Mucopolysaccharide Health Dictionary

Mucopolysaccharide: From 1 Different Sources


n. one of a group of complex carbohydrates functioning mainly as structural components in connective tissue. Mucopolysaccharide molecules are usually built up of two repeating sugar units, one of which is an amino sugar. An example of a mucopolysaccharide is *chondroitin sulphate, occurring in cartilage.
Health Source: Oxford | Concise Colour Medical Dictionary
Author: Jonathan Law, Elizabeth Martin

Connective Tissue

Sometimes called ?brous tissue, this is one of the most abundant tissues in the body, holding together the body’s many di?erent structures. Connective tissue comprises a matrix of substances called mucopolysaccharides in which are embedded various specialist tissues and cells. These include elastic (yellow), collagenous (white) and reticular ?bres as well as macrophages (see MACROPHAGE) and MAST CELLS. Assembled in di?ering proportions, this provides structures with varying functions: bone, cartilage, tendons, ligaments and fatty and elastic tissues. Collagenous connective tissue binds the muscles together and provides the substance of skin. It is also laid down in wound repair, forming the scar tissue. Contracting with time, connective tissue becomes denser, causing the puckering that is typical in serious wounds or burns. (See ADHESION; SCAR; WOUNDS.)... connective tissue

Polysaccharide

A CARBOHYDRATE comprising several monosaccharides linked in long chains. Polysaccharides store energy – as starch in plants and glycogen in animals – and they also form the structural parts of plants (as cellulose) and animals (as mucopolysaccharides).... polysaccharide

Drug Binding

The process of attachment of a drug to a receptor or plasma protein, fat, mucopolysaccharide or other tissue component. This process may be reversible or irreversible.... drug binding

Goitre

SIMPLE GOITRE A benign enlargement of the THYROID GLAND with normal production of hormone. It is ENDEMIC in certain geographical areas where there is IODINE de?ciency. Thus, if iodine intake is de?cient, the production of thyroid hormone is threatened and the anterior PITUITARY GLAND secretes increased amounts of thyrotrophic hormone with consequent overgrowth of the thyroid gland. Simple goitres in non-endemic areas may occur at puberty, during pregnancy and at the menopause, which are times of increased demand for thyroid hormone. The only e?ective treament is thyroid replacement therapy to suppress the enhanced production of thyrotrophic hormone. The prevalence of endemic goitre can be, and has been, reduced by the iodinisation of domestic salt in many countries. NODULAR GOITRES do not respond as well as the di?use goitres to THYROXINE treatment. They are usually the result of alternating episodes of hyperplasia and involution which lead to permanent thyroid enlargement. The only e?ective way of curing a nodular goitre is to excise it, and THYROIDECTOMY should be recommended if the goitre is causing pressure symptoms or if there is a suspicion of malignancy. LYMPHADENOID GOITRES are due to the production of ANTIBODIES against antigens (see ANTIGEN) in the thyroid gland. They are an example of an autoimmune disease. They tend to occur in the third and fourth decade and the gland is much ?rmer than the softer gland of a simple goitre. Lymphadenoid goitres respond to treatment with thyroxine. TOXIC GOITRES may occur in thyrotoxicosis (see below), although much less frequently autonomous nodules of a nodular goitre may be responsible for the increased production of thyroxine and thus cause thyrotoxicosis. Thyrotoxicosis is also an autoimmune disease in which an antibody is produced that stimulates the thyroid to produce excessive amounts of hormone, making the patient thyrotoxic.

Rarely, an enlarged gland may be the result of cancer in the thyroid.

Treatment A symptomless goitre may gradually disappear or be so small as not to merit treatment. If the goitre is large or is causing the patient di?culty in swallowing or breathing, it may need surgical removal by partial or total thyroidectomy. If the patient is de?cient in iodine, ?sh and iodised salt should be included in the diet.

Hyperthyroidism is a common disorder affecting 2–5 per cent of all females at some time in their lives. The most common cause – around 75 per cent of cases – is thyrotoxicosis (see below). An ADENOMA (or multiple adenomas) or nodules in the thyroid also cause hyperthyroidism. There are several other rare causes, including in?ammation caused by a virus, autoimune reactions and cancer. The symptoms of hyperthyroidism affect many of the body’s systems as a consequence of the much-increased metabolic rate.

Thyrotoxicosis is a syndrome consisting of di?use goitre (enlarged thyroid gland), over-activity of the gland and EXOPHTHALMOS (protruding eyes). Patients lose weight and develop an increased appetite, heat intolerance and sweating. They are anxious, irritable, hyperactive, suffer from TACHYCARDIA, breathlessness and muscle weakness and are sometimes depressed. The hyperthyroidism is due to the production of ANTIBODIES to the TSH receptor (see THYROTROPHIN-STIMULATING HORMONE (TSH)) which stimulate the receptor with resultant production of excess thyroid hormones. The goitre is due to antibodies that stimulate the growth of the thyroid gland. The exoph-

thalmos is due to another immunoglobulin called the ophthalmopathic immunoglobulin, which is an antibody to a retro-orbital antigen on the surface of the retro-orbital EYE muscles. This provokes in?ammation in the retro-orbital tissues which is associated with the accumulation of water and mucopolysaccharide which ?lls the orbit and causes the eye to protrude forwards.

