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Thyroid Gland Diseases in Children Riga Olena KhNMU.

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Presentation on theme: "Thyroid Gland Diseases in Children Riga Olena KhNMU."— Presentation transcript:

1 Thyroid Gland Diseases in Children Riga Olena KhNMU

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4 Excretion of iodine ( in urine) 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

5 The steps of thyroid hormone synthesis Monoiodotyrosin (MIT) Diiodotyrosin (DIT) 1 molecula MIT + 2 DIT → thyroxin T 4 1 molecula MIT + 1 DIT → triiodothyroxinT 3 Under thyroperoxidase control

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7 Peripheral conversation T3 from T4 Both T 4 and T 3 circulate in plasma bound to the plasma thyroid hormone- binding protein (TBP) Thyroxine-binding globulin. Thyroid produces only 8 μg of T 4 and 4 μg T 3 daily. Serum T 3 concentration is usually low because of reduced conversation from T 4.

8 The factors that destroyed of conversation T4 → T3 systemic disease starvation, anorexia surgical intervention newborn period gerontological period glucocorticosteroids β-adrenoblocks amiodarone (cordaron) propylthyouracil

9 Action of thyroid hormones Genomic effects: the interaction of TH and its receptors is believed to precede other cellular events of messenger RNA and specific protein synthesis

10 Action of thyroid hormones 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

11 Action of thyroid hormones Maturation of the skeletal and dental system Maintenance of oxidative metabolism and heart production Control of temperature production TH differentiates all tissues and organs

12 Diagnostic of Thyroid gland disease Visual & palpating method Investigation of thyroid function (basal level of freeT3,freeT4) Functional tests (TSH) USG, radiography, scanning, etc. Biopsia

13 Diagnostic of Thyroid gland disease Serological tests: *Markers of autoimmune disease (antibodies to thyroglobulin, thyroperoxidase, to TSH-receptors) *Markers of cancer (thyroglobulin, calcitonin)

14 Goiter WHO (1994) 0 – goiter is absent I – goiter isn’t visualized, but it’s size less than distal phalanx of thumb II – goiter is palpated & visualized

15 Functional condition of Thyroid influence may be as Euthyroidism Hypothyroidism hyperthyroidism

16 Нypothyroidism Hypothyroidism - syndrome with particular or total deficiency T3 and T4 or theirs acts to target cells

17 Classification of hypothyroidism Disturbances PRIMARY - defects of biosynthesis of T 3, T 4 due to pathology of thyroid gland SECONDARY - decreasing T 3, T 4 due to deficiency of TSH (pituitary) or TRH (hypothalamus) or Resistance of receptors for T3, T4

18 Classification of hypothyroidism Onset Congenital Acquired (rare)

19 Classification of hypothyroidism Clinic & biologic data Latent (subclinical) T 3 -N, T 4 –N, TSH > 10 mU/l Manifestation of disease due to ↓ T 4 (at first) & ↓ T 3 Complicate

20 ETIOLOGY OF CONGENITAL HYPOTHYROIDISM 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

21 ETIOLOGY OF CONGENITAL HYPOTHYROIDISM (c’d) 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

22 SYMPTOMS OF CONGENITAL HYPOTHYROIDISM 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

23 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

24 Minority of CH 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

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26 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

27 Biochemical hallmarks of CH 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 TBG- deficiency or hypothalamic-pituitary hypothyrodism

28 Biochemical hallmarks of CH Other: Elevated serum cholesterol Elevated creatinphosphokinase Hyponatriemia

29 Instrumental data 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)

30 Treatment L-thyroxin Preterm 8 – 10 μg/kg 0-3 mo 10 – 15 μg/kg 3-6 mo 8 – 10 μg/kg 6-12 mo 6 – 8 μg/kg 1-3 years 4 – 6 μg/kg 3-10 years 3 – 4 μg/kg 10-15 years 2 – 4 μg/kg > 15 years 2 – 3 μg/kg

31 ETIOLOGY OF ACQUIRED HYPOTHYROIDISM 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

32 ETIOLOGY OF ACQUIRED HYPOTHYROIDISM (c’d) 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

33 SYMPTOMS OF ACQUIRED HYPOTHYROIDISM Slow growth Puffiness Decreased appetite Constipation Swollen thyroid gland Lethargy Drop in school performance Cold intolerance Galactorrhea Menometrorrhagia

34 SIGNS OF ACQUIRED HYPOTHYROIDISM Short stature Decreased growth velocity Increased upper to lower segment ratio Delayed dentition Myxedema or mildly overweight Goiter

35 SIGNS OF ACQUIRED HYPOTHYROIDISM (c’d) Delayed reflex return Dull, placid expression Pale, thick, carotenemic, or cool skin Muscle pseudohypertrophy Delayed puberty or precocious puberty Treatment –same CH

36 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

37 Diagnosis Hashimoto disease T4 total and free, serum TSH Biopsy Antibodies test: antithyroglobulin antibodies to thyroperoxidase antimicrosomal test

38 Causes of thyrotoxicosis Congenital: transient, neonatal Graves’ disease Acquired: Graves’ disease Functional adenoma Thyroid cancer TSH-secreting pituitary tumor Jatrogenic

39 (Graves disease) Diffuse toxity goiter - autoimmune pathology with prolonged elevation T3 & T4 and enlagment of Thyroid gland, and in 70% cases with ophthalmopathy

40 Hyperactivity, irritability, altered mood Fatigue, weakness Goiter Tachycardia and ↑ pul’s pressure Nervousness Graves disease (symptoms)

41 Palpitations Weight loss with ↑ appetite Heat intolerants, increase sweating Increased stool frequency Thirst and polyuria Oligomenorrea, loss of libido

42 Graves disease (sings) Sinus tachycardia, atrial fibrillation Tremor, hyperkinesis Warm, moist skin Palmar erythema, onycholysis Hair loss Muscle weakness & wasting Heart failure, psychosis (rare)

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44 Graves disease Ophthalmopathy A feeling of grittiness & discomfort in the eye Retrobulbar pressure or pain Eyelid lag or retraction Periorbital edema, chemosis, scleral injection

45 Graves disease Ophthalmopathy (c’d) Proptosis Extraocular muscle dysfunction Exposure keratitis Optic neuropathy

46 Treatment of thyrotoxicosis 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

47 Thyroid storm (crisis) Sudden onset Fever Profuse diaphoresis Flushed warm skin Tachycardia Weakness, lethargy and confuson Coma Nausea, vomiting, diarrhea Enlarge liver, jaundice

48 Thyroid storm (crisis) 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

49 GOOD LUCK!


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