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Endocrinology
New set 2023 UWORLD only
| Question | Answer |
|---|---|
| What are some features of pathologic gynecomastia? | Size >4cm, rapid progression, and location eccentric to the nipple areolar complex. |
| What are 2 examples of SGLT-2 inhibitors? | Canagliflozin and Dapagliflozin |
| MOA of SGLT-2 inhibitors | ↓ renal absorption of glucose and Na+ |
| What is the key features of Pancreatic disease in Cystic fibrosis? | Nonselective destruction of α and ß-cells |
| Why is glucagon deficiency seen earlier than insulin deficiency in Pancreatic disease? | α-cells make up the minority of cells in the pancreas, thus creating a faster hormonal deficiency |
| What is another term used for CYP24A1? | 24-hydroxylase |
| What enzyme initiates vitamin D catabolism? | 24-hydroxylase |
| What is the MC anticonvulsant that can cause induction of 24-hydroxylase expression? | Phenytoin |
| What is the result of Phenytoin (or other anticonvulsant) inducing the expression of 24-hydroxylase (CYP24A1)? | ↑↑ breakdown of vitamin D and development of vitamin D deficiency |
| What is the MCC of Acromegaly? | Excess GH due to pituitary somatotroph adenoma |
| What is the cardiovascular complication associated with Acromegaly? | Left ventricular hypertrophy which then causes HF. |
| What is the MC adrenal insufficiency? | 21-hydroxylase deficiency |
| How is 21-hydroxylase deficiency diagnosed based on lab values? | ↑↑↑ 17-hydroprogesterone |
| What is the role of 21-hydroxylase? | Convert Progesterone -----> 11-deoxycorticosterone |
| What stimulates Insulin release in pancreatic ß-cells? | ↑↑ glucose metabolism and ATP production |
| What enzyme functions as a glucose sensor in the Pancreatic ß-cells? | Glucokinase |
| How does Glucokinase regulate insulin release by pancreatic ß-cells? | Control rate of glucose entry into the glycolytic pathway |
| What is the result of mutated glucokinase? | Maturity-onset diabetes of the young |
| Which pouches are involved in DiGeorge syndrome? | Third and fourth brachial pouches |
| What are derivatives of the 3rd pharyngeal pouch? | Inferior PTH gland and Thymus |
| What are the derivatives of the 4th brachial pouch? | Superior PTH gland |
| What neurotransmitter is involved in the rate of prolactin release? | Dopamine |
| Which dopamine receptors are blocked with antipsychotic medications? | D2 |
| What are some values important in Exogenous thyrotoxicosis? | RAIU and serum Thyroglobulin levels are LOW Serum T3 is often elevated |
| Why are chronic DM1 patients at increased risk of hypoglycemic episodes? | Exogenous insulin will be continuously absorbed from the injection site despite falling glucose levels |
| Example of a GnRH agonist often used in BPH? | Leuprolide |
| What is the MOA of chronic GnRH agonist therapy? | Suppression of Pituitary release of LH -->--> ↓ production of testosterone |
| What are the bone effects due to chronic GnRH therapy? | Produces a similar effect to surgical orchiectomy which leads to accelerated bone loss and ↑ risk for osteoporosis |
| What does the term Pituitary apoplexy mean | Acute pituitary hemorrhage |
| What are important clinical features of Pituitary apoplexy? | Headache, bitemporal hemianopsia, and ophthalmoplegia |
| What CN is involved in ophthalmoplegia seen in pituitary apoplexy? | CN III compression |
| What is the initial step of steroid hormone synthesis? | Conversion of cholesterol to pregnenolone in the mitochondria |
| Except for the initial step in steroid hormone synthesis, where does steroid genesis occur? | Smooth Endoplasmic Reticulum |
| What is the MC type of Thyroid cancer? | Papillary Thyroid carcinoma |
| What thyroid cancer is associated with the "Orphan Annie eyes" in histology? | Papillary Thyroid carcinoma |
| What are the 2 most relevant histological findings of Papillary Thyroid carcinoma? | 1. "Orphan Annie eyes" 2. Intranuclear Inclusions and nuclear grooves |
| What is MOA of Erythromycin? | Stimulates upper GI motility by acting as an agonist on Motilin receptors in the muscularis externa. |
