Clinical chemistry
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Pyroglutamic acidemia, a rare metabolic disorder, usually appears in infancy. It is characterized by retardation, ataxia, hemolytic anemia, and chronic acidosis and is caused by a marked deficiency of glutathione synthetase (EC 6.3.2.3) activity. ⋯ The etiology of the acidosis could not be attributed to ketoacidosis, lactic acidosis, or ingestion of methanol, salicylate, or ethylene glycol. Analysis of the patient's plasma and urine for organic acids revealed the presence of high concentrations of pyroglutamate (5-oxoproline), which remained high throughout her hospitalization.
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We describe a procedure for preparing fecal samples for determination of volatile fatty acids (VFAs) by gas-liquid chromatography (GLC) and "high-performance" liquid chromatography (HPLC). The simple, one-step procedure involves only ultrafiltration through a membrane with a molecular-mass cutoff of 3000 Da. As revealed by the GLC chromatograms, ultrafiltration appears to be as effective as steam distillation in sample clean-up. ⋯ The VFA content of the filtrate can also be measured by HPLC. Use of the ion-exclusion mechanism completely resolves isobutyric acid and butyric acid on a cation-exchange column. The mean (+/- SD) percentage distribution values of VFAs (measured by GLC) from five healthy subjects were 56.0 +/- 3.5 (acetic acid), 17.0 +/- 5.3 (propionic acid), 2.9 +/- 1.5 (isobutyric acid), 18.8 +/- 5.8 (butyric acid), 2.3 +/- 1.2 (isovaleric acid), and 2.9 +/- 0.8 (valeric acid).
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Patients who exhibited both hyponatremia and hypernatremia during a single admission were identified by a review of their biochemistry data. The mortality of patients who developed either hypernatremia or hyponatremia, followed by the opposite abnormality within less than or equal to 10 days, was 42%. ⋯ The interval (days) between the maximum and minimum sodium concentrations in the survivors and those who died was not statistically different. We found no specific clinical features that could be used to predict the development of this biochemical abnormality.
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Comparative Study
Performance of an automated six-wavelength photometer (Radiometer OSM3) for routine measurement of hemoglobin derivatives.
A new instrument for spectrophotometric determination of oxygen saturation (SO2) and the fractions of carboxyhemoglobin (FHbCO) and methemoglobin (FHi), the Hemoximeter OSM3 (Radiometer), was tested by comparative measurements with a multiwavelength method. We found that it gave reliable results for freshly drawn samples from patients. ⋯ Because SHb is rarely encountered, a reliable warning for its presence is adequate for clinical practice. Using the instrument in its fetal mode, we obtained accurate results for blood samples from newborns.