Chronic Kidney Disease

Transcription

1 Chronic Kidney Disease Title of Guideline (must include the word Guideline (not protocol, policy, procedure etc) Contact Name and Job Title (author) Directorate & Speciality Guideline for the Identification & Management of Chronic Kidney Disease in Children & Young People Dr Andrew Lunn Paediatric Nephrology Consultant Dr Jonathan Evans Paediatric Nephrology Consultant Family Health Children - Renal Date of submission Jan 2017 Date on which guideline must be reviewed (this should be one to three years) Guideline Number Jan 2022 Explicit definition of patient group to which it applies Children and Young People referred to Nottingham (e.g. inclusion and exclusion criteria, diagnosis) Children s Hospital Renal and Urology Unit with Chronic Kidney Disease. Abstract This guideline describes the identification and management of Chronic Kidney Disease in Children & Young People including pathways for assessment and referral across the EMEESY Children s Renal Network. Key Words Paediatrics, Chronic Kidney Disease, CKD, GFR, Children, Young People, Renal Statement of the evidence base of the guideline has the guideline been peer reviewed by colleagues? 1a meta analysis of randomised controlled trials 2a 2b at least one well-designed controlled study without randomisation at least one other type of well-designed quasi-experimental study 3 well designed non-experimental descriptive studies (ie comparative / correlation and case studies) 4 expert committee reports or opinions and / or clinical experiences of respected authorities 5 recommended best practise based on the clinical experience of the guideline developer Consultation Process Children s Renal Unit guideline review, Paediatric Clinical Guidelines Group Target audience X Clinicians and healthcare professionals caring for children and young people with CKD at Nottingham University Hospitals NHS Trust and across the EMEESY Children s Renal Network. This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date. Document Control Andrew Lunn 1 January 2017

2 Document Amendment Record Version Issue Date Author V1 Jan 2017 Dr Lunn, Dr Evans V2 General Notes: Summary of changes for new version: New guideline Statement of Compliance with Child Health Guidelines SOP This guideline refers to activities of only one specific team and consultation has taken place with relevant members of that team. Therefore this version has not been circulated for wider review. Martin Hewitt Clinical Guideline Lead Andrew Lunn 2 January 2017

3 Chronic Kidney Disease in Children & Young People: Identification & Management 1. Chronic Kidney Disease: Definition and Classification Background Aims and Objectives Family and Person Centred Care, Information and Education Identification of patients with CKD Prevention of disease progression Proteinuria Hypertension Acute Kidney Injury Significant urinary tract abnormalities Preparation for CKD 5 (transplantation and dialysis) Cardiovascular risk The management of CKD complications Bone and Mineral Disorders Acidosis Anaemia Diet and Lifestyle Growth Transition Appendix Quick Reference Guides Quick Reference Guide Stage Quick Reference Guide Stage Quick Reference Guide Stage Quick Reference Guide Stage Quick Reference Guide Stage Andrew Lunn 3 January 2017

4 GFR categories (ml/min/1.73m 2 ), description and range Incre asing risk 1. Chronic Kidney Disease: Definition and Classification A definition of Chronic Kidney Disease (CKD) was developed to identify conditions which had the possibility of progressive loss of kidney function or complications from reduced kidney function. It has been modified to include urine albumin:creatinine ratio (ACR) as an additional marker of disease progression. This is based on adult patient data. Classification of chronic kidney disease using GFR and ACR categories GFR and ACR categories and risk of adverse outcomes ACR categories (mg/mmol), description and range <3 Normal to mildly increased 3 30 Moderately increased >30 Severely increased A1 A2 A3 90 Normal and high Mild reduction related to normal range for a young adult G1 G2 No CKD in the absence of markers of kidney damage Mild moderate reduction G3a Moderate severe reduction G3b Severe reduction G4 <15 Kidney failure G5 Increasing risk Abbreviations: ACR, albumin:creatinine ratio; CKD, chronic kidney disease; GFR, glomerular filtration rate Adapted with permission from Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group (2013) KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney International (Suppl. 3): Andrew Lunn 4 January 2017

