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Kwashiorkor

Description

  • Kwashiorkor is characterized by hypoproteinemia, pitting edema, varying degrees of wasting and/or stunting, dermatosis, and fatty infiltration of the liver.
  • Protein-energy malnutrition (PEM) results when the body’s protein and energy requirements are not adequately met.
    • Primary:
      • Inadequate dietary intake
    • Secondary:
      • Result of other disease processes that limit food ingestion or reduce nutrient absorption, or that increase nutrient requirements or losses
    • Marasmic Kwashiorkor:
      • Combined edema and emaciation associated with acute or chronic protein deficiency and chronic energy deficit.
  • Kwashiorkor was classically described in the mid-1930s by Cicely Williams in her observations of the GA tribe in the Gold Coase (currently Ghana). “Kwashiorkor” is the GA term for “the disease the deposed baby gets when the next one is born.”

Epidemiology

  • Malnutrition underlies 55% of childhood mortality worldwide.
  • Kwashiorkor may occur at any age, but is seen most frequently in children 1–3 years of age.

Etiology

  • Children have relatively high energy and protein requirements per kilogram of body weight.
  • Inappropriate use of infant formula or the introduction of bulky carbohydrate-based staple foods which are low in energy density, protein and fat content may lead to PEM.
  • Food scarcity from drought or other natural disasters, war or civil disturbance may lead to PEM.
  • Aflatoxin poisoning from the fungus Aspergillus flavus has been implicated in the etiology of Kwashiorkor. Aflatoxin concentration has been found to be elevated in the blood and liver of children with Kwashiorkor. Aflatoxins may appear in breast milk.
  • Further investigations have shown an association between selenium deficiency and congestive heart failure in Kwashiorkor.

Other

Pathophysiology of PEM by Systems

  • Temperature regulation is impaired, leading to hypothermia in a cold environment and hyperthermia in a hot environment.
  • Fluid and electrolytes:
    • Increase in total-body sodium and decrease in total-body potassium
    • Increased cell membrane permeability in Kwashiorkor leads to increased intracellular sodium and decreased intracellular potassium.
    • Increased intracellular sodium is accompanied by increased cellular water.
  • Hypophosphatemia is associated with malnutrition and results in high mortality.
  • Liver:
    • Protein synthesis is reduced; particularly albumin, transferrin and apolipoprotein B.
    • Hypoalbuminemia reduces colloid osmotic pressure, leading to edema.
    • Hypertriglyceridemia leads to fatty infiltration of the liver.
  • Gluconeogenesis is reduced which increases risk of hypoglycemia during infection.
  • Cardiovascular system:
    • Pericardial effusion may be present in Kwashiorkor.
    • Reduced cardiac output leads to compromised tissue perfusion and a reduction in renal blood flow and glomerular filtration rate.
    • Increase in ferritin stimulates release of antidiuretic hormone and subsequent fluid retention.
  • Respiratory system:
    • Reduced muscle mass affects respiratory muscles, such as the diaphragm, and reduces pulmonary function.
    • Respiratory muscle weakness may be exacerbated by hypophosphatemia and hypokalemia.
  • GI system:
    • Reduction of gastric acid, intestinal motility and pancreatic digestive enzymes
    • Intestinal mucosa is atrophied resulting in malabsorption
  • Endocrine system:
    • Insulin secretion is reduced.
    • Growth hormone secretion is increased while somatomedin activity is reduced.
    • Glucagon, epinephrine and cortisol levels are increased.
    • Increased epinephrine, growth hormone and corticosteroids leads to lipolysis, an increase in free fatty acid concentration and increased peripheral insulin resistance.
  • Immune system:
    • All aspects of immune function are deminished in malnutrition.

Signs and Symptoms

History

  • Dietary history:
    • Assess typical diet before current illness episode.
    • Assess for adequacy of protein and total calories.
    • Assess food and fluids taken in past few days.
  • Duration and frequency of emesis or diarrhea
  • Loose stools with evidence of malabsorption are common. Stools may be watery and/or tinged with blood.
  • Time when last urine was passed
  • Birth weight
  • Breastfeeding history
  • Developmental milestones
  • Immunization status
  • Exposure to infectious disease
  • Cultural beliefs and practices regarding infant and childhood feeding
  • Growth records: Decreased growth velocity commensurate with poor protein intake

Physical Exam
  • Weight and length/height:
    • Growth failure always occurs to some extent
    • Wasting is also typical, although it may be masked by the presence of edema.
  • Mental status changes:
    • Affected child is usually apathetic and irritable.
    • Child is usually unsmiling and prefers to remain in one position.
  • Edema:
    • There is some degree of edema in all cases of Kwashiorkor.
    • Peripheral edema usually begins in the feet and ascends up the legs.
    • Pitting of the skin above the ankle is diagnostic.
    • The hands and face may become edematous.
    • Facial edema gives the characteristic “moonfaces.”
  • Hair changes:
    • Hair lacks luster and color may change to brown or reddish-brown.
    • Hair is easily pluckable.
    • Bands of discolored hair, representing periods of malnutrition, are termed the “flag sign.”
  • Skin changes:
    • Dermatosis often develops in areas of friction or pressure.
    • Hypo- or hyperpigmented patches may appear which subsequently desquamate in scales or sheets, exposing atrophic ulcers resembling burns.
  • Additional clinical signs of PEM:
    • Signs of B-vitamin deficiency, such as perioral lesions
    • Signs of vitamin A deficiency, such as xerosis and/or xerophthalmia
    • Pale, cold, and cyanotic extremities: Decreased vascular volume secondary to decreased protein concentration
    • Abdomen is frequently protuberant secondary to poor peristalsis, leading to distended stomach and intestinal loops

Tests

Lab

  • Hypoproteinemia, particularly hypoalbuminemia, is commonly seen in Kwashiorkor.
  • Prealbumin and serum transferrin may be useful in determining severity of Kwashiorkor.
  • Retinal binding protein may be reduced in Kwashiorkor.
  • Hemoglobin and hematocrit are usually low.
  • Ratio of nonessential to essential amino acids in plasma is elevated in Kwashiorkor and usually normal in marasmus.
  • Increased serum elevation of free fatty acids
  • Low serum and urine carnitine levels

Differential Diagnosis

  • Nephrosis: Edema is common and albumin is present in urine. There is usually only trace urine albumin in Kwashiorkor. Ascites is common in nephrosis, but rare in Kwashiorkor.
  • Hookworm anemia: May cause edema alone. Hookworm infection is commonly seen in association with Kwashiorkor. Hookworm anemia is not associated with the dermatological findings commonly seen in Kwashiorkor.
  • Chronic dysentery—many children with Kwashiorkor are mistakenly thought to be dehydrated due to hypovolemia and history of frequent mucoid stools reported as diarrhea. Rehydration of these edematous patients with intravenous fluids or nonmodified ORS may lead to heart failure. Edema is not commonly seen in dysentery
  • Pellagra: Dermatosis of pellagra and Kwashiorkor are similar. However, the dermatosis of pellagra is often seen in sun-exposed areas, not in areas such as the groin, as commonly seen in Kwashiorkor.

Management of the child with severe protein-energy malnutrition is divided into 3 phases: Initial Treatment, Rehabilitation and Follow-Up.

Other

Initial Treatment

  • Resolving life-threatening conditions
  • Fluid and electrolyte disturbances, infection, hemodynamic alterations, severe anemia, hypothermia, hypoglycemia and Vitamin A deficiency are of paramount importance.
  • Initial treatment begins with hospitalization and lasts until child is stable and appetite has returned.
  • Whenever possible, a dehydrated child with malnutrition should be rehydrated orally or by nasogasric tube.
  • IV infusion should be avoided except for when it is essential; e.g. severe dehydration and shock.
  • Hypoglycemia is an important cause of death in the 1st 2 days of treatment.
  • Suspected hypoglycemia should be treated with ORS or 10% glucose by mouth or nasogastric tube.
  • Severely malnourished children have high levels of sodium and are deficient in potassium. Standard WHO Oral Rehydration Salts Solution (ORS) does not meet the special electrolyte requirements of the severely malnourished child.
  • ReSoMal is a modified ORS which contains less sodium and more potassium than the standard WHO ORS and is the recommended ORS for severely malnourished children.
  • Breastfeeding should not be interrupted during rehydration.

Rehabilitation

  • Ensure nutritional rehabilitation.
  • Follow-up begins when child is eating satisfactorily and is without complications
  • Introduction of traditional home food and therapy can continue on an outpatient basis.
  • Nutritious use of household foods, personal and environmental hygiene, and dietary management of diarrhea and other diseases is also an aspect of management.
  • Emotional and physical stimulation must be provided.
  • Child and family are followed to prevent relapse.
  • Physical, cognitive, and emotional development of the child should be monitored.

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.
  • Mortality rate in Kwashiorkor can be as high as 40%, but adequate treatment can reduce it to <10%.
  • Some of the factors that indicate poor prognosis:
    • Age <6 months
    • Infections
    • Dehydration and electrolyte abnormalities
    • Persistent tachycardia, signs of heart failure
    • Total serum protein <3 g/100 mL
    • Elevated serum bilirubin
    • Severe anemia with hypoxia
    • Hypoglycemia and/or hypothermia

Complications

  • Fluid and electrolyte disturbances:
    • 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 association with 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 <4 g/100 mL, hypoxia, or impending cardiac failure.
  • Hypothermia and hypoglycemia: Secondary to impaired regulatory mechanisms, reduced fuel substrate, or severe infection
  • Severe vitamin deficiency: Vitamin A deficiency is common and may lead to infection.

ICD-9

260 Kwashiorkor

AUTHOR

Robert D. Karch, MD, MPH

BIBLIOGRAPHY

  1. Carvalho NF , Kenney RD , Carrington PH , et al. Severe nutritional deficiencies in toddlers resulting from health food milk alternatives. Pediatrics. 2001;107:E46.
  2. Golden MH. Oedematous malnutrition. Br Med Bull. 1998;54(2):433–444.
  3. Latham MC . The Dermatosis of Kwashiorkor in Young Children. Ithaca, NY: Cornell University; 1991.
  4. Oumeish OY , Oumeish I. Nutritional skin problems in children. Clin Dermatol. 2003;21:260–263.
  5. World Health Organization. Management of Severe Malnutrition: A Manual for Physicians and other Senior Health Workers. Geneva: WHO; 1999.

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