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Copyright 2012 USMLE Success Academy. All Rights Reserved.

This 500 High-Yield Nuggets book was created to give you a quick review guide for some of the most important facts you should know for the USMLE Step 2 CK examination. While this is not a complete list, it is one that contains high facts you should know before writing the Step 2 CK.

500 High-Yield Nuggets For The Step 2 CK

Surgery 1. Myocardial infarction within the last 6 months is an absolute contraindication to surgery. 2. Before surgery it is important that we stop: Aspirin, NSAIDS, anti-PLT, and Warfarin. 3. Parental consent is not required for the following: STD, Pregnancy, Abortion, Psychiatric problems, and Substance Abuse. 4. Combining Haldol with Succinylcholine gives us the greatest risk of malignant hyperthermia. 5. Treat malignant hyperthermia with Dantrolene. 6. The 5 MCCs of post-operative fever, in order of most recent are: WIND, WOUND, WATER, WALK, WONDER DRUGS. 7. Whenever you are seeing a trauma victim, always consider the ABCDEs. 8. The 4 MCC of shock are: Cardiogenic, Hypovolemic, Septic, Neurogenic. 9. First and Second degree burns are limited to the epidermis and superficial dermis (redness, blistering, pain). 10. Third and Fourth degree burns affect all layers of the skin plus subcutaneous tissues. 11. Epidural hematoma is caused by rupture of the middle meningeal artery. 12. Subdural hematoma is caused by rupture of the bridging veins. 13. The MCC of a subarachnoid hemorrhage is trauma, the 2nd MCC is ruptured Berry aneurysm. 14. Diffuse axonal injury is stretching and/or shearing of brain tissue, MCC by severe motor vehicle accidents. 15. The 1st step in managing increased ICP is to elevate the head of the bed (allowing gravity to pull fluids away from the head). 16. Branchial cleft cysts do not elevate when swallowing, a Thyroglossal duct cyst does.

17. The Papillary thyroid cancer has the greatest survival rate, while the Anaplastic version carries the worst prognosis. 18. Achalasia is caused by loss of the relaxation ability of the lower esophageal sphincter, leading to dilation of the proximal esophagus. 19. Sliding hiatal hernias are more common and associated with GERD, while Rolling hiatal hernias are less common and usually require intervention. 20. Patients who complain of dysphagia and regurgitation + have halitosis will most likely have a Zenkers diverticulum. 21. The MCC of esophageal cancer in the USA is Adenocarcinima. 22. Adenocarcinoma is associated with Barretts esophagus, SCC is associated with smoking and alcohol use. 23. Diets high in fat, high in salt, high in nitrosamines, and low in fiber, all increase the risk for gastric tumors. 24. Common findings in gastric cancer are: Virchovs node, Krukenburg tumor, Sister Mary-Joseph sign, and Blumers shelf. 25. Water-bottle sign on xray is indicative of Linitis Plastica, the most deadly form of gastric cancer. 26. Indirect inguinal hernias protrude lateral to the Inferior Epigastric artery, while a direct inguinal hernia protrudes medial to the Inferior Epigastric artery. 27. Females taking oral contraceptives are most likely to be the victims of a hepatic adenoma. 28. The 4 Fs of Gallstones are: Female, Fertile, Fat, Forty 29. Calcified (porcelain) gallbladder, is indicative of cancer and should warrant a removal of the gallbladder. 30. Ultrasound is usually the correct test to order when looking for gallstones 31. The HIDA scan is a diagnostic procedure that helps us identify stones when an ultrasound is inconclusive. 32. An ERCP is a test that is both diagnostic and therapeutic, as we can visualize and remove an obstruction with this procedure.

33. Lipase is more sensitive for acute pancreatitis, but we still check both Lipase and Amylase. 34. Insulin-producing tumors will be seen by the presence of c-peptide, while exogenous sources of Insulin will lack c-peptide and thus help make a conclusive diagnosis. 35. The MC surgical condition of the GI is a small bowel obstruction (SBO). 36. SBO is identified on xray by visualizing multiple distended loops of small bowel and upright air-fluid levels. 37. The MC form of polyp is the tubular adenoma 38. Villous adenomas have the highest risk of cancer because they are deeper in the GI wall. 39. A true diverticula involves the full extent of the bowel wall, a false diverticula is simply a herniation of the mucosa. 40. A common cause of painless lower GI bleeding in older adults is diverticulosis. 41. A common cause of LLQ pain in older adults is diverticulitis. 42. Treat a volvulus with sigmoidoscopy, which works by decompressing. If the problem lies in the cecum, a Cecopexy can get rid of the problem. 43. With no risk factors (family history, etc), colon cancer screening via colonoscopy should start at 50yr. 44. With the presence of risk factors, screening for colon cancer should begin at 40yr or 10 years before the onset in the family member affected. 45. The initial management steps of hemorrhoids are: Increased fiber and water intake, Sitz baths, hemorrhoidal creams. 46. Drug-related causes of gynecomastia in adult men are: Spironolactone, Digoxin, Cimetidine, Alcohol, and Ketoconazole. 47. The MC breast tumor in teens and young adults is the fibroadenoma. 48. Peau dorange is seen in Pagets disease of the breast and is almost always an indication of underlying ductal carcinoma. 49. Mammography should begin around 35yr of age, but not before due to increased density of the breast tissue.

50. In comparing testicular torsion and appendix testis, the torsion has a transverse lie while the appendix testis sits normally within the scrotum. 51. BPH is caused by excessive stimulation of the prostate tissue via DHT (due to 5-alpha-reductase enzyme). 52. Pain in the anatomical snuff-box is indicative of scaphoid fracture. 53. Wrist splints used in carpal tunnel syndrome should be kept in a slight extension position to best alleviate pressure on the median nerve. 54. Anterior shoulder dislocations occur 95% of the time, a posterior shoulder dislocation is due to only two things: Lightning strike and grand-mal seizure. 55. Hip dislocation shows a shortened and internally rotated leg, while a fracture to the femoral neck results in a leg position that is externally rotated. 56. The unhappy triad consists of injuries to the: MCL, ACL, and Medial meniscus 57. Four major signs of a basilar skull fracture are: Battles sign (mastoid bruising), Periorbital ecchymosis, Hemotympanum, and CSF rhinorrhea. 58. The greatest risk for rupture of a AAA is increasing size (increased diameter = increased risk). 59. The first step in management of the aortic dissection is to control and/or lower the blood pressure. 60. The best way to diagnose Leriche syndrome (aortoiliac disease resulting in claudication of the hip and gluteal muscles), as the Ankle To Brachial Index, whereby an ABI less than 0.7 is indicative of claudication. 61. Venous ulcers are more commonly on the malleolus, while arterial ulcers are more commonly on the dorsum of the foot. 62. When carotid stenosis is >70%, carotid vascular surgery is warranted. 63. The nerve that requires injury assessment in an anterior shoulder dislocation is the axillary nerve. 64. Mastitis is an infection of the skin around the nipple, MC when breastfeeding, MC due to Staph Aureus. This is not an indication for the cessation of breast feeding. 65. Familial Adenomatous Polyposis is an AD disease caused by the inheritance of the APC gene.

66. Peutz-Jeghers syndrome commonly shows up with freckling of the lips. 67. Penetrating chest trauma to the left side will most likely end up in the right ventricle, while on the right side it will most likely end up in the lung. 68. Pain referred to the right shoulder may indicate an inflammatory processes to the liver or liver capsule. 69. Violent acceleration-deceleration injuries are a common cause of diffuse axonal injury. 70. Any patient who is taking Warfarin for DVT yet continues to get PEs, should have an IVC filter placed. 71. A varicocele is a tortuous dilation of the pampiniform plexus of veins that surround the spermatic cord and testes in the scrotum. 72. Any patient who is seen with air under the diaphragm should be rushed into the ER first and foremost.

OB/GYN 73. Nagels Rule: EDC = LMP + 7 days 3 months + 1 year 74. Term pregnancy is defined as 37-42 weeks. 75. Average birth weight is 2500g-3500g 76. The proliferative phase of the menstrual cycle is also known as the Estrogen- dependent phase, and lasts from approximately day 5-13 77. During the proliferative phase, estrogen helps with: Ductal growth, growth of decidua functionalis, and cervical mucus thinning. 78. Progesterone helps prepare a uterus for implantation by increasing spiral arteries in the endometrium and thinning the cervical mucus. 79. Oral contraceptives work by inhibiting the feedback mechanism on the pituitary to keep LH suppressed. 80. Diagnosing menopause requires an FSH level >50. 81. The MOA of Clomiphene is blocking of the Estrogen receptor at the level of the hypothalamus. 82. Any female who fails to lactose 6-7 days post-partum should be evaluated for Sheehans syndrome. 83. Patients with PCOS should be started on Metformin to decrease levels of blood sugar. 84. Congenital adrenal hyperplasia is the MCC of virilization in newborn females. 85. The first three steps in the infertility work-up are: Semen analysis, cervical mucus analysis, and ovulation evaluation. 86. The visualization of prominent blood vessels on colposcopy is the most aggressive invasive cancer finding. 87. The 3 MCCs of vaginitis are: Candida, Gardnerella, and Trichomonas. 88. The classic triad of endometriosis is: Dysmenorrhea, Dyspareunia, and Infertility. 89. Management of idiopathic precocious puberty is with a GnRH analog such as Leupron.

90. The MCC of ectopic pregnancy is history of PID, which is MCC by G&C. 91. The MCC of ovarian torsion is excessive weight due to ovarian cyst. 92. The first step in work-up for an ovarian mass is an ultrasound. 93. The MC malignant tumor of the ovary is the Serous Cystadenocarcinoma. 94. The MC cancer of the reproductive tract is cancer of the endometrium. 95. The causes of endometrial cancer are estrogen-related: Unopposed estrogen replacement therapy, late-onset menopause, obesity, nulliparity, estrogen- secreting tumors, PCOS. 96. Cancer of the vagina/vulva presents with Itching, irritation, and a burning sensation (treat with estrogen cream). 97. A persistent molar pregnancy is also known as a Choriocarcinoma. 98. The first tool used in evaluating a breast mass is a fine-needle aspiration. 99. The MCC of a bloody nipple discharge is Intraductal Papilloma. 100. The MCC of a milky nipple discharge is Galactorrhea. 101. Tamoxifen works well for breast cancer because it blocks the effects of estrogen at the breast, inhibiting its negative effects. 102. The MC sites of breast cancer metastasis are: Lung, Liver, and Bone 103. The true pelvis and the false pelvis are separated by the linea terminalis (true is below, false is above). 104. The classic female pelvis shape is the gynecoid shape, while the android shape is the most common male shape. 105. Gestational diabetes mellitus is caused by the release of human placental lactogen, which is released by the placenta and antagonizes insulin. 106. When the fetus is >4,500g, a c-section is considered. When the fetus is >5,000g, a c-section is mandatory. 107. Pre-eclampsia is HTN >140/90 + mild proteinuria, severe pre-eclampsia is HTN >160/110 + proteinuria >1g/24hr. 108. The first step in preventing a seizure when PIH is present is magnesium sulfate (it beats sodium to the cell and prevents depolarization). 109. The presence of Group B Strep via anal/vaginal swab during pregnancy warrants an intrapartum shot of Penicillin.

110. Three common causes of hyperemesis gravidarum are: High hCG levels, elevated thyroid hormones, elevated GI hormones. 111. Common causes of recurrent abortions ( 2 consecutive or a total of 3 spontaneous abortions) are: Antiphospholipid antibodies or Anti-cardiolipin antibodies. 112. Pre-term labor is defined as the onset of labor between 20-36 weeks. 113. The first step in managing shoulder dystocia is McRoberts maneuver (hyperflexion of the legs into the abdomen). 114. The MCC of post-partum hemorrhage is uterine atony. 115. Breast-feeding supplies the baby with all of the important vitamins except for vitamin K. 116. Three MCC of tender breasts post-partum are: Engorgement, mastitis, and a plugged duct. 117. The MC reason for post-partum uterine infection (Metritis) is a history of c-section. 118. Early fetal heart rate decelerations are an indicator of pressure to the fetal head. 119. Variable decelerations are caused by vagal reflex due to cord compression. 120. Late decelerations are worrisome and are associated with uteroplacental insufficiency (this one is worrisome). 121. Twin-to-twin transfusion syndrome is caused by a shared blood supply that leads to the shunting of blood from a donor twin to an acceptor twin.

PEDIATRICS 122. Infant botulism is MCC by the ingestion of spores found in raw honey. 123. The pathognomonic finding of Measles is Koplik spots (but catching them is difficult because they are transient). 124. The unique features of Rubella is the presence of Suboccipital Lymphadenopathy. 125. Slapped-cheek syndrome is caused by Parvovirus B19, which has the chance of suppressing the bone marrow. 126. Varicella vesicles are infectious until they crust over. 127. The pathognomonic finding of otitis media is decreased tympanic membrane mobility. 128. Steeple sign is seen on xray of Croup. 129. Epiglottitis is caused by H. Influenza B and is a medical emergency. 130. Foreign bodies are MC located in the right bronchus, due to the angle of the anatomy. 131. The MCC of septic arthritis is Staph Aureus. 132. Lateral movement of the femoral head in relation to the femoral shaft is known as Slipped Capital Femoral Epiphysis, which is MC seen in overweight boys 8-17yr. 133. Osteomyelitis is MCC by Staph Aureus. 134. In sickle cell patients, the MCC of osteomyelitis is Salmonella. 135. The treatment for Kawasakis disease is high-dose aspirin (one of the only times when we give Aspirin to a child). 136. The pathognomonic finding in Henoch-Schonlein purpura is palpable purpura on the buttocks and legs. 137. Physiologic jaundice is seen between 24-48hr after birth, before this time it is pathologic. 138. The management for newborn jaundice is UV light exposure, which breaks down bilirubin).

139. Reye syndrome causes a fatty degeneration of the liver in addition to acute encephalopathy (this is why we never give aspirin). 140. Failure to thrive in the 1st year of life has the worst prognosis. 141. The MCC of primary amenorrhea is Turners syndrome. 142. The MCC of mental retardation in boys is fragile x syndrome. 143. The MCC of mental retardation across both genders is Downs syndrome. 144. Folate supplementation should begin at least 12 weeks before a patient plans on getting pregnant (to prevent NTDs). 145. Projectile vomiting in a two-week to two-month old is a sign of congenital pyloric stenosis. 146. The pathognomonic finding of Congenital pyloric stenosis is Olive Sign, which is a palpable mid-epigastrium nodule. 147. The MC type of congenital heart disorder is the VSD. 148. Close a PDA with NSAIDs, keep it open with prostaglandins. 149. Pre-ductal coarctation of the aorta is seen in 5% of Turners syndrome patients. 150. Ebsteins anomaly (low implanted triscuspid valve) is caused by ingestion of Lithium during pregnancy. 151. Adolescents who are of driving age are most likely to die in a MVA. 152. Females attempt suicide more than men, but men are more likely to be successful in their attempt. 153. By the end of high-school, fifty percent of adolescents have used an illicit drug. 154. Sixty percent of males and fifty percent of females have had sex by the end of high-school.

PSYCHIATRIC MEDICINE 155. Major depressive disorder can only be diagnosed if symptoms persist for at least two weeks, with each episode having at least two months in between. 156. Medication + therapy is almost always the best choice on a psychiatric question for the USMLE CK. 157. SSRIs are the mainstay of pharmacological management in MDD. 158. Always rule out cocaine use, amphetamine use, Schizophrenia, and the cluster B personality disorders when bipolar disorder is suspected. 159. Bipolar 1 shows manic phases with or without depressive episodes, while bipolar 2 shows hypomanic phases + depressive episodes. 160. Dopamine blockers are the first-line in medical management of Schizophrenia. 161. Watch for movement disorders as a side-effect of Schizophrenia medications. 162. Panic disorder mimics the signs/symptoms of a myocardial infarction, but is seen MC in females in their twenties. 163. An EKG should be performed the first time you encounter a panic disorder patient. 164. The mainstay of management for panic disorder is SSRIs. 165. Patients with obsessive-compulsive disorder are aware of their problem, while patients with obsessive-compulsive personality disorder do not see a problem with their behavior. 166. Patients are diagnosed with GAD when they worry on most days for at least six months. 167. The mainstay of management in GAD is psychotherapy. 168. PTSD can only be diagnosed after four weeks of signs/symptoms (< 4 weeks is known as acute stress disorder) 169. SSRIs are the mainstay in pharmacological management of PTSD.

170. A personality disorder MUST lead to day-to-day functional impairment. 171. Conduct disorder is a pre-cursor to Anti-Social personality disorder. 172. Munchausen syndrome is a factitious disorder of self, while Munchausen by Proxy is when symptoms are faked by someone who is caring for another. 173. Conversion disorder is a sub-conscious transfer of emotional feeling to a neurological finding. 174. Body dysmorphic disorder is managed with a combination of SSRI + Psychotherapy. 175. Autism is a pervasive developmental disorder that must begin before three years of age. 176. Aspergers syndrome is similar to Autism, however there will be normal language development. 177. Depression in children may be seen as hyperactivity, aggressiveness, irritability, boredom, or anti-social behaviors. 178. Children who experience somatic symptoms during periods of separation from their primary caregiver are said to have separation anxiety. 179. Oppositional defiant disorder is seen when a child acts temperamental with people they know well, while conduct disorder is a child who bullies, fights, and causes harm to other people and their property without any guilt. 180. Conduct disorder is a pre-cursor for antisocial personality disorder. 181. In order to make a proper diagnosis of ADHD, the symptoms must be present before the age of seven years and they must occur in just one place. 182. The first-line DOC for ADHD is Methylphenidate. 183. The DOC for Tourettes disorder is Haldol. 184. There is no requirement for psychotherapy in cases of Tourettes disorder because it is an involuntary disorder. 185. Anorexia is diagnosed with a BMI < 17.5.

INTERNAL MEDICINE 186.The P wave represents atrial depolarization, while the QRS complex represents ventricular depolarization. 187.The left coronary artery supplies eight-five percent of the myocardium. 188.The dominant artery is the one which supplies that SA node, which is usually the right coronary artery). 189.Essential hypertension represents ninety-five percent of all hypertension cases. 190.The first step in managing hypertension is lifestyle modifications for six to twelve months. 191.Hypertensive urgency is seen when blood pressure elevates > 200 systolic or > 110 diastolic without evidence of end-organ damage. 192.Hypertensive emergency is seen when blood pressure elevates > 200 systolic or > 110 diastolic in addition to evidence of end-organ damage. 193.The major risk factors for ischemic heart disease include: Diabetes, HTN, smoking, hypercholesterolemia, family history, and age. 194.The minor risk factors for ischemic heart disease include: Obesity, male sex, post-menopausal female not on HRT, and Homocystinuria. 195.The #1 most preventable risk factor for coronary artery disease is smoking. 196.Prinzmetals angina is caused by coronary artery vasospasm. 197.Normal sinus rhythm with PR interval 0.2ms is the characteristic of a 1st degree heart block. 198.When a PR interval is prolonged from beat-to-beat until it becomes so long that it drops a beat, we call it a 2nd degree, type 1 Wenckeback block. 199.When theres a fixed PR interval with regular non-conducted p-waves, which leads to a dropped beat, we call it a 2nd degree Mobitz heart block. 200.When there is no relationship between P-waves and QRS complexes, we call it a 3rd degree heart block.

201.Ventricular fibrillations usually present with syncope, severe hypotension, and even sudden death defibrillation is the best initial step in management. 202.Left-sided heart failure presents with any of the following: Paroxysmal nocturnal dyspnea, orthopnea, dyspnea on exertion, S3 on auscultation, cardiomegaly, and rales. 203.Right-sided heart failure presents with any of the following: Peripheral edema, hepatic congestion, increased JVP, fatigue, cyanosis, A-fib. 204.The first four drugs used in CHF patients are: ACEIs, Beta blockers, Furosemide, and Digoxin. 205.The main types of cardiomyopathies are: Dilated, Hypertrophic, and Restrictive. 206.Dilated cardiomyopathy is a systolic dysfunction, while restrictive cardiomyopathy is a diastolic dysfunction. 207.Common causes of restrictive cardiomyopathy include: Amyloidosis, Hemochromatosis, Sarcoidosis, Glycogen storage diseases, Scleroderma. 208.Acute endocarditis is MCC by Staph Aureus, while a sub-acute endocarditis is MCC by Viridans Group Strep. 209.The MC findings of endocarditis include: Splinter hemorrhages, Oslers nodes, Roth spots, Janeway lesions, Splenomegaly, Conjunctival petechiae, and focal neuro findings. 210.Rheumatic fever occurs after a Group A Strep infection. 211.The classic triad of pericardial effusion is: Muffled heart sounds + JVD + hypotension. 212.Pansystolic murmurs are those that cross both S1 and S2, they are: Tricuspid regurgitation, Mitral regurgitation, and VSD. 213.Right-sided murmurs generally get louder on inspiration, while left- sided murmurs generally get softer on inspiration. 214. Left-sided murmurs generally get louder on expiration, while right- sided murmurs generally get softer on inspiration.

215.Idiopathic hypertrophic subaortic stenosis is an AD disease that MC presents as death in young athletes. 216.The best initial test in detecting IHSS is the ECHO. 217.Patient with aortic regurgitation + head-bobbing is known as de Mussets sign. 218.Cyanotic congenital heart diseases are asymptomatic until the PDA closes (keep it open with prostaglandins) 219.The MC cyanotic heart disease in infants One to Twelve-months of age is Tetralogy of Fallot. 220.Tetralogy of Fallot has: Over-riding aorta, pulmonary artery stenosis, RVH, and presence of a VSD. 221.Dystrophic calcification is the MCC of aortic stenosis in the elderly. 222.Dystrophic calcification is the MCC of aortic regurgitation in the elderly. 223.The MCC of mitral stenosis is Rheumatic Fever. 224.The MCCs of mitral regurgitation are: MVP, SBE, and Collagen diseases. 225.The MCCs of tricuspid stenosis are: Rheumatic fever and carcinoid syndrome. 226.The MCC of tricuspid regurgitation is Acute endocarditis (caused by use of IV drugs). 227.Wolf-Parkinson-White syndrome is caused by an accessory pathway in the ventricles (bundle of Kent), which leads to pre-excitation of the ventricles and MC presents with palpitations. 228.The anterior lobe of the pituitary secretes: ACTH, TSH, GH, Prolactin, LH, FSH, and MSH. 229.The posterior pituitary secretes both Oxytocin and Vasopressin. 230.Prolactin can be inhibited by Dopamine (is why Psych patients taking dopamine-blockers often experience lactation). 231.The MC functional tumor of the pituitary is the Prolactinoma. 232.Gigantism is due to a GH-secreting tumor in childhood, while Acromegaly is a GH-secreting tumor in adulthood.

233.In hyperthyroidism, there is elevated T3 and T4 with a lowered level of TRH and TSH. 234.Exophthalmos is protrusion of the eye > 18mm. 235.A hot nodule is one that can take up iodine and is indicative of a functioning nodule. 236.The best step in managing the sympathetic symptoms of hyperthyroidism is a beta-1 blocker. 237.Maternal hypothyroidism leads to congenital hypothyroidism, which is known as Cretinism. 238.The MC type of thyroid cancer is Papillary. 239. The most fatal type of thyroid cancer is Anaplastic. 240.The zona glomerulosa produces aldosterone, the zona fasciculate produces cortisol, and the zona reticularis produces sex-steroids. 241.Too much Aldosterone causes Conns syndrome, while too little Aldosterone leads to adrenal insufficiency. 242.Conns syndrome shows hypernatremia and hypokalemia, in addition to metabolic alkalosis. 243.The MCC of adrenal insufficiency is a 21-beta-OH deficiency (90% of time this is the cause). 244.Excess cortisol will lead to proteolysis + gluconeogenesis. 245.Too much cortisol is known as Cushings syndrome, while too little is known as Addisons disease. 246.Pathognomonic findings of Cushings disease are: Buffalo hump, truncal obesity, and striae (abdominal MC). 247.Addisons disease is caused by an insufficiency of the adrenal gland in secreting cortisol, which leads to excessive levels of ACTH being released from the pituitary. 248.An Addisonian crisis warrants immediate IV glucocorticoids. 249.The triad of pheocromocytoma are: Palpitations, Headache, and Intermittent spikes in BP.

