INTRODUCTION — Chronic kidney disease (CKD) refers to a state of irreversible kidney damage and/or reduction of kidney function that is associated with progressive loss of kidney function over time.
An overview of the management of CKD in children will be reviewed here. The etiology, epidemiology, natural course, presentation, and evaluation of CKD in children are discussed separately. (See "Chronic kidney disease in children: Definition, epidemiology, etiology, and course" and "Chronic kidney disease in children: Clinical manifestations and evaluation".)
DEFINITIONS AND DIAGNOSIS — CKD is defined as the presence of structural or functional kidney damage that persists over a minimum period of three months. Functional damage is characterized by sustained reduction of estimated glomerular filtration rate (GFR), persistent elevation of urinary protein excretion, or both.
Clinical practice guidelines from Kidney Disease: Improving Global Outcomes (KDIGO) include the following criteria for diagnosis and staging of pediatric CKD [1]. The are the standard criteria used in clinical practice, research, and public health in the care of children with CKD.
●Diagnosis – The KDIGO diagnosis of pediatric CKD is based on fulfilling one of the following clinical criteria [1]:
•GFR of less than 60 mL/min per 1.73 m2 for greater than three months with implications for health regardless of whether other CKD markers are present.
•GFR greater than 60 mL/min per 1.73 m2 that is accompanied by evidence of structural damage or other markers of functional kidney abnormalities including proteinuria, albuminuria, renal tubular disorders, or pathologic abnormalities detected by histology or inferred by imaging. This category also includes patients with functioning kidney transplants.
●Staging – The KDIGO CKD staging for children older than two years of age stratifies the risk for progression of CKD and its complications based on GFR and is used to guide management (table 1):
•G1 – Normal GFR (≥90 mL/min per 1.73 m2)
•G2 – GFR between 60 and 89 mL/min per 1.73 m2
•G3a – GFR between 45 and 59 mL/min per 1.73 m2
•G3b – GFR between 30 and 44 mL/min per 1.73m2
•G4 – GFR between 15 and 29 mL/min per 1.73 m2
•G5 – GFR <15 mL/min per 1.73 m2, also referred to as kidney failure [2]
Children under two years of age do not fit within the above classification system, because they normally have a low GFR even when corrected for body surface area. In these patients, calculated GFR based on serum creatinine can be compared with normative age-appropriate values to detect kidney impairment (table 2). The KDIGO guideline suggests that a GFR value more than one standard deviation below the mean should raise concern and prompt more intensive monitoring [1].
Equations that can be used to estimate GFR in children and young adults (1 to 25 years) are based on serum creatinine and cystatin C values, respectively [3]. (See "Chronic kidney disease in children: Clinical manifestations and evaluation".)
MANAGEMENT APPROACH BY STAGE OF CHRONIC KIDNEY DISEASE — The general management of the patient with CKD includes the following components:
●Routine health maintenance
●Prevent or slow the progression of kidney disease
●Prevent or treat the complications of CKD
●Preparation for kidney replacement therapy (KRT) when approaching kidney failure (stage G5)
Our practice is consistent with recommendations in the KDIGO Clinical Practice Guideline and the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines [1,4]. Routine health care is provided to all children with CKD. The timing of implementation of the other components of care vary primarily based on the severity of CKD (see "Society guideline links: Chronic kidney disease in children"):
●Early asymptomatic CKD: Stages G1 and G2 – Children with stages G1 and G2 disease are asymptomatic and should be closely followed for deterioration of kidney function. Although not frequent, metabolic derangements may be seen in stage G1 and G2. For these children, there may be an opportunity to treat any reversible cause of kidney dysfunction and prevent or slow the progression of CKD. Educational outreach is initiated so that the child and family can understand and implement care to avoid risk factors that can accelerate the progression of CKD (eg, avoidance of nephrotoxic drugs, recurrent infections, dehydration, obesity, smoking, and use of illicit drugs in adolescents) and incorporate measures (eg, strict blood pressure [BP] control and/ or reducing proteinuria) that may slow the process. (See 'Prevent or slow progression of kidney disease' below.)
