Chronic Kidney Disease in Children and Adolescents

Susan F. Massengill and Maria Ferris

Pediatrics in Review 2014;35;16
DOI: 10.1542/pir.35-1-16

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/35/1/16

Pediatrics in Review is the official journal of the American Academy of Pediatrics. A monthly
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Article

nephrology

Chronic Kidney Disease in Children and

Adolescents
Susan F. Massengill, MD,*
Maria Ferris, MD, MPH,
PhD

Author Disclosure
Drs Massengill and
Ferris have disclosed

Educational Gap
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.

no financial
relationships relevant
to this article. This
commentary does not
contain a discussion of
an unapproved/
investigative use of

Objectives
1.
2.
3.
4.

a commercial product/
device.

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.

Case 1
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 mmol/L]), and
profound hypocalcemia (calcium, 5.6 mg/dL [1.40 mmol/L]), prompting further evaluation where hypocalcemia was conrmed. Urinalysis revealed a specic 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.

Case 2
Abbreviations
1,25(OH)2 D: 1,25-dihydroxyvitamin D
ACE:
angiotensin-converting enzyme
CKD:
chronic kidney disease
CKiD:
Chronic Kidney Disease in Children
CVD:
cardiovascular disease
eGFR:
estimated glomerular ltration rate
ESKD:
end-stage kidney disease
GFR:
glomerular ltration rate
HCT:
health care transition
MBD:
metabolic bone disease

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 mmol/L); estimated glomerular ltration rate (eGFR), 34 mL/min/1.73 m2;
urea nitrogen, 75 mg/dL (26.8 mmol/L); and positive

*Director, Pediatric Nephrology, Levine Childrens 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 Childrens Hospital
TRxANSITION Program, University of North Carolina at Chapel Hill, Chapel Hill, NC.
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nephrology

antinuclear antibody and antidouble-stranded DNA results. Urinalysis reveals blood (3), proteinuria (4), 10
to 20 red blood cells per high-power eld, 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).

Introduction
In 2002, the National Kidney Foundation established
evidence-based clinical practice guidelines entitled the
Kidney Disease Outcomes Quality Initiatives, which
were designed to dene chronic kidney disease (CKD)
based on the presence or absence of markers of kidney
damage and the level of kidney function (glomerular ltration 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:
1. Kidney damage for 3 months or longer as dened
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.
2. 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.

Chronic Kidney Disease

Classification

Table 1.

Stage

Description

Kidney damage with a normal or increased

GFR (>90 mL/min/1.73 m2)
Mild reduction in the GFR
(60-89 mL/min/1.73 m2)
Moderate reduction in the GFR
(30-59 mL/min/1.73 m2)
Severe reduction in the GFR
(15-29 mL/min/1.73 m2)
Kidney failure GFR
(<15 mL/min/1.73 m2 or dialysis)

2
3
4
5

GFRglomerular ltration rate.

chronic kidney disease

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 ltration 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 reux, 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 adultfocused care difcult 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.
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Table 2.

chronic kidney disease

Life Course and Co-Morbidities of Chronic Kidney Disease

Co-Morbidities

Events or Sequelae

Traditional cardiovascular
Hypertension
Dyslipidemia
Abnormal glucose metabolism
Obesity
Tobacco use
Sedentary lifestyle
Nontraditional cardiovascular
Anemia
Volume overload
Abnormal calcium and phosphorus metabolism
Left ventricular hypertrophy
Chronic inflammation
Malnutrition
Metabolic
Hypokalemia and hyperkalemia
Hyponatremia
Hyperphosphatemia and hypocalcemia
Urinary concentrating defects
Acidosis
Mineral metabolism
Impaired phosphate excretion
Decreased vitamin D formation
Secondary hyperparathyroidism
Anemia
Erythropoietin deficiency
Blood loss
Iron deficiency
Bone marrow suppression
Malnutrition
Chronic inflammation
Uncontrolled hyperparathyroidism
Inadequate dialysis
Ongoing systemic disease
Nutrition
Acidosis
Anemia
Anorexia
Volume overload
Endocrine disorders
Chronic inflammation
Low nutrient intake
Taste disturbances
Nausea and vomiting
Impaired gastric emptying
Nutrient loss during dialysis
Growth
Early-onset CKD
Acidosis
Treatment modalities
(corticosteroids)
Associated genetic conditions
Protein-calorie malnutrition

