Yusaku Tazawa

Demonstration of IgG EA (early antigen) and IgM MA (membrane antigen) antibodies in CMV infection of healthy infants and in those with liver disease

The sequence of antibody production to various virus-specific antigens in CMV infection in infancy was studied. In healthy infants, IgG EA antibody was demonstrated in 18% of cord sera, and disappeared within two months after birth in all cases not shedding virus. The nonmaternal EA antibody was produced following virus excretion and decreased rapidly following cessation of virus excretion. Thus, demonstration of EA antibody in infants after 2 months of age was found to indicate acquired CMV infection, even when CMV could not be isolated. IgM MA antibody did not persist as long as EA antibody, disappearing before cessation of virus excretion. Both IgG EA and IgM MA antibodies were more frequently demonstrated in infants with hepatitis than in healthy infants. These findings suggest the possible association of CMV with hepatitis in infants.

Significance of serum lipoprotein-X and gammaglutamyltranspeptidase in the diagnosis of biliary atresia. A preliminary study in 27 cholestatic young infants

As simple and nonsurgical means of differentiating biliary atresia (BA) from intrahepatic cholestasis of unknown origin (IC), liver function tests including serum lipoprotein-X (LP-X) and γ-glutamyltranspeptidase (GGTP) were done and evaluated for their usefulness in the diagnosis of 27 cholestatic Japanese young infants. Except for LP-X and GGTP levels (P<0.01, P<0.001), there were no significant differences between the BA (n=11) and IC (n=13) groups. When values of mean plus 4 standard deviations were used to differentiate BA from IC (89 mg/100 ml for LP-X and 194 IU/l for GGTP), all BA patients gave positive results for either the crtical LP-X of GGTP values. On the other hand, all IC patients gave negative results for both levels, although patients with a paucity of intrahepatic biliary ducts (n=3) were also positive for either the critical LP-X or GGTP values. The combination test with serum LP-X and GGTP is recommended for helping to differntiate BA from IC in cholestatic young infants.

Serum bile acids and their conjugates in breast-fed infants with prolonged jaundice

Serum bile acids and their conjugates were analysed in 20 breast-fed infants with prolonged jaundice. The mean total bile acid levels in serum were increased in the breast-fed infants with jaundice, as compared with those in either breastor bottle-fed infants without jaundice. However, there were no significant differences between the groups. All the breast-fed infants examined, regardless of association with jaundice, had a bile acid pattern dominated by taurine conjugates (the ratio of glycine- to taurine-conjugated bile acid, G/T ratio, less than 1.00). In contrast, the bottle-fed infants without jaundice had a pattern dominated by glycine conjugates (G/T ratio, more than 1.00). Among the breast-fed infants with jaundice, the mean G/T ratio in those who had serum bilirubin levels over 10 mg/100 ml was significantly lower than that in those who had serum bilirubin levels of less than 10 mg/100 ml. The altered bile acid metabolism might be associated with the pathology of breast milk jaundice.

Studies on Steroids CCXXXXIV. Separation and Characterization of C-25 Epimers of Unconjugated and Conjugated Trihydroxycholestanoic Acids in Urine from a Patient with Zell Weger Syndrome by High-Performance Liquid Chromatography

Abstract The separation and characterization of C-25 epimers of unconjugated and glycine- and taurine-conjugated 3α, 7α, 12α - trihydroxy-5β-cholestanoic acid (THCA) in biological fluids by high-performance liquid chromatography (HPLC) are described. The 5β-cholestanoic acid fraction was obtained from a urine specimen from a patient with Zellweger syndrome by passing it through a Sep-pak C18 cartridge. Bile acids were derivatized quantitatively into the fluorescent compounds through the hydroxyl group at C-3 by treatment with 1-anthroyl nitrile. The derivatives were separated into the unconjugated, glycine- and taurine-conjugated fractions by ion-exchange chromatography on alipophilicgel, piperidinohydroxypropyl Sephadex LH-20. Sub-sequent resolution of each fractionin to (25s)- and (25R)-THCA was attained by HPLC on a Cosmosil 5C column. The C-25 epimers of unconjugated and conjugated Tk%A were unequivocally identified on the b asis of the irbehaviors in HPLC using mobile phases of different pHs. The rat...

Unconjugated, Glycine‐conjugated, Taurine‐conjugated Bile Acid Nonsulfates and Sulfates in Urine of Young Infants with Cholestasis

ABSTRACT. A direct assay system for conjugated bile acids using an enzymatic procedure and high‐performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra‐hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3β‐hydroxy‐5‐cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63±2.83 vs. 2.50±1.25, p <0.05, 15.8±9.9 vs. 34.5±9.9%, p < 0.005). The proportion of taurine‐conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7±7.5 vs. 22.7±7.8 %, p < 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine‐conjugated chenodeoxycholate sulfate excretion.

Lipoprotein-X and diagnosis of biliary atresia.

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Lipoprotein X, gamma-glutamyltranspeptidase and biliary atresia

toff value, Dr. Fung may be right. We will deeply consider his suggestion in our 800 future studies. In our experience, however, serum G G T P and LP-X in intra700 hepatic cholestasis of unknown origin (IC) patients varied much less in value and had a tendency to cluster in a lower G00 and more narrow range than in B A patients. Based on these observations we chose the data from the IC group to de500 fine critical values for discrimination of B A patients. ~00 Recently, we reevaluated our combinat ion tests with an increased number of patients (46 with IC and 26 with BA), 300 and the overall accuracy in diagnosis was 89%: 41 of 46 IC patients and 23 of 26 200 B A patients were correctly diagnosed [21.

Application of the New Oral Pancreatic Exocrine Function Test “PFD” Test in Children—Normal Values in Children—

The application of the PFD test, a newly developed technique for testing exocrine pancreatic function, was studied in infants and children.

Syndrome of Inappropriate Secretion of Antidiuretic Hormone (SIADH) in a Case with Guillain-Barre Syndrome

A 10-year-old girl with the syndrome of inappropriate secretion of antidiuretic hormone complicated with Guillain-BarrC syndrome is reported. The patient had been treated as juvenile rheumatoid arthritis for 8 years. O n November 25, 1974, she was admitted with complaints of fever, paraesthesia, bilateral flaccid paralysis of the extremities, and urinary retention. On admission, physical examination revealed neurological abnormalities such as hypotonia, failure to elicite deep tendon reflexes, anesthesia, dysphagia, and dysathria. There was no ophthalmological abnormality. Lumbar puncture carried out on admission showed a clear cerebrospinal fluid which contained one nucleated cell per mms, and 85.4 mg/100 ml of protein. On the basis of these findings, she was diagnosed as Guillain-BarrC syndrome. During the course of the illness, laboratory values included a serum sodium of 113, chloride of 83, potassium of 4.5 mEq/ L, a plasma osmolality of 250, and a urine osmolality 335 mOsm/kg water. A waterloading test subsequently carried out revealed an impairment of urinary dilution, and plasma ADH levels were 2.2 pU/ml (normal: 0.8f 0.2) after the loading. Thus, the diagnosis of SIADH was confirmed, and she showed satisfactory response to restriction of fluid intake.