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Results 401 - 450 of 1009
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Wongsawasdi Lumduan - - 2008
OBJECTIVE: To evaluate diagnostic accuracy of some important clinical manifestations and different investigations in infantile cholestasis. MATERIAL AND METHOD: Infants diagnosed with prolong conjugated hyperbilirubinemia and admitted to Chiang Mai University Hospital between Jan 1999 and Feb 2003. Demographic and clinical data were recorded Routine biochemical tests, and serology for ...
Gura Kathleen M - - 2008
BACKGROUND: Parenteral nutrition-associated liver disease can be a progressive and fatal entity in children with short-bowel syndrome. Soybean-fat emulsions provided as part of standard parenteral nutrition may contribute to its pathophysiology. METHODS: We compared safety and efficacy outcomes of a fish-oil-based fat emulsion in 18 infants with short-bowel syndrome who ...
DeSalvo Daniel - - 2008
Cholestasis occurring in infancy should be evaluated completely to exclude hepatic as well as endocrine or metabolic causes. A rapid diagnosis should be made to ensure that hepatic and neurologic complications are prevented. We describe a rare case of infant cholestasis due to panhypopituitarism resulting in hypoglycemia and liver biopsy ...
Lang T F - - 2008
Making a diagnosis of transient non-ketotic hyperglycinaemia (tNKH) can be difficult. We report an infant who presented in the neonatal period with symptoms of NKH. Metabolic studies performed on day 2 of life showed raised cerebrospinal fluid (CSF) and plasma glycine, and a CSF:plasma glycine ratio consistent with NKH; however, ...
Shimadera Shinichi - - 2008
PURPOSE: Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance. METHODS: Between 1988 and 2005, 31 patients with ...
Naiyereh Najati
Congenital tuberculosis is defined as tuberculosis occurring in infants as a result of infection acquired from their mothers. Congenital tuberculosis maynot be rare and it is necessary to look into this condition more seriously. Tsanscutaneous liver biopsy may help to confirm it`s prenatal origin and explain causes of the multi ...
Jindal B - - 2008
Spontaneous perforation of the extra-hepatic biliary tract presenting with pseudocyst is rare. We report the case of a two-month old infant who presented to us with increasing jaundice and progressive loss of weight. Ultrasonography, magnetic resonance cholangiopancreatography and hepatobiliary iminodiacetic acid scan revealed features of obstructed choledochal cyst. Laparotomy revealed ...
Choi Dongil - - 2008
BACKGROUND AND AIM: A high prevalence of intrahepatic stones in some areas of East Asia has been believed to be related with Clonorchis sinensis infection. The authors conducted a hospital-based case-control study to evaluate the role of Clonorchis sinensis infection as a risk factor for the development of gallstones in ...
Takamizawa Shigeru - - 2007
BACKGROUND/PURPOSE: The triangular cord sign (TCS) is a specific ultrasonographic finding, reflecting a fibrotic mass at the porta hepatis in biliary atresia (BA). We evaluated whether BA can be diagnosed by ultrasonography alone using 3 findings: TCS, gallbladder length (GBL), and gallbladder contractility (GBC). METHODS: Subjects comprised 85 infants (median ...
Ko Jae Sung - - 2007
Citrin is a liver-type mitochondrial aspartate-glutamate carrier encoded by the SLC25A13 gene, and its deficiency causes adult-onset type II citrullinemia and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD). Here, the authors investigated clinical findings in Korean infants with NICCD and performed mutation analysis on the SLC25A13 gene. Of 47 ...
Wales Paul W - - 2007
OBJECTIVE: Parenteral nutrition-associated cholestasis (PNAC) occurs in up to 60% of surgical neonates with intestinal failure, and 10% will develop end-stage liver failure. Our aim was to evaluate the effectiveness of percutaneous transhepatic transcholecystic cholangiography (PTTC) in the treatment of PNAC in surgical neonates. METHODS: A retrospective double cohort study ...
The Natalie S - - 2007
Biliary atresia is a rare birth defect that affects 1 in 12,000 to 1 in 19,500 live births. We used data from the National Birth Defects Prevention Study, a multistate case-control study, to identify potential risk factors for isolated biliary atresia (no additional unrelated major birth defects diagnosed). Infants were ...
Matsui Akira - - 2007
Biliary atresia (BA) is the most frequent single life-threatening hepatic disorder in early childhood in Japan, Europe and North America. It affects one in 9000 live-born infants in our country. BA is characterized by complete inability to excrete bile associated with obstruction, destruction or absence of the extrahepatic bile ducts. ...
Capaccio Pasquale - - 2007
Atresia of the submandibular ducts (particularly the duct orifice) is a rare occurrence that is mainly observed in newborns or infants; moreover, its differential diagnosis with other anomalies of the oral floor is sometimes difficult. Given the lack of data concerning the differences between atresia of the orifice of Wharton's ...
Humphrey Terry M - - 2007
PURPOSE: To evaluate prospectively the sensitivity of ultrasonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard. MATERIALS AND METHODS: After institutional ethical approval and with informed parental consent, 90 consecutive fasting infants with conjugated hyperbilirubinemia underwent detailed US studies performed by a single operator ...
Nwomeh Benedict C - - 2007
Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography. This approach suffers from the disadvantage that sick infants are subjected to a time-consuming and potentially negative surgical exploration. The purpose of this study was to determine if percutaneous cholecystocholangiography (PCC) prevents unnecessary laparotomy in ...
Lazaridis Konstantinos N - - 2007
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disorder that can progress to cirrhosis, shortening life expectancy. PBC patients are often asymptomatic, present with biochemical cholestasis, and test positive (>or=90%) for antimitochondrial antibodies (AMAs) in serum. Although AMA positivity without biochemical cholestasis may indicate increased risk of future PBC ...
Kawano S - - 2007
A 17-month-old girl who had been followed up as an extremely-low-birth-weight infant presented with hepatoblastoma in the right lobe of her liver. Preoperative angiography revealed an absence of the portal vein, and the visceral venous return was through the left renal vein into the inferior vena cava. No liver dysfunction ...
Sokol Ronald J RJ Department of Pediatrics, University of Colorado School of Medicine, The Children's Hospital, Denver, CO, USA. - - 2007
Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States. Earlier diagnosis (<30-45 days of life) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver. However, ...
Lee Way Seah - - 2008
AIMS: To study factors leading to delayed referral in neonatal cholestasis at a tertiary centre in Malaysia. METHODS: A prospective, observational study on consecutive infants with neonatal cholestasis referred to a tertiary unit paediatric liver unit in Malaysia. RESULTS: Thirty-one of the 65 (43%) patients studied encountered delay or had ...
Tang Kuo-Shu - - 2007
BACKGROUND: Early diagnosis of biliary atresia (BA) is important because the prognosis is closely related to timing of Kasai operation. The aim of this study was to test the clinical application of serum gamma-glutamyl transferase (GGT) concentration and the ratio of serum GGT to aspartate aminotransferase (AST) and alanine aminotransferase ...
Isaiah Joel H - - 2007
Subcutaneous fat necrosis of the newborn (SFN) is an uncommon, self-limited condition of neonates and infants. However, hypercalcemia may be a potentially life-threatening complication of the otherwise self-healing process. Rare complications such as thrombocytopenia, hypertriglyceridemia, hypoglycemia, calcium deposits in the liver, inferior vena cava, atrial septum of the heart, and ...
Binder G - - 2007
BACKGROUND: Neonatal cholestatic hepatitis is frequently associated with congenital combined pituitary hormone deficiency (CCPHD). Data on the course of this hepatopathy are scarce. AIM: We retrospectively analyzed the data of all CCPHD infants with cholestasis who presented at the University Children's Hospital, Tuebingen. RESULTS: All infants (n = 9; 2 ...
Serkova Natalie J - - 2007
Thirteen-lined ground squirrels and other circannual hibernators undergo profound physiological changes on an annual basis, transitioning from summer homeothermy [body temperature (T(b)) approximately 37 degrees C] to winter heterothermy (T(b) cycling between 0 degrees C and 37 degrees C). We hypothesize that these physiological changes are reflected in biochemical changes ...
Dryden C - - 2007
Congenital thyrotoxicosis is a rare and potentially fatal illness. We report a case in a preterm infant delivered to a mother known to have autoimmune endocrine disease. Diagnosis was difficult because the infant's presenting symptoms and signs closely resembled congenital viral infection with co-existent hepatic dysfunction and coagulopathy. The associated ...
Carter Beth A - - 2007
Since its introduction into clinical practice, parenteral nutrition has revolutionized the care of premature neonates. Serum transaminase and bilirubin levels are commonly elevated in infants on parenteral nutrition, but their normalization is typical in the setting of short-term administration of parenteral nutrition uncomplicated by sepsis. Premature infants who require long-term ...
Stringer M D - - 2007
Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology. It is a rare condition with an incidence of approximately 1 in 17,000 live births in the UK. BA must be diagnosed promptly in any infant with conjugated hyperbilirubinaemia since prognosis is improved by early detection and timely surgery ...
Hong Li - - 2007
BACKGROUND: This study was designed to explore the mechanisms in parenteral nutrition (PN)-associated hepatic dysfunction, and the possible effectiveness of glutathione (GSH) to alleviate this injury. METHODS: Thirty 1-week-old New Zealand rabbits were divided into 3 groups: 10 in the control group (maternal fed); 10 in the PN group (PN ...
Kosar Feridun - - 2007
In previous studies, it has been shown that QT interval prolongation is related to an increased mortality rate in chronic liver disease (CLD). But QT dispersion (QTd) and its clinical significance in CLD has not been well studied. The objectives of this study were to investigate the relation between QTd ...
Walker Gregor M - - 2007
BACKGROUND/PURPOSE: Pediatric surgeons consider bile vomiting in the neonate a potential surgical emergency. The reported rate of surgical intervention is 30% to 40%, but as most neonates are born outwith pediatric surgical centers, referral of these babies is at the neonatologists' discretion. The aim of this study was to determine ...
Stipsanelli K - - 2007
AIM: The aim of this paper is to identify extrahepatic biliary atresia (EHBA) as the cause of cholestasis in neonates with prolonged jaundice and thus accelerate the decision for surgical intervention, which is critical for prognosis. METHODS: We retrospectively studied 21 infants (13 girls, 8 boys) aged 2-16 weeks who ...
Sala-Vila Aleix - - 2007
PURPOSE OF REVIEW: A lipid emulsion for use in parenteral nutrition containing a significant proportion of olive oil in place of soybean oil (ClinOleic; Baxter, Maurepas, France) is now available. The purpose of this review is to provide background information about the rationale for this emulsion, to collate and synthesize ...
Nasr Ahmed - - 2007
BACKGROUND: The introduction of parenteral nutrition resulted in improved survival of neonates with short bowel syndrome. It is unclear why some may deteriorate to end-stage liver disease (ESLD). Knowledge of when to refer such children for evaluation for transplantation is crucial. A commonly used criterion is conjugated hyperbilirubinemia greater than ...
Galán-Gómez Enrique - - 2007
We describe a patient born to consanguineous parents, who presented with an MCA pattern characterized by low birth weight, duodenal atresia, extrahepatic biliary atresia, hypoplastic pancreas and intestinal malrotation. The infant died 60 days after birth. Chromosomes at 550-600 band levels were normal for a female (46,XX). This patient confirmed ...
Hyatt Melanie A - - 2007
The liver is a major metabolic and endocrine organ in growing neonates, but the extent to which its hormone receptor (R) sensitivity is potentially determined by maternal parity and the mother's nutritional environment is unknown. This was therefore investigated by sampling livers from postnatal sheep born to nulliparous or multiparous ...
Kogure Kimitaka - - 2007
BACKGROUND/PURPOSE: This study was conducted to clarify the real relation between the inferior vena cava (IVC) ligament and the caudate lobe in the human liver and also to elucidate their surgical importance in liver surgery. METHODS: Specimens obtained from 20 adult cadaveric livers were submitted for the study. Histological structures ...
Chen Hung-Wen - - 2007
Two major forms of biliary atresia, the embryonic and perinatal type, are considered to have different pathogeneses and distinct prognoses. Embryonic biliary atresia is associated with worse prognosis. We report a case of embryonic biliary atresia in a preterm male infant of 31 weeks of gestation and weighing 1375 g, ...
