Morning Report May 20, 2015

Valerie Riss, MD/MPH

HPI: Previously healthy 5yo F admitted with unilateral cervical

lymphadenitis. 12 days prior to admission developed dry cough 4 days prior to admission developed low grade fever (99-101),

nausea/emesis, neck stiffness, 2 days prior to admission developed worsening left neck pain

associated with drooling, decreased appetite, decreased range of motion.

Went to PCP, noted to be febrile to 102 with left cervical lymphadenopathy, and sent her to ED.

ED: Met shock protocol (tachycardia, tachypnea, fever, hypertensive), received fluid boluses x 2, ibprofen and CTX (2g) heart rate decreased from 155 to 110. CXR normal. Neck CT showed left anterior and posterior cervical lymphadenitis without abscess. Started on Unasyn (1200mg q6) and toradol and admitted.

Case Presentation

Admission PE: Vitals on admission to floor HD1: T 36.9, HR 114, RR 40, BP: 117/72,

95% on RA Gen: Tired, uncomfortable appearing HEENT: No conjunctival injection, TMs fine, could not open mouth due to

pain, no red/cracked lips. Enlarged and “exquisitely” tender L anterior cervical node, able to move neck through full range of motion

CV: RRR, normal S1/S2, 1/6 SEM at LUSB, no gallop, cap refill < 3 sec Lungs: breathing comfortably, clear lungs Abdomen: Tender to palpation in RUQ and epigastrum, but soft, no

masses, no hepatospenomegaly Extremities: warm and well perfused, no swelling Skin: Erythema over left neck and face, otherwise no rash, no jaundice Neuro: tired, but alert and responsive, grossly normal strength and

tone, symmetric movement of all extremities, CNs grossly intact but not specifically examined

Case Presentation

Overnight HD1 – 2: HR 130s-140s, climbed to 160s with lactate 1.6, preserved bp

HD2: In am noted to be tachycardic up to 160 with preserved blood pressure. 20/kg bolus was given (60/kg over approx 12 hours), approximately ¾ of the way through on re-checking patient noted new S3, new JVD, and hepatomegaly. Fluid bolus stopped. Good mentation, reasonable urine output. HRs did not decrease from fluid bolus.

Additional symptoms noted HD2: abdominal pain and distension, in particular RUQ

Case Presentation

PMH: Had croup 12/2014 and stayed overnight in ED No chronic medical conditions T&A 10/2014 Meds: No home meds, no antibiotics prior to admissionAllergies: NKDAImmunizations: UTD, seasonal flu vaccine receivedFamily History: negative for recurrent infections, MRSA.

Brother with history of SVT s/p ablation. No history of congenital heart disease, other history of arrhythmia, heart failure, SIDS, or sudden death.

Social history: Lives with parents and 2 older brothers, in kindergarten. Has one dog, one cat. No recent travel.

Case Presentation

CBC w/diff: WBC 25.6 (Bands 8%, Neut 83%, Lymph 5%, Mono 4%), Hgb 12.5, Hct 36.7, Plts 313,

Na 134, K 4.4, CO2 20, Glucose 76, iCa 1.23, lactate 1.6

CBG: 7.34/35/25/19/-6 ESR: 93 CRP: 11.8 VRP: negative

Admission Labs

Previously healthy 5yo female presenting on HD2 with unilateral cervical lymphadenitis, not responding to Unasyn, in compensated shock with symptoms of fluid overload, leukocytosis, elevated inflammatory markers, and RUQ pain in the setting of 12 days of cough and 4 days of low grade fever, nausea and vomiting.

One-liner

InfectiousBacterial lymphadenitis:

Staph (rarely MRSA)GASGBSOral anaerobic pathogens

Viral lymphadenitis:CMVEBV

TBTularemiaNon-tuberculous mycobacteriaHIV TularemiaToxoplasmosisSyphillisCat Scratch

GI:CholecystitisCholedocolythiasis

Rheum:Connective Tissue DisorderKawasakiLupusJIAKikuchi-Fujimoto syndromePFAPA

Hem/Onc:LymphomaLeukemiaHLH (hemophagocytic lymphocytosis)Thyroid tumorSuperior mediastinal tumor

CV:AVM (could explain right sided overload with boluses)MyocarditisDilated cardiomyopathy

Differential Diagnosis

HD2:BNP – 758CBG: 7.35/35/45/19/-5UA (clean catch): spec grav > 1.030, 1+ ketones, neg nitirites, neg hgb, trace protein, neg LE, 2 WBC, 0 RBC 1 epi, negative bacteriaRFP: Na 140, K 4.7, Cl 114, CO2 12, BUN 13, Creat 0.58, Alb 2.8, Ca 9.2, Phos 3.9 HD 4:CMP: Na 140, K 3.9, Cl 111, CO2 18, BUN 8, Creat 0.42, Ca 8.6, Prot 5.7, Alb 2.7, TB 0.4, Alk Phos 226, ALT 216, AST 194

Trends:WBC: 25.6 (HD1), 19.7 (HD5), 11.1 (HD8)Hg: 12.5 -> 9.5 -> 9.2Plts: 313 -> 277 -> 358CRP: 11.8-> 11.7 -> 8.8

Labs

ID labs: C.diff negative EBV negative CMV negative Toxoplasmosis: negative HIV negative Cat scratch Abs

(IgG/IgM) negative PPD 0mm Mycoplasma antibodies:

negative

Labs continuedHem/onc labs:

PT/INR: 15.6/1.3 Uric acid/LDH:

normal Ferritin: 241 – mildly

elevated Rheum labs:

ANA negative CPK normal C3/C4: normal

KUB on HD2: non-obstructive bowel gas pattern with possible mild ileus.

Echo on HD2: Dilated right atrium and right ventricle with flow reversal in the descending and abdominal aorta. Trace tricuspid valve insufficiency, trivial pericardial effusion

Hospital Course

HD3 – HRs increased, BPs decreased -> PICU, started on milrinone for a few hrs -> floor HD4

ID, Rheum, Cardiology consulted, lots of work up ensued

Repeat echo HD7 following rheum consult Echo HD7: Dilated distal RCA, posterior descending

artery dilatation mild, mild tricuspid valve regurg, dilated right atrium, dilated right ventricle, normal left ventricular size and function, small posterior pericardial effusion, at least moderate right pleural effusion

Follow up echo at 6 weeks – persistent right coronary artery dilatation, Z-score 1.42

Hospital Course

Temperature Curve

WHAT??

Atypical Kawasaki

Irritability almost always present Fever comes and goes, generally > 39/40 when present America: Fever >= 5 days + 4/5 of:

◦ Bilateral bulbar conjunctival injection◦ Oral mucous membrane changes, including injected or fissured

lips, injected pharynx, or strawberry tongue◦ Peripheral extremity changes

Erythema of palms/soles Edema of hands/feet Peri-ungal desquamation

◦ Polymorphous rash◦ Cervical lymphadenopathy (at least one node > 1.5cm)

Japanese criteria – fever is one of 6 criteria, require 5/6

Typical Kawasaki

CRP > 3 and/or ESR > 40Supplemental labs: Alb <=3, anemia for

age, Increased ALT, Plts >450, WBC > 15, Urine WBC > 10

Atypical Kawasaki

Japanese MR – 8/10,000 – mortality ratio 1.25 overall, 2.35 for boys with cardiac sequlae

US – in-hospital mortality 17/10,000 Peak mortality 15-45 days (combo of bad

coronary vasculitis with thrombocytosis)

Kawasaki Disease

Most common cause of acquired heart disease in children in developed countries

Other findings: pericardial effusion, pericarditis, myocarditis, valvular incompetence, cardiac failure, MI

Hyperdynamic precordium, tachycardia, gallop rhythm, innocent flow murmur, depressed myocardial contractility, low cardiac output syndrome, EKG changes – prolonged PR, non-specific ST/T wave changes

Kawasaki and the Heart

Aneurysms, coronary arteritis, decreased left ventricular contractility, mild valvular regurgitation (primarily mitral valve), and pericardial effusion.

Aneurysms rarely develop before day 10 Early echo findings of arteritis: perivascular

brightness, lack of tapering of the coronary arteries, decreased left ventricular contractility, mild valvular regurgitation (most commonly mitral), and pericardial effusion

Echo findings

Coronary Artery Aneurysm

Resolve within 6 weeksPersistent, <8mm, no stenosis Persistent, >8mm, and/or stenosis

Follow up one year Life long ASA

Life long Warfarin

Life long follow up

ASA until aneurysm resolves

Life long follow up

ArthritisAseptic meningitisPneumonitisUveitisGastroenteritisMeatitisDysuriaOtitisGallbaldder hydrops –

15%GI ischaemia

Other weird symptomsJaundiceCN palsyRenal involvementPetechial rashShock syndromeFebrile convulsionsEncephalopathyAtaxiaMacrophage activation syndromeSIADHTransient sensorineural hearing loss

Diagnostic criteria present prior to day 5 CAA or coronary dilatation are present Persistently elevated inflammatory markers

with no other explanation

When to Treat

KD

No treatment15-25%CAA

ASA17% CAA

Low Dose IVIG11% CAA

High Dose IVIG4.4% CAA

Single Dose2.4% CAA

Rigors (18%) Pruritius (4%) Urticaria Nausea Hypotension Many many more

Side effects of IVIG

Persistently elevated CRP or lack of defervescence despite IVIG, liver dysfunction, hypoalbuminaemia, anemia, HLH, shock

20% are resistant to IVIG Additional therapies – Steroids, TNF-alpha

inhibitors, other monoclonal Abs, plasma exchange, cytotoxic agents

Markers of high severity

Fever >= 7 days unexplained – get labs to evaluate

Patients of any age with unexplained fever >= 5 days and only 2-3 clinical criteria

If there is periungual desquamation after resolution of the fever in someone not meeting epidemiologic criteria for KD

When to Eval

Abdominal CT on HD 5: Mild hepatomegaly, moderate pelvic fluid, large right pleural effusion, small left pleural effusion, possible fecalith right lower abdomen

Neck ultrasound HD5: no fluid collection Echo HD7: Dilated distal RCA, posterior

descending artery dilatation mild, mild tricuspid valve regurg, dilated right atrium, dilated right ventricle, normal left ventricular size and function, small posterior pericardial effusion, at least moderate right pleural effusion

Additional Imaging

Uptodate Newberger et al. Diagnosis, Treatment and

Long-Term Management of Kawasaki Disease. Circulation 2004: 110: 2747-2771

Eleftheriou et al, Management of Kawasaki Disase. Arch Dis Child 2014; 99; 74-83

Dorongpiskitkul et al. The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and Immunoglobulin treatment. Pediatrics 1995 Dec; 96 (6): 1057-61

References