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SLU - SOM Pediatrics Prelim Exams: THYROID

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Hypothyroidism results from?   1. Deficient production from: a) gland (primary Hypothyroidism) b) reduced TSH (Secondary) 2. Defect in receptor activity  
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Cause of CONGENITAL HYPOTHYROIDISM?   >Thyroid Dysgenesis - 85% > Inborn errors of Thyroxine Synthesis - 10% >Transplacental maternal thyrotropine - receptor blocking antibody - 5%  
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Familial Congenital Hypothyroidism is caused by?   Inborn Errors of thyroid hormone synthesis  
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Appearance of manifestations of SEVERE Congenital Hypothyroidism Appears? Less Severe?   Early manifestations - SEVERE Late Manifestations - Less Severe  
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Epidemiology: a) Race b) gender   a) Hispanic and Native Americans > Blacks b) F>M (2:1)  
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Clinical manifestations:   > Large anterior and posterior fontanels - first clues secondary to myxedema of the brain > Prolonged Physiologic Jaundice - >2 weeks, may be the earliest signs > 1st month: feeding difficulties, choking spells, lac of interest, sluggish, somnolence  
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Anemia associated with congenital hypothyroidism   Macrocytic anemia - intractable to treatment  
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Most common Manifestation/s for Congenital Hypothyroidism?   Cardiac Anomalies  
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If untreated or undetected (more than 1 month)   > Retardation of mental and physical development > Stunted growth, HC normal or increased > Fontanels- opened > Dry skin > Fat deposition above clavicles  
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Diseases Detected by Newborn Screening?   > Phenylketonuria > G6PD > Congenital Adrenal Hyperplasia > Congenital Hypothyroidism > Galactosemia  
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Diagnostics for Congenital Hypothyroidism?   > Newborn Screening > (American) T4 determination --> TSH if T4 is low > (European and Japanese) TSH measurement - detects primary hypo and subclinical hypo  
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Thyroid hormones Profile for Congenital Hypothyroidism?   T4 - LOW T3 - NORMAL TSH - HIGH Serum THYROGLOBULIN - LOW ** Serum Prolactin - High  
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Radiologic Findings CHypoThy   >Distal Femoral Epiphysis is ABSENT (epiphyseal dysgenesis) . BEAKING Deformity - T12, L1, L2  
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ECG findings for CHypoThy   Low voltage P and T waves Diminished Amplitude of QRS  
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Treatment for CHypoThy   ORAL LEVOTHYROXINE - 10-25 mcg/kg  
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Monitoring after treatment   T4 and TSH monthly for the first 6 months, then every 2-3 months until 2 y/o  
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How many points in the IQ is reduced?   5-20 points  
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Neuropsychological Sequelae of congenital Hypothyroidism?   > Incoordination > hypotonia/ hypertonia > Short attention span > Speech Problems > neurosensory hearing defects  
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ACQUIRED HYPOTHYROIDISM Epidemiology   > more common that Congenital > F>M (2:1)  
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Cause of Acquired Hypothyroidism   Chronic Lymphocytic Thyroiditis (Hashimoto's Thyroiditis)  
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Other causes of Hypothyroiditis (acquired)   - Autoimmune: Pernicious Anemia, Klinefelter, Down's - Irradiation - Meds: AMIODARONE - liver hemangioma - inc. type III deiodinase  
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Manifestations:   - decelerated growth - 1st clinical sign - Goiter - Enlarged gland, non-tender, firm, rubbery consistency - hypothyroidism s/sx - precocious puberty - inc. TSH  
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What is Goiter?   Enlarged thyroid gland  
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T/F: Goiter is caused by increased thyroid hormone secretion?   FALSE. Increased PITUITARY SECRETION (TSH) can be Euthyroid, Hypothyroid, Hypothyroid, Congenital or acquired , endemic or sporadic  
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Congenital Goiter is caused by?   > Fetal thyroxine Synthetic defect > Administration of Anithyroid drugs during pregnancy  
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Radiologic findings?   Anterior deviation of the Trachea  
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Acquired Goiter is caused by?   > Lyphocytic Thyroiditis (Hashimoto) > Iodine Ingestion > Drugs  
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Thyroid picture of Acquired Goiter?   Euthyroid or Hypothyroid  
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Other name for ENDEMIC GOITER?   Cretinism  
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Cause of Cretinism   IODINE DEFICIENCY  
