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Biomedical subjects

O Arisaka

Publications and source records attributed to O Arisaka.

84 records · Page 5Linked to original sources

Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography.

To elucidate urinary bile acid patterns in patients with biliary atresia (BA), 15 sulfated and nonsulfated bile acids in urine were separately measured by high-performance liquid chromatography. This relatively simple technique for fluorescence detection utilizes the enzyme 3 alpha-hydroxysteroid dehydrogenase (3 alpha-HSD) to reveal urinary bile acid patterns. By this method, recovery rates of sulfated and nonsulfated bile acids in urine were satisfactory, and this analysis was shown to be applicable to clinical situations. In 10 patients with BA, the mean level of total bile acids in urine (23.35 +/- 18.51 mumol/day) was seven times higher than the mean level in eight normal infants (3.05 +/- 2.05 mumol/day). In the infants with BA, the mean level of total sulfated bile acids was about half of the total bile acid level. The main components of urinary nonsulfated bile acids in BA were glycocholic acid (6.21 +/- 5.55 mumol/day) and taurocholic acid (2.28 +/- 1.33 mumol/day), whereas the main components of the urinary sulfated bile acids were glycochenodeoxycholic acid (4.58 +/- 6.97 mumol/day) and taurochenodeoxycholic acid (3.67 +/- 3.54 mumol/day). Chenodeoxycholic acid, which is relatively toxic to the liver, may more easily be conjugated with sulfate and, hence, excreted into urine at a faster rate than cholic acid. Marked individual variations in urinary bile acid patterns were observed not only in BA patients but also in normal controls.

Adult↗

Conjugating ability of bile acids in hepatic failure.

A 5-month-old boy with giant cell hepatitis died of hepatic failure. Analysis of serum bile acids by high pressure liquid chromatography revealed a marked increase in total bile acid concentration and a decrease in the cholate to chenodeoxycholate ratio. However, contrary to expectation, the conjugating ability of bile acids with taurine or glycine was well preserved. This suggests that the amino acid conjugation can occur despite the presence of severe hepatocellular damage.

Bile Acids and Salts↗

Early diagnosis of 11 beta-hydroxylase deficiency in two siblings confirmed by analysis of a novel steroid metabolite in newborn urine.

Plasma and urinary steroid measurements are reported in 2 normotensive newborn female siblings with virilized external genitalia due to 11 beta-hydroxylase deficiency. Plasma 11-deoxycortisol concentrations were markedly elevated whereas 17OH-progesterone concentrations were not raised. Plasma renin activity was suppressed, but increased to levels characteristic of infancy within 4 weeks of treatment. The enzyme defect was confirmed by measurement of increased urinary excretion of tetrahydro-11-deoxycortisol. A more polar steroid metabolite, 6 alpha-hydroxytetrahydro-11-deoxycortisol was also determined by gas chromatographic and mass spectrometric analysis. Analysis of metabolites in urine is an additional specific marker to plasma 11-deoxycortisol measurement for the diagnosis of 11 beta-hydroxylase deficiency in early infancy.

17-Hydroxycorticosteroids↗

Precocious breast development: a case of unilateral hyperplasia of the adrenal cortex.

The case of a 14-month-old girl presenting with precocious breast development due to adrenal hyperplasia is reported. The endocrine studies revealed a slight elevation of the plasma progesterone, 17 alpha-hydroxyprogesterone, testosterone and oestrone levels, and an increased urinary oestrogen excretion. The findings of the ultrasound examination and the evidence that the oestradiol concentration in the left adrenal vein was higher than in the right adrenal vein confirmed the diagnosis of left adrenal hyperfunction. Surgical exploration revealed unilateral hyperplasia of the adrenal cortex. After adrenalectomy the plasma hormone levels and urinary oestrogen excretion fell to normal, with concomitant regression of breast tissue. The girl shows no signs of recurrence of the disorder in a 2 year follow-up. This adrenal hyperplasia might be a benign adrenal disorder causing precocious breast development.

17-alpha-Hydroxyprogesterone↗

Investigation of serum bile acids; seven patients with Alagille syndrome.

To clarify whether an abnormal bile acid pattern has a role in the pathogenesis of Alagille syndrome, we compared serum bile acid patterns in seven with Alagille syndrome with those of patients with congenital biliary atresia (CBA), neonatal hepatitis (NH) and normal infants. Of the seven patients with Alagille syndrome, four patients were younger and three were older than 1 year. The mean total serum bile acid level in the infants was higher than in older subjects. There was a dissociation between the levels of serum total bile acid and bilirubin in three of the seven cases. The mean total bile acid levels in serum were in the following decreasing order: CBA, Alagille syndrome, NH and controls. The ratio of cholate to chenodeoxycholate in the younger patients with Alagille syndrome was significantly higher than CBA (P less than 0.001). However, no specific bile acid pattern was found in Alagille syndrome by high-performance liquid chromatography (HPLC).

Adolescent↗

Primary hyperparathyroidism. Case report and management.

A 13-year-old girl presenting with abdominal pain, polyuria, polydipsia, and radiologically confirmed renal calculi was diagnosed as having primary hyperparathyroidism. Laboratory data revealed markedly elevated serum calcium, low phosphorus, and elevated parathyroid hormone. Other parathyroid function tests also confirmed the diagnosis of primary hyperparathyroidism. Ultrasound examination showed a small echogenic nodule in the parathyroid gland. Following a single gland resection, the extremely high serum calcium level promptly decreased to normal range, and it has remained normal.

Adolescent↗

Chorea as an initial manifestation of systemic lupus erythematosus. A case report of a 10-year-old girl.

Neurological complications of systemic lupus erythematosus (SLE) are relatively common, but isolated chorea as the initial manifestation of SLE is very rare. We report a 10-year-old girl presenting with chorea, who developed clinical and laboratory findings of SLE 10 months after the onset of chorea. In this case, mild depression of complement levels in serum, which had already been found in the early stage of the chorea, was the only abnormal laboratory datum. The low serum complement levels that precede the appearance of other clinical and laboratory findings may be helpful for the diagnosis of isolated chorea involvement in SLE.

Child↗

Spontaneous endocrine cure of gigantism due to pituitary apoplexy.

An 11 year old, tall boy presented with symptoms typical of pituitary apoplexy. A large necrotic and haemorrhagic tumour was removed, which was shown to be an adenoma secreting growth hormone and prolactin. Subsequent treatment comprised cranial irradiation and hormone replacement. Eighteen months after operation growth was static and plasma growth hormone and prolactin concentrations were undetectable. Treatment of pituitary apoplexy should comprise excision of the tumour and postoperative irradiation; such treatment after early recognition of the condition offers the best chance of preserving normal pituitary function in children with gigantism.

Adenoma, Acidophil↗

Effects of early intervention with inhaled sodium cromoglycate in childhood asthma.

International and Japanese guidelines classify childhood asthma as mild, moderate, or severe, and recommend treatment with "as needed" bronchodilators, inhaled sodium cromoglycate, and inhaled corticosteroids, respectively. Alternatively, some investigators proposed inhaled corticosteroids as first-line therapy to prevent airway inflammatory obstruction. This article describes a clinical study assessing the effect of early intervention with inhaled sodium cromoglycate in childhood asthma. This was a retrospective study of 189 asthmatic children treated with inhaled sodium cromoglycate for five years and whose age of onset of asthma was six year of age or younger. An end-of-study questionnaire completed the examination. Children administered oral or inhaled corticosteroids simultaneously with sodium cromoglycate, were excluded. Asthma scores as defined by the Japanese Pediatric Allergic Committee, were reduced continuously during the five years after the start of sodium cromoglycate treatment. The frequency of emergency department visits and hospitalizations also decreased. Significant between-period intervention differences showed improvement of clinical outcomes for children with severe asthma during the five years of sodium cromoglycate inhalation. The early intervention regime of starting sodium cromoglycate inhalation within two years of the onset of asthma shows a large improvement in the long-term prognosis of childhood asthma, especially for children with severe asthma. It is possible that starting inhaled sodium cromoglycate earlier than the present recommendation of corticosteroids could further improve clinical outcomes, but a prospective study should be performed to verify our results.

Administration, Inhalation↗

Prostaglandin metabolism in children with diabetes mellitus. I. Plasma prostaglandin E2, F2 alpha, TXB2, and serum fatty acid levels.

To study prostaglandin (PG) metabolism in children with insulin-dependent diabetes mellitus, plasma levels of PGE2, PGF2 alpha, and thromboxane B2 and the composition of serum fatty acids were measured. Platelet aggregation, a risk factor in diabetic vascular complications, was also measured. The mean levels of plasma PGE2 and PGF2 alpha were high and serum dihomo-gamma-linolenic acid and arachidonic acid were low in the diabetic children. The plasma thromboxane B2 levels of the diabetic children and normal controls were not significantly different. Platelet aggregation was also increased in diabetic patients. These results suggest that the insulin deficiency and high blood sugar in diabetic children may disturb the supply of dihomo-gamma-linolenic acid from cis-linoleic acid, decreasing prostaglandin formation in series 1. Then feedback regulation may increase production of PGE2 and PGF2 alpha in series 2. Altered PG metabolism may be responsible for the occurrence and progression of vascular complications in the diabetic children.

Adolescent↗