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- 1. PELLAGRA
- 2. Also known as Alpine scurvy or St. Ignatius itch Its a disease due to severe deficiency of Niacin ( Vitamin B3) and or Tryptophan. Earliest records were made almost 250 years ago. It was seen spread through Europe following introduction of maize as a staple crop. Through out the 1960s and 1970s, it was still considered a public health in many maize consuming African and Asian countries. In recent time, its only reported in health centers during times of drought and food shortage and in populations depending on food-acids refugee programs where protein is scarce and corn is the staple food.
- 3. Protein and the amino acids are important in metabolizing Niacin. Niacin in corn is in a bound form thats hard for our bodies to absorb. Niacin (Vitamin B3) Nicotinic acid and nicotinamide, biologically equivalent vitamins, are both referred to as niacin. Biosynthesis of this vitamin occurs in all organisms; the conversion ratio of tryptophan to nicotinic acid is 60:1, making it possible for large amounts of tryptophan to meet niacin needs
- 4. Cnt.. ~The body uses Niacin to convert food calories into energy. The Niacin you eat is absorbed through your small intestine into the body tissues where its converted into Nicotinamide Adenine Dinucleotide ( NAD) which helps transfer the potential energy in macronutrients into ATP. NAD is also involved in DNA repair and cellular communications. Iron, Riboflavin (Vitamin B2) and Pyridoxine ( Vitamin B6) are needed in the conversion of Tryptophan to Niacin.
- 5. ~ Sources of Niacin include: Beef liver Oily fish, e.g Salmon and Tuna Peanuts Beets Enriched Breads and Cereals Potatoes Poultry
- 6. Niacin requirements Requirements are expressed in terms of niacin equivalents (NE) One NE equals 1 mg of niacin or 60 mg of tryptophan. RDA for niacin is related to dietary energy intake; the recommended intake is 6.4 to 8 NE/1000 kcal, human milk provides about 8 NE/ 1000 kcal.
- 7. NIACIN DEFICIENCY. Classification. Primary Pellagra; Is caused by inadequate dietary intake of Niacin. Secondary Pellagra; It occurs when the body is unable to absorb the Niacin you consume in the diet. ETIOLOGY Primary Pellagra: Poverty. Poor Nutrition.
- 8. Secondary Pellagra; Malabsorptive states. Hartnup Disease. Fad Diets; Individuals consuming diets high in leucine and low in Tryptophan; excessive leucine alters the normal metabolism of tryptophan hence low levels of niacin. Medications; isoniazid,5- flurouracil, phenobarbital, chloramphenical
- 9. PATHOGENESIS. Pellagra can develop according to several mechanism; 1. Dietary lack of Niacin. 2. From the deficiency of Tryptophan which the body uses to make Niacin. 3. Excess Leucine which inhibits the formation of Niacin to Nicotinamide Mononucleotide. 4. Inflammation of the Jejunum can prevent nutrient absorption. 5. Hartnup Disease; This is an autosomal recessive disorder that comprises renal and intestinal transport of amino acids such as Tryptophan
- 10. 6. Therapeutic Drugs: Example Isoniazid, Decrease the available B6 ( Pyridoxine) by binding it making it inactive hence cannot be used in Niacin synthesis. 7.Carcinoid Tumor; Neuroendocrine tumors along the GI tract that us Tryptophan as the source of serotonin production, limiting the amount of Tryptophan for Niacin synthesis. ( Very Rare in Children)
- 11. RISK FACTORS Digestive disease Low dietary intake of Tryptophan ( needed to produce Niacin in the body) Natural calamities e.g Famine Poverty Iron deficiency anemia
- 12. CLINICAL FEATURES General Features include; 3Ds Dermatitis Diarrhea Dementia. Systemic Features; Dermatological: Rash that resembles sunburn then progress to rough, scaly and hyperpigmented plaques.
- 14. Gastrointestinal; Mouth sores and a red, swollen tongue. Diarrhea leading to dehydration. Abdominal pain. Nausea and vomiting. Indigestion.
- 15. Neurological; Mood Changes. Numbness and tingling in the hands and feet.
- 16. COMPLICATIONS Permanent Dermentia Neurological damage resulting in balance and coordination problems Muscle twitches and tremors
- 17. DIAGNOSIS. History and Physical Examination. Urine test > urinary excretion of N1 -methylnicotinamide is most helpful; normal 24 hour excretion is between 4 and 6 mg, values below 3 mg indicate deficiency. In pellagra these values are usually between 0.5 to 0.8 mg/ day. TREATMENT. The daily dose for treatment is about 10 times the recommended dietary intake. Parenteral therapy is considered when gastrointestinal absorption is deficient.
- 18. Cont Treatment of pellagra consists of oral supplementation of 100 to 200 mg of nicotinamide or nicotinic acid three times daily for 5 days. Niacinamide is generally used to treat deficiency states, because niacin can cause flushing, itching, burning, or tingling sensations, whereas niacinamide does not; however, niacinamide does not possess hypolipidemic or vasodilating properties as does niacin. When oral therapy is precluded because of diarrhea or lack of patient cooperation, 100 to 250 mg should be injected sc bid to tid. In encephalopathic states, 1000 mg po plus 100 to 250 mg IM is recommended.
- 19. PREVENTIVE MEASURES. A well balanced diet Enriched foods and dietary supplements where food choices are limited. *If a child has a chronic health condition that predispose them to Pellagra, long term prevention is required.*