Kwashiorkor Abdullah M. Al-Olayan MBBS, SBP, ABP. Assistant Professor of Pediatrics. Pediatric Pulmonologist. Introduction : Protein-energy malnutrition (or protein-calorie malnutrition) refers to a form of malnutrition where there is inadequate protein intake. Types include : 1- Kwashiorkor (protein malnutrition predominant) 2- Marasmus (deficiency in both calorie and protein nutrition) 3-Marasmic Kwashiorkor (marked protein deficiency and marked calorie insufficiency signs present, sometimes referred to as the most severe form of malnutrition) Introduction : Severe malnutrition is primarily a problem in developing countries. The clinical assessment of the child with malnutrition includes distinguishing between marasmus and kwashiorkor, assessing the severity of the malnutrition, and identifying acute life-threatening complications, including sepsis and acute dehydration. Introduction : These children are at risk for micronutrient deficiencies. Definitions : The World Health Organization (WHO) defines malnutrition as “ the cellular imbalance between the supply of nutrients and energy and the body's demand for them to ensure growth, maintenance, and specific functions." Definitions : Protein-energy malnutrition (PEM) applies to a group of related disorders that include marasmus , kwashiorkor, and intermediate states of marasmus-kwashiorkor. Definitions : Marasmus involves inadequate intake of protein and calories and is characterized by emaciation. Definitions : The term kwashiorkor is taken from the Ga language of Ghana and means "the sickness of the weaning." Williams first used the term in 1933, and it refers to an inadequate protein intake with reasonable caloric (energy) intake. Epidemiology : Malnutrition underlies 55% of childhood mortality worldwide. Kwashiorkor may occur at any age, but is seen most frequently in children 1–3 years of age. Pathophysiology : In kwashiorkor, adequate carbohydrate consumption and decreased protein intake lead to decreased synthesis of visceral proteins. The resulting hypoalbuminemia contributes to extravascular fluid accumulation. Impaired synthesis of B-lipoprotein produces a fatty liver. Clinical Presentation : History : Low intake of calories or an inability to absorb calories is the key factor in the development of kwashiorkor. Acrodermatitis enteropathica. Clinical Presentation : History : Kwashiorkor was reported in an infant presenting with diarrhea and dermatitis, due to infantile Crohn disease. The diarrhea and dermatitis improved in 2 weeks with treatment. Erythematous skin, desquamation, erosions, and diffuse hyperpigmentation Clinical Presentation : Physical : Kwashiorkor typically presents with : 1-Normal or nearly normal weight and height for age. 2-Pitting edema in the lower extremities. 3-moon facies. 4-Distended abdomen with dilated intestinal loops. 5-Dry, atrophic, peeling skin with confluent areas of fatty liver. hyperkeratosis and hyperpigmentation. Clinical Presentation : Physical : 6-Dry, dull, hypopigmented hair that is easily plucked. 7-Wasting is also typical. 8-Mental status changes. 9-There is some degree of edema in all cases of Kwashiorkor. 10-The hands and face may become edematous. 11-Facial edema gives the characteristic “moonfaces.” Causes : Worldwide, the most common cause of malnutrition is 1-inadequate food intake.( PRIMARY ) 2-Ineffective weaning. 3-Gastrointestinal infections. 4-Cystic fibrosis. Causes : 5-Chronic renal failure. 6-Childhood malignancies. 7-Congenital heart disease. 8-Liver cirrhosis. Differential diagnosis : 1-Nephrosis: Edema is common and albumin is present in urine. Ascites is common in nephrosis, but rare in Kwashiorkor. 2-Hookworm anemia: May cause edema alone. Hookworm infection is commonly seen in association with Kwashiorkor. Hookworm anemia is not associated with the dermatological findings commonly seen in Kwashiorkor. 3-Chronic dysentery. Investigations : Laboratory Studies : The WHO recommends the following laboratory tests: 1-Blood glucose. 2-Examination of blood smears. 3-Hemoglobin. 4-Urine examination and culture. 5-Stool examination by microscopy for ova and parasites. 6-Serum albumin. 7-HIV test. 8-Electrolytes. Investigations : Significant findings in kwashiorkor include hypoalbuminemia (10-25 g/L), hypoproteinemia (transferrin, essential amino acids, lipoprotein), and hypoglycemia. Plasma cortisol and growth hormone levels are high. Electrolytes, especially potassium and magnesium, are depleted. Levels of some enzymes (including lactase) are decreased, and circulating lipid levels (especially cholesterol) are low Investigations : Histologic Findings : In kwashiorkor, microscopic studies of hair have revealed a decrease in the proportion of anagen follicles. Treatment : 1-Correct fluid and electrolyte abnormalities and to treat any infections. The most common electrolyte abnormalities are hypokalemia, hypocalcemia, hypophosphatemia, and hypomagnesemia. Macronutrient repletion should be commenced within 48 hours under the supervision of nutrition specialists. Treatment : 2- Supply macronutrients by dietary therapy. Milk-based formulas are the treatment of choice. At the beginning of dietary treatment, patients should be fed ad libitum. After 1 week, intake rates should approach 175 kcal/kg and 4 g/kg of protein for children. A daily multivitamin should also be added. Prognosis : The extent of growth failure and the severity of hypoproteinemia, hypoalbuminemia, and electrolyte imbalances are predictors of a poorer prognosis. Additionally, underlying HIV infection is associated with a poor prognosis.