Myocardial imaging with gallium-67 citrate was used to detect myocarditis in 46 consecutive infants and children (31 boys and 15 girls, mean age 21 months) with Kawasaki disease. In all of them planar imaging (group A) was performed at 6 hours and at 48 or 72 hours after the intravenous administration of a mean (SD) dose of gallium-67 citrate (0.07 (0.02) mCi/kg). Thirty four patients (24 boys and 10 girls, mean age 21 months) also had single photon emission computed tomography imaging (group B) soon after planar imaging. The patients had been ill for from 5 days to 16 days (mean (SD) 10.5 (2.4) days in group A and 10.6 (3.0) days in group B). The colour images obtained at 48 or 72 hours were positive in 41% of group A and in 64% of group B. Among the patients with clinically suspected myocarditis, 63% in group A and 80% in group B had positive myocardial images. Single photon emission computed tomography imaging permitted the identification of tracer in the myocardium, the pericardium only, or in the heart chambers. Myocardial imaging with gallium-67 citrate, especially when used with single photon emission computed tomography imaging, is useful for the detection of myocarditis in the acute phase of Kawasaki disease.

SUMMARYTwo cases of hypoplastic coronary artery (HCA) are presented. Case 1, a 13 year old girl, died suddenly during a long distance race. She had HLCA with marked intimal thickening and an ectopic left coronary ostium above the commisure between the non-coronary and left coronary cusp at post mortem examination. The right coronary artery (RCA) was enlarged and also supplied parts of the area normally supplied by the left coronary artery (LCA). Pathological findings revealed a normal RCA and an extremely hypoplastic LCA with occlusive proliferation of the intima and a myocardial infarction of the left ventricle.Case 2, a 6 year old girl, had a history of effort angina. Selective coronary angiography was performed which failed to demonstrate the orifice of the LCA by aortography. However, the hypoplastic LCA was visualized by RCA angiography as a consequence of anomalous collaterals from the atrioventricular branch of the RCA.We postulate that HCA results from various conditions, including stenosis of the coronary artery orifice, an aberrant course between the pulmonary artery and aorta and ectopic positioning of the coronary artery ostium. In addition, HCA may also be associated with occlusive coronary artery abnormalities.

Abstract.We report an 18-year-old 46,XY phenotypic girl who has been on alternate-day dexamethasone therapy for 10 years. The patient was seen at our hospital for right-sided inguinal hernia at the age of 4 years. Biopsy of the herniated gonad showed testicular tissue, and the karyotype of the peripheral lymphocytes was 46,XY. The diagnosis of 17a-hydroxylase deficiency was established by the evaluation of adrenal steroidogenesis at the age of 6.1 years when hypertension was clearly recognized, and was confirmed later by the gene analysis of CYP17 which disclosed a compound heterozygote. The growth rate was suppressed during the initial treatment with daily administration of 0.25-0.5 mg dexamethasone.Switching to alternate-day regimen of dexamethasone 0.5 mg/dose improved height velocity. Subsequent addition of low-dose estrogen therapy induced pubertal growth spurt. The blood pressure and adrenal hormone levels remained almost within the normal range throughout the course. Adrenal function was evaluated at the age of 15 years. Plasma ACTH and corticosterone levels were high only just before the next administration, when the plasma dexamethasone concentration should be at the nadir. Since corticosterone possesses glucocorticoid activity and can work as a glucocorticoid reserve, it is assumed that this mode of dexamethasone administration can be a safe treatment for this disorder. We conclude that the patient with childhood 17a-hydroxylase deficiency can be safely and effectively treated with alternate-day dexamethasone without interfering with linear growth.

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