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Reactive oxygen species (ROS) and serum ferritin levels are both considered to be important biological factors in the pathogenesis of myelodysplastic syndrome (MDS). This study evaluated the levels of ROS in 40 patients with MDS (19 males and 21 females) using the Free Radical Analytical System, FRAS4, and derivatives of reactive oxygen metabolite kits. The patients' mean age was 67.3 years (range 58–86 years). The sera of 34 (85%) patients exhibited higher levels of oxidative stress than the reference range. There was a positive correlation between ROS levels and serum ferritin levels, and a negative correlation between ROS levels and haemoglobin levels. There was a negative relationship between serum haemoglobin and ferritin levels. The results indicated that iron accumulation or severe anaemia could contribute to oxidative stress in MDS patients. Iron chelation and antioxidant therapy may be suitable for the management of MDS.

Abstract: Twenty-six studies by meta-(131I)-iodobenzylguanidine scintigraphy (131I-MIBG), 26 studies by67Ga-citrate and 3399mTc-hydorxymethylene diphosphate (99mTc-HMDP) scintigraphic studies were performed for 10 patients with abdominal neuroblastoma. Comparing the131I-MIBG images obtained at 24, 48 and 72 h, the 48-h image was the most distinctive for the tumor. Intrabdominal primary lesions, which ranged from bean to fist-size, were visualized in 7/7 cases (100%) by131I-MIBG, 4/7 cases (57%) by67Ga-citrate and 4/8 cases (50%) by99mTc-HMDP before surgery and at diagnosis. In serial follow-up of these patients after starting chemotherapy,131I-MIGB detected 100% of regressing primary tumors. Studies of 5 postoperative patients showed negative images for the primary tumor in all 3 scintigraphies except one in whom131I-MIBG was positive, but not67Ga-citrate or99mTc-HMDP, for an unresectable residual tumor.131I-MIBG also detected metastatic lesions not predicted by67Ga-citrate or99mTc-HMDP and reflected tumor progression more sensitively than known tumor markers such as urinary vanillylmandelic acid (VMA), homovanillic acid (HVA), serum neuron-specific enolase (NSE) and ferritin. These findings indicate that the 48 hr131I-MIBG scintigraphy is superior to67Ga-citrate or99mTc-HMDP images and to other biochemical markers in monitoring the effect of treatment on neuroblastoma.

Objective: The current status of hemophagocytic lymphohistiocytosis (HLH) in infants and children has been studied. Study design: Eighty-two cases of pediatric HLH, for which there was no confirmed familial inheritance, were comparatively studied between 36 patients less than 2 years of age and 46 patients more than 2 years of age. Results: In all cases, persistent fever, cytopenia, liver dysfunction, and hepatosplenomegaly were the most frequently noted symptoms. Hyperferritinemia (>1000 @mg/L) and elevated blood levels of lactate dehydrogenase (>1000 IU/L) were observed in 90% and 89.7%, respectively. These figures were considerably higher than for either hypertriglyceridemia (>2 mmol/L) (50%) or hypofibrinogenemia (<1.5 gm/L) (57.4%), indicating that increased serum ferritin and lactate dehydrogenase concentrations might be good diagnostic parameters for HLH. These parameters are nonspecific but are of follow-up and prognostic value in these HLH cases. No differences were found in clinical signs and symptoms or other laboratory findings for the two age groups. Immunochemotherapy was administered in the similar regimens to patients in both groups. Of the 82 patients, 13 (15.9%) succumbed to a fatal course within 2 months after diagnosis, and Kaplan-Meir analysis for all cases predicted the overall disease-free patient survival at 4 years from the onset of disease to be 57.2% (95% confidence interval [CI], 45.1% to 69.3%). There was a slightly, but not statistically significant, poorer prognosis for the younger patients: 44.2% (95% CI, 26.0% to 62.4%) survival for the infant group versus 67.2% (95% CI, 51.8% to 82.6%) survival for the older group (p= 0.0569). Conclusions:Refinement of the treatment is mandatory to improve the outcome of HLH in both infants and older pediatric patients. (J Pediatr 1997;130:352-7

Summary A case of glycogen storage disease (GSD) type I with vasoconstrictive pulmonary hypertension is described.

