The effectiveness and cost-effectiveness of enzyme and substrate replacement therapies: a longitudinal cohort study of people with lysosomal storage disorders.

Objectives: To determine natural history and estimate effectiveness and cost of enzyme replacement therapy (ERT) and substrate replacement therapy (SRT) for patients with Gaucher disease, Fabry disease, mucopolysaccharidosis type I (MPS I), mucopolysaccharidosis type II (MPS II), Pompe disease and Niemann-Pick type C (NPC) disease.

Design: Cohort study including prospective and retrospective clinical- and patient-reported data. Age- and gender-adjusted treatment effects were estimated using generalised linear mixed models.

The mediating effect of the Mediterranean diet on the role of discretionary and hidden salt intake regarding non-fatal acute coronary syndrome or stroke events: case/case-control study.

Objectives: The aim of the present work was to evaluate the association between salt and salty food consumption on the development of an acute coronary syndrome (ACS) or ischemic stroke, under the context of adherence to the Mediterranean diet.

Methods: During 2009-2010, 1000 participants were enrolled; 250 were consecutive patients with a first ACS, 250 were consecutive patients with a first ischemic stroke and 500 population-based, control subjects, one-for-one matched to the patients by age and sex. Socio-demographic, clinical, psychological, dietary and other lifestyle characteristics were measured.

Presence of cobalt and chromium ions in the seminal fluid of young patients with metal-on-metal total hip arthroplasty.

We aimed to investigate the effect of metal ions on the semen of males of child fathering age with metal-on-metal (MM) total hip arthroplasty (THA). Semen was collected form 11 patients with MM THA and 5 control of comparable age. Cobalt and chromium concentrations were measured in both the seminal plasma and in the blood of patients.

Azithromycin pulses for the treatment of epidermal growth factor receptor inhibitor-related papulopustular eruption: an effective and convenient alternative to tetracyclines.

Background: Papulopustular eruption (PPE) is the most common cutaneous side effect of epidermal growth factor receptor inhibitors (EGFRIs).

Objective: To document the efficacy and safety of pulsed azithromycin doses in the treatment of EGFRI-related PPE.

Methods: A retrospective analysis of patients under EGFRIs who exhibited at least grade 2 PPE and were intolerant or resistant to tetracyclines was performed.

Modelling eating practices in non-fatal acute coronary syndrome or stroke development: a case/case-control study.

Background And Aims: Although significant evidence exists regarding the role of specific foods and dietary patterns on the development of cardiovascular disease, the influence of eating practices has not been thoroughly examined and understood. The aim of the present work was to evaluate the independent role of eating practices on the likelihood of developing an acute coronary syndrome (ACS) or ischemic stroke.

Methods And Results: During 2009-2010, 1000 participants were enrolled; 250 were consecutive patients with a first ACS, 250 were consecutive patients with a first ischemic stroke and 500 were population-based control subjects (250 age-sex matched one-for-one with ACS patients, and 250 age-sex matched one-for-one with stroke patients).

A prospective randomised controlled trial comparing three alternative bearing surfaces in primary total hip replacement.

The ideal bearing surface for young patients undergoing total hip replacement (THR) remains controversial. We report the five-year results of a randomised controlled trial comparing the clinical and radiological outcomes of 102 THRs in 91 patients who were < 65 years of age. These patients were randomised to receive a cobalt-chrome on ultra-high-molecular-weight polyethylene, cobalt-chrome on highly cross-linked polyethylene, or a ceramic-on-ceramic bearing.

Potential impact of the financial crisis on outpatient hospital visits due to otorhinolaryngologic disorders in Crete, Greece.

The public health effect of financial crises has been emphasized in previous studies. In addition, a series of otorhinolaryngologic disorders and manifestations has been related to psychological factors in the literature. Such conditions include temporomandibular joint disorders, laryngopharyngeal reflux, chronic tinnitus, and vertigo.

