Month: May 2011

How Common Is Rheumatic Heart Disease?

Patient Presentation
A third year medical student noted to his attending physician that he was reading about rheumatic fever. He asked the attending how common it was in the United States. The attending said that the last time she had personally seen a case was during her medical school training in older patients who had had the disease as a child, and she knew of a case that had been transferred from a local facility in the past couple of years. She said, “You have to think about it, but it doesn’t happen very much in the U.S. thankfully, because of antibiotics, less overcrowding and public health measures.”

Discussion
Acute rheumatic fever (ARF) is a nonsupprative, auto-inflammatory response after group A streptococcus (GAS) that affects multiple organs, including the heart. Chronic heart effects, particularly of the mitral and other heart valves, is termed rheumatic heart disease (RHD). ARF is thought to be caused by an autoimmune phenomenon where antibodies to the GAS cross react with normal host antigens. About 60% of ARF patients develop RHD and this is correlated to the initial carditis’s severity.

ARF symptoms can present 2-5 weeks after the GAS pharyngitis. Diagnosis of ARF includes 2 major, or 1 major and 2 minor Jones’ criteria in the clinical setting of a preceding GAS infection. The updated Jones’ criteria include:

  • Major criteria
    • Carditis – 30-45% of patients, endocarditis and pancarditis are usually seen. Valve disease usually begins with dilation and regurgitation, but chronic disease can lead to stenosis. Subclinical carditis is common.
    • Arthritis – 60-80% of patients – polyarticular, migratory arthritis, usually of large joints that commonly lasts less than 4 weeks.
    • Syndenham’s chorea – 10% of patients – a.k.a St. Vitus’s dance – purposeless, non-rhythmic involuntary movements usually of extremities and face that can also be accompanied by emotional lability and weakness.
    • Erythema marginatum – pink rash on trunk and extremities that is fleeting.
    • Subcutaneous nodules – painless nodules on extensor surfaces.
  • Minor criteria
    • Arthralgias
    • Fever – > 39° C.
    • Elevated inflammatory markers – erythrocyte sedimentation rate, C-reactive protein
    • Prolonged P-R interval on electrocardiogram
  • Evidence of GAS infection
    • Documented preceding streptococcal infection
    • Positive throat culture
    • Elevated or rising anti-streptolysin O titers.

ARF should be considered in the differential diagnosis of septic arthritis, juvenile idiopathic arthritis, reactive arthritis, patellofemoral syndrome and systemic lupus erythematosis among others.

Primary prevention is the best medicine by treating with antibiotics against GAS pharyngitis, improving access to medical care and social interventions for poverty. Potentially, vaccination may also assist primary prevention.

Secondary prevention is for those with histories of ARF or RHD at risk. Treatment is with daily antibiotics against GAS.

Learning Point

The most common cause of acquired heart disease in children and young adults is RHD.

Over the 20th century, the incidence and prevalence of ARF and RHD has decreased particularly in developed countries because of public health, sanitation, higher socio-economic status and improved medical care.
The highest rates of RHD are in developing countries. However, this is not always true. Australia and New Zealand have some of the highest rates when subpopulations are examined, mainly the First Nations peoples. In Australia the indigenous population incidence is 150-380 cases/100,000 population/year.
In the Maoris it is about 200 cases/year. Other subpopulations at risk include Pacific Islanders and people in Sub-Saharan Africa.

Overall the prevalence of RHD is estimated to be 15.6-19.6 million cases worldwide. About 233,000 deaths and 282,000 new cases are diagnosed each year.
Children and young adults are overrepresented in the statistics though with 2.4 million cases worldwide.
In the United States the incidence is <1 case per 100,000 in the pediatric population.

Questions for Further Discussion
1. How common is ARF and RHD in your location?
2. What treatment is recommended for ARF?
3. What is the role of echocardiograms for evaluation of possible ARF and RHD?
4. List other GAS infections.

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Heart Valve Diseases and Streptococcal Infections.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Carapetis JR. Rheumatic Heart Disease in Developing Countries. NEJM. 2007;357;439-441.

Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Acute rheumatic fever and its consequences: a persistent threat to developing nations in the 21st century. Autoimmun Rev. 2009 Dec;9(2):117-23.

Wilson N. Rheumatic heart disease in indigenous populations–New Zealand experience. Heart Lung Circ. 2010 May-Jun;19(5-6):282-8.

Parnaby MG, Carapetis JR. Rheumatic fever in indigenous Australian children. J Paediatr Child Health. 2010 Sep;46(9):527-33.

ACGME Competencies Highlighted by Case

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.
    18. Using effective nonverbal, explanatory, questioning, and writing skills, the healthcare professional uses effective listening skills and elicits and provides information.
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    • What Causes Vertigo in Children?

    Patient Presentation
    An 8-year-old female came to clinic with a 7 day history of intermittent vertigo. The episodes occurred 3 times where she would suddenly feel like the room was spinning around her or she was riding a roller coaster. She would need to lie down for relief and the episodes lasted about 30 minutes and then resolved. She denied auras, tinnitis, hearing loss, visual field changes, and diplopia. She also complained of nausea and problems walking with the episodes. She was conscious throughout and a teacher told the parents that her eyes were moving funny. She was well rested during the episodes which occurred in the morning and afternoons. She was doing well in school. The family history was positive for her mother who had a history of vertigo in the past and had migraines as an adult. There was no hearing loss, deafness, or other neurological problems in the family. The review of systems was notable for an upper respiratory infection about 3 weeks ago. She had no fevers, chills, cold sores or other problems.

    The pertinent physical exam showed a well-appearing child with growth parameters in the 10-50%. She had a small amount of serous fluid in her left ear. Her neurological examination showed her extraocular movements were intact and pupils were reactive to light and accommodation. DTRs were 2+/2+ with downgoing toes. there wee ormal rapid alternating movements, finger to nose testing, tandem gait and Romberg testing. No soft neurological signs were elicited. Nystagmus could not be elicited during sitting or with rapid changes in movement. The diagnosis of serous otitis media and probable benign paroxysmal vertigo of childhood was made. The physician discussed the natural history of the problem including that the episodes could intensify or remit. He also discussed that this type of vertigo often precedes the development of migraines later including migraines with a vertigenous component. She was warned to avoid doing things that may aggravate it such as merry-go-rounds, teeter-totters, other spinning games and activities. The patient’s clinical course over the next year showed that she had several more vertiginous episodes in the following month, but none since. She also had not developed headaches.

    Discussion
    Dizziness is an abnormal sensation relative to position and space which is often vague. It includes imbalance, motion intolerance, light-headedness, unsteadiness, floating or tilting sensations. Dizziness can be caused by cardiovascular, CNS or systemic diseases. Vertigo is a subtype of dizziness that has a rotary or spinning sensation. Objects rotate around the patient or the patient rotates around the objects.

    Vertigo is usually categorized into peripheral or central causes. Central vertigo emanates from a CNS location, and may have other CNS symptoms such as headaches, aura, motor, sensory or visual symptoms such as tinnitis or hearing loss. Symptoms usually last longer and may increase in number, frequency or intensity. Peripheral vertigo emanates from a non-CNS location and usually but not always has no or fewer CNS symptoms. Symptoms also usually are shorter but can be chronic.

    Treatment includes treatment of underlying disorders such as seizures, migraine, tumor, etc., IV fluids, vestibular suppressants, and avoiding migraine triggers. Positional maneuvers may be helpful for benign postural positional vertigo.

    Learning Point
    The differential diagnosis of vertigo includes:

    • Peripheral
      • Benign paroxysmal vertigo of childhood – Young children present with episodes where they are fearful and grasp onto people or objects or refuse to stand, and additionally have balance problems or falls. Older children may be anxious. Children will often have nystagmus and it is considered an early manifestation of migraines.
      • Benign paroxysmal positional vertigo – Thought to be due to ear debris in the semi-circular canals that irritates the vestibular system. Positioning exercises may help to move the debris and stop the irritation.
      • Congenital deafness
      • Immune-mediated inner-ear disease
      • Infectious disease – e.g. Lyme disease, Epstein Barr Virus
      • Post- head trauma
      • Post- meningitis
      • Migraine
      • Psychosomatic
      • Seizures, vertiginous
      • Semicircular canal pathology – e.g. fistula, dehiscence
      • Vestibular neuronitis/Labyrinthitiss – There are many overlapping features, but usually vestibular neuronitis is diagnosed if the auditory function is not affected. It is often caused by viruses such as herpes simplex.
    • Central
      • Chiari malformation
      • Cerebrovascular disease – vestibulobasilar disease, cerebellar ischemia or hemorrhage
      • Hereditary ataxia
      • Tumor – e.g. posterior fossa, acoustic neuroma
      • Meniere’s disease
      • Multiple sclerosis
    • Misperception of real dizziness caused by dysequilibrium, orthostatic hypotension, presyncope/syncope, etc.

    Questions for Further Discussion
    1. What psychiatric illness could present with perceptual changes such as dizziness or vertigo?
    2. How are presyncope and dizziness and vertigo distinguished?
    3. What are indications for referral to a neurologist for vertigo?
    4. Describe the positioning maneuvers for benign paroxysmal positional vertigo?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Dizziness and Vertigo

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Samy HM, Hamid MA. Dizziness, Vertigo, and Imbalance. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/1159385-overview#showall (rev. 1/14/2010, cited 4/5/11).

    Atunes MB, Ruckenstein MJ. CNS Causes of Vertigo. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/884048-overview (rev. 9/9/10, cited 4/5/11).

    Chan Y. Differential diagnosis of dizziness. Curr Opin Otolaryngol Head Neck Surg. 2009 Jun;17(3):200-3.

    Cuvellier JC, Lepine A. Childhood periodic syndromes. Pediatr Neurol. 2010 Jan;42(1):1-11.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    • When Can Children Use All-Terrain Vehicles?

    Patient Presentation
    While in clinic, a resident asked if another resident had heard about the three teenagers who were involved in an all-terrain vehicle (ATV) accident. The second resident said that he had heard about the accident and both remarked that it was tragic as the teens were in critical condition with uncertain prognoses. The attending physician asked the residents how they might have prevented such an accident. One resident said that she had never discussed ATVs with her patients and the second resident said that it would make sense to wear helmets. The faculty member showed the residents where to find American Academy of Pediatrics policy statements on the Internet and together they reviewed the current policy statement which recommends ATVs not be used until a teenager can drive a car plus other recommendations.

    Discussion
    All-terrain vehicles are 3- or 4- wheeled vehicles designed for one rider to be used in rough-terrain situations. They became available in the 1970s in the United States and because of severe instability, the 3-wheeled variety has not been manufactured since the 1980s. From 1997-2001, the overall exposure to ATVs increased by 36-50% depending on the variable studied and the injuries increased 104%. A 2005 study found 5292 children were hospitalized because of ATV-related injuries over 2 years with 1% dying and an additional 5% needing discharge to long-term care. Adolescent males had the highest risk of injuries and regionally those in the South and Midwest had the highest injury rates. The total hospital cost for the 2 years of hospitalizations was > $74 million.

    Another 2005 study of non-fatal ATV injuries estimated that ~109,000 children were evaluated in hospital emergency departments in the U.S. over 3 years for ATV injuries and there was a 39% increase during the study. Patients (12%) were admitted with teenage males being the highest rate of those injured. However, younger children were more likely to have facial injuries and older children were more likely to have lower extremity injuries.

    Learning Point
    The American Academy of Pediatrics (AAP) policy statement on All-Terrain Vehicle Injury Prevention recommends that:

    • Children should not be allowed to operate an off-road vehicle until they are licensed to drive a car. No one < 16 years of age should operate an ATV.
    • All safety equipment should be used by the rider at all times. Equipment to increase vehicle and driver visibility should be used such as reflectors, flags, etc.
    • Street use of the ATV and use at night should never be allowed.
    • Riders should ride alone and never carry a passenger. ATVs are designed for one person.
    • ATVs should not be used after drinking alcohol.

    During the 1990s, the State of West Virginia had the highest ATV-related deaths in the U.S. and therefore implemented an ATV law in 2004. After implementation, the number of ATV-related deaths continued to increase. The authors of a follow-up research study recommended changing the law to incorporate the AAP’s recommendations including:

    • Requirement of a driver’s license, plus additional certification specific to ATV use.
    • No operation of an ATV by children < 16 years.
    • No operation of an ATV while under the influence of alcohol.
    • No passengers on ATVs.
    • No ATV operation at night.

    The West Virginia authors also suggest that community and school-based education particularly for adolescents in poor communities, use of incentive-based programs such as reduced insurance premiums or safety problems, or extended warranties on helmets might provide help in reducing ATV-related injuries and deaths.

    Questions for Further Discussion
    1. What types of injuries do children with ATV-injuries have?
    2. At what age can children operate lawn mowers?
    3. At what age can children operate farm machinery?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Motor Vehicle Safety and Child Safety.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    American Academy of Pediatrics. All-Terrain Vehicle Injury Prevention: Two-, Three-, and Four-Wheeled Unlicensed Motor Vehicles. Pediatrics. 2000;105:1352-1354.

    Killingsworth JB, Tilford JM, Parker JG, Graham JJ, Dick RM, Aitken ME. National hospitalization impact of pediatric all-terrain vehicle injuries. Pediatrics. 2005 Mar;115(3):e316-21.

    Shults RA, Wiles SD, Vajani M, Helmkamp JC. All-terrain vehicle-related nonfatal injuries among young riders: United States, 2001-2003. Pediatrics. 2005 Nov;116(5):e608-12.

    Centers for Disease Control and Prevention (CDC). All-terrain vehicle fatalities–West Virginia, 1999-2006. MMWR Morb Mortal Wkly Rep. 2008 Mar 28;57(12):312-5.

    ACGME Competencies Highlighted by Case

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

  • Professionalism
    20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital