Miscellaneous Flashcards

Question

Corrected age/age adjusted for gestation

Answer 1

Considered when assessing a premature infant's growth and development milestones. It does not determine timing of vaccine administration.

Answer 2

Sporadic disorder due to maternal uniparental disomy: both copies of a section of ch15 are inherited from the MOTHER instead of 1 from each parent Get deletion of paternal copy of ch 15q11q13 Missing the father's chromosome

Answer 3

Paternal uniparental disomy (both copies inherited from the FATHER; deletion of maternal copy of ch 15q11q13) Short stature, intellectual disability Smiling/laughter, hand-flapping, ataxia, and seizures

Answer 4

Most common sex ch abnormality causing pirmary hypogonadism. Presentation: normal male external genitalia and no apparent dyspmorphic features in newborns; after puberty, small testes and tall stature become apparent Phenotypically male and present wtih hypergonadotrophic hypogonadism (elevated FSH and LH) due to testicular damage during development.

Answer 5

Most common x-lined inherited cause of intellectual disability. Affected boys have a prominent forehead and macroorchidism.

Answer 6

Can cause neuromuscular depression. Presentation: decreased respiratory effort/apnea, muscle paralysis, somnolence, visual disturbances, decreased or absent deep-tendon reflexes Pulmonary edema may occur

Answer 7

Rare cause of precocious puberty (<8 in girls, <9 in boys) Due to a mutation in GNAS gene resulting in G protein activation and overproduction of pituitary hormones = GnRH-independent (ie peripheral) precocious puberty Thyrotoxicosis, acromegaly, cushing syndrome Irregular cafe au lait macules confined to one side of the body, recurrent fractures due to polyostotic fibrous dysplasia

Answer 8

Acute hemorrhagic cystitis, bladder carcinoma, sterility, and myelosuppression. Drinking plenty of fluids, voiding frequently, and taking MESNA help prevent these.

Answer 9

Most common muscular dystrophy in children X-linked recessive Presents at age 2-5 w/ bilateral calf pseudohypertrophy and gower sign Biopsy: fibrosis and fatty infiltration which supports dx Immunochemistry staining of muscle tissue would show absent dystrophin Dx: genetic testing (gold standard) shows deletion of dystrophin gene on ch Xp21

Answer 10

Delayed puberty + anosmia Due to disorder of migration of fetal olfactory and GnRH producing neurons resulting in rhinencephalon hypoplasia + hypogonadotropic hypogonadism; most cases are X-linked but girls can be affected via sporadic AD and AR mutations; 46XX No GnRH-->low FSH and LH Girls: anosmia, no estrogen, short stature, primary amenorrhea, no breast development, no body hair; normal internal reproductive organs Boys: anosmia, no body hair, small phallus + testicles Mgmt: facilitating development of 2nd sex characteristics in adolescents and improving fertility in adults

Answer 11

Normal 2nd sexual characteric development and tall stature. | FSH and LH levels are normal.

Answer 12

The result of nonfunctioning androgen receptors that lead to peripheral androgen resistance Genotypic males (46XY) who present as phenotypic females Breast development but no pubic and axillary hair Primary amenorrhea (no uterus), no ovaries Normal to high FSH and LH

Answer 13

Genotypic males (46XY) who present as phenotypic females Virilization (eg clitoromegaly) at puberty NO breast development Primary amenorrhea (no uterus) Normal to high FSH and LH

Answer 14

Unexpected, dose independent pharmacological effect (SJS w/ lamotrigine)

Answer 15

Dose-dependent increases in normal pharmacologic action (respiratory depression w/ opioids)

Answer 16

More common in women, increased risk in pts w/ DM or thyroid disorders Dry eyes, dry mouth Exocrine output from lacrimal and salivary glands declines w/ age, assoc. w/ atrophy, fibrosis and ductal dilation of the glands Corneal epithelial erosions can lead to impaired vision Mgmt: artificial tears or other ocular lubricants; cyclosprorine drops to increase tear secretion ANA negative

