Abdominal Pain - Case 7-7: 3-Year-Old Girl
I. History of Present Illness
A 3-year-old girl of Sri Lankan descent who was born in the United States
presented to the emergency department with a fever of 40.5
°C for the past 2 days. She complained of abdominal, knee, and elbow pain while
febrile, but she was eating without a change in appetite and was having no
problems ambulating. She had no complaint of headache, sore throat, rhinorrhea,
cough, diarrhea, or vomiting. Her symptoms had been a recurrent problem for 8
months, since the family took a trip to one of the national parks. After
returning from that trip, she had her first episode of fever, with a
temperature to 40
°C, which lasted 5 days. At that time she complained of a frontal headache,
chills, rigors, and abdominal pain. She saw her regular pediatrician, who
diagnosed a viral syndrome. At presentation, her mother reported that the
episodes occurred at the end of every month. During her second febrile episode,
the mother was told that the child
's headache and abdominal pain were secondary to sinusitis with postnasal drip,
and she was treated for clinical sinusitis with a 3-week course of antibiotics.
She continued to have identical episodes every month. She had been treated a
total of three times for acute sinusitis based on clinical diagnosis. The
mother reported no weight loss, rash, upper respiratory tract symptoms, cough,
or joint swelling with these episodes. She has not traveled outside of the
country or had any known tick bites. She was seen by her regular doctor on
multiple occasions during the episodes, with normal examinations, multiple
normal CBCs, and normal Lyme serology. She had had no tuberculosis exposure.
She had had normal growth and development and was completely normal between
episodes. Her mother stated that the doctor often told her that her daughter
's throat was “a little red,” but all throat cultures had been negative.
II. Past Medical History
The patient had a normal birth history. She had had no hospitalizations or
surgeries and had a diagnosis of allergic rhinitis. Her only medications were
acetaminophen and ibuprofen during the febrile episodes. She was appropriately
immunized and had had a negative tuberculin skin test within the last 3 months.
Her family history was negative for GI disease, autoimmune disease, childhood
illnesses, arthritis, cancer, and tuberculosis. She attended day care, and the
family had no pets.
III. Physical Examination
T, 39.9°C; RR, 28/min; HR, 112 bpm; BP, 106/73 mm Hg
Weight, 19.8 kg (95th percentile); height, 104 cm (90th percentile)
In general, the patient was talkative, pleasant, and in no acute distress. Her
sclerae were anicteric, there was no conjunctival injection, her tympanic
membranes were normal, the oropharynx was nonerythematous, and there were no
mouth lesions. Neck examination revealed shotty cervical lymphadenopathy. Chest
and heart examination were normal. Abdominal examination revealed normoactive
bowel sounds, diffuse tenderness without peritoneal signs, and no organomegaly.
Neurologic examination was intact, and the skin showed no rashes or
discoloration.
IV. Diagnostic Studies
A CBC showed 15,800 WBCs/mm3, with 64% segmented neutrophils, 22% lymphocytes, and 13% monocytes. The
hemoglobin was 11.2g/dL, and the platelet count was 323,000/mm
3. Liver function tests, basic metabolic panel, and immunoglobulins were all
normal. Urinalysis, urine culture, blood culture, chest radiograph, sinus
films, rapid streptococcal antigen and throat culture, Lyme antibodies,
Epstein-Barr virus and cytomegalovirus serologies, ANA, stool culture, stool
for ova and parasites, malaria smear, CT scan of the head and sinuses, and
gallium scan with triple-phase bone scan were all negative. ESR was elevated at
66 mm/hour.
V. Course of Illness
Apart from the mild leukocytosis and elevated ESR, the patient's workup was negative. She became afebrile on hospital day 6 and was discharged
home with close follow-up. Her CBC was checked two times a week for 2 months,
and her absolute neutrophil count was always normal. During the febrile
episodes, she always had an elevated WBC count and ESR, which normalized when
she was afebrile. The diagnosis was suggested by the constellation of symptoms
including the recurrent fever and pharyngitis.
Discussion: Case 7-7
I. Differential Diagnosis
Periodic or recurrent fever has several definitions in the literature. The most
common features are that the temperature is at least 38.4
°C (some sources require at least 39°C) and continues for 3 to 6 days, occurs at least three times over a 6-month
period, has no identifiable cause, is not accompanied by symptoms such as upper
respiratory tract infection, and occurs at intervals that are separated by at
least 1 week (and usually less than 4 weeks). The intervals without symptoms
can be of either variable or predictable duration.
There are several causes that could account for recurrent fevers that occur at
irregular intervals. These include infectious diseases such as viral infections
(e.g., Epstein-Barr virus, parvovirus B19, herpes simplex virus, repeated viral
infections), bacterial infections (e.g., occult bacteremia, recurrent upper
respiratory tract infections, relapsing fever caused by Borrelia spp., chronic
meningococcemia, dental abscess, brucellosis, Yersinia infections,
mycobacterial infection), and parasitic infections (e.g., relapsing malaria
with Plasmodium vivax or Plasmodium ovale, reactivation of plasmodium malaria).
Other causes of recurrent fevers that occur at irregular intervals include
inflammatory diseases (e.g., inflammatory bowel disease, systemic juvenile
rheumatoid arthritis, Be
çhet's disease), neoplasms such as lymphoma, and drug fevers. Infections and
undiagnosed causes are the most common causes of recurrent fevers that occur
with irregularity.
