- *Director, Pediatric Nephrology, Levine Children’s Hospital, Adjunct Associate Professor of Pediatrics, University of North Carolina School of Medicine, Charlotte, NC.
- †Director, Pediatric Dialysis and Transplant Programs, UNC Kidney Center, Founder and Director, The UNC Children’s Hospital TRxANSITION Program, University of North Carolina at Chapel Hill, Chapel Hill, NC.
Drs Massengill and Ferris have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.
- 1,25(OH)2 D:
- 1,25-dihydroxyvitamin D
- angiotensin-converting enzyme
- chronic kidney disease
- Chronic Kidney Disease in Children
- cardiovascular disease
- estimated glomerular filtration rate
- end-stage kidney disease
- glomerular filtration rate
- health care transition
- metabolic bone disease
Chronic kidney disease (CKD) is a devastating diagnosis with many co-morbidities, increasing the risk of mortality 30 to 150 times that of the general pediatric population. Recognition of at-risk children can lead to earlier screening and risk reduction. Primary care clinicians are often unaware of the comorbid conditions and long-term consequences of CKD, particularly with respect to cardiovascular disease, nutrition and growth, neurocognitive development, and burden of disease.
After completing this article, readers should be able to:
Be aware of the life course of CKD and its co-morbidities.
Recall the risk factors and complications of pediatric CKD.
Discuss measures to prevent or delay the progression of pediatric CKD.
Optimize the communication between the primary care clinician and nephrologist in treating children, adolescents, and young adults with CKD.
A 13-month-old toddler new to your practice presents for his 1-year health maintenance visit with poor growth and developmental delay. He is just now sitting without support and appears to have occasional leg pain. He is pale, weighs 7.9 kg, and has a normal blood pressure. The results of laboratory studies are remarkable for anemia (hemoglobin, 9 g/dL [90 g/L]), profound acidosis (carbon dioxide, 12 mEq/L [12 mmol/L]), azotemia (urea nitrogen, 117 mg/dL [41.8 mmol/L]; creatinine, 2.44 mg/dL [216 μmol/L]), and profound hypocalcemia (calcium, 5.6 mg/dL [1.40 mmol/L]), prompting further evaluation where hypocalcemia was confirmed. Urinalysis revealed a specific gravity of 1.005 and proteinuria (1+). Renal ultrasonography revealed bilateral renal hypoplasia. Renal replacement therapy was initiated with peritoneal dialysis, and the patient is on the renal transplantation waiting list.
A previously healthy, 14-year-old, African American girl presents with a 3-month history of facial and lower-extremity rash and a 4.5-kg weight loss. Her medical history is unremarkable for contributing conditions. She denies sexual activity, travel, pet ownership, or tick exposure. Her family history is positive for type 1 diabetes mellitus in a younger brother and hypothyroidism in her mother. On physical examination, she is hypertensive (blood pressure, 150/90 mm Hg), with a malar erythematous rash and palpable purpura on the lower extremities. Laboratory studies reveal the following: serum creatinine, 2.5 mg/dL (221 μmol/L); estimated glomerular filtration rate (eGFR), 34 mL/min/1.73 m2; urea nitrogen, 75 mg/dL (26.8 mmol/L); and positive antinuclear antibody and anti–double-stranded DNA results. Urinalysis reveals blood (3+), proteinuria (4+), 10 to 20 red blood cells per high-power field, and 1 red blood cell cast. Urine protein to creatinine ratio is 2.5 (reference range, <0.2). Renal biopsy reveals a crescentic diffuse proliferative glomerulonephritis (World Health Organization class IV systemic lupus erythematosus nephritis).
In 2002, the National Kidney Foundation established evidence-based clinical practice guidelines entitled the Kidney Disease Outcomes Quality Initiatives, which were designed to define chronic kidney disease (CKD) based on the presence or absence of markers of kidney damage and the level of kidney function (glomerular filtration rate [GFR]) irrespective of the type of kidney disease (kidney.org/professionals/kdoqi/guidelines_commentaries.cfm). The 2 independent criteria for CKD are as follows:
Kidney damage for 3 months or longer as defined by structural or functional abnormalities of the kidney, with or without decreased GFR, manifested by either pathologic abnormalities or markers of kidney damage, including abnormalities in the composition of the blood or urine or abnormalities in imaging studies.
GFR less than 60 mL/min/1.73 m2 for 3 months or longer, with or without kidney damage.
In addition, a common nomenclature was proposed for stages of CKD (Table 1) to improve communication between primary care clinicians and nephrologists.
The Chronic Kidney Disease in Children (CKiD) study (a longitudinal cohort study of children with mild to moderate CKD) observes the risk factors related to disease progression, neurocognition and quality of life changes, cardiovascular morbidity, and the growth failure in patients with CKD. (1) From this study, a more precise and accurate estimated glomerular filtration rate (eGFR) using serum creatinine, height, and a constant (k) (eGFR = k [height in centimeters]/serum creatinine) was developed. An updated constant (k) of 0.413 is used (eGFR = 0.413[height in centimeters]/serum creatinine) with children with mild to moderate CKD (http://nephron.com/peds_nic.cgi).
Epidemiology and Etiology of Pediatric CKD
National Health and Nutrition and Examination Survey data from 1999 to 2006 estimated the incidence of CKD among adults at 26,000,000 of a population base of 200 million, with millions more at risk. The prevalence of CKD in children is unknown, but it is estimated at 82 cases per million per year. Conversely, national registries have determined the incidence of pediatric end-stage kidney disease (ESKD), the worst form of CKD, at 15 cases per million per year. The 10-year survival rate for adolescent-onset ESKD is 80%, although lower rates are seen in adult-onset ESKD. This represents a 30-fold increase in mortality compared with the general US adolescent population. In this survey, survival was better for younger adolescents, males, whites, Asians, and transplant recipients. (2)
The cause of ESKD varies by age. Younger patients have increased rates of structural anomalies of the kidneys and urinary tract, whereas older children and adolescents are more likely to have glomerular diseases. (2) The most frequent acquired and congenital forms of CKD include glomerulopathies (33%); vesicoureteral reflux, obstruction, or infections (25%); hereditary nephropathies (16%); hypoplasia or dysplasia (11%); and vascular disorders (5%). African Americans and Latinos are disproportionately affected by CKD in part due to a higher incidence of glomerular conditions. With the increasing incidence of obesity and type 2 diabetes mellitus in youth, the incidence of CKD in adult life is expected to increase. The pediatric ESKD population is only 2% of all patients with this condition, making the transition to adult-focused care difficult because adult nephrologists are unfamiliar with pediatric diagnoses (ie, the most common cause of CKD among adults is diabetes mellitus). The life course of CKD in children and adolescents are discussed in the following sections and summarized in Table 2.
Pathogenesis of CKD
CKD progression is influenced by the severity of the initial renal damage, extent of nephron loss, and the age of nephron loss, which limits renal reserve. An increased risk of progressive structural damage occurs when there is superimposed acute kidney injury from infections, dehydration, drugs, or toxins. The extent of injury can result from a single episode, as seen with acute glomerulonephritis; continuous injury from vesicoureteral reflux, chronic infections, obstructive uropathies; or recurrent injuries from diabetes, lupus, or chronic glomerulopathies. Additional factors that influence progression include host susceptibility, genetic susceptibility, and duration of disease before diagnosis, timing of therapeutic intervention, hypertension, and proteinuria. Periods of rapid growth, such as with infancy and puberty when body mass increases, may result in deterioration of renal function due to the increased filtration demands on the remaining nephron units.
Understanding the presentation of clinical and laboratory features of CKD assists in the early diagnosis. Hypoplastic and dysplastic nephropathies often present with fluid and electrolyte losses and growth failure. Glomerulopathies typically present with hypertension, hematuria (microscopic and macroscopic), edema, and alterations in urine output. In contrast, tubular and interstitial nephropathies present with significant losses of electrolytes (hypokalemia and hypophosphatemia), polyuria, polydipsia, urinary concentrating defects, enuresis, metabolic acidosis, and no clinical edema or hypertension. Despite these factors, progression of CKD is modifiable with the use of angiotensin-converting enzyme (ACE) inhibition. The Effect of Strict Blood Pressure Control and ACE Inhibition on the Progression of Chronic Renal Failure in Pediatric Patients trial was a randomized controlled trial that involved 385 children, ages 3 to 18 years, with CKD who were treated with ramipril, an ACE inhibitor. This trial found that intensified blood pressure control and early decreases in proteinuria effectively slowed the progression of renal disease in those children with CKD due to primary glomerulopathies or renal hypoplasia-dysplasia. (3)
Immunizations and CKD
Children with CKD or ESKD, including those undergoing dialysis or those with kidney transplants, are likely to have reduced responses or reduced duration of immunity after immunizations, placing them at increased risk for vaccine-preventable diseases. (4) Either specific disease states (lupus nephritis, nephrotic syndrome, dialysis, or kidney transplant) or their consequent therapies lead to suboptimal immunization rates from delayed or missed immunizaions. Children with CKD should receive the recommended childhood immunizations as published by the Centers for Disease Control and Prevention (www.aap.org/immunization; http://www.cdc.gov/vaccines/schedules/index.html), with the exception of live viruses in those receiving immunosuppressive medications. Special attention is necessary for immunization against hepatitis B and pneumococcus. Patients undergoing dialysis, particularly hemodialysis, are at risk for hepatitis B infection from suboptimal vaccination responses or a rapid decline in immune response. If postvaccination monitoring does not reveal protective antibody levels after the primary series, then reimmunization should be instituted. If the patient remains nonimmune, then further attempts at immunization are not recommended. Patients with nephrotic syndrome and those with CKD are at risk for invasive disease from Streptococcus pneumoniae, and all should receive the recommended dosages of the 13-valent pneumococcal conjugate vaccine and coverage for additional pneumococcal serotypes with the 23-valent polysaccharide pneumococcal vaccine administered after age 2 years and at least 8 weeks after they have received the 13-valent pneumococcal conjugate vaccine. Annual immunization against influenza is now recommended for all children older than 6 months, with only the inactivated influenza vaccine recommended for those with CKD receiving immunosuppressive therapies. Finally, immunization with live-attenuated vaccines, such as measles-mumps-rubella, varicella zoster virus, and rotavirus, should be avoided in those children with CKD or transplants who are receiving immunosuppressive medications, including corticosteroids.
Cardiovascular Disease in CKD
The long-term survival of children with CKD remains low compared with the general population. Specifically, the lifespan of a pediatric patient undergoing dialysis is shortened by 50 years compared with age- and race-matched controls. Even after successful renal transplantation, their lifespan is reduced by 25 years. As in adult patients, cardiovascular disease (CVD) accounts for most deaths in patients with pediatric-onset CKD, but unlike adults, pediatric-onset CKD patients rarely demonstrate symptomatic atherosclerosis or diabetes mellitus. (5) The prevalence of cardiovascular events with ESKD is 24.3% and 36.9% in children ages 0 to 4 years and 15 to 19 years, respectively. The most common events included arrhythmias (19.6%), valvular heart disease (11.7%), cardiomyopathy (9.6%), and acute cardiac death (2.8%). In a separate analysis, CVD and cardiac death represented 40% and 20%, respectively, of all deaths in pediatric patients with ESKD. In addition, cardiovascular alterations that lead to these terminal events begin in the early stages of CKD possibly as an adaptation to the hemodynamic and biochemical derangements present in CKD.
Pediatric patients with CKD have a high prevalence of traditional risk factors for CVD. One of the most common risk factors in this population is hypertension. It is also recognized that high-risk populations, including patients with diabetes mellitus and CKD, may have normal office-based blood pressures but significant elevations outside the office. Termed masked hypertension, these elevations are associated with development of end-organ damage. Recent data from the CKiD study demonstrate that masked hypertension is prevalent and hypertension is observed in 54% of patients in the early stages of CKD. Furthermore, the prevalence of hypertension increases in patients undergoing long-term dialysis (75%) and remains high after transplantation. The development of hypertension in pediatric CKD is multifactorial. These patients also demonstrate a high prevalence of dyslipidemia and abnormalities in glucose metabolism. Studies have elucidated several nontraditional risk factors that perpetuate CVD in these patients, including anemia, altered calcium-phosphorus metabolism, chronic inflammation, and oxidant stress. Aggressive control of blood pressure, lipid metabolism, and anemia may be critical in these patients. Additional therapeutic options, such as exercise, and anti-inflammatory agents, such as statins, ACE inhibitors, and angiotensin receptor antagonists, must also be examined to enhance the efficacy of traditional cardioprotective agents.
Fluid and Electrolyte Disturbances
Patients with CKD secondary to congenital anomalies of the kidneys and urinary tract are at risk for hypokalemia, hyponatremia, and urinary concentrating defects. Primary care clinicians need to pay particular attention when these patients develop dehydration because the normal tubular response to antidiuretic hormone (vasopressin) is impaired. Patients with early CKD can present with a nongap metabolic acidosis, but as the disease progresses this metabolic derangement becomes an increased anion gap acidosis. Hyperkalemia may become worse with progression of renal disease or in patients receiving ACE inhibitors or angiotensin-receptor blockers for hypertension.
Mineral Metabolism and Vitamin D
One of the most common complications of CKD is metabolic bone disease (MBD) (previously known as renal osteodystrophy). CKD-MBD is caused by the inability of the kidneys to excrete phosphorus and synthesize active 1,25-dihydroxyvitamin D (1,25[OH]2 D). The decreased 1,25(OH)2 D formation was hypothesized to be due to loss of functioning renal tissue or a direct result of hyperphosphatemia. More recently, the role of fibroblast growth factor 23, a bone-derived regulator of phosphate metabolism, has been recognized. With the retention of phosphate, fibroblast growth factor 23 increases and further suppresses 1,25(OH)2 D formation by the kidney. With dysregulation of calcium, phosphorus, and vitamin D, the parathyroid glands are stimulated and secondary hyperparathyroidism occurs. Until the CKD becomes severe, the serum levels of calcium and phosphorus remain normal, but parathyroid levels increase.
The treatment of CKD-MBD begins with restriction of phosphorus intake. This approach is difficult in children without severely affecting their choice of foods. Phosphorus absorption can be blocked by phosphate binders, which can be calcium-based compounds or non–calcium-containing compounds. The role of calcium as phosphate binders is balanced by the newly recognized dangers of vascular calcification as a result of elevated calcium and phosphorus in the blood. Hyperparathyroidism can be treated by administration of active vitamin D metabolites, such as 1,25(OH)2 D, which may raise the serum levels of calcium and phosphorus. Newer artificial vitamin D metabolites, which selectively suppress parathyroid secretion without increasing absorption of calcium and phosphorus, are often used in patients with more severe renal failure. Finally, in children undergoing dialysis who have uncontrolled hyperparathyroidism, activators of the calcium receptors on parathyroid cells have been found to be effective agents to control severe CKD-MBD when all other treatment agents have proven to be ineffective. As result of the availability of these most recent agents, the need for parathyroidectomy in children receiving dialysis has become rare.
Anemia is a common complication of CKD and increases in prevalence with progression of CKD. Although erythropoietin deficiency is the primary cause of anemia, other contributing factors include blood loss, iron deficiency, bone marrow suppression, malnutrition, inflammation, uncontrolled hyperparathyroidism, inadequate dialysis, or ongoing systemic diseases. Aluminum toxicity, a common cause of anemia in the past, is now extremely uncommon unless water supplies at home or in dialysis units are contaminated or when aluminum phosphate binders are used. Chronic anemia in children has significant effects on the child, including fatigue, impaired cognition, sleep disturbances, decreased exercise tolerance, depression, and poor appetite. The Kidney Disease Outcomes Quality Initiatives recommend targeting hemoglobin levels between 11 and 13 g/dL (110-130 g/L) to reduce the need for transfusions; to lessen cardiovascular complications, such as left ventricular hypertrophy; and to enhance overall quality of life. Anemia is best managed with recombinant human erythropoiesis-stimulating agents and iron supplementation. After kidney transplantation, anemia may persist from continuing degrees of CKD, adverse effects of the immunosuppressive medications, or viral infections, such as parvovirus.
