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Flashcards in uWorld 57 Deck (25):
1

what is Goucher disease

most prevalent genetic disorder among Ashkenazi Jews

AR lysosomal storage disease due to BETA-GLUCOCEREBROSIDASE deficiency

leads to accumulation of glucocerebroside, a glycolipid component of leukocyte and erythrocyte membranes

LIPID LADEN MACROPHAGES (VOUCHER CELLS) are classically described as "wrinkled tissue paper", "wrinkled silk" or "compiled newspaper" in a bone marrow aspirate- also in liver, and lymphatic tissue too

2

how does goucher present

bone pain due to marrow invasion/inflammation, abdominal distention due to HEPATOSPLENOMEGALY, and easy bleeding and bruising, pallor, and fatigue due to PANCYTOPENIA splenic enlargement first and can be massive

anemia and thrombocytopenia, leukopenia

3

the splenic red pulp is important for what

destroying aged and ABNORMAL ERYHTROCYTES and serving as an emergency store of blood cells and platelets that can ve delivered into the circulation when needed

clearance of CIRCULATING BACTERIA that become lodged in the chords- macrophages present them to B- and T- cells in the splenic white pulp

splenic OPSONIZING ANTIBODY is of particular importance in the clearance of ENCAPSULATED SPECIES

4

what are type I interferons

interferons ALPHA and BETA- released from virally-infected cells and cause macrophage and cytotoxic T-lyphocyte activation, leading to destruction of infected cells

5

what is midodream

alpha 1 agonist
reflex bradycardia
decrease in cardiac contractility (reflex)
increase in SVR

used for postural hypotension and autonomic insufficiency

may exacerbate supine hypertension

6

what is seen in chronic lung rejection

BRONCHIOLITIS OBLITERANS- producing obstructive lung disease

AIRFLOW LIMITATION with a drop in both FEV1 and the FEV1/FVC ratio

lymphocytic inflammation and destruction of the epithelium of the small airways

fibropurulent exudate and granulation tissue are found in the lumen of the bronchiole, which ultimately results in fibrosis, scarring, and the progressive obliteration of small airways

7

what is seen in a patient in a PINEAL GLAND MASS

GERMINOMA

OBSTRUCTIVE HYDROCEPHALUS form aqueduct stenosis (papilledema, headache, vomiting) and DORSAL MIDBRAIN (PARINAUD) SYNDROME due to direct compression to he PRETECTAL region of the midbrain

paranaud syndrome is LIMITATION of UPWARD GAZE with DOWNWARD GAZE PREFERENCE, bilateral EYELID RETRACTION and LIGHT-NEAR DISSOCIATIONS

8

what are the risks of second hair exposure

prematurity, low birth weight

sudden infant death syndrome

middle ear disease (otitis media)

asthma

respiratory tract infections (bronchitis, pneumonia)

9

what happens if pregnant chick smokes

plantations (previa, abruption), prematurity, perinatal mortality, and significantly reduced birth weight

levels of tobacco are same in fetus and mom

10

hypotension, tachycardia, and cool extremities are indicative of what

HYPOVOLEMIC SHOCK

11

what happens in wound contractures

unusually pronounced MMP activity results in excessive wound contraction

(MMPs encourage myofibroblast accumulation at the wound edges and scar tissue remodeling- myofibroblasts initiate wound contraction during healing by SECOND INTENSION

12

what is alpha 1 effect on renal blood flow

decreases it

(no effect on contractility, DECREASES HEART RATE-refelx)

13

what is seen in multicystic kidney dysplasia

presence of multiple cysts of varying size in the kidney and the absence of normal pelvocaliceal system

associated with ureteral or uretor pelvic atresia

affected kidney is rendered useless

14

lymph proximal to the anal DENTATE LINE drain where

INFERIOR MESENTERIC and INTERNAL ILIAC

the upper rectum and superior part of the middle rectum follow the superior rectal nodes along the superior rectal artery into the inferior mesenteric lymph node

middle to lower third of the rectum can drain upward into the inferior mesenteric nodes or follow the middle rectal nodes along the middle rectal artery to the internal iliac lymph nodes

15

what are complications of varicose veins

painful thromboses, stasis dermatitis, skin ulcerations, poor wound healing, and superficial infections

16

flank pain radiating to the groin with ballot able flank mass that develops within a week of pelvic surgery suggests what

UTERIC OBSTRUCTION

it is vulnerable during PELVIC SURGERY

ureter courses anterior to the iliac vessels (area of resection of the pelvic nodes, which drain the uterus and cervix) and just posterior to the uterine artery near the lateral fornix of the vagina

17

unilateral ureteral ligation causes what

OBSTRUCTION with HYDRONEPHROSIS and FLANK PAIN due to distention of the ureter and renal pelvis

urine output and serum creatinine remain within normal limits in most individuals with unilateral obstruction b/c contralateral kidney functions normally and compensates for decreased functioning of the affected kidney

18

the ureters run close to what in the fell pelvis

PELVIC LYMPH NODES and the UTERINE ARTERY

predisposes them to injury during pelvic surgery

19

renal vein thrombosis is seen in who

NEPHROTIC snydrome

20

what makes up the floor of the anatomical snuff box

scaphoid
trapezium

21

what is DUPUYTREN's CONTRACTURE

slowly progressive fibroproliferative disease of the palmar fascia

nodules form on the fascia, eventually resulting in contractors that draw the fingers into flexion

22

pruritus in palms and soles and is unbearable during the night in a middle aged woman is seen in what

PRIMARY BILIRAY CIRRHOSIS (PBC)

xanthomatous lesions in the eyelids or in the skin and tendons

jaundice, steatorrhea, portal hypertension, and osteopenia

elevated IgM, elevated ALP, cholestasis

associated with Sjogren's, Raynauds, scleroderma, autoimmune thyroiditis, hypothyroidism, and celiac disease

23

what causes the obstruction in hypertrophic cardiomyopathy

mitral valve leaflet (anterior)

interventricular septum

24

what increases the intensity of hypertrophic cardiomyopathy

all decrease preload:

valsalva (straining phase)

abrupt standing (from sitting or supine position)

nitroglycerine administration

25

what decrease the murmur in hypertrophic cardiomyopathy

sustained hand grip

squatting (from standing position)

passive leg raise