Articles in 2016

α1-Antitrypsin deficiency (A1ATD) is an inherited disorder caused by mutations in SERPINA1, leading to liver and lung disease. In this Primer by Greene et al., the classification of A1ATD is discussed, in addition to the mechanisms, diagnosis and management of this disease.

Aortic dissection is a medical emergency that requires prompt diagnosis and treatment to ensure patient survival. This PrimeView highlights the mechanisms and risk factors that contribute to aortic dissection, including hypertension, connective tissue disorders, inflammation and atherosclerosis.

Aortic dissection is a medical emergency that occurs when the layers of the aortic wall are forced apart by bleeding, creating a false route of blood flow. Important risk factors for aortic dissection include hypertension and connective tissue disorders.

Measles is caused by a highly infectious, airborne pathogen: measles virus. This PrimeView focuses on the transmission of this virus and explains how cheap and safe vaccines can be used to prevent transmission and eliminate measles globally.

Measles is an infectious disease caused by the measles virus. In this Primer, Rota et al. cover the pathophysiology and management options, with a focus on the strategies to eliminate endemic transmission of the measles virus by achieving a high level of vaccination coverage

Sjögren syndrome (SjS) is an autoimmune disease that is characterized by the development of sicca symptoms and, in some patients, systemic disease. This PrimeView focuses on the aetiopathogenetic mechanisms of SjS and also discusses the epidemiology, diagnosis and management of this disease.

Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands, resulting in the development of sicca symptoms and systemic disease. In this Primer, the mechanisms as well as the diagnosis and management of SjS are described.

This PrimeView highlights the three main components of managing patients with sepsis: infection control, haemodynamic stabilization and modulation of the septic response.

Sepsis is the dysregulated systemic inflammatory and immune response to infection that produces organ injury. Septic shock is characterized by persistent hypotension that substantially increases the risk of death. In this Primer, the authors outline the controversies in sepsis and septic shock, including the complex mechanistic factors and management of critically ill patients.

Uterine fibroids are common neoplasms of the uterus and can cause heavy or prolonged menstrual bleeding, as well as pain and urinary and gastrointestinal problems. This PrimeView focuses on the mechanisms underlying the development of uterine fibroids.

Uterine fibroids are benign neoplasms of the uterus and are a major source of morbidity in women of reproductive age. In this Primer, Stewart et al. discuss the epidemiology, pathophysiology and management of uterine fibroids, including both surgical and non-surgical therapies.

Systemic lupus erythematosus (SLE) is a potentially fatal, chronic autoimmune disorder. This PrimeView illustrates that SLE is heterogeneous with considerable variability in clinical manifestations and disease severity. Early diagnosis and prevention of flares is important to reduce long-term morbidity and mortality.

Systemic lupus erythematosus is an autoimmune disease that can affect many organs, including the skin, joints, the central nervous system and the kidneys. Here, Kaul et al. highlight the heterogeneity of the disease, the management approaches and provide an outlook on the future research directions.

This PrimeView highlights the pathophysiology of acute-on-chronic liver failure in cirrhosis. In patients with cirrhosis, triggers of inflammation, such as sepsis and alcoholic hepatitis, can cause acute liver decompensation and organ failure, resulting in high short-term mortality.

Patients with cirrhosis can develop acute-on-chronic liver failure, a syndrome characterized by acute decompensation, organ failure and high short-term mortality. Arroyo et al. discuss the mechanisms, diagnosis and management of this evolving concept in the field of liver disease.

Disseminated intravascular coagulation (DIC) is a medical emergency that can be caused by infectious or non-infectious conditions. The mechanisms underlying DIC — highlighted in this PrimeView — are an imbalance in the factors that control haemostasis.

Disseminated intravascular coagulation can be caused by various infectious and non-infectious insults, such as sepsis and trauma, respectively. It is characterized by the widespread activation of coagulation and, depending on the underlying condition, can manifest as bleeding and/or thrombosis.

This PrimeView highlights the mechanisms underlying tuberous sclerosis complex (TSC), a lifelong chronic disease caused by mutations in either TSC1 or TSC2. These mutations lead to signalling changes that promote the growth of benign tumours in various organs.

Tuberous sclerosis complex (TSC) is a rare inherited disease caused by mutations in the tumour-suppressor genes TSC1 or TSC2. As a chronic disease that manifests as benign tumours in the brain, kidneys, lungs and skin, TSC requires lifelong treatment.

Primary hyperparathyroidism (PHPT) is a common disorder characterized by an excessive secretion of parathyroid hormone. This PrimeView accompanies the Primer by Bilezikian and colleagues and shows that PHPT can be caused by a single benign adenoma, multiglandular disease and parathyroid carcinoma.