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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.

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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.

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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.

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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.

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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.

Collins S, Sadler K: ...

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