- Increased ICP signs (nausea and vomiting) in conjunction with cerebellar signs of nystagmus and truncal ataxia in a child…BUZZ… for medulloblastoma - Most common malignant brain tumor in children (arises from cerebellum)

- Nodular collections of lymphoma cells in lymph node - Express bcl-2 (anti-apoptotic gene) as a result of t (14,18) translocation - IgH locus on chromosome 14 - Chromosome 18 = bcl2 - Presents in middle-aged adult with waxing and waning painless LAD - Tx with: RITUXIMAB (non-hodkin B-cell lymphomas, CLL, RA, ITP) and/or antimetabolite or cytotoxic chemotherapies

- Acalculia/dyscalculia (bad math) - Agraphia/dysgraphia (bad handwriting) - Finger Agnosia (whose finger is this?) - Left-right confusion - The ability to read and speak remains intact

RX: Flash 251 Flashcards by Keegan Sturgill

Medulloblastoma

Increased ICP signs (nausea and vomiting) in conjunction with cerebellar signs of nystagmus and truncal ataxia in a child…BUZZ… for medulloblastoma
Most common malignant brain tumor in children (arises from cerebellum)

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Headache and symptoms of ICP (nausea and vomiting)
Hypercellular multinucleated tumor cells with pseudopalisading necrosis
BUTTERFLY MRI

Glioblastoma Multiforme (MOST COMMON PRIMARY ADULT BRAIN TUMOR)

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Fired egg appearance
a relatively rare, slow-growing tumor that is most often found in the frontal lobes
Symptoms= headaches, seizures, changes in cognition, localized weakness, sensory loss, or aphasia

Oligodendroglioma

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Histology: small cells with reduced cytoplasm and crescent-shaped, deeply staining nuclei (due to high mitotic activity). Tightly packed sheets of anaplastic cells forming HOMER-WRIGHT ROSETTETS (considered pseudorosettes because central space is filled with neutrophil as opposed to a lumen in a true rosette.
Radiotherapy for Tx

Medulloblastoma

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Follicular Lymphoma

Nodular collections of lymphoma cells in lymph node
Express bcl-2 (anti-apoptotic gene) as a result of t (14,18) translocation
IgH locus on chromosome 14
Chromosome 18 = bcl2
Presents in middle-aged adult with waxing and waning painless LAD
Tx with: RITUXIMAB (non-hodkin B-cell lymphomas, CLL, RA, ITP) and/or antimetabolite or cytotoxic chemotherapies

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MEN I (multiple endocrine neoplasia type I)
-Mutation in MENIN gene (chromosome 11)

Tumors or hyperplasia of pancreas, pituitary, and parathyroid
Hyperparathyroidism, which causes hypercalcemia and hypophosphatemia.
Galactorrhea can be highly suggestive of pituitary prolactinoma and multiple duodenal ulcers are nearly pathognomonic for Zollinger-Ellison Syndrome, caused by a gastrinoma (gastrin-secreting tumor of the pancreas or duodenum)

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Express bcl-2 (anti-apoptotic gene) as a result of t (14,18) translocation
IgH locus on chromosome 14

Follicular Lymphoma

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the most common supratentorial tumor in children

- derived from remnants of Rathke pouch (surface ectoderm)

Craniopharyngioma

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A benign primary intracranial neoplasm localized to meninges
Clinically: Seizures and neurologic symptoms that worsen due to mass effect
Whorls of uniform meningothelial cells with psammoma bodies (black arrow on image below)

MENINGIOMA

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Craniopharyngioma: symptoms and age?

Symptoms: severe headaches, visual changes, and growth failure secondary to pituitary dysfunction.
Bimodal: children and 5th decade of life

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Ceftriaxone MOA

-Inhibits bacterial transpeptidase

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VHL so called classic triad (even though only seen in 10%)

Flank pain, palpable flank mass, and hematuria
VHL is associated with the deletion of VHL gene on chromosome 3.
Image shows bilateral renal cell carcinoma

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Located in the left posteroinferior frontal gyrus
Responsible for speech production
Lesions here = good speech comprehension, but significant difficulty with written and verbal expression.
Will see prominent frustration over their deficit

Broca Area

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Lymphoblastic leukemia is indicated by greater than 20% leukemic blast.
Neutropenia (because the rapid proliferation of WBCs crowds out production of other cells and causes bone marrow failure)

ALL

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C7 radiculopathy

- Motor defects of triceps and sensory abnormalities of the middle finger

C6-C7 disc herniation

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What paraneoplastic syndromes are thymomas most commonly associated with?

