Chai-ling-tang (Japanese name: sairei-to) as an oral adjuvant.

Immunomodulating and anti-tumor activities of orally administered Chai-Ling-Tang (Japanese name: sairei-to, ST) were investigated. The oral administration of ST into mice augmented the antibody response to intraperitoneally administered 2, 4, 6-trinitrophenyl-haptenated sheep red blood cells (TNP-SRBC). Orally administered ST showed also an enhancing effect on the antibody response to TNP-SRBC administered by the oral route. In addition, orally administered ST markedly activated the peritoneal macrophages to enhanced phagocytic and lysosomal enzyme activities. A significant inhibition of tumor growth was observed in a syngeneic tumor-mouse system when ST was administered orally. These results suggest that ST has an efficiency as an oral adjuvant or an oral biological response modifier (BRM).     

Mutations of syntaxin 11 and SNAP23 genes as causes of familial hemophagocytic lymphohistiocytosis were not found in Japanese people

Although mutations of perforin, MUNC13-4 and syntaxin 11 genes have been found in children with familial hemophagocytic lymphohistiocytosis (FHL), the incidence of each genetic subtype varies in different ethnic groups. We evaluated mutations of syntaxin 11 and SNAP23 genes in 30 Japanese FHL patients. The patients had no mutations and 10% had one polymorphism (146G>A) of syntaxin 11, while no mutation of SNAP23 was observed. Our results indicate that aberrations in the SNARE system may not cause FHL in Japanese families.     

A new type of biological response modifier from Chlorella vulgaris which needs protein moiety to show an antitumour activity

An immunopotentiator obtained from Chlorella vulgaris strain CK22, showed antitumour effects against various lines of syngeneic tumours, especially by intratumour administration. The immunopotentiator exhibited far greater antitumour activity against a rechallenged tumour than against the primary-inoculated tumour in Meth A and BALB/c or CDF1 mouse systems. The antitumour effect was at least comparable to that of a streptococcal preparation, OK-432, which has been widely used for clinical immunotherapy. The active compound was fractionated by anion-exchange and affinity chromatography monitoring by the Meth A rechallenge system. The most active fraction, Q2C2, consisted of galactose-rich carbohydrate (56.1%) and protein (36.3%). Antitumour activity disappeared after protease digestion, but was stable for extreme treatments of acid, alkali, heat and carbohydrate degradation. These facts indicate that the protein moiety of the glycoprotein is mainly related to express the antitumour activity. © 1998 John Wiley & Sons, Ltd.     

Treatment of high-risk acute lymphoblastic leukemia in children using the AL851 and ALHR88 protocols: A report from the Kyushu-Yamaguchi children cancer study group in Japan

A total of 125 children, who were diagnosed as having high-risk acute lymphoblastic leukemia (ALL), were treated with two consecutive protocols designated as AL851 (1985–1988) and ALHR88 (1988–1990). All patients received induction therapy consisting of vincristine (VCR), prednisolone (PSL), daunorubicin (DNR), and l-asparaginase (l-Asp). In the ALHR88 protocol, the patients whose blasts in the bone marrow (BM) were ≥25% on day 14 of induction therapy and who were classified into T-cell type received additional cytosine arabinoside (AraC). After consolidation with intermediate-dose methotrexate (MTX), reinduction therapy including VCR, dexamethasone, and adriamycin followed by high-dose AraC was done for all patients. Intrathecal MTX and 24Gy of cranial irradiation were used to prevent central nervous system leukemia. A maintenance therapy consisting of 6-mercaptopurine, cyclophosphamide, MTX, DNR, VCR, and AraC was administered for 3 years after achieving a complete remission (CR). CR was achieved in 51/55 (92.7%) for AL851 and 68/70 (97.1%) for ALHR88. The 5-year event-free survival rates were 49.1 ± 6.7% in AL851 and 62.5 ± 6.1% in ALHR88. The factors related to a poor prognosis were a high initial leukocyte count of greater than 50 × 10⁹/L (P < 0.001), an L2 morphology of leukemic cells by FAB classification (P = 0.009), the chromosomal abnormality (P = 0.004) and high residual leukemic cells in BM (≥25%) on day 14 of induction therapy (P < 0.001). Taking these factors into consideration, more intensive protocols were started in 1990 for the patients with high-risk ALL. © 1996 Wiley-Liss, Inc.     

Transient extreme spindles in a young child with anti-NMDAR encephalitis: A case report

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a type of immune-mediated encephalitis, which is a new category of treatment-responsive paraneoplastic encephalitis. In patients with this disease, electroencephalography (EEG) shows non-specific findings, but recently, a unique EEG pattern, named the extreme delta brush, was detected in 40% of adult patients and was suggested to be specific to this type of encephalitis. Here, we describe a two-year-old boy with anti-NMDAR encephalitis, who presented with speech arrest and disturbances of gait and cognition several weeks after developing febrile convulsions. In the early stages of the disease, EEG showed 14–16?Hz, continuous, fast waves characterized by a high amplitude (200–500?µV), very diffuse spreading, and a sharp morphology, during light sleep only, which was compatible with extreme spindles. As the patient’s symptoms worsened, this finding was replaced by rhythmic, diffuse, high-voltage, slow waves. Immediately after immunomodulatory therapies, including intravenous methylprednisolone and immunoglobulin, his clinical manifestations and EEG abnormalities appeared to improve. We propose that although the extreme spindle is a non-specific finding of this type of encephalitis, early EEG monitoring might be necessary to detect not only the extreme delta brush pattern, but also non-specific findings, including extreme spindles, which would aid early diagnosis and treatment.     

Craniosynostosis in an infant with an interstitial deletion of 15q [46, XY,del(15)(q15q22.1)]

An interstitial deletion of 15q [46, XY, del(15)(q15q22.1)] was found in a malformed infant with craniosynostosis. Although the parents had normal chromosomes, the study of heteromorphic markers of chromosome 15 showed that the deleted chromosome 15 was of paternal origin. The 2 previously reported cases with an interstitial deletion of the middle portion of 15q were not complicated with craniosynostosis, and their deleted region did not include 15q15 band. The deletion of chromosome band 15q15 might be responsible for craniosynostosis.     

Evaluation of a new automated chemiluminescence immunoassay for FGF23

Fibroblast growth factor 23 (FGF23) is a hormone regulating phosphate and vitamin D metabolism. We have previously established a sandwich enzyme-linked immunosorbent assay (ELISA) for FGF23 and reported that FGF23 values are useful for the differential diagnosis of chronic hypophosphatemia. However, this ELISA has a rather narrow assay range of 3–800 pg/ml, and it was pointed out that the assay performance is not satisfactory when automatic washing is used. Here we evaluated a new automated chemiluminescence immunoassay for FGF23. This assay uses 10 μl sera or plasma samples and requires 20 min to obtain the first result. The assay was linear up to about 15,000 pg/ml and had a detection limit of 1 pg/ml. In addition, this assay showed coefficients of variation of less than 5% using samples with average FGF23 levels of 43.2–2,454.0 pg/ml. When FGF23 levels in 210 samples from chronic hypophosphatemic patients were evaluated by both the previous ELISA and this new assay, there was a good correlation of R ² = 0.96. However, FGF23 levels by the new assay showed lower values, especially in samples with high FGF23 levels. Given that the lowest FGF23 level in patients with FGF23-related hypophosphatemia was 30.8 pg/ml and that the highest FGF23 levels in patients with non-FGF23-related hypophosphatemia was 20.8 pg/ml by this novel assay, the sensitivity and specificity were 100% when the cutoff was set between 20.8 and 30.8 pg/ml. From the aspect of convenience and the coefficients of variation of this assay, we propose that the cutoff be 25 pg/ml. There results indicate that this new assay is ideal for both clinical use and clinical studies, especially when measuring many samples with high FGF23 levels.     

Staged surgical repair for extensive cardiovascular damage by syphilis

A 45-year-old man had aortic regurgitation with a syphilitic true aneurysm of the ascending to transverse arch aorta and a descending aortic aneurysm from chronic Stanford type B aortic dissection. After antibiotic therapy, two-staged surgical repair was performed and there has been no evidence of recurrence in 12 months since the second stage. We describe the successful management of extensive cardiovascular syphilitic damage.