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Biomedical subjects

K Yabuta

Publications and source records attributed to K Yabuta.

At least 163 records · Page 9Linked to original sources

Thyrotropin binding inhibitor immunoglobulin. Its pathogenetic importance in hypothyroidism.

Two patients with hypothyroidism had detectable serum levels of thyrotropin binding inhibitor immunoglobulin (TBII). Patient 1 was a newborn infant who had transient neonatal hypothyroidism due to transfer of TBII from the mother with nongoitrous autoimmune thyroiditis. Patient 2 was an 8-year-old girl with Down's syndrome who presented with signs of myxedema and central precocious puberty. She had no goiter, and the recognition of thyroid disease was delayed; the histological diagnosis of chronic lymphocytic thyroiditis was established by aspiration biopsy, and TBII had strong thyroid adenyl cyclase-inhibiting activity in vitro. It appears that TBII may be pathogenetically important for occurrence of neonatal hypothyroidism and nongoitrous autoimmune thyroiditis without goiter.

Adenylyl Cyclases↗

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↗

Infantile hypertrophic cardiomyopathy with QT prolongation.

An infant who died at 5 months with hypertrophic cardiomyopathy accompanied by QT prolongation was examined at autopsy. At the age of 16 days, serum IgM level was elevated (226 mg/dl). Microscopic examination revealed characteristics of hypertrophic cardiomyopathy with myocardial cell hypertrophy with disarray and healed myocarditis with patchy fibrosis and mononuclear cell infiltration. The histological findings were compatible with those of postmyocarditic cardiomegaly developing during the fetal period.

Arrhythmias, Cardiac↗

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↗

Ring chromosome 10 syndrome: case report and the possibility of clinical diagnosis.

A 2-year-old boy, whose clinical abnormalities included growth retardation(3rd percentile), mild mental retardation (DQ = 67), microcephaly, hypertelorism, strabismus, prominent nasal bridge, stubby nose, pointed chin, undescended testes and hydronephrosis, had a ring chromosome 10. Using high-resolution banding technique, the breakpoints were decided at p15.3 and q26.3 of chromosome 10. The clinical features observed in this case were common in 8 other cases with ring chromosome 10 previously reported. Thus, the terminal deletion of both arms of chromosome 10 seems to be the cause of the specific clinical abnormalities. When a patient has the clinical common abnormalities above mentioned, ring chromosome 10 syndrome will be suspected before chromosome analysis.

Child, Preschool↗

Sarcomatous Wilms tumor associated with consumption coagulopathy.

This is a case report of a sarcomatous Wilms tumor complicating disseminated intravascular coagulation with gastrointestinal bleeding, ascites, pleural effusion, and hepatitis after the second course of actinomycin D. A sarcomatous Wilms tumor is believed to have an unfavorable prognosis even if multimodal therapy is given. Arteriovenous fistulae which we created for purposes of chemotherapy and hyperalimentation by microvascular-sleeve anastomosis functioned well for three years in spite of repeated punctures.

Arteriovenous Shunt, Surgical↗