Prolonged and Recurrent Fevers in Children
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1 Prolonged and Recurrent Fevers in Children Gary S. Marshall, M.D. Professor of Pediatrics Chief, Division of Pediatric Infectious Diseases Director, Pediatric Clinical Trials Unit University of Louisville School of Medicine
2 Disclosures Consultant for Novartis
3 Objectives After this lecture, participants should be able to - Design a diagnostic plan for children with unexplained fever - Differentiate intermittent febrile illnesses and periodic fevers - Manage patients with PFAPA syndrome
4 Measuring Temperature Liquid-in-glass Thermistor-based Direct contact with patient Crawford. J Med Engineer Tech 2006;30:199
5 Defining Fever Core body temperature o F (38.0 o C) Site of measurement - Birth to 3 years: rectal - 3 to 5 years: axillary (add 1 o F or 0.6 o C) - Ages 5 and above: oral Rideout. Contemp Pediatr 2001;18:42
6 Brighton Collaboration Marcy. Vaccine 2004;22:551 (38.0 o C=100.4 o F)
7 Undifferentiated Fever Prolonged Recurrent Fever is the main complaint Not associated with a defined clinical illness Not fever without source Previously healthy
8 Undifferentiated Fever Prolonged Recurrent Not-FUO Patient is not having abnormal temperatures
9 It is a fever
10 It is a fever for him!
11 Undifferentiated Fever Prolonged Recurrent Not-FUO Causes Temperatures usually run low Diurnal temperature variation Meals Ovulation Tobacco and chewing gum Exercise Kleiman. Pediatr Clin N A 1982;29:201
12 Undifferentiated Fever Prolonged Recurrent Not-FUO Clues Healthy appearance Normal growth/development Stable weight School absences for subjective complaints Vulnerable child Behavioral problems Misconceptions about health Fear of malignancy Family stress Normal physical exam Kleiman. Pediatr Clin N A 1982;29:201
13 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO Diagnosis achievable in the primary care setting Clues on history, physical exam, or simple laboratory tests Uncommon presentation of a common disease Separate illnesses that blend together
14 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO Diagnoses URTI (otitis media, sinusitis) Ehrlichiosis LRTI (pneumonia) Endocarditis Central nervous system infection Leukemia Tuberculosis Kawasaki disease Histoplasmosis Juvenile idiopathic arthritis Brucellosis Inflammatory bowel disease
15 Fever for 3 Weeks
16 Fever for 4 Weeks
17 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF
18 Illness >3 weeks Fever >101 o F on several occasions Diagnosis uncertain after 1 week in hospital Petersdorf. Medicine 1961;40:1
19 Defining Prolonged Unexplained Fever Reference Temperature ( ) o C o F Frequency Duration McClung, Multiple occasions Outpatient: 3 wk Inpatient: 1 wk Pizzo, occasions 2 wk Lohr, Multiple occasions Outpatient: 3 wk Inpatient: 1 wk Steele, occasions/wk 3 wk Normal U/A and CXR Jacobs, Daily 2 wk
20 Operational Definition of FUO in Children Core body temperature 38.1 o C ( o F) 1 occasion every day 14 consecutive days Made up by Dr. Marshall
21 Factors Affecting Cause of FUO Geography Age Host factors New diseases Physician experience Referral patterns Availability of laboratory tests and imaging Managed care
22 Causes of FUO in Children U.S s (N=240) 1990s (N=255) Percent McClung. Am J Dis Child 1972;124:544; Pizzo. Pediatrics 1975;55:468; Lohr. Clin Pediatr 1977;16:768; Steele. J Pediatr 1991;119:526; Jacobs. Clin Infect Dis 1998;26:80
23 Infectious Causes of FUO in Children U.S. URTI LRTI UTI CNS TB SBE Mono Osteo Cat Scratch Tularemia Lyme Ehrlichiosis Other 1970s (N=99) 1990s (N=72) Percent McClung. Am J Dis Child 1972;124:544; Pizzo. Pediatrics 1975;55:468; Lohr. Clin Pediatr 1977;16:768; Steele. J Pediatr 1991;119:526; Jacobs. Clin Infect Dis 1998;26:80
24 Fever for 2 Months ESR >130 mm/hr
25 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Inflammatory and autoimmune diseases Inflammatory bowel disease Wegener granulomatosis Juvenile idiopathic arthritis Sarcoidosis Lupus HLH Rheumatic fever Behcet disease Long. Principles and Practice of Pediatric Infectious Diseases; 2008:126
26 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Malignancies Leukemia Lymphoma Neuroblastoma Hepatoma Soft tissue sarcoma Long. Principles and Practice of Pediatric Infectious Diseases; 2008:126
27 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Miscellaneous Munchausen by proxy Factitious fever Drug fever Central fever Pulmonary embolus Dysautonomia Diabetes insipidus Ectodermal dysplasia Hyperthyroidism Hematoma Long. Principles and Practice of Pediatric Infectious Diseases; 2008:126
28 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Approach to diagnosis Severity of findings dictates pace of evaluation Serial evaluations Avoid antimicrobials Use time as a diagnostic tool Tolan. Clin Pediatr 2010;49:207
29 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF If it s infection, there s always a source Travel Animals Raw foods Transfusions Ill persons Recent procedures Insect bites Unusual activities Tolan. Clin Pediatr 2010;49:207
30 Questions Only an ID Doc Could Ask
31 Questions Only an ID Doc Could Ask Tried unpasteurized goat s milk?
32 Questions Only an ID Doc Could Ask Tried unpasteurized goat s milk? Brucellosis
33 Questions Only an ID Doc Could Ask Jumped on any beaver dams?
34 Questions Only an ID Doc Could Ask Jumped on any beaver dams? Blastomycosis
35 Questions Only an ID Doc Could Ask Been around any discarded sheep placentas?
36 Questions Only an ID Doc Could Ask Been around any discarded sheep placentas? Q fever
37 Diagnostic Approach First Visit Initial Hx and PE Previous lab results CBC with smear CMP ESR and CRP U/A and UCx BCx CXR Targeted studies
38 Diagnostic Approach First Visit Initial Hx and PE Previous lab results CBC with smear CMP ESR and CRP U/A and UCx BCx CXR Targeted studies Second Visit Interval Hx and PE CBC with smear CMP ESR and CRP Targeted studies Fever/symptom diary
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41 Diagnostic Approach First Visit Initial Hx and PE Previous lab results CBC with smear CMP ESR and CRP U/A and UCx BCx CXR Targeted studies Second Visit Interval Hx and PE CBC with smear CMP ESR and CRP Targeted studies Fever/symptom diary Fever/symptom diary
42 Diagnostic Approach First Visit Initial Hx and PE Previous lab results CBC with smear CMP ESR and CRP U/A and UCx BCx CXR Targeted studies Second Visit Interval Hx and PE CBC with smear CMP ESR and CRP Targeted studies Third Visit Interval Hx and PE CBC with smear CMP ESR and CRP Targeted studies Fever/symptom diary Fever/symptom diary
43 Targeted Studies Infectious Diseases - TST - Histoplasma serology - EBV and CMV serology - Bartonella serology - Brucella serology - Toxoplasma serology - Tularemia serology - HIV antibody or PCR - Stool Cx Autoimmune/Inflammatory - ANA and RF - ASOT and anti-dnase-b - C3 and C4 Malignancy - Flow cytometry - LDH and uric acid - Bone marrow Miscellaneous - TFTs - Slit lamp exam - Sinus CTS - Echocardiogram - Endoscopy - Abdominal U/S or CTS - Bone scan - PET scan
44 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Statler. JPIDS DOI: /jpids/piv008
45 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Statler. JPIDS DOI: /jpids/piv008
46 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Statler. JPIDS DOI: /jpids/piv008
47 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) Statler. JPIDS DOI: /jpids/piv008
48 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) No diagnosis established (33) Diagnosis established (15) Statler. JPIDS DOI: /jpids/piv008
49 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent
50 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses
51 The picture can't be displayed.
52 The picture can't be displayed. When did the fever start?
53 The picture can't be displayed. The day he was born. When did the fever start?
54 The picture can't be displayed. The day he was born. When did the fever start? Seriously, he s had fever every single day since he was born?
55 The picture can't be displayed. The day he was born. When did the fever start? Yes. Seriously, he s had fever every single day since he was born?
56 The picture can't be displayed. The day he was born. When did the fever start? Yes. Seriously, he s had fever every single day since he was born? How old is he?
57 The picture can't be displayed. The day he was born. When did the fever start? Yes. Seriously, he s had fever every single day since he was born? 12. How old is he?
58 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses Epidemiological clues (e.g., day care attendance) Diagnosis History biopsy Fever and symptom diary Screening laboratory studies Time
59 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses Autoinflammatory diseases Kastner. Cell 2010;140:784
60 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses Autoinflammatory diseases Abnormally increased inflammation Predominantly mediated by cells and molecules of the innate immune system (as opposed to autoantibodies or autoreactive T-cells) Significant host predisposition Kastner. Cell 2010;140:784
61 Autoinflammatory diseases Sequential viral illnesses
62 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses Autoinflammatory diseases Inflammasomopathies Kastner. Cell 2010;140:784
63 The Inflammasome - Macromolecular complex Responds to danger signals Produces inflammatory cytokines - Monocytes, neutrophils, activated T-cells, chondrocytes Masters. Ann Rev Immunol 2009;27:621
64 http-//bmc.erin.utoronto.ca/~michaels/images/portfolio/nalp3 Masters. Ann Rev Immunol 2009;27:621
65 http-//bmc.erin.utoronto.ca/~michaels/images/portfolio/nalp3 Masters. Ann Rev Immunol 2009;27:621
66 http-//bmc.erin.utoronto.ca/~michaels/images/portfolio/nalp3 Masters. Ann Rev Immunol 2009;27:621
67 Intrinsic inflammasomopathies (cryopyrin) Familial cold autoinflammatory syndrome Muckle-Wells syndrome Neonatal-onset multisystem inflammatory disease http-//bmc.erin.utoronto.ca/~michaels/images/portfolio/nalp3 Masters. Ann Rev Immunol 2009;27:621
68 Extrinsic inflammasomopathies Familial Mediterranean fever: pyrin mutation Hyper IgD syndrome: mevalonate kinase def Mevalonate kinase Pyrin http-//bmc.erin.utoronto.ca/~michaels/images/portfolio/nalp3 Masters. Ann Rev Immunol 2009;27:621
69 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Sequential, common, self-limited viral illnesses Autoinflammatory diseases Inflammasomopathies Protein folding disorders Kastner. Cell 2010;140:784
70 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB Proinflammatory gene transcription NUCLEUS CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
71 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB mtnfr1 Proinflammatory gene transcription TNFR1 NUCLEUS ER CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
72 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB Unfolded protein response Ligand-independent signaling Inhibition of apoptosis mtnfr1 Proinflammatory gene transcription TNFR1 NUCLEUS ER CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
73 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB Unfolded protein response Ligand-independent signaling Inhibition of apoptosis mtnfr1 Proinflammatory gene transcription TNFR1 NUCLEUS ER GOLGI APPARATUS CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
74 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB Unfolded protein response Ligand-independent signaling Inhibition of apoptosis mtnfr1 Proinflammatory gene transcription TNFR1 TNF NUCLEUS ER GOLGI APPARATUS CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
75 TNF-Receptor Associated Periodic Syndrome (TRAPS) NF-κB Unfolded protein response Ligand-independent signaling Inhibition of apoptosis mtnfr1 X Proinflammatory gene transcription TNFR1 TNF NUCLEUS ER GOLGI APPARATUS CELL MEMBRANE Kimberly. Arth Res Ther 2007;9:217 Masters. Ann Rev Immunol 2009;27:621
76 Feature Intrinsic Inflammasomopathies Extrinsic FCAS MWS NOMID FMF HIGDS Inheritance AD AD Sporadic AR AR AD Ethnicity Euro Euro Any Med Euro Euro Age at onset <1 yr <20 yr <1 yr <20 yr <1 yr <20 yr Triggers Cold Cold Vaccination --- Protein Folding Disorder TRAPS Frequency Variable Variable Continuous Variable 2-4 wk Variable Duration 1-2 days 2-3 days Continuous 1-3 days 3-7 days >7 days Distinctive features Rash Conjunctivitis Headache Nausea Rash Conjunctivitis Deafness Rash Meningitis Arthropathy Deafness Adenopathy Liver Spleen Serositis Spleen Erysipeloid erythema Abd pain Vomiting Diarrhea Rash Adenopathy Arthralgia Headache Amyloidosis No Yes No Yes No Yes Rash Arthritis Conjunctivitis Spleen Treatment Anti-IL-1 Anti-IL-1 Anti-IL-1 Colchicine Anti-IL-1 Anti-TNF Kastner. Hematol 2005;74-81 Bodar. Br J Hematol 2008;144:279 Lachmann. Clin Exper Immunol 2011;165:301 Anti-IL-1 Anti-TNF
77 Clinical Classification Criteria Federici. Ann Rheum Dis 2015;74:799 Data from Eurofever Registry (N=1215)
78 Clinical Classification Criteria Federici. Ann Rheum Dis 2015;74:799 Data from Eurofever Registry (N=1215)
79 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Periodic Cyclic Neutropenia Lang. Am J Pediatr Hematol Oncol 1981;3:363
80 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Periodic Cyclic Neutropenia Lang. Am J Pediatr Hematol Oncol 1981;3:363
81 Undifferentiated Fever Prolonged Recurrent Not-FUO F-Not-UO PUF Intermittent Periodic Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome
82 Distinctive Features of PFAPA Syndrome - Clockwork periodicity - Episodes are stereotypical, circumspect, and unprovoked - Identifiable prodrome is common - Upper respiratory tract inflammation - No rash or arthritis - Acute phase reaction - Failure to fail to thrive - Episodes are aborted by steroids - Episodes resolve after tonsillectomy - Syndrome usually resolves by adolescence - No long-term sequelae Marshall. J Pediatr 1987;110:43; Thomas. J Pediatr 1999;135:15; Long. J Pediatr 1999;135:1; Feder. Acta Paediatr 2010;99:178; Wurster. J Pediatr 2011;159:958
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86 PFAPA Syndrome Characteristic Number (95% CI) Number of patients 83 Male 57% Age at onset 3 years Duration of episodes 4 days Maximum temperature 40.3 o C Duration of fever >38.3 o C 3 days Episodes per year 10 Symptom-free interval 41 days Thomas. J Pediatr 1999;135:15
87 PFAPA Syndrome: Natural History Resolved N=50 Age=9.2 yr Original Registry N=94 Follow-up 2009 N=59 Death N=1 Argininosuccinate lyase deficiency Duration=6.3 yr Continuing N=9 Age=20 yr Remissions N=8 Duration= 13 mo No remissions N=1 Wurster. J Pediatr 2011;159:958
88 PFAPA Syndrome: Diagnostic Criteria Criterion Marshall 1989 Thomas 1999 Garavello 2009 Onset <5 yr Feder 2010 Fever Abrupt Abrupt >38.9 o C Frequency of episodes Regular Regular Regular 6 episodes, q2-8 wks Duration of episodes ~5 days ~5 days 10 days Constitutional symptoms Aphthous stomatitis Pharyngitis Cervical adenitis Acute phase reaction Asymptomatic intervals <10 wk Exclusion of URTI Exclusion of CN Other exclusions NL growth/development Benign long-term course Response to steroids Other exclusions variously include: FMF, TRAPS, HIGDS, Behcet's, Immunodeficiency, autoimmune disease, chronic infection, arthritis, rash
89 Acute Phase Reaction in PFAPA Syndrome CRP mg/l Days of Fever Forsvoll. Acta Paediatr 2007;96:1670 (serial measurements during 38 episodes in 16 children)
90 The earnestness of this Cochrane review article on the so-called PFAPA syndrome raises the frightening possibility that the American otolaryngology profession has been duped into promoting a brand new acronym for what used to be just called recurrent tonsillitis whose basic prerequisite is the presence of tonsils. Hornibrook J. Otolaryng Head Neck Surg 2011;144:649
91 The earnestness of this Cochrane review article on the so-called PFAPA syndrome raises the frightening possibility that the American otolaryngology profession has been duped into promoting a brand new acronym for what used to be just called recurrent tonsillitis whose basic prerequisite is the presence of tonsils. Hornibrook J. Otolaryng Head Neck Surg 2011;144:649 Is PFAPA a thing?
