patients with DBS, or from normals were cocultured with ...

Exocrine pancreatic insufficiency which is not due to cystic fibrosis is a diagnostic dilemma until attention is paid to the other systemic findings. We present such a child where focusing on other systems helped us reach a diagnosis. A one year one month old male child was brought to us with onset of complaints since the first few months of life. Parents complained of failure to thrive since b...

Shwachman-Diamond syndrome (SDS) is an autosomal-recessive marrow failure syndrome with a predisposition to leukemia. SDS patients harbor biallelic mutations in the SBDS gene, resulting in low levels of SBDS protein. Data from nonhuman models demonstrate that the SBDS protein facilitates the release of eIF6, a factor that prevents ribosome joining. The complete abrogation of Sbds expression in ...

Deficiencies in the SBDS gene result in Shwachman-Diamond syndrome (SDS), an inherited bone marrow failure syndrome associated with leukemia predisposition. SBDS encodes a highly conserved protein previously implicated in ribosome biogenesis. Using human primary bone marrow stromal cells (BMSCs), lymphoblasts, and skin fibroblasts, we show that SBDS stabilized the mitotic spindle to prevent gen...

A10-year boy presented with spontaneous episodes of oral bleeding for the last 6 months. Detailed ENT examination showed no pathology, bleeding profile was normal, endoscopy and dental examination also did not reveal any abnormality. Child abuse or malingering was also ruled out. Initially the child was managed with platelet transfusion and fresh frozen plasma and then put on follow-up treatmen...