Background

Malnutrition, so common in third world countries, may be more common than suspected in modern countries. The two classic forms include kwashiorkor and marasmus. The term protein-energy malnutrition is now generally used.

Disease	Edema	Other
Kwashiorkor	Yes	Total protein deprivation
Marasmus	No	Total caloric deprivation

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGIC ASSOCIATIONS

CHARACTERIZATION

Kwashiorkor in the United States Fad Diets, Perceived and True Milk Allergy, and Nutritional Ignorance Theodore Liu, etal.

Arch Dermatol. 2001;137:630-636 Abstract quote Background Kwashiorkor is the edematous form of protein-energy malnutrition. It is associated with extreme poverty in developing countries and with chronic malabsorptive conditions such as cystic fibrosis in developed countries. Rare cases of kwashiorkor in affluent countries unrelated to chronic illness have been reported. We present 12 cases of kwashiorkor unrelated to chronic illness seen over 9 years by pediatric dermatologists throughout the United States, and discuss common causative themes in this easily preventable condition. Observations Twelve children were diagnosed as having kwashiorkor in 7 tertiary referral centers throughout the United States. The diagnoses were based on the characteristic rash and the overall clinical presentation. The rash consisted of an erosive, crusting, desquamating dermatitis sometimes with classic "pasted-on" scalethe so-called flaky paint sign. Most cases were due to nutritional ignorance, perceived milk intolerance, or food faddism. Half of the cases were the result of a deliberate deviation to a protein-deficient diet because of a perceived intolerance of formula or milk. Financial and social stresses were a factor in only 2 cases, and in both cases social chaos was more of a factor than an absolute lack of financial resources. Misleading dietary histories and the presence of edema masking growth failure obscured the clinical picture in some cases. Conclusions Physicians should consider the diagnosis of kwashiorkor in children with perceived milk allergies resulting in frequent dietary manipulations, in children following fad or unorthodox diets, or in children living in homes with significant social chaos. The presence of edema and "flaky paint" dermatitis should prompt a careful dietary investigation.

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
CYSTIC FIBROSIS
Cystic fibrosis presenting as kwashiorkor in a Sri Lankan infant. Mei-Zahav M, Solomon M, Kawamura A, Coates A, Durie P. Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.	Arch Dis Child. 2003 Aug;88(8):724-5 Abstract quote.   Growth failure is a common presentation of patients with pancreatic insufficient cystic fibrosis. However, full blown kwashiorkor is extremely rare. Cystic fibrosis is also considered to be rare in the South Asian population. This report describes a Sri Lankan infant with cystic fibrosis who presented with clinical features of severe kwashiorkor.

 

PATHOGENESIS

CHARACTERIZATION

Pro-inflammatory cytokines in Turkish children with protein-energy malnutrition. Dulger H, Arik M, Sekeroglu MR, Tarakcioglu M, Noyan T, Cesur Y, Balahoroglu R. Department of Biochemistry, School of Medicine, University of Yuzuncu Yil, Tip Fakultesi, Biyokimya Anabilim Dali, Maras Caddesi, 65300 Van, Turkey.

Mediators Inflamm. 2002 Dec;11(6):363-5 Abstract quote.   BACKGROUND: Protein-energy malnutrition (PEM) results from food insufficiency as well as from poor social and economic conditions. Development of PEM is due to insufficient nutrition. Children with PEM lose their resistance to infections because of a disordered immune system. It has been reported that the changes occurring in mediators referred to as cytokines in the immune system may be indicators of the disorders associated with PEM. AIMS: To determine the concentrations of pro-inflammatory cytokines in children with PEM, and to find out whether there was an association with the clinical presentation of PEM. METHODS: The levels of serum total protein, albumin, tumour necrosis factor-alpha, and interleukin-6 were measured in 25 patients with PEM and in 18 healthy children as a control group. PEM was divided into two groups as kwashiorkor and marasmus. The kwashiorkor group consisted of 15 children and the marasmus group consisted of 10 children. RESULTS: Levels of serum total protein and albumin of the kwashiorkor group were significantly lower than both the marasmus group and controls (p < 0.05). In view of tumour necrosis factor-alpha levels, there was no difference between groups (p > 0.05). While levels of interleukin-6 in both the marasmus group and the kwashiorkor group were significantly higher compared with controls (p < 0.05), there was no significant difference between the groups of marasmus and kwashiorkor (p > 0.05). CONCLUSIONS: It was observed that the inflammatory response had increased in children with malnutrition.

 

LABORATORY/ RADIOLOGY	CHARACTERIZATION
Plasma electrolytes, total cholesterol, liver enzymes, and selected antioxidant status in protein energy malnutrition. Etukudo MH, Agbedana EO, Akinyinka OO, Osifo BO. Department of Chemical Pathology, College of Medicine, University of Ibadan, Ibadan, Nigeria.	Afr J Med Med Sci. 1999 Mar-Jun;28(1-2):81-5. Abstract quote   Golden and Ramdath proposed the free radical theory of kwashiorkor, suggesting that the changes seen in kwashiorkor may be the result of an imbalance between the production and safe disposal of free radicals. In malnourished children, mineral metabolism and antioxidant status need renewed attention especially in relation to cause and functional significance of the changes in concentration of these substances. In the present study, the modified Wellcome classification was used to classify the protein energy malnourished children into kwashiorkor marasmic-kwashiorkor, marasmus and underweight. Twenty-six healthy and normal children were used as controls. Standard procedures were used for the analyses of the biochemical parameters. Our results showed that plasma total cholesterol, sodium, potassium and bicarbonate, beta-carotene, retinol and uric acid were significantly lower in the malnourished group than the control group (P < 0.05), while transaminases were significantly increased in the malnourished group (P < 0.05). These findings suggest an altered electrolyte and antioxidant status in protein energy malnutrition.
Prediction of nutritional status by chemical analysis of urine and anthropometric methods. Adewusi SR, Torimiro SE, Akindahunsi AA. Department of Chemistry, Obafemi Awolowo University, Ile-Ife, Nigeria.	Nutr Health. 2002;16(3):195-202 Abstract quote.   A combination of anthropometric and chemical indices was used to investigate the nutritional status of 26 healthy (H) and kwashiorkor (K) children aged 2-5 years and possibly predict the onset of malnutrition. The healthy children had significantly (p>0.05) higher values for weight, height, middle upper arm circumference and weight/height ratio than kwashiorkor children. The healthy children had significantly higher urinary concentrations of urea, inorganic sulphate and sulphate relative to creatinine, but significantly lower thiocyanate and thiocyanate relative to creatinine than the kwashiorkor children. The results of the present study would tend to indicate that a chemical analysis especially of urea, inorganic sulphate or thiocyanate alone or the urea/creatinine, inorganic sulphate/creatinine or thiocynate/creatinine ratio could be used to predict malnutrition after the initial anthropometric measurements. This study should however be repeated with a large population of volunteers to determine the specific cut-off points for each anthropometric and chemical analysis.
SWEAT TESTING
Sweat test results in children with primary protein energy malnutrition. Yigit H, Selimoglu MA, Altinkaynak S. Ataturk University, Faculty of Medicine, Department of Pediatrics, Erzurum, Turkey.	J Pediatr Gastroenterol Nutr. 2003 Sep;37(3):242-5 Abstract quote. OBJECTIVES: In underdeveloped and developing countries where protein energy malnutrition (PEM) is common, it is sometimes difficult to exclude the diagnosis of cystic fibrosis (CF) in malnourished children because both primary PEM and CF share similar symptoms, signs, and laboratory findings, such as elevated sweat chloride value. This study was performed to investigate sweat test results and determine percentile values in children with primary PEM. METHODS: A total of 90 children with PEM and 30 healthy children were included. PEM was classified according to criteria defined by Gomez, Waterlow, and McLaren. Sweat tests were performed using the Macroduct conductivity system. RESULTS: Patient age and gender did not affect the test results (P > 0.05). The mean sweat conductivity (equivalent NaCl mMol/L) of patients with PEM was higher than that of controls (P < 0.001) and increased with the degree of malnutrition (P < 0.001). Inverse correlations between sweat conductivity and weight for age, height for age, and weight for height were detected (P < 0.001). The highest value was found in children with wasting and stunting, followed by those with stunting (P < 0.05) and those with marasmic kwashiorkor (P < 0.01). Of all children with PEM, 6.7% had elevated sweat test results that normalized after nutritional management; of children with third degree PEM, the figure was 20%. Ninety-fifth percentile values of first, second, and third degree malnutrition were 47 mMol/L, 49 mMol/L, and 69 mMol/L, respectively. CONCLUSION: Elevated sweat test result is not an important problem, especially in first and second degree PEM, but borderline values can be detected in as many as 20% of cases of third degree malnutrition. Sweat conductivity may increase to 69 mMol/L in children with stunting, those with wasting and stunting, and in those with third degree PEM.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General
VARIANTS
Kwashiorkor	Superficial perivascular infiltrate of lymphocytes, pallor of keratinocytes in a band across the upper part of the epidermis, and confluent parakeratosis Pallor, ballooning, and necrosis of keratinocytes in a band across the upper part of the epidermis and is considered nearly pathognomonic for a dermatitis due to a nutritional deficiency Alternative pattern lacks the epidermal pallor and has only psoriasiform epidermal hyperplasia Not pathognomonic for kwashiorkor and may also be found in other uncommon conditions that seem to have a nutritional deficiency

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Other nutritional disorders	Acrodermatitis enteropathica Necrolytic migratory erythema Pellagra Methylmalonic acidemia Essential fatty acid deficiency Cystic fibrosis Multiple carboxylase deficiency

 

PROGNOSIS AND TREATMENT

CHARACTERIZATION

Outpatient care for severely malnourished children in emergency relief programmes: a retrospective cohort study. Collins S, Sadler K. Valid International, Stockwell, London, UK.

Lancet. 2002 Dec 7;360(9348):1824-30 Abstract quote.   BACKGROUND: In emergency nutritional relief programmes, therapeutic feeding centres are the accepted intervention for the treatment of severely malnourished people. These centres often cannot treat all the people requiring care. Consequently, coverage of therapeutic feeding centre programmes can be low, reducing their effectiveness. We aimed to assess the effectiveness of outpatient treatment for severe malnutrition in an emergency relief programme. METHODS: We did a retrospective cohort study in an outpatient therapeutic feeding programme in Ethiopia from September, 2000, to January, 2001. We assessed clinical records for 170 children aged 6-120 months. The children had either marasmus, kwashiorkor, or marasmic kwashiorkor. Outcomes were mortality, default from programme, discharge from programme, rate of weight gain, and length of stay in programme. FINDINGS: 144 (85%) patients recovered, seven (4%) died, 11 (6%) were transferred, and eight (5%) defaulted. Median time to discharge was 42 days (IQR 28-56), days to death 14 (7-26), and days to default 14 (7-28). Median rate of weight gain was 3.16 g kg(-1) x day(-1) (1.86-5.60). In patients who recovered, median rates of weight gain were 4.80 g kg(-1) day(-1) (2.95-8.07) for marasmic patients, 4.03 g x kg(-1) x day(-1) (2.68-4.29) for marasmic kwashiorkor patients, and 2.70 g x kg(-1) x day(-1) (0.00-4.76) for kwashiorkor patients. INTERPRETATION: Outpatient treatment exceeded internationally accepted minimum standards for recovery, default, and mortality rates. Time spent in the programme and rates of weight gain did not meet these standards. Outpatient care could provide a complementary treatment strategy to therapeutic feeding centres. Further research should compare the effectiveness of outpatient and centre-based treatment of severe malnutrition in emergency nutritional interventions.

Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.

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Last Updated 1/5/2004

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