Stephanie Kuhlmann, MD, FAAPBrooke R. Mason, MD, FAAP

KU Pediatric Hospitalist GroupWesley Medical Center

Wichita, KS

7 year old female presents with 3 day history of fever up to 104.9 and myalgia

• Fevers x 3 days• L shoulder and L thigh pain now resolved upon

admission• Febrile episode 3 weeks ago (temp 104)

• refused to bear weight at the time • Evaluated at an ER in Wichita the prior evening

• Diagnosis: viral illness - received IVF and sent home• Recurrent fevers since infancy

• every 2-3 months• lasts 1-2 days now 3-4 days

POSITIVES• CONSTITUTIONAL: fever• GI: constipation, decreased

appetite• RENAL: decreased UOP• MS: thigh pain, shoulder pain• HEME: occasional epistaxis

NEGATIVES• CONSTITUTIONAL: weight loss• HEENT: headache, vision

changes, sore throat, sores in mouth, ear pain

• NECK: swollen lymph nodes, stiffness

• PULM: cough or respiratory symptoms

• GI: abdominal pain, nausea, vomiting, diarrhea

• SKIN: rashes or skin changes• MS: swelling or redness of

joints, swelling of extremities• HEME: no easy bruising

• Medical Conditions:– FTT as infant associated with diarrhea

• tested negative for Celiac but noted partial IgA deficiency

• Hospitalizations: None• Surgeries: None• Meds: Tylenol/Motrin• Developmental History:

– Mild speech delay and receiving speech therapy

• Social History• Military family

• Recent move from North Dakota to Montana to Kansas

• Cat and dog at home• No camping or recent international travel

• Family History• No autoimmune disease• Mom and MGM with SVT

• VITALS: T 101.4 R, HR 151, RR 20, BP 114/59, SaO2 100% on RA, Wt 20.1 kg (20th%), Ht 122 cm (50th%)

• GEN: Alert and oriented, non toxic appearing • HEENT: TMs, conjunctiva and pharynx without erythema, no oral lesions, no

nasal congestion or discharge • NECK: Supple, no stiffness • CHEST: CTAB • CV: Tachycardic, but regular rhythm, no murmur, adequate pulses and cap refill • ABD: Soft, NT, no masses, no HSM • MS: No joint swelling, no decreased ROM, no tenderness along L thigh• EXT: Pink, warm, well perfused , no edema• SKIN: No rashes or lesions• NEURO: No deficits, strength appropriate• LYMPH: No palpable nodes throughout

• CMP normal• UAM negative• CBC

– WBC 8.7– Hgb 10.7– Platelets 164K– 80% neutrophils– 12% lymphs– 15% monos– MCV and RDW WNL

• Infectious (osteomyelitis , pyomyositis, TB, EBV/CMV, HIV)

• Rheum/inflammatory (SLE, JIA)• Periodic fever syndrome (PFAPA, TRAPS, FMF,

NOMID, cyclic neutropenia)• Oncology (leukemia, bone tumor)• Endo (thyroid)

• Received Tylenol PO, Zofran PO, NS bolus of 400 mL

• CBC, CMP, CRP, ESR, CPK, iCa, Mg, Phos, TSH, ANA ordered

• CBC with Hgb 10.6, MCV nml; remainder normal• Blood culture (negative)• ESR, CRP (mild to moderate elevation; 47.2 and 27)• CPK (WNL, 39)• UA (2+ ketones otherwise unremarkable)• ANA (negative)• Thyroid studies (WNL)

• Monitored in hospital overnight • Tmax 105 F• Complained briefly of abdominal pain with a

benign exam • Low blood pressure overnight with SBP of 73

• not tachycardic, well perfused, good urine output• fluid bolus and her BP normalized

• Ordered HIV PCR, Mono spot, Quantiferon• Film of L femur • Consulted ID

“This is presumptive Periodic Fever based on the history of fairly regularly occurring

episodes of fever once a month for 2-3 years and well between the episodes.”

• One small ulcer like lesion on her lip• SBP 73-107, HR 105-135, Tmax 104• Albumin dropped to 2.7 gm/dL• Platelets decreased to 120k • Hgb decreased to 9.1 gm/dL• Ferritin, LDH, iron, haptoglobin ordered• Question if bone marrow biopsy needed• EBV and CMV serology sent

• Continued to be intermittently febrile, (Tmax 104)

• SBP 93-102, HR 98-138• Developed exudative pharyngitis,• Shotty cervical lymphadenopathy• Hgb 9.8 gm/dL, platelets 156 K, WBC 5.9• Strep screen and culture

• Afebrile after midnight• SBP WNL• Developed 2nd lesion on lip• Exudative pharyngitis, anterior and posterior

cervical adenopathy• Given dose of 2 mg/kg of prednisolone for

presumed PFAPA• ANA negative, CMV serology and EBV IgM

WNL• Dismissed to home

• Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis

• Other findings: • Malaise, headache, abdominal pain, arthralgia, HSM,

leukocytosis, and elevated acute phase reactants. • Symptoms begin around 2–6 yr of age • Lasts 4–6 days, regardless of antipyretic or antibiotic

treatment• Occurs at a frequency of 8–12 episodes/yr.

• Frequency and intensity of the episodes diminish over time

• Etiology and the pathogenesis unknown. • Response to a single dose of prednisone (1–2

mg/kg) with prompt resolution of symptoms within 24 hr

• Complete resolution has also been reported after tonsillectomy

• No long term sequelae