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Biomedical subjects

N Hanyu

Publications and source records attributed to N Hanyu.

At least 73 records · Page 4Linked to original sources
PubMed1990

[Clinical and pathological studies on two patients with adult-onset nemaline myopathy].

Clinical and pathological findings of two patients, a 44-year-old male and a 54-year-old female, with adult-onset nemaline myopathy were described. Both patients showed normal motor development through their childhood; Patient 1 ran fast and was involved in powerful heavy labor until the age of 40, and Patient 2 was in good health until 49, when they began to have progressive muscle weakness. They had no family history of neuromuscular diseases. On neurological examination, they had moderate muscle weakness and atrophy in their limb-girdle and paravertebral muscles. Because paravertebral and neck muscles were preferentially involved, they had difficulty in holding the head straight up. They stood in a lordotic posture. They had neither high-arched palate nor facio-skeletal abnormalities which were common findings in congenital nemaline myopathy. Serum enzymes derived from muscle were normal and needle electromyography showed myogenic and neurogenic changes in both patients. On CT scan of the skeletal muscles, the paravertebral muscles were markedly decreased in density suggesting advanced fat tissue replacement in large areas. In patient 2 who was in more advanced stage, the quadriceps femoris, hamstrings and soleus muscles also showed the similar CT findings. Light microscopic examination of biopsied biceps brachii (Patient 1) and quadriceps femoris (Patient 2) demonstrated abundant rod-like structures in the majority of type 1 fibers. In both patients, there was a marked variation in fiber size. Type 1 fibers were atrophic and type 2C fibers increased in number.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Peripheral nerve pathological findings in familial amyloid polyneuropathy: a correlative study of proximal sciatic nerve and sural nerve lesions.

To analyze the peripheral nerve pathological abnormalities in familial amyloid polyneuropathy, a correlative pathological study was carried out on the spinal nerve roots, proximal sciatic nerves, sural nerves, and brachial plexuses from 3 patients with the disease in Japan. The spinal nerve roots appeared to be unaffected except for amyloid deposition on the epineurium. In sciatic nerves and brachial plexuses the nerve lesions had a multifocal distribution, showing prominent interstitial edema in the endoneurium frequently adjacent to deposits of amyloid; in these regions the nerve fibers were severely depleted. A teased-fiber study revealed that segmental demyelination was the predominant type of nerve fiber abnormality. However, these findings were not seen in the sural nerves; instead a diffuse fiber loss with axonal degeneration was observed. It is suggested that multifocal lesions in the proximal portions of the long extremity nerves could summate distally to produce a symmetrical polyneuropathy in the disease. In addition to a space-occupying effect of amyloid deposits in the endoneurium, severe endoneurial edema associated with amyloid deposition in blood vessels and the endoneurial interstitium may induce ischemia in nerve fibers, thus causing the progressive polyneuropathy in this disorder.

Adult↗
PubMed1988

[The effect of vagotomy and gastrectomy on the lower esophageal sphincter motility].

The motor activity of the canine lower esophageal sphincter (LES) was studied under conscious states by means of the strain gage transducer, and gastroesophageal reflux was investigated after injection of the contrast medium through the silastic tube inserted in gastric fundus by x-ray. The motor activity and function of LES were observed in the dogs with selective proximal vagotomy (SPV), truncal vagotomy (TV) and TV + gastrectomy. Results were summarized as follows; 1. In the postprandial period, so called receptive relaxation was observed in gastric body, and tonic contraction was observed in LES. On the other hand, the occurrence of the LES contraction was consistent with gastric periodic motor activity in the interdigestive state. It was found that such a contractile pattern of LES and gastric body prevented the reflux of gastric juice from stomach to esophagus in the digestive and interdigestive state. 2. In the postprandial period, the tonic contraction of LES was inhibited by the venous injection of atropine sulfate. Mechanisms of digestive contractile activity of LES were mainly regulated by cholinergic nerve. 3. After SPV and TV, the motility of LES and gastric body was disturbed. Namely, receptive relaxation disappeared, and tonic contraction was observed in gastric body, but the relaxation of the LES was found after ingestion. These findings suggested that discordant LES contractiles with the contraction of the gastric body after vagotomy produced a gastroesophageal reflux. 4. After TV + gastrectomy, the synchronous motor activity of the LES, remnant stomach and duodenum disappeared. Namely, it was thought that the reflux esophagitis occurred after gastric surgery with vagotomy and lymphnodes dissection was caused by the duodenal juice reflux to remnant stomach and esophagus.

Animals↗
PubMed1987

Hereditary generalized amyloidosis with polyneuropathy. Clinicopathological study of 65 Japanese patients.

A clinicopathological study was made on 65 patients from a small area of Nagano Prefecture, Japan, with hereditary generalized amyloidosis with polyneuropathy to clarify the clinical variety of the disease. Forty-five patients from Ogawa village showed similar clinical features. The age of onset ranged widely from 16 to 62 years. The main neurological manifestations were polyneuropathy starting in the legs and autonomic dysfunction. Lower cranial nerves were also affected in the advanced stages. Severe cardiac and renal involvement was uncommon. All these clinical features are consistent with type I familial amyloid polyneuropathy (FAP). The remaining 20 patients from five unrelated kinships showed unique clinical pictures. Two families from Ogawa village had type I FAP, but 4 out of the 5 affected patients showed marked nephropathy with heavy proteinuria from an early stage. Of the three other families, one, with 10 patients, was notable for the involvement of the central nervous system. Most of the patients showed cerebellar ataxia and pyramidal tract signs in addition to a sensorimotor and autonomic peripheral neuropathy. Another family had 2 siblings who had severe amyloid heart disease from the onset and developed polyneuropathy with autonomic features at an advanced stage. In the third family, onset occurred in the sixth decade in all 3 patients and the course was mild in 2, although the clinical features were those of typical type I FAP. Immunohistochemical study revealed that the amyloid fibril proteins in the patients with all four unusual clinical phenotypes were related to plasma prealbumin. The most common form of hereditary generalized amyloidosis in Japan is type I FAP, but the disease shows considerable variety in the age of onset and involves more systemic organs than previously recognized. The newly recognized clinical forms of hereditary generalized amyloidosis with severe amyloid heart disease or central nervous dysfunction indicate clinical heterogeneity of hereditary amyloidosis with polyneuropathy.

Adolescent↗
PubMed1986

Dramatic events in ciliate evolution: alteration of UAA and UAG termination codons to glutamine codons due to anticodon mutations in two Tetrahymena tRNAs.

The three major glutamine tRNAs of Tetrahymena thermophila were isolated and their nucleotide sequences determined by post-labeling techniques. Two of these tRNAs show unusual codon recognition: a previously isolated tRNA(UmUA) and a second species with CUA in the anticodon (tRNA(CUA)). These two tRNAs recognize two of the three termination codons on natural mRNAs in a reticulocyte system. tRNA(UmUA) reads the UAA codon of alpha-globin mRNA and the UAG codon of tobacco mosaic virus (TMV) RNA, whereas tRNA(CUA) recognizes only UAG. This indicates that Tetrahymena uses UAA and UAG as glutamine codons and that UGA may be the only functional termination codon. A notable feature of these two tRNAs is their unusually strong readthrough efficiency, e.g. purified tRNA(CUA) achieves complete readthrough over the UAG stop codon of TMV RNA. The third major tRNA of Tetrahymena has a UmUG anticodon and presumably reads the two normal glutamine codons CAA and CAG. The sequence homology between tRNA(UmUG) and tRNA(UmUA) is 81%, whereas that between tRNA(CUA) and tRNA(UmUA) is 95%, indicating that the two unusual tRNAs evolved from the normal tRNA early in ciliate evolution. Possible events leading to an altered genetic code in ciliates are discussed.

Journal Article↗
PubMed1986

Influence of age on utilization of supplemental fats by young turkeys.

