Diabetic ketoacidosis, lipid

Conditions that result in increased production of metabolic acids include lactic acidosis and diabetic ketoacidosis. Lactic acidosis occurs with anaerobic metaboUsm in the presence of severe hypoxemia (i.e., Pao < 36 mm Hg). Any condition that prevents adequate oxygenation resulting in hypoxemia, such as respiratory failure or lung cancer, as well as any condition causing a decrease in perfusion to body tissues, such as heart failure or shock of any form, will result in anaerobic metabolism and lactic acid buildup. In diabetic ketoacidosis, the lack of insulin to move glucose into the cells results in a form of starvation and the production and accumulation of ketoacids (i.e., ketosis) owing to the use of lipids for fuel. 

In spite of differences in pathogenesis of the various forms of EHL it is possible to consider their clinical manifestations together since the majority of signs and symptoms seem to vary only in a quantitative manner between syndromes. This, as well as the frequency of asymptomatic cases of EHL, indicates that at least the manifestations of lipemia retinalis, xanthomatosis and probably hepatospleno-megaly and abdominal crises are the results of the lipid elevation rather than primary expressions of the underlying pathogenetic mechanisms. Other evidence for the non-specificity of eruptive xanthomas and lipemia retinalis is their occurrence in secondary hyperlipemias (e.g. diabetic ketoacidosis). 

Once the presence of fasting lipemia has been established, di-ffereyitiation between primary and secondary hyperlipemias can usually be made on the basis of presence or absence of clinical and laboratory findings characteristic of an underlying disease. Plasma lipid and lipoprotein patterns as determined by lipid analyses, ultracentrifugation and electrophoresis are not necessarily diagnostic since similar findings are present in EHL and hyperlipemia of diabetic ketoacidosis, acute pancreatitis, alcoholism, etc. (Schettler 1955, Lees and Fredrickson 1964, Jahnke 1965). 

In accordance with our interpretation of the term lipidoses as hereditary disorders of lipid metabolism, a review of secondary hyperlipidemias has not been attempted here. Since they are considered as associated phenomena of disorders such as diabetic ketoacidosis, nephrosis or pancreatitis, their exclusion seems to be justifiable. 

Concentrations of various carboxylic acids in human body fluids reflect some of the major metabolic processes of the body. These metabolites apparently originate from lipid and amino acid metabolism the major metabolic defects are frequently associated with unbalanced concentrations of these acidic substances. One of the most widely occurring conditions of this kind is ketoacidosis in diabetic disease high concentrations of the so-called ketone bodies (3-hydroxybutyric acids, acetoacetic acid and others) are the traditional hallmarks of ketoacidosis. Many additional acidurias were discovered (particularly during the last 15 years) in major part due to the availability of GC and GC/MS techniques. Acidurias are among the serious medical conditions that are usually a result of genetic aberration (enzyme deficiencies), but environmental factors or nutritional deficiency could occasionally be involved. These conditions are characterized by either (a) drastically enhanced excretion of normal metabolic intermediates, or (b) excretion of unusual metabolites that are produced from the accumulated intermediates via alternate biochemical pathways. Many acidemic conditions have now been documented in the literature, and the role of GC in such medical discoveries has been adequately stressed in the recent reviews of Jellum [15] and Tanaka and Hine [373]. 

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