NEW HY INCORRECT Flashcards
What is the most common congenital anomaly of the GI tract?
Meckel’s Diverticulum
True Diverticulum (all 3 layers) – Persistence of the VITELLINE DUCT
Abnormal fibrous connection b/t ILEUM & Umbilicus
Can cause Vitelline duct cysts
TERMINAL ILEUM
Can cause Iron Deficiency anemia
Markers for Down Syndrome?
INCREASED HCG & Inhibin
DECREASED AFP & Estriol
AML most common BEFORE 5 years old
Atrial Septal Defect
Pancoast Tumor affects what part of the lung?
MC Symptoms?
Carcinoma that occurs in APEX of lung
SHOULDER pain is MC sx – Unilateral symptoms d/t damage to AUTONOMIC GANGLIA
Stellate Ganglion damaged – LMN pattern
Hoarseness (Recurrent laryngeal nerve)
Horner syndrome – Ipsilateral ptosis, Miosis, Anhidrosis
– Pupil Asymmetry more prominent w/ DIM LIGHT
Erythema Multiforme most commonly presents with which type of infection?
Herpes Simplex Virus
Rash looks like Targets with rings surrounding them
G6PD deficiency labs?
What type of Anemia?
Normocytic – Intrinsic hemolytic anemia
Patients present with back / abdominal pain along with DARK URINE a few days after oxidant stress i.e. Sulfa Drugs, Dapsone, Fava beans
Increased Reticulocyte count produced by bone marrow in response to the anemia
Increased Indirect bilirubin from the hemoglobin degradation
Increased Lactate dehydrogenase – breakdown of hemoglobin
DECREASED Haptoglobin – Haptoglobin binds free hemoglobin, so naturally it decreases due to hemoglobin destruction
Heinz Bodies – Denatured hemoglobin inclusions
Bite Cells
Posterior Hip Dislocation affects what nerve?
Sciatic nerve & Inferior gluteal nerve (Gluteus maximus)
Affected leg appears shortened & internally rotated
Arginase Deficiency is a part of what cycle?
Urea Cycle
Arginase is a urea cycle enzyme that produces Urea & Ornithine from Arginine
Deficiency leads to spastic diplegia, growth delay, & abnormal movements
Huntington Disease Inheritance?
Neurotransmitter’s that are high? low?
Presentation?
Autosomal Dominant – CAG repeats – Toxic Gain of Function
INCREASED dopamine (Hence the Chorea)
DECREASED GABA & Acetylcholine
Chorea, Psychiatric symptoms, & Subsequent dementia
Atrophy of the CAUDATE & Putamen
Albinism caused by deficiency of what enzyme?
Tyrosinase
Tyrosinase is responsible for conversion of DOPA to Melanin – w/o it you don’t get melanin you stupid fuck
What is Potter Sequence?
Due to severe Oligohydramnios
Urinary tract anomaly – Bilateral renal agenesis = Decreased Urine output = Very low amniotic fluid = Baby is getting crushed and everything is flat
Lungs get crushed, Pulmonary Hypoplasia = Hypoxia, which is the MCC death
Polyhydramnios associated with which fetal malformations?
Esophageal & Duodenal atresia
So now baby can’t swallow amniotic fluid which results in a LOTTTT of it
Also associated with Anencephaly
What is HY about Charcot-Marie-Tooth?
Abnormal function of Myelin Protein
Progressive hereditary nerve disorders d/t defective production of proteins needed for peripheral nerves / myelin sheath
HY association – Foot Deformities – Hammer toe & Pes Cavus (High Foot Arches that don’t flatten)
LE weakness & Sensory deficits
CMT1A is most common
Differentiate from Duchenne & Becker because these disorders have PSEUDOHYPERTROPHY rather than Atrophy of the calf muscles
Toxic Shock Syndrome pathogenesis?
Superantigens (Exotoxins) bind to MHC-II & then nonspecifically activate T cells – This activation causes a huge release in inflammatory cytokines
Left-Dominant Heart supplies AV node by which artery?
Right-Dominant Heart supplies AV node by which artery?
Left Circumflex in left heart
Right coronary in right heart (90%)
Tenofovir main SE?
Can damage Proximal Tubule – AKI
Specific focal damage to the proximal tubule is the key here
PTH given in a PULSATILE manner has what affect on bone?
ANABOLIC
Stimulates osteoblast proliferation & Increases formation of new bone
- literally opposite of what PTH normally does. Regularly, Increased PTH would induce osteoclasts to breakdown bone which then increases serum calcium
HY for Membranous Nephropathy?
If it’s Primary = IgG-4 Antibodies to Phospholipase-A2 receptor
Secondary = NSAID, Penicillamine, HEPATITIS, SLE
GMB Thickening
GRANULAR d/t immune deposition
SUBEPITHELIAL DEPOSITS
How to Differentiate Kawasaki vs. Hand/foot/mouth
HFM has Oropharyngeal sores
Kawasaki HY?
FEVER for MORE than 5 days – Doesn’t subside w/ medication
Children UNDER 5 YO
Bilateral Conjunctivitis
Fissured lips w/ STRAWBERRY tongue
Distal Extremity changes – erythema & edema
Gastric surgery to place a band around the stomach must pass through which structure?
Lesser Omentum – Divided into hepatogastric & hepatoduodenal ligaments
VSD Murmur type? pathology?
Holosystolic murmur @ left lower sternal border (Tricuspid Area)
Insufficient development of inter-ventricular septum
H. Pylori is Urease positive which means what?
Urease splits Urea into Co2 & AMMONIA – Neutralizes local acidic gastric pH
How to Glucocorticoids lead to loss of bone density?
They inhibit replication & differentiation of OSTEOBLAST precursor cells, Increase osteoclast activity & promote renal calcium wasting
Which diuretic is used for its beneficial effect on calcium homeostasis - thus helping fight osteoporosis?
Thiazide Diuretics – HCTZ
Inhibits NaCl reabsorption in DCT – DECREASES CALCIUM EXCRETION
SE = Hyperglycemia, Hyperlipidemia, Hyperuricemia, Hypercalcemia
Hypokalemic metabolic alkalosis
What cells proliferate during lung injury?
Type II Pneumocytes
Source of pulmonary surfactant & produce Type I Pneumocytes
When they mention alveolus / alveolar in the question… Pick TYPE 2 2 2 2 22 2 2 2 22 2 2 2 2 2
What results in an abnormal connection b/t the bladder & the umbilicus?
Patent Urachus (Median umbilical ligament)
if it’s fully patent – Urinary leakage through your belly button
Partial patency results in bladder diverticulum & Increases UTI risk
Patients with previous BCG vaccination can have what type of TB test?
FALSE POSITIVE – not false negative
Why would a patient with an active TB infection have a negative TB test?
Impaired cell mediated immune response – Inadequate lymphocytic response
Hyperthyroidism causes an up-regulation of beta-adrenergic receptor expression – Leads to increased catecholamine effect
So giving a patient Propranolol works by doing what?
Reduce the conversion of T4 to T3 by inhibiting 5-Monodeiodinase in peripheral tissues (Similar to PTU & Glucocorticoids)
Candida Vulvovaginitis signs / symptoms?
Labs?
Treatment?
Pruritic w/ inflammation & Thick white COTTAGE CHEESE PUSSY
Normal pH & Pseudohyphae
Associated w/ Recent antibiotic use that decrease gram (+) Lactobacillus
FLUCONAZOLE
Bacterial Vaginosis signs / symptoms?
Labs?
Treatment?
Thin white discharge with FISHY SMELLY PUSSY
Clue cells with pH >4.5 (Alkaline) & (+) KOH whiff test
Metronidazole / Clindamycin
Trichomonas Vaginitis signs / symptoms?
Labs?
Treatment?
Inflamed STRAWBERRY PUSSY
Frothy YELLOW GREEN foul discharge
Motile Pear-shaped & pH >4.5 (Alkaline)
Metronidazole & Treat SEXUAL PARTNERS
What causes neonatal hypoglycemia?
