OVERVIEW: What every practitioner needs to know

Are you sure your patient has Marasmus? What are the typical findings for this disease?

Marasmus is a state of protein energy malnutrition that results from a slow and inadequate source of energy and protein intake. In this form of malnutrition, the body has compensated and adapts to the low metabolism. This physiologic change allows the body's vital organs to receive nourishment and prolong survival.

Typical findings in Marasmus include loss of fat mass, especially in the buttocks area. This can also be associated with infectious states, decreased linear growth, decreased heart rate and blood pressure, thin and dry skin, decreased metabolic rate, lethargy, apathy, thin hair, hypothermia, hypoglycemia, electrolyte imbalances, vitamin deficiency.

1. Loss of fat mass, failure to thrive

2. Chronic diarrhea

3. Emaciated body type

What other disease/condition shares some of these symptoms?

Neglect

Child abuse

Mixed Marasmus/Kwashiorkor

What caused this disease to develop at this time?

Severe malnutrition of this type occurs in settings of poor socioeconomic status in developed countries. It can also occur because of child abuse and neglect.

In developing countries, a lack of food resources is the cause of malnutrition. This occurs in areas of political turmoil and famine.

What laboratory studies should you request to help confirm the diagnosis of Marasmus? How should you interpret the results?

• Vital signs: bradycardia, hypothermia

• CBC: to evaluate for anemia, leukocytosis

• CMP: to monitor for hypoglycemia and hypoalbuminemia

• Prealbumin: sensitive index of protein synthesis, which shows severity of malnutrition

• IGF-1: sensitive marker of nutritional status

Would imaging studies be helpful? If so, which ones?

No imaging is needed for diagnosis.

If you are able to confirm that the patient has Marasmus, what treatment should be initiated?

There are three phases in regards to treatment: initial phase, rehabilitation phase, follow up phase.

Initial Phase - This phase occurs within the first 10 days of treatment. You must correct for fluid and electrolyte imbalances by providing oral or IV hydration. any infections must be properly treated. Those susceptible to infection can succumb to septic shock. In this circumstance, the child will require immediate intervention with fluid resuscitation to maintain blood pressure.

Rehabilitation Phase - This phase occurs within 2-6 weeks of treatment when the child is eating well and is no longer malabsorbing. At this point you can start reintroducing nutritious foods. It is important to provide an increase in the daily caloric content, as well as increased protein intake. When starting a balanced diet, it is important to reintroduce foods slowly, as these children are in danger of refeeding syndrome. Calories should be increased gradually. It is recommended that the affected persons should only take in an extra 25 kcal/kg/day every other day, at first. Vitamins and minerals should be supplemented, as these levels will also be deficient in those with malnutrition. It is also important to have a multidisciplinary approach, as many will have emotional pain from the malnutrition.

A child psychologist should monitor the child closely. Encouraging signs of recovery include the return of appetite, decreased apathy, and improved social functioning.

Follow up Phase - Simply entails close monitoring of the child. Though treatment cures the acute symptoms, catch up linear growth may never be fully achieved.

What are the adverse effects associated with each treatment option?

When starting a balanced diet, it is important to reintroduce foods slowly, as these children are in danger of refeeding syndrome. Calories should be increased gradually. It has been recommended that the affected persons should only take in an extra 25 kcal/kg/day every other day, at first. Those that are severely malnourished can also develop an intolerance to lactose containing products.

What are the possible outcomes of Marasmus?

Unfortunately, even if the diet is corrected, most children will be unable to reach their full height and growth potential. But, diet correction will improve their overall general health. If this malnutritive state has occurred for a long period of time, it can result in psychological and physical impairment.

If families are committed to long term interdisciplinary treatment, a positive prognosis is to be expected. However, if this state of malnutrition remains untreated, it can eventually result in death.

What causes this disease and how frequent is it?

• Inadequate intake of calories leads to suppressed gluconeogenesis. This impairs further protein breakdown and allows ketones from fat to become the main energy source for the body, especially vital organs like the brain and heart.

• Chronic underfeeding leads to decreased basal metabolic rates.

• More common in those 5 years old or younger.

• More common in developing countries due to lack of food resources. Can occur in developed countries, especially in families of low socioeconomic status.

• According to the World Health Organization (WHO), the amount of malnourished children worldwide is estimated at 182 million in 2000. Almost 55% of childhood mortality has a factor of malnutrition as a cause.

How do these pathogens/genes/exposures cause the disease?

Other clinical manifestations that might help with diagnosis and management

Anemia

Hypoglycemia

Apathy, flat affect

Prolonged episodes of diarrhea

Reduced muscle and fat mass

What complications might you expect from the disease or treatment of the disease?

Those in a severe malnutrition state are susceptible to infections and sepsis due to an inability to mount a robust inflammatory response. The febrile episode can be reduced. Overwhelming sepsis can result in death.

Electrolyte imbalances are commonly present in this setting. Most common depiction is of hypernatremia with hypokalemia. If hypokalemia is severe, it can result in decreased cardiac contractility, as well as hypotonia.

When reintroducing normal feedings in a patient with marasmus, the patient is at risk for refeeding syndrome if the diet is advanced too rapidly. Findings in refeeding syndrome include acute imbalances in fluid and electrolyte status, including fluid overload or dehydration, hypophosphatemia, hypokalemia, hypomagnesia, and hypoglycemia. Weakness and paresthesias may be seen due to the electrolye abnormalities or vitamin deficiencies.

Are additional laboratory studies available; even some that are not widely available?

How can Marasmus be prevented?

Well balanced diet with adequate protein intake and vitamin supplementation.

In those that are critically ill, early enteral feedings can significantly reduce malnutrition.

Reduce infections in endemic areas by promoting vaccinations, as infections contribute to malnutrition.

Have proper food supplies available in war torn nations or in countries susceptible to famine.

What is the evidence?

http://whqlibdoc.who.int/hq/2000/WHO_NHD_00.6.pdf..

Penny, ME, Walker, WA, Watkins, JB, Duggan, C. "Nutrition in Pediatrics". BC Decker. 2003.

Pelletier, DL, Frongillo, Ea, Schroeder, DG, Habicht, JP. "The effects of malnutrition on child mortality in developing countries". Bull World Health Org.. vol. 73. 1995. pp. 443-8.

Ongoing controversies regarding etiology, diagnosis, treatment

While the common practice is to provide gut rest with diarrhea, there are newer studies that support starting enteral feeds early to provide appropriate healing with the re-establishment of the normal gut function and flora.