UWorld misses 1/16/2021
Sex cord stromal tumors
hormone producing tumors derived from stromal cells _Sertoli, Leydig - usually benign
SGLT2 inhibitors side effects
hypotension, UTIs, vulvovaginal candidiasis, weight loss
Hereditary Pulmonary Arterial Hypertension
inactivating mutation of BMPR2 which is transmitted in an AD pattern with variable penetrance
What does a hyperactive stretch reflex lead to?
increased muscle tone (e.g. spasticity)
diffuse mesangial sclerosis
infantile nephrotic syndrome
insulin analogs
insulin-like polypeptides that has had its amino acid sequence altered to optimize pharmacokinetic properties
stretch reflex definition
involuntary contraction of a muscle due to rapid stretching of that muscle
How does Diabetes cause overflow incontinence?
loss of autonomic afferent innervation (DIABETIC AUTONOMIC NEUROPATHY) and inability to sense a full bladder ; involvement of efferent fibers to the bladder -impaired detrusor contractility and incomplete emptying
Periventricular white matter necrosis what does it lead to
loss of descending inhibitory control from the UMN in spastic CP
Embryonal carcinoma
malignant tumor of the testes - bHCG
autosomal dominant inheritance hint
trait typically appears in every generation
What muscle is cut in a mediolateral episiotomy?
transverse perineal muscle
What is seen on light microscopy in maintenance phase (1-2 weeks) of ATN?
tubular epithelial cell necrosis, sloughing of cells with denuded basement membranes and cast containing degenerating cells and debris
What triggers carotid sinus hypersensitivity?
tight shirt collar
nocturnal enuresis
urinary incontinence during sleep (bed wetting) - pelvic floor relaxation combined with full bladder
What 2 regions does the perianal body separate?
urogenital and anal triangles
Pyruvate kinase deficiency
↓ ATP; RBCs dehydrated; ↑ 2,3-BPG (right-shifted OBC); leads to hemolytic anemia this pallor, scleral icterus and splenomegaly
Schiller-Duval bodies
- Yolk sac tumor - Glomerulus- like structure surrounding vessel in germ cells
Levator ani muscle group
- located in pelvic diaphragm - innervated by origins of sacral nerve roots s3-s5 to pelvic diaphragm - ilicoccygeus - pubococcygeus -puborectalis
What is the stretch reflex responsible for?
DTR, maintenance of muscle tone by constant macular activity occurring even at rest
MOA of metformin
Decreases hepatic glucose production and intestinal glucose absorption; increase peripheral glucose uptake and utilization increase insulin sensitivity
Normal pressure hydrocephalus cause
Increased CSF due to decreased reabsorption by arachnoid granulations, resulting in dilated ventricles; dementia elderly (cog impair MMSE < or = 22); treat with shunt
X-linked dominant
Name the pattern of genetic transmission characterized thus: both M and F affected; no M-to-M transmission; affected M passes trait to all daughters, every generation; affected F passes trait to both sons and daughters; a single mutant allele can produce the disease.
Do thiazolidinediones cause hypoglycemia?
No
What class of drug is glyburide?
Sulfonylurea
What medications stimulate pancreatic insulin secretion independent of blood glucose levels?
Sulfonylureas, meglitinides
Acute rejection what cell is responsible
T lymphocytes (dense mononuclear lymphocytic infiltrate)- cell mediated; 2-5 wks
Chronic rejection
T-cell, antibody mediated (B cell) vascular damage. Months to years after. Irreversible. need new transplant scant inflammatory cells and interstitial fibrosis
What is an example of development of resistant HIV strains?
Poor penetration of antiretroviral meds into the brain allows it to act as an anatomical sanctuary where viral replication proceeds unchecked
Acute intermittent porphyria
What disease causes port-wine colored urine, polyneuropathy and painful abdomen?
Gilbert's syndrome
What disease has a genetically low level of UDPglucuronate transferase, resulting in elevated free unconjugated bilirubin?
Autosomal Recessive Inheritance hints
Skips generations, usually seen in only 1 generation. 25% of offsprings from 2 carrier parents affected. Often due to enzyme deficiencies, more severe than dominant disorders; symptoms presents in childhood.
What drug class are Canagliflozin/Dapagliflozin?
Sodium-glucose transporter-2 inhibitors
Anal triangle contents
external anal sphincter muscle ischioanal fossa
Lead toxicity enzymes inhibited
ferrochelatase, ALA dehydratase
Entomeoba Histolytica colonoscopy findings
flask shaped colonic ulcers with trophozoites
Afferent from carotid body and sinus
glossopharyngeal
Effects of improper repair of a midline episiotomy
pelvic organ collapse, dyspareunia
What structures is involved in a midline episiotomy?
perianal body
Insertion of external anal sphincter
perineal body
What class of drug is amiloride?
potassium sparing diuretic - sodium channel blocker
Where do carbonic anhydrase inhibitors work?
proximal convoluted tubule
Leydig cells (interstitial cells)
secrete testosterone (may lead to precocious puberty); also often produce estrogen leading to feminization (e.g. gynecomastia)
What are the severe side effects of Quetiapine?
sedation, wt gain, metabolic syndrome
Germ Cell tumors types
seminomatous or nonseminomatous
superficial transverse perineal
stabilizes central tendon of perineum
encapsulated bugs: "SHiNE SKiS" (risk in aplenic pts e.g. sickle cell)
streptococcus penumoniae Haemophilus influenzae type B Neisseria meningitidis Escherichia coli Salmonella Klebsiella pneumoniae group B Strep
NPH triad
1. Gait disturbance (wobbly) 2. Urge incontinence (wet) (loss of CNS inhibition of detrusor contraction -->sudden desire to urinate) 3. Memory loss (wacky)
Acute Intermittent Porphyria (AIP)
Autosomal *dominant* mutation in *Porphobilinogen Deaminase* that often presents in puberty ↑Porphobilinogen ↑?-ALA ↑Coporphobilinogen (urine) ↓Heme 5 Ps: Painful abdomen, Purple (wine) urine, Polyneuropathy, Psychological disturbances, Precipitated by Puberty, drugs (P450 inducers), alcohol, starvation Treat with *glucose, heme*, which inhibit ALA synthase
Drug class: metformin
Biguanides (oral antidiabetics)
What can be used as a marker of endogenous insulin secretion?
C-peptide
stretch reflex arc
Components 1. Receptor - muscle lengthening sensed by muscle spindles 2. Sensory Neuron - afferent signal of stretch is conveyed to the spinal cord via DORSAL NERVE FIBERS 3. Motor Neuron - efferent signals conveyed via the VENTRAL ROOT of the spinal cord 4. Effector (Muscle) - extrafusal fibers are stimulated causing muscle contraction
bulbospongiosus female
Compresses and stiffens clitoris; narrows vaginal opening
What glomerular disease causes nephrotic syndrome seen in adolescents and adults and can be related to drug use (e.g. heroin)
FSGS
In what glomerular diseases goes glomerular epithelial proliferation occur?
Glomerulonephritis, diabetic nephropathy (among others)
What accounts for transient pyuria and loss of electrolytes in the recovery phase of ATN?
Gradual nature of tubular cell recovery leads to impaired tubule resorption and decreased renal concentrating ability
What may spasticity be caused by?
Loss of descending inhibitory control from UMN leading to hyperactive stretch reflex resulting in increased muscle tone
What causes overflow incontinence?
INCOMPLETE BLADDER EMPTYING pelvic organ prolapse, prostate enlargement, bladder outlet obstruction, impaired detrusor contractility
Selective Dorsal Rhizotomy (SDR)
Laminectomy in L-S spine -Dorsal roots (sensory) decreases muscle tone without sacrificing motor innervation of the muscles (w/o paralysis)
Lead toxicity
Low-level exposure in housing built before 1978 (neurotoxic combined with incomplete BBB in children) causes long-term behavioral effects, language regression, impaired reading skills, aggression Increases absenteeism and dropout rates; anemia, hemolytic anemia, ALA accumulation and elevated zinc protoporphyrin levels
Yolk sac tumor
MC germ cell tumor in young girl, inc. AFP, Schiller-Duval bodies
Short acting benzodiazepines
Midazolam (Versed) Triazolam (Halcion)
Bulbospongiosus (male)
Origin: base of penis; fibers cross over urethra, insertion: midline and central tendon of perineum, action: compresses base and stiffens penis; ejects urine or semen
variable penetrance
Penetrance refers to the proportion of individuals with a certain allele of a gene that exhibits the phenotype of that gene. Complete penetrance is when any individual who has the allele will show the phenotype (or conditions) of that gene. Incomplete penetrance means the trait is expressed in only part of the population that has the allele.
