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rheumatic fever

 

n.
An acute inflammatory disease occurring during recovery from infection with a strain of streptococcus bacteria, having an onset marked by fever and joint pain and frequently resulting in scarring of the heart valves.


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Britannica Concise Encyclopedia:

rheumatic fever

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Generalized disease caused by certain types of streptococcus bacteria. It occurs mostly in children and young adults. Symptoms may be mild or severe. Sudden fever, joint pain, and inflammation may begin days to weeks after a streptococcal infection, usually of the throat (see pharyngitis). Other symptoms may include skin nodules and rashes, chorea, abdominal pain, nosebleeds, and weight loss. Heart inflammation, with accompanying rapid heartbeat, murmurs, and enlargement, can lead to valve scarring, markedly shortening life. After recovery, survivors are prone to future attacks. Penicillin given when the initial infection is diagnosed can prevent it. Otherwise, salicylic acid derivatives or corticosteroids help the symptoms.

For more information on rheumatic fever, visit Britannica.com.

An illness that follows an upper respiratory infection with the group A streptococcus (Streptococcus pyogenes) and is characterized by inflammation of the joints (arthritis) and the heart (carditis). Arthritis typically involves multiple joints and may migrate from one joint to another. The carditis may involve the outer lining of the heart, the heart muscle itself, or the inner lining of the heart. A minority of affected individuals also develop a rash (erythema marginatum), nodules under the skin, or Sydenham's chorea (a neurologic disorder characterized by involuntary, uncoordinated movements of the legs, arms, and face). Damage to heart valves may be permanent and progressive, leading to severe disability or death from rheumatic heart disease years after the initial attack. The disease occurs an average of 19 days after the infection and is thought to be the result of an abnormal immunologic reaction to the group A streptococcus. Initial attacks of rheumatic fever generally occur among individuals aged 5 to 15. Those who have had one attack are highly susceptible to recurrences after future streptococcal infections.

Initial attacks of rheumatic fever can be prevented by treatment of strep throat with penicillin for at least 10 days. Patients who have had an episode of rheumatic fever should continue taking antibiotics for many years to prevent group A streptococcal infections that may trigger a recurrence of rheumatic fever. See also Heart disorders; Streptococcus.


Definition

Rheumatic fever (RF) is an illness that arises as a complication of untreated or inadequately treated strep throat infection. Rheumatic fever can seriously damage the valves of the heart.

Description

Throat infection with a member of the Group A streptococcus (strep) bacteria is a common problem among school-aged children. It is easily treated with a 10-day course of antibiotics by mouth. However, when such a throat infection occurs without symptoms, or when a course of medication is not taken for the full ten days, there is a 3 percent chance the person will develop rheumatic fever. Other types of strep infections (such as of the skin) do not put the patient at risk for RF.

Demographics

Children between the ages of five and 15 are most susceptible to strep throat, and therefore most susceptible to rheumatic fever. Other risk factors include poverty, overcrowding (as in military camps), and lack of access to good medical care. Just as strep throat occurs most frequently in fall, winter, and early spring, so does rheumatic fever. Rheumatic fever used to be a leading cause of death and disability in children. Since 1960, it has become much less common in the United States, partially because of increasingly accurate and swift diagnosis of strep throat. It is still a large problem in many developing countries. Moreover, children who have family members who have had rheumatic fever are more likely to get rheumatic fever themselves.

Causes and Symptoms

Two different theories exist about how a bacterial throat infection can develop into rheumatic fever. One theory suggests that the bacteria produce some kind of poisonous chemical (toxin). This toxin is sent into circulation throughout the bloodstream, thus affecting other systems of the body.

Research seems to point to a different theory, however. The second theory suggests that the disease is caused by the body's immune system acting inappropriately. The body produces immune cells (called antibodies), that are specifically designed to recognize and destroy invading agent—in this case, streptococcal bacteria. The antibodies are able to recognize the bacteria because the bacteria contain special markers called antigens. Due to a resemblance between Group A streptococcus bacteria's antigens and antigens present on the body's own cells, the antibodies may mistakenly attack the body itself.

It is interesting to note that members of certain families seem to have a greater tendency to develop rheumatic fever than do others. This statistical fact could be related to the above theory, in that these families may have cell antigens that more closely resemble streptococcal antigens than do members of other families.

