Ursodeoxycholic acid in biliary atresia.
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Bile Acids and Salts↗
Biomedical subjects
K Obinata
Publications and source records attributed to K Obinata.
Sulfated and nonsulfated urinary bile acids in cholestasis in children.
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Adult↗
Hypercalcaemia in cerebellar medulloblastoma: pathogenesis of solid tumour-associated hypercalcaemia.
A 7-year-old boy developed acute, severe hypercalcaemia following the partial excision of a cerebellar medulloblastoma. The serum calcium level was extremely high (19.8 mg/100 ml), but a skeletal survey revealed no apparent bone metastatic lesions; such lesions were only detected by X-ray 3 weeks after the onset of hypercalcaemia. Hypercalcaemia was promptly resolved by intravenous mithramycin administration, before which the serum parathyroid hormone level, 1,25-(OH)2-vitamin D level and the nephrogenous cyclic AMP level were low. However, the relation between serum calcium levels and urinary calcium excretions indicated that renal calcium reabsorption was increased in association with hypercalcaemia, suggesting that a parathyroid hormone-like effect was operative on the renal tubules. It is possible that a combination of increased bone resorption by metastatic tumour cells and renal tubular handling of calcium presumably mediated by tumour-produced humoral factors was responsible for the acute development of severe hypercalcaemia in this patient with medulloblastoma.
Bone Neoplasms↗
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↗
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↗
Serum concentrations of glucuronidated and sulfated bile acids in children with cholestasis.
Serum concentrations of nonglucuronidated-nonsulfated, glucuronidated, and sulfated bile acids in 9 control children and 16 children with cholestasis were quantitated by mass fragmentography. Total bile acid levels in control children were 19.55 +/- 2.78 mumol/liter (mean +/- SEM), and glucuronidated and sulfated bile acids comprised 2.6 +/- 0.5 and 17 +/- 3.1%, respectively. In 9 patients with congenital biliary atrasia, total bile acid levels were 167.34 +/- 11.18 mumole/liter of which 2.1 +/- 0.3% were glucuronidated and 15 +/- 1.4% were sulfated. Lithocholic and 3 beta-hydroxy-5-cholenoic acids, which have hepatotoxic effects, were presented in only small amounts in cholestatic children, and they were almost all glucuronidated or sulfated. The percentages of glucuronidated bile acids in control and cholestatic children were lower than in healthy and cholestatic adults, which may be explained by the lower activity of UDP-glucuronyltransferase in neonatal liver.
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↗
Vitamin D metabolism in pre-operative extrahepatic biliary atresia.
In order to clarify the pathogenesis of rickets in preoperative patients with extrahepatic biliary atresia, we evaluated baseline serum 25-OHD and 1,25(OH)2D levels and correlated serum 25-OHD levels with increase in age and season of birth in 16 preoperative patients. Further, parenteral vitamin D2 tolerance tests were performed in 5 cases. Serum 25-OHD and 1,25(OH)2D levels were significantly lower than those in 15 normal controls. There was a negative correlation between the serum 25-OHD levels and increase in age. The patients born during the winter had lower serum 25-OHD concentrations than those born in summer. The mean value of increased 25-OHD levels after the parenteral vitamin D2 tolerance tests did not differ from that of 6 controls. Since there was no impairment of vitamin D 25-hydroxylation, the reduction in serum 25-OHD may therefore be mainly due to disturbed intestinal vitamin D absorption. It was also concluded that season of birth and increase in age are pathogenic factors in the etiology of rickets in preoperative patients with extrahepatic biliary atresia.
Biliary Atresia↗
Human herpesvirus-6 (HHV-6)-associated hemophagocytic syndrome.
Virus-associated hemophagocytic syndrome (VAHS) is characterized by histiocytic proliferation and phagocytosis triggered by virus infections. Viruses in the herpes group, especially the Epstein-Barr virus (EBV), are well known to cause VAHS; however, the relationship between this syndrome and human herpesvirus-6 (HHV-6) infection has rarely been reported. In this study, we describe a 23-month-old girl who exhibited typical manifestations of VAHS associated with HHV-6 infection. To the best of our knowledge, this case is the fifth reported case in the English literature.
Antibodies, Viral↗
Acute lymphoblastic leukemia accompanied by severe hypercalcemia: successful treatment including aminohydroxypropylidene bisphosphonate (pamidronate disodium)
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Child↗
Early ultrastructural changes in the dorsal mucosa of rat tongue after irradiation, with special reference to the microvasculature.
PURPOSE: To clarify the acute effects of irradiation on the ultrastructural conformation of the dorsal mucosa of the rat tongue, with special reference to the changes in microvasculature. METHODS: The proboscis of seventy rats were irradiated. The animals were then perfusion-fixed, followed by India ink-injection or resin casting at 3 to 7 days after irradiation. The bulk, frozen sections, or plastic embedded sections of the treated rat tongues were examined by light and electron microscopy. RESULTS: In the dorsal epithelium of the rat tongue, multi-nucleated cells appeared in the basal layer at 3 days after irradiation. At day 5, the thickness of the epithelial layer and connective tissue papillae decreased dramatically, concomitant with the shortening of the capillary loops. At day 7, lingual papillae and connective tissue papillae disappeared, leaving dissociated epithelial cells and numerous neutrophils migrating throughout the tissue. Subepithelial blood vessels displayed drastic dilation with a number of neutrophils adhering to the endothelial surface, but without ultrastructural abnormalities in its cellular components. CONCLUSION: Early changes in the dorsal mucosa of the irradiated rat tongue were limited to the basal epithelial cells, leading to a total disruption of the epithelial layer. Atrophic changes of the capillary loops is due to the loss of the connective tissue papillae. Dilation and conformational changes of the subepithelial capillaries appear to result from the inflammatory reaction, taking place secondarily to the loss of the epithelial barrier of the irradiated tongue. A difference in radiosensitivity among the epithelial, endothelial, and mesenchymal cell components of the rat tongue in vivo is suggested.
Animals↗