Kawasaki Disease

Brief H&P:

An 8-month old male is brought to the emergency department with fever. He has had four days of fever (temperature ranging from 37-40°C), rash on trunk and extremities, white-colored tongue discoloration, and irritability with decreased oral intake. Temperature on presentation was 39.4°C, examination revealed an erythematous maculopapular rash on the extremities and trunk including soles of the feet. Mucous membrane involvement was noted with oropharyngeal erythema and bilateral conjunctival injection. Neck examination demonstrated right-sided cervical adenopathy.

Labs:

  • WBC: 23.4 (N: 59%, B: 21%)
  • ESR: 100mm/hr
  • CRP: 7.59mg/dL
  • Albumin: 3.3g/dL
  • AST/ALT: 78U/L, 65U/L
  • UA: 7WBC, no bacteria

Hospital Course

The patient was admitted with a diagnosis of Kawasaki Disease and was treated with IVIG and high-dose aspirin. The patient demonstrated marked improvement with treatment and had a normal echocardiogram. He was discharged on hospital day three.

Epidemiology1,2

  • Age: 6 months to 5 years
  • Northeast Asian
  • Possible heritable component
  • Seasonal (winter/spring)

Course

  • Acute febrile (T > 39°C refractory to anti-pyretics)
  • Subacute (coronary vasculitis)
  • Convalescent

Diagnosis

  • Fever >5d
  • Criteria (4/5)
    • Conjunctivitis (bilateral, non-exudative)
    • Oropharynx changes (strawberry tongue, erythema, perioral)
    • Cervical lymphadenopathy (unilateral, >1.5cm)
    • Rash
    • Extremity changes (erythema, edema, palm/sole involvement)
  • Incomplete (2-3 criteria)

Labs

  • CBC: Elevated WBC (neutrophil predominant)
  • Urinalysis: Sterile pyuria
  • Acute phase reactants: Elevated ESR (>40-60mm/hr), CRP (>3.0-3.5mg/dL)
  • CMP: Hyponatremia, hypoalbuminemia, hypoproteinemia, elevated transaminases
  • ECG: AV block, ischemia/infarction (aneurysm/thrombosis)
  • Echocardiography: Decreased LVEF, MR, pericardial effusion

Management

  • Hospital admission
  • IVIG (2g/kg)
  • Aspirin (80mg/kg/day)

Algorithm for the Evaluation of Kawasaki and Incomplete Kawasaki Disease3,4

Algorithm for the Evaluation of Kawasaki and Incomplete Kawasaki Disease

References:

  1. Shiari R. Kawasaki Disease; A Review Article. Arch Pediatr Infect Dis. 2014;2(1 SP 154-159).
  2. Yu JJ. Diagnosis of incomplete Kawasaki disease. Korean J Pediatr. 2012;55(3):83-87. doi:10.3345/kjp.2012.55.3.83.
  3. Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114(6):1708-1733. doi:10.1542/peds.2004-2182.
  4. Yellen ES, Gauvreau K, Takahashi M, et al. Performance of 2004 American Heart Association recommendations for treatment of Kawasaki disease. Pediatrics. 2010;125(2):e234-e241. doi:10.1542/peds.2009-0606.

Sore Throat

Evaluation of Sore Throat

Evaluation of Sore Throat

Physical Examination:

Neck
Stiffness, limitation of extension suggestive of retropharyngeal abscess.
Jaw
Trismus associated with peritonsillar cellulitis or abscess.
Oral Cavity
Dry mucous membranes suggest dehydration (from odynophagia) and indicates severity of symptoms.
Tongue elevation, sublingual/submental induration, poor dentition (particularly of mandibular molars) associated with Ludwig Angina.
Unilateral tonsillar enlargement with contralateral uvular deviation suggests peritonisllar abscess. Fluctuance may be palpated.
Tonsilar exudates suggest infectious pharyngitis (non-specific).
Palatal petechiae suggest bacterial pharyngitis.
Ulcerations of the anterior oral cavity are associated with herpes infection, lesions on the soft palate are suggestive of coxsackievirus infection.
Rarely, a grey membrane in the posterior pharynx will suggest diphtheria.
Lymphadenopathy
Tender anterior cervical lymphadenopathy may suggest bacterial pharyngitis.
Posterior cervical lymphadenopathy is associated with infectious mononucleosis.
Large, firm, non-mobile lymph nodes may suggest malignancy.
Eyes
Presence of conjunctivitis (also rhinorrhea, exanthema) associated with viral pharyngitis.
Skin
Ulcers involving the hands, feet, in addition to pharyngeal lesions suggest coxsackievirus infection.
Scarlatiniform rash associated with pharyngitis (particularly in school-age children) suggests streptococcal pharyngitis.
Abdomen
Splenomegaly is associated with infectious mononucleosis.