The effect of angular mismatch between vertebral endplate and vertebral body replacement endplate on implant subsidence.

Study Design: Comparative biomechanical study.

Objective: To determine whether an angular mismatch between the vertebral body replacement (VBR) endplate and the simulated foam vertebral endplate leads to accelerated subsidence in a cyclic compression model of the VBR-vertebra interface.

Summary Of Background Data: One of the main complications of the VBR surgery is postoperative subsidence and collapse of the VBR implant into the adjacent vertebral bodies.

Melanoma: new insights and new therapies.

Metastatic melanoma has historically been considered as one of the most therapeutically challenging malignancies. However, for the first time after decades of basic research and clinical investigation, new drugs have produced major clinical responses. The discovery of BRAF mutations in melanoma created the first opportunity to develop oncogene-directed therapy in this disease and led to the development of compounds that inhibit aberrant BRAF activity.

Functional outcome and complications using the intramedullary hip screw for intertrochanteric fractures.

We prospectively studied 110 consecutive patients with intertrochanteric hip fractures treated with the 130 degree angle, 10-mm short IMHS intramedullary hip screw (IMHS, Smith & Nephew, Richards, Memphis, TN). Surgery was performed within 36 hours from admission; all patients were mobilized immediately postoperatively. Fracture union, pre- and post-operative mobility status and complications were evaluated.

Adherence to the mediterranean diet in relation to ischemic stroke nonfatal events in nonhypercholesterolemic and hypercholesterolemic participants: results of a case/case-control study.

The aim of the present work was to evaluate the association between adherence to the Mediterranean diet and the development of ischemic stroke according to cholesterol levels. During 2009-2010, 500 participants were enrolled; 250 were consecutive patients (77 ± 9 years, 55.6% men) with a first ischemic stroke and 250 population-based, control participants, matched to the patients by age and sex.

Metal-on-metal total hip arthroplasty - five- to 11-year follow-up.

Metal-on-metal (MoM) total hip arthroplasty (THA) has been introduced in an attempt to reduce the wear rate and the consequent osteolysis around implants. The aim of this study was to present the intermediate to long-term clinical and radiological outcomes and to investigate the metal ion levels in the blood of patients who had undergone primary uncemented MoM THA in our institution. Between July 1997 and November 2003, 166 patients (193 hips), with a mean age of 50 years (range, 18-65 years), underwent primary MoM THA.

Adherence to the Mediterranean diet in relation to acute coronary syndrome or stroke nonfatal events: a comparative analysis of a case/case-control study.

Background: Although the role of Mediterranean diet on cardiovascular disease prevention has long been evaluated and understood, its association with the development of stroke has been rarely examined. The aim of the present work was to comparatively evaluate the association between adherence to the Mediterranean diet and the development of an acute coronary syndrome (ACS) or ischemic stroke.

Methods: During the period from 2009 to 2010, 1,000 participants were enrolled; 250 were consecutive patients with a first ACS, 250 were consecutive patients with a first ischemic stroke, and 500 population-based, control subjects, 1-for-1 matched to the patients by age and sex.

Use of femoral shaft fracture classification for predicting the risk of associated injuries.

Objectives: To investigate the hypothesis that specific fracture patterns in patients with femoral shaft fractures can predict the likelihood of associated injuries.

Design: Retrospective cohort study.

Setting: Level I trauma center.

Comprehensive mutational analysis of CDKN2A and CDK4 in Greek patients with cutaneous melanoma.

Background: The penetrance of CDKN2A mutations is subject to geographical and latitudinal variation and is presumably dictated by ultraviolet radiation exposure and possibly other co-inherited genetic factors. The frequency of mutations increases with the number of family members affected and the number of primary tumours, and also fluctuates with geography. To date, little is known about the prevalence of CDKN2A mutations in patients with melanoma from Greece.

Epidemiologic risk factors of basal cell carcinoma development and age at onset in a Southern European population from Greece.

