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.
Based on this definition, clinical practice guidelines from Kidney Disease: Improving Global Outcomes (KDIGO) in 2012 published criteria for diagnosis and staging of pediatric CKD [1]. The KDIGO diagnosis criteria and staging classification are the standard used in clinical practice, research, and public health in the care of children with CKD and will be used throughout this topic.
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.
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 of <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 upon 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. (See "Chronic kidney disease in children: Definition, epidemiology, etiology, and course", section on 'Estimated glomerular filtration rate'.)
MANAGEMENT APPROACH — 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 when approaching kidney failure (stage G5, previously referred to as end-stage kidney disease)
Our practice is consistent with recommendations in the Kidney Disease: Improving Global Outcome (KDIGO) Clinical Practice Guideline and the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative (KDOQI) guidelines [1,3]. 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 chronic kidney disease. Educational outreach is initiated so 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, and smoking in adolescents) and incorporate measures (eg, strict blood pressure 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 (MBD), 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 end-stage kidney failure: Stages G4 and G5 – Patients who continue to have progressive disease need to be identified well in advance of the time that kidney replacement therapy is required so that adequate preparation and education can be provided to both the patient and family. The preparation for kidney replacement therapy generally starts with stage G4 CKD when GFR falls below 30 mL/min per 1.73 m2. (See 'Preparation for end-stage kidney disease' below.)
ROUTINE HEALTH MAINTENANCE
Components — 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. The following parameters are closely monitored [3-12]:
●Growth, including measurements of weight and height. For patients who are three years of age and younger, head circumference is also monitored.
●Nutritional status.
●BP.
●Neurodevelopment assessment.
●Laboratory tests to monitor kidney function and detect associated complications, including serum creatinine, electrolytes, calcium and phosphorus, alkaline phosphatase, 25-hydroxyvitamin D, parathyroid hormone level, hemoglobin, iron, total iron binding capacity, ferritin, lipid profile, urinalysis, and urinary protein-to-creatinine ratio. The frequency of assessment is based on the severity of kidney dysfunction.
●Immunizations.
Nutrition — Appetite and nutritional intake decrease with progression of CKD in children [13,14]. 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) [15]. Weight loss primarily occurs when the GFR decreases to <35 mL/min per 1.73 m2, and this weight loss is associated with a higher risk for renal failure (previously referred to as end-stage kidney disease) [16]. In our practice, ongoing assessment and follow-up for growth and nutrition is based on the 2008 Kidney Disease Outcomes Quality Initiative (KDOQI) Clinical Practice Guideline (CPG) for Nutrition in Children with Chronic Kidney Disease: Update 2008 (table 3) and the recommendations from the Pediatric Renal Nutrition Taskforce [17-21]. These guidelines provide a schedule for health and nutritional surveillance in children with CKD based on severity of kidney function and age. More frequent assessments are recommended in younger children and in children with severe impairment of kidney function regardless of age (ie, stages G4 and G5). At each of the scheduled visits, in addition to the growth measurements, the dietary intake is assessed using either a three-day diet record or three 24-hour dietary recall in children who are at increased risk.
Nutritional therapy based upon 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 based upon the estimated energy requirement (EER) for chronological age (table 4) [18,22]. Further supplementation should be considered when the initial intake fails to meet the child's EER 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 [19] (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 – Protein intake should be between 100 and 140 percent of the Dietary Reference Intake (DRI) based upon age and gender for children with CKD stage G3 and between 100 and 120 percent for those with CKD stages G4 and G5 (table 5) [18,22]. For 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. Protein supplementation should be considered if the oral and/or enteral protein intake is inadequate [22-24].
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 [23,25,26].
●Vitamins and minerals – Children with CKD should receive 100 percent of the DRIs for the following vitamins: thiamine (B1); riboflavin (B2); pyridoxine (B6); vitamins B12, A, C, E, K; and folic acid (table 6 and table 7) and the following minerals: copper and zinc [22]. Vitamin D deficiency is common in children with CKD and the formulation of vitamin D supplementation is dependent 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'.)
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 with the addition of vitamin A only if they have very low dietary intake of vitamin A.
Blood pressure and targeted goals — 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 BP control' below.)
It remains unclear what optimal target BP goals should be for children with CKD. Guidelines from several professional societies have been published using both office BP measurement and ambulatory blood pressure monitoring (ABPM) [27-29]. We use the following target BP goals for office measurements (table 8). These targets represent the thresholds for normal BP for pediatric patients with CKD, as defined by the 2017 American Academy of Pediatrics and American Heart Association (AAP/AHA). The AAP/AHA BP guidelines are based on normal-weight children and exclude overweight/obese children in their normative data. guidelines [27]:
●In children with CKD (<13 years of age), we use target systolic and diastolic BPs of less than the 90th percentile of normative data for age, gender, and height (table 9 and table 10).
●In adolescents (≥13 years of age), we use a target BP of ≤120/80 mmHg.
If ABPM is performed, we use a target goal of a 24-hour mean arterial BP (MAP) below the 50th percentile based on pediatric ABPM normative data for gender and height as recommended by the 2017 AAP guidelines (table 11 and table 12 and table 13 and table 14) [27].
We recommend strict BP control in all children with CKD, as aggressive BP control slows the progression of CKD. 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'.)
Additional studies include the following:
●Ambulatory blood pressure monitoring (ABPM) – In accordance with the AAP/AHA guidelines, we perform ABPM in children with CKD periodically in the following settings:
•To evaluate for the presence of masked hypertension (defined as normal office BP but elevated BP on ABPM)
•When consistent blood pressure data are difficult to obtain with office measurements
•To evaluate the efficacy of antihypertensive therapy, especially when hypertension is difficult to control
•If the patient is symptomatic with complaints that could be attributed to adverse effects from antihypertensive therapy
•To detect white coat hypertension (defined as elevated office BP but normal BP on ABPM)
The AAP/AHA guidelines recommend that ABPM should be used to identify any child suspected to have white coat hypertension, and children with CKD should be periodically evaluated with ABPM to evaluate for the presence of masked hypertension [30-32]. The AAP/AHA recommendations do not change for a child with CKD if overweight/obese. (See "Ambulatory blood pressure monitoring in children".)
●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 [33]. We suggest echocardiogram be performed annually especially in children with CKD, especially for those with uncontrolled hypertension and/or documented end-organ damage. ECG is not recommended for evaluation of LVH in children as it is not a sensitive tool [27]. Detection of LVH is an indication that 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 impairment in neurodevelopment. 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, and uremia), which can negatively impact on neurodevelopmental performance. For patients with impaired neurodevelopmental function, referral to early intervention programs or special educational services may be beneficial. (See "Chronic kidney disease in children: Complications", section on 'Neurodevelopmental impairment' and "Developmental-behavioral surveillance and screening in primary care" and "Specific learning disabilities in children: Educational management".)
Laboratory testing — Laboratory testing is used to monitor kidney function and detect associated-CKD complications. Tests include serum creatinine, urea, electrolytes, bicarbonate, calcium, phosphorus and alkaline phosphatase, hemoglobin, indices of iron status, fasting lipid profile, 25-hydroxyvitamin D, parathyroid hormone level, urinalysis, and urinary protein-to-creatinine ratio. The frequency of assessment is based on the severity of kidney dysfunction (table 15).
Immunization — All childhood vaccinations should be administered in children with CKD 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.
●Hepatitis B vaccination should be provided to all children with CKD or undergoing dialysis.
●Human papillomavirus (HPV) vaccine should be given to individuals based on the normal schedule. Limited data have shown a robust and sustained response to human papillomavirus (HPV) vaccination in children with predialysis CKD and children on dialysis, but a less robust response was observed in kidney transplant recipients [34,35].
●Varicella vaccine should be given to all children with CKD [36,37]. 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 (BCG) vaccine at birth. In some countries with a low incidence of tuberculosis, BCG is administered to children with CKD prior to kidney transplantation [38].
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 [39-41]. (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 SSNS' 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 [42]. 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 anti-inflammatory 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 anti-inflammatory agents, 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 (BUN), as they interfere with either the tubular secretion of creatinine or the laboratory assay for creatinine [43]. As a result, they do not directly impact on kidney function.
Slow progression of CKD — Reported interventions to slow CKD progression include BP control, reducing protein excretion, correcting anemia and maintaining normal 25-hydroxyvitamin D levels [44-46]. However, BP control has been the only intervention that has been shown to have a beneficial effect on slowing CKD progression in children. (See "Chronic kidney disease in children: Definition, epidemiology, etiology, and course", section on 'Interventions'.)
Strict BP control — We suggest that blood pressure (BP) be strictly controlled to reach targeted BP goals (table 8), including the use of antihypertensive therapy in children with CKD when necessary, based on evidence that strict BP control reduced the rate of progression of CKD in children [47-49].
For children who require antihypertensive therapy, our preferred agents are an ACE inhibitor or ARB consistent with 2017 AAP Clinical Practice Guideline for screening and management of high BP in children and adolescents [27]. 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 [27,47,48,50-53]. The management of hypertension in children with CKD is discussed in more detail below. (See "Chronic kidney disease in children: Definition, epidemiology, etiology, and course", section on 'Blood pressure control' and "Chronic kidney disease in children: Complications", section on 'Hypertension' and "Overview of hypertension in acute and chronic kidney disease".)
Reduction of proteinuria — Data in both patients and animal models provide evidence that angiotensin blockade using either an ACE inhibitor or ARB may also slow CKD progression in patients with hereditary nephritis without elevated BP. (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, acidosis, and correction of anemia. However, results are inconclusive with respect to the impact of these interventions upon delaying the progression of pediatric CKD.
