Understanding Kawasaki Disease in Children

 

Kawasaki disease (KD) is named after the Japanese pediatrician Tomisaku Kawasaki who in 1967 described 50 cases of infants with persistent fever, accompanied by rash, lymphadenopathy, edema, conjunctival injection, redness and cracking of the lips, "strawberry tongue," and convalescent desquamation.

Kawasaki Disease, also known as mucocutaneous lymph node syndrome, is a self-limited medium-sized vasculitis that primarily affects children. It is the leading cause of acquired heart disease in children in developed countries. Let’s break down the key points:

Classic KD (Typical KD)

Clinical diagnosis based on at least 4 of 5 clinical criteria.

Typically occurs in children aged 6 months to 6 years.

Criteria include:

• Fever lasting ≥ 4 days.
• Presence of at least 4 of the following features:
• Polymorphous rash (desquamation, morbilliform, erythema multiforme).
• Cervical lymphadenopathy (usually unilateral, ≥ 1.5 cm).
• Changes in lips/oral mucosa (strawberry tongue/ulcerations).
• Extremity changes (swelling, erythema).
• Nonexudative conjunctivitis with limbic sparing.

Diagnosis is clinical, but lab studies (elevated white blood cell count, inflammation markers, liver enzymes) can assist.

Incomplete KD (Atypical KD)

Challenging diagnosis.

Patients lack atypical features but have fewer classic features.

Criteria: 5 days of fever and 2-3 clinical features.

Infants ≤ 6 months are at higher risk for coronary artery lesions.

Treatment and Timing

Intravenous immunoglobulin (IVIG) reduces coronary artery disease risk (from 25% to 4%) if appropriately timed.

Early treatment is essential to prevent cardiac abnormalities.

Emergency Care of Kawasaki Disease (KD)

Patients often arrive in the emergency room with prolonged fevers. A thorough history and physical exam are crucial to differentiate KD from other illnesses.

Classic KD (Typical KD):

Diagnosed clinically.

Emergency center (EC) management involves obtaining labs, echocardiogram, and administering intravenous immunoglobulin (IVIG).

Incomplete KD (Atypical KD):

More challenging to diagnose.

Patients without elevated inflammatory markers are less likely to have KD, but clinical judgment matters.

Clinical Criteria for Classic KD:

Fever lasting ≥ 4 consecutive days (measured over 38°C).

At least 4 clinical symptoms suggestive of KD (e.g., mucositis, rash, large lymph nodes).

Fever alone doesn’t confirm Classic KD.

Other Considerations:

Viral and bacterial illnesses (e.g., influenza, adenovirus, strep pharyngitis) can trigger KD.

Age matters: Classic KD usually occurs between 6 months and 6 years, while Incomplete KD can happen at any age.

Consult an Infectious Disease specialist if the diagnosis is uncertain.

Multisystem Inflammatory Syndrome in Children (MIS-C)

In 2020, institutions noted severe inflammatory syndrome resembling KD in pediatric patients with recent COVID-19 infection.

MIS-C should be considered in patients with KD-like features and a history of COVID infection.

Inpatient management for Kawasaki Disease (KD)

Primary Treatment:

IVIG (Intravenous Immunoglobulin):

Administer high-dose IVIG (2g/kg) as a single infusion within 10 days of illness onset.

Do not delay treatment for echocardiogram or specialty consultation.

IVIG Beyond 10 Days:

Generally avoid administering IVIG beyond the tenth day of illness unless specific criteria are met:

Persistent fever without other explanation.

Coronary artery abnormalities with ongoing systemic inflammation (elevated ESR or CRP >3 mg/dL).

Avoid Routine Methylprednisolone with IVIG:

Single-dose pulse methylprednisolone is not recommended as routine primary therapy.

Consider Corticosteroids for High-Risk Patients:

Consider longer corticosteroid course (tapering over 2–3 weeks) along with IVIG and Aspirin (ASA) for high-risk patients.

High-risk criteria: infants <6 months, patients with large or giant aneurysms.

No consensus on prediction scores for IVIG resistance in North America.

ASA Administration:

Reasonably administer moderate (30–50 mg/kg/day) to high-dose (80–100 mg/kg/day) ASA until the patient is afebrile.

Evidence does not conclusively show reduction in coronary artery aneurysms.

Transition to Low-Dose ASA:

Consider transitioning to low-dose ASA (3-5 mg/kg once daily) upon discharge or when the patient returns to baseline.

If no improvement within 7 days, immediate follow-up with a physician is recommended.

IVIG-resistant patients in the context of Kawasaki Disease (KD)

IVIG Resistance:

Defined as persistent or recrudescent fever at least 36 hours and <7 days after the first IVIG infusion.

Treatment options:

Second IVIG Infusion:

Administer pooled polyclonal IG (2 g/kg) intravenously.

IVIG + Steroid:

Administer IVIG (2 g/kg) intravenously along with methylprednisolone (2 mg/kg/day) intravenously divided every 8 hours until afebrile.

Then switch to oral prednisone or prednisolone until C-reactive protein (CRP) normalizes, followed by a taper over 2–3 weeks.

Infliximab:

Monoclonal antibody against TNF-α.

Single infusion: 5 mg/kg intravenously over 2 hours.

Additional Options:

Cyclosporine:

Consider in refractory KD patients who have failed other treatments.

Immunomodulatory Monoclonal Antibodies (except TNF-α blockers), Cytotoxic Agents, or Plasma Exchange:

Rarely considered for highly refractory patients who haven’t responded to previous treatments.

Echocardiography Recommendations

Perform echocardiography when considering KD diagnosis.

Unavailability or technical limitations should not delay treatment.

Image coronary arteries and assess luminal dimensions using Z scores adjusted for body surface area.

Classification of coronary artery abnormalities:

• No involvement: Always Z-score <2.
• Dilation only: Z-score 2 to <2.5.
• Small aneurysm: Z-score >2.5 to <5.
• Medium aneurysm: Z-score >5 to <10 with absolute dimension <8 mm.
• Large or giant aneurysm: Z-score >10 or absolute dimension >8 mm.

Positive Echocardiogram for Kawasaki Disease (KD) Includes:

Left anterior descending (LAD) or right coronary artery Z-score >2.5.

Any coronary artery aneurysm.

Three or more of the following:

Decreased left ventricular function.

Mitral regurgitation.

Pericardial effusion.

Left anterior descending (LAD) or right coronary artery Z-score between 2 and <2.5.

Note that other cardiac lesions associated with KD (e.g., valvular disease, myocarditis) should be discussed with cardiology and infectious disease specialists. 

Discharge Criteria & Follow-Up

Patient should be afebrile for >24 hours and show improved clinical status (less irritability, adequate hydration, and voiding).

Ensure all medications are sent to the pharmacy in advance.

Arrange consultations and follow-up appointments.

Recommend influenza vaccine (unless contraindicated) to prevent Reye’s Syndrome.

Avoid ibuprofen in children with coronary aneurysms taking aspirin.

Address transportation issues for families.

Ensure family understands follow-up instructions and when to seek immediate medical attention.

No live vaccines within 11 months after IVIG administration (e.g., MMR, varicella).

Follow-up:

Primary Care Physician within 1 week.

Cardiology outpatient follow-up (echocardiogram) in 1-2 weeks.

Infectious Disease follow-up in 1-2 weeks.

Documentation

Document concerns for KD:

Note if the patient is at higher risk (e.g., infants ≤ 6 months).

Explain why the patient is considered at higher risk (e.g., age-related factors).

Consider Other Diagnoses:

1. Dehydration:

Document at least 2 symptoms related to dehydration (e.g., capillary refill time, absent tears, dry mucous membranes, weight loss).

2. Acute kidney injury:

Consider and document any signs or symptoms suggestive of kidney dysfunction.

3. Transaminitis:

Be alert for elevated liver enzymes and document accordingly.