Arrhythmias
Valvular heart disease
Cardiomyopathy (dilated)
Acute cardiac death

g
g

Arrhythmias
Metabolic bone disease
Diurnal or nocturnal enuresis
Poor growth
Metabolic bone disease

Fatigue
Impaired cognition
Sleep disturbances
Decreased exercise tolerance
Depression
Poor appetite

Malnutrition
Cachexia
Protein-energy wasting

Decreased linear growth

Poor health-related quality of life

Continued

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nephrology

Table 2.

chronic kidney disease

(Continued)

Co-Morbidities
Neurocognitive
Hypertension
Lower IQ
Impaired memory and executive functioning
Sleep disturbances
School absenteeism and grade retention
Learning disabilities and ADHD
Disease burden
Number of medications
Medication dosing schedule
Medication route (oral, injections, infusions)
Monitoring (blood pressure, urine)
Dietary restrictions
Need for dialysis
Depression

Immunologic
Immunosuppressed or underimmunization
Reproductive
Impaired fertility
Pregnancy-related events
Psychosocial
School absences
Dysmorphic features
Medication adverse effects
Psychiatric
Depression
Sleep disturbances
Family related
Economic burden
Parental work or health insurance loss
Divorce
Healthy siblings

Events or Sequelae

Poor school performance

Decreased quality of life

Depression
ADHD
Poor self-esteem
Family stress
Financial burden
Frequent school absences
Frequent parental work absences
Marital discord
Sibling(s) feeling neglected
Susceptibility to infections by common
and opportunistic organisms
Infertility and/or fetal loss
Prematurity, teratogenicity
Underemployment
Few romantic relations, bullying, low self-esteem
Nonadherence (high risk in adolescents)
Depression
Anxiety
Posttraumatic stress disorder
Poverty
Maladjustment
Poor family function

ADHDattention-decit/hyperactivity disorder.

Pathogenesis of CKD
CKD progression is inuenced 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 reux,
chronic infections, obstructive uropathies; or recurrent
injuries from diabetes, lupus, or chronic glomerulopathies.
Additional factors that inuence 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
ltration 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
uid 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 signicant losses of electrolytes
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chronic kidney disease

(hypokalemia and hypophosphatemia), polyuria, polydipsia, urinary concentrating defects, enuresis, metabolic acidosis, and no clinical edema or hypertension. Despite
these factors, progression of CKD is modiable 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 intensied 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 hypoplasiadysplasia. (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 vaccinepreventable diseases. (4) Either specic 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 inuenza is now recommended for
all children older than 6 months, with only the inactivated

inuenza vaccine recommended for those with CKD receiving immunosuppressive therapies. Finally, immunization
with live-attenuated vaccines, such as measles-mumpsrubella, 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. Specically,
the lifespan of a pediatric patient undergoing dialysis is
shortened by 50 years compared with age- and racematched 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
ofce-based blood pressures but signicant elevations
outside the ofce. 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 inammation,

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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-inammatory agents, such as statins, ACE inhibitors, and angiotensin receptor antagonists, must also
be examined to enhance the efcacy of traditional cardioprotective agents.

Metabolic Derangements
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 broblast
growth factor 23, a bone-derived regulator of phosphate
metabolism, has been recognized. With the retention of
phosphate, broblast 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 difcult in children without severely affecting their choice of foods.
Phosphorus absorption can be blocked by phosphate
binders, which can be calcium-based compounds or
noncalcium-containing compounds. The role of calcium as phosphate binders is balanced by the newly recognized dangers of vascular calcication as a result of
elevated calcium and phosphorus in the blood.

chronic kidney disease

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 articial 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.

Hematologic Complications
Anemia is a common complication of CKD and increases
in prevalence with progression of CKD. Although erythropoietin deciency is the primary cause of anemia, other
contributing factors include blood loss, iron deciency,
bone marrow suppression, malnutrition, inammation,
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 signicant 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
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chronic kidney disease

are 3 major pathophysiologic features. Other contributing

factors implicated are systemic inammation, 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 reux, delayed gastric emptying, elevated cytokine levels, and alterations in appetite-regulating hormones, such as leptin and
ghrelin. Aggressive nutritional management with the guidance of qualied dieticians is critical. Dietary modications
should be individualized and may include alterations to
calories, protein, fat, sodium, potassium, calcium, phosphorus, and uid 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, uids, 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 folliclestimulating 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 rst 6-12
months after transplantation). (9) The complexity of care
also includes procedures such as self-catheterization several times per day, uid and dietary restrictions, blood
pressure measurements daily, injections (erythropoiesisstimulating 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 signicantly 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

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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-decit/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 nancial 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 patients chronic condition can lead to feelings of neglect by siblings and affects
the familys nancial 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 childs diagnosis and
treatment by performing periodic screening of family
health and encouraging communication during periods
of family distress.