| What condition is treated with Erythromycin? | Gastroparesis |
| What condition is often associated with gastroparesis? | Chronic diabetes mellitus |
| What serum level is the most sensitive for dx of primary hypothyroidism? | Serum TSH |
| Inheritance mode of Niemann-Pick disease | Autosomal Recessive |
| What substance is deficiency and accumulated in Niemann-Pick? | Sphingomyelinase deficiency leads to sphingomyelin (phospholipid) accumulation |
| What are the important clinical features of Niemann-Pick disease? | - Neurologic regression - Hepatosplenomegaly - Retinal opacification (cherry-red macula) |
| Describe pathogenesis of CF-related diabetes | Progressive destruction of pancreatic ß-cells leads to ↓↓↓ insulin production |
| What are the CATABOLIC effects of glucocorticoids? | 1. Muscle weakness 2. Impaired wound healing, osteoporosis, and 3. Immunosuppression |
| What is an important ANABOLIC effect by glucocorticoids? | ↑ hepatic synthesis of gluconeogenic and glycogenic proteins to ↑ glucose availability |
| What is MCC of type 1 DM? | Autoimmune insulitis w/ progressive ß-cell loss |
| What are 3 examples of sympathomimetic weight loss medications? | Phentermine, diethylpropion, and benzphetamine |
| Stimulant weight loss medications are contraindicated in pts with: | Hx of CVD |
| Intestinal lipase inhibitor | Orlistat |
| What are the functions of insulin? | 1. ↑ glucose uptake by skeletal muscle, adipose, and liver 2. ↑ glycogen synthesis/ ↓ glycogenolysis, 3. ↓ glucagon secretion 4. ↓ Lipolysis and ketogenesis 5. ↑ protein synthesis in muscle |
| What receptor is stimulated by Thiazolidinediones? | PPAR-γ |
| What are the results of thiazolidinediones? | 1. ↓insulin resistance ----> ↓↓↓ blood glucose 2. Reverse non-alcoholic steatohepatitis |
| What is the MCC of adrenal insufficiency? | Suppression of HPA axis by glucocorticoid therapy |
| What is rare cause of an Adrenal crisis? | Failure to ↑↑ glucocorticoid dose during stressful situations |
| What is the main clinical feature of Primary hyperaldosteronism? | Hypertension with hypokalemia |
| What cells give rise to Medullary Thyroid cancer? | Parafollicular Calcitonin-secreting C cells |
| What is the histology of Medullary Thyroid cancer? | Nests or sheets of polygonal or spindle-shaped cells with extracellular amyloid deposits derived from calcitonin |
| How is TSH and T4 in Thyroid dysgenesis? | ↑ TSH and ↓ T4 |
| MCC of primary congenital hypothyroidism | Thyroid dysgenesis |
| What enzyme is inhibited by Metyrapone? | 11-ß-hydroxylase |
| What is the direct effect of Metyrapone? | ↓ cortisol synthesis via inhibition of 11-ß-hydroxylase |
| What urinary lab effect is seen with use of Metyrapone? | ↑ urinary 17-hydroxycorticosteroid level |
| What are the defects in Type III hyperlipidemia? | Defective ApoE3 and ApoE4 |
| What are the dermatologic manifestations seen in Type III hyperlipidemia? | Eruptive and palmar xanthomas |
| How does metformin aid in reducing glucose levels? | Reduce hepatic gluconeogenesis and ↑↑ insulin-dependent peripheral glucose uptake |
| Which patients are to be closely monitored if given metformin? | Renal insufficiency patients |
| How is the "growing" done in Acromegaly? | Hyperplasia of articular chondrocytes and synovial hypertrophy --> degeneration of articular cartilage and periarticular bone |
| How is Rickets characterized? | Defective mineralization at sites of rapid bone growth |
| Which deficiencies can cause Rickets? | Calcium or phosphorus |
| What is the MCC of low-phosphorus - induced rickets? | Impaired renal reabsorption |
| What is the MCC of Homocystinuria? | Cystathionine synthase deficiency |
| What is the ocular deficit in Homocystinuria? | Ectopia lentis |
| What is MCC of death in Homocystinuria? | Thromboembolism |
| What supplementation has proven to improve Homocystinuria symptoms and progress? | Pyridoxine (vitamin B6) supplementation |
| What are physiological normal age-related changes in male testosterone production? | Slow decline in gonadal testosterone synthesis which is compensated by ↑ LH and ↑↑ SHBG |