Recommendations for Pregnancy in Rare Inherited Anemias.

Rare inherited anemias are a subset of anemias caused by a genetic defect along one of the several stages of erythropoiesis or in different cellular components that affect red blood cell integrity, and thus its lifespan. Due to their low prevalence, several complications on growth and development, and multi-organ system damage are not yet well defined. Moreover, during the last decade there has been a lack of proper understanding of the impact of rare anemias on maternal and fetal outcomes.

The Minimally Invasive-Guided Genioplasty Technique using Piezosurgery and 3D printed surgical guide: An innovative technique.

Introduction: Mental nerve injuries with neurosensory deficits, asymmetries, and intra-operative bleeding are the main immediate complications of genioplasty. Following a recent systematic review, three-dimensional (3D)-printed cutting guide could improve the predictability and accuracy of this surgical technique avoiding postoperative asymmetries. Furthermore, anatomical structures in the surgical area (mental nerve and teeth roots) are better protected, reducing the morbidity and providing safer results.

Recommendations for skin cancer consultation and surgery during COVID-19 pandemic.

: COVID‐19 SPECIAL FORUM. 2020; : e433–e466.

Position statement of the European Academy of Dermatology and Venereology Task Force on Quality of Life and Patient Oriented Outcomes on quality of life issues in dermatologic patients during the COVID-19 pandemic.

The pandemic of COVID-19 is a global challenge for health care, and dermatologists are not standing apart from trying to meet this challenge. The European Academy of Dermatology and Venereology (EADV) has collected recommendations from its Task Forces (TFs) related to COVID-19. The Journal of the EADV has established a COVID-19 Special Forum giving free access to related articles.

The prognostic value of patient-reported Health-Related Quality of Life and Geriatric Assessment in predicting early death in 6769 older (≥70 years) patients with different cancer tumors.

Objectives: We aimed to determine the prognostic value of baseline Health-Related Quality Of Life (HRQOL) and geriatric assessment (GA) to predict three-month mortality in older patients with cancer undergoing treatment.

Methods: Logistic regressions analysed HRQOL, as measured with the EORTC Global Health Status (GHS) scale, and geriatric information prognostic for early mortality controlling for oncology variables. The assessment was established with the odds ratio (OR), 95% confidence interval (CI) and level of significance set at p < 0.

Late-onset Pompe disease (LOPD) in Belgium: clinical characteristics and outcome measures.

Background: Late-onset Pompe disease (LOPD) is a rare, hereditary, progressive disorder that is usually characterized by limb-girdle muscle weakness and/or respiratory insufficiency. LOPD is caused by mutations in the acid alpha-glucosidase (GAA) gene and treated with enzyme replacement therapy (ERT).

Methods: We studied the clinical, brain imaging, and genetic features of the Belgian cohort of late-onset Pompe disease patients (N = 52), and explored the sensitivity of different outcome measures, during a longitudinal period of 7 years (2010-2017), including the activity limitations ActivLim score, 6 min walking distance (6MWD), 10 m walk test (10MWT), MRC sum score, and forced vital capacity (FVC) sitting/supine.

Optimum imaging of chronic pancreatitis.

Chronic pancreatitis is an inflammatory process of the pancreas characterized by progressive parenchyma destruction, resulting in pain and exocrine and endocrine insufficiency. In the advanced stages the diagnosis by imaging is usually straightforward, while in the early phases of the disease there can be a paucity of findings at imaging, thus making an early diagnosis challenging. Different imaging modalities can have a role in the initial diagnosis and in the longitudinal follow-up of patients affected by chronic pancreatitis, also enabling to assess the complications of the disease.

Three cases of molecularly confirmed Knobloch syndrome.

Knobloch syndrome (OMIM 267750) is a rare autosomal recessive disorder due to genetic defects in the gene. The triad of high myopia, occipital defect, vitreoretinal degeneration has been described as pathognomonic for this condition. Patients with Knobloch syndrome have also extraocular problems as brain and kidney malformations.

European interdisciplinary guideline on invasive squamous cell carcinoma of the skin: Part 2. Treatment.

In order to update recommendations on treatment, supportive care, education and follow-up of patients with invasive cutaneous squamous cell carcinoma (cSCC), a multidisciplinary panel of experts from the European Dermatology Forum, the European Association of Dermato-Oncology and the European Organization of Research and Treatment of Cancer was formed. Recommendations were based on evidence-based literature review, guidelines and expert consensus. Treatment recommendations are presented for common primary cSCC (low risk, high risk), locally advanced cSCC, regional metastatic cSCC (operable or inoperable) and distant metastatic cSCC.

European interdisciplinary guideline on invasive squamous cell carcinoma of the skin: Part 1. epidemiology, diagnostics and prevention.

Invasive cutaneous squamous cell carcinoma (cSCC) is one of the most common cancers in the white populations, accounting for 20% of all cutaneous malignancies. Factors implicated in cSCC etiopathogenesis include ultraviolet radiation exposure and chronic photoaging, age, male sex, immunosuppression, smoking and genetic factors. A collaboration of multidisciplinary experts from the European Dermatology Forum (EDF), the European Association of Dermato-Oncology (EADO) and the European Organisation of Research and Treatment of Cancer (EORTC) was formed to update recommendations on cSCC classification, diagnosis, risk stratification, staging and prevention, based on current literature, staging systems and expert consensus.

