Heart Attacks in Children? The Kawasaki Disease Epidemic

A recent study out of Australia published in the Journal of Pediatrics revealed that cases of the potentially deadly Kawasaki Disease are steadily increasing. Kawasaki Disease (KD) is a poorly understood condition known as vasculitis, which is the inflammation of small blood vessels. The disease is known for attacking the coronary arteries of children, the vital blood vessels supplying the heart muscle with oxygen and other nutrients. The child suffers from an acute fever and severe pain, essentially symptoms of both infection and a heart attack.

Doctors do not know the cause of KD, but they believe it may be infectious in origin because most cases occur between the winter and spring, and epidemics occur frequently.  KD most commonly affects Japanese children and those of Japanese ancestry: the incidence in Japan has been steadily increasing, from 102 per every 100,000 children <5 years old to 188 in 2006.  In the United States, it is 17.1 per 100,000 children <5 years old, but clinicians fear this number will continue to increase.

85% of children suffering from KD are <5 years old, and the median age is 2 years old. The symptoms begin with a high fever that lasts a minimum of 5 days and lasts as long as 4 weeks. The child typically experiences redness and swelling of his or her hands and feet and a morbilliform rash of the face and extremities. The lips and mouth will typically dry out, crack and blister.

Meanwhile, inside the coronary arteries, the body is attacking the vascular smooth muscle and causing inflammation that will lead to scarring and poor vessel integrity.  It is for this reason that coronary artery aneurysm (ballooning and tearing) occurs in 25% of cases, particularly if the child is less than 6 months or older than 6 years old. Other complications include myocardial infarction, cardiac arrest, heart failure, myocarditis and pericarditis. Two reports have noted children becoming insulin dependent diabetic within 4 months of the vasculitis.

When the pediatrician recognizes these harrowing symptoms, he or she will order an echocardiogram to visualize the heart.  When a blood test reveals high white blood cells (leukocytosis), high erythrocyte sedimentation rate (ESR) and high C-reactive protein (CRP), a diagnosis can be made.

The treatment for KD is intravenous immunoglobulin (IVIG), which is an injection of IgG (long-term) antibodies pooled from over 1,000 blood donors. High dose aspirin with or without corticosteroids is also administered to help reduce swelling and prevent a heart attack. The child should follow up with a cardiologist for repeated echocardiograms and long-term monitoring, since the highest risk for a heart attack is in the first year after diagnosis.

Until more is learned about KD and its origins, it will be added to a long list of autoimmune diseases that are poorly understood but well respected in the medical community.  

References

Saundankar J1, Yim D, Itotoh B, Payne R, Maslin K, Jape G, Ramsay J, Kothari D, Cheng A, Burgner D. The epidemiology and clinical features of Kawasaki disease in Australia. Pediatrics. 2014; 133(4):e1009-14.

Kawasaki Disease. Dynamed Database. Updated June 9, 2014. Accessed June 13, 2014.

Protect Your Baby From Bronchiolitis

Bronchiolitis is a relatively common respiratory condition seen in children younger than 2 years old.  According to a recent study out of Finland published in the Scandanavian journal Acta Paediatrica, bronchiolitis is a major cause of lower respiratory tract illness and hospitalization in babies, especially those younger than 6 months old. The condition is mostly caused by the respiratory syncytial virus (RSV), which is the infectious agent in over 75% of cases.  Other viruses, including influenza (flu) and adenovirus represent the remaining 25% and in many cases exist as co-infections.

RSV season typically begins in October and ends in April.  The virus is spread via the hands of caregivers and other inanimate surfaces (fomites), and can stay alive on them for several hours. The virus enters the respiratory tract and infiltrates the top cell layer of the lungs, known as bronchiolar epithelium, causing inflammation and swelling and obstructing the small airways. Once this happens, air passing through these small spaces emits a whistle-like sound that can be heard as a wheeze outside of the body.

This wheeze, along with a runny nose (rhinitis), cough, and fever, is suggestive of  RSV bronchiolitis. It is important, however, for the pediatrician to rule out other disease processes, such as pneumonia or foreign body aspiration. Studies have shown that infants <12 weeks old, particularly those who were premature at birth, have an increased risk of requiring hospitalization and medical intervention. For many babies, though, this disease is self-limiting and no tests or treatments are necessary.

There are established risk factors for RSV that you can’t avoid; childcare attendance, school aged siblings, prematurity, congenital cardiopulmonary disease and immunodeficiency. But the steps listed below have been shown to reduce the risk of spreading RSV:

• When in a group setting such as daycare or doctors offices, be sure that you and others caring for your children decontaminate their hands with alcohol-based sanitizer before and after direct contact with children or inanimate objects

• Do not smoke tobacco or wear clothing that has been around cigarette smoke unless it has been thoroughly washed

• Avoid exposing your infant to air pollutants

• Always breastfeed your infant whenever possible to allow your infant's immune system to strengthen

• Always follow current vaccination guidelines, including the flu vaccine

References

Bronchiolitis. Dynamed Database. Updated 2014 May 12 02:17:00 PM. Accessed June 6 2014. 