●Mild to moderate CKD: Stages G3a and G3b – Children who progress to stages G3a and G3b may begin to display CKD-associated complications. These include disorders of fluid and electrolytes, anemia, hypertension, dyslipidemia, endocrine abnormalities, growth impairment, mineral and bone disorder, and decreased clearance of substances normally excreted from the body by the kidney (uremia). In these patients, management is focused on preventing and treating these complications. In addition, avoidance of risk factors, as outlined above, should be maintained to slow CKD progression. (See "Chronic kidney disease in children: Complications".)
●Severe CKD and kidney failure: Stages G4 and G5 – Patients who continue to have progressive disease need to be identified well in advance of the time that KRT is required so that adequate preparation and education can be provided to both the patient and family. The preparation for KRT generally starts with stage G4 CKD when glomerular filtration rate (GFR) falls below 30 mL/min per 1.73 m2. (See 'Preparation for kidney failure' below.)
ROUTINE HEALTH MAINTENANCE — As CKD progresses, children are at increased risk for developing complications such as poor growth, elevated blood pressure (BP), increased cardiovascular risk (eg, dyslipidemia), anemia, vitamin D deficiency, and fluid and electrolyte abnormalities (see "Chronic kidney disease in children: Complications"). As a result, routine health maintenance, in addition to the care provided normally to all children, requires more intense monitoring of kidney function, growth, and nutritional status, with intervention as needed and screening for associated complications as CKD progresses [4-15].
Growth and nutrition — Appetite and nutritional intake decrease with progression of CKD in children [16,17]. As a result, malnutrition is common in children with CKD because of poor appetite; decreased intestinal absorption of nutrients; and metabolic acidosis affecting physical growth, neurocognitive development, and overall health status (ie, fragility) [18]. Weight loss primarily occurs when the glomerular filtration rate (GFR) decreases to <35 mL/min per 1.73 m2, and this weight loss is associated with a higher risk for kidney failure [19].
A protocol for monitoring growth and nutrition in children with CKD is outlined in the table (table 3), consistent with recommendations from the 2008 KDOQI and Pediatric Renal Nutrition Taskforce (PRNT) [20-27]. The assessments are more frequent for younger children and those with severe impairment of kidney function regardless of age (ie, stages G4 and G5).
●Growth – For all children, measure height and weight at each visit. For children <3 years, also monitor head circumference.
●Nutritional assessments – At each scheduled visit, assess dietary intake using either a three-day diet record or three 24-hour dietary recalls.
Nutritional therapy based on growth parameters is developed for each child with CKD and should ideally be coordinated by a dietician with expertise in pediatrics and renal nutrition. Nutritional management should address the energy, protein, vitamin, mineral, and electrolyte needs of the individual patient.
•Energy – The initial prescribed energy (calorie) intake for children with CKD is similar to that for healthy children of the same chronologic age, which can be calculated from prediction equations (table 4) or found in summary tables [21,28] (see "Dietary recommendations for toddlers and preschool and school-age children", section on 'Energy and macronutrient balance'). The recommendations of KDOQI are expressed in terms of the dietary reference intakes (DRIs). Recommendations from the PRNT are expressed in terms of the suggested dietary intake, which takes into consideration the range of published values. For children with suboptimal growth, the target should be adjusted toward the higher end of the suggested energy intake.
Further supplementation should be considered when the initial intake fails to meet the child's estimated energy requirement and they are not achieving expected rates of weight gain and/or growth. Although supplementation by the oral route is preferred, one may have to resort to tube feedings with a gastrostomy, transpyloric tube, or nasogastric tube to ensure adequate energy intake [22] (see "Overview of enteral nutrition in infants and children"). A trial of intradialytic parenteral nutrition may be considered in children undergoing chronic hemodialysis therapy. (See "Hemodialysis for children with chronic kidney disease", section on 'Inadequate nutrition'.)
