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

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Presentation on theme: "Stephanie Kuhlmann, MD, FAAP Brooke R. Mason, MD, FAAP KU Pediatric Hospitalist Group Wesley Medical Center Wichita, KS."— Presentation transcript:

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

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

3 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 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

5 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 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 VITALS: T 101.4 R, HR 151, RR 20, BP 114/59, SaO2 100% on RA, Wt 20.1 kg (20 th %), Ht 122 cm (50 th %) 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 CMP normal UAM negative CBC – WBC 8.7 – Hgb 10.7 – Platelets 164K – 80% neutrophils – 12% lymphs – 15% monos – MCV and RDW WNL

9

10 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 Received Tylenol PO, Zofran PO, NS bolus of 400 mL CBC, CMP, CRP, ESR, CPK, iCa, Mg, Phos, TSH, ANA ordered

12 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 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 “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 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 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 Afebrile after midnight SBP WNL Developed 2 nd 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

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19 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 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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