Clinical efficacy of the enzyme replacement therapy in patients with late-onset Pompe disease: a systematic review and a meta-analysis.

In patients with late-onset Pompe disease (LOPD), the efficacy of the enzyme replacement therapy (ERT) with recombinant human alpha-glucosidase (rhGAA) is difficult to evaluate, due to the clinical heterogeneity and the small sample sizes in published studies. Therefore, we conduct a systematic literature review and meta-analysis of the literature to evaluate the efficacy of ERT in LOPD patients considering the walking distance, respiratory function and muscle strength. Particularly, six-minute walk test (6MWT), forced vital capacity (FVC), medical research council (MRC) grading, quantitative muscle testing (QMT), and quick motor function test (QMFT) were outcomes of interest.

Preparedness of Frontline Doctors in Jordan Healthcare Facilities to COVID-19 Outbreak.

The number of COVID-19 (Coronavirus Disease of 2019) cases in Jordan is rising rapidly. A serious threat to the healthcare system appears on the horizon. Our study aims to evaluate preparedness of Jordanian frontline doctors to the worsening scenario.

Sexual and urinary function following anterior lumbar surgery in females.

Aims: Anterior lumbar interbody fusion procedures (ALIF) and total disc replacement (TDR) with anterior exposure of the lumbar spine entail a risk of a vascular injury and dysfunction of the sympathetic and parasympathetic nerves due to disturbance of the inferior and superior hypogastric plexus. While retrograde ejaculation is a known complication of the anterior spinal approach in males, post-operative sexual as well as urinary function in females has not yet been thoroughly investigated and was hence the aim of this study.

Methods: Fifteen female patients documented their sexual and urinary function preoperatively, 3 months and 6 months postoperatively, using the validated questionnaires FSFI (Female Sexual Function Index) and ICIQ (International Consultation of Incontinence Questionnaire).

Regional Versus General Anesthesia: Effect of Anesthetic Techniques on Clinical Outcome in Lumbar Spine Surgery: A Prospective Randomized Controlled Trial.

Background: There are only a few prospective clinical trials investigating the effects of different anesthetic techniques on clinical outcomes after lumbar spine surgery. The purpose of this study was to evaluate clinical outcomes in patients receiving general (GA) and regional anesthesia (RA) for lumbar spine surgery.

Methods: This was a single-center, 2-arm, trial in which 100 patients undergoing lumbar spine surgery were randomized to receive either RA or GA (50 per group).

Death receptor-independent FADD signalling triggers hepatitis and hepatocellular carcinoma in mice with liver parenchymal cell-specific NEMO knockout.

Hepatocellular carcinoma (HCC) usually develops in the context of chronic hepatitis triggered by viruses or toxic substances causing hepatocyte death, inflammation and compensatory proliferation of liver cells. Death receptors of the TNFR superfamily regulate cell death and inflammation and are implicated in liver disease and cancer. Liver parenchymal cell-specific ablation of NEMO/IKKγ, a subunit of the IκB kinase (IKK) complex that is essential for the activation of canonical NF-κB signalling, sensitized hepatocytes to apoptosis and caused the spontaneous development of chronic hepatitis and HCC in mice.