Male poults were fed diets containing 0, 4, 8, or 12% tallow or 4, 8, or 12% animal-vegetable fat blend (A-V Fat) from 1 day to 8 weeks of age. Supplemental fat improved 8-week body weight and feed efficiency, irrespective of fat source. Fat retention (fat consumed-fat excreted) was not affected by fat level or by fat source after 2 weeks of age. At 2 weeks, fat retention by poults fed tallow was less than that of poults fed A-V Fat. Fat retention increased with age, irrespective of fat source or level. Fat retentions ranged from 66.4 to 83.7 at 2 weeks and from 90.8 to 96.5 at 8 weeks of age. Changes in metabolizable energy (ME) values of the fats with age paralleled those of fat retention, whether ME were estimated by multiplying fat retention percentages times gross energy of the fats or by regression analysis of changes in diet ME associated with increments of fats. On the basis of changes in diet ME, the ME of tallow was 6808 kcal/kg at 2 weeks and 8551 kcal/kg at 8 weeks. Analagous values for A-V Fat were 7114 and 8924 kcal/kg, respectively. Fatty acid analysis of dietary and excreta fat indicated that, as turkeys aged, improvements in retention were greatest for palmitic and stearic acids, especially in the instance of tallow. Fatty acid binding protein (FABP) assay of intestines from poults fed no added fat, 12% tallow, or 12% A-V Fat showed that fat level did not significantly affect FABP activity. There was, however, a marked increase in intestinal FABP in 6- and 8-week-old poults as compared with 2- or 4-week-old poults.

Age Factors↗
PubMed1985

Type III (chronic) GM1-gangliosidosis. Histochemical and ultrastructural studies of rectal biopsy.

Type III GM1-gangliosidosis is a rare hereditary storage disease caused by lack of lysosomal beta-galactosidase and characterized by a slowly progressive course, and extrapyramidal signs, but without prominent skeletal changes or visceromegaly. The storage substance was reported to be located only in the basal ganglia. There has been no detailed report on visceral lesions in type III GM1-gangliosidosis. In this report we describe a case of type III GM1-gangliosidosis, and the histochemical and ultrastructural findings from biopsied rectum. The patient was a 22-year-old female who exhibited dysarthria, gait disturbance, and generalized dystonia with rigidity. Beta-galactosidase activity in leukocytes was absent and sialidase activity in cultured fibroblasts was normal. Many histiocytes were found in biopsied rectal mucosa. Histochemical studies showed that the granules of histiocytes contained acidic glycoconjugates, beta-galactose, beta-N-acetylgalactosamine and sialic acid. Ultrastructural investigations revealed that ganglion cells of Meissner's plexus had many osmiophilic lamellar inclusions, similar to "membranous cytoplasmic bodies". These findings are crucial for the clinical diagnosis of type III GM1-gangliosidosis.

Adult↗
PubMed1985

Tetrahymena thermophila glutamine tRNA and its gene that corresponds to UAA termination codon.

Nucleotide sequence analysis of one of several tRNA genes cloned from Tetrahymena thermophila macronuclear DNA indicated that it corresponds to a tRNA species having TTA as anticodon. Subsequently, the tRNA species corresponding to that gene was isolated and its nucleotide sequence was determined by post-labeling techniques. The nucleotide sequence was found to be pG-G-U-U-C-C-A-U-A-m2G-U-A-psi-A-G-D-G-G-D- D-A-G-U-A-C-U-G-G-G-G-A-Cm-U-Um-U-A-i6A-A-psi-C-C-C-U-U-G-A-C- m5C-U-G-G-G-U-psi-C-G-m1A-A-U-C-C-C-A-G-U-G-G-G-A-C-C-U-C-C-AOH. This tRNA sequence exactly matched the DNA sequence of the corresponding tRNA gene. The first position of anticodon is 2'-O-methyluridine (Um), forming UmUA as the anticodon, which presumably recognizes the ochre termination codon UAA. This tRNA species is aminoacylated with glutamine by a Tetrahymena crude aminoacyl tRNA synthetase fraction, suggesting that ochre termination codon is used as a glutamine codon during cytoplasmic protein synthesis in Tetrahymena.

