• Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

Card Range To Study

through

image

Play button

image

Play button

image

Progress

1/197

Click to flip

Use LEFT and RIGHT arrow keys to navigate between flashcards;

Use UP and DOWN arrow keys to flip the card;

H to show hint;

A reads text to speech;

197 Cards in this Set

  • Front
  • Back
Poststreptococcal Glomerulonephritis
Nephritic Syndrome
Most common in children
Type III Hypersensitivity
SUBEPITHELIAL Immune Complex Deposition; Granular on IF
Self Limited
Rapidly Progressive Glomerulonephritis (Crescentic Glomerulonephritis) [RPGN]
Nephritic Syndrome
Very Poor Prognosis
Causes: Pulmonary-Renal Syndromes
1. Goodpasture's Syndrome - Type II Hypersensitivity to GBM - Linear on IF - Male>Female
2. Wegener's Granulomatosis - Granulomatous disease - ELK - c-ANCA
3. Microscopic Polyarteritis - No granulomas - ELK - p-ANCA
Diffuse Proliferative Glomerulonephritis (SLE-Nephritis)
Normally a nephritic syndrome can also be nephrotic
Type III Hypersensitivity to dsDNA
SUBENDOTHELIAL IC Deposition - Granular IF
#1 cause of death in Lupus patients
Berger's Disease
Nephritic Syndrome, but often presents as Nephrotic Syndrome
Increased IgA Production
OFTEN FOLLOWS URI
MESANGIAL IC deposition
Other name: IgA Nephropathy
Alport's Syndrome
Nephritic Syndrome
Mutation in Type IV Collagen - BM dysfunction
Ass'd with eyes, ears (deafness), and kidneys
Membranous Glomerulonephritis
Nephrotic Syndrome
Most common cause of nephrotic syndrome in adults
Caused by Drugs, Infection, SLE
EM: Spike & Dome appearance; IF: Granular
SLEs nephrotic presentation
Minimal Change Disease
Nephrotic Syndrome - Massive proteinuria (>3.5g) and edema
Most common in kids (postinfectious)
RESPONDS TO CORTICOSTEROIDS
EM: podocyte foot process effacement. LM: Normal
Amyloidosis
Nephrotic Syndrome
Ass'd with MM, TB, RA, chronic conditions
Congo-red apple green birefringence
Diabetic Glomerulonephropathy
Basement Membrane gets glycosylated from high glucose load; causes thickening of GBM and efferent arterioles. --> increased GFR --> mesangial damage.
Kimmelstiel-Wilson "wire loop" lesions - acellular ovoid lesions in periphery.
Focal Segmental Glomerulosclerosis (FSGS)
Nephrotic syndrome - Proteinuria & Edema
Findings: LM: focal sclerosis and hyalinosis
Most common glomerular disease in HIV patients. Very severe in HIV pts
Membranoproliferative Glomerulonephritis (MPGN)
Nephrotic Syndrome
"Tram Track" appearance due to mesangial splitting.
Subendothelial IC deposition. Granular IF
HBV>HCV
Leads to Chronic Kidney Failure
Renal Tubular Acidosis
Type I - Distal - H+/K+ pump failure - Hypokalemia
Type II - Proximal - Defect in HCO3- reabsorption - leads to hypokalemia
Type IV - Hyperkalemic - Hypoaldosteronism --> High K+ --> Failure of Prox. Tubule to secrete NH3 to buffer H+ (Mech (I think): Cl-/NH3 countertxporter in prox. tubule activity decreased because Cl- needs to stay in tubule to maintain charge balance with high K+)
Calcium Kidney Stones
Most common Kidney stones (75-85%)
Often RECUR
Can be calcium phosphate or calcium oxalate.
Oxalate stones = ETHYLENE GLYCOL or Vit. C abuse.
Due to HYPERCALCEMIA:
Cancer
Hyperparathyroidism
High Vit. D
RADIOPAQUE
Struvite (Ammonium Magnesium Phosphate) Stones
2nd most common
ALKALINE STONES - WORSE with alkaluria
RADIOLUCENT
2ndary to UTI infxn with UREASE POSITIVE BUGS:
Proteus
Staph
Klebsiella
Can form Staghorn Calculi
Uric Acid Stones
2ndary to high uric acid levels
Common in:
Gout
Conditions with high cell turnover (Heme malignancies)
RADIOLUCENT
Cysteine Stones
2ndary to high cysteine levels in urine (such as Fanconi's syndrome or other AA wasting diseases)
Tx: Alkalinize Urine
Renal Cell Carcinoma
Most common renal MALIGNANCY
Men 50-70 most often affected
Invasion of IVC and hematogenous spread
Sx: Flank Pain, Hematuria, Fever, Weight Loss, 2ndary polycythemia
PARANEOPLASTIC SYNDROMES (common):
PTH --> Hypercalcemia
Epo --> 2ndary polycythemia
ACTH --> Cushing's Syndrome
Prolactin --> Gynecomastia & Infertility
Wilms' Tumor
Most common renal malignancy in YOUNG CHILDREN (2-4 yrs)
Associated with deletion of WT1 gene on Chromosome 11
Sx: Large Abdominal Mass.
Can be part of WAGR syndrome:
W = Wilms Tumor
A = Aniridia - ALWAYS PRESENT (malformation of iris)
G = Genitourinary malformation
R = Retardation
Transitional Cell Carcinoma
Can occur anywhere from Renal Pelvis to Bladder
Associated with Pee SAC:
P = Phenacetin (used as analgesic until 1983)
S = SMOKING
A = ANILINE Dyes
C = CYCLOPHOSPHAMIDE (also causes hemorrhagic cystitis)
Painless hematuria = BLADDER CANCER
Diffuse Cortical Necrosis
Bilateral Cortical Necrosis due to Infarction of both kidneys.
Most likely due to simultaneous Vasospasm & DIC
Causes:
OB complications (abruptio placentae)
SEPSIS
Pyelonephritis
ACUTE - WBC casts = pathognomic
Neutrophilic infiltrate
Sx: Fever; CVA tenderness
CHRONIC - Scarring
Tubules contain eosinophilic casts
Lymphocytic infiltrate
Drug Induced Interstitial Nephritis
Acute interstitial inflammation
Sx: Fever, Rash, Eosinophilia
TYPE IV HYPERSENSITIVITY - delayed; 2wks after administration
DRUGS:
Penicillin & derivatives
NSAIDs
Diuretics
Acute Tubular Necrosis
Most Common Cause of Acute Renal Failure in HOSPITAL
Causes:
Hypotension
Renal Ischemia
Crush Injury
Toxic Injury
GRANULAR CASTS (Epithelial Cell or Muddy-Brown)
REVERSIBLE (2-3 wks recovery)
Progression:
Insult --> Oliguria --> Recovery
Renal Papillary Necrosis
Sx: Gross Hematuria; Proteinuria
Causes:
Diabetes Mellitus
Acute Pyelo
Sickle Cell Anemia
Chronic Phenacetin Use (Acetaminophen)
Acute Renal Failure
Def: Acute Decline in Renal Function with ↑ CREATININE and ↑ BUN (Uremia).
Three types:
1. Prerenal
2. Intrinsic Renal
3. Post Renal
Consequences:
1. ↓ Epo --> Anemia
2. ↓ Vit. D --> Renal Osteodystrophy
3. ↓ K+ exc --> Hyperkalemia --> arrhythmias
4. ↓ H+ secretion --> ↓ HCO3 generation --> Metabolic Acidosis
5. Uremia --> Encephalopathy & Pericarditis (Serous & Fibrinous)
6. ↑ Na+ & H2O --> Fluid overload --> CHF/Pulm. Edema/HTN
Acute Renal Failure - Prerenal Azotemia
Due to ↓ RBF --> ↓ GFR.
No intrinsic kidney problem.
Kidney tries to ↑ blood volume. So ↑ Na/H2O/Urea Retention.
Findings:
↑ Urine Osmolality (>500)
↓ Urine Sodium
↓ Fraction of excreted Na (<1%)
↑ Serum BUN:Cr (Urea is reabsorbed)
Acute Renal Failure - Intrinsic Renal Problem
ARF --> ↑ BUN & Cr.
Normal causes:
ATN
Ischemia
Toxins
Necrosis of tubules causes impaired reabsorption. ↓ BUN, ↑ Cr. --> ↓ BUN:Cr. Ratio
Findings:
↓ BUN:Cr Ratio
Acute Renal Failure - Post-Renal
ARF --> ↑ BUN & Cr.
BILATERAL outflow obstruction
Causes:
Stones
BPH
Neoplasm
Fanconi's Syndrome
↓ Txport of EVERYTHING in PROXIMAL TUBULE
Common cause of Type II RTA
Causes:
Wilson's Disease
Glycogen Storage Disease
Drugs (Cisplatin)
Consequences:
Rickets/Osteomalacia (↓ PO3 reabs.)
Metabolic Acidosis (↓ HCO3- reabs.)
Hypokalemia (↓ proximal Na+ reabs --> ↑ distal Na+ reabs.)
Adult Polycystic Kidney Disease (ADPKD)
MULTIPLE, LARGE, BILATERAL RENAL CYSTS causing DESTRUCTION of PARENCHYMA

