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