The Kwashiorkor Syndrome
( Originally Published 1956 )
In many areas of the world, infants and young children develop a syndrome of protein malnutrition that is of serious import. This condition is designated Kwashiorkor in Africa (38) and Syndrome Policarencial Infantil in Central and South America (45). The primary cause of kwashiorkor is a diet deficient in protein of good quality and the disease appears in the age groups where protein need is greatest. Manifestations of this syndrome vary somewhat from one area to another due, in large part, to differences in diet; in some localities, caloric undernutrition and vitamin deficiency exist concomitantly with deficiency of protein. Kwashiorkor is extremely rare in the United States although isolated cases have been observed.
The fundamental signs of kwashiorkor in Africa are retarded growth in the late breast-feeding, weaning and post weaning period, alterations in pigmentation of the skin and hair, edema, fatty infiltration, cellular necrosis or fibrosis of the liver and a high mortality unless good dietary protein is provided . Atrophy of the acini of the pancreas is considered by several investigators to be a fundamental lesion.
In central America, deficiency of calories and vitamins often complicate the clinical picture. In this area, unlike Africa, the incidence of kwashiorkor is relatively high in children more than four years of age and may be seen up to the age of twelve.
Other features of kwashiorkor which are commonly encountered include dermatoses, gastrointestinal disturbances such as anorexia, digestive upsets, diarrhea and steatorrhea, peevishness and mental apathy, and anemia. The skin lesions are multiple in type, bizarre in appearance, and have been designated "crazy pavement" dermatosis. Hyperpigmentation is frequent, with lesions which resemble pellagra, except for their distribution which is generalized rather than limited to areas exposed to sunlight or trauma. Vascular changes, hyperkeratinization and secondary infection are often observed. The etiology of the skin lesions is uncertain; vitamin, amino acid, or multiple deficiencies have been implicated. The hair becomes "dyspigmented," often assuming a reddish color, and falls out readily. In older children, the "flag" sign may be observed: when the hair is held up away from the scalp, stripes of normal color and of depigmentation may be noted, presumably signifying periods of dietary improvement and inadequacy, respectively. Factors responsible for these changes in the hair have not been elucidated. It has been postulated that deficiencies of sulfur-containing amino acids or of B complex vitamins may be responsible.
Alterations in function of the kidneys and heart have been reported in kwashiorkor. Oliguria is frequent and electrocardiographic changes have been observed. The latter include diminished amplitude of all deflections and prolongation of the Q-T interval
Laboratory findings in kwashiorkor include anemia which may be normocytic or slightly macrocytic; the bone marrow shows relative hypoplasia of the erythroid series (46). There is a decrease in pancreatic enzymes as determined by duodenal intubation and low levels of serum amylase, esterase, lipase and alkaline phosphatase are observed. Serum protein concentration is reduced markedly, especially the albumin fraction. Specimens of liver obtained by biopsy show fatty infiltration, cellular necrosis, fibrosis or a combination of these changes. Waterlow has found a fall in liver pseudo-cholinesterase paralleling the fall in plasma proteins.
Skim milk powder has been found to be effective in the treatment of kwashiorkor and its use has led to marked reduction in mortality. Recently, pure casein and amino acid mixtures have been shown to initiate recovery (48). Complete rehabilitation may require many months even though an adequate diet high in protein is administered.
Undoubtedly many mild cases of kwashiorkor go unrecognized, even in endemic areas, until intercurrent infection or parasitism precipitates the characteristic syndrome. Further investigation should elicit early findings and lead to recognition before the disorder has become advanced.
In regions of the world where kwashiorkor is common, a high incidence of chronic liver disease, not only cirrhosis but also primary carcinoma, is observed in adults. Relationships of these pathologic changes to chronic protein malnutrition require further investigation.