Deficiency Disorders Dr.K.V BEENA DISTRICT PROGRAMME MANAGER NRHM, Ernakulam
Diseases caused by the deficiency of • Protein • Fat • Carbohydrates • Minerals • Vitamins
OVERVIEW OF PEM Protein calorie malnutrition is one of the most important public health problems in many developing countries including India, South East Asia and Africa.
OVERVIEW OF PEM It is a wide spread deficiency disease among children in low socio economic group 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) Those who survive may suffer from impaired growth and perhaps mental retardation
PRECIPITATING FACTORS • LACK OF FOOD (famine, poverty) • Inadequate breast feeding • Wrong concepts about nutrition • Diarrhoea & malabsorption • INFECTIONS (worms, measles, T.B)
CLASSIFICATION • A. CLINICAL ( WELLCOME ) • Parameter: weight for age + oedema • Reference standard (50th percentile) • Grades: • 80-60 % without oedema is under weight • 80-60% with oedema is Kwashiorkor • < 60 % with oedema is Marasmus-Kwash • < 60 % without oedema is Marasmus
KWASHIORKOR • Cecilly Williams, a paediatrician ,working in west africa had introduced the word Kwashiorkor to the medical literature in 1933. The word is described as displaced child meaning the sickness which a child develops when the next baby is born and the older one is deprived of breast milk
Definitions of Malnutrition Kwashiorkor: protein deficiency Marasmus: energy deficiency Marasmic/ Kwashiorkor: combination of chronic energy deficiency and chronic or acute protein deficiency
The effect of protein deficiency in adults may show itself in the form of • loss of weight, • reduced subcutaneous fat • Anaemia • Great susceptibility to infection • Frequent loose stools • General lethargy • Inability to do sustained hard work • Delay in healing of wounds and oedema
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 also operative.
CONSTANT FEATURES OF KWASHIORKAR • Oedema – starts with feet and lower legs • Psychomotor changes – no interest in suroundings, cheerless, no appetite • Growth retardation – body wt deminished to 11-40% • Muscle wasting – unable to hold the head when gently pulled from a lying to the sitting position
USUALLY PRESENT SIGNS • Moon face – oedema leads to full well rounded face • Hair changes – red hair, puckability of hair • Skin depigmentation – dry rough, scaly skin • Loose stools – due to gastro intestinal disturbances
OCCASIONAL FEATURES Fatty liver - Assosiated vitamin deficiency – occurance of keratomalacia and angular stomatisis Susceptibilty to infection – more prone to infectious diseases
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.
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 • Severe wasting of muscle & s/c fats • Severe growth retardation • Dehydration occurs due to watery diarrhoea • No edema or hair changes • The abdomen may be shrunken or distendend with gas • Continued feeling of hunger • The eyes become large in size and give the appearnace of staring
CLINICAL ASSESSMENT Interrogation & physical exam including detailed dietary history. Anthropometric measurements Team approach with involvement of dieticians, social workers & community support groups.
Investigations for PEM Full blood counts Blood glucose profile Septic screening Stool & urine for parasites & germs Electrolytes, Ca, Ph & ALP, serum proteins CXR & Mantoux test Exclude HIV & malabsorption
Complications of P.E.M Hypoglycemia Hypothermia Hypokalemia Hyponatremia Heart failure Dehydration & shock Infections (bacterial, viral & thrush)
TREATMENT • Correction of water & electrolyte imbalance • Treat infection & worm infestations • Dietary support: 3-4 g protein & 200 Cal /kg body wt/day + vitamins & minerals • Prevention of hypothermia • Counsel parents & plan future care including immunization & diet supplements
KEY POINT FEEDING Continue breast feeding Add frequent small feeds Use liquid diet Give vitamin A & folic acid on admission With diarrhea use lactose-free or soya bean formula
Cereal pulse combination • Khichadi • Green gram payasam • Puffed rice • Idli • Dosa • Cereal, Pulse vegetable porridge • Ragi, dhal roti • Paushtic ladoo
PROGNOSIS Kwashiorkar & Marasmus- Kwashiorkar have greater risk of morbidity & mortality compared to Marasmus and under weight Early detection & adequate treatment are associated with good outcome Late ill-effects on IQ, behavior & cognitive functions are doubtful and not proven
Prevention and Control Nutrition education of the correct feeding practices Promotion of breast feeding Development of low cost yet nutritive weaning foods Control of infections Promotion of food hygiene and personnel hygiene
Prevention and Control Detection an treatment of hook worm and other parasitic infestation Provision of a mineral, multi vitamin supplementation
Definitions • Micronutrients: Those required in small quantity; i.e, mg/mcg • Trace elements: Those present in very small quantity in the body; i.e, <0.01% or 0.1 mg/g ash weight • MDD- Micronutrient deficiency disorders • MNM- Micronutrient Malnutrition • Hidden Hunger- Subclinical MNM
VITAMIN A DEFICIENCY • Eye signs were dryness, clouding, corneal clouding, and liquefaction and extrusion of lens • Increased deaths from infection; especially pneumonia, diarrhea, measles • Body barriers to infection damaged (i.e., skin, all mucous membranes, eye covering); immune function impaired • Fat soluble Vitamin deficiency in liver diseases • Associated with blindness and signs of “toad skin” & Mobidity and mortality • (Ophthalmologist Sommers noted that in populations with eye signs of VAD, the children had very high levels of mortality and morbidity) • Surface protection- Vit A, SIgA
Vitamin A deficiency Lack of intake of vitamin A - containing fruits and vegetables, milk and organ meat Beta carotene – GYOR Blindness leading cause- Preventable Night blindness (6 mo – 6 yrs)> 1% in community- VAD-Public Health problem Corneal impression cytology, S, retinol, S RBP
Eye diseases due to VAD (WHO) Night blindness (XN) Conjunctival xerosis (X1A) Bitot’s spots (X1B) Corneal xerosis (X2) Corneal ulceration/keratomalacia <1/3 of cornea(X3A) Corneal ulceration/keratomalacia >1/3 of cornea(X3B) Corneal scar (XS) Xerophthalmic fundus (XF)
Approaches- VAD Nutrition education, cultivation of vitamin A-rich fruits and vegetables (sweet potato, carrots, tomatoes, green leafy vegetables) GYOR- Rainbow Revolution Food fortification Prophylaxis: high-dose vitamin A capsule/ concentrate distribution to children under five years of age every six months, low doses to pregnant women and also to nursing mothers 0.5 L to < 6 mo, < 1yr. 1 L, >1 yr- 2 L 9 megadoses – integrated with immunisation.
Vitamin A Deficiency (VAD) • 2 doses on consecutive days and then after 1 mo, every 6 months • PEM- repeat monthly till PEM resolves • Aquasol A- Water Soluble & No Storage • Hypervitaminosis- Gulf syndrome • Hypervitaminosis A- Pseudotumor cerebri/ Benign intracranial hypertension • 1 ml/2 ml spoon Vs 1-2 ml syringe
Suggested recipes for Vitamin A deficiency • Boiled egg, Egg Custard • Coriander and mint chutney with chapathi • Carrot halwa, Carrot salad, Carrot kheer, Carrot Juice • Papaya • Orange juice, Tomato Juice, Mango Juice • Green leafy vegetable with dhal
Vitamin D Deficiency Rickets is the term signifying a failure (defective) in the mineralization of growing bone or osteoid tissue (ends of long bones) Osteomalacia- defective mineralisation in the adult
What are the various forms of Vitamin D Two forms of vit D are of practical importance D2 or calciferol –available as irradiated ergosterol & fish liver oil D3-available synthetically.naturally present in human skin in pro vitamin stage as 7 dehydrocholestrol.
Sources of Vit D Sunlight Breastmilk(4-100 IU/liters) Cows milk(5-40IU/liters) Egg yolk(3-10mcg) RDA of vit D 400 IU/day (1mcg = 40 IU)