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Health Supervision for Children With Down Syndrome

Marilyn J. Bull and the Committee on Genetics


Pediatrics 2011;128;393; originally published online July 25, 2011;
DOI: 10.1542/peds.2011-1605

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly


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Health Supervision for Children With Down Syndrome
Marilyn J. Bull and the Committee on Genetics
Pediatrics 2011;128;393; originally published online July 25, 2011;
DOI: 10.1542/peds.2011-1605

PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly


publication, it has been published continuously since 1948. PEDIATRICS is owned, published,
and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk
Grove Village, Illinois, 60007. Copyright © 2011 by the American Academy of Pediatrics. All
rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly


publication, it has been published continuously since 1948. PEDIATRICS is owned, published,
and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk
Grove Village, Illinois, 60007. Copyright © 2011 by the American Academy of Pediatrics. All
rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.

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FROM THE AMERICAN ACADEMY OF PEDIATRICS
Guidance for the Clinician in
Rendering Pediatric Care

Clinical Report—Health Supervision for Children With


Down Syndrome
Marilyn J. Bull, MD, and the COMMITTEE ON GENETICS
ABBREVIATIONS abstract
BAER—brainstem auditory evoked response
TSH—thyroid-stimulating hormone
These guidelines are designed to assist the pediatrician in caring for
CRP—C-reactive protein the child in whom a diagnosis of Down syndrome has been confirmed
This document is copyrighted and is property of the American by chromosome analysis. Although a pediatrician’s initial contact with
Academy of Pediatrics and its Board of Directors. All authors the child is usually during infancy, occasionally the pregnant woman
have filed conflict of interest statements with the American who has been given a prenatal diagnosis of Down syndrome will be
Academy of Pediatrics. Any conflicts have been resolved through
a process approved by the Board of Directors. The American referred for review of the condition and the genetic counseling pro-
Academy of Pediatrics has neither solicited nor accepted any vided. Therefore, this report offers guidance for this situation as well.
commercial involvement in the development of the content of Pediatrics 2011;128:393–406
this publication.
The guidance in this report does not indicate an exclusive INTRODUCTION
course of treatment or serve as a standard of medical care.
Variations, taking into account individual circumstances, may be Children with Down syndrome have multiple malformations, medical
appropriate. conditions, and cognitive impairment because of the presence of extra
genetic material from chromosome 21.1,2 Although the phenotype is
variable, there typically are multiple features that enable the experi-
enced clinician to suspect the diagnosis. Among the more common
physical findings are hypotonia, small brachycephalic head, epicanthal
folds, flat nasal bridge, upward-slanting palpebral fissures, Brushfield
spots, small mouth, small ears, excessive skin at the nape of the neck,
single transverse palmar crease, and short fifth finger with clinodac-
tyly and wide spacing, often with a deep plantar groove between the
first and second toes. The degree of cognitive impairment is variable
www.pediatrics.org/cgi/doi/10.1542/peds.2011-1605 and may be mild (IQ of 50 –70), moderate (IQ of 35–50), or occasionally
doi:10.1542/peds.2011-1605 severe (IQ of 20 –35). There is a significant risk of hearing loss (75%);
All clinical reports from the American Academy of Pediatrics obstructive sleep apnea (50%–79%); otitis media (50%–70%); eye dis-
automatically expire 5 years after publication unless reaffirmed, ease (60%), including cataracts (15%) and severe refractive errors
revised, or retired at or before that time.
(50%); congenital heart defects (50%); neurologic dysfunction (1%–
PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).
13%); gastrointestinal atresias (12%); hip dislocation (6%); thyroid
Copyright © 2011 by the American Academy of Pediatrics disease (4%–18%)3–6; and, less commonly, transient myeloproliferative
disorder (4%–10%) and later leukemia (1%) and Hirschsprung disease
(⬍1%) (Table 1). The social quotient may be improved with early-
intervention techniques, although the level of function is exceedingly
variable. Children with Down syndrome often function more effectively
in social situations than would be predicted on the basis of cognitive
assessment results.
In approximately 95% of children with Down syndrome, the condition is
sporadic because of nonfamilial trisomy 21, in which there are 47
chromosomes with a free extra chromosome 21 being present. In ap-
proximately 3% to 4% of persons with the Down syndrome phenotype,
the extra chromosomal material is the result of an unbalanced trans-
location between chromosome 21 and another acrocentric chromo-
some, usually chromosome 14. Approximately three-quarters of these

