Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy.

Two children with influenza A-related encephalopathy were treated with a combination of mild hypothermia (deep body temperature of the forehead: 35 degrees C) and anticytokine agents (high-dose methylprednisolone and ulinastatin), while receiving amantadine. One of the cases exhibited acute necrotizing encephalopathy on computed tomography (CT). Although no severe complications occurred, correctable hypokalemia and hyperglycemia occurred in both cases.

Molecular identification of a rat novel organic anion transporter moat1, which transports prostaglandin D(2), leukotriene C(4), and taurocholate.

We have isolated a rat novel multispecific organic anion transporter, moat1. The isolated clones were originated by alternative splicing of the moat1 mRNA. The nucleotide sequences predict a protein of 682 amino acids with moderate sequence similarity to LST-1, the oatp family, and the prostaglandin transporter.

Gonadal suppression by a GnRH analogue does not alter somatic growth in rats with complete GH deficiency.

Sexual dimorphism of somatic growth in rats appears to reflect differing actions of sex steroids. However, mechanisms of gonadal steroid effects on the somatotropic axis are incompletely understood. To evaluate whether GH is involved in the effects of long-term gonadal suppression on somatic growth in rats, a GnRH agonistic analogue (GnRHa) was administered to normal Sprague-Dawley rats (controls) and to a strain of rats with complete growth hormone deficiency (GHD; n=4-6 in each group).

[A case with mild subdural hematoma presenting with a transient cluster of convulsions--problems concerning differentiation from benign infantile convulsion and benign complex partial epilepsies in infancy].

A 1-month-old girl showed frequent partial seizures of sudden onset. Continuous spikes were observed in left central area during the ictal period, although interictal EEG showed neither epileptiform discharges nor focal signs. Optimal seizures control was obtained with intravenous administration of diazepam and subsequent oral administration of phenobarbital.

[Nation-wide survey on hemimegalencephaly in Japan].

We report here 38 Japanese patients with hemimegalencephaly collected by a national survey study. All the patients were sporadic. There was no familial occurrence or sex difference.

The vasocontractive action of norepinephrine and serotonin in deep arterioles of rat cerebral gray matter.

To examine the direct effects of norepinephrine (NE) and serotonin (5-HT) on the contractility of arterioles in the gray matter of the rat cerebrum, we micro-perfused arterioles in vitro and observed the changes in luminal diameter under the stop-flow condition with constant intraluminal pressure. While the average diameter of the lumen of arterioles was 39.9 +/- 9.

The perfusion defect seen with SPECT in West syndrome is not correlated with seizure prognosis or developmental outcome.

We used interictal single photon emission computed tomography (SPECT) on 40 patients with West syndrome to determine whether cortical perfusion abnormalities are closely related to the development of West syndrome and whether they are correlated with the long-term seizure prognosis or the developmental outcome. Localized cortical perfusion abnormalities were seen in 24 patients (60%), while 15 patients (38%) were classified as normal. The remaining patient showed hyperperfusion of the basal ganglia bilaterally.

Genotypic analysis of hepatitis C virus in blood donors in Indonesia.

A study was conducted to describe the genetic diversity of hepatitis C virus (HCV) in a population of positive blood donors from throughout Indonesia. Repeat analysis by reverse transcription-polymerase chain reaction (RT-PCR) of 102 anti-HCV positive samples showed that 67 gave HCV-specific positive signals by the PCR for the 5'-untranslated genomic region of HCV. Further genotypic analysis on 64 HCV RNA-positive samples indicated that 57 belonged to the following individual genotypes: 1a, 1b, 2a, 2b, and 3b.

Biosynthesis of PF1022A and related cyclooctadepsipeptides.

PF1022A belongs to a recently identified class of N-methylated cyclooctadepsipeptides (CODPs) with strong anthelmintic properties. Described here is the cell-free synthesis of this CODP and related structures, as well as the purification and enzymatic characterization of the responsible synthetase. For PF1022A synthesis extracts of Mycelia sterilia were incubated with the precursors L-leucine, D-lactate, D-phenyllactate, and S-adenosyl-L-methionine in the presence of ATP and MgCl(2).

Glycogen storage disease type Ia: molecular diagnosis of 51 Japanese patients and characterization of splicing mutations by analysis of ectopically transcribed mRNA from lymphoblastoid cells.

Glycogen storage disease type Ia (GSD-Ia) is an autosomal recessive disorder of glycogen metabolism caused by a deficiency of glucose-6-phosphatase (G6Pase) that is expressed in the liver, kidney, and intestinal mucosa. Clinical manifestations include short stature, hepatomegaly, hypoglycemia, hyperuricemia, and lactic acidemia. To elucidate a spectrum of the G6Pase gene mutations and their frequencies, we analyzed mutations in 51 unrelated Japanese patients with GSD-Ia.

Strong immunoreactivity of platelet-derived growth factor and its receptor at human and mouse neuromuscular junctions.

Platelet-derived growth factor (PDGF) and its alpha-receptor were localized at human and mouse neuromuscular junctions (NMJs) using specific polyclonal antibodies against each, anti-PDGF-A and anti-PDGF alpha-receptor, respectively. By applying double fluorescence labeling, immunoreactivity for PDGF and its receptor was closely co-localized with acetylcholine receptors, which were identified with alpha-bungarotoxin. PDGF might be involved in the interaction between the presynaptic and postsynaptic components.

Use of cultured tubular cells isolated from human urine for investigation of renal transporter.

Aim: To facilitate the understanding of the transporter function of human renal tubular cells, we have developed a simple method using primary cultured proximal tubule (PT) cells isolated from voided urine.

Methods: PT cells grown to confluence on glass coverslips could be identified by parallel arrays of spindle cells and hemicyst formation. Brush-border gamma-glutamyl transpeptidase (gammaGTP) activity was histochemically identified.

Mutation detection by TaqMan-allele specific amplification: application to molecular diagnosis of glycogen storage disease type Ia and medium-chain acyl-CoA dehydrogenase deficiency.

We have devised an allele-specific amplification method with a TaqMan fluorogenic probe (TaqMan-ASA) for the detection of point mutations. Pairwise PCR amplification using two sets of allele-specific primers in the presence of a TaqMan probe was monitored in real time with a fluorescence detector. Difference in amplification efficiency between the two PCR reactions was determined by "threshold" cycles to differentiate mutant and normal alleles without post-PCR processing.

17beta-hydroxysteroid dehydrogenase type 1 and 2 expression in the human fetus.

The present study investigates the expression patterns of 17beta-hydroxysteroid dehydrogenase (17betaHSD) isozymes in human fetal tissues to understand how estrogenic activity is regulated in the human fetus. Using enzyme assay, high 17betaHSD activity was detected in the placenta and liver, and low levels of 17betaHSD activity were also present in the gastrointestinal tract and kidney. After Northern blot analysis, we detected the messenger ribonucleic acid for 17betaHSD type 1 (17betaHSD1) only in the placenta, whereas that for 17betaHSD type 2 (17betaHSD2) was detected in the placenta, liver, gastrointestinal tract, and urinary tract at 20 gestational weeks.

G-CSF induces apoptosis of a human acute promyelocytic leukemia cell line, UF-1: possible involvement of Stat3 activation and altered Bax expression.

Granulocyte colony-stimulating factor (G-CSF) is a cytokine that regulates the proliferation, differentiation and survival of cells in the granulocytic lineage. In this study, however, we found that G-CSF or interleukin-6 (IL-6) induced UF-1, a human acute promyelocytic leukemia cell line, into apoptosis that was confirmed by morphological features and DNA fragmentation. This rare response is demonstrated for the first time with human acute promyelocytic leukemia cell line.

CagA antibodies in Japanese children with nodular gastritis or peptic ulcer disease.

cagA(+) Helicobacter pylori strains have been linked to more severe gastric inflammation, peptic ulcer disease, and gastric cancer in adults, but there have been few studies of cagA in children. We examined the relationship between H. pylori cagA status and clinical status in Japanese children.

Urinary bile alcohol profile in infants with intrahepatic cholestasis: identification of 5beta-cholestane-3alpha,7alpha,24,25-tetrol.

Urinary bile acids and bile alcohols were examined in six infants aged between 1 and 6 mo who had intrahepatic cholestasis. Following extraction, hydrolysis and solvolysis, cholanoids were analysed by gas-liquid chromatography and gas-liquid chromatography-mass spectrometry. The relative ratio of the urinary excretion of bile alcohols to bile acids was very low (0.

Molecular analysis of Japanese patients with steroid 21-hydroxylase deficiency.

We have designed a rapid and convenient strategy to determine nine of the most common mutations in the 21-hydroxylase gene (CYP21). The frequency of the mutations was investigated in 34 Japanese patients affected with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency. We characterized 82% of the CAH chromosomes.

Detection of apoptosis in acute promyelocytic leukemia cells in vivo during differentiation-induction with all-trans retinoic acid in combination with chemotherapy.

In two patients with acute promyelocytic leukemia (APL) treated with all-trans retinoic acid (ATRA) in combination with chemotherapy, we demonstrated that cells with apoptotic morphology were recognized in a small fraction of whole blood cells (0.2-0.4%) at the regression phase of leukocytosis with mostly maturing aberrant granulocytes of APL clone origin.

Disseminated Fusarium infection identified by the immunohistochemical staining in a patient with a refractory leukemia.

The difficulty and uncertainty encountered in diagnosing a systemic mycosis often lead to a delay in starting antifungal therapy. We reported a disseminated infection of multiple fungal isolates including Fusarium species during donor leukocyte transfusion (DLT) after allogeneic bone marrow transplantation in a 20-year-old woman with a refractory leukemia. Skin lesions are the feature of Fusarium and occur in the early period of the infection.

Long-term follow-up study of serum immunoglobulin G and immunoglobulin A antibodies after Helicobacter pylori eradication.

Objective: There have been few studies concerning serum titers of anti-Helicobacter pylori immunoglobulin G (IgG) antibody >12 months after eradication of the original infection. Moreover, clinical usefulness of immunoglobulin A (IgA) antibody levels remains to be established. The purpose of this study was to investigate long-term responses of serum IgG-specific and IgA-specific antibodies to H pylori in children after eradication therapy.

[General principles of treatment and effects of childhood intractable epilepsy].

Intractable epilepsy can be defined as (1) refractory to the treatment, (2) with frequent and severe seizures, and (3) with association of mental disability. Main disorders of childhood intractable epilepsy include Ohtahara syndrome, West syndrome, Lennox-Gastaut syndrome, severe myoclonic epilepsy in infancy, and symptomatic localization related epilepsy. Our results of long-term prognosis of West syndrome showed seizure free patients were 11 of 14 (79%) in cryptogenic and 10 of 29 (34%) in symptomatic cases.