In this article we will discuss about the Kwashiorkor:- 1. Meaning of Kwashiorkor 2. Clinical Features of Kwashiorkor 3. Biochemical and Metabolic Disorders 4. Changes in the Organs and Systems of the Body 5. Treatment 6. Prognosis.
Contents:
- Meaning of Kwashiorkor
- Clinical Features of Kwashiorkor
- Biochemical and Metabolic Disorders of Kwashiorkor
- Changes in the Organs and Systems of the Body
- Treatment for Kwashiorkor
- Prognosis of Kwashiorkor
1.
Meaning of Kwashiorkor:
Kwashiorkor is due to a quantitative and qualitative deficiency of protein, but in which energy in take may be adequate. It is mainly a disease of rural areas occurring in the second year of life. This disease occurs when the child is weaned into the traditional family diet; this may be low in protein because of poverty, insufficient land and poor agricultural practice.
There is no supplement of milk. The disease is frequently precipitated by outbreaks of febrile illnesses, such as malaria, measles or gastroenteritis. It arises as a result of poverty and ignorance. Many mothers have received no satisfactory instruction in infant feeding although there is money to buy food. In Africa, the disease is commoner.
2. Clinical Features of Kwashiorkor:
a. There are oedema, anorexia, diarrhoea and a generalized unhappiness or apathy. An infection often precipitates at the onset for which the child is brought to the doctor.
b. Failure of growth is an early sign. Oedema is more marked in the lower limbs.
c. The characteristic dermatosis consists of areas of both hypo- and hyperpigmentation. The skin first becomes thickened as if varnished. This then peels and appears like “flaky paint” leaving cracks or denuded areas of shallow ulceration. In moderate cases the dermatosis resembles crazy paving; when severe, the desquamated part looks as if there has been a burn. The lower limbs, buttocks and perineum are usually most affected but ulcers can occur over pressure points and deep cracks in skinfolds.
d. The hair is sparse, soft and thin. Negro children lose their characteristic curl. There may be changes in pigmentation with diffuse patches or streaks which may be red or grey in colour.
e. Angular stomatitis, cheilosis and a smooth atrophic tongue are commonly seen, as in ulceration around the anus.
6. Watery diarrhoea or large semisolid, acid stools are usual. The liver can generally be palpated and is firm, not tender.
g. The muscles are always wasted and as a result many children may no longer be able to walk or crawl.
h. Some degree of anemia is always present and may be severe.
i. Apathy is a characteristic feature and the child appears constantly unhappy. Neurological features are unusual, but some children during recovery have tremors resembling parkinsonism.
j. Many patients show a mixture of some of the features of both marasmus and kwashiorkor. These children are said to have marasmic kwashiorkor.
k. Some children adapt to prolonged insufficiency of food by a marked retardation of growth. They resemble children a year or more younger.
3. Biochemical and Metabolic Disorders of Kwashiorkor:
a. A high body water content, loss of the fat stores, and loss of protein from the wasted muscles and other tissues greatly alter the chemical composition of the child.
b. Plasma concentrations of essential amino acids, especially branched chain amino acids and tyrosine are low, but those of some non-essential amino acids may be higher than normal. Sooner the treatment starts with protein, the concentration of amino acids in the plasma rises and there may be an overflow aminoaciduria.
The plasma albumin level is low owing to a failure of synthesis in the liver. In severe cases, it is usually below 20 and sometimes below 10 g/1. Plasma IgG is often raised if infections are present but other immuno-globulins are usually normal.
Plasma transferrin is lowered, especially in severe cases, and may be a better guide to prognosis than plasma albumin. The plasma concentrations of some enzymes such as cholinesterase, alkaline phosphatase, amylase, and lipase are lowered.
The blood urea is usually low and may fall to 6 mg/100 ml. This indicates a reduced protein intake rather than a lowered rate of protein catabolism. Urinary creatinine is also reduced reflecting decreased muscle mass.
c. There is fatty liver and the excess of fat in the liver is triglyceride. Plasma triglyceride and cholesterol are low due to a decreased ability of the liver cells to mobilize lipid in the form of lipoproteins.
d. Blood glucose is usually normal. Hypoglycemia may occur and is a complicated matter to be kept in mind.
e. Plasma potassium level is below normal due to diarrhoea. Plasma magnesium level is also low due to increased losses in the stool.
f. Plasma [H+] may be either raised or lowered. Acidosis is probably due to poor circulation and consequent tissue hypoxia. Alkalosis may be associated with potassium depletion and a failure of the kidneys to excrete bicarbonate.
g. The total body water may increase from 60 per cent to 80 per cent. The severity of clinical oedema is not closely associated with the size of the increase in total body water or with the level of plasma albumin. The cause and nature of the oedema still remains in part a mystery.
The oedema may shift from one part of the body to another. Clinical dehydration and shock may be found in a child, associated with gross oedema in parts of the body.
h. Malnourished children are likely to have infections and may have complications requiring drug treatment. Some antibiotics and antimalarial act by interfering with nutrition more in the micro-organism than in the human host, and they need to be used with care in Protein Energy Malnutrition (PEM).
The antibiotics streptomycin, chloramphenicol and the tetracyclines inhibit protein synthesis by interfering with the action of messenger or transfer RNA. The antimalarial trimethoprim is folate antagonist.
Some drugs are carried in the circulation bound to plasma proteins. Kwashiorkor plasma with a low albumin content has a reduced binding capacity with the drugs. Higher concentrations of the free form of the drug increase the risk of toxic effects. Many drugs are detoxicated in the liver by the microsomal enzyme-oxidising system and its function may be impaired in PEM. Therefore, all drugs should be used with caution.
4. Changes in the Organs and Systems of the Body:
a. The atrophy of the cells of the pancreas and intestinal mucosa cannot produce digestive enzymes in normal amounts. Duodenal contents contain reduced amounts of amylase, trypsin and lipase. The activity of lactase, sucrase, and maltase are greatly reduced in the atrophic mucosa which is also associated with impaired absorption of nutrients.
b. The fat first accumulates in small droplets within liver cells, situated at the periphery of the lobules. The droplets increase in size and extend from the periphery to the centre of the lobules. In severe cases, all the liver cells may be filled with big fat droplets, pushing aside the cell nucleus and reducing the cytoplasm to a narrow rim.
Yet the liver function is well maintained and severe liver failure is unusual. Plasma bilirubin is normal; prothrombin concentrations are often reduced, but return to normal on treatment with vitamin K. In case the plasma concentrations of alanine amino-transferase and isocitrate dehydrogenase are found raised, they suggest the presence of damage from a bacterial or viral infection.
With proper treatment the lipid accumulated in the liver cells is all cleared with return to a normal structure.
c. Plasma concentration of growth hormone may be raised as the pituitary responds effectively to the stimulus of protein depletion. Plasma concentrations of Cortisol and other adrenocorticosteroids are normal or raised. Plasma thyroxine is often low, but free T4 is usually normal or raised.
d. Atrophy of the heart leads to a reduced cardiac output and a poor circulation. In many severe cases the extremities are cold and cyanosed and the pulse small or impalpable. The electrocardiogram (ECG) shows low-voltage changes in the QRS complex and the T-wave may be depressed or inverted. Some of the changes are rapidly reversed by potassium therapy.
e. There is no specific structural or functional abnormality of kidneys. The glomerular filtration rate may be low, but this is probably due to dehydration or reduced cardiac output. The concentrating power of the kidneys is often poor, but this may be due to the depression of tubular function by electrolyte deficiencies. They are all reversible by treatment.
f. The immune responses of the body are produced by cells arising in the thymus, lymph nodes and spleen, the lympho-reticular organs. These are very immature at birth and develop rapidly in the first two years of life. In kwashiorkor, the thymus, tonsils, spleen, and other lymphoid tissues are atrophied.
These changes are accompanied by a delayed or absent tuberculin response and other skin hypersensitivity reactions; reduced complement activity in the serum, especially the C3 component; reduced numbers of thymus- dependent lymphocytes (T cells) in the blood; reduced lymphocytes transformation in response to phytohemagglutinin. The bactericidal action of neutrophil leucocytes is also impaired.
These signs of reduced cell-mediated immunity are in contrast to a usually unimpaired humoral immunity; the response to injected antigens is often normal and plasma IgG may be higher than normal. The depression of cell-mediated immunity is mainly due to protein deficiency. Once the immunological system has reached maturity it is much less susceptible to malnutrition.
5. Treatment for Kwashiorkor:
a. Children who are seriously ill require treatment in hospital as their recovery depends on high standards of clinical skill and nursing care with some laboratory support.
When the acute phase of the illness is over, they need several weeks of special feeding and some medical supervision before recovery is complete.
b. Most children admitted to hospital have had repeated attacks of diarrhoea and often of vomiting and as a result are dehydrated. Some of these respond to oral rehydration therapy but if the response is not prompt or the dehydration is severe, intravenous fluid should be given at once.
c. A small infusion of plasma is beneficial when there is severe peripheral circulatory failure and of whole blood or red cells when there is severe anemia. Under these conditions the myocardium may be damaged by hypoxia and there may be acute heart failure, infusion should be low and a fast diuretic (frusemide) given at the start.
d. Many patients have malaria, pneumonia, dysentery and other infections and infections do not cause fever in the malnourished. It is better to give a short course of procaine benzyl-penicillin and ampicillin. If ampicillin is not available, chloramphenicol and tetracycline may also be given.
e. Hypothermia is often present and needs urgent treatment. It is, therefore, wise to let their mothers sleep with them in the hospital.
f. The child should be given a dilute milk feed with added sugar from the first or second day. Thus the strength can be increased and a vegetable oil added to give extra energy.
g. Infants who are seriously ill will improve when given 1 gm. protein per kg body weight daily. Good recovery can be obtained with 2 gm. and recovery is not accelerated by giving more than 3.5 gm.
h. A high energy diet is required and this can only be made by including large amounts of fat. Some infants can tolerate at first the amount of fat in whole cow milk. Feeds, therefore, be made up from skimmed milk powder.
i. As soon as children are able to take normal food and infection or other complications are controlled, they should be discharged to a centre where their nutritional rehabilitation can be supervised.
6. Prognosis of Kwashiorkor:
a. A child may suffer for a short period from one of the forms of PEM and make a complete recovery. If growth is retarded slightly, the child may reach the normal size for its age quickly, provided the dietary supply is satisfactory. If growth is retarded for a long period, the child may be stunted and develop into a small size, but healthy adult.
b. If the disease is so severe as to demand treatment in hospital, the prognosis is uncertain and often bad.
c. Children who survive severe PEM in early childhood perform less well in intelligence tests than controls. A poor performance can be due to their growing up in an unfavorable psychological environment rather than to a short period of malnutrition in early life.
d. It is proper to request government that failure to provide adequate nutritional services for mothers and young children may well lead to a school population with a diminished capacity for learning. On the other hand, a mother whose child has suffered a period of severe malnutrition and made a good recovery may be reassured that subsequent mental development is not likely to be impaired seriously, if at all.