[Biventricular repair with end-to-side aorta to pulmonary artery central shunt for ventricular septal defect, severe pulmonary stenosis with hypoplastic pulmonary artery].

The case was 11-month-old girl diagnosed as tetralogy of Fallot with severe pulmonary valve stenosis and suffering from severe cyanosis. A catheter study demonstrated pulmonary artery (PA) was well arborized, but severely hypoplastic in spite of previous transcatheter pulmonary valve dilatation; PA index was calculated as 69 mm²/m². A central end-to-side aorta to PA shunt was created. Cyanosis was well improved, but congestive heart failure occurred after 1 month from the operation. Subsequent catheter study demonstrated pulmonary artery growth, 166 mm²/m² of PA index and major aortopulmonary collateral artery (MAPCA) coil embolization was performed. Patient underwent Rastelli type definitive repair 9 month after palliation. The central end-to-side aorta to PA shunt is reported as useful measure for promoting PA size increase and definitive repair achievement in patient with pulmonary atresia, ventricular septal defect and severely hypoplastic PA. Appropriate consideration of criteria, carefull follow up and treatments are necessary.

Evaluation of right ventricular contraction by myocardial strain in children using a two-dimensional tissue tracking method.

Two-dimensional tissue tracking makes it possible to detect myocardial strain in any direction. Consequently, this method is applicable for evaluation of myocardial dyssynchrony. This study enrolled 22 healthy volunteers (11 boys and 11 girls) ages 1.6 to 10.8 years (mean, 6.8 years). Echocardiography (subxiphoid right anterior oblique view) of the right ventricle was examined. Three tracking points were put on the right ventricle, and time-strain curves of the inflow tract (strain at the inlet) and the outflow tract (strain at the outlet) as well as time-strain curve of the pulmonary annulus diameter were made. The strain at the inlet was larger than the strain at the outlet (0.31 vs 0.15; p = 0.0003). The time to peak negative strain at the inlet was longer than at the outlet (0.48 vs 0.42 s; p = 0.001). The diameter of the pulmonary annulus shortened in systole, and the time to peak negative strain of the pulmonary annulus was longer than that of the outlet (0.48 vs 0.42; p = 0.001). There was no significant difference in the times between the pulmonary annulus and the inlet (0.48 vs 0.48; p = 0.78). Two-dimensional tissue tracking allows assessment for quantification of myocardial performance and timing of the right ventricle.

Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation.

An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.

Analysis of genetic diversity in the VP1 unique region gene of human parvovirus B19 using the mismatch detection method and direct nucleotide sequencing.

To assess the prevalent genetic types of human parvovirus B19 strains derived from various sources and their relation to particular clinical symptoms, the genetic diversity in the VP1 unique region, which is important for the neutralizing response to human parvovirus B19, was examined by the mismatch detection method using the Non-isotopic RNase Cleavage Assay (NIRCA) and direct nucleotide sequencing. Twenty three samples obtained between 1986 and 1997 were examined. Three electrophoresis patterns were observed with NIRCA. The nucleotide sequence showed that there were 14 nucleotide changes and 4 amino acid substitutions in comparison with Au strains employed as a standard strain. The nucleotide variability of all samples ranged from 0.3 to 2.7% and the amino acid variability ranged from 1.0 to 3.0%. They were classified into three types according to NIRCA. Types 1 and 3 had similar sequences, but the type 2 sequence was quite different. Although there were some nucleotide variations in the same NIRCA type, these were silent. However, there was no relationship between the clinical features and NIRCA types or between clinical features and the nucleotide sequence. All samples obtained before 1987 were NIRCA type 2. On the other hand, 19 of 20 samples obtained after 1989 were NIRCA type 1. The other sample obtained in 1992 was type 3. The results suggest that the B19 strain of type 2 disappeared by 1988 and changed to other B19 strains such as type 1 and type 3 after 1988, indicating a correlation between genome type and prevalence. NIRCA is a convenient method for screening mutations due to its simplicity and quickness.

Respiratory syncytial virus infection of human alveolar epithelial cells enhances interferon regulatory factor 1 and interleukin-1beta-converting enzyme gene expression but does not cause apoptosis.

The induction kinetics of the transcriptional activities of interferon regulatory factor 1 (IRF-1), interleukin-1beta-converting enzyme (ICE), and CPP32 by respiratory syncytial virus (RSV) infection of human type II alveolar epithelial cells (A549 cells) were analyzed semiquantitatively by reverse transcriptase PCR. The appearance of ICE and CPP32 protein in cell lysate was examined by Western blotting analysis. The induction of apoptosis by RSV infection was examined by the appearance of DNA fragmentation detected by terminal deoxynucleotidyltransferase-mediated dUTP-biotin nick end labeling. RSV moderately enhanced IRF-1 mRNA as early as 4 h after infection, and this enhancement lasted several hours. Following induction of the IRF-1 gene, ICE gene expression increased significantly, and an increase of ICE protein was observed in the RSV-infected cell lysate. These increments were observed in cells treated with live RSV but not in cells treated with inactivated RSV or control antigen. However, no infection-specific increase of CPP32 gene expression or the protein was observed. No nucleosomal fragmentation was observed in RSV-infected cells during the whole course of infection, despite the appearance of extensive cytopathic change and cell death. These observations suggest that RSV infection of human alveolar epithelial cells induces the ICE gene and its protein as a result of increased IRF-1 induction but that the increased ICE was insufficient to cause apoptosis in the RSV-infected cells. ICE might not be able to activate CPP32, which is thought to be the more important protease for apoptosis.

[Nephrotic syndrome related to chronic graft versus host disease after allogeneic bone marrow transplantation in a patient with malignant lymphoma].

A 13-yr-old boy was diagnosed as T cell lymphoma. After the second remission, he underwent BMT from an HLA-identical, MLC negative sibling donor. After BMT, he developed grade II acute GVHD. GVHD was improved by pulsed steroid therapy using prednisolone. About 12 months after BMT, he developed bronchiolitis obliterans, sicca syndrome, and leukoderma, which were related to chronic GVHD. Pulsed steroid therapy was carried out twice, and his condition improved. Twenty-seven months after BMT, he developed nephrotic syndrome. A renal biopsy was performed, and the diagnosis was histologically membranous nephropathy and focal glomerular sclerosis. The response to steroids was not satisfactory. After 5 weeks, dipyridamole was added, but proteinuria persisted. Proteinuria disappeared 8 weeks after the addition of cyclosporine. The second biopsy after 5 months of treatment revealed an improvement in the renal lesions. The patient showed a low T4 to T8 ratio of T-lymphocytes at the onset of nephrotic syndrome. However after treatment with cyclosporine, the ratio gradually increased. These findings suggested the nephrotic syndrome in this patient was related to renal involvement in the course of chronic GVHD.

Human parvovirus B19 infection associated with acute hepatitis.

BACKGROUND: Human parvovirus (HPV) B19 infection produces a range of clinical manifestations including erythema infectiosum in children. Here we describe seven children who had acute hepatitis with HPV B19 infection. METHODS: Hepatic dysfunction was noted in three children referred to our hospital during the course of erythema infectiosum caused by HPV B19 infection diagnosed by ELISA and PCR. The role of HPV B19 in the pathogenesis of hepatic involvement was investigated retrospectively by PCR assay of stored serum samples from 773 patients admitted to our hospital. FINDINGS: 15 patients admitted to our hospital from January, 1991, to June, 1992, were HPV B19 DNA positive, of whom four had acute hepatitis of unknown origin. These four patients were aged between 7 months and 5 years. Of the seven patients, infection with hepatitis A, B, or C viruses or Epstein-Barr virus was ruled out in six by virological examinations. INTERPRETATION: Epidemiological evidence suggests that HPV B19 can be the cause of acute hepatitis.

Incidence of human parvovirus B19 DNA detection in blood donors.

1000 serum samples from blood donors were tested for human parvovirus B19 (B19) DNA by a nested PCR assay: six samples were positive for B19 DNA. The frequency was 1/167 (0.6%), considerably higher than previous surveys (0.004-0.03%). Five of the six samples were also positive for anti-B19 IgM, indicating an acute phase of infection. It is recommended to screen for B19 DNA in blood products to prevent transfusion mediated viral infection for those susceptible such as immunocompromised patients and pregnant women.

Large-scale screening for human parvovirus B19 DNA in clinical specimens by dot blot hybridization and polymerase chain reaction.

Large-scale screening for human parvovirus B19 (B19) DNA in serum samples was carried out by both dot blot hybridization and the polymerase chain reaction (PCR). Dot blot hybridization was undertaken with a digoxigenin-labeled DNA probe. Serum samples from four patients were pooled and tested by a dot blot hybridization assay. When a dot was positive, each of the four samples was tested separately to identify the positive sample. The PCR template was the DNA extracted from mixed serum samples from 10 patients. When B19 DNA was positive by PCR, each of the ten samples was tested separately. A total of 7,969 serum samples were tested by dot blot hybridization and 15 samples (11 patients) were positive for B19 DNA; 7,038 serum samples were tested by PCR and 71 samples (50 patients) were positive. Large-scale screening for B19 DNA by PCR suggested a broader spectrum of clinical manifestations associated with B19 infection.

[Serological diagnosis for human parvovirus B19 infection by an enzyme immunoassay kit with recombinant antigens synthesized in a baculovirus expression system].

Propagation of human parvovirus B19 (B19) in cell cultures are not applicable to the source of viral antigens for serological assays at present. Enzyme immunoassay (EIA) kits with recombinant B19 capsids by E. coli or baculovirus expression system have been developed. We tested serum samples from the patients with erythema infectiosum and aplastic crisis by EIA kit with recombinant antigens synthesized in a baculovirus expression system (Denka Seiken Co., Tokyo, Japan). The antigens used in the kit are self-assembled recombinants containing both VP-1 and VP-2 with the same proportion as found in native B19 capsids. B19 IgM is detected by antibody capture methods and IgG by indirect methods. All of the samples were positive for B19 DNA by nested PCR. Thirty-six (97%) of the 37 patients with erythema infectiosum and all (100%) of the 4 patients with aplastic crisis were positive for B19 IgM. The EIA kit with recombinant antigens synthesized in a baculovirus expression system has proved to be reliable and useful for the diagnosis of B19 infection.