Cellular origin of microfibrils explored by monensin-induced perturbation of secretory activity in embryonic primary cultures

Fibrillin is a primary component of elastin-associated microfibrils. Since microfibrils are distributed rather ubiquitously in embryonic tissues, attention has focused on the types of cells responsible for producing fibrillin. To clarify this issue, we employed monensin-induced perturbation of secretory activity in embryonic primary cultures, as this would allow examination of both the secreted protein and the formation of extracellular fibrils in the same culture. Micromasses of avian limb bud mesoderm, its ectodermal covering and several explants from other sources were cultured in the presence and absence of monensin, and evaluated immunohistochemically using antibodies against fibrillin and cell lineage markers. The results indicated that monensin perturbation induced intracellular accumulation of fibrillin and prevented the formation of microfibrils. It was shown specifically that not only mesodermally derived fibrogenic cells and myogenic cells of skeletal and smooth muscle cell lineage, but also epithelial-type cells such as endothelial and ectodermal cells, are producers of fibrillin. This dual cellular origin of fibrillin at the ectomesenchymal interface is considered significant for understanding the formation and remodeling of microfibrils originating from the basal lamina.     

(GγAγδβ)0$({}^{\rm G}{\gamma}{}^{\rm A}{\gamma}\delta\beta)^0$-Thalassemia: Report of two cases in a family

Case 1 presented with severe anemia and received an intrauterine blood cell transfusion at 33 weeks of gestation. The anemia spontaneously improved in early infancy. Case 2, the father of Case 1, had an uneventful birth with no evidence of anemia, though microcytic anemia was observed during childhood. The genetic analysis of the β-globin gene cluster identified a novel heterozygous deletion of DNA extending from the Gγ-globin gene downstream to the β-globin gene, confirming a diagnosis of (^Gγ^Aγδβ)⁰-thalassemia. In cases where thalassemia is suspected based on blood tests, a genetic diagnosis should be performed for the sake of the offspring.     

Surgical treatment of selected tumors via the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach without rerouting of the facial nerve

Neurosurgical Review - The feasibility of a novel skull base approach — the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach (NaMIPSI-A) without rerouting of...     

Epidemiological study of Candida infections in blood: susceptibilities of Candida spp. to antifungal agents, and clinical features associated with the candidemia

The purpose of this study was to evaluate the susceptibility to antifungal agents of Candida spp. isolated from blood samples from patients in our hospital, located in Osaka, Japan. We also examined the clinical background of these patients. We analyzed fungi isolated from clinical blood samples obtained in our hospital over a period of 10 years (1993 to 2002). Antifungal susceptibility testing was carried out for six agents, using the National Committee of Clinical Laboratory Standards (NCCLS) M-27-A2 method. The clinical backgrounds were reviewed using the medical records of 125 patients who were diagnosed as having candidemia. The major fungi isolated were Candida parapsilosis (39.2%) and C. albicans (30.1%), and both were sensitive to fluconazole. One strain of C. glabrata and six strains of C. krusei were resistant to fluconazole, and they constituted 4.4% of all Candida spp. isolated. With the exception of C. parapsilosis, most fungi were susceptible to micafungin, although there is no universally agreed breakpoint for this drug. Analysis of the patients' clinical backgrounds revealed that the major underlying disease was cancer (46.4% excluding hematological malignancies). C. krusei was detected almost exclusively in patients with hematological malignancies. Indwelling venous catheters had been responsible for infection in 93.6% of the infected patients. The clinical outcomes of the 125 patients were favorable in 52% and poor in 48%, and subsequent removal of the indwelling catheters was effective in about half of the patients in whom this was done, with good prognosis. To prevent mycosis and its complications, indwelling catheters should be avoided as much as possible. Attention must be paid to the possibility that resistant isolates of Candida spp. can be selected as a result of the use of antifungal agents.     

Hepatocyte growth factor: from diagnosis to clinical applications

Hepatocyte growth factor (HGF), initially identified and molecularly cloned as a potent mitogen of primary cultured hepatocytes, has multiple activities in a variety of tissues during the course of development and also in various disease states. HGF plays key roles in the attenuation of disease progression as an intrinsic repair factor. It is also evident that HGF levels are regulated under different conditions, for example, during the course of pregnancy, aging, and disease. This review focuses on the levels of HGF in normal and pathophysiological situations and examines the relationships between HGF levels and disease, disease stage, and disease prognosis. The clinical potential of HGF as a treatment for subjects with various diseases is also given attention.