BABY BLUES
| MY BABY TURNED BLUE !Raymond D. Pitetti, MD, MPH, Assistant Professor of Pediatrics, University
of Pittsburgh School of Medicine, and Childrens Hospital of Pittsburgh, PA
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| Apnea: defined as any episode of not breathing >15 to 20 sec, or any episode associated with changes in color
or heart rate; parents sometimes worry about normal breathing patterns
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 | Periodic breathing: periods of rapid, shallow breathing while infant sleeps, lasting 20 to 30 sec, followed by 2- to 5-
sec pause, after which child resumes normal breathing; thought to be immature breathing pattern reflecting development
of breathing centers in brain; not worrisome unless accompanied by changes in color or heart rate
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 | Apnea of prematurity: expected in infants born at <35 wk gestation; occurs until 2 to 3 mo of age; characterized
by periods of apnea lasting 15 to 20 sec, often associated with changes in heart rate to <80 bpm (occasionally
associated with color changes); in hospital, often treated with caffeine
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| Apparent life-threatening events (ALTEs): fit or spell in which child appears to choke or turn blue, but recovers
and appears well by time of arrival at emergency department (ED); occur in ≈5% of children; frightening
to parent, who thinks child about to die, and frustrating to physician, who must decide whether to
send child home or admit for tests (no good guidelines); prognosis also unclear
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 | Definition: developed in 1987 at National Institutes of Health (NIH); consists of some combination of apnea,
color change, possible changes in mental status, muscle tone, choking or gagging, and caregivers fear that
child may die; many cases benign and never recur; challenge to identify children at high risk for serious
consequences; ALTE itself not diagnosis but description (many underlying causes possible)
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| Possible causes of ALTEs: ≈40% idiopathic; reflux; arrhythmia; infection; inborn errors of metabolism; child
abuse; seizures
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 | Reflux: now known that apnea often occurs first, followed by relaxation of lower esophageal sphincter and reflux;
should raise suspicion that something else caused apnea; however, in some cases, reflux does lead to apnea,
due either to laryngospasm or true obstruction
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 | Cardiac disease: could be congenital heart problem or arrhythmia (suspect prolonged QTc interval); look for
other signs of autonomic dysfunction, including increased QTc dispersion and altered heart rate variability
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 | Child abuse: look for other clues, eg, retinal hemorrhages, subdural hematoma, or familial patterns associated
with risk for abuse; important to identify as early as possible because abuse escalates with time; probably
cause of ALTEs most likely to lead to morbidity or mortality; in speakers experience, types of abuse include
suffocation, Munchausens syndrome by proxy, and poisoning
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| Evaluation: obtain thorough history, including nature of changes in color, muscle tone, or mental status; information
to gatherwhat happened just before ALTE; whether it has occurred before or in siblings; associated
symptoms; feeding history; family history, including heart disease or prolonged QTc; social history
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 | Physical examination: should include complete neurologic examination, including dilated fundoscopic examination
and Woods lamp evaluation of body (may detect bruises invisible to naked eye or conditions such
as tuberous sclerosis)
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 | Laboratory studies: at Pittsburgh Childrens Hospital, children with ALTEs undergo battery of tests and followed
for 6 to 12 mo as part of prospective study to determine outcomes and identify most helpful tests;
also trying to identify combination of factors associated with high risk for repeat events; ≈60% of these patients
have carboxyhemoglobin of 3% to 4% (normal 0.65%-1%; exact significance unknown); urine toxicology
reveals over-the-counter medication in ≈20% of patients (inappropriate for babies this young; may
contribute to event); electrocardiography (ECG) also may be informative; radiographic studiesinclude
chest x-rays; computed tomography (CT) or magnetic resonance imaging (MRI) of head performed only on
follow-up, if initial evaluation reveals something questionable, eg, retinal hemorrhage; ancillary studies
might include electroencephalography (EEG) to look for evidence of seizures; upper gastrointestinal series
or pH probe if child has strong history of reflux, or if ALTE occurred within 15 to 20 min of feeding
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| Prognosis: 30% to 40% of children have recurrent episodes, most likely from underlying condition; episode
severity probably will not escalate, unless related to child abuse; literature states that 5% to 10% of patients
may have learning disabilities later in childhood (not confirmed); mortality estimates range from 0% to 13%
(probably closer to 1%-2%, but depends on condition underlying event, eg, arrhythmia or seizure)
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| Management: standard of care to admit all children to assuage parental anxiety, learn more about episode, and
observe interaction between child and parents; home monitors often not used because they cannot prevent or
stop event and may intensify parents frustration; close follow-up by pediatrician recommended
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| Sudden infant death syndrome (SIDS): may share similar pathophysiology with ALTE; defined as sudden
death of infant <1 yr of age that remains unexplained after thorough case investigation, including performance
