What is Dyshormonogenetic goiter?

What is Dyshormonogenetic goiter?

Dyshormonogenetic goiters (DG) are genetically determined thyroid hyperplasias due to enzyme defects in thyroid-hormone synthesis. We report 56 cases of DG occurring in 34 females and 22 males. The patients age ranged from newborn to 52 yr (median 16), 75% of the cases occurring before the age of 24.

What is neonatal goiter?

Congenital goiter is enlargement of the thyroid gland that is present at birth. The enlarged thyroid gland may produce too much thyroid hormone (hyperthyroidism. Hyperthyroidism in the newborn is usually caused by Graves disease in the mother.

Is a goiter a nodule?

A goiter may be an overall enlargement of the thyroid, or it may be the result of irregular cell growth that forms one or more lumps (nodules) in the thyroid. A goiter may be associated with no change in thyroid function or with an increase or decrease in thyroid hormones.

What causes congenital hypothyroidism in newborns?

The most common cause worldwide is a shortage of iodine in the diet of the mother and the affected infant. Iodine is essential for the production of thyroid hormones. Genetic causes account for about 15 to 20 percent of cases of congenital hypothyroidism.

Which are the two types of goitre?

There are 2 main types of goitre:

  • diffuse goitre – where the entire thyroid gland swells and feels smooth to the touch.
  • nodular goitre – where solid or fluid-filled lumps called nodules develop within the thyroid and make the thyroid gland feel lumpy to touch; the nodules can be single or multiple and may contain fluid.

Can a baby be born with a goiter?

Kids can be born with a goiter or develop one later in life. A goiter that’s present at birth is called a congenital goiter. These can be caused by: a genetic disease that prevents the baby from producing thyroid hormone properly.

What is Myxedematous cretinism?

Myxedematous cretinism is considered to result from iodine deficiency and hypothyroidism in the fetus during late pregnancy or in the neonatal period, resulting in mental retardation, short stature, goiter, and hypothyroidism.

Is cretinism Hyposecretion or hypersecretion?

Anterior pituitary gland – 7 hormones and bone cells. [Hyper secretion of it during childhood causes Gigantism, hypersecretion during adulthood causes Acromegaly, and hyposecretion causes Dwarfism]. hyposecretion causes cretinism in children and myxedema in adults].

What is the difference between goiter and thyroid nodules?

What Are Goiters and Thyroid Nodules? An enlarged thyroid gland can be felt as a lump under the skin at the front of the neck. When it is large enough to see easily, it’s called a goiter. A thyroid nodule is a lump or enlarged area in the thyroid gland.

What are dyshormonogenetic goiters?

Dyshormonogenetic goiters (DG) are genetically determined thyroid hyperplasias due to enzyme defects in thyroid-hormone synthesis. We report 56 cases of DG occurring in 34 females and 22 males. The patients age ranged from newborn to 52 yr (median 16), 75% of the cases occurring before the age of 24 …

What is the pathophysiology of goiter?

Dyshormonogenetic goiter is a benign thyroid hyperplasia due to hereditary defects in thyroid hormone synthesis. It lacks malignant features common for papillary (Orphan Annie nuclei, psammoma bodies), follicular (capsular invasion) or high grade (necrosis) thyroid cancer.

What are the diagnostic criteria for goiter?

Dyshormonogenetic goiter should be suspected in a child or young adult with diffuse follicular hyperplasia, nodularity, scant colloid and nuclear atypia Only strict criteria for diagnosing malignancy should be applied, e.g. true capsular or vascular invasion in case of follicular carcinoma

What are the histologic hallmarks of goiter?

Histologic hallmarks are markedly hypercellular nodules with papillary hyperplasia, absence of colloid, frequent internodular bizarre cells and bridging fibrosis Also known as inherited congenital / familial goiter and congenital / sporadic goitrous cretinism (more vague terms)

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