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Biomedical subjects

O Nose

Publications and source records attributed to O Nose.

At least 37 records · Page 2Linked to original sources

Transient infantile hyperthyrotrophinaemia.

Sixteen cases of transient infantile hyperthyrotrophinaemia were followed up for two to seven years. Concentrations of serum triiodothyronine, thyroxine, and free thyroxine were maintained within the normal range in all cases. All but one child, who had a hearing disturbance, showed normal mental development with normal physical and skeletal maturation. Eleven children had normal concentrations of serum thyroid stimulating hormone and no signs or symptoms of thyroid dysfunction; in three children, diffuse small goitres developed and two further children showed relapse with slightly raised concentrations of thyroid stimulating hormone. It is concluded that 'transient infantile hyperthyrotrophinaemia' is a syndrome, which differs from typical transient neonatal hypothyroidism, and that careful follow up is necessary because some children show signs of mild pituitary-thyroid dysfunction in later childhood.

Child Development↗

Multiple associated endocrine abnormalities in a patient with pseudohypoparathyroidism type 1a.

A girl with type 1a pseudohypoparathyroidism (PHP) presented several hormonal abnormalities. Although she had eluded neonatal thyroid screening, she was diagnosed as having hypothyroidism at the age of 5 months. Thereafter, a diagnosis of PHP was made on the basis of skeletal features of Albright osteodystrophy and lack of both cyclic adenosine monophosphate (c-AMP) and phosphaturic responses after parathyroid hormone (PTH) infusion. The basal levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) were higher than normal and showed exaggerated responses to luteinizing hormone-releasing hormone (LH-RH). There was no growth hormone (GH) response to arginine infusion, and the prolactin (PRL) response after thyrotropin-releasing hormone (TRH) infusion, was also impaired. The stimulating guanine nucleotide-binding protein (Ns) activity of the erythrocytes was reduced to 66.9%. The skeletal age was not delayed at the age of 5 months in spite of the hypothyroid state, and it advanced following thyroxine and vitamin D treatments.

Age Determination by Skeleton↗

Universal predictive criteria for neonatal overt thyrotoxicosis requiring treatment.

The universal predictive criteria for neonatal overt thyrotoxicosis requiring treatment were examined in 108 neonates (including a pair of twins) born to mothers with Graves' disease (36 patients under treatment with antithyroid drugs [group A] and 71 in remission [group B]). Anti-thyroid-stimulating hormone (TSH) receptor antibody activity was measured by both radioreceptor assay (TSH-binding inhibitor immunoglobulin [TBII]) and biologic stimulation assay (thyroid stimulating antibody [TSAb]). For generalization of the predictive criteria, the expression of TBII activity was standardized using standard serum made taking units of Medical Research Council long-acting thyroid stimulator, standard B as a reference, and expression of TSAb activity was standardized using bovine TSH as a standard. TBII activity was positive in 22 mothers at delivery, and TSAb activity was positive in 18. In 12 cases, both activities were positive. Both the TBII and the TSAb activity of maternal serum at delivery correlated well with that of the cord serum. Neonatal thyrotoxicosis occurred in 9 of 108 neonates (8%), of whom five (5%) had clinical overt symptoms requiring antithyroid drug treatment. In all nine cases the TBII and TSAb activities were both positive, but no neonate without TBII or TSAb activity developed thyrotoxicosis. The prediction rate (42%) of neonatal overt thyrotoxicosis was higher when both TBII and TSAb were measured than when only TBII (23%) or TSAb (28%) was measured. Clinical overt thyrotoxicosis could be predicted in five of six neonates (83%) of mothers when the cutoff levels of antibody activities were increased to a TBII activity of above 8 U/ml and TSAb activity of above 1.0 TSH microUEq.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies↗

Urinary iodide excretion measured with an iodide-selective ion electrode: studies on normal subjects of varying ages and patients with thyroid diseases.

As a part of studies concerning clinical application of the measurement of urinary iodide with an iodide-selective ion electrode, we report here the reference values for the iodide content or concentration in morning spot urine specimens from normal subjects of varying ages and studies with patients with thyroid diseases in Japan. The number distribution of the iodide content or concentration in the morning specimens appeared logarithmic normal in adults, children and infants. Normal ranges found in 95 per cent of populations of healthy subjects were 5.3 to 62.0 mumoles/g Creatinine (Cr) for adults, 5.3 to 42 mumoles/g Cr for children, and 1.9 to 56 microM for infants, respectively. Urinary iodide concentrations in breast-fed infants varied widely compared with those in bottle-fed infants. Mean values were 16.4 microM for breast-fed infants and 8.6 microM for bottle-fed infants, and they were not statistically different. Urine samples from the infants with transient hypothyroidism, who had undergone amniofetography showed extraordinarily high iodide concentrations, even though they were measured at 20th, 29th and 30th days after birth. Although urinary iodide excretion in patients with simple goiter was within normal limits, the mean was statistically lower than that in normal controls (p less than 0.001). Because of the simplicity and rapidity of the electrode method, we strongly recommend it for use in examining iodide excretion in patients with various thyroid diseases.

Adolescent↗

Efficacy of a multiple insulin injection regimen in teenagers with insulin-dependent diabetes. Carbohydrate and lipid oxidation measured by continuous indirect calorimetry.

Our study of insulin-dependent diabetic teenagers proved that a multiple insulin injection regimen (MIR) can be an acceptable and effective method of glycemic control. Further, the artificial beta cell can be used to determine insulin requirements for MIR. And finally, continuous indirect calorimetry can be used to assess metabolic control in diabetes.

Adolescent↗

Effect of the energy source on changes in energy expenditure, respiratory quotient, and nitrogen balance during total parenteral nutrition in children.

The effects of three isocaloric intravenous nutritional regimens were studied in seven infants and children, ages 2 months to 9 yr, with congenital gastrointestinal anomalies (four patients) or with prior history of malignant disease admitted in remission for bone marrow transplantation (three patients). Energy metabolism, as measured by the basal metabolic rate (BMR), and substrate utilization, as measured by the respiratory quotient (RQ), were studied to determine the effect of different levels of carbohydrate and fat on nitrogen retention in each patient. Solution A provided 8% of energy as amino acids, 87% as carbohydrate, and 5% as fat. Solution B provided 8% of energy as amino acids, 60% as carbohydrate, and 32% as fat. Solution C provided 8% of energy as amino acids, 34% as carbohydrate, and 58% as fat. Administration of solution A (high carbohydrate, low fat) was associated with moderately increased mean (+/- SD) BMR and RQ and with low nitrogen retention (19.1 +/- 12.7%, 1.06 +/- 0.14, and 98 +/- 28 mg N/kg/day). Both the BMR and the RQ decreased when less carbohydrate and more lipid was given: BMR 4.3 +/- 11.6% (p less than 0.005), RQ 0.92 +/- 0.09 (p less than 0.001) for solution B; BMR 3.94 +/- 10.6% (p less than 0.005), RQ 0.86 +/- 0.09 (p less than 0.001) for solution C. Among the solutions tested, optimal nitrogen retention [163 +/- 60 mg N/kg/day (p less than 0.01)] was noted with solution B. Our data support the conclusion that a physiologic balance of fat and carbohydrate results in optimal nitrogen retention.

Child↗

Analysis of steroid 21-hydroxylase gene in five unrelated Japanese patients with 21-hydroxylase deficiency.

DNA samples from five unrelated Japanese patients with 21-hydroxylase (21-OHase) deficiency were studied by Southern analysis using human 21-OHase cDNA. Patterns seen after digestion with not only TaqI but also KpnI showed that two out of the five patients were homozygous for a deletion of the 21-OHase B gene. This result supports the report that the 21-OHase B gene is functional. In the other three, smaller mutations might be responsible for the disorder. The parents of one of the two patients with the deletion had a common ancestor. Hybridization patterns of DNA from members of the family of the patient were consistent with an autosomal recessive mode of inheritance of the deletion that correlates with the clinical phenotype. The deletion segregated with HLA-Aw 24; Bw 61; Cw 3. Heterozygous carriers of 21-OHase deficiency could be detected by comparing the patterns as well as the HLA haplotypes in this family. The application of the family study to the prenatal diagnosis is also discussed.

Adrenal Hyperplasia, Congenital↗

Enzyme immunoassay of free thyroxin in dried blood samples on filter paper.

We describe a double-antibody enzyme immunoassay for determination of free thyroxin (FT4) in dried blood samples on filter paper, with use of a T4-beta-D-galactosidase complex. The measurable range of FT4 concentration in two 3-mm blood discs, each of which contained about 2.7 microL of blood, was 1.9 to 93 ng/L, as determined by comparison with concentrations of FT4 in known serum standards. FT4 in blood samples dried on filter paper was stable for at least four weeks when kept dry at -20 degrees C, room temperature, or 37 degrees C. The mean coefficients of variation were 7.6% (within assay) and 6.4% (between assays). Results for FT4 by this method correlated well with those for serum determined by radioimmunoassay (r = 0.98). The proposed method can be used to differentiate persons with hyper- and hypothyroidism from normal subjects and those with abnormal concentrations of thyroxin-binding globulin. The procedure seems suited for screening studies.

Adult↗

Breath hydrogen test in infants and children with blind loop syndrome.

Breath hydrogen production after oral lactose loading was examined in infants and children with stagnant loop syndrome, blind loop syndrome, or both. All six infants under 3 years of age had bacteriological evidence of small intestinal colonization. The characteristics of the breath hydrogen test in this syndrome are: (a) extremely high basal excretion of breath hydrogen (after overnight fasting); (b) an earlier and greater breath hydrogen value (0.293 +/- 0.201 ml/min/m2: mean +/- SD) after oral lactose administration than formed in lactose malabsorption alone (0.050 +/- 0.041 ml/min/m2); and (c) the observation of a sustained hydrogen rise over several hours. This investigation demonstrated that the breath hydrogen test is a promising and noninvasive tool for diagnosing blind (or stagnant) loop syndrome.

Blind Loop Syndrome↗

Total parenteral nutrition with a new amino acid solution for infants.

The currently available, commercially prepared amino acid mixtures for intravenous usage do not result in completely normal plasma amino acid patterns. Taking into consideration the known imbalances that occur with the commercial preparations, we designed a new amino acid solution (AF). This solution was prepared to contain a low concentration of those amino acids usually found in increased concentrations in plasma during the course of total parenteral nutrition (TPN) with a commercially prepared product (CF) and a high concentration of branched-chain amino acids and arginine. Plasma amino acid concentrations were determined for nine infants receiving CF (Group A) and eight infants receiving AF (Group B) as the protein source while on TPN for 5 days. These data were compared to 2-h postprandial values obtained from breast-fed, growing infants serving as controls. In comparison with controls, the plasma concentrations of threonine, glycine, methionine, phenylalanine, tryptophan, and lysine were higher in infants in group A, while those of taurine, alanine, valine, cystine, isoleucine, and proline were lower. In group B, the levels of valine and leucine were slightly higher, while those of alanine, cystine, tyrosine, histidine, and proline were lower. Mean nitrogen balances in groups A and B were + 112 and + 170 mg/kg/day, respectively. Therefore, our new amino acid solution may be better for infants receiving TPN.

Amino Acids↗

HLA-A and -B antigens in Japanese patients with congenital hypothyroidism and their parents.

The frequency of HLA-A and B antigens were studied in 32 Japanese patients with congenital hypothyroidism due to thyroid dysgenesis, and in their parents. The incidence of the Aw24 antigen was significantly higher in 27 mothers of patients with ectopic thyroid (91.3%, corrected P less than 0.037) and it seemed to be slightly higher in patients (77.7%) than in controls (56.8%). The Aw24 antigen was also found in 4 patients with thyroid hypoplasia and their mothers. No difference was found in the incidences of the antigen in the fathers of patients and in controls. The haplotype frequencies were not significantly different in controls, patients and their parents. These findings suggest that the gene for susceptibility to congenital hypothyroidism due to thyroid dysgenesis is closely linked to the gene for the HLA-A locus of the patients' mothers.

Adolescent↗

Semiautomated enzyme immunoassay of thyrotropin as a mass screening test for neonatal hypothyroidism.

A sensitive, simple, and rapid semiautomated sandwich enzyme immunoassay (EIA) was developed for measuring thyrotropin in dried blood samples on filter paper for use in screening for neonatal hypothyroidism. Good correlation was found between values for thyrotropin determined by this method and those determined by radioimmunoassay (RIA) (r=0.94). In pilot tests on 17,160 newborn infants in the general population, five cases of primary hypothyroidism were detected by both EIA and RIA. The recall rate was slightly highter in EIA than in RIA.

Autoanalysis↗

A live varicella vaccine in a pediatric community.

A total of 663 children with various underlying diseases were immunized with a live varicella vaccine at the vaccine clinic of Osaka University Hospital during a period of seven years from October, 1975. Clinical reactions after vaccination occurred in 32.4% (24/74) of the children with malignancies and in 0.3% (2/591) of those in other groups. Vaccine-induced immunity was detected for more than 6 years, by FAMA (fluorescent antibody to membrane antigen) and IAHA (immune adherence hemagglutination) tests, and a skin-test for varicella-zoster virus (VZV). During an observation period of more than 7 years, clinical varicella developed in 12 children, 8 of whom were in the group with malignancies. Zoster occurred in only 4 (9.1%) of 44 vaccinees with acute leukemia, this incidence being significantly less (p less than 0.05) than that (21.6%, 8/37) in un-vaccinated leukemic children.