•Protein – Targets for protein intake suggested by KDOQI are (table 5) [21,28]:
-CKD stage G3 – 100 to 140 percent of the DRI
-CKD stages G4 and G5 – 100 to 120 percent of the DRI
-Patients on peritoneal dialysis – Protein intake should be higher (additional average 0.15 to 0.3 g/kg per day) to compensate for dialytic protein loss
The PRNT recommendations are similar, except for the suggested dietary intake as the reference value for protein requirement. Protein supplementation should be considered if the oral and/or enteral protein intake is inadequate [28,29].
Protein restriction is not recommended in children, as it has not been shown to influence the decrease in kidney function in children with CKD and may impair growth [29-31]. (See 'Other interventions with insufficient evidence' below.)
•Vitamins and minerals – Children with CKD should receive 100 percent of the DRIs for the following nutrients [28]:
-Vitamins – Thiamine (B1); riboflavin (B2); pyridoxine (B6); vitamins B12, A, C, E, K; and folic acid (table 6 and table 7)
-Minerals – Copper and zinc
Special considerations are:
-Vitamin D – Vitamin D deficiency is common in children with CKD, and the formulation of vitamin D supplementation depends on the severity of kidney function, as discussed separately. (See "Pediatric chronic kidney disease-mineral and bone disorder (CKD-MBD)", section on 'Vitamin D deficiency'.)
-Vitamin A – In children with advanced CKD (ie, stage G5), the loss of kidney clearance of vitamin A metabolites places them at risk for developing hypervitaminosis A. These children should receive a water-soluble vitamin supplement that includes vitamin A only if they have very low dietary intake of vitamin A.
-Vitamins E and K – There are no formal recommendations regarding supplementation of vitamin E and K in children and adults with CKD or those undergoing dialysis, due to the lack of data. However, if a child with CKD has an underlying condition that increases their risk for deficiency, supplementation should be considered after confirming low serum levels of vitamin E or coagulopathy (elevated prothrombin time and partial thromboplastin time) secondary to vitamin K deficiency.
Blood pressure and targeted goals
●Office BP – Office BP measurement should be performed at each supervised health visit. As noted below, strict BP control has shown a beneficial effect on slowing CKD progression in children. (See 'Strict blood pressure control' below.)
We use the following target BP goals for office measurements for children with CKD (table 8), which are the upper thresholds for normal BP for pediatric patients, as defined by the 2017 American Academy of Pediatrics and American Heart Association [32]:
•Children <13 years of age – Systolic and diastolic BPs <90th percentile of normative data for age, sex, and height (table 9 and table 10)
•Adolescents ≥13 years of age – BP ≤120/80 mmHg
We recommend strict BP control in all children with CKD because aggressive BP control slows the progression of CKD [33]. Treatment of hypertension (defined as BP greater than the BP targeted goal on three separate occasions) should be initiated using nonpharmacologic therapy and, if needed, antihypertensive therapy. (See "Chronic kidney disease in children: Complications", section on 'Hypertension'.)
●Ambulatory BP monitoring (ABPM) – In accordance with the American Academy of Pediatrics/American Heart Association guidelines, children with CKD should be evaluated periodically with ABPM to evaluate for the presence of masked hypertension (normal clinic BPs but abnormal ABPM) [34,35]. ABPM is also valuable for optimizing antihypertensive therapy to slow disease progression and reverse organ damage and for diagnosing white coat hypertension (elevated clinic BP but normal ABPM). (See "Ambulatory blood pressure monitoring in children".)
If ABPM is performed, we use a target goal of a 24-hour mean arterial BP below the 50th percentile for pediatric ABPM normative data based on age (table 11A-B) or height (table 12A-B), as recommended by the KDIGO guideline for management of BP in CKD [35].
●Echocardiogram – We obtain echocardiograms in patients with CKD and elevated BP as these children are at risk for left ventricular hypertrophy (LVH), which is associated with adverse cardiovascular disease [36]. The echocardiogram should be repeated annually in children with CKD, especially for those with uncontrolled hypertension and/or documented end-organ damage. Adiposity in children with CKD is an additional independent risk factor for LVH, especially in girls [37]. Electrocardiography is not recommended for evaluation of LVH in children, as it is not a sensitive tool [32]. The diagnosis of LVH in children is outlined separately. (See "Evaluation of hypertension in children and adolescents", section on 'Echocardiography'.)
