NBME CBSE STUDY GUIDE/ TEST BANK

NBME CBSE STUDY GUIDE/ TEST BANK Type II pneumocytes surfactant (lecithin) Proliferate after injury Type I progenitors Neonatal Respiratory Distress Syndrome Polio live v killed vaccine Killed = Salk = IgG Live = Sabin = IgG + IgA - can be shed in feces Neonatal Respiratory Distress: Etiology + Tx Maternal DM (high insulin) or C-section (low cortisol) TX: dexamethasone before birth Lung maturity determined with Amniocentesis of Phospholipids (*type II pneumocytes) L S Type I pneumocytes Squamous gas diffusion Elastase in lungs macrophage: lysosomes PMN: azuronphilic granules Elastin stretches and recoils due to Lysine interchain crosslinks air pressure and intrapleural pressure at FRC Air pressure = 0 Intrapleural pressure = -5 Pulm Vasc Resistance is lowest during Exhale of Tidal Volume Lung Compliance is decreased by LHF, pulmonary edema, pulmonary fibrosis Lung Compliance is increased by emphysema, age Obesity affects ERV and FRC DECREASE ERV & FRC Blood flow/min (pulmonary v systemic) pulmonary = systemic Anatomic pulmonary shunting Bronchial circulation causes decreased PO2 in LA/LV than in pulmonary capillaries More ventilation is at the BASE O2-Hgb dissociation LEFT shift basic, cold, low 2,3 BPG low pO2 (compensatory erythrocytosis) O2-Hgb dissociation RIGHT shift low pH, high 2,3BPG, high T HOT, ACIDIC CO2 transport to lungs carbonic anhydrase Cl shift Haldane: CO2 released to lung (Bohr: O2 release to tissue) CO poisoning causes carboxyhemoglobin no affect on PaO2 Cyanide poisoning causes lactic acidosis How to treat cyanide poisoning Amyl nitrite -- Methemoglobin THEN Thiosulfate (hydroxycobalamin) Normal A-a gradient 5-15 Hypoventilation: Heroin OD or high altitude Increased A-a gradient Diffusion impairment (fibrosis) R-L shunt (aspiration, ARDS) V/Q mismatch (pulmonary edema AT -- AT II where and how ACE (- high in sarcoidosis) In small pulmonary bV C5a induces what PMN influx (ie: in lungs) Korotkoff sound BP cuff - appear and disappear in inflation/deflation Pulsus Paradoxus 10mmHg difference in Korotkoff sound Pulsus Paradoxus occurs in Cardiac Tamponade Kussmaul sign JVP rises during inspiration Constrictive Pericardiditis Restrictive/Interstitial Lung Disease: A-a, FVC, FEV1, EFR Airway widening due to radial traction from fibrosis increase Aa decreased FVC & FEV1 Increased EFR Sarcoidosis Th1 noncaseating granulmona bilateral hilar adenopathy increased ACE increased IL2, IFNg 1-a-hydroxylase in macrophages: vit D -- HyperCa Hyper Ca causes stones, thrones, groans, psych overtones 1-a-hydroxylase in macrophages PTH independent conversion of Calcifediol to calcitriol (bioactive Vit D) Vit D -- Hyper Ca Idiopathic pulmonary fibrosis Honeycomb pattern loss of Type 1 pneumocytes hyperplasia Type II pneumocytes Goodpasture HS II Auto-Ab against BM destroys lung alveoli (restrictive) and renal glomeruli Obstructive Lung Disease DECREASED FEV1, Decreased FVC increased RV, FRC, TLC **different shape COPD PMN, mo, CD8 V/Q mismatch: O2 induced hypercapnia; physio dead space Myeloperoxidase causes Green sputum/pus Do not give O2 supplement to COPD patient Decreased stimulation of carotid bodies = decreased RR TX COPD with Fluticasone (glucocorticoid) inhibit cellular reaction a1-antitrypsin deficiency Serine protease inhibitor LIVER LUNG: inc PMN elastase -- emphysema Asthma dx Methacholine (maCh) challenge = induce bronchoconstriction to reduce FEV1 + test = Airways ARE reactive B2 agonist MOA B2 (Gs) -- AC -- increase cAMP Corticosteroid MOA inhibit cytokine synthesis suppress T lymphocyte mACh Antagonist ("tropium") MOA inhibit Vagal via ACh -- decreased Ca OSA causes pulmonary HTN and RHF increases EPO which worsens HTN EPO can do what on Cardiovascular worsen HTN Pulmonary Arterial HTN BMPR2 