Medicine for Africa - Medical Information Service
WASHIORKOR
Definition:
Kwashiorkor describes the severe form of protein malnutrition, due to inadequate protein intake, thus, it is a so-called macronutrient deficiency. It is largely a nutrition problem in developing countries, especially in Africa, and even more prevalent during times of drought, famine, or social unrest, disrupting the already modest food supplies.
The term Kwashiorkor is derived from the Ga language of coastal Ghana and means ‘the sickness of the weaning’ or ‘the disease of the displaced (from the mother’s breast) child’. Dr. Cicely D. Williams, a Jamaican pediatrician, first used the name kwashiorkor in 1933, when she described the insufficient intake of protein containing foods, in spite of acceptable overall caloric (energy) intake, especially in young children.
Other names for the disease are ‘obwosi’ in Uganda, and ‘diboba’ in the Democratic Republic of Congo (DRC).
Children, especially under the age of five, are most at risk to develop kwashiorkor, and make up the largest contingency of patients. This is due to the fact that after the mother has discontinued breast feeding her child, the dietary needs of the growing infant is usually grossly inadequate, caused by the widespread lack of maternal understanding regarding a balanced diet of sufficient total calories and protein derived calories. However, in many countries, poverty and inadequate availability of appropriate food products have to be blamed equally. Overall, close to 50% of an estimated 10 million deaths each year in developing countries, are the result of malnutrition in infants and children under the age of five. In kwashiorkor, the mortality rate (death rate) tends to decrease at an older age.
In recent research, the causes of kwashiorkor have been expanded, by identifying other essential nutrient deficiencies, beyond protein, such as iron, folic acid, riboflavin, vitamins A and C, selenium and iodine. Other nutrients that provide anti-oxidant protection are also important, as it has been shown that children suffering kwashiorkor have reduced anti-oxidants such as albumin, glutathione, polyunsaturated fats and vitamin E in their system.
Another factor that is attributed to the development of kwashiorkor is aflatoxin poisoning. Aflatoxins are produced by certain molds, and are ingested in connection with moldy foods. They can damage the liver DNA and subsequently interfere with the production of serum albumin. Considering that kwashiorkor mostly occurs in countries with warm and humid climates which encourage the growth of mold, it is important to consider this fact prior to starting treatment.
Furthermore, kwashiorkor occurs most often in countries where the staple diet consists mostly of cassava, rice, yams or plantains. The intake of protein is usually minimal in these countries, falling well below the basic needs for a growing child.
Symptoms:
Early symptoms of kwashiorkor resemble the general symptoms of any type of malnutrition and include fatigue, lethargy, irritability, diarrhea and failure to gain weight.
With progressive protein deficiency, the more typical symptoms of kwashiorkor appear, such as failure to grow in height, generalized edema (usually extracellular), a large protuberant (swollen) abdomen (the ‘pot belly’ is a rather characteristic sign for kwashiorkor, due to the largely increased fatty liver), and often pronounced loss of muscle mass. Typical skin changes include the peeling of dark and dry skin, and vitiligo (discoloration of the skin – white spots). Hair becomes thin, dry, straight and brittle, and also loses its color, causing it to turn reddish to white. Hair loss usually starts at the back of the head and can become extreme. The eyelashes can show similar changes, expressing the so-called broomstick appearance.
Physiologic changes may include a fatty liver, more or less severe anemia, decreased immunity, and renal failure; while shock and coma often precede death.
Since the immune system can be severely impaired, children with kwashiorkor are more likely to have malaria, and are more susceptible to recurrent infections of the gastrointestinal tract (bacterial, viral or parasitic), to viral pneumonia, disseminated herpes simplex infections, and to tuberculosis. Caveat – kwashiorkor patients may have negative tuberculin skin tests (PPD or Mantoux).
Diagnosis:
The first step, a physical examination of the patient will usually show an enlarged liver (hepatomegaly) and more or less widespread edema (swelling).
The second step, blood tests will reveal severe metabolic changes, and a marked deficiency of amino acids. The determination of serum electrolytes and a complete blood count (CBC) will show a low total plasma protein level (hypoproteinemia), with albumin between 1 and 2 gm per 100 ml (hypoalbuminemia; normal levels are 3.5 to 5.5 gm/100 ml), alpha and beta globulins will also be low, while gamma globulin levels are often increased due to recurrent infections. Potassium (K) can be severely depleted, while sodium (Na) and chloride (Cl) levels will be elevated. The measurement of the 24 hour excretion of creatinine in the urine usually provides a good way of assessing the extent of the actual protein deficiency.
Blood glucose levels will be low (hypoglycemia), and the protein deficiency will cause an iron deficiency anemia and metabolic acidosis. While the anemia will respond to a high protein diet if caused by kwashiorkor, many children may also be anemic due to other reasons such as parasites or chronic sepsis.
Plasma cortisol and growth hormone levels are high, trying to make up for the failure of linear growth, while insulin secretion is decreased.
The physical examination should of course include a detailed dietary history, growth measurements, and the evaluation of the body mass index (BMI).
A skin biopsy can be taken, if available, which will show hypertrophy (overgrowth, callus formation) of the outer skin layer (stratum corneum), with concomitant atrophy of the intermediate and lower skin layers (stratum granulosum, prickle cell layer).
Microscopic examination of hair reveals severe atrophy and shaft constriction, with loss of pigment due to the lack of melanin production.
Radiological abnormalities include some delay in bone ossification, similar to general osteoporosis, and white lines in the metaphyses of the long bones, especially in the distal radius and tibia.
Barium studies showed that the entire alimentary tract, from esophagus to rectum, can be affected. Marked disturbances of propulsive motor function of the gastrointestinal tract, abnormal, delayed transit time, and changes in the intestinal mucous membrane are frequently found.
Sonography can be a reliable way of demonstrating the extent of present muscle mass wasting, and can also evaluate the results of treatment.
Differential diagnosis of kwashiorkor has to exclude possible cases of strongyloidiasis, a parasitic disease caused by a nematode (roundworm), in which x-ray findings of the intestinal tract can be quite similar. In addition, other parasitic diseases such as giardiasis (caused by the protozoan Giardia lamblia), hookworm disease, and schistosomiasis japonicum, all of which can cause mucosal edema and irritabe bowel syndromes, should also be excluded. Caveat – parasitic infections are an almost normal and to be expected concomitant illness of kwashiorkor, thus, the finding of parasites in the stool does not exclude kwashiorkor. On the other hand, treatment of kwashiorkor has to consider a therapy that may go well beyond the dietary needs to treat the protein deficiency.
Therapy:
The first step of treating kwashiorkor consists of rehydrating the patient with isotonic solutions. Subsequently, slowly increasing amounts of food intake should be implemented – starting with carbohydrate rich meals, simple sugar substitutes and fat, followed by protein supplementation. Of course, vitamin and mineral supplements are essential adjuncts.
In children, the caloric intake should consist of 175 kcal/kg of body weight, during the second week of feeding, including 4 gm/kg body weight of protein.
The earlier treatment has been initiated, the more likely the regression of most symptoms can be achieved; although, normal height and weight potential will probably never be reached again.
Overall, early treatment can produce good results. Treatment started late in the disease process, will likely leave permanent physical and intellectual disabilities. No treatment, or treatment that is started too late, will be fatal.
Other disease processes such as parasitic infections, or diseases caused by the immunosuppressed status of the patient have to be treated concomitantly.
Topical and even oral, zinc paste may be very effective for healing areas of skin breakdown.
Successful treatment will restore the loss of muscle mass, as well as the changes in skin, hair, and mucous membranes. Even a liver with severe fatty changes can recover completely, as can the intestinal mucosa, which, however, may take many months.
Prevention:
An adequate, balanced diet, consisting of carbohydrates, proteins (12% of total calories) and fats (10% of total calories), can easily prevent kwashiorkor.
It has often been shown that kwashiorkor is the result of ignorance of mothers to dietary needs and provisions of food products, aside from the fact that the availability of any food is often an insurmountable problem for many families, especially in Africa and other developing nations.
Thus, especially in developing countries, it is adamant for the national health care institutions to include dietary education into any Family Planning courses or classes.
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