Health Care Transition, Disease SelfManagement, 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

chronic kidney disease

this tool suggests that older age is signicantly associated

with greater acquisition of HCT skills and disease selfmanagement. 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 identication 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 denition of what constitutes adherence
and how to measure adherence consistently. The primary
care clinician and subspecialists can conrm adherence in
patients who have biomarkers, such as drug levels. In
CKD patients, phosphorus levels, prescription rell rates,
or medical appointment attendance can be used to determine adherence.

Discussion of Cases
Case 1
Prenatal ultrasonography may have identied small kidneys, leading to an earlier diagnosis and medical intervention. His poor growth is attributable to metabolic acidosis,
uid and electrolyte losses, inadequate nutritional intake,
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Primary Care Clinicians Activities as Comanager of PediatricOnset Chronic Kidney Disease

Table 3.

Activity

Action Plan

Monitoring

Identify at risk
individuals

LBW infant, history of prior acute kidney

injury, obesity, diabetes, HTN
History of HUS, HSP, lupus, or other
glomerulopathies
History of urologic disorders (UTI,
obstructive uropathy)
Positive family history for CKD, PKD, ESKD
History of HTN

Immunizationsa

Routine
Pneumococcal (23-valent Pneumovax and
Prevnar 13)
HPV
Influenza
Tobacco, alcohol, drugs

Yearly BP, urinalysis, periodic serum

chemical analyses
Yearly BP, urinalysis, serum chemical
analyses
High index of suspicion for UTI, stones,
obstruction, monitor BP
Yearly BP, urinalysis
Monitor BP at routine health screenings
(http://www.nhlbi.nih.gov/guidelines/
hypertension/child_tbl.htm), annual
echocardiogram, and 24-hour ABPM
www.aap.org/immunization,
http://www.cdc.gov/vaccines/schedules/
index.html

Counseling/prevention

Nutrition, adequate dietary calcium

Avoidance of physical inactivity, obesity
Sexual practices
Self-examinations (breast, testicular)
Sun exposure
School absenteeism and social isolation
Screen for depression, anxiety, ADHD
Screen for ability to read prescription or
food labels and patient education material
Monitor adherence

Inquire about substance use at each clinic

visit in private
Show the growth chart to patients and
families, establishing ideal anthropometric
measurements and discuss physical activity
at each visit
Discuss healthy sexual behavior at each
adolescent visit
Yearly: skin cancer prevention
Yearly: school performance and need for
individualized education
Each visit: screen for psychosocial distress
and coping
Each visit: reconcile medications and ask the
patient to read labels to you and use the
teach-back method when patient
education takes place
Each visit: discuss adherence strategies
(eg, alarms, pill-boxes)

ABPMambulatory blood pressure monitoring, ADHDattention-decit/hyperactivity disorder; BPblood pressure; ESKDend-stage kidney disease;
HPVhuman papillomavirus; HSPHenoch-Schnlein purpura; HTNhypertension; HUShemolytic uremic syndrome; LBWlow birth weight;
PKDpolycystic kidney disease; UTIurinary tract infection.
a
Note vaccine response may be reduced in dialysis or transplant population.

and MBD. The leg pains are likely due to hypocalcemicinduced 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 childs 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.

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Pediatric CKD: Health Maintenance Activities by Primary Care

Clinicians

Table 4.

Surveillance and
Risk Reduction

Conditions
Stages of CKD
1 (GFR >90)

Urinalysis and BP
measurement
If low birth weight
or other factors
for Y renal mass
BP measurement

2 (GFR 60-89)
3 (GFR 30-59)
4 (GFR 15-29)
5 (Transplant)

Growth, nutrition
Urinalysis and BP
measurement
Exercise
Encourage adherence
and hydration

Peritoneal dialysis

Growth, nutrition

Hemodialysis

Growth, maintain
fluid restriction

Selective conditions or
psychosocial issues
Congenital anomalies
of the kidney and
urinary tract

Acute kidney injury

recovery

Hypertension

Universal medical services,

universal literacy and
numeracy appropriate
services

Universal Psychosocial Services

Diagnosis and
Treatment of
Comorbid Conditions

Reduce Negative Effect of

Comorbid Conditions and
Treat Complications

Monitor growth

Modify CVD risk

factors

Monitor for
proteinuria
Normalize BP
Monitor growth
Growth hormone as
Monitor proteinuria,
needed, modify
normalize BP
CVD risk factors
Rapid fluid resuscitation
Viral infection
in dehydration
surveillance
(CMV, EBV):
lymphadenopathy,
enlarged tonsils,
fever, bone
marrow
suppression
Monitor for signs of
Encourage living donor
peritonitis, optimize
transplantation
protein intake
High index of
suspicion
for infection

Teratogen avoidance and

prenatal vitamins in
future pregnancies

Postnatal
ultrasonography,
high index of
suspicion for UTI

Urinalysis and BP
measurement
Serum chemical
analyses
Monitor growth
Encourage healthy
diet and exercise

Encourage hydration,
avoid nephrotoxic
agents

Surgical correction as
needed, healthy
toilet habits
Psychosocial support of
long-term enuresis
Voiding history not reliable
during dehydration
(poor ADH response)
Nephrology referral for
abnormal growth or
laboratory values

Monitor serial BP

Annual echocardiogram,
urinalysis, eye
examination; modify
CVD risk factors
Screen for modifiable CVD factors: normal BP, hyperlipidemia, exercise, and healthy
weight and diet
Provide immunizations
Assume low functional and health literacy and numeracy and provide patient
education and counseling at a fourth grade or below level. Monitor parent and
patients ability to read prescription and food labels. Use teach-back method to
ensure comprehension of education sessions and material.
Monitor: psychosocial evaluation, health-related quality of life, individualized
education plan
Encourage: therapeutic camp participation
Continued
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Table 4. (Continued)

Conditions
Health care transition and
treatment adherence

Reduce Negative Effect of

Diagnosis and
Comorbid Conditions and
Treatment of
Surveillance and
Treat Complications
Comorbid Conditions
Risk Reduction
Assess patient and parent literacy to customize patient and family
education efforts;
Monitor ability to read prescriptions and food labels; prescription refill rate or
drug levels (when applicable) and support the health care transition process;
adherence and successful disease self-management

ADHantidiuretic hormone; BPblood pressure; CKDchronic kidney disease; CMVcytomegalovirus; CVDcardiovascular disease; EBVEpstein-Barr
virus; ESKDend-stage kidney disease; UTIurinary tract infections.

Case 2
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 angiotensinreceptor 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 benet 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 rell rate or therapeutic drug levels will assist
the primary care clinicians 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

identication 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 educationalrelated services will help with adjustment to this condition. We nd 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, nding a living donor among the family members
or friends is difcult in part due to public misconceptions
about donation. The primary care clinician can assist with
patient education and identication 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 difcult 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.

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nephrology

ACKNOWLEDGMENTS. 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 Childrens
Hospital for their insightful comments.

Summary
On the basis of evidence, children, adolescents, and
young adults with chronic kidney disease or end-stage
kidney disease may have:
Great morbidity in other organ systems, affecting their
long-term survival. (1)(2)(3)(5)(6)(7)(8)
Lower immunization rates, placing them at risk for
preventable conditions. (4)
Psychosocial and neurocognitive issues, affecting
their self-esteem and school or job performance. (1)
(8)(10)(11)
Psychiatric conditions, such as depression, anxiety,
attention-deficit/hyperactivity disorder,
maladjustment, and posttraumatic stress disorder. (1)
(10)(11)
Nonadherence (14) and great treatment burden, (9)
particularly in adolescence and young adulthood,
placing these patients at risk for transplant loss and
hospitalizations.