Answer 17

Chromosomal abnormality caused by complete or partial loss of x ch (45, XO) Presentation: short stature, aortic coarctation (blood pressure differential), absent menarche Ovarian dysgenesis leads to "streak ovaries" (small ovaries with little to no follicles) and primary ovarian insufficiency No estrogen-->amenorrhea, no breast development Estrogen normally inhibits osteoclast-mediated bone resorption so these pts are at increased risk for osteoporotic fractures Bicuspid aortic valve, aortic root dilation place pts at increased risk for aortic dissection, risk is further increased during pregnancy Tx: estrogen replacement therapy to promote normal sexual maturation and reduce risk of fractures

Answer 18

Swelling in hands and feet due to congenital lymphedema which occurs in over 1/2 of TS pts Etiology: lymphatic network dysgenesis; dysfunctional lymphatic system causes accumulation of protein rich interstitial fluid in the hands, feet, and neck Severe obstruction of lymphatic vessels can result in cystic hygroma of neck and fetal hydrops Congenital lymphedema presents at birth w/ edema that is generally NON-PITTING due to high protein content in intersitital fluid In contrast, pitting edema seen with liver failure (hypoalbuminemia), nephrotic syndrome (proteinuria), and CHF (ventricular hypocontractility) is caused by low protein concentration in the interstitial fluid.

Answer 19

Any penetrating wound below the 4th ICS (level of the nipples) is considered to involve the abdomen and requires and exploratory laparatomy in unstable pts. Exlap should be done for: HD instability, peritonitis (rebound, guarding), evisceration (externally exposed intestines), or blood from NG tube or rectal exam)

Answer 20

Etiology: arsenic binds to sulfhydryl groups and interferes w/ arious enzymes RF: working w/ antique pressure-treated wood preserved w/ arsenic; pesticide manufacturing, metalworking, mining Presentation: sensorimotor neuropathy in stocking glove distribution w/ burning, painful hypersensitivity, distal weakness, and hyporeflexia; hypo/hyperpigmentation of skin, mees lines (horizontal striation of fingernails); pancytopenia, hepatitis Dx: measurement of urine arsenic levels Tx: chelation (dimercaprol, dimercaptosuccinic acid) for severe toxicity

Answer 21

A defect of the NADPH oxidase in phagocytic cells, which leads to impaired killing of catalase positive organisms (eg s. aureus, serratia) Affected pts have recurrent skin and soft tissue infection Normal lymphocyte and Ig concentrations

Answer 22

An x-linked recessive disease Presents early in infancy w/ eczema and bleeding (eg post circumcision, bleeding from umbilical stump) due to thrombocytopenia T cell dysfunction and hypogammaglobulinemia worsens with age and present as bacterial, viral, and opportunistic infections.

Answer 23

Primary tx for moderate hypothermia: active external warming, warm baths, and warmed IVF. Due to decreased reactivity of the pacemaker cells, the bradycardia assoc. w/ hypothermia is often refractory to treatment w/ atropine and cardiac pacing. However, it will improve with tx of the hypothermia. Levothyroxine is used to treat hypothermia caused by myxedema.

Answer 24

An IgE mediated hypersensitivty reaction that can result from exposure to latex (eg surgical gloves, Foley catheter) Pts typically present w/ hypotension, poor organ perfusion tachycardia, urticarial rash, wheezing, and they may develop life-threatening upper airway edema. Some cases require additional doses of epinephrine for refractory symptoms.

Answer 25

In case of amputation injury, amputated parts should be wrapped in saline-moisterized gauze, sealed in a plastic bag, and placed on ice and brought to the ED w/ pt. Amputated parts viable for up to 24h.

Answer 26

Irreversible loss of function of the whole brain, including the brain stem. Brain death is a legally acceptable definition of death, and artificial life support does not need to be continued. Family permission is not legally required to discontinue mechanical ventilation in a pt w/ brain death.

Answer 27

Hydrogen cyanide and carbon monoxide are the 2 major products of combustion in closed spaces. Cyanide toxicity can be treated w/ hydroxocobalamin or sodium thiosulfate, which directly binds cyanide molecules. Alt tx: Induction of methemoglobinemia w/ nitrites to increase ferric iron (Fe3+) in circulating hemoglobin. Cyanide binds avidly to Fe3+.

Answer 28

A full body XR | Indicated when non-accidental trauma is suspected.