High endothelin, Low NO SMC hypertophy, fibrosis, narrow lumen P2 louder than A2 When is P2 louder than A2 Pulmonary Artherial Hypertension TX pulmonary arterial hypertension Endothelin-R antagonist: - Bosentan, Ambisentan PGEi (inc cGMP): - Sildenafil Pulmonary Embolism perfusion defect (V/Q mismatch) sudden SOB + calf swelling Hypoxemia -- Hyperventilate -- Respiratory Alkalosis -- Metabolic compensation in 2 days dx pulmonary embolism D-dimer test CT angiogram Lines of Zahn Homan's sign (DVT calf pain on dorsiflex) TX pulmonary embolism Heparin/LMWH THEN Warfarin Fat embolism syndrome Long bone/pelvic fracture -- neuro, hypoxemia, rash Fat microglobules in pulmonary arterioles Spontaenous pneumothorax nontraumatic rupture of subpleural blebs **20 yo thin TALL man who smokes DECREASED PRELOAD Tension pneumothorax Treachea deviates REQUIRES INTUBATION ARDS bilateral infiltrate **PANCREATITIS RISK 1. EXUDATIVE (capillary permeability) 2. Proliferative (collagen) 3. Fibrotic (pulmonary fiborsis + HTN) What are the risks from ARDS Sepsis Pancreatitis Pneumo cystic fibrosis genetics dF508 frameshift CFT protein - post-tln HypoNa Cystic Fibrosis complications Dec Vit A -- Pancreatic (squamous metaplasia) Def Vit E -- Neuromuscular, hemolytic anemia Def Vit K -- Intracranial hemorrhage Meconium Ileus; No Vas deferns; Digital clubbing DEATH FROM PNEUMO Hemorrhagic infarct is what color and why RED Dual blood supply Empyema Infected exudative pleural effusion Meniscus opacity Increased LDH COMPLICATES PNEUMO Lobar pneumonia consolidation Ex: strep, legionella -- Red (3-4 d) -- Grey hepatization (5-7d) -- Resolution (Type II regen in 8 d) Broncho Pneumonia Patchy ex: Staph, Strep, Kleb, H flu Interstitial Pneumonia Alveolar walls Ex: Mycoplasma, Chlamydia, Legionella, RSV/CMV/flu/adeno Tuberculosis Th1: IFNg -- activates Macrophage (CD14): TNF-a Lung Harmatoma hyaline cartilage, fat, SMC lined by respiratory pneumoepithelium Pancoast syndrome NSCLC at lung apex (superior sulcus) compression of brachial plexus -- Shoulder pain, Horner, Upper edema, paraplegia Asbestos #1 cancer Bronchiogenic carcinoma (Adenocarcinoma in situ) Asbestos change in lung pleural thickening, calcifications of posterolateral midlung and diaphragm Mesothelioma Asbestos Calcifcations, spindle cells, cytokeratins, calretinin Bronchioalveolar carcinoma is also known as Adenocarcinoma in situ Does Bronchioalveolar carcinoma have a good px YES even though malignant #1 lung cancer Adenocarcinoma Bronchiolaveolar carcinoma characteristics thickened columnar cells along alveolar walls Bronchiolaveolar carcinoma genes KRAS EGFR ALK SCC in lungs characteristics keratin pearls hilar mass HyperCa -- PTHrP (stones, bones, groans, psych overtones) SCLC neuroendocrine (*neural cell adhesion molecule) HORMONES: ACTH, ADH, Lambert Eaton Acute transplant rejection in lungs perivascular mononuclear infiltrates in smalll bv Chronic transplant rejection in lungs Bronchiolitis obliterans in small airways Gastroschisis not covered by periotoneum poor GI function Omphalocele Midline herniation covered by periotoneum normal GI function *Trisomy 13/18, CL/P Diverticulitis outpouching of mucosa + submucosa **Dysphagia most diverticulitis are FALSE (pulsion, Zenker) Meckel Diverticulum TRUE = traction bulge at ileum Mallory Weiss Mucosal tears due to intraabdominal pressure Boerhaave Transmural distal rupture due to increased intraabdominal pressure Esophagel cancer in upper 2/3 SCC Esophageal cancer in lower 1/3 Adenocarcinoma Splenic vein thrombosis causes gastric varices where fundus esophageal dysfunction can be due to enlarged left atrium GERD - what happens to LES tone DECREASED LES tone Achalasia - what happens to LES tone INCREASED LES tone Krukenberg tumor gastric cancer signet ring cells: mucin