Family History of Early Infant Death Correlates with Earlier Age at Diagnosis But Not Shorter Time to Diagnosis for Severe Combined Immunodeficiency

published_or_final_versio

Osteopontin as potential biomarker and therapeutic target in gastric and liver cancers

published_or_final_versio

Pathway to mental health recovery: a qualitative and quantitative study on the needs of Chinese psychiatric inpatients

published_or_final_versio

Klebsiella infection in patients with thalassemia

Klebsiella infection has previously been reported in a few patients with transfusion-dependent thalassemia. The incidence and clinical spectrum of this infection in our cohort of patients were reviewed retrospectively. Among 160 patients observed for 12 years, there were 15 episodes of Klebsiella infection that occurred in 12 patients (7.5%), resulting in an incidence of 0.78 infections per 100 patient-years. The clinical spectrum included sinusitis (4 cases), intracranial infection (5 cases), septicemia (4 cases), and abscesses of the liver, lung, kidney, and parotid gland (1 case each). Three patients had recurrent infections involving different sites, 2 (16%) died of fulminant septicemia, and 3 (25%) had significant permanent neurological deficits. The antibiotic susceptibility pattern for the isolates was similar to the pattern for isolates recovered in the community. With regard to predisposing factors, iron overload and liver function derangement were found to be significant on univariate analysis (P = .046 and P = .049, respectively) but insignificant on multivariate analysis. Klebsiella infection was a serious and frequently encountered complication in our patients with transfusion-dependent thalassemia, resulting in high mortality and morbidity rates.published_or_final_versio

The Embryotrophic Activity of Oviductal Cell-derived Complement C3b and iC3b, a Novel Function of Complement Protein in Reproduction

The oviduct-derived embryotrophic factor, ETF-3, enhances the development of trophectoderm and the hatching process of treated embryos. Monoclonal anti-ETF-3 antibody that abolishes the embryotrophic activity of ETF-3 recognized a 115-kDa protein from the conditioned medium of immortalized human oviductal cells. Mass spectrometry analysis showed that the protein was complement C3. Western blot analysis using an antibody against C3 confirmed the cross-reactivities between anti-C3 antibody with ETF-3 and anti-ETF-3 antibody with C3 and its derivatives, C3b and iC3b. Both derivatives, but not C3, were embryotrophic. iC3b was most efficient in enhancing the development of blastocysts with larger size and higher hatching rate, consistent with the previous reported embryotrophic activity of ETF-3. Embryos treated with iC3b contained iC3b immunoreactivity. The oviductal epithelium produced C3 as evidenced by the presence of C3 immunoreactivity and mRNA in the human oviduct and cultured oviductal cells. Cyclical changes in the expression of C3 immunoreactivity and mRNA were also found in the mouse oviduct with the highest expression at the estrus stage. Molecules involving in the conversion of C3b to iC3b and binding of iC3b were present in the human oviduct (factor I) and mouse preimplantation embryo (Crry and CR3), respectively. In conclusion, the present data showed that the oviduct produced C3/C3b, which was converted to iC3b to stimulate embryo development.postprin

Suppression of nickel release in nickeltitanium alloys by plasma immersion ion implantation surface treatment: towards a new generation of "smart" orthopaedic implants

Summary form only given. Nickel-titanium shape memory alloys (NiTi) are potentially very useful in spinal deformity correction due to their super elastic properties and their ability to change shape with temperature. However, release of toxic nickel particulate debris remains a major concern. We have developed a novel method of altering the surface of the material to reduce nickel release by using plasma immersion ion implantation (PIII). This study compares the corrosion resistance and mechanical properties of PIII treated samples with untreated NiTi. NiTi discs containing 50.8% Ni were implanted with nitrogen using PIII technique. Their elemental depth profile, surface chemical composition, surface hardness and corrosion resistance were compared with untreated NiTi. The amount of Ni released into simulated body fluids after the accelerated corrosion tests were determined. The biocompatibility was assessed by culturing mouse osteoblasts expressing an enhanced green fluorescent protein on the surface of these materials. After PIII treatment, a layer of titanium nitride formed on the surface. Compared to untreated NiTi, the corrosion resistance is better by five times, and the surface hardness and elastic modulus are better by a factor of 2. The concentration of Ni in the simulated body fluid for the untreated sample was 30 ppm compared to undetectable levels in the PIII treated sample. There was no difference in the ability of cells to grow on either surface. PIII results in enhanced corrosion and wear resistance, and negligible Ni release. This technique will allow NiTi alloys to be safely implanted in the human body. A new generation of "smart" orthopaedic implants will likely result.published_or_final_versio

New plasma surface-treated memory alloys: Towards a new generation of "smart" orthopaedic materials

This paper describes the corrosion resistance, surface mechanical properties, cyto-compatibility, and in-vivo performance of plasma-treated and untreated NiTi samples. Nickel-titanium discs containing 50.8% Ni were treated by nitrogen and carbon plasma immersion ion implantation (PIII). After nitrogen plasma treatment, a layer of stable titanium nitride is formed on the NiTi surface. Titanium carbide is also found at the surface after carbon plasma implantation. Compared to the untreated samples, the corrosion resistances of the plasma PIII samples are better by a factor of five and the surface hardness and elastic modulus are better by a factor of two. The concentration of Ni leached into the simulated body fluids from the untreated samples is 30 ppm, whereas that from the plasma-treated PIII are undetectable. Although there is no significant difference in the ability of cells to grow on either surface, bone formation is found to be better on the nitrogen and carbon PIII sample surfaces at post-operation 2 weeks. All these improvements can be attributed to the formation of titanium nitride and titanium carbide on the surface. © 2007 Elsevier B.V. All rights reserved.postprin

Measurement of cosmic-ray muons and muon-induced neutrons in the Aberdeen Tunnel Underground Laboratory

postprin

Next-generation sequencing with a myeloid gene panel in core-binding factor AML showed KIT activation loop and TET2 mutations predictive of outcome

Clinical outcome and mutations of 96 core-binding factor acute myeloid leukemia (AML) patients 18-60 years old were examined. Complete remission (CR) after induction was 94.6%. There was no significant difference in CR, leukemia-free-survival (LFS) and overall survival (OS) between t(8;21) (N=67) and inv(16) patients (N=29). Univariate analysis showed hematopoietic stem cell transplantation at CR1 as the only clinical parameter associated with superior LFS. Next-generation sequencing based on a myeloid gene panel was performed in 72 patients. Mutations in genes involved in cell signaling were associated with inferior LFS and OS, whereas those in genes involved in DNA methylation were associated with inferior LFS. KIT activation loop (AL) mutations occurred in 25 patients, and were associated with inferior LFS (P=0.003) and OS (P=0.001). TET2 mutations occurred in 8 patients, and were associated with significantly shorter LFS (P=0.015) but not OS. Patients negative for KIT-AL and TET2 mutations (N=41) had significantly better LFS (P<0.001) and OS (P=0.012) than those positive for both or either mutation. Multivariate analysis showed that KIT-AL and TET2 mutations were associated with inferior LFS, whereas age ⩾40 years and marrow blast ⩾70% were associated with inferior OS. These observations provide new insights that may guide better treatment for this AML subtype.published_or_final_versio

An apparatus for studying spallation neutrons in the Aberdeen Tunnel laboratory

postprin