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NBME

NBME

QuestionAnswer
What is sclerotomes the ventral and mesial portion of a somite that proliferates mesenchyme which migrates about the notochord to form the axial skeleton and ribs Failure of fusion of sclerotomes leads to Spinabifida
What are the signs of glossopharngeal nerve lesion? difficulty swallowing; impairment of taste over the posterior one-third of the tongue and palate; impaired sensation over the posterior one-third of the tongue, palate, and pharynx; an absent gag reflex; and dysfunction of the parotid gland
Vagus nerve lesions presentation? palatal and pharyngeal paralysis; laryngeal paralysis; abnormalities of esophageal motility, gastric acid secretion, gallbladder emptying, heart rate; and other autonomic dysfunction.
Effect on squatting on blood pressure and venous return Squatting produces a simultaneous increase in venous return first to the right then to the left side of the heart, increase in peripheral vascular resistance. This results in an increase in systemic blood pressure and in stroke volume
Aortic Stenosis murmur of aortic stenosis is a high-pitched, crescendo-decrescendo ("diamond shaped"), midsystolic murmur located at the aortic listening post and radiating toward the neck. aortic stenosis murmur is also well known to radiate to the cardiac apex on occasion,
pineal gland tumor Pinealoma Parinaud syndrome (compression of tectum → vertical gaze palsy) obstructive hydrocephalus (compression of cerebral aqueduct) precocious puberty in males (hCG production)
presenilin familial form of Alzheimer disease presenilin-1, (CH 14) presenilin-2: (CH 1) familial forms (10%) with earlier onset associated with degrading APP
Transfusion-related acute lung injury Donor anti-leukocyte antibodies against recipient neutrophils and pulmonary endothelial cells. presentation Respiratory distress noncardiogenic pulmonary edema. fever, hypotension
patient on infliximab or entracept RX for rheumatoid arthritis reactivation of TB TB presentation
Cyclosporine binds cyclophilin. inhibits calcineurin ---> blockade of T cell transcription Psoriasis, rheumatoid arthritis. Nephrotoxicity, hypertension, hyperlipidemia, neurotoxicity, gingival hyperplasia, hirsutism.
Mycophenolate Mofeti reversibly inhibits IMP dehydrogenase ---> prevention of purine synthesis of B and T cells Lupus nephritis. GI upset, pancytopenia, hypertension, hyperglycemia. Less nephrotoxic and neurotoxic
Sirolimus (Rapamycin) mTOR inhibitor; binds FKBP. prevents response to IL-2 pancytopenia, insulin resistance, hyperlipidemia kidney transplant rejection prophylaxis
pt with restrictive lung disease decrease lung volumes ( FVC and TLC) PFTs: FEV1/FVC ratio increase A-a gradient
pt with proliferative glomerulonephritis post respiratory infection RBC casts (shows lesion is glomerula origin or tubular) Proteinuria often in the subnephrotic range (< 3.5 g/day) in severe cases may be in nephrotic range.
pt with tension pneumothorax presents with respiratory acidosis Hyperresonant to percussion Trachea deviated Away from side of lesion - because of air in affected lungs decreased breath sounds and decreased fremitus **fremitus is increased in lobar pneumonia and pulmonary edema
Thyroglossal duct cyst anterior midline neck mass - moves with swallowing or protrusion of the tongue - below the hyoid most likely derived from the tongue - Thyroid diverticulum arises from floor of primitive pharynx & descends into neck - Connected to tongue by thyroglossal duct Foramen cecum is the remnant
Addison disease HLA B8, HLA DR3, DR4 -Due to adrenal atrophy or autoimmune destruction hypotension (hyponatremic volume contraction), hyperkalemia, metabolic acidosis, skin/mucosal hyperpigmentation ( melanin synthesis due to MSH, a byproduct of ACTH production from POMC
6 month pt with failure to thrive, dermatitis, diarrhea is found to have a mutation in the gene encoding FOXP3 regulatory T cells (Tregs) express CD3, CD4, CD25, and FOXP3. maintain specific immune tolerance by suppressing CD4 and CD8 T-cell effector functions. produce anti-in ammatory cytokines (eg, IL-10, TGF-β) --- downregulation of CD4 , CD8 genetic deficiency of FOXP3 -autoimmunity -enteropathy, endocrinopathy, nail dystrophy, dermatitis, /autoimmune dermatologic conditions - x linked. - IPEX
Bortezomib - Proteasome inhibitor -induce arrest at G2-M phase and apoptosis. - affects presentation of antigens to CD8 Tcells proteasome - protein complex that degrades damaged or ubiquitin-tagged proteins RX- Multiple myeloma, mantle cell lymphoma. AVR; Peripheral neuropathy, herpes zoster reactivation.
A 5 month old recently recovered from a viral infection develops easy bruising, petechia, decreased plt count with normocytic monochromic RBC Immune thrombocytopenia -megakaryocytes on bone marrow biopsy, platelet count, increase bleeding time - RX; steroids, IVIG, rituximab, TPO receptor agonists (eg, eltrombopag, romiplostim), or splenectomy for refractory ITP. Destruction of platelets in spleen. Anti-GpIIb/IIIa antibodies -->splenic macrophages phagocytose platelets. idiopathic or 2° to autoimmune disorders (eg, SLE), viral illness, malignancy (eg, CLL), or drug reactions.
Lipoprotein lipase deficiency Pt with yellowish white papules (eruptive pruritic xanthomas), hepatosplenomegaly, abd pain labs ; Chylomicrons, TG, cholesterol lipoprotein lipase Degrades TGs in circulating chylomicrons. deficient in Familial dyslipidemia 1. - hyperchylomicronemia Apoc II might be deficient too -Lipoprotein lipase Cofactor that Catalyzes Cleavage Pancreatitis hepatosplenomegaly, and eruptive/pruritic xanthomas (no risk for atherosclerosis). Creamy layer in supernatant
Margination and rolling E-selectin (upregulated by TNF and IL-1) on endothelial cells ---> binds to sialyl LewisX on WBC - leukocyte adhesion deficiency type 2 ( Sialyl LewisX) P-selectin (released from Weibel- Palade bodies) binds to Sialyl LewisX GlyCAM-1, CD34 on endothelial cells binds to L-selectin on WBC
Tight binding (adhesion) defective in leukocyte adhesion deficiency type 1 ( CD18 integrin subunit ICAM-1 (CD54) binds to CD11/18 integrins (LFA-1, Mac-1) on WBC VCAM-1 (CD106) --> VLA-4 integrin on WBC
Diapedesis (transmigration WBC travels between endothelial cells and exits blood vessel PECAM-1 (CD31 on WBC and endothelial cells bind
Migration—WBC travels through interstitium stimulated by chemotactic factors C5a, IL-8, - LTB4, - platelet-activating factor kallikrein,
Calcineurin inhibitor Cyclosporine -binds cyclophilin. avr; Nephrotoxicity, hypertension, hyperlipidemia, neurotoxicity, gingival hyperplasia, hirsutism. Tacrolimus (FK506) -binds FK506 binding protein (FKBP) avr; Similar to cyclosporine, - risk of diabetes and neurotoxicity; no gingival hyperplasia or hirsutism. Blocks T-cell activation by preventing IL-2 transcription.
Sirolimus (Rapamycin) mTOR inhibitor; binds FKBP. -preventing response to IL-2. -Blocks T-cell activation and B-cell differentiation avr: pancytopenia), - insulin resistance, - hyperlipidemia; - kidney transplant rejection prophlaxis -Synergistic with cyclosporine
Basiliximab Monoclonal antibody; blocks IL-2R. Edema, hypertension, tremor.
Glucocorticoids Inhibit NF-κB. - Suppress both B- and T-cell function by transcription of many cytokines - Induce T cell apoptosis - autoimmune and inflammatory disorders - asthma first line therapy - CLL - non-Hodgkin lymphoma. - amenorrhea - peptic ulcers, - psychosis, - cataracts, - avascular necrosis (femoral head). - Demargination of WBCs causes artiFIcial leukocytosis
Created by: beccao