Pediatric emergency medicine trisk 554

baseline
8. Elevated
reticulocyte count
9. Elevated bilirubin
Aplastic anemia 1. Fatigue
2. Pallor
crisis
3. Tachycardia
4. Absence of
jaundice
5. Altered mental
status (severe
anemia)
6. Headache
7. Dyspnea
8. Fall in the
hematocrit from
baseline
9. Low reticulocyte
count
10. Absence of acute
hemolysis
Bacteremia/sepsis 1. Fever
2. Tachycardia
3. Hypotension
4. Lethargy

1. Usually self-limited,
Small
routine supportive care
transfusions

2. Transfusion of pRBCs
often sufficient
if severe symptoms
to support
(altered mental status,
oxygen
impaired oxygen
delivery while
delivery, significant
awaiting bone
tachycardia)
marrow
recovery
Often parvo B19
related, though
other infections
as well

1. Cultures of blood, and High risk for
if indicated, urine and
infection with
spinal fluid.
Streptococcus
2. Prompt antibiotic
pneumoniae,
administration (don’t
Haemophilus
delay if culture difficult
influenzae,
to obtain). ThirdSalmonella,

generation
Staphylococcus
cephalosporin, consider
aureus,
addition of
Escherichia
vancomycin. If allergy
coli
to cephalosporins,
Can rapidly
consider
deteriorate
vancomycin/gentamicin
in hospitalizated
patients; for wellappearing allergic
patients, consider
quinolones (e.g.,
levofloxacin), or
clindamycin
3. IV fluids


4. Simple or exchange
transfusion for sepsis,
acidosis, or hypoxia
5. Admission
(consideration of
outpatient management
in stable patients with
isolated fever)

Management/Diagnostic Testing
If the patient describes pain typical in location and quality to a vasoocclusive crisis,
begin fluids, provide analgesia, and observe the patient for improvement. Elevated
aspartate aminotransferase (AST) and hyperbilirubinemia may be present in infarction
as well as biliary disease. Abdominal ultrasound can assess the anatomy of the liver, gall
bladder, and biliary tree.
Cholelithiasis is the most common hepatic and biliary tract complication in children
with sickle cell disease, with an incidence of 12% in 2- to 5-year olds and approximately
40% by the age of 15 to 18 years. Patients can present with acute right upper quadrant
pain and tenderness, hyperbilirubinemia, and elevated liver enzyme levels. Typically,
surgery is delayed until acute inflammation has subsided to reduce the risk of
complications. The optimal treatment includes elective laparoscopic cholecystectomy
after adequate preparation for surgery (e.g., transfusions).
Rarely, acute intrahepatic sickling or viral hepatitis can result in a similar clinical
presentation with massive hyperbilirubinemia and elevated enzyme levels. Fulminant
hepatic failure with hepatic encephalopathy and shock can also occur as a rare, often
fatal, syndrome that may be amenable to exchange transfusion.

Priapism
Initial Assessment
Priapism is a painful vasoocclusive crisis causing a tender and engorged penis that may
persist for hours. Urination may be difficult.
Management/Diagnostic Testing
Treatment is similar to other painful crises with management directed at hydration and
analgesia. A number of systemic therapies can also be utilized. Pseudoephedrine can be
given orally, while terbutaline delivery is either oral or subcutaneous. Terbutaline has
been studied for the treatment of priapism in nonsickle cell disease patients and has
some demonstrated efficacy in the sickle cell disease group. Consider early consultation
with a pediatric hematologist and urologist.

