Systemic Disease Flashcards
Inflammation
Defense response that elimates the products of cellular injuries. Inflammation plays a role in the healing of injured cells bi diluting or destroying the agent responsible for injury.
Acute inflammation
Occurs in 1-2 minutes post injury. Leukocytes help clear up the injury site by invading bacteria and degrade necrotic byproducts of the damage. Acute inflammation is deterministic-it happens the same way every tine
What are the three major types of acute inflammation
- Vascular size changes (dilation) to facilitate increased blood flow
- Structural changes in the microvasculature (increased permeability) facilitate the arrival of plasma proteins and leukocytes from the circulation
- Immigration of neutrophils (PMNs) from circulation to the site of injury
What does acute inflammation generate
RUBOR (redness)
CALOR (heat)
DOLOR (pain)
TUMOR (swelling)
Also the presence of WBCs
Possible outcomes of acute inflammation
Complete resolution
Scarring of fibrosis
Abscess formation
Progression to chronic inflammation
Chronic inflammation
Last weeks to years. Active inflammation, tissue injury, and healing progress at the same rate.
Chronic inflammation is characterized by
- Infiltration with mononuclear cells (macrophages, lymphocytes, and plasma cells)
- Tissue destruction
- Repair involving new vessel proliferation (neovascularization) and fibrosis
Chronic inflammation will arise from
- Persistent infections (H. Pylori)
- Prolonged exposure to potential toxic agents (asbestos)
- AI disease (RA)
Presentation of chronic inflammation
Not all chronic inflammations are identical. In fact the body adjusts the response according to the type of injury. One type of chronic inflammation is granulomatous inflammation
Granulomatous inflammation
Is marked by collections of large, activated macrophages with a squamous cell-like appearance
Examples of granulomatous inflammation
Bacterial: TB, leprosy, syphilis
Fungal: histoplasmosis, blastomycosis
FB: suture, vascular graft
Unknown: sarcoidosis
Local factors that affect wound healing
Prolonged healing: local infection, decreased blood supply, and the inabilialty to form clots
Systemic factors that can prolong healing
Diabetes, immunocompromised states, decreased peripheral blood flow, systemic infection, malnutrition, and increases glucocorticoid production (stress)
Reversible cellular injury
Process is marked by decrease in blood supply to a cell and a corresponding decrease in oxygen supply (hypoxia)
Lack of oxygen to a cell will cause
- increase in glycolysis and anaerobic respiration-the lack of oxygen leads to increases lactic acid concentration and a decrease in tissue pH
- a decrease ATP production, which disrupts the Na and K gradient across the cell; this causes accumulation of intracellular Na and subsequent cellular edema
If oxygen is restored to a cell during injury
ATP increases, the Na-K pump is restored, and anaerobic respiration ceases. Persistent ischemia will lead to irreversible cell injury
Irreversible cell injury
- Insuffient ATP resutls in Na accumulation and the cell, in turn, becomes edematous, which disrupts the cell membrane. This causes crucial cellular components needed for the reconstruction of ATP to leak out, and thereby further facilitates the depletion of high energy phosphates
- lack of oxygen will ultimately increase ischemia, causing irreversible tissue necrosis.
