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Tagge D U - - 1991
Thirty-four infants with biliary atresia were primarily treated at our institution between 1974 and 1987. The mean age at diagnosis was 8.8 weeks. The Kasai portoenterostomy was used in 11 patients (32%) and the Sawaguchi modification in 23 infants (68%). Overall survival was 47% (16/34), with a mean follow-up of ...
Gaboardi F - - 1991
A 32-year-old man with azoospermia is presented. Transperineal echographically guided vesiculography of the vas deferens showed absence of the ejaculatory duct and presence of a cystic müllerian duct in which both enlarged seminal vesicles joined. Transurethrally, by means of a contact tip over the verumontanum a new channel was created ...
Glassman M S - - 1991
A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants ...
Davenport M - - 1991
Spontaneous perforation of the bile duct is a rare but well documented condition of infants. Six infants are described, five presenting within 2 months of birth without apparent antecedent factors and one associated with an 'acquired' type I biliary atresia at 8 months. All infants underwent definitive surgery, which included ...
Guelrud M - - 1991
This study assessed the usefulness of ERCP in the diagnosis of biliary atresia. We evaluated 57 infants with prolonged cholestasis with abdominal ultrasound, liver biopsy, and ERCP. Using clinical observations alone, 22 infants were thought to have biliary atresia; whereas 35 children were thought to have neonatal hepatitis. The ERCP ...
Matsura T - - 1991
In order to determine whether coenzyme Q (CoQ) homologs which coexist in mammals play the same or different roles, the concentrations of coenzyme Q9 (CoQ9) and coenzyme Q10 (CoQ10) were analyzed in Japanese White (JW) rabbit tissues during growth, together with the intracellular distribution of these two CoQ homologs. In ...
Geller J D - - 1991
Diffuse neonatal hemangiomatosis is a rare, frequently fatal disease characterized by multiple cutaneous and visceral hemangiomas. The skin, liver, lungs, intestines, and central nervous system are the most commonly affected organs. Death can result from high-output cardiac failure as a result of arteriovenous shunting. In this report we present a ...
Yoshida M - - 1991
Neonatal guinea pigs were exposed to mercury vapor on the day of birth, and the following mercury distribution and concentrations of metallothioneins (MTs) in the liver and kidney were observed. In the liver, two hours after exposure, an appreciable amount of mercury accumulated and a substantial portion of this was ...
Krishna L - - 1991
An outbreak of aflatoxicosis in Angora rabbits involving a large number of rabbitries was investigated. Mortality was more in weaners than in adults. Affected animals showed anorexia, dullness and weight loss followed by jaundice in terminal stages. Death occurred within 3-4 d of the appearance of clinical signs. Livers were ...
Tazawa Y - - 1991
To clarify the relationship between hyperbilirubinaemia and abnormal results of biochemical liver function tests in infants with breast milk jaundice (BMJ), 58 breast-fed infants with indirect hyperbilirubinaemia were enrolled in this study. Sera obtained from the above infants were subjected to routine liver function tests. Although serum transaminases were within ...
Pittschieler K - - 1991
From 1985 to 1988 14,938 newborns were screened during the first days of life to determine their protease inhibitor phenotype (Pi) and 467 PiMZ and 456 PiMS were identified. Of these 101 PiMZ and 135 PiMS were followed-up and their clinical, biochemical and, in selected cases, histological data were recorded ...
Smith C L - - 1991
In plasma, glucocorticoids are transported by corticosteroid-binding globulin (CBG), which is synthesized primarily in the liver. Plasma levels of maternal and fetal CBG fluctuate during gestation, and this may be due to changes in the biosynthesis and/or clearance of the protein. We have, therefore, studied the ontogeny of CBG biosynthesis ...
Kristidis P - - 1991
Infantile hepatic haemangiomas are benign tumours which tend to regress in late infancy. We describe five infants with hepatic haemangioma, three of whom developed congestive cardiac failure. Technetium-99m labelled red blood cell scan was useful in the diagnosis and follow-up of the lesion. The three symptomatic infants were treated with ...