Sx: Hematuria, Flank Pain, HTN (↑ Renin), Prog. Renal Failure

Autosomal Dominant mutation in APKD2 gene

Ass'd with:
1. Polycystic Liver Disease
2. Berry Aneurysms (2° to HTN)
3. Mitral Valve Prolapse
Infantile Polycystic Kidney Disease (ARPKD)
Infantile Presentation of MULTIPLE, LARGE, BILATERAL RENAL CYSTS causing DESTRUCTION of PARENCHYMA

Autosomal Recessive

Ass'd with:
Hepatic Cysts
Fibrosis
Dialysis Cysts of Kidneys
CORTICAL and MEDULLARY cysts due to dialysis
Simple Cysts of Kidneys
Benign finding. CORTEX only
Medullary Cystic Disease of Kidneys
Medullary Cysts.
Poor Prognosis.
US shows Small Kidney
Medullary Sponge Disease of Kidneys
Cysts of COLLECTING DUCT.
Good prognosis
Hyponatremia
v.
Hypernatremia
Hyponatremia:
Disorientation
Stupor
Coma

Hypernatremia:
Irritability, delirium, coma
Hypochloremia
v.
Hyperchloremia
Hypochloremia: 2° to metabolic acidosis, hypokalemia, hypovolemia, hyperaldosteronism

Hyperchloremia: 2° to anion-gap acidosis
Hypokalemia
v.
Hyperkalemia
Hypokalemia:
U-waves on EKG
Flattened T-waves on EKG
Arrhythmias
Paralysis

Hyperkalemia:
Tall-peaked T-waves (larger than QRS)
Wide QRS (longer QT)
Arrhythmias
Hypocalcemia
v.
Hypercalcemia
Hypocalcemia:
Tetany
Neuromuscular irritability

Hypercalcemia:
"Stones, Moans, Abdominal Groans"
Kidney stones
Delirium
Abdominal Pain
Hypomagnesemia
v.
Hypermagnesemia
Hypomagnesemia:
Neuromuscular irritability
Arrhythmias

Hypermagnesemia:
Delirium
↓ DTRs
Cardiopulmonary arrest
Hypophosphatemia
v.
Hyperphosphatemia
Hypophosphatemia:
Bone loss
Osteomalacia

Hyperphosphatemia:
Renal Stones
Metastatic Calcification
Congenital Adrenal Hyperplasia
Enzyme deficiency leads to cortisol deficiency which causes increased ACTH and bilateral hyperplasia of adrenal glands

Enzyme-Deficiencies:
17α-hydroxylase deficiency
21α-hydroxylase deficiency (most common)
18β-hydroxylase deficiency
17α-hydroxylase deficiency
17α-hydroxylase required to make cortisol and sex hormones
Symptoms:
Hypertension
Hypokalemia (aldo effect)
Male pseudohermaphroditism - external female genitalia (no DHT), testes, and no internal female genitalia (MIF by Sertoli cells)
Female Sexual Infantilism - failure to develop 2° sex characteristics. Default development is female, but estrogen required for breast development and ovulation
21α-hydroxylase deficiency
21α-hydroxylase required to make Cortisol and Aldosterone
Symptoms:
Hypotension
Hyperkalemia (No Aldo)
Salt-wasting
↑ sex hormones
Female Pseudohermaphroditism - ↑ androstenedione and testosterone
Masculinization - ↑ androstenedione and testosterone

Hypovolemic shock in newborn - salt-wasting leads to ↓ blood volume --> ↓ Renal perfusion --> ↑ Renin --> ↑ AT II --> Efferent arteriole constriction --> ↑ GFR which perpetuates salt-wasting and hypovolemia
11β-hydroxylase deficiency
11β-hydroxylase is step later in pathway than 21α-hydroxylase. Milder form of disease. Still unable to produce cortisol and aldosterone.
Symptoms:
Hypertension (deoxycorticosterone is mineralocorticoid and has similar effects as aldosterone)
Masculinization - ↑ sex hormones

Since this is less severe form of disease, female pseudohermaphroditism is less likely.
Cushing's Syndrome
↑ Cortisol
Causes:
1. Pituitary Adenoma --> ↑ ACTH --> ↑ Cortisol (Cushing's DISEASE)
2. Adrenal Hyperplasia --> ↑ Cortisol --> ↓ ACTH
3. Ectopic ACTH Prod. --> ↑ ACTH --> ↑ Cortisol (small-cell lung cancer, RCC, etc.)
4. Iatrogenic (chronic corticosteroid use) --> once steroids are stopped, body hasn't been producing ACTH or cortisol, so it takes a while to start back up. Reason for tapering steroids.

Sx: HTN, moon facies, truncal obesity, skin thinning & striae, buffalo hump, hyperglycemia (insulin resistance), osteoporosis, etc.

Dex test: Only useful with ↑ ACTH
1. ACTH tumor (pituitary): ↑ cortisol with low dose Dex; ↓ cortisol with high dose Dex
2. ACTH tumor (ectopic): ↑ cortisol with both high and low dose dex.
**Normal - ↓ cortisol after low dose
Cushing's Disease
ACTH producing tumor of pituitary leading to ↑ cortisol.

Cortisol will ↓ with high dose dexamethasone (Dex test).
Dexamethasone Test
Use for ↑ cortisol levels (primarily with ↑ ACTH also)
Key idea: If it is pituitary in nature, then the neg feedback is semi-normal. So, high dose dex will suppress and lead to decrease.

In ectopic or adrenal, feedback doesn't matter, so dex will not suppress production of cortisol.
1° Hyperaldosteronism (Conn's Syndrome)
Adrenal tumor producing Aldosterone
↓Renin, ↑ aldosterone
Symptoms:
Hypertension
Hypokalemia
Metabolic Alkalosis (Shift of K+ out of cells and H+ into cells)
2° Hyperaldosteronism
Condition that leads to normal mechanisms being overactive --> ↑ Aldosterone
↑ Renin, ↑ Aldosterone

Causes:
Renal Artery Stenosis
JG cell tumor producing Renin
CHF (↓ RBF due to ↓ CO)
Cirrhosis (↓ albumin --> ↓ oncotic pressure --> edema --> low intravascular volume)
Nephrotic syndrome (same as cirrhosis)
Addison's Disease
1° Adrenal Insufficiency caused by autoimmune destruction of adrenal cortex
All 3 layers of cortex affected
Symptoms:
Hypotension
Hyponatremic volume contraction
Hypokalemia (not 2°)
Skin Pigmentation (not 2°)
2° Adrenal Insufficiency
↓ ACTH --> ↓ Cortisol production
** Hyperpigmentation and Hypokalemia NOT found in 2° adrenal insufficiency because MSH is byproduct of ACTH production (from POMC) and ACTH is not main controller of aldosterone**
Waterhouse-Friedrichsen Syndrome
Adrenal insufficiency 2° to adrenal hemorrhage due to MENINGOCOCCEMIA
Sheehan's Syndrome
Postpartum hypopituitarism.

Normally following delivery with severe bleeding or maternal hypoperfusion (pituitary ↑ in size without ↑ blood supply leading to susceptibility to infarction (Hemorrhagic due to portal system)).

Symptoms:
Fatigue (Cortisol ↓)
Poor Lactation (Prolactin ↓)
Loss of Pubic/Axillary Hair (↓ Sex Hormones)
Pheochromocytoma
Catecholamine producing tumor of the adrenal medulla

Findings: ↑ Catecholamines in blood and ↑ VMA/Metanephrine (brkdwn products) in urine

Ass'd with NF & MEN 2 syndromes

KEY: EPISODIC

Sx: 5 Ps (Hypersympathetic)
P = Palpitations (↑ HR)
P = Pressure (↑ B/P)
P = Pain (Headache - ↑ B/P)
P = Pallor (vasoconstriction)
P = Perspiration

Tx: Phenoxybenzamine (irrev. α blocker)

Rule of 10s
10% malignant
10% bilateral
10% kids
10% familial
10% calcify
10% extra-adrenal
Neuroblastoma
Most common adrenal tumor in KIDS
N-myc oncogene ass'd
Not ass'd with EPISODIC HTN (Pheo)
MEN Type 1 (Wermer's Syndrome)
Familial endocrine cancer syndrome
3 Ps
P = Parathyroid Tumors
P = Pituitary Tumors (Prolactin>GH)
P= Pancreatic Endocrine Tumors
(Ex: Zollinger-Ellison, Insulinoma, VIPoma)

Common Presentation: Kidney Stones (↑ PTH) & Stomach Ulcers (↑ Gastrin (ZE))
MEN Type 2
Medullary Thyroid Carcinoma
Pheochromocytoma
Ass'd with Ret gene

Type A (Sipple's Syndrome) = Parathyroid also
Type B = Ganglioneuromas also
Hypothyroidism
Symptoms: Cold intolerance, ↓ Activity, ↓ Reflexes, Myxedema, Weight Gain, ↓ Metabolic Rate, ↓ appetite, Constipation, Cold Skin, Brittle Hair
Hyperthyroidism
Weight Loss, Heat Intolerance, Diarrhea, ↑ Reflexes, ↑BMR, Weight Loss, ↑ Appetite, Warm skin
Hashimoto's Thyroiditis
Autoimmune disorder resulting in hypothyroidism.
ANTIMICROSOMAL and ANTI-THYROID PEROXIDASE, and Antithryroglobulin Ab

Slow course, mild non-tender goiter

Findings: Lymphocytic infiltrate with germinal centers & Hurthle Cells
Subacute Thyroiditis (de Quervain's)
Hypothyroidism following flu-like illness. Self-limited (can by hyperthyroid --> hypothyroid)

Sx: VERY TENDER THYROID, Elevated ESR

Biopsy shows Granulomatous inflammation
Riedel's thyroiditis
Fibrous replacement of thyroid tissue.
Painless, fixed, hard goiter.
Most patients are EUTHYROID.
Graves' Disease
Type II Hypersensitivity - Autoimmune disorder resulting in HYPERTHYROIDISM.
Ass'd with TSI (thyroid stimulating Immunoglobulin)

Symptoms:
Graves Ophtalmopathy (proptosis & EOM swelling)
Goiter
Pretibial Myxedema

Sudden death can occur with significant stress. Catecholamine surge --> Arrhythmia --> Death
Toxic Multinodular Goiter
Multiple thyroid nodules that can lead to thyrotoxicosis.

Commonly ass'd with making iodine deficient patients iodine replete.
Papillary Thyroid Carcinoma
Most common type
Good Prognosis
Path: Orphan Annie Nuclei and Psammoma Bodies
Ass'd with childhood irradiation
Follicular Thyroid Carcinoma
UNIFORM Follicles
Good prognosis
Medullary Thyroid Carcinoma
Cancer of Parafollicular, Calcitonin-producing "C-cells"
THINK MEN 2!!
Anaplastic Thyroid Carcinoma
Older patients
VERY POOR PROGNOSIS
Thyroid Lymphoma
Ass'd with Hashimoto's Thyroiditis
Cretinism
Severe Fetal Iodine Deficiency
Common in places with Endemic Goiter (China)
Short, Pot-bellied, MR kids with protruding umbilicus and tongue