250.The first step in diagnosing a pheocromocytoma is to take a 24-hr urinary VMA and metanephrine level. 251.Polycythemia + elevated erythropoietin = Hypoxia or Tumor. 252.Polycythemia without erythropoietin elevation = BM problem. 253.Insulin is released from beta cells, glucagon is released from alpha cells. 254.Presence of insulin + C-peptide is indicative of endogenous insulin. 255Presence of insulin without C-peptide is indicative of exogenous insulin administration. 256.Sulfonylureas work by increasing the amount of insulin released from the beta cells of the pancreas. 257.Glucagonoma can cause up to a 1000-fold increase in serum glucagon. 258.Osteoblasts will build bone and lower serum calcium, while osteoclasts will destroy bone to increase serum calcium. 259.The superior parathyroid glands develop from the fourth pharyngeal pouch, while the inferior parathyroids develop from the third pharyngeal pouch. 260.Osteopenia is a condition of decreased bone density, osteomalacia is a nutritional deficiency leading to bone softening, and osteosclerosis is an increase in bone density due to trauma. 261.The first diagnostic test for osteoporosis is the DEXA scan. 262.Bisphosphonates help osteoporosis patients by inhibiting osteoclastic activity (ie. Mimics calcium). 263.Rickets is bone softening in children due to impaired metabolism of vitamin D, phosphate, or calcium. 264.Osteomalacia is bone softening due to defective bone mineralization secondary to inadequate amounts of available calcium and phosphate. 265.Klinefelters syndrome is an XXY disorder whereby males have: Increased height, small testes, gynecomastia, wide hips, female pubic hair pattern, low testosterone/high LH:FSH. 266.XXY syndrome presents as a male with mild mental retardation, severe acne, violent behavior (may be anti-social behavior).

267.Testicular feminization is a result of a defect on the DHT receptor. 268.A 5-alpha-reductase deficiency leads to ambiguous genitalia at birth with a masculinization of genitalia at puberty due to rise of testosterone. 269. Any patient with amenorrhea + loss of ability to smell has Kallmanns syndrome, an AD disorder caused by hypogonadism and anosmia. 270.Male and Female genital differentiation begins around the eight gestational week. 271.The mullerian ducts develop into: Ovaries, Fallopian tubes, Uterus, Cervix, and upper 1/3 of vagina. 272.The lower 2/3 of the vagina is formed from the urogenital sinus. 273.The Mesonephric Ducts develop into the: Testes, Epididymis, Vas deferens, and Seminal Vesicle. 274.The prostate, prostatic urethra, and bulbourethral glands develop from the urogenital sinus. 275.A cyst between the umbilicus and bladder is a Urachal Cyst, which presents with: Abdominal pain, dysuria, persistent umbilical discharge, and frequent UTIs. 276.Varicocele is most common in the left testicle because of the angle of drainage into the left renal vein. 277.Acetazolamide and Dorzolamide work by inhibition of Carbonic Anhydrase enzyme. 278.Loop diuretics work by inhibiting the Na/K/2Cl co-transport system in the thick ascending loo. 279.Common side-effects of loop diuretics are: Ototoxicity, Hypokalemia, Dehydration, Allergy, Nephritis and Gout. 280.Bartters syndrome is a genetic mutation that results in a non-functional triple transporter (It looks as though they are using a Loop diuretic when they are not). 281.Thiazide diuretics work by shutting down the Na/Cl co-transport in the distal convoluted tubule.

282.Common side-effects of Thiazide diuretics are: hyperglycemia, hyperlipidemia, hyperuricemia, and hypercalcemia). 283.Central diabetes insipidus is a result of lack of anti-diuretic hormone release from the posterior pituitary gland. 284.Desmopressin is the management of choice from central DI. 285.Nephrogenic diabetes insipidus is a result of a non-responsive ADH receptor on the kidney. 286.A thiazide diuretic given to a patient with nephrogenic DI will lead to a paradoxical fluid conservation. 287.The activation of renin leads to a series of events that cause the following four things to occur in hopes of increasing blood pressure: Increase circulating aldosterone, alpha-1-vasoconstriction, ADH released from posterior pituitary, thirst mechanism stimulation in the hypothalamus. 288.The side-effects of ACE Inhibitors are: Cough, Angioedema, Proteinuria, Taste disturbance, Hypotension, Pregnancy contraindication, Rash, Increased Renin, Lower A2. 289.Any patient taking an ACEI who develops a cough should be switched to an ARB. 290.The initial five steps to attacking an acid/base disturbance are: Check pH, check C02/HC03, determine whether compensation is appropriate, calculate the anion gap, check to see if HC03 change matches anion gap change. 291.Causes of increased anion gap metabolic acidosis are: Methanol, Uremia, DKA, Paraldehyde, Isoniazid, Lactate, Ethanol/Ethylene glycol, Salicylates. 292. Causes of normal anion gap metabolic acidosis are: Hyperalimentation, Acetazolamide, Renal tubular acidosis, Diarrhea, Uremia, Post-intubation hypocapnia. 293.Hyperkalemia in DKA is actually pseudohyperkalemia because there is a K+/H+ exchange in the cells. 294.Type 1 distal RTA is the MC form and is caused by a failure of the collecting ducts to secrete acid.

295.Fanconis syndrome is caused by an inability to reabsorb via the PCT, resulting in glucose, AA, uric acid, phosphate, and HC03- being passed into the urine. 296.The Gold-Standard for determining GFR and renal plasma flow is to measure the Para-Aminohippuric acid levels. 297.Oliguria is defined as urine output <400cc/day and Anuria is defined as urine output <100cc/day. 298.Pre-renal azotemia is caused by any problem inhibiting the flow of fluids into the kidneys, think: Volume depletion, cardiovascular disease, decreased oncotic pressure, renal artery stenosis, or shock. 299.Pre-renal azotemia shows a BUN:Cr >20:1. 300.Intra-renal azotemia is MCC by drugs, crystals, or crush injuries. 301.MCC of drug-induced intra-renal azotemia are: Ampho B, Cisplatin, and Aminoglycosides. 302.Intra-renal azotemia shows a BUN:Cr of <20:1. 303.For post-renal azotemia to occur, there must be a bilateral blockage (think prostate enlargement, pelvic tumors, or urethral strictures). 304. The main findings of nephritic syndromes are: HTN, macroscopic/microscopic hematuria, oliguria, and edema. 305.The main findings of nephrotic syndromes are: Proteinuria >3.5g/day, generalized edema, hypoalbuminemia, and hyperlipidemia. 306.Eosinophils in the urine should make you think of drug-induced hypersensitivity, MCC by Cephalosporin. 307.RBC casts in the urine should make you think of glomerular nephritis. 308.WBCs and bacteria in the urine should make you think of infection. 309.Crystals in the urine should have you checking birefringence (negatively birefringent = Gout). 310.Bence-Jones proteins in the urine should tip you off to Multiple Myeloma. 311.If the urine is positive for blood but negative for RBCs, look for myoglobinuria. 312.Fat casts in the urine should make you think of nephrotic syndromes.

313. Waxy casts in the urine should make you think of chronic end-stage renal failure. 314.Crescent-shaped casts in the urine should make you think of rapidly progressive glomerulonephritis, Goodpastures syndrome, and Wegeners. 315.Horse-shoe kidney gets caught under the Inferior Mesenteric Artery during development. 316.The MCC of a small kidney is renal artery stenosis. 317.We must avoid ACE inhibitors when a patient has renal artery stenosis. 318. Medullary cystic kidney disease presenting later in life is AD, while if earlier in life it is AR. 319.The three MC areas where a kidney stone might get caught are: Hilum, Mid-ureter, and Ureteropelvic junction. 320.The presentation of kidney stones is usually radiating pain from the flank, moving anteriorly and descending into the groin. 321.The MCC of kidney stones is calcium stones, which are radio-opaque on xray (MCC by hypercalcemia). 322.MCC of staghorn calculi are: Proteus, Pseudomonas, Ureaplasma, Nocardia, Cryptococcus, H. Pylori, Staph Saprophyticus, and Brucellosis. 323.Cysteine stones are caused by an AD disorder where there is a defect in the renal transport of COLA (Cysteine, Ornithine, Lysine, Arginine). 324.The MCC of pyelonephritis are E. Coli and E. Faecalis. 325.The steps in diagnosing a pyelonephritis are: 1st KUB, 2nd spiral CT, and 3rd IVP 326.The MCC of hydronephrosis in newborns is posterior urethral valves. 327.The MCC of urinary obstruction in adult males is BPH. 328.TURP syndrome is hyponatremia caused by water intoxication (due to fluid overload and absorption during the TURP procedure). 329.We do a one-time ultrasound in long-time smokers for AAA at sixty years of age. 330.The MC malignancy of the kidney is renal cell carcinoma, seen MC in male smokers who are fifty to seventy years of age.

331.The most common renal malignancy in children is Wilms tumor (MC from two to four years of age). 332.Wilms tumor is associated with WAGR (Wilms, Aniridia, GU malformations, and Retardation). 333.Urge incontinence is caused by instability of the detrusor muscle. 334.Children with urge incontinence can be managed with Imipramine (has strong anti-cholinergic properties). 335.Hypospadius is the MC GU abnormality and is associated with increased risk of UTIs. 336.Balantitis = foreskin inflammation, Phimosis = Foreskin scarring, Paraphimosis = scarring of the foreskin that adheres to the base of the penis. 337.The two MCCs of urethritis are: Chlamydia and Gonorrhea. 338.Barretts esophagus is metaplasia of the columnar epithelium into squamous epithelium in the distal esophagus (MCC by chronic GERD). 339.Barretts esophagus leads to increased risk of adenocarcinoma. 340.Mallory-Weis tears are caused by mucosal tearing at the gastroesophageal junction, while Boerhaaves syndrome is a rupture of the esophagus. 341.Plummer-Vinson syndrome presents with the triad of: Dysphagia, Glossitis, and Iron-deficiency anemia. 342.Diffuse esophageal spasm is caused by un-coordinated contraction of the entire esophageal body (presents as severe chest pain that mimics an MI). 343.A baby who becomes cyanotic when feeding should be checked for choanal atresia (one or both nasal passages are blocked by soft/body tissue). 344.Two main factors that slow gastric emptying are: Acid in the duodenum, Fats in the stomach. 345.Prostaglandins help protect the stomach and are inhibited when a patient uses aspirin. 346.Type A gastritis affects the fundus, type B gastritis affects the antrum. 347.Management for H. Pylori gastritis is: PPI + 2 Abs + Bismuth.

348.Gastric ulcer pain is worsened during a meal, while a duodenal ulcer pain is improved during a meal. 349.Surgery is warranted for peptic ulcer disease in the following situations: Intractable pain, Hemorrhage, Obstruction, Perforation. 350.The main signs/symptoms of Carcinoid syndrome are: Watery diarrhea, abdominal cramping, flushing, and bronchospasm. 351.Recurring bouts of pancreatitis-mimicking symptoms is likely a pancreatic pseudocyst. 352.Management of choice for a pancreatic pseudocyst is drainage. 353.Presence of Grey-Turner sign (flank bruising) and Cullen sign (periumbilical bruising) are caused by hemorrhagic pancreatitis. 354.Twisting of a segment of bowel around its mesentery is known as Volvulus, which shows up as a kidney bean shape on xray. 355.Treat a volvulus with a barium enema. 356.Telescoping of one segment of bowel into another is known as Intussusception (sausage-shaped mass on presentation). 357.Intussusception is treated with sigmoidoscopy and rectal tube placement. 358.Internal hemorrhoids are painless because they are above the pectinate line. 359.External hemorrhoids are painful because they are below the pectinate line. 360.The best initial management for hemorrhoids are: Sitz bath, increased dietary fiber intake, and hemorrhoidal cream. 361.Up to eight-percent of migraine sufferers have a family member who also gets them (ie. Strong genetic component). 362.Acute management of migraine headaches is with a Triptan drug (5-HT agonist leads to vasoconstriction). 363.The mildest type of headache is the tension headache, which has a band- like quality and is not debilitating.

364.Cluster headaches present as severe unilateral headaches that are localized to the temporal/periorbital area. 365.Decorticate posturing is caused by cortical lesions and result in arm flexion, while decerebrate posturing is caused by midbrain/lower lesions and results in arm extension. 366.Patients seen within three to six hours of stroke should be given tissue plasminogen activator (TPA) assuming it is an occlusive infarct. 367.Cryptococcal meningitis is the MCC of meningitis is AIDS patients. 368.MRI of a MS patient demonstrates multiple areas of focal demyelination. 369.Guillain-Barre is a rapidly ascending demyelinating disease that affects the peripheral nerves, the most worrisome complication is respiratory depression. 370.Rapidly correcting hyponatremia can lead to central pontine myelinosis. 371.Initial management of status epilepticus is benzodiazepine. 372.MRI of Alzheimers disease demonstrates diffuse cerebral atrophy. 373.AchE inhibitors can slow the progress of Alzheimers disease. 374.Picks disease looks like Alzheimers except that it mainly affects the frontal lobe and thus personality changes are more common. 375.Parkinsons disease is caused by a loss of dopaminergic neurons in the substantia nigra, and is initially managed with a combination of Carbidopa + Levedopa. 376.Diphenhydramine is the DOC for dealing with the tremor associated with Parkinsons disease. 377.Huntingtons disease is a triplet repeat disorder characterized by choreiform movements and severe clinical depression. 378.ALS patients should be managed with supportive measures (intubation, feeding tube, etc). 379.The MCCs of hypoxemia are: Shunts, V/Q mismatch, Hypoventilation, Diffuse impairment, and low inspired FI02. 380.Initial management of hypoxemia includes: Nasal 02, CPAP, intubation, and/or tracheostomy.

