Kwashiorkor E40.x0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Flour damage; malignant; Malnutrition syndrome; Malnutrition syndrome malignant; Protein deficiency syndrome; protein-energy malnutrition

History
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Williams, 1933

Definition
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Severe chronic malnutrition (protein deficiency syndrome) in infants and small children (so-called infantile pellagra), especially in warm countries. Kwashiorkor is taken from the indigenous language of the African Gold Coast (Kwashi = boy; orkor = red).

Occurrence/Epidemiology
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Mainly infants in Africa, South America, West India.

Etiopathogenesis
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Deficit in energy metabolism due to reduced protein intake with simultaneous action of other noxious agents, infections and nutritive noxious agents, e.g. aflatoxin, are discussed. The pathogenesis is unclear in detail. Edema is not necessarily associated with hypoproteinemia.

Clinical features
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  • Skin changes: Parakeratotic keratinization tendency. Inflammatory and squamous-crusty changes, especially in the diaper area, trochanteric, knees, elbows. Pressure points on the trunk, leaving out the areas of skin exposed to light. Bluish-red or reddish-brownish erythema, later clear scaling, rhagade formation in the large joint bends. Characteristic depigmentation with hypopigmentation periorbital and on the legs and early oedematous swelling. Possible hyperpigmentation after healing of the inflammatory changes. Dry, fine, easily broken, lustreless hair with a reddish-brownish colour. Ribbon-like change of normal and abnormal hair colour results in the characteristic flag-sign. In advanced cases development of dark, almost purple spots in the groin area.
  • Extracutaneous manifestations: anorexia, edema especially on hands and feet, hypalbumin dysproteinemia (inconstant). Gastrointestinal disorders: diarrhoea, vomiting. Irritability, irritability, apathy. Signs of polyavitaminosis. Hepatomegaly, retardation of growth and mental development and muscular atrophy.

Differential diagnosis
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Therapy
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Protein intake through a high-protein diet.

Progression/forecast
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In mild cases with appropriate diet and protein intake regression. 10-30% mortality in severe and recurrent cases.

Literature
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  1. Alleyne GAO, Hay RW, Picou DI et al (1977) Protein-energy malnutrition. Arnold, London
  2. Collins S et al (2002) Outpatient care for severely malnourished children in emergency relief programmes: a retrospective cohort study. Lancet 360: 1824-1830
  3. Golden M (1985) The consequences of protein deficiency in man and its relationship to the features of Kwashiorkor. In: Blaxter K, Waterlow JC (eds) Nutritional adaption in man. Libbey, London, S. 169-187
  4. Niggemann B et al (2003) Side-effects of complementary and alternative medicine. Allergy 58: 707-716
  5. Williams CD (1933) A nutritional disease of childhood associated with a maize diet. Arch Dis Child 8: 423-433

Outgoing links (2)

Pellagra; Scale ;

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Last updated on: 29.10.2020