PEDIATRICS Volume 128, Number 2, August 2011 393


TABLE 1 Medical Problems Common in Down syndrome and facilitate their transi- incorporates maternal age risk with
Syndrome
tion to adulthood. The following outline measurement of maternal serum hCG,
Condition %
is designed to help the pediatrician unconjugated estriol, ␣-fetoprotein
Hearing problems 75
provide care for children with Down (AFP), and inhibin levels. The detection
Vision problems 60
Cataracts 15 syndrome and their families in the rate of Down syndrome by first-
Refractive errors 50 medical home. It is organized by the trimester screening is 82% to 87%, by
Obstructive sleep apnea 50–75 issues that need to be addressed in second-trimester screening is 80%,
Otitis media 50–70
Congenital heart disease 40–50 various age groups (see Appendix 1). and by combined first- and second-
Hypodontia and delayed dental eruption 23 Several areas require ongoing assess- trimester screening (referred to as in-
Gastrointestinal atresias 12 tegrated screening) is approximately
Thyroid disease 4–18
ment throughout childhood and
Seizures 1–13 should be reviewed at every physician 95%. These screening tests are re-
Hematologic problems visit and at least annually. These areas ported to have a 5% false-positive
Anemia 3
include: rate.9–12
Iron deficiency 10
Transient myeloproliferative disorder 10 ● personal support available to family; Pediatricians may be asked to counsel
Leukemia 1 a family whose fetus has been identi-
Celiac disease 5 ● participation in a family-centered
fied with or is at increased risk of
Atlantoaxial instability 1–2 medical home;
Autism 1 Down syndrome. In some settings, the
Hirschsprung disease ⬍1
● age-specific Down syndrome–re- pediatrician may be the primary re-
lated medical and developmental source for counseling. At other times,
conditions; counseling may have been provided
● financial and medical support pro- for the family by a certified genetic
unbalanced translocations are de
grams for which the child and fam- counselor, a clinical geneticist, obste-
novo, and the remainder result from
ily may be eligible; trician, or developmental-behavioral
familial translocations. If the child has
● injury and abuse prevention with pediatrician. In addition, parents may
a translocation, a balanced transloca-
special consideration of develop- have received information from a
tion must be excluded in the parents.
mental skills; and Down syndrome program, a national
When there is a translocation in a par-
Down syndrome organization, or an In-
ent, additional familial studies and ge- ● nutrition and activity to maintain ap-
ternet site. Because the pediatrician
netic counseling should be provided. In propriate weight.
often has a previous relationship with
the remaining 1% to 2% of persons
THE PRENATAL VISIT the family, he or she should be pre-
with the Down syndrome phenotype, a
pared to review this information and
mix of 2 cell lines is present: one nor- The American College of Obstetricians assist in the decision-making process.
mal and the other with trisomy 21. This and Gynecologists recommends that When asked, the pediatrician should
condition is called mosaicism. Persons all pregnant women, regardless of discuss the following topics with the
with mosaicism may be more mildly age, be offered the option of diagnostic family:
affected than persons with complete testing for Down syndrome and consider
trisomy 21 or translocation chromo- 1. The prenatal laboratory studies
less invasive screening options.7,8
some 21, but this is not always the that lead to the diagnosis and any
Screening options have improved signif-
case, and their condition may include fetal imaging studies that have
icantly with the introduction of first-
any of the associated medical prob- been or will be performed.
trimester screening, which incorporates
lems and be indistinguishable from tri- maternal age, nuchal translucency ul- 2. The mechanism for occurrence of the
somy 21. Recurrence risks for families trasonography, and measurement of disorder in the fetus and the potential
with an affected child depend on maternal serum human chorionic go- recurrence rate for the family as pro-
many factors, and families benefit nadotropin (␤-hCG) and pregnancy- vided by genetic counseling.
from counseling by a clinical genetic associated plasma protein A (PAPP-A). 3. The prognosis and phenotypic man-
professional. Second-trimester screening is available ifestations, including the wide
Medical management, home environ- for patients who first seek medical range of variability seen in infants
ment, early intervention, education, care in the second trimester or in loca- and children with Down syndrome.
and vocational training can signifi- tions where first-trimester screening is Families benefit from hearing a fair
cantly affect the level of functioning of not available. The second-trimester and balanced perspective, includ-
children and adolescents with Down screening, often called the quad screen, ing the many positive outcomes of

394 FROM THE AMERICAN ACADEMY OF PEDIATRICS


FROM THE AMERICAN ACADEMY OF PEDIATRICS

children with Down syndrome and 3. Offer referral to a clinical geneticist positive FISH-test result should be con-
their effect on the family. for a more extended discussion of firmed by a complete chromosome
4. Any additional studies performed clinical outcomes and variability, analysis to identify translocations that
that may refine the estimation of recurrence rates, future reproduc- may have implications for further re-
the prognosis (eg, fetal echocardio- tive options, and evaluation of the productive counseling for the parents
gram, ultrasonographic examina- risks for other family members. and possibly other family members.
tion for gastrointestinal tract mal- The mother should be allowed to re-
formations). Consultation with an HEALTH SUPERVISION FROM BIRTH cover from the immediate delivery of
appropriate medical subspecialist, TO 1 MONTH: NEWBORN INFANTS the infant and have her partner or sup-
such as a pediatric cardiologist or a Examination port person present before the diagno-
pediatric surgeon, should occur The first step in evaluating a newborn sis is given. The information should be
prenatally if abnormal findings are infant for trisomy 21 is a careful review relayed in a private setting by the phy-
detected. of the family history and prenatal in- sicians involved, optimally by the pri-
5. Currently available treatments and formation, particularly if prenatal mary care provider for the infant and
interventions. This discussion chromosome studies were performed. the delivering physician.13 It is recom-
needs to include the efficacy, poten- Previous children born with trisomy 21 mended that hospitals coordinate the
tial complications and adverse ef- or developmental differences or preg- delivery of the information and offer a
fects, costs, and other burdens as- nancies that ended in miscarriage may private hospital room pending confir-
sociated with treatments. Discuss be significant clues that a family may mation of the diagnosis.
early-intervention resources, par- carry a balanced translocation that An important aspect of providing in-
ent support programs, and any ap- predisposes them to having children formation about Down syndrome to
propriate future treatments. with trisomy 21. For children who have families includes first congratulat-
6. The options available to the family had the diagnosis made prenatally, a ing parents on the birth of their in-
for management and rearing of formal copy of the chromosome report fant. Obstetricians and pediatricians
the child should be discussed us- should be obtained. This report allows should coordinate their messaging
ing a nondirective approach. In the clinician to confirm the diagnosis, and inform parents of their suspicion
cases of early prenatal diagnosis, review the results with the family, and immediately, in a private setting and,
this may include discussion of add the formal diagnosis to the child’s where appropriate, with both parents
pregnancy continuation or termi- medical record. If the results of prena- together. Physicians should use their
nation, raising the child in the tal testing are not available, a blood experience and expertise in providing
family, foster care placement, and sample should be obtained for postna- support and guidance for families. Cli-
adoption. tal cytogenetic analysis to confirm the nicians should ensure a balanced ap-
diagnosis and rule out a chromosome proach rather than their personal
7. Availability of genetic counseling
translocation. opinions, give current printed materi-
or meeting with a genetics
A physical examination is the most sen- als, and offer access to other families
professional.
sitive test in the first 24 hours of life to who have children with Down syn-
If the pregnancy is continued: drome and support organizations if lo-
diagnose trisomy 21 in an infant. If the
1. Develop a plan for delivery and neo- clinician feels that enough criteria are cally available. It is important that cli-
natal care with the obstetrician and present on physical examination, then nicians be cognizant of the realities
the family. As the pregnancy pro- a blood sample should be sent for and possibilities for healthy, produc-
gresses, additional studies should chromosome evaluation. The clinician tive lives of people with Down syn-
be performed if available, if recom- should alert the laboratory and re- drome in society.13
mended by subspecialty consul- quest rapid results. A study that uses Confirm the laboratory diagnosis of
tants, and/or if desired by the fam- fluorescent in situ hybridization (FISH) Down syndrome and review the karyo-
ily for modifying this management technology should be available within type with the parents when the final
plan (eg, detection of a complex 24 to 48 hours to facilitate diagnosis result is available. Discuss the specific
heart defect by echocardiography). and parent counseling. A FISH study findings with both parents whenever
2. Offer parent-to-parent contact and can only indicate that an extra copy of possible, and talk about the potential
information about local and na- chromosome 21 is present; it cannot clinical manifestations associated
tional support organizations. detect translocations. Therefore, a with the syndrome. These topics

