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Sedlák Kamil - - 2004
Three captive female nilgais (Boselaphus tragocamelus) either showed late-term abortion or their newborn calves died within 2 days of birth. Toxoplasma gondii DNA was demonstrated in the brain and liver of each fetus and in one of the two neonates by single-stage polymerase chain reaction (PCR) with TGR1E and by ...
Latini G - - 2004
BACKGROUND/AIMS: Epidemiological studies in humans link adult disease to abnormal growth in utero. In addition to general malnutrition of the foetus, preferential blood flow to the brain and heart may furthermore deprive organs such as the liver, spleen and kidneys of oxygen and macro- and micronutrients. As a consequence, these ...
Venigalla Sridevi - - 2004
Any infant who is jaundiced beyond two to three weeks of life should be evaluated for neonatal cholestasis. Neonatal cholestasis is defined as accumulation of bile substances in blood due to impaired excretion. These infants should always have fractionated serum bilirubin levels checked to differentiate the conjugated hyperbilirubinemia of cholestasis ...
Baserga Mariana C - - 2004
BACKGROUND: Hepatobiliary dysfunction is well recognized as a complication of long-term total parenteral nutrition (TPN). Because intrauterine growth restriction (IUGR) alters a number of metabolic and physiologic variables in the fetus that probably affect the hepatocyte function and tolerance to feedings in the IUGR extremely low birth weight (ELBW), we ...
Suskind David L DL Department of Pediatrics, University of California, San Francisco, USA. - - 2004
Biliary atresia (BA) is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. Many similarities exist between BA and graft versus host disease suggesting engraftment of maternal cells during gestation could result in immune responses that lead to BA. The aim of ...
Haywood S - - 2004
Copper-sensitive North Ronaldsay sheep represent a possible model for certain hepatic-overload syndromes of infancy and childhood that are clinically, pathologically and genetically distinct from Wilson's disease. The purpose of this study was to simulate in artificially reared lambs the syndrome produced by copper exposure in susceptible human infants. Twenty four ...
Davenport Mark - - 2004
BACKGROUND: Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centres, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centres where both ...
Old J M - - 2004
The development of the liver, bone marrow and spleen have been investigated in the stripe-faced dunnart. At birth, the liver was undergoing haematopoiesis but the level declined rapidly and by day 50 after birth the liver was histologically mature. Both the bone marrow and spleen were non-haematopoietic at birth but ...
Davenport M - - 2004
BACKGROUND: There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis. METHODS: ...
Ji Eun-Kyung - - 2004
OBJECTIVES: To evaluate fetuses with echogenic foci in the left upper quadrant of the abdomen by sonographic examination. METHODS: Between November 2000 and October 2001, we prospectively assessed fetuses with echogenic foci in the abdominal left upper quadrant on routine prenatal sonography. The location, number, and size of the foci ...
Mandel D - - 2004
OBJECTIVE: To retrospectively study the epidemiology of nosocomial cutaneous abscesses in 46 consecutive septic infants. RESULTS: Ten infants had one abscess or more. Surviving infants with abscesses had a longer duration of bacteraemia, which disappeared within 24 hours of drainage. CONCLUSION: Infants with persistent bacteraemia should be examined regularly for ...
Sharma Shyam B - - 2004
Spontaneous gall bladder perforation in infants is rare. We report a 3-month-old male infant who presented with progressive abdominal distension, low-grade fever, bilateral hydrocele and acholic stools. Ultrasonography showed free fluid in the peritoneal cavity, which was bile-stained on paracentesis. Surgical exploration revealed sterile biliary peritonitis and a gangrenous gall ...
Bellig Linda L - - 2004
Acute fatty liver disease of pregnancy has been recognized as a clinical problem since the 1980s. In the past 8 years, the association of this disease with a genetic inborn error of metabolism in the infant has been recognized. Women who are heterozygous for this disorder are usually asymptomatic until ...
Hu Weimin K - - 2004
This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age. At ...