Schmidt Beate - - 2007
BACKGROUND: Neonatal reference values of gallbladder size have been assessed in healthy newborns with enteral feeding regimen. Their applicability to critically ill patients under total parenteral nutrition (TPN) remains to be questioned. OBJECTIVE: Our aim was to evaluate the impact of short-term TPN versus enteral nutrition (EN), gender and birth ...
Massoud Samir N - - 2007
We evaluated histologically 10 biopsy specimens taken preoperatively from the anterior-inferior glenohumeral ligament from patients with atraumatic instability who had undergone radiofrequency capsular shrinkage, 10 taken immediately postoperatively, and 13 taken before revision. The synovial and subsynovial layers returned to normal histology in biopsy specimens taken from 6 months onwards. ...
Sari Neriman - - 2006
Infantile hemangioendothelioma is the most common hepatic vascular tumor in infants less than 6 months of age, with a prevalence of 1%. Serum alpha-fetoprotein levels have been used as an important tumor marker for hepatoblastoma, hepatocellular carcinoma, and germ cell tumors. It is rarely elevated in hepatic hemangioendothelioma. The authors ...
Amaral Joao Guilherme - - 2006
OBJECTIVE: The purpose of our study was to assess the technical success and complication rate of sonographically guided percutaneous liver biopsies performed in infants under 1 year old at a tertiary pediatric center. MATERIALS AND METHODS: Retrospective analysis of 65 biopsies performed in 61 infants between January 1999 and December ...
Enomoto Yuki - - 2006
We used dexmedetomidine for more than 2 months in a mechanically ventilated infant without serious adverse effects. An infant with liver cirrhosis of unknown cause underwent living donor liver transplantation at the age of 9 months. Long-term mechanical ventilation was required postoperatively, and midazolam with fentanyl had been used to ...
Allen Steven R - - 2007
Biliary atresia is a devastating disorder of the newborn in which afflicted infants develop inflammation and fibrosis of the extrahepatic biliary tract, resulting in cirrhosis and end-stage liver disease. Infection with a virus is thought to be a contributing factor in the etiology of biliary atresia. In the murine model ...
Yang Jian - - 2007
The maternal to fetal transfer of monophenyltin (MPT), diphenyltin (DPT), and triphenyltin (TPT) was investigated for the first time in a cetacean species, a mother-fetus pair of Dall's porpoises (Phocoenoides dalli) collected off the Sanriku coast, Japan. The mother individual was contaminated by phenyltin compounds, and the muscle, blubber, and ...
Valayannopoulos Vassili - - 2006
Transaldolase (TALDO) deficiency is a newly recognized metabolic disease, which has been reported so far in 2 patients presenting with liver failure and cirrhosis. We report a new sibship of 4 infants born to the same consanguineous parents; all presented at birth or in the antenatal period with dysmorphic features, ...
Tan Josephine J - - 2006
AIM: Alpha 1-antitrypsin (AAT) deficiency is the most common genetic cause of liver disease in children. The Pi*S carrier rate among Filipinos is <1%. Its significance in Filipino infants with neonatal cholestasis has not been investigated. The aim of the study was to determine the incidence of AAT deficiency among ...
Wolf Dennis - - 2006
Carbimazole embryopathy is a recently recognized and defined phenotype. Choanal atresia; gastrointestinal anomalies, particularly esophageal atresia; athelia or hypothelia; developmental delay; hearing loss; aplasia cutis; and dysmorphic facial features all can occur after exposure to the antithyroid drugs carbimazole or methimazole during gestation. Although full expression of this phenotype appears ...
Vijayaraghavan Paari - - 2006
The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. The clinical features, liver histology and the outcome after surgery in 14 cases of CDCs treated before ...
Judson Marc A - - 2006
STUDY OBJECTIVE: To test the hypothesis that sibling pairs, who share genes and environmental exposures, might have similar phenotypic expressions of sarcoidosis beyond what would be expected by chance alone. DESIGN: Multicenter family study with study subjects recruited from 11 clinical centers. SUBJECTS: Subjects were African-American sibling pairs with sarcoidosis. ...
Davenport Mark - - 2006
We carried out a retrospective review of infants with biliary atresia splenic malformation (BASM). We found that 56 infants (10.2%) met the criteria for inclusion from a series of 548 infants (from January 1977 to December 2004). Syndromic infants were more likely to be female (P = .04) and to ...
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