Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Significant liver disease developed in 14 patients after renal transplantation. Nine patients had morphologic and functional evidence of chronic active hepatitis. In general, these patients had few symptoms of liver disease, even though the course of chronic active hepatitis was progressive. Despite large doses of prednisone, cirrhosis ultimately developed in five patients. The cause of chronic active hepatitis could not be related to azathioprine or methyldopa therapy because there was no perceptible change in the course of liver disease after treatment with these drugs was stopped. Three patients were persistently positive for hepatitis B surface antigen. Isolated instances of granulomatous hepatitis (Mycobacterium kansasii) and of prolonged intrahepatic cholestasis were encountered in patients with chronic active hepatitis. Two patients had acute cytomegalovirus hepatitis. There was one episode each of fulminant herpes simplex hepatitis and severe fatty metamorphosis.
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PMID:Liver disease in renal transplant recipients. 18 93

During the last 31 months, 50 children between 3 months and 15 years of age have undergone living related liver transplantation (LRLT) for end-stage liver diseases (39 biliary atresia, 2 Budd-Chiari syndrome, 2 progressive intrahepatic cholestasis, 3 liver cirrhosis, 1 Wilson disease, 1 protoporphyria, 1 tyrosinemia, and 1 fulminant hepatitis). Combined FK-506 and low-dose steroids were routinely used for immunosuppression. There were seven deaths, two of which were related to infection (Candida pneumonia and Epstein-Barr virus [EBV]-associated lymphoproliferative syndrome [LPS]). Five patients had a bacterial infection, all of which were associated with surgical complications. Three patients had Candida infection, all of which were malnourished, had biliary atresia, and had been managed with prolonged antibiotics against obstinate ascending cholangitis. There were 14 symptomatic viral infections (1 herpes simplex virus, 1 herpes zoster virus, 5 cytomegalovirus [CMV], 6 EBV, and 1 EBV-associated LPS). Three of the five CMV infections appeared in patients whose graft was ABO-incompatible, who were managed with prophylactic OKT-3. Most of the viral infections (except 1 EBV-associated LPS) were minor and were treated successfully. The low incidence and successful treatment of CMV infection are related to the high compatibility and low incidence of allograft rejection in LRLT. Bacterial and fungal infections can be decreased by greater refinement of surgical technique and more aggressive preoperative management. Treatment of EBV infection is still an unsolved problem.
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PMID:Infectious complications in living related liver transplantation. 801 5

Liver disease occurring in pregnancy can be categorized into three groups. The first group includes diseases unique to pregnancy and caused by it. Among these are hyperemesis gravidarum, cholestasis of pregnancy, and disorders associated with preeclampsia. Liver involvement may be expected in 50% of patients with hyperemesis gravidarum. Preeclampsia has been associated with both the HELLP syndrome (hemolysis, elevated liver tests, and low platelets), which includes hepatic infarction and rupture, and with acute fatty liver of pregnancy (AFLP). In patients with HELLP syndrome, liver test abnormalities do not correlate with liver injury. Therefore, this and other disorders associated with preeclampsia require aggressive treatment, primarily with delivery. The second group of liver diseases are those exacerbated by pregnancy. Viral infections involving the liver that are usually benign, such as hepatitis E and herpes simplex, are more likely to be exacerbated in pregnant women and are more likely to lead to fulminant hepatic failure. Cholelithiasis and Budd-Chiari syndrome are more prevalent in pregnant women. The third group is comprised of liver diseases that are preexisting in the pregnant patient and includes autoimmune chronic active hepatitis and Wilson's disease. The number of patients in the last group is small, as chronic liver disease is rare in women who are able to bear children.
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PMID:Hepatic disease in pregnancy. 810 83

In the case of the central nervous system or hepatic involvement, the prognosis of neonatal herpes simplex infection remains poor, despite antiviral drugs, presumably effective if given early. We report the case of a neonate with herpes simplex hepatitis, where the course of the illness was unusual with chronic, ultimately fatal, cholestasis. The treatment was not effective, because its administration was delayed, because of high infant C reactive protein level and the absence of clinical maternal genital infection, and because it was interrupted due to misleading information: clinical improvement, negative viral tests and raised herpes IgG antibody titer.
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PMID:Herpes simplex hepatitis with chronic cholestasis in a newborn. 849 96

Due to the urgency in choosing either clinical treatment or immediate surgical intervention, the study of the prolonged neonatal cholestasis involves two basic aims: the differential diagnosis between biliary atresia and neonatal hepatitis and the research into the associated etiological agents. So, in a prospective trial carried out in the 70's, 77 children with prolonged neonatal cholestasis were studied in order to establish the differential diagnosis between biliary atresia and neonatal hepatitis, followed by the evaluation of 108 children towards a pathogenesis of the prolonged neonatal cholestasis. The results of the differential diagnosis showed that within 18 items examined only 8 proved to be good biliary atresia indicators. They are as follows (in decreasing order): ductular proliferation (portal tracts), fibrosis (portal tracts), cholestasis (portal tracts), stools colour--acholia, hepatomegaly, canalicular cholestasis (lobule), infiltrate (portal tracts), giant cells (lobule). These eight items were then gathered in a sole indicator of great discriminative power, with a confidence level of 99%. The figures regarding the pathogenesis are: rubella virus 0%, herpes simplex virus 0%, listeriosis 0%, cytomegalovirus 2.2%, hepatitis B virus 2.4%, toxoplasmosis 2.8%, alpha-1-antitrypsin deficiency 13.1%, syphilis 21.1%, autoantibodies against the liver 58.4%. Such work thus revealed that those eight most important factors when differentiating biliary atresia from neonatal hepatitis remain as fundamental indicators and, when employed alongside other diagnostic methods, can help in the assembling of a multifactorial strategy less and less invasive and more precise. The pathogenic study, with its heavy dependency on time and place, has become more complete with the introduction of new diagnostic methods, evolving to the ideal progressive reduction of idiopathic processes.
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PMID:[Prolonged neonatal cholestasis: prospective study]. 1088 10

Syncytial giant cell hepatitis in the neonatal period has been associated with many different etiologic agents and may present initially as cholestasis. Infectious causes are most common and include: (1 ) generalized bacterial sepsis, (2) viral agents, (3) toxoplasmosis, (4) syphilis, (5) listeriosis, and (6) tuberculosis. Viral hepatitis may be due to cytomegalovirus, rubella virus, herpes simplex, HHV-6, varicella, coxsackievirus, echovirus, reovirus 3, parvovirus B19, HIV, enteroviruses, paramyxovirus, and hepatitis A, B, or C (rare). Giant cell hepatitis may result in fulminant liver failure with massive hepatocyte necrosis and severe liver dysfunction leading to death, resolution with severely compromised liver function, or liver transplantation. The authors report a 6-week-old male who had an unremarkable perinatal period, became jaundiced after developing diarrhea, and subsequently developed liver dysfunction with massively increased liver enzymes and a coagulopathy. Open wedge and core liver biopsies were performed to determine if the patient should be listed for liver transplantation. Giant cell hepatitis with a significant mixed lymphocytic and neutrophilic infiltrate was present on both the wedge and core biopsies. The residual 60% of hepatocytes had ballooning degeneration and many possessed pyknotic nuclei. The hepatocytes were arranged in a pseudoacinar pattern. Electron microscopy showed paramyxoviral-like inclusions in the giant cells, characterized as large inclusions with fine filamentous, beaded substructures (18-20 nm). Paramyxoviridae are nonsegmented, negative-sense, single-stranded RNA viruses. This family is divided into the Paramyxovirinae subfamily containing respirovirus (Sendai virus, parainfluenza virus type 3), rubulavirus (mumps, parainfluenza virus type 2), and morbillivirus genera (measles); and Pneumovirinae subfamily (pneumovirus genus [respiratory syncytial virus]). Supportive care to determine if hepatic function resolves following the viral episode, liver transplantation with fulminant liver failure, and ongoing evaluation in those who recover to assess chronic liver disease are necessary. Ultrastructural evaluation may unmask the etiologic agent for hepatitis and direct therapy.
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PMID:Neonatal syncytial giant cell hepatitis with paramyxoviral-like inclusions. 1129 22

Liver diseases specific of pregnancy, the most common hepatic complications of pregnancy, are always associated with a sometimes asymptomatic increase in serum aminotransferase activity. The most frequent of the liver diseases specific of pregnancy in normotensive pregnant women is cholestasis of pregnancy, the cause of generalised pruritus, and, in those with pregnancy-induced hypertension, preeclampsia which requires short-term cessation of pregnancy. Similar treatment is required by acute fatty liver of pregnancy the diagnosis of which must be done in the third trimester when recent polydipsia, nausea or vomiting occurs. Moreover, pregnancy increases the incidence and/or the severity of herpes simplex hepatitis (for which acyclovir therapy is urgently required) and hepatitis type E. Pregnancy may also unmask untreated cases of autoimmune hepatitis, Wilson's disease or Budd-Chiari syndrome.
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PMID:[Hepatic complications of pregnancy]. 1472 76

A 23-year-old woman presented to our polyclinic complaining of itching, generalized dermatitis, and jaundice. She was in her 31st gestational week and had developed pruritus and the dermatitis since the first month of pregnancy; her jaundice started about a month before presentation. Her history included similar complaints in a previous pregnancy, which resulted in premature birth of a baby with a permanent brain defect. One of her sisters had had jaundice and itching in her 27th gestational week and delivered a healthy baby; a second sister had experienced itching and dermatitis in her second trimester and delivered a healthy baby. Physical examination of the patient showed that her eyes were jaundiced (Figure 1); skin examination revealed generalized erythematous excoriated papules, symmetrically distributed all over her body (Figure 2 Figure 3). Laboratory analyses revealed the following results: leukocyte count, 14.30/mm(3) (3.8-10.3/mm(3)); erythrocyte sedimentation rate, 25 mm/h (<20 mm/h); aspartate aminotransferase, 44 U/L (5-40 U/L); alanine aminotransferase, 63 U/L (5-40 U/L); lactate dehydrogenase, 1158 U/L (220-450 U/L); total bilirubin, 6.88 mg/dL (<1.10 mg/dL); and direct bilirubin, 3.27 mg/dL (<0.35 mg/dL). Urinalysis results were positive for bilirubin and urobilinogen. Positive serologic findings included rubella immunoglobulin G, 93 AU/mL (<15) and cytomegalovirus, 188 AU/mL (<10); negative findings included herpes simplex virus type 2 and hepatitis. Histopathologic examination of material collected from the left breast via punch biopsy showed parakeratosis, acanthosis, and perivascular lymphocyte infiltration in dermal vessels. Treatment with 2 g/d cholestyramine and a topical corticosteroid was effective in the patient, who was diagnosed with intrahepatic cholestasis of pregnancy and prurigo of pregnancy based on the clinical, histopathologic, and laboratory findings. To the authors' knowledge, this is the first such reported case in the literature.
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PMID:Intrahepatic cholestasis occurring with prurigo of pregnancy. 1797 52

Hepatic disorders severely affected by pregnancy include choledochal cysts that can be compressed by the gravid uterus and potentially rupture; hepatic adenomas that exhibit accelerated growth because of hyperestrogenemia during pregnancy; acute intermittent porphyria that is exacerbated by increased female sex hormones during pregnancy; splenic artery aneurysms that can rupture during pregnancy because of compression by the gravid uterus; Budd-Chiari syndrome that is promoted by hyperestrogenemia; and hepatitis E and herpes simplex hepatitis that are particularly severe during pregnancy. Hepatic disorders unique to pregnancy include intrahepatic cholestasis of pregnancy; acute fatty liver of pregnancy; preeclampsia and eclampsia; and hemolysis, elevated liver function tests, and low platelet count (HELLP) syndrome. Most disorders uniquely related to pregnancy are treated by prompt fetal delivery as soon as the fetus is sufficiently mature.
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PMID:Hepatic disorders severely affected by pregnancy: medical and obstetric management. 1857 Sep 41

A middle-aged man received a kidney transplant from a deceased multi-organ donor. The recipient suffered cardiac arrest several days post-operatively and sustained hypoxic brain injury and was declared brain dead. Following the family's consent, the allograft kidney was retrieved and re-transplanted into a man with end-stage renal failure secondary to reflux nephropathy. The liver was not transplanted due to suspicion of fatty changes based on macroscopic appearance. After transplantation of other organs, liver histology revealed coagulative parenchymal necrosis with nuclear inclusions and moderate parenchymal cholestasis, suggestive of herpes viral hepatitis. Renal implantation biopsy showed histiocytes with enlarged nuclei containing viral inclusions in the capsular fibrous tissue, with positive immunostaining for herpes simplex virus (HSV). Anti-viral therapy was commenced immediately after obtaining histological evidence of donor HSV infection. Our recipient had pre-formed immunoglobulin G antibodies to HSV-1 and HSV-2, and was immunoglobulin M negative pre-transplant. HSV viraemia was detected day 5 post-transplant with a viral load of 7688 copies/mL by polymerase chain reaction assay. The recipient completed a 30 day course of intravenous ganciclovir before switching to oral valganciclovir as standard cytomegalovirus prophylaxis. The HSV polymerase chain reaction became undetectable on day 7 of intravenous ganciclovir and has remained undetectable. The patient remains well 9 months post-transplant with an estimated glomerular filtration rate of 61 mL/min per 1.73 m(2). Although renal allograft re-use has been shown to be technically possible with a good outcome in this recipient, this does raise issues including assessment of allografts that have undergone repeated severe ischaemic insults and the potential of transmission of infections.
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PMID:Renal allograft re-use and herpetic re-infection. 2580 53


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