The events leading to irreversible tissue necrosis from cell ischemia
- progressive loss of membrane phospholipids
- cytoskeleton abnormalities
- toxic oxygen radicals, which damage the cell membrane and other cell components
- lipid breakdown products, which accumulate in ischemic cells and result in phospholipid degradation
Necrosis is a result of
Irreversible cell death
The death of one or more cells as a result of irreversible damage
Necrosis
What are the two processes that occur with necrosis
- Enzymatic digestion of the cell
2. Desaturation of the proteins
What are the 4 types of necrosis
- Cogaulative necrosis
- Liquefactive necrosis
- Caseous necrosis
- Fat necrosis
Coagulative necrosis
The structural boundary of the coagulated cell, tissue, or vessel is maintained, but integral structural proteins are denatured. This type of necrosis often occurs following MIs
Liquefactive necrosis
Cell with a well-defined boundary remains, but is consists of dull, gray-white remains. This is seen with fungal infections and often occurs in the lungs
Fungal, lungs
Caseous necrosis
Most often seen in TB infections. The term casesou comes from “cheesy” because the central necrotic tissue appears white and cheesy
Fat necrosis
Death to adipose tissue. Small white lesions are formed
Apoptosis
Well organized self destruction of cells; commonly referred to as programmed cell death. It occurs during embryogenesis, endometrium shedding during the menstrual cycle, or cell depletion in tumors. Apoptosis is critical in fine-tuning the developing retina. Over 70% of ganglion cells die via this process during development
Apoptosis and the retina
Critical in fine-tuning the developing retina. Over 70% of ganglion cells die this way during development
What are the hypersensitivity reactions
Anaphylactic
cytotoxic
Immune complex
Delayed
Anaphylactic HS components
Type 1
IgE
Histamine
Anaphylactic HS
An allergen activates a B lymphocytes and IgE Abs are produced and bind to the surface of mast cells and basophils. A second exposure to the allergen causes cross linking of IgE, allowing calcium to enter and resulting in degranulation of the cell
Common causes of a type I HS
Peanuts, shellfish, drugs (PCNs), snake venom, and winged insects
Initial response of a type I hS
5-30m and resolves in 30-60m. Late phase response (4-6 hours later) can occur, resulting in tissue damage
Histamine and type I HS
Primary mediator released from mast cells and basophils during a type I allergic reaction
Symptoms of histamine during type I HS
Itching Redness Rhinitis Wheezing Hypotension (vasodilation) Tachycardia Nausea/vomiting HA, syncope, seizures Life threatening anaphylactic shock
Which HS is fast and first
Type I anaphylactic
Requires EpiPen
What are the players of a type 2 HS
IgG and IgM
Ex. Rheumatic fever
Destroy part of the body in attempt to destroy the toxic cells
What is type II HS facilitates by
Abs against antigens absorbed on various tissue components, such as cell surfaces. IgM and IgG Ab bind to antigen or enemy cell, which leads to its destruction
- transfusion reaction with the prime example being Rh disease. Maternal IgG ab capable of crossing the placenta attack fetal erythrocyte antigens.
- rheumatic fever is another example
Which HS reaction has no Ag/Ab
Delayed
Which HS reaction has two players
Cytotoxic
Which HS involves Ca2+ reentering the cell
Anaphylactic
Comments of type III HS
Ag/Ab complexes
RA/SLE
Immune complex mediated HS
Type III
- mediated by Ag/Ab complexes either in the systemic circulation of those formed at the location of antigen deposition
- these Ag/Ab complexes activate the complement response. This triggers the attack on neutrophils, which then release lysosomal enzymes
- SLE
- serum sickness is also an immune complex disorder in which Ab are formed due to the intake of large amounts of foreign proteins
Delayed HS Components
T cells (memory)
2-3 days
TB skin test example
Delayed or cell-mediated HS
Type IV
Sensitized T lymphocytes encounter an antigen and release leukokinin, leading to macrophage activation
-examples: TB skin test, contact dermatitis, and cornal transplant rejection, phlyctenules is
2-3 days
SLE
AI disease affects multiple systems including the skin, kidneys, joints, and heart. Female to male is 10:1 and often arises in the 2-3rd decades of life. Symptoms include a butterfly (malar rash), discoid lupus, photosensitivity, arthritis, renal disorders, neuro disorders, immunological disorders, and hemolytic anemia. SLE patients will produce ANA and 90% will have joint pain.
Ocular findings of SLE
Dry eye, recurrent episcleritis, peripheral keratitis, and photophobia
Neuroophthalmic implications of SLE
Disc edema and papilledema
RA
The most common systemic inflammatory disease classically causes systemic arthritis in multiple locations that leads to destruction of articular cartilage. Symptoms are often worse in the AM and can include pain in the hands, wrist, feet, and small joints. Women are affected mroe commonly than men, age 40-50. These patients will have a positive RF test. Appx 25% of patients with RA will have ocular manifestations
Ocular manifestations of RA
Keratoconjunctivitis sicca
Scleromalacia perforans
Choroiditis, retinal vasculitis
Papilledema is less common but possible
Scleritis in RA patients
Necrotizing without inflammation=scleromalacia perforans. No pain
JIA
Formerly known as JRA. Predilection for young females and can affect multiple joints, these patients will have a negative RF, but can have a positive ANA. Patients under the age of 6 with recent onset of the disease and positive ANA are at a higher risk of ocular manifestation
They usually just have one joint affected
What is the most common cause of uveitis in children
JIA q
Classic clinical picture of JIA
Female with asymptomatic, chronic, bilateral, non-granulomatous, anterior uveitis
Often present with a low grade fever
Sjögren’s syndrome
Affects females between 40-60. Classified as primary and secondary
Primary Sjogrens
Aqueous deficient dry eye
Dry mouth
Evidence of reduced salivary secretion, positive focus score on a minor salivary gland biopsy, and the presence of autoAb
Secondary Sjogrens
Primary Sjogrens AND an autoimmune CT disease (RA)
Dry eye, dry mouth, arthritis
Classic triad of secondary Sjogrens
Dry eyes
Dry mouth
RA
What are Sjogrens patietns at a risk of
Appx 5% of Sjogrens patients develop a malignant B cell lymphoma
- primary Sjogrens more likely
- mean development of it is 7 years
When should you evaluate someone for Sjogrens
Refractory dry eye disease
Patients with oncurrent symptoms of dry eye, dry mouth, and/or arthritis
Sarcoidosis
Idiopathic condition that classically affects middle aged AA females. Characterized by non-caseating granulomas and increased levels of serum angiotensin converting enzyme (ACE). Up to 40% of active cases of sarcoidosis can have normal ACE resutls. 90% of patients have lung invovlemt-a chest X ray is indicated if the disease is suspected. Patients are often asymptomatic, but can have complaints of breathing difficulties, dry cough, and unusual rashes. 25% of patients will have ocular manifestation
Ocular manifestations of Sarcodosis
- chonric dacryadenitits
- dry eye disease
- 19% of patients will have chronic, bilateral, anterior granulomatous uveitis
- CN VII palsy
- vasculitis, vitritis
- possible ON disease (unilateral disc edema, papilledema)
Ankylosing spondylitis
Chronic inflammatory condition of the spine with large joints that usually affects young males 10-30 years old. 90% of patients with be HLA-B27 positive
Manifestations of ankylosing spondylitis
- bamboo spine
- sacroiliitis causing lower back pain that IMPROVES with exercise and responds well to NSAIDs
- Acute anterior, unilateral (or alternating) non-granulomatous uveitis
- aortic regurgitation
Order a sacroiliac X ray
Most common cause of acute unilateral (or alternating) anterior non-granulomatous uveitis
Ankylosing spondylitis
Reactive arthritis
AKA reiters syndrome
- classic triad of urethritis, conjunctivitis, and/or anterior uveitis, and arthritis
- urinary symptoms will usually appear first. Low grade fevers, conjunctivis, and arthritis will then develop over the next several weeks
- affects young males more than females and is typically HLA-B27 positive
Can’t see, cant pee, cant climb a tree
Things that can cause episcleritis
RA
Lupus
UCRAP
Things that can cause anteiror nongranulomatous uveitis
UCRAP
JIA
Syphilis
Things that can cause anteiror granulomatous uveitis
TB
Sarcoidosis
Syphilis
AI things that can cause ONH disease
Sarcoidosis
Lupus
Psoriatic arthritis
Symmetrical, peripheral, small joint pain with accompanying psoriatic lesions found on the knees, elbows, and scalp. 7% of patients with psoriatic arthritis may develop anterior uveitis. These patients will have a positive HLA-B27 test. Treatment includes UV-B light exposure and methotrexate
What are the HLA-B27 conditions
UCRAP
- ulcerative colitis
- Crohns disease
- reactive arthritis
- ankylosing spondylitis
- psoriatic arthritis
Temporal arteritis (GCA)
Systemic vasculitis that affects the medium to large vessels including temporal artery. These patients are typically older than 55 years old and present with complaints of jaw claudication, jabbing neck pain, anorexia, scalp tenderness, temporal HA, and fever. A dialted and nodular temporal artery may also be present. 50% of patients with GCA may also develop polymayalgia rheumatica, which characterized by fatigue and morning stiffness in the hips and shoulders
Testing for GCA
STAT ESR
STAT CRP
STAT CBC with differential and platelets
Temporal artery biopsy (gold standard)
-can have false negatives due to skip lesions
Elevated ESR
> age/2 for men
>age +10/2 for women
Elevated CRP
> 2.45 mg/DL
Elevated platelets
> 400,000 cells/uL
What does the ESR/CRP look like in GCA patietns
90% will have an ESR>50 mm/hr. An elevated ESR and CRP is 97% specific for GCA
Eye problem and GCA
AION due to occlusion from the SPCA
Ischemic optic neuropathy may not be present for the first 24-48 hours after the onset of blindness
If GCA is suspected, what must you do
Begin therapy with prednisone immediately. Low dose aspirin (81mg) should also be considered to reduce the chance of visual loss or stroke
Granulomatosis with polyangitis (Wegner’s)
Systemic vasculitis involving the URT, lungs, kidneys. 60% have ocular invovlvement
Ocular complications of Wegner’s
Granulomatous sclerouveitis, retroorbital mass lesion with proptosis, conjunctivis, episcerlitis, scleritis, and ciliary vessel vasculitis
Peripheral sclerokeratitis may also occur and lead to corneal ulceration
Scleroderma
Multisystem disorder causing inflammation and vascular changes of the skin and internal organs. Ocular effects:
-dry eye and shrinkage of the areas of the skin, including the conjunctiva
Gout
- caused by the formation of monosodium urate crystals in joints in response to increased Uris acid levels
- occurs most frequently in the MTP joint of the big toe, which is called podagra
- more common in men. Presents with sudden onset of red, hot joints
What ocular complication can occur secondary to gout
Band K
What drug is used to reduce gout flare ups
Allopurinol: reduces incidence of gout flare ups by inhibiting xanthine oxidase
The most common causes of acute, anterior, non-granulomatous uveitis are
Ankylosing spondylitis (90%) IBD (85-90%) Reactive arthritis (60%)
Since they are all HLA-B27, ordering this test fails to differentiate between them if you suspect uveitits is due to an HLA-B27 disease
Diseases of immunodeficiency
May develop due to inherited defects in the development of the immune system or may be secondary to disease that affect the normal immune system. An ex alpine of inherited immunodeficny is immunoglobulin A deficiency
Deficiency of immunoglobulin A
- IgA deficiency is the most common of the primary immunodeficiency diseases
- IgA is the prominent immunoglobulin in external secretions, including the tear film; involved in mucosal defenses
- patient will show a significant decrease in IgA in both serum and secretions
- may be asymptomatic, or may suffer from recurrent respiratory infections, keratinization of the cornea, weight loss, and diarrhea
AIDS
Caused by HIV, and RNA virus that uses reverse transcriptase to make viral DNA within effected cells. AIDs is marked by severe immunosuppression and resulting opportunistic infections. The smog common way to acquire HIV is through sexual contact. The most common symptoms include fever, lymphadenopathy, sore throat, rash, myalgia/arthralgia, and HA. Prolonged duration of these vague symptoms with the presence of mucocutaneous ulcers is suggestive of the disease
What do all drugs for HIV target
Reverse transcriptase
What CD4 count do you have to reach to have AIDS
<200
Testing for HIV
ELISA test is used as the screening test for HIV. A western blot is then used to confirm the ELISA test. Collectively these tests have a 99.9% specificity rate. Although these tests are still recommended for testing saliva and dried spot blood samples, the CDC recommends new guidelines for testing blood samples for HIV that involves Ag/Ab immunoassay to detect HIV Ab and HIV Ag within the blood
The most common ocular infection and the leading cause of blindness in AIDS
CMV retinitis
-CD4 <200 at risk, <50 at high risk
Treatment for CMV retinitis
Gancyclovir
Foscarnet
What is the most common opportunistic infection in HIV
Pneumocystic pneumonia
Parasitic opportunistic infections in HIV
Toxoplasmosis
- papilledema
- CN palsies
Bacterial opportunistic infections of HIV
Mycobacterium TB
Viral opportunistic infections of HIV
CMV and herpes simplex
Kaposi’s sarcoma is a malignancy caused by HHV-8
Kaposi’s Sarcoma
Herpes virus 8
HIV
Malignant
Red/purple lesions on the lids or conj
Seborrheic keratitis
Crusty, plaque like tan lesions that have a classic elevated “Stuck on” appearance. These are found most commonly in males over 30. Seborrheic keratosis is usually not treated; for smaller lesions, shave excision or curretage is recommended; larger lesions rewuire complete surgical excision
Keratoacanthoma
Isolated, dome shaped nodules usually seen on the face and mimics SSC. It has a spontaneous remission over a period of several months
Papilloma
Common slow growing epithelial tumors (viral warts) that may be caused by HPV
- characterized by finger like or cauliflower like appearance (skin tag); usually elevated and multilobulated with a central vascular core
- treatment for papillomas can range from no intervention, to excision or snipping, chemical cauterization, surgical excision, or cryotherapy
Xanthelasma
Yellow, elevated, plaque like lesions that are typically bialteral, symmetric, and located within the medial portion of the eyelids
- associated with older age, female gender, and high cholesterol, although most patients with xanthelasma have normal cholesterol
- treatment includes full thickness surgical excision, carbon dioxide laser treatment, and chemical cauterization; recurrences after treatment are common
Molluscum contagiosum
- Chronic infection (direct contact) skin condition caused by DNA pox virus. The condition is common in kids and young adults in communities with poor hygiene.
- classic presentation is a single or multiple dome shaped waxy umbilicated nodules on the eyelid or eyelid margin. Patients are typically asymptomatic, but nodules can spontaneously open, resulting in a follicular conjunctivitis
- if multiple are presently, HIV should be considered
- treatment is incision, curretage, shaving excision, cauteriation, or cryotherapy
What causes follicles
CHAT
- chlamydia
- herpes
- adenovirus
- toxic (molluscum)
Acne rosacea
Syndrome of undertermined etiology that affects sebaceous glands of the face and eyelids. It is characterized by vascular abnormalities and papulopustular lesion on the cheeks and forehead. Classic signs include superficial telangiectasia, rhinophyma (late stage), and facial flushing. The latter is associated with triggers such as alcohol, spicy foods, caffeine, and increased sun exposure
Ocular findings of acne rosacea
Due to inflammation of the meibomian and zeiss glands
- inspissated meibomian glands
- blepharitis
- hordeola
- chalazia
- DED
- phlyctenules
- staph marginal keratitis
- SPK
- corneal neo
Allergic contact dermatitis
Delayed type 4 HS response of inflammation to any substance that comes in contact with the skin. Classically results from cosmetics, makeup, shampoo, hairspray, fingernail polish, perfume, jewelry, poison ivy, CL solution. Medications such as aminoglycosides, trifluridine, cyclo/myds, glaucoma meds, and preservatives
Ocular signs of contact dermatitis
Acute periorbital swelling
Conjunctival chemosis, redness, itching, and tearing
Impetigo
Gram + infection with classic honey colored crusted lesion
Very common in kids
HSV
HSV I and II. Both can cause ocular infections but type I is significantly more commonly (98%) of cases. Type I HSV usually occurs above the belt and is transmitted by close persons along contact. Type II HSV usually causes infections below the belt and is STD
What HSV is most common cause of ocular manifestations
Type I
Which HSV is the most common cause of herpetic keratitis in neonates
HSV II
90% of adults wtih HSV I obtain it from
Primary infection as a child
After initial infection of HSV
Virus hides in the trigeminal ganglion and can reoccur at any time
Reactivation of HSC can be triggered by
Physical and emotional stress, including sun exposure, hormonal changes, fever, trauma, and immunosupression
Primary ocular exposure of HSV
Blepharitis with focal vesicular lesion on the eyelids and periorbital skin
Acute unilateral follicular conjunctivitis with PAL
Recurrent HSV ocular manifestations
- dendritic keratitis
- marginal or GA ulcers
- neurotrophic Keratopathy
- interstitial keratitis
- disciform endotheliitis
- acute anterior unilateral non-granulomatous uveitits with trabeculitis
- acute retinal necrosis
After an inital corneal HSV epithelial infection, there is a ___ chance of having a recurrent.
