The genotype of HCV (III) and a reduction in the core antibody titre appear to be useful parameters for predicting the response to IFN-alpha therapy.

The relationship between late ventricular potentials (LP) and myocardial ischemic changes or ventricular arrhythmias was investigated in patients with Duchenne muscular dystrophy (DMD). Twenty-six DMD patients aged 10-33 years (mean 18.2 years) and 27 age-matched healthy volunteers were studied. Ventricular arrhythmias were detected by 24-h Holter ECGs and LPs were determined using signal-averaged ECGs. In DMD patients filtered QRS duration, late duration, and low-amplitude signal under 40 microV were significantly prolonged compared with those of the controls. The root mean square voltage of the f-QRS complex in the last 40 ms was lower in DMD patients than in the controls. None of the control subjects had LP. However, LP was detected in 8 (31%) of the 26 DMD patients. The patients with LP had more frequent ST-T depression and ventricular arrhythmias than the patients without LP. LP had 60% sensitivity and 87% specificity for documented ventricular arrhythmias. It is concluded that LP in DMD patients indicates the presence of substrate for ventricular arrhythmias associated with local myocardial fibrosis, and is useful in identifying those at high risk for malignant ventricular arrhythmias.

Retrograde flow in the abdominal aorta is increased in patients with Kawasaki disease. Further studies are needed to clarify the causal relationship between the abnormal flow and the overproduction of nitric oxide.

Objective. We studied the time course of hepatic dysfunction, seropositivity to hepatitis C virus (HCV) antibodies, viremia, and histologic evidence of hepatic injury to evaluate the course of HCV infection in children infected by blood transfusion. Patients and methods. Twenty-nine patients (ages 4 to 18 years) who underwent open-heart surgeries for congenital heart disease were grouped into three categories based on alterations in serum alanine aminotransferase (ALT) levels: Group A, acute infection; Group B, subacute infection; and Group C, chronic infection. Results. In Group C, all 13 patients had detectable HCV RNA in serum. In contrast, all patients in Group A had no detectable HCV RNA. In Group B, one of nine patients had detectable HCV RNA and two of four patients examined had persistent chronic hepatitis by histologic criteria. Antibodies directed against C100-3 antigen or core-antigen were more useful than second-generation HCV antibody assays in determining the relationship between viremia and immunologic response. Infection with HCV genotype II and the presence of higher HCV RNA copy numbers were associated with histologic evidence of hepatic damage. Conclusion. An abnormal ALT value is frequently associated with viremia, and biochemically resolved acute infection reflects clearance of HCV. However, a normal ALT does not always reflect an absence of hepatocyte damage and HCV replication in patients with subacute disease. The measures outlined in this study are useful indicators of disease activity during the chronic phase of post-transfusion HCV infection.

We report the case of a male infant with a variant of congenital dyserythropoietic anemia (CDA), who developed severe hyperbilirabinemia on the day of birth, subsequent severe anemia, and hyperferritinemia. Bone marrow and laboratory examinations revealed features of CDA including trilineage myelodysplasia and erythroblasts with a binucleated nuclear morphology and ineffective erythropoiesis. The CDA in this patient was assumed to be a new variant type because of: the lack of internuclear chromatin bridges in the erythroblasts with abnormal nuclear morphology; a negative acid serum test; the presence of erythrocyte antigen I, and the effect of splenectomy. Trilineage myelodysplasia in CDA is not known. An abnormality in the stem cells was suggested to be the cause of CDA in this case.