Meyers Rebecka L - - 2004
BACKGROUND: Once it is established that a jaundiced infant has an elevated direct bilirubin level, the principal diagnostic concern is the differentiation of hepatocellular from obstructive cholestasis, of disorders of physiology from disorders of anatomy, and of disease that is managed medically from disease that is managed surgically. Traditional tests ...
Hinds Rupert - - 2004
OBJECTIVES: To investigate the incidence, presence of associated anomalies, type of malformation, and natural history of children with biliary atresia (BA) who presented antenatally. Study design The database of 194 infants diagnosed with BA at our tertiary referral unit between 1991 and 2002 was reviewed for infants in whom routine ...
Shimada Akira - - 2004
A transient myeloproliferative disorder (TMD) occurs in 10% of the infants with Down syndrome. While most cases resolve within a few months, in 20% of them TMDs are life-threatening or fatal. We encountered 4 patients with TMD, including 1 patient who died of liver failure and disseminated intravascular coagulation. Suspecting ...
Kanegawa Kimio - - 2003
OBJECTIVE: A retrospective review was performed to evaluate the importance of the "triangular cord" sign in comparison with gallbladder length and contraction for the diagnosis of biliary atresia in pediatric patients. MATERIALS AND METHODS: Fifty-five fasting infants with cholestatic jaundice were examined on sonography. The examinations focused on the visualization ...
Kobayashi Hiroyuki - - 2003
Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology, affecting both the intra- and extrahepatic bile ducts. Although relatively rare, BA must be excluded in any infant with conjugated hyperbilirubinaemia since the prognosis is improved by early diagnosis and prompt surgery. At least two phenotypes of BA are ...
Roberts Eve A - - 2003
Conjugated hyperbilirubinaemia in an infant indicates neonatal liver disease. This neonatal hepatitis syndrome has numerous possible causes, classified as infective, anatomic/structural, metabolic, genetic, neoplastic, vascular, toxic, immune and idiopathic. Any infant who is jaundiced at 2-4 weeks old needs to have the serum conjugated bilirubin measured, even if he/she looks ...
Davenport Mark - - 2003
BACKGROUND: Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life. METHODS: A retrospective review was conducted of all ...
Mancuso Michelangelo - - 2003
OBJECTIVES: To document 2 apparently incongruous clinical disorders occurring in the same infant: congenital myopathy with myophosphorylase deficiency (McArdle disease) and mitochondrial hepatopathy with liver failure and mitochondrial DNA depletion. METHODS: An infant girl born to consanguineous Moroccan parents had severe congenital hypotonia and hepatomegaly, developed liver failure, and died ...
Arnold Chris J - - 2003
Parenteral nutrition (PN) is an essential component in the care of premature and ill infants. The incidence of parenteral nutrition-associated cholestasis (PNAC) ranges from 7.4 to 84%. One substance in PN solutions that has been implicated in PNAC is aluminum. Aluminum loading in animals and humans causes hepatic accumulation and ...
Sundaram Shikha S - - 2003
Orthotopic liver transplantation (OLT) has evolved over the past two decades to become the standard of care for end-stage liver disease in infants and children. Technical advances, particularly the use of technical variant allografts, have permitted extension of OLT into a much younger and smaller population than previously possible. Major ...
Zeitlin Leonid - - 2003
The extracellular matrix (ECM) expression is subject to distinct changes during ontogeny, and the natural course of liver fibrosis in neonates is thought to differ from that in adults. We compared the expression and distribution of main ECM components between neonatal and adult liver fibrosis. Liver biopsies from infants with ...
Zietz Björn P - - 2003
Copper in drinking water has been associated with non-Indian childhood cirrhosis (NICC), a form of liver cirrhosis in childhood. This epidemiological study examines the exposure of infants to increased copper concentrations through drinking water from public water supplies in southern Lower Saxony, Germany, and whether this dietary copper intake causes ...
Lee W S - - 2003
A six-week-old male infant was admitted for investigation of cholestasis and pale stools. He became lethargic and apnoeic with prolonged seizures after a percutaneous liver biopsy. Subsequent investigations showed conjugated hyperbilirubinaemia, elevated liver enzymes, and hypoglycaemia. The radinuclide hepatobiliary scintigraphy was non-excretory. After an operative cholangiogram, the infant developed Addisonian-like ...
Fahnehjelm Kristina Teär - - 2003
PURPOSE: To present four infants with optic nerve hypoplasia and cholestasis. METHODS: All patients underwent detailed ophthalmological and hepatological assessment. Their endocrinological and neuroradiological examinations were re-evaluated. RESULTS: All four infants presented with cholestasis and were subsequently found to have optic nerve hypoplasia. One child was blind according to the ...
Moens Els - - 2003
We describe two neonates with a liver abscess after umbilical venous catheterisation. The first case was a female neonate, born at 32 weeks of gestation. After persistence of elevated inflammatory parameters, an abscess in the right lobe of the liver was diagnosed. Percutaneous drainage under CT guidance was performed. The ...
Miller Kirsten K - - 2003
The case of a term infant with disseminated neonatal hemangiomatosis and congenital right ventricular diverticulum is reported. Visceral hemangiomas were present in the liver and kidneys and the patient had lower gastrointestinal bleeding that resolved. The right ventricular diverticulum remained clinically silent. The association of right ventricular diverticula and disseminated ...
Meyers Rebecka L - - 2003
BACKGROUND/PURPOSE: Early reports suggest that the use of steroids after Kasai portoenterostomy may improve bile flow and outcome in infants with biliary atresia. METHODS: Of 28 infants with biliary atresia, half received adjuvant high-dose steroids, and half received standard therapy. Infants in the steroid group (n = 14) received intravenous ...
Tréluyer J M - - 2003
Amprenavir is a human immunodeficiency virus-1 (HIV-1) protease inhibitor intended to be used to treat HIV-infected children. Although a pediatric dosage is proposed by the manufacturer, no data are currently available on the pharmacokinetics of amprenavir in neonates and infants. Amprenavir being primarily eliminated after oxidative biotransformation, we explored its ...
Weimann E - - 2003
Bloody nipple discharge is a rare but distressing finding in neonates and infants. We report an 8-month-old boy who showed bilateral bloody nipple discharge for 5 months without signs of infection. Ultrasound examination revealed dilated mammary ducts. This benign phenomenon is most likely caused by mammary duct ectasia. On the ...
Herzog D D Department of Pediatrics, Sainte-Justine Hospital, Montreal, Quebec, - - 2003
In asphyxiated newborn infants, cholestasis often leads to extensive investigations and a cause can rarely be found. To assess the frequency of transient neonatal cholestasis in an unselected group of asphyxiated newborn infants in a mother-child centre. Charts of 181 asphyxiated newborn infants born with appropriate birth weight for gestational ...
Ohura Toshihiro - - 2003
Adult-onset type II citrullinaemia, caused by deficiency of the citrin protein encoded by the SLC25A13 gene, is characterised by a liver-specific argininosuccinate synthetase deficiency. DNA analysis for citrin deficiency revealed that SLC25A13 mutations are the cause of a particular type of neonatal intrahepatic cholestasis. We retrospectively investigated nine infants with ...
Zietz Björn P - - 2003
Copper in drinking water has been associated with Non-Indian Childhood Cirrhosis (NICC), a form of early childhood liver cirrhosis. This epidemiological study examines the exposition of infants to increased copper concentrations through drinking water from public water supplies in Berlin, Germany, and if this dietary copper intake can cause liver ...
van Weeren P R - - 2003
REASON FOR PERFORMING STUDY: To elucidate the highly contentious role of copper in the pathogenesis of osteochondrosis. HYPOTHESIS: There would be no relationship between liver copper concentration of mares and foals and incidence of radiographically detectable osteochondrotic lesions in foals and yearlings was tested. METHODS: Liver copper concentration was assessed ...
Feo C F - - 2003
AIM: To evaluate the aetiopathogenetic factors in cases of retroperitoneal abscess in young infants, particularly the correlation with omphalitis. METHODS: We describe the cases of two infants, aged 8 and 3 wk, respectively, with a history of omphalitis during the first weeks of life and subsequent development of a retroperitoneal ...