In addition to fever, in about 75 percent of all cases of RF one of the first symptoms is arthritis. The joints (especially those of the ankles, knees, elbows, and wrists) become red, hot, swollen, shiny, and extraordinarily painful. Unlike many other forms of arthritis, this arthritis may not occur symmetrically (affecting a particular joint on both the right and left sides, simultaneously). The arthritis of RF rarely strikes the fingers, toes, or spine. The joints become so tender that even the touch of bed sheets or clothing is terribly painful.

A particular type of involuntary movement, coupled with emotional instability, occurs in about 10 percent of all RF patients. The patient begins experiencing a change in coordination, often first noted by changes in handwriting. The arms or legs may flail or jerk uncontrollably. The patient seems to develop a low threshold for anger and sadness. This feature of RF is called Sydenham's chorea or St. Vitus' dance.

A number of skin changes are common to RF. A rash called erythema marginatum often develops (especially in those patients who will develop heart problems from their illness), composed of pink splotches that may eventually spread into each other. The rash does not itch. Bumps the size of peas may occur under the skin. These are called subcutaneous nodules. They are hard to the touch, but not painful. These nodules most commonly occur over the knee and elbow joint, as well as over the spine.

The most serious problem occurring in RF is called pancarditis ("pan" means total; "carditis" refers to inflammation of the heart). Pancarditis is an inflammation that affects all aspects of the heart, including the lining of the heart (endocardium), the sac containing the heart (pericardium), and the heart muscle itself (myocardium). About 40 to 80 percent of all RF patients develop pancarditis. This RF complication has the most serious, long-term effects. The valves within the heart (structures that allow the blood to flow only in the correct direction and only at the correct time in the heart's pumping cycle) are frequently damaged during the course of pancarditis. This effect may result in blood that either leaks back in the wrong direction or has a difficult time passing a stiff, poorly moving valve. Either way, damage to a valve can result in the heart having to work very hard in order to move the blood properly. The heart may not be able to "work around" the damaged valve, which may result in a consistently inadequate amount of blood entering the circulation.

When to Call the Doctor

The doctor should be contacted if the child is displaying any of the signs or symptoms of rheumatic fever. If they are not indications of rheumatic fever, they could be indicative of another disease or disorder. The doctor should also be contacted if the child has had a sore throat and fever for more than 24 hours. The doctor will do a strep test, and if the child does have strep throat the doctor can administer antibiotics that will help prevent rheumatic fever.

Diagnosis

There are no laboratory tests that can determine with complete certainty if a child has rheumatic fever. Some laboratory tests may be used in conjunction with careful examination of the patient to determine if the child has RF. A list of diagnostic criteria has been created. These "Jones Criteria" are divided into major and minor criteria. A patient can be diagnosed with RF if he or she has either two major criteria (conditions) or one major and two minor criteria. In either case, it must also be proved that the individual has had a previous infection with streptococcus.

The major criteria include:

  • carditis
  • arthritis
  • chorea
  • subcutaneous nodules
  • erythema marginatum

The minor criteria include:

  • fever
  • joint pain (without actual arthritis)
  • evidence of electrical changes in the heart (determined by measuring electrical characteristics of the heart's functioning during a test called an electrocardiogram, or EKG)
  • evidence (through a blood test) of the presence in the blood of certain proteins that are produced early in an inflammatory/infectious disease

Tests are also performed to provide evidence of recent infection with group A streptococcal bacteria. A swab of the throat can be taken and smeared on a gel-like substance in a petri dish to see if bacteria will multiply and grow over 24 to 72 hours. These bacteria can then be specially processed and examined under a microscope to identify streptococcal bacteria. Other tests can be performed to see if the patient is producing specific antibodies that are only made in response to a recent strep infection.

Treatment

A 10-day course of penicillin by mouth or a single injection of penicillin G is usually the first line of treatment for RF. If the child does not tolerate or is allergic to penicillin, other antibiotics can be used effectively. These antibiotics are given to help cure a strep infection, if the child still has one. Patients will need to remain on some regular dose of antibiotic to prevent recurrence of RF. This can mean a small daily dose of antibiotic by mouth or an injection every three to four weeks. Some practitioners keep patients on this regimen for five years or until they reach 18 years of age whichever comes first. Other practitioners prefer to continue treating those patients who will be regularly exposed to streptococcal bacteria (teachers, medical workers), as well as those patients with known RF heart disease.

Arthritis quickly improves when the patient is given a preparation containing aspirin or some other anti-inflammatory agent (e.g. ibuprofen). Mild carditis also improves with such anti-inflammatory agents, although more severe cases of carditis require steroid medications. A number of medications are available to treat the involuntary movements of chorea, including diazepam for mild cases and haloperidol for more severe cases.

Prognosis

The long-term prognosis of an RF patient depends primarily on whether he or she develops carditis. This manifestation of RF is the only one that can have permanent effects. Those patients with no or mild carditis have an excellent prognosis. Those with more severe carditis have a risk of heart failure, as well as a risk of future heart problems that may lead to the need for valve replacement surgery. Patients who have had rheumatic fever are at an increased risk of getting it again.

Prevention

Prevention of the development of RF involves proper diagnosis of initial strep throat infections and adequate treatment within 10 days with an appropriate antibiotic. Prevention of RF recurrence requires continued antibiotic treatment, perhaps for life. Prevention of complications of already-existing RF heart disease requires that the patient always take a special course of antibiotics when he or she undergoes any kind of procedure (even dental cleanings) that might allow bacteria to gain access to the bloodstream.

Parental Concerns

Rheumatic fever can be life-threatening if not treated. It can also lead to lifelong heart problems. The best way for parents to prevent rheumatic fever is to take seriously sore throats that are accompanied with fever and to take the child to a doctor to test for strep throat. Children who have had rheumatic fever need to take extra precautions to ensure they do not have repeat attacks triggered by strep infections.

See also Strep throat.

Resources

Books

Margulies, Phillip. Everything You Need to Know about Rheumatic Fever. New York: Rosen Publishing Group, 2004.

Periodicals

Mercadante, Marcos T., et al. "The Psychiatric Symptoms of Rheumatic Fever." American Journal of Psychiatry 157, i.12 (December 2000): 2036.

Steeg, Carl N., et al. "Rheumatic Fever: No Cause for Complacence." Patient Care 34, i.14 (July 30, 2000): 40.

Stollerman, Gene H. "Rheumatic Fever in the 21st Century." Clinical Infectious Diseases 33, no. 16 (September 15, 2001): 806.

Organizations

American Heart Association. 7272 Greenville Ave., Dallas, TX 75231. Web site: www.americanheart.org.

[Article by: Tish Davidson, A.M.
Rosalyn Carson-DeWitt, MD]



Columbia Encyclopedia:

rheumatic fever

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rheumatic fever (rūmăt'ĭk), systemic inflammatory disease, extremely variable in its manifestation, severity, duration, and aftereffects. It is frequently followed by serious heart disease, especially when there are repeated attacks. Rheumatic fever usually affects children. It is closely related to a preceding streptococcal infection (e.g., streptococcal tonsillitis or pharyngitis). Some of its symptoms are tenderness and inflammation about the joints, fever, jerky movements, nodules under the skin, and skin rash. If inflammation of the heart, or myocarditis, is mild, there is no permanent heart damage, but if the valves of the heart become inflamed, they may become scarred and deformed, permanently impairing their function. Such heart damage can sometimes be corrected by surgery.

Treatment of rheumatic fever is with penicillin, salicylates, and steroids; extended rest is usually necessary. Rheumatic fever may be prevented by prompt treatment of all streptococcal infections. Cardiac damage may possibly be avoided if prophylactic measures are taken after a first attack of rheumatic fever, i.e., long-term maintenance doses of antibiotics, to discourage streptococcal infections and recurrences of rheumatic fever. Rheumatic fever has declined in incidence in the industrialized countries, but has increased in prevalence in the Third World. See also streptococcus.


(rooh-mat-ik)

An infectious disease occurring most often in children who have had a previous infection with a strain of streptococcus. Rheumatic fever, which is characterized by fever and joint pain, can cause permanent damage to the heart if left untreated. Antibiotics, such as penicillin, are used in treating the disease.

Mosby's Dental Dictionary:

rheumatic fever

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(rōōmat′ik)
n

A severe, apparently infectious disease produced by hemolytic streptococci organisms or associated with their presence in the body; characterized by upper respiratory tract inflammation, cervical lymphadenopathy and lymphadenitis, polyarthritis, cardiac involvement, and subcutaneous nodules. The disease may be produced by an autoantibody reaction.

Random House Word Menu:

categories related to 'rheumatic fever'

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Random House Word Menu by Stephen Glazier
For a list of words related to rheumatic fever, see:
  • Diseases and Infestations - rheumatic fever: delayed complication of upper respiratory streptococcus infection, esp. in young, characterized by fever, arthritis of joints, chorea, carditis, and erythematosus rash


Wikipedia on Answers.com:

Rheumatic fever

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Rheumatic fever
Classification and external resources

Streptococcus pyogenes bacteria, Pappenheim's stain
ICD-10 I00-I02
ICD-9 390392
DiseasesDB 11487
MedlinePlus 003940
eMedicine med/3435 med/2922 emerg/509 ped/2006
MeSH D012213

Rheumatic fever is an inflammatory disease that occurs following a Streptococcus pyogenes infection, such as streptococcal pharyngitis or scarlet fever. Believed to be caused by antibody cross-reactivity that can involve the heart, joints, skin, and brain,[1] the illness typically develops two to three weeks after a streptococcal infection. Acute rheumatic fever commonly appears in children between the ages of 6 and 15, with only 20% of first-time attacks occurring in adults.[1] The illness is so named because of its similarity in presentation to rheumatism.[2]

Contents

Diagnosis

Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium).

Modified Jones criteria were first published in 1944 by T. Duckett Jones, MD.[3] They have been periodically revised by the American Heart Association in collaboration with other groups.[4] According to revised Jones criteria, the diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of streptococcal infection: elevated or rising antistreptolysin O titre or DNAase.[1] Exceptions are chorea and indolent carditis, each of which by itself can indicate rheumatic fever.[5][6][7]

Major criteria

  • Polyarthritis: A temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
  • Carditis: Inflammation of the heart muscle which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
  • Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.
  • Erythema marginatum: A long lasting rash that begins on the trunk or arms as macules and spreads outward to form a snake like ring while clearing in the middle. This rash never starts on the face and it is made worse with heat.
  • Sydenham's chorea (St. Vitus' dance): A characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease for at least three months from onset of infection.

Minor criteria

Other signs and symptoms

  • Abdominal pain
  • Nose bleeds
  • Preceding streptococcal infection: recent scarlet fever, raised antistreptolysin O or other streptococcal antibody titre, or positive throat culture.[9]

Pathophysiology

Pathophysiology of rheumatic heart disease

Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated Group A Beta hemolytic streptococcal pharyngeal infection. It is believed to be caused by antibody cross-reactivity. This cross-reactivity is a Type II hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a Streptococcus infection, mature antigen presenting cells such as B cells present the bacterial antigen to CD4-T cells which differentiate into helper T2 cells. Helper T2 cells subsequently activate the B cells to become plasma cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints,[10] producing the symptoms of rheumatic fever.

Group A streptococcus pyogenes has a cell wall composed of branched polymers which sometimes contain M protein that are highly antigenic. The antibodies which the immune system generates against the M protein may cross react with cardiac myofiber protein myosin,[11] heart muscle glycogen and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. However, the only proven cross reaction is with perivascular connective tissue.[citation needed] This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Aschoff giant cells. Acute rheumatic valvular lesions may also involve a cell-mediated immunity reaction as these lesions predominantly contain T-helper cells and macrophages.[12]

In acute rheumatic fever, these lesions can be found in any layer of the heart and is hence called pancarditis. The inflammation may cause a serofibrinous pericardial exudate described as "bread-and-butter" pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and verrucae formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendothelial lesions may induce irregular thickenings called MacCallum plaques.

Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion and shortening and thickening of the tendinous cords.[12]

Prevention

Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis with antibiotics. The American Heart Association recommends that daily or monthly prophylaxis continue long-term, perhaps for life.[13][dead link]

Treatment

The management of acute rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics. Aspirin is the drug of choice and should be given at high doses of 100 mg/kg/day. One should watch for side effects like gastritis and salicylate poisoning. In children and teenagers, the use of aspirin and aspirin-containing products can be associated with Reye's syndrome, a serious and potentially deadly condition. The risks, benefits and alternative treatments must always be considered when administering aspirin and aspirin-containing products in children and teenagers. Ibuprofen for pain and discomfort and corticosteroids for moderate to severe inflammatory reactions manifested by rheumatic fever should be considered in children and teenagers. Steroids are reserved for cases where there is evidence of involvement of heart. The use of steroids may prevent further scarring of tissue and may prevent development of sequelae such as mitral stenosis. Monthly injections of longacting penicillin must be given for a period of five years in patients having one attack of rheumatic fever. If there is evidence of carditis, the length of Penidure therapy may be up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low-dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.

