Retrospective evaluation of patients with X-linked adrenoleukodystrophy with a wide range of clinical presentations: a single center experience.

Objectives: X-linked adrenoleukodystrophy (X-ALD), is a peroxisomal inborn error of metabolism caused due to the loss of function variants of gene that leads to accumulation of very long chain fatty acids (VLCFAs) in several tissues including the neurological system. Childhood cerebral X-ALD (CCALD) is the most common and severe form of X-ALD, if left untreated. Allogenic hematopoietic stem cell transplantation (HSCT) is the only available therapy that halts neurological deterioration in CCALD.

Determinants of reverse dipping blood pressure in normotensive, non-diabetic population with an office measurement below 130/85mmHg.

: The role of dipping blood pressure pattern in normotensives is unclear. The study aims to search the circadian blood pressure rhythm and the clinical determinants related to reverse dipping pattern in a strictly selected, normotensive population.: The study population was divided into three groups depending on the nocturnal dipping pattern as dipping, non-dipping, and reverse dipping.

Evaluation of clinical, endocrine and metabolic findings in obese children with and without hepatosteatosis.

Objectives: Non-alcoholic fatty liver disease (NAFLD) is a common obesity-related comorbidity in childhood. In this study, we aimed to evaluate predictors of NAFLD by comparing clinical, endocrine and metabolic findings in obese children with and without hepatosteatosis.

Methods: Two hundred and eight obese children aged 6-18 years were included.

The Effect of Discharging Patients with Low Hemoglobin Levels on Hospital Readmission and Quality of Life after Adult Spinal Deformity Surgery.

Study Design: Retrospective cohort.

Purpose: This study aims to evaluate the impact of anemia on functional outcomes, health-related quality of life (HRQoL), and early hospital readmission (EHR) rates after adult spinal deformity (ASD) surgery at the time of discharge from the hospital.

Overview Of Literature: Concerns with risks of transfusion, insufficient evidence for its benefits, and the possibility of associated adverse outcomes have led to restrictive transfusion practices.

Improved Outcomes of Haploidentical Hematopoietic Cell Transplantation with Total Body Irradiation-Based Myeloablative Conditioning in Acute Lymphoblastic Leukemia.

The optimal myeloablative conditioning (MAC) for patients undergoing haploidentical hematopoietic cell transplantation (haplo-HCT) is unknown. We studied the outcomes of total body irradiation (TBI)-based versus chemotherapy (CT)-based MAC regimens in patients with acute lymphoblastic leukemia (ALL). The study included 427 patients who underwent first haplo-HCT with post-transplantation cyclophosphamide (PTCy), following TBI-based (n = 188; 44%) or CT-based (n = 239; 56%) MAC.

ERS International Congress 2020: highlights from the Thoracic Surgery and Transplantation Assembly.

The Thoracic Surgery and Lung Transplantation Assembly of the European Respiratory Society is delighted to present the highlights from the 2020 Virtual International Congress. We have selected four sessions that discussed recent advances in a wide range of topics. From the use of robotic surgery in thoracic surgery and extracorporeal life support as a bridge to lung transplantation, to lung transplantation in the era of new drugs.

Aortic arch calcification: a novel parameter for prediction of masked hypertension.

Background: Masked hypertension is directly related to increased cardiovascular morbidity and mortality but remains underdiagnosed in clinical practice. The aim of the study was to search the role of aortic arch calcification (AAC) in the diagnosis of masked hypertension.

Methods And Results: Among the patients who underwent ambulatory blood pressure monitoring (ABPM) in our clinic, those with office blood pressure (OBP) <140/90 mmHg were included in the study population.

Real-world efficacy and safety of Ledipasvir + Sofosbuvir and Ombitasvir/Paritaprevir/Ritonavir ± Dasabuvir combination therapies for chronic hepatitis C: A Turkish experience.

Background/aims: This study aimed to evaluate the real-life efficacy and tolerability of direct-acting antiviral treatments for patients with chronic hepatitis C (CHC) with/without cirrhosis in the Turkish population.

Material And Methods: A total of 4,352 patients with CHC from 36 different institutions in Turkey were enrolled. They received ledipasvir (LDV) and sofosbuvir (SOF)±ribavirin (RBV) orombitasvir/paritaprevir/ritonavir±dasabuvir (PrOD)±RBV for 12 or 24 weeks.

Prognostic utility of the assessment of diastolic function in patients undergoing cardiac resynchronization therapy.

Conflicting data exist about the relationship between cardiac resynchronization therapy (CRT) and diastolic function. Aims of the study are to assess diastolic patterns in patients undergoing CRT according to the 2016 recommendations of the American Society of Echocardiography/European Association of Cardiovascular Imaging and to evaluate the prognostic value of diastolic dysfunction (DD) in CRT candidates. METHODS AND RESULTS: One-hundred ninety-three patients (age: 67 ± 11 years, QRS width: 167 ± 21 ms) were included in this multicentre prospective study.

Importance of Systematic Right Ventricular Assessment in Cardiac Resynchronization Therapy Candidates: A Machine Learning Approach.

Background: Despite all having systolic heart failure and broad QRS intervals, patients screened for cardiac resynchronization therapy (CRT) are highly heterogeneous, and it remains extremely challenging to predict the impact of CRT devices on left ventricular function and outcomes. The aim of this study was to evaluate the relative impact of clinical, electrocardiographic, and echocardiographic data on the left ventricular remodeling and prognosis of CRT candidates by the application of machine learning approaches.

