DEFICIENCY DISEASES (1)

PROTEIN DEFICIENCY DISEASES : 

PROTEIN DEFICIENCY DISEASES PRESENTED BY RANJANA TIWARI DIETICIAN

PROTEIN DEFICIENCY: 

PROTEIN DEFICIENCY A nutritional condition produced by a deficiency of proteins in the diet characterised by adaptive enzyme changes in the liver, increase in amino acid synthetases, and diminution of urea formation, thus conserving nitrogen and reducing its loss in the urine. Growth, immune response, repair and production of enzymes and hormones are all impaired in severe protein deficiency. Protein deficiency may also arise in the face of adequate protein intake if the protein is of poorquality (i.e., the content of one or more amino acids is inadequate and thus becomes the limiting in protein utilization).

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The majority of world’s children live in developing countries Lack of food & clean water, poor sanitation, infection & social unrest lead to LBW & PEM Malnutrition is implicated in >50% of deaths of <5 children (5 million/yr)

PROTEIN ENERGY MALNUTRITION: 

PROTEIN ENERGY MALNUTRITION Protein energy malnutrition (PEM) is a type of disease caused due to decreased intake of food and deficient protein in the food most commonly seen in children and to the debilitated and uncared. Protein energy malnutrition is a disease of the poor , undernourished and chronically ill patient characterized by the imbalance between the supply of nutrient and energy and the body’s demand for them to ensure growth, maintenance of health and function of the body.

PREVELENCE: 

PREVELENCE The World Bank estimates that India is ranked 2 nd in the world of the number of children suffering from malnutrition after Bangladesh (in 1998), where 47% of the children exhibit a degree of malnutrition. The prevalence of underweight children in India is among the highest in the world, and is nearly double that of Sub-Saharan Africa with dire consequences for mobility, mortality, productivity and economic growth. The UN estimates that 2.1 million Indian children die before reaching the age of 5 every year – four every minute - mostly from preventable illnesses such as diarrhea, typhoid, malaria, measles and pneumonia.

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India is one of the fastest growing countries in terms of population and economics, sitting at a population of 1,139.96 million (2009) and growing at 10-14% annually (from 2001–2007)India's economy is growing where its GDP growth is 9.0% from 2007 to 2008; since Independence in 1947, its economic status has been classified as a low-income country with majority of the population at or below the poverty line. Though most of the population is still living below the National Poverty Line, its economic growth indicates new opportunities and a movement towards increase in the prevalence of chronic diseases which is observed in at high rates in developed countries such as United States, Canada and Australia.

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According to the World Food Program and the M.S. Swaminathan Research Foundation (MSSRF), over the past decade there has been a decrease in stunting among children in rural India, but inadequate calorie intake and chronic energy deficiency levels remain steady. [5] Today child malnutrition is prevalent in 7 percent of children under the age of 5 in China and 28 percent in sub-Saharan African compared to a prevalence of 43 percent in India. [6] Undernutrition is found mostly in rural areas and is concentrated in a relatively small number of districts and villages with 10 percent of villages and districts accounting for 27-28 percent of all underweight children. [7]

PREVELENCE OF PEM IN WORLD: 

PREVELENCE OF PEM IN WORLD

ETIOLOGY: 

ETIOLOGY The most common cause of malnutrition is poverty. PEM is primarily due to: An inadequate intake of food both in quantity and quality Infections Diarrhoea Respiratory Infections Measles Intestinal worm infestation These infections increase requirements for calories, proteins and other nutrients, while decreasing their absorption and utilization.

CAUSES OF PEM: 

CAUSES OF PEM Poor environmental conditions Large family size Poor maternal health and nutritional status Failure of lactation Premature termination of breast feeding Delayed weaning Social and cultural feeding practices Low birth weight Mal absorption states Short bowel syndrome Small bowel bacterial overgrowth Celiac disease Immunodeficiency states Enzyme and transport defects

KWASHIORKOR: 

KWASHIORKOR Cecilly Williams, a British nurse, had introduced the word Kwashiorkor to the medical literature in 1933 . The word is taken from the Ga language in Ghana & used to describe the sickness of weaning . Kwashiorkor: The word kwashiorkor comes from the Ivory Coast. It means the deposed (no longer suckled) child. Kwashiorkor is a childhood disease due to protein deprivation.

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Early signs are vague: apathy (indifference), lethargy (drowsiness) and irritability. More advanced signs are poor growth, lack of stamina, loss of muscle mass, swelling, abnormal hair (sparse, thin, often streaky red or gray in dark-skinned children) and abnormal skin (darkening in irritated but not sun-exposed areas). Kwashiorkor disables the immune system so the child is susceptible to a host of infectious diseases. Kwashiorkor is responsible for much morbidity (illness) and mortality (death) among children worldwide. Also known as protein malnutrition. and protein-calorie malnutrition (PCM).

ETIOLOGY: 

ETIOLOGY Kwashiorkor can occur in infancy but its maximal incidence is in the 2nd yr of life following abrupt weaning. Kwashiorkor is not only dietary in origin. Infective, psycho-socical, and cultural factors are aso operative. Kwashiorkor is an example of lack of physiological adaptation to unbalanced deficiency where the body utilized proteins and conserve S/C fat.

ETIOLOGY (2): 

ETIOLOGY (2) One theory says Kwash is a result of liver insult with hypoproteinemia and oedema. Food toxins like aflatoxins have been suggested as precipitating factors.

CONSTANT FEATURES OF KWASH: 

CONSTANT FEATURES OF KWASH OEDEMA PSYCHOMOTOR CHANGES GROWTH RETARDATION MUSCLE WASTING

USUALLY PRESENT SIGNS: 

USUALLY PRESENT SIGNS MOON FACE HAIR CHANGES SKIN DEPIGMENTATION ANAEMIA

OCCASIONALLY PRESENT SIGNS: 

OCCASIONALLY PRESENT S IGNS HEPATOMEGALY FLAKY PAINT DERMAT ITI S CARDIOMYOPATHY & FAILURE D EHYDRATION (Diarrh. & Vomiting) SIGNS OF VITAMIN DEFICIENCIES SIGNS OF INFECTIONS

DD of Kwash Dermatitis: 

DD of Kwash Dermatitis Acrodermatitis Entropathica Scurvy Pellagra Dermatitis Herpitiformis

MARASMUS: 

MARASMUS The term marasmus is derived from the Greek marasmos, which means wasting. Marasmus involves inadequate intake of protein and calories and is characterized by emaciation. Marasmus represents the end result of starvation where both proteins and calories are deficient . A form of protein-energy malnutrition occurring chiefly among very young children in developing countries particularly under famine conditions, in which a mother's milk supply is greatly reduced .

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. Marasmus results from the inadequate intake of both protein and calories; persons with a similar type of protein-energy malnutrition, Marasmus is characterized by growth retardation (in weight more than in height) and progressive wasting of subcutaneous fat and muscle. Other symptoms may include diarrhea; dehydration; behavioral changes; dry, loose skin; and dry, brittle hair. Marasmus can be treated with a high-calorie, protein-rich diet. Severe, prolonged marasmus may result in permanent mental retardation and impaired growth

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Marasmus represents an adaptive response to starvation, whereas kwashiorkor represents a maladaptive response to starvation I n Marasmus t he body utilizes all fat stores before using muscles.

EPIDEMIOLOGY & ETIOLOGY: 

EPIDEMIOLOGY & ETIOLOGY Seen most commonly in the first year of life due to lack of breast feeding and the use of dilute animal milk . Poverty or famine and diarrhoea are the usual precipitating factors Ignorance & poor maternal nutrition are also contributory .

Clinical Features of Marasmus: 

Clinical Features of Marasmus Severe wasting of muscle & s/c fats Severe growth retardation Child looks older than his age No edema or h air changes Alert but miserable Hungry Diarrhoea & Dehydration

Complications of P.E.M: 

Complications of P.E.M Hypoglycemia Hypothermia Hypokalemia Hyponatremia Heart failure Dehydration & shock Infections (bacterial, viral & thrush)

COMPARISION OF KWASHIORKOR AND MARASMUS: 

COMPARISION OF KWASHIORKOR AND MARASMUS FEATURES MARASMUS KWASHIORKOR Muscle wasting Obvious Some time hidden by edema and fat Fat wasting Severe loss of subcutaneous fat Fat often retained but not firm Edema None Present in lower leg and usually in face and lower arms Weight and Height Very low Low but may be masked by edema Mental changes Sometime quite and apathetic Irritable ,moaning, apathatic CLINICAL FEATURES

CLINICAL FEATURES: 

CLINICAL FEATURES FEATURES MARASMUS KWASHIORKOR Appetite Usually good Poor Diarrhea often Often Skin changes Usually none Diffuse pigmentation, some times flaky paint dermatosis Hair changes Seldom Sparse, easily pulled out Hepatic enlargement None Sometimes due to accumulation of fat

PATHOLOGICAL FEATURES: 

PATHOLOGICAL FEATURES Serum albumin Normal or slightly decreased Low (less than 3g/100mlblood) Urinary urea per g creatinine Normal or decrease Low Hydroxy proline /creatinine ratio Low Low Plasma /amino acid ratio Normal Elevated Anemia Uncommon Common Liver biopsy Normal or atrophic Fatty changes

THANK YOU: 

THANK YOU