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)

References
1. Copelovitch L, Warady BA, Furth SL. Insights from the Chronic
Kidney Disease in Children (CKiD) study. Clin J Am Soc Nephrol.
2011;6(8):20472053

chronic kidney disease

2. Ferris ME, Gipson DS, Kimmel PL, Eggers PW. Trends in

treatment and outcomes of survival of adolescents initiating endstage renal disease care in the United States of America. Pediatr
Nephrol. 2006;21(7):10201026
3. Whl E, Trivelli A, Picca S, et al; ESCAPE Trial Group. Strict
blood-pressure control and progression of renal failure in children.
N Engl J Med. 2009;361(17):16391650
4. Neu AM. Immunizations in children with chronic kidney
disease. Pediatr Nephrol. 2012;27(8):12571263
5. Shroff R, Weaver DJ Jr, Mitsnefes MM. Cardiovascular complications in children with chronic kidney disease. Nat Rev Nephrol.
2011;7(11):642649
6. Mak RH, Cheung WW, Zhan J-Y, Shen Q, Foster BJ. Cachexia
and protein-energy wasting in children with chronic kidney disease.
Pediatr Nephrol. 2012;27(2):173181
7. Foster BJ, McCauley L, Mak RH. Nutrition in infants and very
young children with chronic kidney disease. Pediatr Nephrol. 2012;
27(9):14271439
8. Lande MB, Gerson AC, Hooper SR, et al. Casual blood
pressure and neurocognitive function in children with chronic
kidney disease: a report of the children with chronic kidney
disease cohort study. Clin J Am Soc Nephrol. 2011;6(8):
18311837
9. So TY, Layton JB, Bozik K, et al. Cognitive pharmacy services at
a pediatric nephrology and hypertension clinic. Ren Fail. 2011;33
(1):1925
10. Sinha R, Davis ID, Matsuda-Abedini M. Sleep disturbances in children and adolescents with non-dialysis-dependent
chronic kidney disease. Arch Pediatr Adolesc Med. 2009;163
(9):850855
11. Davis ID, Greenbaum LA, Gipson D, et al. Prevalence of sleep
disturbances in children and adolescents with chronic kidney
disease. Pediatr Nephrol. 2012;27(3):451459
12. Ferris ME, Mahan JD. Pediatric chronic kidney disease and the
process of health care transition. Semin Nephrol. 2009;29(4):
435444
13. Ferris ME, Harward DH, Bickford K, et al. A clinical tool to
measure the components of health-care transition from pediatric
care to adult care: the UNC TR(x)ANSITION scale. Ren Fail.
2012;34(6):744753
14. Kiley DJ, Lam CS, Pollak R. A study of treatment compliance
following kidney transplantation. Transplantation. 1993;55(1):
5156

Parent Resources From the AAP at HealthyChildren.org

English: http://www.healthychildren.org/English/family-life/health-management/pediatric-specialists/Pages/What-is-a-

Pediatric-Nephrologist.aspx
Spanish: http://www.healthychildren.org/spanish/family-life/health-management/pediatric-specialists/paginas/what-is-a-

pediatric-nephrologist.aspx
English: http://www.healthychildren.org/English/health-issues/conditions/genitourinary-tract/Pages/Children-with-a-Single-

Kidney.aspx

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PIR Quiz Requirements

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1. A 5-year-old child presents with short stature and anemia. Growth parameters have indicated that he has
a downward trend on his percentile curves for age from the 25th percentile for weight and height at age 1 year
to below the third percentile at present. He weighs 14 kg, and his height is 100 cm. Physical examination
reveals heart rate of 100/min, respiratory rate of 26/min, and blood pressure of 120/80 mm Hg. Laboratory
studies are as follows: hemoglobin, 9 g/dL; hematocrit, 27; serum sodium, 134 mEq/L; serum potassium, 4.2
mEq/L; serum chloride, 104 mEq/L; serum bicarbonate, 18 mEq/L; serum calcium, 7.0 mg/dL; serum
phosphorous, 6.8 mg/dL; serum creatinine, 1.1 mg/dL; and blood urea nitrogen, 22 mg/dL. Abdominal
ultrasonography reveals small kidneys bilaterally. In addition to his serum creatinine concentration which of
the following is most helpful in determining his estimated glomerular filtration rate?
A. Blood urea nitrogen.
B. Fractional excretion of phosphorous.
C. Height.
D. Mean blood pressure.
E. Weight.
2. A 13-year-old boy with reflux nephropathy and hypertension has been treated since age 5 years with an
angiotensin-converting enzyme (ACE) inhibitor and dietary salt restriction. His vesicoureteral reflux was
successfully repaired surgically. He is being followed up regularly by his pediatrician in consultation with
a pediatric nephrologist. His blood pressure has been well controlled in the past. He has been in the 50th
percentile for height and weight. Two years ago his serum calcium and phosphorous levels were normal, and his
serum creatinine level was 1.1 mg/dL. On his visit today, he says that he has been tired and not able to sleep
well at night. He also has had a runny nose and sore throat for the last 2 days. He has gained 10 cm in height
and 10 kg in weight in the last 2 years and he remains in the 50th percentile for his age. His blood pressure and
other vital signs are normal. Physical examination reveals pubic hair and genital development consistent with
Tanner stage IV. His serum creatinine level is 1.7 mg/dL. Which of the following is the most likely reason for
increase in his serum creatinine?
A.
B.
C.
D.
E.

ACE inhibitor nephropathy.

Expected increase according to age.
Growth spurt.
Intercurrent infection.
Recurrence of vesicoureteral reflux.

3. A 9-year-old girl presents with fever, facial rash, and joint pains. On examination she appears ill, with a heart
rate of 124/min, axillary temperature of 39C, respiratory rate of 28/min, and blood pressure of 146/90 mm Hg.
Physical examination reveals erythematous rash in the malar area and petechial rash on extremities. Spleen is
enlarged 2 cm below the costal margin. Laboratory studies are remarkable for the following: platelets, 70 3
103/mL (70 3 109/L); serum creatinine, 2.5 mg/dL; urea nitrogen, 54 mg/dL; and positive antinuclear antibody
and antidouble-stranded DNA. Urinalysis reveals 3D blood, proteinuria (4D), and 20 red blood cells per
high-power field. The urine protein to creatinine ratio is 2.5. Renal biopsy specimen reveals crescentic
proliferative glomerulonephritis. The patient responds favorably to appropriate treatment with
corticosteroids and cyclophosphamide. Which of the following antihypertensive medication for strict
control of blood pressure will result in maximum protection from progression of chronic renal failure?
A.
B.
C.
D.
E.

Darusentan (endothelin receptor antagonist).

Hydralazine (vasodilator).
Nifedipine (calcium channel blocker).
Propranolol (b blocker).
Ramipril (ACE inhibitor).

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4. A 5-year-old girl who underwent renal transplantation a year ago for dysplastic kidneys is under your care as
her primary pediatrician. She is receiving corticosteroids and cyclosporine to prevent graft rejection. She is
doing well and now is scheduled to enter kindergarten. If administered, which of the following vaccines poses
the greatest risk to her health?
A.
B.
C.
D.
E.

Diphtheria, tetanus, and acellular pertussis.

Hepatitis B.
Inactivated influenza.
23-valent polysaccharide pneumococcal vaccine.
Varicella zoster.

5. A 14-year-old girl is awaiting renal transplantation for end-stage renal disease from focal segmental
glomerulosclerosis. She is receiving hemodialysis 3 times a week and is taking multiple medications to control
her hypertension. She was doing well until 6 months ago, when she started to feel constantly tired and was not
able to sleep well at night. She has daytime somnolence and loss of appetite. She complains of frontal
headaches in the morning and often refuses to go to school. Her school grades have fallen from As and Bs to
mostly Ds. Last week her mother caught her smoking marijuana in her bedroom. Evaluation by a neurologist
has revealed no abnormality. Referral to psychology services is required to evaluate her for which of the
following conditions?
A. Chemical dependency.
B. Childhood-onset schizophrenia.
C. Depression.
D. Munchausen syndrome.
E. Oppositional defiant disorder.

Corrections
In the November 2013 article Cephem Antibiotics: Wise Use Today Preserves Cure for Tomorrow Parker S, Mitchell M,
Child J. Pediatr Rev. 2013;34(11):510524, doi: 10.1542/pir.34-11510), in Table 3, under the Cefotaxime column, in the
Escherichia coli row, the missing value should be S: 1.
Also, in the print version of that article, in the Selected References introduction, the link to the complete reference list
should be: http://pedsinreview.aappublications.org/content/34/11/510/suppl/DCSupplementary_Data. The link is correct in
the online version of the journal.
In the October 2013 article Pneumonia (Gereige, RS, Laufer, PM. Pediatr Rev. 2013;34(10):438456), ampicillin dosing
in the first paragraph of Empiric Therapy should read, If this dose is desired, a combination of ampicillin-sulbactam at 300
mg/kg daily (dosing ampicillin at 200 mg/kg daily) and regular ampicillin at 100 to 200 mg/kg daily is a recommended
regimen. The online version of the article was resupplied with correct dosage.
The journal regrets this errors.

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Chronic Kidney Disease in Children and Adolescents

Susan F. Massengill and Maria Ferris
Pediatrics in Review 2014;35;16
DOI: 10.1542/pir.35-1-16

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