In particular, data have not shown a benefit of a low-protein diet upon the progression of kidney disease in children [23]. The current consensus in pediatric nephrology is to provide children with CKD the age-appropriate recommended daily allowance for protein. (See '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, an observational study reported that a serum uric acid level greater than 7.5 mg/dL was an independent risk factor for accelerated progression of CKD in children and adolescents [54]. However, there are no recommendations for intervention or monitoring of serum uric acid in children or adults with CKD. Whereas the Kidney Disease: Improving Global Outcomes (KDIGO) 2012 Clinical Practice Guideline for Evaluation and Management of Chronic Kidney Disease acknowledges the growing body of evidence regarding the association of hyperuricemia and CKD, they also acknowledge the lack of reliable evidence to warrant intervention to lower serum uric acid in order 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'.)
COMPLICATIONS OF CKD — 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):
•Uremic pericarditis
•Uremic bleeding
These complications and their management are discussed in greater detail separately. (See "Chronic kidney disease in children: Complications".)
PREPARATION FOR END-STAGE KIDNEY DISEASE — 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 the family for KRT [3]. The family and patient should be provided information related to the timing and choice of KRT (preemptive kidney transplantation, peritoneal dialysis, and hemodialysis).
When, on a rare occasion, parents of a child with stage G5 CKD elect 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, the health care providers, 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 [55]. (See "Kidney palliative care: Principles, benefits, and core components" and "Kidney palliative care: Withdrawal of dialysis".)
The timing and choice of KRT for children are discussed in greater detail separately. (See "Overview of kidney replacement therapy (KRT) for children with chronic kidney disease".)
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 (KRT) for children with chronic kidney disease", section on 'KRT options'.)
Almost three-quarters of children diagnosed with renal failure undergo dialysis in the United States, and the mortality rate for these children is reported to be at least 30 times higher than in the general pediatric population [56]. However, data from the United States Renal Data System (USRDS) and the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) database have shown a decrease in mortality for children who received chronic dialysis [57-59]. The 2018 USRDS Annual Data Report, which includes all children who receive KRT (both kidney transplant and dialysis), found that the one-year adjusted all-cause mortality had decreased from 49 to 39 per 1000 patient years between 2006 to 2010 and 2011 to 2015 [60]. Based on the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) database, survival of infants who initiate chronic peritoneal dialysis in the first year of life has improved over time from 2000 to 2012 compared with an earlier cohort from 1992 to 1999 [61].
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 [62]. 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 (QOL) for both the child and family [13,63-66]. In particular, psychological (eg, depression) and social stresses are found in children with CKD and their families [65,66]. 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 [65]. The prospect of a lifetime with kidney replacement therapy (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 QOL surveys [67-70]. Whereas many adults with childhood renal failure are able to attain social autonomy, employment remains challenging due to an inability to work because of medical issues and a lack of understanding among teachers and employers about the medical burden of CKD/dialysis that these young adults have to deal with [70,71]. This results in loss of self-esteem, social isolation, fatigue, and low mood [70].
The negative impact of chronic disease on the emotional status of the patient's siblings is also well recognized [72]. 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: Bone problems caused by kidney disease (The Basics)" and "Patient education: Medicines for chronic kidney disease (The Basics)")
SUMMARY AND RECOMMENDATIONS
●The general management of children with chronic kidney disease (CKD) 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' above.)
●In addition to care measures provided to all children, routine health maintenance for children with CKD requires more intense monitoring of kidney function and potential complications (table 15), growth, nutrition (table 3) and diet, neurodevelopment, blood pressure (BP) control and immunization. (See 'Routine health maintenance' above.)
●Prevention and slowing progression of CKD includes:
•Treatment of the underlying primary kidney disease if possible. (See 'Treat underlying cause of kidney disease' above.)
•Prevention of subsequent kidney injury by avoiding episodes of decreased kidney perfusion or the administration of nephrotoxic agents. (See 'Avoid subsequent kidney injury' above.)
•Strict BP control 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 children with CKD who require antihypertensive therapy, we suggest an agent that blocks the renin-angiotensin system (angiotensin-converting enzyme [ACE] inhibitors, and angiotensin II receptor blockers [ARB]) versus other classes of antihypertensive drugs (Grade 2B). (See 'Strict BP control' above and 'Blood pressure and targeted goals' above.)
•In children with CKD, there is no evidence that a low-protein diet slows the progression of kidney disease. There is also concern that such a diet may impair growth. We suggest that children with CKD be given a daily diet containing at least 100 percent of the dietary reference intake for protein based upon age and gender (table 5) (Grade 2C). (See 'Other interventions with insufficient evidence' above and 'Nutrition' above.)
●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
●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 end-stage kidney disease' above.)
●For children with renal failure (stage G5, previously referred to as end-stage kidney disease), there is a significant increased risk for morbidity and mortality that persists through adulthood. (See 'Long-term outcome' above.)
●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.)
