Carbamoyl phosphate synthetase I deficiency: molecular genetic findings and prenatal diagnosis.

We report a Japanese boy who died at Day 28 of life because of severe carbamoyl phosphate synthetase I (CPS1) deficiency that was proven by enzyme assay. By analysis of cDNA and genomic DNA, he was shown to be a compound heterozygote with two point mutations of the CPS1 gene, 840G>C leading to an aberrant splicing and 1123C>T (predicting Q375X). The 840G>C was a mutation described in another Japanese family.

Interleukin-3 and stem cell factor modulate cell cycle regulatory factors in mast cells: negative regulation of p27Kip1 in proliferation of mast cells induced by interleukin-3 but not stem cell factor.

Objective: Interleukin-3 (IL-3) and stem cell factor (SCF) are able to promote survival and proliferation of mast cells. However, the precise signal transduction cascades leading to mast cell proliferation are not clearly understood. Thus, we sought to define the mechanism of mast cell proliferation induced by IL-3 and SCF.

Maternal serum placental leucine aminopeptidase (P-LAP)/oxytocinase and preterm delivery.

Objective: To test the applicability of maternal serum placental leucine aminopeptidase (P-LAP) as a parameter for predicting preterm delivery.

Patients And Methods: Maternal serum P-LAP activity of 61 consecutive women with preterm labor at admission were assayed and examined to assess the relationship of levels to the incidence of preterm and term deliveries.

Results: When P-LAP activity of study patient serum was at or below the 10th percentile, we found a greater ratio of preterm delivery (P=0.

Atopic NC/Nga mice as a model for allergic asthma: severe allergic responses by single intranasal challenge with protein antigen.

Since certain characters of allergic asthma are common with other allergic disorders like atopic dermatitis, the possible relationship in etiology is expected. Herein, we investigated whether NC/Nga mice, an inherent animal model for human atopic dermatitis, are inclined to allergic asthma. A single intranasal challenge of NC/Nga mice immunized with ovalbumin (OVA) resulted in an increase in plasma levels of OVA-specific IgE, and typical pathological aspects of allergic asthma characterized by infiltration of numerous eosinophils, mucus hyper production of bronchial epithelial cells.

Generalized scaling laws of the formation and effects of plasma-confining potentials for tandem-mirror operations in GAMMA 10.

The main operations from 1979 to 2000 in the GAMMA 10 tandem-mirror, characterized in terms of the high-potential mode having kV-order plasma-confining potentials and the hot-ion mode yielding fusion neutrons with 10-20 keV bulk-ion temperatures, are summarized and generalized as a result of scalings of the formation and the effects of the potentials. The wide validity of potential-formation physics from Cohen's theory and the validity of the generalized Pastukhov's theory for the effects of thermal-barrier potentials on electron confinement are verified and consolidated through electron-energy balance.

Placental leucine aminopeptidase/oxytocinase in maternal serum and placenta during normal pregnancy.

Placental leucine aminopeptidase (P-LAP), which is identical with cystine aminopeptidase as oxytocinase, was found to be homologous with rat insulin-regulated membrane aminopeptidase (IRAP) by sequence comparison. In the current study, we determined the P-LAP levels in maternal serum and placenta during healthy pregnancy. P-LAP activities in maternal serum increased with gestation and rose to the peak of 80 IU/ml at 38 weeks of gestation.

Necessity of thromboxane A2 for initiation of platelet-mediated contact sensitivity: dual activation of platelets and vascular endothelial cells.

To investigate the crucial role of platelet-derived thromboxane A(2) (TXA(2)) in initiating Ag-specific contact sensitivity (CS), a platelet-dependent CS model using genetically mast cell-deficient W/W(v) mice, was provided. In vivo treatment with BAYu3405, a TXA(2) receptor antagonist, markedly suppressed CS responses in a dose-dependent manner. This inhibitory effect occurred when BAYu3405 was administered before an early initiating phase, suggesting that TXA(2) may be a potent initiator of platelet-mediated CS responses.

Placental aminopeptidase A as a possible barrier of angiotensin II between mother and fetus.

Aminopeptidase A (AP-A EC 3.4.11.

Nerve growth factor functions as a chemoattractant for mast cells through both mitogen-activated protein kinase and phosphatidylinositol 3-kinase signaling pathways.

Despite being a well-characterized neurotrophic factor, nerve growth factor (NGF) influences survival, differentiation, and functions of mast cells. We investigated whether NGF was able to induce directional migration of rat peritoneal mast cells (PMCs). NGF clearly induced chemotactic movement of PMCs in a dose-dependent manner with the drastic morphological change and distribution of F-actin, which was completely blocked by pretreatment with Clostridium botulinum C(2) toxin, an actin-polymerization inhibitor.

Human mast cells produce matrix metalloproteinase 9.

Extracellular matrix-destructive enzymes, like matrix metalloproteinases (MMP), have been recognized in the process of inflammation and tissue remodeling and repair. The affected tissues often contain markedly increased numbers of mast cells. Although mast cells are capable of activating latent collagenase and proMMP, it has so far been unknown whether human mast cells themselves produce and secrete MMP9.

Fetal sacrococcygeal teratoma visualized by ultra-fast T2 weighted magnetic resonance imaging.

We report here a fetal sacrococcygeal teratoma found at 26 weeks of gestation. An ultra-fast T2 weighted imaging method enables the clear visualization of morphological details of the fetus without motion artifacts. Complete surgical resection was performed immediately after cesarean birth, and no evidence of tumor recurrence was confirmed at 1 year of age.

