[Case of renal hypouricemia causing exercise-induced acute renal failure].
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Acute Kidney Injury↗
Biomedical subjects
T Hosoya
Publications and source records attributed to T Hosoya.
Evidence of association of the ecNOS gene polymorphism with plasma NO metabolite levels in humans.
Nitric oxide (NO) synthesized by the vascular endothelium regulates mammalian blood vessels and other systems in humans. Recently, an endothelial nitric oxide synthase (ecNOS) gene polymorphism, the 27-bp repeat in intron 4 (ecNOS4), was reported to be related to the pathogenesis of coronary heart disease and terminal renal failure. We analyzed this polymorphism in a group of 413 healthy subjects, and measured their plasma nitrite and nitrate (NOx) levels. The frequency of the b allele was 89.8% , and the frequency of the a allele was 10.2%. The frequency of ecNOS4 b/b, ecNOS4 b/a, and ecNOS4 a/a in the healthy subjects in this study was 0.814 (n=336), 0.169 (n=70) and 0.017 (n=7), respectively. Using this polymorphism as a DNA marker, we found a strong association between the alleles of the ecNOS gene polymorphism and the plasma NOx levels. The basal NO metabolite levels were 28.8 micromol/L in subjects with ecNOS4 a/a, 31.4 micromol/L in those with ecNOS4 b/a, and 35.5 micromol/L in those with ecNOS4 b/b. The mean plasma NOx level of the subjects who were homozygous for the a allele was nearly 20% lower than in the subjects with the b allele. The plasma NOx level of the subjects with the a allele was 31.2+/-2.00 micromol/L, and significantly lower than the 35.5+/-0.93 micromol/L in those without the a allele (P <0.05). The results of this study indicate that the ecNOS4 gene locus may be responsible for variations in the genetic control of plasma NOx and that analysis of ecNOS4 gene polymorphism may be a useful tool for studying the relation between NO and diseases.
Adult↗
Mutations in xanthine dehydrogenase gene in subjects with hereditary xanthinuria.
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Base Sequence↗
Dilated Virchow-Robin spaces: MRI pathological study.
We performed a histopathological study of two human brains to look at dilated Virchow-Robin (V-R) spaces in the anterior perforated substance and putamen. We measured the diameter of 74 arteries in 54 dilated V-R spaces. In 28 patients without neurological deficits we ascertained the characteristic location of dilated V-R spaces in the anterior perforated substance and basal ganglia on MRI, measuring the distance from 64 foci of cerebrospinal fluid signal intensity to the centre of the mamillary body on 1 mm thick images. In the histopathological study, the mean diameter of the arteries was 39.0 +/- 36.0 microns. Dilatation of the V-R space was observed from the end of the indentation of the pial membrane towards the brain surface along the perforating artery. In the MR images, the mean distance from the dilated V-R space to the mamillary body was 10.0 +/- 4.5 mm. The V-R space was confined to a fixed level in the lower part of the basal ganglia, and not found near the brain surface.
Adult↗
MRI anatomy of white matter layers around the trigone of the lateral ventricle.
MRI was obtained in eight normal volunteers and seven patients with brain oedema around the trigone. In addition to the conventional sequences, diffusion-weighted and intravoxel-incoherent-motion images using motion-proving anteroposterior and/or lateral direction gradients were obtained to show the white matter pathways better. Coronal proton-density-weighted images showed three thin relatively high-intensity layers in addition to the tapetum and the internal and external sagittal strata. Although they have not been confirmed anatomically, the thin layer between the internal and the external sagittal strata was corroborated by diffusion-weighted and intravoxel-incoherent-motion images, and by characteristics of the spread of oedema into the sagittal stratum. We propose that this layer be named the central sagittal lamina. The other two layers medial and lateral to the sagittal stratum were outside, but in contact with the medial and lateral parts of the sagittal stratum, respectively. We provisionally named them medial and lateral sagittal laminae; they were not evident on any other images. The low-intensity layer on T2-weighting was the internal sagittal stratum. The optic radiation, comprising the external sagittal stratum, appeared as an intermediate to slightly high-intensity layer on T2-weighted images and a low-intensity layer on T1-weighted images as did the corticospinal tract in the posterior internal capsule.
Adult↗
Alteration of cell fate by ectopic expression of Drosophila glial cells missing in non-neural cells.
The glial cells missing (gcm) gene encodes an essential transcription factor that converts neuronal precursor cells to glial fate in the Drosophila nervous system. In this study, we tested effects of gcm ectopic expression on fate of non-neural cells. When gcm expression was continuously induced in epidermal cells from around stage 9, these cells started to exhibit mesenchymal cell morphology at stage 13, which was preceded by the onset of expression of Repo, a glial marker. The morphological change was coincident with loss of expression of an epidermal cell-adhesion molecule. In addition to the epidermis, fate of mesodermal cells was also affected by gcm ectopic expression. These findings suggest that gcm can convert gene expression and cell morphology even outside the neuroectoderm.
Animals↗
Mesalazine-associated severe aplastic anemia successfully treated with antithymocyte globulin, cyclosporine and granulocyte colony-stimulating factor.
A 20-year-old male with ulcerative colitis complicated by mesalazine-associated severe aplastic anemia is described. The patient developed aplastic anemia four months after the start of mesalazine therapy. He was treated with antithymocyte globulin, cyclosporine, and granulocyte colony-stimulating factor (G-CSF) and responded well. Hematological complications of mesalazine are rare, but if bone marrow suppression is detected, immediate cessation of the drug and intensive immunosuppressive treatment with G-CSF should be considered.
Adult↗
Long-term administration of G-CSF for aplastic anaemia is closely related to the early evolution of monosomy 7 MDS in adults.
There is an increasing incidence of the evolution of myelodysplastic syndrome (MDS) from aplastic anaemia (AA) with immunosuppressive treatment. In paediatric patients G-CSF is also reported to increase MDS evolution, but this process is not precisely understood in children or in adults. Therefore risk factors of MDS evolution in adults are evaluated here. Of 72 patients, five developed MDS. In 47 patients without cyclosporine (CyA) or antithymocyte globulin (ATG) therapy, only one developed MDS with trisomy 8, 242 months after diagnosis. But of 25 patients treated with either CyA or ATG, four developed monosomy 7 MDS within 3 years. Of these 25 patients, 18 were treated with G-CSF and the four patients (22.2%) who developed MDS were found in this group. The cumulative dose and the duration of G-CSF administration were significantly elevated in patients who developed MDS when compared with those who did not, 822.3 +/- 185.0 v 205.4 +/- 25.5 microg/kg (P<0.05) and 187.5 +/- 52.5 v 72.0 +/- 24.6 d (P<0.002), respectively. However these two values for CyA did not differ significantly. Statistically, treatment with CyA, G-CSF and combined G-CSF and CyA were significantly related to MDS evolution. The administration of G-CSF for more than a year was the most important factor (P=0.00). These results suggested that a close relationship exists between G-CSF and subsequent monosomy 7 MDS from AA in adults who receive immunosuppressive therapy. Long-term administration of G-CSF should be prohibited in order to prevent MDS evolution.
Adolescent↗
Apa I polymorphism in the vitamin D receptor gene may affect the parathyroid response in Japanese with end-stage renal disease.
Vitamin D [1,25(OH)2D3] plays a key role in the pathogenesis of secondary hyperparathyroidism. A polymorphism in the vitamin D receptor (VDR) gene is reported to be involved in bone mineral density and the serum level of intact-osteocalcin (i-OC) in patients with osteoporosis. We investigated the relationship between VDR gene polymorphisms and the levels of intact PTH (i-PTH) and i-OC in 129 Japanese patients with end-stage renal disease (ESRD). The VDR gene sequences were PCR-amplified, and the product was cleaved with the restriction enzymes Bsm I and Apa I. Undigested alleles were designated as B and A, and the digested alleles as b and a, respectively. The frequencies for the Bsm I polymorphism were 0.0% BB, 19.4% Bb, and 80.6% bb, while those for Apa I polymorphism were 14.2% AA, 47.2% Aa, and 38.6% aa. The Bsm I polymorphism of VDR was greatly biased in Japanese people. The i-PTH level in the aa group was about twice as high as those in the both AA group and Aa group (P < or = 0.04). The i-OC concentrations in the aa group was also approximately double those in both the AA group and Aa group (P < or = 0.03). In contrast, no significant differences in age, duration of dialysis, male/female ratio, or the incidence of diabetic nephropathy were observed among these three groups. On the other hand, there was no significant differences in i-PTH and i-OC between the Bb and bb groups. These results suggest that VDR gene polymorphisms can affect parathyroid response in ESRD patients, and the Apa I polymorphism is more informative in Japanese patients than the Bsm I polymorphism. The VDR a gene allele may define the pathogenesis of secondary hyperparathyroidism and of high turnover bone disease in patients with ESRD.
Alleles↗
Correlation of antithyroglobulin and antithyroid-peroxidase antibody profiles with clinical and ultrasound characteristics of chronic thyroiditis.
Patients with chronic thyroiditis were selected for study according to specific selection criteria, and comparisons of an enzyme-linked immunosorbent assay (ELISA) method to a hemagglutination method were performed. The ELISA assays contained antithyroglobulin (TG) antibody and antithyroid-peroxidase (PO) antibody, the latter using recombinant human TPO. Four groups were established from 437 patients with chronic thyroiditis; 316 with both antibodies (TG + PO group), 75 with TG antibody alone (TG group), 10 with PO antibody alone (PO group), and 36 with no such antibodies (N group). The hemagglutination assay resulted in much different antibody profiles: PO group based on ELISA occurred in only 2%, whereas a group with microsome antibody alone based on hemagglutination assay occurred in 39%. Subsequently, studies of disease characteristics including age, gender, familial predisposition, thyroid function, and morphology of the gland were performed among the four groups of patients based on ELISA profiles but not on hemagglutination results. The subgroups of TG + PO and TG were derived from randomly selected patients (40 out of a much larger number of patients in each). The results showed that the PO group had smaller thyroid volume (25+/-16 mL, mean +/- SD) with normal echogenicity, and 50% prevalence of hypothyroidism. The TG group had larger thyroid volume (57+/-42 mL) with frequent association of small nodular formation (53%) but less frequent hypothyroidism (23%). In summary, compared to the hemagglutination method, the ELISA was noted to have both a higher sensitivity and specificity for detection of chronic thyroiditis. Correlation of ELISA profiles with ultrasonography of the thyroid gland detected subtle differences in subgroups that may account for differences in thyroid gland morphology and prevalence of hypothyroidism: the PO group has normal echogenicity but high incidence of impaired thyroid function, whereas in the TG group small nodules were associated with a lesser incidence of hypothyroidism.
Adult↗
Oxidative stress suppresses the endothelial secretion of endothelin.
To address endothelial function on vascular walls exposed to oxidative stress, we investigated the effect of oxidative stress on the secretion of endothelin-1 (ET-1) from cultured bovine carotid artery endothelial cells (BAECs). Concentrations of ET-1 in the media were measured by a specific radioimmunoassay and ET-1 mRNA expression was estimated by Northern blot analysis. Treatment of BAECs with 0.5-2.0 mM H2O2 for 3 h suppressed both ET-1 secretion and ET-1 mRNA expression in a dose-dependent manner compared to control. Attenuation of ET-1 mRNA expression by H2O2 was revealed to take place at the transcriptional level. The addition of NG-nitro-L-arginine-methyl ester (L-NAME) 10 microns, a specific nitric oxide synthase inhibitor, had no effect on H2O2-induced suppression of ET-1 mRNA expression. Suppression of ET secretion under oxidative stress observed in the present study is proposed to be a compensatory mechanism of endothelial cells to inhibit vasoconstriction and proliferation during oxidative stress.
Animals↗
Combination chemotherapy with G-CSF, M-CSF and EPO: successful treatment for acute myelogenous leukemia without blood transfusion at lower medical costs.
A 55-year-old Jehova's Witness was treated for acute myelogenous leukemia (AML) by intensive chemotherapy with enocitabine, 6-mercaptopurine and daunorubicin. G-CSF, M-CSF and EPO were subsequently administered. Even though no blood transfusion was given for religious reasons, complete remission was achieved without serious infection and hemorrhage. The total cost for induction chemotherapy was less expensive than is the case for elderly AML patients. This case indicates that the administration of cytokines might reduce the incidence of infection and the necessity for blood products, which would result in favorable cost effectiveness for the treatment of elderly patients with AML.
Antineoplastic Combined Chemotherapy Protocols↗
Cyclosporine and entrapment neuropathy. Report of two cases.
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Cyclosporine↗
Role of nitric oxide in hypotension during hemodialysis.
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Female↗
Malignant insulinoma with extensive liver metastases presenting as disturbance of consciousness.
A 56-year-old man was referred to our hospital for evaluation of episodic disturbance of consciousness. Hypoglycemic symptoms were noted and Whipple's triad was satisfied. The 75 g OGTT and the glucagon test revealed a high baseline insulin level and hyperreactivity to glucagon. A pancreatic tumor and liver metastases were found by abdominal computed tomography (CT). Based on the finding of liver biopsy, the final diagnosis was malignant insulinoma with liver metastasis. He selected conservative treatment and no hypoglycemic crisis has occurred for one year since discharge. Early diagnosis and long-term follow-up is necessary since this tumor is slow growing.
Blood Glucose↗
Two siblings with classical xanthinuria type 1: significance of allopurinol loading test.
Two brothers with classical xanthinuria who lacked xanthine dehydrogenase activity were encountered. Their hypouricemia was caused by underproduction of uric acid. In their duodenal mucosa, no xanthine dehydrogenase (oxidase) activity was detected. The patients had no symptoms except for duodenal ulcer in one case. The conversion of allopurinol to oxipurinol during an allopurinol loading test for determining the type of classical xanthinuria revealed that the patients had classical type 1 xanthinuria, because aldehyde oxidase activity was present. Furthermore, the allopurinol loading test was conducted to determine the optimal examination times and specimens required for this test.
Adult↗
Partial deficiency of hypoxanthine-guanine phosphoribosyltransferase manifesting as acute renal damage.
A 32-year-old man who had had frequent gouty arthritis over the past 17 years, was admitted for acute renal failure. Acute renal failure was improved rapidly after medication was resumed and the patient was sufficiently hydrated. The hypoxanthine-guanine phosphoribosyltransferase (HPRT) activity in the patient had been reduced to about 30% of the normal control. Therefore we considered that this patient suffered from a partial deficiency of HPRT. A point mutation of HPRT gene 68G (guanine) to T (thymine) was detected. This is a mutation that has not been previously reported. Familial analysis indicated that his mother and sister were heterozygotes.
Acute Kidney Injury↗
[Renal damage in a chronic active hepatitis C patient receiving interferon-alpha therapy].
A 68-year-old male patient with chronic active hepatitis C was treated with interferon-alpha (IFN-alpha) for a period of 5 months. The patient responded well to the IFN therapy showing substantial improvement in liver function and disappearance of HCV-RNA. However, one year after the treatment he was found to have developed proteinuria and showed a reduction in Ccr. Renal biopsy findings were as follows: Light microscopy showed diffuse expansion of mesangial cells with a focal/local increase in cellularity accompanied by capillary loop thickening. Splitting of the basement membrane was also present. An immunofluorescent study showed that IgA was localized predominantly in the peripheral capillary wall. Electron microscopy showed that there was mesangial cell interposition between the peripheral capillary wall and endothelial cells. Furthermore, endothelial cells were expanded and numerous platelets were seen in the capillary lumen. These findings were compatible with focal MPGN accompanied by activation of endothelial cells. These histological data suggest two clinical disease entities: late-onset renal damage induced by IFN-alpha alone, and HCV-induced renal damage possibly modified by the direct effect of IFN-alpha on the endothelium. The present case suggests that IFN therapy for HCV may produce a particular type of renal damage, under the influence of either IFN or HCV infection, and/or both.
Aged↗