5 2. Background Chronic kidney disease is rare in childhood with a prevalence of CKD stage 3-4 of 50 per million population and stage 5 (dialysis and transplantation) of 56 per million population. Optimum management therefore needs centralised expertise co-ordinating with local centres through a network to deliver care close to the patients home whenever possible. There is considerable emerging evidence in relation to prevention of CKD, management of CKD progression and the high cardiovascular risks associated with CKD. There is now a significant evidence base in support of stricter control of blood pressure, reduction in proteinuria, optimising vitamin D levels and control of calcium intake and vitamin D analogues. If we are to be effective in adopting new preventative and management strategies we will need to identify children with early CKD across our network. This therefore requires a co-ordinated approach across our clinical network. This document sets out guidelines for our own practice and how they should be applied across our clinical network. The quick reference guides (QRG) define how the patient pathway should be continuous across the network. Andrew Lunn 5 January 2017

6 3. Aims and Objectives The aim of this document is to provide guidelines for local practice and across our network to improve the care of children with CKD. The objective of this guideline is therefore to; 1) Increase identification of all patients with CKD 2) Increase use of appropriate treatment to prevent progression of CKD 3) Identify, prevent and treat cardiovascular risk factors 4) Prevent and manage other complications of CKD principally a. Anaemia b. Calcium, Phosphate and Vitamin D metabolism 5) Ensure achievement of growth potential 6) Ensure achievement of educational potential in children with CKD 7) Identify and address psychosocial concerns 8) Improve the co-ordination of care and the transition between care delivered locally by locally teams, care delivered locally by specialist teams and care delivered in a specialist centre by specialist teams 9) Help prepare children with CKD stage 4 and their families for end-stage renal disease management (dialysis and/or transplantation). Andrew Lunn 6 January 2017

7 4. Family and Person Centred Care, Information and Education We will involve young people, children and their families in all decisions about their care using explanations relevant to their needs and level of understanding taking into account their religious, cultural and language needs. We will offer resources to the young people, children and their families including; Discussion in clinic Written information through copies of clinic letters and additional correspondence Online information i.e. Information leaflets at Access to Patient View Offer participation in registry and research studies as applicable. As required this may also include Specific meetings for discussion eg. CKD assessment day Specific training to allow delivery of care at home e.g. dialysis training Play preparation for procedures Home visits School visits The young person, child and families will be offered support as relevant and as needed. This includes support from Treatment specific specialist nurses Play therapist Youth worker Social Worker Child psychologist Hospital schoolteacher All families can contact specialist nurses using the renal pager. Members of the team will contact and, if required, arrange a visit to the child s or young person s home to ensure that they are informed and supported regarding the treatment. Members of the team will contact and, if required, arrange a visit to the child s nursery, school or college to ensure that they are informed and supported regarding the treatment. Appropriate links will be established with local resources and services for the families if they live some distance from the unit. We will offer children, young people and their families suport through our Facebook page including information about parent support groups or family days. Andrew Lunn 7 January 2017

8 The needs of siblings of children and young people with CKD are also considered and addressed as needed. Social workers will offer emotional and practical support to increase the well-being of children, young people and their families. They will also help to reduce the financial impact of looking after a child or young person with CKD. Adolescent patients will require an additional profile of education plans, social issues and careers advice. Transition to adult units will also be discussed in conjunction with the family, youth worker and other team members using the Ready, Steady, Go framework. Referral to the child psychologist will be made when appropriate. A hospital schoolteacher will link with all children and their schools and ensure continuity in teaching when required. Departmental meetings will include the psychological and social needs of the child, young person and their families as part of the development and discussion of individual management plans. In return we ask that parents and young people; 1. Update us with contact details ie home address and telephone number to allow us to communicate effectively 2. Treat us politely and with respect Andrew Lunn 8 January 2017

