[Nutrition in cholestasis and long-term impact on trasnplant]. / Nutritia în colestaza si impactul pe termen lung dupa transplant.

AIM: Plurietiological hepatic cholestasis involves an increased risk of protein-caloric malnutrition and specifical nutritional deficiencies. Biological investigations to determine deficiencies in fat-soluble vitamins are essential for specific nutritional therapy . Although malnutrition is not an absolute contraindication for liver transplantation, its gravity has complex consequences in relation to this intervention. MATERIAL AND METHOD: The authors present a study conducted over a period of 5 years following the analysis of 293 children diagnosed with intra- or extrahepatic abnormalities that caused varying degrees of cholestasis. RESULTS: In the study group, the percentage of infants with cholestasis was 45.39% (133 cases) and among them 62.12% had malnutrition (82 children). Clinical evaluation of fat-soluble vitamins deficiency, in particular, but also of the soluble and minerals was performed in all patients. Liver transplantation was successfully performed in three cases. CONCLUSIONS: Most important factors affecting growth after transplantation were age at the time of the liver transplant and primary diagnosis that required transplant. The role of nutritional support prior to liver transplantation is of great importance. Quality nutritional support change impact that malnutrition has on survival after liver transplantation.

Extrahepatic manifestations of cholestasis.

Pruritus, fatigue and metabolic bone disease represent three major extrahepatic manifestations of chronic cholestatic liver disease that considerably affect the patient's quality of life. The present article reviews pathogenetic aspects of and current therapeutic approaches to extrahepatic manifestations of cholestatic liver disease. Pathogenesis of pruritus of cholestasis remains poorly understood. The involvement of putative peripherally acting pruritogens, such as bile acids or endogenous opioids, is being discussed. More recently, central mechanisms, including an increased central opioidergic tone and pertubations in the serotonergic system have been proposed. Treatment of the underlying disease is beneficial also for the control of cholestasis-associated pruritus. Current therapeutic recommendations include ursodeoxycholic acid, cholestyramine, rifampicin and opioid antagonists. Liver transplantation may be indicated when severe pruritus is refractory to medical treatment. Fatigue is being recognized as the most frequent and one of the most disabling complaints in chronic cholestasis. Fatigue is presumably of central origin and its association with other neuropsychiatric disorders (e.g. depression, obsessive-compulsive disorders) is consistent with defective central neurotransmission. No specific therapies are currently available and a healthy lifestyle, regular sleep and avoidance of unnecessary stress and other precipiting factors are recommended. Antidepressant therapy may be warranted in selected patients. Osteopenia and osteoporosis are common in chronic cholestatic liver disease, whereas osteomalacia is rare. The pathophysiology of cholestasis-associated metabolic bone disease is regarded as multifactorial. Therapeutic recommendations include regular exercise, calcium and vitamin D supplementation in late stage disease, hormone replacement therapy in postmenopausal women and bisphosphonates.

Microwave coagulation versus insertion of a second stent for occluded biliary metal stent.

BACKGROUND/AIMS: There is no consensus regarding optimal management of self-expandable metallic stent occlusion. We investigated the efficacy of microwave coagulation therapy for recanalization as compared to second stent placement. METHODOLOGY: Sixty patients with malignant obstruction of the common bile duct were treated with metal stent placement from January 1992 to July 1999. Of these, 13 patients subsequently developed stent occlusion due to tumor ingrowth. We compared stent patency and patient survival rates after microwave coagulation to those after insertion of a second stent. The influence of the duration of patency of the first stent on the second stent patency was also evaluated. RESULTS: Of the 13 patients with stent occlusion, 7 were treated with microwave coagulation therapy, and 6 with insertion of a second metal stent. In all cases, occluded stents were successfully recanalized without any complications. There was no significant difference in duration of first stent patency between the two groups. The median duration of second stent patency was prolonged in microwave-treated patients (152 days vs. 104 days, P > 0.05). The median duration of patient survival after last recanalizing procedure was also prolonged in microwave-treated patients (131 days vs. 78 days, P > 0.05). Microwave energy did not induce destruction of the stent filament. CONCLUSIONS: Microwave coagulation did not offer significantly longer duration of stent patency and patient survival compared to insertion of a second metal stent. However, this procedure is safe, feasible, and certainly as good as a second stent placement. It may be an alternative to insertion of a second stent within the occluded stent.

Tissue distribution and general safety of MnDPDP in male beagle dogs, with or without total common bile duct obstruction.

PURPOSE: Evaluation of the tissue distribution of manganese (Mn) and general safety in normal and cholestatic male beagle dogs after i.v. administration of mangafodipir trisodium (MnDPDP, Teslascan). MATERIAL AND METHODS: Male beagle dogs, with or without surgical obstruction of the common bile duct, received a single i.v. bolus injection of saline (control), or MnDPDP at doses of 10 or 50 mumol/kg b.w. and were sacrificed 1 or 7 days after treatment. Toxicity was assessed and tissue concentrations of Mn were measured. RESULTS: Increased tissue Mn concentrations were found in all dogs treated with MnDPDP and were greatest in those with biliary obstruction. Although Mn concentrations decreased with time in most tissues in each of the treated groups, this was not the case for the brain and adrenal glands in dogs with total biliary obstruction in which further increases in Mn concentrations were seen at the later time point. This suggested a re-distribution of Mn from the major body depots such as the liver. There were no effects of MnDPDP on clinical signs/behaviour, organ weights, histomorphology or clinical biochemistry. CONCLUSION: These findings indicate that a single clinical dose of 5 mumol/kg MnDPDP is likely to be well tolerated in patients with cholestasis.

Colangite obstrutiva aguda / Acute obstructive cholangitis

Os autores discutem vários aspectos da colangite obstrutiva aguda com ênfase especial ao quadro clínico, diagnóstico diferencial e tratamento. Com relaçäo à etiologia referem que a coledocolitíase constituía-se na principal causa da colangite aguda. Entretanto, nos últimos anos, especialmente em centros de atendimento terciário, as manipulaçöes biliares näo cirúrgicas, geralmente em pacientes com neoplasias malignas irressecáveis, tornaram-se as causas mais frequentes de colangite. Enfatizam que o prognóstico é ruim, se a terapêutica instituida for retardada ou mal indicada. Finalmente, referem que cabe ao médico entender que pacientes com colangite obstrutiva aguda devem ser sempre tratados em caráter de urgência, tanto no que diz respeito ao diagnóstico como à terapêutica

The bile ducts after a fatty meal: further sonographic observations.

The sonographic appearance of the response of the common hepatic duct to physiologic stimulation by a fatty meal was assessed in 131 patients referred because of right upper quadrant symptoms or abnormal liver chemical studies. In the determination of the presence or absence of biliary obstruction, the sensitivity of the examination was 84%, the accuracy of a positive test was 84%, and the accuracy of a negative test was 93%. This test proved helpful in several circumstances: equivocal duct caliber (6-10 mm); abnormal caliber (6-14 mm) with normal laboratory values; normal caliber duct with abnormal laboratory values; persistent question of cholelithiasis or asymptomatic pancreatic duct dilatation. Measurements of bile duct caliber alone may be insufficient to ascertain the presence of bile duct obstruction and fatty meal stimulation significantly improves diagnostic accuracy.