Mass Screening for Neuroblastoma and Estimation of Costs

ABSTRACT. On the basis of epidemiological data and medical costs for patients with neuroblastoma, we have calculated the cost of mass screening for neuroblastoma with high performance liquid chromatography (HPLC) compared to the cost when it is not performed. If the sensitivity of the mass screening is 80 % and 22 000 infants are screened annually the cost will be 27809000 yen ($191800). If mass screening is not performed, the cost will be 28 446 000 yen ($196 200). The difference in cost (637 000 yen or $4 400) is fairly small. If the sensitivity is 75 % and 16 500 infants are screened, the difference is also small (174000 yen or $1 200). Therefore, mass screening with the HPLC method will not be an undue financial burden. But re-screening at an older age will be done with less financially favorable results, considering that the sensitivity may not be as high as that of the first screening and that mothers are somewhat reluctant about re-screening. The balance of the cost of mass screening by qualitative methods may also be less favorable, since the detection rate is low.     

Renal Na+, K+-ATPase in Dahl salt-sensitive rats: K+ dependence,effect of cell environment and protein kinases

Na⁺, K⁺-ATPase in renal epithelial cells plays an important role in the regulation of Na⁺ balance, extracellular volume and blood pressure. The function of renal Na⁺, K⁺-ATPase in Dahl salt-sensitive (DS) rats, an animal model for salt-sensitive hypertension, and Dahl salt-resistant (DR) rats has been studied. In Na⁺, K⁺-ATPase partially purified from renal cortex, affinities and the Hill coefficients for Na⁺ and K⁺ activation were similar in DS and DR rats. Only one component of low ouabain affinity site was found in both strains, indicating the presence of the al isoform. Protein kinase C and cAMP-dependent protein kinase phosphorylated Na⁺, K⁺-ATPase α subunit in DS and DR rats, and the phosphorylation by protein kinase C was associated with an inhibition of enzyme activity. The kinetic parameters for K⁺ activation were also studied in a preparation of basolateral membranes and were found to be similar in DS and DR rats. In a preparation of cortical tubule cells, Na⁺, K⁺-ATPase activity was determined as ouabain-sensitive oxygen consumption (OS Qo₂). Maximal OS Qo₂, measured in Na⁺ loaded cells, was the same in DS and DR rats. The K₀₆ for K⁺ was significantly lower in DS than DR rats (0.163 ±0.042 vs. 0.447 + 0.061 mM, P < 0.05), indicating that factors regulating Na⁺, K⁺-ATPase activity in intact cells are altered in DS rats. Kinetic parameters for Na⁺ activation in cells were the same in both strains. In summary, the function of renal Na⁺, K⁺-ATPase molecule is not altered in DS rats. The intracellular systems that regulate renal Na⁺, K⁺-ATPase activity might be different in DS and DR rats.     

A Sensitive and Simple Determination of Human α-fetoprotein by Enzyme-electroimmunodiffusion

A simple and sensitive determination method of α-fetoprotein was developed by introducing α-fetoprotein horseradish peroxidase conjugate into electroimmunodiffusion.
The precipitin line formed was directly visualized by staining for peroxidase activities. The sensitivity was 32 ng/ml. Electrophoretic activities of AFP were analyzed by two dimensional electrophoresis applying the present method to the second electrophoresis.
AFP from normal adults and several patients was confirmed to migrate as an α-globulin as AFP of hepatoma, yolk sac tumor and fetuses did.
AFP-like activities were detected in β-γ-globulin region in some samples but these were not due to AFP-anti-AFP interactions.     

Three cases of pyogenic sacro-iliitis,and factors in the relapse of the disease

Pyogenic sacro-iliitis (PS) is a rare disease in childhood. Three cases of PS are reported that were difficult to diagnose. Scintigraphy and magnetic resonance imaging (MRI) were useful for diagnosis. One patient suffered from an episode of relapse. Seventeen other cases of PS were reviewed in the literature to investigate the incidence of abnormal imaging findings and various factors in disease relapse. It was found that the incidence of abnormal findings by scintigraphy was significantly higher than that by computed tomography (P = 0.0057). The duration of intravenous antibiotic administration of the relapse group (14.7 ± 4.7 days) was significantly shorter than that of the non-relapse group (24.3 ± 10.7 days; P = 0.0376). The statistical analysis suggested that intravenous antibiotic administration is necessary at least for 20 days to prevent a relapse of PS.     

Trial of duplication procedure for complete dentures by CAD/CAM

summary The purpose of this study was to duplicate the shapes of complete dentures by using a computer-aided design/manufacturing (CAD/CAM) system. The shapes of the complete dentures of an edentulous patient were measured using a non-contact type shape measurement system and morphological data at the interval of 0.25 mm were obtained in the X-axis and Y-axis directions. Measurement was performed from the occlusal surface and mucosal surface sides. Based on the three-dimensional morphological data, cutter paths for cutting were generated. For cutting, the three-step method consisting of rough cutting, finish cutting, and partial finish cutting was used and for duplicating the dentures the modelling wax was cut using a computerized numerical control (CNC) processor and ball-end mills with diameters of 6 mm and I mm. The method for the controlling of three axes (X, Y, and Z) of CNC machine was used, and cutting was performed only from two directions. Although further improvements are needed in the measurement and cutting in acute slope areas, the duplication of complete dentures appears to be possible using the CAD/CAM system.     

Adrenocorticoid regulation of Na+, K+-ATPase in adult rat kidney: effects on post-translational processing and mRNA abundance

The mechanisms by which adreno-corticoid hormones regulate Na⁺, K⁺-ATPase in adult kidney were studied in adrenalectomized (Adx) rats. Five days after adrenalectomy, Na⁺, K⁺-ATPase activity was significantly reduced in the renal cortex homogenate (C = 13.0±0.8 vs. Adx = 7.1±0.7 μmol Pi mg^-1 protein h^-1) and in renal microsomes (C = 30.3 ± 1.9 vs Adx = 14.6 ± 1.3 μmol Pi mg^-1 protein h^-1). Glucocorticoid replacement treatment of adrenalectomized rats with betamethasone (20 μg kg^-1 body wt twice daily for 5 days) effectively counteracted the observed reduction in Na⁺, K⁺-ATPase activity. In cortical homogenate the protein level of α1 and β1 subunits measured in immunoblots was not significantly different in Adx and control rats, indicating that 5 days after adrenalectomy the α1 and β1 subunits were present in renal cortical cells to almost normal extent but could not be assembled into a transmembrane functional unit. In support of this conclusion we found that the protein level of both the α1 and β1 subunits was significantly lower (P < 0.001 for both subunits) in microsomes from Adx than in control rats. The mRNA abundance for α1 and β1 subunits were not lower in Adx as compared to control rats 1 and 5 days after surgery. However, if Adx rats were given a single dose of betamethasone (600 μg kg^-1 body wt), a significant 2-fold increase in both α1 and β1 mRNAs was observed (P < 0.05 for both subunits). These data suggest that glucocorticoids can upregulate the mRNA of both Na⁺, K⁺-ATPase subunits but that the low renal Na⁺, K⁺-ATPase activity in adult Adx rats is mainly due to loss of glucocorticoid regulation of the post-translational processing of the enzyme.     

No linkage to the COL4A3 gene locus in Japanese thin basement membrane disease families

Summary: Patients with thin basement membrane disease (TBMD) exhibit persistent haematuria with a diffuse thinning of the glomerular basement membrane (GBM), especially of the lamina densa. It appears to be an autosomal dominant trait. It has been reported that the Goodpasture epitope, which is located in the non-collagenous domain of type IV collagen α 3 chain, may be reduced in patients with TBMD. We speculated that the candidate gene for TBMD could be the type IV collagen α 3 chain gene ( COL4A3 ), which is present closely to type IV collagen α 4 chain gene ( COL4A4 ) on chromosome 2q35–37. We conducted a linkage analysis to investigate the relationship between familial TBMD and COL4A3 gene, using COL4A3 cDNA polymorphism and a (CA)_n microsatellite marker located in the COL4A3 gene. We examined 32 individuals from four Japanese families with TBMD. There were no associations between the patients with haematuria and certain alleles of the two markers in the pedigrees of three families. It has been reported that type IV collagen α 1 chain gene ( COL4A1 ) and α 2 chain gene ( COL4A2 ) are not involved in TBMD, and that α 5 chain gene ( COL4A5 ) and a 6 chain gene ( COL4A6 ) map to chromosome X. In conclusion, our findings suggested that familial TBMD is not caused by the genetic abnormalities of type IV collagen genes isolated thus far.     

Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.