Fawn Mumbulo
2013
670 Intermediate Family Level
Instructor: Melissa Muha, FNP
Encounter was 8/26/2013 @ Child Adolescent
Healthcare Associations
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Accompanied &
encounter is reported
by mother
CC: Fever for two days,
swollen cervical nodes
for one day.
HPI: Recurrent fever
with lymphadenopathy
and rash. Irritable &
restless.
Vital signs:
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Temp 39.5 degrees; HR
84 bpm; RR 84/42 mmHg;
Ht 45.75 in.; Wt 39.6
lbs.; BMI 13.3; visual
acuity 20/20 bilaterally
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ROS:
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General- Mother reports no fatigue, or wt. loss,
positive fever, no night sweats, see HPI
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EENT- Mother reports no reduced visual acuity,
strabismus, diplopia, blurred vision,
photophobia, eye redness, itching, discharge, or
pain in eyes. Mother reports no ear discharge,
pain, or hearing loss bilaterally. Reports no
nasal congestion, discharge, postnasal drip,
sneezing, epistaxis, or hoarseness. Reports pos.
for sore throat.
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Respiratory- Reports no dyspnea, cough,
wheezing, or pleuritic pain.
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Cardiovascular- Reports no chest pain, nocturnal
dyspnea, or edema.
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Gastrointestinal- Reports no loss of appetite,
dysphagia, abdominal pain, nausea, vomiting,
change in bowel habits, diarrhea, constipation,
or blood in stool.
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Lymphatic- Reports positive swollen lymphnodes
with tenderness bilateral neck.
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Skin- Mother denies skin rash, lesions, dry skin,
or pruritus.
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Allergic/immunologic- Reports no urticaria,
wound healing not impaired.
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Social History
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Lives with both parents, 5 yr old
brother, 1 dog, 1 cat, fish
Is in the 2nd grade
Rides bike
ties shoe laces
reading at grade level
math at grade level
has no issues with school or friends at
this time
Anticipatory guidance
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Balanced diet
Positive fluoride use
Dental care, brushes teeth daily
Set appropriate bed time
Encourage reading
Regular physical activity
Uses seat belts, wares helmets,
bicycle safety, uses sunscreen
Avoids tobacco & smoke exposure
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Family History is insignificant
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Health risks are insignificant
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8/26/13 Upper respiratory
infection; wax in ear
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5/4/13 Throat pain
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2/10/13 Acute Tonsillitis
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12/5/12 Fever, acute
tonsillitis
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2/10/12 Gastroesophageal
reflux; acute tonsillitis
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Medications- none at this
time
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Allergy to Penicillin's (Hives)
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Signs & Symptoms
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Periodic fever (>39 degrees
Celsius), lasting 3-6 days, recurs
every 3-4 wks
Aphthous ulcer, sm. lesions
which are often asymptomatic
Pharyngitis
Cervical lymphadenopathy
Child is well between monthly
exacerbations
Non-contagious inflammatory
process
Malaise
Headache
Arthralgia’s
Abdominal pain & vomiting
hepatosplenomegaly
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Epidemiology
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1st episode in children under 5
years (can occur in ages 3mo.-12
yrs)
Most common recurrent fever in
children
Most children have resolution of
syndrome in the 2nd decade of
life
Differential Diagnosis
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Recurrent fever
Aphthous Ulcer
Pharyngitis
Cyclic neutropenia
Behcet’s disease
Juvenile idiopathic arthritis
Familial Mediterranean fever
Familial Hibernian fever
Hyperglobulinemia D syndrome
(Berlucchi & Nicolai, 2004; fpnotebook.com, 2013; Galanakis, Papadakis, Giannoussi, Karatzanis, Bitsori, & Helidonis, 2002)
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Constitutional: Appear well, alert, cooperative
EENT: Negative findings for abnormality with ocular motility, conjunctiva,
eyelids, sclera, pupil size/shape. Visual acuity 20/20 bilaterally.
Negative findings for abnormality in external ear, ear canal, tympanic
membrane, conversational hearing. External nose, nasal mucosa, & oral
mucosa wet, dry, pink. Dentition with no decaying seen. Tonsils +3
bilaterally with errythemia, negative for Aphthous ulcers.
Neck: symmetrical, no thyromegaly. Positive for tonsilar
lymphadenopathy bilaterally, tenderness upon palpation. ROM within
limits, no tenderness.
Respiratory: Chest movement is symmetrical, lungs clear bilaterally. No
axillary lymphadenopathy.
Cardiac: Heart rate 84 bpm, no deviations with rhythm, no heart
murmurs.
Gastrointestinal: Bowel sounds heard in all four quadrants, abdomen
soft, no hepatosplenomegaly, no abdominal tenderness, no masses or
hernias.
Genitalia: Tanner stage 1, Breasts Tanner stage 1.
Musculoskeletal: Gait with no deviations, no muscle weakness in
extremities X 4. Full ROM in all extremities.