Centor Criteria (Modified)

  • +1: Fever
  • +1: Tonsillar Exudate
  • +1: Tender anterior cervical lymphadenopathy
  • +1: Absence of cough
  • -1: Age >45yo

Incidence of GABHS by Centor Criteria

  • 0, -1: 1%
  • 1: 10%
  • 2: 17%
  • 3: 35%
  • 4: 51%

References:

  1. Newman, D., & Shreves, A. (2013). Sore Throat. In Rosen’s Emergency Medicine – Concepts and Clinical Practice (8th ed., Vol. 1, pp. 198-202). Elsevier Health Sciences.
  2. King, B. R., & Charles, R. A. (2004). Pharyngitis In The ED Diagnostic Challenges And Management Dilemmas. Emergency medicine practice, 6(5), 1–24.

Fever

Causes of Fever

Causes of Fever

Key Features

  • Morbidity and mortality increase with age and comborbidities
  • Most common sources in elderly: respiratory, genitourinary, skin/soft-tissue
  • Atypical presentations: functional decline, altered mental status

Immediate Evaluation and Management

  • Critical Findings

    • Altered mental status
    • Respiratory distress
    • Hemodynamic instability
  • Critical Interventions

    • Airway management, supplemental O2
    • Cardiac monitoring
    • Fluid resuscitation
    • Empiric antibiotics
    • Cooling measures (T>41.0°C)

Pathophysiology of Fever

Production of endogenous or exogenous pyrogens
Increase temperature set point in hypothalamus
Patient experiences chills when core temperature < set point
Vasoconstriction, shivering causes fever
Patient experiences euthermia, though may feel malaise, fatigue
Resolution
Patient experiences sweats until core temperature returns to normal set point

References

  1. Blum, F., & Biros, M. (2013). Fever in the Adult Patient. In Rosen’s Emergency Medicine – Concepts and Clinical Practice (8th ed., Vol. 1, pp. 119-123). Elsevier Health Sciences.

Fever in Systemic Lupus Erythematosus

HPI:

48F with a history of SLE presenting with two weeks of fevers and joint pain. The patient reports progressive development of these symptoms associated with malaise and muscle aches. She also reports two days of cough productive of yellow sputum, but denies chest pain, shortness of breath or hemoptysis. She states that these symptoms are comparable to prior lupus flares but have persisted longer than usual. She has not travelled or been hospitalized recently and has no sick contacts.

PMH:

  • SLE
  • AVN left hip

PSH:

  • Total hip arthroplasty

FH:

Non-contributory

SHx:

  • Denies tobacco, alcohol or drug use.
  • Works as an accountant.
  • No history of TB exposure.

Meds:

  • Methotrexate 50mg p.o. weekly
  • Plaquenil 400mg p.o. daily
  • MVI

Allergies:

NKDA

Physical Exam:

VS: T 39.4 HR 138 RR 19 BP 97/61 O2 98% 2L NC
Gen: Alert, responsive and in no acute distress.
HEENT: PERRL, dry mucous membranes, no oropharyngeal lesions or erythema, TM intact bilaterally, no cervical lymphadenopathy, neck supple.
CV: Tachycardia, regular rhythm without additional heart sounds.
Lungs: Clear to auscultation bilaterally.
Abd: +BS, soft, non-tender, non-distended, no rebound/guarding.
Ext: No peripheral edema, extremities warm and well-perfused, diffuse tenderness to palpation and resisted range of motion of joints with particular involvement of bilateral shoulders, elbows, knees and ankles. No effusion noted, no erythema or warmth.
Neuro: Alert, oriented to self, location and time. PERRL, EOMI, facial sensation intact, facial muscles symmetric, palate rises symmetrically, tongue protrudes midline without fasciculation, peripheral sensation and motor strength grossly intact with normal gait.

Labs/Studies:

  • 0h:
  • CBC: 6.0/7.7/23.5/259
  • BMP: 138/2.8/116/18/12/0.66/71
  • Ca: 5.0 (corrected for hypoalbuminemia: 6.9)
  • Mg: 1.1
  • Lactate: 0.7
  • ESR: 81 (0-20)
  • CRP: 7.99 (0-0.74)
  • C3: 60 (79-152)
  • C4: 13 (16-38)
  • 12h:
  • BMP: 142/4.6/113/26/15/0.75/128
  • Ca: 7.8

Imaging:

  • CXR: Negative for acute cardiopulmonary process.

Assessment/Plan:

48F with a history of SLE presenting with fever and polyarticular arthralgia.

#SIRS: Fever and tachycardia in the setting of immunosuppression. The differential diagnosis includes pneumonia (bacterial, viral, less-likely fungal), which would be community-acquired. Association with polyarticular arthralgia suggests symptoms may represent lupus flare given no leukocytosis and elevated CRP.

#Hypocalcemia: Asymptomatic, likely due to hypoalbuminemia and hypomagnesemia. Improved after IV fluids and correction of hypokalemia, hypomagnesemia.

Hospital Course:

The patient was admitted and completed a 7-day course of ceftriaxone and azithromycin. Rheumatology was consulted for management of lupus flare, which included resuming home medications and a prednisone taper upon discharge.
The patient was admitted ten days later, presenting with fevers, productive cough and pleuritic chest pain. Found to have a left lower lobe sub-segmental pulmonary embolus and antiphospholipid syndrome. She was also treated empirically for healthcare-associated pneumonia with cefepime and vancomycin given fever and productive cough though there were no imaging findings suggestive of consolidation and sputum cultures were negative.

Differential Diagnosis of Hypocalcemia: 1,2

Differential Diagnosis of Hypocalcemia

Laboratory Evaluation of Hypocalcemia: 1,2

Laboratory Evaluation of Hypocalcemia

Clinical Manifestations of Hypocalcemia: 1

  • Neuromuscular
    • Hyperexcitability
    • Perioral paresthesias
    • Muscle weakness, cramps, fasciulations, tetany
  • CNS
    • Depression
    • Irritability
    • Confusion
    • Seizure
  • Cardiac
    • Decreased contractility/conduction
    • QT prolongation

Management of Symptomatic Hypocalcemia: 1

  • 10mL 10% Calcium Gluconate
  • Dilute in 100mL D5W

Fever in SLE:

It is important to differentiate whether fever in a patient with SLE is due to disease activity (flare) or active infection.

Risk Factors for Infection:3

  • Neutropenia/Lymphopenia
  • Hypocomplementemia
  • Immunosuppressive therapy (especially Azathioprine4)

Laboratory Studies:3

  • CRP: sensitivity 100%, specificity 90% >1.35mg/dL 5
  • PCT: sensitivity 75%, specificity 75% 6

References:

  1. Cooper, M. S., & Gittoes, N. J. L. (2008). Diagnosis and management of hypocalcaemia. BMJ (Clinical research ed.), 336(7656), 1298–1302. doi:10.1136/bmj.39582.589433.BE
  2. Hannan, F. M., & Thakker, R. V. (2013). Investigating hypocalcaemia. BMJ (Clinical research ed.), 346(may09 1), f2213–f2213. doi:10.1136/bmj.f2213
  3. Cuchacovich, R., & Gedalia, A. (2009). Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheumatic diseases clinics of North America, 35(1), 75–93. doi:10.1016/j.rdc.2009.03.003
  4. Zhou, W. J., & Yang, C.-D. (2009). The causes and clinical significance of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalised patients. Lupus, 18(9), 807–812. doi:10.1177/0961203309103870
  5. Kim, H.-A., Jeon, J.-Y., An, J.-M., Koh, B.-R., & Suh, C.-H. (2012). C-reactive protein is a more sensitive and specific marker for diagnosing bacterial infections in systemic lupus erythematosus compared to S100A8/A9 and procalcitonin. The Journal of rheumatology, 39(4), 728–734. doi:10.3899/jrheum.111044
  6. Scirè, C. A., Cavagna, L., Perotti, C., Bruschi, E., Caporali, R., & Montecucco, C. (2006). Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clinical and experimental rheumatology, 24(2), 123–128.

Sore Throat

Oropharynx AnatomyID:

17 year-old female presenting to the pediatric ED with sore throat for 2 days.

HPI:

The patient reports steadily worsening sore throat over the past 2 days, associated with a sensation of swelling. The pain is described as sharp, 4/10 in severity, located on the left side of her throat, and worsened with swallowing. She denies inability to swallow or difficulty breathing, she also denies fever, cough, new skin rashes or genital lesions.

She has no PMH/PSH, takes no medications, denies t/e/d use and is not currently sexually active.

PE:

  • VS: 111/65mmHg, 80bpm, 97.8°, 16/min, 100% RA
  • Gen: Well-appearing, NAD
  • HEENT: PERRL, no conjunctival injection, TM clear b/l, minimal pharyngeal erythema on left with 6mm white circular lesion on left tonsil, no tonsillar enlargement, no uvular deviation, no cervical LAD, neck supple no masses, normal neck ROM
  • CV: RRR, no M/R/G, Lungs: CTAB
  • Abdomen: +BS, soft, NT/ND
  • Ext: Warm, well-perfused, normal peripheral pulses

Assessment & Plan:

17yo female with no significant PMH with acute pharyngitis for 2 days. The most likely cause of the patient’s symptoms is viral pharyngitis, potentially herpangina (given the appearance of the tonsillar lesion). A more serious viral/bacterial pharyngitis is less likely given the absence of fever or significant erythema/exudate. There was no uvular deviation to suggest peritonsillar abscess and no evidence of airway obstruction to suggest other acute processes (epiglottitis, retropharyngeal abscess). The plan is to recommend supportive care and ibuprofen for symptomatic relief. The patient will be discharged home in good condition with precautions to return if symptoms worsen or she begins to have difficulty swallowing/breathing.