of complete autopsy, examination of death scene, and review of clinical history; all 3 components
required for diagnosis, so diagnosis in ED not possible
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 | Triple-risk model: current theory holds that babies have underlying genetic defect, brainstem abnormality, or
cardiac dysfunction set in motion by triggering event (eg, maternal smoking, infection) at critical time in
development of immune or central nervous system
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 | Brainstem abnormality: abnormal development of neural network responsible for arousal; results in deficit of
serotonin receptors in ventral medulla, which affects responses to life-threatening challenges during sleep;
serotonin receptors drive sodium-driven pacemakers that govern gasp reflex; when serotonin receptors deficient,
infant unable to gasp and auto-resuscitate; found in animal models and some cases of SIDS; similar
findings in some studies of ALTE
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 | Epidemiology: SIDS third leading cause of infant mortality in United States; leading cause of death among
infants <1 yr of age; mortality decreased significantly starting in 1994 with start of Back to Sleep campaign
promoted by American Academy of Pediatrics, but leveled off around 2000; death rate had started
drifting downward before 1994 as clinicians increased ability to find accurate diagnosis for deaths formerly
labeled SIDS; incidence peaks at 2 to 3 mo of age (rare during first month of life); seasonal variation
(higher in winter) no longer exists, as infectious illnesses formerly labeled as SIDS identified
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 | Risk factors: prone sleeping position; soft sleeping surface (increases risk by ≈5 times, independent of sleeping
position); maternal smoking during pregnancy (increases risk by factor of 3); preterm birth; low birth weight;
male sex; overheating; late or no prenatal care; young maternal age; race (risk 3 times greater among black,
American Indian, and Alaskan native babies than among whites); bed sharing (controversial; may increase
risk of smothering and therefore SIDS); presence of secondary caregiver, eg, babysitter, day care staff
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 | Management: get complete history; perform thorough physical examination; complete autopsy recommended;
visit scene of death whenever possible for clues to what transpired
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 | Prevention: put child to sleep on back; prone and side sleeping not recommended until child older than 2 to 3
mo; use firm sleep surface; keep pillows and other soft objects out of crib; do not smoke around child;
maintain separate but proximate sleep environment to encourage bonding but avoid risks of bed sharing
(shown to reduce rate of SIDS); avoid overheating; protective effect of breast-feeding not established; some
research suggests that pacifiers reduce SIDS risk, although they may increase risk for otitis media
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| Breath holding: noiseless state of expiration associated with color changes, loss of consciousness (sometimes),
and loss of postural tone; often occurs when child angry; can occur in children as young as 1 to 2 mo of age
(might actually be ALTEs), while ALTEs reported in children as old as 12 to 24 mo (might actually be
breath-holding spells); cyanotic spellschild turns blue; related to excessive sympathetic activity; accounts
for ≈50% of spells; pallid spellschild turns pale; related to excessive parasympathetic activity (25% of
spells); mixed spellscombines features of cyanotic and pallid spells (25% of cases)
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 | Aspects of breath holding: runs in families; autosomal dominant trait; occurs in ≈5% of well children, with
onset during first year of life and continuing to ≈6 yr of age; frequency varies widely, ranging from yearly
to multiple times daily; often occurs when child angry, but involuntary and nonvolitional (reflex); current
evidence points to autonomic dysregulation as common mediating pathway; association with iron deficiency
anemia found (iron supplements associated with reduced frequency of events, even in absence of
frank anemia; similar relationship between iron deficiency and risk for ALTEs reported)
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| ECTOPIC PREGNANCY Elliot S. Nipomnick, MD, Regional Medical Director, Emergency Physicians
Medical Group, and Staff Physician, Chinese Hospital, San Francisco, CA
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| Incidence: 20 per 1000 known pregnancies; mortality rate 1 per 1000; ectopic pregnancies responsible for 10% of
maternal deaths; most occur in women 25 to 34 yr of age, but risk for death increased 2.5 times among women 35
to 39 yr of age, and 6 times among women >40 yr of age
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 | Heterotopic pregnancy: one pregnancy in endometrial cavity and another elsewhere; overall, occurs in 1 of
every 3000 pregnancies, but some fertility clinics report it in 1 of every 100 pregnancies (ask all patients
about history of fertility treatments)
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| Natural history of pregnancy: implantation occurs 14 days before first day of missed period; normally,
quantitative human chorionic gonadotropin (hCG) levels double every second day well into second trimester;
hCG levels rise more slowly when blood supply inadequate or pregnancy abnormal, making them harder to
correlate with age, so ectopic pregnancy cannot be diagnosed by having patient return for another measurement
24 to 48 hr later; yolk sac usually present on ultrasonography by 4.5 to 5.5 wk; heartbeat present by 6 to
6.5 wk; ectopic pregnancy may resorb or bleed, but rarely develops into advanced pregnancy; if ectopic pregnancy
suspected, look for lack of intrauterine pregnancy on endovaginal or transabdominal examination
