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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 158 (1999), S. S070 
    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
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1989), S. 344-348 
    ISSN: 1432-1076
    Keywords: Methylmalonic acidaemia ; Chronic renal failure ; Tubulo-interstitial nephritis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The renal function of 12 patients with non vitamin B12 responsive methylmalonic acidaemia has been investigated. Eight patients had reduced glomerular filtration rates, but the plasma creatinine concentration was only raised in those with values of less than 40ml/min per 1.73m2 surface area. The reduction in glomerular filtration was a function of the age and the severity of the disease. Plasma urate concentrations were increased in four patients but this may be secondary to the renal disease rather than its cause.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Environmental biology of fishes 28 (1990), S. 33-75 
    ISSN: 1573-5133
    Keywords: Sharks ; Rays ; Chimaeras ; Chondrichthyes ; Evolution ; Success ; Diversity ; Ecology ; Ecomorphotypes ; Reproductive modes ; Conservation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Synopsis Cartilaginous fishes, the sharks, rays and chimaeras (class Chondrichthyes), are a very old and successful group of jawed fishes that currently contains between 900 and 1100 known living species. Chondrichthyians show a high morphological diversity during most of their evolutionary career from the Paleozoic to the present day. They are relatively large predators which have remained a major, competitive element of marine ecosystems despite the varied rivalry of numerous other marine vertebrate groups over at least 400 million years. Although restricted in their ecological roles by morphology, reproduction and other factors, the living cartilaginous fishes are highly diverse and show numerous alternative life-history styles which are multiple answers to exploiting available niches permitted by chondrichthyian limitations. Chondrichthyians living and fossil can be divided into at least eighteen ecomorphotypes, of which the littoral ecomorphotype is perhaps the most primitive and can serve as an evolutionary origin for numerous specialist ecomorphotypes with benthic, high-speed, superpredatory, deep-slope and oceanic components. Reproductive modes in cartilaginous fishes are of six types, ranging from primitive extended oviparity through retained oviparity and yolk-sac viviparity (previously ovoviviparity) to three derived forms of viviparity. Reproductive modes are not strongly correlated with ecomorphotypes and with the phylogeny of living elasmobranchs. The success and importance of cartilaginous fishes is largely underrated by marine biologists and by the public, and requires new and ‘heretical’ emphasis to overcome the present inadequacies of chondrichthyian research and the problems of overexploitation that cartilaginous fishes face.
    Type of Medium: Electronic Resource
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