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Biomedical subjects

K Tada

Publications and source records attributed to K Tada.

At least 415 records · Page 23Linked to original sources
PubMed1984

Ulnar ray deficiency: its various manifestations.

Eighty-eight upper extremities of 65 patients with ulnar ray deficiency were reviewed with regard to clinical manifestations. Based on the findings, a subclassification into four types was established: type I, hypoplasia or partial defect of the ulna; type II, total defect of the ulna; type III, total or partial defect of the ulna with humeroradial synostosis; and type IV, ulnar defect with congenital amputation at the wrist. Various manifestations of deficiency were evident not only within the ulnar ray but also in other rays. Hypoplasia of the shoulder and/or proximal part of the humerus was present in some cases of types III and IV. Elbow involvement varied from functioning (type I) to acute flexion contracture (type II) to fusion (type III). In 57 hands the digits and carpal bones in the radial ray showed hypoplasia and/or defect. Central digits and carpal bones were also influenced by ulnar ray deficiency, presenting carpal bone fusion, syndactyly, and delta phalanx.

Abnormalities, Multiple↗
PubMed1984

Cytochrome C oxidase deficiency in two siblings with Leigh encephalomyelopathy.

Two siblings with cytochrome c oxidase deficiency are described. One of them died of subacute necrotizing encephalomyelopathy which was proven by autopsy. The other was also suspected of having Leigh encephalomyelopathy by the findings on brain CT scans. The former, a younger brother, was in good health until the age of 10 months when progressive dysphagia, muscular hypotonia and abnormal eye movements became apparent. Six months later he suddenly died due to respiratory insufficiency. The latter, an elder brother, started to show nystagmus, abnormal eye movements and ataxia at the age of 5 years. A deficiency of cytochrome c oxidase in the younger brother was demonstrated in autopsied liver and brain, while such a deficiency in the elder brother was shown in biopsied peripheral muscle tissue and in cultured skin fibroblasts. Both patients showed a marked heat lability of cytochrome c oxidase. These results suggest that the biochemical defect observed in the siblings is due to a genetic defect. This seems to be the first case of a generalized defect in cytochrome c oxidase.

Brain↗
PubMed1984

Serum vitamin E levels in children with corrected biliary atresia.

In 37 children with long-standing cholestasis who had undergone a Kasai's procedure (double Roux-en-Y hepatic portoenterostomy), serum vitamin E levels were determined. In addition, serum bile acid levels were simultaneously tested as a marker of cholestasis. Eighteen of 37 children had vitamin E levels of less than 0.50 mg/100 ml, and two showed neurological abnormalities including hypoactive deep tendon reflexes and ataxia. Serum vitamin E levels were inversely correlated with serum bile acid levels (p less than 0.01). Older patients have mild cholestasis and high serum vitamin E levels in comparison with younger ones. Improvement in bile excretion into the intestinal tract with age seemed to be responsible for an increase of serum vitamin E levels. Oral supplements of alpha-tocopherol in doses of 5 to 10 mg/kg/day were needed to maintain the normal serum vitamin E levels in postoperative infants.

Ataxia↗
PubMed1984

Fecal and biliary bile acid patterns in children with bile acid malabsorption.

Bile acid metabolism was examined in two children with bile acid malabsorption, who were being treated with intravenous hyperalimentation. Fecal bile acid excretion was 1,261 mumol/m2/day in a child with bile acid malabsorption of unknown origin, and 1,877 mumol/m2/day in a child with secondary bile acid malabsorption after an operation for long-segment aganglionosis. These values were approximately 10 times higher than those in diarrheal or nondiarrheal children without apparent abnormalities in bile acid metabolism. Fecal bile acids in these patients with bile acid malabsorption were almost completely conjugated, with little unconjugated bile acid present. It is possible that the disturbed bile acid deconjugation in the intestine might be caused by a rapid intestinal transit time, which was found in our patients with bile acid malabsorption. In the analysis of biliary lipid composition, children with bile acid malabsorption were shown to have a chenodeoxycholate-dominant pattern, an increased glycine- to taurine-conjugated bile acid ratio, and markedly supersaturated cholesterol. Such profiles may be related not only to bile acid malabsorption but also to cholestasis, presumably due to intravenous hyperalimentation.