Infants of Diabetic mothers
Maternal hyperglycemia causes fetal hyperglycemia w/ a compensatory HYPERFUNCTIONING PANCREAS
This hyperfunctioning causes hyperinsulinemia – so after birth this increased insulin leads to transient hypoglycemia
Growth of Candida on sputum cultures almost always indicates what?
Oral contamination of normal flora rather than pulmonary infection
Normal morphological changes of an aged heart?
Decreased LV chamber
Sigmoid shaped ventricular septum
Myocardial atrophy w/ collagen deposition
Brown Lipofuscin pigment (lipid oxidation)
Which NRTI is contraindicated in patients with the HLA-B*57:01 Allele?
Abacavir
Which Hepatitis virus Lacks a 3’-5’ Exonuclease activity?
Hepatitis C
– So it can’t proofread, which then causes antigenic variation of the enveloped proteins
Cavernous sinus syndrome has what affect on CN?
Compresses / stretches the Abducens Nerve causing weakness of the Lateral rectus muscle
What effect does low BP have on Baroreceptor firing & ANP?
Low BP = Decreased arterial distension = REDUCED firing of aortic & carotid baroreceptors
This leads to Increase in sympathetics = Increased contractility
& since we have Hypovolemia, there is less atrial stretch which means DECREASED ANP
Overall – INCREASED contractility & DECREASED baroreceptor firing & ANP
Narcolepsy CSF levels of Hypocretin-1?
DECREASED Hypocretin-1 in CSF
Shortened REM latency – knocking the fuck out fast
Cataplexy
Urinary concentration of which substance is indicative of early diabetic nephropathy?
ALBUMIN
Polymyositis characterized by what?
Most often involves what body part?
Overexpression of MHC-1 leads to infiltration with CD8+ T Lymphocytes w/ endomysial inflammation
Anti-Jo-1
How to differentiate b/t Polymyositis & Dermatomyositis?
Dermatomyositis Involves Gottron papules – Heliotrope edema of the eyelids
Hyperkeratosis of the skin
Perimysial inflammation w/ CD4+ T cells
(Vs. CD8+ in Polymyositis)
Polymyalgia Rheumatica characterized by? What does it NOT have that polymyositis has?
Myalgias of the shoulder & pelvic girdle w/ fever & weight loss
NO WEAKNESS (differ from polymyositis)
Almost exclusively in patients over 50 (& Females)
Increase in ESR & CRP
Digoxin MOA & SE?
Direct inhibition of Na/K ATPase
Increases intracellular calcium to increase contractility. Stimulates Vagus nerve = Decreases HR
HYPERKALEMIA – sign of digoxin toxicity
Blurry yellow vision
Underlying Hypokalemia is the MCC of Digoxin toxicity
How does infection lead to leukocytosis?
Neutrophils are most prominent in blood, however, the vast majority are held in reserves in the BONE MARROW
Infection mobilizes the BM – Mediated by increased cytokines i.e. TNF alpha – This then demarginalizes neutrophils from endothelial attachment & contributes to leukocytosis
What is the greatest risk factor for infection from Clostridium Septicum?
Underlying colonic malignancy
C. Septicum is the MCC of spontaneous gas gangrene
Spironolactone in patients w/ cirrhosis is used to induce natriuresis without blocking what?
W/o blocking the critical vasoconstrictive effects of Angiotensin
Commonly used w/ Furosemide (Loop)
Background: Cirrhosis – Portal hypertension leads to splanchnic vasodilation – this decreases the effective arterial volume & Lowers BP – This drop causes RAAS activation which promotes vasoconstriction (Angiotensin) & Fluid & sodium retention by kidneys (Aldosterone)
Aldosterone antagonist (Spironolactone) interrupts the cycle but allows the vasoconstriction to occur while getting rid of the ascites
HOCM leads to obstruction of what?
Left ventricular outflow tract d/t hypertrophied interventricular septum & abnormal anterior motion of the MITRAL VALVE
Placental abruption pathophysiology?
Rupture of the maternal vessels @ the uteroplacental interface – Leads to placental separation from the myometrium
Tender & firm uterus
Abrupt, Painful vaginal bleeding in 3rd trimester
Placenta Accreta pathophys?
