PubMed Health⌕ Search

Biomedical subjects

I Matsuda

Publications and source records attributed to I Matsuda.

At least 19 recordsLinked to original sources
PubMed1979

Urinary glycopeptides of fucosidosis.

Fucosidosis is unique among congenital exoglycosidase deficiencies, because not only oligosaccharides but large amount of glycopeptides are excreted in the urine. The structures of 22 major glycopeptides isolated from urine of a fucosidosis patient were determined. One of the prominent features common to all these glycopeptides was that they all contain 1 fucosyl residue at either C-3 or C-6 position of the N-acetylglucosamine which is linked to asparagine. Possibly, accumulation of glycopeptides in fucosidosis urine is caused by the inability of human endo-beta-N-acetylglucosaminidase to cleave the asparagine-linked sugar chains, which have a fucose at the innermost N-acetylglucosamine residue.

Carbohydrates↗
PubMed1979

Lactose intolerance associated with cataracts.

A 50-day-old boy with severe lactose intolerance is described. In addition to vomiting, failure to thrive, dehydration, metabolic acidosis and amino aciduria, bilateral cataracts were also found. At three months of age, a computerized axial tomography scan and an electroencephalogram were abnormal, and myoclonic spasms began at the age of seven months.

Cataract↗
PubMed1979

Metabolic acidosis in patients receiving anticonvulsants.

Blood pH, bicarbonate, PCO2, serum calcium, alkaline phosphatase and red cell carbonic anhydrase were measured in 37 selected patients receiving anticonvulsants. Patients with metabolic acidosis showed a high incidence of hypocalcemia with increased alkaline phosphatase and a significant reduction of carbonic anhydrase-B activity. High iPTH levels were found in 13 patients, but this was not correlated with acid-base balance status. Anticonvulsant drugs seemed to inactive carbonic anhydrase-B activity. Metabolic acidosis might be one of the factors causing a disturbance of calcium metabolism in these patients.

Acidosis↗
PubMed1979

Iatrogenic and transient hyperglycinemia in patients with phenylketonuria.

Two patients with phenylketonuria detected by newborn screening for inborn errors of metabolism were treated with low phenylanine formulae. Serum phenylalanine levels were well controlled, but serum glycine levels were elevated until 4 or 5 months of age. This was probable due to the high content of glycine in the formulae. Glycine level returned to be normal in these patients, even though they were kept on the same formula, suggesting, immature metabolism of glycine during this period. No clinical problems were encountered in either patient.

Glycine↗
PubMed1979

Pseudohypoparathyroidism type II and anticonvulsant rickets.

A patient treated with anticonvulsants showed hypocalcemia and hyperphosphatemia in association with increased serum parathyroid hormone, reduced serum 25-hydroxy D3, diminished response in phosphorus excretion to exogenous parathyroid hormone and normal response in cyclic AMP excretion. Oral administration of Vitamin D3 resulted in normalization of serum 25-hydroxy D3, calcium, and phosphorus. At this stage, phosphorus excretion after parathyroid hormone returned to normal. These findings suggest that the patient had pseudohypoparathyroidism type II with anti-convulsant medication as a complicating factor.

Adolescent↗
PubMed1979

Lysine intolerance in a variant form of citrullinemia.

An oral loading of lysine (100 mg of lysine-HCL/kg was performed in two patients, 18-and 23-yr-old, with a variant form of citrullinemia. Serum citrulline levels were approximately 10 times higher than control level and lysine levels were within the normal range, in contrast to the classical form of the disease in which serum citrulline is approximately 100 times normal levels and hyperlysinemia is usually present. After lysine loading, lysine levels rose sharply and clearance was decreased. Blood ammonia rose approximately 2.5 times. Lysine, citrulline, and arginine were markedly elevated in urine, collected 90--210 min after the lysine loading. Baseline homocitrulline and homoarginine excretion was elevated and increased further after the load.

Adolescent↗
PubMed1979

Embolization and superselective angiography by means of balloon catheters.

Three types of thin flexible balloon catheters were made. One of them is a releasable balloon catheter, which is prepared for embolization of intracranial aneurysms, arteriovenous malformations and fistulas. The other two are nonreleasable balloon catheters that are utilized for temporary occlusion, catheterization, and superselective angiography of the intracranial arteries. These catheters are useful experimentally for the above mentioned purposes. Our clinical experiences with catheterization and superselective angiography with our balloon catheter are reported.

Animals↗
PubMed1979

Intracavernous giant aneurysm associated with Marfan's syndrome.

A case of intracavernous giant aneurysm associated with Marfan's syndrome is reported. The patient was treated with a superficial temporal artery-middle cerebral artery bypass and a ligation of the internal carotid artery in the neck. Although they are common in aorta and pulmonary artery, the vascular abnormalities in Marfan's syndrome are very rarely reported in intracranial arteries. The patient also had an aberrant internal carotid artery which presented as a red mass behind the eardrum.

Adult↗
PubMed1979

Balloon embolization of a giant aneurysm using a newly developed catheter.

A new catheter with a releasable balloon has been developed for the controllable embolization of intracranial vascular lesions located beyond many arterial curves such as the carotid siphon. Embolization is accomplished by introduction of the balloon to the lesion, followed by inflating the balloon with a polymerizable liquid. After this liquid sets to a gel, the catheter is twisted off to detach the balloon at a specially designed joint. This balloon catheter has been used clinically to embolize an aneurysm of the middle cerebral artery.

Aged↗
PubMed1978

Delayed cutaneous hypersensitivity in children with severe multiple handicaps treated with phenytoin.

Peripheral lymphocyte counts, T-cell and B-cell populations, DNCB skin reactions, Mantoux reactions, mitogenic transformation using PHA, and determination of serum phenytoin concentration were carried out in 45 children with severe handicaps. Twenty two of these patients were receiving phenytoin treatment. When compared with control subjects, the skin reactions were significantly depressed in the handicapped children, amongst whom those treated with phenytoin had more impaired reactions than those who were untreated. No significant relationship between the results of the skin reactions and the other parameters was found.

Adolescent↗
PubMed1978

Glucose-6-phosphatase activity in liver and blood platelets of two patients with glycogen storage disease type I.

Glucose-6-phosphatase (G-6-Pase) activity in liver and blood platelets of two patients with glycogen storage disease (GSD) type I is described. Both patients had a reduced activity of G-6-Pase in liver. The km value for glucose 6-phosphate (G-6-P) of residual activity in liver of both patients was similar to that of control liver. We could not demonstrate any reduced activity of platelet G-6-Pase in the patients. Platelet G-6-Pase with our assay method seems to represent a nonspecific phosphatase activity. Our observation suggests that it is necessary to examine platelet G-6-Pase of many other patients with GSD type I to confirm that G-6-Pase deficiency can be diagnosed by enzyme assay performed on blood platelets.

Alanine↗