PEDIATRICS Volume 128, Number 2, August 2011 395


should be reviewed again at a subse- ence in managing the child with TMD usually regresses spontaneously
quent meeting. Parents should be re- Down syndrome. within the first 3 months of life, but
ferred for genetic counseling if it ● Congenital hearing loss, with objec- there is an increased risk of later on-
was not conducted prenatally. tive testing, such as brainstem audi- set of leukemia for these patients
Newborn care is often provided in a tory evoked response or otoacous- (10%–30%).20 Polycythemia is also
hospital setting by a physician who will tic emission, at birth, according to common in infants with Down syn-
not be the primary care provider, and the universal newborn hearing drome (18%– 64%)21 and may require
extreme care is required to be certain screening guidelines. Complete any careful management. Infants with
that a smooth transition occurs for the needed follow-up assessment by 3 TMD and polycythemia should be fol-
family. months.16,17 lowed according to subspecialty con-
sultation recommendations. Parents
● Duodenal atresia or anorectal atre-
Discuss and Review of infants with TMD should be coun-
sia/stenosis by performing a his-
● Hypotonia. seled regarding the risk of leukemia
tory and clinical examination.
and made aware of the signs, includ-
● Facial appearance, and acknowl- ● Apnea, bradycardia, or oxygen de-
ing easy bruising, petechiae, onset of
edge the presence of familial saturation in a car safety seat for lethargy, or change in feeding pat-
characteristics. infants who are at increased risk terns. Leukemia is more common in
● Feeding issues. Children with Down because they have had cardiac sur- children with Down syndrome than in
syndrome can usually nurse, and gery or are hypotonic. A car safety the general population but still rare
many can breastfeed successfully. seat evaluation should be con-
(1%).
Occasionally, some will need early ducted for these infants before hos-
pital discharge.18 ● Congenital hypothyroidism (1% risk).
supplementation until a successful
Obtain thyroid-stimulating hormone
nursing pattern is established. ● Constipation. If constipation is pres-
(TSH) concentration if state newborn
Some infants will also sleep for pro- ent, evaluate for restricted diet or
screening only measures free thyrox-
longed periods and need to be limited fluid intake, hypotonia, hypo-
ine (T4); congenital hypothyroidism
awakened to feed to maintain ade- thyroidism, or gastrointestinal tract
can be missed if only the T4 concen-
quate calorie intake. malformation, including stenoses
tration is obtained in the newborn
or Hirschsprung disease, for which
screening. Many children with Down
Evaluate for there is an increased risk.
syndrome have mildly elevated TSH
● Heart defects (⬃50% risk). Perform ● Gastroesophageal reflux, which is and normal free T4 levels. Manage-
an echocardiogram, to be read by a usually diagnosed and managed ment of children with abnormal thyro-
pediatric cardiologist, regardless of clinically. If severe or contributing tropin or T4 concentrations should be
whether a fetal echocardiogram to cardiorespiratory problems or discussed with a pediatric
was performed. Refer to a pediatric failure to thrive, refer for subspe- endocrinologist.
cardiologist for evaluation any in- cialty intervention.
fant whose postnatal echocardio- ● Stridor, wheezing, or noisy breath- Anticipatory Guidance Given at
gram results are abnormal. ing. If severe or contributing to car- Least Once Between Birth and 1
● Feeding problems. Refer all infants diorespiratory problems or feeding Month of Age
who have marked hypotonia as well difficulty, refer to pediatric pul- ● Discuss increased susceptibility to
as infants with slow feeding, chok- monologist to assess for airway respiratory tract infection. Children
ing with feeds, recurrent pneumo- anomalies. Tracheal anomalies and with signs and symptoms of lower
nia, or other recurrent or persistent small tracheal size may also make respiratory tract infection should
respiratory symptoms and unex- intubation more difficult. be evaluated acutely by a medical
plained failure to thrive for a radio- ● Hematologic abnormalities. Obtain a provider, and in the presence of
graphic swallowing assessment.14,15 complete blood cell count. Leukemoid cardiac or chronic respiratory
● Cataracts at birth by looking for a reactions, or transient myeloprolifer- disease, aggressive treatment
red reflex. Cataracts may prog- ative disorder (TMD). TMD is found al- should be instituted.14 Children
ress slowly and, if detected, need most exclusively in newborn infants with comorbid conditions who
prompt evaluation and treatment with Down syndrome and is relatively qualify should have respiratory
by an ophthalmologist with experi- common in this population (10%).19 syncytial virus prophylaxis.22

396 FROM THE AMERICAN ACADEMY OF PEDIATRICS


FROM THE AMERICAN ACADEMY OF PEDIATRICS

● Discuss with parents the importance suggested.29 Until studies confirm awakening, daytime sleepiness,
of cervical spine-positioning precau- this finding and document that apneic pauses, and behavior prob-
tions to avoid excessive extension or screening improves outcomes, rou- lems that could be associated with
flexion to protect the cervical spine tine renal and urologic screening is poor sleep. Refer to a physician
during any anesthetic, surgical, or ra- not recommended. with expertise in pediatric sleep
diographic procedure.23,24 disorders for examination and fur-
● Discuss efficacy of early interven-
HEALTH SUPERVISION FROM 1 ther evaluation of a possible sleep
MONTH TO 1 YEAR: INFANCY disorder if any of the above-
tion and availability of early-
intervention services and therapies Physical Examination and mentioned symptoms occur.32,33
in the community. Initiate referral Laboratory Studies ● At each well-child visit, discuss with
as appropriate.25 parents the importance of maintain-
Review the risk of serous otitis media
ing the cervical spine in a neutral
● Inform the family of the availability of (50%–70%). Review the previous hear-
position during any anesthetic, sur-
support and advice from parents of ing evaluation (brainstem auditory
gical, or radiographic procedure to
other children with Down syndrome. evoked response [BAER, ABR] or otoa-
minimize the risk of spinal cord in-
● Supply names of Down syndrome coustic emission). If the child passed
jury and review the signs and
support groups and current books the screening study, rescreen at 6
symptoms of myelopathy. Perform
and pamphlets (see “Resources for months of age for confirmation. If the
careful history and physical exami-
Parents”). infant failed to pass screening studies,
nation, and pay attention for myelo-
refer to an otolaryngologist who is
● Discuss the strengths of the child pathic signs and symptoms.
comfortable with examining infants
and positive family experiences. ● Within the first 6 months of life, re-
with stenotic external canals to deter-
● Discuss the individual resources for mine if a middle-ear abnormality is fer to a pediatric ophthalmologist
support, such as family, clergy, and present. Tympanometry may be neces- or ophthalmologist with expertise
friends. sary if the tympanic membrane is and experience with infants with
● Talk about how and what to tell sib- poorly visualized. Middle-ear disease disabilities to evaluate for strabis-
lings, other family members, and should be treated promptly. Once a mus, cataracts, and nystagmus.34
friends. Review methods of coping clear ear is established, a diagnostic Check the infant’s vision at each
with long-term disabilities. BAER should be performed to accu- visit and use developmentally ap-
rately establish hearing status. In chil- propriate subjective and objective
● Review the recurrence risk in sub- criteria. If lacrimal duct obstruction
dren with stenotic canals, in which
sequent pregnancies and the avail- is present, refer for evaluation for
the tympanic membranes cannot be
ability of prenatal diagnosis as pro- surgical repair of drainage system
seen, refer to an otolaryngologist for
vided in genetic counseling. if not resolved by 9 to 12 months of
examination under an office micro-
● Discuss treatments that are consid- scope. Interval ear examinations age.35
ered complementary and alterna- should be performed by the otolaryn- ● Verify results of newborn thyroid-
tive. Parents need an opportunity to gologist every 3 to 6 months until the function screen if not previously
learn objectively which therapies tympanic membrane can be visual- performed. Because of increased
are safe and which are potentially ized by the pediatrician and tympa- risk of acquired thyroid disease, re-
dangerous (eg, cell therapy that nometry can be performed reliably. A peat measurement of TSH at 6 and
may transmit slow viruses and fat- behavioral audiogram may be at- 12 months of age and then annually.
soluble vitamins that can cause tox- tempted at 1 year of age, but many chil- ● Monitor infants with cardiac de-
icity). Several articles and Internet dren will not be able to complete the fects, typically ventricular or atrio-
sites evaluate the legitimacy of study and may need additional testing ventricular septal defects that
claims that are made.26–28 by BAER.30,31 cause intracardiac left-to-right
● Renal and urinary tract anomalies ● At least once during the first 6 shunts, for symptoms and signs of
have been reported to occur at in- months of life, discuss with par- congestive heart failure as pulmo-
creased frequency among persons ents symptoms of obstructive nary vascular resistance decreases
with Down syndrome, and screen- sleep apnea, including heavy and pulmonary blood flow in-
ing for these anomalies for all chil- breathing, snoring, uncommon creases. Tachypnea, feeding diffi-
dren with Down syndrome has been sleep positions, frequent night culties, and poor weight gain may

PEDIATRICS Volume 128, Number 2, August 2011 397


indicate heart failure. Medical man- iron deficiency on the basis of a his- prenatal diagnosis at least once in
agement, including nutritional sup- tory of decreased iron intake.38–42 the first year of life and more often if
port, may be needed until the infant ● Monitor for signs of neurologic dys- judged necessary by the clinician.
can undergo cardiac surgery to re- function that may occur. Children Refer for genetic counseling if not
pair the defects. For patients with with Down syndrome have an in- already provided.
large ventricular septal defects and creased risk of seizures, including ● Be prepared to discuss and answer
without obstruction to pulmonary infantile spasms (1%–13%)43,44 and questions about treatments that are
blood flow, repair should be per- other conditions including Moya- considered complementary and alter-
formed before 4 months of age to moya disease.45 native at each well-child visit.
limit the potential for development
● Administer immunizations, includ-
of pulmonary hypertension and as- HEALTH SUPERVISION FROM 1 TO 5
ing influenza vaccine and other
sociated complications. Infants and vaccines recommended for all chil- YEARS: EARLY CHILDHOOD
children with Down syndrome are dren, unless there are specific ● Obtain a history and perform a
also at increased risk of pulmonary contraindications.46 physical examination, and give at-
hypertension even in the absence of tention to growth and developmen-
intracardiac structural defects. Anticipatory Guidance tal status at every well-child visit.
● Obtain hemoglobin concentration ● Monitor weight and follow weight- ● Review the risk of hearing loss asso-
beginning at 1 year of age and annu- for-height trends at each health ciated with serous otitis media. For
ally thereafter. Children with Down care visit. Review the infant’s a child who passed diagnostic hear-
syndrome have been shown to have growth and plot it by using the stan- ing testing, additional screening or
significantly lower dietary intakes dard growth charts of the National behavioral audiogram and tympa-
of iron than their typically develop- Center for Health Statistics or the nometry should be performed every
ing peers.36 Increased erythrocyte World Health Organization.47 The 6 months until normal hearing lev-
mean corpuscular volume (MCV) previously used Down syndrome- els are established bilaterally by
has been reported in 45% of pa- specific growth charts no longer re- ear-specific testing (usually after 4
tients with Down syndrome with and flect the current population styles years of age). Subsequently, behav-
without heart disease, and when and body proportion. Until new ioral hearing tests should be per-
MCV is decreased, it occurs at ap- charts are developed, patterns of formed annually. If normal hearing
proximately the same time as ane- growth and weight gain should be is not established by behavioral
mia.37 Therefore, MCV is not useful followed on the available standard testing, additional screening by
in screening for the diagnoses of growth charts and should include otoacoustic emissions or diagnos-
iron deficiency, lead toxicity, or use of weight for height and BMI.48 tic BAER should be performed with
thalassemia in children with Down sedation if necessary. Children
● Review availability of Down syn-
syndrome. Serum ferritin concen- who demonstrate a hearing loss
drome support groups at least once
tration is a sensitive parameter for should be referred to an otolaryn-
in the first year of life (see “Re-
assessment of iron stores in healthy gologist who is comfortable with
sources for Parents”).
subjects but is an acute-phase reac- the examination of children with
● Assess the emotional status of par- stenotic ear canals. The risk of se-
tant and may be increased in the
presence of chronic inflammation ents and intrafamilial relationships rous otitis media between 3 and 5
or infection and should be evaluated at each well-child visit. Educate and years of age is approximately 50%
support siblings and discuss sibling to 70%.
together with C-reactive protein
adjustments.
(CRP) concentration. An elevated ● Check the child’s vision, and use de-
CRP level is an indication that a nor- ● Review connection to early- velopmentally appropriate subjec-
mal ferritin level may be falsely ele- intervention services and their rela- tive and objective criteria at each
vated and is not a reliable indication tionship to the strengths and needs well-child visit. Refer the child
of normal iron status. Serum ferri- of the infant and family at each well- annually to a pediatric ophthalmol-
tin and CRP or reticulocyte hemoglo- child visit. ogist or ophthalmologist with spe-
bin (CHr) concentrations should be ● Review the family’s understanding cial expertise and experience with
obtained at annual visits for pa- of the risk of recurrence of Down children with disabilities. Children
tients who are at increased risk of syndrome and the availability of with Down syndrome have a 50%

398 FROM THE AMERICAN ACADEMY OF PEDIATRICS


FROM THE AMERICAN ACADEMY OF PEDIATRICS

risk of refractive errors that lead ipation in some sports, including con- multaneous quantitative IgA. The
to amblyopia between 3 and 5 tact sports such as football and soccer quantitative IgA is important, be-
years of age. Addressing refrac- and gymnastics (usually at older cause a low IgA level will result in a
tive errors and strabismus at an ages), places children at increased false-negative tissue transglutami-
early age can help prevent ambly- risk of spinal cord injury65 and that nase IgA result. Refer patients with
opia and encourage normal visual trampoline use should be avoided by abnormal laboratory values for
development.34,49–51 all children with or without Down syn- specialty assessment. There is no
drome younger than 6 years and by evidence showing routine screen-
Atlantoaxial Instability older children unless under direct ing of asymptomatic individuals as
Discuss with parents, at least bienni- professional supervision.66,67 Special being beneficial. There are neither
ally, the importance of cervical spine- Olympics has specific screening re- data nor consensus that would in-
positioning precautions for protection quirements for participation in some dicate whether patients with per-
of the cervical spine during any anes- sports.68 sistent symptoms who had normal
thetic, surgical, or radiographic proce- laboratory values on initial evalu-
dure. Perform careful history and The Symptomatic Child ation should have further labora-
physical examination with attention to Any child who has significant neck tory tests.
myelopathic signs and symptoms at pain, radicular pain, weakness, spas- ● Discuss symptoms of obstructive
every well-child visit or when symp- ticity or change in tone, gait difficul- sleep apnea, including heavy
toms possibly attributable to spinal ties, hyperreflexia, change in bowel or breathing, snoring, restless sleep,
cord impingement are reported. Par- bladder function, or other signs or uncommon sleep positions, fre-
ents should also be instructed to con- symptoms of myelopathy must un- quent night awakening, daytime
tact their physician for new onset of dergo plain cervical spine radiography sleepiness, apneic pauses, and be-
symptoms of change in gait or use of in the neutral position.55,65 If significant havior problems, that could be as-
arms or hands, change in bowel or radiographic abnormalities are pres- sociated with poor sleep at each
bladder function, neck pain, stiff neck, ent in the neutral position, no further well-child visit. There is poor corre-
head tilt, torticollis, how the child posi- radiographs should be taken and the lation between parent report and
tions his or her head, change in gen- patient should be referred as quickly polysomnogram results.33,72 There-
eral function, or weakness. as possible to a pediatric neurosur- fore, referral to a pediatric sleep
geon or pediatric orthopedic surgeon laboratory for a sleep study or poly-
The Asymptomatic Child
with expertise in evaluating and treat- somnogram for all children with
Children with Down syndrome are at ing atlantoaxial instability. If no signif- Down syndrome by 4 years of age is
increased risk of atlantoaxial sublux- icant radiographic abnormalities are recommended. Refer to a physician
ation. However, the child must be 3 with expertise in pediatric sleep any
present, flexion and extension radio-
years of age to have adequate verte- child with signs or symptoms of ob-
graphs may be obtained before the pa-
bral mineralization and epiphyseal de- structive sleep apnea or abnormal
tient is promptly referred.23,62,63
velopment for accurate radiographic sleep-study results. Discuss obesity
evaluation of the cervical spine.52 Plain ● Measure TSH annually or sooner if
child has symptoms that could be re- as a risk factor for sleep apnea.34 It
radiographs do not predict well which is recognized that access to a pedi-
children are at increased risk of devel- lated to thyroid dysfunction.
atric sleep laboratory or specialist
oping spine problems, and normal ra- ● For children on a diet that contains
may be limited for some popula-
diographs do not provide assurance gluten, at each preventative care tions and geographic areas.
that a child will not develop spine prob- visit review for symptoms poten-
● Maintain follow-up with a pediatric
lems later.53,54 For these reasons, rou- tially related to celiac disease, in-
cardiologist for patients with car-
tine radiologic evaluation of the cervi- cluding diarrhea or protracted con-
diac lesions even after complete re-
cal spine in asymptomatic children is stipation, slow growth, unexplained
pair to monitor for recurrent/resid-
not recommended. Current evidence failure to thrive, anemia, abdominal
ual lesions as well as development
does not support performing routine pain or bloating, or refractory devel-
of pulmonary hypertension.
screening radiographs for assess- opmental or behavioral prob-
ment of potential atlantoaxial instabil- lems.69–71 For those with symptoms, ● Monitor for neurologic dysfunction,
ity in asymptomatic children.55–64 obtain a tissue transglutaminase including seizures.
Parents should be advised that partic- immunoglobulin A (IgA) level and si- ● Obtain hemoglobin concentration

