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Biomedical subjects

M Sudo

Publications and source records attributed to M Sudo.

At least 145 records · Page 8Linked to original sources

Urinary sugar phosphates and related organic acids in fructose-1,6-diphosphatase deficiency.

Two sisters with fructose-1,6-diphosphatase deficiency are reported. They presented with ketonuria, elevated plasma transaminase activity and severe metabolic acidosis during hypoglycaemic crises, which resembled Reye syndrome. Intravenous fructose tolerance tests provoked severe hypoglycaemia and metabolic acidosis. Fructose-1,6-diphosphatase activities in both peripheral leukocytes and cultured lymphocytes were below the limit of detection. Urinary organic acid analysis during crises revealed markedly increased excretion of lactate, ketone bodies, glycerol and glycerol-3-phosphate. We newly identified other glycolytic intermediates, glyceraldehyde, 3-phosphoglycerate and fructose-1,6-diphosphate, in the urine during hypoglycaemic attacks or after fructose tolerance tests. Identification of such compounds may be useful in the early diagnosis of this disease.

Female↗

Ultrasonographic evaluation of bladder volume in children.

Although ultrasound has been recently used to estimate bladder volume, several different formulae are used. We analysed the accuracy of ultrasound evaluation of bladder volume with an ultrasound scanner equipped with a videocassette recorder. We determined three dimensions of the bladder, i.e. depth (D), height (H) and width (W) by reviewing videotapes in 53 children. D x H x W (DHW) showed a good linear relationship with actual bladder volume (r = 0.96). Estimation of bladder volume by the regression equation gave errors of 12.5 +/- 8.6% (mean +/- SD) with errors above 25% in only 6% of children. Estimation of the volume by simply multiplying DHW by a correction factor of 0.80 gave a greater error of 18.9 +/- 14.0% (P < 0.001) with errors above 25% in 24% of children. Thus, the use of the formula volume (ml) = DHW (cm3) x 0.68+4 was sufficiently accurate in the ultrasound estimation of bladder volume in children.

Adolescent↗

Clinical course and outcome of idiopathic membranous nephropathy in Japanese children.

We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9-15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3-1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6-11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed; it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.

Adolescent↗

Urinary alpha 1-microglobulin as an index of proximal tubular function in early infancy.

Urinary alpha 1-microglobulin (U-A1M) was measured in healthy term infants on days 1, 4, 7, 14, 28, 90 and 180 of life. U-A1M was high until day 14 and declined thereafter. It was significantly correlated with urinary beta 2-microglobulin (U-B2M) throughout the study, but not with serum A1M on days 1 or 7. Similar to U-B2M, U-A1M in the clinically stable term infants with intrauterine growth retardation (n = 4-7) was not elevated on days 1-7. In the sick infants who needed immediate resuscitation at birth (n = 4-8), U-A1M was well as U-B2M was high on days 1-7 and then decreased to normal levels, suggesting that U-A1M can be used as a sensitive marker of acute proximal tubular damage and its recovery. These observations indicate that U-A1M is a useful index of proximal tubular function in early infancy.

Alpha-Globulins↗

The effects of biotin deficiency on organic acid metabolism: increase in propionyl coenzyme A-related organic acids in biotin-deficient rats.

Volatile organic acid levels in plasma and tissues and nonvolatile organic acid levels in urine of biotin-deficient (BD) rats were measured and compared with other factors of biotin deficiency. Biotin levels and the activities of propionyl coenzyme A (CoA) carboxylase (PCC) in the livers of these rats were decreased, respectively, to 22% +/- 3% and 3.6% +/- 0.3% of the average values of pair-fed controls. Plasma concentrations of propionate were higher (15 to 223 micrograms/mL) than those of controls (5 to 7 micrograms/mL), whereas plasma levels of 3-methylcrotonate were only minimally increased as compared with those of controls. Concentrations of these volatile acids in the tissues were similarly increased, although those in brain showed less remarkable increases as compared with levels in other tissues. In the urine of BD rats, large amounts of organic acids derived from propionyl CoA, as well as those from 3-methylcrotonyl CoA, were excreted. Plasma propionate levels were not apparently related to the severity of clinical symptoms, biotin levels, or carboxylase activities, but were related to the amounts of urinary ketone bodies, lactate, and some of the organic acids derived from branched-chain amino acids, including those from propionyl CoA.

Acyl Coenzyme A↗

Developmental features of the brain in preterm and fullterm infants on MR imaging.

Normal development and maturation in pre- and postnatal periods were studied using MR imaging. For this purpose, we performed MR imaging at a postmenstrual age of 37-44 weeks in both low-risk preterm and fullterm infants and assessed the myelination pattern and the dimension of the pituitary gland, corpus callosum, pons and cerebellar vermis. There were no differences in stages of myelination and the size of these structures in both groups. MR imaging was also performed in infants with various cerebral injuries. Focal or diffuse areas of hyperintensity on T2-weighted images and delayed myelination were the commonly observed abnormal findings in these infants.

Brain↗

Evaluation of proximal tubular function in preterm infants by urinary alpha 1-microglobulin.

alpha 1-Microglobulin is a low molecular weight protein that is relatively stable in urine of low pH. There have been few reports on urinary alpha 1-microglobulin (U-A1M) excretion in preterm infants. This study was designed to establish the ranges for U-A1M in clinically stable preterm infants and to investigate changes observed in sick preterm infants. We measured U-A1M and urinary beta 2-microglobulin (U-B2M) levels at 1, 4, 7, 14, 28 and 90 days after birth in stable preterm infants (Group 1) and sick preterm infants who were depressed at birth and required immediate resuscitation (Group 2). In Group 1 infants, both parameters were high during the first 28 days and appeared to decline thereafter. U-A1M in Group 2 infants was only significantly increased compared with Group 1 on day 1, as was U-B2M. On each day of the study, U-A1M had significant positive correlations with U-B2M for all the infants studied. The changes of the two parameters observed in Group 1 probably reflect postnatal evolution of proximal tubular function in stable preterm infants. A comparison of groups 1 and 2 shows a high prevalence of acute tubular injury at birth in sick infants and also suggests that U-A1M as well as U-B2M may be a sensitive index for detecting acute tubular damage and for following its course in preterm infants.

Age Factors↗

A long-term suppression by alpha-interferon of Philadelphia chromosome in children with chronic myelogenous leukemia.

An 8 year old girl with adult type Philadelphia (Ph1)-positive chronic myelogenous leukemia received natural alpha-interferon therapy in the chronic phase. Complete suppression of the Ph1 clone of bone marrow cells was achieved after 1 month of therapy, which was determined by disappearance of rearranged breakpoint cluster region (BCR) gene in Southern blot analysis. Complete hematological remission was also attained following 2 months of therapy. Both the suppression and the hematological remission have been sustained for 24 months with alpha-interferon, in spite of the detection of the chimeric BCR/ABL mRNA in her bone marrow by polymerase chain reaction assay 12 months after therapy.

Amino Acid Sequence↗

Urinary growth hormone excretion in preterm neonates.

We measured urinary growth hormone (U-GH), beta 2-microglobulin (U-B2) and serum growth hormone (S-GH) in preterm neonates on days 1, 4, 7, 14 and 28 of age. U-GH as well as U-B2 were high, particularly in the more premature and sick neonates with respiratory failure requiring mechanical ventilation. U-GH showed significant positive correlations with U-B2 throughout the study but with S-GH only on day 7. Therefore, we conclude that in preterm neonates, U-GH mainly reflects the degree of renal proximal tubular function, which is determined by the degree of renal maturation of the subject and of tubular injury due to disease states such as respiratory failure.

Aging↗

Measurement of lumbar spinal bone mineral density in preterm infants by dual-energy X-ray absorptiometry.

Lumbar spinal bone mineral density (BMD) was measured in 40 preterm infants by dual-energy X-ray absorptiometry (DXA). During the first several months of life, their BMD was considerably lower than that of normal term infants and the osteopenia was more pronounced in the more preterm and smaller infants. Weak (inverse) correlations were found between the BMD and urinary calcium/creatinine or tubular phosphorus reabsorption ratio. Rickets-like changes in the forearm bones did not predict the greater spinal osteopenia. Follow-up study was performed in 10 preterms. In 3 of the 4 who underwent the last DXA between 8 and 12 months, BMD had improved remarkably. Our present study shows the potential of DXA for the assessment and management of osteopenia of prematurity.

Absorptiometry, Photon↗

Human glioblastoma cells produce granulocyte-macrophage colony-stimulating factor in vitro, but not in vivo, without expressing its receptor.

Granulocyte-macrophage colony-stimulating factor (GM-CSF) production and receptor expression by human glioblastomas was studied. Enzyme-linked immunosorbent assay showed four of 10 glioblastoma cell lines spontaneously released GM-CSF (2.9-9.2 pg GM-CSF protein/ml culture medium), which was enhanced by stimulation with tumor necrosis factor-alpha (TNF) (10 U/ml) up to 410 pg/ml. TNF also induced secretion of GM-CSF by another cell line. Northern blot analysis identified increasing GM-CSF gene expression by cells following TNF stimulation. However, no GM-CSF protein was detectable in the cerebrospinal fluid of three malignant glioma patients. Intratumoral administration of TNF in the patients also failed to stimulate GM-CSF levels in the cerebrospinal fluid. A binding assay using flow cytometry with biotinylated GM-CSF and Scatchard analysis using 125I-labeled GM-CSF failed to demonstrate GM-CSF receptor expression on the 13 cell lines. Exogenous GM-CSF stimulation had no effect on production of prostaglandin E2, interleukin-6, or interleukin-8 by glioma cells. Human glioblastoma cells secrete GM-CSF without expressing the receptor in vitro, but there was no evidence of GM-CSF production in vivo.

Glioblastoma↗

Diagnosis of urinary tract infection by urine microscopy using a disposable counting chamber.

Routine urinalysis is performed as a screening test for urinary tract infection (UTI), but is not very reliable. We assessed the usefulness of microscopic examination of unspun urine using a disposable slide with counting chambers for the diagnosis of UTI caused by a variety of species of bacilli. One hundred and seventy-two urine samples were obtained from 113 subjects (60 male and 53 female), including 84 inpatients, aged 20-96 years. The urine samples were examined for bacteriuria and pyuria using a counting chamber, and the reliability of this method in predicting significant bacteriuria defined by routine urine culture and Gram stain of urine smears was analyzed. Significant bacteriuria was diagnosed in 68 urine samples, including 34 from indwelling catheters, from 52 patients mostly having underlying diseases. Only 12 of the positive urine samples contained E. coli, with a variety of other bacilli including cocci found in the rest. The counting chamber method detected bacteriuria in 64 of these 68 positive samples (sensitivity = 94%). Specificity was 88%. While the sensitivity and specificity of pyuria (WBC > 10 microliters-1) were 79 and 71%, respectively, both sensitivity and negative predictive value were as high as 97% when bacteriuria or pyuria was present. We demonstrated that urine microscopy on a disposable counting chamber is a simple, sensitive and time- and cost-saving method for the diagnosis of UTI caused by a variety of bacterial species including cocci.

Adult↗

Calcium channel current in cultured rat mesangial cells.

The presence of voltage-dependent calcium channels has been suggested in mesangial cells by using calcium-sensitive fluorescent probes. However, direct electrophysiological evidence for voltage-dependent calcium channels has not yet been presented. In this study voltage-dependent calcium channels were studied in cultured rat mesangial cells. Whole-cell patch-clamp experiments were done with 50 mM Ba2+ as a charge carrier. Step depolarizing pulses from a holding potential of -50 mV produced an inward barium current at potentials more positive than -10 mV, and a peak current (10-45 pA) was obtained at a membrane potential of approximately +30 mV. The inward current was augmented by 100 nM Bay K 8644, attenuated by 1 microM nifedipine, and abolished by 50 microM Cd2+. These results indicate that the inward current is a barium current flowing through L-type calcium channels. This may be the first study that demonstrates the presence of L-type calcium channels in mesangial cells.