Can be due to developmental failure of thyroid or defect in T4 formation
Acromegaly
Excess GH in adults
Sx: Large Jaw, Hands, Feet; Deep-voice; & Insulin-resistance
Dx: high serum IGF-1
Treatment: Remove pituitary adenoma & then give Octreotide (Somatostatin)
Gigantism
Excess Growth Hormone in Children
1° Hyperparathyroidism
↑Ca, ↓ PO3 (diff. from 2°)
Parathyroid adenoma --> ↑ PTH
Actions:
1. ↑ PO3 excretion by blocking Na/PO3 cotxporter in proximal tubule
2. ↑ 1α-hydroxylase activity in proximal tubule (↑ conversion of 25 OH Vitamin D to 1, 25 (OH)2 Vit. D)
3. Increases Ca2+ reabsorption in DCT
4. Stimulates osteoblasts --> Stimulation of osteoclasts --> ↑ Bone resorption

**1, 25 OH Vit D also increases PO3 and Ca absorption from gut

Symptoms:
Hypercalcemia, Kidney Stones, ↑ PTH, ↑ ALP
Osteitis Fibrosis Cystica (Von Recklinghausen's Syndrome)
Cystic bone spaces filled with fibrous tissue leading to bone pain
Renal Osteodystrophy
Bone Lesions due to ↑ resorption of bone due to 2° hyperparathyroidism from renal disease
2° Hyperparathyroidism
↑PO3, ↓ Ca
Hyperparathyroidism often 2° to chronic renal disease (CKD)
Cause:
Chronic Kidney Disease --> ↓ PO3 excretion --> ↑ serum PO3 --> ↑ PTH --> Free calcium binds extra PO3 --> ↓ Ca --> ↑ PTH

If bone lesions present: called Renal Osteodystrophy
Hypoparathyroidism
Due to excision of parathyroids during thyroid surgery or Di George Syndrome (22.11q deletion)

Findings: ↓ Ca, Tetany

Chvostek's sign - tapping face causes facial spasms

Trosseau's Sign - BP cuff use --> contraction of carpal muscles
Pseudohypoparathyroidism
Autosomal Dominant
Kidney is unresponsive to PTH

Signs: hypocalcemia, shortened 4th/5th digits, short stature

Dx: Check PTH
Hypercalcemia Causes:
CHIMPANZEES
C = Calcium Ingestion
H = Hyperparathyriodism
I = Iatrogenic
M = Multiple Myeloma
P = Paget's Dz
A = Addison's dz
N = Neoplasms
Z = Zollinger-Ellison syndrome
E = Excess Vit. D
E = Excess Vit. A
S = Sarcoidosis
Diabetic Ketoacidosis
One of the most important complications of Diabetes
More common in Type I Diabetics due to lack of inhibitory control of glucagon by insulin.
High Glucagon necessary for DKA
↑ Glucagon --> ↑ Lipolysis --> ↑ CoA (brkdwn product) --> metabolized to ketones (β-hydroxybutyrate/acetoacetate)
Sx:
Kussmaul's respirations
Hyperglycemia
Anion Gap Metabolic Acidosis
Hyperkalemia (shift out of cells due to acidosis)
Fruity odor to breath
Altered Mental Status

Complications: Life-threatening Mucormycosis, Rhizopus infection, cerebral edema, arryhthmias

Tx: Fluids, Insulin, K+
Central Diabetes Insipidus
Due to ↓ ADH secretion
Causes:
Pituitary Tumor
Surgery/Trauma
Histiocytosis X
Sx: Intense Thirst and Polyuria. Serum Osmolality ↑

Patients will respond to desmopressin

Tx: Nasal Desmopressin
Nephrogenic Diabetes Insipidus
Lack of response by kidney to ADH.
Causes:
Genetic
2° to hypercalcemia or lithium
Demeclocyline (ADH Antagonist)

Findings: Serum Osmolality ↑

Patients will not respond to desmopressin stimulation test

Tx: Hydrochlorthiazide, Amiloride, or Indomethacin
SIADH
↑ ADH
Causes:
1. Ectopic ADH (Small cell lung cancer)
2. CNS disorder/trauma
3. Pulmonary Disease
4. Drugs (Cyclophosphamide)
Findings:
Excessive H2O Reabsorption
Hyponatremia (Dilution effect from H2O)
Urine Osmolality>Serum Osmolality
Tx: Demeclocycline or ↓ H20 Intake

**Correct sodium slowly. low sodium causes brain cells to swell, if you correct too quickly, they shrink and can cause seizures
Carcinoid Syndrome
Neuroendocrine, serotonin (5-HT) secreting tumor outside of GI Tract (within GI tract, serotonin is inactivated by liver. No Sx)
Most common appendiceal tumor
Sx:
Flushing (↑ 5-HT --> ↑ Bradykinin --> potent vasodilation)
Wheezing (↑ 5-HT ---> ↑ Bradykinin --> bronchoconstriction)
Right-sided valvular disease (fibrosis)
2° Pellagra - Tryptophan can be broken down in 2 ways: to serotonin (5-HT) or to Niacin (B3). In carcinoid syndrome, all the tryptophan is shunted toward serotonin production, and ↓↓ niacin. --> Pellagra (Dermatitis, Dementia, Diarrhea)

Findings: ↑ 5-HIAA in urine (brkdwn product of serotonin)

Tx: Octreotide (↓ Production of serotonin by tumors)
Zollinger-Ellison Syndrome
Gastrin-secreting tumor of duodenum or pancreas. Causes recurrent ulcers.
Ass'd with MEN-1 (3Ps)
Simple Partial Seizure
1 area of brain affected, no acute change in CONSCIOUSNESS
Most often from Mesial Temporal Lobe
Complex Partial Seizure
1 area of brain affected accompanied by CHANGE IN CONSCIOUSNESS
Most often from Mesial Temporal Lobe
Absence Seizure
Generalized seizure type
Blank stare
Tx: Ethosuximide (Valproate with concurrent Grand-Mal)
Myoclonic Seizure
Quick Repetitive Jerks
Tx: Valproate
Tonic-Clonic (Grand-Mal) Seizure
Alternating Stiffening and Movement
Generalized Seizure Type
Tonic Seizure
Stiffening Seizure
Generalized Seizure Type
Atonic Seizure
Drop Seizure - falls to floor
Migraine Headache
Unilateral pulsating pain with nausea, photophobia, or phonophobia. F>M. Due to irritation of CN V and release of vasoactive peptides and substance P. ± aura (sensory, visual, or speech disturbance).
Tension Headache
>30 min. of bilateral steady pain.
Cluster Headache
Repetitive, Brief, Unilateral periorbital pain with ipsilateral lacrimation, rhinorrhea, or Horner's syndrome. M>>F
Poliomyelitis
ss (+) RNA virus (picornavirus). Replicates in oropharynx & GI tract, Oral-Fecal Txmission. Spreads hematogenously to CNS

CNS symptoms: Degeneration of anterior horns (α motor neuron destruction). LMN findings only.

CSF Findings: Lymphocytic pleocytosis, normal glucose, normal to slightly ↑ protein

**West Nile presents similarly (mosquito bite)
Werdnig-Hoffman disease
Infantile spinal muscular atrophy.
Autosomal Recessive
Degeneration of anterior horns.
All LMN findings. Tongue fasciculations
Avg age of death <7mo.
Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) [ALS]
Destruction of Lateral Corticospinal Tract and Anterior Horns (α motor neurons) leading to UMN and LMN signs.

NO SENSORY DEFECTS! PURE MOTOR
**Kind of like MS of the spine**
Tabes Dorsalis (3° Syphilis)
3° syphilis causing destruction of the DORSAL ROOTS and Dorsal Columns. COMPLETE SENSORY LOSS (due to Dorsal root loss), Areflexia,
Occlusion of Anterior Spinal Artery
Spares only Dorsal Columns. All others lost.
Subacute Combined Degeneration
Loss of Dorsal Columns and Lateral Corticospinal Tract. Due to B12 deficiency.

Findings: ataxic gait, + Romberg, impaired position and vibration sense.
Syringomyelia
Central cord syndrome. Formation of cystic cavity in center of spinal cord.

Bilateral loss of pain and temperature at or 1-2 levels below the level of the lesion.
Friedrich's Ataxia
Autosomal Recessive
Trinucleotide (GAA) in frataxin gene
Presents in childhood with kyphoscoliosis
Impaired Mitochondrial functioning

Findings: Frequent falls, Hypertrophic Cardiomyopathy, staggering gait.
Brown-Sequard Syndrome
Hemisection of spinal cord

Findings:
1. Ipsilateral UMN findings below lesion
2. Ipsilateral vibration, proprioception (Dorsal Columns) below level of lesion
3. Contralateral Pain and Temp loss below lesion
4. Ipsilateral Loss of ALL sensation @ level of lesion
5. LMN signs @ level of lesion

**Above T1, Horner's syndrome.
Parinaud Syndrome
Paralysis of conjugate vertical gaze due to lesion of superior colliculi (normally pineal pathology)
Strabismus
Misalignment of eyes.
Amblyopia
Reduction of vision from disuse during critical development period. Brain senses that one eye is different (blurry) from the other, and blocks its input. If this is not corrected before age 8, patient will have, essentially, 2° blindness.
Conductive Hearing Loss
Due to blockage of ear canal.

Weber's test (tuning fork in midline) - louder in AFFECTED ear (no ambient noise, so it sounds louder)

Rinne's test - bone conduction will be better than air conduction. This is abnormal.
Sensorineural Hearing Loss
This is due to an organic problem either with the nerve or the sensory hair follicles of the cochlea.

Weber's test - will be louder in unaffected ear because patient can't hear as well with affected ear.

Rinne's test - air conduction will be louder than bone conduction (normal) because the hearing is normal, but it simply doesn't hear as well as the other.
Deficit due to CN III palsy
3 Possibilities:
1. Whole nerve affected - Horner's syndrome and eyeball "down and out"
2. Only parasympathetic (outer) nerve fibers affected - Horner's syndrome only with NORMAL ocular movement. Due to compression of the nerve.
3. Only Motor (central) nerve fibers affected - Down and out eyeball, but no Horner's syndrome. Due to vascular problems such as diabetes.
Deficit due to CN VI palsy
Ipsilateral eyeball pulled inward (esotropia) with horizontal nystagmus
Deficit due to CN IV palsy
May look normal, but patient will get diplopia with downward gaze. Test by looking down and in. (tests superior oblique muscle)
Open Angle Glaucoma
Increased intraocular pressure due to obstruction of outflow from anterior chamber (such as occlusion of Canal of Schlemm).

Risk factors: myopia, age, African-American

Sx: Silent or painless
Closed Angle Glaucoma
Increased Intraocular Pressure (posterior chamber behind iris) due to narrow angle or obstruction of flow between posterior and anterior chambers (between iris and lens).

Sx: PAINFUL, ↓ vision,

DON'T GIVE EPINEPHRINE
MLF Syndrome
Normally due to MS since MLF is a white matter tract

MLF normally is a connection between CN VI and the contralateral CN III. This coordinates abduction of the eye with adduction of the contralateral eye.

In MLF syndrome, the MLF is destroyed, so when the CN VI nucleus fires to abduct the eye, the signal is not transmitted to the contralateral CN III nucleus. The contralateral eye doesn't move, so the patient will have nystagmus beating back toward the midline.
Peripheral Vertigo
Due to inner ear pathology (CN VIII infection, Benign Paroxysmal Positional Vertigo (semicircular canal debris), or Meniere's disease).

Positional testing causes DELAYED HORIZONTAL nystagmus
Central Vertigo
Cerebellar or brain stem lesion (vestibular nuclei, tumor of posterior fossa)

Positional testing: IMMEDIATE nystagmus in ANY DIRECTION. can Δ directions
Glioblastoma Multiforme (Grade IV astrocytoma)
Most common 1° brain tumor of adults. Found in cerebral hemispheres (ST) and can cross corpus callosum (butterfly). GFAP will stain for tumor.

"Pseudopalisading" pleomorphic tumor cells. CENTRAL AREAS OF NECROSIS/HEMORRHAGE
Meningioma
2nd most common 1° brain tumor of adults.
Arises from Arachnoid Cells
VERY GOOD PROGNOSIS (resectable)

Spindle cells concentrically arranged in whorl pattern. PSAMMOMA BODIES (laminated calcifications)
Schwannoma
3rd most common 1° brain tumor of adults. Often CNVIII --> acoustic neuroma. RESECTABLE

**Bilateral = NF-2
Oligodendroglioma
Rare, slow growing, most often in frontal lobes.

Oligodendrocytes look like "fried eggs". Often calcified.
Pituitary Adenoma
Most often Prolactinoma.
Can cause Bitemporal hemianopsia due to pressure on optic chiasm.

Derived from RATHKE'S POUCH
Adult 1° Brain Tumors
Primarily SUPRATENTORIAL

Do not normally metastasize

HALF OF ADULT BRAIN TUMORS ARE METASTASES FROM SOMEWHERE ELSE!!!
Pediatric 1° Brain Tumors
Most commonly INFRATENTORIAL
Pilocytic Astrocytoma
Well-circumscribed, posterior fossa tumor.
GFAP Positive
VERY GOOD PROGNOSIS (Benign)

EOSINOPHILIC CORKSCREW FIBERS (Rosenthal fibers)
Medulloblastoma
HIGHLY MALIGNANT CEREBELLAR TUMOR

ROSETTES or pseudorosettes.

RADIOSENSITIVE
Ependymoma
Most commonly found in 4th ventricle
Can cause Obstructive Hydrocephalus
POOR PROGNOSIS

Characteristic Perivascular Pseudorosettes and Blepharoplasts near nucleus

Ass'd with 3° Syphilis
Hemangioblastoma
Often Cerebellar.

Ass'd with VON-HIPPEL LINDAU when found with renal angiomyolipomas

Can produce EPO and lead to 2° polycythemia

Foamy cells with high vascularity
Craniopharyngioma
Benign childhood tumor

Same location as pituitary

MOST COMMON SUPRATENTORIAL CHILDHOOD TUMOR.

