Peds Thyroid issues Flashcards

1
Q

In the endoplasmic reticulum, thyroglobulin molecules are produced, packaged in vesicles by the Golgi, and exocytosed into the lumen of the follicle and then Iodide (I − ) (from the diet) enters the follicle cell via basolateral :

A

Na + /I − cotransporters (the I‐trap)

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2
Q

In the follicular lumen, I − is oxidized to iodine by ______and substituted for H + on the benzene ring of tyrosine residues of thyroglobulin.

A

thyroid peroxidase

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3
Q

Binding of one iodine molecule will form monoiodotyrosine (MIT), and binding of two iodine moieties will form diiodotyrosine (DIT). This reaction is termed________. Thyroid peroxidase also catalyzes the binding of DIT to another DIT, forming T4. Some DIT will also bind to an MIT, forming T3. These products remain linked to the

A

organification

thyroglobulin (Tg).

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4
Q

The mature Tg, containing MIT, DIT, T4, and T3 (in order of greater to lesser abundance), is endocytosed back into the follicle cell and can be stored as______ until secreted

A

colloid

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5
Q

Proteolysis of the colloid is stimulated by____ and releases the constituent molecules. MIT and DIT reenters the synthetic pool, and T3 and T4 exit the basolateral membrane into the blood.

A

TSH

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6
Q

Most iodine in the diet is excreted into the urine without being taken up by the thyroid gland. Only about____% of ingested iodine is taken up by the thyroid. Of that, about ___ is organified; the other ¼ leaks out and winds up in the urine.

A

20

¾

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7
Q

Most of the _____ iodine is removed in the tissue and excreted in the urine. Only a very small percentage of the ingested iodine winds up in the stool (still in the organified state)

A

hormone‐associated

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8
Q

In the initial steady state, TBG is ____ saturated with T4.

As TBG concentration increases, causing more T4 to be bound, we see reducing of the_____ concentration. This stimulates___ secretion, which leads to an increase in the release of T4 from the thyroid

A

one‐third

free T4

TSH

*) T4 becomes redistributed between the bound and the free states, leading to a new steady state with the same free T4 concentration but an increased total T4.

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9
Q

Because T4 and T3 are bound >99% to TBG in plasma, the half‐life of T4 is ___ days and T3 is __ day. When making adjustments in LT4 therapy, it takes weeks to see a physiological effec

A
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10
Q

Congenital hypothyroidism

how common is it

gender preference?

A

Hypothyroidism present at birth is seen in 1/2000 to 1/4000 babies (Wisconsin 1/3350). It is most common in Hispanics and least common in African‐Americans. Girls are affected more often than boys (2:1).

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11
Q

The most common underlying reason of congenital hypothyroidism is__________seen in 75‐85% of cases. Most of these cases are sporadic (only 2% familial)

A

failure of the gland to form properly (absent, ectopic or dysplastic gland)

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12
Q

What causes Congenital hypothyroidism in 10% of the children?

A

About 10% of the cases are due to an enzyme defect in thyroid hormone synthesis (dyshormonogenesis) due to genetic defects (mostly autosomal recessive)

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13
Q

Worldwide, many cases are due to ______ but this is uncommon in developed countries (due to use of iodized salt).

A

iodine deficiency

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14
Q

About 5% of congenital hypothyroidism are cases are due to _______(lack of TSH secretion), mostly in association with other missing pituitary hormones.

A

central hypothyroidism

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15
Q

Is congenital hypothyroidism a lifelong disease?

A

sometimes, but sometimes not: about 10% of all newborns diagnosed, the hypothyroidism turns out to be transient (either due to late maturation of the thyroid axis or due to transient suppression by maternal antibodies).

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16
Q

WHy is it important to screen for hypothyroidism at birth in all babies?

A

bc its often asymptomatic and can cause permanent brain damage if left untreated

17
Q

What are symptoms of congentital hypothyroidism?

A

lethargy, slow movement, hoarse cry, feeding problems, constipation, macroglossia, umbilical hernia, large fontanels, hypotonia, dry skin, hypothermia, and prolonged jaundice will become evident,

18
Q

What is the newborn screening for hypothyroidism?

A

Blood is collected using a filter paper and tested for either TSH or both TSH and T4. “TSH alone” is more commonly used because of higher sensitivity and specificity but it is not a perfect test.

19
Q

Why are TSH levels increased in newborns ?

A

TSH increases because of the decrease in negative feedback inhibition by thyroid hormone

ALSO dt a neonatal TSH surge immediately after birth and screening too early may lead to false positives. I

20
Q

What does the TSH screen fail to detect in newborn screening?

A

Central Hypothyroidism

21
Q

What is the tx for congenital hypothyroidism?

A

Hypothyroidism is treated using levothyroxine (T4), which is the main form of thyroid hormone produced normally in the thyroid gland.

22
Q

W

A
23
Q

What is the goal for T4 and TSH levels during tx for hypothyroidism?

A

o keep the serum T4 concentration in the upper normal range and to keep serum TSH concentration in the normal range (optimal 0.5‐2 mU/L)

24
Q

What is the relationshop btwn age of diagnosisng hypothyroidism and IQ?

A

inverse relationship between IQ and the age at diagnosis with normal outcome likely in >90% of those treated within the first two weeks

*Since even brief interruption can result in irreversible brain damage, compliance is critical in the first 3 years of life

25
Q

Brain development is not affected by hypothyroidism developing after age

A

3 years.

26
Q

main features of hypothyroidism in older children include

A

short stature, lack of energy, cold intolerance, constipation, hair loss and dry skin.

27
Q

In Primary hypothyroidism, elevated TSH levels are a result of

A

loss of negative feedback by T4 and T3 on atnerior pituitary

28
Q

What are some common features we see of children with hypothryoidism thats left untreated?

A

short stature, severe mental retardation, coarse facial features, a large tongue