Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency

BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase) deficiency, even under stable conditions. The aim of the present study was to determine whether glycerol solution was used for brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis in patients with unrecognized FBPase deficiency in Japan and to examine a long-term prognosis of the patients who had this kind of severe metabolic decompensation with or without glycerol therapy. METHODS: A retrospective study of 20 children with FBPase deficiency was conducted, based on their medical records. RESULTS: Six of the 20 children were given glycerol solution for the presence or possibility of brain edema during acute metabolic decompensation of hypoglycemia and metabolic acidosis; two of the six patients administered with glycerol were given dialysis. In four patients treated with glycerol alone without dialysis, two had no brain edema before glycerol administration but it developed later after the administration. These four patients treated with glycerol alone died or developed severe neurological complications. Fourteen patients who were not treated with glycerol solution had no brain edema and showed good prognosis. CONCLUSIONS: Glycerol solution, which contains fructose in Asian countries including Japan, should not be used as an osmotic agent for treatment of brain edema in patients who have hypoglycemia and retention-type metabolic acidosis, until FBPase deficiency is ruled out by measuring blood concentration of lactate.     

Epidemiology of school urinary screening over a 30 year period in Tokyo

BACKGROUND: A school urinary screening (SUS) system has been conducted for 30 years in Japan, but the cross-sectional data have never been reported or analyzed. The purpose of the present study was to analyze the data epidemiologically. METHODS: All elementary and junior high school children in public school in Tokyo who had SUS performed by the Tokyo Health Service Association from 1974 to 2002 (approx. 400,000-600,000 children per year) were involved. The cross-sectional data were analyzed with Pearson's correlation coefficient. RESULTS: During the first 10 years of SUS, the prevalence of abnormal urinalysis in both the first and second screenings varied widely, and the result of the second screening was affected by that of the first screening. The results of both first and second screening were highly correlated with the prevalence of hematuria, especially microhematuria, in both elementary and junior high school children. They were also correlated with the prevalence of proteinuria in junior high school children. Important factors that affected the prevalence of hematuria and/or proteinuria were reagent strips and sampling method of urinalysis. CONCLUSIONS: In order to validate SUS, attention should be paid to quality controls of the screening method, such as the selection of reagent strips, and the participants should be instructed to strictly adhere to the sampling method.     

Delta infection in Japan: Immunohistological and immuno-electron microscopic study of delta antigen in liver tissue

Abstract Although the vast majority of hepatitis B surface antigen (HBsAg) carriers of the world inhabit South-east Asia, very little is known about delta infection in this area. Therefore, a serological and immunohistological study was made in the Tokyo-Chiba area. One of 58 (1.7%) HBsAg carriers had anti-delta antibody in a high titre in serum. Delta antigen was immuno-histologically localized in the liver in two of 146 (1.4%) HBsAg carriers studied. The antigen was strongly stained in the nuclei, and positive cells were diffusely scattered throughout the liver in both cases. Neither subject was an illicit drug user: one had travelled to Italy 10 years earlier and the other had a blood transfusion during a 5-year residence in Bangkok in the past.
Thus, there is delta infection among non-intravenous drug users in Japan. Delta infection has been linked to severe liver damage, occasionally fatal. Once introduced, it could become epidemic in a country where hepatitis B virus infection is endemic, and might spread among non-drug users.     

Varicella infection complicated with meningitis after immunization

A healthy 45 month old boy who had received varicella vaccine 21 months previously developed aseptic meningitis along with an episode of varicella. The presence of viral DNA in cerebrospinal fluid (CSF) detected by polymerase chain reaction (PCR) with Southern blot hybridization confirmed the relationship between the symptoms and the CSF pleocytosis. This is the first reported case of this complication of varicella meningitis occurring in a child with documented immunization and seroconversion.     

