Pediatric emergency medicine trisk 604

Possible
Unlikely

Serologic criterion one or two
with ≤1 minor criterion
Maternal history of adequate
treatment during pregnancy
(penicillin-based regimen) +
nonreactive serologic test
for syphilis

XR, radiographs; CNS, central nervous system; CSF, cerebrospinal fluid; RPR, rapid plasma reagin; VDRL,
venereal disease research laboratory; FTA-ABS, fluorescent treponemal antibody absorption; TP-PA,
Treponema pallidum particle agglutination; MHA-TP, microhemagglutination test for antibodies to
Treponema pallidum.
Modified from Mascola L, Pelosi R, Blount JH, et al. Congenital syphilis revisited. Am J Dis Child
1985;139:575–580.

e-TABLE 94.32
TREATMENT OF SYPHILIS
Stage
Primary, secondary, or early latent
(infection within the last 12 mo)

Treatment

Penicillin G benzathine 50,000
units/kg (maximum: 2.4 million
units) IM once
Late latent (>1 yr since acquisition),
Penicillin G benzathine 50,000

latent syphilis of unknown duration,
units/kg (maximum: 2.4 million
or tertiary syphilis
units) IM weekly for 3 wks
Congenital syphilis or neurosyphilis
Aqueous penicillin G 50,000
units/kg/dose IV every 12 hrs in 0–7
do, then every 8 hrs in infants > 7
days (maximum daily dose: 24
million units/day) for 10 days
Alternative regimen for adults:
Procaine penicillin G 24 million
units IM daily with probenecid 500
mg four times daily for 10–14 days
IM, intramuscular; IV, intravenous.


e-TABLE 94.33
STAGES OF SYPHILIS


Stage

Symptoms

Time frame

Primary

Chancre: nontender, firm red lesion,

often with associated nontender
regional adenopathy. As often
found in the cervix or vagina,
may not be noted by many
women
Due to disseminated spirochetemia.
Fever, influenza-like illness,
generalized adenopathy, and
dermal finding predominate in
adults but are less prominent in
young children. These include
condylomata lata and lesions on
palms and soles (contact
precautions should be used).
Reactive serologic tests without
symptoms

10–90 days after
exposure

Secondary

Latent

2–10 wks after primary
disease

Early latent: <1 yr
Late latent: >1 yr
Tertiary

Gummas: indolent localized dermal 3–10 yrs after secondary
nodules caused by the host
disease
inflammatory reaction; in
contrast to secondary syphilis,
few spirochetes are found
Neurosyphilis Paralytic dementia, meningitis,
≥5 yrs after primary
seizures, gummas of the spinal
disease
cord, optic atrophy, or tabes
dorsalis (myelopathy of the
posterior columns of the spinal
cord, resulting in loss of
proprioception and vibration
sensation)
Cardiovascular Arteritis of the aorta and pulmonary 10–40 yrs after primary
vessels that can result in valvular
disease
regurgitation or myocardial
insufficiency


CHAPTER 95 ■ METABOLIC EMERGENCIES
BRET L. BOSTWICK, JUSTIN R. DAVIS

GOALS OF EMERGENCY THERAPY
Recognition and understanding of inborn errors of metabolism (IEMs) in the acutely ill child in the
emergency department (ED) is critical for appropriate, and possibly lifesaving management. Individually,
metabolic diseases are rare, but collectively are common with the incidence approaching 1 in every 800

to 2,500 newborns. Goals of emergency care are to consider the possibility of IEM in the differential
diagnosis of acutely ill, previously undiagnosed patients, and to identify, treat, and prevent acute
metabolic derangements in patients with suspected or known IEMs, including the asymptomatic neonate
with a positive newborn screening (NBS).
KEY POINTS
IEMs usually manifest in the neonatal period or infancy but can present at any age, even
during adulthood.
Newborn screening (NBS) results may not be available in the first days to weeks of life: false
negatives and false positives occur.
ED care does not require an extensive knowledge of individual metabolic diseases or
biochemical pathways, but rather an understanding of the pathophysiology of categories of
IEMs.
High index of suspicion is the most important element in making the diagnosis of metabolic
disease. Initial laboratory evaluation should include CBC, blood gas, glucose, ammonia,
chemistries, uric acid, liver function studies, and urinalysis.
Successful emergency treatment of suspected and known IEMs depends on prompt
institution of therapy to correct and prevent further metabolic derangement and is critical to
prevent acute and long-term morbidity and mortality.
Specialists with expertise in inborn errors of metabolism should be consulted to guide
diagnosis and management, and consideration should be given to consultation even if this
requires referral outside of your facility.

UNKNOWN SUSPECTED IEM
Goals of Treatment
Recognizing the possibility of an IEM is critical for optimal management of the child with unknown
IEM. Immediate goals of treatment are to stabilize cardiopulmonary function, correct metabolic
derangements, and avoid intake and/or endogenous production of potentially toxic substances. Early
consultation with an IEM specialist, including prior to transport of a child being transported from an
outside facility, is advised to guide treatment and collection of appropriate specimens for diagnosis.
CLINICAL PEARLS AND PITFALLS