uWorld 57 Flashcards
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
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
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
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
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
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
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
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)
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
hypotension, tachycardia, and cool extremities are indicative of what
HYPOVOLEMIC SHOCK
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
what is alpha 1 effect on renal blood flow
decreases it
no effect on contractility, DECREASES HEART RATE-refelx
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
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
what are complications of varicose veins
painful thromboses, stasis dermatitis, skin ulcerations, poor wound healing, and superficial infections