-Myasthenia gravis, pure RBC aplasia and hypogammaglobinemia

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-Results from damage to the visual association cortex, which is located in the angular gyrus

Gerstmann Syndrome

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Iron deficiency anemia, beefy-red tongue, dysphagia, and esophageal web
Anemia may also present with atrophic glossitis, angular cheilitis and koilonychia

Plummer-Vinson Syndrome

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Gerstmann Syndrome

Acalculia/dyscalculia (bad math)
Agraphia/dysgraphia (bad handwriting)
Finger Agnosia (whose finger is this?)
Left-right confusion
The ability to read and speak remains intact

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Infarcted radial nerve (BEST)

Wrist drop
BEST (radial nerve innervates)
Brachioradialis, Extensors of wrist and fingers, Supinator, and Triceps
Can be caused by diabetes, vasculitis, sarcoid, leprosy, and HIV

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Subscapularis muscle action?

Medially rotates and adducts the arm

- Innervated by upper and lower subscapular nerves

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Four muscles of mastication

Lateral pterygoids lowers/opens the jaw
Lateral lowers, M’s Munch
Medial pterygoids, Masseter, teMporalis close the jaw

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Adults with vitamin D and calcium deficiencies

- May present with pathologic fractures or bone pains and a waddling gait

Osteomalacia

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Hypercalcemia
Dehydration
Acute Renal failure
Anemia
Bone pain
Punched out boney lesions
Pathological fracture

Common multiple myeloma findings:

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Wernicke Aphasia

- Fluent aphasia with marked impaired comprehension - Lesion in the superior temporal gyrus aka Wernicke area (associate) - Wernicke area supplied by MCA

Myxoma

- Most common primary cardiac tumor. - Amorphous extracellular matrix - Left atrium blocks mitral valve (M. Cyrus story)

Trouble swallowing after head and neck surgery

- Injury to superior laryngeal nerve | - Branchial (pharyngeal) arch 4

Do you remember the different causes of dilated cardiomyopathy?

- ETOH: directly toxic (disrupt cytochrome c) - Beriberi: B1 (thiamine) deprived from mito of myocytes - Chagas: T. Cruzi neurotoxin directly damages specialized conduction cells of heart - Cocaine: Disrupts mito membrane - Doxorubicin: intercalates myocytes DNA - Hemochromatosis: ROS

Damage to myocytes→ decrease in systolic function→ increase in wall tension→addition of sarcomeres in SERIES (eccentric)/ can stretch→increase wall thickness to decrease tension → CHF over time due to: decreased ventricular function→decreased EF→Decreased CO

Dilated cardiomyopathy

- Dyspnea, orthopnea, fatigue - Lateral displacement of the point of maximal impulse - S3 gallop - Weight gain - Heart 2-3X normal size

Dilated cardiomyopathy

Medications that have a proven mortality benefit in heart failure with systolic dysfunction include:

- Beta-blockers (can not use in decompensated heart failure) - ACE inhibitors (could use furosemide for decompensated heart failure but it has only symptomatic benefit and no mortality benefit) - Spironolactone

- Triad: confusion, nystagmus and ataxia - History of alcoholism or malnutrition - Treat with supplemental thiamine - Thiamine (a cofactor for oxidative decarboxylation of alpha-keto acids) - Reversible if hasn’t progressed to Korsakoff syndrome (irreversible)

Wernicke Encephalopathy

Bulbus cordis

-Smooth parts of both left and right ventricles out flow tracts

Wernicke Encephalopathy: triad?

-Triad: confusion, nystagmus and ataxia

Primitive atrium/ventricle

-Trabeculated regions of atria/ventricles

Sinus Venosus

- Coronary sinus (left horn) | - Smooth part of right atrium (right horn)

Truncus arteriosus

- Origin of the aorta and pulmonary trunk | - With neural crest cells forming the aorticopulmonary septum between the two arteries

- Berry like cytoplasmic inclusion in macrophages and neutrophils - Tick vector - Fever, headache and rash - Parasite Ehrlichia chaffeensis (Order: Rickettsia)

Ehrlichiosis (monocytic)

- UMN and LMN deficits (weakness seen in both) | - Characterized by anterior horn and lateral corticospinal degeneration

ALS

UMN signs

increased activity of neuromuscular junction thus increased muscle tone, spasticity and hyperreflexia. Positive Babinski.