9 5. Identification of patients with CKD Rationale; Early identification of patients with CKD allows treatment to prevent progression of CKD and reduce complications. Methods of Identification 1) Serum creatinine and estimated GFR (egfr) All patients who have a serum creatinine checked should have a GFR estimated. In Nottingham this is done automatically for children > 2yrs of age. If automated egfr reporting is not available then the following estimate should be used; egfr = k x Height (cm) ml/min per 1.73m 2 Serum Creatinine (µmol/l) Where possible this constant, k, should be verified with local laboratory findings. For example in Nottingham it has been determined that the most accurate constant is k=30 for all females and for males under 13 years and k=36 for males 13 years or older. In the absence of this data the k value most commonly used is 40. An egfr less than 90 ml/min per 1.73m 2 should prompt clinical evaluation and consideration of further investigation and referral. See guideline Abnormal GFR referral Andrew Lunn 9 January 2017

10 2) Measured GFR ( 51 Cr-EDTA GFR) A number of blood tests of significant volume are required for a measured GFR. Usually children 5 years or older are able to undertake this procedure and an estimated GFR is often sufficient in children below this age. A measured GFR should be done in all patients over 5 years of age with: a. Bilateral renal abnormalities e.g. bilateral renal scarring b. Significant bilateral obstructive uropathy e.g. posterior urethral valve c. Significant abnormality in solitary functioning kidney d. One year following acute kidney injury requiring specialist nephrology care e. Patients with non-renal disease receiving frequent nephrotoxic drugs e.g. cystic fibrosis, following chemotherapy A GFR should also be considered in patients over 5 years of age with: a. Evidence of renal disease in patients with abnormal body habitus such that the egfr may be inaccurate. b. Significantly abnormal egfr but with no evidence of renal disease on further investigation. c. Patients at risk of reduced nephron mass e.g ex-preterm infants, IUGR, significant neonatal hypoxia d. A formal GFR may be repeated in patients with known renal disease usually 5 years or more after a previous GFR to document progression. This is usually done once growth is complete to document final GFR as part of transition planning 3) Proteinuria a. All patients at risk of CKD should have a urine albumin:creatinine quantified at least annually b. All patients at risk of CKD should have a urine dipstick tested for protein at each clinic visit 4) Hypertension a. All patients at risk of CKD should have a blood pressure measured at each clinic visit and the blood pressure centile documented Andrew Lunn 10 January 2017

11 b. Annual 24hr ABPM should be considered for patients in CKD3 or worse and target BP control at 50 th 75 th centile with ACE inhibition. 5) Other Patients may also be identified with abnormal renal tract investigations, hypertension and / or abnormal urinalysis. The management is that of the underlying disease with assessment of GFR as required. Further investigations and management would then proceed as per the stage of CKD. Andrew Lunn 11 January 2017

12 6. Prevention of disease progression There is variable evidence to clearly define risk factors for progression of CKD in children. The following however have some evidence and are amenable to treatment; Proteinuria Hypertension AKI Severe urinary tract abnormalities particularly if associated with recurrent UTI or voiding dysfunction Hypercalcaemia Andrew Lunn 12 January 2017

13 Proteinuria 1) Albumin:creatinine ratio (ACR) is more sensitive than protein:creatinine ratio at lower levels of proteinuria and should be used in patients with CKD 2) Proteinuria should be confirmed on a first morning urine sample unless ACR > 70 mg/mmol 3) The following groups of patients with CKD and proteinuria should be treated with an ACE inhibitor unless contraindicated: a. Diabetes with ACR > 3 mg/mmol b. Hypertension and ACR > 30 mg/mmol c. ACR > 70 mg/mmol Andrew Lunn 13 January 2017

14 Hypertension 1) See separate guideline for investigation and management of patients presenting with hypertension who are not known to have CKD. 2) Hypertension is a risk factor for progression of CKD 3) ACE inhibitors unless contraindicated should be used as a first line agent in patients with known CKD 4) Treatment should be instigated if a manual blood pressure is persistently above the 75 th centile 5) Treatment should be increased as tolerated until the blood pressure is maintained below the 75 th centile 6) Patients on treatment for hypertension should have an annual ECHO for LVH 7) Patients with CKD 4/5 should have an annual ECHO. 8) 24 hour ABPM or home blood pressure monitoring can be used in patients to monitor blood pressure control or if white coat hypertension is suspected 9) 24 hour ABPM or home blood pressure monitoring should be considered annually in patients with CKD 3-5 Andrew Lunn 14 January 2017

15 Acute Kidney Injury Patients with CKD are at greater risk of acute kidney injury (AKI). This can lead to progression of CKD. Early identification of AKI and appropriate management is important to reduce the impact of AKI on CKD progression. More details can be found in the AKI guideline and at the Think Kidneys website. Points of particular reference to patients with CKD are: 1) Discuss all patients with CKD and AKI with a paediatric nephrologist or SPIN 2) Avoidance of nephrotoxic medication and appropriate dose adjustment a. Avoid NSAID b. Avoid aminoglycosides and vancomycin if possible. If cannot be avoided then adjust dose for level of CKD and ensure appropriate monitoring (see for more pharmacy information) c. Ensure patients on ACE-Inhibitors are informed of sick day rules and the medication stopped if they have AKI or are dehydrated d. Monitor drug levels of patients on calcineurin inhibitors if they develop diarrhoea or have AKI 3) Check U&E in patients with CKD who have a clinical history consistent with an illness that may cause dehydration even if no clinical signs of dehydration. 4) Ensure appropriate fluid management to prevent dehydration see guideline on fluid management of patients with CKD. 5) Ensure adequate monitoring of CKD patients admitted with AKI to include blood pressure, strict input and output monitoring, daily weights and age appropriate early warning score. 6) Patients with CKD who are undergoing surgery should be discussed with the relevant consultant (either paediatric nephrologist or SPIN) to ensure appropriate peri-operative fluid management. Andrew Lunn 15 January 2017

16 Significant urinary tract abnormalities Patients with a significant urinary tract abnormality particularly if associated with recurrent UTI or voiding dysfunction should be reviewed in conjunction with a MDT including urologists, specialist nurses, radiologist and nephrologists. Advice and measures should be taken to avoid recurrent UTI (See UTI guideline and leaflet) Assessment of bladder function should be considered including, where relevant, Home assessment of voiding pattern (frequency / volume chart) Assessment of residual bladder volumes Assessment of urinary flow rates Assessment of bladder pressure studies Andrew Lunn 16 January 2017

17 Preparation for CKD 5 (transplantation and dialysis) Unfortunately despite the above measures patients with CKD can still progress. Consideration in all patients should therefore be given to the following; 1) Immunisations - A full vaccination history should be taken, and verified with the patients General Practitioner (G.P). The schedule of immunisations set out in current Guidelines on Immunisations and Immunity testing for children with Chronic Renal Failure or a Renal Transplant should be followed. 2) Approximately half of children with CKD stage 3 progress to needing renal replacement therapy (RTT) prior to adulthood. 3) Children with CKD 4 will inevitably progress to needing RRT. 4) Children with CKD 4 should be under the care of a multi-disciplinary paediatric nephrology team with expertise in dialysis and transplantation. 5) Children with CKD 4 and their carers should a. Have treatment of CKD optimised b. Be given information about options regarding RRT including as relevant i. Living donor renal transplantation ii. Deceased donor renal transplantation iii. Peritoneal dialysis iv. Haemodialysis v. Palliative care 6) In suitable patients the intention would be to prepare the child for a preemptive renal transplant from a living donor if available. Andrew Lunn 17 January 2017

18 7. Cardiovascular risk CKD is a risk factor for future cardiovascular disease. Reducing cardiovascular risk includes optimal management of CKD and monitoring for other risk factors for CKD. There is evidence of sub-clinical cardiovascular changes in children with CKD. Optimise CKD management to reduce cardiovascular risk includes treatment of: 1) Hypertension 2) Proteinuria 3) CKD related calcium, phosphate and vitamin D abnormalities (see below) Other modifiable factors to be monitored include: 1) Obesity Those identified as being obese should be encouraged to have diet modification, lifestyle modification and where available referred to local services for the management of childhood obesity. 2) Hyperlipidaemia in those at risk and consider a. Familial hypercholesterolaemia b. Patients with uncontrolled nephrotic range proteinuria c. Obesity Management includes treatment of the underlying cause, diet modification and consideration of the use of statins. 3) Vitamin D deficiency ( see below) Andrew Lunn 18 January 2017