Infection

Patients with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not present. This treatment will not alter the course of the acute disease.

The most appropriate treatment stated in the Oxford Handbook of Clinical Medicine for rheumatic fever is benzathine benzylpenicillin.

Inflammation

Patients with significant symptoms may require corticosteroids. Salicylates are useful for pain.

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual treatment for heart failure: ACE Inhibitors, diuretics, beta blockers and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids.

Epidemiology

Disability-adjusted life year for rheumatic heart disease per 100,000 inhabitants in 2004.[14]
  no data
  less than 20
  20-40
  40-60
  60-80
  80-100
  100-120
  120-140
  140-160
  160-180
  180-200
  200-330
  more than 330

Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. In Western countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to treat streptococcus infections. While it has been far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a case-fatality rate of 2–5%.[15]

Rheumatic fever primarily affects children between ages 5 and 17 years and occurs approximately 20 days after strep throat. In up to a third of cases, the underlying strep infection may not have caused any symptoms.

The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%).[16] The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Heart complications may be long-term and severe, particularly if valves are involved.

Survivors of rheumatic fever often have to take penicillin to prevent streptococcal infection which could possibly lead to another case of rheumatic fever that could prove fatal.

References

  1. ^ a b c Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; & Mitchell, Richard N. (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 403-406 ISBN 978-1-4160-2973-1
  2. ^ "rheumatic fever" at Dorland's Medical Dictionary
  3. ^ Jones TD (1944). "The diagnosis of rheumatic fever.". JAMA 126: 481–4. 
  4. ^ Ferrieri P; Jones Criteria Working, Group (2002). "Proceedings of the Jones Criteria workshop". Circulation 106 (19): 2521–3. doi:10.1161/01.CIR.0000037745.65929.FA. PMID 12417554. http://circ.ahajournals.org/cgi/content/full/106/19/2521?ck=nck. 
  5. ^ Steven J Parrillo, DO, FACOEP, FACEP. "eMedicine — Rheumatic Fever". http://www.emedicine.com/emerg/topic509.htm. Retrieved 2007-07-14. 
  6. ^ "Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association". JAMA 268 (15): 2069–73. 1992. doi:10.1001/jama.268.15.2069. PMID 1404745. 
  7. ^ Saxena, Anita (2000). "Diagnosis of rheumatic fever: Current status of Jones criteria and role of echocardiography". Indian Journal of Pediatrics 67 (4): 283–6. doi:10.1007/BF02758174. PMID 11129913. 
  8. ^ Aly, Ashraf (2008). "Rheumatic Fever". Core Concepts of Pediatrics. University of Texas. http://www.utmb.edu/pedi_ed/CORE/Cardiology/page_40.htm. Retrieved 2011-08-06. 
  9. ^ a b Ed Boon, Davidson's General Practice of Medicine, 20th edition. P. 617.
  10. ^ Abbas, Abul K.; Lichtman, Andrew H.; Baker, David L.; et al (2004). Basic immunology: functions and disorders of the immune system (2 ed.). Philadelphia, Pennsylvania: Elsevier Saunders. ISBN 978-1416024033. 
  11. ^ Faé KC, da Silva DD, Oshiro SE, et al. (May 2006). "Mimicry in recognition of cardiac myosin peptides by heart-intralesional T cell clones from rheumatic heart disease". J. Immunol. 176 (9): 5662–70. PMID 16622036. http://www.jimmunol.org/cgi/pmidlookup?view=long&pmid=16622036. 
  12. ^ a b Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. ISBN 0-7216-0187-1. http://www.robbinspathology.com/. 
  13. ^ "Rheumatic Heart Disease/Rheumatic Fever". American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=4709. Retrieved 2008-02-17. 
  14. ^ "WHO Disease and injury country estimates". World Health Organization. 2009. http://www.who.int/healthinfo/global_burden_disease/estimates_country/en/index.html. Retrieved Nov. 11, 2009. 
  15. ^ NLM/NIH: Medline Plus Medical Encyclopedia: Rheumatic fever
  16. ^ Porth, Carol (2007). Essentials of pathophysiology: concepts of altered health states. Hagerstown, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7087-4. 

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