381.The four types of asthma are: Intermittent, mild persistent, moderate persistent, and severe persistent. 382.The initial management of mild asthma cases is a beta-2 agonist (Albuterol). 383.Any patient who uses their asthma inhaler more than once per day should also be started on an inhaled corticosteroid. 384.Ninety-five percent of pulmonary embolisms come from a DVT. 385.The three main RFs for a PE include: Stasis, Hypercoagulable state, and damage to the epithelium. 386.The best initial test to rule out a PE is a spiral CT of the chest + V/Q scan. 387. The gold-standard diagnostic test for PE is the pulmonary angiography. 388.Chronic hypoxia tends to lead to clubbing of the digits. 389.CXR of a pleural effusion demonstrates costophrenic angle blunting. 390.Acute onset of ipsilateral chest pain + decreased breath sounds is most likely a PTX. 391.Positive pressure in the pleural space will collapse the ipsilateral lung and shift the mediastinum away from the PTX (known as tension PTX). 392.Tension PTX is a medical emergency that should be managed urgently with a large-bore needle inserted into the mid-clavicular line at the 2nd-3rd intercostal space. 393.Asbestosis leads to an increased risk of bronchogenic carcinoma and malignant mesothelioma. 394.Sarcoidosis is a chronic systemic granulomatous disease that is characterized by widespread non-caseating granulomas. 395.Common findings of Sarcoidosis include: Bilateral hilar adenopathy on CXR, elevated ACE enzyme, and hypercalcemia/hypercalcuria. 396.Four drugs that commonly cause interstitial lung diseases include: Amiodarone, Bleomycin, Phenytoin, and Nitrofurantoin. 397.Ground glass or honeycombing on CXR is an indication of idiopathic pulmonary fibrosis.

398.Lung cancer is the MCC of cancer-related deaths, and is the second MC category of cancer. 399.Common findings of lung cancer are: Coughing, Hemoptysis, Hoarseness, Weight-Loss, Fatigue, Infection risk. 400.Pancoast tumor commonly leads to Horners syndrome (Ptosis, Anhydrosis, and Miosis). 401.Patients with facial swelling, cyanosis, and neck/head vein dilation should be examined for superior vena cava syndrome. 402.The best initial step in examining a lung mass is to check old CXRs. 403.Primary tuberculosis affects the lower lobes of the lung, while secondary tuberculosis affects the upper lobes (apex). 404.Diagnosis of TB is based on three things: Clinical assessment, +CXR, and + sputum sample. 405.Patients using Isoniazid commonly encounter peripheral neuropathies, which is a result of depletion of vitamin B6 (Pyridoxine). 406.Common causes of osteoporosis include: Decreased estrogen levels, lack of physical activity, hypercortisolism, calcium deficiency, and hyperthyroidism. 407.Signs of Rickets include: Craniotabes, Rachitic rosary, Harrisons groove, and Pectus carinatum. 408.Patients with bleeding gums, bleeding from hair follicles, ecchymosis, and sub-periosteal hemorrhage should be evaluated for Scurvy (vitamin C deficiency). 409.Pagets disease is managed first with Bisphosphonates and secondly with Calcitonin. 410.Calcitonin works by inhibiting calcium absorption in the intestines and inhibiting renal tubular cell reabsorption of calcium. 411.MCCs of pyogenic osteomyelitis are: S. Aureus, S. Epidermidis, and Strep species. 412.Pyogenic osteomyelitis is diagnosed by finding of periosteal elevation on xray.

413.Osteochondroma is seen in those less than twenty-five years of age, MC at the distal femur/proximal tibia. 414.Giant cell cancer of the bone is MC seen in those twenty to forty years of age, MC at the epiphyseal ends of long bones. 415.Soap Bubble sign is an x-ray finding of Giant cell bone cancer. 416.Osteosarcoma is seen MC in those ten to twenty years of age, and is the MC primary bone malignancy. Is seen at the distal femur/proximal tibia. 417.Osteosarcoma is diagnosed by finding: Increased alkaline phosphatase, Codmans triangle on x-ray, and Sun-burst sign on x-ray. 418.Ewings sarcoma is most commonly seen in those less than fifteen years of age, mostly in males. 419. Ewings sarcoma has the 11;22 translocation and shows onion skinning on x-ray. 420.Rheumatoid arthritis is best diagnosed based on clinical findings, as the lab values are not always going to be sufficient for diagnosis. 421.First-line management of RA is Methotrexate. 422.Antinuclear antibody is 98% sensitive for SLE. 423.Anti-phospholipid autoantibodies can cause false positive lab test in SLE patients and a false positive RPR/VDRL for syphilis. 424.Drug-induced lupus will show the presence of anti-histone antibody. 425.Drug-induced lupus is MCC by: Procainamide, Phenytoin, Hydralazine, INH, and Sulfonamides. 426.Sjogrens syndrome causes: Keratoconjunctivitis, Xerostomia, and Arthritis. 427.Behcets syndrome will show: Arthritis, Vasculitis, Neurologic symptoms, and painful ulcers of the oral/genital region. 428.The seronegative spondyloarthropathies include: Osteoarthritis, Ankylosing Spondylitis, Reiters syndrome, and Psoriatic Arthritis. 429.Osteoarthritis and psoriatic arthritis are the only two types that affect the DIPs.

