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  • 1
    Electronic Resource
    Electronic Resource
    Metabolism of paracetamol in children with chronic liver disease (1996)
    Springer
    European journal of clinical pharmacology 50 (1996), S. 69-76 
    Details
    ISSN: 1432-1041
    Keywords: Key words Acetaminophen ; Liver disease in children ; toxicity ; analgesics ; antipyretics pharmacokinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Abstract Objective: To study the metabolism of single doses of paracetamol in paediatric patients with chronic liver disease admitted to a hospital liver disease clinic. Results: Thirteen paediatric patients, aged 7 months to 12 years, with chronic liver disease of varying severity were studied. In these children, paracetamol elimination half-life was negatively correlated with serum albumin and positively with prothrombin time, as previously reported in adults with liver disease. The rate constant of glucuronide formation was higher in the children with liver disease compared to the value reported in healthy children of similar ages. The rate constant of the formation of paracetamol sulphate was no different from that in normal children. The 36 h urinary paracetamol glucuronide to sulphate ratio was 1.4 (95% CI 0.8 to 1.7). This mean ratio was higher than in healthy children (0.81 and 0.75) but not significantly so, probably because of a Type 1 error due to the inevitable small sample size arising from the nature of the population being studied. Conclusion: The present study provides reassuring additional data to indicate that, at least for single doses, there is no cause for concern in the use of paracetamol in children with chronic liver disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002280050071
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  • 2
    Electronic Resource
    Electronic Resource
    Indications and outcome of liver transplantation in tyrosinaemia type 1 (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S049 
    Details
    ISSN: 1432-1076
    Keywords: Key words Tyrosinaemia type 1 ; Liver transplantation ; AbbreviationsAFPα-fetoprotein ; ALF acute liver failure ; cGFR glomerular ; filtration rate using height:creatinine ratio ; CLF chronic liver failure ; FAH fumarylacetoacetase ; HCC hepatocellular carcinoma ; HD hepatic dysplasia ; NTBC 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cylohexanedione ; OLT orthotopic liver transplantation ; SA succinylacetone ; TT1 tyrosinaemia type 1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A retrospective analysis was performed on 17 patients presenting with tyrosinaemia type 1 (TT1) between 1989–1997; 7 pre 1992 prior to the introduction of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) therapy and 10 post 1992. During this time, eight children (5 males) underwent orthotopic liver transplantation (OLT); six prior to the introduction of NTBC in 1992 and two on NTBC therapy. The primary indications for OLT pre-1992 were risk of hepatocellular carcinoma with evidence of hepatic dysplasia in all, associated with liver failure in two, and rise in α-fetoprotein (AFP) in four. Two of the ten treated with NTBC required OLT. The indications were non-response to NTBC in one child and development of hepatic dysplasia associated with poor quality of life in the second patient. Median age for OLT was 64 months (range 5–127 months) with a median weight of 24 kg (range 6–25 kg). The histology of hepatectomy specimens at transplantation showed: cirrhosis in 8, hepatic dysplasia in 6 and hepatocellular carcinoma in 1. Plasma tyrosine and AFP returned to normal in all cases. Urinary succinylacetone reduced but persisted in small amounts (median 7.7 μmol/mmol creatinine). Hypertrophic cardiomyopathy resolved in 3/3 patients. Hypoglycaemia, not responding to dietary therapy or NTBC treatment, resolved post-transplant in one patient. There were two deaths, one from primary non-function and one from chronic rejection. Late complications in survivors (n=6) include post-transplant lymphoproliferative disease of the iris in one child which resolved and renal dysfunction with a fall in glomerular filtration rate in three (50%). Median follow up post OLT is 6.7 years (range 1–7 years). Quality of life post-transplant in survivors is good with unrestricted diet in all. Conclusion Liver transplantation is an effective treatment for TT1 with good quality of life. The current indications of OLT in TT1 are non-response to NTBC, risk of malignancy and poor quality of life related to dietary restriction and frequency of blood sampling.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014321
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  • 3
    Electronic Resource
    Electronic Resource
    Liver transplantation for methylmalonic acidaemia (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S070 
    Details
    ISSN: 1432-1076
    Keywords: Key words Methylmalonic acidaemia ; Liver transplantation ; Kidney transplantation ; Chronic renal failure ; AbbreviationsMMA methylmalonic acidaemia ; CRF chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The outcome for children with severe forms of methylmalonic acidaemia remains poor. Patients have recurrent episodes of metabolic decompensation; many have neurodevelopmental complications and the mortality is high. Long-term survivors develop chronic renal failure. Because of the poor prognosis, transplantation has been considered. In young patients with early onset disease, liver transplantation might prevent complications and, for those in end-stage renal failure, kidney transplantation could be combined with that of the liver. The results of liver transplantation in the early onset patients have generally been disappointing. In particular there appears to be a high risk of neurological complications. The optimal management of those in end-stage renal failure has not yet been determined although combined liver and kidney transplantation has been successful. Conclusion The role of transplantation in methylmalonic acidaemia has yet to be established and follow up of all patients who are considered for transplantation is essential.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014326
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  • 4
    Electronic Resource
    Electronic Resource
    Liver transplantation in mitochondrial respiratory chain disorders (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S081 
    Details
    ISSN: 1432-1076
    Keywords: Key words Respiratory chain ; Mitochondria ; Children ; Infancy ; Liver failure ; AbbreviationsMRCD mitochondrial respiratory chain disease ; OLT orthotopic ; liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mitochondrial respiratory chain disease may lead to neonatal or late onset liver failure, requiring liver transplantation. In rare cases, the disease is restricted to the liver and the patient is cured after surgery. More frequently, other organs are simultaneously involved and neuromuscular or other extra-hepatic symptoms may pre-exist, or appear in the post-transplant follow up. Pre-transplant evaluation should aim to rule out neurological disease, which may be difficult to differentiate from signs accompanying liver insufficiency. Cerebrospinal fluid lactic acid levels, compared to blood lactate, may be suggestive of central nervous system involvement. Of 11 cases with respiratory chain disorders who had liver transplantation in various centres, 4 are alive and well on follow up, and 6 died, three of them having developed neurological disease post orthotopic liver transplantation. All three patients with initial liver and gastro-intestinal disease died early after transplantation, indicating that these may be poor candidates for this procedure. Conclusion Liver transplantation is feasible in hepatic respiratory chain disorders, but extra-hepatic disease should be ruled out before transplantation. Extra-hepatic manifestations may, however, appear and cause patient death despite successful transplantation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014328
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