A Delphi Survey Study to Formulate Statements on the Treatability of Inherited Metabolic Disorders to Decide on Eligibility for Newborn Screening.

The Wilson and Jungner (W&J) and Andermann criteria are meant to help select diseases eligible for population-based screening. With the introduction of next-generation sequencing (NGS) methods for newborn screening (NBS), more inherited metabolic diseases (IMDs) can technically be included, and a revision of the criteria was attempted. This study aimed to formulate statements and investigate whether those statements could elaborate on the criterion of for IMDs to decide on eligibility for NBS.

Population-based study on coverage and healthcare processes for cancer during implementation of national healthcare insurance in Indonesia.

Background: A national healthcare insurance has been implemented in Indonesia since 2014. Although cancer care currently represents a smaller part of the healthcare support, the demographic development will lead to a rapid growth of the population within age groups at cancer risk. This requires strategic and developmental planning of cancer care resources.

Decision Conflicts in Clinical Care during COVID-19: A Multi-Perspective Inquiry.

Background: The early COVID-19-pandemic was characterized by changes in decision making, decision-relevant value systems and the related perception of decisional uncertainties and conflicts resulting in decisional burden and stress. The vulnerability of clinical care professionals to these decisional dilemmas has not been characterized yet. Methods: A cross-sectional questionnaire study (540 patients, 322 physicians and 369 nurses in 11 institutions throughout Germany) was carried out.

A pyramid model to describe changing decision making under high uncertainty during the COVID-19 pandemic.

The COVID-19 pandemic put healthcare systems, hospitals and medical personal under great pressure. Based on observations in Germany, we theorise a general model of rapid decision-making that makes sense of the growing complexity, risks and impact of missing evidence. While adapting decision-making algorithms, management, physicians, nurses and other healthcare professionals had to move into uncharted territory while addressing practical challenges and resolving normative (legal and ethical) conflicts.

Economic Perspective of Cancer Care and Its Consequences for Vulnerable Groups.

Within healthcare systems in all countries, vulnerable groups of patients can be identified and are characterized by the reduced utilization of available healthcare. Many different reasons can be attributed to this observation, summarized as implementation barriers involving acceptance, accessibility, affordability, acceptability and quality of care. For many patients, cancer care is specifically associated with the occurrence of vulnerability due to the complex disease, very different target groups and delivery situations (from prevention to palliative care) as well as cost-intensive care.

Single-Center Experience With the Thoraflex™ Hybrid Prosthesis: Indications, Implantation Technique and Results.

Objective: The aim of this study was to evaluate the early and mid-term results after the frozen elephant trunk (FET) procedure for the treatment of complex arch and proximal descending aortic disease in a single-center institution.

Methods: From April 2015 to July 2021, 72 patients (25 women, 60.4 ± 10.

Bilateral Implantation of Double-Branched Iliac Branch Devices for Endovascular Repair of Aorto-Bi-Iliac Aneurysm With Concomitant Hypogastric Aneurysms: The Quadruple Branch.

Purpose: The purpose of this report is to describe the use of a double-branched custom-made iliac branch device (IBD) for the endovascular repair of an aorto-bi-iliac aneurysm with concomitant bilateral hypogastric aneurysms.

Technique: A 61-year-old man on peritoneal dialysis underwent a computed tomography (CT) of the infrarenal aorta before planned kidney transplantation. The CT showed an asymptomatic aorto-bi-iliac aneurysm of 54 mm involving the hypogastric artery (HA) bilaterally (right HA 31 mm; left HA 40 mm).

Realignment of Migrated Celiac Stent Graft After Branched Stent Graft Implantation Through Retrograde Cannulation of the Superior Mesenteric Artery Using a Single Vascular Access.

Purpose: The purpose of the study was to present an endovascular management of a type IIIc endoleak (EL) in a patient with migration of the bridging stent graft of the celiac trunk (CT) after branched aortic aneurysm repair with retrograde cannulation of the superior mesenteric artery (SMA).

Technique: The therapy was applied in a 62-year-old man who underwent a branched EVAR 2 years ago. Meanwhile, the patient was treated due to type Ia EL 6 months ago.

Retrograde Approach for Antegrade Inner Branches in a Precannulated Off-the-Shelf Multibranch Device.

Purpose: The purpose of this technical note was to describe the application of the combination of precannulated branches and a femoral approach for bridging stent graft deployment in branched endovascular aneurysm repair.

Technique: The technique is shown in a 65-year-old woman treated for thoracoabdominal aneurysm type I with endovascular repair using a multibranched device. The stent graft is an off-the-shelf device with 4 precannulated inner branches.

Availability and Accessibility of Primary Care for the Remote, Rural, and Poor Population of Indonesia.

Adopting Universal Health Coverage for implementation of a national health insurance system [Jaminan Kesehatan Nasional (JKN)/Badan Penyelenggara Jaminan Sosial or the Indonesian National Social Health Insurance Scheme (BPJS)] targets the 255 million population of Indonesia. The availability, accessibility, and acceptance of healthcare services are the most important challenges during implementation. Referral behavior and the utilization of primary care structures for underserved (rural/remote regions) populations are key guiding elements.

Congenital disorders of glycosylation with defective fucosylation.

