Primers and PrimeViews

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  • Primary lymphoedema is a condition, often caused by a genetic mutation, characterized by alterations in the structure or function of the lymphatic system.

    PrimeView
  • Primary lymphoedema (PLE) refers to swelling of parts of the body (usually limbs) caused by anomalies in the development or functioning of the lymphatic system; PLE can result from genetic mutations and can be the only symptom or one of the manifestations of a syndrome.

    • Pascal Brouillard
    • Marlys H. Witte
    • Miikka Vikkula
    Primer
  • This PrimeView highlights the epidemiology, diagnosis, pathophysiology and management of Wilms tumour, and summarizes future research needs. It accompanies the Primer on Wilms tumour by Spreafico and colleagues.

    PrimeView
  • Wilms tumour (WT) is the most common renal tumour in infants and young children. This Primer reviews the epidemiology, mechanisms, diagnosis and management of WT. In addition, the authors outline potential opportunities to translate novel biological targets to improve clinical outcomes.

    • Filippo Spreafico
    • Conrad V. Fernandez
    • Kathy Pritchard-Jones
    Primer
  • Histiocytic disorders are a heterogeneous family of diseases with a wide range of clinical manifestations, from self-resolving skin lesions to multi-organ, life-threatening disease.

    PrimeView
  • Histiocytic disorders are characterized by proliferation of cells with macrophage or dendritic cell phenotype and intense inflammation. Clinical signs and symptoms depend on the specific disease and the tissues and organs affected.

    • Kenneth L. McClain
    • Camille Bigenwald
    • Carl E. Allen
    Primer
  • This PrimeView highlights the epidemiology, diagnosis and management of membranous nephropathy, and summarizes the mechanisms of this kidney glomeruli autoimmune disease. It accompanies the Primer on membranous nephropathy by Ronco and colleagues.

    PrimeView
  • Membranous nephropathy is a glomerular disease that is the most frequent cause of nephrotic syndrome in adults. This Primer reviews the epidemiology, pathophysiology, diagnosis and management of this disease and discusses how to improve long-term renal prognosis and quality of life.

    • Pierre Ronco
    • Laurence Beck
    • Jack Wetzels
    Primer
  • This PrimeView describes the epidemiology, pathophysiology, diagnosis and management of Behçet syndrome, as well as highlighting patient quality of life and avenues for future research.

    PrimeView
  • Behçet syndrome is a recurrent multiorgan inflammatory disorder and a systemic vasculitis that predominantly affects veins. This Primer reviews the epidemiology, pathophysiology, diagnosis and treatment of Behçet syndrome and describes its effect on patient quality of life and the future outlook for the field.

    • Yusuf Yazici
    • Gulen Hatemi
    • Hasan Yazici
    Primer
  • This PrimeView highlights the diagnosis and prevention of cholangiocarcinoma, and summarizes its epidemiology, mechanisms, management and outlook. It accompanies the Primer on cholangiocarcinoma by Brindley and colleagues.

    PrimeView
  • Cholangiocarcinoma is a highly lethal adenocarcinoma of the hepatobiliary system that can be associated with liver fluke infection but often has no identifiable cause. This Primer reviews the epidemiology, pathophysiological mechanisms, diagnosis and management of cholangiocarcinoma, and highlights the patient experience and future directions.

    • Paul J. Brindley
    • Melinda Bachini
    • Gregory J. Gores
    Primer
  • This PrimeView highlights the clinical presentations of Marfan syndrome, a genetic disorder caused by pathogenetic variants in FBN1, encoding the extracellular matrix protein fibrillin-1.

    PrimeView
  • Marfan syndrome (MFS) is a genetic disorder affecting the connective tissue, caused by mutations in FBN1 (which encodes fibrillin-1, a structural component of the extracellular matrix); individuals with MFS usually present with cardiovascular (aortic aneurysms and dissections), skeletal and ocular manifestations.

    • Dianna M. Milewicz
    • Alan C. Braverman
    • Julie De Backer
    Primer
  • This illustrated PrimeView highlights the mechanisms of cutaneous T cell lymphoma, and accompanies the Primer article by Dummer and colleagues.

    PrimeView
  • Cutaneous T cell lymphoma is a group of lymphomas that initially manifest in the skin. This Primer summarizes the pathophysiology, epidemiology, diagnosis and management of these disorders.

    • Reinhard Dummer
    • Maarten H. Vermeer
    • Egle Ramelyte
    Primer
  • Psoriatic arthritis is a chronic immune-mediated disease with heterogeneous clinical features. This PrimeView highlights the mechanisms of psoriatic arthritis and accompanies the Primer article by Fitzgerald and colleagues.

    PrimeView
  • Psoriatic arthritis (PsA) is a chronic immune-mediated form of arthritis that occurs in some patients with psoriasis. This Primer reviews the epidemiology and pathophysiology of PsA and highlights the challenges in diagnosis and advances in treatment. In addition, the authors discuss the quality of life of patients and outstanding questions in the field.

    • Oliver FitzGerald
    • Alexis Ogdie
    • Philip J. Mease
    Primer
  • A non-union bone fracture represents a chronic medical condition affecting an individual’s psychosocial and economic wellbeing. This PrimeView discusses the epidemiology, pathophysiology and diagnosis of non-union as well as highlights its management and the patient quality of life.

    PrimeView
  • Non-union, defined as a fractured bone that does not heal within the expected time or is deemed unable to heal without intervention, represents a complex chronic medical condition characterized by pain and functional and psychosocial disability. In this Primer, Jupiter and colleagues discuss the epidemiology, advances in pathophysiology, diagnosis and management, and the quality of life of patients with non-union.

    • Britt Wildemann
    • Anita Ignatius
    • Jesse B. Jupiter
    Primer