Cerebral oedema with coning in diabetic keto-acidosis: Report of 2 survivors

Two children presented with a first episode of diabetic ketoacidosis. Initially both patients made a good clinical and biochemical recovery, but suddenly developed neurological signs consistent with a diagnosis of tentorial herniation. Cranial computed tomography showed signs of cerebral oedema in both cases with evidence of uncal and tentorial herniation in 1 patient, which resolved after the appropriate treatment. The excellent neurological outcome emphasises the need for early recognition and treatment of sudden onset brain oedema in diabetic keto-acidosis

Infective endocarditis in children

Infective endocarditis (IE) has high mortality and morbidity rates despite available treatment. Because of the changing predisposing cardiac abnormalities and causative organisms, a retrospective study of 29 children with this disease was undertaken to review our experience in its diagnosis and treatment. Forty-eight per cent of the patients presented with the classic clinical picture of IE. Rheumatic heart disease (69%) was the most common underlying cardiac condition. Sixty-five per cent of the blood cultures were negative, possibly because 82,5% of patients received antibiotic treatment before being referred to our institution. The organisms cultured were Staphylococcus aureus (3), S. epidermidis (2), Streptococcus viridans (2), S. faecalis (1), Pseudomonas (1) and Aeromonas (1). Two-dimensional echocardiography was found to be one of the most useful special investigations to confirm the diagnosis in children in whom IE was suspected. Despite early diagnosis and treatment, the mortality rate in this series was 24%, with serious complications occurring in a further 21% of patients. The value of having a committed individual to care postoperatively for children undergoing surgery for heart lesions cannot be overemphasised and might be an important factor in containing post-surgical IE.Articl

Sydenham's chorea - Analysis of 27 patients and a review of the literature

The first documented epidemic of Sydenham's chorea was in 1418. In 1686 Thomas Sydenham was the first to describe the condition, but it was Richard Bright who in 1831 first made the association between chorea and rheumatic fever. Chorea is one of the major diagnostic criteria for rheumatic fever. Chorea is often considered a benign self-limiting condition. Little is known about the pathophysiological process. A link between the group A β-haemolytic streptococcus through an antibody-mediated immune response targeting the basal ganglia has been described. Special investigations have been done to ascertain the pathophysiology, but none is of diagnostic value. The aims of this study were to analyse the clinical findings, the role of special investigations in the management and course of Sydenham's chorea and to review the literature. Data on 27 patients were analysed. Special investigations in these patients can be divided into those necessary to assist in diagnosing acute rheumatic fever and those to exclude other causes that mimic chorea. None of these tests was helpful in diagnosing chorea due to other causes; neither did they influence the management of these patients. In conclusion, Sydenham's chorea remains a clinical diagnosis and extensive and expensive special investigations are seldom warranted.Revie

Simultaneous occurrence of mumps meningoencephalitis and tuberculosis meningitis

Accurate early diagnosis of meningitis and institution of appropriate therapy are matters of urgency. The clinical and cerebrospinal fluid (CSF) findings in viral meningoencephalitis, however, may at times be difficult to distinguish from the early stages of tuberculous meningitis. This case report describes the diagnostic confusion arising from the unusual simultaneous occurrence of tuberculous meningitis and mumps meningoencephalitis.Articl

Genetic basis of congenital heart defects

[No abstract available]Revie

The extent of progressive familial heart block type 1 - A new perspective

New data on the inheritance of progressive familial heart block type I (PFHB1) does not favour the theory that the original immigrant (founder of PFHB1 families) introduced the genetic abnormality into South Africa and suggests that a new mutation in family member b6 was possibly responsible for the origin of the disease. If the mutation occurred in family member b6, this would reduce the number of affected family members from a previously estimated 9000 to a mere 860 with a revised prevalence of 1:5000 South Africans of European descent.Articl

A comparison of heart rate variability in affected and unaffected members of families with progressive familial heart block type I

The presence of sinus bradycardia (less than 60/min) in patients affected by progressive familial heart block type I (PFHB1) and their inability to reach a target heart rate during exercise testing, prompted an investigation of the influence of the autonomic nervous system on cardiac conduction in these family members. Forty members of PFHB1 families, 20 affected and 20 not affected, were included in the study. All were subjected to 24-hour Holter monitoring and treadmill exercise testing (Bruce protocol). Spectral and non-spectral analyses of heart rate variability were performed to evaluate the autonomic nervous system. The results showed that there were no differences between the two groups and that they had normal autonomic nervous systems. It is therefore possible to conclude that the slow heart rate at rest of affected PFHB1 family members and their inability to reach a target heart rate during exercise testing is due to a primarily diseased heart conduction system.Articl

Cervical aortic arch

A cervical aortic arch was diagnosed in an 8-year-old girl. The role of digital subtraction imaging as an ideal diagnostic tool for this condition is emphasised.Articl

Isolation of the left subclavian artery in tetralogy of Fallot and bronchial anomalies

In the normal left-sided aorta, the vertebral arteries arise from the respective subclavian arteries. Isolation of the left subclavian artery takes place when the distal subclavian artery arises as an extension of a patent ductus arteriosus. This report describes a patient with tetralogy of Fallot and a right-sided aortic arch with isolation of the left subclavian artery. In addition, the patient also had congenital tracheal and bronchial stenosis, with a horseshoe lung.Articl

Pseudocoarctation (congenital kinking) of the aorta with aneurysm formation a case report

Pseudocoarctation of the aorta with aneurysmal dilatation is a rare condition. We documented a case in an asymptomatic child who presented with widened mediastinum on chest radiography. The pseudocoarctation was documented by echocardiography and digital subtraction angiography. A review of the literature is presented.Articl