Rubinstein-Taybi syndrome: report of a case in a Nigerian child.

The first case of Rubinstein-Taybi syndrome in an African is reported. It is a syndrome of unknown etiology and prognosis, characterized by broad thumbs and great toes, mental retardation, high arched palate, distinctive facies, and undescended testes. Approximately 25 percent of patients have congenital cardiac defects, of which patent ductus arteriosus is the most common.

Childhood osteomyelitis. A five-year analysis of 118 cases in Nigerian children.

During the years 1976 through 1980, 118 children with osteomyelitis were seen at our hospital, an incidence of almost 24 cases per year. Twenty-eight of these had sickle cell disease. Males were more commonly affected than females, with a ratio of 2.1 to 1, and bones of the lower extremities were more commonly involved, than those of the upper extremities with a 2 to 1 ratio. Seventy patients were anemic, with hemoglobin levels of 10 g/dl or less. Staphylococcus aureus was the most common organism isolated from patients with sickle cell disease, as well as those with normal genotype.

Postpneumonic pleural suppuration in children.

Postpneumonic pleural suppuration is a common condition seen in paediatric practice in Nigeria. One hundred and twenty cases seen at the University of Nigeria Teaching Hospital, Enugu, over a 4-year period were reviewed. The patients were aged between 1 1/2 months and 16 years. History of antecedent measles was elicited in 27 of the children, and 70% of the patients presented to the hospital later than 7 days after the onset of symptoms of pleura suppuration. From the pleural aspirates of 106 cases (88.3%) Staphylococcus aureus was cultured in 31.2%, but there were no organisms cultured in 39.4%. Twenty-nine children were treated by chemotherapy only; 11 of them (37.9%) died. Sixty-eight cases had tube drainage of the pleural collections, with 6 deaths (8.8%). Twenty-three patients had thoracotomy, evacuation of the suppurative lesion and decortication of the lung, with no mortality. There was overall hospital mortality of 14.2%, the highest mortality being in children who had associated measles, gastroenteritis, anaemia or malnutrition. Early surgical drainage by tube thoracostomy or by thoracotomy and decortication in addition to appropriate and adequate antibiotic therapy is the treatment of choice.

Rheumatic fever in Nigerian children. A prospective study of 66 patients.

Sixty-six Nigerian children with rheumatic fever were studied between 1975 and 1979. Equal numbers of patients had acute and chronic disease. Carditis was the most common major manifestation, being present in 98% of the cases, whereas subcutaneous nodules, erythema marginatum, and chorea were relatively rare. Twelve patients died within two years of the disease's appearance and eight have become unavailable for follow-up. Our findings resemble those from other tropical countries, in contrast with results from temperate countries: rheumatic fever manifests differently and rheumatic carditis runs a more fulminant course in a person from a tropical area.

Primitive ventricle with normally related great vessels and stenotic subpulmonary outlet chamber. Angiographic differentiation from tetralogy of Fallot.

Four patients with primitive ventricle and normally related great vessels with stenotic subpulmonary outlet chamber (Holmes' heart with pulmonary stenosis) are reported. The history, physical examination, and chest x-ray film are not helpful in distinguishing Holmes' heart with pulmonary stenosis from tetralogy of Fallot. Electrocardiogram often provides the first clue to the presence of Holmes' heart; left axis deviation with or without left ventricular hypertrophy is an unusual finding in tetralogy of Fallot, but common in Holmes' heart. Selective ventriculography is diagnostic: the right ventricular outflow chamber overlies the aortic root and aortic valve in the frontal view in Holmes' heart with pulmonary stenosis, but is to the left of the aortic valve in tetralogy of Fallot; no ventricular septum can be identified in Holmes' heart. The diagnosis can be suspected in a child with clinical features of tetralogy of Fallot but atypical electrocardiogram, and can be established by angiography.

Aortico-left ventricular tunnel. Clinical profile, diagnostic features, and surgical consideration.

Case histories of 2 patients with aortico-left ventricular tunnel are reported. A review of the literature on this subject shows a unique clinical profile. The electrocardiographic (ECG) and roentgenographic features as well as the hemodynamic and angiographic data of 18 previously reported patients are reviewed. The anatomy and pathophysiology of the defect are discussed and surgical management is described. It is recommended that surgical correction be carried out as soon as the diagnosis is established.

Acute bacterial percarditis in children: report of 25 cases.

Twenty-five patients, aged 5 months to 14 years, with acute bacterial pericarditis are reported. Thirteen (52 per cent) of the patients died. The presenting symptoms, associated illness and physical findings, bacteriology, and response to therapy are reviewed. Optimum therapy consists of intravenous administration of specific antibiotics combined with surgical drainage; 90 per cent of our patients treated in this fashion survived. Antibiotic therapy alone is usually inadequate, especially in the presence of significant effusion, and among our patients only three of 10 patients so treated survived. One patient developed constrictive pericarditis 1 month after the initial attack with meningococcal pericarditis and required pericardectomy.

Etiology of right bundle-branch block pattern after surgical closure of ventricular-septal defects.

An incidence of 60 per cent of postoperative RBBB in the ECG's and available VCG's of 26 patients with isolated muscular VSD repaired was noted after ventriculotomy. In the 38 patients with VSD's near the membranous septum who underwent repair via the tricuspid valve, the incidence of postoperative RBBB was 44 per cent. Results suggested that either ventriculotomy or injury to the right bundle near the VSD can cause RBBB after surgical closure of the defect. Changes in the initial 0.02 second electrovectocardiographic forces in patients with postoperative RBBB were thought to result from central injury to the specialized conduction tissue supplying the interventricular septum. Peripheral RBBB, therefore, could be separated from central RBBB, by the appearance of the initial electrovectorcardiographic forces. For detection of these changes in initial forces, both the ECG recorded at 50 mm. per second and the Frank VCG were useful.