If LVH is detected, efforts should be made to improve BP control. (See "Nonemergent treatment of hypertension in children and adolescents", section on 'Cardiovascular disease'.)
Neurodevelopment assessment — CKD is associated with impaired neurodevelopment [38]. Commonly reported neurocognitive difficulties include deficits in attention regulation and executive function [39]. As a result, routine health maintenance includes early developmental surveillance and screening to identify children with or at risk for neurodevelopmental delay and formal assessment for older children, especially if they have poor school performance. Assessment should include identifying modifiable risk factors (eg, anemia, poor nutrition, hypertension, and uremia) that can negatively impact neurodevelopmental performance. For patients with impaired neurodevelopmental function, referral to early intervention programs or special educational services may be beneficial [38]. (See "Chronic kidney disease in children: Complications", section on 'Neurodevelopmental impairment' and "Developmental-behavioral surveillance and screening in primary care" and "Specific learning disorders in children: Educational management".)
Laboratory testing — Laboratory testing is used to monitor kidney function and detect associated CKD complications. The frequency of assessment is based on the severity of kidney dysfunction (table 13). Tests include:
●Serum creatinine, urea, electrolytes, bicarbonate
●Calcium, phosphorus, alkaline phosphatase
●Albumin
●Hemoglobin
●Indices of iron status (ferritin, iron, total iron-binding capacity)
●Fasting lipid profile
●25-hydroxyvitamin D
●Parathyroid hormone
●Urinalysis and urinary protein-to-creatinine ratio
Immunization — Children with CKD should receive all childhood vaccinations, with the caveat that live-attenuated vaccines should not be administered to children who are immunosuppressed. As an example, live-attenuated influenza vaccine should not be given to children with nephrotic syndrome or those receiving immunosuppressive therapy post-kidney transplantation [1]. After receiving a live-attenuated vaccine, a mandatory minimum waiting period of four weeks is necessary prior to using immunosuppression for kidney transplantation.
Management for specific vaccinations include:
●Pneumococcal vaccine should be given to all children with nephrotic syndrome and those with CKD, using protocols for children at high risk of invasive pneumococcal disease. This is discussed separately. (See "Pneumococcal vaccination in children", section on 'Immunization of high-risk children and adolescents'.)
●Hepatitis B vaccination should be provided to all children with CKD or undergoing dialysis.
●Human papillomavirus vaccine should be given to individuals based on the normal schedule. Limited data have shown a robust and sustained response to human papillomavirus vaccination in children with predialysis CKD and those on dialysis; a less robust response was observed in kidney transplant recipients [40,41].
●Varicella vaccine should be given to all children with CKD [42,43]. However, since it is a live attenuated vaccine, it is contraindicated in patients with severe immunocompromise, including children receiving high doses of corticosteroids. It is ideally administered as a two-dose regimen when the child is on low-dose regimen of corticosteroids (eg, less than 2 mg/kg of body weight on alternate days) or off of corticosteroid therapy. (See "Vaccination for the prevention of chickenpox (primary varicella infection)", section on 'Immunocompromised hosts' and "Symptomatic management of nephrotic syndrome in children", section on 'Varicella'.)
●Tuberculosis in endemic countries is prevented by the universal administration of the Bacillus Calmette-Guérin vaccine at birth. In some countries with a low incidence of tuberculosis, the vaccine is administered to children with CKD prior to kidney transplantation [44].
PREVENT OR SLOW PROGRESSION OF KIDNEY DISEASE
Treat underlying cause of kidney disease — In some cases, identifying and treating the underlying primary kidney disease can prevent or slow the progression of disease. Examples include:
●Correction of obstructive uropathy or high-grade vesicoureteral reflux [45-47]. (See "Management of vesicoureteral reflux", section on 'Grades III to V'.)
●Immunosuppressive therapy for primary nephrotic syndrome and primary and secondary glomerulonephritis (eg, lupus nephritis). (See "Treatment of idiopathic nephrotic syndrome in children", section on 'Long-term outcome of steroid-sensitive nephrotic syndrome' and "C3 glomerulopathies: Dense deposit disease and C3 glomerulonephritis" and "Lupus nephritis: Initial and subsequent therapy for focal or diffuse lupus nephritis" and "C3 glomerulopathies: Dense deposit disease and C3 glomerulonephritis", section on 'Prognosis'.)
Avoid subsequent kidney injury — Episodes of acute kidney injury can result in a faster decline in kidney function in children with CKD [48]. Decreased kidney perfusion or the administration of nephrotoxic agents are two common conditions that may result in further kidney injury in children with CKD.
●Avoid acute episodes of kidney hypoperfusion – A subset of children with CKD have impaired tubular sodium reabsorption (salt losers) and urinary concentrating ability, which increases their risk for hypovolemia and hypoperfusion with minor illnesses. At-risk patients should be identified at the onset of an intercurrent illness associated with hypovolemia or hypotension so that fluid repletion can be provided prior to a significant decrease in kidney blood flow.
Kidney hypoperfusion is caused by hypotension (eg, septic shock); administration of drugs that lower the kidney perfusion (such as nonsteroidal antiinflammatory drugs, angiotensin-converting enzyme [ACE] inhibitors, and angiotensin II receptor blockers [ARBs]); and volume depletion from vomiting, diarrhea, diuretic use, burns, major surgeries (eg, cardiac surgery performed on cardiopulmonary bypass, orthopedic spine surgeries), and/or bleeding. (See "Etiology and diagnosis of prerenal disease and acute tubular necrosis in acute kidney injury in adults", section on 'Causes of prerenal disease' and "Acute kidney injury in children: Clinical features, etiology, evaluation, and diagnosis".)
●Avoid nephrotoxic drugs – Common nephrotoxic drugs include nonsteroidal antiinflammatory drugs, diagnostic agents (eg, radiographic contrast materials), aminoglycosides, amphotericin B, cyclosporine, and tacrolimus. The administration of such drugs should be avoided or used with caution in patients with underlying CKD, with the assistance of therapeutic drug level monitoring. When drugs that have a narrow therapeutic index are used and precision in dosing is critical (eg, cisplatinum in bone marrow transplant induction protocol) based on kidney function, measurement of glomerular filtration rate (GFR) should be made using iohexol or one of the radioisotopes (51Cr-EDTA, 125Iothalamate, or 99Tc-DTPA). In this situation, drug dosing ideally should not be based on an estimated GFR using a regression equation. (See "Manifestations of and risk factors for aminoglycoside nephrotoxicity" and "NSAIDs: Acute kidney injury" and "Contrast-associated and contrast-induced acute kidney injury: Clinical features, diagnosis, and management" and "Assessment of kidney function".)
Certain drugs, such as cimetidine, trimethoprim, ciprofloxacin, and flucytosine, lead to an elevation in serum creatinine but not blood urea nitrogen, because they interfere with either the tubular secretion of creatinine or the laboratory assay for creatinine [49]. As a result, they do not directly affect kidney function.
Slow progression of chronic kidney disease — Reported interventions to slow CKD progression include blood pressure (BP) control, reducing protein excretion, correcting anemia, and maintaining normal 25-hydroxyvitamin D levels [50-52]. However, BP control has been the only intervention that has been shown to have a beneficial effect on slowing CKD progression in children.
Strict blood pressure control — BP should be strictly controlled to reach targeted BP goals (table 8), including the use of antihypertensive therapy in children with CKD when necessary. Strict BP control reduces the rate of progression of CKD in children [53-55].
For children who require antihypertensive therapy, our preferred agents are an ACE inhibitor or ARB, consistent with 2017 American Academy of Pediatrics Clinical Practice Guideline for screening and management of high BP in children and adolescents [32]. There is good evidence that these agents are more protective than other antihypertensive drugs in slowing the progression of CKD as they control BP and also decrease proteinuria, even in children with advanced CKD [32,53,54,56-59].
