[Successful surgical ablation of an epicardial accessory pathway on the right coronary artery].

We present a case of successful surgical ablation of an oblique epicardial accessory pathway on the right coronary artery in a 56-year-old female patient with Wolff-Parkinson-White (WPW) syndrome, for which the radio-frequency (RF) catheter ablation had not been effective 3 times. The heart was exposed via a T-shaped sternotomy, and positioned for adequate exposure using a suction device. Epicardial mapping was performed with a multi-electrode catheter fixed on the atrioventricular sulcus. The epicardium just above the right coronary was dissected with an ultrasonic scalpel and we confirmed complete electrophysiological block of the accessory pathway. For 3 years since the surgery, there has been no recurrence of arrhythmia in the patient. Although an RF ablation through a transcutaneous intrapericardial approach can be an alternative, surgical ablation seems to be a safer and more curative approach to failed RF catheter ablation of accessory pathway. Such surgical skills should be maintained.

3beta-hydroxy-delta5 -C27-steroid dehydrogenase deficiency: diagnosis and treatment.

The aim of this study was to evaluate the effects of bile acid treatment and to obtain further information about the pathway of bile acid biosynthesis in a patient with 3beta-hydroxy-delta5-C27-steroid dehydrogenase/isomerase (3beta-HSD) deficiency by gas chromatography-mass spectrometry. Results showed that at 2 months of age, 3beta-hydroxy-5-cholen-24-oic acid (3.0 micromol/mmol Cr, 7.9%) was detected in the urine in essentially the same relative amount as 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids (3.7 micromol/mmol Cr, 9.8%) during ursodeoxycholic acid treatment combined with prednisolone. As a result, diagnosis was delayed until 18 months of age. One month later with substitution of chenodeoxycholic acid treatment, urinary 3beta,7alpha-dihydroxy- and 3beta,7alpha,12alpha-trihydroxy-5-cholen-24-oic acids decreased significantly, and subsequent improvement of liver dysfunction was accelerated. Chenodeoxycholic acid treatment is useful in 3beta-HSD deficiency. However, in the diagnosis of this disease in early life, it should be noted that the acidic pathway may be the major route for bile acid biosynthesis in the neonatal period. Diagnosis of 3beta-HSD deficiency may have been delayed by administration of ursodeoxycholic acid, resulting in prolonged diagnostic investigation in this child with cholestasis. Further, use of prednisolone may have been contraindicated.

Transmission of hepatitis C virus from mothers to infants. The Vertical Transmission of Hepatitis C Virus Collaborative Study Group.

BACKGROUND: Although there are case reports of vertical transmission of hepatitis C virus (HCV), it remains uncertain to what extent infected mothers transmit this virus to their infants. METHODS: We investigated the transmission of HCV from infected mothers to their babies by analyzing HCV RNA in the blood. Three independent studies were performed. First, 7698 parturient women were tested for anti-HCV antibodies; 53 were positive. Their 54 infants (including one set of twins) were followed prospectively for at least six months and tested for HCV disease were prospectively studied. Third, the families of three HCV-infected infants were examined retrospectively. RESULTS: Of the 53 antibody-positive mothers, 31 were also positive for serum HCV RNA: Three of the 54 babies born to these mothers (5.6 percent) became positive for HCV RNA during the follow-up period. None of the babies of the 22 women who were antibody-positive but HCV RNA-negative became positive for HCV RNA: In the second study, HCV RNA was detected in one of the six infants of infected mothers. In the third study, HCV RNA was detected in the mothers of the three HCV-infected infants. In each of the seven infected infants we studied, the genomic sequence of HCV was almost identical to that from the mother. These seven mothers had significantly higher titers of HCV RNA than did the mothers of infants with no evidence of infection (mean [+/- SD], 10(6.4 +/- 0.5) vs. 10(4.4 +/- 1.5) per milliliter; P < 0.001). CONCLUSIONS: HCV is vertically transmitted from mother to infant, and the risk of transmission is correlated with the titer of HCV RNA in the mother.

Significance of measurement of pre-S2 antigen for the prevention of vertical transmission of hepatitis B virus in infants born to HBsAg carrier mothers.

The significance of pre-S2 antigen (pre-S2 Ag) as a marker of hepatitis B virus (HBV) infection, especially in infants born to HBsAg carrier mothers who are HBeAg-negative or HBeAg-positive, was evaluated. Pre-S2 Ag was measured by enzyme immunoassay. HBsAg carrier mothers who were HBeAg-negative and HBeAb-positive were divided into two groups: group A, mothers whose infants were not infected with HBV (n = 10) and group B, mothers whose infants were infected with HBV (n = 13). Absorption rates of pre-S2 Ag in group A and B were 0.09 +/- 0.04 and 1.36 +/- 0.95, respectively. The values for pre-S2 Ag in group B were significantly higher than those in group A. Values for pre-S2 Ag among HBsAg carrier mothers who were HBeAg-positive and HBeAb-negative were also measured by reverse passive hemagglutination. In the same way, HBsAg carrier mothers who were HBeAg-positive and HBeAb-negative were divided into two groups: group C, mothers whose infants did not become HBsAg carriers (n = 15) and group D, mothers whose infants became HBsAg carriers (n = 11). The titers of pre-S2 Ag (reverse passive hemagglutination) in group C and D were 2(5.75) +/- 1.68 and 2(10.45 +/- 1.69), respectively. The values for pre-S2 Ag in group D were significantly higher than those in group C. The values for pre-S2 Ag as markers of infectivity became higher with increasing amounts of HBV-DNA.(ABSTRACT TRUNCATED AT 250 WORDS)

Hepatitis B virus mutants with precore-region defects in two babies with fulminant hepatitis and their mothers positive for antibody to hepatitis B e antigen.

Clones of hepatitis B virus (HBV) DNA were propagated from sera of two babies who developed neonatal fulminant hepatitis B, as well as from sera of their mothers who carried HBV with antibody to hepatitis B e antigen, and the precore-region sequences were determined. A point mutation from guanine to adenine, converting codon 28 for tryptophan (TGG) to a stop codon (TAG), was detected in 18 of 20 HBV DNA clones from mother and all 31 clones from baby in one family, and invariably in 55 clones from mother and three clones from baby in the other family. These results indicate that HBV mutants defective in the precore region in some carrier mothers with antibody to hepatitis B e antigen may transmit fulminant hepatitis B to their babies.