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Biomedical subjects

M Miyao

Publications and source records attributed to M Miyao.

At least 127 records · Page 7Linked to original sources

[A case of generalized komuragaeri disease (Satoyoshi disease) treated with glucocorticoid].

Generalized komuragaeri disease (Satoyoshi disease) is a rare disorder of unknown etiology, characterized by painful muscle spasms, alopecia, diarrhea and various endocrine disorders. We administered glucocorticoid to a girl with this disease, resulting in a marked improvement of all clinical features. The patient was a 15-year-old girl. Since the age of 13 years, she had had intermittent painful muscle spasms, which affected any skeletal muscles 5 to 15 times a day at exercise and at rest and lasted for a few minutes. At the age of 14 years, she had idiopathic thrombocytopenic purpura which responded to the glucocorticoid treatment. Amenorrhea and orthostatic hypotension developed at the age of 14 years. Then the loss of body and head hair was noticed and progressed slowly. She had not experienced severe diarrhea. On admission, her physical and neurological examinations showed no abnormalities except for the thin hair and frequent muscle spasms. Laboratory examinations showed elevated levels of serum creatine kinase and aldolase, positive antinuclear antibody of speckled pattern and a mild disturbance in carbohydrate absorption. Endocrinological tests suggested the dysfunction of hypothalamus as a cause of amenorrhea. Electromyogram showed large action potentials on spasms. She was treated with glucocorticoid, 2 mg/kg on alternate days. The muscle spasms decreased gradually in frequency and duration in 1 month of treatment, and disappeared in 4 months. The growth of her hair was noticed and orthostatic hypotension disappeared in 4 months. Menstruation became regular in 7 months. The muscle spasms worsened when the dosage of glucocorticoid was reduced, and they improved on the increased dosage. She was free of symptoms at 6 months after the successful diminution of glucocorticoid. The etiology of this disease has not been revealed. The association of autoimmune disorders and the responsiveness of all clinical features to glucocorticoid suggest that an autoimmune process is involved in the pathogenesis of generalized komuragaeri disease.

Adolescent↗

Skin sympathetic activity in the tibial nerve triggered by vibration applied to the hand.

The effect of vibration applied to the hand on skin sympathetic activity (SSA) in the lower limbs was studied in five healthy subjects. SSA from the right tibial nerve at the popliteal fossa, plethysmogram from the right index toe and perspiration from the sole of the right foot were measured when vibration of 100 m/s2 at 60 Hz was applied to the left palm for 1 min. The SSA response to vibration exposure differed among subjects, but every subject showed an increase in SSA from the tibial nerve when vibration was applied to the hand. Decrease in amplitude of plethysmogram from the toe was also found in all subjects. One subject displayed a remarkable increase in perspiration on the sole of the foot together with a great increase in SSA. The present findings indicated that even vibration exposure of the hand triggers sympathetic activity in the tibial nerve innervating the foot, and causes vasoconstriction of the toe and perspiration on the sole of the foot.

Adult↗

Serial imaging of Menkes disease.

The patient developed myoclonic seizures at 3 months of age and his hair demonstrated the pili torti pattern. The low serum copper content and ceruloplasmin confirmed the diagnosis of Menkes disease. The characteristic signs on the images were: high echo level regions in the cerebrum as detected by brain ultrasonography, low density areas of white matter detected by CT scan, and low signal intensities of white matter by both T1 and balanced MR images.

Brain↗

Multi-institutional survey of the Rett syndrome in Japan.

The results of the first multi-institutional survey of the Rett syndrome in Japan are reported. The survey was performed during 1985 and 1986. Eighty-nine cases were collected, of which 54 cases were confirmed to have the Rett syndrome. The ages of the patients ranged between 1 and 26 years. The clinical onsets occurred after 18 months of age in 28 cases, and before the age of one year in 26. No familial cases nor consanguinity was found. The incidence of perinatal abnormalities among the patients was not significantly higher than in other diseases in which no perinatal factors are involved.

Adult↗

Pontocerebellar hypoplasia associated with infantile motor neuron disease (Norman's disease).

A Japanese female, floppy since birth, died at the age of 1 year and 10 months. Fasciculation of the tongue, neurogenic patterns on an electromyograph, and an empty posterior fossa on a cranial computerized tomogram suggested a motor neuron disorder resembling Werdnig-Hoffmann disease with cerebellar hypoplasia. Autopsy revealed a very small cerebellum and brain stem. The cerebellar cortex showed thin molecular and granular layers with total absence of Purkinje cells. Degeneration of the motor neurons with central chromatolysis, a change typical of Werdnig-Hoffmann disease, was noted throughout the anterior horn of the spinal cord as well as in the motor nuclei of the brain stem. The clinical features and pathological findings of this case were almost identical with those first detected and described by Norman in 1961. Six similar autopsy cases have been reported since the original description. In addition to pontocerebellar hypoplasia, the presence of severe mental retardation and a probable autosomal recessive inheritance make the disease a distinct entity, which we have called Norman's disease.

Abnormalities, Multiple↗

[Radiographic evaluation of 12% Au-Ag-Pd alloy cast lower denture plates constructed by dental student as a laboratory exercise].

We evaluated 12% Au-Ag-Pd alloy cast lower denture plates prepared by dental students in laboratory exercises for casting defects. First, we determined the most suitable exposure conditions for nondestructive radiographic test of 12% Au-Ag-Pd alloy. Subsequently, 130 plates were nondestructively evaluated by radiography under the most suitable exposure conditions. In radiographic testing using X-Omat RP films (Eastman Kodak Co.) and X-Omatic Fine Screen (Eastman Kodak Co.), the most suitable exposure conditions involved a target-to-film distance of 1.9 m, tube voltage of 148 Kvp, tube current of 10 mA, and exposure time of 1.5 sec. Preliminary radiographic examination of artificial casting defects revealed semispherical defects in the diameter of 0.25 mm in 1.6 mm-thick 12% Au-Ag-Pd alloy plates and detects of 0.26 mm in 2.4 mm thick plates. In the 130 cast plates, 56 casting defects were detected. These defects were found at the left webbing part in 40 (30.8%), right webbing part in 36 (27.7%), lingual bar in 7 (5.4%), I-bar in 4 (3.0%), and at other regions of the metal frames in 3 (2.3%) cases. These results indicate that nondestructive radiographic testing is as effective in detection of casting defects in 12% Au-Ag-Pd alloy cast plates as it is in Co-Cr and Ni-Cr alloy plates. This method was also found to be effective in evaluation of the practice metal frames constructed by dental students.

Dental Casting Technique↗

Congenital hypoplasia of the dorsal pancreas: with special reference to duodenal papillary dysfunction.

We report a case of dorsal pancreatic hypoplasia complicated with atresia of the vagina, type-A chronic atrophic gastritis, duodenal papillary dysfunction, and insulin-requiring diabetes mellitus, in a 32-yr-old woman. The laboratory data showed elevated hepatobiliary enzymes. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a slightly dilated common bile duct and a short major pancreatic duct connected with a minor pancreatic duct. Ultrasonography and computerized tomography could not identify any pancreatic tissue in the region of the body or tail of the pancreas. The pancreatic tissue weight calculated by the serial thin slice of computerized tomography was 43.1 g, approximately 45% of the standard Japanese adult pancreas. Reevaluated pancreatic exocrine function based on this weight showed a hypersecretory state. The pancreatic ductal pressure was slightly increased, and the motility of the sphincter of Oddi (SO) was abnormal when measured with a 4Fr. microtransducer inserted through a duodenoscope. These findings suggest that dysfunction of the sphincter of Oddi may play some role in the pathophysiology in the hypoplasia of the dorsal pancreas and pancreaticobiliary diseases associated with it.

Adult↗

[Endocrinological analysis of chronic hypernatremia in two cases of hydranencephaly].

We reported two infants with hydranencephaly and chronic hypernatremia. Their plasma sodium concentration gradually increased during the first week and remained between 150-160 mEq/L thereafter. They showed no signs of thirst. A water deprivation test demonstrated low urine osmolality and low plasma ADH concentration despite markedly elevated plasma osmolality in both cases. Urine was significantly concentrated when vasopressin was given. Thus, it was concluded that both thirst mechanism and ADH secretion were disturbed in these two cases. ADH producing cells, the thirst center and the osmoreceptor are all located in the hypothalamus. Radiographic measures showed dysplasia of the hypothalamus, providing the anatomical basis for their dysfunction.

Anencephaly↗

A longitudinal study of obesity in Japan: relationship of body habitus between at birth and at age 17.

To clarify the relationship between body habitus from birth to adolescence, changes in weight and height of 309 boys and 335 girls were observed at birth, 3, 6, 11, 14 and 17 years old. Body mass index (BMI = W/H2) was calculated to estimate their habitus at birth, 3 and 17 years, and Rohrer index (W/H3) was used for 6, 11 and 14 years habitus. We define the children in the top decile as obese and those in the bottom decile as lean. Among girls obesity at 17 years old is already related to habitus at birth, while among boys it is related to habitus at 3 years old. Based on these results, we tentatively recommend that prevention of obesity should start as early as possible, at the least from 3 years old.

