H&0 CLINICAL CASE STUDIES 616 Clinical Advances in Hematology & Oncology Volume 10, Issue 9 September 2012 Active Rheumatic Heart Disease in a Yemeni Adolescent With Sickle Cell Disease: A Case Report and Review of the Literature Moustafa Abd El-Aal Hegazi, MD Mansoura University Children’s Hospital, Mansoura, Egypt, Ibn Sina College Hospital, and Al-Jedaani Hospital, Jeddah, Saudi Arabia Address correspondence to: Moustafa Abd El-Aal Hegazi, MD, Associate Professor of Pediatrics, Mansoura University Children’s Hospital, El-Gomhoria St., PO: 35516, Mansoura, Egypt; Tel: +20101020941; Fax: +20502234092; E-mail: mhegazi712003@yahoo.co.uk. Case Presentation A 14-year-old Yemeni male adolescent presented to Al- Jedaani Hospital in southern Jeddah, Saudi Arabia, with inability to walk due to bilateral leg and ankle pain of 1-week duration. Consultation with other hospitals established him as a known patient with sickle cell disease (SCD). Te patient reported a history of recurrent similar attacks that had been diagnosed as sickle cell crises. On examination, the patient was fully conscious and in pain. His vital signs showed a temperature of 38.5 0 C, a pulse rate of 100 beats/min, blood pressure of 110/70 mmHg, and a respiratory rate of 30 breaths/min. He was pale and jaundiced without cyanosis. Bilateral ankle arthritis, tender legs, and mild hepatosplenomegaly were observed. Cardiac examination showed cardiomeg- aly (the apex was in the sixth left intercostal space in the midaxillary line), mufed frst heart sound, and grade II apical pansystolic murmur propagated to the axilla, but there was no thrill or gallop. Results of initial laboratory tests are presented in Table 1. Erythrocyte sedimentation rates (ESR) of 60.0 mm and 110.0 mm for the frst and second hour, respectively, were recorded. An electrocar- diogram (ECG) revealed sinus tachycardia, prolongation of the PR interval, and left ventricular hypertrophy. Chest x-ray showed cardiomegaly. Patients with SCD usually have a low ESR. In this patient, the high ESR, along with arthritis and cardiac manifestations, raised the suspicion of rheumatic fever. Te diagnosis was confrmed by echocardiography, which revealed a dilated left atrium and left ventricle, thickened mitral and aortic valve leafets, moderate mitral regurgita- tion, and mild aortic regurgitation with normal global ventricular functions. Tere was evidence of streptococcal infection with antistreptolysin O titer of 400 IU/mL, and a throat culture was positive for group A streptococcus. Te patient was given long-acting benzathine penicillin and prednisolone 60 mg/day for 15 days for treatment of acute rheumatic fever with carditis, cardiomegaly, and multi- valvular heart disease. Salicylates were given while predniso- lone was gradually withdrawn and were continued until the ESR became normal, with signifcant clinical improvement. Bone and Joint Manifestations in Sickle Cell Disease SCD is primarily a disease of the hematopoietic system, in which the skeleton bears the brunt of its complications. Bone changes in sickle cell anemia occur due to marrow hyperpla- sia, tissue ischemia, and infarction from vaso-occlusion. 1 Acute sickle dactylitis is frequently the frst overt evidence that SCD is present in an infant. Its associated fndings include painful, usually symmetric, swelling of the hands and feet. In young children, pain often involves the extremities; in older patients, pain in the chest, abdo- men, and back is more common. 2 Te most frequent bone and joint manifestations in children with SCD are bone and joint pain, osteomyeli- tis, joint efusion, and septic arthritis, with a frequency of 81%, 61%, 14%, and 3.5%, respectively. It can be difcult to distinguish between vaso-occlusive crises and osteomyelitis. However, osteomyelitis can be diagnosed by the presence of positive cultures or multiple areas of bone involvement, which are often symmetric, and longi- tudinal fssuring of the cortex. 1 Long bones and joints of the lower limbs are more commonly afected, especially in children younger than 10 years. 3 Cardiac and Echocardiographic Findings in Sickle Cell Disease Cardiac enlargement has been recognized as a part of the clinical spectrum of sickle cell anemia since its earliest description by Herrick. 4 Cardiac hypertrophy, interstitial fbrosis, and degenerative myocardial changes have also been described in autopsy studies. 5