Nutrition and Growth
Provision of adequate calories, particularly in infants and young children with CKD, is paramount given the effect of nutritional status on growth and neurocognitive development. (6)(7) Although cachexia and protein-energy wasting are well described among adult patients with advanced CKD, data concerning the prevalence in children with CKD are limited. Anorexia, increased energy expenditure despite adequate caloric intake, and muscle wasting are 3 major pathophysiologic features. Other contributing factors implicated are systemic inflammation, endocrine disturbances, and maladaptive neuropeptide signaling. Hypoalbuminemia and malnutrition in dialysis patients are predictors of mortality in this patient population. Anorexia, particularly in infants, may be related to altered tastes, oral food aversions, gastroesophageal reflux, delayed gastric emptying, elevated cytokine levels, and alterations in appetite-regulating hormones, such as leptin and ghrelin. Aggressive nutritional management with the guidance of qualified dieticians is critical. Dietary modifications should be individualized and may include alterations to calories, protein, fat, sodium, potassium, calcium, phosphorus, and fluid intake (http://kidney.org/professionals/KDOQI/guidelines_ped_ckd/cpr1.htm). Acknowledgment of cultural food preferences may improve adherence to dietary changes. If nutrition cannot be maximized through the addition of carbohydrate or fat sources, then placement of gastrostomy tubes, particularly in infants and small toddlers, is often necessary to provide adequate nutrition, fluids, and/or medications via bolus or continuous infusions.
Decreased linear growth is one of the most apparent effects of CKD in children. The mean height of children with CKD is 1.5 SDs below the mean. Factors that contribute to impaired growth include age at the onset of CKD, duration of CKD, metabolic acidosis, treatment modalities for primary renal disease (corticosteroids), associated genetic disorders, protein-calorie malnutrition, residual urine volume, and hormonal disturbances of the gonadotropic axis (luteinizing hormone and follicle-stimulating hormone) and somatropic axis (growth hormone, insulinlike growth factor 1, and thyroid hormone). Short stature is the most common concern associated with low health-related quality of life among survivors of pediatric-onset CKD. Optimization of growth can be achieved with appropriate caloric intake and the use of growth hormone replacement.
Neurologic and Neurocognitive Effects of CKD
Children, adolescents, and young adults with CKD are at risk for neurocognitive function impairment. This is particularly true for patients with CKD who also have hypertension because they have a lower IQ score compared with patients in similar age groups who have CKD without hypertension. (8) When compared with healthy controls, children and adolescents with CKD are at higher risk for grade retention, absenteeism, and impairments on measures of intelligence and reading. In-depth neurocognitive tests, memory, and executive function impairment are both directly associated with the level of renal function, especially in patients undergoing dialysis.
Burden of Care, Quality of Life Issues, and Psychosocial Issues in CKD
The burden of care for children and adolescents with CKD correlates directly with the level of kidney damage and requires time and attention by patients and their family. An example of this care includes the number of medications (mean [SD], 5.7 [4.8]) patients take once to several times per day, with dialysis and transplant patients requiring the largest number (particularly the first 6-12 months after transplantation). (9) The complexity of care also includes procedures such as self-catheterization several times per day, fluid and dietary restrictions, blood pressure measurements daily, injections (erythropoiesis-stimulating agents once to thrice weekly, growth hormone daily, or insulin several times per day), and/or home peritoneal (daily) or hemodialysis (thrice weekly) in ESKD cases.
Compared with healthy children and adolescents, patients with CKD have significantly lower health-related quality of life in the physical, school, emotional, and social domains. Interestingly, in a longitudinal national cohort of pediatric CKD patients, longer disease duration and older age were associated with higher quality of life scores in the physical, emotional, and social functioning domains, but older age was associated with lower school domain scores, likely related to prolonged neurocognitive abnormalities. Maternal education of 16 years or more was associated with higher Pediatric Quality of Life scores in the domains of physical, school, and social functioning. Short stature has been associated with lower quality of life. Moreover, patients with CKD and those with anemia have greater limitations in physical functioning, school work or activities with friends as a result of physical health, and parental effect on time and family activities.
On the basis of a sleep questionnaire in a Canadian cohort, sleep disorders occur in approximately 30% of children and adolescents with CKD (including dialysis and transplant), particularly restless leg syndrome and periodic limb movements. (10) In a regional US multi-institution study, 58.5% of patients with CKD had symptoms of a sleep disturbance (restless leg syndrome, periodic limb movements, excessive daytime sleepiness, or sleep disordered breathing), correlating with a decrease in quality of life, independent of the level of kidney function. (11) In the North American CKiD cohort, parents of children and adolescents with lower levels of renal function were more likely to report low energy, severe weakness, or daytime sleepiness and, consequently, overall poorer quality of life.
