{"title":"Degenerative mitral regurgitation","authors":"","doi":"10.1038/s41572-023-00485-8","DOIUrl":"https://doi.org/10.1038/s41572-023-00485-8","url":null,"abstract":"This PrimeView summarizes the pathophysiology of degenerative mitral regurgitation, a form of valvular heart disease.","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":81.5,"publicationDate":"2023-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138547862","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eleni Stamellou, Claudia Seikrit, Sydney C W Tang, Peter Boor, Vladimir Tesař, Jürgen Floege, Jonathan Barratt, Rafael Kramann
{"title":"IgA nephropathy.","authors":"Eleni Stamellou, Claudia Seikrit, Sydney C W Tang, Peter Boor, Vladimir Tesař, Jürgen Floege, Jonathan Barratt, Rafael Kramann","doi":"10.1038/s41572-023-00476-9","DOIUrl":"10.1038/s41572-023-00476-9","url":null,"abstract":"<p><p>IgA nephropathy (IgAN), the most prevalent primary glomerulonephritis worldwide, carries a considerable lifetime risk of kidney failure. Clinical manifestations of IgAN vary from asymptomatic with microscopic or intermittent macroscopic haematuria and stable kidney function to rapidly progressive glomerulonephritis. IgAN has been proposed to develop through a 'four-hit' process, commencing with overproduction and increased systemic presence of poorly O-glycosylated galactose-deficient IgA1 (Gd-IgA1), followed by recognition of Gd-IgA1 by antiglycan autoantibodies, aggregation of Gd-IgA1 and formation of polymeric IgA1 immune complexes and, lastly, deposition of these immune complexes in the glomerular mesangium, leading to kidney inflammation and scarring. IgAN can only be diagnosed by kidney biopsy. Extensive, optimized supportive care is the mainstay of therapy for patients with IgAN. For those at high risk of disease progression, the 2021 KDIGO Clinical Practice Guideline suggests considering a 6-month course of systemic corticosteroid therapy; however, the efficacy of systemic steroid treatment is under debate and serious adverse effects are common. Advances in understanding the pathophysiology of IgAN have led to clinical trials of novel targeted therapies with acceptable safety profiles, including SGLT2 inhibitors, endothelin receptor blockers, targeted-release budesonide, B cell proliferation and differentiation inhibitors, as well as blockade of complement components.</p>","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":81.5,"publicationDate":"2023-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138461113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Potential and pitfalls of conversational agents in health care.","authors":"Kerstin Denecke","doi":"10.1038/s41572-023-00482-x","DOIUrl":"10.1038/s41572-023-00482-x","url":null,"abstract":"","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":81.5,"publicationDate":"2023-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138299588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marc G Jeschke, Fiona M Wood, Esther Middelkoop, Ardeshir Bayat, Luc Teot, Rei Ogawa, Gerd G Gauglitz
{"title":"Scars.","authors":"Marc G Jeschke, Fiona M Wood, Esther Middelkoop, Ardeshir Bayat, Luc Teot, Rei Ogawa, Gerd G Gauglitz","doi":"10.1038/s41572-023-00474-x","DOIUrl":"10.1038/s41572-023-00474-x","url":null,"abstract":"<p><p>Wound healing occurs as a response to disruption of the epidermis and dermis. It is an intricate and well-orchestrated response with the goal to restore skin integrity and function. However, in hundreds of millions of patients, skin wound healing results in abnormal scarring, including keloid lesions or hypertrophic scarring. Although the underlying mechanisms of hypertrophic scars and keloid lesions are not well defined, evidence suggests that the changes in the extracellular matrix are perpetuated by ongoing inflammation in susceptible individuals, resulting in a fibrotic phenotype. The lesions then become established, with ongoing deposition of excess disordered collagen. Not only can abnormal scarring be debilitating and painful, it can also cause functional impairment and profound changes in appearance, thereby substantially affecting patients' lives. Despite the vast demand on patient health and the medical society, very little progress has been made in the care of patients with abnormal scarring. To improve the outcome of pathological scarring, standardized and innovative approaches are required.</p>","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":81.5,"publicationDate":"2023-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136398290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lillian Tugume, Kenneth Ssebambulidde, John Kasibante, Jayne Ellis, Rachel M Wake, Jane Gakuru, David S Lawrence, Mahsa Abassi, Radha Rajasingham, David B Meya, David R Boulware
{"title":"Cryptococcal meningitis.","authors":"Lillian Tugume, Kenneth Ssebambulidde, John Kasibante, Jayne Ellis, Rachel M Wake, Jane Gakuru, David S Lawrence, Mahsa Abassi, Radha Rajasingham, David B Meya, David R Boulware","doi":"10.1038/s41572-023-00472-z","DOIUrl":"10.1038/s41572-023-00472-z","url":null,"abstract":"<p><p>Cryptococcus neoformans and Cryptococcus gattii species complexes cause meningoencephalitis with high fatality rates and considerable morbidity, particularly in persons with deficient T cell-mediated immunity, most commonly affecting people living with HIV. Whereas the global incidence of HIV-associated cryptococcal meningitis (HIV-CM) has decreased over the past decade, cryptococcosis still accounts for one in five AIDS-related deaths globally due to the persistent burden of advanced HIV disease. Moreover, mortality remains high (~50%) in low-resource settings. The armamentarium to decrease cryptococcosis-associated mortality is expanding: cryptococcal antigen screening in the serum and pre-emptive azole therapy for cryptococcal antigenaemia are well established, whereas enhanced pre-emptive combination treatment regimens to improve survival of persons with cryptococcal antigenaemia are in clinical trials. Short courses (≤7 days) of amphotericin-based therapy combined with flucytosine are currently the preferred options for induction therapy of cryptococcal meningitis. Whether short-course induction regimens improve long-term morbidity such as depression, reduced neurocognitive performance and physical disability among survivors is the subject of further study. Here, we discuss underlying immunology, changing epidemiology, and updates on the management of cryptococcal meningitis with emphasis on HIV-associated disease.</p>","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":76.9,"publicationDate":"2023-11-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72014677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Federica Pederiva, Steven S Rothenberg, Nigel Hall, Hanneke Ijsselstijn, Kenneth K Y Wong, Jan von der Thüsen, Pierluigi Ciet, Reuven Achiron, Adamo Pio d'Adamo, J Marco Schnater
{"title":"Congenital lung malformations.","authors":"Federica Pederiva, Steven S Rothenberg, Nigel Hall, Hanneke Ijsselstijn, Kenneth K Y Wong, Jan von der Thüsen, Pierluigi Ciet, Reuven Achiron, Adamo Pio d'Adamo, J Marco Schnater","doi":"10.1038/s41572-023-00470-1","DOIUrl":"10.1038/s41572-023-00470-1","url":null,"abstract":"<p><p>Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.</p>","PeriodicalId":18910,"journal":{"name":"Nature Reviews Disease Primers","volume":null,"pages":null},"PeriodicalIF":81.5,"publicationDate":"2023-11-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71425192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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