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Chronic kidney disease

Background

Chronic kidney disease (CKD) in children is a significant health problem that affects growth, development, and overall quality of life. CKD is characterized by the gradual loss of kidney function over time, resulting in the accumulation of waste products in the body, disturbances in electrolyte balance, and other systemic complications. It can also be defined by a glomerular filtration rate (GFR) of less than 60 mL/min/1.73 m² for more than three months or by kidney damage markers, such as proteinuria, for more than three months. While CKD is more commonly discussed in the context of adults, it is critical to understand its impact on pediatric patients, as early onset can lead to lifelong health challenges. The management of CKD in children requires a multidisciplinary approach to address the diverse and complex needs of these patients.

 

Discussion
Etiology

Top of FormBottom of FormThe causes of CKD in children vary by age and can include congenital abnormalities, hereditary conditions, and acquired diseases. Some of the common etiological factors include:

  • Congenital Anomalies of the Kidney and Urinary Tract (CAKUT):
    • These are the most common causes of CKD in children, accounting for up to 50% of cases. CAKUT encompasses a range of disorders, including renal dysplasia, obstructive uropathy, vesicoureteral reflux, and renal agenesis.
  • Hereditary Conditions:
    • Conditions such as polycystic kidney disease, Alport syndrome, and nephronophthisis can lead to CKD. These genetic disorders often manifest early in life and have a progressive course.
  • Glomerular Diseases:
    • Diseases such as focal segmental glomerulosclerosis (FSGS), membranoproliferative glomerulonephritis, and lupus nephritis can cause CKD. These conditions typically involve inflammation and damage to the glomeruli, the filtering units of the kidney.
  • Acquired Conditions:
    • Acquired causes include hemolytic uremic syndrome (HUS), chronic pyelonephritis, and systemic diseases such as sickle cell diseases, diabetes, and hypertension, though these are less common in pediatric populations compared to adults.
Pathophysiology

The pathophysiology of CKD involves a progressive decline in kidney function due to the loss of nephrons, the functional units of the kidney. This process occurs over several stages:

  • Initial Injury:
    • The initial injury to the kidneys, whether due to congenital, hereditary, or acquired causes, leads to damage of the nephrons. This damage results in a reduced glomerular filtration rate (GFR).
  • Compensatory Hypertrophy and Hyperfiltration:
    • In response to nephron loss, the remaining nephrons undergo hypertrophy and hyperfiltration to maintain overall kidney function. While this compensatory mechanism is initially beneficial, it eventually leads to further damage and scarring of the nephrons.
  • Progressive Nephron Loss:
    • As more nephrons are lost, the compensatory mechanisms become insufficient, and kidney function declines further. This leads to an accumulation of waste products, electrolyte imbalances, and other systemic effects.
  • End-Stage Renal Disease (ESRD):
    • When the GFR falls below a critical threshold, the kidneys can no longer sustain life without intervention, leading to ESRD. At this stage, renal replacement therapy, such as dialysis or kidney transplantation, becomes necessary.
Clinical Features

The clinical presentation of CKD in children can vary widely depending on the underlying cause, the stage of the disease, and the age of the child. Common clinical features include:

  • Growth Retardation:
    • Poor growth is a hallmark of CKD in children due to factors such as malnutrition, metabolic acidosis, and disturbances in hormone regulation.
  • Electrolyte Imbalances:
    • Hyperkalemia, hyponatremia, hypocalcemia, and hyperphosphatemia are common in CKD and can lead to symptoms such as muscle weakness, cramps, and cardiac arrhythmias.
  • Anemia:
    • Reduced production of erythropoietin by the diseased kidneys leads to anemia, resulting in fatigue, pallor, and decreased exercise tolerance.
  • Bone Disease:
    • CKD-mineral and bone disorder (CKD-MBD) results from disturbances in calcium, phosphorus, parathyroid hormone, and vitamin D metabolism, leading to bone pain, deformities, and fractures.
  • Cardiovascular Complications:
    • Hypertension is common in CKD due to fluid overload and dysregulation of the renin-angiotensin-aldosterone system (RAAS). Cardiovascular disease is a leading cause of morbidity and mortality in these patients. Examples include hypertension, congestive cardiac failure
  • Uremic Symptoms:
    • As CKD progresses, accumulation of uremic toxins can cause nausea, vomiting, pruritus, and neurological symptoms such as confusion and seizures.
Diagnosis

The Kidney Disease Improving Global Outcomes (KDIGO) staged CKD using GFR as follows:

The management of CKD in children is multifaceted and aims to slow disease progression, treat complications, and improve quality of life. Key aspects of management include:

  • Medical Management:
    • Blood Pressure Control: Angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin II receptor blockers (ARBs) are often used to manage hypertension and reduce proteinuria.
    • Electrolyte Management:
      • Interventions to correct electrolyte imbalances include dietary modifications, phosphate binders, and vitamin D analogs.
    • Anemia Management:
      • Erythropoiesis-stimulating agents (ESAs) and iron supplements are used to treat anemia.
    • Growth Hormone Therapy:
      • Recombinant human growth hormone (rhGH) can be used to promote growth in children with growth retardation.
  • Nutritional Management:
    • Nutritional support is critical for growth and development. This includes ensuring adequate calorie and protein intake, managing electrolyte balance, and supplementing vitamins and minerals as needed.
  • Psychosocial Support:
    • Children with CKD and their families often require psychological and social support to cope with the chronic nature of the disease, the demands of treatment, and the impact on quality of life.
  • Renal Replacement Therapy:
    • When CKD progresses to ESRD, renal replacement therapy becomes necessary. Options include:
      • Dialysis:
        • Hemodialysis and peritoneal dialysis are two main types. The choice depends on factors such as age, lifestyle, and medical condition.
      • Kidney Transplantation:
        • Transplantation offers the best long-term outcomes and quality of life for children with ESRD. Preemptive transplantation, before the need for dialysis, is often pursued when feasible.
  • Multidisciplinary Care:
    • A multidisciplinary team approach, involving pediatric nephrologists, dietitians, social workers, psychologists, and other specialists, is essential for comprehensive care of children with CKD.

Chronic Kidney Disease (CKD) in children is a progressive condition that can lead to severe health complications if untreated, especially in low-income settings where access to specialized care is limited. Early detection, regular monitoring, and management of underlying causes, such as infections and malnutrition, are essential to slow disease progression. Strengthening healthcare infrastructure, improving access to medications, and raising awareness about preventive measures are key to reducing the impact of CKD in children in resource-limited regions.

Interesting patient case

A 12-year-old boy with initials AB was diagnosed with focal segmental glomerulosclerosis (FSGS) and required emergency management. Despite initial medical treatments, his kidney function progressively deteriorated over several months with a progressively declining glomerular filtration rate, GFR. leading to the need for hemodialysis. Over several months, AB underwent regular hemodialysis sessions, showing resilience and courage. However, due to the lack of facilities for kidney transplantation in the facility, his condition became critical. Tragically, AB passed away, highlighting the urgent need for better healthcare infrastructure and resources for children with chronic kidney diseases.

 

 

 

Further readings
  1. Warady, B. A., & Chadha, V. (2007). Chronic kidney disease in children: the global perspective. Pediatric Nephrology, 22(12), 1999-2009.
  2. Harambat, J., van Stralen, K. J., Kim, J. J., & Tizard, E. J. (2012). Epidemiology of chronic kidney disease in children. Pediatric Nephrology, 27(3), 363-373.
  3. National Kidney Foundation. (2012). KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney International Supplements, 3(1), 1-150.
  4. Rees, L., & Shroff, R. (2015). The demise of CKD-MBD? Pediatric Nephrology, 30(2), 203-213.
  5. Greenbaum, L. A., Warady, B. A., & Furth, S. L. (2009). Current advances in chronic kidney disease in children: growth, cardiovascular, and neurocognitive risk factors. Seminars in Nephrology, 29(4), 425-434.
  6. Hogg, R. J., Furth, S., Lemley, K. V., Portman, R., Schwartz, G. J., Coresh, J., … & Warady, B. (2003). National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics, 111(6), 1416-1421.
  7. Wong, C. S., Pierce, C. B., Cole, S. R., Warady, B. A., Mak, R. H., Benfield, M. R., … & Furth, S. L. (2009). Association of proteinuria with race, cause of chronic kidney disease, and glomerular filtration rate in children. Clinical Journal of the American Society of Nephrology, 4(4), 812-819.
  8. Haffner, D., & Schaefer, F. (2010). Obesity and cardiovascular risk in children with chronic kidney disease. Nature Reviews Nephrology, 6(8), 495-503.
  9. National Institute for Health and Care Excellence (NICE). (2013). Chronic kidney disease (stage 4 or 5): management of hyperphosphataemia. NICE clinical guideline [CG157].
  10. Gipson, D. S., Wiggins, R. C., & Fitzgibbons, L. (2006). Pediatric chronic kidney disease. Pediatric Clinics, 53(4), 773-797.
  11. Cochat, P., & Shaw, V. (2013). Nutrition in pediatric chronic kidney disease. Pediatric Nephrology, 28

Author's details

Reviewer's details

Chronic kidney disease

Chronic kidney disease (CKD) in children is a significant health problem that affects growth, development, and overall quality of life. CKD is characterized by the gradual loss of kidney function over time, resulting in the accumulation of waste products in the body, disturbances in electrolyte balance, and other systemic complications. It can also be defined by a glomerular filtration rate (GFR) of less than 60 mL/min/1.73 m² for more than three months or by kidney damage markers, such as proteinuria, for more than three months. While CKD is more commonly discussed in the context of adults, it is critical to understand its impact on pediatric patients, as early onset can lead to lifelong health challenges. The management of CKD in children requires a multidisciplinary approach to address the diverse and complex needs of these patients.