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| Frequency of claims related to failure to diagnose ectopic pregnancy: 1% of total claims, but cost accounts
for 2% of total claims because incidence increasing, perhaps due to infertility treatments
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 | Reasons physicians may not diagnose ectopic pregnancy: no typical presentation; 30% to 40% of patients examined
by another physician before presenting to ED; mistaken reliance on normal menstrual history (order
pregnancy test for any female patient between 10 and 60 yr of age who presents with abdominal pain, back
pain, or any medical condition requiring medication inappropriate for pregnant woman); mistaken reliance on
lack of adnexal mass on examination or ultrasonography; failure to measure hCG levels; delayed transvaginal
pelvic ultrasonography; discharge before ectopic pregnancy fully excluded or consultation obtained; inadequate
follow-up; if pregnancy suspected despite negative urinary hCG, order blood test
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| Patient evaluation: should include complete obstetric and gynecologic history, including last normal menstrual
period, any abnormal vaginal bleeding, history of sexual activity, birth control, use of fertility drugs,
any previous ectopic pregnancies; location, duration, and quality of pain; associated symptoms, including
urinary symptoms, syncopal episodes, or lightheadedness; risk factors include previous use of intrauterine
device, pelvic inflammatory disease, infertility, endometriosis, and tubal ligation
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 | Examination: take vital signs, including orthostatic changes and unusual tachycardia; presence or absence of abdominal
tenderness; cervical motion tenderness may result from peritoneal irritation due to bleeding; address
presence and quantity of vaginal bleeding; look for adnexal mass or tenderness and uterine enlargement; if tissue
at os, take sample for analysis (presence of fetal or gestational tissue makes diagnosis)
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 | Tests: quantitative and qualitative hCG; transvaginal or transabdominal ultrasonography; Rh factor mandatory;
urinalysis also important, as is complete blood count to test for anemia
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Suggested Reading
Bharadwaj P, Erskine K: Heterotopic pregnancy: still a diagnostic dilemma. J Obstet Gynaecol 25:720, 2005;
Blaivas M, Lyon M: Reliability of adnexal mass mobility in distinguishing possible ectopic pregnancy from
corpus luteum cysts. J Ultrasound Med 24:599, 2005; Cote A: Home and hospital monitoring for ALTE. Paediatr
Respir Rev 7 Suppl 1:S199, 2006; Cresi F et al: Relationship between gastro-oesophageal reflux and gastric
activity in newborns assessed by combined intraluminal impedance, pH metry and epigastric impedance.
Neurogastroenterol Motil 18:361, 2006; Dammann O: Paediatric neurology: the many faces of development.
Lancet Neurol 6:12, 2007; Hall KL, Zalman B: Evaluation and management of apparent life-threatening
events in children. Am Fam Physician 71:2301, 2005; Kiechl-Kohlendorfer U et al: Epidemiology of apparent
life-threatening events. Arch Dis Child 90:297, 2005; Mousa H et al: Testing the association between gastroesophageal
reflux and apnea in infants. J Pediatr Gastroenterol Nutr 41:169, 2005; Piccirillo B: Rapid
diagnosis of ectopic pregnancy using emergency bedside ultrasonography. JAAPA 20:29, 2007; Pitetti RD et
al: Prevalence of retinal hemorrhages and child abuse in children who present with an apparent life-threatening
event. Pediatrics 110:557, 2002; Pitetti RD, et al: Prevalence of anemia in children presenting with apparent
life-threatening events. Acad Emerg Med 12:926, 2005; Saririan S, Hauck FR: New recommendations to reduce
the risk of SIDS: what should we advise parents? Am Fam Physician 74:1839, 2006; Schnitzer PG: Prevention
of unintentional childhood injuries. Am Fam Physician 74:1864, 2006; Silva C et al: Human chorionic
gonadotropin profile for women with ectopic pregnancy. Obstet Gynecol 107:605, 2006; Waseem M, Pinkert
H: Apparent life-threatening event or child abuse? Pediatr Emerg Care 22:245, 2006.
Educational Objectives
| The goal of this program is to review the causes and presentation of common apneic conditions in infants, and
manifestations of ectopic pregnancy. After hearing and assimilating this program, the listener will be better able
to:
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 | 1. Determine when an infant has experienced an apparent life-threatening event (ALTE).
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 | 2. Recognize signs of child abuse associated with an ALTE.
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 | 3. Name the risk factors for sudden infant death syndrome (SIDS).
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 | 4. List measures that may lower the risk for SIDS.
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 | 5. Recognize the signs and symptoms of ectopic pregnancy.
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Faculty Disclosure
In adherence to ACCME guidelines, the Audio-Digest Foundation requests all lecturers to disclose any significant
financial relationship with the manufacturer or provider of any commercial product or service discussed.
For this issue, the faculty reported nothing to disclose.
Acknowledgements
Dr. Pitetti spoke at the 2006 Annual Scientific Assembly, held April 25-28, 2006, in Pittsburgh, PA, and sponsored
by the Pennsylvania Chapter of the American College of Emergency Physicians. Dr. Nipomnick was recorded
at High Risk Emergency Medicine, held May 23-24, 2006, in Las Vegas, NV, and sponsored by the Center
for Emergency Medical Education, The Emergency Physicians Medical Group, Emergency Medicine Physicians,
and the Ohio Chapter of the American College of Emergency Physicians. The Audio-Digest Foundation
thanks the speakers and the sponsors for their cooperation in the production of this program.
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