Polyclonal antibodies that had been raised against particular PDI (protein disulphide-isomerase) family proteins did not cross-react with other PDI family proteins. To evade immune tolerance to the important self-motif Cys-Xaa-Xaa-Cys, which is present in PDI family proteins, we used the phage display library [established by Griffiths, Williams, Hartley, Tomlinson, Waterhouse, Crosby, Kontermann, Jones, Low, Allison et al. (1994) EMBO J. 13, 3245-3260] to isolate successfully the phage antibodies that can cross-react with human and bovine PDIs, human P5, human PDI-related protein and yeast PDI. By measuring the binding of scFv (single-chain antibody fragment of variable region) to synthetic peptides and to mutants of PDI family proteins in a surface plasmon resonance apparatus, we identified clones that recognized sequences containing the CGHC motif or the CGHCK sequence. By using the isolated phage antibodies, we demonstrated for the first time that a lysine residue following the CXXC motif significantly increases the isomerase activities of PDI family proteins. Moreover, we demonstrated that the affinity of isolated scFvs for mutant PDI family proteins is proportional to the isomerase activities of their active sites.

We report a neonate with aneurysmal dilatation of the vein of Galen diagnosed prenatally by color Doppler sonography and MRI at 37 weeks' gestation. The child was treated by transarterial embolization of micro-coils 2 and 8 days after birth. The aneurysmal dilatation of the vein of Galen markedly decreased after embolization and the patient is developing normally at 2 years 5 months of age. Prenatal diagnosis and early intervention by transarterial embolization produced in a good outcome in this patient.

To elucidate the incidence and natural history of mitral valve prolapse (MVP) during childhood, we investigated a total of 4,238 children (aged from 1 day to 15 years) classified by age into 4 groups: Group 1 : 1 to 28-day-old full-term normal newborns (n = 108), Group 2: 6 to 18-month-old infants (n = 391), Group 3: 6 to 7-year-old children (n = 2,801), and Group 4: 12 to 15-year-old children (n = 938). The incidence of MVP was determined by videorecorded two-dimensional echocardiography in a double-blind method twice-over. There were 109 cases diagnosed as having MVP. The incidence rates of MVP were as follows: Group 1: O%, Group 2: 0.25%, Group 3: 2.1% and Group 4: 5.1%. Arrhythmias were detected in 49% (27/55) by Holter ECG, and by exercise stress test in 4.7% (2/43). Eighty-three (77%) of 108 cases in Groups 3 and 4, excluding the 1 case in Group 2, showed no symptoms. Ventricular premature contraction (VPC) was the most common arrhythmia, and was benign in all cases. A mid-systolic click (MSC), late systolic murmur (LSM), MSC -t LSM, and a pansystolic murmur were detected in 23.1%, 3.7%, 4.6% and 5.6%, respectively. Symptoms caused by MVP increased and appeared more apparently with age. Further prospective long-term follow-up studies to adulthood are necessary. population. However, there are few studies reporting the incidence and clinical presentations of MVP in children. The incidence of MVP in adults is still uncertain and published figures vary in a wide range from 1% [2] to nearly 20% [ 11. To elucidate the incidence and natural history of childhood MVP, we have investigated 4,238 healthy infants and children by two-dimensional echocardiography and have studied their clinical presentations, symptoms and incidence of arrhythmias.

Percutaneous balloon aortic valvuloplasty (BAV) was performed in 14 patients, including one critically ill infant with congenital valvular aortic stenosis (AS). BAV was effective in 13 patients (except the infant). The peak systolic pressure gradient between the left ventricle (LV) and the ascending aorta decreased from 76.6 t 21.6 to 29.5 -t 15.3 mmHg (P < 0.001). Follow-up cardiac catheterization was performed for eight patients between 1 and 3 years (1.6 k 1.1 years) after BAV. Restenosis was found in only one patient, and the efficacy of BAV continued significantly. Aortic regurgitation developed or increased in severity in 5 of 13 children immediately after BAV. Any other severe complication was not observed.Dilatation by BAV was not sufficient for the infant with critical AS, and acute myocardial infarction (AMI) in the lateral wall of the LV occurred during the BAV procedure. The infant died 3 days after the procedure due to AMI. It was concluded that the retrograde double balloon technique was superior to the retrograde single balloon technique. In two cases, the single balloon technique was ineffective because it was impossible to fix the balloon at the aortic annulus. However, the double balloon technique was effective in every patient.BAV is effective for AS in children, and an optional repeat trial may enable BAV to be the first choice for AS. Although BAV may be effective for neonates and infants with critical AS as an emergency treatment, much attention must be paid during the procedure.

Balloon pulmonary valvuloplasty (BPV) was attempted in 38 cases of congenital pulmonary valve stenosis. It was effective and was done without complication in 36 cases, however it was not effective in two cases of pulmonary valve dysplasia. The balloon used was 20-50% larger in diameter than the pulmonary valve annulus. In the seven cases in which the transvalvular