[Clinical investigation on childhood HCM; long-term follow-up study with special reference to electrophysiology, histopathology by endomyocardial biopsy and ambulatory and exercise electrocardiography].

In order to determine the prognosis and risk factors of childhood HCM, we investigated clinical parameters, such as the age of onset, symptoms and signs related to HCM, dysrhythmias and ST-T and changes by Holter ECG and exercise ECG, electrophysiological study and histopathological study by endomyocardial biopsy. Heart murmur was the commonest sign although only one patient revealed a significant left ventricular outflow tract gradient of more than 20 mmHg. The peak age of patients diagnosed as having HCM was 10 to 15 years. The major ECG manifestations of our series showed specific findings and in some cases ECG changed remarkably during the follow-up period. The diagnosis of apical hypertrophy should be made with caution in childhood. Holter ECG seemed to be indispensable for the follow-up of childhood HCM because Holter ECG in our patients manifested such significant findings as ventricular premature contractions or couplets or ST depression. The degree of clinical severity did not always correlate with the histopathological severity obtained by endomyocardial biopsy. As HCM in childhood is heterogeneous and multifaceted, there is no one therapy, and prognosis and proper treatment with close observation should be carried out in each individual case.

Myelopathy, ascites and pleural effusion in systemic lupus erythematosus.

A 12-year-old girl with transverse myelopathy, massive pleural effusion and ascites as the presenting features of systemic lupus erythematosus (SLE), is described. All these features developed within a week, but other manifestations of SLE such as malar rash and arthritis were not seen during the illness. Investigations revealed positive direct Coombs' test, high titer of antinuclear antibodies, elevation of serum anti-DNA antibodies, depressed complement activity, thrombocytopenia and proteinuria, indicating that she had SLE. A week after starting treatment with prednisolone 35 mg/day, the thrombocytopenia improved, and following an increase in dosage to 60 mg/day the pleural effusion and ascites diminished after two weeks, and the serological abnormalities improved after two months. The neurological disturbances were unchanged in spite of injections of methylprednisolone 1 g/day for three days from the 89th day of her illness. Review of other cases reported to be improved neurologically, suggested that the earlier initiation of corticosteroids in higher dosage might be beneficial in transverse myelopathy in SLE.