Animals↗
PubMed1985

[G-I hormones and gastric functions--with special reference to the pyloroplasty for the selective proximal vagotomy].

UNLABELLED: Serum gastrin changes before and after selective proximal vagotomy (SPV) were studied in relation to the gastric motility and the acid secretion clinically and experimentally and the effects of pyloroplasty were investigated. RESULTS AND CONCLUSIONS: Experimental studies revealed that test meals provoked gastrin secretion to stimulate gastric motility. Periodical gastric acid secretions were observed during hunger period, along with elevated serum gastrin levels. After SPV, gastrin responses to the test meals were significantly increased so that the pyloric ring motility was disturbed. Clinical studies revealed that adrenaline infusion test provoked significant gastrin responses to secrete acid secretions in duodenal ulcer patients. After SPV, increased gastrin responses to adrenaline restored acid secretions as much as preoperative acid secretions responded to the adrenaline test. Pyloroplasty for the SPV inhibited the gastrin and acid secretions responded to the adrenaline test.

Animals↗
PubMed1983

[Effects of truncal vagotomy on serum motilin levels, gastric motility patterns and gastric emptying: an experimental study].

Using adult mongrel dogs, gastroduodenal motility, gastric emptying and pertinent serum motilin levels were measured under conscious states before and after transthoracic truncal vagotomy. Gastroduodenal motility was recorded by chronically planted strain gage transducers along the gastrointestinal tract. Gastric emptying was measured by X-ray examinations after barium-meat-meal ingestions or by gastric contents sampling method through the total pouch cannula after normal dog food ingestions. Results were as follows; Gastric motility patterns recorded by strain gage transducers were classified into two patterns that were a digestive pattern (D.P.) and an interdigestive pattern (I.P.). Serum motilin levels were elevated during high-amplitude contractions and lowered during resting phase of interdigestive periods. After vagotomy, strengths (heights) of antral contractions and phasic changes were decreased with the lack of I.P. These postvagotomy changes were thought to be related with the delayed gastric emptying after vagotomy. Gastric lavage through the cannula or pyloroplasty produced no effect on recovery of I.P. This fact suggested that the lack of I.P. was the direct effect of vagotomy and not due to delayed gastric contents emptying. On the assumption that motilin had the major role on the regulation of I.P., vagotomy seemed to produce an effect to prevent phasic motilin release.

Animals↗
PubMed1982

Degeneration and regeneration of ventral root motor fibers in amyotrophic lateral sclerosis. Morphometric studies of cervical ventral roots.

Morphometric studies by histological and teased-fiber methods on myelinated fibers of the C6 and C8 ventral roots were made in cases of myotrophic lateral sclerosis (ALS) and in age-matched controls. In 11 controls myelinated fibers were divided into two groups: large and small fibers. The fiber density was calculated and the actual number of fibers was counted in both groups. The number of large fibers was significantly decreased in 14 of 17 cases with ALS, and correlated with the muscle strength of the ipsilateral upper limb as determined by manual muscle testing before death. The absolute number of small fibers of the C6 ventral root was increased in 3 of 5 cases of ALS. In these cases, there was a group of small myelinated fibers showing very thin myelin lamellae compared to axon circumference, and these were considered as regenerating myelinated fibers. On teased-fiber study, the frequency of fibers with axonal degeneration was 23.8% in ALS in contrast to 0.6% in controls (P less than 0.001). In addition, 5.5% of fibers showed segmental demyelination, and remyelination after segmental demyelination was found in 16.4% of fibers in ALS. These figures were also significantly larger (P less than 0.01) than controls (2.4% and 10.1%, respectively). These results suggest that the essential pathological change in the ventral spinal root in ALS is axonal degeneration of large myelinated fibers, and that Schwann cell involvement may also occur.

Adult↗