- UTI (untreated)→infect ureters and kidneys→acute kidney failure→hyperkalemia→cardiac arrhythmias→peaked T-waves on ECG - Peaked T-waves on ECG = dangerous levels K+

Hyperkalemia (weird representation)

- Centrally located bronchogenic carcinoma - Hypercalcemia, smoking history, mass in lung on x-ray suggest lung tumor that secretes PTHrP - Excess PTHrP→leads to decreased serum phosphate level and hypercalcemia (constipation and polyuria…large amount of dilute urine)

Squamous cell carcinoma

Wallerian degeneration (orthograde degeneration)

- Process whereby peripheral nerve fibers (generally the axon) are cut off from the soma (source of metabolic nourishment) - After nerve dies (calcium is liberated)→activates enzymes that destroy the axon - Process begins within a day after the injury→becomes apparent about one week after injury. - Over next one to two months’ muscle will undergo atrophy (angulated, small muscle fibers)

- Presents with dyspnea on exertion and minimal physical exam finding - Murmur= an opening snap followed by a late diastolic rumble - Left atrial enlargement (hoarseness in voice)

Mitral Stenosis

- Anti-microbial agent used for Pneumocystis jirovecii prophylaxis in patients who are not candidates for trimethoprim-sulfamthoxazole. - Acts via inhibition of mitochondrial electron transport.

Atovaquone

- Most common type or arthritis in children - Younger than 16 usually (more females) - Non-migratory polyarthritis - Joint swelling - Migratory rash - Fever, HSM (and other systemic symptoms. - Positive RF in less than 15% of cases - Elevated ESR and neutrophils in absence of infectious cause - Uveitis can develop and lead to BLINDNESS

Juvenile idiopathic arthritis (JIA)

- Failure of aortopulmonary septum to SPIRAL - Presents as neonatal cyanosis - Associated with diabetic mothers

Transposition of great vessels

Mid-shaft injuries or fractures of the humerus

- Can damage the two structures that run in the radial groove: - Brachial artery - Radial nerve- forearm weakness due to atrophy wrist extenders

What does the 3rd aortic arch gives rise to?

-Common carotids as well as proximal part of the internal carotids

- Acute, autoimmune, demyelinating polyradiculoneuropathy that affects the PNS. - Ascending paralysis - Loss of deep tendon reflex - Autonomic dysfunction - Tx: respiratory support, IV immunoglobulin’s or PLASMAPHERESIS

Guillain-Barre

In children 0-6 months old what are the most common causes of bacterial meningitis?

Group B streptococci- gram-positive cocci (most common due to vagina colonization) - Escherichia coli- gram-negative rod. Ferments lactose quickly (pink) as seen on MacConkey agar below. - Listeria- gram-positive rod

- Infarct of the anterior spinal artery - Characterized by: contralateral muscle weakness, ipsilateral tongue deviation and contralateral loss of sensation from the body (sparing the face)

Medial medullary syndrome

-Codes for a protein involved in cytoskeleton components responsible for contact inhibition of tumor progression

Merlin

- Lyme disease - Reservoir= white-footed mouse - Vector= Ixodes tick

Borrelia burgdorferi

- Inflammation of subcutaneous fat - Fever and malaise may also be present - Associate with IBDs (Crohn and UC), sarcoidosis, meds (oral contraceptives and sulfonamides), certain malignant neoplasms and infections (tuberculosis, beta-hemolytic streptococci, coccidioidmycosis, histoplasmosis and leprosy).

Erythema Nodosum

- NF2 (AD) - Chromosome 22 - Codes for a protein involved in cytoskeleton components responsible for contact inhibition of tumor progression - NF2 findings can include: blurry or cloudy vision due to juvenile cataracts and meningioma’s.

Merlin

- Chromosome 3 defect in VHL gene | - Produce hemangioblastomas that appear as highly vascular foamy cells

VHL

- Autoimmune hypothyroidism - Cold intolerance, hypoactivity, weakness, diminished reflexes, dry and cool skin and coarse hair. - Increased TSH (most sensitive test)

Hashimoto thyroiditis

- Increased TSH (most sensitive test) - Goitrous and firm thyroid gland - Lymphocyte and plasma cell infiltrate with germinal centers - Colloid-sparse follicle and HURTHLE CELLS (acidophilic cells) - HLA-DR5 and B5 - Anti-TPO (thyroid peroxidase) or anti-thyroglobulin

Hashimoto thyroiditis

Anti-smooth muscle

-Hepatitis

Anti-centromere

-Crest syndrome

Anti-U1 RNP

-Mixed connective tissue disease (pre-mRNA splicing defect)

Congenital Diaphragmatic Hernia

- Bowl protrudes through hernia (left usually) - Lungs can’t develop fully (hypoplasia) - Pushes mediastinum to right (hear heart sounds on right)*NOT KARTs.. - Pleuroperitoneal folds failing to fuse with other components of the diaphragm or failing to develop all together.

What four fetal structures is the diaphragm derived from? | Hint- Several Parts Build Diaphragm

1) Septum transversum 2) Pleuroperitoneal folds 3) Body wall 4) Dorsal mesentery esophagus

-Pleuroperitoneal folds failing to fuse with other components of the diaphragm or failing to develop all together.