 

  1. Warady, B. A., & Chadha, V. (2007). Chronic kidney disease in children: the global perspective. Pediatric Nephrology, 22(12), 1999-2009.
  2. Harambat, J., van Stralen, K. J., Kim, J. J., & Tizard, E. J. (2012). Epidemiology of chronic kidney disease in children. Pediatric Nephrology, 27(3), 363-373.
  3. National Kidney Foundation. (2012). KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney International Supplements, 3(1), 1-150.
  4. Rees, L., & Shroff, R. (2015). The demise of CKD-MBD? Pediatric Nephrology, 30(2), 203-213.
  5. Greenbaum, L. A., Warady, B. A., & Furth, S. L. (2009). Current advances in chronic kidney disease in children: growth, cardiovascular, and neurocognitive risk factors. Seminars in Nephrology, 29(4), 425-434.
  6. Hogg, R. J., Furth, S., Lemley, K. V., Portman, R., Schwartz, G. J., Coresh, J., … & Warady, B. (2003). National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics, 111(6), 1416-1421.
  7. Wong, C. S., Pierce, C. B., Cole, S. R., Warady, B. A., Mak, R. H., Benfield, M. R., … & Furth, S. L. (2009). Association of proteinuria with race, cause of chronic kidney disease, and glomerular filtration rate in children. Clinical Journal of the American Society of Nephrology, 4(4), 812-819.
  8. Haffner, D., & Schaefer, F. (2010). Obesity and cardiovascular risk in children with chronic kidney disease. Nature Reviews Nephrology, 6(8), 495-503.
  9. National Institute for Health and Care Excellence (NICE). (2013). Chronic kidney disease (stage 4 or 5): management of hyperphosphataemia. NICE clinical guideline [CG157].
  10. Gipson, D. S., Wiggins, R. C., & Fitzgibbons, L. (2006). Pediatric chronic kidney disease. Pediatric Clinics, 53(4), 773-797.
  11. Cochat, P., & Shaw, V. (2013). Nutrition in pediatric chronic kidney disease. Pediatric Nephrology, 28

Content

Author's details

Reviewer's details

Chronic kidney disease

Chronic kidney disease (CKD) in children is a significant health problem that affects growth, development, and overall quality of life. CKD is characterized by the gradual loss of kidney function over time, resulting in the accumulation of waste products in the body, disturbances in electrolyte balance, and other systemic complications. It can also be defined by a glomerular filtration rate (GFR) of less than 60 mL/min/1.73 m² for more than three months or by kidney damage markers, such as proteinuria, for more than three months. While CKD is more commonly discussed in the context of adults, it is critical to understand its impact on pediatric patients, as early onset can lead to lifelong health challenges. The management of CKD in children requires a multidisciplinary approach to address the diverse and complex needs of these patients.

 

  1. Warady, B. A., & Chadha, V. (2007). Chronic kidney disease in children: the global perspective. Pediatric Nephrology, 22(12), 1999-2009.
  2. Harambat, J., van Stralen, K. J., Kim, J. J., & Tizard, E. J. (2012). Epidemiology of chronic kidney disease in children. Pediatric Nephrology, 27(3), 363-373.
  3. National Kidney Foundation. (2012). KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney International Supplements, 3(1), 1-150.
  4. Rees, L., & Shroff, R. (2015). The demise of CKD-MBD? Pediatric Nephrology, 30(2), 203-213.
  5. Greenbaum, L. A., Warady, B. A., & Furth, S. L. (2009). Current advances in chronic kidney disease in children: growth, cardiovascular, and neurocognitive risk factors. Seminars in Nephrology, 29(4), 425-434.
  6. Hogg, R. J., Furth, S., Lemley, K. V., Portman, R., Schwartz, G. J., Coresh, J., … & Warady, B. (2003). National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics, 111(6), 1416-1421.
  7. Wong, C. S., Pierce, C. B., Cole, S. R., Warady, B. A., Mak, R. H., Benfield, M. R., … & Furth, S. L. (2009). Association of proteinuria with race, cause of chronic kidney disease, and glomerular filtration rate in children. Clinical Journal of the American Society of Nephrology, 4(4), 812-819.
  8. Haffner, D., & Schaefer, F. (2010). Obesity and cardiovascular risk in children with chronic kidney disease. Nature Reviews Nephrology, 6(8), 495-503.
  9. National Institute for Health and Care Excellence (NICE). (2013). Chronic kidney disease (stage 4 or 5): management of hyperphosphataemia. NICE clinical guideline [CG157].
  10. Gipson, D. S., Wiggins, R. C., & Fitzgibbons, L. (2006). Pediatric chronic kidney disease. Pediatric Clinics, 53(4), 773-797.
  11. Cochat, P., & Shaw, V. (2013). Nutrition in pediatric chronic kidney disease. Pediatric Nephrology, 28
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