Titlebook: Inborn Metabolic Diseases; Diagnosis and Treatm John Fernandes (Professor Emeritus of Pediatrics), Book 20003rd edition Springer-Verlag Ber

clarify

Disorders of Fatty Acid Oxidationhreatening episodes of hypoketotic, hypoglycemic coma induced by fasting [1–3]. In some of the disorders, there may also be chronic skeletal-muscle weakness or acute exercise-induced rhabdomyolysis and acute or chronic cardiomyopathy. Recognition of the fatty acid-oxidation disorders is often diffic

放逐某人

上下连贯

Defects of the Respiratory Chaintricted to the neuromuscular system but proceeds in all cells that contain mitochondria (Fig. 13.1). Most non-neuromuscular organs and tissues are, therefore, also dependent upon mitochondrial energy supply. Therefore, due to the twofold genetic origin of respiratory enzymes [nuclear DNA and mitocho

Inexorable

The Hyperphenylalaninaemiaste normal ranges for blood concentrations: phenylalanine 35–120 μmol/1; tyrosine 40–130 μmol/1). Hyperphenylalaninaemia arises due to a block in the conversion of phenylalanine into tyrosine due to a defect in either the enzyme phenylalanine hydroxylase (98% of subjects) or in the metabolism of the

abject

Disorders of Tyrosine Metabolismver disease and renal tubular dysfunction with rickets. Hereditary tyrosinaemia type II (Richner-Hanhart syndrome) presents with keratitis and blisterous lesions of the palms and soles. Tyrosinaemia type III may be asymptomatic or associated with mental retardation. Hawkinsinuria may be asymptomatic

Irremediable

矛盾

不如乐死去

Georges Van den Berghesure that their marketing spend produces reasonable returns. In my view, the field still lacks comprehensive models to link marketing spending and customer action. A number of customer-management authorities have developed customer life-cycle planning with conceptual notions of customer journey. Pro

仪式

Pascale de Lonlay,Jean-Marie Saudubrayegy. Traditional strategy development process applies tools and concepts like mission (defining the company’s purpose), vision (inspiring future results) and value statements to guide the company’s strategy development. However, due to changes and competition, a company cannot thrive by its internal

engender

equently, the product-mix of companies, spread across the product categories and product lines, are stuffed with a variety of brands with marginal differentiation. Such business situations exhibit chaos of brands within the product line triggering cannibalization of brands within the product line of