19 8. The management of CKD complications Bone and Mineral Disorders Abnormal calcium, phosphate and vitamin D metabolism can be detected from CKD stage 2 onwards. Adverse effects included Abnormal bone growth (both adynamic bone disease and renal osteodystrophy) Cardiovascular calcification Increased risk of cardiovascular disease in adults with CKD Principles of treatment include early intervention and prevention address phosphate with dietary restriction in the first instance minimise dose of phosphate binders and use of the smallest dose of vitamin D analogues to maintain PTH in target range For more information see separate guideline. Andrew Lunn 19 January 2017

20 Acidosis 1) Acidosis can be present as a primary consequence of renal tract abnormality e.g. renal tubular acidosis or secondary to CKD. 2) If secondary to CKD acidosis is usually associated with advanced CKD (stage 4-5). If acidosis is present in CKD stages 1-3 an alternative cause should be considered. 3) Treatment of acidosis should be commenced with an oral sodium bicarbonate supplement if the serum bicarbonate is persistently below 20 mmol/l. Andrew Lunn 20 January 2017

21 Anaemia Anaemia of CKD is the result of deficient erythropoesis due to reduced renal erythropoietin synthesis. It is rare in CKD1-2 but is found in 30-70% of children in CKD 3-5. Investigation The cause of anaemia is usually easily identified and iron deficiency and anaemia of CKD account for most cases. Additional investigations are only required if the clinical picture suggests an alternative cause (e.g severe anaemia in CKD1-2) or there is a poor response to treatment. Management The aim is to correct Hb levels to a level that is safe and prevents symptoms or CVS complications of anaemia without introducing the cardiovascular risks associated with overuse of erythropoietin-stimulating agent (ESA) and IV iron. More details can be found in our Anaemia guideline. Andrew Lunn 21 January 2017

22 9. Diet and Lifestyle All children with CKD will be referred to a named Paediatric Dietitian for nutritional assessment and on-going dietary support. Patients with CKD stage 2/3 in shared care clinics will be referred to a paediatric dietitian for a moderate phosphate restriction and a reduced salt diet. Local paediatric dietitians will have support from specialist renal dietitians based in Nottingham and the opportunity to attend an annual training day. All children with CKD stages 3-5 that are transferred to clinic in Nottingham will be referred to a paediatric renal dietitian for nutritional assessment and on-going dietary support. Each patient requires an evaluation of dietetic care at clinic visits and during ward admissions. Telephone contact at home should be available for each family in between ward and clinic visits. A visit to home and school/nursery with other team members will be co-ordinated if required. The oral route should always be considered as the preferred route for providing nutrition. The enteral tube feeding route (gastrostomy or nasogastric tube) with use of nutritional supplements (Appendix) should be considered when: 1. the child s oral intake fails to meet 80% of Reference Nutrient Intake (RNI) 2. the child fails to maintain adequate growth velocity (for example, when weight, height and/or head circumference measurements begin to fall from percentiles and height velocity is consistently below the 25th percentile) 3. and other non-nutritional causes are excluded. Dietary Assessment In order to assess nutritional status upon referral a 24 hour recall or 3-day dietary diary should be undertaken and repeated as required. In infants or children / young people with specific dietary concerns frequent adjustments may be required. Andrew Lunn 22 January 2017

23 Where there are concerns that dietary intake is inadequate computer analysis of a 3 day food diary should be completed and compared with recommended nutrient intakes for children of the same sex and chronological age: If the child is within the normal percentile ranges for height (>2nd percentile), energy and micronutrient requirements can be based upon the recommendations for children of the same chronological age. If the child falls below the normal percentile ranges for height (<2nd percentile), energy and micronutrient requirements may be based upon the recommendations for children of the same height age. The dietary assessment should include: 1. Adequacy of energy and regulation of protein intakes sufficient for growth but regulated to minimise blood urea levels. 2. Fluid balance 3. Review of calcium and phosphate intake and the use of binders. 4. Review of electrolyte intake especially potassium. 5. Adequacy of micronutrient and iron intakes. Micronutrient Supplements Children and young people should be prescribed a vitamin and mineral supplement if dietary intake does not meet 100% of recommended nutrient intake (RNI). At present there is no specific vitamin and mineral supplement for children with CKD. At present we are using: 1. Ketovite Contains B vitamins, Folate, Vitamin E, K and C. < 5 years of age 1 tablet daily > 5 years x 2 tablets daily 2. Renavit B and C vitamin complex for adult patients with CKD, can be used from 15 years Amounts/doses can be adjusted depending on patients assessed intake. Lifestyle Patients with CKD should be encouraged to have a healthy diet and a healthy amount of exercise. Andrew Lunn 23 January 2017

