Kawasaki Disease, (Mucocutaneous Lymph Node Syndrome), Pregnancy, and Hereditary Risks

kawasaki diseaseThe study, “Pregnancy in women with a history of Kawasaki disease: management and outcomes”, published March 6th 2014 in BJOG adds an important piece of information to our understanding of this disease.  Although a small study (10 women with 21 children) these 21 pregnancies resulted in two children developing Kawasaki’s, or a 10% chance of your child developing Kawasaki’s disease, if you, the mom, had Kawasaki’s in the past. The good news was that all the moms did well with their pregnancies. 

So why should you care about this obscure disorder?  Mostly because if you figure out your child has this and they get treatment within the first week to 10 days or so, (intravenous immune globulin), you significantly reduce the risk of your child ending up with coronary aneurysms (balloon-like swellings of the aorta, which can be fatal).  Immune globulin and low dose aspirin will reduce the risk from 25% to 5%.  It is for this reason that  physicians and parents should think of this when there is a prolonged unexplained high fever, especially if there are other findings typical of Kawasaki.  

The diagnosis is made when there are several days of high fever and four of five criteria are met:  rash, red lips or tongue, red bulbar conjunctivitis (the white part of the eye is red), swollen lymph nodes, and swollen hands and feet.  The peeling of hands and feet tends to come later and the tips of the fingers can even peel. Often urine will have white cells but not be infected (sterile pyuria) and ESR and CRP (markers of inflammation) are very elevated.  The challenge in diagnosis comes when you have just a few of these symptoms.  It can be confused with other infections or malignancy, and in those tough cases where it is not classic, if you have access to an infectious disease expert, then this is an appropriate consult. Because the treatment with IV immune globulin generally requires hospitalization and is very expensive, this treatment is not done without reasonable suspicion that this is what you are dealing with.  Once the diagnosis is suspected, consider a cardiology consult as well. Usually the cardiac findings are 2-3 weeks out or later from the onset of the disease, but a recent atypical case in my practice suggests that when in doubt, get the cardiology and infectious disease consults and consider treatment if the fever is relentless and the inflammatory markers really high.  

Dr. Kawasaki first published a paper in 1967  titled, “Infantile acute febrile mucocutaneous lymph node syndrome with specific desquamation of the fingers and toes. Clinical observation of 50 cases”. This was the first report on what is now called Kawasaki disease. Below, is a link to  “The new history of Kawasaki”, article by Dr. Kawasaki. 
The cause is still not known, but it appears to have an immune system over-activation.  Some think there may be an unknown viral cause, although that seems less and less likely as so many cases have had viral cultures and tests sent over the decades.  This is a disorder we are seeing more and more often, so it may somehow be related to our modern world.  When I think of things to balance the immune system, here are a few of the things I would do and recommend to all my patients and family:

1.  Take Vitamin D3.   I recommend 1000 IUs for newborns up to 40 lbs, 2,000IUs for school age children,  and 5000 IU for teens and adults. Stay faithful on these and check your Vitamin D levels.

2.  Eat organic and drink filtered water (avoiding toxins is a huge part of staying healthy in our world today).

3.  Take probiotics and eat fermented foods.  The good bacteria in our GI track have a lot to do with a good and balanced immune system.

4.  Get enough Omega-3. This can be in the form of flax oil, fish oil (purified), or (in moderation) low toxin fish like salmon.

5.  Reduce stress in your life.  High stress raises cortisol, not good for your immune system.

6.  If you have the option to do so (offered at Integrative Pediatrics and Natura) get your micronutrients tested so you can replace deficiencies.

7.  If you are an adult, consider getting your hormones checked. This is offered at Natura and can be found here…

Pregnancy in women with a history of Kawasaki disease: management and outcomes

The (New) History of Kawasaki Disease

Diagnosis and classification of Kawasaki disease

 

Dr. Paul

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