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Acquired disorder caused by malnutrition secondary to
insufficient protein intake resulting in multisystemic chronic failure with
generalized edemas.
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No genetic component. Acquired malnutrition
disease.
++
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 criteria in
making the 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.
++
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.
++
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.
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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.
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Consider altered hepatic and renal function
and albumin concentration when selecting anesthetic agents and other drugs.
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Danbolt-Closs Syndrome: An inherited vesiculobullous disorder
characterized by intermittent simultaneous occurrence of diarrhea and
bullous dermatitis (dry lesions surrounding the mouth, ears, nose and eyes,
but also affecting the fingers, feet, and knees) and failure to thrive in
premature babies. In children, periorificial lesions of the face and
anogenital region occur. Alopecia and absence of eyebrows, eyelashes, and thymus
are common.
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Keshan Disease: A condition caused by a deficiency of the essential
trace mineral selenium as well as an infection with an enterovirus (coxsackie).
It is named after the Chinese province Keshan, where this disease is endemic and
was first described. It has since been reported from other parts of the world (e.g.,
New Zealand, Finland). The clinical picture consists of a potentially fatal cardiomyopathy of
childhood characterized by multiple patchy areas of myocardial
necrosis (later fibrosis) mainly affecting the left ventricle, but also the atria and the
right ventricle. The necrotic lesions are isolated and lack a vascular or
inflammatory reaction.