Complete Atrioventricular Block in an Adolescent With Rheumatic Fever

Rheumatic fever is an acute inflammatory sequela following a group A, β-hemolytic streptococcal infection. Rheumatic fever is characterized by polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum as the major diagnostic criteria. Rarely, advanced heart block may also occur. A 13-year-old boy was admitted to the Pediatric Department for evaluation and management of complete atrioventricular block. The patient had exertional dyspnea for 1 month. Based on the findings of mitral regurgitation, fever, elevated acute phase reactants, and a high antistreptolysin O titer, the patient was diagnosed with rheumatic fever. A benzathine penicillin injection was administered, as well as salicylate therapy. On the 5th day of hospitalization, the electrocardiogram revealed a normal sinus rhythm with a 1st degree atrioventricular block. After discharge, the electrocardiogram normalized with the monthly penicillin injections. Herein we report a case of complete atrioventricular block associated with rheumatic fever. The heart block resolved without specific cardiac treatment, other than a non-steroidal anti-inflammatory medication

Beneficial use of immunoglobulins in the treatment of Sydenham chorea

This double case report indicates that treatment with intravenous immunoglobulins (IVIG) is effective in patients with Sydenham chorea (SC). SC is a rare but impressive clinical manifestation following streptococcal infection. This movement disorder characterised by chorea, emotional lability and muscle weakness, is one of the major criteria of acute rheumatic fever. Treatment of SC is typically limited to supportive care and palliative medications. Curative treatment is still in the experimental stage. Recent research on patients with SC proved that antibodies against the group A streptococcus cross-react with epitopes of neurons in the basal ganglia, namely, intracellular tubulin and extracellular lysoganglioside. Therefore, immune modulating therapy by means of prednisone, plasma exchange and IVIG are mentioned in the literature as possible effective treatment. Beneficial effect of IVIG has been shown in several diseases with molecular mimicry as the underlying pathophysiology. In this paper, we describe two girls aged 11 and 13 years, respectively, who presented with SC having severe disabilities in their daily live. We treated both patients with IVIG 400 mg/kg/day for 5 days. Treatment was tolerated well and had a pronounced positive effect. Shortly after the drug was administered, all signs and symptoms disappeared in both patients. Based upon these patients, we highlight IVIG as a serious treatment option for SC

Diagnosis, Treatment, and Long-term Management of Kawasaki Disease: a Scientific Statement for Health Professionals from the American Heart Association

BACKGROUND: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in approximately 25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS: To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS: These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances