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Biomedical subjects

K Tada

Publications and source records attributed to K Tada.

At least 433 records · Page 24Linked to original sources
PubMed1984

Birth palsy: natural recovery course and combined root avulsion.

Seventy-six patients with birth palsy were reviewed for type of palsy and mode of delivery. High incidences of root avulsion associated with lesser birth weight were identified in patients of breech deliveries. In 15 patients with serial neurological assessment for more than 5 years, the natural recovery course was evaluated, based on the assessment of particular key muscle and dermatomes mainly innervated by single nerve roots. Rapid and useful motor recovery was observed in upper-type palsy and the upper root levels (C5 and C6) of whole-type palsy, while poor prognosis of motor function was demonstrated in the lower root levels (C8 and D1) of whole-type palsy. Recovery of sensory function was far more predominant than recovery of motor function. Of 12 patients with root avulsion, 10 patients (27 avulsed roots) over 5 years of age were neurologically assessed. Of avulsed roots, 70.4% had useful sensory recovery and 33.3% had useful motor recovery. The patho-mechanism of those with a good prognosis in root avulsion of birth palsy is discussed, based on the findings of this series.

Birth Weight↗
PubMed1983

Biliary lipid compositions in cholestatic diseases of infancy.

Biliary lipid compositions in infants with cholestasis were analysed. Bile acid and phospholipid values were appreciably lower in infants with idiopathic neonatal hepatitis syndrome or corrected biliary atresia than in control infants. Cholesterol values were not, however, notably lower in infants with cholestasis. When biliary lipid components were determined in terms of molar percent, bile acid values were considerably lower in infants with cholestasis than in controls, and cholesterol and phospholipids were appreciably higher, as was the lithogenic index.

Age Factors↗
PubMed1983

Ring chromosome 10 and its clinical features.

A 2-year-old boy with mental and growth retardation is presented; he has a 46,XY,r(10)(p15q26) chromosome complement. Five previously reported cases of ring chromosome 10 were reviewed and compared with the present case in an attempt to delineate a clinical syndrome. Since the first description, identified by Giemsa banding by Lansky et al, four other r(10) patients have been described. Their common features were mental and growth retardation, low birth weight, microcephaly, stubby nose, hypertelorism, strabismus, wide set nipples, single transverse palmar creases, undescended testes, and hypoplastic scrotum. In some of the cases congenital heart disease was present.

Child, Preschool↗
PubMed1983

Glycogen storage disease type 1b: microsomal glucose-6-phosphatase system in two patients with different clinical findings.

The basic defect in glycogen storage disease (GSD) type 1b was investigated in two patients: one, (Y.S.), a severely affected infant and the other, (Y.M.), an adult with mild clinical symptoms. The enzymatic studies on liver needle biopsy specimens from the two patients indicated that glucose-6-phosphate (G-6-P) phosphohydrolase activity of the "intact microsomes" was partially deficient (20% of that in controls) in Y.M. and undetectable in Y.S. Activities of G-6-P phosphohydrolase in the disrupted microsomes of Y.S. and Y.M. are higher than those in the disrupted microsomes of controls (12.60 mumole/min/g liver in Y.S., 9.18 in Y.M. and 6.26 +/- 1.22, mean +/- S.D. in controls). Our study also shows that PPi phosphohydrolase activities of the "intact microsomes" from both patients (6.07 mumol/min/g liver in Y.S. and 5.36 in Y.M.) were greater than those of the controls (3.23 +/- 0.77 mumole/min/g wet weight liver). These results indicate that the G-6-P translocase was the locus of the defect in both patients with GSD type 1b. Clinical symptoms and enzymatic studies suggest that the clinical severity of this disorder depends on the level of residual activities of G-6-P translocase. Kinetic studies showed an abnormally high Km of the residual G-6-P translocase in Y.M., suggesting a structural gene mutation. The systematic assay method for glucose-6-phosphatase system, which requires only 15 mg of liver tissues, is also described.

Adult↗
PubMed1983

Nonketotic hyperglycinemia: two patients with primary defects of P-protein and T-protein, respectively, in the glycine cleavage system.

The glycine cleavage system was investigated in the livers and brains of two patients with typical nonketotic hyperglycinemia who died in the neonatal period. The overall activity of the glycine cleavage system was found to be extremely low in both the liver and brain of each patient. In one patient, the disturbance of the glycine cleavage system was due to absence of activity of the P-protein. Immunochemical analysis indicated that this resulted from an absence of the enzyme protein. In the other patient, the activity of the T-protein was undetectable in the brain and was extremely low in the liver. Clinically classic nonketotic hyperglycinemia resulted from molecular defects in two different protein components of the glycine cleavage system.

Amino Acid Metabolism, Inborn Errors↗
PubMed1983

Ultrastructural alterations of Paneth cells in infants associated with gastrointestinal symptoms.

Paneth cells containing abundant and large inclusion bodies found in patients with acrodermatitis enteropathica have been considered as a consistent and characteristic abnormality of this disease. We examined 34 children with gastrointestinal symptoms due to a wide variety of basic diseases and found similar abnormal Paneth cells in most of them, particularly in infants under the age of one year. This abnormality, according to our findings, is attributed to the secondary effect of zinc deficiency due to malnutrition.

Acrodermatitis↗
PubMed1983

Dietary therapy in a girl with propionic acidemia: supplement with leucine resulted in catch up growth.

A 15-month-old girl with propionic acidemia presented a failure to thrive and muscular hypotonia, who had been treated with diets low in leucine, valine, isoleucine, threonine and methionine. Supplementation with leucine gave her a catch up growth and brought about an improvement in muscular hypotonia in parallel with restoration of plasma leucine which had been below normal range during the treatment with the above diets. Oral loading test of leucine produced no ketoacidosis. These findings indicate that the restriction of leucine is not necessary in the dietary management of propionic acidemia.

Amino Acid Metabolism, Inborn Errors↗
PubMed1983

Microautoradiographic study on the tissue localization of liposome-entrapped or unentrapped 3H-labeled beta-galactosidase injected into rats.

The localization of intravenously injected liposome-entrapped or unentrapped 3H- beta-ga lactosidase in various tissues of rats was investigated by microautoradiography. The microautoradiographic silver grains, indicating the uptake of both forms of the enzyme were observed in all of the rat tissues studied, such as the liver, kidneys, spleen, lungs, heart, muscle and brain. The liver was found to be the most active in taking up both forms of the enzyme and probably Kupffer cells were primarily involved in the uptake of the enzyme. Also in the brain, the uptake of both forms of the enzyme was observed, though far less extensively, through the blood-brain barrier. The uptake of the enzyme in the brain was somewhat greater in the gray matter than in the white matter.

Animals↗
PubMed1983

Epstein-Barr virus-induced precursor B cell lines from patients with congenital agammaglobulinemia.

We have established lymphoid cell lines with diversity of immunoglobulin expression by infection of bone marrow cells of four patients with congenital agammaglobulinemia with Epstein-Barr virus. Immunofluorescent study revealed that the cells of many of these lymphoid cell lines had characteristics of possible precursor B cells, permitting classification as follows: 1) cell lines without any surface or cytoplasmic immunoglobulins (17/28 lines), 2) cell lines which had only cytoplasmic mu heavy chains (line K4 and O2), 3) cell lines with both mu and delta heavy chains in the cytoplasm (line O6 and M5), 4) cell lines which bore surface mu chains and possessed cytoplasmic mu chains but lacked light chain expression (line S5), 5) cell lines which had surface and cytoplasmic delta and lambda chains, and secreted the immunoglobulins (line K5), and 6) cell lines which had surface IgM and cytoplasmic mu and light chains, and secreted the immunoglobulins (5/28 lines). Biosynthetic studies with 14C-leucine confirmed the patterns of immunoglobulin expression found by immunofluorescent analysis. These cell lines may represent some stages of B cell development and provide useful information on the pathophysiology of congenital agammaglobulinemia.

Agammaglobulinemia↗
PubMed1983

Ecto-5'-nucleotidase activities in lymphocytes and B lymphoblastoid cell lines from patients with congenital agammaglobulinemia.

Ecto-5'-nucleotidase (ecto-5'-NT) activity was measured in Epstein-Barr virus (EBV)-induced B lymphoblastoid cell lines (LCL) derived from bone marrow cells and peripheral lymphocytes of four patients with congenital agammaglobulinemia (CAG). In control subjects there was no appreciable difference in the range of ecto-5'-NT activity between immunoglobulin (Ig)-producing B LCL and their peripheral lymphocytes. Though peripheral lymphocytes of the patients showed lower ecto-5'-NT activities than those of control subjects, ecto-5'-NT activities in Ig-producing B LCL from peripheral lymphocytes of the patients fell into the same range as those in B LCL from control subjects. Ecto-5'-NT activities in non-Ig producing precursor B cell lines derived from bone marrow cells of the patients also registered within the range of the enzyme activity of Ig-producing B LCL from control subjects. B LCL from mothers of the patients, who were presumed to be heterozygotes, also had the same range of ecto-5'-NT activity as the control subjects. These results indicate that ecto-5'-NT does not appear to be involved in the pathogenesis of congenital agammaglobulinemia.

Agammaglobulinemia↗
PubMed1983

Endothelial alterations of skeletal muscle capillaries in childhood myopathies.

Intramuscular blood capillaries in thirteen biopsied specimens obtained from eleven patients with various childhood myopathies were investigated ultrastructurally. The patients were classified into 4 different groups according to the percent distribution of various stages of endothelial alteration; normal endothelial type (E0 Type), slightly abnormal type (E1 Type), moderately abnormal type (E2 Type) and markedly abnormal type (E3 Type). Endothelial types in Duchenne dystrophy progressed from E0 to E1 and further to E2 Type, which kept pace with muscle degeneration. The endothelial types in Werdnig-Hoffmann disease and central core disease were E0 and those in multicore disease and Fukuyama type congenital muscular dystrophy were E1. Two cases of mitochondrial myopathy showed E3 Type, which was characterized by markedly swollen endothelial cells and narrowed lumens. The endothelium in these two cases showed more marked changes than in other childhood myopathies.

Adolescent↗