Adolescent↗

Tricho-rhino-phalangeal syndrome type I with severe mental retardation due to interstitial deletion of 8q23.3-24.13.

Here we report on a 13-year-old boy who had an interstitial deletion of the long arm of chromosome 8 [46,XY,del(8)(pter----q23.3::q24.13----qter)]. He had the facial features of the tricho-rhino-phalangeal (TRP) syndrome and severe mental retardation, but lacked multiple exostoses. This is the first report with such a peculiar combination of abnormalities and interstitial deletion of 8q.

Chromosome Banding↗

Prometaphase chromosomes in the tricho-rhino-phalangeal syndrome type I.

Prometaphase chromosome analysis was undertaken in a patient with familial tricho-rhino-phalangeal syndrome type I (TRPS-I). The patient had apparently normal chromosomes and produced counterevidence to part of Bühler's hypothesis. Thus, although some cases of typical TRPS-I may be derived from a deletion of 8q24.12, others might be caused by gene mutation or submicroscopic deletion involving the corresponding locus within the band 8q24.11----24.13.

Abnormalities, Multiple↗

Combined effects of vibration and noise on palmar sweating in healthy subjects.

The combined effects of vibration and noise on palmar sweating were studied experimentally in healthy subjects. Ten subjects were exposed to vibration at an acceleration of 100 m.sec-2 root mean square at a frequency of 125 Hz, a noise level of 105 dB(A) at a frequency of 1000 Hz and both stimuli together. Vibration was applied to the right hand and noise to both ears from headphones. Palmar sweating was measured by the ventilated capsule method. Vibration caused a marked increase in palmar sweating of the right hand directly exposed to vibration and also of the left hand not exposed to vibration. Simultaneous vibration and noise caused a greater increase in palmar sweating than by each of the factors separately. The combined effects tended to be greater in subjects who were more susceptible to vibration or noise.

Adult↗

Effects of VDT resolution on visual fatigue and readability: an eye movement approach.

The effects of VDT resolution on visual fatigue and readability were studied. Two kinds of displays with different resolutions (1664 x 1200 pixels and 720 x 350 pixels) and fonts were used. In the first experiment, the subjects read from each display for 1 h to induce fatigue. Reading speed and blink rate while reading were measured. Eye movements during visual smooth pursuit tracking tasks were studied before and after reading; quantitative scoring of eye movement performance showed no significant changes. In the second experiment, readability tests with three different character sizes on both displays were conducted and resulting reading eye movements were analysed. For readability of sufficiently large characters, no significant difference between the high and the standard VDT could be detected. However, for very small characters, higher resolution improved readability.

Adult↗

[Analysis of subjective symptoms of coldness and numbness in the upper and lower limbs among patients with vibration syndrome].

Coldness and numbness of the lower limbs in patients with vibration syndrome were studied in correlation with the frequency of attacks of vibration-induced white finger (VWF), coldness and numbness of the upper limbs, and awakening at night due to numbness of the arm. Subjects were 229 patients aged 50-69 years without disease other than vibration syndrome who had mainly operated chain saws. The present study showed that patients with more frequent VWF attacks had a higher prevalence and frequency of coldness in the fingers, awakening at night due to numbness of the arm, and coldness and numbness in the legs. Particularly patients having almost daily attacks of VWF had a high prevalence of these symptoms. Positive correlation was observed in prevalence and frequency between coldness of the fingers and that of the legs, and between coldness and numbness in the legs. The prevalence of VWF, numbness and coldness of the fingers, and coldness of the legs was higher the longer the total chain saw operating period. The present findings suggest that patients with severe circulatory disturbances in the upper limbs have severe circulatory disturbance in the lower limbs. Further studies on circulatory disturbances in the lower limbs are indicated, and their mechanism involved should be also pursued from the pathogenetic point of view.

Aged↗

[Clinical experience with clarithromycin in the pediatric field].

Clarithromycin (TE-031, A-56268) was orally administered to 20 children with the following acute bacterial infections; 1 case of acute pharyngitis, 3 cases of acute tonsillitis, 11 cases of acute bronchitis, 1 case of exacerbation of chronic bronchitis, 1 case of acute bronchopneumonia, 2 cases of Mycoplasma pneumonia and 1 case of impetigo. Clinical effectiveness was obtained in 15 out of the 20 cases (75.0%). No clinical side effects and laboratory abnormalities were observed. The above results suggest that TE-031 is a useful antibiotics for treating pediatric patients with various bacterial infections.

Administration, Oral↗