Trophoblast attaches to myometrium w/o invading it
Diagnosed after baby delivery – Postpartum hemorrhage
Placenta Previa pathophys?
Extension of placental tissue over the cervix
MCC of PAINLESS vaginal bleeding in the 3rd trimester
Hyper-IgM Syndrome pathophysiology?
Labs?
Defective CD40L on CD4+ Lymphocytes – Impaired T cell activation of B cells
NO GERMINAL CENTERS
ONLY increased IgM, everything else is decreased
Recurrent Sinopulmonary infections, GI (Giardia) & Pneumocystis
Fungal & CMV
Background - CD40 Ligand is on the surface of CD4 cells. This CD40 Ligand binds to the CD40 on B cells – a necessary step for class-switching, allowing them to produce IgE, IgA, & IgG INSTEAD of IgM & IgD
So, defective CD40L defectiveness is unable to generate anything other than IgM
CD40 is expressed on B cells. CD40-Ligand is expressed on T cells
Nonclassical congenital adrenal hyperplasia is due to a mild deficiency in what enzyme?
21-Hydroxylase
21-Hydroxylase converts Progesterone to 11-Deoxycorticosterone
It also converts 17-Hydroxyprogesterone into 11-Deoxycortisol
Since this is Nonclassical (Meaning not @ birth) – Presents as adolescent girls w/ signs of Hyperandrogenism (Hirsutism, acne)
INCREASE in 17-Hydroxyprogesterone & Testosterone
Increase in potassium & sex hormones
Decrease in mineralocorticoids & BP & Cortisol
Which enzyme converts Testosterone to Estradiol?
Aromatase
Aromatase deficiency therefore leads to primary amenorrhea, NO breasts, & virilized genitalia (BIG CLIT)
What is a Pathognomonic finding of Chediak-Higashi syndrome?
GIANT CYTOPLASMIC GRANULES within neutrophils
CHS = Defect in lysosomal trafficking
Microtubule dysfunction in phagosome-lysosome fusion
ALBINISM & Neurologic sx (Nystagmus)
Background: Neutrophil phagosome-lysosome fusion is defected = prevents release of cytotoxic granules = Can’t kill bacteria
What’s the best medication to use in patient’s having a Hypertensive emergency but with Renal insufficiency?
Fenoldopam – Peripheral Dopamine-1 Receptor AGONIST
Vasodilates systemic & RENAL ARTERIOLES to lower BP but also increasing renal perfusion & NATRIURESIS
Donepezil is 1st line TX for?
Alzheimer’s – provides improvement of cognitive symptoms BUT does NOT alter the inevitable disease progression
AChE Inhibitor
When you hear FIXED WIDE SPLITTING OF S2 – Automatically what?
What murmur is associated?
ASD
Midsystolic ejection murmur – due to increased flow from pulmonic valve
Background: ASD cause fixed wide splitting of S2 d/t right-sided volume overload d/t left-to-right shunting
When left uncorrected, defects lead to irreversible hypertrophy of the pulmonary arteries w/ pulmonary HTN & reversal right-to-left shunting (i.e. Eisenmenger syndrome)
Old man with bilateral flank pain & Renal dysfunction - What is causing it?
Urethral compression due to BPH
NF type 1 has what type of inheritance?
Autosomal Dominant w/ 100% penetrance – Gene located on Chromosome 17
* Cafe Au Lait Spots
* Optic gliomas
* Seizures
* Pheochromocytomas
NF type 2 is also AD but on CH. 22
During skeletal muscle contraction – Calcium released from SER binds to what?
Calcium binds to Troponin C – Allowing the binidng of actin to myosin
Lats innervated by what nerve?
Thoracodorsal Nerve
What is a Capitation Payment model?
The Payor pays a fixed & predetermined fee to provide all services required by a patient
Used by some HMOs
MEN Type 1 classified by what?
Primary Hyperparathyroidism (Parathyroid adenomas or hyperplasaia)
Pitutiary tumors (Prolactin, visual defects)
Pancreatic tumors (Gastrinomas)
Only MEN with Pituitary & Pancreatic involvement & MEN-1 Mutation
MEN type 2 classifed by what?
