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Celayir S - - 1998
Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile ...
Clayton P T - - 1998
Long-term parenteral nutrition of infants who have had major gut resections is associated with a high incidence of cholestatic liver disease. Affected infants have high plasma concentrations of phytosterols--compounds that resemble cholesterol but have an alkylated side chain. The phytosterols that accumulate in patients receiving parenteral nutrition are derived from ...
Karnameedi S - - 1997
Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary ...
Sokol R J - - 1997
Administration of parenteral nutrition to the small infant has decreased morbidity but is associated with the development of cholestasis and liver dysfunction in a high proportion of infants. In this review, contributing factors of the etiopathogenesis of parenteral nutrition-associated liver disease will be outlined, forming the basis for a unifying ...
Park W H - - 1997
BACKGROUND/PURPOSE: The authors evaluated prospectively the utility of ultrasonography, Tc-99m-DISIDA hepatobiliary scintigraphy, and liver needle biopsy in differentiating biliary atresia (BA) from intrahepatic cholestasis in 73 consecutive infants who had cholestasis. METHODS: Sixty three ultrasonographic examinations of 61 infants with 7.0-MHz transducer were carried out, focusing on the fibrous tissue ...
Siafakas C G - - 1997
Inborn errors of bile acid synthesis are newly recognized disorders that may cause the phenotypic appearance of neonatal hepatitis or neonatal cholestasis. This is a clinicopathologic study of two sets of siblings with cholestatic neonatal liver failure. In 3 of the infants, diagnostic evaluation, including analysis of urinary bile salts, ...
Scher H - - 1997
OBJECTIVE: To describe two infants with cholestatic jaundice treated with ursodeoxycholic acid (UDCA). CASE SUMMARY: Two infants with cystic fibrosis (CF)-associated hepatobiliary disease, manifesting as cholestatic jaundice and elevated liver enzymes within the first 6 weeks of life, had improved biochemical indices of liver function following treatment with UDCA 20-40 ...
Vajro P - - 1997
OBJECTIVE: The aim of this study was to characterize the liver involvement in infants who have both neonatal asphyxia and neonatal cholestasis. METHODS: We describe four asphyctic newborn infants (three born at term) who developed early (age 3.8 +/- 2.1 days) intrahepatic neonatal cholestasis and in whom tests for causes ...
Watanabe T - - 1997
A case is reported of sudden rupture of a subcapsular hematoma of the liver (SHL) due to birth injury occurring after complete repair of a total anomalous pulmonary venous connection in a 3-day-old male infant. Autopsy showed a wide bruised surface of liver parenchyma. Although making a diagnosis of SHL ...
Mirkin L D - - 1997
Hyaline cartilage was found on microscopy of sections of the extrahepatic biliary tree in two infants with extrahepatic biliary atresia (EHBA). Respiratory epithelium was not present, and the cartilage did not seem to block the bile duct lumen. Hyperbilirubinemia was manifest in one infant on the second postnatal day, but ...
Schambeck C M - - 1997
We report on a 27-year-old healthy female with transient hyperphosphatasaemia of adulthood (it is the eighth case ever recorded). A maximum alkaline phosphatase activity of 1950 U/l, 11-fold the upper reference limit, was measured. The activity normalized within 11 weeks. Electrophoresis revealed the typical pattern for alkaline phosphatase isoenzymes observed ...
Abdullah A M - - 1997
In the King Khalid University Hospital (Central Province) and King Fahad Hospital of the University (Eastern Province) Saudi Arabia, we identified 64 infants with cholestasis. The causes of cholestasis were idiopathic neonatal hepatitis in 29; extrahepatic biliary atresia in 17; neonatal hepatitis secondary to Rubella and Cytomegalovirus in six and ...
Uchiyama K - - 1997
The gallbladder ejection fraction (GBEF) obtained with Tc-99m-pyridoxyl-5-methyl-tryptophan (99mTc-PMT) hepatobiliary scintigraphy has been used as a parameter of gallbladder function. To determine the accuracy of GBEF, the relationship with the contraction ratio of the gallbladder (GBCR) obtained with three-dimensional helical computed tomography (3D-CT) was studied. PATIENTS AND METHODS: A normal ...