Depression and attention-deficit/hyperactivity disorder are common comorbidities in patients with CKD. Screening for these conditions and referral to psychological services are paramount in ensuring adjustment to the diagnosis of CKD. Families with a child with CKD experience emotional, physical, and financial stress; 2-parent households have better adaptation to this family challenge. Additional psychological burdens to the family include increased school absences for patients and their siblings and missed opportunities for family activities. Parental distractions related to the patient’s chronic condition can lead to feelings of neglect by siblings and affects the family’s financial well-being. Financial burdens result from interrupted work schedules, insurance copayments for medical visits or medications, and poor reimbursement for travel costs, meals, or parking. In general, parents of a chronically ill child have higher marital distress and decreased marital harmony when compared with parents of healthy children. The primary care clinician can help CKD families adapt to their child’s diagnosis and treatment by performing periodic screening of family health and encouraging communication during periods of family distress.
Health Care Transition, Disease Self-Management, and Treatment Adherence
The survival of patients with pediatric-onset CKD necessitates a coordinated health care transition (HCT) preparation from pediatric- to adult-focused health services and coordination between the primary care and nephrology teams. HCT is a process that should begin between ages 12 and 14 years, considering patient characteristics (cognition, developmental stage, culture, and literacy level), family factors, and health-related resources within the clinical and community settings. (12) Parental or caregiver willingness to relinquish responsibilities for disease management requires reassurance that the children will be successful health self-managers. Patient and family education with learning tools that meet health literacy standards is fundamental for successful HCT preparation.
Measuring transition readiness to guide HCT preparation is critical to ensure guided patient education strategies. Our pediatric nephrology practices are using a clinician-administered clinical tool termed the TRxANSITION Scale, developed among pediatric patients with CKD and ESKD. (13) Preliminary work with this tool suggests that older age is significantly associated with greater acquisition of HCT skills and disease self-management. A self-administered HCT readiness tool has also been created for patients with CKD and is termed The STARx Transition-readiness survey, and our preliminary data reveal that older age and female sex appear to be associated with greater transition readiness. (13)
To ensure a successful HCT process, an interdisciplinary collaboration among the patient, family, and primary and subspecialty pediatric clinicians must occur. Communication between the pediatric- and adult-focused health care teams and the patient is paramount to ensure continuity of care, optimized health outcomes, health-related quality of life, and continuous quality improvement. An example of a tool that can assist with this communication is the TRxANSITION Passport, a patient portable medical summary with information on diagnoses and medications in the form of a wallet-size identification card. (12) Transition preparation is optimized with the services of a dedicated transition coordinator.
Adherence to medical treatment among pediatric patients with CKD and ESKD is multifactorial and a major challenge, particularly in the adolescent and young adult populations. CKD can be silent, and the consequences of nonadherence are not palpable to patients who have such complex medical and dietary regimens. In fact, despite medical advances, kidney transplant loss in adolescents exceeds that of any other population. (14) Adherence among adolescents is also compromised by poor understanding and poor consequence recognition, leading to an inconsistent commitment to the treatment regimens. In our practice, low parental and child literacy has been correlated with lower adherence to medical appointments, greater emergency department use, and greater morbidity (peritonitis and transplant rejection). A problem with measuring treatment adherence is the lack of a standardized definition of what constitutes adherence and how to measure adherence consistently. The primary care clinician and subspecialists can confirm adherence in patients who have biomarkers, such as drug levels. In CKD patients, phosphorus levels, prescription refill rates, or medical appointment attendance can be used to determine adherence.
Discussion of Cases
Prenatal ultrasonography may have identified small kidneys, leading to an earlier diagnosis and medical intervention. His poor growth is attributable to metabolic acidosis, fluid and electrolyte losses, inadequate nutritional intake, and MBD. The leg pains are likely due to hypocalcemic-induced cramps. The developmental delay that resulted from progressive, unrecognized kidney failure places him at risk for poor school performance and neurocognitive abnormalities. He will require aggressive nutritional monitoring and likely a gastrostomy tube to meet caloric demands. Early intervention programs and individualized education plans will optimize vocational milestones. The likelihood of high urinary output from renal tubular damage impairs urinary concentrating ability regardless of hydration status. This high urinary output translates into constant thirst and frequently daytime and nighttime incontinence, with its psychosocial effect of adjustment disorder. Thorough family history is indicated because this is a congenital anomaly. He needs to get to a weight of approximately 10 kg to physically be able to receive a kidney transplant. If this child’s diagnosis were posterior urethral valves, many of these complications would also apply. Although posterior urethral valves can be diagnosed prenatally, in many instances and despite surgical valve ablation, patients are still at risk for CKD or ESKD, particularly during adolescence.