24 Where relevant advice regarding smoking and alcohol intake should be given to young people with CKD. Andrew Lunn 24 January 2017

25 10. Growth Basic Growth parameters (Weight, Height or Length) should be measured and interpreted ie growth chart centile noted at each clinic visit. Head Circumference should be measured and interpreted ie growth chart centile noted in children under 2 years of age. The reliable assessment of growth requires staff that have received training in the use of appropriate measuring techniques and equipment. Weight* - Supine Length* - Standing Height - Head circumference Body Mass Index - Infants should be weighed in the nude Older children should be weighed in light clothing without shoes Recommended up to the age of 2 years on a flat surface Taken without shoes, with the child standing with heels and back in contact with an upright wall using a height stadiometer Measured using a non-stretchable measuring tape Wt. (kg)/ht 2 (cm) and plotted on a BMI chart. To be assessed yearly in dialysis patients and when required in all other patients *For premature infants (<30/40) correct for gestational age up to 2 years of age, for premature infants (>30/40) correct for gestational age up to 1 year of age Andrew Lunn 25 January 2017

26 11. Transition 1. The issue of transition to adult services is a particularly important to young people with CKD because of the extent of contact time with hospital services. 2. A transition plan agreed by the multi-professional team and young person should be initiated when transfer is first discussed, usually as the young person is starting secondary school. 3. The aim is to prepare young people for adulthood in general and also to provide information to enable them to advocate on their own behalf in relation to their treatment using the Ready, Steady, Go framework. 4. When time of transition approaches, the named consultant should approach a consultant at the young person s local renal services provider, to make them aware of the patient s needs and agree with the young person a schedule of transition appointments and a plan for a transfer date. 5. For patients who are on dialysis or have received a kidney transplant the named nurse should create links with their appropriate counterpart. 6. For patients who are on dialysis the named nurse should enquire about practices and policies in the adult unit and whenever possible, these should be mimicked at the paediatric unit. 7. For patients who are on in-centre haemodialysis at least 2-3 months before transfer a visit should be arranged to the adult unit for the young person and family to look around and meet relevant team members there. 8. Ultimately the age of transition should be multi-factorial o Young people should participate in all discussions related to transfer, and their wishes should be respected. o Whenever possible the young person s condition should be stable at the time of transfer. o Other factors should also be considered e.g. Growth Educational needs. Andrew Lunn 26 January 2017

27 12. Appendix Quick Reference Guides Quick Reference Guide Stage 1 Definition Kidney damage with GFR 90 ml/min/1.73m 2 Frequency of monitoring Referral As per underlying diagnosis Management would usually be co-ordinated by general paediatrician in district general hospital. Comments Consideration of formal GFR. Specialist services such as urology and nephrology to be considered on case by case basis. All cases with abnormality in solitary kidney should be discussed with tertiary nephrology and / or arrange review in outreach clinic. Anaemia Investigate for iron deficiency. Target normal range. Unlikely to be related to CKD Hypertension Target normal blood pressure. Initial treatment is diet and lifestyle advice. If medical treatment required consider ACE-inhibitor unless renal artery disease or other contraindication. Discuss with SPIN doctors or tertiary nephrologist and / or arrange review in outreach clinic. Acidosis Calcium, phosphate and vitamin D Proteinuria Conventional cardiovascular risk factors Diet and lifestyle Growth Transition Treat persistent metabolic acidosis with sodium bicarbonate If abnormal calcium or phosphate investigate for vitamin D deficiency. Persistent proteinuria on early morning sample is risk of disease progression. Consideration of smoking, weight and co-morbidities e.g. hyperlipidaemia, diabetes Low salt diet and healthy lifestyle Expectation is of normal growth. Nephrology care likely to be via GP If not consistent with underlying renal disorder then consider other diagnosis. Treat vitamin D deficiency. Unlikely to be related to CKD. Discuss with SPIN doctors or tertiary nephrologist and / or arrange review in outreach clinic. Routine growth monitoring at clinic reviews. May need referral to adult specialists for other follow-up e.g. urology. Andrew Lunn 27 January 2017

