Irukandji SyndromeIsabel M. Algaze Gonzalez, MD
Assistant Professor of Emergency Medicine
Wilderness Medicine Fellowship Co-Director
University of California, Irvine
Physician at Catalina Island Medical Center
Avalon, Catalina
Funded by: U54MD008149RTRN, NIMHDPI Angel Yanagihara
Objectives
• Brief bio-chemical review
• Pathophysiology
• Clinical presentation
• Most recent treatment strategies
No disclosures
Why is this important to you? -Goals
• You can not diagnose what, to your knowledge, does not exist
• Increasingly prevalent around the world coastal areas
• Need to prevent:• Misdiagnosis
• Underreporting
• Improper treatment strategies
Why is this important to you? -Goals
• Has a great burden of morbidity and mortality
• Associated financial costs are high
Credit: Dianna Emmanuelli
Jellyfish Background Information
Distribution
Alatina alata Nematocyst
Sting Mechanism
What do we know
• Severity increases with:
• Dose• Jellyfish size
• Extent of contact
• Delicacy of the victim's skin
• Fish have nucleated cells• Capability to repair makes them 1,000 times more resistant than humans
• Nematocysts Injects Spines • Mineralized Chitin- Triggers Mannose protein mediated prolonged immune response
• Porin • Approximately 1% if the venom
• Causes holes in cells
• Platelets are affected first• Releases catecholamines
• Anthrax porin (anthrolysin O) as a model• Copper (better than zinc) Gluconate inhibits Calcium mediated porin assembly
• Death • Marker of death is potassium
• After well intentioned epinephrine
King Slayer, Carukia
barnesi, Malo kingi
Chironex fleckeri
Literature
Pathophysiology of cubozoan envenomation
Clinical presentation
• 1 to 2 hours after the sting• May not have cutaneous marks
• Cutaneous:• May progress over a course of days to local necrosis, skin
ulceration, and secondary infection• Can resolve 2w-2mo• Residual hyperpigmentation to lichenification
• Ocular: • Conjunctivitis, chemosis, corneal ulcers, corneal
epithelial edema, keratitis, iridocyclitis, elevated intraocular pressure, synechiae, iris depigmentation, chronic unilateral glaucoma, and lacrimation
• Systemic:• “Feeling of doom”, Trembling, Acute renal failure,
lymphadenopathy, chills, fever, and nightmares
Credit: Dianna Emmanuelli
Clinical presentation
• Neurologic: • Malaise, headache, aphonia, diminished touch and temperature sensation, vertigo, ataxia, spastic or
flaccid paralysis, mononeuritis multiplex, Guillain-Barré syndrome, parasympathetic dysautonomia, plexopathy, radial–ulnar–median nerve palsies, brainstem infarction, delirium, loss of consciousness, convulsions, coma, and death
• Cardiovascular: • Hemolysis, hypotension, small artery spasm, bradyarrhythmias, tachyarrhythmias, elevated serum
troponin I level in the absence of myocardial injury, vascular spasm, deep venous thrombosis, thrombophlebitis, acute myocardial infarction, congestive heart failure, and ventricular fibrillation
• Respiratory: • Rhinitis, bronchospasm, laryngeal edema, dyspnea, cyanosis, pulmonary edema, and respiratory failure
• Musculoskeletal or rheumatologic: • Abdominal rigidity, diffuse myalgia and muscle cramps, muscle spasm, fat atrophy, arthralgias, reactive
arthritis, and thoracolumbar pain
• Gastrointestinal: • Nausea, vomiting, diarrhea, paralytic ileus, dysphagia, hypersalivation, and thirst
Treatment
Clinical trial and piglet model
• Collaboration • DOD
• Puerto Rico Sea Grant
• Hawaii Sea Grant
• NIH grant
Treatment
• ACLS/BLS protocols acutely
• Anticipate drowning
• Hypothermia
• Decompression sickness or arterial air embolism
• If only local symptoms:
• Treat symptomatically-• Vinegar and then hot water
• Sting no More products
• Observe until the victim is asymptomatic for 6 hours*
• Antivenom vs inhibitor • Technical limitations
Treatment
• What NOT to do:
• Rinse with fresh water
• Rinse with cold water
• Apply ice
• Scrape with a credit card
• Shave with shaving cream
• Rub sand
• Rinse with urine
• Rinse with alcoholic drinks or rubbing alcohol
• Touch with bare hands
• Administer Epinephrine
Literature
Literature
Without blood skin agar With skin blood agar
Prevention
• Beach monitoring
• Flag system and beach signs
• Bay Watch and EMS most have updated protocols
• Monitoring of victims*
• Poison center reporting
Credit: Dianna Emmanuelli
Management
• If symptomatic:
• Monitor- some end up admitted to ICU
• Opioids – spasm pains, careful of pulmonary edema
• Nausea medication
• Phentolamine- Alpha blockers have been recommended for the control of hypertension, as a result of catecholamine release.
• Mag- for arrhythmias?
Draft by Irukandji clinical working group
You have an expert in your backyard
Please forward cases and pictures to :
Angel A. Yanagihara, Ph.D.
Associate Research Professor
Bekesy Laboratory of Neurobiology, PBRC
and Dept of Trop Med, JABSOM
University of Hawaii at Manoa
1993 East West Road
Honolulu, Hawaii 96822 USA
Email [email protected]
Tel 808 956-8328
FAX 808 956-8713
http://manoa-hawaii.academia.edu/AngelYanagihara
https://www.researchgate.net/profile/Angel_Yanagihara
Isabel M. Algaze Gonzalez, MD
Assistant Professor of Emergency Medicine
Wilderness Medicine Fellowship Co-Director
University of California, Irvine
333 City Boulevard West, 640, Orange, CA, 92868
Physician at Catalina Island Medical Center
Avalon, Catalina
100 Falls Canyon Rd, Avalon, CA 90704
Email [email protected]
Tel 714 456-3713
FAX 714-456-3714
http://www.ucirvinehealth.org/find-a-doctor/a/isabel-m-algaze-gonzalez
For the future
• More research is needed on
• Species
• Distribution
• Syndrome
• Variations in signs and symptoms between species
• Validated Treatment
• Prevention strategies
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