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Biomedical subjects

K Fujieda

Publications and source records attributed to K Fujieda.

At least 73 records · Page 4Linked to original sources
PubMed1994

Gestational age assessment in Japanese low birthweight infants.

We evaluated the validity of the Ballard scoring system for assessing gestational age in Japanese low birthweight infants. Infants included in this study were 116 neonates who were admitted to seven hospitals in Hokkaido. Gestational ages of all infants were prenatally established by ultrasonographic measurement of the crown-rump length performed between 8 and 12 weeks of gestation (clinical age). Of these, 74 infants were appropriate for gestational age (AGA) and the remaining were small for gestational age (SGA). Mean birthweight was 1703 g and 34% (39/116) were < 1500 g. The correlation coefficient for the total population was 0.93 (P < 0.001). Although mean Ballard age tended to overestimate clinical age, mean differences between Ballard age and clinical age did not exceed 0.6 weeks. Agreement within 2 weeks among all infants was 86.2%. Percentages of agreement within 2 weeks did not differ to a statistically significant degree between AGA and SGA. These results indicate that the Ballard method is of value in assessing gestational age in Japanese low birthweight infants.

Evaluation Studies as Topic↗
PubMed1994

[Characteristics of myocardial diseases in the elderly].

Myocardial diseases consist of cardiomyopathy of unknown origin and specific myocardial diseases of known origin. The former consists mainly of dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). In the latter, cardiac amyloidosis may be most frequently seen in the elderly. One hundred and twenty patients with cardiomyopathy were studied concerning their clinical courses. They were divided into 2 groups; i) young-middle-aged (Y) and ii) elderly (0). Group 1 was divided into 2 subgroups: 1a) followed up to an age less than 60 years old, and 1b) followed up to beyond age 60. In DCM, left ventricular posterior wall thickness and left atrial diameter increased significantly in the elderly. In HCM, young patients had obstructive type disease more frequently than the elderly. A history of mild hypertension was found more frequently in the middle-aged or elderly than in the young. Left ventricular end-diastolic diameter increased and left ventricular wall thickness decreased significantly in the elderly. Many patients with DCM usually die of congestive heart failure with ventricular arrhythmia, and those with HCM, both young or middle-aged, often die suddenly during sports activity. If there is an adaptive system, such as increased wall thickness in DCM or decreased wall thickness and increased diameter in HCM, which may contribute to the normalization of left ventricular wall stress, the patients might be able to survive to old age.

Adult↗
PubMed1993

Molecular analysis of patient and carrier genes with congenital steroid 21-hydroxylase deficiency by using polymerase chain reaction and single strand conformation polymorphism.

Steroid 21-hydroxylase deficiency is a major cause of congenital adrenal hyperplasia and is caused by genetic impairment of this enzyme. Since approximately 80% of cases are caused by point mutations of the CYP21B (CYP21A2) gene, whereas the remaining 20% are due to deletion of this gene, we used the polymerase chain reaction single strand conformation polymorphism technique for rapid and accurate diagnosis of this disease. Of 23 patients examined, 1 had a hemizygous CYP21B gene. 18 patient's genes localized their harmful mutations or deletion on both the alleles, while 4 of them found their causative mutations on one of the two alleles, and 1 failed to find any responsible mutation. All the mutations (four nucleotide substitutions) detected are also found in the CYP21A (CYP21A1) pseudogene. A mutation at the intron 2 site is most prevalent in both salt-wasting and simple virilizing forms of the disease, and accounts for 37% of the patient's genes (17/46). Pedigree analysis of these mutations revealed that the mutations (at least four of them) occurred de novo at a considerable frequency on both the paternally and maternally inherited chromosomes. This result could explain occasional discordance of the diagnosis using HLA typing with the clinical symptoms.

Adrenal Hyperplasia, Congenital↗
PubMed1993

de novo mutation causes steroid 21-hydroxylase deficiency in one family of HLA-identical affected and unaffected siblings.

Over 90% of congenital adrenal hyperplasia (CAH) results from 21-hydroxylase deficiency. Because the CYP21B gene is located within the HLA complex and is very tightly linked to HLA markers, HLA typing is widely used for prenatal diagnosis and identifying heterozygous family members. In the course of a study on identification of heterozygous family members with HLA typing, we recognized an unusual family case in which three siblings share the same HLA haplotype, and only one of them had the simple virilizing form; her two siblings did not have any endocrinological abnormalities. We investigated the mode of genetic transmission by using polymerase chain reaction and single stranded conformation polymorphism. The present study revealed that the proband was a compound heterozygote with the intron 2 mutation that causes aberrant RNA splicing and the missense mutation of exon 4, while the other siblings and the father had only one allele of a missense mutation in exon 4; the mother is a normal homozygote. This result together with DNA fingerprint analysis strongly suggest that the intron 2 mutation occurred de novo in the maternally inherited gene of the proband. This seems to be the first case of a de novo mutation of the CYP21B gene that causes CAH.

Adrenal Hyperplasia, Congenital↗
PubMed1993

Human corticotropin-releasing hormone (hCRH) test: sex and age differences in plasma ACTH and cortisol responses and their reproducibility in healthy adults.

The effectiveness and safety of MCI-028, a synthetic human corticotropin-releasing hormone (hCRH), as a diagnostic drug were examined in 65 healthy male and 24 healthy female adult volunteers. Mean maximum concentrations of plasma ACTH and cortisol after intravenous administration of 100 micrograms of MCI-028 were 3.0 and 2.0 times their basal concentrations, respectively, and there were no significant age or sex differences in the responses. Good reproducibility was observed in the responses in 59 male subjects who received a second administration after 1 to 2 weeks. Although slight adverse reactions such as mild and transient hot flushing were observed, these were not serious.

Adrenal Cortex Function Tests↗
PubMed1993

Evaluation of hypothalamo-pituitary-adrenocortical function in children by human corticotropin-releasing hormone (MCI-028) test.

A dose of 1.5 micrograms/kg of MCI-028, human corticotropin-releasing hormone (hCRH), was administered intravenously to 38 children with non-endocrine short stature with normal function in the hypothalamo-pituitary-adrenocortical axis and to 71 children with a disorder in the same axis. Blood levels of adrenocorticotropic hormone (ACTH) and cortisol were determined to evaluate the axis. The 95% confidence limits of peak responses of ACTH and cortisol in non-endocrine short stature were between 17.2 and 135.3 pg/ml, and between 13.1 and 35.6 micrograms/dl, respectively, and were used as standards for children. When compared with these standards, the hormonal responses in children with various disorders in the hypothalamo-pituitary-adrenocortical axis were as follows: in two children with Cushing's syndrome caused by adrenal tumor, ACTH values were decreased and were not responsive to hCRH, while cortisol values, though within the normal limit, were not responsive; in children with primary adrenal insufficiency or congenital adrenal hyperplasia, cortisol values were decreased and not responsive, whereas ACTH values tended to be increased and ACTH response high except for 21 alpha-hydroxylase deficiency of congenital adrenal hyperplasia. In two cases of pituitary dwarfism complicated with ACTH deficiency, both ACTH and cortisol values were decreased and poorly responsive; and in children who were receiving glucocorticoid, both ACTH and cortisol values tended to be decreased and to respond poorly to hCRH. As for side effects, hot flushing was observed among 8.0% of the subjects after administration of hCRH. But this symptom was not severe and no other side effects of clinical importance were observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Endocrine functions in children with suprasellar germinoma.

Clinical manifestations and endocrine functions were evaluated in 17 children with suprasellar germinoma. Polyuria and growth retardation were the most common initial symptoms. Physical and neurological examinations revealed diabetes insipidus in 17, growth retardation in five, and visual disturbance in three of the patients. Assessment of hypothalamic-pituitary function before treatment revealed pituitary deficits in all patients involving growth hormone, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone, and adrenocorticotropic hormone. These pituitary dysfunctions persisted after tumor remission due to radiation therapy. These results indicate that children with suprasellar germinoma need long-term hormone replacement therapy to prevent growth retardation, thyroid dysfunction, and delayed secondary sexual development.

Adolescent↗
PubMed1993

Capillarization and venularization of hepatic sinusoids in porcine serum-induced rat liver fibrosis: a mechanism to maintain liver blood flow.

The processes of capillarization and venularization of sinusoids after porcine serum-induced rat liver fibrosis were studied by light and electron microscopy. Accompanying the development of fibrosis in the walls of central veins, most of the sinusoidal outlets collapsed, resulting in the formation of hepatic limiting plates around central veins. A few remaining sinusoids underwent capillarization (the development of a basal lamina and the defenestration of the sinusoidal endothelial cell), followed by venularization (the transformation into venules of sinusoids, characterized by the enlargement of the diameters with the lumina being lined with several endothelial cells, which lose fenestrae and develop a basal lamina). These newly formed venules served to maintain blood flow from sinusoids into central veins and thus have been designated the "outlet venules." Diameters of these venules could reach about 25 microns. They were classified into two types: (a) the septal outlet venules, which developed inside the septa; and (b) the angular outlet venules, which drained blood directly from the parenchyma into the fibrotic central veins at the angles between two septa. Associated with venularization, perisinusoidal stellate cells (fat-storing cells or Ito cells) differentiated to myofibroblasts.

Animals↗
PubMed1993

[Measurement of bone mineral density using dual energy X-ray absorptiometry in asthmatic patients receiving prednisolone therapy].

Bone mineral density (BMD) of the lumbar spine (L2-L4) was measured using dual-energy X-ray absorptiometry (DEXA), and its relationship to total dose of prednisolone and duration of prednisolone therapy was studied in 57 patients with bronchial asthma. There was a significant negative correlation between BMD and total dose of prednisolone (r = -0.463, p < 0.001) and between BMD and duration of prednisolone treatment (r = -0.30, p < 0.05). The half-yearly percent decrease of BMD measured in 17 asthmatic patients was 0.83% (p < 0.01) after correction for age-associated decline. These findings suggest that the reduction of BMD was related to the total dose of prednisolone and the duration of therapy in asthmatics.

Absorptiometry, Photon↗
PubMed1992

A symptomatic female patient with Duchenne muscular dystrophy diagnosed by dystrophin-staining: a case report.

We report a case of symptomatic carrier of Duchenne muscular dystrophy (DMD) in a 14-year-old girl with no prior family history of DMD. She presented with chest pain, enlarged calf muscles, an elevated serum creatine kinase (CK), and decreased left ventricular function. Histological examination of skeletal muscle revealed myopathic changes and immunostaining with anti-dystrophin antiserum demonstrated a mosaic pattern which are compatible with the observations in carriers of DMD. Southern blots using the dystrophin cDNA revealed no evidence of a deletion within the DMD gene in the patient or in her mother. We found this observation interesting for two reasons: 1. Cardiomyopathy is rare in female DMD patients. 2. Immunostaining of a muscle biopsy with anti-dystrophin serum proved to be valuable in the diagnosed for symptomatic carriers of the dystrophin gene mutation.

Adolescent↗
PubMed1991

Molecular cloning and mapping of 10 new probes on the human Y chromosome.

We have developed a novel positive cloning vector whose use precludes the cloning of any fragments less than 0.8 kb as well as 3.4-kb EcoRI fragments of DYZ1, the largest repeating-DNA family on the long arm of the human Y chromosome. Using this vector, we subcloned inserts of a Y-chromosome-specific phage library constructed from EcoRI-digested flow-sorted Y-chromosome DNA. Ten novel Y-specific fragments were obtained. Their localization on the Y chromosome was determined by deletion mapping using clinical samples with structurally abnormal Y chromosomes. The long arm of the Y chromosome was divided into 12 segments by the novel probes in combination with established probes. The amelogenin-like sequence, mapped on the long arm in Human Gene Mapping 10, has been mapped on the short arm.

Amelogenin↗
PubMed1991

Age-related change of pulsatile gonadotropin secretion in Turner syndrome.

In an attempt to understand the dynamic change of the gonadotropin-releasing hormone-pituitary axis during the transitional stage from prepuberty to puberty, we investigated gonadotropin secretory patterns using a highly sensitive assay system and frequent blood sampling technique in children with Turner syndrome aged 5-17 y. Blood samples were collected every 20 min for 24 h in 16 cases, or every 30 min for 9 h (daytime 5 h, nighttime 4 h) in nine cases. Serum LH and FSH were measured by time-resolved fluoroimmunoassay. A 24-h profile of LH and FSH was analyzed by a computerized pulse detection program (PC-PULSAR). As early as 5 to 6 y of age, mean daytime LH concentration was significantly higher than nighttime concentration and pulsatile LH secretion existed throughout the day and night. At about 9 to 11 y of age, corresponding to the early stage of puberty, a dramatic increase in LH concentration and amplitude was observed, and both concentration and pulse amplitude were much higher during the night than during the day. However, these day-night differences became less clear at ages corresponding to late pubertal stages. Pulse frequency of LH secretion remained almost constant throughout the day and night at all ages investigated. As for FSH concentration, a trend similar to that of LH was observed, although day-night differences and age-related changes were less remarkable. Furthermore, pulsatile FSH secretion was detected in only a small number of the cases. These findings suggest that in Turner syndrome the hypothalamic gonadotropin-releasing hormone oscillator is functioning actively with constant frequency before the onset of puberty.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Comparison of atrophic and goitrous auto-immune thyroiditis in children: clinical, laboratory and TSH-receptor antibody studies.

We studied the clinical features, laboratory and thyroid functions and thyrotropin (TSH)-receptor and thyroid-stimulation antibodies in 21 patients with atrophic auto-immune thyroiditis (AAT) and 48 patients with goitrous auto-immune thyroiditis (GAT) of childhood onset. The clinical features of patient with AAT were cessation of growth and obesity, while asymptomatic enlargement of the thyroid gland was the sole symptom in most patients with GAT. Although the ages at diagnosis were comparable in both groups, the estimated ages at onset were much lower in patients with AAT than in those with GAT. Patients with AAT exhibited more severe hypothyroidism when evaluated by serum thyroxine (T4), tri-iodothyronine (T3), TSH, cholesterol levels and basal metabolic rates. The 24 h 123I-thyroidal uptake was significantly lower in patients with AAT than in those with GAT. None of the 19 patients with AAT possessed TSH-binding inhibitor immunoglobulins (TBII). On the other hand, 3 of the 32 GAT patients tested, possessed weak to potent TBII activities. Three TBII-positive patients with GAT also possessed thyroid-stimulation blocking antibodies. These findings suggest that: 1. Pathogenesis of AAT in children whose onset of hypothyroidism was before puberty is not due to TSH-receptor blocking antibodies, which are often found in patients with AAT of postpubertal onset. 2. AAT in children is considered not to be due to the later stage of GAT. 3. Some patients with GAT possessed TSH-receptor blocking antibodies. The aetiology and pathogenesis of AAT in children have yet to be elucidated.

Adult↗
PubMed1990

[A study on sex chromosome mosaicism--endocrinological and immunohistochemical findings with glutathion S-transferase in the gonads].

In order to clarify the clinical aspects of sex chromosomal mosaicism, we evaluated the karyotypes, the anatomy of external genitalia and internal ductal system, the pituitary-gonadal function, and the histopathology of the gonads by immuno-staining for glutathion S-transferase (GST) in 5 patients who had been all raised as female. Three patients have the 45, X/46, XYq- karyotype in the initial lymphocyte culture or the subsequent culture of skin fibroblasts. Another two karyotypes were 45, X/46, XYq-/47, XYq-, Yq- and 45, X/46, XdicY. Thus, Y chromosome of all patients retained short arms in which the testis determining factor is encoded. Three prepubertal patients were referred to us for their ambigious external genitalia and two postpubertal patients were for the short statures. Although the vaginal orifice was separated from the urethral meatus in all of them, the phallic enlargement was noted in 4 patients and the posterior labial fusion in 2 patients. The oldest patient had a normal female appearance of external genitalia except the vaginal septum. Serum gonadotrophin (GnH) levels were basically high in the postopubertal patients and the responses of GnH to LH-RH were significantly increased in the prepubertal patients. Serum testosterone levels to hCG stimulation ranged from no response to low normal response. All patients underwent the exploratory laparotomy together with the feminizing genitoplasty. The gonads in 3 patients, diagnosed as mixed gonadal dysgenesis (MGD), consisted of a unilateral testis and a contralateral streak gonad. Two patients had variants, including one with bilateral dysgenetic testis and another with bilateral streak gonads.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

[Diaphragmatic fatigue under inspiratory resistive load in healthy subjects and the effect of several selections of frequency bands on the H/L (high/low) ratio].

In seven normal adult males the authors studied diaphragmatic fatigue by analysis of the power spectrum of the diaphragmatic electromyogram obtained with an esophageal electrode, and of the change of transdiaphragmatic pressure. Diaphragmatic fatigue was produced by inspiring through a resistive load, and mean value of the index of the tension time of the diaphragm (TTdi) maintained until fatigue was 0.33 +/- 0.09, which has been known to produce fatigue. The mean value of ratios of power at high frequencies to power at low frequencies (H/L) decreased to 61.2 +/- 16.8% of the initial value at just before breaking time. Breaking time was 4.88 +/- 1.37 minutes in average. The selection of low and high band widths for analysis of H/L ratio has differed slightly in the literature, but such differences do not have any substantial influence on the estimation of the changes of H/L ratio.

Adult↗
PubMed1990

Diastolic properties of hypertrophied hearts in essential hypertension: classification by left ventricular wall stress.

Hypertensive cardiac hypertrophy of 20 patients was classified as inappropriate hypertrophy (HH-I) and appropriate hypertrophy (HH-II) according to their end-systolic wall stress, as measured by echocardiography. The differences in systolic and diastolic performances among the HH-I and HH-II subjects and 10 normal controls (NC) before and during isoproterenol infusion were investigated. Eight patients had subnormal end-systolic wall stress (inappropriate hypertrophy) and 12, normal end-systolic wall stress (appropriate hypertrophy). Before isoproterenol infusion, normalized peak rate of a change in left ventricular diameter during systole was significantly greater in HH-I (3.5 +/- 0.8/s) than in NC (2.3 +/- 0.5/s) and HH-II (2.6 +/- 0.6/s) (p less than 0.01 and p less than 0.005), but there was no significant difference between HH-II and NC. There was no significant difference in normalized peak rate of a change of left ventricular diameter during the rapid filling phase among the three groups (4.5 +/- 1.2/s in HH-I, 4.0 +/- 1.6/s in HH-II, and 4.2 +/- 0.8/s in NC). During isoproterenol infusion, normalized peak rate of a change of left ventricular diameter during systole was significantly greater in HH-I (7.0 +/- 1.9/s) than in HH-II (4.8 +/- 1.7/s) and NC (4.8 +/- 0.8/s) (p less than 0.05 and p less than 0.01, respectively), but there was no significant difference between HH-II and NC. Normalized peak rate of a change of left ventricular diameter during rapid filling was significantly less in HH-II (4.8 +/- 1.7/s) than in HH-I (7.3 +/- 1.3/s) and NC (6.5 +/- 0.8/s) (p less than 0.005 and p less than 0.005, respectively), but there was no significant difference between HH-I and NC. These results suggest that hypertensive patients with inappropriate hypertrophy have relatively diminished diastolic velocity (supernormal systolic velocity and normal diastolic velocity) before and during isoproterenol infusion, and that hypertensive patients with appropriate hypertrophy have absolutely diminished diastolic velocity during isoproterenol infusion, in spite of normal diastolic velocity before the infusion.

Adult↗
PubMed1989

Hyperglucagonemia of insulin autoimmune syndrome induced by methimazole in a patient with Graves' disease.

A 47-year-old man with Graves' disease suffered from a feeling of hunger and sweating in the night, polyarthralgia and fever one month after the start of treatment with methimazole. The above symptoms were ascribed to the side effects of methimazole; insulin autoimmune syndrome and lupus-like syndrome. The change in the antithyroid drug to propylthiouracil caused an amelioration of the symptoms. In addition to an anti-insulin antibody with a high binding capacity, hyperglucagonemia (260 pg/ml with a plasma glucose level of 61 mg/dl) was observed, which returned to normal in parallel with the decrease in the insulin binding capacity of the plasma one month after beginning the treatment with propylthiouracil. A normal decrease in the plasma glucagon level due to exogenous insulin (2 mU/kg/min) was observed with the euglycemic clamp. However, the plasma glucagon level was not suppressed by the oral glucose loading and elicited a poor response to the arginine infusion. Taking previous reports into account, this basal hyperglucagonemia seems to be a characteristic finding in the insulin autoimmune syndrome, while a sluggish response of glucagon to oral glucose or arginine infusion might be ascribed to hyperthyroidism. This is the first case report concerning a kinetical study of the glucagon secretion in insulin autoimmune syndrome with Graves' disease.

Arginine↗