Kwashiorkor
The 5 Minute Pediatric Consult
Helen Anita John-Kelly
The 5 Minute Pediatric Consult
DEFINITION
- Extreme expression of protein-energy malnutrition
- Characterized by edema, growth failure, hypoalbuminemia, fatty infiltration of the liver, and specific dermatosis
- First described in West Africa by Cecily Williams. The word Kwashiorkor in Ghanian means red or yellow boy.
CAUSES
Diet deficient in protein and a low protein-to-energy ratio are important factors in the development of Kwashiorkor. Other factors postulated include:
- Carbohydrate overloading of a severely malnourished child
- Aflatoxin poisoning
- Imbalance between the production of toxic free radical and its disposal
- Essential fatty acid deficiency
- Deficiency of trace minerals (e.g., zinc, copper, manganese, and selenium)
PATHOPHYSIOLOGY
- Hypoalbuminemia reduces colloid osmotic pressure, leading to edema.
- Reduction in renal blood flow and glomerular filtration rate due to decreased plasma volume and decreased cardiac output as a consequence of hypoalbuminemia.
- Increase in ferritin stimulates release of antidiuretic hormone and subsequent fluid retention.
EPIDEMIOLOGY
- Most common age group are children younger than 2 years of age.
- Prevalent in Third World countries; occurs in developed countries secondary to nutritional ignorance rather than food deprivation
- Other contributory factors include misconception concerning the use of foods, unstable home environment, high prevalence of alcoholism, poor sanitary conditions, and societal beliefs that prohibit the use of many nutritious foods.
COMPLICATIONS
- Fluid and electrolyte disturbances
- Hypoosmolality with moderate hyponatremia
- Mild-to-moderate metabolic acidosis
- Hypocalcemia
- Decreased body potassium without hypokalemia
- Decreased body magnesium with or without hypomagnesemia
- Infections
- Gram-positive and gram-negative organisms; the latter is more common in severe protein-energy malnutrition
- Cardiac failure
- May occur in the midst of severe anemia, during rehydration, and shortly after the introduction of high-protein and high-energy feedings
- Severe anemia
- Hemoglobin levels usually improve with proper dietary management.
- Blood transfusion should be reserved for patients with hemoglobin less than 4 g/100 mL, hypoxia, or impending cardiac failure.
- Hypothermia and hypoglycemia
- Secondary to either impaired non-regulatory mechanisms, reduced fuel substrate, or severe infection
- Severe vitamin deficiency
- Vitamin A deficiency is common
- Protein energy malnutrition due to the impairment in protein absorption or metabolism
HISTORY
Question: Dietary history
Significance: Assess for adequacy of protein and total calories.
Question: Cultural beliefs about feeding
Significance: May contribute to low protein intake.
Question: Breastfeeding
Significance: May protect infant but expose older sibling to protein deficiency.
Question: Possible protein loss, diarrhea, renal disease
Significance: Nondietary cause of decreased serum protein.
Question: Skin rash
Significance: See Physical Examination.
Question: Growth records
Significance: Decreased growth velocity commensurate with poor protein intake.
- Children appear apathetic, irritable, and sad.
- Predominant soft, pitting, painless edema of the soft tissues usually in the feet and legs, perineum, upper extremities, and face.
Finding: Dyspigmentation of the skin
Significance: Hair develops a red-brown color, loses its luster, becomes fragile and easily pluckable. Alternating bands of depigmentation and normal hair known as the flag sign.
Finding: Characteristic dermatosis of Kwashiorkor
Significance: Also known as flaky dermatosis. Appears in areas of the body subject to friction or pressure, notably the flexures, groin, buttocks, behind the knees, or at the elbows. It is described as patchy areas of darkly pigmented skin with a clear margin and a slightly raised edge. As the lesions progress, these lesions peel or desquamate. In severe cases, the skin peels away in patches leaving pale, ulcerated lesions lacking pigmentation. The borders of these lesions may have new highly pigmented dark plaques. These severe peeling lesions may resemble second-degree burns, but unlike burns, the dermatosis of Kwashiorkor lacks a surrounding of erythema and is accompanied by edema.
Finding: Vesiculations of skin
Significance: Resulting in weeping lesions
Finding: Height
Significance: May be normal or retarded, depending on the chronicity of the illness.
Finding: Pale, cold, and cyanotic extremities
Significance:
Finding: Abdomen is frequently protuberant secondary to poor peristalsis
Significance: Leading to distended stomach and intestinal loops.
Test: Common biochemical findings
Significance:
- Significant reduction in serum concentration of total protein and albumin.
- Hemoglobin and hematocrit are usually low.
- Ratio of non-essential to essential amino acids in plasma is elevated in Kwashiorkor and usually normal in marasmus.
- Increased serum elevation of free fatty acids.
Treatment protocol is usually divided into three stages:
- Resolving life-threatening conditions
- Restoration can be achieved by oral rehydration solution. As soon as the patient improves, liquid elemental feeds can be initiated. Intravenous fluids must be used when there is persistent vomiting or abdominal distention. Electrolyte imbalances should be treated.
- Restoring nutritional status
- Nutritional status can be improved by naso-gastric feeds with 6 to 12 feedings per day. High-protein, high-energy formulas are used. Initial treatment should provide average energy and protein requirements, followed by a gradual increase to 1.5 times the energy and 3 to 4 times the protein requirements by the seventh day. No change in weight or a decrease caused by loss of edema, accompanied by large diuresis, can occur.
- Ensuring nutritional rehabilitation
- Intravenous alimentation is rarely justified in primary protein-energy malnutrition and can increase mortality rates.
- Introduction of traditional home food and therapy can continue on an outpatient basis. Emotional and physical stimulation must be provided. Emphasizing nutritious use of household foods, personal and environmental hygiene, and dietary management of diarrhea and other diseases is also an aspect of management.
PROGNOSIS
Early recognition is important in treating Kwashiorkor. Treatment corrects the acute signs of the disease, but catch-up growth in height may never be achieved. A higher mortality rate is associated with severe anthropometric deficits. Mortality rate in Kwashiorkor can be as high as 40%, but adequate treatment can reduce it to less than 10%. Some of the factors that indicate poor prognosis are:
- Age of less than 6 months
- Infections
- Dehydration and electrolyte abnormalities
- Persistent tachycardia, signs of heart failure
- Total serum protein less than 3 g/100 mL
- Elevated serum bilirubin
- Severe anemia with hypoxia
- Hypoglycemia and/or hypothermia
ICD-9-CM 260
Buno IJ, Morelli JG, Weston WL. The enamel paint sign in the dermatologic diagnosis of early-onset Kwashiorkor. Arch Dermatol 1998;134(1):107108.
Chase HP, Kirmar V, Caldwell RJ, OBrien D. Kwashiorkor in the United States. Pediatrics 1980;66:972976.
Latham MC. The dermatosis of Kwashiorkor in young children. Ithaca, NY: Cornell University, 1991.
Rossouw JE. Kwashiorkor in North America. Am J Clin Nutr 1989;49:588592.
Copyright © 2000 Lippincott Williams & Wilkins
M. William Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F. Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult