Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Abnormal lipoprotein (LP-X) represents a specific parameter for the presence of obstructive jaundice in the adult. Since LP-X has also been detected in the serum of newborn infants, both full-term and premature, and in early infancy, in the absence of clinical evidence of obstructive jaundice, extensive investigations were undertaken in infants during the neonatal period to clarify this phenomenon. The present study reports the data obtained in over 2000 sera from over 370 infants (mature newborn and premature newborn and young infants), tested more or less continuously by means of the Rapidophor method, initially on a qualitative, and subsequently, on a semi-quantitative basis. LP-X appears within the first fortnight in newborn infants, irrespective of the mode of feeding. The LP-X concentration was correlated to the birth weight. Premature infants displaying signs of immaturity possessed markedly higher LP-X levels than mature newborn infants. LP-X was not correlated to the alkaline phosphatase level, nor to the gammaglutamyl transferase activity; the bilirubin level, likewise, had no connection with the LP-X concentration. Patients with proven obstructive jaundice showed distinctly higher LP-X concentrations (greater than 56 mg/100 ml), whereby the rise in LP-X level in some cases preceded the appearance of the clinical manifestations of obstructive jaundice. The following hypotheses are advanced in order to explain the presence of LP-X during the neonatal period and are discussed on the basis of clinical observations in adults, the physiological conditions in the newborn infant and the results of the present study: The liver, which occupies the central position amongst metabolic organs, also in the case of the lipoproteins, is at a physiological stage of organic and functional maturation during this early period of life. Under these circumstances, a pseudo-obstructive mechanism on the basis of insufficient excretion of biliary lipoproteins, in conjunction with a simultaneous "physiological" deficiency of lecithin: cholesterol acyl transferase could lead to the appearance of LP-X in the serum. Catabolism of the resultant LP-X cannot take place owing to an inadequate activity of lipoprotein lipase. Functional immaturity can be presumed in the case of both enzyme systems during the neonatal period. On attainment of a degree of maturity compatible with the appropriate neonatal stage, the LP-X values become negative between the 7th and the 16th week of life. It is conceivable that the appearance of LP-X in the newborn infant can be ascribed to LP-X1, since the "physiological" LP-X concentrations in the neonatal period (values of up to 20 mg/100 ml) are distinctly lower than the values found in obstructive jaundice. LP-X determination can be rated as a useful supplementary investigation in the differential diagnosis of extrahepatic biliary atresia during the first weeks or months of life...
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PMID:[Abnormal lipoprotein (LP-X) in the first months of life with particular reference to obstructive jaundice (author's transl)]. 26 15

Serum bile acid measurements now available by radioimmunoassay have proven to be the most sensitive procedure developed to date to assess diseases of the hepatobiliary system in both adult and pediatric liver disease. Their clinical utility appears to hold particular promise in establishing the early diagnosis of liver disease when conventional liver function test such as SGOT, alkaline phosphatase, bilirubin and albumin are still normal. Serum bile acid determinations have been shown to be particularly useful in the diagnosis of alcoholic liver disease, drug-induced liver disease, viral hepatitis and cholestasis of intra- and extrahepatic origin. In infants, serum bile acid measurements can be used to establish the diagnosis of biliary atresia. When serum bile acids are determined post-prandially, they are the most sensitive indicator of liver dysfunction developed to date.
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PMID:Serum bile acids (a new advance in the diagnosis of liver disease). 49 9

Fourteen patients with "noncorrectable" biliary atresia are living without jaundice for more than 2 yr after hepatic portoenterostomy or its modification. Retardation of physical growth was observed in one of them, and mental retardation in another, both of which seemed irrelevant to biliary atresia. Serial tests for liver function after operation revealed early recovery of serum bilirubin, transminase, and turbidity, and delayed improvement of alkaline phosphatase. Postoperative needle biopsy of the liver disclosed that changes in hepatic parenchyma and ductular proliferation were rapidly improved after successful operation. Improvement of fibrosis of the liver was delayed, and it was not satisfactory in patients whose preoperative changes in the liver were severe or in whom ascending cholangitis had been a frequent complication. Histologic features of hepatic cirrhosis were observed in the liver in three cases, in two of which there had been frequent episodes of cholangitis. Only one of these showed clinical signs of portal hypertension. Functional and morphologic cure can be achieved in "noncorrectable" biliary atresia by hepatic portoenterostomy or its modifications, although varying degree of hepatic fibrosis may remain according to severity of preoperative changes of the liver and postoperative complication of ascending cholangitis.
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PMID:Follow-up studies of long term survivors after hepatic portoenterostomy for "noncorrectible" biliary atresia. 112 98

This modified lectin affinity electrophoresis method is suitable for simultaneous measurement of liver, bone, and high-molecular-mass (high-Mr) isoforms of alkaline phosphatase (ALP; EC 3.1.3.1) in children. Age-related isoform reference ranges were derived for 247 children, ages 0-13 years. Liver ALP did not appear in plasma until after six months of age, and remained constant after one year of age. Bone ALP was highest in the youngest age group, and declined to relatively constant activities thereafter. High-MrALP was not detected in normal children. In bone disease, the bone isoform was increased in all age groups. In liver disease, only 5 of 30 infants younger than six months had detectable liver ALP, although half had increased bone ALP. Among children older than six months, 5 patients with biliary atresia and 15 patients with hepatitis A all had increased liver isoform activity. Liver ALP was also a sensitive index of early hepatobiliary complications in 27 nonjaundiced children with cystic fibrosis. Measurement of ALP isoforms therefore yields useful clinical information in children older than six months but is of doubtful value in younger infants.
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PMID:Wheat-germ lectin affinity electrophoresis for alkaline phosphatase isoforms in children: age-dependent reference ranges and changes in liver and bone disease. 158 17