Bile↗
PubMed1984

Familial intrahepatic cholestasis associated with progressive neuromuscular disease and vitamin E deficiency.

Three Japanese patients with familial progressive intrahepatic cholestasis developed complications involving neurologic abnormalities characterized by ataxia and pigmentary retinopathy. Serum vitamin E concentrations were extremely low in all patients, suggesting a long-term vitamin E deficiency. High dose oral supplementation of alpha-tocopherol produced normal serum vitamin E levels in two patients. Parenteral administration of vitamin E resulted in no clinical improvement in one patient who first received the treatment at 14 years of age. In the other two patients, the progression of neurological abnormalities was slowed by vitamin E supplementation. Cholestyramine treatment resulted in an apparent decrease in serum vitamin E levels despite oral alpha-tocopherol supplementation.

Adolescent↗
PubMed1984

Serum bile acid patterns determined by an enzymatic method and high-performance liquid chromatography in young infants with cholestasis.

Serum unconjugated and conjugated bile acids in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome, n = 8) or extra-hepatic cholestasis (preoperative extrahepatic biliary atresia, n = 8) were examined by an enzymatic procedure and high-performance liquid chromatography. In comparison with the mean level of total serum bile acid of controls having no liver or gastrointestinal diseases, those of each group markedly increased (15.6 +/- 5.1 vs. 120.9 +/- 64.0 and 161.8 +/- 54.2 nmol/ml), but those of unconjugated bile acid were almost unchanged (1.4 +/- 0.5 vs. 1.0 +/- 0.6 and 0.6 +/- 0.2 nmol/ml). The ratios of cholate to chenodeoxycholate and glycine- to taurine-conjugated bile acids (G/T) were not significantly different between the groups of intrahepatic and extrahepatic cholestasis. However, in the patients with intrahepatic cholestasis, the G/T ratio varied greatly and the quantitative determination of individual conjugated bile acids in serum revealed that a half of the patients examined had very low levels of taurine-conjugated cholate and chenodeoxycholate, suggesting a bile acid metabolism alternation specific for underlying intrahepatic cholestasis.

3-Hydroxysteroid Dehydrogenases↗
PubMed1984

Congenital constriction band syndrome.

Eighty-three patients with congenital constriction band syndrome were reviewed. Clinical manifestations and associated anomalies were analyzed with attention directed to distribution of the involvement. Constriction bands, amputation, and acrosyndactyly were the main clinical manifestations of this syndrome. Involvement of the distal portions of the extremity was most common. In the hand, the central digits were involved most frequently, and the thumb was only minimally affected in most cases. Of the 19 cases with an associated clubfoot deformity, 10 were proven to be a paralytic clubfoot due to compression neuropathy of the peroneal nerve caused by a deep constriction band below the knee, whereas 9 had a normal peripheral nerve.

Amniotic Band Syndrome↗
PubMed1984

Unconjugated, glycine-conjugated, taurine-conjugated bile acid nonsulfates and sulfates in urine of young infants with cholestasis.

A direct assay system for conjugated bile acids using an enzymatic procedure and high-performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra-hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3 beta-hydroxy-5-cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63 +/- 2.83 vs. 2.50 +/- 1.25, p less than 0.05, 15.8 +/- 9.9 vs. 34.5 +/- 9.9%, p less than 0.005). The proportion of taurine-conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7 +/- 7.5 vs 22.7% +/- 7.8%, p less than 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine-conjugated chenodeoxycholate sulfate excretion.

Bile Acids and Salts↗
PubMed1984

Evidence that two sizes of ventromedial hypothalamic neurones project to the mesencephalic central grey matter in rats.

The spatial spread of the extracellular antidromic action potentials was measured in eighty-three neurones in the ventromedial nucleus region of the female rat hypothalamus following electrical stimulation of the mesencephalic central grey matter. The positive-negative configuration of the antidromic action potentials across the extracellular field suggested that the potentials were generated predominantly by neuronal soma with simple geometries. When represented by the distance travelled by the electrode, at which peak-to-peak spike amplitude exceeded the half maximum value, the spatial spread of the extracellular field ranged from 25 to 174 micron. The frequency distribution for the field size was distinctively bimodal and could be divided into large and small groups at 85 micron. Antidromic action potentials with larger extracellular fields had significantly larger maximum spike amplitude and shorter duration, indicating that differences in field size were associated with neuronal size. At least 55% of the central grey projection of the ventromedial nucleus originated from small neurones. Taking into account the sampling bias, a much greater proportion of the central grey projection may arise from small neurones. The lack of a systematic difference in the antidromic spike latencies between large and small cells indicated that axonal thickness is not the major factor in determining the latency of the responses of the ventromedial hypothalamic neurones to stimulation of the central grey matter.

