Transcript Thyroid Gland Diseases

Thyroid Gland Diseases
in Children
Riga Olena
KhNMU
Excretion of iodine ( in urine)
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100-200 μg/l – normal level
201-299 μg/l – increase level
> 300 μg/l - increase of intake in food
Deficiency:
< 20 μg/l – severe
20-49 μg/l - moderate
50-99 μg/l - mild
The steps of thyroid hormone synthesis
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Monoiodotyrosin (MIT)
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Diiodotyrosin (DIT)
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1 molecula MIT + 2 DIT → thyroxin T4
 1 molecula MIT + 1 DIT →
triiodothyroxinT3
Under thyroperoxidase control
Peripheral conversation T3
from T4
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Both T4 and T3 circulate in plasma
bound to the plasma thyroid hormonebinding protein (TBP) Thyroxine-binding
globulin.
Thyroid produces only 8 μg of T4 and
4 μg T3 daily.
Serum T3 concentration is usually low
because of reduced conversation from
T4.
The factors that destroyed of
conversation T4→T3
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systemic disease
starvation, anorexia
surgical intervention
newborn period
gerontological period
glucocorticosteroids
β-adrenoblocks
amiodarone (cordaron)
propylthyouracil
Action of thyroid hormones
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Genomic effects: the interaction of
TH and its receptors is believed to
precede other cellular events of
messenger RNA and specific protein
synthesis
Action of thyroid hormones
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Maturation of the CNS: lack of TH in
the first year or two results in
decreased brain cell size and number.
Myelinization of axons is retarded
leading to abnormalities and dendritic
arborization
Action of thyroid hormones
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Maturation of the skeletal and
dental system
Maintenance of oxidative
metabolism and heart production
Control of temperature production
TH differentiates all tissues and
organs
Diagnostic of Thyroid
gland disease
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Visual & palpating method
Investigation of thyroid function (basal
level of freeT3 ,freeT4)
Functional tests (TSH)
USG, radiography, scanning, etc.
Biopsia
Diagnostic of Thyroid
gland disease
Serological tests:
*Markers of autoimmune disease
(antibodies to thyroglobulin,
thyroperoxidase, to TSH-receptors)
*Markers of cancer (thyroglobulin,
calcitonin)
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Goiter WHO (1994)
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0 – goiter is absent
I – goiter isn’t visualized, but it’s size
less than distal phalanx of thumb
II – goiter is palpated & visualized
Functional condition of
Thyroid influence may be as
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Euthyroidism
Hypothyroidism
hyperthyroidism
Нypothyroidism
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Hypothyroidism - syndrome with
particular or total deficiency T3 and T4
or theirs acts to target cells
Classification of
hypothyroidism
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Disturbances
PRIMARY - defects of biosynthesis of
T3, T4 due to pathology of thyroid
gland
SECONDARY - decreasing T3, T4 due
to deficiency of TSH (pituitary) or TRH
(hypothalamus) or Resistance of
receptors for T3, T4
Classification of
hypothyroidism
Onset
Congenital
Acquired (rare)
Classification of
hypothyroidism
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Clinic & biologic data
Latent (subclinical) T3 -N, T4 –N,
TSH > 10 mU/l
Manifestation of disease due to ↓ T4 (at
first) & ↓ T3
Complicate
ETIOLOGY OF CONGENITAL
HYPOTHYROIDISM
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Primary hypothyroidism Thyroid
dysgenesis (aplasia, hypoplasia, or ectopic
gland)
Inborn error of thyroid hormone synthesis,
secretion, or utilization
Maternal goitrogen ingestion or radioactive
iodine treatment
Iodine deficiency (endemic goiter)
Autoimmune thyroiditis
ETIOLOGY OF CONGENITAL
HYPOTHYROIDISM (c’d)
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Hypothalamic or pituitary
hypothyroidism
Pituitary aplasia
Septo-optic dysplasia
PIT1 mutation (deficiency TSH, GH, Prol
PROP-1 mutation (deficiency TSH, GH,
Prol,Lh,FSH,ACTH)
Thyrotropin unresponsiveness
SYMPTOMS OF CONGENITAL
HYPOTHYROIDISM
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There is a tendency towards prolonged
gestation with 1/3 of pregnancies
lasting 42 weeks or more
Prolonged jaundice
Lethargy
Constipation
Feeding problems
Cold to touch
SIGNS OF CONGENITAL
HYPOTHYROIDISM
Skin mottling and Dry skin
Umbilical hernia and Distended abdomen
Jaundice
Macroglossia
Large fontanels
Wide sutures
Hoarse cry
Muscle Hypotonia
Slow reflexes
Minority of CH
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Puffy myxedematous face
Depressed nasal bridge with hypertelorism
Large protruding tongue with an open mouth
Cold, motted skin
Short neck
Palpebral fissures are narrow
Short fingers
Fat deposits between neck and shoulders
Hiar is coarse, brittle and scanty
Hiarline reaches far down on the forehead
DIAGNOSTIC STUDIES IN
HYPOTHYROIDISM
Thyroid scan – 99mTc or 123 IT3 resin
uptake
Bone age
TSH !!!