Methods: One hundred ninety-three patients with systolic heart failure receiving CRT according to current recommendations were prospectively included in this multicenter study.

Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease.

Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for the medical treatment of patients with CHAPLE disease. We observed cessation of gastrointestinal pathology together with restoration of normal immunity and metabolism.

Comparison between body composition parameters and response to neoadjuvant chemotherapy by using pre-treatment PET CT in locally advanced breast cancer.

Purpose: To compare the adipose and muscle tissue areas in patients who responded differently to neoadjuvant chemotherapy.

Methods: One hundred and eighty six patients diagnosed with breast cancer who underwent neoadjuvant chemotherapy between January 2015- October 2019 and were operated after the treatment were retrospectively included in the study. Pathological results were divided into five groups using the Miller-Payne grading systems.

Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage.

Objective: To review our experience with fetal abnormality of the umbilical-portal-DV complex and to discuss the new classification system for umbilical portal systemic venous shunts (UPSVS) according to our cases.

Methods: This study was a retrospective analysis of fetuses with a prenatal diagnosis of abnormality of the umbilical-portal-DV complex. The integrity of the fetal umbilical-portal ductus venosus complex and the hepatic venous system were evaluated using two-dimensional color Doppler sonography.

Post-transplantation cyclophosphamide versus antithymocyte globulin in patients with acute myeloid leukemia undergoing allogeneic stem cell transplantation from HLA-identical sibling donors: A retrospective analysis from the Acute Leukemia Working Party of the European Society for Blood and Marrow Transplantation.

Background: Graft-versus-host disease (GVHD) is a major complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Addition of antithymocyte globulin (ATG) or post-transplantation cyclophosphamide (PTCY) to standard immunosuppressive agents reduces GVHD in different donor settings.

Methods: We compared the outcomes of adults with acute myeloid leukemia undergoing allo-HSCT from HLA-identical sibling donors after the use of PTCY (n = 197) or ATG (n = 1913).

Role of Point-of-Care Lung and Inferior Vena Cava Ultrasound in Clinical Decisions for Patients Presenting to the Emergency Department With Symptoms of Acute Decompensated Heart Failure.

Objectives: This prospective study was performed to evaluate the diagnostic role of point-of-care lung ultrasound (LUS) and inferior vena cava (IVC) ultrasound in patients with acute decompensated heart failure (ADHF).

Methods: A prospective cohort study was conducted between January 2018 and November 2018 on patients with a diagnosis of ADHF in the emergency department (ED). On admission, LUS findings, inspiratory and expiratory IVC diameters, and the inferior vena cava collapsibility index (IVCCI) were obtained.

Comparison of Rapid Absorbable Sutures with Nonabsorbable Sutures in Closing Transcolumellar Incision in Septorhinoplasty: Short-term Outcomes.

Background: Following open rhinoplasty, a postoperative scar at the columellar incision line is a common morbidity. The aim of this study is to compare absorbable and nonabsorbable suture materials which had been used for closing the transcolumellar incision, in the aspect of risk of postoperative infection, wound healing, postoperative columellar scar and patient satisfaction.

Method: A prospective, randomized, single-blind study was conducted between May 2017 and February 2018.

Spinal anesthesia in surgical treatment of lumbar spine tumors.

Objective: This study aims to show the feasibility of spinal anesthesia in lumbar spinal tumor surgery; share our data and experience.

Patients And Methods: A retrospective review of 14 patients with high risk for general anesthesia due to severe comorbidities was carried out. Patients who underwent surgeries under spinal anesthesia for lumbar vertebral column or spinal cord tumors at a single center between 2007-2019 were evaluated.

Comparison of Clinical and Radiological Parameters with Two Different Surgical Methods for Anterior Cruciate Ligament Reconstruction.

This study compared the clinical and radiological findings of nonanatomic transtibial (TT) technique with intraspongious fixation and anatomical anteromedial portal (AMP) technique with extracortical button implant in anterior cruciate ligament (ACL) reconstruction. A total of 54 patients with isolated ACL rupture were included in this prospective study. The patients who had the intraspongious fixation by nonanatomical TT technique were allocated to Group 1 ( = 27).

Post-transplant cyclophosphamide versus anti-thymocyte globulin for graft-versus-host disease prevention in haploidentical transplantation for adult acute lymphoblastic leukemia.

Graft-versus-host disease (GVHD) prophylaxis for unmanipulated haploidentical hematopoietic cell transplantation (haplo-HCT) include post-transplant cyclophosphamide (PTCy) and anti-thymocyte globulin (ATG). Utilizing EBMT registry, we compared ATG versus PTCy based GVHD prophylaxis in adult acute lymphoblastic leukemia (ALL) patients undergoing haplo-HCT. Included were 434 patients; ATG (n=98) and PTCy (n=336).

Comparison of MOLLI and ShMOLLI in Terms of T1 Reactivity and the Relationship between T1 Reactivity and Conventional Signs of Response during Adenosine Stress Perfusion CMR.

Background: One of the most important techniques of cardiac magnetic resonance in assessment of coronary heart diseases is adenosine stress myocardial first-pass perfusion imaging. Using this imaging method, there should be an adequate response to the drug adenosine to make an accurate evaluation. The conventional signs of drug response are not always observed and are often subjective.

Platelet-rich plasma injection in a patient with adhesive capsulitis due to chronic kidney disease.

Adhesive capsulitis is a common problem in patients with chronic kidney disease. Patients suffer from joint stiffness and painful joint movement.Conservative treatments consist non-steroid antiinflamatory drugs, intraarticular injections and physical therapy.