Answer 29

Placed when IV access cannot be obtained in emergency cases Requires less skill and practice than central line placement; safer and faster than central lines Provides cannula large enough to deliver fluids and meds rapidly and to obtain blood samples for lab testing Most common site for access: proximal tibia Contraindications: infection (cellulitis) over the access site, fracture or previous IO attempts in chosen extremity, or bone fragility (osteogenesis, imperfecta)

Answer 30

Autosomal recessive; degeneration of spinal tracts (spinocerebellar tracts, posterior columns) A neurodegenerative disorder that classically presents in adolescence w/ progressive ataxia and dysarthria. Most common cause of death: cardiac dysfunction (eg arrhythmia, CHF) due to hypertrophic cardiomyopathy.

Answer 31

Chronic: due to progressive deposition of subcutaneous collagen and adipose tissue leading to development of firm, thickened skin and nonpitting edema Stemmer sign: Inability to lift skin on dorsum of 2nd toe is highly specific for lymphedema

Answer 32

Hypotonia is a common finding in neonates with DS. Poor muscle tone and weak suck are key findings. Additional features: upslanting palpebral fissues, protruding tongue, single palmar crease.

Answer 33

Temp <36.5 (97.7) or >39 (102.2) Tachypnic >30 Tachycardia >90 Hypotension SBP <90

Answer 34

For pt who present soon after a single, potentially toxic intestion (>7.5g) of acetaminophen, the first step in mgmt is gastric decontamination with activated charcoal and measurement of acetaminophen levels if they present w/in 4h of ingestion. Pts can be asymptomatic during the first 24h after ingestion.

Answer 35

Mgmt options for acute pain (including opioids) will be similar for all pts regardless of substance abuse history, although those w/ a hx of opioid addiction who are given opioid analgesics may need close follow-up care to avoid relapse. So, it's okay to give opioids.

Answer 36

Primarily given to pts w/ mild or moderate pain who are unable to take oral or rectal preparations. For pts w/ severe pain, it is usually administered w/ opioid analgesics.

Answer 37

Indicated for long-term mgmt of pain in pts who require daily, continuous pain relief. Onset of action too slow for mgmt of acute pain.

Answer 38

Occurs when a pt fails to regain consciousness w/in expected window (15-60m); due to - drug effect: preop drug ingestion (opioids, benzos, illict drugs, anticholinergics, antihistamines) may potentiate anesthetic effects; prolonged anesthesia duration or higher medication doses may also delay emergency - metabolic disorder: hyper- or hypoglycemia, hyper or hyponatremia, and liver disease - neuro disorder: intraop stroke, seizure (or postictal state), or elevation of intracranial pressure can cause prolonged alterations in mental status

Answer 39

In a nonemergency situation in which a parent refuses potentially life-saving treatment for their child, the physician should seek a court order mandating treatment. [after hospital ethics committe, social services, and hospital risk mgmt have been consulted] If a delay for consent is life-threatening, emergency treatment for the child is legally authorized regardless of the parent's wishes.

Answer 40

LR is a balanced fluid and is preferred for burn victims. It contains near physiologic levels of cl, K, and ca and includes sodium lactate a buffer hepatically metabolized to bicarb which helps correct acidosis and maintain normal blood ph. NS is an unbalanced fluid because its cl concentration is supraphysiologic (154 v 103) and can cause a hyperchloremic metabolic acidosis. It has also been assoc. w/ hypocoagulability.

Answer 41

Physicians are ethically obligated to protect patient confidentiality. Unless a pt is at active risk of harm to self or others, physicians cannot disclose information to family members w/o the pt's consent. Cannot disclose if pt is or is not pt a clinic = ANY information

Answer 42

LV dysfunction (tiredness, poor feeding, diaphoresis during feeds), coronary artery abnormalities during a prolonged febrile illness Injected conjunctiva, mucositis, edema/erythema of hands and feet due to progressive LV overload, polymorphous rash, cervical lympadenopathy Tachypnea, tachycardia, and S3 gallops on auscultation Prolonged fevers >10d, delayed treatment w/ IVIG, and age <1y are RF for cardiac complications Complications: coronary artery aneurysm

Answer 43

Most common cause of pediatric myocarditis and presents w/ fever, LV dysfunction, and HF. Coronary artery abnormalities are not seen. Rash classically presents as papulovesicular lesion on hands, feet, and mouth.