This patient has been diagnosed as having CKD at a crucial developmental age. She is at risk for psychological distress and must adjust to her life-changing diagnosis and the medical treatment. This form of nephritis will need aggressive immunosuppression (corticosteroids and cyclophosphamide), which has many adverse effects, some of which can affect appearance, placing her at risk for treatment nonadherence. Her hypertension with proteinuria will require the use of ACE inhibitors or angiotensin-receptor blockers for their renoprotective effect. She needs to know that if she becomes pregnant the fetus is at risk for ACE fetopathy, affecting the fetal kidneys.
Cases 1 and 2
Both patients are at risk for early cardiovascular events during young adulthood. They would benefit from participating in therapeutic camps and psychosocial services. Both families will need to receive counseling and support to optimize family function. Living kidney donation for transplantation by family members of friends needs to be encouraged because this type of donation has better outcomes. The HCT preparation will need to start between ages 12 and 14 years to ensure successful disease self-management when they transfer to adult-focused health care clinicians. Both patients are at greater risk of nonadherence during the adolescent years, and prescription refill rate or therapeutic drug levels will assist the primary care clinician’s team to counsel these patients and encourage treatment concordance.
Primary Care Clinician Activities to Overcome the Challenges in the Diagnosis and Management of Pediatric-Onset CKD
The 2 major barriers for early diagnosis and treatment of CKD are low awareness by the patient, family, and health care clinicians of risk factors for this condition and the silent nature of this condition in its initial stages. Early identification of pediatric CKD in patients at risk is paramount to optimize patient outcomes. We suggest activities to achieve this goal in Table 3. Patients often present to emergency departments with CKD or ESKD in its late stages, and adjustment to the diagnosis is a major challenge for both the patients and their families. Regular screening for CKD factors as suggested in Table 3 and Table 4 will ensure early diagnosis and referral for prevention of unnecessary complications. As soon as CKD is diagnosed, referrals for psychosocial and educational-related services will help with adjustment to this condition. We find that if we refer all patients and families with a new diagnosis of CKD to obtain a baseline psychological evaluation there is less resistance by these families when a future need for these services arise. Establishing a relationship between the psychology team and the patient and family decreases the anxiety and stigma effect that this type of referral may create.
Starting HCT-related activities in the early stages of adolescence will ensure successful disease self-management on transferring to adult-focused health care clinicians. Monitoring and encouraging adherence to treatment and medical appointments prevents CKD complications (transplant rejection, volume overload, and hypertensive crisis) along with optimizing the longevity of renal transplants. Once the diagnosis of CKD or ESKD is established, finding a living donor among the family members or friends is difficult in part due to public misconceptions about donation. The primary care clinician can assist with patient education and identification of potential donors. Ensuring close collaboration between the medical home (primary care clinicians) and medical neighbors (subspecialty clinicians, educators, community workers, and agencies) can be difficult given that electronic medical records are not readily available to all, but close communication among health care clinicians that includes the patient and family will positively affect the long-term outcomes of youth with pediatric-onset CKD.
On the basis of evidence, children, adolescents, and young adults with chronic kidney disease or end-stage kidney disease may have:
Lower immunization rates, placing them at risk for preventable conditions. (4)
On the basis of research evidence and expert consensus, health care transition preparation to self-manage their condition will ensure successful outcomes and improved health-related quality of life. (12)(14)
Parent Resources From the AAP at HealthyChildren.org
We thank Wallace Brown, MD, Andrew Wallace, MS4, William Primack, MD, and Keisha Gibson, MD, MPH, from the University of North Carolina School of Medicine and Donald Jack Weaver, MD, PhD, and Charles McKay, MD, from Levine Children’s Hospital for their insightful comments.
- Copelovitch L,
- Warady BA,
- Furth SL
- Lande MB,
- Gerson AC,
- Hooper SR,
- et al
- © American Academy of Pediatrics, 2014. All rights reserved.