displaces nucleus **spreads to ovary Zollinger-Ellison tumor Gastrin secreting causes PUD dx: increased secretin Vipoma watery diarrhea + hypoK tx: octreotide Carcinoid syndrome intestinal tumor (*enterochromaffin) -- 5HT, Bradykinin, PGE watery diarrhea telangiectasis Bronchospasm **RIGHT VALVULAR FIBROUS PLAQUES METASTASIS Levels increased in Carcinoid syndrome 5-HIAA in urine high histamine, VIP 5HT, Bradykinin, PGE **LOW NIACIN Tx Carcinoid syndrome Octreotide (somatostatin w longer t1/2) Gastrin is from G cells Gastrin causes mucosal growth H secretion from parietal Gastrin released due to Vagal (GRP) Phenylalanine Distension Secretin is from S cells Secretin causes HCO3 = increase pH (pancreas) Bile excretion Secretin is released due to low pH fatty acid Pepsin is from Chief cells Pepsin is released due to Vagal (ACh) low pH (digest protein) Gastric acid is from Parietal cells Gastric acid is released due to Gastrin: CCKb-R -- ECL/mast -- histamine (cAMP) Vagal (ACh) Somatostatin is from D cells (INHIBITS gastric acid & pancreatic/gb contraction) Somatostatin action Inhibit gastric acid and pancreatic/gb contraction Somatostatin is released due to Low pH, Vagal (inhibit gastric acid & panc/gb contraction) Cholecystokinin is from I cells (duodenum) Cholecystokinin causes pancreatic/gb contraction CCK is released due to fatty acids and aa VIP causes increase pH via H2O/e excretion Relax sphincter NO in GI causes relaxation of LES Ghrelin causes increased appetitie GIP/GLP is released from L cells GIP/GLP causes Dexcrease H Increase insulin Acute gastritis is caused by NSAID (low PGE2 in PMN) Burns = Curling (hypovolemic) Brain = Cushing (high Vagal, ACh) H pylori gastritis is located antrum H pylori gastritis increases risk for MALT or adenocarcinoma H pylori gastritis histo patchy infiltrate + multifocal atrophy Autoimmune gastritis is located body/fundus Autoimmune gastritis can risk pernicious anemia H pylori duodenal PUD tx (use 2): Amoxicillin / clarithromycin / metronidazole / tetracycline + PPI (omeprazole) MOA of Bismuth, Sucralfate, Misoprostol Bind ulcer base PGE-i tx: traveler's diarrhea "-tidine" H2 antagonist decrease H from parietal H2 antagonist "-tidine" decrease H from parietal Cimetidine AE p450 inhibitor increase AST/ALT/Creatinine gynecomastia (H2 antagonist = "tidine") "prazole" PPI inhibit H/K ATPase in parietal cell PPI "prazole" inhibit H/K ATPase in parietal cell PPI AE C dif Nephritis decrease: Mg, Ca, B12, Fe, Vit C Metoclopramide D2-R agonist inhibit ACh MOTILITY tx: GERD, Gastroparesis Loperamide Mu agonist without CNS penetration SLOW motility Enteric bacteria produce what vitamins Vit K and Folate Watershed areas in GI Rectosigmoid + Splenic flexure Fat malabsorption stain with Sudan III What is absorbed at brush border D-xylose and lactose Duodenal atresia is associated with Down Syndrome Duodenal atresia failure of recanalization Double bubble (tri21) Jejunum/Ileum atresia Vascular occlusion Apple peel Jejunum/Ileum atresia example Gastrocschesis Colonic Atresia example Hirschsprung Colonic Atresia submucosal of RECTUM = Meissner plexus absent Abetalipoproteinemia foamy cytoplasm at villi tips NO APO-B - No VLDL or Chylomicron Mesenteric Ischemia SMA (A fib embolus) -- Extreme pain = Food aversion Lactose intolerance low stool pH high Breath H high Stool osmolality Celiac - genes Gliadin HS IV HLA-DQ2/8 Anti-tTG (IgA) Antiendomysial Celiac malabsorption complication Malabsorption of vit D in duodenum decreased Ca/PO4 high PTH -- Osteomalacia/rickets Celiac association Dermatitis Herpetiformis Dermatitis herpetiformis IgA in dermal papilla (Celiac) Crohns ASCA Fistula; Transmural thickening Non-caseating granuloma Bile acid malabsorption (*TERMINAL ILEUM) Crohns complications Ileum resection causes B12 