Pruikkonen H1, Uhari M, Dunder T, Pokka T, Renko M. Infants Under Six Months With Bronchiolitis Are Most Likely To Need Major Medical Interventions In The Five Days After Onset. Acta Paediatr. 2014 [Epub ahead of print]

How to Recognize Croup in Your Child

Pay close attention to your child’s next cough- it may suggest a common but serious childhood condition known as croup. Experienced pediatricians can recognize this particular cough in a crowded room; a sound often described as a “seals bark” that carries a potentially deadly omen. The medical term for this condition is laryngotracheobronchitis- a complicated word for inflammation of the upper respiratory tract, specifically the trachea and larynx.

Croup is most commonly caused by any one of several viral infections, including parainfluenza, influenza (flu), rhinovirus (common cold), adenovirus, respiratory syncitial virus (RSV), enterovirus and echovirus. In about 20% of cases, bacterial infections such as Mycoplasma pneumoniae, Corynebacterium diptheriae or staph or strep species are to blame.

The typical case of croup begins in a child under 6 years old between the fall and early winter months: The child has a history of an upper respiratory infection with fever and a cough that worsens at night.  The child's breathing becomes increasingly more labored, and a high pitched sound on inspiration known as stridor develops. This characteristic barking cough may follow:

How does this happen? When the upper airways become inflamed by an infection, swelling begins to narrow the passageway below the glottis through which air travels. As the airway narrows, our bodies attempt to compensate for poor airflow by increasing the respiratory rate (breaths per minute). The result is rapid air exchange through a narrowed airway (stridor), poor ventilation/oxygenation and respiratory distress.

It’s important that a pediatrician evaluates any child with stridor. Though many cases are self limiting and resolve in less than 48 hours, the doctor will rule out more serious conditions that present in a similar fashion, particularly epiglottitis, bacterial tracheitis and aspiration of a foreign budy. A simple oral dose of steroids (dexamethasone 0.15-0.60 mg/kg) will rectify the child’s breathing difficulties, though moderate to severe cases require inhaled (nebulized) racemic epinephrine.

• A diagnosis of epiglottitis is more likely when the child is experiencing excessive drooling and leaning over in a “sniffing position”.

• If the child is not treated, croup may lead to severe respiratory distress or become complicated with a pneumonia (known as laryngotracheobronciopneumonia)

• There are reports of repeated cases of croup, known as  spasmodic croup, that may be caused by gastroesophageal reflux disease (GERD) or extreme sensitivity to the parainfluenza virus.

• Any time your child is suffering from an upper respiratory infection, he or she is at an increased risk for developing croup

References

Croup. Dynamed Database. Updated December 27, 2013. Accessed June 2, 2014.

Petrocheilou A, Tanou K, Kalampouka E, Malakasioti G, Giannios C, Kaditis AG. Viral croup: diagnosis and treatment algorithm. Pediatr pulmonol. 2014; 49(5): 421-9.

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS)

Does a simple sore throat lead to psychological symptoms such as obsessive compulsive disorder (OCD) or Tourette syndrome?

In 1998, physicians began reporting odd encephalitis (brain inflammation)-like symptoms following an infection with Group A beta-hemolytic Streptococcus (GABHS), a bacterial infection.

Currently, PANDAS is not an ICD-9 recognized disease, and there is no recommended treatment. The suggested diagnostic criteria for PANDAS is abrupt onset (or dramatic exacerbations) of OCD or tic disorder, beginning between 3yo and the start of puberty, that occurs following an infection with GABHS. The neurologic examination would reveal hyperactivity, choreiform (snake-like) movements and/or tics.

Several studies have explored this phenomenon with conflicting results. Some authors found no correlation, while others found only elevated ASO (antibody) titers or the worsening of preexisting tic or OCD disorders. While some retrospective studies verified an association between infection and these neurological changes, a direct relationship has not been established.

Many clinicians think that PANDAS is simply a misdiagnosis of Sydenham’s chorea, a criteria for acute rheumatic fever (also caused by streptococcal infection) that is characterized by spastic and purposeless movements of the face or arms. Similarly to rheumatic fever, researchers believe that PANDAS may be due to the accidental production of antibodies that attack an area of the brain known as the basal ganglia.

•  If your child develops a sudden tic or movement disorder, or begins to display uncharacteristic psychological behavior, it is important to contact your physician. While PANDAS may be only temporary, acute rheumatic fever can lead to serious heart problems.

Reference

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS). Dynamed Database. Updated February 19, 2013, Accessed May 24, 2014.

Preterm infant mortality falling, prevalence of cerebral palsy rising

A recent study by Vincer et al published in the Journal of Pediatrics and Child Health reported on the rates of preterm infant mortality and cerebral palsy (CP) over the last 20 years. They found that the birth prevalence of CP among very preterm infants (less than 31 weeks) is on the rise, but the reasons are unclear.

CP is a permanent condition caused by damage to motor control centers in the brain before or shortly after birth, resulting in non-progressive impaired movement and permanent physical disability. The affected child typically has problems with uncontrolled spastic movements and/or impaired coordination of movement. Some cases are associated with epilepsy, poor cognition and/or difficulty with communication.

Between 1988 and 2007, very preterm infant mortality steadily declined while CP has been on the upswing. Vincer et al notes that maternal risk factors, anemia and tocolytic use have all been on the rise as well. Whether or not these represent a correlation has yet to be determined.

Reference

Vincer MJ, Allen AC, Allen VM, Baskett TF, O’Connell CM. Trends in the prevalence of cerebral palsy among very preterm infants (<31 weeks’ gestational age). Paediatr Child Health. 2014; 19(4):185-9.