Seminars in nephrology最新文献

Glomerular diseases are common causes of chronic kidney disease in childhood, adolescence, and adulthood. The epidemiology of glomerular diseases differs between different age groups, with minimal change disease being the leading cause of nephrotic syndrome in childhood, while membranous nephropathy and focal segmental glomerulosclerosis are more common in adulthood. IgA vasculitis is also more common in childhood. Moreover, there is a difference in disease severity with more children presenting with a relapsing form of nephrotic syndrome and a more acute presentation of antineutrophil cytoplasmic antibody-associated vasculitis and concomitant glomerulonephritis, as highlighted by the higher percentage of cellular crescents on kidney biopsy specimens in comparison with older patients. There is also a female preponderance in antineutrophil cytoplasmic antibody-associated vasculitis and more children present with tracheobroncholaryngeal disease. This article aims to summarize differences in the presentation of different glomerular diseases that are encountered commonly by pediatric and adult nephrologists and potential differences in the management.

Cystic kidney diseases, when broadly defined, have a wide differential diagnosis extending from recessive diseases with a prenatal or pediatric diagnosis, to the most common autosomal-dominant polycystic kidney disease primarily affecting adults, and several other genetic or acquired etiologies that can manifest with kidney cysts. The most likely diagnoses to consider when assessing a patient with cystic kidney disease differ depending on family history, age stratum, radiologic characteristics, and extrarenal features. Accurate identification of the underlying condition is crucial to estimate the prognosis and initiate the appropriate management, identification of extrarenal manifestations, and counseling on recurrence risk in future pregnancies. There are significant differences in the clinical approach to investigating and managing kidney cysts in children compared with adults. Next-generation sequencing has revolutionized the diagnosis of inherited disorders of the kidney, despite limitations in access and challenges in interpreting the data. Disease-modifying treatments are lacking in the majority of kidney cystic diseases. For adults with rapid progressive autosomal-dominant polycystic kidney disease, tolvaptan (V2-receptor antagonist) has been approved to slow the rate of decline in kidney function. In this article, we examine the differences in the differential diagnosis and clinical management of cystic kidney disease in children versus adults, and we highlight the progress in molecular diagnostics and therapeutics, as well as some of the gaps meriting further attention.

The tubular system of the kidneys is a complex series of morphologic and functional units orchestrating the content of tubular fluid as it flows along the nephron and collecting ducts. Renal tubules maintain body water, regulate electrolytes and acid-base balance, reabsorb precious organic solutes, and eliminate specific metabolites, toxins, and drugs. In addition, decisive mechanisms to adjust blood pressure are governed by the renal tubules. Genetic as well as acquired disorders of these tubular functions may cause serious diseases that manifest both in childhood and adulthood. This article addresses a selection of tubulopathies and the underlying pathomechanisms, while highlighting the important differences in pediatric and adult nephrology care. These range from rare monogenic conditions such as nephrogenic diabetes insipidus, cystinosis, and Bartter syndrome that present in childhood, to the genetic and acquired tubular pathologies causing hypertension or nephrolithiasis that are more prevalent in adults. Both pediatric and adult nephrologists must be aware of these conditions and the age-dependent manifestations that warrant close interaction between the two subspecialties.

Acute kidney injury (AKI) occurs frequently in hospitalized patients, regardless of age or prior medical history. Increasing awareness of the epidemiologic problem of AKI has directly led to increased study of global recognition, diagnostic tools, both reactive and proactive management, and analysis of long-term sequelae. Many gaps remain, however, and in this article we highlight opportunities to add significantly to the increasing bodies of evidence surrounding AKI. Practical considerations related to initiation, prescription, anticoagulation, and monitoring are discussed. In addition, the importance of AKI follow-up evaluation, particularly for those surviving the receipt of renal replacement therapy, is highlighted as a push for global equity in the realm of critical care nephrology is broached. Addressing these gaps presents an opportunity to impact patient care directly and improve patient outcomes.

Common goals of nutritional therapy across the spectrum of pediatric and adult chronic kidney disease (CKD) include maintaining normal body mass and composition and reducing associated morbidity and mortality. Adult nephrologists caring for children and adolescents may be challenged by the existing complexities in identifying and interpreting the nutritional status and growth in children. Pediatric nephrologists may face situations that call for a sound knowledge of assessing nutritional status and providing nutrition therapy for adolescents and young adults. One important additional nutrition goal in children is to achieve normal growth and development. Children are growing and therefore need more calories and nutrients than just maintaining their body weight and composition. Lack of weight and height gain actually is considered failure to thrive in children. Some fundamental differences in approaches to nutritional therapy in CKD are necessitated based on the etiology of CKD. A large proportion of adults with CKD are diabetics, so the approach would be a low-carbohydrate diet. Children with CKD, especially young ones, often are anorexic, so calorie supplements that could include quite a lot of carbohydrates often are prescribed. More adults with CKD have hypertension and atherosclerotic comorbidities, which result in recommendations for low-salt and low-fat diets. Children with CKD often have salt and electrolyte wasting disease states and would require normal- or even high-salt diets, and fats often are included in supplements to bolster calorie intake. Low-protein diets often are recommended in adults with predialysis CKD to slow disease progression. Children are growing and have a higher protein daily requirement. Low-protein diets have not been found to be efficacious in children with CKD, in achieving normal growth, or in slowing disease progression. Adult nephrologists caring for children and adolescents may be challenged by the existing complexities in identifying and interpreting nutritional status and growth in children. Pediatric nephrologists may face situations that call for a sound knowledge of assessing nutritional status and providing nutrition therapy for adolescents and young adults. This article discusses the differences in the assessment of nutritional status between children and adults, as well as provides a comprehensive approach to nutritional management for CKD across the age spectrum. Semin Nephrol 43:x-xx © 2023 Elsevier Inc. All rights reserved.