Search Results
Results 551 - 600 of 1008
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Miyazaki T - - 2001
Propionic acidemia (PA) is an inborn error of metabolism caused by the genetic deficiency of propionyl-CoA carboxylase (PCC). By disrupting the alpha-subunit gene of PCC, we created a mouse model of PA (PCCA(-/-)), which died in 24-36 h after birth due to accelerated ketoacidosis. A postnatal, liver-specific PCC expression via ...
Amicarelli F - - 2001
This work is aimed at detecting the expression and location of embryonic Bufo bufo GST (bbGSTP1-1) and adult B. bufo GST (bbGSTP2-2) during toad development, in order to assign a putative role to these enzymes also on the basis of their compartmentalization and to verify whether during the premetamorphic liver ...
Bezerra J A - - 2001
Cholestasis results from structural and functional impairment of the hepatobiliary system, which is often the target of several environmental factors and disease processes. This review focuses on the clinical consequences of this impairment. When evaluating an infant or child with cholestasis, a broad differential diagnosis must be considered; viral infections, ...
Larrosa-Haro A - - 2001
BACKGROUND: Biliary atresia (BA) is the main cause of severe liver damage in infants. Successful surgical treatment is related directly to an early and rapid diagnosis. The aim of this study was to determine specificity, sensitivity, and predictive value of the duodenal tube test (DTT) in the diagnosis of BA ...
Senyüz O F - - 2001
BACKGROUND: The early diagnosis of surgical jaundice in a neonate is an important step for the surgical success in extrahepatic biliary atresia. Diagnostic laparoscopy, as in many areas in surgery, is included in the conventional diagnostic methods of extrahepatic biliary atresia. METHODS: Since 1992, 24 infants with prolonged jaundice, in ...
Stormon M O - - 2001
OBJECTIVE: Cholestatic liver disease in infancy is caused by a wide range of conditions. This study reviews the pattern of diagnosis of infants with cholestasis presenting to a tertiary referral paediatric hospital in Sydney, Australia, during a 12-year period (1985-96). METHODOLOGY: Infants aged less than 6 months with cholestasis were ...
Tajiri H - - 2001
We present 3 cases of immunocompetent infants with CMV infection who showed prolonged liver dysfunction. In all cases the CMV genome was detectable in hepatocytes using the in situ hybridization method. Combination therapy with ganciclovir (GCV) and hyperimmune gammaglobulin (HGG) was instituted in 2 cases and successfully suppressed the replication ...
Shaw S - - 2001
Survival following hemorrhagic necrosis of a lobe of the liver in preterm infants is very uncommon. We present the clinical and radiologic findings of such a case, discuss the clinical management of the hemorrhage that resulted in the infant's survival, and present a basis for the evolution of the liver ...
Fischler B - - 2001
The aim of the study was to investigate factors of possible importance for the aetiology of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. The most common diagnoses were extrahepatic biliary atresia (n = 30 patients), alpha1-antitrypsin deficiency (n = 11) and progressive familial intrahepatic cholestasis ...
Sreenan C - - 2000
OBJECTIVE: We report a newborn infant who was successfully treated with chelation therapy having developed severe liver disease secondary to iron overload following multiple intrauterine, intravascular transfusions (IVTs). STUDY DESIGN: Case report with review of the literature. RESULTS: An infant was born at 33 weeks' gestation having received multiple IVTs ...
Farrant P - - 2000
An initial investigation of 313 patients suggested that the ultrasound appearances of gall bladder shape and wall structure might be abnormal in infants with biliary atresia. These observations were then tested in a prospective study. In this study, 346 infants, aged 12 weeks or less, presenting with conjugated hyperbilirubinaemia underwent ...
Noguchi H - - 2000
The expression of tenascin in the extrahepatic bile duct remnants in biliary atresia was investigated using an immunohistochemical method. Tenascin is thought to be a marker of repair of injured tissues based on an immunohistochemical study of a variety of tissues. Twenty-three specimens were obtained from 23 cases (2 correctable ...
Kimura T - - 2000
Although transjugular intrahepatic portosystemic shunt (TIPS) is widely accepted in adults, there have been few successful reports in infants. The authors describe a 2-year-old boy with postoperative biliary atresia who underwent TIPS for uncontrollable lower intestinal bleeding and achieved hemostasis. Massive melena developed, which was not controlled by conservative therapy ...
Wagner C - - 2000
STUDY OBJECTIVE: To determine those infants at high risk for perioperative complications and mortality following living, related liver transplantation. DESIGN: Retrospective chart review. SETTING: Large metropolitan teaching hospital. MEASUREMENTS AND MAIN RESULTS: The charts and anesthetic records of the 12 infants and children who received the left lateral hepatic segment ...
Tomar B S - - 2000
Hepatobiliary dysfunctions (TPN-HBD) occur during parenteral nutrition. In older children these are usually reversible whereas in newborns and infants these hepatobiliary abnormalities play a significant role in the morbidity. Cholestasis is a commonly occurring TPN-HBD. It correlates directly with the decreasing gestational age, low birth weight and increasing duration of ...
Vijayan V - - 2000
BACKGROUND/PURPOSE: In biliary atresia (BA), although the intrahepatic bile ducts are reported to appear in the fetal configuration in about 20% to 60% of cases, information about the 3-dimensional (3-D) morphology of the hilar biliary structures is limited. The authors, therefore, have applied the technique of computer-generated 3-D reconstruction, to ...
Spray C H - - 2000
Congenital hypopituitarism is a recognized cause of neonatal hepatitis, but the diagnosis may be difficult to establish even if clinically suspected. In order to determine the natural history of this disorder, the outcome of 12 infants with neonatal hepatitis secondary to hypopituitarism is reviewed. The clinical diagnosis of hypopituitarism was ...
Hume R - - 2000
BACKGROUND: At discharge from neonatal units, many preterm infants are vulnerable to preprandial hypoglycemia due to insufficient liver glucose production. In most preterm infants, hepatic glucose-6-phosphatase activity (the terminal step of liver glucose production) remains abnormally low postnatally. OBJECTIVE: To determine what perinatal factors are associated with changes in hepatic ...
Lee C H - - 2000
The purpose of this study was to determine whether gallbladder visualization can help exclude biliary atresia in hepatobiliary scintigraphic studies of infants with persistent jaundice. METHODS: One hundred fifty-two infants with persistent jaundice (49 patients with a final diagnosis of biliary atresia and 103 with biliary patency) were studied using ...
Hollifield J L - - 2000
Systemic erysipelas infection was seen in 2-to-4-day-old poults from three separate ranches owned by the same company. The affected poults were all from the same breeder source; poults from other breeder sources were seemingly unaffected. Mortality increased on days 2 and 3, ranging from 2% to 8.5%. Birds submitted were ...
Uges D R - - 2000
A patient is described who died with the diagnosis of septicemia. After her death the delayed results of a clozapine determination for TDM were sent to the clinician. The clozapine serum level was 4034 microg/L, which was considered to be the primary cause of death. However, a forensic autopsy revealed ...
Hartman B J - - 2000
Rupturing of the liver due to delivery is an uncommon but severe birth trauma. Although described in the preterm neonate with very low birthweight or after complicated delivery, we present a case of capsular liver haemorrhage in a term newborn with normal birthweight after a seemingly uncomplicated delivery. The infant ...
Shamir R - - 2000
BACKGROUND: Hyperbilirubinemia and liver enzyme abnormalities are commonly observed in sepsis. However, the frequency in premature neonates and the specific relation to gram-negative bacteria are not known. PATIENTS AND METHODS: Charts of all preterm infants who had positive blood cultures for either gram-negative bacteria or coagulase-negative staphylococci were reviewed. Neonates ...
Owings E - - 2000
Infants with very low birth weight (VLBW) are at increased risk of cholestasis when compared with older infants and children. Factors associated with this increased risk of cholestasis include immaturity of the biliary excretory system, a diminished immune response to sepsis, an increased incidence of necrotizing enterocolitis and short bowel ...
Lapillonne A - - 2000
BACKGROUND: The long-chain polyunsaturated fatty acid (LC-PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this reverses poor LC-PUFA status ...
Tan Kendrick A P - - 2000
BACKGROUND: To evaluate the accuracy and utility of the triangular cord sign and gallbladder length in diagnosing biliary atresia by sonography. MATERIALS AND METHODS: Sixty fasted infants with cholestatic jaundice aged 2-12 weeks were examined sonographically using a 5-10 MHz linear array transducer, focusing on the triangular cord sign (as ...
Ono S - - 2000
We report an 8-month-old male presenting with gingival hemorrhages and nasal bleeding as the first symptom of a choledochal cyst (CC). On physical examination, there was a large cystic mass in the right upper abdominal quadrant. Laboratory studies on admission revealed moderate liver dysfunction and a bleeding tendency due to ...
Gubernick J A - - 2000
High-resolution real-time ultrasonography (US) serves as an important tool for differentiation of obstructive and nonobstructive causes of jaundice in infants and children, independent of liver function. Unconjugated hyperbilirubinemia occurs in approximately 60% of normal term infants and in 80% of preterm infants. Persistence of neonatal jaundice beyond 2 weeks of ...
Green D W - - 2000
Extrahepatic biliary atresia (EHBA) is an uncommon condition presenting in the first few weeks of life. It has an incidence of 0. 5-1 per 10 000 live births and is the end result of a destructive inflammatory process involving the extrahepatic biliary system of unknown aetiology occurring in utero. The ...
Dassel de Vergara J - - 1999
During the past 20 years there has been much discussion about copper in connection with a form of Early Childhood Liver Cirrhosis (ECLC) known as Non Indian Childhood Cirrhosis (NICC). NICC is similar to Indian Childhood Cirrhosis (ICC) which occurs in India, and has already been known for many years. ...
Morris J - - 1999
BACKGROUND: Infantile hepatic hemangioma is a benign liver tumor with potentially fatal complications. Response of these lesions to steroid therapy has been well documented in infants. Successful antenatal treatment of a fetal hepatic hemangioma with maternal corticosteroids is reported. CASE: A 36-year-old woman, gravida 2, para 1, had an ultrasound ...
Tolia V - - 1999
PURPOSE: Hepatobiliary scintigraphy is used routinely to evaluate infants with neonatal cholestasis. Hepatobiliary scintigraphy determines biliary patency by detecting radioactivity in the bowel on imaging, in duodenal and gastric aspirates, or all of these. During hepatobiliary scintigraphy, the hepatocyte extraction fraction (HEF) is calculated by deconvolution analysis. Normal values of ...
Zeolla M M - - 1999
OBJECTIVE: To report a case of successful clopidogrel use in a patient who developed suspected ticlopidine-induced hepatotoxicity during therapy for a cerebrovascular accident. CASE REPORT: A 79-year-old white woman with a history of hypertension, type 2 diabetes, Alzheimer disease, and coronary artery disease started receiving ticlopidine 250 mg twice daily ...
Bianchi A - - 1999
Over a 16-year period, 20 neonates and infants with short-bowel syndrome underwent longitudinal intestinal lengthening and tailoring because of a dysfunctional dilated jejunum. There was no operative mortality, and morbidity was limited to 2 hemiloop anastomotic stenoses and 1 spontaneously resolving air and bile leak. Long-term survival was 45%. Survivors ...
Ghosh P - - 1999
Management of a patient with foreign body (FB) in the ENT areas is an art. FB in the respiratory tract is fraught with respiratory obstruction and even death, rarely though, especially in infants and children, if not intervened in time. Fair prediction can be made as to the site and ...
Bennett M J - - 1999
This report describes the clinical, biochemical, and pathological findings in three infants with hepatic short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase (SCHAD) deficiency, a recently recognized disorder of the mitochondrial oxidation of straight-chain fatty acids. Candidate subjects were identified from an ongoing study of infant deaths. SCHAD analysis was performed on previously frozen ...
Mandhan P - - 1999
Cholelithiasis in infants and children is unusual and is reputedly associated with underlying hemolytic disorders, total parenteral nutrition, ileal disease, and congenital anomalies of the biliary tree. We report a case of cholecystitis with pigmented stones in a 3-month-old infant associated with portal vein thrombosis (PVT) and biliary cirrhosis without ...
Gilbert-Barness E - - 1999
Isovaleric acidemia, an autosomal recessive disorder, is due to isovaleryl-coenzyme A dehydrogenase deficiency and is one of the branched-chain aminoacidopathies. Isovaleric acidemia may present in the neonatal period with an acute episode of severe metabolic acidosis, ketosis, and vomiting and may lead to coma and death in the first 2 ...