(Note: Please follow your hospital protocol for the diagnosis and management of Kawasaki Disease. The information in this blog is derived from the references below)

References:

https://www.cdc.gov/kawasaki/about/index.html

Owens AM, Plewa MC. Kawasaki Disease. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537163/

LiJiang MDa†, et al COVID-19 and multisystem inflammatory syndrome in children and adolescents, The Lancet Infectious Diseases, Volume 20, Issue 11, November 2020, Pages e276-e288

McCrindle, B., Rowley, A., Newburger, J., et al. (2017). Diagnosis, treatment, and long-term management of Kawasaki disease: A scientific statement for health professionals from the American Heart Association. Circulation, 135, e927. http://circ.ahajournals.org/content/early/2017/03/29/CIR.0000000000000484

Newburger, J., Takahashi, M., Gerber, M., et al. (2004). 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. Circulation, 110(17), 2748. http://circ.ahajournals.org/content/110/17/2747.ful

Management of Pyloric Stenosis- Pediatric Nursing

  Pyloric Stenosis Overview:

Pyloric stenosis is a common cause of persistent vomiting in infants during their first weeks of life.

It typically presents between 2 and 8 weeks of age but can appear as late as 12 weeks.

The exact cause is unknown, but genetic and environmental factors are believed to play a role.

Males are more commonly affected than females.

The hallmark symptom is projectile, non-bilious vomiting.

Bile in the vomit rules out pyloric stenosis as the cause.

Definition:

Hypertrophic pyloric stenosis (HPS) results from thickening of the pyloric muscles, narrowing the pyloric canal.

It’s the most common surgical cause of vomiting in infants.

From: Pyloric Stenosis jpg.jpg (1280×733) (kidshealth.org.nz)

Symptoms:

Progressive projectile vomiting after feeding (typically non-bilious).

An upper abdominal mass or visible gastric wave may or may not be present.

Untreated vomiting can lead to dehydration, hypochloremia, hypokalemia, and metabolic alkalosis.

Epidemiology:

More common in males and firstborn children.

Lower incidence in African and Asian populations.

Differential Diagnosis:

Consider other conditions like overfeeding, gastroesophageal reflux, malrotation, duodenal stenosis, and intracranial lesions.

Management:

Suspected Pyloric Stenosis:

1. Perform a basic metabolic panel (BMP).

2. Ensure the patient is nil per os (NPO).

3. Administer a Normal Saline bolus.

4. Conduct a pyloric ultrasound.

5. Consider repeat ultrasound or upper GI contrast study if inconclusive.

Confirmed Pyloric Stenosis:

1. Consult with pediatric surgery.

2. Review serum electrolyte results.

3. Place a nasogastric tube.

4. Monitor the infant closely.

Emergency Considerations:

Pyloric stenosis is a medical emergency.

Correct electrolyte imbalances before surgery.

Rehydration protocols depend on clinical condition.

Inpatient Management for Pyloric Stenosis:

Pre-Surgery:

Ensure the patient is fully hydrated and correct electrolyte imbalances.

Surgical intervention (pyloromyotomy) is necessary.

Pyloromyotomy can be performed openly or laparoscopically.

Post-Surgery:

Feeding can resume within two hours after the procedure.

Pain management with Tylenol only.

Feeding regimen at the discretion of the attending physician (usually breastmilk or Enfamil).

Some initial vomiting is common; parents should be reassured unless advised otherwise.

Discharge:

Expect discharge on postoperative day (POD) 1 or 2.

Documentation Recommendations:

Document any electrolyte or acid-base abnormalities.

Note presence and severity of dehydration at admission.

Document shock status at admission.

Note: Always follow your hospital protocol for managing patients with pyloric stenosis

References: 

Dalton BG, Gonzalez KW, Boda SR, Thomas PG, Sherman AK, St Peter SD, Optimizing fluid resuscitation in hypertrophic pyloric stenosis. J Pediatr Surg. 2016 Aug; 51(8):1279- 82

Jobson M, Hall N, Bchir MB, Contemporary management of pyloric stenosis. Seminars in Pediatric Surgery 2016 Aug; 25(4):219-224. 

Markel TA, Scott MR, Stokes SM, Ladd AP, A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. J Pediatr Surg. 2017 Apr;52(4):534-539  

Understanding Cardiomyopathies: Definition, Diagnosis, Treatment, and Prognosis

 

Definition:

Cardiomyopathy refers to diseases of the heart muscle that affect its size, shape, or structure, leading to impaired function. These conditions can lead to heart failure, arrhythmias, and other serious cardiac issues.

Types:

1. Dilated Cardiomyopathy (DCM):

   • The heart's ventricles enlarge and weaken, reducing the heart's ability to pump blood efficiently.
   • Common causes include genetic factors, infections, and toxins such as alcohol.

2. Hypertrophic Cardiomyopathy (HCM):

   • The heart muscle thickens abnormally, often leading to obstructed blood flow.
   • It is usually inherited and can cause sudden cardiac death, especially in young athletes.

3. Restrictive Cardiomyopathy (RCM):

   • The heart muscle becomes rigid and less elastic, preventing proper filling of the heart chambers.
   • It is often associated with conditions like amyloidosis and can result from radiation therapy or connective tissue disorders.

4. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC):

   • Fat and fibrous tissue replace the muscle of the right ventricle, leading to arrhythmias.
   • This type is typically genetic.

Fig. 1 Cardiomyopathies according to pathophysiologic types a. normal, b. dilated, c. hypertrophic (obstructive), d. hypertrophic (non-obstructive), e. hypertrophic (obliterative), f. restrictive (obliterative), g. restrictive (non-obliterative) (From: Overview of Cardiomyopathies - Cardiovascular Disorders - Merck Manual Professional Edition (merckmanuals.com) )

Diagnosis:

• Medical History and Physical Examination: Key to identifying symptoms and risk factors.
• Electrocardiogram (ECG): Detects electrical activity abnormalities.
• Echocardiogram: Visualizes heart structure and function.
• Cardiac MRI: Provides detailed images of heart tissues.
• Blood Tests: Identify markers of heart damage or underlying conditions.
• Genetic Testing: Important for detecting inherited forms of cardiomyopathy.
• Biopsy: In some cases, a small sample of heart tissue is examined.

Prognosis

The prognosis for patients with cardiomyopathy varies widely depending on several factors, including the type and severity of the cardiomyopathy, the patient's overall health, the presence of other medical conditions, and how well the condition is managed. Here is an overview of the prognosis for different types of cardiomyopathy:

1. Dilated Cardiomyopathy (DCM):

   • Prognosis: The prognosis can vary significantly. Some patients may remain stable for many years with appropriate treatment, while others may experience progressive heart failure.
   • Factors Influencing Prognosis: Severity of symptoms, response to treatment, presence of complications such as arrhythmias, and whether the underlying cause (e.g., alcohol use, viral infection) is reversible.

2. Hypertrophic Cardiomyopathy (HCM):

   • Prognosis: Many people with HCM live normal lifespans with few symptoms. However, there is a risk of sudden cardiac death, particularly in younger individuals and athletes.
   • Factors Influencing Prognosis: Family history of sudden cardiac death, severity of left ventricular hypertrophy, presence of arrhythmias, and adherence to treatment and lifestyle recommendations.

3. Restrictive Cardiomyopathy (RCM):

   • Prognosis: This type generally has a poorer prognosis compared to other forms of cardiomyopathy due to its association with underlying conditions such as amyloidosis or other systemic diseases.
   • Factors Influencing Prognosis: The underlying cause of the restrictive cardiomyopathy, the extent of heart damage, and the patient's response to treatment for the underlying condition.

4. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC):

   • Prognosis: The prognosis can be quite variable. Some individuals may experience only mild symptoms, while others may develop severe heart failure or life-threatening arrhythmias.
   • Factors Influencing Prognosis: The presence of arrhythmias, the extent of right ventricular involvement, and adherence to treatment including the use of ICDs to prevent sudden cardiac death.

General Factors Influencing Prognosis:

• Early Diagnosis and Treatment: Early detection and management of cardiomyopathy can improve outcomes significantly.
• Adherence to Treatment: Following prescribed medications, lifestyle modifications, and regular follow-ups with a healthcare provider.
• Management of Comorbid Conditions: Controlling conditions such as hypertension, diabetes, and coronary artery disease.
• Access to Advanced Therapies: Availability of advanced treatments such as heart transplantation or gene therapy can impact prognosis.

Here is a table comparing the three main types of cardiomyopathies: Dilated Cardiomyopathy (DCM), Hypertrophic Cardiomyopathy (HCM), and Restrictive Cardiomyopathy (RCM).

Feature	Dilated Cardiomyopathy (DCM)	Hypertrophic Cardiomyopathy (HCM)	Restrictive Cardiomyopathy (RCM)
Definition	Enlarged and weakened ventricles	Abnormally thickened heart muscle	Rigid and less elastic heart muscle
Primary Cause	Genetic, viral infections, toxins	Mostly genetic	Often related to systemic diseases
Symptoms	Fatigue, shortness of breath, edema	Chest pain, shortness of breath, palpitations	Shortness of breath, fatigue, edema
Heart Chamber Affected	Mainly left ventricle	Typically left ventricle	Both ventricles
Heart Function	Reduced ejection fraction	Often preserved ejection fraction but with outflow obstruction	Preserved ejection fraction but impaired filling
Risk of Arrhythmias	High	High	Moderate to high
Common Complications	Heart failure, arrhythmias, thromboembolism	Sudden cardiac death, heart failure	Heart failure, arrhythmias
Diagnostic Tools	Echocardiogram, MRI, ECG, blood tests, genetic testing	Echocardiogram, MRI, ECG, genetic testing	Echocardiogram, MRI, ECG, biopsy
Treatment Options	Medications, lifestyle changes, ICD, heart transplant	Medications, lifestyle changes, ICD, septal myectomy	Medications, lifestyle changes, treat underlying cause, heart transplant
Prognosis	Variable; can be stable or progressive	Generally good with treatment, but risk of sudden death	Poorer prognosis due to underlying conditions
Lifestyle Modifications	Avoid alcohol, low-sodium diet, regular exercise	Avoid intense exercise, maintain healthy diet	Low-sodium diet, regular but moderate exercise

Treatment:

• Medications:
  • Beta-blockers, ACE inhibitors, or ARBs to reduce heart strain.
  • Diuretics to manage fluid retention.
  • Anti-arrhythmic drugs to control irregular heartbeats.
• Lifestyle Changes:
  • Dietary modifications, exercise, and avoiding alcohol.
• Implantable Devices:
  • Pacemakers or Implantable Cardioverter Defibrillators (ICDs) to regulate heartbeat.
• Surgery:
  • Septal myectomy (removal of thickened heart muscle) in severe HCM.
  • Heart transplant in advanced cases.
• Gene Therapy: Emerging treatments aimed at addressing underlying genetic causes.

In summary, while cardiomyopathy can be a serious condition, many patients manage well with appropriate treatment and lifestyle changes. The prognosis is highly individualized and depends on a combination of factors specific to each patient. Early diagnosis and treatment are crucial to managing cardiomyopathy and improving the quality of life and prognosis for affected individuals. Regular follow-up with a cardiologist is crucial for monitoring the condition and adjusting treatment as necessary.

Just for fun: Emergency Department Roadsigns and Acronyms

I have worked in the Emergency Room for 18 years as a staff nurse and an educator. If you are an ER nurse, you know you will not survive if you don’t have any sense of humor. No, we do not laugh at the patients. We usually laugh at the situation and we enjoy reading funny posts about the ER like the ones below:

Just for fun: Emergency Department Roadsigns and Acronyms

from "The Back Passage" 

by Ian Miller, the impactednurse.com

Too much information! At least that’s what it seems like on any given second in the Emergency Department. What with all those flyers on the walls and all those medication sheets and special orders and acronyms and abbreviations. What we need is a roadmap to guide us through this sensory overload. Our own department has begun using some specialized signage to clarify information and highlight patient needs. Here are a few of the more important ones:

Smelly bedpan. 

Danger: nurse carrying extremely smelly bedpan at arms length. Whenever you see a nurse walking in this manner take immediate evasive action. 

Urinal hazard.

Do not carry urinal in this position.Walking around for the remainder of

the shift with squishy shoes is to say the least: sub-optimal.  

Sleep deprived nurse.

Danger: nursing staff have worked too many consecutive night shifts Divergent gaze is causing double vision. Brain gone bad. 

Fecal loading hazard.

This patient has not had their bowels open for a very long time.

But, they have just had an enormously potent enema.

Avoid contact at all costs. Transfer to ward ASAP. 

Failure to launch. 

This patient is in need of Viagra.If unable to obtain from pharmacy may substitute: madixafloppin or macoxafillin. 