Fucosylation is essential for intercellular and intracellular recognition, cell-cell interaction, fertilization, and inflammatory processes. Only five types of congenital disorders of glycosylation (CDG) related to an impaired fucosylation have been described to date: FUT8-CDG, FCSK-CDG, POFUT1-CDG SLC35C1-CDG, and the only recently described GFUS-CDG. This review summarizes the clinical findings of all hitherto known 25 patients affected with those defects with regard to their pathophysiology and genotype.

Presymptomatic treatment of classic late-infantile neuronal ceroid lipofuscinosis with cerliponase alfa.

Background: Neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is a rare rapidly progressive neurodegenerative disorder, resulting in early death. Intracerebroventricular enzyme replacement therapy (ERT) with cerliponase alfa is now available and has shown to delay disease progression in symptomatic patients. It is yet unknown if cerliponase alfa can prevent disease onset in presymptomatic patients.

Radiation doses for endovascular aortic repairs performed on mobile and fixed C-arm fluoroscopes and procedure phase-specific radiation distribution.

Objective: The objective of this study was to analyze radiation risk to patients during endovascular aneurysm repair (EVAR) using mobile C-arm (MA) or fixed C-arm (FA) fluoroscopes and to describe the dose distribution during the different phases of the procedure.

Methods: Patients treated with EVAR using a single stent graft system between November 2009 and June 2016 were included in this study. The patients were divided into one of two groups (MA or FA) according to the type of C-arm used in the procedure.

Outcomes of Surgical Explantation of Infected Aortic Grafts After Endovascular and Open Abdominal Aneurysm Repair.

Objectives: Infection of the vascular graft represents one of the most threatening complications after aortic repair. It is rare and associated with high morbidity and mortality rates. The aim of this study was to present short-term outcomes after surgical treatment of infected aortic grafts after endovascular and open repair of abdominal aortic aneurysms (AAAs).

Late-onset aortoesophageal fistula after treatment of a chronic type B aortic dissection with a three-step approach.

Aortoesophageal fistula is a rare but lethal complication after thoracic endovascular repair for thoracic aortic diseases. Extensive treatment is reserved for patients fit for surgery. Various technical approaches have been described; however, mortality rates are still high.

Outcome After Crossing Femoropopliteal Chronic Total Occlusions Based on Optical Coherence Tomography Guidance.

Aims: The crossing of chronic total occlusions (CTOs) is the key step for an endovascular treatment. The Ocelot system is a novel device that combines a steerable drilling tip with optical coherence tomography (OCT) technology. It provides intraluminal imaging to help the crossing of CTOs in the femoropopliteal segment.

Clinical presentation and outcome of children with central diabetes insipidus associated with a self-limited or transient pituitary stalk thickening, diagnosed as infundibuloneurohypophysitis.

Objective: Despite lymphocytic or autoimmune infundibuloneurohypophysitis (INH) is an increasingly recognized aetiology in children with central diabetes insipidus (CDI); clinical data on epidemiology (clinical evolution, predisposing factors, complications), diagnosis and management of this entity are limited and mostly based on published case reports. The aim of this study was to gain a broader insight in the natural history of this disease by analysing the clinical presentation, radiological pituitary stalk changes, associated autoimmunity and hormonal deficiencies in children with CDI and a self-limiting or transient stalk thickening (ST), diagnosed as autoimmune infundibuloneurohypophysitis, during the last 15 years in four Belgian university hospitals.

Design And Patients: The medical files of nine CDI patients with a ST at initial presentation and no signs of Langerhans cell histiocytosis or germinoma at presentation and/or during follow-up of more than 1.

Native arteriovenous fistula placement in three patients after implantation of a left ventricular assist device with non-pulsatile blood flow.

Patients with left ventricular assist device have an increased risk for postoperative acute kidney injury and long-term requirement of dialysis. As a chronic access for dialysis, native arteriovenous fistulae are uncommon options due to suspected maturation failure. We present three patients who received successful arteriovenous fistulae placement after left ventricular assist device implantation with assisted long term patency.

Soft tissue swelling in children: case report, differential diagnosis, and diagnostic delay.

A general practitioner faces regularly soft tissue swelling in otherwise healthy children. Delay in diagnosis of soft tissue malignancies is often due to asymptomatic nature and the unfamiliarity with the age-dependent differential diagnosis. Hence, an accurate knowledge is important to prevent important delay in diagnosis of potential malignancies.

Crossing chronic total occlusions with the Ocelot system: the initial European experience.

Aims: The aim of the study was to determine the safety, efficacy and feasibility of a new chronic total occlusion (CTO) device using optical coherence tomography (OCT) technology, the Ocelot catheter (Avinger, Inc., Redwood City, CA, USA), for crossing of SFA CTOs following guidewire failure.

Methods And Results: Prospective, multicentre, market preference testing.

Serum levels of N-terminal proB-type natriuretic peptide in mechanically ventilated critically ill patients--relation to tidal volume size and development of acute respiratory distress syndrome.

Background: Serum levels of N-terminal proB-type natriuretic peptide (NT-proBNP) are elevated in patients acute respiratory distress syndrome (ARDS). Recent studies showed a lower incidence of acute cor pulmonale in ARDS patients ventilated with lower tidal volumes. Consequently, serum levels of NT-proBNP may be lower in these patients.