IgE hyperproduction through enhanced tyrosine phosphorylation of Janus kinase 3 in NC/Nga mice, a model for human atopic dermatitis.

IgE hyperproduction frequently observed in patients with atopic dermatitis (AD) may greatly contribute to the pathogenesis of AD, but its mechanisms are still unclear. NC/Nga mice raised in nonsterile circumstances spontaneously suffered from AD-like skin lesions with elevation of plasma IgE levels. We investigated mechanisms of the IgE hyperproduction in NC/Nga mice.

Preeclampsia with fetal death in a patient with schistosomiasis japonica.

A case of preeclampsia with fetal death at 32 weeks' gestation is reported. Liver examination of the patient revealed network patterns on ultrasonography and linear calcifications on unenhanced CT scans in the liver. These findings are typical of those of chronic schistosomal infection.

Role of nerve growth factor in cutaneous wound healing: accelerating effects in normal and healing-impaired diabetic mice.

Four full-thickness skin wounds made in normal mice led to the significant increase in levels of nerve growth factor (NGF) in sera and in wounded skin tissues. Since sialoadenectomy before the wounds inhibited the rise in serum levels of NGF, the NGF may be released from the salivary gland into the blood stream after the wounds. In contrast, the fact that messenger RNA and protein of NGF were detected in newly formed epithelial cells at the edge of the wound and fibroblasts consistent with the granulation tissue produced in the wound space, suggests that NGF was also produced at the wounded skin site.

Conservative handling of the uterus in a 10-week cervical pregnancy case.

A patient with a living 10-week old cervical pregnancy who desired to preserve fertility was successfully treated with methotrexate, intraamniotic KCI injection and endocervical curettage. In the case of living cervical pregnancy even after 10 weeks, conservative treatment remains an option, although intensive management and care should be given.

Hepatocyte growth factor in human amniotic fluid promotes the migration of fetal small intestinal epithelial cells.

Objective: Previously we reported on the abundant existence of hepatocyte growth factor in amniotic fluid. This study was conducted to clarify the effects of hepatocyte growth factor in amniotic fluid on fetal intestinal epithelial cells.

Study Design: Amniotic fluid samples were obtained from 22 cases at various gestational ages.

Involvement of hepatocyte growth factor in formation of bronchoalveolar structures in embryonic rat lung in primary culture.

To clarify the role of hepatocyte growth factor (HGF) in embryonic lung development, organoids from fetal rat lung were cultured in collagen gels with or without HGF antisense oligonucleotides. Cyst-like structures formed within 24 h in organoids isolated from fetuses after 14 days' gestation, but this was abolished by the oligonucleotide addition, apparently by interference with the endogenous expression of HGF. Electron microscopy revealed two types of structure: an alveolar type characterized by osmiophilic lamellar bodies in the cytoplasm and lumen, and a bronchial type consisting of epithelial cells bearing microvilli on their apical surfaces.

Timing of insults causing abnormal outcome in preterm infants 1989-1992.

Objective: The purpose of this study was to clarify the influence of timing of brain insults causing abnormal outcome in preterm infants.

Methods: One hundred and thirty-one preterm infants were examined. The timing of brain insult was estimated from EEG or clinical findings.

Levels of hepatocyte growth factor and its messenger ribonucleic acid in uncomplicated pregnancies and those complicated by preeclampsia.

The purpose of this study was to elucidate the possible relationship between hepatocyte growth factor (HGF) expression and the pathogenesis of preeclampsia. The concentration of immunoreactive HGF was measured and the expression of HGF messenger ribonucleic acid (mRNA) assessed in human placentas obtained from two groups: uncomplicated and preeclamptic pregnancies at various gestational weeks. In addition, the localization of HGF mRNA and c-met protein was analyzed using in situ hybridization and immunohistochemical staining, respectively.

Human amniotic fluid motogenic activity for fetal alveolar type II cells by way of hepatocyte growth factor.

Objective: To find out if hepatocyte growth factor (HGF) in amniotic fluid (HGF-AF) has a direct effect on fetal lung development, we investigated the effects of AF as well as recombinant human HGF (rhHGF) on proliferation, migration, and morphogenesis of fetal alveolar type II cells in vitro.

Methods: Amniotic fluid samples were obtained from 37 women at various gestational ages. Mitogenic, motogenic, and morphogenic activity was investigated by 5-bromo-2'-deoxyuridine incorporation, Boyden chamber assay, and culture in collagen-gels, respectively.

Increased mitochondrial damage by lipid peroxidation in trophoblast cells of preeclamptic placentas.

Lipid peroxides and their related free radicals have been implicated in the pathogenesis of placental dysfunction in preeclampsia. Recent studies suggest that the placenta is a source of the increased lipid peroxides in the maternal circulation of women with preeclampsia. We examined intracellular localization of 4-hydroxy-2-nonenal (HNE: a major aldehydic product of lipid peroxidation)-modified proteins in human placentas by immunohistochemistry, and immunoblotting.

A live birth from intracytoplasmic injection of a testicular spermatozoon.

Testicular sperm was retrieved from a man with complete epididymal obstruction, and intracytoplasmic sperm injection was performed on his wife's oocytes. In four mature treated, two fertilized eggs were obtained, and a clinical pregnancy was established with embryo transfers. One healthy girl (2715 g) was delivered by cesarean section at 38 weeks' gestation.