Infection/Sepsis


Initial Assessment
Sickle cell patients have a higher risk of bacterial infection than the general population
due to functional asplenia, low serum immunoglobulin levels, and abnormal
opsonization and complement activation. The risk is highest for serious infections with
encapsulated bacteria such as Streptococcus pneumoniae, Haemophilus influenzae, and
Salmonella species, as well as S. aureus and E. coli. Sepsis, meningitis, pneumonia,
osteomyelitis, abscesses, and septic arthritis are all possible. The use of oral penicillin
prophylaxis until age 5 and vaccination for H. influenzae and S. pneumoniae have
decreased the rate of serious bacterial infection; however, infection remains a
meaningful concern in the setting of an acutely ill, febrile patient with sickle cell
anemia. In one large retrospective study at a tertiary care center, the bacteremia rate in
1,118 acutely febrile patients with sickle cell was 0.8%; the nine cases of bacteremia
included four cases of Salmonella, two S. pneumoniae, two E. coli, and one S. aureus.
Management/Diagnostic Testing
In the emergency department, a toxic-appearing febrile child with sickle cell disease
requires careful and immediate evaluation, monitoring, fluid resuscitation, and rapid
administration of antibiotics. A pathway for the management of sickle cell disease with
fever is available at . Obtain cultures of blood, and, if indicated, urine and spinal
fluid. Include a type and cross. Do not delay antibiotic administration due to difficulty
obtaining labs or performing procedures. In regions with endemic resistant S.
pneumoniae strains, use vancomycin in addition to a third-generation cephalosporin.
Consider broader-spectrum antibiotics as indicated based on concern for abdominal
process or for severe presentations. As in other patients with reduced or absent splenic
function, clinical deterioration may be extremely rapid. The patient who arrives alert to
the ED may become moribund and hypotensive within 30 minutes. In the ill patient with
sepsis, acidosis, or hypoxia, provide simple or exchange transfusions to decrease
massive sickling, which can cause secondary organ damage. Mortality from bacteremia

may approach 20% to 30%.
Have a high level of suspicion for meningitis in the young, irritable child with sickle
cell disease and unexplained fever. Perform a lumbar puncture on toxic children and
anyone with signs or symptoms of meningitis. Use the same antibiotic therapy for
meningitis as recommended for children with meningitis with hematologic disease.
Simple or exchange transfusion to lower the percentage of sickle hemoglobin may
reduce the risk of intracerebral sickling in areas of brain swelling that can lead to
infarction. When hemoglobin S is less than 30% of the total hemoglobin, sickling is
unlikely, and decisions regarding fluid management can be directed by the central
nervous system findings as opposed to the need to ameliorate sickling.
The nontoxic, febrile child (temperature >38.5°C) with sickle cell disease requires a
thorough history and physical examination. Detailed history should focus on length,


duration, and height of fever, and the presence of associated symptoms such as cough,
respiratory distress, and abdominal pain. Elicit immunization status and compliance
with prophylactic antibiotics. Without a clear source of infection, laboratory assessment
should include a CBC with a reticulocyte count, blood culture, and a type and screen.
Other evaluations may include a chest x-ray, urinalysis with culture, or cerebrospinal
fluid analysis as clinically indicated. A type and cross should be sent if history suggests
splenic sequestration, acute chest syndrome, or significant neurologic symptoms.
Administer antibiotics promptly, prior to knowledge of laboratory results. Typically,
administer ceftriaxone (50 mg/kg up to 2,000 mg) for broad-spectrum antibiotic
coverage lasting 24 hours while cultures are pending; outpatient management is
appropriate in stable patients in whom there are no early concerns for developing
complications and who have reliable caretakers with the means to return if necessary.

Acute Chest Syndrome
Initial Assessment
Acute chest syndrome is a life-threatening complication of sickle cell disease indicative

of pneumonia, pulmonary infarction, or both. Lifetime risk of acute chest syndrome is
48% and causes 25% of sickle cell disease–related deaths. The patient usually presents
with fever, tachypnea, chest pain, rales, and hypoxia. In a dehydrated patient, the initial
physical examination findings may be minimal.
Management/Diagnostic Testing
Any sickle cell patient who presents with chest pain requires immediate assessment,
monitoring, and treatment including IV access, fluids, oxygen administration, and
laboratory testing including CBC with reticulocyte count, type and screen, blood
culture, and chest x-ray (see Table 93.6 ). Any acute infiltrate should be concerning for
acute chest syndrome and the patient should be treated with antibiotics including a thirdgeneration cephalosporin such as ceftriaxone and atypical coverage with a macrolide.
Add vancomycin in the severely ill patient. All patients with suspected acute chest
syndrome require admission for further management. Closely monitor patients with
chest pain without radiographic findings or hypoxia and encourage incentive spirometry,
as splinting puts them at risk for the development of acute chest syndrome which may
not be present on presentation. Consider simple transfusion in the presence of severe
anemia (hemoglobin <6 to 7 g/dL) and simple or exchange transfusion for hypoxia and
in patients with radiologic or clinical evidence of severe or rapidly progressive disease.
The distinction between pneumonia and an acute chest/vasoocclusive crisis can be
difficult, making aggressive management prudent. Euvolemia is the goal of fluid
resuscitation in acute chest, as pulmonary edema can worsen respiratory distress and
hypovolemia can increase sickling. Corticosteroids are not routinely used in the
management of acute chest syndrome. Patients with comorbid asthma presenting with