28 Quick Reference Guide Stage 2 Definition Kidney damage with GFR ml/min/1.73m 2 Frequency of monitoring Referral Minimum annual review. More frequent review if deterioration of GFR or proteinuria category A2/A3 Initial referral to SPIN or tertiary nephrology (likely to be in shared care clinic). Comments Consideration of formal GFR. Further management may then be co-ordinated by general paediatrician in district general hospital with review by SPIN or tertiary nephrologist as required. Anaemia Investigate for iron deficiency. Unlikely to be related to CKD Hypertension Acidosis Calcium, phosphate and vitamin D Proteinuria Diet and lifestyle Conventional cardiovascular risk factors Target blood pressure 50 th -75 th centile for age, gender and height. Treat with ACE inhibitor unless renal artery disease or other contra-indication. Treat metabolic acidosis with sodium bicarbonate If abnormal calcium or phosphate investigate for vitamin D deficiency Persistent proteinuria on early morning sample is risk of disease progression. If ACR persistently > 70 mg/mmol or hypertensive and ACR > 30mg/mmol then treat with ACE inhibitor unless contraindicated. Low salt diet and healthy lifestyle. Moderate phosphate restriction. Consideration of smoking, weight and co-morbidities e.g. hyperlipidaemia, diabetes Discuss with SPIN doctors or tertiary nephrologist and / or arrange review in local sharedcare clinic. If not consistent with underlying renal disorder then consider other diagnosis. Unlikely to be related to CKD Discuss with SPIN doctors or tertiary nephrologist and / or arrange review in outreach clinic. Dietetic input. Growth Transition Expectation is of normal growth. Nephrology care likely to be via GP Routine growth monitoring at clinic reviews. May need referral to adult specialists for other follow-up. Andrew Lunn 28 January 2017

29 Quick Reference Guide Stage 3 Definition GFR ml/min/1.73m 2 Frequency of monitoring Referral Anaemia Hypertension Acidosis Calcium, phosphate and vitamin D Proteinuria Conventional cardiovascular risk factors Diet and lifestyle Growth Transition Minimum 6 monthly. More frequent review if deterioration of GFR. Refer to SPIN doctor or tertiary nephrologist. Investigate for iron deficiency and treat. Consider ESA if anaemic despite correcting iron deficiency. Target blood pressure 50 th -75 th centile. Treat with ACE inhibitor unless renal artery disease or other contra-indication. Consider 24hr ABPM annually Treat metabolic acidosis with sodium bicarbonate. If abnormal calcium, phosphate or PTH investigate for vitamin D deficiency. Persistent proteinuria on early morning sample is risk of disease progression. If ACR persistently > 70 mg/mmol or hypertensive and ACR > 30mg/mmol then treat with ACE inhibitor unless contraindicated. Consideration of smoking, weight and co-morbidities e.g. hyperlipidaemia, diabetes Low salt diet and healthy lifestyle. Phosphate restriction. Nutrition optimised for growth. Monitor growth at 6 monthly assessment. Nephrology care likely to be via adult nephrologists. Comments Consideration of formal GFR. Care co-ordinated in specialist centre or in local shared-care clinic. Target Hb g/l. Treatment co-ordinated by SPIN doctors or tertiary nephrologist. Additional treatment such as phosphate binders, alfacalcidol co-ordinated by SPIN doctors or tertiary nephrologist Treatment co-ordinated by SPIN doctors or tertiary nephrologist. Monitor fasting lipids and glucose annually if CKD plus additional cardiovascular risk factor e.g. hypertension, obesity Specialist dietetic input at least annually. Consideration of growth hormone (GH) if poor growth despite optimal nutrition. GH coordinated with tertiary nephrologist and local endocrinologist. May need referral to adult specialists for other follow-up e.g. urology. Andrew Lunn 29 January 2017

