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Recent Wilson Disease News and Articles

• New Molecular Bioscience Findings from University of Bordeaux Described
• Findings from Michigan State University Advance Knowledge in Ophthalmology
• Investigators from University of Saskatchewan Release New Data on Biological Chemistry
• Research from University of Ulsan College of Medicine Yields New Findings on Genetics and Hepatolenticular Degeneration
• Study Results from Chhatrapati Shahuji Maharaj Medical University Provide New Insights into Science
• New Genetics Research from Shiraz University of Medical Sciences Described

Data from University of Cairo Provide New Insights into Bacterial Infections

Our news editors obtained a quote from the research from the University of Cairo, "There are three main goals for the management of portal hypertension: (i) prevention of the first episode of variceal bleeding largely by non-selective ß-adrenoceptor antagonists, which is not generally recommended in children; (ii) control of bleeding by using a stepwise approach from the least to most invasive strategies; (iii) and prevention of re-bleeding using bypass operations, with particular enthusiasm for the use of meso-Rex bypass in the pediatric population. Hepatic encephalopathy management also consists of three main aspects: (i) ruling out other causes of encephalopathy; (ii) identifying and treating precipitating factors; and (iii) starting empiric treatment with drugs such as lactulose, rifaximin, sodium benzoate, and flumazenil. Treatment of mild ascites and peripheral edema should begin with the restriction of sodium and water, followed by careful diuresis, then large-volume paracentesis associated with colloid volume expansion in severe cases. Empiric broad spectrum antimicrobial therapy should be used for the treatment of spontaneous bacterial peritonitis, bacterial and fungal sepsis, and cholangitis, after taking appropriate cultures, with appropriate changes in therapy after sensitivity testing. Empirical therapies continue to be the standard practice for pruritus; these consist of bile acid binding agents, phenobarbital (phenobarbitone), ursodeoxycholic acid, antihistamines, rifampin (rifampicin), and carbamazepine. Partial external biliary diversion can be used in refractory cases. Once hepatorenal syndrome is suspected, treatment should be initiated early in order to prevent the progression of renal failure; approaches consist of general supportive measures, management of concomitant complications, screening for sepsis, treatment with antibiotics, use of vasopressin analogs (terlipressin), and renal replacement therapy if needed. Hepatopulmonary syndrome and portopulmonary hypertension are best managed by liver transplantation. Provision of an adequate caloric supply, nutrition, and vitamin/mineral supplements for the management of growth failure, required vaccinations, and special care for ensuring psychologic well-being should be ensured. Anticoagulation might be attempted in acute portal vein thrombosis. Some CLDs, such as extrahepatic biliary atresia (EHBA), Crigler-Najjar syndrome, and Indian childhood cirrhosis, require special considerations. For EHBA, Kasai hepatoportoenterostomy is the current standard surgical approach in combination with nutritional therapy and supplemental fat and water soluble vitamins, minerals, and trace elements. In type 1 Crigler-Najjar syndrome, extensive phototherapy is the mainstay of treatment, in association with adjuvant therapy to bind photobilirubin such as calcium phosphate, cholestyramine, or agar, until liver transplantation can be carried out. Treating Indian childhood cirrhosis with penicillamine early in the course of the disease and at doses similar to those used to treat Wilson disease decreases the mortality rate by half. New hopes for the future include extracorporeal liver support devices (the molecular adsorbent recirculating system [MARS®] and Prometheus®), hepatocyte transplantation, liver-directed gene therapy, genetically engineered enzymes, and therapeutic modalities targeting fibrogenesis."

According to the news editors, the research concluded: "Hepapoietin, a naturally occurring cytokine that promotes hepatocyte growth, is under extensive research."

For more information on this research see: Medical management of chronic liver diseases (CLD) in children (part II): focus on the complications of CLD, and CLD that require special considerations. Paediatric Drugs, 2011;13(6):371-83. Paediatric Drugs can be contacted at: Adis International LTD, 41 Centorian Dr, Private Bag 65901, Mairangi Bay, Auckland 10, New Zealand.

The news editors report that additional information may be obtained by contacting M.H. El-Shabrawi, Faculty of Medicine, Cairo University, Mohandesseen, Cairo, Egypt.

Publisher contact information for the journal Paediatric Drugs is: Adis International LTD, 41 Centorian Dr, Private Bag 65901, Mairangi Bay, Auckland 10, New Zealand.

Keywords for this news article include: Cairo, Egypt, Africa, Genetics, Hepatology, Pediatrics, Bacterial Infections, Chronic Liver Diseases, Digestive System Diseases.

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