Calcification common.
Uncal Herniation Syndrome
1. Ipsilateral dilated pupil/ptosis --> stretching of CN III
2. Contralateral homonymous hemianopsia --> Compression of ipsilateral PCA (no blood to visual cortex)
3. Ipsilateral Paresis --> compression of contralateral cerebral peduncle against falx tentorium
4. Duret Hemorrhages/Paramedian artery rupture --> Caudal displacement of brainstem.
Ataxia-Telangectasia
Defect in DNA repair mechanisms.
Autosomal Recessive.
Leads to cerebellar ataxia and blanching nests of distended capillaries.
Hypersensitive to X-RAY Radiation

Triad:
1. Ataxia (normally narrow-based)
2. Telangectasias (blanching)
3. Sinopulmonary infections (IgA deficiency)
Mitochondrial Myopathies
Ragged Red Fiber Diseases
Mitochondrially-inherited
1. Myoclonic Epilepsy with Ragged Red Fibers
2. Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-like episodes (MELAS)
Acute Intermittent Porphyria (AIP)
Triad:
1. Abdominal Pain
2. Neuropsychiatric Manifestations
3. Darkened Urine upon Standing

Enzyme deficiencies in early steps of porphyrin synthesis will have photosensitivity. Enzyme deficiencies later like AIP will not.

5Ps of Porphyria
Precipitated by Drugs (EtOH and Barbiturates)
Painful Abdomen
Pink Urine
Psychological Distubances
Polyneuropathy

Glucose will improve symptoms because it blocks ALA Synthase
Unhappy Triad
1. Medial Meniscus Damage
2. MCL Damage
3. ACL Damage

Sx:
MCL tear: Abnormal Passive Abduction
ACL tear: Positive Anterior Drawer Sign

Usually caused by lateral hit with knee extended (football)
Upper Brachial Plexus Injury
Erb-Duchenne's Palsy
"Waiter's Tip" - Injury to abductors, external rotatos, and biceps
Caused by stretching of neck and arm in opposite directions. Blow to shoulder (inferiorly) or birth complication
Lower Brachial Plexus Injury
Klumpke's Palsy
Increased Risk with Cervical Rib
Caused by Shoulder Dysotcia at birth (think grabbing a tree branch while falling)

Sx: Atrophy of Thenar and Hypothenar Eminences (Median and Ulnar nerves)
2. Interosseus Muscle atrophy (Ulnar)
3. Sensory deficits on medial side of forearm (C8-T2 distribution)
4. Disappearance of radial pulse upon moving head toward OPPOSITE side (compression of subclavian artery)
Distal Ulnar n. Lesion
Fracture of Hook of Hamate (Falling on outstretched hand)
Causes Pope's Blessing hand
Loss of lateral lumbricals leads to MCP flexion and PIP & DIP extension of 4th & 5th digits
Distal Median n. Lesion
Caused by Carpal Tunnel Syndrome or Dislocated Lunate
Causes Median Claw hand
Loss of medial lumbricals (2nd & 3rd digits) leads to MCP flexion and PIP & DIP extension
Proximal Median n. Lesion
Caused by Supracondylar humerus Fx
Causes "ape hand" - inability to oppose thumb
Total Claw Hand
Klumpke's Palsy - Clawing of all digits due to loss of all Lumbrical Function
Radial n. Palsy
Can be caused by crutches or by "saturday night palsy" (compression in spiral groove)
WRIST DROP
Motor deficit: Wrist extension, Finger extension at MCP, Supination, Thumb adduction and extension.
If Proximal/Posterior Cord: Triceps Weakness
If only motor deficit in forearm: caused by subluxation of radius. Deep radial n. only
Axillary n. Palsy
Caused by: fx of surgical neck of humerus, dislocation of humerus
Motor deficit: Shoulder Abduction
Sensory Deficit: over Deltoid m.
Musculocutaneous n. palsy
Motor deficit: Flexion of arm at elbow
Sensory deficit: Lateral forearm
Obturator n. Palsy
Cause: Anterior Hip Dislocation
Motor Deficit: Thigh Adduction
Sensory Deficit: Medial Thigh
Femoral n. Palsy
Cause: Pelvic Fracture
Motor Deficit: Thigh Flexion and Leg Extension
Sensory Deficit: Anterior Thigh and Medial Leg
Common Peroneal n. Palsy
Cause: Fibular neck Fx // Trauma to lateral aspect of leg
Motor deficit: Foot Eversion & Dorsiflexion // Toe Flexion
Sensory Deficit: Anterolateral leg // Dorsal aspect of foot

FOOT DROP (Foot is dropPED --> Peroneal Everts and Dorsiflexes)
Tibial n. Palsy
Cause: Knee trauma
Motor: Foot Inversion and Plantarflexion // toe flexion
Sensory: Sole of foot

Can't stand on TIPtoes --> Tibial Inverts and Plantarflexes
Superior Gluteal n. Palsy
Cause: Posterior hip dislocation
Motor: Thigh Abduction // Trendelenburg sign
Inferior Gluteal n. Palsy
Cause: Posterior hip dislocation
Motor: Can't jump, climb stairs, or rise from seated position
Achondroplasia
Autosomal Dominant // Ass'd with ↑ paternal age
Mech: Fibroblast growth factor receptor (FGFR3) defective causes ↓ cartilage --> ↓ long bone growth (head and flat bones are normal in size)

DWARFISM
Osteoporosis
Reduction of bone mass. Labs normal.
Type I - Postmenopausal.
↓ estrogen --> ↓ OPC --> ↑ RANK-RANKL interaction --> ↑ OsteoCLAST activation --> ↑ bone resorption

Type II - Senile osteoporosis
↓ Ca ingestion --> ↑ bone resorption

Findings: Vertebral Crush Fx; Distal Radius (Colles' Fx)

Prophylaxis: Ca ingestion BEFORE 30!

Tx:
Bisphosphonates
Pulsatile PTH
Estrogen (Type I Osteoporosis)
Osteopetrosis
THICK, DENSE bones due to ↓ bone resorption.
Pathogenesis: Defect in Carbonic Anhydrase II --> abnormal osteoclast function because osteoclast can't form acidic environment necessary to resorb bone!
Osteomalacia
Rickets
Osteomalacia = adults
Rickets = Children

Vitamin D deficiency. Reversible if replaced.

↓ Vitamin D --> ↓ Ca and PO3 absorption from the gut --> ↓ serum Ca --> ↑ PTH --> ↑ Ca, but also leads to ↓ PO3 (sum total = ↑ bone resorption with ↓ bone formation).

Abnormal mineralization comes from ↓ phosphate. Osteoid formation, but doesn't harden due to lack of phosphate.

Cause of 2° Hyperparathyroidism
Osteitis Fibrosa Cystica (Von Recklinghausen's disease)
Hyperparathyroidism --> formation of cystic spaces in bone filled with fibrous stroma and blood.

