Core Curriculum in Nephrology
Installments of the Core Curriculum in Nephrology provide trainees in nephrology with a strong knowledge base in core topics in the specialty by providing an overview of the topic and citing key references, including the foundational literature that led to current clinical approaches.
- Renal cystic disease encompasses a large variety of illnesses with various phenotypic expressions that can manifest in utero, in infancy, and in childhood. These diseases may be unilateral or bilateral and present with single or multiple cysts. Various cystic diseases may also progress to chronic kidney disease (CKD), including kidney failure, and hepatic disease, thus potentially being life threatening. The prevalence and serious complications of CKD in the pediatric population make it vital that health care providers detect these conditions early and provide effective management.
- Total-body potassium (K+) content and appropriate distribution of K+ across the cell membrane is vitally important for normal cellular function. Total-body K+ content is determined by changes in excretion of K+ by the kidneys in response to intake levels. Under normal conditions, insulin and β-adrenergic tone also make important contributions in maintaining internal distribution of K+. However, despite these homeostatic pathways, disorders of altered K+ homeostasis are common. Appreciating the pathophysiology and regulatory influences that determine the internal distribution and external balance of K+ is critical in designing effective treatments to restore K+ homeostasis.
- This Core Curriculum article models a person-centered approach to care for older adults with kidney disease. We provide background information on the principles of person-centered care and outline ways in which this approach contrasts with the more disease-based approaches that dominate current medical education and practice. Using hypothetical cases, we discuss 3 clinical scenarios that arise commonly when caring for older adults with kidney disease: (1) a moderate reduction in estimated glomerular filtration rate, (2) new-onset nephrotic-range proteinuria, and (3) the prospect of starting dialysis.
- Nephrotic syndrome (NS) is one of the most challenging conditions to manage and treat, partly because we lack a specific molecular understanding of its pathogenesis and progression. This limits our ability to provide targeted therapy or precise prognostications. Fortunately, genomic discovery in NS and its translation to genomic-informed medicine is allowing us to improve our understanding of the molecular anatomy of NS and our ability to care for patients with NS. In this Core Curriculum, we review the specific genes and loci discovered in childhood NS, specifically NS of Mendelian origin, APOL1-associated NS in black patients, HLA region variants associated with steroid-sensitive NS, their biological impacts, prevalence across populations, and clinical correlates.