Kwashiorkor

Kwashiorkor
One of many children with kwashiorkor in relief camps during the Biafra War
Pronunciation /kwɑːʃiˈɔːrkər/
Classification and external resources
Specialty Pediatrics
ICD-10 E40, E42
ICD-9-CM 260
DiseasesDB 7211
MedlinePlus 001604
MeSH D007732

Kwashiorkor is a form of severe protein–energy malnutrition characterized by edema, irritability, ulcerating dermatoses, and an enlarged liver with fatty infiltrates. Sufficient calorie intake, but with insufficient protein consumption, distinguishes it from marasmus. Kwashiorkor cases occur in areas of famine or poor food supply.[1] Cases in the developed world are rare.[2]

Jamaican pediatrician Cicely Williams introduced the name into the medical community in a 1935 Lancet article, two years after she published the disease's first formal description in the Western medical literature.[3][4] The name is derived from the Ga language of coastal Ghana, translated as "the sickness the baby gets when the new baby comes" or "the disease of the deposed child",[5] and reflecting the development of the condition in an older child who has been weaned from the breast when a younger sibling comes.[6] Breast milk contains proteins and amino acids vital to a child's growth. In at-risk populations, kwashiorkor may develop after a mother weans her child from breast milk, replacing it with a diet high in carbohydrates, especially sugar.

Signs and symptoms

The defining sign of kwashiorkor in a malnourished child is pitting edema (swelling of the ankles and feet). Other signs include a distended abdomen, an enlarged liver with fatty infiltrates, thinning hair, loss of teeth, skin depigmentation and dermatitis. Children with kwashiorkor often develop irritability and anorexia. Generally, the disease can be treated by adding protein to the diet; however, it can have a long-term impact on a child's physical and mental development, and in severe cases may lead to death.

In dry climates, marasmus is the more frequent disease associated with malnutrition. Another malnutrition syndrome includes cachexia, although it is often caused by underlying illnesses. These are important considerations in the treatment of the patients.

Causes

Child in the United States with symptoms of kwashiorkor
Typical ulcerating dermatosis seen on a Malawian child with kwashiorkor
Disability-adjusted life year for protein–energy malnutrition per 100,000 inhabitants in 2002.[7]
  no data
  fewer than 10
  10–100
  100–200
  200–300
  300–400
  400–500
  500–600
  600–700
  700–800
  800–1000
  1000–1350
  more than 1350

Kwashiorkor is a severe form of malnutrition, caused by a deficiency in dietary protein. The extreme lack of protein causes an osmotic imbalance in the gastro-intestinal system causing swelling of the gut diagnosed as an edema or retention of water.[4]

Extreme fluid retention observed in individuals suffering from kwashiorkor is a direct result of irregularities in the lymphatic system and an indication of capillary exchange. The lymphatic system serves three major purposes: fluid recovery, immunity, and lipid absorption. Victims of kwashiorkor commonly exhibit reduced ability to recover fluids, immune system failure, and low lipid absorption, all of which result from a state of severe undernourishment. Fluid recovery in the lymphatic system is accomplished by re-absorption of water and proteins which are then returned to the blood. Compromised fluid recovery results in the characteristic belly distension observed in highly malnourished children.[8]

Capillary exchange between the lymphatic system and the bloodstream is stunted due to the inability of the body to effectively overcome the hydrostatic pressure gradient. Proteins, mainly albumin, are responsible for creating the colloid osmotic pressure (COP) observed in the blood and tissue fluids. The difference in the COP of the blood and tissue is called the oncotic pressure. The oncotic pressure is in direct opposition with the hydrostatic pressure and tends to draw water back into the capillary by osmosis. However, due to the lack of proteins, no substantial pressure gradient can be established to draw fluids from the tissue back into the blood stream. This results in the pooling of fluids, causing the swelling and distention of the abdomen.[9]

The low protein intake leads to some specific signs: edema of the hands and feet, irritability, anorexia, a desquamative rash, hair discolouration, and a large fatty liver. The typical swollen abdomen is due to two causes: ascites because of hypoalbuminemia (low oncotic pressure), and enlarged fatty liver.[10]

Ignorance of nutrition can be a cause. Latham, director of the Program in International Nutrition at Cornell University, along with Keith Rosenberg cited a case where parents who fed their child cassava failed to recognize malnutrition because of the edema caused by the syndrome and insisted the child was well-nourished despite the lack of dietary protein.

Protein should be supplied only for anabolic purposes. The catabolic needs should be satisfied with carbohydrate and fat. Protein catabolism involves the urea cycle, which is located in the liver and can easily overwhelm the capacity of an already damaged organ. The resulting liver failure can be fatal. This means in patients suffering from kwashiorkor, protein must be introduced back into the diet gradually. Clinical solutions include weaning the affected with milk products and increasing the intake of proteinaceous material to daily recommended amounts.

See also

Notes

  1. ↑ Krebs NF, Primak LE, Hambridge KM. Normal childhood nutrition & its disorders. In: Current Pediatric Diagnosis & Treatment. McGraw-Hill.
  2. ↑ Liu, Theodore; Howard, Renée M.; Mancini, Anthony J.; Weston, William L.; Paller, Amy S.; Drolet, Beth A.; Esterly, Nancy B.; Levy, Moise L.; et al. (2001). "Kwashiorkor in the United States: Fad Diets, Perceived and True Milk Allergy, and Nutritional Ignorance". Archives of Dermatology. 137 (5): 630–6. PMID 11346341.
  3. ↑ Williams, Cicely (1983) [1933]. "A nutritional disease of childhood associated with a maize diet". Archives of Disease in Childhood. 58: 550–560. doi:10.1136/adc.58.7.550. PMC 1628206Freely accessible. PMID 6347092.
  4. 1 2 Williams, Cicely (1935). "Kwashiorkor: a nutritional disease of children associated with a maize diet". The Lancet. 226 (5855): 1151–1152. doi:10.1016/S0140-6736(00)94666-X. Reprint: Williams CD, Oxon BM, Lond H (2003). "Kwashiorkor: a nutritional disease of children associated with a maize diet. 1935". Bull. World Health Organ. 81: 912–3. PMC 2572388Freely accessible. PMID 14997245.
  5. ↑ Stanton J (2001). "Listening to the Ga: Cicely Williams' Discovery of Kwashiorkor on the Gold Coast". Clio Medica: Studies in the History of Medicine and Health. 71: 149–171. PMID 11603151.
  6. ↑ "Merriam Webster Dictionary". Retrieved 2009-04-05.
  7. ↑ "Mortality and Burden of Disease Estimates for WHO Member States in 2002" (xls). World Health Organization. 2002.
  8. ↑ "Nova et Vetera". The British Medical Journal. 2 (4673): 284. 1950. doi:10.1136/bmj.2.4673.267.
  9. ↑ Saladin, Kenneth (2012). Anatomy and Physiology (6th ed.). New York: McGraw Hill. pp. 766–767, 809–811. ISBN 978-0-07-337825-1.
  10. ↑ Tierney, Emily P.; Sage, Robert J.; Shwayder, Tor (1 May 2010). "Kwashiorkor from a severe dietary restriction in an 8-month infant in suburban Detroit, Michigan: case report and review of the literature". International Journal of Dermatology. 49 (5): 500–506. doi:10.1111/j.1365-4632.2010.04253.x. PMID 20534082.

References

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