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

[A case of hyperimmunoglobulin E syndrome with normal interferon-gamma production].

An 18-year-old male with a lifelong history of atopic dermatitis and recurrent severe Staphylococcus aureus abscesses was found to have hyperimmunoglobulinemia E. We measured IFN-gamma production by peripheral blood mononuclear cells cultured with a protein A non-producing strain of staphylococcal antigen and serum specific IgG subclass antibodies to this antigen in this patient. IFN-gamma production, when cultured with non protein A produced by this antigen in this patient was almost the same as that in healthy subjects. IFN gamma m-RNA was detected at the dose of 0.0625 micrograms total RNA. The levels of IgG1, IgG3 and IgG4 were higher in this patient than in healthy subjects, while IgG2 was lower. We thus identified a case of hyperimmunoglobulinemia E syndrome with normal IFN-gamma production.

Abscess↗

Autosomal dominant polycystic kidney disease in a child diagnosed by ultrasonography.

A 10-year-old boy with a chief complaint of nocturnal enuresis was identified as having three cysts in each kidney on ultrasonographic examination. These findings together with a positive family history for renal failure prompted us to examine his father ultrasonographically on the same day, resulting in the identification of multiple large cysts in both kidneys. These observations supported a diagnosis of autosomal dominant polycystic kidney disease. Enhanced X-ray CT demonstrated more sensitively the presence of renal cysts in the boy. He had a normal urinary concentrating ability and no other abnormalities except for mild hypertension, which was successfully treated only by salt restriction. Ultrasonography thus appears to be a valuable tool for diagnosing polycystic kidney diseases.

Child↗

Dual-energy X-ray absorptiometry in the lumbar spine, proximal femur and distal radius in children.

Dual-energy X-ray absorptiometry was used to measure bone mineral density (BMD) in the lumbar spine, proximal femur and distal radius in 48 Japanese children aged 3-18 years. In the normal children (n = 32), BMD increased with age in all locations, with a nearly twofold increase from preschool age to adolescence. Most of the children with chronic diseases known to affect bone metabolism (e.g., steroid osteoporosis) (n = 16) had low BMD in every region, indicating that these disease states probably affect multiple sites of the skeleton in children.

Absorptiometry, Photon↗

Urinary leukotriene E4 after exercise challenge in children with asthma.

To assess the role of sulfidopeptide leukotrienes in the pathogenesis of exercise-induced asthma (EIA), the urinary levels of leukotriene E4 (LTE4), a metabolite of LTC4 and LTD4, were measured by RIA before and after exercise in 13 children with EIA and 10 healthy children. Mass spectrometry was used to confirm the presence of LTE4 in urine and the specificity of the RIA. There was no significant difference in the urinary LTE4 levels before exercise between the children with asthma and healthy children (109 [21 to 265] versus 122 [45 to 156] pg/mg of creatinine; median and range). Urinary LTE4 levels increased significantly after exercise in the children with EIA (from 109 [21 to 265] to 196 [40 to 655] pg/mg of creatinine; median and range; p less than 0.05) but not in the healthy children. The children with asthma demonstrated no significant correlation between the LTE4 level after exercise and the degree of bronchoconstriction, as revealed by the maximal percent fall in the peak expiratory flow rate. Taken together with a recent study that pretreatment with a potent and selective LTD4 antagonist markedly attenuated EIA, our findings suggest that sulfidopeptide leukotrienes may play some role in the pathogenesis of this type of asthma with other factors also being involved in determining the overall airway response.

Asthma, Exercise-Induced↗