Ohnuma N - - 1997
BACKGROUND: It is not easy to discriminate between infantile hepatitis and biliary atresia in spite of several diagnostic tests including laboratory analyses, ultrasound, and hepatobiliary scans. ERCP is the most useful procedure for visualization of the extrahepatic biliary system, but ERCP is still an uncommon procedure in children. METHODS: ERCP ...
Yoon P W - - 1997
OBJECTIVE: Biliary atresia is the leading cause of extrahepatic obstructive jaundice in the newborn and is the single most frequent indication for liver transplantation in children. The cause of biliary atresia is unknown, although several mechanisms have been postulated to explain the inflammatory process that obliterates the bile ducts. Most ...
Greenholz S K - - 1997
Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three ...
Kreuzer P E - - 1997
Contents of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) and of 16 further congeners--polychlorinated dibenzodioxins and dibenzofuranes (PCDD/PCDF)--were determined in lipids of adipose tissue and of livers of 3 stillborns and of 17 infants (0.43-44 weeks old) who died from sudden infant death syndrome. International toxic equivalents (I-TEq) calculated for the sum of TCDD together ...
Tateishi T - - 1997
We immunochemically measured the contents of 9 different cytochrome P450 (CYP) isoenzymes expressed in the liver and compared them between two groups: one group of 6 infant and 4 perinatal patients and one group of 10 patients after infancy (over 1 year old). CYP protein expressed in human liver can ...
Matsui A - - 1996
Two infants with biliary atresia who exhibited three-fold increased trough levels of tacrolimus and required reduced doses during episodes of acute infantile diarrhea within 5 months of liver transplantation are described. The cause of the increase was not explained simply by hemoconcentration as a result of significant loss of extracellular ...
Haque A K - - 1996
An autopsy study was conducted to investigate whether there is transplacental transfer of asbestos in humans. The asbestos burden of lung, liver, skeletal muscle, and placenta digests of 40 stillborn infants was determined using a bleach digestion method. The fibers detected in the tissue digests were characterized as to the ...
Papadopoulou F - - 1996
OBJECTIVE: Inversion of the superior mesenteric vessels is associated with various conditions. The purpose of this study was to prospectively evaluate the position of these vessels in infants with idiopathic ileocolic intussusception. SUBJECTS AND METHODS: Abdominal sonography was performed before and after reduction of ileocolic intussusception in 16 infants. RESULTS: ...
Yamagiwa I - - 1996
In order to investigate the possibility of early discrimination of extrahepatic biliary atresia from other cholestatic diseases, a series of results of liver function tests in infants with cholestatic diseases were reviewed. The results of routine liver function tests (LFT) recorded in patients' charts were reviewed within 12 weeks after ...
Bunchman T E - - 1996
The care of an infant with primary hyperoxaluria and oxalosis is discussed. After an unheralded presentation, followed by 9 months of intensive treatment that included combined hemodialysis and peritoneal dialysis, the infant successfully underwent combined liver and kidney transplantation to definitely address both kidney failure and the underlying metabolic defect. ...
Shneider B L - - 1996
Neonatal liver failure (NLF) is a distinct and unusual clinical syndrome that requires a specialized approach to its management. This review establishes a definition of NLF and provides a literature-supported differential diagnosis. A specific approach to the diagnostic work-up of an infant with NLF based on this differential diagnosis is ...
Davenport M - - 1996
A number of features (eg, age at time of surgery, size of microscopic biliary ductules) have been suggested to be useful predictors of the efficacy of portoenterostomy in the treatment of biliary atresia. However, no previous study has attempted to quantify the macroscopic appearance of the degree of hepatobiliary damage ...
Yachha S K - - 1996
OBJECTIVE: To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS). DESIGN: Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years. SETTING: Tertiary level ...