Contribution of Monoaminergic Nervous System in Potentiation of 2-sec-Butylphenyl N-Methylcarbamate (BPMC) Toxicity by Malathion in Male Mice

Contribution of Monoaminergic Nervous System in Potentiationof 2-sec-Butylphenyl N-Methylcarbamate (BPMC) Toxicity by Malathionin Male Mice. TAKAHASHI, H., TANAKA, J., TSUDA, S., and SHIRASU,Y. (1987). Fundam. Appl. Toxicol. 8, 415–422. Malathion-inducedmarked potentiation of BPMC toxicity (about fivefold) was analyzedby measuring LD50 as an index of acute toxicity. The acute lethalityof BPMC was decreased by muscarinic blockers (atropine, methylatropine,or trihexyphenidyl) or a monoamine oxidase inhibitor (pargyline)and increased by a monoamine depleter (reserpine) or a dopaminergicblocker (haloperidol). The potentiation observed with BPMC andmalathion was decreased by the muscarinic blockers, monoaminedepleters (reserpine, -methyl-p-tyrosine), an -noradrenergicblocker (phen-tolamine), or haloperidol. The acute toxicitiesof other N-methylcarbamates MPMC (3,4-di-methylphenyl N-methylcarbamate),MTMC (3-methylphenyl N-methylcarbamate), NAC (1-naphthyl N-methylcarbamate),and XMC (3,5-dimethylphenyl N-methylcarbamate) were potentiatedby malathion to a lesser degree than that of BPMC. Atropineprotected against the lethalities of all N-methylcarbamates.Reserpine or haloperidol potentiated the lethalities of N-methylcarbamateswith a similar tendency toward malathion. When the inhibitoryeffect of each N-methylcarbamate on brain acetylcholinesterase(AChE) was compared with its LD50, among five N-methylcarbamatesBPMC had particularly strong anti-AChE activity. This characteristicof BPMC was not observed after the treatment with reserpine.These results suggest that BPMC may act not only on cholinergicnerves as an anti-AChE, but also on monoaminergic nerves whichantagonize the lethal cholinergic effect. Malathion might inhibitthe effect of BPMC on the monoaminergic nerves, thereby markedlypotentiating the lethal effect of BPMC.     

Malignancy: Subsequential Alterations of Telomeric DNA Length Correlate with Cytogenetic Response in Chronic Myeloid Leukemia Treated with Interferon alpha

Telomeres consist of simple tandem hexametric (TTAGGG) repeats and progressively shorten with cell replication. To determine a relationship between telomeric erosion and response to treatment, we measured telomere length following treatment in patients with chronic myeloid leukemia (CML) in the chronic phase. We used 70 samples of bone marrow mononuclear cells obtained from 26 patients with CML in the chronic phase subsequently. Telomere length was determined by a Southern hybridization of HinfI-digested DNA using a (TTAGGG)(4) probe, and the terminal restriction fragment (TRF) length was measured. Telomerase activity was also measured in 14 CML patients at the time of diagnosis using a telomeric repeat amplification protocol (TRAP) assay. Of the 26 patients with CML at the time of diagnosis, 14 had normal TRF lengths and the remaining 12 had shortened TRFs compared to those of age-matched normal individuals. In a group of CML patients treated with interferon alpha (IFNalpha), 80% of those who showed normal TRFs obtained cytogenetic responses. Approximately 50% of patients with shortened TRFs and treated with IFNalpha showed normalization of TRFs after IFNalpha treatment and all of them were cytogenetic responders. None of the CML patients with shortened TRFs before and after IFNalpha treatment achieved major cytogenetic response and they had high levels of telomerase activity. In the group of CML patients treated with hydroxyurea alone, although some patients showed normalization of TRF lengths after treatment, none of them showed major cytogenetic response. Telomere length before treatment may be related to CML disease severity. Cytogenetic response could be expected in CML patients with normal TRF lengths and treated with IFNalpha. Thus, measurement of telomere length after treatment might provide important information in managing CML patients.     

Treatment of a stage I testicular yolk sac tumor with vascular invasion

There is controversy concerning the treatment of stage I yolk sac tumor of the testis, particularly of those with histological factors that indicate a high risk of relapse. Usually orchiectomy alone is sufficient and adjuvant chemotherapy is unnecessary. Retroperitoneal lymphadenectomy is indicated for patients with persistently high alpha-fetoprotein. Once recurred, treatment at that time is thought to be curative. However, postoperative chemotherapy may be necessary for patients with a tumor expressing histological factors that predict possible relapse. In this paper we report on a case of a 2 year old boy whose tumor invaded the testicular veins. The patient suffered from recurrent disease but was successfully treated by chemotherapeutic regimens including cisplatin and retroperitoneal lymphadenectomy. The importance of the histological factors in making a decision on the treatment strategy for stage I testicular yolk sac tumor is discussed.