NBME CBSE Latest Updated 2022, graded A+

NBME CBSE Latest Updated 2022 Bulbus cordis Correct Answer-Smooth parts (outflow tract) of left and right ventricles endocardial cushions Correct Answer-Atrial septum, membranous interventricular septum; AV and semilu ...

NBME CBSE Latest Updated 2022 Bulbus cordis Correct Answer-Smooth parts (outflow tract) of left and right ventricles endocardial cushions Correct Answer-Atrial septum, membranous interventricular septum; AV and semilunar valves neural crest left horn of the sinus venosus Correct Answer-coronary sinus posterior, sub cardinal, and supra cardinal veins Correct Answer-IVC Right common cardinal vein and right anterior cardinal vein Correct Answer-SVC Right horn of sinus venosus Correct Answer-Smooth part of right atrium (sinus venarum) Patent foramen ovale Correct Answer-failure of septum primum and septum secundum to fuse after birthTransposition of the great vessels Tetralogy of Fallot Persistent truncus arteriosus Correct Answer-Conotruncal abnormalities associated with failure of neural crest cells to migrate ductus venosus Correct Answer-connects the umbilical vein to the inferior vena cava, bypassing the liver becomes ligamentum venosum phrenic nerve Correct Answer-innervates the diaphragm and pericardium S3 heart sound Correct Answer-Increased ventricular filling pressure (e.g., mitral regurgitation, HF), common in dilated ventricles normal in kids and pregnant women S4 heart sound Correct Answer-atrial kick late diastole, right before S1 best heard at apex in LLD positionHigh atrial pressure. Stiff/hypertrophic ventricle (aortic stenosis, restrictive cardiomyopathy) Always abnormal atria contract Correct Answer-a wave of JVP c wave Correct Answer-RV contraction (closed tricuspid valve bulging into atrium) wave of JVP x descent Correct Answer-JVP wave corresponding to downward displacement of closed tricuspid valve during rapid ventricular ejection phase reduced or absent in tricuspid regurge V wave Correct Answer-JVP wave corresponding to inc'd RA pressure due to filling against closed tricuspid valve y descent Correct Answer-JVP wave corresponding to RA emptying into RVabsent in cardiac tamponade plusus parvus et tardus Correct Answer-pulses are weak with delayed peak Aortic stenosis PR interval Correct Answer-0.12-0.20 seconds 120 milliseconds QT interval length Correct Answer-9 - 11 squares = .36 to .44 seconds Hypokalemia Correct Answer-U wave present on ECG Mg sulfate Correct Answer-for torsades de pointe, hypokalemia (can lengthen QT and cause torsades), and pre-eclampsia (prevent seizures) Romano-Ward syndrome Correct Answer--Congenital long QT syndrome -Autosomal dominant, pure cardiac phenotype (no deafness).Jervell and Lange-Nielsen syndrome Correct Answer--Congenital long QT syndrome -Autosomal recessive, sensorineural deafness Brugada syndrome Correct Answer--Autosomal dominant disorder affecting Na channels most common in Asian males. -ECG pattern of pseudo-right bundle branch block and ST elevations in V1-V3 (anterior ventricular septum) -inc risk of ventricular tachyarrhythmias and sudden cardiac deatgh Prevent SCD with implantable cardioverter-defibrillator (ICD). Wolff-Parkinson-White Syndrome Correct Answer-Most common type of ventriuclar pre-excitation sydnrome. Abnormal fast accessory conduction pathway from atria to venricle bypasses the rateslowing AV node causing a delta wave and widening QRS with shortened PR interval. Could lead to a reentrant circuit and suprvaventicular tachy. First degree AV block Correct Answer-- PRI >5 boxes/.20 sec (200 msec) - Fixed but prolonged PRI (consistent but long) - normally get bradycardia heresecond degree AV block mobitz type 2 Correct Answer--PR interval is constant -atrial conduction to ventricle is intermittent: dropped QRS without increasing PR interval length -disease below AV node in His bundle may progress to 3rd degree/complete AV block Second Degree AV Block Mobitz Type 1 (wenckebach) Correct Answer-Progressive lengthening of pr interval leading to dropped QRS third degree AV block Correct Answer-The atria and Ventricles are totally dissociated. -So, the QRSs and the P waves have no relation to each other. PCWP Correct Answer-4-12 mmHg est of LA pressure Williams Syndrome Correct Answer-a genetic condition characterized by mental retardation in most regards but surprisingly good use of language relative to their other abilities, elfin facies Chromosome 7 assoc with supravalvular aortic stenosisDiGeorge Syndrome Correct Answer-Maldevelopment of 3 and 4 pharyngeal pouches, fascial dysmorphia, cardiac shunt (trunks arteriosus, tetralogy of Fallot), lack of T-cells, undeveloped paracortex Corneal arcus Correct Answer-Lipid deposits in the cornea. Common in the elderly, but appears earlier in life with hypercholesterolemia Stanford A aortic dissection Correct