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Biomedical subjects

M Sudo

Publications and source records attributed to M Sudo.

At least 127 records · Page 7Linked to original sources

Scaphoid megalourethra with multiple urogenital anomalies.

A male infant with scaphoid megalourethra, which is a rare congenital anomaly, is presented. At birth he exhibited a grossly swollen, malformed penis and cryptorchidism. The corpus spongiosum was not palpable. The infant had congenital renal failure. Retrograde urethrography revealed a crescent-shaped dilatation of the anterior urethra, and the diagnosis of scaphoid megalourethra was made. Subsequent uroradiological examinations showed that there were also severe urogenital anomalies, i.e. renal hypoplasia-dysplasia, hydronephrosis, hydroureter and vesicoureteral reflux. This disorder is frequently associated with other urogenital anomalies. Therefore, once the diagnosis is reached, a careful investigation for other associated abnormalities should be undertaken.

Abnormalities, Multiple↗

Uncooked cornstarch treatment for hepatic phosphorylase kinase deficiency.

UNLABELLED: A 5-year-old boy with short stature, hepatomegaly and motor weakness due to hepatic phosphorylase kinase deficiency is described. Laboratory data showed mild hypoglycaemia and metabolic acidosis, hepatic dysfunction, and a low insulin-like growth factor-I level. Mild hypoglycaemia, marked ketosis and insufficient growth hormone secretion were revealed at night. Serum total and free carnitine levels were low and the acyl/total carnitine ratio was high. Urinary acylcarnitine profile using fast atom bombardment and tandem mass spectrometry showed increased excretion of acetylcarnitine and dicarboxylylcarnitines. These endocrinological and metabolic abnormalities and clinical symptoms were improved with uncooked cornstarch treatment. CONCLUSION: Uncooked cornstarch treatment may be helpful in hepatic phosphorylase deficiency.

Child↗

Corpus callosum development in preterm and term infants.

The development of the corpus callosum was documented by magnetic resonance imaging in low-risk preterm infants and results were compared with those of term infants. Thirty-three magnetic resonance imaging studies were performed on 21 preterm infants whose corrected ages were 1-13 months. Twenty-one magnetic resonance imaging studies were performed on 17 term infants. All infants exhibited normal development. The thickness of the corpus callosum was measured at a point one-third of the length of the entire corpus callosum from the most anterior aspect of the genu. The thickening of the corpus callosum was related to age in the preterm group. The development of the corpus callosum in preterm infants was suggested to be the same as that of term infants matched by conceptional age.

Birth Weight↗

Corpus callosum in developmentally retarded infants.

The development of the corpus callosum was examined by magnetic resonance imaging in developmentally retarded infants ranging in age from 1-13 months. Results were compared with those of normal infants. Eighteen magnetic resonance imaging studies were performed on 18 developmentally retarded infants. Fifty-four magnetic resonance imaging studies were performed on 38 normally developed infants. The thickness of the corpus callosum was measured at a point one-third of the length of the entire corpus callosum from the most anterior aspect of the genu. The development of the corpus callosum was related to aging in both groups. There was significant difference in the thickening of the corpus callosum between normal and developmentally retarded infants.

Birth Weight↗

Urinary N-acetyl-beta-D-glucosaminidase excretion in term and preterm neonates.

Urinary N-acetyl-beta-D-glucosaminidase (NAG) excretion was measured in term and preterm neonates on days 1, 4, 7, 14 and 28 of life. Urinary NAG showed a peak level on day 4 or 7 in these infants. In addition, it tended to be higher with the degree of prematurity. In sick preterms who were depressed at birth and had respiratory failure, the NAG activity was further elevated during the first 2 weeks, suggesting the presence of renal tubular injury in this period. These observations thus suggest that urinary NAG may be a sensitive measure of renal maturation or damage in neonates.

Acetylglucosaminidase↗

Rapid dipstick test for diagnosis of urinary tract infection.

The rapid dipstick test of urine leukocyte esterase (LE) activity and nitrite has not been studied fully in pediatric clinical situations. We investigated the usefulness of the dipstick LE and nitrite test in the screening of urinary tract infection (UTI) in pediatric patients. Ninety-two fresh urine samples were obtained from children suspected of having UTI. Leukocyte esterase activity and nitrite were measured in the urine specimens read by a photometer. Leukocytes were also counted on a disposable slide. Urine samples were examined for bacteriuria by the standard culture method. The results of the urine dipstick test of LE showed a close relationship with leukocyte counts on a counting chamber. Leukocyte esterase (-) indicated leukocyte counts of less than 10/uL with a probability of 97% (58/60). Of the 22 urine samples with significant bacteriuria diagnosed by standard urine culture, the nitrite test did not detect bacteriuria in 10. While the sensitivities of the dipstick tests of nitrite (+) and LE +/- or more for the diagnosis of significant bacteriuria were 55% (12/22) and 86% (19/22), respectively, the sensitivity and negative predictive value of the combined test were 100%. These results suggest that use of the dipstick test of LE and nitrite can avoid a large part of the cost incurred by urine culture and is useful for screening UTI in children.

Adolescent↗

A case of familial thyroxine binding globulin excess associated with growth hormone deficiency.

A 7 years 3 months old Japanese boy with familial thyroxine binding globulin (TBG) excess associated with growth hormone (GH) deficiency is reported. The patients height was 106.4 cm (-2.86 s.d.) and his bone age was 5 years and 3 months. He had no goiter and his developmental milestones were normal. The serum thyroid stimulating hormone (TSH) was 2.8 microU/mL, triiodothyronine (T3) 3.1 ng/mL, thyroxine (T4) 23.4 micrograms/dL and free T4 1.8 ng/dL. The serum TBG level was beyond 80.0 micrograms/mL, with normal TSH response to the thyrotropin-releasing hormone (TRH) test. Familial study revealed that his grandmother, mother, uncle, younger sister and younger brother had high TBG and T3 levels, thus an X-linked co-dominant transmission was suggested. The peak GH responses to insulin and clonidine hydrochloride were 5.8 and 8.2 ng/mL, respectively. The mean nocturnal GH concentration was 2.5 ng/mL. His growth velocity increased from 4.8 to 8.4 cm/year and his serum TBG levels decreased gradually after human growth hormone (hGH) treatment.

Body Height↗

Development of posture in prone and supine positions during the prenatal period in low risk preterm infants.

The development of posture in the prone and supine positions was studied longitudinally in 10 low risk preterm infants between 31 and 39 weeks' postmenstrual age whose subsequent neurological development at 18 months old was normal. The infants were observed from one week after birth until the expected date of delivery. All postures were analysed according to the procedures described by Prechtl et al. There were large intraindividual and interindividual differences in posture in terms of the duration and incidence of the two positions. There was no age specific preferred posture found in either position. The posture duration in the prone position was longer than that in the supine position, but overall flexed and abducted posture were observed more often in the former. Turning of the head to the right side was also observed more often in the prone than in the supine position.

Female↗

Attenuation of sodium nitroprusside responses after prolonged incubation of rat aorta with endotoxin.

We investigated the effects of prolonged treatment with Escherichia coli lipopolysaccharide (LPS) on the responses to sodium nitroprusside (SNP) in endothelium-denuded rat aortic strips. Incubation of the aortic strips with LPS for 24 h dramatically attenuated relaxation and guanosine 3',5'-cyclic monophosphate (cGMP) formation by SNP, which were significantly restored by the inhibition of nitric oxide (NO) production with N omega-nitro-L-arginine. In the aorta coincubated with LPS and protein synthesis inhibitor (dexamethasone or cycloheximide, which prevents induction of endotoxin-inducible NO synthase), no attenuation of the relaxation was observed and the cGMP formation was significantly restored. Relaxation response to 8-bromo-cGMP or papaverine was not attenuated, even after 24 h of incubation. These results suggest that the attenuation of SNP responses is mainly associated with a decrease in the activation of guanylate cyclase (GC) as a consequence of the prolonged exposure to muscle-derived NO. Moreover SNP in the presence of methylene blue evoked a small but apparent relaxation of 24-h-incubated aorta without significant elevation of cGMP, suggesting the involvement of cGMP-independent pathways in the remaining relaxation produced by SNP.

Amino Acid Oxidoreductases↗

Renal handling of albumin and beta-2-microglobulin in neonates.

Urinary albumin and beta 2-microglobulin (B2M) were measured during the neonatal period. Urinary albumin decreased postnatally in term neonates, while it remained almost constant in preterm neonates. Urinary B2M showed a peak level on day 7 both in term and preterm neonates. There was some trend towards higher levels of albumin and B2M with decreasing gestation, showing that glomerular permeability increases and proximal tubular protein reabsorption decreases with increasing degrees of prematurity. In sick preterms who were depressed at birth and had respiratory failure, both parameters were elevated during the first 2 weeks, indicating the presence of glomerular and tubular damage in this period. The changes in B2M with gestation or clinical condition were more pronounced than those in albumin.

Age Factors↗

Antiproliferative effect of tumor necrosis factor-alpha on human glioblastoma cells linked with cell cycle arrest in G1 phase.

The effects of tumor necrosis factor-alpha (TNF) on proliferation and cell cycle alterations in human malignant glioma cell lines, SF-188 and LN-382, were investigated by flow cytometry with the bromodeoxyuridine-propidium iodide dual staining technique. Low concentrations of TNF (1-100 U/ml) suppressed the growth of SF-188 assessed by cell count, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide assay, and thymidine incorporation assay, but not that of LN-382. After TNF treatment, the percentage of SF-188 cells in the G0/G1 phase increased, while the percentage of cells in the S phase decreased. LN-382 cells did not show any marked change in cell kinetics. TNF arrests certain human glioma cells in the G0/G1 phase resulting in reduction of deoxyribonucleic acid synthesis in the subsequent S phase, suppressing the proliferation pathway.

Brain Neoplasms↗

Abnormal fatty acid composition of lymphocytes of biotin-deficient rats.

The relative proportions (% of total fatty acids) of odd-chain (15:0-29:0) and long-chain (22:0-30:0) saturated fatty acids in phospholipids of biotin-deficient rat lymphocytes were significantly increased as compared with biotin-supplemented rats, and the ratio of unsaturated fatty acids to saturated fatty acids in the former was significantly decreased mainly due to the reduced composition of polyunsaturated fatty acids in the omega-3, omega-6, and omega-9 pathway. The ratio of cis-vaccenic acid to palmitoleic acid in biotin-deficient rats was significantly lower than that in control rats, and was thought to be another important, but previously unreported indicator of biotin deficiency. These changes imply that the elongation and desaturation of unsaturated fatty acids are depressed in lymphocytes of biotin-deficient rats, and may contribute to the associated immunological dysfunction in biotin deficiency through abnormal prostaglandin metabolism and/or cell membrane functions.

Animals↗

Low serum aminotransferase activity in patients undergoing regular hemodialysis.

Of 150 patients undergoing regular hemodialysis (HD), 14 (9.3%) and 12 (8.0%), respectively, showed low serum activity of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). An investigation was conducted to elucidate the underlying mechanisms in 20 patients with low serum AST and/or ALT activity. Fifty-five percent of the patients with low aminotransferase activity manifested serum levels of pyridoxal phosphate (PLP) that were lower than normal. Serum PLP levels correlated neither with AST nor with ALT activity. Oral administration of vitamin B6 to the cases with low aminotransferase activity resulted in an increase in pre-hemodialysis aminotransferase activity. Addition of vitamin B6 in vitro to the sera from the patients with low aminotransferase activity did not increase the values when the added vitamin B6 was within the physiological range, but did increase when added in larger (pharmacological) amounts. However, aminotransferase activity increased, but PLP levels remained unchanged when these values were compared before and after HD. On the other hand, guanase being within the normal range in all cases studied, did not change after HD. Although our study does not correlate with vitamin B6 deficiency, but rather with some uremic substance(s) which interfere(s) with the enzyme reaction as a cause of low aminotransferase activity, the fact that less than 10% of our patients showed low AST and/or ALT points to the latter possibility, suggesting the need of further study.

Adult↗

Morphological characterization of glomerular endothelial cells cultured on a basement membrane matrix.

We characterized the morphological expression of glomerular endothelial cells (GEC) cultured on a basement membrane matrix, Matrigel. GEC cultured on this matrix formed abundant networks of branching and anastomosing cords of cells, and formed capillary-like structures with a lumen and interdigitating junctional processes. In contrast, GEC cultured on fibronectin-coated dishes had elongated spindle shapes without any interdigitating junctional processes. These findings suggest that the culture system with Matrigel is useful for inducing and maintaining characteristics close to in vivo GEC.

Animals↗

Detection of heterozygotes for fructose-1,6-diphosphatase deficiency by measuring fructose-1,6-diphosphatase activity in monocytes cultured with calcitriol.

The increase of fructose-1,6-diphosphatase activity during culture with calcitriol, which was reported in monocytes, was found not to occur in lymphocytes. Monocytes cultured with calcitriol were accordingly used as more reliable diagnosis of heterozygotes for fructose-1,6-diphosphatase deficiency, instead of mononuclear cells (lymphocyte-fraction-containing monocytes) cultured without calcitriol by a conventional method. Variation of fructose-1,6-diphosphatase values in leukocytes from nine healthy adults was smallest in monocytes cultured with calcitriol, among four different experimental conditions: monocytes cultured with or without calcitriol and mononuclear cells cultured with or without calcitriol. Both parents of two sisters with fructose-1,6-diphosphatase deficiency were successfully confirmed as carriers of fructose-1,6-diphosphatase deficiency by this method. However, confirmation by the conventional method using mononuclear cells cultured without calcitriol was possible only in the father, not in the mother. Thus, the new method using monocytes cultured with calcitriol seems more reliable for detecting heterozygotes for fructose-1,6-diphosphatase deficiency.

Calcitriol↗