Although thyrotoxicosis may affect any age-group, the peak incidence is in the third decade. Females are affected ten times as often as males; the prevalence in females is one in 500. As with many other autoimmune diseases, there is an increased prevalence of autoimmune thyroid disease in the relatives of patients with thyrotoxicosis. Some of these patients may have hypothyroidism (see below) and others, thyrotoxicosis. Patients with thyrotoxicosis may present with a goitre or with the eye signs or, most commonly, with the symptoms of excess thyroid hormone production. Thyroid hormone controls the metabolic rate of the body so that the symptoms of hyperthyroidism are those of excess metabolism.

The diagnosis of thyrotoxicosis is con?rmed by the measurement of the circulating levels of the two thyroid hormones, thyroxine and TRIIODOTHYRONINE.

Treatment There are several e?ective treatments for thyrotoxicosis. ANTITHYROID DRUGS These drugs inhibit the iodination of tyrosine and hence the formation of the thyroid hormones. The most commonly used drugs are carbimazole and propylthiouricil: these will control the excess production of thyroid hormones in virtually all cases. Once the patient’s thyroid is functioning normally, the dose can be reduced to a maintenance level and is usually continued for two years. The disadvantage of antithyroid drugs is that after two years’ treatment nearly half the patients will relapse and will then require more de?nitive therapy. PARTIAL THYROIDECTOMY Removal of three-quarters of the thyroid gland is e?ective treatment of thyrotoxicosis. It is the treatment of choice in those patients with large goitres. The patient must however be treated with medication so that they are euthyroid (have a normally functioning thyroid) before surgery is undertaken, or thyroid crisis and cardiac arrhythmias may complicate the operation. RADIOACTIVE IODINE THERAPY This has been in use for many years, and is an e?ective means of controlling hyperthyroidism. One of the disadvantages of radioactive iodine is that the incidence of hypothyroidism is much greater than with other forms of treatment. However, the management of hypothyroidism is simple and requires thyroxine tablets and regular monitoring for hypothyroidism. There is no evidence of any increased incidence of cancer of the thyroid or LEUKAEMIA following radio-iodine therapy. It has been the pattern in Britain to reserve radio-iodine treatment to those over the age of 35, or those whose prognosis is unlikely to be more than 30 years as a result of cardiac or respiratory disease. Radioactive iodine treatment should not be given to a seriously thyrotoxic patient. BETA-ADRENOCEPTOR-BLOCKING DRUGS Usually PROPRANOLOL HYDROCHLORIDE: useful for symptomatic treatment during the ?rst 4–8 weeks until the longer-term drugs have reduced thyroid activity.

Hypothyroidism A condition resulting from underactivity of the thyroid gland. One form, in which the skin and subcutaneous tissues thicken and result in a coarse appearance, is called myxoedema. The thyroid gland secretes two hormones – thyroxine and triiodothyronine – and these hormones are responsible for the metabolic activity of the body. Hypothyroidism may result from developmental abnormalities of the gland, or from a de?ciency of the enzymes necessary for the synthesis of the hormones. It may be a feature of endemic goitre and retarded development, but the most common cause of hypothyroidism is the autoimmune destruction of the thyroid known as chronic thyroiditis. It may also occur as a result of radio-iodine treatment of thyroid overactivity (see above) and is occasionally secondary to pituitary disease in which inadequate TSH production occurs. It is a common disorder, occurring in 14 per 1,000 females and one per 1,000 males. Most patients present between the age of 30 and 60 years.

Symptoms As thyroid hormones are responsible for the metabolic rate of the body, hypothyroidism usually presents with a general sluggishness: this affects both physical and mental activities. The intellectual functions become slow, the speech deliberate and the formation of ideas and the answers to questions take longer than in healthy people. Physical energy is reduced and patients frequently complain of lethargy and generalised muscle aches and pains. Patients become intolerant of the cold and the skin becomes dry and swollen. The LARYNX also becomes swollen and gives rise to a hoarseness of the voice. Most patients gain weight and develop constipation. The skin becomes dry and yellow due to the presence of increased carotene. Hair becomes thinned and brittle and even baldness may develop. Swelling of the soft tissues may give rise to a CARPAL TUNNEL SYNDROME and middle-ear deafness. The diagnosis is con?rmed by measuring the levels of thyroid hormones in the blood, which are low, and of the pituitary TSH which is raised in primary hypothyroidism.

Treatment consists of the administration of thyroxine. Although tri-iodothyronine is the metabolically active hormone, thyroxine is converted to tri-iodothyronine by the tissues of the body. Treatment should be started cautiously and slowly increased to 0·2 mg daily – the equivalent of the maximum output of the thyroid gland. If too large a dose is given initially, palpitations and tachycardia are likely to result; in the elderly, heart failure may be precipitated.

Congenital hypothyroidism Babies may be born hypothyroid as a result of having little or no functioning thyroid-gland tissue. In the developed world the condition is diagnosed by screening, all newborn babies having a blood test to analyse TSH levels. Those found positive have a repeat test and, if the diagnosis is con?rmed, start on thyroid replacement therapy within a few weeks of birth. As a result most of the ill-effects of cretinism can be avoided and the children lead normal lives.

Thyroiditis In?ammation of the thyroid gland. The acute form is usually caused by a bacterial infection elsewhere in the body: treatment with antibiotics is needed. Occasionally a virus may be the infectious agent. Hashimoto’s thyroiditis is an autoimmune disorder causing hypothyroidism (reduced activity of the gland). Subacute thyroiditis is in?ammation of unknown cause in which the gland becomes painful and the patient suffers fever, weight loss and malaise. It sometimes lasts for several months but is usually self-limiting.

Thyrotoxic adenoma A variety of thyrotoxicosis (see hyperthyroidism above) in which one of the nodules of a multinodular goitre becomes autonomous and secretes excess thyroid hormone. The symptoms that result are similar to those of thyrotoxicosis, but there are minor di?erences.

Treatment The ?rst line of treatment is to render the patient euthyroid by treatment with antithyroid drugs. Then the nodule should be removed surgically or destroyed using radioactive iodine.

Thyrotoxicosis A disorder of the thyroid gland in which excessive amounts of thyroid hormones are secreted into the bloodstream. Resultant symptoms are tachycardia, tremor, anxiety, sweating, increased appetite, weight loss and dislike of heat. (See hyperthyroidism above.)... goitre

Hurler’s Syndrome

A rare, inherited condition caused by an enzyme defect. The syndrome is a type of inborn error of metabolism (see metabolism, inborn errors of) in which there is an abnormal accumulation of substances known as mucopolysaccharides in the tissues.

Affected children may appear normal at birth but, at 6–12 months of age, they develop cardiac abnormalities, umbilical hernia, skeletal deformities, and enlargement of the tongue, liver, and spleen.

Growth is limited and mental development slows.

If the condition is diagnosed in early infancy, a bone marrow transplant may be curative.... hurler’s syndrome

Mucopolysaccharidosis

A group of rare inherited metabolic disorders (see metabolism, inborn errors of) of which Hurler’s syndrome is the best known. All mucopolysaccharidoses are genetic disorders in which there is an abnormality of a specific enzyme. This leads to the accumulation within body cells of substances called mucopolysaccharides.

Features may include abnormalities of the skeleton and/or the central nervous system, with learning difficulties and, in some cases, a characteristic facial appearance. There may also be clouding of the cornea, liver enlargement, and joint stiffness. No specific treatment is available. However, a bone marrow transplant may successfully be used to treat Hurler’s syndrome.

Mild forms of mucopolysaccharidoses allow a child to have a relatively normal life.

More severe types usually cause death during childhood or adolescence.... mucopolysaccharidosis

Basement Membrane

the thin delicate membrane that lies at the base of an *epithelium. It is composed of mucopolysaccharide and fibres of protein.... basement membrane

Chondroitin Sulphate

a mucopolysaccharide that forms an important constituent of cartilage, bone, and other connective tissues. It is composed of glucuronic acid and N-acetyl-D-galactosamine units. Although it is sold as a supplement to help with joint pain, evidence of any benefit is limited.... chondroitin sulphate

Glucosamine

n. the amino sugar of glucose, i.e. glucose in which the hydroxyl group is replaced by an amino group. Glucosamine is a component of *mucopolysaccharides and *glycoproteins: for example, *hyaluronic acid, a mucopolysaccharide found in synovial fluid, and *heparin. Glucosamine is taken as a supplement to help manage osteoarthritis.... glucosamine

Hyaluronic Acid

an acid *mucopolysaccharide that acts as the binding and protective agent of the ground substance of connective tissue. It is also present in the synovial fluid around joints and in the vitreous and aqueous humours of the eye.... hyaluronic acid

Morquio–brailsford Disease

a defect of *mucopolysaccharide metabolism (see inborn error of metabolism) that causes dwarfism with a *kyphosis, a short neck, *knock-knee, and an angulated sternum in affected children. Intelligence is normal. [L. Morquio (1865–1935), Uruguayan physician; J. F. Brailsford (1888–1961), British radiologist]... morquio–brailsford disease

Periodic Acid–schiff Reaction

(PAS reaction) a test for the presence of glycoproteins, polysaccharides, certain mucopolysaccharides, glycolipids, and certain fatty acids in tissue sections. The tissue is treated with periodic acid, followed by *Schiff’s reagent. A positive reaction is the development of a red or magenta coloration.... periodic acid–schiff reaction



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