Validation of the 2HELPS2B Seizure Risk Score in Acute Brain Injury Patients.

Background And Objective: Seizures are common after traumatic brain injury (TBI), aneurysmal subarachnoid hemorrhage (aSAH), subdural hematoma (SDH), and non-traumatic intraparenchymal hemorrhage (IPH)-collectively defined herein as acute brain injury (ABI). Most seizures in ABI are subclinical, meaning that they are only detectable with EEG. A method is required to identify patients at greatest risk of seizures and thereby in need of prolonged continuous EEG monitoring.

A management algorithm for adult patients with both brain oxygen and intracranial pressure monitoring: the Seattle International Severe Traumatic Brain Injury Consensus Conference (SIBICC).

Background: Current guidelines for the treatment of adult severe traumatic brain injury (sTBI) consist of high-quality evidence reports, but they are no longer accompanied by management protocols, as these require expert opinion to bridge the gap between published evidence and patient care. We aimed to establish a modern sTBI protocol for adult patients with both intracranial pressure (ICP) and brain oxygen monitors in place.

Methods: Our consensus working group consisted of 42 experienced and actively practicing sTBI opinion leaders from six continents.

Decision-Analytic Modeling Studies in Prevention and Treatment of Iodine Deficiency and Thyroid Disorders: A Systematic Overview.

Prevention and treatment of iodine deficiency-related diseases remain an important public health challenge. Iodine deficiency can have severe health consequences, such as cretinism, goiter, or other thyroid disorders, and it has economic implications. Our aim was to give an overview of studies applying decision-analytic modeling to evaluate the effectiveness and/or cost-effectiveness of iodine deficiency-related prevention strategies or treatments related to thyroid disorders.

Tissue oxygen saturation changes and postoperative complications in cardiac surgery: a prospective observational study.

Background: Cardiac surgery with extracorporeal circulation (ECC) can induce microvascular dysfunction and tissue hypoperfusion. We hypothesized that the alterations in near-infrared spectroscopy (NIRS)-derived parameters would be associated with post-operative complications in cardiac surgery patients.

Methods: Prospective observational study performed at two University Hospitals.

Evaluation of clinical and anatomical outcome of staged stenting after acute coiling of ruptured intracranial aneurysms.

Introduction: Stent-assisted coiling has widened indications and improved stability of endovascular treatment of intracranial aneurysms. However, stent-assisted coiling is usually not used to treat acutely ruptured intracranial aneurysms to avoid antiplatelet therapy. The objective of this study is to evaluate a strategy of staged endovascular treatment of ruptured intracranial aneurysms including coiling at the acute phase with complementary stenting with or without coiling at the subacute phase.

Clinical Performance Measures for Neurocritical Care: A Statement for Healthcare Professionals from the Neurocritical Care Society.

Background: Performance measures are tools to measure the quality of clinical care. To date, there is no organized set of performance measures for neurocritical care.

Methods: The Neurocritical Care Society convened a multidisciplinary writing committee to develop performance measures relevant to neurocritical care delivery in the inpatient setting.

A management algorithm for patients with intracranial pressure monitoring: the Seattle International Severe Traumatic Brain Injury Consensus Conference (SIBICC).

Background: Management algorithms for adult severe traumatic brain injury (sTBI) were omitted in later editions of the Brain Trauma Foundation's sTBI Management Guidelines, as they were not evidence-based.

Methods: We used a Delphi-method-based consensus approach to address management of sTBI patients undergoing intracranial pressure (ICP) monitoring. Forty-two experienced, clinically active sTBI specialists from six continents comprised the panel.

Measuring the nursing workload in intensive care with the Nursing Activities Score (NAS): A prospective study in 16 hospitals in Belgium.

Purpose: The evaluation of nursing workload is a common practice in intensive care units (ICUs). It allows the calculation of an optimal nurse/patient ratio (N/P) which is a major challenge to ensuring the quality of care while controlling the costs of health care. The objectives of this study were, therefore, to evaluate the N/P ratio and to study nursing activities in intensive care in French-speaking Belgium.

Detection of a Large Novel α-Thalassemia Deletion in an Autochthonous Belgian Family.

α-Thalassemia (α-thal) is a common hemoglobinopathy mainly caused by deletion of one or both α-globin genes. We describe an autochthonous Belgian family diagnosed with α-thal trait. Molecular analysis revealed a novel large deletion of at least 170 kb between 226.

Post-operative cerebellar mutism syndrome: rehabilitation issues.

Introduction: Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language.

Different shades of pancreatic ductal adenocarcinoma, different paths towards precision therapeutic applications.

Background: Different histological and molecular subtypes of pancreatic ductal adenocarcinoma (PDAC), with different molecular composition and survival statistics, have recently been recognised.

Materials And Methods: This review describes the currently available studies regarding molecular and histological subtypes in PDAC. Studies from major cohorts such as International Cancer Genome Consortium as well as smaller cohorts are reviewed.