What happens to GRP in the recovery phase of ATN? Why?
Recovers tubules clear of casts and debris
Heme biosynthesis
Required for efficient erythropoiesis - glycine, B6 and iron requirements. Impaired biosynthesis results in disorders called Porphyrias. These disorders result in cutaneous and/or neurologic manifestations. Lead inhibits final step of adding Iron cofactor.
What are common infections in Sickle cell patients?
S. pneumo, N. meningitidis, H. influenzae, Salmonella
Class of Quetiapine (Seroquel)
Second generation antipsychotic
episiotomy prevents
Severe perineal lacerations thus fecal incontinence due to anal sphincter damage
Vessels in series are sided by side on one on top of another?
Side by Side
drug class of pioglitazone and rosiglitazone
Thiazolidinedione
What is the recovery phase of ATN characterized by?
Tubular re-epithelialization
Porphyria cutanea tarda (PCT)
Uroporphyrinogen decarboxylase deficiency, resulting in abdominal pain, neuro symptoms, and photosensitive hyper pigmented blistering and elevated uroporphyrinogen in the urine
X-linked recessive
What pattern of genetic transmission affects only M and has no M-to-M transmission, and mother is usually an unaffected carrier?
Mitochondrial inheritance
What pattern of genetic transmission is characterized by no transmission from M, maternal inheritance, and the potential for the disease to affect both sons and daughters of affected F?
P-glycoprotein
a multidrug transmembrane transporter protein responsible for transporting various molecules , including drugs, across cell membranes; in areas of high expression, it reduces drug absorption; highly expressed on the luminal membrane of brain capillary endothelial cells; ATP driven efflux pump
MOA of thiazolidinediones (glitazones)
agonist of PPAR gamma upregulates adiponectin (increased insulin sensitivity & FA oxidation): increases TAG storage & FA oxidation-> decreased serum TAGs increases the differentiation & # of adipocytes upregulate GLUT4 in peripheral tissues (increased glucose uptake) increase insulin sensitivity
intermediate benzos
alprazolam, lorazepam, temazepam, oxazepam, clonazepam
contact dependent cytotoxicity
amebic lectin binds host cell introduces an amebic porin into the host cell membrane and causes cell lysis (e.g. invasive strains of E. histolytica)
Entamoeba histolytica
amoebic dysentery (blood and pus due to colonic damage); extraintestinal (liver abscess most common); fecal oral
hematocolpos
an accumulation of menstrual blood in the vagina
Mitochondrial myopathies
are rare disorders often presenting with myopathy, lactic acidosis, and CNS disease secondary to failure of oxidative phosphorylation. biopsy shows ragged red fibers. MELAS is one such example.
Is the carotid sinus a baroreceptor or chemoreceptor?
baroreceptor
What structures are anchored to the perianal body?
bulbospongiosis m., external anal sphincter muscle, superficial and deep transverse perineal mm., fibers from the external urethral sphincter, levator ani and muscular coat of the rectum
Saddle Anesthesia, DEC DTR, DEC rectal sphincter tone, loss of bowel and bladder control are indicative of what?
cauda equina syndrome which is commonly due to epidural cord compression from a malignancy - pts develop urinary retention late in the course associated with fecal incontinence
Is aortic body a baro or chemoreceptor?
chemoreceptor
What does the external anal sphincter arise from?
coccyx and anococcygeal ligament
sacrotuberous ligament
connects sacrum to ischial tuberosity
Seminomas
contain large uniform germ cells that retain the phenotypic features of sperantogonia
urogenital triangle
contains the urethral and vaginal orifices in females and the base of the penis and scrotum in males
obturator fascia
covers the pelvic surface of the obturator internus muscle
Entomeoba Histolytica stool O&P
cysts and trophozoites
MOA of SGLT2 inhibitors
decrease resorption of filtered glucose
Where do osmotic diuretics work?
descending limb loop of henle and proximal tubule
Long acting benzodiazepines
diazepam (Valium), chlordiazepoxide, flurazepam
nonseminomatous tumors
embryonal carcinoma, teratoma, choriocarcinoma, and yolk sac tumor, mized
Teratoma
neoplasm of a testis or ovary containing multiple tissues from other sites in the body -terminally differentiated germ cells
Do exogenous insulin and insulin analogs elevate C-peptide?
no
Afferent from aortic body and arch
vagus
anococcygeal body
what is the fibrous mass called between the tip of the coccyx and the anal canal?