Obeid M

Rheumatic Fever in Children: A 15-Year Experience in a Developing Country

Abstract. Clinical data from 91 patients with rheumatic fever (RF), who were hospitalized at a tertiary hospital in Lebanon between 1980 and 1995, were reviewed retrospectively. Age on hospitalization was 11.1 ± 2.9 years (mean ± SD, range 3–17 years). Nineteen patients were <6 years of age. Manifestations included carditis (93%), arthritis (39%), Sydenhams chorea (2%), erythema marginatum (4%), subcutaneous nodules (1%), fever (62%), arthralgia (55%), and acute congestive heart failure (CHF) on initial presentation (44%). Pericardial effusion occurred in 11%. There was positive family history of RF in 14%. Mitral insufficiency and aortic insufficiency occurred in 67 and 35%, respectively. Both mitral and aortic valves were involved in 30% of cases. Tricuspid insufficiency developed in 3% and pulmonary insufficiency in 1%. Mitral stenosis developed in 19%. Twenty-eight patients underwent surgical intervention: mitral valve repair and commissurotomy in 9/91 (10%), mitral valve replacement in 18/91 (20%), and aortic valve replacement in 9/91 (10%). Overall mortality was 12%: 5 following surgical intervention (3 after mitral valve surgery and 2 after mitral and aortic valve surgery). All patients that died had CHF on initial presentation (p= 0.006). This study includes hospitalized patients with predominant rheumatic heart disease. Initial presentation with CHF is a risk factor for surgical intervention and mortality. A significant high surgical intervention rate is noted that is probably related to the nature of the selected group studied. This study emphasizes the significant morbidity and death in patients with RF and carditis.

Paediatric infective endocarditis: 19-year experience at a tertiary care hospital in a developing country.

A retrospective study was undertaken to study children who presented with infective endocarditis (IE) to a university teaching hospital in Beirut, Lebanon, between January 1977 and May 1995. Of 41 patients with IE (24F, 17M), 28 (68%) were diagnosed between 1977 and 1985. Patients ages ranged from 3 to 18 y (mean age 11.3+/-2.8 y), and 13 patients were <10 y of age. Clinical presentations included: fever (in 88%), heart failure (in 39%), neurologic findings (in 20%) and embolic phenomena (in 22%). Nineteen patients (46%) had underlying congenital heart disease (CHD) with tetralogy of Fallot and pulmonary stenosis being the most common. Sixteen patients (39%) had underlying rheumatic heart disease (RHD). A total of 5 children (12%) with normal cardiac anatomy had IE. One had underlying acquired viral myocarditis with mitral insufficiency. Echocardiography showed vegetations in 60%. Blood cultures were positive in 31 patients (76%). IE occurred in three patients following cardiac surgery. In one patient it occurred within 2 mo of surgery and in the other two it occurred within 6 mo. Streptococcus viridans and Staphylococcus aureus were the two most commonly isolated bacteria. Overall mortality rate was 29% (not statistically significant between patients presenting between 1977-1985 and 1986-1995; p = 0.17). There was no statistically significant difference in mortality among the groups (five in the group with CHD, six with RHD and one with structurally normal heart). This study demonstrates that RHD is an important underlying cause of IE in children in our community. This finding is similar to those in other developing countries and different from those in developed countries. Distribution of pathogens and CHD in our study is comparable to some reports in the literature, except for the higher proportion of patients with underlying pulmonary stenosis. Bacterial endocarditis prophylaxis should be emphasized in patients with RHD or pulmonary stenosis.

A new patient with pure trisomy 4p resulting from isochromosome formation and whole arm translocation

Short arm isochromosome formation with translocation of the entire long arm of the same chromosome is an unusual constitutional abnormality that has been observed, to our knowledge, in 18 cases. Only one of these previously reported cases involved chromosome 4, resulting in pure trisomy 4p. Pure trisomy 4p has been reported in a number of cases, the majority of them due to familial chromosome rearrangements, and is associated with a distinct pattern of abnormal findings. We report here a second case of a de novo chromosome 4 whole arm translocation with short‐arm isochromosome formation, which we have delineated further by FISH studies.