Characteristic "Brown Tumors"

Findings: ↑ Ca, ↓ PO3, ↑ ALP
Paget's Disease (osteitis deformans)
↑ osteoBLAST AND ↑ osteoCLAST activity --> abnormal bone architecture

Probably post-infectious (paramyxovirus)

Findings: normal labs except ↑ Alk Phos

Sx: ↑ hat size, hearing loss (auditory foramen narrowing); Chalk-stick fractures. High output CHF (↑ formation of AV shunts --> ↑ blood flow)

↑ risk for OSTEOGENIC SARCOMA
Polyostotic Fibrous Dysplasia
Bone replaced by fibroblasts & collagen.

McCune Albright is a good example
McCune-Albright syndrome
Sx: multiple lesions of bone being replaced by fibroblasts & collagen associated with precocious puberty and hyperpigmented skin lesions "coast of Maine" spots.

Type of Polyostotic fibrous dysplasia
Osteoarthritis
Mechanical mechanism of joint destruction. Wear and tear of joints leads to destruction of articular cartilage

Findings: subchondral cysts, osteophytes (bone spurs), joint space narrowing, Heberden's (DIP) & Bouchard's (PIP) nodes.

↑ risk with AGE and OBESITY
Rheumatoid Arthritis
Autoimmune Inflammatory disorder ass'd with Anti Ig-G antibodies (RF)
Ass'd with HLA-DR4
Females>Males

Symptoms: Pannus formation of MCP and PIP joints, rheumatoid nodules, subluxation, ulnar deviation

Symptoms IMPROVE with use, worse in the morning and at night

NO DIP INVOLVEMENT!!

**Synovial joints only. C1 & C2 joint is a synovial joint. In patients with RA, it is important to be careful when intubating; can cause subluxation with manipulation

Swan-Neck Deformity
MCP - flexed
PIP - extended
DIP - Flexed

Boutoineirre-Button hook
MCP - Extended
PIP - Flexed
DIP - Extended
Sjogren's syndrome
Autoimmune disorder associated with antibodies to ribonucleotide proteins (RNPs) SS-A (Ro) & SS-B (La). F>M

Triad: (CLASSIC)
1. Dry eyes
2. Dry mouth
3. Arthritis

Can be ass'd with SICCA syndrome (no arthritis) + vaginal and nasal dryness and chronic infections

Findings: ↑ size of parotid gland

↑ risk of B-CELL LYMPHOMAS
Sicca syndrome
Dry eyes, dry mouth, dry nose, dry vagina, chronic bronchitis.

Like Sjogren's, but no ARTHRITIS
Gout
Precipitation of MONOSODIUM URATE crystals due to HYPERURICEMIA. M>F

Crystals are needle-shaped and NEGATIVELY BIREFRINGENT. (yellow under parallel light)

Sx: Swollen, red, painful joint. Asymmetric. Most often the 1st MTP joint (podagra). Can be tophaceous (small calcifications)

Acute gout normally after LARGE MEAL or ALCOHOL intake (EtOH & uric acid use same pathway, competitive inhibition)

Treatment: Indomethacin (NSAID) (Acute Gout), colchicine, probenecid (chronic only), allopurinol (chronic only)

Causes: Lesch-Nyhan syndrome (MR & self-mutilation), PRPP excess, ↑ cell turnover (ACUTE LEUKEMIA), von Gierke's disease (Glycogen storage type I);
THIAZIDE DIURETICS

** Do NOT use SALICYLATES in patients with gout. They ↓ renal clearance of uric acid
Pseudogout
Due to calcium pyrophosphate crystals within joint space.

Rhomboid crystals, M=F >50 years old, primarily LARGE JOINTS (knee)
Ankylosing Spondylitis
Seronegative (RF neg) Spondyloarthropathy

Chronic inflammatory disease of spine

M>F

Primarily SACROILIAC (SI) Joints
Characteristic BAMBOO SPINE radiograph - know it.

Ass'd with uveitis and AORTIC REGURGITATION

Ass'd with HLA-B27 (PAIR)
Reiter's Syndrome
"Can't See, Can't Pee, Can't Climb a Tree"
Triad:
1. Conjunctivitis & Uveitis
2. Urethritis (non-gonococcal; ass'd with Chlamydia D-K)
3. Arthritis
Psoriatic Arthritis
Joint Pain/Stiffness with Psoriasis

Asymmetric and Patchy (like Psoriasis)

"PENCIL IN A CUP" deformity on radiograph

Less than 1/3 of psoriasis patients
Lupus Erythematosus
Autoimmune, diffuse disorder characterized primarily by multiple autoantibodies and a variety of symptoms.

90% Female (14-45); ↑ incidence and severity in African Americans

Autoantibodies:
Antinuclear Ab (ANA) - SENSITIVE // Not specific
ds-DNA Ab (anti-dsDNA) - very SPECIFIC // POOR Prognosis
Anti-Smith antibodies (Anti-Sm) - Very SPECIFIC, not prognostic
Antiphospholipid Ab
Antihistone Ab - drug-induced lupus

Symptoms: I'M DAMN SHARP
I = Immunoglobulins (anti-dsDNA, anti-Sm, anti-phospholipid)
M = Malar Rash
D = Discoid Rash (Discoid Lupus)
A = ANA
M = Mucositis (Oropharyngeal ulcers)
N = Neurologic deficits
S = Serositis (Pleuritis, Pericarditis)
H = Hematologic disorders
A = Arthritis
R = Renal Disorders
P = Photosensitivity
Other: Libman-Sacks (verrucuous) endocarditis, hilar adenopathy, fever, fatigue, weight loss Raynaud's.

Associated with Renal Pathology - Membranous Glomerulopathy (Nephrotic) and Diffuse Proliferative Glomerulonephritis (DPGN) (Nephritic)

Death from Renal Failure and Infection

False positives on VRDL/RPR Syphilis test - due to Anti-Phospholipid Antibodies
Sarcoidosis
Immune-mediated disease with NON-CASEATING GRANULOMA formation and ↑ serum ACE levels

Symptoms:
G = Gammaglobulinemia
R = Rheumatoid Arthritis
A = ACE ↑
I = Interstitial Fibrosis (Restrictive Lung Disease)
N = Noncaseating Granulomas

Ass'd with:
Restrictive Lung Disease
Bell's Palsy
Bilateral HILAR LYMPHADENOPATHY

**Often presents with HYPERCALCEMIA due to conversion to calcitriol in epithelioid macrophages

Treatment: STEROIDS!
Polymyalgia Rheumatica
Pain and Stiffness in shoulders and hips in patients with Giant Cell (Temporal) Arteritis

↑ESR

Tx: Prednisone
Polymyositis
Autoimmune, Progressive Symmetric PROXIMAL Muscle Weakness

Myofiber damage by CD8 T-cells

Muscle biopsy shows inflammation
Dermatomyositis
Autoimmune, Progressive Symmetric Proximal Muscle Weakness WITH DERMATOLOGICAL FINDINGS

Derm: Heliotropic Rash; malar rash; or face-shawl Rash

↑ Risk of Malignancy.

