Two Cases of Cystic Fibrosis in Japanese Children: Studies on the Essential Fatty Acid and Prostaglandin Metabolism

We describe the fatty acid (FA) and prostaglandin (PG) metabolism in two Japanese cases of cystic fibrosis (CF) with or without pancreatic insufficiency (PI). The diagnosis of CF was based on the elevated sweat chloride concentration by pilocarpine iontophoresis. A 1-month-old boy (case 1) showed poor weight gain, steatorrhea and scaly dermatitis, but no respiratory symptoms were noted. He had decreased levels of serum linoleate and arachido-nate, and increased palmitoleate and oleate levels, indicating essential fatty acid (EFA) deficiency. Supplementation of fat-emulsion improved his skin lesions and the altered FA pattern within a few months, associated with the definite reduction of the urinary PC F_za levels. Until two years of age, he has been free from respiratory symptoms. A 12-year-old girl (case 2) had had recurrent respiratory tract infections due to Pseudomonas ueruginoso and Staphylococcus aureus for several years, and her pancreatic functions were preserved. The FA patterns of her serum lipid were almost within the normal range. These results indicate that 1) the altered FA composition appeared to be a secondary consequence of PI commonly complicating CF and 2) the correction of the altered FA and PC metabolism might have a beneficial effect on the respiratory function of CF patients with EFA deficiency.     

Leukocyte Function-associated Antigen-1-dependent Adhesion of Rat Ascites Hepatoma AH66F to Mesentery-derived Mesothelial Cells

Rat ascites hepatoma AH66F cells adhered better than AH130 cells to mesentery-derived mesothelial cells (M-cells), though both cells secreted M , 92,000 matrix metalloproteinase on a gelatin zymogram with similar activity. AH66F cells expressed leukocyte function-associated antigen-1 (LFA-1) and intercellular adhesion molecule-1 (ICAM-1), a ligand of LFA-1, on the cell surface, while AH130 cells had ICAM-1 alone. The adhesion to M-cells of AH66F cells was inhibited to the adhesion level of AH130 cells by anti-rat LFA-1 α- and/or β-chain monoclonal antibody (mAb) and also by anti-rat ICAM-1 mAb. This is the first report to show the LFA-1-dependent adhesion of cells other than leukocytes, because AH66F cells did not express CD45, T cell-αβ receptor or CD11b/c (Mac-1/p150,95). These results indicate that a part of the adhesion of AH66F cells to M-cells is due to LFA-1/ICAM-1 interaction, and we suggest that this characteristic feature of AH66F cells may be related to the malignant properties.     

Complete penetration of the optic chiasm by an unruptured aneurysm of the ophthalmic segment: case report

BACKGROUND: It is well known that aneurysms of the ophthalmic segment sometimes elevate the optic nerve or chiasm, and in case of large or giant aneurysms, the optic apparatus can be dramatically thinned. Nonetheless, they rarely penetrate the optic pathway completely. To our knowledge, no previous reports have dealt with the complete penetration of the optic chiasm by unruptured aneurysms of the ophthalmic segment. CASE DESCRIPTION: A 70-year-old woman presented with visual dysfunction in her left eye that she had experienced for several months. Her left visual acuity had rapidly deteriorated to the level of finger counting and visual field testing demonstrated nasal hemianopsia in the left eye and upper temporal quadrant hemianopsia in the right eye. Left internal carotid angiograms and three-dimensional digital subtraction angiograms showed an aneurysm of the ophthalmic segment projecting superomedially. Intraoperative findings revealed complete penetration of the optic chiasm by the fundus of the aneurysm. The optic pathway adjacent to the dome had become remarkably thin and dark yellow. After clipping was completed, the fundus of the aneurysm was punctured to decompress the optic chiasm. Postoperatively, patient's visual acuity in the left eye gradually recovered, but the visual field deficit persisted after the operation. CONCLUSION: This rare case demonstrates the potentially aggressive behavior of unruptured aneurysms of the ophthalmic segment. Patients with unruptured aneurysms of the ophthalmic segment who present with visual symptoms should be treated with surgical clipping to decompress the optic pathway as soon as possible.     

Age-dependent emergence of oscillatory signal flow between the primary and secondary visual cortices in rat brain slices

Developmental changes in dynamics of signal propagation between the primary (Oc1) and secondary visual cortex (Oc2) were investigated by using optical recording methods with voltage-sensitive dyes. Propagating oscillatory optical responses were evoked by our previously reported procedure, and were recorded on stimulation to white matter of Oc1 in rat visual cortex slices. In immature slices, evoked signals spread out from the stimulation site by way of deep layers, but were restricted largely to Oc1. In mature slices, however, evoked signals spread upward from the stimulation site at first, and then spread out along layer II/III, finally to penetrate Oc2. More remarkably, after this initial signal was attenuated, oscillatory responses emerged and spread back from Oc2 to Oc1 by way of layer II/III, suggesting that the origin of backpropagating oscillation is located in Oc2. The initial forward component was dependent on both N-methyl-D-aspartate (NMDA) and non-NMDA receptors, and the subsequent backward components were dependent only on NMDA receptors. These results suggest that the extent of corticocoritcal signal propagation within the visual cortex grows wider horizontally during maturation, so that information interchange may become easier between the Oc1 and Oc2.     

