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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cholestasis often occurs in infants on total parenteral nutrition (TPN) for long periods. Amino acid formulations developed specifically for infants, namely Aminosyn PF and Trophamine, may protect against cholestasis associated with total parenteral nutrition (CATPN). The development of cholestasis may also be caused by other risk factors such as prematurity, surgery, sepsis, and extracorporeal membrane oxygenation (ECMO). To evaluate the relative effectiveness of the pediatric amino acid formulations in reducing CATPN, the courses of 70 infants < 1 year of age who received TPN for at least 14 days were reviewed. Cholestasis was defined as a conjugated serum bilirubin > or = 2 mg/dl subsequent to the initiation of TPN; CATPN was considered present when other factors related to cholestasis were ruled out. Liver function tests were recorded 24 h before starting TPN and at day 7, 15, and 21 during TPN infusion. Thirty infants (42.8%) developed cholestasis. CATPN was judged to have occurred in 15 (21.4%) of 70 infants, while 15 (21.4%) developed cholestasis secondary to other factors. Of the 15 CATPN patients, 7 had received Trophamine, 6 had received Aminosyn PF, and 2 had received both solutions. Aminosyn PF and Trophamine, along with other potential risk factors for CATPN such as antecedent surgery, sepsis, ECMO, prematurity, and nitrogen/calorie intake were analyzed by regression-analysis methods. None was statistically significant except the length of TPN (p = 0.0063). In conclusion, we cannot support the view that Trophamine is more effective than Aminosyn PF in the prevention of CATPN.
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PMID:Aminosyn PF or trophamine: which provides more protection from cholestasis associated with total parenteral nutrition? 858 87

The medical records of 74 neonates dependent on parenteral nutrition for at least 21 days after emergency abdominal surgery (performed between 1988 and 1992) were reviewed respectively. The role of enteral starvation, prematurity, composition and duration of parenteral nutrition, and sepsis in the evolution of parenteral nutrition-related cholestasis was evaluated by multiple regression analysis. The most important factors for cholestasis were low gestational age (median, 34 weeks), early exposure to parenteral nutrition, and sepsis. Episodes of sepsis were associated with a 30% increase in the bilirubin level. Enteral starvation and composition and the duration of parenteral nutrition solutions did not correlate significantly with the development of cholestasis. Prevention of sepsis should be the priority in minimising cholestasis in postsurgical neonates who are dependent on parenteral nutrition.
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PMID:Parenteral nutrition-related cholestasis in postsurgical neonates: multivariate analysis of risk factors. 880 24

To evaluate the incidence, clinical course, and possible risk factors of cholestasis in very low-birth-weight infants. A retrospective study of 143 very low-birth-weight infants was performed. Cholestasis was defined as direct-reacting bilirubin > 2 mg/dL for more than 14 days. The clinical course of cholestasis was described, and perinatal risk factors were evaluated for associations with the development and severity of cholestasis. Cholestasis was present in 31 infants (21.7%). The mean (SD) age of onset was 30.3(15.3) days after birth or 26.0 (15.6) days after receiving parenteral nutrition, and the mean (SD) duration was 77.1 (33.8) days. In half of the cholestatic infants, bilirubin continued to rise after discontinuing parenteral nutrition. One infant developed signs of liver cirrhosis and died, two infants died with progressive cholestasis, while the other 28 patients recovered. Analysis of risk factors revealed that birthweight and duration of fasting significantly correlated with the development of cholestasis, and that sepsis significantly influenced the severity of cholestasis. Cholestasis is a common complication of extreme prematurity. The clinical course seems benign but long-term sequelae are unknown. Immature liver function and absence of stimuli for intestinal motility and hormonal secretion predispose to decreased bile flow, while sepsis further impairs hepatic ductular secretion and aggravates cholestasis.
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PMID:Risk factors of cholestasis in very low-birth-weight infants. 885 50

The aim of this prospective study was to analyze the characteristics of intrahepatic cholestasis of pregnancy (ICP) in a French population. From 1989 to 1995 we studied 50 consecutive pregnant women with ICP (41 single, 7 twin, and 2 triplet pregnancies) referred for hepatologic consultation. All patients suffered from pruritus and/or jaundice associated with elevated fasting serum levels of total bile acids (mean 49 micromol/L, range 7-290). No patients had concomitant liver disease and all recovered normal liver function after delivery. Overall prematurity rate was 60%: 100% in multiple pregnancies and 41% in single pregnancies. Three of 61 babies died. Systematic clinical interviews revealed that 34 patients had been treated with oral micronized natural progesterone (200-1,000 mg/d) during the current pregnancy for risk of premature delivery, including at least 32 (64%) before the onset of pruritus. Onset of pruritus was statistically earlier in patients previously receiving progesterone than in patients not receiving progesterone (217 +/- 21 vs. 240 +/- 26 days, P < .01). This was also found in the single pregnancy subgroup of patients (222 +/- 19 vs. 240 +/- 26 days, P < .05). Pruritus disappeared before delivery in 10 of 50 patients, i.e., after withdrawal of progesterone in 7 patients (only one concurrently treated with cholestyramine), after decrease in dose of progesterone in 1 patient, and spontaneously in 2 patients. During the same period, the percentage of pregnant women without ICP who had been treated with progesterone during pregnancy was statistically lower than the percentage of patients treated with progesterone before the onset of pruritus in our group of patients with ICP (36% vs. 64%, P < .01, odds ratio 3.16, 95% CI:1.29-7.80). These results suggest that orally administered progesterone might be an exogenous factor which triggers ICP in predisposed women.
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PMID:Intrahepatic cholestasis of pregnancy: a French prospective study. 925 46

Total parenteral nutrition (TPN)-induced liver disease develops in 40-60% of infants who require long-term TPN for intestinal failure. The clinical spectrum includes cholestasis, cholelithiasis, hepatic fibrosis with progression to biliary cirrhosis, and the development of portal hypertension and liver failure in a significant number of children who are totally parenterally fed. The pathogenesis is multifactorial and is related to prematurity, low birth weight, and duration of TPN. The degree and severity of the liver disease is related to recurrent sepsis including catheter sepsis, bacterial translocation, and cholangitis. Lack of enteral feeding leading to reduced gut hormone secretion, reduction of bile flow, and biliary stasis may be important mechanisms in the development of cholestasis, biliary sludge, and cholelithiasis. Although it is unlikely that modern TPN solutions have a major role in the etiology of TPN liver disease, manganese toxicity recently has been recognized in children with hepatic dysfunction on TPN. Although there is a definite relationship with the degree of manganese toxicity and hepatic decompensation, it is not yet clear whether this is a primary mechanism or whether the high levels are related to reduced biliary excretion of manganese. The management strategies for the prevention of TPN-induced liver disease include early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition, and aseptic catheter techniques to reduce sepsis. The administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder and intestinal stasis. As survival from isolated intestinal transplantation improves, this therapeutic option should be considered before TPN liver disease becomes irreversible and combined liver and small bowel transplantation is required.
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PMID:Liver complications of pediatric parenteral nutrition--epidemiology. 943 2

The women who are suffering from chronic renal failure in an advanced stage have a deficient fertility but they are not sterile. Hemodialysis has improved considerably the fertility of these patients. The aim of this study is to give the results of our experience, from 1990 to 1996, about pregnancies among the uremic patients, dialysed or no and to make a literature review about this subject. We have noticed that pregnancies in the dialysis patients are rare and their evolution is precarious. We have also noticed more miscarriage or pregnancy interruption. Complications are frequent. Mothers have a high risk of hemorrhagic accident (ablatio placentae), of anemia aggravation, of thrombosis of the vascular approach and a high risk of liver anomalies (gravidic cholestasis). The fetus suffers from the maternal anemia and from chronic hypoxia. He's threatened by hydramnios in the case of bad volemic supervision. The intra uterine delayed developement and the prematurity are usual. The absence of high blood pressure and a residual renal function are representing the favourable elements of the good march of pregnancy. A therapeutic intensification is necessary in order to lead this pregnancies to a viable term. The management is heavy not only for the nephrologic, the obstetrical and the neonatal physicians, but also for the patient who is the only one who can decide to continue or to interrupt the pregnancy. It seems better to inform the patient rather than to procure her abortion by proposing her an effective and inoffensive contraceptive method meanwhile to be pregnant after renal transplantation.
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PMID:[Chronic renal failure and pregnancy]. 1126 43

Parenteral nutrition is a life-saving therapy for patients with intestinal failure. It may be associated with transient elevations of liver enzyme concentrations, which return to normal after parenteral nutrition is discontinued. Prolonged parenteral nutrition is associated with complications affecting the hepatobiliary system, such as cholelithiasis, cholestasis, and steatosis. The most common of these is parenteral nutrition-associated cholestasis (PNAC), which may occur in children and may progress to liver failure. The pathophysiology of PNAC is poorly understood, and the etiology is multifactorial. Risk factors include prematurity, long duration of parenteral nutrition, sepsis, lack of bowel motility, and short bowel syndrome. Possible etiologies include excessive caloric administration, parenteral nutrition components, and nutritional deficiencies. Several measures can be undertaken to prevent PNAC, such as avoiding overfeeding, providing a balanced source of energy, weaning parenteral nutrition, starting enteral feeding, and avoiding sepsis.
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PMID:Parenteral nutrition-associated liver complications in children. 1183 58

A heterogenic group The dermatoses specifically associated with pregnancy are generally benign, even if they lead to great discomfort. Some of them require appropriate management because of the risk of maternal or foetal complications. Pruritus of pregnancy Corresponds to intra-hepatic cholestasis of pregnancy, present in 1 to 2% of pregnant women, it may place the foetus at risk of delayed growth, prematurity or even death in utero. Treatment relies on cholestyramine. Pemphigoid gestationis or herpes gestationis This is an autoimmune disease of pregnancy occurring in multiparous women between the 28th and 32nd week of amenorrhoea (once out of 5 times within the first 7 days postpartum). Diagnosis is confirmed by histological examination of a bullous lesion. The disease usually regresses spontaneously after delivery, generally within a period of 1 to 17 months. Relapses, earlier on and more severe than during the initial episode, occur in 50 to 70% of subsequent pregnancies. Treatment is essentially based on corticosteroids: local Class II for the pauci-bullous and/or limited forms and general corticosteroid therapy for the severe forms. Polymorphic dermatitis of pregnancy This regroups various and similar entities described during pregnancy. They start during the third trimester of pregnancy, their clinical aspects are similar, their histology is non specific, the biological explorations are normal, direct immunofluorescence is negative, the progression is favourable for the mother and the child and the pathogenesis is unknown. Impetigo herpetiformis This exceptional dermatosis affects the primiparous woman in 80% of cases and the eruption usually appears during the 3rd trimester of pregnancy, or even after delivery. Relapses are constant during subsequent pregnancies.
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PMID:[Specific dermatoses of pregnancy]. 1466 85

Intrahepatic cholestasis of pregnancy (ICP) is a liver disorder associated with increased risk of intrauterine fetal death and prematurity. There is increasing evidence that genetically determined dysfunction in the canalicular ABC transporters bile salt export pump (BSEP, ABCB11) and multidrug resistance protein 3 (MDR3, ABCB4) might be risk factors for ICP development. This study aimed to (i). describe the extent of genetic variability in BSEP and MDR3 in ICP and (ii). identify new disease-causing mutations. Twenty-one women with ICP and 40 women with uneventful pregnancies were recruited between April 2001 and April 2003. Sequencing of BSEP and MDR3 spanned 8-10 kb per gene and comprised the promoter region and 100-350 bp of the flanking intronic region around each exon. DNA sequencing of polymerase chain reaction fragments was performed on an ABI3700 capillary sequencer. MDR3 promoter activity of promoter constructs carrying different ICP-specific mutations was studied using reporter assays. A total of 37 and 51 variant sites were detected in BSEP and MDR3, respectively. Three non-synonymous sites in codons for evolutionarily conserved amino acids were specific for the ICP collective (BSEP, N591S; MDR3, S320F and G762E). Furthermore, four ICP-specific splicing mutations were detected in MDR3 [intron 21, G(+1)A; intron 25, G(+5)C and C(-3)G; and intron 26, T(+2)A]. Activity of the mutated MDR3 promoter was similar to that observed for the wild-type promoter. Our data further support an involvement of MDR3 genetic variation in the pathogenesis of ICP, whereas analysis of BSEP sequence variation indicates that this gene is probably less important for the development of pregnancy-associated cholestasis.
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PMID:Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p-glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy. 1507 10

The increasing number of successfully treated newborns with disturbances of the adaptive period can measure effectiveness of neonatal intensive care units (NICU). The authors summarized some health problems of the former NICU patients. Central nervous system (CNS) of the premature, especially low (LBW) and very low birth weight (VLBW) infant, is in serious danger of improper development. The lack of neurological disturbances in early infancy does not exclude further pathological events and such patients require long-term observation and follow-up. High-risk newborns are of great need of precise imaging and diagnosis of CNS, which should be performed before discharge. Early stimulation and developmental care program are the crucial points in the management of newborns with neurological abnormalities. Sensorial problems, especially the rate of vision and hearing impairments, can be significantly reduced when prophylactic schedule is applied. Both, etiological factors leading to respiratory insufficiency and some iatrogenic effects of the therapy, can influence respiratory system. Symptoms of different intensity--from sneezing caused by nasal mucous membrane edema to the severe forms of bronchopulmonary dysplasia (BPD)--may last for a long time and influence the child's general condition. The surgical closure of Botall's duct (persistent ductus arteriosus--PDA) usually normalizes the circulatory system. In serious heart defects or isolated shunt-type defects, the compensation of the circulatory system can easily be disturbed. Digestive tract problems belong to the two main groups: first--connected with the prematurity and immature interstitial functioning (necrotizing enterocolitis, digestive and absorption abnormalities, hepatic cholestasis), and second--caused by congenital defects requiring immediate surgical intervention. Despite different types of the diseases, the treatment and care have to be focused on necessary for the growing organism, optimal nutrients and calories uptake. Disturbances of social development, improper behavior, adaptation and emotional problems are mainly presented in VLBW patients and those with organic defects of the central nervous system.
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PMID:[Neonatal intensive care unit--therapeutical success and what next?]. 1586 51


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