Organic Acids in Man

1. Front Matter

   Pages i-xiii

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2. Introduction

   1. Introduction

      • R. A. Chalmers, A. M. Lawson

      Pages 1-8

3. Methods

   1. Front Matter

      Pages 9-10

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   2. Extraction of organic acids from physiological fluids

      • R. A. Chalmers, A. M. Lawson

      Pages 11-26

   3. Derivatives of organic acids

      • R. A. Chalmers, A. M. Lawson

      Pages 27-45

   4. Separation, quantification and identification

      • R. A. Chalmers, A. M. Lawson

      Pages 46-82

   5. Gas chromatography-mass spectrometry

      • R. A. Chalmers, A. M. Lawson

      Pages 83-127

   6. Volatile carboxylic acids

      • R. A. Chalmers, A. M. Lawson

      Pages 128-135

4. Back Matter

   Pages 136-160

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5. Organic acids in physiological fluids from normal subjects

   1. Front Matter

      Pages 161-162

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   2. Organic acids in blood and urine from normal healthy adults and children

      • R. A. Chalmers, A. M. Lawson

      Pages 163-195

   3. Organic acids in physiological fluids other than urine and blood

      • R. A. Chalmers, A. M. Lawson

      Pages 196-203

6. Back Matter

   Pages 204-208

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7. Organic acids in human metabolic diseases (the organic acidurias)

   1. Front Matter

      Pages 209-209

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   2. Organic acids in the study of metabolic diseases

      • R. A. Chalmers, A. M. Lawson

      Pages 211-238

   3. Disorders of branched-chain amino acid metabolism

      • R. A. Chalmers, A. M. Lawson

      Pages 239-295

   4. Disorders of propionate and methylmalonate metabolism

      • R. A. Chalmers, A. M. Lawson

      Pages 296-331

   5. Specific causes of congenital keto acidosis in infants

      • R. A. Chalmers, A. M. Lawson

      Pages 332-337

   6. Disorders of L-lysine metabolism

      • R. A. Chalmers, A. M. Lawson

      Pages 338-349

   7. The dicarboxylic acidurias

      • R. A. Chalmers, A. M. Lawson

      Pages 350-382

The writing of this book was prompted by the need for a comprehensive of current data on organic acids suitable for both newcomers and collection established researchers in this field. The only previous text of the kind was the excellent review by Nordmann and Nordmann (1961), and at that time the main method of analysis was paper chromatography with liquid chromatography being used in a limited way. Only three diseases in which organic acids accumulate were known (primary hyperoxaluria, phenylketonuria and alcaptonuria). Since then, with the development of gas chromatography and mass spectrometry, and the further development ofliquid chromatography, knowledge concerning the nature of the organic acids in physiological fluids has been greatly extended. At the same time, the number of organic acidurias has increased dramatically, there being now some 40-50 known diseases of this type. During the past 15 years or so, there have been several reviews, dealing with either specific diseases or groups of diseases (Gompertz, 1972, 1974; Tanaka, 1975), or presenting the proceedings of symposia (Stern and Toothill, 1972) or workshops (Marner et al. , 1974). This present text deals comprehensively and in detail with the organic acids in human physiological fluids in health and in disease states, and is particularly concerned with the methods necessary for their separation, determination and indentification.

• Alanin
• amino acid
• biochemistry
• chromatography
• metabolism
• organic acid
• physiological fluid

• M.R.C. Clinical Research Centre, Harrow, UK

  R. A. Chalmers, A. M. Lawson

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