430.Bouchards nodes affect the PIPs, while Heberdens nodes affect the DIPs. 431.X-ray of osteoarthritis demonstrates osteophytes and joint-space narrowing. 432.Ankylosing spondylitis can only be diagnosed when the SI joint is affected. 433. Ankylosing spondylitis is HLA-B27 positive. 434.Management of AS is NSAIDs and back-specific strengthening exercises. 435.Reiters syndrome is an HLA-B27+ disease with the classic triad of: Conjunctivitis, Non-gonococcal urethritis, and reactive Arthritis. 436.The pathognomonic finding of psoriatic arthritis is pitting of the nails. 437.CREST syndrome = Calcinosis, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. 438.Diagnose Scleroderma with ANA (is 95% +) and anti-centromere antibody (80% sensitive). 439.Acute management of Gout is Colchisine (is a microtubule inhibitor). 440.Long-term Gout management is with Allopurinol (inhibits xanthine oxidase). 441.Gout caused by under secretion of uric acid is managed by Probenecid. 442.Negatively birefringent crystals = Gout. Positively birefringent crystals = Pseudogout. 443.Septic arthritis is MCC by gonorrhea in sexually active patients. 444. Pain and weakness in the muscles of the shoulder-girdle is Polymyalgia Rheumatica. 445.Myasthenia Gravis is caused by autoantibodies against the post-synaptic Ach receptors. 446. Diagnosis of MG is made by administration of Edrophonium (short- acting AchE inhibitor). 447.Management of MG is with Pyridostigmine (long-acting AchE inhibitor). 448.Lambert-Eaton syndrome is caused by autoantibodies against the pre- synaptic calcium channels.

449. Lambert-Eaton syndrome may be a sequelae of small cell cancer of the lung. 450.The most important step before prescribing Retinoic acid to a female is to obtain a negative pregnancy test. 451.Infection of the skin surrounding the nail margin is known as Paronychia. 452.The MCC of Necrotizing Fasciitis is Group A Strep (pyogenes). 453.The initial step in Necrotizing Fasciitis is surgical debridement + PCN/Clindamycin to prevent spread. 454.Scarlet fever may be caused by Group A Strep (Pyogenes). 455. Findings of Scarlet Fever include: Truncal rash, Strawberry tongue, Sandpaper-like skin, hand/foot desquamation, circumoral pallor, bright-red pharynx. 456.A swollen and painful mass in the axilla or groin is likely Hidradenitis Suppurativa, which is caused by a plugged apocrine gland. 457.The management for Psoriasis is topical steroids and UVA light. 458.The MCCs of contact dermatitis are: Poison Ivy, Nickel, Poison Oak, and Chemicals. 459.Urticaria is caused by mast-cell degranulation and histamine release, and IgE-mediated process. 460.Albinism is caused by a deficiency of Tyrosinase. 461.Patients with Albinism should avoid the sun and use sunscreen at all times. 462.Mask-like hyperpigmentation of the face in pregnancy is known as Melasma. 463.MCCs of Erythema Nodosum include: Drugs (Sulfonamides, OCPs), Pregnancy, and Infections (Chlamydia, M. Leprae, Mycoplasma, Coccidioides Immitis). 464.Bronze diabetes is caused by Primary Hemochromatosis, caused by excessive iron absorption.

465.Primary hemochromatosis is associated with cardiomyopathy, pituitary disorders, and arthropathy. 466.Diagnose Primary hemochromatosis via transferrin saturation percentage, management with Phlebotomy. 467.HPV types 6 & 11 = anorectal and genital warts 468.HPV types 16, 18, 31, 33, and 35 = potential to cause cervical cancer. 469.Basal cell carcinoma is the MC skin cancer and is associated with a pearly grey ulcer-like appearance, and is classically seen on the face. 470.Squamous cell carcinoma is MC seen in the elderly and looks as ulcerated/crusty nodules seen on sun-exposed areas. 471.Malignant melanoma is MC seen in fair-skinned individuals who are overly exposed to the sun. 472.Malignant melanoma has a high risk of metastasis and the most worrisome characteristic is the depth of invasion. 473.Kaposis sarcoma is caused by HHV-8 and is seen mainly in AIDS patients. 474.Ash-leaf spots + thickening of the skin is indicative of Tuberous Sclerosis. 475.Pemphigus Vulgaris is an autoimmune disorder against the type 1 and 3 desmoglein, which destroys the connection between the epidermis and keratinocytes. 476.Blistering of sun-exposed areas without any abdominal pain is characteristic of Porphyria Cutanea Tarda, caused by deficiency of Uroporphyrinogen Decarboxylase enzyme). 477.The pathognomonic finding of Lyme disease is the bulls-eye rash, but can also diagnose with PCR for Borrelia DNA. 478.A rash that begins on the wrists and ankles and moves centrally is most likely Rocky Mountain Spotted Fever. 479.Scabies is managed with Permethrin cream 5% over the entire body. 480.Candida is managed with a Nystatin rinse. 481.Onychomycosis is managed with oral Itraconazole or Fluconazole. 482.Children with anisocoria have increased ICP until proven otherwise.

483.Amblyopia (lazy eye), is caused by weakening of CNs 3, 4, and 6. 484.Astigmatism is the MC reason for optometrist visits and is caused by abnormal curvature of the cornea. 485.A white reflex is indicative of either Cataracts or Retinoblastoma. 486.Retinoblastoma is a rare retinal cancer caused by mutated RB1 gene and is associated with the 11;22 translocation. 487.Marcus-Gunn pupil is due to a CN 2 defect that decreases pupillary reaction when exposed to light. 488.Parinauds syndrome is a bilateral paralysis of the upward gaze due to a lesion of the midbrain tectum. 489.Argyll-Robertson pupil is the pathognomonic finding for tertiary syphilis. 490.Argyll-Robertson pupil is a condition whereby the pupil constricts with accommodation but not to direct light stimulation. 491.Lens dislocation is seen MC in conditions such as Marfans, Alports, and Homocystinuria. 492.The pathognomonic finding for Wilsons disease is the Kayser-Fleischer ring. 493.Pain upon movement of the eye is known as Retrobulbar Neuritis, caused by inflammation of the optic nerve and is a common initial sign of MS. 494.Common causes of red-eye include: Conjunctivitis, hyphema, xerophthalmia, corneal abrasion, keratitis, uveitis, close-angle glaucoma. 495.Yellow vision is commonly caused by Digoxin toxicity. 496.Blue sclera is commonly seen in Marfans syndrome and Osteogenesis imperfect. 497.Painless loss of vision is caused MC by macular degeneration and is age- related. 498.Definitive management of macular degeneration is laser therapy. 499.Patient who describes a curtain coming down over their eye is experiencing retinal detachment, which must be managed with surgical re- attachment.

500.Cherry-red spots on the macula are caused by: Tay-Sachs disease, Niemann-Pick disease, and central retinal artery occlusion.