Triage Nurse Hazards.

Do not come to the Emergency Department with that splinter in your thumb. Go to your own doctor or get it out with a pair of tweezers you big pillow. 

Extreme Triage Nurse Hazard.

If you have amputated your hands or feet, do not enter the emergency department dripping or squirting blood over the newly cleaned floors

Catheter insertion hazard. 

This patient needs a large bore catheter passed. He is intoxicated, angry and has the words LOVE and HATE tattooed on either side of his penis. The doctor asks you to pass the catheter.

Caution with Catheter Insertion.

This person has not passed urine for 48 hours. Their bladder is the size of the Hindenburg and she is ready to blow. 

High wind speeds. 

Caution. High velocity flatus in this area. Patient has habit of *busting a grumpy* every time they roll over cough or think nobody is watching.

Rectal Foreign Body

This patient has inserted a beetroot far up into their descending colon. Listen to their explanation with a straight face. Pass patient off to student nurse. 

Found objects. 

Do not give little yellow tablets that you find lying on the floor to your patients. 

Nurse taking a shower. 

Nothing will pour iced water on those flames of passion like a subtle musky aroma of Melena wafting between you. Or the fragment top notes of a semi-digested Quarter Pounder with Cheese hanging in the air. Make a bee line from the front door to the shower and scrub-a dub dub.

Emergency Room Accepted Acronyms 

Documentation is a most important aspect of your craft as an Emergency Department nurse. The ability to communicate fluently and accurately is not only an essential competency, it is a legal fingerprint of the care you deliver. So… you should make your charts, history’s and notes a justifiable reflection of the quality care you deliver. 

With this foremost in our minds, we present the following list of useful acronyms to add to your literary kitbag. Use them to squeeze some vital juices into your notes. Use them to add an objective and accurate sub-context to your reporting. Use them to tell it like it is. Use them at your peril. 

AOX3 

Alert, Oriented times 3 (person, place, time). 

AFOL 

All fine on leaving. 

AGA 

Acute Gravity Attack. (Patient fell over) 

ART 

Assuming Room Temperature. (dead) 

AWOL 

All Well On Leaving.

AWTF 

Away With The Fairies. 

BBSS 

Big Boobs, See Soon. 

BUNDY 

But Unfortunately Not Dead Yet. 

BVA 

Breathing Valuable Air. 

BWS 

Beached Whale Syndrome.

CTD

Circling the Drain/Close To Death.

DTS

Danger To Shipping.

DRT

Dead Right There.

DRTTTT

Dead Right There, There, There, and There.

FITH

F****d In The Head.

FLK

Funny Looking Kid.

FND

Friggin’ Nearly Died.

FRACS

Fornicates Regularly And Chain Smokes.

GAK

God Alone Knows.

GOMER

Get Out of My Emergency Room.

HIVI

Husband Is Village Idiot.

NFN

Normal For Nurses.

NOONG

Not One Of Nature’s Gentlemen.

NQR

Not Quite Right.

NYDN

Not Yet Diagnosed - Nervous.

OSINTOT

Oh Shit I Never Thought Of That.

PAFO

Pissed And Fell Over.

PANIC

Pressured And Not In Control. Descriptive

and useful acronym for all sorts of situations.

A reminder also that pressure alone does

not produce panic - it’s whether you can

control it.

PRATFO

Patient Reassured And Told to F**k Off.

SIG

Stroppy Ignorant Git.

SOB

Shortness Of Breath.

STIO/SIO SupraTentorial in Origin.

(psychosomatic)

TATT

Tired All The Time.

TEETH

Tried Everything Else?..Try Homeopathy.

TOBASH

Take Out Back And SHoot.

TTFO

Told To F**k Off.

TUBE

Totally Unnecessary Breast Examination.

TWOFT

Total Waste Of Frigging Time.

UBI

Unexplained Beer Injury.

UNIVAC

Unusually Nasty Infection; Vultures Are Circling. 

Source: impactednurse.com

(Note: Thank goodness I saved this article because I used to show it to my ER friends a long time ago. I wanted to reach out to the writer before posting this but his website is no longer active. So credit goes to nurse Ian Miller.)