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Thyroid Gland Diseases in Children Riga Olena KhNMU 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 The steps of thyroid hormone synthesis Monoiodotyrosin (MIT) Diiodotyrosin (DIT) 1 molecula MIT + 2 DIT → thyroxin T4 1 molecula MIT + 1 DIT → triiodothyroxinT3 Under thyroperoxidase control Peripheral conversation T3 from T4 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 systemic disease starvation, anorexia surgical intervention newborn period gerontological period glucocorticosteroids β-adrenoblocks amiodarone (cordaron) propylthyouracil 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 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 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 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 Diagnostic of Thyroid gland disease Serological tests: *Markers of autoimmune disease (antibodies to thyroglobulin, thyroperoxidase, to TSH-receptors) *Markers of cancer (thyroglobulin, calcitonin) 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 Functional condition of Thyroid influence may be as Euthyroidism Hypothyroidism hyperthyroidism Нypothyroidism Hypothyroidism - syndrome with particular or total deficiency T3 and T4 or theirs acts to target cells Classification of hypothyroidism 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 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 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) 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 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 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 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 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 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 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) 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 Slow growth Puffiness Decreased appetite Constipation Swollen thyroid gland Lethargy Drop in school performance Cold intolerance Galactorrhea Menometrorrhagia SIGNS OF ACQUIRED HYPOTHYROIDISM Short stature Decreased growth velocity Increased upper to lower segment ratio Delayed dentition Myxedema or mildly overweight Goiter 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 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 T4 total and free, serum TSH Biopsy Antibodies test: antithyroglobulin antibodies to thyroperoxidase antimicrosomal test Causes of thyrotoxicosis Congenital: transient, neonatal Graves’ disease Acquired: Graves’ disease Functional adenoma Thyroid cancer TSH-secreting pituitary tumor Jatrogenic (Graves disease) Diffuse toxity goiter - autoimmune pathology with prolonged elevation T3 & T4 and enlagment of Thyroid gland, and in 70% cases with ophthalmopathy Graves disease (symptoms) Hyperactivity, irritability, altered mood Fatigue, weakness Goiter Tachycardia and ↑ pul’s pressure Nervousness Graves disease (symptoms) Palpitations Weight loss with ↑ appetite Heat intolerants, increase sweating Increased stool frequency Thirst and polyuria Oligomenorrea, loss of libido Graves disease (sings) Sinus tachycardia, atrial fibrillation Tremor, hyperkinesis Warm, moist skin Palmar erythema, onycholysis Hair loss Muscle weakness & wasting Heart failure, psychosis (rare) Graves disease Ophthalmopathy 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) Proptosis Extraocular muscle dysfunction Exposure keratitis Optic neuropathy 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 Thyroid storm (crisis) Sudden onset Fever Profuse diaphoresis Flushed warm skin Tachycardia Weakness, lethargy and confuson Coma Nausea, vomiting, diarrhea Enlarge liver, jaundice 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 GOOD LUCK!