Background: Basal cell carcinoma (BCC) is the most common form of skin cancer with increasing incidence rates worldwide.

Methods: To assess the association of BCC with epidemiologic risk factors in a Southern European population from Greece, we conducted a hospital-based case-control study of 199 patients with BCC and 200 controls.

Results: In the multivariate analysis, fair skin colour was associated with increased risk of BCC (OR: 4.

Inflammatory pseudotumor complicating metal-on-highly cross-linked polyethylene total hip arthroplasty.

Inflammatory masses or cysts occurring in the pelvis, thigh, and gluteal regions, often mimicking infection, occasionally arise after total hip arthroplasty procedures. Inflammatory pseudotumors comprise a subgroup of these lesions. Pseudotumors have been associated with pain, rashes, instability, neuropathy, and premature loosening of prosthetic components, often leading to early and difficult revision surgery.

Experimental osteomyelitis caused by methicillin-resistant Staphylococcus aureus treated with a polylactide carrier releasing linezolid.

Background: The effectiveness of a new delivery system consisting of polymerized dilactide (PLA) with incorporated linezolid was investigated in a rabbit model as a means of treating methicillin-resistant Staphylococcus aureus (MRSA) osteomyelitis.

Methods: The PLA-linezolid system was prepared after thorough stirring of PLA with linezolid at a 10:1 ratio. Experimental osteomyelitis was established in 40 rabbits by a modification of the Norden model with MRSA as the test isolate.

Intra-articular morphine enhances analgesic efficacy of ropivacaine for knee arthroscopy in ambulatory patients.

The aim of this double-blind, randomized control trial was to compare the effectiveness of intra-articular ropivacaine alone or with morphine or ketoprofen for controlling pain after arthroscopic knee surgery. One hundred fifty-six patients scheduled for elective knee arthroscopy were recruited. All patients received general anesthesia and were randomly assigned to 4 groups to receive intra-articular ropivacaine 40 mg (group R), ropivacaine 24 mg plus morphine 8 mg (group R+M), ropivacaine 36 mg plus ketoprofen 100 mg (group R+K), or normal saline (group N/S).

Proximal tibial fractures: early experience using polyaxial locking-plate technology.

Between 2004 and 2009, 60 patients with proximal tibial fractures were included in this prospective study. All fractures were treated with the polyaxial locked-plate fixation system (DePuy, Warsaw, IN, USA). Clinical and radiographic data, including fracture pattern, changes in alignment, local and systemic complications, hardware failure and fracture union were analysed.

Total knee replacement and chemical thromboprophylaxis: current evidence.

Thromboembolic disease remains one of the most devastating and potentially lethal complications after elective total knee replacement (TKR) surgery. Studies have shown that 40-85% of patients undergoing TKR will develop venographically confirmed deep vein thrombosis (DVT) if they are not given any type of post-operative thromboprophylaxis and approximately 0.1 to 1.

Novel anticoagulants: new evidence for emerging drugs and their potential application in major lower limb surgery.

For decades, parenteral drugs, such as the low molecular weight heparins and unfractionated heparins or vitamin K antagonists, have been used as anticoagulants for prevention of venous thromboembolism following major lower limb surgery. However, these regiments have limitations that rendered the quest for new anticoagulants mandatory. Recently, research has been focused on the development of orally active small molecules that directly target thrombin or activated factor X (FXa).

PUVA plus interferon α2b in the treatment of advanced or refractory to PUVA early stage mycosis fungoides: a case series.

Background:   The combination of PUVA with variable doses of systemically administered interferon α2b (IFN-α2b) reduces the number of PUVA treatments and the dose of IFN-α2b required to produce remission in all mycosis fungoides (MF) stages.

Objectives:   To evaluate the efficacy of the combination of PUVA and IFN-α2b in patients with late stage or refractory to treatment early stage MF.

Methods:   The combination of PUVA three times weekly and IFN-α2b 2-5 MU three times weekly was retrospectively reviewed in 22 patients.