Halasz R - - 1999
The role of GB virus C/hepatitis G virus (GBV-C/HGV) in adult and pediatric liver disease is unclear. We detected serum GBV-C/HGV RNA by reverse transcriptase polymerase chain reaction in 1 (3%) of 38 cholestatic infants, in 4 (4%) of 95 children without liver disease, and in none of 30 children ...
Sergi C - - 1999
We describe the fatal course of a preterm infant of 34 weeks' gestation with veno-occlusive disease of the liver and refractory ascites. Despite aggressive medical management, the baby died twenty-two hours post partum because of cerebral haemorrhage before potentially life-saving organ transplantation could take place. At autopsy, paucity of lymphoid ...
Pelsang R E - - 1999
OBJECTIVE: Traditionally, barium paste has been used for performing defecography. Because this substance is not stool-like, barium defecography may not accurately represent defecatory function. Our aim was to prospectively compare the utility of a new artificial stool, "FECOM"--a silicon-filled and barium-coated, deformable device the shape and consistency of which mimicked ...
Loff S - - 1999
We analyzed clinical, biochemical, and histo- logic parameters of ten infants with parenteral nutrition-induced hepatobiliary dysfunction. The data were compared with the results of a rabbit model. All infants were born prematurely with low birth weight. Their clinical diagnoses were necrotizing enterocolitis (6), gastroschisis (1), intrauterine volvulus (1), and lung ...
Risseeuw J J - - 1999
Liver rupture is a rare perinatal complication with high maternal mortality. In a multiparous woman with preeclampsia and Hemolysis, Elevated Liver enzymes, and Low Platelet count (HELLP) syndrome, liver rupture was suspected 10 h after a cesarean section. Laparotomy revealed liver rupture which was treated by perihepatic packing. Eventually, the ...
Onda Koichi K Department of Psychiatry, Jichi Medical School, - - 1999
The monthly birth distribution of schizophrenic inpatients was examined in the Tochigi prefecture, Japan. A significant excess (26%) above the expected birth counts was found for patients born in the winter months (December-February). These data are consistent with the previous reports from European and North American countries.
Johnson K - - 1998
BACKGROUND: The prognosis of infants with prolonged neonatal jaundice is dependent on early diagnosis because of the need for prompt surgical management of biliary atresia. OBJECTIVE: To evaluate the usefulness of 99 mTcm-trimethylbromo-iminodiacetic acid (TBIDA, mebrofenin) in the investigation of infantile jaundice. MATERIALS AND METHODS: A retrospective study was undertaken ...
Peng S S - - 1998
Distinguishing extrahepatic biliary atresia from other causes of cholestasis in neonates and infants is important because surgical intervention before 2 months of age allows for long-term survival. The purpose of this prospective study was to evaluate the usefulness of magnetic resonance (MR) cholangiography in differentiating biliary atresia from other causes ...
Johal J S - - 1998
We report a neonate with neonatal hemochromatosis (NH), renal tubular dysgenesis (RTD), and hypocalvaria. NH is a fatal condition of the newborn, characterized by severe idiopathic liver failure of intrauterine onset and siderosis, intra- and extrahepatic, with sparing of the reticuloendothelial system. RTD is characterized by short, abnormally developed cortical ...
Berbero─člu M - - 1998
BACKGROUND: Isolated deficiency of glucocorticoids is characterized by elevated levels of adrenocorticotropin (ACTH) and normal aldosterone production. It is rare for isolated deficiency of glucocorticoids to be associated with liver involvement. A case of an infant with isolated deficiency of glucocorticoids presenting with cholestasis is presented in this article. A ...
Uckun F M - - 1998
Leukemic cells from bone marrow (BM) of 17 infants and 127 children with newly diagnosed ALL, as well as fetal liver and BM and normal infant BM samples, were analyzed for presence of a t(4;11) translocation using standard cytogenetic techniques and expression of an MLL-AF4 fusion transcript using standard reverse ...
Richard K - - 1998
The role of the deiodinases D1, D2, and D3 in the tissue-specific and time-dependent regulation of thyroid hormone bioactivity during fetal development has been investigated in animals but little is known about the ontogeny of these enzymes in humans. We analyzed D1, D2, and D3 activities in liver microsomes from ...
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