Congenital Diaphragmatic Hernia

- Analogs for gonadotropin-releasing hormone. - Make GnRH signaling flat instead of pulsatile (like normal) - FSH and LH levels fall - Lower LH → testosterone synthesis by Leydig cells is reduced

Leuprolide and Goserelin

- Granulocyte colony-stimulating factor - Recombinant cytokine used to augment bone marrow recovery - Post-chemo - Treats aplastic anemia and congenital neutropenia

Filgrastim

-Anti-androgen that acts by blocking androgen receptors and preventing the body from using sex steroids

Flutamide

- Suppresses cellular immunity by binding to intracellular protein FKBP-12 - Inhibiting the activation of T-lymphocytes

Tacrolimus

-Chemical modification of DNA, impairing replication -Inhibits topoisomerase II (unwinds supercoiled DNA) -W/out the enzyme→DNA breaks→degradation of DNA -Rx for small cell carcinoma of lung and prostate -Rx for testicular carcinoma ADR: myelosuppression, alopecia, nausea and vomiting

Etoposide

- Pain in the upper right quadrant (intermittent) - Eating typically exacerbates pain - Tx is surgical CHOLECYSTECTOMY

Cholelithiasis (GALLSTONES)

- Periumbilical pain that migrates to the right lower quadrant - Accompanied by nausea/vomiting and decreased appetite

Appendicitis

Fluoxetine

-SSRI used for depression and anxiety (and others)

Crohn Disease vs. Ulcerative colitis: Inflammation

Crohns: transmural UC: mucosal/submucosal

True or false? Crohns disease has noncaseating granulomas?

True. | Note UC has crypt abscesses and ulcers, bleeding, NO granulomas

friable mucosal polyps with hanging mesentery

Ulcerative Colitis (UC)

Cobblestone thickening of intestinal wall, linear ulcers, and fistulas

Crohn Disease

Samter’s Triad: Aspirin-sensitive asthma

-Nasal polyps, asthma and aspirin sensitivity

- Used for acute mania and mood stabilization in BPD | - Very narrow therapeutic window (MONITOR SERUM LEVELS)

Lithium

Drugs commonly associated with ASTHMA attacks:

- Aspirin, coloring agents like tartrazine, Beta-antagonists, sulfating agents - Asthma is triggered by: exercise (especially in the cold), viral URIs, allergens, and stress

Myasthenia Gravis Treatment: PYRIDOSTIGMINE

- Pyridostigmine is an acetylcholinesterase inhibitor used in management of CHRONIC myasthenia gravis (duration of 3-6 hours) - Neostigmine has same action but duration of only 2-4 hours - Edrophonium has same action but is only used in the Tensilon test to help diagnose myasthenia gravis (duration is only 19 minutes)

- Stimulates muscarinic cholinergic receptors | - DRUG OF CHOICE: for emergency lowering of intraocular pressure of both closed-angle and open-angle glaucoma

Pilocarpine

Lipid soluble substances and ADEK (fat soluble vitamins) absorption

Ex: Vitamin A→enters lacteals (small lymphatic capillaries in the villi of intestinal epithelium→lacteals merge and the chyle flows into progressively larger lymphatics until→thoracic duct Lipid soluble substances are not transported via the portal venous system but rather absorbed by the lymphatic system. The lymphatic system bypasses the liver at first, avoiding the first-pass effect.

Cystic Fibrosis (AR)

- Recurrent respiratory infections - Chromosome 7 defect in CFTR gene - Defective chloride channel in lungs and GI - Channel normally reabsorbs chloride from sweat and secretes chloride into the lungs and GI tract - High Cl- in sweat (diagnostic chloride sweat test) - Abnormally thick mucus in lungs - Mucus plugs block airways, facilitating infections thus… - N-Acetylcysteine acts to loosen mucus plugs that develop in lungs - Nutritional deficiencies also…some risk for Rickets

- Lymphoproliferative neoplasm - Typically young adults (especially young men) - Fever, night sweats, and weight loss - Reed-Sternberg (RS) cells (more= worse prognosis) - Tx: Vincristine, vinblastine, bleomycin, or doxorubicin

Hodgkin Lymphoma

- Microtubule inhibitor | - Treatment for the HODGKIN LYMPHOMA

Vincristine

- Stimulates host immune system (NK and cytotoxic T-lymphocytes) - First line treatment for hepatitis B - Also treats hepatitis C and some cancers - Dose-dependent neutropenia during first 4 weeks of therapy (returns to normal)

-Alpha-Interferon

RX: Flash 251 Flashcards

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