06 April 2010

Biliary Tumors Cholangiocarcinoma and Cancer of the Gall Bladder



Biliary Tumors Cholangiocarcinoma and Cancer of the Gall Bladder
By: Larry Pennington, MD

Cholangiocarcinoma
Etiology
Ulcerative Colitis
Thorotrast Exposure
Sclerosing Cholangitis
Typhoid Carrier
Choledochal Cysts
Adult Polycystic Kidney Disease
Hepatolithiasis
Liver Flukes

Papillomatosis of Bile Ducts
Cholangiocarcinoma
Extra-hepatic: Distribution
Diagnosis and Initial Workup
Intra and Extra-hepatic Cholangiocarcinoma
Cholangiocarcinoma Intra-hepatic Disease
* Suspicious mass on CT. Quadruple phase CT with 0.5 cm cuts through the liver and portal hepatitis. Consider CTA reconstruction.
* Bx
* If adenoncarcinoma: look for primary with a chest CT and upper/lower endoscopy.
* Colon, pancreas, and stomach are common primary sites.

Cholangiocarcinoma Intra-hepatic Disease-Surgery/Ablation
* Extent of surgical therapy is determined by the location, hepatic function, and underlying cirrhosis.
* Anatomic resections have lowest recurrence rates. However nonanatomic resection increases potential surgical candidates and improves survival.
* Hepatic devascularization prior to resection is preferred
* Ablative therapy gives good local control.

Child’s Classification
Intra-hepatic Disease: Extent of Resection
Intra-hepatic Disease
Representative Case
MRCP of Extra-hepatic Cholangiocarcinoma at the Bifurcation
Klatskin tumor
Cholangiocarcinoma Extra-hepatic
Cholangiocarcinoma Pathology
Extra-hepatic Disease: Surgical Therapy
ERCP: Distal CBD Cancer
Ca of CBD Bifurcation
Node Dissection in Bile Duct Excision
Roux-en-Y Hepaticojejunostomy
Extra-hepatic Disease: Positive Margins or Unresectable
Extra-hepatic Disease: Unstentable
* Bypass if possible
* If not use proximal decompression and feeding jejunostomy
* Chemotherapy/Radiation Therapy/Brachy therapy as tolerated or clinical trial.
Cholangiocarcinoma Prognosis
* Best Result are with distal CBD tumors completely excised. Cure = 40%
* Incomplete resection plus radiation gives a median survival of 30 m.
* Stenting plus chemo/radiation gives a median survival of 17 to 27m
* Those stented alone live only a few months

Cancer of the Gall Bladder
Gall Bladder Cancer
Presentation (1)
Presentation 2
PET Scan and Cholangiocarcinoma
Sclerosing type of Cholangiocarcinoma
Cytological Brushing of Cholangiocarcinoma

Biliary Tumors Cholangiocarcinoma and Cancer of the Gall Bladder.ppt

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04 April 2010

OKAP Glaucoma Review



OKAP Glaucoma Review
By:Yara Catoira-Boyle MD

Introduction to Glaucoma

* What is the definition of glaucoma?
* Group of diseases that have in common a characteristic optic neuropathy associated with visual field loss for which elevated IOP is the primary risk factor
* What are the 3 factors that determine IOP?
* Rate of aqueous production by the CB, resistance to aqueous flow (Juxtacanalicular TM) and the level of EVP
* Of those factors, what is the most common cause of increased IOP?
* Increased resistance to outflow
* What is the prevalence of glaucoma in the general population?
* 1.5-2%
* What the percentage of 1st degree relatives of POAG patients will develop the disease?
* 10-15%
* How many people over 45 y/o are estimated to have glaucoma in the US? What % is bilaterally blind?
* 2.25 million / 4%
* 10 million /2%
* 1 million / 5%
* 5 million /4%
* What is the most common cause of nonreversible blindness in AA in the US?
* Glaucoma (prevalence 3-4X higher than whites)
* What are the leading causes of blindness worldwide?
* Trachoma Cataract glaucoma
* Which one is NOT a risk factor for glaucoma?
* Increased IOP and AA race
* Positive family history
* Advanced age
* Thin central cornea
* Male gender
* DM, myopia, HTN, ischemic vascular disease, arteriosclerosis are all inconclusive
* Which one is correct about the prevalence of Chronic angle closure glaucoma?
* Inuit from Arctic Asians Whites
* Men women
* Myopes hyperopes
* Younger older
* Which one is correct about heredity of glaucoma?
* Prevalence among siblings of pts is 20%
* Lifetime absolute risk at age 89 is 10 x higher for relatives of glaucoma patients
* A single gene is likely to be discovered as the culprit
* The gene responsible for mutations of the TIGR (myocilin) protein is located on chromos 5