The management of hypertension in children with CKD is discussed in more detail separately. (See "Chronic kidney disease in children: Complications", section on 'Hypertension'.)
Reduction of proteinuria — For patients with proteinuria, we also treat with an ACE inhibitor or ARB even if BP is not elevated, because data in patients and animal models suggest that this antiproteinuric treatment may slow CKD progression. (See "Clinical manifestations, diagnosis, and treatment of Alport syndrome (hereditary nephritis)", section on 'Renin-angiotensin blockade'.)
Other interventions with insufficient evidence — Additional interventions that have been studied in adults with CKD include dietary protein restriction, lipid-lowering therapy, and correction of acidosis and anemia. However, results are inconclusive with respect to the impact of these interventions on delaying the progression of pediatric CKD.
In particular, data have not shown a benefit of a low-protein diet on the progression of kidney disease in children [29]. The consensus in pediatric nephrology is to provide children with CKD the age-appropriate recommended daily allowance for protein. (See 'Growth and nutrition' above.)
Hyperuricemia, which is due to decreases in urinary excretion, has been proposed to contribute to CKD progression, in part by decreasing kidney perfusion via stimulation of afferent arteriolar vascular smooth muscle cell proliferation. In addition to adult data that suggest an association between hyperuricemia and progressive CKD, a report from the Chronic Kidney Disease in Children (CKiD) study found that a serum uric acid level >7.5 mg/dL was an independent risk factor for accelerated progression of CKD in children and adolescents [60]. This was followed by longitudinal data from CKiD that provided further evidence of the relationship between elevated uric acid and risk for CKD progression [61]. However, there are no recommendations for intervention or monitoring of serum uric acid in children or adults with CKD. The 2012 KDIGO Guideline acknowledges the growing body of evidence regarding the association of hyperuricemia and CKD but concludes that there is insufficient evidence to warrant intervention to lower serum uric acid to slow the rate of GFR decline among both adult and pediatric patients with CKD [1]. (See "Secondary factors and progression of chronic kidney disease", section on 'Hyperuricemia'.)
Anemia is often linked with progression of CKD. However, its ability to predict the development of kidney failure is not well established. While anemia was found to be a predictor of disease progression in the nonglomerular group of the CKiD study, its impact was not observed in other studies [50,62]. However, it is crucial to manage and correct anemia because it can significantly impact growth, quality of life, and executive functioning of children with CKD [63-65].
While dyslipidemia is a prevalent issue in CKD patients, the impact of lipid-lowering medications on slowing the progression of CKD remains uncertain [66,67].
Acidosis has also been identified as a risk factor for progression of CKD [62,68]. Observational cohort studies have suggested that correcting acidosis could potentially reduce the risk of CKD progression [68]. However, to validate these findings, a formal prospective long-term therapy study is required. Acidosis has also been associated with adverse effects on muscle strength, specifically grip strength, as well as lower height Z-scores in children with CKD, highlighting the importance of addressing and managing acidosis [69,70].
COMPLICATIONS OF CHRONIC KIDNEY DISEASE — Complications due to kidney impairment become more prevalent with decreasing glomerular filtration rate (GFR) in children as CKD advances from stages G3 to G5 (table 1). They include:
●Fluid and electrolytes abnormalities
●Mineral and bone disorder (see "Pediatric chronic kidney disease-mineral and bone disorder (CKD-MBD)")
●Anemia
●Hypertension
●Dyslipidemia
●Endocrine abnormalities
●Growth impairment
●Uremia (decreased clearance of substances excreted by the kidneys), potentially causing uremic bleeding or uremic pericarditis
These complications and their management are discussed in greater detail separately. (See "Chronic kidney disease in children: Complications".)
PREPARATION FOR KIDNEY FAILURE — In children with CKD, kidney replacement therapy (KRT) will generally be needed when the glomerular filtration rate (GFR) falls below 15 mL/min per 1.73 m² (stage G5 CKD, kidney failure) and, in some circumstances, prior to that. Thus, once the estimated GFR declines to <30 mL/min per 1.73 m² (stage G4), it is time to start preparing the child and family for KRT [4]. They should be provided information related to the timing and choice of KRT (preemptive kidney transplantation, peritoneal dialysis, and hemodialysis). The timing and choice of KRT for children are discussed in greater detail separately. (See "Overview of kidney replacement therapy for children with chronic kidney disease".)
Rarely, parents or guardians of a child with stage G5 CKD choose conservative management and death over a lifetime of dialysis and transplantation for their child. This should be considered a choice that may, on occasion, be medically, ethically, and legally acceptable. A host of factors need to be considered by the family, clinicians, and, often, the institution's ethics committee. When a decision to forego KRT is deemed acceptable, the family should be supported emotionally and provided with whatever care is necessary to maintain the child in a pain-free state [71]. (See "Kidney palliative care: Principles, benefits, and core components" and "Kidney palliative care: Withdrawal of dialysis".)
LONG-TERM OUTCOME
Kidney failure
Pediatric mortality — Children with kidney failure (previously referred to as end-stage kidney disease) have a shortened life expectancy compared with children without CKD. Kidney transplantation remains the treatment of choice to maximize survival, growth, and development. (See "Overview of kidney replacement therapy for children with chronic kidney disease", section on 'Preemptive transplantation as preferred kidney replacement therapy modality'.)
Almost three-quarters of children diagnosed with kidney failure in the United States undergo dialysis, and the mortality rate for these children is reported to be at least 30 times higher than in the general pediatric population [72]. However, mortality rates for children receiving chronic dialysis have decreased over time, as shown by data from the United States Renal Data System (USRDS) and the North American Pediatric Renal Trials and Collaborative Studies database [73-75]. The USRDS Annual Data Report, which includes all children who receive kidney replacement therapy (KRT; both kidney transplant and dialysis), found that the one-year adjusted all-cause mortality decreased from 49 per 1000 patient years in 2006-2010 to 39 per 1000 patient years in 2011-2015 [76]. Additional improvement was noted in the 2022 USRDS annual report, especially for the youngest age group (<1 year) [77]. Similarly, survival of infants who initiate peritoneal dialysis during the first year of life has improved substantially since the turn of the century [78,79].
The leading causes of death are cardiovascular disease and infection. The authors speculate that the reduction in mortality is probably due to improved predialysis care, advances in dialysis technology, and increased clinical experience in caring for these patients.
Adulthood outcome — Patients placed on KRT before 15 years of age have a greater mortality and morbidity risk than age-matched controls. This was illustrated in a long-term Dutch follow-up study (median time 25.5 years) that observed 30 times greater mortality risk for adults who began pediatric KRT (median time of KRT 25.5 years) than age-matched peers [80]. These individuals were more likely to have motor disabilities, skin cancer, and severe fatigue when they were >40 years old.
QUALITY OF LIFE — CKD, as is true for any chronic condition, impacts the quality of life for both the child and family [16,81-84]. In particular, psychological (eg, depression) and social stresses are found in children with CKD and their families [83,84]. The normal progression of the child to independence is impeded, and concerns about body composition and image are greatly magnified in children whose growth and pubertal development are delayed or altered, especially those who undergo chronic dialysis [83]. The prospect of a lifetime with kidney replacement therapy (KRT; dialysis and/or transplant) and the potential for catastrophic complications and/or death makes it difficult to achieve normal childhood and adolescent developmental goals.
This difficulty continues into adulthood. Compared with age-matched population normative data, patients who had renal failure were more likely to be unemployed and to have lower income and scores on quality-of-life surveys [85-88]. Whereas many adults with childhood kidney failure are able to attain social autonomy, employment remains challenging due to an inability to work because of medical issues and because teachers and employers often fail to recognize and make accommodations for the medical burden of CKD/dialysis [88,89]. This results in loss of self-esteem, social isolation, fatigue, and low mood [88]. These issues were emphasized in an assessment of patient-reported outcomes in pediatric CKD patients and their parents [90].