Medullary thyroid cancer (Calcitonin)
Pheochromocytoma
Primary Hyperparathyroidism
RET Mutation
MEN type 3 classified by what?
Medullary Thyroid cancer (Calcitonin)
Pheochromocytoma
Mucosal Neuromas & Marfanoid habitus
RET mutation – Only one without hyperparahyroidism
What is the most common cause of T1D?
Autoimmune Insulitis w/ Progressive beta cell loss
Leukocytic infiltration of islets by inflammatory cells is called insulitis
PPI MOA? SE?
Irreversibly inhibit parietal cell Hydrogen-Potassium-ATPase
MALABSORPTION of Calcium, Iron, Magnesium, & B12
However, enhances Fat absorption in patients given pancreatic enzyme replacement therapy
Pneumonia, Osteoporosis
Loperamide MOA?
Opioid AGONIST – Binds to mu opiate receptors – inhibits the release of Acetylcholine release – Slows Peristalsis
Traveler’s Diarrhea
DOES NOT CROSS BBB – Avoiding systemic opiate-related adverse events
Jersey Finger affected tendon?
Flexor Digitorum Profundus
Susceptible to rupture when an actively flexed DIP joint is forcefully hyperextended
Rupture = Inability to flex
Guillain-Barre Syndrome mechanism? Presentation?
Immune mediated polyneuropathy due to cross reacting Antibodies – MOLECULAR MIMICRY
Following Viral illness or mainly Campylobacter
Affects Schwann cells & Myelin sheaths
SYMMETRICAL ASCENDING WEAKNESS beginning @ legs
Postpartum thyroiditis differentiating features?
Hyperthyroid phase for first 1-3 months d/t release of PREFORMED THYROID HORMONE
Hypothyroid phase d/t depletion of thyroid hormone stores
Recovery phase w/ euthyroid state
Autoimmune disorder w/ lymphocytic thyroid inflammation
DECREASED Radioiodine uptake & Decreased blood flow on doppler US
Positive TPO
Polyarteritis Nodosa mainly involves which vessels?
What is it commonly associated with?
Typically involves Renal & Visceral vessels – NOT pulmonary
Transmural inflammation w/ Fibrinoid necrosis
Hepatitis B Seropositivity (Sometimes Hep C)
Rhabdomyolysis pathogenesis? Cause?
Myoglobin, Electrolyte release d/t myocyte injury
Common with crush injuries, SEIZURES, & Statins
Can cause ATN due to heme pigment released from myoglobin
Positive BLOOD on urine dipstick in the ABSENCE of RBC suggests myoglobinuria
Severe tetraology of Fallot neonate w/ cyanosis immediate administration of which drug?
Prostaglandin E1 – Prevents closure of the ductus arteriosus
Regardless of hydration status – majority of water reabsorption in the nephron occurs where?
Proximal tubule
Apixaban, Edoxaban, & Rivaroxaban MOA?
Uses?
Reversal?
Binds active site of Xa & Prevents the conversion of Prothrombin to Thrombin
Used as stroke prophylaxis in patients w/ AFib
Bleeding reversed with AndeXAnet
Acute Promyelocytic Leukemia is associated with which translocation?
Responds to what?
MC presentation?
15;17 Translocation
Responds to All-Trans Retinoic Acid
DIC is the most common presentation
Glucocorticoids increase the Apoptosis of which cells?
Increase the production of which Interluekin?
Eosinophils, T cells, & Monocytes
Increase IL-10 (Anti-inflammatory)
Testes drain into?
Scortum drains into?
Testes = Para-Aortic
Scrotum = Superificial inguinal
Anytime SVR is decreased + SVO2 Increased – What type of shock is it?
Distributive Shock – Sepsis / Anaphylaxis
Why is the Right Ventricle relatively protected after an MI?
Because it is perfused throughout the cardiac cycle
Vs. the LV only diffused during diastole
21-Hydroxylase catalyzes the conversion of what?
Conversion of Progesterone to 11-Deoxycorticosterone (Aldosterone precursor) – Zona Glomerulosa
Also Converts 17-Hydroxyprogesterone to 11-Deoxycortisol (Cortisol Precursor) – Zona Fasiculata
Deficiency means ACTH levels are increased as a result of low cortisol levels
High serum 17-Hydrooxyprogesterone confirms the DX
Barrett Esophagus epithelium?
What type of cancer are you prone to when you have Barrett’s?
You go from Nonkeratinized Stratified Squamous epithelium to Nonciliated COLUMNAR epithelium w/ Goblet cells
Increased risk of Esophageal ADENOCARCINOMA
Adenocarcinoma of the Distal 1/3 of Esophagus
Hydralazine MOA?
ARTERIAL Vasodilator that has little effect on veins
Increases cGMP & Reduces Afterload
Nitroprusside MOA?
SE?
Strong acting Vasodilator for both arteries & veins
SE = Cyanide poisoning
TX = Amylnitrite w/ B12 & then Methylene blue
What is Acanthosis Nigricans?
Hyperpigmented plaques @ flexural areas – Neck / & Armpit
Associated with Diabetes, Obesity, Malignancy
What is Actinic Keratosis?
Hyperkeratotic lesions in SUN EXPOSED areas of scalp, ears, face, & hands
Unpigmented
Risk of squamous cell carcinoma
What is Lichen Planus?
Polygonal, Planar, Pruritic, Purplish Plauqes on wrists, hands, trunk, legs
Fine white lines (Wickham Striae) present on plaque surface
Which nerve root is MC affected in patients with cervical radiculopathy?
C7 root
Deficits across the median & radial distributions
What arterial blood gas has the most powerful effect on cerebral blood flow?
PaCO2
Drop in PaCO2 d/t hyperventilation causes vasoconstriction
Lowering PaCO2 is one of the measures employed to reduce ICP in mechanically ventilated patients with cerebral edema
Which test can you run to confirm the diagnosis of Chronic Granulomatous disease?
Absence of fluorescence on dihydrorhodamine flow cytometry = Confirmatory
Carcinoid tumors are typically confined to the intestine – The secretory products of these tumors go through 1st pass metabolism and therefore don’t reach systemic circulation – But when they do, it is now called what?
Carcinoid Syndrome
METS has already reached the liver – Avoid 1st pass metabolism & now systemic issues
Flushing, Diarrhea, Bronchospasm
Increased 5-HIAA in urine
Can also cause Pellagra – Niacin deficiency
TX = Octreotide
Hepatic Angiosarcoma associated with exposure to?
Vinyl Chloride / Arsenic
Express CD31
Hepatic Adenoma MC in which population?
What are main risk facotrs?
Women
OCP’s & Steroids
Rupture & shock
What is increased in Hemophilia?
Only PTT; all other tests normal
Autosomal Recessive
Colorectal cancer has bad prognosis when the tumor penetrates what strcuture?
Muscularis Propria
Anal Squamous Cell Carcinoma strong association with which virus?
Histology?
Strong HPV association
Presents w/ Rectal bleeding, Pruritis, Pain, Ulcerated anal lesion
Histology will show Eosinophilic squamous cells arranged in islands
VIPoma Leads to hypersecretion of VIP – What electrolyte abnormalities do you see?
Increased VIP = Excess chloride LOSS in the stool along w/ Water, Na+ & K+
HYPOKALEMIA
WATERY DIARRHEA
VIP inhibits Gastric acid secretion
TX = Somatostatin
Glucokinase where?
Hexokinase where?
Glucokinase – Liver
Hexokinase – Brain, Heart, Kidney
D-Xylose in stool is a sign of what?
Osmotic Diarrhea
D-Xylose test is ABNORMAL in Celiac disease
4 causes of Secretory diarrhea?
Vibrio Cholera – Curved
VIPoma
ETEC - E. Coli
Cryptosporidium
V-VEC
Increase in Insulin & C-Peptide?
Endogenous insulin production / Insulinoma
i.e. Sulfonyureas (Glyburide)
This is considered endogenous because these medications are increasing insulin production – therefore it is also producing C-peptide vs. exogenous insulin which is just an analog
Also increased in Insulinomas