Free T4 – if hypothalamic- pituitary
hypothyroidism suspected
TBG – if TBG deficiency suspected
Anti-thyroid antibodies – if history of
maternal thyroiditis
Biochemical hallmarks of CH
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Low serum T4 and T3 with evaluated
TSH (primary)
T3 –normal, T4 ↓- severe or
longstanding
T4 –normal but TSH is elevated –
compensative CH, transient or
subclinical
T4↓ but TSH normal- congenital TBGdeficiency or hypothalamic-pituitary
hypothyrodism
Biochemical hallmarks of CH
Other:
 Elevated serum cholesterol
 Elevated creatinphosphokinase
 Hyponatriemia
Instrumental data
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Slightly decrease heart rate and
amplitude of R wave (ECG)
Increase projection period, left
ventricular wall diameter, decrease LV
chamber size and decrease cardiac
output (EchoCG)
Low-amplitude diffuse slowing (EEG)
Treatment L-thyroxin
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Preterm
0-3 mo
3-6 mo
6-12 mo
1-3 years
3-10 years
10-15 years
> 15 years
8 – 10 μg/kg
10 – 15 μg/kg
8 – 10 μg/kg
6 – 8 μg/kg
4 – 6 μg/kg
3 – 4 μg/kg
2 – 4 μg/kg
2 – 3 μg/kg
ETIOLOGY OF ACQUIRED
HYPOTHYROIDISM
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Chronic lymphocytic (Hashimoto`s)
thyroiditis (CLT)
Subacute thyroiditis (De Quervain`s)
Goitrogens (iodide, thiouracil, etc.)
Thyroidectomy or ablation following
radioactive iodine
Infiltrative disease (e.g., cystinosis,
histiocytosis X)-systemic disease
ETIOLOGY OF ACQUIRED
HYPOTHYROIDISM (c’d)
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Hypothalamic or pituitary disease
Congenital thyroid disorders, e.g., ectopia,
may not decompensate until later childhood
and thus may appear acquired
Peripheral resistance to thyroid hormones,
including receptor defects
Jatrogenic (propylthiouracil, methimazole,
iodides, lithium,amiodarone)
Hemangiomas of the liver
SYMPTOMS OF ACQUIRED
HYPOTHYROIDISM
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Slow growth
Puffiness
Decreased appetite
Constipation
Swollen thyroid gland
Lethargy
Drop in school performance
Cold intolerance
Galactorrhea
Menometrorrhagia
SIGNS OF ACQUIRED
HYPOTHYROIDISM
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Short stature
Decreased growth velocity
Increased upper to lower segment ratio
Delayed dentition
Myxedema or mildly overweight
Goiter
SIGNS OF ACQUIRED
HYPOTHYROIDISM (c’d)
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Delayed reflex return
Dull, placid expression
Pale, thick, carotenemic, or cool skin
Muscle pseudohypertrophy
Delayed puberty or precocious puberty
Treatment –same CH
Chronic thyroiditis Hashimoto
disease
Clinical presentation:
 goiter with euthyroidism
 Thoxic thyroiditis
 Hypothyroidism with or without
thyromegaly
 Dysphagia, pain or pressure sensation
in the neck, cough and headache have
been reported
Diagnosis Hashimoto disease
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T4 total and free, serum TSH
Biopsy
Antibodies test: antithyroglobulin
antibodies to thyroperoxidase
antimicrosomal test
Causes of thyrotoxicosis
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Congenital: transient, neonatal Graves’
disease
Acquired: Graves’ disease
Functional adenoma
Thyroid cancer
TSH-secreting pituitary tumor
Jatrogenic
(Graves disease)
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Diffuse toxity goiter - autoimmune
pathology with prolonged elevation
T3 & T4 and enlagment of Thyroid
gland, and in 70% cases with
ophthalmopathy
Graves disease (symptoms)
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Hyperactivity, irritability, altered mood
Fatigue, weakness
Goiter
Tachycardia and ↑ pul’s pressure
Nervousness
Graves disease (symptoms)
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Palpitations
Weight loss with ↑ appetite
Heat intolerants, increase sweating
Increased stool frequency
Thirst and polyuria
Oligomenorrea, loss of libido
Graves disease (sings)
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Sinus tachycardia, atrial fibrillation
Tremor, hyperkinesis
Warm, moist skin
Palmar erythema, onycholysis
Hair loss
Muscle weakness & wasting
Heart failure, psychosis (rare)
Graves disease
Ophthalmopathy
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A feeling of grittiness & discomfort in
the eye
Retrobulbar pressure or pain
Eyelid lag or retraction
Periorbital edema, chemosis, scleral
injection
Graves disease
Ophthalmopathy (c’d)
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Proptosis
Extraocular muscle dysfunction
Exposure keratitis
Optic neuropathy
Treatment of thyrotoxicosis
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Thionamids: mercasolyl 0.3-0.5 mg/kg
divided 2 -3 times – 14-21 days , than
supportive dose – 2.5-7.5 mg/daily 1
time
Β ab (anaprilin) 1-2 mg/kg divided 3
times
Euthyrosis – mercasolil 5-10 mg/daily
with L-thyroxin 25-50 μg/daily
Surgical treatment
Thyroid storm (crisis)
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Sudden onset
Fever
Profuse diaphoresis
Flushed warm skin
Tachycardia
Weakness, lethargy and confuson
Coma
Nausea, vomiting, diarrhea
Enlarge liver, jaundice
Thyroid storm (crisis)
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NaJ 1-2 g daily IV immediately
Propylthiouracil 200-300 mg every 6
hours by nasogastric tube
Β ab (propranolol) 0.1 mg/kg IV or 4
mg/kg orally
Dexamethasone 1-2 mg every 6 hours
Supportive: correction of dehydratation,
antipyretics, digitalis to patients with
cardiac failure
GOOD LUCK!