The negative impact of chronic disease on the emotional status of the patient's siblings is also well recognized [91]. These siblings frequently feel "neglected" because the parents must provide substantial physical and psychological support to the sick child. Furthermore, the well child may simultaneously feel jealous of the attention provided to the sick child, as well as guilt about being well while the sibling is severely ill.
Optimal comprehensive management of these issues involves a multidisciplinary approach that proactively addresses these concerns. Key members of the team include social workers and mental health specialists.
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Chronic kidney disease in children" and "Society guideline links: Chronic kidney disease-mineral and bone disorder".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Basics topics (see "Patient education: Chronic kidney disease (The Basics)" and "Patient education: Medicines for chronic kidney disease (The Basics)" and "Patient education: Bone problems caused by kidney disease (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Overview – The general management of children with chronic kidney disease (CKD) evolves with the stage of CKD (table 1) and includes routine health maintenance, measures to prevent or slow progression of CKD, treatment of the complications associated with CKD, and identifying and preparing patients with advancing CKD and their families for kidney replacement therapy (KRT). (See 'Management approach by stage of chronic kidney disease' above.)
●Routine health maintenance – In addition to care provided to all children, routine health maintenance for children with CKD requires more intense monitoring of kidney function and potential complications (table 13), neurodevelopment, blood pressure (BP) control, and immunization. (See 'Routine health maintenance' above.)
Growth and nutrition should be monitored closely, with dietary counseling and supplements if needed (table 3). Initial targets for energy intake are similar to those for healthy children, but children with CKD and growth failure may require higher energy intakes. We suggest that children with CKD be given a daily diet containing at least 100 percent of the dietary reference intake (DRI) for protein based on age and sex (table 5) (Grade 2C). There is no evidence that a low-protein diet slows the progression of kidney disease, and such a diet might impair growth. (See 'Growth and nutrition' above.)
●Measures to slow progression of CKD:
•Treat the underlying primary kidney disease if possible. (See 'Treat underlying cause of kidney disease' above.)
•Prevent further kidney injury by avoiding episodes of decreased kidney perfusion or the administration of nephrotoxic agents. (See 'Avoid subsequent kidney injury' above.)
•Maintain strict BP control. This has been the only intervention shown to slow the progression of kidney disease in children. In children with CKD, we suggest that BP be strictly controlled to reach targeted BP goals, including through the use of antihypertensive therapy if needed (table 8) (Grade 2B). For antihypertensive therapy, the first-line approach is an agent that blocks the renin-angiotensin system (angiotensin-converting enzyme [ACE] inhibitors and angiotensin II receptor blockers [ARBs]). (See 'Blood pressure and targeted goals' above and 'Strict blood pressure control' above and "Chronic kidney disease in children: Complications", section on 'Pharmacologic therapy'.)
●Complications – Complications due to kidney impairment become more prevalent as CKD progresses (see "Chronic kidney disease in children: Complications"). They include:
•Fluid and electrolytes abnormalities
•Mineral and bone disorder (see "Pediatric chronic kidney disease-mineral and bone disorder (CKD-MBD)")
•Anemia
•Hypertension
•Dyslipidemia
•Endocrine abnormalities
•Growth impairment
•Uremia including uremic pericarditis and bleeding
●KRT – Preparations for the child and family are initiated for KRT when the estimated glomerular filtration rate (GFR) declines to less than 30 mL/min per 1.73 m2 (stage G4 CKD). The patient (if appropriate) and family should be provided with information related to timing and choice of KRT (preemptive kidney transplantation, peritoneal dialysis, and hemodialysis). (See 'Preparation for kidney failure' above.)
For children with kidney failure (stage G5), there is a significant increased risk for morbidity and mortality that persists through adulthood. (See 'Long-term outcome' above.)
●Psychosocial considerations – CKD impacts on the quality of life for both the child and family and includes psychological (eg, depression) and social stresses for both. Comprehensive management of these issues involves a multidisciplinary approach that proactively addresses these concerns. (See 'Quality of life' above.)
Do you want to add Medilib to your home screen?