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Biomedical subjects

A Okuno

Publications and source records attributed to A Okuno.

16 recordsLinked to original sources

[Athyreotic congenital hypothyroidism in two sisters].

Two sisters with athyreotic congenital hypothyroidism are described. This is the fifth report on athyreotic congenital hypothyroidism in siblings. The elder sister is 14 years old and the younger one is 12. The parents have no consanguinity or family history of thyroid disease. Both of the patients were born before the start of neonatal screening tests for congenital hypothyroidism. After birth, they had jaundice, abdominal distention and constipation, which are typical symptoms of congenital hypothyroidism. Serum T4 levels were decreased, and the serum TSH levels were markedly increased. Therefore we diagnosed them as having congenital hypothyroidism. They have received replacement therapy with thyroxine since diagnosis and have shown normal development physically and psychologically. They were tested for thyroid scintigram when the elder sister was 9 years old and the younger one was 7. 123I thyroid uptakes were 0.69% and 0.64%, respectively. The thyroid scans demonstrated no focus of accumulation of 123I. They do not have trapping defect of iodine, because 123I ratio of saliva and serum were 41.3 and 46.3, respectively. From these results, we diagnosed them as having athyreotic congenital hypothyroidism. They and their mother do not have any antithyroid antibodies. We suppose that some genetic factor is responsible for the athyreotic congenital hypothyroidism.

Child

Sonographic imaging of the thyroid gland in congenital hypothyroidism.

We have attempted to facilitate differential diagnosis of etiological types of congenital hypothyroidism using real-time ultrasonography. Sonography of the thyroid gland was performed on 418 normal children, and 23 patients with congenital hypothyroidism and hyperthyrotropinemia. The thyroid gland was imaged by transverse scanning at the neck; the maximum thickness and the maximum width of the right and left lobe were measured. On the basis of the normal thyroid gland size obtained from normal children, the thyroid gland image of the patients were classified into 4 types: large image, normal image, small image, and no image of the thyroid gland at the neck: no image of the thyroid gland indicated agenesis or ectopia; large thyroid gland image indicated goitorous hypothyroidism. On the other hand, normal or small thyroid gland image probably included mild or transient forms of hypothyroidism, and transient hyperthyrotropinemia; these 2 types required further examination to complete the diagnosis. We concluded that real-time ultrasonography of the thyroid gland was a useful diagnostic imaging technic for patients who revealed elevated serum thyrotropin on neonatal mass-screening.

Adolescent

Cytochrome b positive X-linked chronic granulomatous disease: a normal cell surface expression of cytochrome b.

The polymorphonuclear (PMN) cells from a patient with cytochrome b positive X-linked chronic granulomatous disease (Xb+ CGD) were studied using flow cytometry. Both the cell surface expression of monoclonal antibody defined cytochrome b and the superoxide production (intracellular 2',7'-Dichlorofluorescin Diacetate oxidation) were investigated at a single cell level. Flow cytometry clearly demonstrated the complete absence of superoxide production in the patient's PMN cells, the mosaicism in his mother's PMN cells and also indicated the normal cell surface expression of cytochrome b. The results obtained by Western blot analysis and reduced-minus-oxidized spectra confirmed the presence of functional and normal amounts of cytochrome b. We concluded that this is a case of Xb+ CGD with a normal cell surface expression of cytochrome b.

Adolescent

Growth hormone secretory status in patients with Turner syndrome.

The growth hormone (GH) secretory capacities in patients with Turner syndrome aged 5.1-15.9 years and those with constitutional short stature (CSS) aged 5.2-14.2 years were evaluated by pharmacological and physiological means. The GH response to hypoglycemia in the patients with Turner syndrome was lower than that in the patients with CSS. However, the GH response to arginine was not significantly different between the two patient groups. For the physiological test, the integrated concentration of GH (ICGH), the number of episodic peaks and their mean height were evaluated using blood obtained from the patients every 20 minutes for a period of 24 hours. The ICGH and the mean height of the episodic peaks in the patients with Turner syndrome were significantly lower than those in the CSS patients during the night but not during the day. Negative correlation between the bone age and the night-time values of ICGH was observed in the patients with Turner syndrome. Such correlation was not observed in the CSS patients. The patients with CSS showed a significant day-night difference in the ICGH and the mean height of the episodic peaks, but the patients with Turner syndrome did not show any significant day-night difference in either the ICGH or the mean height of episodic peaks. In conclusion, the GH secretory capacity in patients with Turner syndrome is lower than that in CSS patients.

Adolescent

Pharmacokinetics of high-dose methylprednisolone in children.

Disposition of methylprednisolone was characterized in 11 children receiving the high-dose therapy (26.0 mg/kg on average). After intravenous infusion, methylprednisolone hemisuccinate was rapidly converted to methylprednisolone with a half-life of about 20 min. Methylprednisolone in serum, eliminated monoexponentially in 8 patients and biexponentially in the remaining three, had the mean residence time of about 3 h, and a terminal half-life of 2.5 h. The volume of distribution at steady state, and the clearance were 1.3 liters/kg and 0.5 liters/kg/h, respectively. Although these average pharmacokinetic parameters were comparable to those determined in other studies with conventional low doses, the clearance values in our data were characterized by 5-fold interindividual difference, suggesting large variations in exposures to methylprednisolone among children on the high-dose pulse therapy.

Adolescent

[Relationship between recurrence of esophageal varices and changes of portal circulation after endoscopic injection sclerotherapy: evaluation by single photon emission CT].

The relationship between recurrence of esophageal varices after endoscopic injection sclerotherapy (EIS) and changes of the blood pool of portosystemic collaterals was studied in 36 patients with liver cirrhosis. Examination of the blood pool of portosystemic collaterals was performed by single photon emission CT (SPECT). Seven hundreds and forty MBq of 99mTc-RBCs, labeled by an in vivo technique, were given intravenously, and tomographic imaging of the intraabdominal vascular blood pool was performed. Before EIS, the blood pool images of the coronary vein were demonstrated in 34 cases (94.4%). According to changes of SPECT images, the patients were divided into 3 groups, that is, the groups showing a disappearance, decrease, and no changes of the blood pool images of the coronary vein. The recurrence rates of esophageal varices after EIS were 11.1% (1 of 9 patients), 40.0% (6 of 15 patients), and 90.0% (9 of 10 patients) in the disappeared, decreased and unchanged groups respectively. These values were significantly different between the disappeared group and the unchanged group (P less than 0.01), and between the decreased group and the unchanged group (P less than 0.05). These results indicate that the abdominal blood pool SPECT is useful for evaluating the therapeutic effectiveness of EIS.

Adult

Aminophylline suppository-induced acute proctitis.

A 9-yr-old boy revealed anal hemorrhage after administration of an aminophylline suppository. Proctosigmoidoscopy demonstrated edema of the rectal mucosa with hyperemia and petechiae. We performed a provocation test and obtained the diagnosis of aminophylline suppository-induced acute hemorrhagic proctitis. One should recognize that acute hemorrhagic proctitis is a rare but important side effect of aminophylline suppositories, because they are commonly used for asthmatic children.

Acute Disease

Hypocalcemic focal seizures in a one-month-old infant of a mother with a low circulating level of vitamin D.

We present a case of a one-month-old infant with hypocalcemia and rickets, with symptoms of focal seizures. The ictal EEG showed left occipital spikes spreading over all of the left hemisphere. From the laboratory studies, we concluded that a low maternal circulating level of vitamin D would cause infantile hypocalcemia and rickets, while immature renal response to parathyroid hormone and transient hypoparathyroidism in infancy would induce hyperphosphatemia. Hypocalcemia may be an important factor in the cause of focal seizures which start even after the age of one month. Further, investigation of maternal vitamin D levels should be done in infantile hypocalcemia.

Adult

Acute suppurative thyroiditis in children.

Ten cases of acute suppurative thyroiditis were studied. All patients remained euthyroid clinically during the course of this disease and during the follow-up. Of seven cases in which bacterial cultures were performed, an aerobic bacterium alone was isolated in one patient and anaerobic bacteria alone in four patients. Mixed aerobic and anaerobic microorganisms were recovered from one patient and cultures were negative in one case. Plasma TSH, T4, T3, and PBI levels were normal except in one case. The 131I uptake values were normal in six of seven cases. Plasma TSH response to TRH stimulation was normal in four of five cases. Antibodies against thyroglobulin and thyroid microsome were negative in four of six cases. These findings suggest that anaerobic bacteria may play an important role in the pathogenic process of acute suppurative thyroiditis and that the pituitary-thyroid functions are not impaired.

Bacterial Infections

Kinetic analysis of plasma TSH dynamics after TRH stimulation.

Kinetic analysis of the time course of plasma TSH after TRH stimulation was performed by means of a single compartment model with first order input. This kinetic model showed satisfactory fit to the data, and was found to be useful enough for the characterization of plasma TSH dynamics. In endocrinologically normal short children, the amount of TSH release per unit volume of distribution space (Q0/V), the rate constant for the TSH release (alpha), and the rate constant for TSH elimination (beta) were averaged 23.4 microU/ml, 6.981 hr-1 and 0.813 hr-1, respectively. Elevated Q0/V values with lowered alpha and beta were obtained in the hypothyroid children. Variable results, with the exception of low alpha values, were obtained in the children with pituitary dwarfism.

Adolescent

Recurrent acute suppurative thyroiditis.

Two patients had two recurrent episodes each of acute suppurative thyroiditis. Circulating concentrations of plasma thyrotrophin, thyroxine, and triiodothyronine were within the range of normal. Antibodies to thyroid microsome or thyroglobulin were not found. Anaerobic bacteria such as Bacteroides, Peptostreptococcus, and Peptococcus were identified as causative agents, indicating that anaerobic organisms may play an important role in the pathologic process. The present cases are the third and fourth reported cases of recurrent acute suppurative thyroiditis in the literature.

Acute Disease