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The infant in "A rapidly deteriorating baby girl: Can you judge this book by its cover?" (Puzzler, June 2005) received an extensive work-up, including many blood examinations. She was given intravenous therapy for dehydration and intubated for impending respiratory failure. An electroencephalogram was performed for CNS changes, and a broad list of possible diagnoses was entertained before arriving at kwashiorkor. Having seen hundreds of children with severe protein-energy malnutrition (both kwashiorkor and marasmus) during my work in Africa, Latin America, and Asia, I would like to emphasize that severe protein-energy malnutrition is a clinical diagnosis; that is, one that can be made without laboratory or other examinations.
This infant's dietary intake was the major finding on history: During the four months before admission she had been given a diet very low in protein and her birth weight had not doubled at 6 months. There is no description of edema or pallor, although hours later in the pediatric intensive care unit both hepatomegaly and edema are noted. When she is rehydrated with IV fluids her hemoglobin is recorded as 10.3 g/dL, down from the initial concentrated value of 14.1 g/dL, and albumin was 2.3% when she was hemoconcentrated.
Given the central nervous system change of appearing tired, with a distended abdomen and hepatomegaly along with edema and poor growth, she falls directly into the category of kwashiorkor, with the confirmation of hypoalbuminemia. Infants with kwashiorkor can have a convulsion based on the edema, which is generalized and can certainly be found in the CNS. Because edema is dependent, I would often elevate the head of the child's cot to reduce that possibility.
Roy E. Brown, MD, MPH, DTMHNew York, N.Y.
Author reply: Dr. Brown's letter serves to remind us of the steps in arriving at a correct diagnosis. An accurate diagnosis requires a thorough history, followed by a comprehensive physical examination, supplemented by laboratory and radiographic tests as needed, all leading to formulation of a list of possible diagnoses. In this case, severe malnutrition was not at the top of our first list for a number of reasons: It is uncommon, in our experience; the patient didn't "look" malnourished and was not obviously edematous until after IV fluids; so many signs of illness (e.g., fever, dehydration, altered mental status, seizure, hepatomegaly) were present that other diagnoses were considered first; and, last, the abnormalities just mentioned required immediate treatment to prevent further instability. After the patient was stabilized, we were able to go back and reconsider the history (Did her diet really consist of almost pure fat for several months?), thereby placing malnutrition back on the list of possible diagnoses.
Dr. Brown's vast experience with protein-energy malnutrition provides an important perspective on the diagnosis and management of kwashiorkor, and we thank him for sharing his knowledge.
Jennifer Twente, MDRichard Strauss, MD