Differential Diagnosis of Acute Pharyngitis:

Acute Pharyngitis

 

Evaluation (history):

  • Respiratory distress: epiglottitis, retropharyngeal abscess, peritonsillar abscess, EBV (obstruction in or near pharynx)
  • Fatigue: infectious mononucleuosis
  • Abrupt onset: epiglottitis

Evaluation (physical examination):

  • Vesicles anterior: herpetic stomatitis, SJS, Behcet
  • Vesicles posterior: herpangina (± involvement of extremities)
  • Asymmetry: peritonsillar abscess
  • Stridor, drooling, respiratory distress: airway obstruction
  • Generalized inflammation: Kawasaki

Pediatric Fever

CXR with infiltrates

ID:

5yo girl brought to the pediatric emergency department by her mother due to 3 days of fever.

HPI:

The patient’s fever was first noted 3 days ago, measured at home to 103°F. It is associated with a moist cough, vomiting, and decreased PO intake. Her mother reports that she appears lethargic and has been urinating less frequently. The patient denies headache, changes in vision, burning with urination, or ear pain. No known sick contacts, attends day care.

PMH (Birth History):

No significant medical/surgical history. Ex-term born NSVD with no complications.

PE:

  • VS: 95/65mmHg, 100bpm, 102.6°, 22/min
  • General: Well-appearing, mildly irritated but consolable
  • HEENT: NC/AT, PERRL, oropharynx without erythema, no cervical LAD
  • CV: RRR, no M/G/R
  • Lungs: No evidence of respiratory distress (retractions, flaring), faint crackles over right inferior lung fields
  • Abd: +BS, soft, non-distended, TTP RLQ > LLQ, no rebound/guarding
  • Back: No CVAT

Labs/Imaging:

  • CXR PA/Lateral: RML/RLL infiltrate

Assessment:

5yo with 3 days persistent high fever and cough. These symptoms along with examination findings of crackles warranted further imaging (CXR) which revealed infiltrate in the right inferior lung field. The patient appeared clinically stable and was tolerating PO intake in the ED and was discharged home with azithromycin 5mg/kg/dose (with loading dose), clinic follow-up and strict return precautions.

Evaluation and Management of Pediatric Fever

Algorithm for the Evaluation of Pediatric Fever

A System for Pediatric Fever:

Pathophysiology:

Pathophysiology

Diagnosis:

  • <3mo: 38.0°C, 100.4°F
  • 3-36mo: 39.0°C, 102.2°F
  • Rectal > oral > axillary

Differential Diagnosis of Pediatric Fever:

Causes Of Fever

Serious Bacterial Illness (SBI):

1) UTI and pyelonephritis

  • Most common cause of SBI
  • Accounts for 3-8% of uncharacterized fevers
  • Female > male, uncircumcised > circumcised
  • Consider BCx, CSF evaluation as 5-10% bacteremic at presentation
  • Urinalysis: LE 75% specificity, Nitrites 97% specificity

2) Pneumonia and sinusitis

  • Sinusitis uncommon <3yo (sinuses unformed)
  • PNA diagnosed with CXR, obtain if findings of respiratory distress (grunting, tachypnea, hypoxemia) or rales on exam

3) Meningitis

  • Diagnose with LP
  • Meningitis suggested if:
    • ANC > 1,000
    • Protein > 80
    • Seizure (particularly complex febrile seizure)

Diagnosis by Age Group:

<3mo

  • Physical exam findings:
    • Tachypnea, hypoxemia → LRT infection
    • Irritability, inconsolability, bulging anterior fontanelle → meningitis
    • Vomiting/diarrhea → non-specific, GE, AOM, UTI, meningitis
  • History
    • Recent immunization: increased risk of SBI (usually UTI) 24-72h after immunization
    • Confirmed bronchiolitis (viral): enterovirus/parainfluenza associated with SBI

3-36mo

  • Physical exam findings:
    • Viral (URTI, GE) → vomiting, diarrhea, rhinorrhea, cough, rash; still playful and responsive
    • UTI → fever, foul-smelling urine, crying when urinating
    • Meningitis → irritability with handling, vomiting, bulging anterior fontanelle, complex febrile seizures

>36mo

  • Physical exam findings: presentation more adult-like
  • Watch for:
    • Group A Streptococcal pharyngitis
    • Infectious mononulceosis
    • Kawasaki: high fever (>5d), strawberry tongue, conjunctivitis, desquamating rash on palms/soles

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