We investigated the adhesion of three morphologically distinct human neuroblastoma cell lines (NCG, GOTO and SK-N-DZ) to intact fibronectin, central cell binding domain fragment (CBF) and CS peptide-IgG conjugates in the fibronectin molecule. Each cell line was found to express different integrin fibronectin receptors (α3β1,α4β1and α5β1), although similarly attached on intact fibronectin. To CBF, NCG attached well, while GOTO moderately and SK-N-DZ poorly attached. Only GOTO adhered to CS1-IgG. RGDS inhibited the spreading of NCG and SK-N-DZ on intact fibronectin, but it barely inhibited that of GOTO. The analysis by fluorescence-activated cell sorting (FACS) revealed that NCG expressed abundant α3β1and α5β1, but little α4β1, while GOTO expressed a large amount of α4β1as well as α5β1. SK-N-DZ was undetectable in any of these molecules, but expressed αvβ1, which was identified by immunoprecipitation and immunoblotting. Polyclonal antibody to αvβ3inhibited the adhesion of SK-N-DZ but not that of NCG or GOTO on intact fibronectin. These results suggest the existence of a distinct mechanism of cell adhesion to fibronectin among human neuroblastoma cell lines. It remains to be determined if such heterogeneous adhesion properties are related to the unique metastatic character of human neuroblastoma.

The effects of various cations (Mg2+, In3+, Yb3+, Tm3+, Er3+, Ho3+, Gd3+, Nd3+, La3+, Bi3+, and Ga3+) were examined as dopants into the B site of perovskite barium zirconate. The solubility of In, Yb, Tm, Er, and Ho, whose ionic radii are close to that of zirconium ion, into the B site is more than 0.075 (XMO1.5=0.075)at 1600°C. In contrast, when there is a large difference of ionic radii between trivalent cations (Nd3+, La3+, Bi3+, and Ga3+) and zirconium ion, the solubility of trivalent cations into barium zirconate is less than XMO1.5=0.075. The microstructure of sintered pellets and their conductivity in wet atmosphere were investigated. We could divide dopants into two classes based on the extent of proton conductivity and microstructure: “Y-type” and “Sc-type” dopants. Yb, Ho, Er, and Tm belong to Y-type dopants, which have a microstructural mixture of coarse and fine grains and higher proton conductivity. In belongs to Sc-type dopants, which have well-grown grains and lower proton conductivity.

Scandium and yttrium co-doped barium zirconate [BaZr0.85ScxY0.15-xO3-δ(x=0,0.05,0.075,0.10,0.15)] have been investigated in terms of phase relationship, microstructures, and electrical conductivity. The bulk conductivity of the scandium and yttrium co-doped barium zirconate increased with the dopant ratio of yttria. BaZr0.85Sc0.05Y0.10O3-δhad the highest grain-boundary conductivity among the scandium and yttrium co-doped barium zirconates in this study. But, BaZr0.85Sc0.15O3-δ, BaZr0.85Sc0.10Y0.05O3-δ, BaZr0.85Sc0.75Y0.075O3-δ, and BaZr0.85Sc0.05Y0.10O3-δconsisted of a single cubic perovskite phase at 1600°C and their densities of grain-boundary were smaller than that of BaZr0.85Y0.15O3-δ. From the observation of microstructure and results of grain boundary-conductivity measurement, we can say that yttrium is a dopant that increases specific grain-boundary conductivity and bulk conductivity, and scandium is a dopant that increases the grain size. Thus, there is a trade-off relation between the grain size and specific grain-boundary conductivity based on the mixing ratio of scandia to yttria. The total conductivity of BaZr0.85Sc0.05Y0.10O3-δat 600°C was estimated to be 1.6×10−2Scm−1, which is the highest-class conductivity among reported trivalent cation-doped barium zirconates.

Typical microstructures of BaZr0.85Y0.15O3−δsintered for 4–24hat 1600°Cwere mixtures of larger and smaller grains. Well-grown and homogeneous grains of BaZr0.85Y0.15O3−δwere observed by adding an extra amount of barium oxide from stoichiometric composition or extending the sintering time to 100h. Such an extra amount of barium oxide is expected to decrease chemical stability in a wet atmosphere, and we observed the precipitation of yttria after 100hsintering. However, we did not meet such difficulties in sintering BaZr0.85Sc0.15O3−δ. Large and uniform grains were observed in BaZr0.85Sc0.15O3−δby sintering for 24h.

Tissue content of cyclic AMP was as much as 10 times greater in ganglioneuroma than in neuroblastoma. This high cyclic AMP in ganglioneuroma was not significantly different from that of sympathetic ganglia.