A little genetics of glaucoma
* TIGR protein produced by TM cells was id’d in Juvenile glaucoma, and later found to affect up to 3% of OAG
* GLC1A, the gene responsible for TIGR mutations is on chromosome 1
* It seems to be an autossomal dominant inheritance of the polygenic type with late or variable age of onset, incomplete penetrance and substantial environmental influence

IOP and Aqueous Dynamics
* What is correct about the aqueous composition?
* Has less hydrogen and chloride than plasma
* Has deficit of ascorbate
* Has excess bicarbonate
* Contains lysozyme, cAMP, steroid hormones and hyaluronic acid
* Which one is true of rate of aqueous production?
* It has a turnover of 1% per minute
* It increases during sleep
* It increases with age
* It increases during ocular inflammation
* What are the 2 major outflow pathways?
* Pressure-dependent/ TM/ conventional
* Pressure-indepdt/ Uveoscleral/ nonconventio
* What is true about facility of outflow?
* Increases with age
* Not affected by surgery, trauma, medications
* Varies widely in normal eyes, mean 0.22 to 0.28 microL/min/mmHg
* What are the 3 parts of the TM?
* Uveal, corneoscleral and juxtacanalicular
* What is false of the TM anatomy?
* TM is composed of many layers of colagenous tissue covered with endothelium
* TM has pressure-dependent flow
* TM functions as a one way valve
* The aqueous leaves the eye by bulk flow
* Laser trabeculoplasty causes apoptosis of Trabecular cells
* What is false about Schlemm’s canal?
* It is lined by endothelium and transversed by tubules
* It is a multiple channel
* Its average diameter is 370 microns
* The inner wall contains giant vacuoles that have direct communication with the intertrabecular spaces
* When IOP is low, blood may reflux into the canal
* The venous path of aqueous includes episcleral veins, anterior cyliary and superior ophthalmic veins and the cavernous sinus
* What is false about the uveoscleral outflow?
* It refers to any nontrabecular outflow
* Aqueous passes from the AC into the CBM and then into the supraciliary and suprachoroidal spaces
* It is decreased by cycloplegia, adrenergic agents, PG analogs and cyclodyalisis surgery
* It is also decreased by miotics
* It accounts for at least 5-15% of outflow

IOP and Aqueous
* What effect the following medications have on uveoscleral outflow?
* Cycloplegics
* Miotics
* Epinephrine
* Xalatan
* Brimonidine
* Timoptic ....

Clinical Evaluation
* Associate the external adnexae finding with a diagnosis associated with glaucoma
* “ash-leaf” sign
* Plexiform neuroma of upper lid
* Nevus of Ota
* Microdontia or hypodontia
* Port-wine stain (facial cutaneous angioma)
* Yellow or orange papules of skin of head/neck
* EOM restriction, proptosis, pulsating exophthalmos
* What is the type of glaucoma associated with the following corneal signs?
* Krukenberg spindle
* Exfoliating material on anterior chamber
* Keratic precipitates
* “stelate” KP’s
* “beaten bronze” appearance or corneal edema with iris changes
* What is not a possible cause of blood in Schlemm’s canal on gonioscopy
* Compression of episcleral veins with lip of goniolens
* Hypotony
* CC fistula
* Sturge-Weber syndrome
* Dilation of the pupil
* What is false about gonioscopy:
* Normal angle vessels are usually radial along the iris or circumferential on the CB
* Abnormal vessels cross the SS to reach the TM
* The nonpigmented TM is posterior to the pigmented TM
* A C angle indicates that SS is visible
* List potential causes for increased TM pigmentation:
* PDS
* PXF
* Malignant melanoma
* Trauma
* Surgery
* Hyphema

Gonioscopy
* It is false about gonioscopy
* It is necessary to see the angle due to total internal reflection at the tear-air interface (critical angle approximat 46’)
* Koeppe and Barkan lenses are examples of direct gonioscopy, most used in the OR
* Goldmann, Zeiss and Sussman lenses are used for indirect gonioscopy in the office
* Dynamic gonioscopy is done by asking the patient to move his eye
* It is false about gonioscopy
* The order of structures from anterior to posterior is: Schwalbe’s line-nonpigm TM-pigment TM-scleral spur-ciliary body-iris root
* Multiple methods of classification exist
* A Shaffer grade 4 angle is the narrowest
* Spaeth’s classification C40R indicates a normal and open angle

Clinical Evaluation
* What is angle recession and how is it different from cyclodyalisis on gonio:
* Angle recession is a tear between the longitudinal and circular muscles of CB= widened CB band
* Cyclodyalisis is a separation of the CB from the SS= gap between CB and sclera
* What is false of the Optic Nerve:
* Consists of about 1.2-1.5 million RGS axons
* The RGC cell body is in the ganglion cell layer of the retina
* The diameter of the intraocular ONH is about 2.5 mm
* There are 2 types of RGC’s: Magnocellular and Parvocellular...
OAG
* What percentage of patients with OAG have a screening IOP below 22?
* 30-50%
* What is the average corneal thickness by optical and ultrasound measurements?
* 534 optical and 544 ultrasound
* In the AGIS, patients had significantly better outcomes if their IOP was:
* Below 18 at all visits
* Below 18 50% of visits
* Below 14 at all visits
* Below 14 50% of visits....
Secondary OAG
* What is false about PXF?
* The odds of exfoliation glaucoma are 40% in 10 years
* The angle is often narrow with a +4 pigmented TM and a Sampaolesi line inferior
* The hyaline material is found on the lens, pupil margin, CB epithelium, iris PE, iris stroma, iris blood vessels and subconjunctiva
* SLE features include iris TI at pupil margin and iridodonesis or phacodonesis....
Traumatic OAG
* About hyphemas, is false:
* Sicke cell patients may have severe glaucoma from small hyphemas
* Acute IOP elevations may lead to AION or CRAO in patients with sickle cell
* Treatment consists of corticosteroids, cycloplegics, eye shield, limited activity, head elevation
* Aminocaproic acid is advocated since it has very few side effects and decreases rebleed
* Which drug is the best choice to control IOP in a hyphema patient with sickle cell diz?
* IV Diamox
* Iopidine
* Manitol
* Pilocarpine
* Timolol
OAG
* Regarding secondary glaucomas, which one is false?
* UGH syndrome can happen secondary to erosion of IOL haptic into the iris or CB
* UGH presents with chronic inflammation, iris NV, recurrent hyphemas
* Schwartz-Matsuo syndrome refers to elevated IOP after RD due to photoreceptor outer segmemts blocking of TM
* PKP related glaucomas can be of open or closed angle mechanism
* About 50% of people will have some elevation of IOP with the use of topical steroids
ACG
* About ACG epidemiology, what is incorrect?
* 10% of the 67 million cases of glaucoma worldwide are of ACG
* Predominant form of glaucoma in East Asia
* PACG is responsible for 91% of the bilateral blindness in China
* About the mechanisms of ACG, the incorrect is:
* Pupillary block is the most common cause of angle closure, including primary AC
* NVG is a type of ACG without pupillary block
* Marfan’s synd and Homocystinuria can lead to pupillary block by lens dislocation
* In Aniridia, Glaucoma is of open type since pupillary block can’t happen due to lack of iris
Primary ACG
* About the risk factors for ACG, the wrong one is:
* The prevalence varies with race: AA whites Japanese East asians Inuits
* White pts tend to have acute AC, while AA and asians have chronic asymptomatic diz
* Shallow AC, thick lens, short AL, small corneal diameter and radius increase risk
* Ages 30-50 are the highest risk
* More common in women and hyperopes