PEDIATRICS Volume 128, Number 2, August 2011 399


annually. Also, obtain serum ferritin ing into child’s personal space or on the basis of history of cardiac
and CRP concentrations for any performing a procedure. Remind defects.
child at risk of iron deficiency. patient and family that the only rea- ● Obtain hemoglobin concentration
son anyone should be looking at or annually and serum ferritin and CRP
Anticipatory Guidance
touching private body parts is for or reticulocyte hemoglobin concen-
● Review early intervention, including health (doctor office visits) or hy- trations at annual visits for any
physical therapy, occupational ther- giene (bathing or showering).79 child at risk of iron deficiency on the
apy, and speech therapy, at all
● On at least 1 well-child visit educate basis of history of decreased iron
health maintenance visits.
parents about increased risk of sex- intake.
● Discuss at the 30-month visit the ual exploitation, and remind them
transition from early intervention ● For children on a diet that contains
that likely perpetrators are people
to preschool, which occurs at 36 gluten, review for symptoms poten-
their child knows and trusts, not
months of age. Help the family un- tially related to celiac disease at ev-
strangers.
derstand the change from the In- ery health maintenance visit and
● At least once between 1 and 5 years evaluate if indicated.
dividualized Family Service Plan
of age, as with discussion in the first
(IFSP) in early intervention to the ● At each well-child visit, discuss with
year of life, discuss future preg-
Individualized Education Plan (IEP) parents the importance of universal
nancy planning and review risk of
through public education. precautions for protection of the
recurrence of Down syndrome and
● Discuss with caregivers at every cervical spine during any anes-
availability of prenatal diagnosis.
visit the child’s behavioral and so- thetic, surgical, or radiographic
● Assess the child’s behavior and talk procedure. Perform careful history
cial progress. Refer children who
may have autism, attention-deficit/ about behavioral management, sib- and physical examination with at-
hyperactivity disorder, or other psy- ling adjustments, socialization, and tention to myelopathic signs and
chiatric or behavioral problems for recreational skills. symptoms. Parents should also be
appropriate evaluation and inter- ● Encourage families to establish op- instructed to contact their physi-
vention as soon as suspected. Au- timal dietary and physical exercise cian immediately for new onset of
tism and other behavioral problems patterns that will prevent obesity. symptoms of myelopathy.
occur with increased frequency in ● Be prepared to discuss and an- ● Counsel parents that some sports
children with Down syndrome, and swer questions about treatments place children at increased risk of
symptoms may manifest as early as that are considered complemen- spinal cord injury.65–67
2 or 3 years of age.73–76 tary and alternative.
● Monitor for neurologic dysfunction,
● Provide influenza vaccine annually.
HEALTH SUPERVISION FROM 5 TO including seizures.
Children with chronic cardiac or
pulmonary disease should be given 13 YEARS: LATE CHILDHOOD ● Very dry skin, which may be a sign of
the 23-valent pneumococcal poly- ● Obtain a history and perform a hypothyroidism, and other skin
saccharide vaccine (PPS23) at 2 physical examination with attention problems are particularly common
years or older.22 to growth and developmental status in patients with Down syndrome.
● Reassure parents that delayed and at each annual well-child visit. Therefore, be attentive to these der-
irregular dental eruption patterns matologic problems and discuss
● Monitor growth patterns, especially
are common and that hypodontia them with the patient and family.
BMI, and emphasize healthy diet and
occurs with increased frequency lifestyle for preventing obesity. ● Discuss symptoms related to ob-
(23%).77,78 structive sleep apnea at every well-
● Obtain annual ear-specific audio-
● Encourage and model use of accu- child visit, including snoring, rest-
logic evaluation.
rate terms for genitalia and other less sleep, daytime sleepiness,
● Obtain ophthalmologic evaluation
private body parts (penis, vulva) any nighttime awakening, behavior
every 2 years. problems, and abnormal sleep posi-
time these body parts are discussed
or examined. Model respect for body ● Measure TSH annually; the risk of tion. Refer to a physician with exper-
rights by reminding patients that hypothyroidism increases with tise in pediatric sleep any child with
their body is their own and explain to age. signs or symptoms of obstructive
the child what you will do before mov- ● Individualize cardiology follow-up sleep apnea or abnormal sleep-

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FROM THE AMERICAN ACADEMY OF PEDIATRICS

study results. Discuss obesity as a of psychotropic medications among wish to discuss sterilization, and
risk factor of sleep apnea. children with Down syndrome, an- the pediatrician may review the
ecdotal reports indicate that such topic in the American Academy of
Anticipatory Guidance at Every children may differ in their re- Pediatrics policy statement “Ster-
Health Maintenance Visit sponse to medications. ilization of Minors With Develop-
● Review the child’s development and ● Counsel families regarding the tran- mental Disabilities.”86
appropriateness of school placement sition from elementary to middle ● Be prepared to discuss and answer
and developmental intervention. school, when major change often questions regarding treatments
● Discuss socialization, family status, occurs, from 1 to many teachers that are considered complementary
and relationships, including finan- and from 1 class to changing and alternative.
cial arrangements, health insur- classes. Prepare them to facilitate
ance, and guardianship. adjustment at a time when the ac- HEALTH SUPERVISION FROM 13
ademic disparity becomes greater TO 21 YEARS OR OLDER:
● Discuss the development of age-
and full inclusion becomes more ADOLESCENCE TO EARLY
appropriate social skills, self-help
difficult. ADULTHOOD
skills, and development of a sense
of responsibility. ● Refer children who may have autism Physical Examination and
● Monitor for behavior problems that for appropriate evaluation and inter- Laboratory Values
interfere with function in the home, vention as soon as suspected. ● Measure hemoglobin concentra-
community, or school. Attention ● Continue to assess, monitor, and en- tion annually.
problems, attention-deficit/hyper- courage independence with hygiene ● Measure TSH concentration annually.
activity disorder, obsessive compul- and self-care. Encourage parents to ● Obtain annual ear-specific audio-
sive behaviors, noncompliant be- teach, model, and respect privacy at logic evaluation.
havior, and wandering off are some home and in the community. Discuss
● For children on a diet that contains
of the common behavior concerns appropriate management of sexual
reported. Psychiatric disorders gluten, review for symptoms poten-
behaviors such as masturbation.
seen in typically developing children tially related to celiac disease at ev-
● Discuss progression of physical ery health maintenance visit, and
may also occur. Evaluate for medi-
and psychosocial changes through evaluate if indicated.
cal problems that can be associated
puberty and issues of fertility and ● Individualize cardiology follow-up
with behavior changes, including
contraception.79,80 Remind parents on the basis of history of cardiac
thyroid abnormalities, celiac dis-
that physical development usually defects. Discuss symptoms re-
ease, sleep apnea, gastroesopha-
follows patterns similar to those lated to obstructive sleep apnea,
geal reflux, and constipation. Inter-
found in the general population, including snoring, restless sleep,
vention strategies depend on the
but the child with Down syndrome daytime sleepiness, nighttime
child’s age, the severity of the prob-
will likely need more preparation awakening, behavior problems,
lem, and the setting in which the
in understanding and managing and sleep position at every health
problem occurs. Referral to com-
them.81 maintenance visit. Refer to a phy-
munity treatment programs, psy-
chosocial services for consultative ● Discuss the need for gynecologic sician with expertise in pediatric
care, or behavioral specialists expe- care in the pubescent girl. Talk with sleep any child with signs or symp-
rienced in working with children the patient and her family about the toms of obstructive sleep apnea or
with special needs may be neces- recurrence risk of Down syndrome an abnormal sleep-study result.
sary. The use of medication for be- (50%) were she to become preg- Discuss the risk factor of obesity
havior management should be dis- nant.82,83 Although males with Down for sleep apnea.
cussed between the primary care syndrome are usually infertile, ● Discuss with parents and the pa-
physician and specialists involved in there have been rare instances in tient at every visit the importance of
the child’s care, because children which a male has reproduced.83–85 cervical spine-positioning precau-
with Down syndrome may be more Birth control and prevention of tions for protection of the cervical
sensitive to certain medications. Al- sexually transmitted diseases spine during any anesthetic, surgi-
though there has been little re- should be discussed with patients cal, or radiographic procedure. Per-
search to directly address the use and their families. Families may form careful history and physical

PEDIATRICS Volume 128, Number 2, August 2011 401


examination with attention to myo- ● Discuss appropriateness of school drome could be enhanced through
pathic signs and symptoms. Parents placement, and emphasize planning population-based research. A rigorous
and patients should also be in- for transition to adulthood and ade- evidence-based review of screening
structed to contact their physician quate vocational training within the and treatment for atlantoaxial instabil-
immediately for new onset of symp- school curriculum.90,91 ity, for example, is needed,94 and con-
toms of myopathy. ● Talk with the female patient and her tinuing research is critical for direct-
● Counsel parents that some sports family about the recurrence risk of ing the care for optimal outcomes of
place children at increased risk of Down syndrome should she become persons with Down syndrome.1,95,96
spinal cord injury.65–67 pregnant. ACKNOWLEDGMENT
● Monitor for signs of other neurologic ● Continue to assess, monitor, and en-
The mentoring and contributions of Dr.
dysfunction, including seizures. courage independence with hygiene
William Cohen have been sincerely ap-
● Obtain ophthalmologic evaluation and self-care. Provide guidance on
preciated and were integral to the de-
every 3 years. Check for onset of healthy, normal, and typical sexual
velopment of this clinical report. His
cataracts, refractive errors, and development and behaviors. Empha-
untimely death is a great loss to his
keratoconus, which can cause size the need for understandable in-
patients and their families, his col-
blurred vision, corneal thinning, formation, and encourage opportu-
leagues, and the greater medical com-
or corneal haze and is typically di- nities for advancing comprehension
munity. This clinical report is dedi-
agnosed after puberty. of sexuality. Discuss the need for
cated to his memory.
contraception and prevention of
● Examine annually for acquired mi- sexually transmitted diseases and LEAD AUTHOR
tral and aortic valvular disease in the degree of supervision required. Marilyn J. Bull, MD
older patients with Down syndrome. Advocate for the least invasive and COMMITTEE ON GENETICS, 2010 –2011
An echocardiogram should be ob- least permanent method of birth Howard M. Saal, MD, Chairperson
tained if there is a history of in- control and be familiar with local Stephen R. Braddock, MD
creasing fatigue, shortness of Gregory M. Enns, MB, ChB
law and resources to assist the fam- Jeffrey R. Gruen, MD
breath, or exertional dyspnea or ab- ily in their decision-making regard- James M. Perrin, MD
normal physical examination find- ing questions about sterilization.86 Robert A. Saul, MD
ings, such as a new murmur or gallop. Beth Anne Tarini, MD
● Make recommendations and provide
Discuss skin, hair, and scalp care at FORMER COMMITTEE ON GENETICS
or refer for routine gynecologic care
each preventive health care visit. MEMBERS
if not already provided. Discuss pre-
Marilyn J. Bull, MD
menstrual behavioral problems and Joseph H. Hersh, MD
Anticipatory Guidance at Every
management of menses.92 Nancy J. Mendelsohn, MD
Health Maintenance Visit
● Discuss group homes and indepen- LIAISONS
● Discuss issues related to transition James W. Hanson, MD – American College of
dent living opportunities, workshop
into adulthood, including guardian- Medical Genetics
settings, and other community- Michele Ann Lloyd-Puryear, MD, PhD – Health
ship and long-term financial planning
supported employment. Resources and Services Administration
from early adolescence. Potential Thomas J. Musci, MD – American College of
adult morbidities including apparent ● Discuss intrafamily relationships, fi-
Obstetricians and Gynecologists
tendency toward premature aging nancial planning, and guardianship. Sonja Ann Rasmussen, MD, MS – Centers for
● Facilitate transition to adult medical Disease Control and Prevention
and increased risk of Alzheimer dis-
ease may also be discussed.87 care.93 CONTRIBUTOR
Stephen M. Downs, MD—Partnership for
● Monitor growth patterns, especially FUTURE CONSIDERATIONS Policy Implementation
BMI, and counsel regarding healthy diet Many issues related to the develop- STAFF
and a structured exercise program. ment and health of people with Down Paul Spire
● Discuss behavioral and social syndrome remain to be evaluated, and
RESOURCES FOR PARENTS
states and refer patients who have research agendas for addressing both
chronic behavioral problems or public health and basic science topics National Down Syndrome Society:
manifest acute deterioration in have been developed. Knowledge in www.ndss.org.
function for specialized evaluation several topics of great importance to National Down Syndrome Congress:
and intervention.88,89 the care of children with Down syn- www.ndsccenter.org.

402 FROM THE AMERICAN ACADEMY OF PEDIATRICS


FROM THE AMERICAN ACADEMY OF PEDIATRICS

Canadian Down Syndrome Society: Skallerup SJ. Babies With Down Syn- brookespublishing.com/store/books/
www.cdss.ca. drome: A New Parents Guide. 3rd ed. pueschel-4528/index.htm.
March of Dimes: www.marchofdimes. Bethesda, MD: Woodbine House; 2009 Simmons J. The Down Syndrome Transi-
(English and Spanish editions available at tion Handbook: Charting Your Child’s
com.
www.woodbinehouse.com.) Course to Adulthood. Bethesda, MD:
Down Syndrome International Educa-
Pueschel SM, ed. A Parent’s Guide to Woodbine House; 2010. Available at:
tion: www.downsed.org. Down Syndrome: Toward a Brighter www.woodbinehouse.com/main.asp_
Brighter Tomorrows-Supporting Fami- Future. Bethesda, MD: Brookes Pub- Q_product_id_E_978-1-890627-87-4_
lies: www.brightertomorrows.org. lishing; 2001. Available at: www. A_.asp.
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PEDIATRICS Volume 128, Number 2, August 2011 405


