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?