Acute PACG
* What is false about Acute PACG?
* Mild attacks can be broken with Pilocarpine 1-2%, but should avoid stronger miotics
* At IOP 40-50 the pupillary sphincter is too ischemic to respond to miotics and should use aqueous supressants, diamox or manitol
* The chance of acute attack in the fellow eye is 40-80% in 1 year
* High IOP during an acute attack may lead to ischemic nerve damage or retinal vascular occlusion

ACG
* What is false of iris and ACG?
* Both mydriasis and miosis can cause acute angle closure in a predisposed eye with shallow AC
* Miotics relax the lens zonules allowing it to sit forward in the AC, decreasing irido-lenticular touch
* Systemic medications including allergy, cold medicines, antidepressants, anticholinergics and topamax carry warning against glaucoma
* Peripheral iridectomy is indicated in patients with critically narrow angles, PAS, h/o previous attack, AC depth 2.0mm, +family history, +provocative test...
Childhood Glaucoma
* Mark the false :
* Primary congenital is the same as infantile glaucoma
* Glaucoma recognized after age 3 is termed Juvenile Glaucoma
* Glaucoma may present with buphthalmos if the IOP elevation starts after age 3
* Developmental glaucoma refers also to secondary glaucomas associated with inflammatory, neoplastic, hamartomatous, metabolic or congenital ocular or systemic anomalies
* Mark the false
* Congenital glaucoma is usually primary(50-70%)
* It is a rare disease (1:10,000 births)
* 60% are diagnosed in the first 6 months, 80% by one year
* 65% are female and bilateral in 70%
* Axenfeld-Rieger syndrome and aniridia are conditions associated with infantile glaucoma...
Medical therapy of glaucoma
* About beta blockers, which one is false:
* Betaxolol is selective for Beta 1 receptors
* They reduce aqueous production by 20-50%
* Are very additive to epinephrine
* Metipranolol was reported to cause iritis
* Effect starts within one hour and may last 4 weeks
* Tachyphylaxis and short term scape are seen
* Are contra-indicated in myasthenia gravis
Surgical Therapy
* What is false about surgical therapy of OAG?
* Usually undertaken when medical therapy fails
* LTP may be considered as a initial step in therapy since it decreases IOP by 20-25%
* The GLT showed that LTP patients did better than medication patients
* LTP is particularly effective in XFG and PG....

OKAP Glaucoma Review.ppt

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03 April 2010

Fetal Tissue Transplants



Fetal Tissue Transplants
By:Michelle Gomez

Words You May Need To Know
* Definitions-
+ Fetal tissue transplants
+ Parkinson’s Disease (PD)
+ Huntington’s Disease (HD)
+ Dopamine (DA)

Development of the Fetus
First Month (conception to 6 weeks)
Second Month (7-10 weeks)
Third Month (11-14 weeks)
Fourth Month (beginning of second trimester)
Fifth month (19- 22 weeks)
Seventh Month (beginning of third trimester 27-30 weeks)
Sixth Months (23-26 weeks)
Eighth month (31-34 weeks)
Ninth month (35 weeks to delivery)

History
Fun Facts
* The History of Fetal Tissue Transplants

Parkinson’s Disease
* Parkinson’s Disease
Huntington’s Disease
What does it treat?
* Parkinson’s Disease
* Huntington’s Disease
* Retina Repair
* Future:
o Epilepsy
Biological Concepts

The Process: Cell Therapy Development in PD
* Parkinson’s Disease-
o Graft Efficiency has to be increased and variability reduced
+ Patient selection
+ Graft Placement
+ Composition and Preparation of the Graft Tissue
+ Developing immunological mechanisms

2 Years After Transplantation
The Process: Cell Therapy Development in PD
Evidence and Experiments
Society for Neuroscience-
* October 24, 1999- a study was presented at the Society for Neuroscience’s annual meeting, showing that the fetal cells can produce a critical neurotransmitter, reducing patient's tremors and paralysis. (Helmuth, 886)
Retina Repair-
o The procedure that Elisabeth Bryant, from the previous slide, underwent was retinal repair. Robert Aramant at the University of Louisville in Kentucky developed the transplant technique with Magdalene Seiler. (image on next slide)

