A 6 year-old boy who became aggressive

BASIC CONCEPT

Please read the following text before attempting the question below.

The carbohydrates are a group of naturally occurring carbonyl compounds (aldehydes or ketones) that also contain several hydroxyl groups. The carbohydrates include single sugars (monosaccharides) and their polymers, the oligosaccharides and polysaccharides. Glucose is the most important carbohydrate in mammalian system because nearly all carbohydrate in food is converted to glucose for metabolism. Sugars have large numbers of stereoisomers because they contain several asymmetric carbon atoms. The physiologically important monosaccharides include glucose, the “blood sugar,” and ribose, an important constituent of nucleotides and nucleic acids. The important disaccharides include maltose (Glucosyl glucose), an intermediate in the digestion of starch; sucrose (Glucosyl fructose), important as a dietary constituent containing fructose; and lactose (Galactosyl glucose), in milk. Starch and glycogen are storage polymers of glucose in plants and animals, respectively. Starch is the major source of energy in the diet. Cellulose is the most abundant organic substance in nature. Almost half of the total biomass consists of cellulose. Some 40–50% of plant cell walls are formed by cellulose.

Complex carbohydrates contain other sugar derivatives such as amino sugars, uronic acids, and sialic acids. They include proteoglycans and glycosaminoglycans, which are associated with structural elements of the tissues, and glycoproteins, which are proteins containing oligosaccharide chains; they are found in many situations including the cell membrane.

 CLINICAL CASE – A 6 year-old boy who became aggressive  [14/01/2014]

A 6-year-old boy is seen by a pediatrician because his parents are concerned about his aggressive behaviour, hyperactivity, and a loss of language skills. He also has recently become increasingly unsteady on his feet and has experienced a recent seizure. Slight facial feature coarsening is noted. In which of the following processes is this child most likely to have a disorder?

A. Mobilization of glycogen

B. Gluconeogenesis

C. Salvage of purine bases

D. Degradation of glycosaminoglycans

E. Cholesterol metabolism

 NOTE: Please discuss the answer in the “LEAVE A REPLY” box below.

ANSWER

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4 Responses

  1. Answer is D – Degradation of glycoaminoglycans. The disorder of which leads to Sanfilippo syndrome.

  2. Chitra Dhume says:

    answer is D -Degradation of glycosoaminoglycans.

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