Marasmus and Kwashiorkor

marasmus are two separate diseases, but the former is so often superimposed on the
latter that most clinical cases of kwashiorkor are better characterized as “marasmic
kwashiorkor.”

Kwashiorkor is a disease syndrome resulting from a severe deficiency of dietary protein

relative to caloric intake. It is characterized typically by retardation of growth and
development, edema, fatty enlarged liver, anorexia, and alterations in the color and
texture of the hair and the skin. The child is severely apathetic, and at the same time,
irritable. Pneumonia and diarrhea are almost always present.

Kwashiorkor occurs in young children because at this period of life, protein

requirements per kilo of body weight are relatively high, diets are more likely to be
deficient in protein, and complicating infections that increase the need for protein are
most common.

The name kwashiorkor comes from the Ga language of Ghana and means literally “first-
second,” i.e. the disease of the first child when it is displaced at the mother’s breast. It
has had many other names in different parts of the world, but the name kwashiorkor,
given in its original description, is now universally accepted.

In the early 20th century, a form of kwashiorkor given the name mehlnahrschaden
(starch dystrophy) was described in Europe in infants fed excessive starch and little else.
Cases have been reported in Jamaica in children with a history of excessive calories
from cassava starch and very little dietary protein. Kwashiorkor has been observed in
older children and adults, but only rarely.

Inadequate dietary intake of protein is always part of the history. A deficient intake of
some micronutrients is common, and this contributes to clinical variations among
individuals and geographic regions.

Caloric intake is also usually low, and accounts for the marasmus, on which
kwashiorkor is commonly superimposed, as a result of the adverse metabolic effects of
infection on protein nutrition. These effects include anorexia (which causes a further
decrease in protein intake) and an increase in the catabolic loss of nitrogen in the urine.
In addition, diarrhea may reduce nitrogen adsorption from the gastrointestinal tract.

Characteristic findings on physical examination

The skin lesions and edema are diagnostic. These symptoms may be limited to the
extremities, or extended over the entire body. Characteristically, the skin becomes
hyperkeratotic, hyperpigmented, dry, cracked, scaly and peels. In Africa, it is sometimes
referred to as “crocodile skin,” “flaky paint,” or “crazy pavement dermatosis.”

Changes in the color and texture of hair are a constant finding, but differ with racial
origin. In black Africans, the hair takes on a reddish color, but in Latinos and Asians, it
becomes almost white. In both cases it is fine, and easily and painlessly pluckable.
On admission, lifting the hair will usually reveal darker roots and a lighter band
corresponding to the development of the acute episode of kwashiorkor. This has been
referred to as the “flag sign.” Upon discharge, the roots are seen to be again growing out
darker.

Marasmus is the name applied to wasting in infants and young children as a result of
severe food deprivation. It is a chronic disease that develops over many months or even
years.

Marasmic children are usually stunted. Atrophy of cutaneous and subcutaneous tissue
occurs, but this is true of any wasting disease and is not specific to marasmus. In the
final stages when all adipose tissue as a source of energy has been exhausted, death is
near.

In most developing countries in the past century and in some today, it was kwashiorkor
superimposed on marasmus that was the problem. Unlike kwashiorkor, marasmus does
not show the pathological changes characteristic of protein deficiency . A cytokine-
mediated response mobilizes amino acids from muscle and other tissues for
gluconeogenesis and essential new protein synthesis.

Under conditions of extreme poverty or disaster, during which food is not available,
marasmus is still seen and is a common cause of death in young children in refugee
populations, usually because of dehydration and complicating diarrhea or respiratory
infections.

In the early and mid-20th century, marasmus coexisted with kwashiorkor in most
developing countries and was the prevailing form of severe protein calorie deprivation
in some of them. It can also occur in adults under conditions of extreme poverty or
disaster, when sufficient food is not available.

The etiology of marasmus is a prolonged lack of adequate food, i.e. slow starvation. As
a result, such individuals must draw on their own tissues for as long as possible. At first,
this provides sufficient nutrients to meet internal metabolic needs, but once body fat as a
major internal source of energy is exhausted, death soon follows. At any time before
this point is reached, it is possible to reverse the process by appropriate feeding, unless
the child becomes severely dehydrated or is overwhelmed by a superimposed infection.

Marasmus will not be discussed further because it is not a dermatological problem

except in combination with kwashiorkor.

Expected results of diagnostic studies

The medical history and physical examination must be sufficiently thorough to

determine the type and severity of the case and the degree of hydration, as well as the
presence of any infection. The determination of urine volume, density, chloride, and pH
is essential to evaluate the dehydration and electrolyte imbalance, and to guide clinical
decisions. Microscopic examination of the urine sediment may reveal a urinary
infection, which requires prompt treatment. The severity and type of anemia should be
determined as a guide to prompt therapy.
As a result of the protein deficiency, many serum enzymes will be lowered. Examples
are: serum amylase, esterase, choline esterase, lipase, and alkaline phosphatase. If
convenient, one or two of these are worth obtaining for further confirmation, although
serum albumin is sufficient for this purpose. Serum choline esterase or amylase can be
profitably followed as further measure of recovery. The blood examination must include
basic hematological data. A stool examination should be done to check for amoeba and
other intestinal parasites.

While kwashiorkor can usually be easily diagnosed by physical appearance and history,
it should not be forgotten that a wide variety of pathological and biochemical changes
are occurring. These include an enlarged fatty liver, atrophied pancreas, and serum
biochemistry changes characteristic of protein deficiency. Episodes of diarrhea, as well
as respiratory and other infections precipitate kwashiorkor, but both diarrhea and
pneumonia are almost always present in patients with kwashiorkor, although sometimes
pneumonia may be identified only by x-ray.

Diagnosis confirmation

The signs and symptoms of kwashiorkor are so consistent and characteristic that the
diagnosis is an obvious one to anyone familiar with them. Nevertheless, kwashiorkor
has been misdiagnosed as pellagra in some countries because it also sometimes causes
darkly pigmented scaly skin. However, the distribution of the skin lesions is quite
different in the two conditions, and edema is not present in pellagra.

The first manifestation of pellagra may look very much like a sunburn and then become
more like a severe burn. This can become secondarily infected. The appearance soon
changes as the skin turns a somewhat dirty brown and frequently acquires a parchment
appearance. It then quickly becomes more pigmented, scaling or cracking wherever
exposure to the sun occurs (i.e. on the backs of the hands and forearms, elbows, knees,
and around the neck as “Casal’s necklace”).

With pellagra, there may be intriginous maceration of the sensitive skin induced by poor
personal hygiene, heat, and chaffing, not characteristic of kwashiorkor. The skin lesions
stop itching and respond quickly to niacin or tryptophan therapy.

Who is at Risk for Developing this Disease?

These conditions are characteristically found in impoverished areas, where there is not
adequate nutrition. Other circumstances include fad dieting (by choice or by psychiatric
illness) and extreme conditions such as massive weight loss following bariatric surgery.

This is rarely seen in the United States or Europe. It can still be seen in parts of South
and Central America, Africa, and Asia.

What is the Cause of the Disease?

Etiology

Pathophysiology
A history of an inadequate diet, low in both protein and energy, is common to marasmus
and kwashiorkor, but the appearance of the signs and symptoms of kwashiorkor
described above are usually precipitated by one or more recent episodes of infection,
most commonly diarrhea, a lower respiratory infection, or one of the common
communicable diseases of childhood. Measles, whooping cough, or repeated episodes
of diarrhea are particularly likely to be followed by kwashiorkor in a child with
marasmus.

Systemic Implications and Complications

The pathology of kwashiorkor is diverse because most body functions depend on a

steady supply of essential amino acids in appropriate proportions, as found in protein
from animals, legumes, and oil seeds, plus additional available nitrogen for the essential
amino acids that the body can synthesize from any biologically available source of
nitrogen.

In both human subjects and experimental animals, a diet that is deficient in either total
nitrogen or one or more essential amino acids will lead to physiological and
pathological changes in most body systems characteristic of kwashiorkor. These
changes include:

 Cardiovascular: The heart is relatively small, but its size increases rapidly with
treatment. Electrocardiographic findings include low voltage and alterations in
the T wave and rhythm.
 Exocrine glands: The liver is consistently enlarged due to a diffuse fatty change,
which starts at the center of the lobule and eventually affects nearly all of the
parenchymal cells. As a result, the sinusoids are generally collapsed. Acute
inflammatory foci, but not cirrhosis, may also be found at autopsy. Changes in
the exocrine pancreas include shrinkage in the size and number of the acinar
cells with no evidence of a central lumen. Their enzyme granules are decreased.
Salivary and intestinal glands are similarly affected, and the intestinal wall is
sometimes so atrophic as to be nearly transparent.
 Endocrine: The urinary excretion of 17-ketosteroids and 17- hydroxysteroids is
decreased. The thyroid gland contains follicles that are smaller than normal and
have cuboidal epithelium. The testes show only minimal signs of maturation as
compared to those of normal children of the same age. The adrenals tend to be
small in size and weight. Histopathological changes in the adrenal cortex
indicate hypofunction.
 Renal: A reduction in the glomerular filtration rate is reduced, initially in
proportion to the degree of dehydration. The glomerular filtration rate and inulin
and endogenous creatinine clearances are also reduced.
 Neurological: Electroencephalographic studies have shown diminished voltage
and excessively slow rhythmic activity. Some authors have reported abnormal
focal activity and other alterations. These abnormalities disappear as recovery
progresses.

Treatment Options

THE THERAPEUTIC LADDER

(for children 1-5 years old* with kwashiorkor)

First Day:

• Correct dehydration and electrolyte imbalance

• Start penicillin

• Keep patient warm

• Introduce gastric feeding tube and begin feeding formula of acidified half-skim milk,
half strength (protein 1-2 g/kg/day, calories 30-60g/kg/day)

Second to Fourth Day:

• Continue electrolyte solution

• Continue penicillin

• Diet: half-strength acidified skim milk (protein 2-5g/kg/day, calories 60-100/day) 5%

sugar may be added.

As soon as appetite returns, withdraw tube and begin oral feeding.

First to Second Week:

• Add electrolytes or potassium to the formula

• Continue penicillin

• Diet: whole milk (protein 5-7g/kg/day, calories 100-130/kg/day) plus banana, orange
juice, vegetables

Second to Fourth Week:

• Diet: Same (protein 5-7g/kg/day, calories 130-150/kg/day) plus meat, eggs, cereals,
and vegetables

Convalescence:

• Diet: balanced high-protein high-calorie diet (protein 5-6g/kg, calories 140-

150/kg/day)

• Physical and mental stimulation

*This age range will cover the great majority of all cases of kwashiorkor. Obvious
modifications will have to be made for infants and the very rare older child or adult with
kwashiorkor .

MANAGEMENT OF ELECTROLYTE IMBALANCES

Mild (kwashiorkor with no clinical signs of dehydration)

In very mild cases, adding sufficient potassium chloride to provide a total of 4 to 6mEq
of potassium per kg of body weight daily is sufficient. One gram of potassium chloride
provides 13mEq, and whole milk contains about 40mEq per liter. In mild cases,
treatment for the first 2-3 days is sufficient.

Moderate and Severe (kwashiorkor with marked clinical signs of dehydration)

The basic problems in these cases are severe dehydration, hyperosmolarity, metabolic
acidosis, and multiple anion and cation loss, especially of potassium. As soon as
possible after admission, the child should be given a buffered hypotonic solution
intravenously to counteract the acidosis and hyperosmolarity, and should begin the
correction of the dehydration and oliguria. If laboratory facilities for determining CO2
combining power and serum electrolytes are not available, this therapy must depend on
clinical observation.

A so-called 1-2-3 solution of one part 1/6 molar sodium lactate, two parts Ringer’s
solution, and three parts 5% glucose is recommended. The lactate in this solution
combats the acidosis, the glucose diminishes the ketosis, and the Ringer’s solution,
which contains 6mEq of calcium and 4mEq of potassium per liter helps to correct the
hyperkalemia and potassium depletion. The dosage varies from 40ml per kg to 50ml per
kg, administered intravenously at a rate of 40-50 drops per minute.

If facilities are not available for preparing this solution, the Ringer-lactate solution of
Hartman can be used, which, however, contains less lactate. Ringer’s Solution contains
per 1,000ml: NaCl 8.5g, KCl 0.3g, CaCl2 0.2g, and Na lactate 3.1g.

In most cases, diuresis begins after the preceding treatment has been administered. If
not, the solution can be continued some hours longer at a slower rate. It is advisable to
continue with Darrow’s solution more slowly, approximately 20-25 drops per minute at
a dosage of 90-110ml per kg per day, and to give an additional 5% glucose, in either
normal saline or Ringer’s solution, to help replace the total water loss. It is usually
necessary to give between 160ml and 200ml of fluid per kg in the first 24 hours.

Close and frequent examination is required to guide this therapy, since clinical signs of
diuresis are the best indices of the amount of fluids required. Oral feeding should begin
as soon as the patient’s condition permits, even within a few hours after admission.

Ringer’s Solution contains per 1000ml: NaCl 8.5g, KCl 0.3g, CaCl2 0.2g

Ringer’s Lactate Solution (Hartmann’s solution) contains per 1000ml: NaCl 6.0g, KCl
0.3g, CaCl2 0.2g, and Na lactate 3.1g

Darrow’s Solution contains per 1000ml: NaCl 3.0g, KCl 2.7g, and NaHCO2 4.4g

Moderate (kwashiorkor with moderate to severe vomiting as well as diarrhea)

These cases are rare and present a slightly different problem due to the loss of HCl from
the stomach and the superimposition of metabolic alkalosis on the fluid and electrolyte
disturbances described. If vomiting has been sufficiently acute to produce alkalosis, the
sodium lactate should be omitted from the solution given. The remainder of the
treatment is essentially the same.

Skim milk has been found most readily available for the dietary treatment of
kwashiorkor and marasmus, but some workers have obtained good therapeutic results
with vegetable protein mixtures. In Guatemala, a mixture of cottonseed flour and lime-
treated maize with added nutrients has given results equivalent to those with skim milk
powder. Properly processed soybean, peanut meal, or cotton seed meal can also be used
in this manner if skim milk is not available.

General Treatment Measures

Because the skin of the child with kwashiorkor is likely to be excoriated, good nursing
care is required to control secondary infection of the involved areas.

Because of their initial apathy and anorexia, these children will not ordinarily eat well
enough of their own accord for satisfactory recovery and may stay in one position
without moving. This must be taken into consideration by the nursing staff, particularly
in the feeding of children during early treatment.

It is important from the beginning to try to get these children’s attention and treat them
sympathetically and with understanding. In general, they are backward in psychosocial
and psychomotor development because of neglect and a longstanding lack of good
nutrition. A friendly attitude on part of the staff and a play area with toys will result in a
better outcome.

Advantage should be taken of the visits by the parents, as this is a good time to explain
to them the purely dietary nature of the treatment and the fact that a poor diet was
responsible for the development of the disease in their child.

MANAGEMENT OF OTHER COMORBIDITIES

Infections

It is important to recognize that children with kwashiorkor are very susceptible to

infections and almost always have pneumonia on admission. Even when the child is not
febrile and pneumonia cannot be detected by auscultation, pneumonia will be evident on
an x-ray of the lungs. It is for this reason that penicillin or another antibiotic should be
started on admission and continued throughout the initial recovery.

Even when infections are not initially present, the susceptibility of the child with
kwashiorkor to infections is so great that routine antibiotic therapy is recommended. In
advanced kwashiorkor, neither fever nor elevated leucocyte count may develop, even
when the infection is severe. Best results are obtained when penicillin or a broad-
spectrum antibiotic is given as soon as possible after admission for 6 to 8 more days.

During treatment, the child with kwashiorkor should be isolated from cross infections as
much as possible. In early studies, children with kwashiorkor treated on an open
pediatric ward frequently showed little or no growth for weeks after initial recovery.
When it was possible to study kwashiorkor children in individual cubicles, such a
stationary growth period was never again observed.

There is no doubt that episodes of infectious diarrhea are frequently precipitating factors
for kwashiorkor in an already malnourished child, but diarrhea is such a constant
accompaniment of kwashiorkor that it may or may not be due entirely to infection. This
cannot be said, however, of the diarrhea that persists after initial recovery. This must be
considered and treated as infectious.

Children with human immunodefficiency virus/acquired immunodeficiency syndrome

(HIV/AIDS) and tuberculosis are frequently severely malnourished and may develop
either marasmus or marasmic kwashiorkor. In addition to the treatment described for
severe malnutrition, specific treatment for these infectious diseases should be provided
promptly.

Children living under circumstances responsible for their severe malnutrition are almost
certain to have one or more common intestinal parasites such as Ascaris, Tricuris,
Giardia, and others. These are not likely to be a major factor in the development of
either marasmus or kwashiorkor, but after initial recovery, should be eliminated by
specific treatment.

Anemias

Children with kwashiorkor usually have anemia, but its cause may be complex. When
potential etiological factors are explored sequentially, there is a mild reticulocyte
response to the protein therapy that soon ends. The administration of iron usually results
in a more marked and prolonged reticulocyte response and recovery from the anemia.
However, there often remains some anemia that responds only to B vitamins. It is best
to give iron and B vitamins routinely early in therapy.

Optimal Therapeutic Approach for this Disease

The principles of therapy do not differ with severity, but the rapidity of moving from
one stage of treatment to the next may vary. The need for this is usually evident from
the response of the child, guided by both clinical response and the rate of improvement
in the laboratory findings.

Since nearly all kwashiorkor patients will be young children, it is important to spend
time explaining to the mother the cause of her child’s life-threatening illness and
helping her find ways that she can prevent its reoccurrence.

Any permanent cognitive damage is due to dietary and other factors affecting the child’s
earlier development. The child’s performance on cognitive tests will be reduced during
acute kwashiorkor, but usually will recover to the level achieved before the acute
disease.

Opportunities for exercise, games, and other stimulation appear to hasten complete
recovery. These principles also apply to the rare older child or adult that may be seen
with this disease.
Patient Management

Weeks 2-9 can be considered the rehabilitation period, during which close attention
must be given to continuing the initial treatments, as required. The World Health
Organization manual suggests a follow-up period of 7-26 weeks, depending upon the
individual case. This is consistent with the experience of the author in Central America.

Unusual Clinical Scenarios to Consider in Patient Management

The first week is the period of initial treatment. In the first 1-2 days, hypoglycemia,
hypothermia, and dehydration should receive priority attention. Low body potassium is
always present in individuals with severe malnutrition and can have fatal consequences.
Therefore, the correction of electrolyte imbalances must be given a given high priority.

Correction of electrolyte imbalances must begin immediately and may need to be

continued for at least 4 weeks. Correction of potassium deficiency within cells is rapid
(about 1 week). Replenishing total body potassium takes much longer. On admission,
treatment must be initiated for any infections present.

What is the Evidence?

Scrimshaw, NS, Viteri, FE. “INCAP studies of kwashiorkor and marasmus”. Food Nutr
Bull. vol. 31. 2010 Mar. pp. 34-41. (This recent publication summarizes and integrates
more than 25 years of experience of Institute of Central America and Panama [INCAP]
scientists studying all aspects of marasmus and kwashiorkor, and describes their
interrelations during the period when these diseases were still highly prevalent
worldwide.)

Williams, CD. ” A nutritional disease of childhood associated with a maize diet”. Arch
Dis Child. vol. 8. 1933. pp. 423-33. (This is the excellent original description of the
epidemiology, etiology, treatment, prevention, and naming of kwashiorkor.
Unfortunately, World War II soon followed. The author spent it in a Japanese prison
camp in Malaysia and her work was overlooked until a WHO/FAO mission in 1952
found the widespread distribution of the condition in Africa and determined the nature
of the disease. . .)

Béhar, M, FViteri, FE, Scrimshaw, NS. “Treatment of severe protein deficiency in

children (kwashiorkor)”. Am J Clin Nutr. vol. 5. 1957. pp. 506-15. (This is the single
most useful reference for treatment. In the 1950s, marasmic kwashiorkor was first
recognized as an almost universal problem in developing countries . Intensive
experience by INCAP clinicians in Guatemala, and their treatment of a large number of
cases [many of them severe], resulted in the development of a treatment regimen that
minimized case fatality and expedited recovery. It was widely adopted and served as the
basis for later World Health Organization recommendations.)

Goldberger, J, Milton, T. “Pellagra: its nature and prevention”. Goldberger on pellagra.

1964. pp. 373-80. (This is the original description of the clinical signs of pellagra by the
person whose studies in the southern United States during the Great Depression were
the first to identify it as a dietary deficiency disease. His work led to the recognition that
dietary niacin and tryptophan deficiency was the cause of this disease. It provides the
best detailed descriptions of the skin lesions.)

Béhar, M, Viteri, FE, Bressani, B, Arroyave, G, Squibb, RL, Scrimshaw, NS. Principles
of treatment and prevention of severe protein malnutrition in children (kwashiorkor).
1959. pp. 954-Guatemala City, Guatemala. (This paper recapitulates the advice on
treatment of kwashiorkor and goes on to describe factors responsible for the
development of clinical cases and principles of prevention. It also describes the full
range of protein sources available for the prevention and recuperation of cases of
kwashiorkor in developing countries.)

Scrimshaw, NS, Béhar, M. “Protein malnutrition in young children”. Science. vol. 3470.
1961. pp. 2039-46. (This paper recapitulates and details further experience with the
various forms and combination of kwashiorkor and marasmus. It contains good pictures
of edema and skin lesions, as well as hair changes, in kwashiorkor. It clarifies and
illustrates the multiple combinations of kwashiorkor and marasmus and how this
impacts treatment and recovery.)

Management of severe malnutrition: a manual for physicians and other senior health
workers. 1999. (This manual was developed as a cooperative effort of experts in
different parts of the world with first-hand experience in the treatment of kwashiorkor
and other forms of severe protein energy malnutrition. The advice on treatment takes
into account the necessity of adapting treatment procedures to regional variations in the
signs, symptoms, and pathologies in different populations. The treatment and case
management of kwashiorkor suggested in this chapter are consistent with its
recommendation.)

Severe malnutrition: report of a consultation to review current literature. 2004. (This is

the report of an informal consultation convened by WHO in Geneva in 2004.
Temporary advisers for this meeting included representatives from Bangladesh,
England [3], Germany, South Africa, and the United States , all of whom had in-depth
experience with the management of severe protein malnutrition in one or more
developing countries. The charge given to this group was to critically review new
evidence relevant to the current WHO guidelines, consider if changes to the guidelines
are required as a result of new evidence, and assess the guidelines in relation to care of
the poorest severely malnourished children with HIV/AIDS.The consultation found
only very limited new research data and no need to revise current WHO guidelines for
the dietary management of kwashiorkor; however, experience with the treatment of
kwashiorkor has programmatic implications for the management of patients with
advanced HIV/AIDS.)

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