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T Takayanagi

Publications and source records attributed to T Takayanagi.

At least 55 records · Page 3Linked to original sources
PubMed1998

[A case of Bartter's syndrome with chronic renal failure due to chronic interstitial nephritis].

We report a case of 45-year-old women with Bartter's syndrome and concomitant renal dysfunction. In 1986, the patient demonstrated muscle weakness and serum potassium levels as low as 1.1 mEq/l. She was suspected of having Bartter's syndrome because of hypokalemia, metabolic alkalosis, hyperreninemia, hyperaldosteronism and normotension. Pretibial edema developed in 1989 for which she received 40 to 100 mg/week of furosemide intermittently for the next 5 years. Her serum potassium level ranged from 1.5 to 3.9 mEq/l. In 1991, her serum creatinine level rose to 2.1 mg/dl, then continued to increase gradually. She was admitted to our hospital in 1994 for evaluation of the renal dysfunction. Decreased creatinine clearance (44 ml/min) and a defect in urinary concentrating capacity (Fishberg's test, 370 mOsm/kg.H2O) were detected. Renal biopsy revealed juxtaglomerular cell hyperplasia. These findings resulted in the diagnosis of Bartter's syndrome. The renal biopsy also showed diffuse interstitial fibrosis and marked tubular atrophy. We postulate in this case that long-term hypokalemia due to Bartter's syndrome and the administration of furosemide led to chronic interstitial nephritis and renal dysfunction.

Bartter Syndrome↗
PubMed1998

[A report of two siblings with both maternal dentato-rubro-pallido-luysian atrophy and paternal Ehlers-Danlos syndrome type III].

The report deals with the first description of two siblings, a 43-year-old woman and 39-year-old man, who have developed cerebellar ataxia, choreoathetosis, dementia, epilepsy, hyperelasticity of the skin, and hypermobility and dislocation of joints. The frequent dislocations of joints sometimes could not be corrected surgically. Their mother and maternal uncle have the same neurological signs and symptoms as the siblings do. However, they do not present with the signs of skin and joints. Gene analysis of peripheral blood lymphocytes from these 4 patients revealed that the CAG repeat length of the dentato-rubro-pallido-luysian atrophy (DRPLA) gene is increased in all four. On the contrary, their father has displayed only hyperelasticity of the skin. From the clinical signs, family history and skin biopsy, we consider that the siblings and their father have Ehlers-Danlos syndrome (EDS) type III. In conclusion, the previously unreported coincidental development of maternal DRPLA and paternal EDS observed in two siblings deserves to be described.

Adult↗
PubMed1998

[Screaming during sleep in patients with Parkinson disease].

We studied 12 patients with Parkinson disease who scream while sleeping. All 12 patients showed clinical manifestations and brain images of typical idiopathic Parkinson disease. On average, the screaming began 4.8 years after the onset signs and symptoms of Parkinson disease. In many cases, sleep talking started before the onset of Parkinson disease. All patients reported that the screaming disrupted the sleep of their families, and half of the patients reported that the screams disturbed their own sleep. The screams were incorporated into their dreams. Clonazepam was effective to alleviate this screaming in 8 out of 9 cases. We considered this screaming to be caused by similar mechanisms as rapid eye movement (REM) sleep behavior disorder in which muscle atonia characterizing normal REM sleep is absent. The screams were not accompanied by other abnormal behaviors. We postulate that the screaming is a symptom closely related to that of mid or lower brainstem lesion in Parkinson disease because the neural activity of the locus ceruleus or the pudunculopontine nucleus are responsible for muscle atonia in REM sleep.

Aged↗
PubMed1997

International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology.

Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to various types of pharmacological agents, but the scales used for assessment were all different. With the long-term goal of double-blind controlled trials-multicentric and international-an ad hoc Committee of the World Federation of Neurology has worked to propose a one-hundred-point semi-quantitative International Cooperative Ataxia Rating Scale (ICARS). The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied. The weight of each symptomatologic compartment has been carefully designed. The members of the Committee agreed upon precise definitions of the tests, to minimize interobserver variations. The validation of this scale is in progress.

Cerebellar Ataxia↗
PubMed1997

Influence of graft versus host reaction on the T cell repertoire differentiating from bone marrow precursors following allogeneic bone marrow transplantation.

When lethally irradiated AKR (Mls-1a) mice were reconstituted with bone marrow (BM) cells plus a small number (0.5%) of mature T cells from allogeneic B10.AQR or B10 (Mls-1b) mice and minor GVHR was induced in the recipients, almost complete donor chimerism was accomplished in the early stages after reconstitution. By contrast, in irradiated AKR mice reconstituted with T cell-depleted BM cells alone from B10 or B10.AQR mice, radio-resistant T cells of recipient origin persisted for a relatively long period in peripheral lymphoid tissues. In this paper the influence of residual T cells in the chimeric mice on generation of the T cell repertoire derived from donor BM is discussed. It will be demonstrated that the recipient (AKR) T cells are capable of producing Mls-1a antigens (Ag) after lethal irradiation in vivo. These recipient T cells eventually induce clonal elimination of Mls-1a reactive V beta 6+, V beta 8.1+ and V beta 9+ T cells derived from developing thymocytes of donor BM origin. The Mls-1a reactive T cells are not eliminated in GVHR chimeras in which recipient T cells are absent. However, V beta 5+ T cells reactive to I-E plus Etc-1 Ag are deleted in the chimeras undergoing GVHR. These results indicate that recipient cells which produce tissue-specific antigens (tolerogens) should be taken into consideration when generation of the T cell repertoire of donor origin following allogeneic BM transplantation is investigated.

Animals↗
PubMed1997

Effects of non-major histocompatibility antigens on acute graft-versus-host reaction after allogeneic bone marrow transplantation.

In the present study using an experimental BMT system we analyzed the effects of disparity at non-MHC Ag including minor lymphocyte stimulatory-1a (Mls-1a) Ag on the acute GVH reaction (GVHR) induced by MHC class I Ag. Mismatch at MHC (class I) Ag alone did not induce clinically detectable acute GVHR in this model. However, BMT mice prepared with a combination of both class I and non-MHC Ag mismatches showed signs of clinical GVHR and various cytokines were produced by the spleen cells at an early stage (4 days) after BMT. Although no clinical GVHR was detected in BMT chimeras prepared with a non-MHC mismatched but MHC matched combination, large amounts of various cytokines were secreted by spleen cells. Cytokine production in the latter two kinds of chimeras paralleled the increase of Mls-1a reactive Vbeta6+ T cells in the host spleen. Marked cytokine production induced by Mls-1a Ag was confirmed by MLR. Thus, these cytokines appeared to be produced by T cells responding to Mls-1a (ie Vbeta6+ T cells) and to augment the T cell responses to MHC class I which resulted in clinically detectable GVHR in chimeras prepared with the combination mismatched at both MHC class I and non-MHC loci.

Animals↗
PubMed1997

Spinocerebellar ataxia type 6. Molecular and clinical features of 35 Japanese patients including one homozygous for the CAG repeat expansion.

Spinocerebellar ataxia type 6 (SCA6) is a newly classified autosomal-dominant cerebellar ataxia (ADCA) associated with CAG repeat expansion. We screened 111 patients with cerebellar ataxia for the SCA6 mutation. Of these, 35 patients were found to have expanded CAG repeats in the SCA6 gene, indicating that second to SCA3, SCA6 is the most common ADCA in Japan. Expanded alleles ranged from 21 to 29 repeats, whereas normal alleles had seven to 17 repeats. There was no change in the CAG repeat length during meiosis. The age at onset was inversely correlated with the repeat length. The main clinical feature of the 35 patients with SCA6 was slowly progressive cerebellar ataxia; multisystem involvement was not common. The 35 patients included nine cases without apparent family history of cerebellar ataxia. The sporadic cases had smaller CAG repeats (21 or 22 repeats) and a later age at onset (64.9 +/- 4.9 years) than the other cases with established family history. We also identified one patient who was homozygous for the SCA6 repeat expansion. The homozygote showed an earlier age of onset and more severe clinical manifestations than her sister, a heterozygote carrying an expanded allele with the same repeat length as the homozygote. This finding suggests that the dosage of the CAG repeat expansion plays an important role in phenotypic expression in SCA6.

Adult↗
PubMed1997

[A family with dentato-rubro-pallido-luysian atrophy (DRPLA): an intergenerational contraction of the CAG repeat].

We describe the relation of the CAG repeat length to the genetic anticipation in a Japanese family with dentato-rubro-pallido-luysian atrophy (DRPLA). The proband, a 21-year-old woman, developed epilepsy at age 19. Her mother has displayed cerebellar ataxia, choreoathetosis, and dementia since age 40, and the grandmother has shown cerebellar ataxia since age 52. So a genetic anticipation was observed. The CAG repeat sizes of peripheral blood lymphocytes from the proband, mother, and grandmother were found to be 61, 59, and 60, respectively. Thus, the mother showed earlier onset but a smaller CAG repeat length as compared to the grandmother. This case is thought to be rare and valuable in respect that the intergenerational contraction of the CAG repeat was shown in spite of the genetic anticipation was observed.

Adult↗
PubMed1997

[A case of dystrophia myotonica with homozygous DM kinase abnormalities].

We report a case of dystrophia myotonica (DM) in a 38-year-old man with homozygous DM kinase abnormality. His distal muscle strength was reduced moderately; muscle biopsy showed type 1 predominance and type 1 fiber atrophy. The patient's WAIS total IQ score was under 60. Since his childhood, his cognitive deficit has been more severe than his muscle weakness. MRI demonstrated many abnormal changes in the brain of this patient, but these changes were mild in comparison to the severity of his reduced cognition and low IQ. The relationship between mental dysfunction and DM kinase abnormalities is quite different from that seen in cases of heterozygous DM kinase abnormalities. This case demonstrated severe mental changes in spite of mild DM kinase abnormalities. We suspect the homozygous DM kinase abnormality to be a cause of the different clinical presentations of this patient.

Adult↗
PubMed1997

[An apparently sporadic case with spinocerebellar ataxia type 1 (SCA1)].

We reported a sporadic case with late onset SCA1. There was no family history of neurological diseases. His parents had been healthy until they died at the age of 77 and 89 years, respectively. The patient noticed gait disturbance at age of 60. Thereafter, he gradually developed cerebellar ataxia, hyporeflexia, mild atrophy of the facial and limb muscles and moderate deep sensory disturbance. MRI of the brain showed moderate atrophy of the cerebellum and brainstem. Sequencing analysis of SCA1 gene demonstrated that the patient had an expanded allele with 40 CAG repeats and no CAT interruption. Consequently, he was diagnosed as having SCA1. These results suggest the possibility that among apparently sporadic cases with cerebellar ataxia, there are some cases of SCA1 with mild CAG repeat expansion.

Atrophy↗
PubMed1997

[A case of idiopathic hypereosinophilic syndrome with special reference to mental disorders and brain images].

We reported a case of idiopathic hypereosinophilic syndrome with psychiatric symptoms and abnormal areas in subcortical white matter on brain MRI. The 25-year-old man was referred to us because of fever, confusional state and emotional incontinence. He soon became apathetic and emotionless. While he was confused, we studied brain CT twice and brain MRI four times. In deep white matter around the lateral ventricles and the cortices of parietal lobe, occipital lobe and cerebellar hemispheres, T1-weighted MRI revealed low intensity areas and T2-weighted MRI showed high intensity areas. After gadolinium infusion, some above-stated areas were enhanced. The lesions in the deep white matter disappeared when symptoms diminished, while lesions in the occipital cortex remained unchanged. Brain MRI studies were beneficial and useful to analyse the rare idiopathic hypereosinophilic syndrome with psychic disorders.

Adult↗
PubMed1997

[Pharmacokinetic and clinical evaluation of cefozopran in newborn patients].

Pharmacokinetic and clinical evaluation of an injectable cephem antibiotics, cefozopran (SCE-2787, CZOP), was conducted in newborn patients and the following results were obtained: 1. Clinical results The clinical efficacy of CZOP was evaluated in one each patient with intrauterine infection and suspected septicemia. The efficacy was "excellent" in both patients. No clinically serious adverse drug reactions of signs and symptoms and abnormal alterations of the laboratory test values were recognized. 2. Pharmacokinetics CZOP was intravenously given to newborn patients at doses of 25.0, 20.0, and 18.75 mg/kg. The blood CZOP concentrations were 44.7 +/- 7.0 micrograms/ml (n = 3), 48.3 micrograms/ml and 48.2 micrograms/ml at one hour after administration, respectively. The elimination half life (T 1/2) was 4.22 +/- 1.17 hours (n = 3) in the patients given 25.0 mg/kg and 2.74 hours in the patient given 20.0 mg/kg. The urinary drug excretion rate was 44.5 +/- 8.7% and 31.3 +/- 9.7% of dose within 8 hours after administration of 25.0 mg/kg and 20.0 mg/kg, respectively.

Bacterial Infections↗
PubMed1996

Coronary artery spasm does not depend on the intracellular calcium store but is substantially mediated by the protein kinase C-mediated pathway in a swine model with interleukin-1 beta in vivo.

BACKGROUND: The intracellular mechanism for coronary artery spasm is still unknown. Since the protein kinase C (PKC)-mediated pathway and Ca2+ release from sarcoplasmic reticulum (SR) are important intracellular mechanisms of vascular smooth muscle contraction, we examined the possible role of these two mechanisms in the pathogenesis of coronary spasm in our swine model in vivo. METHODS AND RESULTS: In 25 pigs, interleukin-1 beta (IL-1 beta) was applied chronically to the coronary arteries from the adventitia to induce an inflammatory/proliferative lesion. Two weeks after the operation, either intracoronary serotonin or histamine repeatedly induced coronary spasm at the IL-1 beta-treated site. At those spastic sites, phorbol-12, 13-dibutyrate, a PKC-activating phorbol ester, also induced coronary spasm, which was blocked by pretreatment with the PKC inhibitors staurosporine and sphingosine. Serotonin- and histamine-induced coronary spasm was also significantly inhibited by pretreatment with staurosporine, sphingosine, or nifedipine (an L-type Ca2+ channel antagonist) but not by ryanodine (an inhibitor of Ca(2+)-induced Ca2+ release from SR) or thapsigargin (an inhibitor of Ca(2+)-ATPase of SR). Bay K 8644 (an L-type Ca2+ channel agonist) also induced coronary spasm at the IL-1 beta-treated site, which was significantly inhibited by pretreatment with staurosporine, sphingosine, and nifedipine. In contrast, coronary vasoconstriction induced by prostaglandin F2 alpha was not affected by pretreatment with staurosporine or sphingosine but was significantly inhibited by pretreatment with ryanodine, thapsigargin, or nifedipine. CONCLUSIONS: These results suggest that (1) PKC activation largely accounts for the serotonin- and histamine-induced coronary spasm; (2) at the spastic site, the calcium influx through L-type Ca2+ channels may be augmented via the PKC-mediated pathway; and (3) the Ca2+ release from the SR into the cytosol may not play a primary role in coronary spasm.

3-Pyridinecarboxylic acid, 1,4-dihydro-2,6-dimethy↗
PubMed1996

Chronic treatment with interleukin-1 beta induces coronary intimal lesions and vasospastic responses in pigs in vivo. The role of platelet-derived growth factor.

Studies in vitro have suggested that inflammatory cytokines may play an important role in the pathogenesis of atherosclerosis. However, little is known about their effects in vivo. Thus, the present study was designed to determine in vivo what histological and functional changes may be induced by chronic treatment with IL-1 beta, one of the major inflammatory cytokines, and also to clarify what mechanisms are involved in those changes. Under aseptic conditions, proximal segments of the left porcine coronary arteries were gently wrapped with cotton mesh absorbing Sepharose beads either with or without recombinant human IL-1 beta. From 1 to 4 wk after the operation, coronary vasospastic responses to intracoronary serotonin or histamine were noted at the IL-1 beta-treated site but not at the control site. Histologically, intimal thickening was greater at the IL-1 beta-treated site than at the control site. Those functional and histological changes induced by the chronic treatment with IL-1 beta were significantly inhibited by the simultaneous treatment with a neutralizing antibody to either IL-1 beta or PDGF. These results indicate that chronic treatment with Il-1 beta induces coronary intimal lesions and vasospastic responses in porcine coronary arteries in vivo and also suggest that these changes are substantially mediated by PDGF.

Animals↗
PubMed1996

Autosomal dominant cerebellar ataxias in the Kinki area of Japan.

The autosomal dominant cerebellar ataxias are a heterogeneous group of neurodegenerative disorders characterized by slowly progressive cerebellar ataxia. Recently, among the ataxias, spinocerebellar ataxia type 1 (SCA1), Machado-Joseph disease (MJD) and dentatorubral-pallidoluysian atrophy have been found to be caused by expansion of a CAG trinucleotide repeat in the coding region of the disease genes. We have analyzed the CAG repeats of 67 patients from 47 families with dominantly inherited ataxia who lived in the Kinki area of Japan. The following results were obtained. First, 31 patients from 22 families were found to be positive for the MJD repeat expansion, indicating that MJD is the most common dominantly inherited ataxia in the Kinki area of Japan. Second, no SCA1 repeat expansion was found among the families studied. This presents a striking contrast to the fact that there are many families with SCA1 in Hokkaido and the Tohoku area of Japan. These findings suggest geographic variation in autosomal dominant cerebellar ataxias in Japan.

Asian People↗
PubMed1996

Relationship of (CAG)nC configuration to repeat instability of the Machado-Joseph disease gene.

The mutation responsible for Machado-Joseph disease (MJD) has been identified as an expansion of a CAG trinucleotide repeat in a novel gene on chromosome 14q32.1. The CAG repeat tract is followed by C or G, and alleles are thereby divided into two types on the basis of molecular configuration, (CAG)nC and (CAG)nG. We have studied the relationship between the repeat length and the configuration in 38 patients from 28 Japanese families with MJD, and 31 unrelated normal Japanese subjects. The CAG repeat length in 100 normal alleles ranged from 13 to 37 repeats, while 38 MJD patients had one expanded allele with 64 to 84 repeats. Surprisingly, the expanded alleles had exclusively the (CAG)nC configuration, while both (CAG)nC and (CAG)nG were seen in normal alleles from MJD and control subjects. Furthermore, in normal alleles, the CAG repeat tract was significantly longer in (CAG)nC than in (CAG)nG. These findings suggest that the (CAG)nC configuration is related to repeat instability of the MJD gene.

Alleles↗
PubMed1996

The importance of the hyperpolarizing mechanism increases as the vessel size decreases in endothelium-dependent relaxations in rat mesenteric circulation.

Endothelium-dependent relaxations are achieved by a combination of endothelium-derived prostacyclin (PGI2), nitric oxide (NO), and endothelium-derived hyperpolarizing factor (EDHF). However, it remains to be fully clarified whether the relative contribution of these three mechanisms to endothelium-dependent relaxations varies as a function of the vessel size. This study was designed to clarify this point. Acetylcholine (ACh)-induced endothelium-dependent relaxations were examined in isolated blood vessels taken from the aorta and the proximal and distal mesenteric arteries of the rat. The contributions of PGI2, NO, and EDHF were evaluated by the inhibitory effects of indomethacin, N omega-nitro-L-arginine methyl ester (L-NAME) in the presence of indomethacin, and KCl in the presence of indomethacin and L-NAME, respectively. The membrane potentials were recorded with microelectrodes. The expression of endothelial No synthase (eNOS) was examined by both immunostaining and immunoblotting. The contribution of PGI2 was negligible in three different-sized blood vessels. The contribution of NO was most prominent in the aorta, whereas that of EDHF was most prominent in the distal mesenteric arteries. The resting membrane potential was significantly deeper and the ACh-induced hyperpolarization was greater in the distal mesenteric arteries than those in the aorta. The expression of eNOS was the highest in the aorta and the lowest in the distal mesenteric arteries. These results indicate that the importance of EDHF increases as the vessel size decreases in endothelium-dependent relaxations in the rat mesenteric circulation.

Acetylcholine↗