Fetal tissue inserted here
Frontier Issues
Cost and Finances
* Surgery Alone- $43,500
* Pre-Operative Costs- $4,000
* Total- Approx.- $50,000
Controversy
Pro-life vs. Fetal Tissue Transplant Supporters
Ethical Issues
* It takes six fetuses to provide enough material to treat one Parkinson’s patient.
* Who is donating?
* Cell supply is limited.
* Better areas for transplantation?
Politics
Bibliography/ References
* Barinaga, M. “Fetal neuron grafts pave the was for stem cell therapies.” Science. 5 Feb 2000 287:5457 p.1421-2. General Science Index. COSLibrary, Visalia, CA. 19 Sept 2005
* Björklund, Anders and Olle Lindvall. “Cell Therapy in Parkinson’s Disease.” NeuroRx. Oct 2004. the American Society for Experimental Therapeutics, Inc. Lund, Sweden. 13 Oct 2005. http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=534947
* Cimons, Marlene. “Bush is a threat to US stem-cell research”. Nature Medicine. 2001. COS Library, Visalia, CA. 24 Oct. 2005. http://www.nature.com/nm/journal/v7/n3/full/nm0301_263a.html
* “Dopamine.” Columbia Encyclopedia, 6th Edition. 2005. 19 Sept 2005 http://www.encyclopedia.com
* Dunnett, Stephen B. and Anne E Rosser. “Cell Therapy in HD.” NeuroRx. Oct 2004. American Society for Experimental NeuroTherapeutics, Inc. Wales, UK. 13 Oct 2005. http://www.pubmedcentral.gov/articlerender.fcgi?tool=pmcentrez&artid=534947

Bibliography/ References
* Fackelmann, K.A. “Study sizes up fetal cells for transplant.” Science News. 7 Jan 1995. 149:1 p.6. Academic Abstracts. COS Library, Visalia, CA. 15 Sept 2005
* "Fetal Tissue Implant." Columbia Encyclopedia, 6th Edition. 2005. 19 Sept. 2005 http://www.encyclopedia.com
* Hawaleshka, Dan. “The debate over fetal tissue. (cover-story).” Maclean’s. 1996 109:4 p.48. Academic Abstracts. COS Library, Visalia, CA. 15 Sept 2005
* Helmuth, L. “Fetal cells Help Parkinson’s patients.” Science. 29 Oct 1999. 286:5441 p.886-7. General Science Index. COS Library, Visalia, CA. 19 Sept 2005
* Hopkins, John. About Huntington's Disease and Related Disorders. 2002. John Hopkins Medicine. 24 Oct 2005. http://www.hopkinsmedicine.org/bhdc/about/
* “Huntington’s Disease.” Columbia Encyclopedia, 6th Edition. 2005. 19 Sept. 2005 http://www.encyclopedia.com
* “Introduction.” Parkinson’s Disease: Hope Through Research. Sept 2003. National Institute of Neurological Disorders and Stroke. 22 Sept 2005. http://www.ninds.nih.gov/disorders/parkinsons_disease/detail_parkinsons_disease.htm
* Rae, Scott B. “The Ethics of Fetal Tissue Transplantation.” CHRISTIAN RESEARCH INSTITUTE. 2005. Talbot School of Theology. 24 Oct 2005. http://www.equip.org/free/DE192.htm

Images-
* “Development of the Fetus.” Illustration. 1998. Development of the Baby. Parents Magazine. 24 Oct 2005 http://www.csulb.edu/divisions/students2/departments/Health_Resource_Center/pregnancy.htm
* Levivier, Marc. “PET scan in patient with PD after transplantation of human fetal neurons (before and after)”. Illustration. 7 July 2003. Neural Transplants in Parkinson’s Disease: do they work?. Lancet Neurology. 6 Oct 2005 http://www.thelancet.com/journals/laneur/article/PIIS1474442203004423/fulltext.
* Rowe, Duncan. “Retina Repair.” Illustration. 31 Jan 2003. Fetal Tissue Transplants Improve Adult Sight. New Scientist Magazine. 6 Oct 2005. http://www.newscientist.com/article.ns?id=dn3319

Fetal Tissue Transplants.ppt

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