Acquired cystic kidney disease: an under-recognized condition in children with end-stage renal disease

Eugene Y H Chan1, Bradley A Warady2

  • 1Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.

Insights

Acquired cystic kidney disease (ACKD) affects patients with end-stage renal disease (ESRD). Surveillance is recommended due to potential complications like renal cell carcinoma.

Area of Science:

  • Nephrology
  • Oncology
  • Pediatric Nephrology

Background:

  • Acquired cystic kidney disease (ACKD) is prevalent in end-stage renal disease (ESRD) patients.
  • ACKD involves small kidneys with multiple cysts, distinct from hereditary forms.
  • Pediatric data indicate a high ACKD incidence (21.6-45.8%) in children on dialysis.

Observation:

  • ACKD incidence correlates with longer dialysis duration in children.
  • Pathogenesis involves proto-oncogene activation.
  • Most ACKD cases are asymptomatic.

Findings:

  • High incidence of ACKD in pediatric dialysis patients mirrors adult rates.
  • Proto-oncogene activation is a key factor in ACKD development.
  • Renal cell carcinoma is a significant complication of ACKD.

Implications:

  • Routine surveillance for ACKD is crucial in at-risk populations.
  • Understanding ACKD pathogenesis may lead to targeted therapies.
  • Early detection of ACKD complications can improve patient outcomes.

Related Concept Videos

Chronic Kidney Disease I: Introduction01:25

Chronic Kidney Disease I: Introduction

Chronic Kidney Disease (CKD) arises when the kidneys progressively lose their ability to function, ultimately leading to end-stage renal disease. At this advanced stage, the kidneys can no longer filter waste or maintain essential body functions, requiring renal replacement therapy (RRT) through dialysis or a kidney transplant for survival.Early-stage chronic kidney disease and detection challengesIn CKD's early stages, symptoms often remain absent because healthy nephrons compensate for...
885
Chronic Kidney Disease III: Interprofessional Care01:28

Chronic Kidney Disease III: Interprofessional Care

Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
554
Acute Kidney Injury IV: Diagnostic Studies and Prevention01:30

Acute Kidney Injury IV: Diagnostic Studies and Prevention

Accurate diagnosis and effective prevention are critical in managing Acute Kidney Injury (AKI), which is linked to high mortality rates ranging from 10% to 80%. Timely recognition of at-risk patients and careful monitoring can significantly reduce the likelihood of kidney damage.Diagnostic Assessments:The diagnostic process starts with a comprehensive medical history to identify prerenal, intrarenal, and postrenal causes.Prerenal causes, such as dehydration, hypotension, or blood loss, should...
410
Nephrons01:10

Nephrons

The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
7.7K
Kidney Transplant I: Introduction01:28

Kidney Transplant I: Introduction

A kidney transplant is a surgical approach that involves replacing a non-functioning kidney with a healthy one from a donor. This procedure is often a treatment option for end-stage renal disease (ESRD) patients. The method requires careful recipient selection, including evaluating various medical and psychosocial factors. These criteria vary between transplant centers but generally include assessments of the patient's overall health, adherence to medical recommendations, and lifestyle...
600
Acute Kidney Injury I: Introduction01:22

Acute Kidney Injury I: Introduction

Introduction:Acute Kidney Injury (AKI) describes a swift decrease in kidney function occurring over hours to days, characterized by the kidneys' failure to remove waste products from the bloodstream. This leads to dangerous complications like metabolic acidosis, fluid overload, and electrolyte imbalances, such as hyperkalemia, which can cause life-threatening arrhythmias. AKI is common in both hospital and outpatient settings, often triggered by dehydration, sepsis, or exposure to nephrotoxic...
946