diet4life
fssai

Prevalence

Worldwide, the incidence of IEM is more than 1/1000. In India, diagnosis or new born screening is limited leading to many cases going undiagnosed. According to WHO figures, 5 million children die in the first month of life in developing countries and 4 million children are born with some congenital anomaly. Thousands of children die of no definable reason. Almost 27 - 30 % of these babies are now proved to be having some IEM disease. About 5 to 15 % of all sick neonates in NICU are expected to have some Inborn Error of Metabolism

Prevalence

IEM Conditions

S. No
Medical Condition
Description
1
Maple syrup urine disease
Isoleucine, Leucine and Valine free diet
2
Homocystinuria
Lysine free and Tryptophan Low/free diet powder
3
Homocystinuria
Methionine free diet
4
Isovaleric Acidemia, 3-MethylcrotonyI-CoA Carboxylase Deficiency, 3-MethylglutaconyI-CoA Hydratase Deficiency
Leucine free diet
5
Methylmalonic Acidemias, Propionic Acidemia
Isoleucine, Methionine, Threonine and Valine free diet
6
Amino acid metabolic disorders
Protein and amino acid free diet (with and without fat)
7
Phenylketonuria (PKU)
Phenylalanine free diet
8
Urea Cycle Disorders, Argininemia, Argininosuccinic Aciduria, Carbamoylphosphate Synthetase Deficiency1, Citrullinemia
Non-essential amino acid free diet
S. No
Medical Condition
Description
9
Tyrosinemia
Phenylalanine and Tyrosine free diet
10
Galactosemia
Galactose free formula
11
3-Hydroxy Long Chain Acyl-CoA Dehydrogenase Deficiency
LCHAD Deficiency
12
Defects in the intraluminal hydrolysis of fat; defective mucosal fat absorption; defective lymphatic transport of fat
Milk protein-based diet with medium-chain triglycerides (MCT) for children and adults
13
Disaccharidase deficiencies; Disorders of carbohydrate metabolism Sucrase/ isomaltase deficiency, Fructose intolerance
Protein hydrolysate formula base diet with iron for use with added carbohydrate.
14
Non-ketotic Hyperglycinemia, Lysinuric protein intolerance
Protein free formula
15
Glucose transport defect(Glut1 def), Pyruvate dehydrogenase complex deficiency
Low carbohydrate, sucrose, fructose, sugar free formula

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