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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Gastrointestinal problems and, in particular, diarrhoeal illnesses are the commonest cause of morbidity and mortality among young children in Jordan. Children with diarrhea constitute about 20% of admissions to Jordan University Hospital (JUH). The aetiology of diarrhoea is multifactorial and bacterial pathogens are seldom isolated. Stool viruses have not been investigated. There is a steady fall in the prevalence of breast-feeding with a consequent increase in gastroenteritis, especially among the poor. The use of electrolyte solutions for oral rehydration therapy is encouraged, and the use of antibiotics in diarrhoeal illness is discouraged. The addition of sucrose instead of glucose to electrolyte solutions has also been adopted. Cow's milk protein intolerance, coeliac disease and chronic inflammatory bowel disease are rarely encountered. Hepatitis is common and is a major cause of morbidity among school children. Cystic fibrosis has been recognized in Jordan but is rare. JUH has been selected as the referral centre for cystic fibrosis in the Middle East.
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PMID:Gastroenterological problems in childhood in Jordan. 618 81

Technetium-99m mebrofenin hepatobillary excretory patterns were assessed in 36 infants with hyperbilirubinemia. Phenobarbital was administered to 22 patients before imaging. Final diagnoses included: intrahepatic cholestasis (14 patients), neonatal hepatitis (nine patients), biliary atresia (eight patients), alpha-1-antitrypsin deficiency (two patients), Alagille's syndrome (two patients), and cystic fibrosis (one patient). No patient with biliary atresia showed bowel activity by 24 hours. Of the 28 infants without biliary atresia, 23 (82%) had bowel activity visualized by 6-8 hours and 26 (90%) had bowel activity by 24 hours. Two had no bowel activity at 24 hours: one had cystic fibrosis and one had neonatal hepatitis. Of the 26 patients with bowel visualization, the time to visualize bowel did not differ between patient groups with and without phenobarbital induction. All of the patients with hepatitis, including those with marked dysfunction, showed good hepatic uptake. Mebrofenin scintigraphy is an important imaging technique in the diagnostic evaluation of infants with hyperbilrubinemia. In addition to biliary atresia, intrahepatic cholestasis due to cystic fibrosis and severe neonatal hepatitis may also cause bowel nonvisualization up to 24 hours. The results of this study suggest phenobarbital induction may not be needed when Tc-99m mebrofenin scintigraphy is used for the assessment of infantile jaundice.
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PMID:Utility of Tc-99m mebrofenin scintigraphy in the assessment of infantile jaundice. 772 Mar 8

Cystic fibrosis (CF) is an autosomal recessive disorder that is reported frequently among Caucasians. In view of the high rate of consanguinity in Saudi Arabia and the high number of children dying so young, many Saudi infants with CF remain undiagnosed owing to lack of clinical suspicion and proper diagnostic facilities. Over a 6-year period (1986-1992), we have made a diagnosis of CF in 36 cases. The aim of this report is to increase awareness of the hepatic presentation of CF in the developing word. Nine patients (25%) were originally referred to us as having liver disorders but subsequent investigations confirmed the diagnosis of CF. The referral diagnoses, number of patients in parentheses, were jaundice for investigation (four), glycogen storage disease (three), hepatomegaly for investigation (one) and neonatal hepatitis (one). Liver biopsies, obtained in five cases, demonstrated portal fibrosis and some steatosis. Consanguinity was present in eight cases, two were siblings. Four patients died. Three families had previously lost seven siblings during infancy with clinical features consistent with CF. It is concluded that hepatic presentation among Saudi patients with CF is relatively common, with serious sequelae.
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PMID:Cystic fibrosis and the liver--a Saudi experience. 782 91

Seventy-two long-surviving liver transplant recipients were evaluated prospectively, including a baseline allograft biopsy for weaning off of immunosuppression. Thirteen were removed from candidacy because of chronic rejection (n = 4), hepatitis (n = 2), patient anxiety (n = 5), or lack of cooperation by the local physician (n = 2). The other 59, aged 12-68 years, had stepwise drug weaning with weekly or biweekly monitoring of liver function tests. Their original diagnoses were PBC (n = 9), HCC (n = 1), Wilson's disease (n = 4), hepatitides (n = 15), Laennec's cirrhosis (n = 1), biliary atresia (n = 16), cystic fibrosis (n = 1), hemochromatosis (n = 1), hepatic trauma (n = 1), alpha-1-antitrypsin deficiency (n = 9), and secondary biliary cirrhosis (n = 1). Most of the patients had complications of long-term immunosuppression, of which the most significant were renal dysfunction (n = 8), squamous cell carcinoma (n = 2) or verruca vulgaris of skin (n = 9), osteoporosis and/or arthritis (n = 12), obesity (n = 3), hypertension (n = 11), and opportunistic infections (n = 2). When azathioprine was a third drug, it was stopped first. Otherwise, weaning began with prednisone, using the results of corticotropin stimulation testing as a guide. If adrenal insufficiency was diagnosed, patients reduced to < 5 mg/day prednisone were considered off of steroids. The baseline agents (azathioprine, cyclosporine, or FK506) were then gradually reduced in monthly decrements. Complete weaning was accomplished in 16 patients (27.1%) with 3-19 months drug-free follow-up, is progressing in 28 (47.4%), and failed in 15 (25.4%) without graft losses or demonstrable loss of graft function from the rejections. This and our previous experience with self-weaned and other patients off of immunosuppression indicate that a significant percentage of appropriately selected long-surviving liver recipients can unknowingly achieve drug-free graft acceptance. Such attempts should not be contemplated until 5-10 years posttransplantation and then only with careful case selection, close monitoring, and prompt reinstitution of immunosuppression when necessary.
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PMID:Weaning of immunosuppression in long-term liver transplant recipients. 783 42

Molecular biology has revolutionized our understanding of the life of the cell. The development of concepts has been accompanied by the development of new technologies, many of which have clinical applicability. This new armamentarium includes methods for detection and amplification of minuscule amounts of DNA, automated methods for DNA and RNA sequencing, the ability to express a gene in cultured cells and harvest its product, approaches to identify genes whose product is unknown, methods for a detailed understanding of the regulation of gene expression, and innovative methods for gene therapy. These developments have already had an impact on gastroenterology, exemplified by the identification of hepatitis viruses and the agent causing Whipple's disease, development of a recombinant vaccine for hepatitis B, and isolation of the cystic fibrosis gene, which will lead to the diagnosis and treatment of this disease. This editorial outlines these recent changes in the conceptual and practical framework of gastroenterology.
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PMID:Molecular gastroenterology--implications for medical practice. 822 75

We present a case of meconium peritonitis which was associated with a short bowel and complicated by progressive bowel distension and difficulty in making a definitive diagnosis of cystic fibrosis. Treatment was by bowel resection and an ileostomy (and later bowel anastomosis), followed by parenteral nutrition which was complicated by hepatitis. The literature is reviewed and management dilemmas and options are discussed.
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PMID:Antenatal diagnosis and management of meconium peritonitis: a case report and review of the literature. 852 7

Hepatobiliary scintigraphy using iminodiacetic (IDA) radiopharmaceuticals provides clinically useful information on the function of the biliary tract in a variety of pathological processes in children, including neonatal jaundice, gallbladder dysfunction, trauma, and liver transplantation. Phenobarbital premedication (5 mg/kg per day for a minimum of 5 days in divided doses) is used in infants who are being examined for neonatal jaundice to increase the accuracy of 99mTc-IDA scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. Biliary atresia can be ruled out in an infant if a patent biliary tree is shown with passage of activity into the bowel. If no radiopharmaceutical is noted in the bowel on imaging up to 24 hours, distinction between severe hepatocellular disease and biliary atresia cannot be made. The literature reports 91% accuracy, 97% sensitivity, and 82% specificity for hepatobiliary imaging in the diagnosis of biliary atresia. The impairment of both intrahepatic and extrahepatic biliary drainage is an important cause of liver disease in cystic fibrosis. Hepatobiliary scintigraphy in cystic fibrosis has shown characteristic patterns of dilatation of mainly the left hepatic duct, narrowing of the distal common bile duct, gallbladder dysfunction, and delayed bowel transit. Cholecystitis in children may be acalculous. Sensitivity and specificity for the scintigraphic diagnosis of acute acalculous cholecystitis is reported to range from 68% to 93% and 38% to 93%, respectively. Cholescintigraphy in a suspected bile leak provides information generally not available with other techniques, except for direct cholangiography. If the amount of intraperitoneal accumulation of the tracer is greater than that entering the gastrointestinal tract, surgery is usually indicated. Hepatobiliary imaging in children who have undergone liver transplantation will assess graft vascularity, parenchymal function, biliary drainage, presence of a leak, and obstruction.
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PMID:Hepatobiliary scintigraphy in children. 862 49

The relative importance of molecular biology in clinical practice is often underestimated. However, numerous procedures in clinical diagnosis and new therapeutic drugs have resulted from basic molecular research. Furthermore, understanding of the physiological and physiopathological mechanisms underlying several human diseases has been improved by the results of basic molecular research. For example, cloning of the gene encoding leptin has provided spectacular insights into the understanding of the mechanisms involved in the control of food intake and body weight maintenance in man. In cystic fibrosis, the cloning and identification of several mutations in the gene encoding the chloride channel transmembrane regulator (CFTR) have resolved several important issues in clinical practice: cystic fibrosis constitutes a molecular defect of a single gene. There is a strong correlation between the clinical manifestations or the severity of the disease (phenotype) with the type of mutations present in the CFTR gene (genotype). More recently, identification of mutations in the gene encoding a subunit of the renal sodium channel in the Liddle syndrome has provided important insight into the physiopathological understanding of mechanisms involved in this form of hereditary hypertension. Salt retention and secondary high blood pressure are the result of constitutive activation of the renal sodium channel by mutations in the gene encoding the renal sodium channel. It is speculated that less severe mutations in this channel could result in a less severe form of hypertension which may correspond to patients suffering from high blood pressure with low plasma renin activity. Several tools, most notably PCR, are derived from molecular research and are used in everyday practice, i.e. in prenatal diagnosis and in the diagnosis of several infectious diseases including tuberculosis and hepatitis. Finally, the production of recombinant proteins at lower cost and with fewer side effects is used in everyday clinical practice. Gene therapy remains an extraordinary challenge in correcting severe hereditary or acquired diseases. The use of genetically modified animal cell lines producing growth factors, insulin or erythropoetin, which are subsequently encapsulated and transferred to man, represents an attractive approach for gene therapy.
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PMID:[Is molecular biology useful to the practitioner?]. 919 Jun 68

Hepatobiliary scintigraphy is the only non-invasive technique providing real-time assessment of hepatocytes function and bile progression from the liver to the intestine; for this reason it is of great importance in the study of jaundice and many other disorders of the liver and the biliary tract in children. Ultrasonography is the initial method of evaluating the intra- and extrahepatic bile ducts dilatation; the differential diagnosis between biliary atresia and neonatal hepatitis cannot however be done without hepatobiliary scintigraphy. Cystic fibrosis patients also require hepatobiliary scintigraphy; liver and biliary tract disease can really occur independently of the underlying disease severity and the presence of steatorrhea. Hepatobiliary imaging in children who have undergone liver transplantation is of major importance; it can assess vascularity, parenchymal function, biliary drainage, possible presence of a bile leak and obstruction; it has very good sensitivity and specificity for the detection of biliary leak and biliary stricture. Due to these important clinical conditions being studied in pediatrics, both qualitative and quantitative informations (functional indices obtained from radioactivity/time curve analysis) need to be extracted; in addition, SPECT acquisition allows more accurate evaluation of abdominal activity during the excretory phase.
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PMID:Hepatobiliary scintigraphy in current pediatric practice. 969 63

Primary sclerosing cholangitis (PSC), a chronic inflammatory process affecting the extrahepatic and/or medium to large bile ducts, is not rare in children. It has features suggesting an autoimmune pathogenesis, although the mechanism of tissue damage remains unknown. The clinical presentation of childhood primary sclerosing cholangitis is highly variable and frequently without obvious features of cholestasis. Clinical similarity to autoimmune hepatitis is common. Association with chronic colitis is less common than in adults. Cholangiography is essential for the diagnosis and examination of the medium to large intrahepatic ducts is mandatory, as 40% of children lack extrahepatic duct involvement. Histological findings may help to distinguish childhood PSC from autoimmune hepatitis. In children, sclerosing cholangitis may also develop secondary to other disease processes, notably Langerhans histiocytosis, congenital immunodeficiencies and cystic fibrosis. Neonatal sclerosing cholangitis is chronic inflammatory disease of bile ducts which presents initially with neonatal cholestasis; its pathogenesis remains uncertain and may not be the same as for primary sclerosing cholangitis. Effective treatment modalities for childhood PSC remain undetermined. Liver transplantation is required for children who progress to biliary cirrhosis and hepatic decompensation.
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PMID:Primary sclerosing cholangitis in children. 1038 70


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