Hypoglycosylation is a common finding in antithrombin deficiency in the absence of a SERPINC1 gene defect.

Unlabelled: Essentials We investigated the molecular base of antithrombin deficiency in cases without SERPINC1 defects. 27% of cases presented hypoglycosylation, transient in 62% and not restricted to antithrombin. Variations in genes involved in N-glycosylation underline this phenotype.

Inflammation and the bone-vascular axis in end-stage renal disease.

Unlabelled: Bone loss and vascular calcification coincide in patients with end-stage renal disease, similar as to what is observed in the general population. In the present bone biopsy study, we provide further evidence that (micro-)inflammation may represent a common soil for both diseases.

Introduction: Vascular calcification is a common complication of end-stage renal disease (ESRD) and is predictive of subsequent cardiovascular disease and mortality.

General practitioners' judgement of chronic heart failure in the oldest old: Insights from the BELFRAIL study.

Background And Objectives: Conflicting evidence exists about the value of general practitioners' (GPs') diagnoses of chronic heart failure (CHF), especially in older persons. Therefore, the relationship between GPs' judgement of CHF and objective cardiac abnormalities and their respective prognostic value for 5-year mortality in patients aged 80 and older was studied.

Methods And Results: These analyses were embedded within the prospective, population-based BELFRAIL study.

Plasma GAD65, a Marker for Early β-Cell Loss After Intraportal Islet Cell Transplantation in Diabetic Patients.

Context And Objective: Intraportal islet transplantation can restore insulin production in type 1 diabetes patients, but its effect is subject to several interfering processes. To assess the influence of β-cell loss before and during engraftment, we searched for a real-time marker of β-cell destruction. Previous studies showed that 65-kDa isoform of glutamate decarboxylase (GAD65) is discharged by chemically damaged rat β-cells.

Renal Ischemia and Function After Partial Nephrectomy: A Collaborative Review of the Literature.

Context: Partial nephrectomy (PN) is the current gold standard treatment for small localized renal tumors.; however, the impact of duration and type of intraoperative ischemia on renal function (RF) after PN is a subject of significant debate.

Objective: To review the current evidence on the relationship of intraoperative ischemia and RF after PN.

High numbers of mobilized CD34+ cells collected in AML in first remission are associated with high relapse risk irrespective of treatment with autologous peripheral blood SCT or autologous BMT.

The faster hematopoietic recovery after autologous peripheral blood SCT (APBSCT) in patients with AML may be offset by an increased relapse risk as compared with autologous BMT (ABMT). The EORTC and GIMEMA Leukemia Groups conducted a trial (AML-10) in which they compared, as second randomization, APBSCT and ABMT in first CR patients without an HLA compatible donor. A total of 292 patients were randomized.

Randomized phase III trial of amrubicin versus topotecan as second-line treatment for patients with small-cell lung cancer.

Purpose: Amrubicin, a third-generation anthracycline and potent topoisomerase II inhibitor, showed promising activity in small-cell lung cancer (SCLC) in phase II trials. This phase III trial compared the safety and efficacy of amrubicin versus topotecan as second-line treatment for SCLC.

Patients And Methods: A total of 637 patients with refractory or sensitive SCLC were randomly assigned at a ratio of 2:1 to 21-day cycles of amrubicin 40 mg/m(2) intravenously (IV) on days 1 to 3 or topotecan 1.

Mechanical biocompatibility of prosthetic meshes: a comprehensive protocol for mechanical characterization.

This study is aimed at a comprehensive and extensive characterization of the mechanical biocompatibility of mesh prostheses. A robust and simple experimental protocol and a set of parameters is proposed, addressing stiffness under uniaxial and biaxial loading conditions, anisotropy, influence of prior deformation history, local mismatch of deformation mechanisms, and changes in mechanical properties when embedded in a homogeneous matrix. These parameters can form the basis for comparison of different mesh types and for evaluation of their mechanical biocompatibility.

Contemporary role of salvage lymphadenectomy in patients with recurrence following radical prostatectomy.

Context: Prostate cancer (PCa) patients with isolated clinical lymph node (LN) relapse, limited to the regional and/or retroperitoneal LNs, may represent a distinct group of patients who have a more favorable outcome than men with progression to the bone or to other visceral organs. Some data indirectly denote a beneficial impact of pelvic LN dissection on survival in these patients.

Objective: To provide an overview of the currently available literature regarding salvage LN dissection (SLND) in PCa patients with clinical relapse limited to LNs after radical prostatectomy (RP).

Adequate iron chelation therapy for at least six months improves survival in transfusion-dependent patients with lower risk myelodysplastic syndromes.

Background: Most patients with myelodysplastic syndromes (MDS) require transfusions at the risk of iron overload and associated organ damage, and death. Emerging evidence indicates that iron chelation therapy (ICT) could reduce mortality and improve survival in transfusion-dependent MDS patients, especially those classified as International Prognostic Scoring System (IPSS) Low or Intermediate-1 (Low/Int-1).