Antibodies, Viral↗

Study of ammonia metabolism in a patient with ornithine transcarbamylase deficiency using an 15N tracer.

Ammonia metabolism was studied in an 8-year-old girl with ornithine transcarbamylase (OTC) deficiency, using 15N-tracer. Changes in the incorporation of 15N into amino acids and urea were examined after 15NH4Cl administration. The recovery of total 15N in the urine of the patient in 3 days was 28.5% of the administered 15N whereas that of a control was 69.3%. They were mostly urea. The recovery of 15N-urea in the patient was 28.8% of the control in 1 day, 32% in 2 days and 33.3% in 3 days after the administration of 15NH4Cl. A larger amount of 15N was incorporated into glutamine (alpha-amino N) and glutamate and 15N was incorporated more rapidly into alanine, asparagine and serine in the patient than in the control. The incorporation into ornithine was less in the patient than in the control.

Amino Acids↗

Thyroid hormones in human milk and their influence on thyroid function of breast-fed babies.

Various assay methods for detection of thyroid hormones in human milk were evaluated in recovery and dilution experiments after which the concentrations of thyroxine (T4) and 3,5,3'-triiodothyronine (T3) were measured and compared with those in serum. The effect of breast feeding on pituitary thyroid function of normal babies also was studied. Competitive protein-binding analysis (CPBA) was found to be unsuitable for measurement of T4 in milk. T4 was not detected in samples of human milk by four radioimmunoassays (RIA), although more than 100% of T4 was recovered in the assays. RIA (double antibody-ANS system) seemed to be reliable for detection of T3 in milk, judging from recovery and dilution experiments. T3 was detectable in all samples obtained 1-4 months postpartum. The T3 concentration in milk was not correlated with protein concentration or daily volume. The concentration of T3 in milk was lower than that in serum and the mean ratio of serum T3 to milk T3 was 2.8 +/- 1.7 (mean +/- S.D.). No correlation was observed between the T3 concentration or daily T3 excretion in milk and the T3 concentration in serum. The total amount of T3 excreted in milk was estimated as only 5-1000 ng/day. The serum levels of thyrotropin, T4, free T4 and T3 were not significantly different between breast-fed and bottle-fed babies. These results indicate that T3 excretion in milk cannot be explained by simple diffusion from the blood into the mother's milk and that breast feeding has no influence on the pituitary thyroid axis of normal babies.

Breast Feeding↗

Activity of the glycine cleavage system in hyperammonemia treated with benzoate.

We investigated levels of the glycine cleavage system in livers of spf-fur mutant mice with ornithine transcarbamylase (OTC) deficiency treated with sodium benzoate. The activities of the glycine cleavage system in benzoate-treated spf/Y males and the control mice livers are not significantly different. We examined plasma folate level and Vit. B6 status in a patient with OTC deficiency during the therapy with benzoate. Plasma folate level and vitamin B6 status during the therapy period and during control period are not different and these data were within normal ranges. The observation suggests that the glycine cleavage system is not the candidate for the increase of the de novo synthesis of glycine in hyperammonemic patients treated with benzoate.

Ammonia↗

Enzyme immunoassay of thyroxin-binding globulin in dried blood samples on filter paper.

A double-antibody enzyme immunoassay was developed for determination of thyroxin-binding globulin in dried blood samples on filter paper. The measurable concentration range of thyroxin-binding globulin in two 3-mm blood discs was 3.3 to 52 mg/L equivalent of serum (i.e., equivalent to the concentrations in known serum standards). Thyroxin-binding globulin in dried blood samples on filter paper was stable for at least four weeks when kept dry at -20 degrees C, 4 degrees C, or room temperature. The mean coefficients of variation were 6.6% (within assay) and 5.9% (between assays). The concentrations of thyroxin-binding globulin in dried blood samples determined by this method correlated well with those in serum determined by radioimmunoassay (r = 0.95) and by enzyme immunoassay (r = 0.96). This method is applicable for detecting cases of thyroxin-binding globulin deficiency and avoids the false-positive results for neonatal hypothyroidism obtained by measuring thyroxin.

Adolescent↗