Viewing All Flashcards for Diseases - Chapters 37-39
Kwashiorkor, a common problem of children in Third World countries, is caused by a deficiency of protein in a diet that is adequate in calories.
Muscle wasting and decreased concentration of plasma proteins, particularly albumin. The result is an increase in interstitial fluid that causes edema and a distended abdomen, which makes the children appear "plump".
A lack essential amino acids in the diet; existing proteins must be broken down to produce these amino acids for new protein synthesis. These problems may be compounded by a decreased ability to produce digestive enzymes and new intestinal epithelial cells because of a decreased availability of amino acids for the synthesis of new proteins.
In neutrophils, which are white blood cells that engulf and destroy invading bacteria. Neutrophils frequently act in the lung, and elastase is sometimes released into the lung as the neutrophils work. In normal individuals, the released elastase is blocked from destroying lung cells by the action of circulating a-1 antitrypsin, a protease inhibitor.
Certain individuals have a genetic mutation that leads to the production of an inactive a-1-antitrypsin protein. The lack of this enzyme activity leads to the development of emphysema caused by proteolytic destruction of lung cells, which results in a reduction in the expansion/contraction capability of the lungs.
Using a dried blood spot. The blood is solubilized using a buffer, and then various amounts of the blood sample are incubated with antibodies specific for a-1-antitrypsin. The antigen-antibody complexes formed will disperse light, and the extent of light scattering is proportional to the concentration of a-1-antitrypsin in the solution. This procedure is known as rate nephelometry. Obtaining blood samples from a finger prick and allowing the blood to dry on a card enables rapid screening of individuals for this disorder.
In secretory granules. The pancreas also synthesizes a secretory trypsin inhibitor. The need for the inhibitor is to block any trypsin activity that may occur from accidental trypsinogen activation. If the inhibitor were not present, trypsinogen activation would lead to the activation of all of the zymogens in the pancreas, which would lead to the digestion of intracellular pancreatic proteins. Such episodes can lead to pancreatitis.
Relatively rare autosomal recessive disorder. It is caused by a defect in the transport of neutral amino acids across both intestinal and renal epithelial cells. The signs and symptoms are caused, in part, by a deficiency of essential amino acids.
They involve defects in two different transport proteins. In each case, the defect is present both in intestinal cells, causing malabsorption of the amino acids from the digestive products in teh intestinal lumen, and in kidney tubular cells, causing a decreased resorption of these amino acids from the glomerular filtrate and increased concentration of the amino acids in the urine.