Wilkinson M L - - 1991
The difficulty of distinguishing surgically correctable causes of conjugated hyperbilirubinaemia in infants from other causes means that some infants may undergo laparotomy and intraoperative cholangiography unnecessarily, and others may be referred for surgery too late. In an attempt to improve the diagnostic accuracy in infants with conjugated hyperbilirubinaemia when standard ...
Bux J - - 1991
A significant association of autoimmune neutropenia (AIN) in infants due to NA1-specific autoantibodies and HLA-DR2 is reported. Nineteen of 26 infants presenting with AIN and NA1 autoantibodies possessed the DR2 antigen, while only one out of six children with AIN, but non-NA1-specific autoantibodies, was DR2 positive. Furthermore, one adult patient ...
Furuya K N - - 1991
A male infant presenting with neonatal hepatitis syndrome, characterized by conjugated hyperbilirubinemia and very mild liver function test abnormalities, at 2 weeks of age was found to have no excretion of radioisotope into the intestinal tract on hepatobiliary scan. Liver biopsy revealed severe interlobular bile duct paucity. Other features of ...
Garel D - - 1991
An infant presented with enlarged liver and spleen and profound pancytopenia. Severe marrow fibrosis impeded the diagnosis which was achieved by liver biopsy: Fibrosis surrounded foci of blasts which were shown by immunologic markers to originate from the megakaryocytic lineage. Complete sustained remission was obtained by low-dose aracytine therapy. The ...
Szilágyi L - - 1991
The case of a 3-month-old premature infant deceased by massive hemorrhage from a giant hemangioma of the right thigh, exhibiting also biliary lithiasis, is discussed. The six dark-green-blackish faceted calculi contained by the gall bladder appear to be formed of biliary pigment and the consequence of repeated inflammatory hemolytic episodes ...
Pittschieler K - - 1991
Low levels of alpha-1-antitrypsin can predispose deficient infants to the development of hepatitis and cirrhosis. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement during their first half year of life. One pathogenic hypothesis of liver damage is that the process seems to be mediated by the activity ...
Takehara H - - 1990
Cholestasis associated with total parenteral nutrition (TPN) has been reported to be an incidence of 15% to 50% in surgical neonates. This study was designed to prevent the liver dysfunction, especially intrahepatic cholestasis, caused by TPN for surgical neonates. In the last three years, 10 surgical neonates ranged from 1868g ...
Jonas A - - 1990
During the 3-year period, January 1986 through December 1988, 7 of 24 infants evaluated for cholestatic liver disease were found to have choledocholithiasis with or without associated gallbladder disease. Five were born prematurely with associated predisposing factors, especially systemic infection and total parenteral nutrition. The clinical course varied from spontaneous ...
Blair G K - - 1990
Intussusception occurs most commonly between the fifth and ninth month of life. Affected infants are usually healthy and born at full gestation age. We describe a case of intussusception occurring in a 10-day-old, 700-g neonate born at 28 weeks' gestation. The diagnosis was made at laparotomy 7 days after colostomy ...
Netherland D - - 1990
Hydrostatic reduction of intussusception is definitive therapy in most infants with this abnormality. In the older child, adolescent, and adult, a polyp or tumor is often present. Operative intervention should be considered earlier in the clinical course both to relieve the intussusception and to define the nature of the lead ...
Hancock B J - - 1990
Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included ...
Holgersen L O - - 1990
Three infants aged 2 days to 11 weeks with conjugated hyperbilirubinenemia, had sonographically documented dilated common hepatic bile ducts, and echogenic material in the gallbladder. A 2-day-old infant, born to a diabetic mother, had none of the classic predisposing factors for cholelithiasis, and two infants had received total parenteral nutrition ...
Chilukuri S - - 1990
Spontaneous perforation of the biliary system is an unusual neonatal phenomenon that is rarely recognized at birth. To date, it has not been reported antenatally. A 16-year-old pregnant adolescent had an ultrasonogram at 25 weeks of pregnancy that revealed ascites in the fetus. After the infant was delivered at 32 ...
Yoshida A - - 1990
Previous reports suggested that the major cytosolic aldehyde dehydrogenase (ALDH1) was present in fetal and infant livers, but the major mitochondrial isozyme (ALDH2) was absent or severely diminished. Re-examination by means of starch gel electrophoresis followed by enzyme activity staining, and by means of dot blot immuno-hybridization of liver samples ...
Brzezińska-Slebodzińska E - - 1990
The maturation of the 5'- and 5-monodeiodinase system in liver, kidney and brown adipose tissue of rabbits, during the foetal period (from 21 days of gestation to birth) and the neonatal period (from birth to 3 weeks of life) was studied. A sudden increase of 5'- and 5-monodeiodinase activity in ...
Fuller C E - - 1990
The relationship between immunohistochemically demonstrable hepatic metallothionein (MT) and age was studied in histologically normal livers from 12 fetuses (13-28 weeks' gestation) and 19 children (birth-4 years). In addition, the relationships between copper (rubeanic acid-positive), copper-associated protein (orcein-positive), lipofuscin (periodic acid Schiff after diastase digestion and Schmorls-positive), and MT (immunostaining) ...
Fung K P - - 1990
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 ...
Flye M W - - 1990
Two infants with hereditary tyrosinemia secondary to fumarylacetoacetate hydrolase (FAH) deficiency underwent orthotopic liver transplantation at 14 and 16 weeks of age due to poor clinical and biochemical response to medical therapy. Prompt clearance of abnormal metabolites with improved mental alertness and appetite occurred with minimal perioperative complications. Both infants ...
Brown S C SC Department of Paediatrics and Child Health, University of the Orange Free State, - - 1990
Visual inspection of stools for the presence or absence of bile pigment was evaluated prospectively to determine its value in differentiating between biliary atresia and hepatitis. In a group of 23 infants with prolonged neonatal cholestasis the presence of stool pigment excluded extrahepatic biliary atresia, while its absence was suggestive ...
Iles R A - - 1990
Localized 31P NMR spectroscopy was used to study the developing human liver in three neonates and one infant, all with neonatal intracranial problems, but normal liver function. A prominent resonance was present in the phosphomonoester (PME) region of the spectrum of the neonates; the PME/ATP ratio was 1.0 +/- 0.4 ...
Pawłowska J - - 1990
Serum concentrations of bile acids and bilirubin, and activity of alanine transferase and alkaline phosphatase as well as bile acid and bilirubin levels in duodenal contents were determined in 90 infants aged 1-44 weeks (including 49 under 10 weeks of age) admitted to hospital for prolonged jaundice. Infants with extrahepatic ...
Verbeke C - - 1990
Graphic reconstruction of dilated intrahepatic lymphatics in a 36 week old fetus displayed an anastomosing network of channels with intraluminal valves. Examination of the liver in 42 fetuses between the gestational age of 15 to 40 weeks showed that lymph vessels were visible as early as the 15th week. The ...
Brown D M - - 1990
Common bile duct obstruction, in an infant with bile plug syndrome, was relieved at surgery with a mucolytic agent, after an unsuccessful attempt at clearing the biliary tree with saline irrigation. This procedure obviated choledochotomy in this patient, and may be of use in other infants with ductal obstruction complicating ...
Laxer R M - - 1990
We report the cases of neonatal lupus erythematosus associated with significant hepatic involvement in three living infants and in one infant who died 3 hours after delivery. The three living infants had neonatal cholestasis as a major component of their clinical findings. Pathologic changes included giant cell transformation, ductal obstruction, ...
