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 |