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Clinical Manifestations   > Mild - no noticeable neck mass > Moderate - evident neck mass and may disappear with maturity may reappear during pregnancy > Severe - large neck mass  
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Types of Endemic Cretinism?   1. Neurologic Type - Zaire 2. Myxedematous Type - Papua New Guinea  
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Manifestations of Neurologic Type?   > MR > Deaf mutism > gait and standing disturbances > normal pubertal development  
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Pyramidal Signs of Neurologic type?   > Babinski > hyperreflexia > ankle clonus  
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Thyroid Picture of Neurologic type?   EUTHYROID  
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Manifestations of Myxedematous type?   > MR > deaf with neurologic symptoms > delayed growth and sexual development > absent goiter on UTZ  
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Thyroid picture?   T4 LOW; TSH marked elevation  
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Pathogenesis of Neurologic type?   Direct effects of maternal and fetal iodine deficiency (hypothyroxinemia)  
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Pathogenesis of Myxedematous type?   >Selenium Deficiency > auto-antibodies/ autoimmune  
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Treatment for Cretinism?   IODINATED POPPY SEED OIL to women - prevents iodine deficiency in future pregnancies for 5 years  
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HYPERTHYROIDISM   excessive secretion of Thyroid Hormones  
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Cause of GRAVE'S DISEASE?   production of thyroid stimulating immunoglobulin  
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Peak incidence?   11-15 y/o  
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Gender ratio?   F>M (5:1)  
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Earliest manifestation of Grave's?   Emotional Disturbances with motor hyperactivity  
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Description of Exophthalmos   > lagging upper eyelid > impaired convergence >  
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Anxious Stare   EXOPHTHALMOS  
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An acute condition that is manifested by hyperthermia, severe tachardia, rapid progression of coma, and is precipitated by stressful conditions. Hx: Hyperthyroidism   Thyroid crisis/Thyroid Storm  
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diagnostics for Thyroid storm?   > inc Serum T4, Free T4 and T3 > low TSH > (+) antithyroid antibodies and thyroid peroxidase antibodies > (+) thyrotropin stimulating antibodies  
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Treatment for Thyroid storm?   > Anti-thyroid: PTU > Hydrocortisone > Propanolol > Supportive mngt. (treat underlying cause)  
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conditions for radioiodine treatment of surgery?   > cooperation for medical management is not possible > trial of meds has failed > side effects preclude futher use of antithyroid meds  
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Ocular manifestations of Grave's Disease?   Pain, Lid erythema, Chemosis, decrease EOM function, Decreased Visual Acuity  
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Papillary Carcinoma   - RET rearrangements - Familial; Autosomal Dominant  
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Anaplastic Thyroid Carcinoma   - p53 point mutations  
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Procedure that causes Thyroid carcinomas?   Radiation Therapy  
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Amount of Grey that causes a 7.7 relative risk   1 Gy  
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Most Common Histologic type of Thyroid Ca   Papillary/Follicular  
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Most fatal Histologic type?   Anaplastic  
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MC site of mets   Lungs  
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Gold standard for diagnosis   FNAB  
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Determines Solidity of the tumor   Ultrasound  
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Determines radionucleid uptake   Thyroid Scan  
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Radioisotopes used for DIAGNOSIS   Iodine - 123 99m Technetium pertechnetate  
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cold nodules   most are BENIGN  
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Hot Nodules   Malignant  
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Treatment for tumors <1 cm   subtotal thyroidectomy followed by suppressive doses of thyroid hormone  
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Small but multicentric tumors   Total Thyroidectomy  
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Tumors >1 cm   Total thyroidectomy with LN excision  
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Radioisotopes used for TREATMENT   Iodine - 131 (30-100 mCi)  
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Drugs given after Surgery?   Na L - thyroxine  
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