Answer-Dissection of the ascending aorta Tx with surgery Stanford B aortic dissection Correct Answer-Dissection of the descending aorta below the level o the left subclavian artery Tx: Beta Blockers then vasodilators Left bundle branch block Correct Answer-QRS> 120 msec Deep, broad S waves in V1 and V2 Broad R waves in in V5 and V6 hypertrophic obstructive cardiomyopathy Correct Answer-common AD inherited heart defect of a thick septal wallmutations in sarcomeric proteins (myosin binding protein C; Beta myosin heavy chain) sudden death in young athletes Loffler endocarditis Correct Answer-assoc w hypereosinophilic syndrome; histology shows eosinophilic infiltrates in myocardium cause restrictive/infiltrative cardiomyopathy with low voltage ECG Endocardial fibroelastosis Correct Answer-Dense layer of fibrosis and elastic tissue in the endocardium; cause of restrictive cardiomyopathy in children low voltage ECG Culture negative bacterial endocarditis Correct Answer-Coxiella Bartonella HACEK (Haemophilus, Aggregatibacter/Actinobacillus, Cardiobacterium, Eikenella, Kingella) Erythema marginatum Correct Answer-rheumatic feverErythema multiforme Correct Answer-skin disorder resulting from a generalized allergic reaction to an illness, infection, or medication Erythema migrans Correct Answer-Lyme disease pulsus paradoxus Correct Answer-beats have weaker amplitude with respiratory inspiration, stronger with expiration seen in cardiac tamponade, asthma, obstructive sleep apnea, pericarditis, croup Polyarteritis nodosa Correct Answer-affects multiple medium-sized arteries EXCEPT PULMONARY Transmural inflammation with fibrinoid necrosis assoc with Hep B Bechet's syndrome Correct Answer-recurrent oral ulcers, genital ulcers, uveitis immune complex vasculitis, assoc with HLA-B51cutaneous small vessel vasculitis Correct Answer-Occurs 7-10 days after certain medication (pen, cephalosporins, phenytoin, allopurinol) or infection (HCV,HIV). Palpable purpura, no visceral involvement Immune complex mediated leukocytoclastic vasculitis mixed cryoglobulinemia Correct Answer-Often due to viral infection, especially HCV Triad of palpable purpura, weakness, arthralgia. May also have peripheral neuropathy and renal disease. Mixed IgG and IgA immune complex deposition vasculitis Cardiac myxoma Correct Answer-benign tumor left atrium; embolization; syncope Gelatinous Hear early diastolic "tumor plop" Produce IL-6 leading to constitutional symptoms of fever or weight loss Kussmaul sign Correct Answer-Inc in JVP on inspiration Seen in constrictive pericarditis, restrictive cardiomyopathies, right atrial or ventricular tumorsOsler-Weber-Rendu syndrome/Hereditary hemorrhagic telangiectasia Correct Answer-AD disorder of blood vessels Telangiectasia on skin and mucous membranes, recurrent epistaxis, skin discoloration, AV malformations, GI bleeding, hematuria paroxysmal atrial fibrillation Correct Answer-"holiday heart syndrome" subsides in ~7 days See irregular RR intervals, no discernible P waves, and tachycardia with narrow QRS Friedrich's ataxia Correct Answer-GAA trinucleotide repeat expansion on chromosome 9 results in defective frataxin production, which is involved in the synthesis of iron-sulfur clusters, in the mitochondrial respiratory chain. A deficiency of the protein causes iron to accumulate within cells, including in the heart, resulting hypertrophic cardiomyopathy, in pancreatic islets, causing diabetes, and in the spinal cord, causing neurologic dysfunction. Gs protein coupled receptor Correct Answer-inc's cAMP and activate protein kinase A B1, B2, B3 D1 H2V2 Occlusion of the paramedic branches of the anterior spinal artery and/or vertebral a. Correct AnswerMedial medullary syndrome Infarct of corticospinal tract: Contralateral hemiparesis; medial lemniscus: contralateral loss of proprioception; hypoglossal nucleus: ipsilateral tongue deviation Retroperitoneal fibrosis/Ormond's disease Correct Answer-rare disease of unknown etiology, characterized by inflammation and fibrosisof the retroperitoneum resulting in compression and encasement of the ureter, and/or the retroperitoneal blood vessels Contrast CTis the diagnostic test of choice and reveals a retroperitonealmass encasing and obstructing the ureters and/or the aorta and IVC Physiologic dead space (Vd) Correct Answer-=(PaCO2-PeCO2)/PaCO2 apprx equal to anatomic dead space in healthy lungs Alveolar ventilation Correct Answer-the amount of air that reaches the alveoli each minute=(Tidal vol - Physiologic dead space) x RR atmospheric pressure, negative Correct Answer-At FRC, airway and alveolar pressures equal ___________________ (called zero), and intrapleural pressure is _________. Normal A-a gradient Correct Answer-10-15 mmHg (age/4)+4 Alveolar Gas Equation (PAO2) Correct Answer-= PIO2 - (PaCO2 / R) inspired O2 arterial O2 Resp Quotient = CO2 produced/O2 consumed @sea level = 150mmHg - (PaCO2/0.8) Edwards syndrome (trisomy 18) Correct Answer-have small jaws, small eyes, and malfored low set ears. They also have rocker bottom feet. But the key buzzword is clenched hands with overlapping fingers. Patau syndrome (trisomy 13) Correct Answer-Infant with cleft lip/palate, microcephaly or holoprosencephaly, polydactyly, cutis aplasia Thromboangiitis obliterans (Buerger disease) Correct Answer-Vasculitis with segmental thrombosing inflammation with sparing of the internal elastic lamina Meyer loop Correct Answer-Optic fibers of the geniculocalcarine tract radiating through the anterior and lateral temporal lobe before reaching the occipital cortex. The involvement of these fibers results in upper quadrantanopsia. Superior nasal meatus Correct Answer-Drains the sphenoid and posterior ethmoid sinus Middle nasal meatus Correct Answer-Drains the frontal, maxillary, and anterior ethmoid Inferior nasal meatus Correct Answer-drains nasolacrimal duct Moraxella catarrhalis Correct Answer-fastidious, nonmotile, Gram-negative, aerobic, oxidase-positive diplococcus that can cause infections of the respiratory system, middle ear, eye, central nervous system, and joints of humans.Hamman's sign Correct Answer-Crepitus/Crunching sound on auscultation=> subcutaneous emphysema, pneumomediastinum a1-hydroxylase activity of macrophages Correct Answer-Is responsible for the hypercalcemia of sarcoidosis Caplan syndrome Correct Answer-pneumoconiosis with intrapulmonary nodules + rheumatoid arthritis asbestos coal silica Mesothelioma Correct Answer-Calretinin + staining Psammoma bodies Neimann-Pick disease Correct Answer-hepatosplenomegaly, cherry-red macular spots, and psychomotor retardation, liver biopsy findings show foam cells due to accumulation of sphingomyelin primarily in lysosomes of the reticuloendothelial system and CNS normal development until approximately one yearTay-Sachs disease Correct Answer-AR disorder of hexosaminidase A Accumulate GM2 ganglioside, onion skin appearance of lysosomes with whorled membranes Cherry red spot, developmental delay and death in early childhood NO HEPATOMEGALY, no recurrent pneumonia Fabry disease Correct Answer-Accumulation of ceramide trihexoside The only X-linked recessive sphingolipidosis (more likely in males) and typically includes involvement of the eyes (e.g., cataracts, corneal clouding). No cherry-red macular spots, hepatosplenomegaly, or recurrent pneumonia Furthermore, clinical features usually only start to develop during late childhood or early adolescence. Gaucher cells Correct Answer-lipid laden macrophages resembling crumpled tissue paper, accumulation of glucocerebroside Krabbe disease (globoid cell leukodystrophy) Correct Answer-rapidly progressive, fatal, autosomal recessive neurologic disorder caused by deficiency of beta-galactocerebrosidase; appears in infancy and is characterized by "globoid cells" in the white matter (macrophages containing undigested galactosylceramide) Pi Z variant Correct Answer-glutamic acid to lysine substitution at position 342 of a1-AT gene on chrom 14Riboflavin (B2) deficiency Correct Answer-assessed by measuring the activity coefficient of erythrocyte glutathione reductase increase in activity upon addition of deficient vitamin indicates a large amount of unsaturated enzyme due to deficiency Sx: seborrheic dermatitis (erythematous rash with fine yellow scales), pharyngeal hyperemia, glossitis, cheilitis, angular stomatitis, and normocytic-normochromic anemia Friedriech ataxia Correct Answer-presents with progressive bilateral limb ataxia and weakness, loss of deep tendon reflexes, pallhypesthesia, dysarthria, and skeletal deformities, Pes cavus (inverted feet), hammertoes, and kyphoscoliosis are typical skeletal deformities seen most common cause of death is heart failure due to hypertrophic cardiomyopathy or arrhythmia Icatibant Correct Answer-antagonizing the bradykinin-B2-receptor Tx of hereditary angioedema Ecallantide Correct Answer-kallekrein inhibitor; decreases conversion of kininogen to bradykinin Tx of hereditary angioedemaWinter formula Correct Answer-expected respiratory compensation in patients with metabolic acidosis. Expected pCO₂ (in mm Hg) = (1.5 x HCO3-) + 8 (+/- 2). CD14 Correct Answer-surface marker of monocyte-macrophage cell lineage eRPF Correct Answer-= U(PAH)xV/P(PAH) = C(PAH) slight underestimation Renal blood flow Correct Answer-= RPF / (1 - Hct) Filtration fraction Correct Answer-= GFR/RPF normal ~20% RTA type 1 Correct Answer-Inability of a-intercalated cells to secrete H+, no new bicarb formed leading to acidosis Urine pH>5.5Hypokalemia Causes: Ampho B, analgesic nephropathy, congenital obstruction of urinary tract, AI disease RTA type 2 Correct Answer-Defect in PCT bicarb reabsorption leading to acidosis Urine pH <5.5 due to acidification in a-intercal