ERKNet

The European Rare Kidney Disease Reference Network

  

 

 

Thematic Area: CKD-MBD

 
The following guidance documents have been adopted based on standardized reviews and are followed in all ERKNet centers:

1) KDIGO Clinical Practice Guideline for the Diagnosis, Evaluation, Prevention, and Treatment of Chronic Kidney Disease-Mineral and Bone Disorder (CKD-MBD) (including 2017 update)

 Kidney Int Supp. 2009; 113: S1-130 


Core Recommendations:
  1. In children with CKD 3-5D maintain serum calcium in the age-appropriate normal range.  
  2. It is reasonable to base the choice of phosphate-lowering treatment on serum calcium levels  
  3. Calcitriol and vitamin D analogs may be considered to maintain serum calcium levels in the age-appropriate normal range  
 
Comments by evaluators:
  • In some guidelines the distinction between adults and children is not always clear.
  • The described rationale is solid and based on systematically gathered information.

 

 

2) Prevention and Treatment of Renal Osteodystrophy in Children on Chronic Renal Failure: European Guidelines. 

Pediatr Nephrol 2006; 21: 151-9 

 

 

Core Recommendations:
  1. Clinical, biochemical and radiological markers of renal bone disease should be monitored regularly  
  2. Metabolic acidosis should be corrected  
  3. For control of hyperphosphataemia aluminium free phosphate binders should be administered  
  4. Vitamin D deficiency should be avoided 
  5. Marked hyperparathyroidism should be avoided and PTH levels should be kept at two to three times the upper limit of the normal range in end-stage renal disease
  6. Treatment with growth hormone should not be started in the presence of severe hyperparathyroid bone disease
  7. In case of hypercalcemia, active vitamin D metabolites and calcium-containing phosphate binders should be stopped and dialysate changed to low-calcium solutions.
  8. Parathyroidectomy has to be considered in case of severe, therapy-refractory hyperparathyroidism with radiological signs in combination with hypercalcemia and/or elevated calcium phosphorus product
 
Comments by evaluators:
  • This guideline was published in 2006 before rigorous guideline development methodology was defined and systems such as GRADE were available. The guideline is widely quoted and used in many paediatric nephrology centres around Europe. Currently, there is no better evidence to challenge or change these recommendations.
  • The recommendation on parathyroidectomy may be outdated, as cinacalcet use should be considered before considering surgery.
  • The discipline/content expertise and the description of the members’ role in the guideline is not always clear.

 

 

3) Clinical Practice Recommendations for Native Vitamin D Therapy in Children with CKD Stages 2-5 and on Dialysis

 Nephrol Dial Transplant  2017 Jul 1;32(7):1098-1113

Core Recommendations:
  1. Measure serum 25(OH)D concentration for assessing the vitamin D status of children with CKD 2-5D. Measurement is recommended 6 – 12 monthly, depending on the CKD stage  
  2. Maintain serum 25(OH)D levels above 75nMol/L  (>30ng/ml)  
  3. A treatment regimen, guided by age and vitamin D concentration, for the prevention and treatment of vitamin D deficiency is suggested. An intensive replacement phase followed by a maintenance phase of treatment is recommended  
  4. Mega-dose vitamin D therapy is not recommended 
  5. Vitamin D supplementation must be stopped at serum 25(OH)D concentrations above 120nMol/L (48ng/ml). Symptomatic toxicity from Vitamin D is defined as serum 25(OH)D above 250nMol/L with hypercalcaemia, hypercalciuria and suppressed PTH
 
Comments by evaluators:
  • Very helpful guideline in a field where evidence and evidence-based guidelines are missing

 

4) Clinical Practice Recommodations for Treatment with Active Vitamin D Analogues in Children with CKD Stage 2-5 and on Dialysis

    Nephrol Dial Transplant 2017 Jul 1;32(7):1114-1127

Core Recommendations:
  1. Suggest vitamin D analogues in children with CKD 2-5D who have persistently increased serum PTH concentrations above the CKD specific target range
  2. Any vitamin D analogue can be used to reduce PTH levels.
  3. Start vitamin D analogues in the lowest dose to achieve target PTH concentrations and maintain normocalcemia. Subsequent titration of vitamin D therapy may be performed based on trends in serum calcium, phosphate and PTH level. 
 
Comments by evaluators: