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.
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.*