22 research outputs found

    Energy-financial flows of an enterprise

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    Energy, financial, material and other interrelation set common presentation formed on enterprises can be sum in energy-financial flows. Such balances composition and analysis regular practice provides during several flows forming determine contradictions in time, put value of energy efficiency indexes. And, in case of their increasing quickly make management decision. In this way energy-financial balance is enterprise operating managemen

    S085 Hepatic complications of protoporphyria

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    Homozygous acute intermittent porphyria in a 7-year-old boy with massive excretions of porphyrins and porphyrin precursors.

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    Item does not contain fulltextA 7-year-old boy demonstrating hepatosplenomegaly, mild anaemia, mild mental retardation, yellow-brown teeth and dark red urine had excessively elevated levels of urinary delta-aminolevulinic acid, porphobilinogen and uroporphyrin. Furthermore hepta-, hexa-, penta- and copro(I)porphyrins were highly increased in urine. This pattern of porphyrin precursor and metabolite excretion is characteristic of acute intermittent porphyria. The decreased copro(III)/copro(I+III) ratio, normally not found in acute intermittent porphyria, is discussed. The porphobilinogen deaminase activity in red cells was decreased to 2-4%. Mutation analysis revealed a novel homozygous L81P mutation in exon 6 of the porphobilinogen deaminase gene. The father and mother, shown to be gene carriers of the same mutation, are asymptomatic and have normal urinary porphyrin precursor and metabolite excretion
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