Discriminant analysis was used to differentiate between extrahepatic biliary atresia and intrahepatic cholestasis. Among the ten laboratory variables tested, three (gamma-glutamyl transpeptidase, alkaline phosphatase and total serum bilirubin) were useful in the differential diagnosis. gamma-Glutamyl transpeptidase contributed most to the discrimination (85%). From a population study of 28 babies with extrahepatic biliary atresia and 24 infants with intrahepatic cholestasis, the procedure achieved a diagnostic accuracy and specificity of 92.9% and an efficiency of 92.3%. The jackknife procedure has also confirmed that the mathematical model was robust for discriminant analysis and therefore it may be valid for screening infants with cholestasis for early surgical intervention. Discriminant analysis is a useful adjunct for differentiation between intrahepatic cholestasis and extrahepatic biliary atresia.
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PMID:Differentiation between extrahepatic and intrahepatic cholestasis by discriminant analysis. 197 25

Investigations of bone mineral content, X-ray of the radius and ulna and serum biochemical analysis were performed in 10 children with biliary atresia (B.A.) and 150 healthy children. The patients were classified into 2 groups, namely; group A, comprised of 5 patients whose postoperative courses went well, and group B, comprised of 5 patients who developed various degrees of liver disturbance postoperatively. The ratio of bone mineral content (BMC) to bone width (BW) (BMC/BW) increased in accordance with age in the healthy children and the patients of group A, but was relatively slow in the patients of group B. Signs of rickets were found on the X-rays of 4 of the 10 patients, while serum levels of calcium and 25-OH-vitamin D in the group B patients were significantly lower than those in the group A patients or healthy children. Serum levels of alkaline phosphatase (Alp) fluctuated, but serum Alp was elevated in all the patients with rickets.
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PMID:Bone mineral analysis and X-ray examination of the bone in patients with biliary atresia. 224 46

The differentiation between extrahepatic biliary atresia (EHBA) and neonatal hepatitis (NH) was studied in 53 Thai infants (39 males, 14 females) with obstructive cholangiopathy at the Department of Pediatrics, Siriraj hospital. Prevalence of EHBA was 25 per cent (13 of 15) overall. A higher prevalence of EHBA was found in females (8 of 13) while a higher prevalence of NH occurred in males (34 of 40). No difference was demonstrated in serum conjugated and total bilirubin, SGOT or alkaline phosphatase between EHBA and NH groups. The EHBA group had a significantly higher mean serum GGTP (184 +/- 77 IU/L) than the NH group (58 +/- 40 IU/L). The diagnostic usefulness of GGTP was confirmed, but variation in laboratory measurements could limit its generalizability.
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PMID:Use of serum gamma glutamyl transpeptidase to differentiate between extrahepatic biliary atresia and neonatal hepatitis. 257 63

Surgical restoration of bile flow in patients with extrahepatic biliary atresia (EHBA) results in the disappearance of clinical cholestasis in about 30% of cases. It is suggested that early postoperative administration of phenobarbital (PB) or cholestyramine (Ch) may improve this percentage. Eighty patients were randomly divided into three subgroups comprising 27 who were treated with Ch (4 g/d), 27 who were given PB (7.5 to 10 mg/kg/d) during the first 3 postoperative months and 26 untreated patients, who served as controls. Cholestasis was observed to disappear in 38 (group I) patients and to persist in 42 (group II) patients, as judged from their total blood bilirubin levels, the conjugated/total bilirubin ratio, measurements of cholesterol, bile acids and alkaline phosphatase, and of Rose Bengal fecal excretion. Neither Ch nor PB significantly improved the degree or duration of cholestasis in either group.
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PMID:Effects of postoperative cholestyramine and phenobarbital administration on bile flow restoration in infants with extrahepatic biliary atresia. 351 84

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.
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PMID:Corticosteroid therapy in biliary atresia. 408

Hepatic secretory functions were measured postoperatively in 17 infants undergoing hepatoportoenterostomy for biliary atresia. These studies were compared in surviving and nonsurviving infants to attempt early identification of infants who might require reoperation. Statistically significant differences between surviving and nonsurviving infants were found in alkaline phosphatase clearances at 1, 4, 12 and 24 weeks postoperatively. In addition, bilirubin clearance and secreted 24 hour bilirubin measurements were significantly increased in survivors 12 and 24 weeks postoperatively. Iodine-131 rose bengal excretion, measured 6 months postoperatively, was likewise significantly increased in survivors. Six of the 10 surviving infants required reoperation when their secretory functions deteriorated; all improved postoperatively. None of the seven nonsurviving infants underwent reoperation. Biliary secretory functions have early prognostic significance and apparently can predict which infants require reoperation. Reexploration and higher transection of atretic ducts may improve survival.
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PMID:Prognostic determinants after hepatoportoenterostomy for biliary atresia. 745 28


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