The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–3, 2015 Copyright Ó 2015 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter

http://dx.doi.org/10.1016/j.jemermed.2014.12.057

Clinical Communications: Pediatrics GROUP C STREPTOCOCCUS CAUSING RHEUMATIC HEART DISEASE IN A CHILD Harsha K. Chandnani, MD, MBA, MPH, Renu Jain, MD, and Pisespong Patamasucon, MD Department of Pediatrics, University of Nevada School of Medicine, Las Vegas, Nevada Reprint Address: Harsha K. Chandnani, MD, MBA, MPH, Department of Pediatrics, University of Nevada School of Medicine, 2040 West Charleston Boulevard, Suite 402, Las Vegas, NV 89102

, Keywords—group C Streptococcus; carditis; rheumatic heart disease; pediatrics

, Abstract—Background: Human infection with group C Streptococcus is extremely rare and a select number of cases have been reported to cause acute pharyngitis, acute glomerulonephritis, skin and soft tissue infections, septic arthritis, osteomyelitis, pneumonitis, and bacteremia. In pediatrics, this bacteria is known to cause epidemic food-borne pharyngitis, pneumonia, endocarditis, and meningitis, and has reportedly been isolated in the blood, meninges, sinuses, fingernail, peritonsillar abscess, and thyroglossal duct cyst, among others. Case Report: Our patient was a 7-year-old previously healthy female who presented with abnormal movements of her upper body and grimaces of her face that progressively worsened over time. Initial laboratory resulted revealed 3+ protein on urinalysis and elevated antistreptolysin-O and anti-DNAse antibody levels, and echocardiogram showed mild-to-moderate mitral regurgitation. We describe a rare case of group C Streptococcus resulting in rheumatic heart disease in a child, with a detailed review of the literature pertaining to the diagnosis and management of this infection. Why Should an Emergency Physician Be Aware of This?: Early recognition of rheumatic heart disease is crucial in the overall outcome of the condition and therefore knowledge of the symptoms associated with condition is also imperative. Group C Streptococcus is rarely associated with rheumatic heart disease and most children exhibiting acute onset of common symptoms, such as chorea, fever, carditis, and rash (erythema marginatum) will present to the emergency department first. Increased awareness and prompt recognition, as done with this child, will result in proper follow-up and adequate management of this condition in all patients. Ó 2015 Elsevier Inc.

INTRODUCTION Children present to the emergency department (ED) with many different kinds of infections. Some are very common, and others are quite rare. Human infection with group C Streptococcus is extremely rare and a select number of cases have been reported to cause acute pharyngitis, acute glomerulonephritis, skin and soft tissue infections, septic arthritis, osteomyelitis, pneumonitis, and bacteremia. In pediatrics, infection with this organism is not known to cause rheumatic heart disease, but prompt recognition and appropriate management are essential for emergency medicine physicians, as they usually are the first health care providers at initial presentation. CASE REPORT The patient was a 7-year-old female, previously healthy, presenting to the ED with abnormal movements. She described experiencing abnormal twitching, primarily of her upper body, with right-sided prominence, as well as grimaces of her face that progressively worsened during the previous 1 month. It was worse soon after she woke up in the morning and just before going to bed at night. She had not used any medications recently. She

RECEIVED: 18 October 2014; ACCEPTED: 21 December 2014 1

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denied fever, trauma, head injury, drug use, headaches, vision changes, vomiting, or focal neurologic symptoms. Patient did report a history of an upper respiratory infection with a ruptured right acute otitis media diagnosed 2 months before, for which she was treated adequately with amoxicillin. There was no family history of neurologic disorders and no recent travel. Two days before presentation, the patient’s 5-year-old sister tested positive on the Rapid Strep test. Her immunizations were up to date. Physical examination revealed a child with temperature 98.8 F, blood pressure 107/62 mm Hg, pulse 102 beats/min, respiratory rate 28 breaths/min, and pulse oximetry of 98% on room air. She weighed 26.7 kg and was alert and active with pupils that are equally round and reactive to light, sharp disk margins on fundoscopic examination, clear tympanic membranes, no lymphadenopathy, no murmurs or gallops on cardiovascular examination, lungs clear to auscultation bilaterally, and no rashes or other skin abnormalities. Neurologic examination revealed no abnormalities, with 5/5 strength in all extremities, intact sensation to deep and vibratory sensation in all extremities, 2+ deep tendon reflexes, and normal gait. Patient did exhibit difficulty maintaining balance with tandem gait and when attempting alternating movements. Laboratory investigation included a complete blood count, complete metabolic panel, urinalysis, erythrocyte sedimentation rate, C-reactive protein, antistreptolysinO (ASO) titer, and an anti-DNAse antibody level. Except 3+ protein on urinalysis, an ASO titer of 543 IU/mL, and an anti-DNAse antibody level of 930 U/mL, all results were within normal limits. Electrocardiogram was read as normal sinus rhythm, with a normal PR interval and no QT prolongation. Initial echocardiogram revealed a structurally normal heart with mild-to-moderate mitral valve regurgitation, and no pericardial effusion. Eleven months later, patient’s ASO titer and antiDNAse antibody level remained elevated at 782 IU/mL and 1,200 U/mL, respectively. DISCUSSION This patient’s initial presentation demonstrated evidence of Sydenham’s chorea and carditis, suggesting acute rheumatic fever and rheumatic heart disease. She was started on acute treatment with high-dose amoxicillin in the ED and then subsequently followed closely by the cardiologist and infectious disease specialist, requiring monthly benzathine penicillin doses for rheumatic fever prophylaxis, to be continued until age 21 years. Nine months after initial presentation, the patient was found to grow group C Streptococcus on throat culture with relapse of chorea and mitral valve regurgitation, requiring another course of acute treatment.

Group C Streptococcus is a b-hemolytic aerobic, facultatively anaerobic, coprophilic, and catalasenegative organism (1). Two individual species have been identified: S. dysgalactiae and S. equi, each of which has two subspecies. The S. equismilis subspecies of S. dysgalactiae is known to most often colonize and cause infection in humans, found in the nasopharynx, throat, skin, and genital tract of asymptomatic individuals. A small proportion of people living in temperate climates carry the organisms in the upper respiratory tract. Infection is elicited with an antibody response to streptokinase and streptolysin O production from the equisimilis strain, similar to group A Streptococcus. Patients with acute pharyngitis have also had M protein isolated from this strain. The S. equi subspecies zooepidermicus, although rare in humans, is known to cause infections in domestic animals, including cattle, pigs, sheep, and horses (1). Humans can acquire this strain with consumption of unpasteurized milk or homemade cheese or other forms of animal contact. Group C streptococci organisms are frequently pathogenic in animals rather than humans, where there is low virulence and higher prevalence of infection in those with an underlying medical condition, such as diabetes mellitus, immunosuppression, and malignancy. The most common clinical presentation is acute pharyngitis with fever, sore throat, pharyngeal exudates, and cervical lymphadenitis (2). In addition, individuals with a group C Streptococcus infection can also present with acute glomerulonephritis, skin and soft tissue infections, ostemyelitis, septic arthritis, urinary tract infections, epiglottitis, meningitis, bacteremia, infective endocarditis, pericarditis, and sinusitis. Some rare cases have also reported puerperal sepsis and endometritis, and others have established an association with reactive arthritis and toxic shock-like syndrome. Specifically in pediatrics, group C streptococcal infections have been identified as causing epidemic foodborne pharyngitis, pneumonia, endocarditis, pericarditis, and meningitis, but not rheumatic heart disease. A case review published in 1989 identified 9 pediatric patients under the age of 19 years with group C Streptococcus isolated in the blood, meninges and sinuses, fingernail, frontal bone aspirate, thyroglossal duct cyst, peritonsillar abscess, orbital abscess, and appendiceal abscess, of but one recovered without sequelae (2). Our patient was found to have recurrent symptoms and requires longterm prophylaxis for rheumatic fever. Substantial evidence exists to establish a link between group A streptococcal pharyngitis and acute rheumatic fever and rheumatic heart disease. This case, however, highlights an association between group C Streptococcus and rheumatic heart disease, which is known as the most

Group C Streptococcus and Rheumatic Heart Disease

common form of acquired heart disease in all ages around the world (3). At the beginning of the 20th century, the United States saw rheumatic heart disease emerging as a leading cause of heart disease in adults younger than 40 years old, with rates gradually declining in recent decades. Two theories about the pathogenesis of rheumatic heart disease exist. The cytotoxicity theory indicates that enzymes produced by the organism, such as streptolysin O, have a direct cytotoxic effect on cardiac cells and other tissues in the body; however, it does not explain the latent period between the onset of initial infection (pharyngitis, meningitis, abscess formation) and the onset of acute rheumatic fever. The immune-mediated theory, on the other hand, reveals that there are common antigenic determinants between components of the organism (ie, M protein) and specific tissues, such as the heart, brain, and joint, resulting in immunologic cross reactivity. To diagnose acute rheumatic fever and the development of rheumatic heart disease, the Jones criteria have been revised by the American Heart Association for the initial attack. There are five major and four minor criteria with evidence of recent group A streptococcal infection (4). Diagnosis can be established if a patient has two major criteria or one major and two minor criteria, along with recent evidence. Refer to Table 1 for specific criteria (4). Carditis and chronic rheumatic heart disease are the most serious complications of acute rheumatic fever. Cardiac involvement can range from mild to fulminant in affected patients. Most cases will present with only mitral valve pathology or combined atrial and mitral valve involvement. Rheumatic carditis can be diagnosed with a new murmur, cardiomegaly, congestive heart failure, or valvular involvement. Regurgitation and valvular insufficiency are indicative of both acute and convalescent stages of acute rheumatic fever, which our patient was found to have on echocardiogram, while valvular stenosis can appear several years after initial presentation. Sydenham chorea is also a major criterion in the diagnosis of acute rheumatic fever, occurring in approximately 10%–15% of diagnosed patients. As in our patient, it usually presents as an isolated, often subtle, physical finding that is exacerbated by stress and improved with sleep, and rarely with any neurologic sequelae. It is characterized by the following clinical movements: milkmaid’s grip, which demonstrates irregular contractions of hand muscles while forming a grip around the examiner’s finger; pronation of hands with arms fully extended; and wormian tongue movements when protruded. Although these criteria have been defined with group A Streptococcus, our patient exhibited two major criteria with group C Streptococcus, namely carditis and Sydenham chorea, and was therefore diagnosed as having acute rheumatic fever and rheumatic heart disease, and was managed with thorough follow-up and guidance.

3 Table 1. Jones Criteria (4) Major Criteria

Minor Criteria

Carditis Migratory polyarthritis Erythema marginatum

Arthralgia Fever Elevated acute phase reactants: ESR, CRP Prolonged PR interval

Subcutaneous nodules Chorea

CRP = C-reactive protein; ESR = erythrocyte sedimentation rate.

Once diagnosed, all patients should be monitored closely for development or progression of carditis and placed on bed rest until signs of acute inflammation have resolved. Upon initial evaluation and diagnosis, patients should also be started on an oral penicillin or erythromycin course or be given a single intramuscular benzathine penicillin dose (5). After completion of acute therapy, patient should be started on long-term antibiotic prophylaxis, as our patient did with monthly benzathine penicillin. Sedatives, such as phenobarbital, haloperidol, or chlorpromazine, can be used for the acute phase of Sydenham chorea as needed. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS? Early recognition of rheumatic heart disease is crucial in the overall outcome of the condition and therefore knowledge of the symptoms associated with condition is also imperative. Group C Streptococcus is rarely associated with rheumatic heart disease and most children exhibiting acute onset of common symptoms, such as chorea, fever, carditis, and rash (erythema marginatum) will present to the ED first. Increased awareness and prompt recognition, as done with this child, will result in proper follow-up and adequate management of this condition in all patients.

REFERENCES 1. Feigin RD, Cherry JD, Demmler GJ, Kaplan SL. Group C and Group G streptococcal infections. In: Feigin RD, ed. Textbook of pediatric infectious diseases, 1. Philadelphia: Saunders; 2004:1151–6. 2. Arditi M, Shulman ST, David AT, Yogev R. Group C beta-hemolytic streptococcal infections in children: nine pediatric cases and review. Rev Infect Dis 1989;11:34–45. 3. Dajani AS, Ayoub E, Bierman FZ, et al. Guidelines for the diagnosis of rheumatic fever: Jones criteria, 1992 update. JAMA 1992;268: 2069–73. 4. Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE. Rheumatic fever. In: Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE, eds. Nelson textbook of pediatrics. 19th edn. Philadelphia: Elsevier; 2011:914–25. 5. Johnson CC, Tunkel AR. Viridans streptococci and groups C and G streptococci. In: Mandell GL, Bennett JE, Dolin R, eds. Principles and practice of infectious diseases. 5th edn. Philadelphia: Churchill Livingstone; 2000:2167–83.

Group C Streptococcus Causing Rheumatic Heart Disease in a Child.

Human infection with group C Streptococcus is extremely rare and a select number of cases have been reported to cause acute pharyngitis, acute glomeru...
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