Cretinism - Symptom, Causes, Treatment of Cretinism

Cretinism is a condition caused by a deficiency of thyroid hormone at birth and during infancy, as a result of abnormal development of the thyroid gland About 1 in 4000 babies is affected. Actual cretinism is a form of hypothyroidism, where lack of thyroid gland activity, often due to an iodine deficiency, stunts growth. Cretinism causes very serious retardation of physical and mental development; if the condition is left untreated, growth is stunted and the physical stature attained is that of a dwarf. In addition, the skin is thick, flabby, and waxy in color, the nose is flattened, the abdomen protrudes, and there is a general slowness of movement and speech.

Cretinism (also called congenital hypothyroidism) may not obvious at birth because thyroid hormone frornt mother's blood can benefit the baby before and several months after birth. The three characteristic features of neurological endemic cretinism in its fully developed form are extremely severe mental deficiency together with squint, deaf mutism and motor spasticity with disorders of the arms and legs of a characteristic nature.Most newborn babies are routinely screened for thyroid deficiency, which can be detected by a blood test even if there are symptoms. If the condition is not detected at birth, symptoms will have developed within 6 months.

Symptoms of Cretinism

Symptoms include stunted growth, distorted facial features, enlarged tongue and mental retardation. The typical

• floppy infant
• thick, protruding tongue
• poor feeding
• choking episodes
• constipation
• prolonged jaundice
• short stature

Common Signs and tests:

• sutures - separated widely and a large posterior fontanelle
• large fontanelle and posterior fontanelle (soft spots)
• dull-appearing facial features
• dry, brittle hair and low hairline
• short, thick neck
• growth failure
• short extremities
• broad hands with short fingers
• myxedema
• hypotonia
• hoarse-sounding cry or voice

Types of Cretinism

Myxedematous Cretinism has a less severe degree of mental retardation than the neurological cretin. It has all the features of extremely severe hypothyroidism present since early life, as in non recognized sporadic congenital hypothyroidism, severe growth retardation, incomplete maturation of the features including the naso-orbital configuration, atrophy of the mandibles, puffy features, myxedematous, thickened and dry skin, dry and rare hair, eyelashes and eyebrows and much delayed sexual maturation. Other signs may include thickened skin and a protruding abdomen.

Endemic cretinism arises from a diet deficient in iodine and has affected far more people worldwide and continues to be a major public health problem in many countries. Iodine is an essential trace element, necessary primarily for the synthesis of thyroid hormones. Although it is found in many foods it is not universally present in all soils in adequate amounts. The soils of many inland areas on all continents are iodine deficient, and plants and animals grown there are correspondingly deficient. Populations living in those areas without outside food sources are most at risk for iodine deficiency diseases.

Diagnosis and Treatment of Cretinism

Early diagnosis, and life long treatment with thyroid hormone by mouth, give the child a good chance of developing normally. Newborns diagnosed and treated in the first month to month and a half generally develop normal intelligence. Absence of thyroid hormone during early life gives a poor outlook in terms of mental development. Replacement therapy with thyroxine is the standard approach to treatment of hypothyroidism. Once medication starts, the blood levels of T3 and T4 are monitored to keep the values within a normal range.

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