Rosenthal P - - 1990
To assess the utility of the serum aspartate aminotransferase/alanine aminotransferase (AST/ALT) ratio in a group of infants with liver disorders, we retrospectively analyzed the charts of 73 infants with chronic liver disorders. Patients were considered as having either a good outcome (n = 40) or a poor outcome (n = ...
Becroft D M - - 1990
Two infants with Down syndrome, one 4 weeks old and the other stillborn, at necropsy showed hepatic and pancreatic fibrosis, which was very severe in the liver of the liveborn infant and in the pancreas of the stillbirth. The liveborn infant had typical hematological features of the transient congenital leukemoid ...
Hagenmaier H - - 1990
A defined mixture of polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDDs and PCDFs) was subcutaneously administered to a pregnant marmoset monkey (Callithrix jacchus) 11 weeks prior to delivery. Transfer of PCDDs and PCDFs via placenta and mother's milk was investigated by measurement of concentrations in a newborn 1 day after birth and ...
Bielańska-Osuchowska Z - - 1990
Quantitative changes in the developing liver of the pig embryo have been studied in the light microscope by stereological methods. Between days 18 and 112 of gestation the volumetric fraction of hepatocytes in the liver significantly increased and the volumetric fraction of blood vessels decreased. The haemopoietic cells reached the ...
Conover P T - - 1990
Disseminated intravascular coagulation (DIC) and other clotting abnormalities are common in sick newborn infants who have a variety of conditions. To document evidence of DIC at autopsy, immunoperoxidase staining of fibrin-related antigens (FRA) was used to detect intravascular microthrombi in liver, kidney, and lung from 127 newborns. Patients were selected ...
Drongowski R A - - 1989
The risk of developmental of total parenteral nutrition (TPN)-associated cholestatic jaundice in neonates receiving intravenous hyperalimentation is high. Numerous factors have been cited as contributing to TPN cholestasis; however, the exact etiology remains obscure. This retrospective study was undertaken in order to identify any factors which might contribute to this ...
Tolia V - - 1989
A prospective study was undertaken on 49 infants with prolonged neonatal cholestasis to determine whether the specificity, sensitivity, and accuracy of 99mTc-disofenin scintigraphy can be improved by performing simultaneous radioactivity measurements on duodenal and gastric aspirates along with routine imaging. Specificity of routine scintigraphy was improved by this modification from ...
Baill I C - - 1989
A recently submitted placenta was found to be histopathologically unique. Funicitis was present at the early gestational age of 22 1/7 weeks, and an abscessed allantoic duct remnant was present. The latter finding had not previously been seen at this pathology department, nor could reference to it be found in ...
Metzman R - - 1989
A hydropic premature infant with intrauterine growth retardation died at 4 days of age and was found at necropsy to have advanced liver disease. Clinical and serologic findings in mother and infant were consistent with recent parvovirus B19 infection. Parvovirus can cause fetal liver disease in animals, and some instances ...
Vajaradul C - - 1989
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 ...
Kahn E - - 1989
Paucity of interlobular bile ducts is a common feature of cholestatic liver disease in premature infants. Whereas a bile duct to portal space ratio of 0.9 to 1.8 is cited by Alagille as the norm for children, there are no data regarding the normal bile duct to portal space ratio ...
Beckett G J - - 1989
Concentrations of glutathione S-transferase (glutathione transferase; EC 2.5.1.18) B1 and B2 subunits (B1 and B2) and activity of alanine aminotransferase (ALT; EC 2.6.1.2) were measured in sequential plasma samples taken from 14 infants with birth asphyxia. Within 6 h of asphyxia, abnormal concentrations of B1 were found in 11 infants, ...
Brotman B - - 1989
Two chimpanzees were born to parents with chronic non-A, non-B hepatitis and remained with their mothers until 12 and 18 months, respectively. The infants were followed from 7 to 8 weeks of age with biweekly or monthly blood samples and with monthly liver biopsies from 4 to 7 months after ...
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