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Clinical Conundrum

Clinical Conundrum. Stephanie Kuhlmann, MD, FAAP Brooke R. Mason, MD, FAAP KU Pediatric Hospitalist Group Wesley Medical Center Wichita, KS. Chief Complaint. 7 year old female presents with 3 day history of fever up to 104.9 and myalgia. History of the Present Illness. Fevers x 3 days

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Clinical Conundrum

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  1. Clinical Conundrum Stephanie Kuhlmann, MD, FAAP Brooke R. Mason, MD, FAAP KU Pediatric Hospitalist Group Wesley Medical Center Wichita, KS

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

  3. History of the Present Illness • 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

  4. Review of Systems POSITIVES 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 • CONSTITUTIONAL: fever • GI: constipation, decreased appetite • RENAL: decreased UOP • MS: thigh pain, shoulder pain • HEME: occasional epistaxis

  5. Past Medical History • 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

  6. Social and Family History • 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

  7. Physical Exam • 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

  8. Lab from Transferring ER • CMP normal • UAM negative • CBC • WBC 8.7 • Hgb 10.7 • Platelets 164K • 80% neutrophils • 12% lymphs • 15% monos • MCV and RDW WNL

  9. Differential Diagnosis?

  10. Differential Diagnosis • 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)

  11. Wesley ER • Received Tylenol PO, Zofran PO, NS bolus of 400 mL • CBC, CMP, CRP, ESR, CPK, iCa, Mg, Phos, TSH, ANA ordered

  12. Day 1 Workup • 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)

  13. Hospital Course: Day 1 • 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

  14. ID Consult “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.”

  15. Hospital Course: Day 2 • 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

  16. Day 3 • 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

  17. Day 4 • 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

  18. Fever Curve

  19. PFAPA/Marshall Syndrome • 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.

  20. PFAPA • 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

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