92 You Know When You ve Seen One
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96 PFAPA Citations in PubMed
97 PFAPA Milestones 1987 Marshall. Original case series (N=12) 1999 Thomas. Vanderbilt registry (N=94) Padeh. Israel (N=28) 2006 Tasher. Israel (N=54) 2010 Feder. University of Connecticut (N=105) 2011 Wurster. Vanderbilt registry follow-up (N=59) 2013 Forsvoll. Population-based study in Norway (incidence 2.3 per 10,000) 2014 Hofer. Pediatric Rheumatology European Society cohort (N=301) Clinical features, surrogate markers, natural history, epidemiology
98 PFAPA Milestones 2002 Galanakis. PFAPA among children undergoing tonsillectomy 2007 Renko. Randomized controlled trial in Finland (N=26) 2009 Garavello. Randomized controlled trial in Italy (N=39) 2010 Burton. Cochrane Review Garavello. Systematic review Baugh. AAO-HNS guidelines 2012 Licameli. Case series (N=102) 2014 Burton. Cochrane Review 2 Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
99 PFAPA Milestones 2006 Stojanov. Cytokine profile 2010 Brown. Inflammatory mediators 2011 Stojanov. Disorder of innate immunity Berkun. MEFV mutations Yazgan. CRP and procalcitonin 2013 Kolly. Dysregulated IL-1β production Forsvoll. Elevated CXCL10 Sundqvist. Neutrophil oxygen radical production Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
100 PFAPA Milestones 2008 Gattorno. Diagnostic score for monogenic PFS 2009 Gattorno. Gaslini score to identify patients at low risk for PFS mutations Differentiation Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
101 PFAPA Milestones 2010 Cochard. Familial clustering in Europe 2011 Adachi. Family cluster in Japan Antón-Martín. Occurrence in sibs Families Differentiation Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
102 PFAPA Milestones 2015 Di Gioia. Genome-wide SNPs, whole-exome sequencing autosomal dominant incomplete penetrance unlikely monogenic Genetics Families Differentiation Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
103 PFAPA Milestones 2013 Valenzuela. Inflammatory mediators in tonsils 2015 Petra. Phenotype of T-cells in tonsils Forsvoll. Decreased CD8+ T-cells in tonsils Tonsils Genetics Families Differentiation Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
104 PFAPA Milestones 2014 Freeman. Microbial RNA sequencing in tonsils 2016 Tejesvi. Bacterial rrna gene sequencing Microbiome Tonsils Genetics Families Differentiation Inflammation Tonsillectomy Clinical features, surrogate markers, natural history, epidemiology
105 Gaslini Score Variable Coding Coefficient Age at onset Months Abdominal pain Never= 0 Sometimes or often= 2 Always= 3 Aphthosis Never= 0 Sometimes or often= 1 Always= 2 Thoracic pain Absent= 0 Present= 1 Diarrhea Never= 0 Sometimes= 1 Often= 2 Family history Negative= 0 Positive= Gattorno. Arth Rheum 2008;58:1823
106 Differential Gene Expression Hereditary Periodic Fever PFAPA Flare PFAPA Non-flare Control Principal Component Analysis Stojanov. PNAS 2011;108:7148 N=6 in each group Hereditary periodic fevers: CAPS=3, FMF=2, TRAPS=1
107 Differential Gene Expression Stojanov. PNAS 2011;108:7148
108 Inflammatory Proteins in Serum Stojanov. PNAS 2011;108:7148
109 Tonsillectomy: Meta-analysis Source, year Observed/Total Complete Resolution (%) Garavello. J Pediatr 2011;159:138
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111 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) No diagnosis established (33) Diagnosis established (15) Statler. JPIDS DOI: /jpids/piv008
112 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Intermittent (92) Periodic (60) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) No diagnosis established (33) Diagnosis established (15) Statler. JPIDS DOI: /jpids/piv008
113 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Intermittent (92) Periodic (60) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) Self-limited illnesses or no diagnosis established (84) Diagnosis established (8) No diagnosis established (33) Diagnosis established (15) Statler. JPIDS DOI: /jpids/piv008
114 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Intermittent (92) Periodic (60) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) Self-limited illnesses or no diagnosis established (84) Diagnosis established (8) No diagnosis established (33) Diagnosis established (15) No diagnosis established (39) Periodic fever syndrome (21) Statler. JPIDS DOI: /jpids/piv008
115 Clinic visit 1/1/08-12/31/12 (N=4586) Referred for unexplained fever (221) Exclusions Fever, febrile, FUO not included in diagnosis (4118) Established patient visit (159) Initial consult in hospital (51) Not referred for fever (17) Initial visit before 2008 (8) Records not available (3) Referred for typhoid fever (2) Referred for scarlet fever (1) Referred for fever blisters (1) Prolonged (69) Recurrent (152) Not having fever (10) Having fever (59) Intermittent (92) Periodic (60) Diagnosis apparent on initial visit (11) Fever of unknown origin (48) Self-limited illnesses or no diagnosis established (84) Diagnosis established (8) No diagnosis established (33) Diagnosis established (15) No diagnosis established (39) Periodic fever syndrome (21) Statler. JPIDS DOI: /jpids/piv008 PFAPA (20) FMF (1)
116 Undifferentiated Fever in Pediatric ID Clinic Final Diagnostic Category No illness, self-limited illness, or no diagnosis made Prolonged (N=69) Fever Pattern Recurrent (N=152) All Patients (N=221) 43 (62%) 123 (81%) 166 (75%) Mild or moderate illness 18 (26%) 25 (16%) 43 (20%) Serious illness 8 (12%) 4 (3%) 12 (5%) Statler. IDSA Meeting, October 2014
117 Undifferentiated Fever in Pediatric ID Clinic Final Diagnostic Category No illness, self-limited illness, or no diagnosis made Prolonged (N=69) Fever Pattern Recurrent (N=152) All Patients (N=221) 43 (62%) 123 (81%) 166 (75%) Mild or moderate illness 18 (26%) 25 (16%) 43 (20%) Serious illness 8 (12%) 4 (3%) 12 (5%) Statler. IDSA Meeting, October 2014
118 Undifferentiated Fever in Pediatric ID Clinic Final Diagnostic Category No illness, self-limited illness, or no diagnosis made Prolonged (N=69) Fever Pattern Recurrent (N=152) All Patients (N=221) 43 (62%) 123 (81%) 166 (75%) Mild or moderate illness 18 (26%) 25 (16%) 43 (20%) Serious illness 8 (12%) 4 (3%) 12 (5%) Inflammatory bowel disease (3) Juvenile idiopathic arthritis (2) Acute lymphoblastic leukemia (1) Familial Mediterranean fever (1) Systemic lupus (1) Malaria (1) Rheumatic fever (1) Typhoid fever (1) EBV infection w/hlh (1) Statler. IDSA Meeting, October 2014
119 He s got the fever Oh, he s got the fever Nothing a po boy can do
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