Halpern Z - - 1996
In the past, biliary lipids of infants and children were studied in duodenal aspirates. This study was performed on original bile aspirated from the gallbladder. The analysis of lipids in bile demonstrated a significantly lower total lipid content in the bile of infants than in children (3.3 g/dl vs. 9.1 ...
Araikum S - - 1996
A compartmental model describing the extraction and disposition of 99mTc-acetanilidoiminodiacetic acid (IDA) compounds by the liver has been applied to 5 adult patients admitted for cholecystitis investigations and 29 jaundiced infants the majority of whom were clinically differentiable into neonatal hepatitic and biliary atretic groups. METHODS: In each case kinetic ...
Coni P - - 1996
The newborn liver is a proven model for the study of liver storage of copper and iron. We analyzed zinc concentration and distribution in the livers of newborns and infants using a systematic tissue-sampling technique. We studied 14 newborns of 26-41 weeks of gestation (WG). One stillborn, and three infants ...
Winslow B T - - 1996
Intussusception is the invagination of one bowel segment into another. It is an emergent condition that most commonly affects infants between five and nine months of age, but it can also occur in other age groups. The etiology is usually idiopathic in infants five to nine months of age; neonates, ...
Wu T J - - 1996
To evaluate the incidence, clinical course, and possible risk factors of cholestasis in very low-birth-weight infants. A retrospective study of 143 very low-birth-weight infants was performed. Cholestasis was defined as direct-reacting bilirubin > 2 mg/dL for more than 14 days. The clinical course of cholestasis was described, and perinatal risk ...
Ishitani M B - - 1996
Choledocholithiasis in neonates and infants has been reported only rarely. Infants with complications of prematurity are more predisposed to development of biliary calculi. With the current widespread use of diagnostic ultrasonography, more neonates may be found to have gallstones and common bile duct stones. We describe a case of choledocholithiasis ...
Andres J M - - 1996
Neonatal hepatobiliary disorders are now better understood due primarily to new discoveries in molecular genetics, virology, and immunology. Because they are almost always pathologic and require early intervention, the neonatologist must recognize infants with these hepatocellular and ductal cholestatic problems occurring in the first few weeks of life. This article ...
Penrith M L - - 1996
Ataxia associated with myelopathy and low liver copper concentrations is described in a blesbok (Damaliscus dorcas phillipsi) and black wildebeest (Connochaetes gnou) from the Karoo Nature Reserve, Graaff-Reinet. This syndrome, which occurs in neonatal and delayed forms, has been described in sheep, goats, pigs and domesticated red deer, but reports ...
Park W H - - 1996
Eleven cases of biliary atresia (BA) and eight of neonatal hepatitis (NH) were studied, using transmission electron microscopy, to document their different ultrastructural characteristics and to elucidate the possible pathogenesis of biliary atresia. Among 30 consecutive liver biopsies obtained from 19 infants with BA or NH, 21 specimens composed (13 ...
Kok T - - 1996
The ability of ultrasound to detect biliary obstruction, bile leakage and generalized ductal changes after orthotopic liver transplantation (OLT) was compared to cholangiography. Cholangiography was considered to be the gold standard. Adequate opacification of the biliary tree was achieved in 139 cholangiograms. Biliary obstruction, intermediate or large bile leakage, and ...
Suchy F J - - 1996
Extrahepatic biliary atresia is a disease of unknown cause, leading to profound cholestasis and progres-sive biliary cirrhosis. This paper discusses the diagnosis and management of this condition. It is stressed that bile flow can be established in 80-90% of infants referred to surgery within 60 days after birth. Liver transplantation ...
Roberts E A - - 1995
Family physicians should see all infants between 2 and 4 weeks of age to exclude persistent jaundice. Early identification of infants with jaundice due to liver disease might lead to better outcomes not only for infants with extrahepatic biliary atresia but for all infants with neonatal liver diseases. The case ...
Forchielli M L - - 1995
Cholestasis often occurs in infants on total parenteral nutrition (TPN) for long periods. Amino acid formulations developed specifically for infants, namely Aminosyn PF and Trophamine, may protect against cholestasis associated with total parenteral nutrition (CATPN). The development of cholestasis may also be caused by other risk factors such as prematurity, ...
Stein B - - 1995
PURPOSE: To determine the importance of intrahepatic calcifications seen sonographically in the fetus. MATERIALS AND METHODS: The authors retrospectively reviewed sonograms obtained in fetuses scanned between 16 and 40 gestational weeks in whom intrahepatic calcifications were seen. The number and character of these calcifications were determined. Follow-up scans obtained in ...
Jacquemin E - - 1995
We report 18 premature infants (gestational age: 31.1 weeks +/- 2.6 [mean +/- SD] (range: 28-36]) with necrotizing enterocolitis (NEC) who developed total parenteral nutrition (TPN) associated cholestasis. Liver function tests were performed at the start of TPN (D1) and repeated once a week. Considering the date of cholestasis onset ...
Jazrawi R P - - 1995
BACKGROUND & AIMS: Impaired gallbladder emptying is implicated in gallstone disease. Ultrasonography and scintigraphy have shown conflicting results because the former is influenced by postprandial refilling, whereas the latter is not influenced by refilling. The aim of this study was to measure postprandial refilling and turnover of bile by combining ...
Harris J - - 1995
A study has been made on certain epidemiological characteristics of infants with alimentary tract atresia: esophageal atresia, small and large gut atresia, and anal atresia. Data were collected from three malformation registries and represent a material of more than 4.5 million births. A total of 3,550 infants with alimentary atresia ...
Lugo Vicente H L - - 1995
Biliary Atresia is the most common cause of persistently direct (conjugated) hyperbilirubinemia in the first three months of life. It is a progressive inflammatory obliteration of the extra- and intrahepatic bile ducts whose pathogenesis remains speculative. This review updates the history, etio-pathological considerations, clinical manifestations, methods of diagnosis, surgical management, ...
Endo M - - 1995
This study was undertaken to define the role of an ileocolic conduit that was devised as anti-reflux procedure in comparative study with Roux-en-Y type reconstruction in infants with biliary atresia treated at a single institution. The ileocolic conduit (IC) consisted of 30 cm of distal ileum anastomosed to the portahepatis ...
Fu L S - - 1995
A three-month old Chinese male infant was a victim of neonatal hepatitis presenting with prolonged jaundice, poor body weight gain, progressive hepatosplenomegaly and extremely elevated serum alpha-fetoprotein level. Niemann-Pick disease (NPD) type C was confirmed by autopsy, which revealed sphingolmyelin deposition in multiple visceral organs, and normal sphingomyelinase activity in ...
Lamont A C - - 1995
RATIONALE AND OBJECTIVES: Ultrasonic image analysis aims to provide information related to tissue histology. Routine ultrasonography uses subjective criteria for analysis. Quantitation of the image is expected to aid diagnosis and may be helpful in follow-up of pathology. The authors describe normal findings in infant liver and kidney. METHODS: Texture ...
Bhagwat S S - - 1995
Isolated jejunal varices are an uncommon manifestation of portal hypertension. A one and a half year old boy presented with recurrent, massive gastrointestinal bleeding from jejunal varices. The bleeding site was identified at exploratory laparotomy. Jejunal resection and anastomosis resulted in complete resolution of the bleeding and there has been ...
von Schweinitz D - - 1995
We report on 26 infants under 3 months of age with various liver tumors, who were treated in our hospital since 1977 and/or registered in the German Cooperative Pediatric Liver Tumor Study HB-89. 17 of these had an infantile hemangioendothelioma (inf HE), 7 a hepatoblastoma (HB), one a mesenchymal hamartoma ...
Bruce S - - 1995
A preterm infant presented with high-output cardiac failure. Color Doppler imaging of the liver revealed a large hepatic hemangioma. Pulsed-wave Doppler of the proximal aorta was used to monitor noninvasively the reduction in flow through the arteriovenous malformation in response to steroid therapy. The hemangioma resolved completely by 7 weeks ...
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