 Labs:
Rapid strep negative, culture sent.
 There are no laboratory tests specific to
diagnose. Diagnosis is based on symptoms,
history, & physical exam. WBC, ERCP, & CRP
will show elevation due to inflammatory
response.
 Exclusions: Streptococcus & disease’s that
have recurrent FUO.
 Diagnosis can be confirmed after throat
culture results. If negative then diagnosis
would be consistent with A.K. history.
(American College of Rheumatology, 2013)
Named Marshall’s syndrome in 1987 & given the
acronym PFAPA (periodic fever, aphthous
stomatits, pharyngitis, cervical adenitis).
 In 1989, diagnostic criteria was proposed which
include:
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Regularly recurring fevers early onset <5yrs age
Symptoms in the absence of URI with at least one
clinical signs of aphthous stomatitis, cervical
lymphadenitis, or pharyngitis.
Exclusion of cyclic neutropenia
With complete asymptomatic in-between episodes
Normal growth & development
If suspected refer to infectious disease,
rheumatology, or immunology for definitive diagnosis.
(Berlucchi & Nicolai, 2004; Tasher, Somekh, & Dalal, 2006)
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Aim of treatment is to control
the symptoms during episodes of
fever & to shorten the duration.
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Fever does not respond well with Tylenol
or NSAIDS, antibiotics have no affect.
A single dose of prednisone (2 mg/kg)
given when the symptoms 1st begin as
shown to shorten or end the episode
(although studies have shown that this
can cause the next cyclic fever to
present earlier). If the fever disappears
within the first or second dose of steroid
then it can be used to confirm diagnosis
of PFAPA.
Cimetidine & Colchicine used for
prophylaxis may prevent future episodes
in 1/3 of children.
Tonsillectomy is confirmed as an
effective treatment, although studies
show that most children stop having
PFAPA episodes in their 2nd decade of
life.
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Studies have shown with
tonsillectomy’s in children diagnosed
with PFAPA will have no recurrence
after 10 months
It is not uncommon for children who are
diagnosed with PFAPA to have been
diagnosed multiple times for tonsillitis.
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Education
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Encourage parents that PFAPA
syndrome resolves spontaneously
after several years in about 40% of
children.
Multiple days of school will be
missed which can stigmatize the
child to social issues. Allow child
to socialize as much as possible.
Missed school days and missed
work days for parents can put
strain on the family dynamics.
Consider counseling if this evolves.
Prednisone can cause transient
hyperactivity in children.
PFAPA is not contagious or
infectious.
(American College of Rheumatology, 2013; Berlucchi & Nicolai, 2004; Galanakis et., al. 2002; fpnotebook.com, 2013;
National Institutes of Health, 2011; Stojanov et., al. 2011; Tasher, Somekh, & Dalal, 2006)
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American College of Rheumatology, (2013). Periodic fever, aphthous stomatitis, pharyngitis,
adenitis syndrome (PFAPA) (juvenile). Retrieved from website
http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Conditions/PFAP
A
Berlucchi, M., & Nicolai, P. (2004). Marshall’s syndrome or PFAPA (periodic fever, aphthous
stomatitis, pharyngitis, cervical adenitis) syndrome. Retrieved from website
http://www.orpha.net/data/patho/GB/uk-PFAPA.pdf
fpnotebook.com, (2013). PFAPA syndrome. Retrieved from website
http://www.fpnotebook.com/id/Peds/PfpSyndm.htm
Feder, H. M., & Salazar, J. C. (2010). A clinical review of 105 patients with PFAPA (a periodic fever
syndrome). Acta Paediatrica, 99, 178-184. doi: 10.1111/j.1651-2227.2009.01554.x
Galanakis, C. E., Papadakis, C. E., Giannoussi, E., Karatzanis, A. D., Bitsori, M., & Helidonis, E. S.
(2002). PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child, 86, 434435.
National Institutes of Health, (2011). Scientists identify new treatment to combat PFAPA
syndrome in children. Retrieved from website http://www.newsmedical.net/news/20110411/Scientists-identify-new-treatment-to-combat-PFAPAsyndrome-in-children.aspx
Stojanov, S., Lapidus, S., Chitkara, P., Feder, H., Salazar, J. C., Fleisher, T. A., Brown, M. R>,
Edwards, K. M., Ward, M. M., Colbert, R. A., Sun, H., Wood, G. M., Barham, B. K., Jones,
A., Aksentijevich, I., Goldbach-Mansky, R., Athreya, B., Barron, K. S., & Kastner, D. L.
(2011). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a
disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Pnas,
108(17), 7148-7153. doi: 10.1073/pnas.1103681108.
Tasher, D., Somekh, E., & Dalal, I. (2006). PFAPA syndrome: New clinical aspects disclosed. Arch
Dis Child, 91, 981-984. doi: 10/1136/adc.2005.084731.