Methods: Follow-up of a retrospective study.

Proteomic analysis of platelet N-glycoproteins in PMM2-CDG patients.

Unlabelled: PMM2-CDG, the most frequent congenital disorder of N-glycosylation, is an autosomal recessive disease with a multisystem presentation. PMM2-CDG patients show an increased risk for thrombosis, which might be in part due to spontaneous platelet aggregations as previously described. A potential hypoglycosylation of platelet proteins in these patients might explain this increased reactivity, as removal of sialic acid from platelets, particularly of GPIbα, leads to enhance platelet aggregation and clearance from the circulation.

The site and nature of airway obstruction after lung transplantation.

Rationale: The chronic rejection of lung allografts is attributable to progressive small airway obstruction.

Objectives: To determine precisely the site and nature of this type of airway obstruction.

Methods: Lungs from patients with rejected lung allografts treated by a second transplant (n = 7) were compared with unused donor (control) lungs (n = 7) using multidetector computed tomography (MDCT) to determine the percentage of visible airways obstructed in each airway generation, micro-computed tomography (microCT) to visualize the site of obstruction, and histology to determine the nature of this obstruction.

Outcome following laparoscopic and open total mesorectal excision for rectal cancer.

Background: There are few reports on the oncological quality of resection and outcome after laparoscopic versus open total mesorectal excision (TME) for rectal cancer in everyday surgical practice.

Methods: Between January 2006 and October 2011, data for patients with mid or low rectal adenocarcinoma who underwent elective TME were recorded in the PROCARE database. A multivariable model and the propensity score as a co-variable in Cox or logistic regression models were used for adjustment of differences in patient mix and non-random assignment of surgical approach.

New array approaches to explore single cells genomes.

Microarray analysis enables the genome-wide detection of copy number variations and the investigation of chromosomal instability. Whereas array techniques have been well established for the analysis of unamplified DNA derived from many cells, it has been more challenging to enable the accurate analysis of single cell genomes. In this review, we provide an overview of single cell DNA amplification techniques, the different array approaches, and discuss their potential applications to study human embryos.

[Lipoblastoma and lipoblastomatosis: especially in children].

Lipoblastoma and lipoblastomatosis are rare benign fatty tumours that mainly occur in children under the age of 3 years. Several body sites can be affected. The term 'lipoblastoma' is reserved for an encapsulated neoplasm; 'lipoblastomatosis' for tumours demonstrating infiltrative growth.

New strategies and designs in pancreatic cancer research: consensus guidelines report from a European expert panel.

Although the treatment of pancreatic ductal adenocarcinoma (PDAC) remains a huge challenge, it is entering a new era with the development of new strategies and trial designs. Because there is an increasing number of novel therapeutic agents and potential combinations available to test in patients with PDAC, the identification of robust prognostic and predictive markers and of new targets and relevant pathways is a top priority as well as the design of adequate trials incorporating molecular-driven hypothesis. We presently report a consensus strategy for research in pancreatic cancer that was developed by a multidisciplinary panel of experts from different European institutions and collaborative groups involved in pancreatic cancer.

Persistence of polypropylene mesh anisotropy after implantation: an experimental study.

Objective: To determine whether anisotropy persisted after incorporation into the host, using a standardised rabbit model for abdominal wall reconstruction.

Design: Investigator-initiated prospective-controlled experimental study.

Setting: Centre for Surgical Technologies, Medical Faculty KU-Leuven.

Shrinkage and biomechanical evaluation of lightweight synthetics in a rabbit model for primary fascial repair.

Introduction And Hypothesis: The experiment evaluated different lightweights (<32 g/m(2)) in terms of shrinkage and biomechanics.

Methods: PP-8 (polypropylene of 7.6 g/m(2)), PP-s (PP-8 with absorbable sheets), PP-32 (PP with absorbable fibers; 32.

Congenital disorders of glycosylation (CDG): it's (nearly) all in it!

Congenital disorders of glycosylation (CDG) is a booming class of metabolic diseases. Its number has increased nearly fourfold (to 45) since 2003, the year of the Komrower lecture, entitled 'Congenital disorders of glycosylation CDG): It's all in it!'. This paper presents an overview of recently discovered CDG and CDG phenotypes, of a diagnostic approach, of (the lack of) treatment, of CDG genetics, of a novel CDG nomenclature and classification, and of some future directions in the CDG field.

Malignant involvement of the knee synovium in a patient with metastastic oesophageal adenocarcinoma.

We describe the case of a malignant arthritis of the knee. This is the first description with an oesophageal adenocarcinoma being the primary site of the malignancy. Diagnosis of this rare condition can be made by cytological examination of joint fluid or by synovial biopsy.

Niacin, an old drug with new perspectives for the management of dyslipidaemia.

Niacin has been used for decades to treat dyslipidaemic disorders. Niacin is the most effective agent currently available for increasing levels of high-density lipoprotein. Moreover, significant improvements in cardiovascular outcomes in niacin treated patients have been demonstrated.