Kwashiorkor Protein

Intestinal diseases that injure the absorptive cells of the intestinal villi diminish lactase activity along the intestine, producing a condition known as secondary lactase deficiency. Kwashiorkor (protein malnutrition), colitis, gastroenteritis, tropical and... 

Organoselenium compounds in particular, once ingested, are slowly released over prolonged periods and result in foul-smelling breath and perspiration. The element is also highly toxic towards grazing sheep, cattle and other animals, and, at concentrations above about 5 ppm, causes severe disorders. Despite this, Se was found (in 1957) to play an essential dietary role in animals and also in humans — it is required in the formation of the enzyme glutathione peroxidase which is involved in fat metabolism. It has also been found that the Incidence of kwashiorkor (severe protein malnutrition) in children is associated with inadequate uptake of Se, and it may well be involved in protection... 

All 20 of the amino acids present in proteins are essential for health. While comparatively rare in the Western world, amino acid deficiency states are endemic in certain regions of West Africa where the diet relies heavily on grains that are poor sources of amino acids such as tryptophan and lysine. These disorders include kwashiorkor, which results when a child is weaned onto a starchy diet poor in protein and marasmus, in which both caloric intake and specific amino acids are deficient. 

In addition to the wasting of muscle tissue, loss of intestinal mucosa, and impaired immune responses seen in marasmus, children with kwashiorkor show a number of characteristic feamres. The defining characteristic is edema, associated with a decreased concentration of plasma proteins. In addition, there is enlargement of... 

Types of protein-energy malnutrition are marasmus (deficiency in total intake or nutrient utilization), kwashiorkor (relative protein deficiency), and mixed marasmus-kwashiorkor. 

Diseases of people come in many flavors. There are infectious diseases (measles, mumps, influenza, AIDS,...), nutritional deficiency diseases (scurvy, beriberi, kwashiorkor,...), degenerative diseases (Alzheimer s disease, osteoporosis,...), cancer (of the lung, breast, prostate, liver,...), and single-gene inherited diseases or molecular diseases. In the last category, an important and instructive example is provided by sickle cell anemia. Let s consider this disease and begin to develop a sense of how we can understand it on the basis of what we now know about proteins. 

A special form of undemutrition that particularly affects children is known as protein-energy malnutrition (PEM) which is a spectrum of syndromes from marasmus (lack of energy intake) to kwashiorkor (deficient protein intake). 

Marasmus is considered to be due to inadequate food intake. It is not usually the quantity but the quality of the food that is deficient, e.g. low nutritional value of bulky vegetables. Kwashiorkor is considered to be caused, more specifically, by a low-protein diet. This condition frequently develops at the time of weaning when protein-rich milk is replaced by protein-deficient solid food. It did not appear in the medical literature until 1934 when it was reported by Cicely Williams who studied the condition while she was working among tribes of Western Africa. She gave it the name kwashiorkor, which was used by the Ga tribe to describe the condition that develops when the baby is taken away from mother s breast, usually because another baby has been bom. It has generally been held that the oedema is a consequence of a low plasma albumin concentration and a reduction in the colloid osmotic pressure which reduces the movement of water from tissue fluid back into capillaries. The low albumin level results from a decreased rate of synthesis of albumin by the liver. However, if marasmus is due entirely to lack of energy... 

Donbts have, however, been expressed about protein deficiency being the sole cause of kwashiorkor. There is only a poor connection between its occurrence and the protein content of the diet and recovery from kwashiorkor is not simply related to protein consumption. Other factors may be involved, such as ... 

The serum protein concentrations, especially of albumin, are low in kwashiorkor but normal in marasmus. 

Fatty liver occurs in kwashiorkor, probably due to lack of protein in the diet, which reduces the synthesis of the structural protein for VLDL (apolipoprotein B). The increased triacylglycerol produced in the liver from fatty acids removed from the blood (i.e. the inter-tissue triac-... 

The ratio of essential to non-essential amino acids is high in kwashiorkor but normal in marasmus. The cause of this may be low activities of the enzymes for metabolising the essential amino acids. These are required for any protein synthesis that must take place even in kwashiorkor. 

The higher than normal serum IgA in many children with protein calorie malnutrition may be related to increased synthesis of IgA by the intestinal lamina propria in resjionse to increased antigenic stimuli from bacteria and virus. This is probably supported by the observation that children with kwashiorkor were found to maintain their polio antibodies during malnutrition, and their immune mechanism seemed to be quite capable of inhibiting poliovirus infection, indicating that the intestinal receptor cell for poliovirus operates normally in kwashiorkor (B8). It is now known that polio antiliodies are mainly associated with IgA. 

The antibody response to yellow fever vaccine was impaired in protein-deficient children with kwashiorkor compared to the well-nourished controls. Polio antibody production was normal in the malnourished children, all of whom also responded in the normal fashion to smallpox vaccination. They had no evidence of disseminated vaccinia (B8). In Guatemala, on the other hand, smallpox vaccination of children who had fully recovered from severe protein-calorie malnutrition led to a drop in their nitrogen retention with the added complication of disseminated vaccinia (V3). 

In countries where food Is In short supply or the diet is inadequate, protein-calorie malnutrition can take two extreme forms, kwashiorkor and marasmus. 

Kwashiorkor arises in children due to deprivation of protein relative to calories, eg, a starch-dominated diet. 

The nutritional status of an animal may affect the disposition of a foreign compound in vivo as well as the metabolism. Many drugs are protein-bound in the plasma, and alteration of the extent of binding for compounds extensively bound may have important toxicological implications. Thus, the decreased plasma levels of albumin after low-protein diets, such as occur in the human deficiency disease Kwashiorkor, might lead to significantly increased plasma levels of the free drug and therefore the possibility of increased toxicity. 

Marasmus Marasmus occurs when calorie deprivation is rela tively greater than the reduction in protein. Marasmus usually occurs in children younger than one year of age when the mother s breast milk is supplemented with thin watery gruels of native cereals, which are usually deficient in protein and calories. Typical symptoms include arrested growth, extreme muscle wast ing (emaciation), weakness, and anemia. Victims of marasmus do not show the edema or changes in plasma proteins observed in kwashiorkor. 

Kwashiorkor is caused by inadequate intake of protein. Marasmus Marasmus occurs when calorie deprivation is relatively greater than the reduction in protein. 

Kwashiorkor usually occurs in the second or third year in the life of a child. Edema is the principal symptom. The condition arises from a combination of circumstances, but the primary cause appears to be a weaning diet that is both inadequate and indigestible and, notably, is lacking of protein. The principal calories are supplied by carbohydrate. The condition is accelerated by repeated infections of a bacterial, parasitic, or vital nature. Without treatment, the disease is fatal in most cases. 

It is only when the renal tubular function becomes disturbed that hyperamino aciduria appears, in comparison to normal subjects, under the influence of protein overloading in the diet (S34). Dubois et al. attribute also to the same renal tubular deficiency the temporary rise in amino aciduria observed in the first days of high-protein dietary treatment of kwashiorkor cases (D25, D26), a situation which has also been seen by others (C9, M4). It has been attributed both to hepatic and renal deficiencies (C3). 

In our laboratory, nine cases of colored children from Central Africa, suffering from typical kwashiorkor, have been examined, using Moore and Stein s 1954 method. Abnormal amino aciduria was observed in most cases, both before and in the early stage of protein treatment, by comparison with normal subjects of the same region. Taurine and (3-amino-isobutyric acid were abundantly excreted (Fig. 6). Under protein treatment the drop in amino acid output is a most striking feature of the... 

The signs and symptoms of protein-energy malnutrition (PEM) depend on various factors, including the duration of the nutritional inadequacy, age at onset, and frequency/types of concomitant infections. Figure 24-1 shows children diagnosed with kwashiorkor and marasmus and outlines some of the diagnostic features discussed in this section (Scrimshaw and Behar, 1961). 

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