30 Quick Reference Guide Stage 4 Definition GFR ml/min/1.73m 2 Frequency of monitoring 1-2 monthly. Comments Referral Refer to tertiary nephrologist. Care may still be delivered in local shared-care clinic but will need some visits to Nottingham to begin preparation for dialysis and transplantation. Anaemia Hypertension Acidosis Calcium, phosphate and vitamin D Proteinuria Conventional cardiovascular risk factors Diet and lifestyle Investigate for iron deficiency and treat. Consider ESA if anaemic despite correcting iron deficiency. Target blood pressure 50 th -75 th centile for age, gender and height. 24 hr ABPM annually Treat metabolic acidosis with sodium bicarbonate. If abnormal calcium, phosphate or PTH investigate for vitamin D deficiency. Persistent proteinuria on early morning sample is risk of disease progression. Consideration of smoking, weight and co-morbidities e.g. hyperlipidaemia, diabetes Low salt diet and healthy lifestyle. Phosphate restriction. Nutritional intake modified to optimise growth. Target Hb g/l. Treatment co-ordinated by tertiary nephrologist. ACE inhibitors should be considered but may not be tolerated (e.g. hyperkalaemia) and studies in adults are looking at the advantages / disadvantages of stopping ACE inhibitors in patients with CKD 4. Additional treatment such as phosphate binders, alfacalcidol coordinated by tertiary nephrologist Treatment if required co-ordinated by tertiary nephrologist. ACE inhibitors should be considered but may not be tolerated (see above). Monitor fasting lipids and glucose annually if CKD plus additional cardiovascular risk factor e.g. hypertension, obesity Specialist dietetic input at least 6- monthly. Consider gastrostomy feeding if not meeting nutritional requirements Growth Monitor growth at assessment. Consideration of growth hormone (GH) if poor growth despite optimal nutrition. GH co-ordinated with tertiary nephrologist and local endocrinologist. Transition Ongoing nephrology care with adult nephrologists. May need referral to adult specialists for other follow-up e.g. urology. Andrew Lunn 30 January 2017

31 Quick Reference Guide Stage 5 Definition Kidney damage with GFR <15 ml/min/1.73m 2 or on dialysis. Minimum monthly. Frequency of monitoring Referral Comments Refer to tertiary nephrologist. Care co-ordinated in specialist centre. Preparation for dialysis (if not yet instigated) and transplant to continue. Anaemia Investigate for iron deficiency Target Hb g/l. and treat. Consider ESA if anaemic despite correcting iron deficiency. Hypertension Target blood pressure 50 th -75 th centile. Treatment co-ordinated by tertiary nephrologist. Acidosis Treat metabolic acidosis. Often controlled with dialysis. Calcium, Phosphate and Vitamin D Proteinuria Conventional cardiovascular risk factors Diet and lifestyle If abnormal calcium, phosphate or PTH investigate for Vitamin D deficiency. Ongoing nephrotic syndrome is pro-thrombotic risk. Consideration of smoking, weight and co-morbidities e.g. hyperlipidaemia, diabetes Low salt diet and healthy lifestyle. Phosphate restriction. Nutritional intake modified to optimise growth. Additional treatment such as phosphate binders, alfacalcidol co-ordinated by tertiary nephrologist Treatment if required coordinated by tertiary nephrologist. Monitor fasting lipids and glucose annually. Specialist dietetic input 1-2 monthly. Consider gastrostomy feeding if not meeting nutritional requirements Growth Monitor growth at assessment. Consideration of growth hormone (GH) if poor growth despite optimal nutrition. GH coordinated with tertiary nephrologist and local endocrinologist. Transition Ongoing nephrology care with adult nephrologists. May need referral to adult specialists for other follow-up e.g. urology. Andrew Lunn 31 January 2017