Age-dependent appearance of an insulo-parietal cortical signal propagation that elicits a synchronized population oscillation in the parietal cortex in rats

We investigated postnatal development of a functional connectivity from the gustatory insular cortex to the parietal cortex, which is known to contain many more neurons responding to oral somesthetic stimulation than the insular cortex, in slices obtained from 9-35-day-old rats. Field potentials were evoked by stimulation to the insular cortex. In the mature cortex, insular stimulation elicited a solitary field potential in both the insular and parietal cortices and, as the simulation continued, the initial solitary potential came to be followed by a population oscillation of field potential in the parietal cortex, but not in the insular cortex. In the immature cortex, by contrast, insular stimulation failed to evoke both the initial solitary potential and the subsequent population oscillation in the parietal cortex. In the mature cortex, application of neither thapsigargin nor AP5 prevented elicitation of the initial solitary potential in the parietal cortex, but either of them abolished the parietal oscillation. In immature cortex bathed with low Mg(2+) medium, insular stimulation elicited both the initial solitary potential and the subsequent parietal oscillation, which were both prevented by thapsigargin or AP5. Theses results suggest that the insular and parietal cortices are anatomically connected but functionally unlinked at an early postnatal stage, and that a functional linkage, dependent both on NMDA and ryanodine receptors, is formed during the first postnatal month.     

Chemiluminescent detection of induced reactive oxygen metabolite production of human polymorphonuclear leucocytes by anthophyllite asbestos

Incidences of lung cancer and pleural plaque have been reported in relation to exposure to anthophyllite asbestos. To investigate the pathogenic mechanisms of anthophyllite, chemiluminescence (CL) detection of reactive oxygen metabolite (ROM) generation of human polymorphonuclear leucocytes (PMN) stimulated by anthophyllite asbestos was determined and compared with that of other asbestos and mineral fiber samples. When anthophyllite fiber sample was mixed with the luminol-primed PMN, high levels of CL which exhibited a specific time course characterized by two separate peaks were induced. The CL induced by anthophyllite sample was greater than that induced by chrysotile, crocidolite, and amosite asbestos. We further investigated the two peaks of CL using specific inhibitors of signal transduction mechanisms. The two peaks of CL by anthophyllite sample were different in sensitivity to cytochalasin B and genistein; the former relates to the cytoskeleton-dependent mechanism and the latter has been shown to inhibit tyrosine kinase, which resides in the pathway to cause PMN activation. The strong ROM reaction of PMN by anthophyllite suggests that the surface characteristics of the fiber may participate in the pathogenic mechanisms of anthophyllite asbestos.     

Symptoms of sick house syndrome and contributory factors; study of general dwellings in Hokkaido

OBJECTIVE: The aim of this study was to clarify the "Sick House Syndrome" which has recently received increasing attention, and to investigate relationships between symptoms and the state of general dwellings in Hokkaido. METHODS: Questionnaires were sent to residents in 1775 dwellings, mainly solitary houses built or remodeled within the past few years by 24 construction companies in Sapporo and its environs, and answers was received from 564. The questionnaires included queries about building structure and characteristics, the residents' habits in the home, and subjective symptoms. We requested one resident who had the most severe symptoms in the dwelling to answer a questionnaire about symptoms. We classified the symptoms into 11 categories, and selected those that developed or were aggravated after the building or remodeling. We defined dwellings in which inhabitants complained of one or more categories of symptoms as the group with sick-house-related disease (developed or aggravated group: DA group), and those in which the inhabitants complained of two or more symptoms as the group with sick house syndrome (more than one organic symptom group: MO group)". Associations between symptoms and dwellings were then studied. RESULTS: There were 201 dwellings for which residents complained of symptoms (37.2%). Of these, 94 were in the DA group (16.7%), and 57 (10.1%) in the MO group. The symptoms that developed or were aggravated after building or remodeling of the dwellings were throat, 7.1%, dermal, 6.9%, psychoneural, 5.3%, eye, 5.1%, and nasal problems, 4.1%. Unpleasant odors form furniture were significant in both groups (DA: crude odds ratio (OR) 2.66, MO: OR 3.24). Use of aromatics was significant in group DA (OR 1.78). Condensation on windows and mold growth in the dwellings were significant in both groups (condensation on windows; DA: OR 2.98, MO: OR 3.32, mold growth; DA: OR 3.11, MO: OR 3.24). In addition, the percentage of dwellings for which residents complained of symptoms increased with signs of dampness (condensation on windows and mold growth). On logistic regression analysis, condensation on windows and mold growth were significant in both groups, and unpleasant odors from furniture in the MO group. CONCLUSION: It is suggested that symptoms of sick house syndrome are associated with high humidity such as condensation on windows and mold growth, odors from furniture and use of aromatics.     

Pleomorphic Primitive Neuroectodermal Tumor with Glial and Neuronal Differentiation: Clinical,Pathological, Cultural,and Chromosomal Analysis of a Case

This paper examines a case of pleomorphic primitive neuroectodermal tumor (PNET) with glial and neuronal differentiation in a 7-year-old girl who was clinicopathologically reported with immunohistochemical and chromosomal analysis. Clinically, a highly aggressive behavior leading to early recurrence with cerebrospinal fluid seedings was characteristic despite total removal and chemoradiation therapy. Pathologically, severe pleomorphism was noted and large ganglioid cells were predominant.Immunohistochemically, the expression of low-molecular neurofilament was recognized in the surgical specimens and increased in the recurrence. Coexpression of vimentin and neurofilament/GFAP was recognized in the culture. Chromosomal analysis showed near-diploidy, but different karyotype from that PNETs previously reported. These findings suggested that PNETs with pleomorphism and differentiation into both glial and neuronal lineages may show aggressiveness and require more aggressive therapy.