There are very few diseases that cause recurrent fevers at predictable and
regular intervals. Causes include cyclic neutropenia (which usually recurs at
intervals of 21 to 28 days), relapsing fever caused by Borrelia spp. (every 14
to 21 days), and PFAPA (periodic fever, aphthous ulcers, pharyngitis, and
adenopathy) syndrome (every 21 to 28 days). There are also a group of
hereditary causes of periodic fever that can occur at either regular or
irregular intervals and should be considered. These include familial
Mediterranean fever, hyperimmunoglobulin D syndrome (HIDS), and tumor necrosis
factor receptor-associated periodic syndrome (TRAPS, or familial Hibernian
fever). PFAPA and undiagnosed causes are the most common causes of recurrent
fevers that occur at predictable intervals.
The clue to this patient's disease was the regularity of her febrile episodes.
II. Diagnosis
During one of the episodes of fever, the patient was found to have three
aphthous mouth ulcers and an erythematous throat.
The diagnosis is PFAPA syndrome.
III. Incidence and Epidemiology
PFAPA syndrome is defined by recurrent and periodic episodes of fever (greater
than 39
°C) that last for 3 to 6 days, occur approximately every 21 to 28 days, and are
accompanied by certain clinical findings. There is a slight male predominance,
and the disease usually manifests before 5 years of age. There seems to be no
pattern of familial inheritance and no seasonal or geographic predilection.
Although the etiology of PFAPA is not known, infectious and autoimmune causes
have been implicated. PFAPA is considered the most common cause of recurrent
fever that occurs at regular and predictable intervals.
IV. Clinical Presentation
Children with PFAPA are generally well in terms of growth and development and
present with predictable patterns of fever, as described previously. The three
most common clinical findings are aphthous mouth ulcers, pharyngitis, and
cervical adenopathy. The aphthous mouth ulcers are frequently missed because
these are very small (less 5 mm), few (usually two or three), and painless and
resolve very quickly. One case series found reports of associated aphthous
mouth ulcers in only 70% of cases of PFAPA. Pharyngitis is found in only 72% of
cases and is usually nonexudative. Cervical adenopathy is the most commonly
seen sign (88% of cases); it is usually confined to the cervical region and is
relatively unremarkable (nodes less than 5 mm). Although symptoms such as
abdominal pain, nausea, and headache are sometimes present, upper respiratory
tract symptoms are rarely seen and usually indicate a viral upper respiratory
tract infection. The most striking features of PFAPA are its predictable
recurrence and the overall well-being of the patient, both during the episode
and after the episode resolves.
V. Diagnostic Approach
Diagnostic criteria that have been used are clinical in nature and include much
of what has already been discussed: (a) regularly recurring fevers with early
age at onset (younger than 5 years); (b) constitutional symptoms without upper
respiratory tract infection and either aphthous stomatitis, cervical
lymphadenitis, or pharyngitis; (c) asymptomatic intervals between episodes; (d)
normal growth and development; and (e) exclusion of cyclic neutropenia. Other
fever syndromes also have characteristic features (Table 7-3).
Complete blood count. Included in the diagnostic criteria of PFAPA is exclusion of the diagnosis of
cyclic neutropenia. This distinction can be difficult because the two disorders
have similar features. In cyclic neutropenia, neutropenia recurs approximately
every 21 days. Although the fever in cyclic neutropenia usually occurs without
accompanying infection, bacterial infection secondary to the neutropenia can
occur. The best way to distinguish between the two conditions is with a CBC. In
PFAPA, the CBC shows a mild leukocytosis, and the ESR is also mildly elevated
during the episode. These values return to normal in the absence of fever. To
diagnose cyclic neutropenia, CBCs with differential must be checked regularly
(at least twice per week for two consecutive months), because the neutropenia
does not always occur at the time of the fever. In cyclic neutropenia, there is
an absolute neutrophil count of less than 500 cells/mm
3 that count recovers on its own.
Other studies. Additional studies may be directed at other diseases in the differential
diagnosis. Testing for mutation detection in patients with suspected familial
Hibernian fever (TRAPS) is performed by some laboratories. The immunoglobulin D
levels are increased in patients with hyperimmunoglobulin D syndrome, although
several measurements may be required for confirmation. Genetic testing is also
available for familial Mediterranean fever.
VI. Treatment
PFAPA usually persists for several years (more than 4 years) before spontaneous
remission occurs. This remission begins with a period during which the episodes
occur with decreasing frequency before resolving completely. During the years
in which the episodes occur, treatment includes supportive care and planning of
family events around the predictable fever. A single dose of corticosteroids at
the beginning of an episode has been found to help to shorten the duration and
should be the first line of treatment. If this is not effective, prophylaxis
with cimetidine can be attempted. Tonsillectomy has been found to effectively
stop the episodes in some patients and can be considered if neither
corticosteroids nor cimetidine is effective.
VII. References
1. Chandy CJ, Gilsdorf JR. Recurrent fever in children. Pediatr Infect Dis J 2002;21:1071–1080.
2. Thomas KT, Feder HM Jr, Lawton AL, et al. Periodic fever syndrome in
children.
J Pediatr 1999;135:15–21.
3. Long SS. Syndrome of periodic fever, aphthous stomatitis, pharyngitis, and
adenitis (PFAPA): what it isn
't, what it is. J Pediatr 1999;135:1–5.
4. Drenth JPH, van der Meer JWM. Hereditary periodic fever. N Engl J Med 2001;345:1748–1757.
5. Centola M, Aksentijevich I, Kastner DL. The hereditary periodic fever
syndromes: molecular analysis of a new family of inflammatory diseases.
Hum Mol Genet 1998;7:1581–1588.
Pictures
Book Source Details
- Book Title: Pediatric Complaints and Diagnostic Dilemmas
- Author(s): Samir S Shah MD; Stephen Ludwig MD
- Year of Publication: 2003
- Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.
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