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At a glance

Acquired disorder caused by malnutrition secondary to insufficient protein intake resulting in multisystemic chronic failure with generalized edemas. Irritability, ulcerating dermatoses, and enlarged liver with fatty infiltrates complete the clinical presentation. It is caused by insufficient protein intake even though there is sufficient calorie intake. This is distinguishing it from another medical condition called Marasmus (see below).

History

This medical condition was first presented by Dr Cicely Williams, a Jamaican pediatrician, which introduced the name into the medical community in the Lancet journal in 1935. It is in 1937 that she subsequently published a more complete description of the disease. The name is derived from the Ga language of the coastal Ghana which means “the sickness the baby gets when the new baby comes” or “the disease of the deposed child.”

Genetic inheritance

No genetic component. Acquired malnutrition disease.

Pathophysiology

A condition in which almost all systems may be involved. A multifactorial process related primarily to dietary and environmental factors. The presence of edema is an important criterion in making the diagnosis.

Diagnosis

Decreased protein intake, and thus production, results in a decrease in albumin. Increase in total body water (particularly extracellular) relative to body weight. Decreased total body potassium and magnesium. Low plasma sodium, but increased total body sodium. Bone demineralization. Anemia is common and is related to iron, protein, and vitamin deficiencies. Impaired immune status as a result of thymic atrophy and impaired polymorphonuclear cell chemotaxis. Hepatocellular damage is a poor prognostic sign.

Clinical aspects

Presence of edema is necessary to make the diagnosis. Patients usually have stunted growth and wasting. There is relative sparing of subcutaneous adipose tissue. Anorexia, diarrhea, and skin excoriation are common. Wasting of cardiac muscle with decrease in stroke volume and prolongation of circulation time. Concentrating and diluting ability of the kidneys may be impaired.

Precautions before anesthesia

Evaluate cardiac function. Obtain echocardiography if necessary. Blood examination: should include a complete blood count, electrolytes, acid-base status, and liver function tests, including albumin. Coagulation profile and bleeding time should be obtained.

Anesthetic considerations

Low albumin concentration may increase the free fraction of protein-bound drugs, but this may be offset by an increase in total body water. Low cardiac output may slow induction with intravenous induction agents. Children with Kwashiorkor are unable to control their body temperature within a narrow range and may develop hypothermia or hyperthermia depending on the circumstances. Pressure points must be well protected in view of the friable skin.

Pharmacological implications

Consider altered hepatic and renal function and albumin concentration when selecting anesthetic agents and other drugs.

Other conditions to be considered

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