APPENDIX 1 Health Supervision for Children With Down Syndrome
Prenatal Birth–1 mo 1 mo–1 y 1–5 y 5–13 y 13–21 y
Counseling regarding prenatal screening test & imaging results
Plan for delivery
Referral to genecist
Parent-to-parent contact, support groups, current books and
pamphlets
Physical exam for evidence of trisomy 21
Chromosomal analysis to confirm dx
Discuss risk of recurrence of Down syndrome
Echocardiogram
Radiographic swallowing assessment if marked hypotonia, slow
feeding, choking with feeds, recurrent or persistent respiratory
sx, FTT
Eye exam for cataracts
Newborn hearing screen and follow-up
Hx and PE assessment for duodenal or anorectal atresia
Reassure parents delayed and irregular dental erupon,
hypodona are common
If conspaon, evaluate for limited diet or fluids, hypotonia,
Any visit
hypothyroidism, GI malformaon, Hirschsprung
CBC to R/O transient myeloproliferave disorder, polycythemia
Hb annually; CRP & ferrin or CHr if possible risk iron deficiency
Annually
or Hb <11 g.
Hemoglobin Annually
6 and 12
TSH (may be part of newborn screening) Annually
mo
Discuss risk of respiratory infecon
If cardiac surgery or hypotonic: evaluate apnea, bradycardia, or
oxygen desaturaon in car seat before discharge
Discuss complementary & alternave therapies All health maint. visits
Discuss cervical spine posioning, especially for anesthesia or
All health maint. visits
surgical or radiologic procedures
Review signs and symptoms of myopathy All health maint. visits
If myopathic signs or symptoms: obtain neutral posion spine
films and, if normal, obtain flexion & extension films & refer to
Any visit
pediatric neurosurgeon or orthopedic surgeon with experse in
evaluang and treang atlanto-axial instability
Instruct to contact physician for change in gait, change in use of
arms or hands, change in bowel or bladder funcon, neck pain, Biennially
head lt, torcollis, or new-onset weakness
Advise risk of some contact sports, trampolines All health maint. visits
Audiology evaluaon at 6 mo
If normal hearing established, behavioral audiogram and
Every 6
tympanometry unl bilateral ear specific tesng possible. Refer
mo
child with abnormal hearing to ot
If normal ear-specific hearing established, behavioral audiogram Annually
Assess for obstrucve sleep apnea Sx All health maint. visits
Sleep study by age 4 years
Ophthalmology referral to assess for strabismus, cataracts, and
nystagmus
Refer to pediatric ophthalmologist or ophthalmologist with
Annually Every 2 y Every 3 y
experience with Down syndrome
If congenital heart disease, monitor for signs & Sx of Congesve
All visits
heart failure
Assess the emoonal status of parents and intrafamilial
All health maint. visits
relaonships
Check for Sx of celiac disease; if Sx present, obtain ssue
All health maint. visits
transglutaminase IgA & quantave IgA
Early intervenon: physical, occupaonal, and speech therapy Health maint. visits
At 30 months, discuss transion to preschool and development
of IEP
Discuss behavioral and social progress Health maint. visits
Health
Discuss self-help skills, ADHD, OCD, wandering off, transion to
maint.
middle school
visits
If chronic cardiac or pulmonary disease, 23-valent pneumococcal
vaccine at age >2 y
Reassure regarding delayed and irregular dental erupon
Health
Establish opmal dietary and physical exercise paerns maint.
visits
Discuss dermatologic issues with parents
Discuss physical and psychosocial changes though puberty, need
for gynecologic care in the pubescent female
Facilitate transion: guardianship, financial planning, behavioral Health
problems, school placement, vocaonal training, independence maint.
with hygiene and self-care, group homes, work sengs visits
Health
Discuss sexual development and behaviors, contracepon,
maint.
sexually transmied diseases, recurrence risk for offspring
visits

Do once at this age


Do if not done previously
Repeat at indicated intervals

Maint. indicates maintenance; dx, diagnosis; sx, symptoms; FTT, failure to thrive; Hx, history; PE, physician examination; Gl, gastrointestinal; CBC, complete blood count; R/O, rule out; Hb,
hemoglobin; ot, occupational therapy; CHr, reticulocyte hemoglobin; IgA, immunoglobulin A; IEP, Individualized Education Plan; ADHD, attention-deficit/hyperactivity disorder; OCD, obsessive
compulsive disorder.

406 FROM THE AMERICAN ACADEMY OF PEDIATRICS


ERRATA Takata et al. Principles of Patient Safety: Reducing Harm Due to Medical Care.
Pediatrics. 2011;127(6):1199 –1210
An error occurred in the American Academy of Pediatrics policy statement “Prin-
ciples of Patient Safety: Reducing Harm Due to Medical Care” published in the
June 2011 issue of Pediatrics (2011;127[6]:1199-1210; doi:10.1542/2011-0967). Ref-
erence 82 (Takata GS et al, 2008) was incorrectly cited in the definition of Error on
page 1205. It should have been cited in the definition of Trigger tool on page 1206,
along with reference 79. We regret the error.
doi:10.1542/peds.2011-1758

Rabi et al. Room-Air Versus Oxygen Administration for Resuscitation of Preterm


Infants: The ROAR Study. Pediatrics. 2011;128(2):e374 – e381
An error occurred in this article by Rabi et al, titled “Room-Air Versus Oxygen
Administration for Resuscitation of Preterm Infants: The ROAR Study” published in
the August, 2011 issue of Pediatrics (2011; 128[2]: e374-e381; originally published
online July 11, 2011; doi: 10.1542/2010-3130). On page e379, in the second para-
graph under the heading Discussion, this reads: “In a recent study by Vento et al,
preterm infants resuscitated with 90% oxygen needed fewer days of mechanical
ventilation and oxygen supplementation compared with those resuscitated with
30% oxygen.” This should have read: “In a recent study by Vento et al, preterm
infants resuscitated with 30% oxygen needed fewer days of mechanical ventila-
tion and oxygen supplementation compared with those resuscitated with 90%
oxygen.”
doi:10.1542/peds.2011-2853

American Academy of Pediatrics. Health Supervision for Children with Down


Syndrome. Pediatrics. 2011;128(2):393– 406
An error occurred in the American Academy of Pediatrics clinical report “Health
Supervision for Children with Down Syndrome” published in the August 2011 issue
of Pediatrics (2011;128[2]:393-406; originally published online July 25, 2011;
doi10.1542/2011-1605). In Appendix 1 on page 406, the 24th row of the first column
should read “If myelopathic signs or symptoms:” rather than “If myopathic signs
or symptoms:”. We regret the error.
doi:10.1542/peds.2011-3113

1212 ERRATA