The behavior of vascular endothelial cells (EC) is an important factor in the processes involved in angiogenesis, but the regulatory mechanisms of angiogenesis, especially underlying the tubulogenesis by EC are not yet clear. Although a number of in vitro experimental models of tubulogenesis have been developed by use of cultured EC, most of those models are too complex to be easily handled and further, the culture media are usually supplemented with serum, creating problems in interpretation of experimental results. To generate a simple in vitro angiogenesis study model under serum-free culture conditions, we adapted a murine microvascular endothelial cell line, F-2, to a chemically defined medium, Cos Medium 001, and successfully established a subline of F-2, designated F-2C, which revealed a unique growth pattern. In Cos Medium 001, F-2C proliferates in a cobblestone pattern at an early growth stage, but, at a late growth stage, spontaneously differentiates to form three-dimensional honeycomblike tubular structures without the supplementation of any specific factors. The cell aggregation activity of F-2C in the presence of Ca2+ was much greater than that of F-2. The amount of subendothelial matrix deposited by F-2C was significantly higher than that by F-2, and increased prominently after the F-2C cells reached the differentiating stage of tubulogenesis. These findings indicate that F-2C is a new EC line in which tubulogenesis is spontaneously induced by the marked deposition of basement membrane analog to the subendothelial matrix and by the enhancement of presumable cadherin activity. We suggest that this cell line, F-2C, represents a simple and useful in vitro angiogenesis model.

Animals↗

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Dominantly inherited early-onset non-progressive cerebellar ataxia syndrome.

A mother and daughter with suspected dominantly inherited, early-onset, non-progressive cerebellar ataxia syndrome have been reported. A review of the literature and the clinical features of the present cases revealed the nosologic features of this rare disorder, possibly dominant inheritance, floppiness and delayed milestones preceding early-onset mild cerebellar ataxia, non-progressive clinical course, retained or even brisk tendon reflexes without pyramidal tract involvement, normal or slightly delayed intelligence, and occasional nystagmus. Neuroimaging reveals selective involvement of the cerebellum, which is prominent in the vermis and the anterior part of the hemispheres.

Adult↗

PubMedDate not supplied

Muscle pathology in Marinesco-Sjogren syndrome: a unique ultrastructural feature.

The light- and electron-microscopical findings in the skeletal muscle of a patient with Marinesco-Sjogren syndrome (MSS) are presented. Muscle biopsy specimens showed myopathy with slightly dystrophic changes including variation in fiber size, muscle fiber necrosis, regeneration and rimmed vacuole formation. Fiber type analysis with myosin ATPase staining showed a mild increase in type 2C fibers. Electron microscopy revealed autophagic vacuoles with numerous myeloid bodies, and a unique dense membranous structure associated with nuclei. We consider that this unique membranous structure is an important feature in the muscle pathology of MSS.

Biopsy↗

PubMedDate not supplied

Early-onset benign autosomal-dominant limb-girdle myopathy with contractures (Bethlem myopathy).

We report the first Japanese patients, a mother and son, with early-onset, benign, autosomal-dominant, limb-girdle myopathy with contractures (Bethlem myopathy). The clinical features revealed predominantly proximal muscle weakness--especially in the limb-girdle muscles--joint contractures increasing with age, a benign course, and the absence of cardiac involvement. Muscle histology revealed nonspecific myopathy changes without dystrophic features. Electromyogram revealed a reduced interference pattern with a giant spike suggesting a neurogenic process.

Adult↗

PubMedDate not supplied

Mosaic pattern of dystrophins in Duchenne muscular dystrophy.

Dystrophin is the gene product affected in Duchenne muscular dystrophy (DMD). Dystrophin is demonstrably absent with immunocytochemical staining and undetectable by western blotting of DMD muscles. We report an isolated 7-year-old girl with DMD. Analysis of the patient's and her mother's DNA, with probes covering the DMD gene, disclosed no deletion. We have studied dystrophin in biopsied muscle from the patient using antidystrophin antibody in combination with immunofluorescence. Random presence of normal and dystrophin-deficient fibers were indicative of mosaic expression. Dystrophin immunocytochemistry may be useful for accurate diagnosis of affected females.

Biopsy↗

PubMedDate not supplied

Skin diseases first described in Japan.

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Eponyms↗

PubMedDate not supplied

Cutaneous vasculitis in Japan.

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Female↗

PubMedDate not supplied

Pathogenesis of experimental aural cholesteatoma in the chinchilla.

Histopathological observation of celloidin serial sections of the chinchilla middle ear after treatment with propylene glycol disclosed the development of severe inflammation of the middle ear mucosa and tympanic membrane, papillary proliferation of the epidermis of the tympanic membrane and external auditory meatus, and retraction and adhesion of the tympanic membrane. The findings for the tympanic membrane, impedance testing and histopathological examination suggested that there were two types of acquired cholesteatoma formation, probably with a difference in the pathogenesis. In one type, the proliferated epidermal layer of the tympanic membrane penetrated into the middle ear cavity making tympanic perforations. In the other type, there was progressive retraction of the tympanic membrane forming a retraction pocket. We discuss the two different patterns of cholesteatoma development.

Animals↗

PubMedDate not supplied

Efficacy of long-term administration of isosorbide for Ménière's disease.

The long-term therapeutic efficacy of isosorbide, an oral hyperosmotic diuretic, was clinically evaluated in 30 patients (14 males and 16 females) who were followed up in our clinic for more than 2 years. The patients ranged in age from 14 to 73 years, with a mean age of 49.2 years. Isosorbide was given orally, continuously or intermittently, for 27-297 weeks, depending on each patient's condition. The efficacy of the drug was determined according to the evaluation criteria of the American Academy of Ophthalmology and Otolaryngology (AAOO) and the American Academy of Otolaryngology, Head and Neck Surgery (AAO-HNS) for Ménière's disease. Definitive episodes were successfully controlled in 15 of the 20 patients (75%) who received isosorbide continuously according to both the AAOO and the AAO-HNS criteria. Of the 10 patients who were given the drug intermittently, definitive episodes were successfully controlled in 6 patients (60%) according to the AAOO and in 7 patients (70%) according to the AAO-HNS criteria. During the treatment periods, hearing improved in 6 patients (20%), and tinnitus subjectively lessened in 15 patients (50%). Although some patients complained of abdominal discomfort, the side effects of this drug were minimal. Among the patients who responded well to the treatment, the doses of isosorbide could be tapered to a daily dose of 30 ml, less than one half of the usual daily of this drug. The results suggest that most patients should be asked to continue this medical treatment for at least several months before a decision can be made regarding whether the medical response is good or poor.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗

PubMedDate not supplied

Study on orthostatic dysregulation and the Schellong test in healthy young females.

We carried out a questionnaire survey regarding the symptoms of orthostatic dysregulation (OD) and administered the Schellong test to 301 healthy young females ranging in age from 18 to 21 years. OD was identified in 62 (20.6%) of the subjects based on the questionnaire. Although orthostatic dizziness was relatively common in the entire group of young females (171/301, 56.8%), this symptom was much more prevalent in the subjects with OD (58/62, 93.5%). There was also a highly significant difference between the systolic pressure decrease during the Schellong test procedure in 22 subjects with OD with frequent orthostatic dizziness and that in the 239 subjects without OD. Although the clinical value of the Schellong test in the assessment of OD and other related conditions has been brought into question because of its relatively poor specificity, we conclude that this test procedure may be useful in screening for OD or autonomic nerve dysfunction, since it presents the advantage of easy application in clinical practice.

Adolescent↗

PubMedDate not supplied

Effects of local hyperthermia on natural killer activity in mice.

The effect of local hyperthermia on natural killer (NK) activity in C3H mice was investigated, because there have been reports on both the enhancing and suppressing effects of hyperthermia on NK activity. When right hind legs of mice were treated at 43 degrees C for 45 min, NK activity was first suppressed. It reached its lowest level 2 days after the treatment, then recovered, and was significantly enhanced on the 7th day. When mice were treated at a lower temperature (41 degrees C), NK activity was enhanced even 2 days after the treatment. In addition, the suppression of NK activity, which was observed 2 days after the treatment at 43 degrees C, was diminished to some extent by i.p. injection of liposomal recombinant human superoxide dismutase (L-r-hSOD). From these results it is suggested that local hyperthermia had an enhancing effect on NK activity, which plays an important role in the anti-tumour mechanism of hyperthermia, and that transient suppression of NK activity after hyperthermia at 43 degrees C was partially cancelled by the administration of L-r-hSOD.

Animals↗