Findings: ↑ CK, ↑ aldolase, ANA and Anti-Jo 1 Ab (specific)

Treatment: Steroids
Myasthenia Gravis
Neuromuscular Junction Disorder characterized by autoantibodies against POSTsynaptic ACh receptors.

**Symptoms WORSEN with use**

Symptoms: Ptosis, Diplopia, General Weakness

Tx: AChE inhibitors
Lambert-Eaton Syndrome
Neuromuscular Junction Disease with autoantibodies to PREsynaptic Ca channels. Results in ↓ ACh release.

Symptoms: Proximal muscle weakness

PARANEOPLASTIC syndrome (Ass'd with Small Cell Lung Cancer)

**Symptoms IMPROVE with use**
Mixed Connective Tissue Disease
Disease with varying symptoms ass'd with autoantibodies to U1RNP

Symptoms:
Raynaud's
Arthralgias
Myalgias
Fatigue
ESOPHAGEAL HYPOMOTILITY

Tx: Steroids
Scleroderma (Progressive Systemic Sclerosis)
Autoimmune disorder resulting in excessive fibrosis and collagen deposition

Symptoms:
SCLEROSIS of SKIN
Sclerosis of RENAL, CARDIAC, PULMONARY, and GI symptoms

75% Female

Two Types:
Diffuse and CREST Syndrome
Diffuse Scleroderma
Progressive Sclerosis of skin, GI, Cardiac, Renal, and Pulmonary systems

Widespread skin involvement (above elbows and knees), RAPID progression, EARLY VISCERAL involvement

Ass'd with Anti-DNA Topoisomerase I antibody aka Anti-Scl-70
CREST Syndrome
More Limited form of Scleroderma

C = Calcinosis
R = Raynaud's
E = Esophageal dysmotility
S = Sclerodactyly
T = Telangectasia

Limited skin involvement (normally fingers and face)

Ass'd with Anti-centromere Ab (C for CREST)
Rhabdomyosarcoma
Most common soft tissue tumor in CHILDREN
Malignant skeletal muscle tumor
Commonly in HEAD/NECK
Lipoma
Benign, soft, well-encapsulated fat tumor.

Tx: Excision
Liposarcoma
Malignant fat tumor.
High possibility of RECURRENCE if not excised properly.
Osteoma
Benign bone tumor ass'd with Gardner's Syndrome

Gardner's = FAP colon cancer, retinal hyperplasia and osteomas
Osteoid Osteoma
<2cm woven bone tumor with interlacing trabeculae and SURROUNDED by OSTEOBLASTS

Most common in Men<25 years old

PROXIMAL TIBIA & FEMUR
Osteoblastoma
Bone tumor with woven bone and interlacing trabeculae SURROUNDED by OSTEOBLASTS

Larger form of Osteoid Osteoma (>2cm)

Most common in VERTEBRAL COLUMN
Giant Cell Tumor of Bone (Osteoclastoma)
Locally Aggressive Benign tumor of the EPIPHYSIS of Long Bones.

Characterized by Multinucleated GIANT cells and spindle-shaped cells

Characteristic "Soap Bubble" appearance on radiograph

Most common in Proximal Tibia/Distal Femur

Often RECUR
Osteochondroma (exostosis)
MOST COMMON BENIGN BONE TUMOR

Mature bone growing out of a long bone with CARTILAGENOUS cap.

Found in METAPHYSIS of long bones (distal femur and proximal tibia)

VERY RARELY converts to chondrosarcoma
Enchondroma
Benign CARTILAGENOUS bone neoplasm. Found IN THE MIDDLE (intramedullary) of the bone

Usually DISTAL extremities (Distal Tibia, Ulna, or Radius)
Osteosarcoma (Osteogenic Sarcoma)
2nd most common 1° malignant bone tumor.

Characteristic "Sunburst" pattern (Codman's triangle).

Young MEN (10-20 years old); POOR Prognosis

DISTAL FEMUR // PROXIMAL TIBIA

Predisposing factors: Paget's dz, familial Retinoblastoma, bone infarcts, RADIATION
Ewing's Sarcoma
Anaplastic small blue cell Malignant Tumor. Extremely Aggressive with Early Mets. Ass'd with t(11;22)

Responsive to Chemo

Most common in BOYS <15

Characteristic "onion-skin" appearance of bone

Commonly appears in Diaphysis of long bones, PELVIS, SCAPULA, and RIBS
Chondrosarcoma
Malignant INTRAMEDULLARY (inside bone) Cartilagenous Tumor. Characteristic "glistening mass"

Can be located in Pelvis, Spine, Humerus, Tibia, or Femur

Most common in MALES 30-60
Aspiration pneumonia
Normally of the Right lung because Right bronchus is more vertical and wider than left. Due to dependence, most commonly in Right Inferior Lobe.

Supine: SUPERIOR portion of RIGHT INFERIOR lobe

Upright: LOWER portion of RIGHT INFERIOR Lobe
Pulmonary Hypertension
Pulmonary HTN = pulm. art. pressure>= 25 mm Hg. Severe respiratory distress --> cyanosis and RVH --> death from cor pulmonale
1° - Inactivating mutation in BMPR2 gene --> ↑ smooth muscle proliferation --> pulm. HTN; poor prognosis

2° Pulmonary HTN
COPD - ↑ air trapping --> destruction of lung parenchyma
Mitral Stenosis - ↑ resistance --> ↑ pulmonary pressure
Recurrent VTE - Infarcts in lung --> ↓ cross-sectional area
Autoimmune dz - inflammation --> intimal fibrosis --> sm. muscle hypertrophy
Left to Right Shunt - ↑ shear stress --> endothelial injury
Sleep Apnea / Altitude - hypoxic vasoconstriction
Centriacinar Emphysema
SMOKING, SMOKING, SMOKING

Central Acinii closest to where smoke enters, so has most interaction with carcinogens
Panacinar Emphysema
α1-antitrypsin
Paraseptal Emphysema
Young healthy males. Normally causes bullae around the outside of the lung. Often causes spontaneous pneumothorax
Sleep Apnea
Person stops breathing for >10 sec.
Central Sleep Apnea
No Respiratory Effort
Obstructive Sleep Apnea
Respiratory Effort against Closed Glottis
Assd: with obesity, pulmonary HTN

**Daytime Sleepiness**

Tx: Weight Loss, CPAP, Surgery
Asbestosis
Diffuse pulmonary interstitial fibrosis after inhaling asbestos.

↑ risk of mesothelioma and bronchogenic carcinoma

Ferruginous bodies (Asbestos coated with hemosiderin).

Mainly lower lobes (all other pneumoconioses are upper lobe)

Shipbuilders, Roofers, Plumbers