Amygdala↗
PubMed1984

T-cell subsets analyzed by monoclonal antibodies in patients with primary immunodeficiency diseases.

The proportion of T-cell subsets was normal in all patients with X-linked agammaglobulinemia except for an 8-month patient who showed a decrease in OKT8+ T-cell population. In two patients with hypogammaglobulinemia with IgM production, T-cell subsets were normal in distribution and the patients' B cells produced only IgM in culture with autologous T cells. A patient with common variable immunodeficiency showed no imbalance in distribution of T-cell subsets. Imbalance of regulatory T-cell subsets was found in a patient with DiGeorge syndrome and severe combined immunodeficiency.

Adolescent↗
PubMed1984

An Ia-like antigen positive null cell leukemia cell line (THP-5) lacking in the stimulating capacity in autologous and allogeneic mixed lymphocyte reaction.

A null cell leukemia cell line, THP-5, was established from the peripheral blood of a patient with acute null cell leukemia. THP-5 cells were characterized by the presence of Ia-like antigen and the absence of other cell surface markers examined. Despite the presence of Ia like antigen, THP-5 cells had no stimulating capacity in autologous or allogeneic mixed lymphocyte reaction.

Adolescent↗
PubMed1984

Direct measurement of urinary bile acids of infants by high-performance liquid chromatography connected with an enzyme immobilized column.

An accurate and sensitive method by high-performance liquid chromatography connected with 3 alpha-hydroxysteroid dehydrogenase immobilized column for the measurement of urinary bile acids of noncholestatic and cholestatic infants was performed in this study. The purification and group separation of urinary bile acids were done through a piperidinohydroxypropyl Sephadex LH-20 column. By ultraviolet spectromonitor, non 3 alpha-hydroxy bile acids such as 3 beta-hydroxy-5-cholenate were determined, and 3 alpha-hydroxy bile acids by fluorescence spectromonitor. Urinary bile acids in the specimens obtained from noncholestatic and cholestatic infants were determined by this procedure. Major bile acids were cholate, chenodeoxycholate conjugates and cholate unconjugate in healthy infants. In cholestatic infants, total and individual bile acids were extremely increased, and 3 beta-hydroxy-5-cholenate conjugates appeared in the sulfated fraction.

3-Hydroxysteroid Dehydrogenases↗
PubMed1984

Interleukin-2 activated peripheral blood lymphocytes and the correlation of lytic activity and peanut agglutinin receptor expression on malignant cells.

The possibility that the peanut agglutinin (PNA) receptor and/or its co-ordinate structures on the surface of leukaemia cells might be recognized by lectin free crude interleukin-2 (IL-2) activated human peripheral blood lymphocytes (LAK) was investigated. Analysis was undertaken of the correlation between the sensitivity to allogeneic LAK lysis and the proportion of PNA receptor on different fresh malignant cells. The results obtained suggested that PNA receptor detected by rhodamine isothiocyanate labelled PNA binding assay appeared to be well expressed on leukaemia cells which were sensitive targets for LAK. PNA receptor and/or its coordinate structures seemed to provide a 'target' structure for LAK.

Cytotoxicity, Immunologic↗
PubMed1984

Arthrogryposis of the hand.

Forty-five cases of arthrogryposis multiplex congenita were reviewed with respect to deformities and function of the hand. A distal predominance of the involvement and severity was apparent; hand deformity was a common manifestation in the arthrogrypotic patients. The majority of the hand deformities consisted of two types: one was a thumb-in-palm deformity with the fingers in intrinsic plus position, and another type was flexion contractures of the fingers at the interphalangeal joints. Regardless of type, hand function was impaired with severe or moderate deformity. Twenty-nine hands of 17 patients were successfully treated by surgery. Surgical methods are discussed based on an analysis of hand deformity and function.

Adolescent↗