Answer 44

Adults should receive Tdap as a one time dose in place of Td. If pt has not received Tdap as adult, or prior vaccine hx is unknown, Tdap is given followed by Td every 10y after.

Answer 45

Live attenuated vaccine Safety not established for pt w/ medical comordities such as DM, which would increase their risk for influenza complications.

Answer 46

19-64 - PPSV23 alone: chronic heart, lung, or liver disease; DM, smokers, alcoholics [For heart or lung disease, DM, smoking, chronic liver disease - PPSV23 alone is recommended followed by 13 and 23 at 65yo.] 13-valent pneumococcal conjuate (PCV13) then 23-valent pneumococcal polysaccharide vaccine (PPSV23) for adults >=65yo and for those <65 w/ high risk comorbid conditions (CSF leaks, sickle cell, cochlear implants, congenital or acquired asplenia, IC pts)

Answer 47

Consent is not required from an absent parent in an emergency situation. Attempting to contact them would delay life-saving treatment. Grandparents cannot provide consent unless thay are legal guardians.

Answer 48

Most reliable and predictive sign: decreased respiratory rate Other evidence: decreased bowel sounds and hypotension Hypothermia results from environmental exposure and impaired thermogenesis, and can occur even at room temperature in severely intoxicated pts Absence of miosis does not exclude dx as normal or enlarged pupils may be seen if there is co-exposure Tx: naloxone

Answer 49

A culture-bound syndrome of South Asia that manifests w/ psychological and somatic symptoms that are attributed to loss of semen. When evaluating culture-bound syndromes, it is important to take a patient-centered approach and allow the patient to explain the cultural explanations, manifestations, and consequences of a given syndrome.

Answer 50

Recommend for travel to North Africa, Asia As well as hep B, typhoid + polio booster vaccine. A single dose of hep A vaccine provides adequate protection for a young immunocompetent adult; the second dose should be administered for long-term immunity

Answer 51

Mosquito born viral hemorrhagic fever endemic to tropical regions of sub-Saharan Africa and South America. Vaccination is recommended for travel to those regions.

Answer 52

Required for people traveling to some Asian countries and to sub-Saharan Africa. Legally required for pilgrims who make the Hajj to Mecca, Saudi Arabia.

Answer 53

Toxicity impaires O2 delivery: reduces O2 carrying capacity Decreases O2 unloading in tissues Disrupts oxidative phosphorylation in mitochondria Cerebral hypoxia: headache, dizziness, confusion Pulse ox is usually normal b/c oximeter cannot differentiate b/n oxyhemoglobin and carboxyhemoglobin AG metabolic acidos due to lactic acidosis from peripheral tissue hypoxia Permanent hpoxic brain injury: bilateral enlargement of GLOBUS PALLIDUS, an area highly sensitive to hypoxic conditions

Answer 54

Unresponsiveness AG metabolic acidosis Papilledema - methanol

Answer 55

Initial managnement based on rapid rewarming in 37-39F WATER BATH. Hot air rewarming not recommended due to difficulty of maintaining precise temperature control. Debridement not indicated until the tissue is rewarmed and an accurate survey of devitalized tissue can be performed.

Answer 56

Tetanus Ig (TIG) provides immediate passive (but temporary) immunity. Used to treat symptomatic tetanus. Never administered w/o tetanus vaccination. Should be given to pts w/ significant or dirty wounds who have not previously received 3 doses of tetanus vaccine or whose vaccine status is uncertain.

Answer 57

Primary humoral immunodeficiency syndromes present w/ recurrent or severe sinopulmonary infections. Hyper IgM syndrome is due to a defect in the CD40 ligand. Characterized by high IgM levels, low IgA and IgG, and normal lymphocyte populations.

Answer 58

Defective integrins on leukocyte surface Presents w/ delayed umbilical cord separation, recurrent skin (cellulitis, abscess, omphalitis) and mucosal bacterial infections (periodonatal) (w/o purulence) and poor wound healing. Marked leukocytosis w/ neutrophil predominance is common. Inflamamation w/ lack of purulence Culture: s. aureus or GN bacilli

Answer 59

You can only accept nonmonetary gifts from interested parties only if the gifts directly benefit patient care and are of small monetary value (eg unbiased education materials, drug samples) No gifts of cash or high value, or w/ implied reciprocity, should be accepted.

Answer 60

Continuity of care for medications at the time of transitions of care, between inpatient and outpatient facilities and w/in inpatient facilities, is a potential source of medical error. Interventions that target pharmacy personnel and high risk pts appear to be the most effective in improving the quality of patient care.

Answer 61

Rapid transfusion of O negative (Rh negative) blood cells is the best management of hypovolemic shock due to acute hemorrhage as it restores blood volume and O2 carrying capacity while minimizing risk of hemolytic transfusion reactions.

Answer 62

Type 3 hypersensitivity reaction Most commonly caused by beta-lactams (PCN, amox, cefaclor) and sulfa drugs (TMP-SMX). Symptoms arise 1-2 weeks after exposure and include fever, urticarial rash, arthralgia, and lymphadenopathy. The abnormalities should resolve w/ withdrawal of the offending agent.

Answer 63

The prevalence of measles has been reduced drastically by MMR vaccination. The vaccine has an excellent safety profile, but w/in 1-3 weeks after immunization a small fraction of pts may develop a fever and mild rash due to infection w/ vaccine-strain measles that resolve w/o treatment. Although virus is attenuated, it is a live virus and can cause a mild version of measles w/in 1-3week of immunization. Vaccine strain is still transmissable to others. So ppl should avoid contact w/ immunocompromised individuals until rash has resolved.

Answer 64

Wheezing, urticaria, anaphylaxis

Answer 65

The timing of fever (eg immediate, acute, subacute, delayed) after surgery usually indicates the likely dx. Febrile nonhemolytic transfusion reaction can occur w/in 1-6h of transfusion and can cause immediate postop fever in pts receiving blood during or after surgery. During blood storage, leukocytes release cytokines that when transfused can cause transient fevers, chills, and malaise (w/o hemolysis) w/in 1-6h of transfusion. Mgmt: stopping transfusion to r/o other serious causes of fever (eg acute hemolytic reaction) and antipyretics (avoid aspirin in thrombocytopenic pts) Other causes of immediate (w/in a few h) postop fever include prior infection or trauma, inflammation due to surgery, malignant hyperthermia, and meds (anesthetics).

Answer 66

Appear phenotypically male Develop nodulocystic acnea at puberty They require neurodevelopmental evaluation due to the increased incidence of learning disabilities, behavioral problems, and developmental delay.

Answer 67

Salicylate (eg aspirin) poisoning presents w/ nausea, vomiting, and a metabolic acidosis w/ respiratory compensation. However, tinnitus is a common early sign, and aspirin tablets are not visualized on XR.

Answer 68

Pts w/ acute iron poisoning have abdominal pain, diarrhea, and hematemsis. In addition, they may develop hypovolemic shock w/in a few hours due to GI losses. Labs: AG metabolic acidosis XR: radiopaque tablets (visualized) Tx: IV deferoxamine chelation therapy

Answer 69

Methanol poisoning: damages the eyes (blurred vision, hyperemic optic disc) Ethanol poisoning: damages the kidneys

Answer 70

Autosomal dominant Assoc. w/ alterations of tumor protein p53 gene Usually results in early onset of malignancies such as sarcomas, breast cancer, and adrenal carcinomas. CNS disease is characterized by gliomas and medulloblastomas.

Answer 71

Causes hereditary fructose intolerance. Introduction of fruits and vegetables into the diet is followed by accumulaton of fructose 1 phosphate. Affected infants can present w/ vomiting, poor feeding, and lethargy. Seizures or encephalopathy follow if fructose is not removed from the diet.

Answer 72

Autosomal recessive Characterized by partial oculocutaneous alblinism and recurrent cutaneous infections. S. aureus and Strep pyogenes are common.

Answer 73

Systemic disorder characterized by chronic rhinosinustitis, including nasal polyps, asthma, and prominent eosinophilia. Typically diagnosed in adulthood and does not present w/ pancreatic insufficiency.

Miscellaneous Flashcards

(97 cards)