deficiecny Crohn tx Corticosteroids Sulfasalazine (*act by bacteria) Ulcerative Colitis pANCA pseudopolyps crypt abscess friable ulcers CONTINUOUS FROM RECTUM Ulcerative Colitis tx Mesalamine (NSAID) + resection Colon Cancer most commonly where Rectosigmoid Ascending Descending Familial adenomatous polyposis 2 hit chromo instability APC (adenoma) KRAS (size) p53 (carcinoma KRAS oncogene resistant to anti-EGFR -- constitutive active RAS (which normally requires GTP) RAS normally requires what to be activated GTP Colitis becomes carcinoma how multifocal poorly differentiated tumors early p53/late APC HNPCC/Lynch microsatellite instability MSH2/MLH1 = mismatch repair HEREDITARY colorectal, endometrial, ovarian LN drainage proximal to dentate (UPPER RECTUM) Inferior Mesenteric Internal Iliac LN drainage Distal to dentate (ANUS) Inguinal Hemorrhoids - internal above dentate PAINLESS superior rectal -- inferior mesenteric v -- PORTAL Hemorrhoid - External below dentate PAINFUL Inferior rectal -- internal pudendal = IVC Indirect hernia is common in child Indirect hernia is a fluctuant hydrocele due to patent processus vaginalis Indirect hernia is located through inguinal ring into scrotum LATERAL to epigastrics Direct hernia is due to weakened transversalis fascia Direct hernia is located Hesselbach triangle MEDIAL to epigastrics Femoral hernia is common in female What becomes the main pancreatic duct Ventral pancreatic bud Annular pancreas is caused from abnormal ventral pancreatic bud MIGRATION Encircles duodenum Vit a deficiency caused from squamous metaplasia of pancreatic ducts OR biliary obstruction What activates trypsin Enteropeptidase Pancreas K Channels are sensitive to what ATP Epi does what to insulin a2 = inhibit insulin B2 = stimulate insulin Acute pancreatitis is due to **Gallstones **Alcoholism HyperTAG (tx: Niacin) HyperCa Drugs Surgery Infection CF IgG4 Alcoholic Pancreatitis 2 AST:ALT no gallstones high MCV Acute Pancreatitis causes what deficiency Trypsin autodigestion -- Macrocytosis (high MCV) = folate deficient Chronic Alcoholic Pancreatitis Calcification Ductal plugs Steatorrhea DM Pancreatic adenocarcinomia CA19-9 Anorexia Obstructive Jaundice (high bili, dark urine/pale stools) Migratory thrombophlebitis What are risks to getting Pancreatic Adenocarcinoma KRAS, SMAD4 65 Smoking Peutz-Jeghers, MEN, HNPCC tx of sarcoidosis corticosteroids Where is Fe homeostasis liver AST v ALT usually ALT AST Alcoholic = AST ALK (2x) Alk phos is elevated when biliary issues GGTP is increased in what disease? Cholestasis due to alcohol (*liver and pancreas) cirrhosis prognosis determined by prolonged PT and albumin lipophilic drugs are metabolized in liver Vit A toxicity cirrhosis, papilledema, dry skin, alopecia fatty streaks are caused by MACROPHAGES in bV -- foam cells CCl4 causes ROS injury (lipid peroxidation) = liver fatty change/necrosis Hepatic steatosis process Alcohol -- high NADH = low FFA Box = high TAG A1 antitrypsin deficiency in liver swelling, poor feeding, PAS+ Hemolytic disease of newborn nucleated RBC extramedullary hematopoeisis Wilson's Disease AR - ATP7B decreased Copper metabolism = low ceruloplasmin, no Cu in bile high transaminase = ROS to hepatocytes -- hemolytic anemia Features of Wilson's Disease Cornea Kayser Fleisher Ring Basal ganglia atrophy = Parkinson/impulsive Tx Wilson's Disease D-penicillamine + Zinc LPL deficiecny increased TAG Acute pancreatitis Lipemia retinalis (milky) dx LPL deficiency heparin Xanthelasma lipid histiocytes in dermis + mo -- FOAMY cytoplasm **Achilles tendon G1P -- UDP-glucose -- Glycogen -- G1P Glycogen -- G1P - Pompe (Acid a-glucosidase) -McArdle (glycogen phosphorylase) - Cori (debranching) Pompe Acid a-glucosidase (glycogen -- G1P) cardiomegaly hypotonia McArdle Glycogen phosphorylase (glycogen -- G1P) exercise intolerance (arrhythmia, m cramps) Cori Debranching (glycogen--G1P) hypoglycemia; Hepatomegaly; Ketoacidosis increased limit dextrin Reye VZV/flu + ASPIRIN microvesicular steatosis encephalopathy NH4 damage mt Hemochromatosis AR - HFE inactive transferrin-R -- GI absorbes FE -- HEMOSIDERIN Black liver, Blue on stain Hemochromatosis complications HCC, DM, Pigment, Hypogonad, Arrhythmia, Dilated cardiomyopathy, MCP arthropathy Hepatic encephalopathy CAUSE: HCV + GI bleed (N absorption) high NH3 = high GLUTAMINE ATAXIA Tx of hepatic encephalopathy Lactulose - decrease GI pH, increase NH4 Rifaximin - decrease synth/abs of NH3 - Metabolized by CYP3A4 HAV Self limiting Ballooning degeneration Monocellular infiltrates Eosinophilic apoptosis Portal vein thrombosis causes portal hypertension esophageal varices large spleen Portosystemic anastomoses PORTAL // IVC -Left gastric // esophageal -Paraumbilical // superficial, inferior epigastric ...........................................--internal iliac -superior rectal // middle, inferior rectal --IMV--Splenic..........--internal iliac CD31 PECAM-1 CD31/PECAM1 vascular endothelial tumor LIVER ANGIOSARCOMA liver angiosarcoma caused by what toxins arsenic and polyvinyl Cl Aspergillus aflatoxin induces what mutation that causes what in the liver p53 mutation -- HCC bile rate limiting step cholesterol 7a-hydroxylase requires NADPH (inhibited by fibrates) what "cofactor" does bile require NADPH What starts the process of bilirubin syntehsis heme Bilirubin synthesis heme -- biliverdin -- unconj -- conj -- unconj -- urobilinogen Unconj bili to Conj bili UGT (UDP glycoronsyl transferase) UGT low in Gilbert: jaundice w fasting/exercise Crigler Najjar (none): kernicterus Crigler Najjar no UGT = death from kernicterus (super high unconj) Gilbert low UGT = high unconj jaundice w fasting/exercise Conj bili to Unconj bili B-glucuronidase high conj Dubin Johnson + Rotor Cholestasis / Obstruction high unconj Hemolysis Gilbert Crigler Najjar Dubin Johnson + Rotor obstruction (PBS, PBC) = high conj BLACK liver PBC FEMALE lymphocytes destroy intralobar ducts (granulomas) ITCHING PBC dx high AlkPhos +anti-mt IgM tx PBC UDC acid to replace BA PSC MALE onion skin beading of intra+extralobar ducts CANCER assoc PSC dx pANCA IgM Biliary atresia 2 yo high GGT obstruct extrahepatic = high DIRECT dark urine, pale stool, jaundice Gallbladder hypomobility causes biliary sludge Cholelithiasis LUCENT = high Chol OPAQUE = high bilirubin low BA low phosphatidylcholine cholelithiasis dissolves with Bile Acid (ursoeoxycholic acid) pigment cholelithiasis (what increases risk?) UNCONJ - not visualized on scan Forty, Fat, Female, Fertile Female: E = chol synth Fertile: P inhibit gb empty low B-glucoronidase brown pigment stones assoc w E coli + Helminths Cholecystitis located cystic duct Colic cholelithiasis located at neck Acalculous cholecystitis inflammation, no stone in SICK pt (sepsis) Gallstone ileus SI obstruction w air in gb and biliary tree prolonged obstruction of gb causes fat sol vit deficiency (ex: vit A) porcelain gb has a risk for adenocarcinoma avoid WHAT in GB disease fibrates (inhibit chol-7a-hydroxylase) causes stones Cholestyramine BA binding low LDL high TAG Fibrates inhibit chol-7a-hydroxylase do not take w statin OR if GB disease Ezetimibe inhibit cholesterol absorption (via inhibiting NPC1L1) increase LDL-R Statin HMG-CoA reductase inhibitor increase LDL-R density Help HEART statin AE renal failure if taken w P450 inhibitor Niacin release PGE2/D2 increase HDL Niacin AE skin flushing PBC looks morphologically similar to GvHD The ureteric bud forms Collecting tubules Major and minor calyces Renal pelvis Ureters the Metanephric Mesoderm forms Glomeruli to DCT a horseshoe kidney is due to