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Transcript
Recurrent Fever in the Pediatric Patient Ping-Wei Chen Emergency Medicine Resident Much thanks to: Drs. Bryan Young, Graham Thompson, Susan Kuhn,Chris Waterhouse, Paivi Miettunen, Ron Anderson +++ Concerned Parent • 4 year old boy • 5 weeks of intermittent fevers (Tmax 389C) – – – – last “a few days” fatigue, malaise Unsure if ever completely gone ?red rash the first few days • Walk-in clinic x 2 – “viral illness” – “the flu” • Negative urine dip • PLC ER – Today is visit #2 PLC ER Visit #1 • CBC – Hgb 96 – WBC 9.7 – Plt 530 • Electrolytes, Creatinine, BUN = normal • Urine dip, R+M negative PLC ER Visit #2 • “5 weeks intermittent fevers” • • • • • • • • • • • Tmax 389C ✓ fatigue/malaise Øvomiting, Ødiarrhea Ørespiratory symptoms Øgenitourinary symptoms ✓red rash “first few days” Øsick contacts From Turkey 6 months ago Preschool student Previously healthy Immunizations UTD On exam • 379C, 100/65, 102bpm, 100% RAO2 • HNT normal • CV normal • Resp – clear, equal BS • Abdo – soft, nontender • MSK – Ørash/joint pain Objectives • Discuss definitions – Recurrent Fever/Periodic Fever – Fever of Unknown Origin (FUO) • Outline differential diagnoses – Regular VS Irregular fever intervals • Describe an approach • Expert opinion – ID, GI, Rheumatology, Oncology Definitions Working Definition • Recurrent/Periodic Fever – Repeating episodes of fever separated by periods of normal temperature that return at regular or irregular intervals • Fever of Unknown Origin – Fever of greater than 3 weeks duration and uncertain diagnosis after 1 week of intensive investigation Recurrent Fever • John and Gilsdorf 2003 – “≥3 episodes of fever in a 6 month period with no defined medical illness to explain the fever and with an interval of at least 7 days in between febrile episodes” Recurrent/Periodic Fever • Long 2005 • Recurrent Fever – “A single illness in which fever and other signs and symptoms wane and wax” • Periodic Fever – “Recurring episodes of illness for which fever is the cardinal feature…with intervening intervals of weeks to months of complete well-being. Episodes can have either clockwork or irregular periodicity” Fever of Unknown Origin • Petersdorf and Beeson 1961 – “fever persisting more than 3 weeks in duration, with documented temperatures of 38.3oC on several occasions, and uncertain diagnosis after intensive study of at least 1 weeks duration” “Throw me a frickin’ bone here” Etiology • “Common disorders with uncommon presentations” 1. INFECTION 2. Inflammatory/Autoimmune 3. Undiagnosed (recurrent)/Neoplasms (FUO) Etiology Infectious Autoimmune/I nflammatory Malignant No diagnosis Misc McClung 1972 (n=99) 28% 14% 8% 11% 16% Pizzo et al. 1975 (n=100) 52% 20% 6% 12% 10% Feigen and Shearer 1976 (n=20) 35% 20% 5% 30% 10% Lohr and Hendley 1977 (n=54) 33% 21% 13% 19% 15% Etiology Ciftci et al. 2003 • Etiology FUO (n=102) – – – – – Infection 44.2% Collagen Vascular 6.8% Malignancy 11.7% Misc. 24.5% Undiagnosed 12.8% Pasic et al. 2006 • Etiology FUO (n=185) – – – – – – Infection 37.8% Autoimmune 12.9% Kawasaki Disease 6.4% Malignancy 6.4% Misc. 8.1% Undiagnosed 30% Recurrent Fever Differential Diagnosis Fever Intervals Regular? Irregular? Fevers at Regular Intervals • Fever occurring at regular intervals – PFAPA syndrome* – Cyclic neutropenia – Relapsing fever (Borrelia spp. other than burgdorferri) – Undiagnosed cause* • Fever occasionally at regular intervals – Familial Mediterranean Fever – Hyper-IgD syndrome – EBV infection John and Gilsdorf 2002 Fever at Regular Intervals • Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenopathy (PFAPA) – high fever q21-28 days – Leukocytosis, ESR – well/investigations normal between episodes – Tx: prednisone, cimetidine – No long-term sequelae Fever at Regular Intervals • Cyclic Neutropenia – Uncommon – May be clinically indistinguishable from PFAPA – Usually no bacterial infection during neutropenia – Diagnosis: • CBC X2-3/week for 6 weeks (ANC <500) and spontaneous recovery • Bone marrow – If symptomatic, G-CSF Fever at Regular Intervals • Relapsing Fever – Spirochetes of Borrelia genus (not burgdorferi) – Fevers 1-6 days separated by 4-14 days – “crisis” (BP,HR) followed by profuse diaphoresis, falling temperature, and BP. • Mortality for untreated fever during crisis and its aftermath – Treatment: penicillin or tetracycline Fevers at Irregular Intervals John and Gilsdorf 2002 An Approach • Careful history & physical exam – Establish pattern of fever (fever diary) • Constant VS Recurrent • Duration • Associated symptoms – Hematologic exam • Hepatosplenomegaly • Lymphadenopathy When to refer? • Dr. Susan Kuhn (Pediatric Infectious Diseases) • ≥3 episodes of recurrent fever What to order? • Infectious Disease – Order: • • • • • • CBC Urine dip/R+M/C+S Blood culture ESR/CRP EBV serology (IgM/IgG) Quantitative immunoglobulins – Maybe: – CXRay – resp symptoms – Stool C+S/O+P – diarrhea – Thin/Thick blood smear – travel to endemic area ≤1 year What to Order? • Dr. Chris Waterhouse (Paediatric GI) – Add: • • • • Hepatobiliary studies (ALT, GGT, ALP, lipase) Iron studies (ferritin, iron) Albumin (losses/decreased production) Stool studies (C+S, O+P, C. diff) What to order? • Dr. Paivi Miettunen (Pediatric Rheumatology) – If referring directly to Rheumatology Clinic • Order: – – – – – – – CBC Creatinine, Urea ESR/CRP on days 1, 5, 10 of fever Ferritin IgD Urine R+M Urine Mevalonic Acid What to Order? • Dr. Ron Anderson (Pediatric Oncology) – Order: • CBC • CXRay – if lymphadenopathy, hepatomegaly, splenomegaly, abdominal mass Prognosis • Generally excellent – If no diagnosis after investigations • Fevers resolve • Growth/Development unaffected – No further testing unless new signs/symptoms Back to our Case • Referred to urgent paediatrics – Bloodwork/Urine investgations unremarkable – No diagnosis • Fevers resolved – Still being followed by paediatrics Conclusions 1. 2. 3. 4. Recurrent Fever ≠ Fever of Unknown Origin Differential Diagnosis are not the same Urgent Paediatrics/Outpatient Paediatrics Workup guided by ID/GI suggestions