Powell J E - - 2003
To evaluate a community-based screening programme for detecting neonatal liver disease by the quantitation of conjugated bilirubin in blood. Prospective cohort/observational study using spare plasma from routinely collected liquid neonatal screening specimens from babies born in Birmingham over a two-year period. Babies with a conjugated bilirubin above 18 mumol/l and ...
Kim M C - - 2002
Intussusception is a common but life threatening gastrointestinal emergency that occurs in the infant or young child. A three-year-old boy presenting with abdominal pain and vomiting was diagnosed to have the target sign on ultrasonography. An ileocolic intussusception was initially reduced using air enema. Recurrent intussusception 12 hours later was ...
Kullendorff C-M - - 2002
A small number of cavernous liver hemangiomas in infants cause serious symptoms, requiring active treatment. We report two newborns with giant liver hemangiomas, treated by intra-arterial embolization. The babies presented at 2 - 8 days after birth with tachypnoea and cardiac dilation. A giant liver hemangioma located in the right ...
Rhoads Kim - - 2002
An infant presented with abdominal distension and failure to thrive. This patient was a 3.5-month-old, ex-26-week premature infant at the time of presentation who required supplemental parenteral nutrition until day 9 of life. Workup found ascites and a complex cystic mass in the porta hepatis. A perforated gallbladder with gallstones ...
Rohana J - - 2002
A term newborn infant developed hypovolaemic shock shortly after birth. She was pale with gross hepatomegaly. She required multiple boluses of intravenous fluids, blood products as well as inotropic support. Blood investigations showed persistent thrombocytopenia, anaemia and disseminated intravascular coagulopathy (DIC). She also developed heart failure. She finally succumbed on ...
Boberg Kirsten Muri - - 2002
The incidence and characteristics of AIH differ in various geographic regions. Based on limited epidemiologic studies, the incidence of type 1 AIH among Caucasoid populations of Europe and North America ranges from 0.1 to 1.9/100,000/year. The disease is considerably less frequent in Japan. The relative proportion of AIH among cases ...
Yerushalmi Baruch - - 2002
OBJECTIVE: To determine the frequency of Niemann-Pick disease type C (NPC) among children being evaluated for neonatal cholestasis during a 2-year period. METHODS: Medical records were reviewed from all infants with cholestasis and all patients with NPC evaluated at our center from January 1997 through December 1998. RESULTS: Forty neonates ...
Basaran Umit Nusret - - 2002
Spontaneous prenatal closure of gastroschisis (GS) is rare and usually associated with atresia of the midgut. We describe a case of GS diagnosed at 20 weeks' gestation that resolved spontaneously in utero. At delivery the infant had an ileus. A laparotomy with a jejunocolostomy was performed, but she died at ...
Bode C O - - 2002
Idiopathic intussusception is associated with viral pathogens of gastrointestinal respiratory and febrile diseases of infancy and early childhood. These agents are known to vary from one region to another. No such specific viruses have, however, been reported from Nigeria. We therefore collected stools from 28 infants with intussusception as well ...
Frey Bernhard - - 2002
OBJECTIVE: To report a case of transient cholestatic hepatitis occurring in an infant between the third and seventh weeks of life, most likely due to carbamazepine exposure during pregnancy and breast feeding. CASE SUMMARY: A boy, born to an epileptic mother who had been treated with carbamazepine monotherapy throughout pregnancy ...
McKiernan P J - - 2002
Neonatal cholestasis must always be considered in a newborn who is jaundiced for more than 14-21 days and a measurement of the serum total and conjugated bilirubin in these infants is mandatory. Conjugated hyperbilirubinaemia, dark urine and pale stools are pathognomic of the neonatal hepatitis syndrome which should be investigated ...
Rapola Juhani - - 2002
Autopsy study of 17 newborn infants with lethal autosomal recessive disease presenting as growth retardation with lactic acidosis, Fanconi aminoaciduria, and hepatic hemosiderosis is reported. The patients succumbed between day 1 and 4 months of life; 9 patients died within the first month. All patients showed severe pathologic changes of ...
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