25%
The risk increases to 40-45% after a 2nd episode
HZV
Initially presents at chicken pox. After the inital infection, the virus lays dormant in the nerve roots. HZV is the reactivation of VZV in the dermatome that was assocaited with the latent virus. 66% of patients are over the age of 50. Consider a medical evaluation in patients younger than 40 to determine if they are immunocompromised
HZO may involve the following
- blepharitis with vesicles on the eyelid margin
- acute follicular conjunctivitis on the affected side
- episcleritis
- pseudodendritic keratitis
- acute anterior unilateral non-granulomatous uveitis with trabeculitis
- acute retinal necrosis
- optic neuritis
- EOM palsies
- proptosis
Hutchinson’s sign
Vesicular rash on the tip of the nose. Indicates involvement of V1, which increases the risk of ocular involvement
Nasociliary
Behçet’s disease
Inflammtory disease causing multissytem complications. Recurrent oral aphthous ulcers and two of the following features are needed for diagnosis: genital ulcers, eye lesions, or skin lesions. Most commonly seen in Asian and middle eastern young adults. Ocular findings are
- acute recurrent hypopyon,
- iritis
- posterior and anterior uveitis
- retinal vasculitis
- vitritis
- secondary cataracts
- glaucoma
- neovascular lesions
What is the only systemic condition that presents with a hypopyon
Behçet’s disease
Malignant melanoma
- most common cancer of young women
- depth of invasion is the number one prognostic factor
- risk: age, skin color, fam Hx, an repeated irritation and sun exposure
- characteristics of suspicious skin lesions for malignant melanoma include ABCDE; asymmetry, border, color, diameter, and enlarging
Most common cancer of young women
Malignant melanoma
What is the number one prognostic factor for malignant melanoma
Depth of invasion
What is the most common variant of melanoma
Superficial spreading melanoma
-it has rapid growth and is classically found on non-exposed skin
BCC
- malignancy of the basal cell layer of the epidermis. Often appears as a shiny, firm, pearly nodule with superficial telangiectasia. Progression can lead to central ulceration (rodent ulcer)
- treatment is 5-FU or biopsy with surgical removal
SCC
Malignancy of the stratum spinosum layer of the epidermis. Non-healing ulcer that often appears an an erythematous plaque. It can arise from a pre cancerous lesion called actinic keratosis
Sturge Weber Syndrome
Rare congenital vascular disorder characterized by a facial capillary malformation (overabundance) known as a port wine stain. These patietns may have seizures, focal neurological deficits, or mental retardation. Ocular findings
- unilateral glaucoma due to increased EVP (obstruct outflow)
- vascular malformations of the conjunctiva, episclera, choroid, and retina
- iris heterochromia
Tuberous sclerosis
Multisystem genetic disease that causes benign tumors to grow in the brain and other organs. About 90% of these patietns with TSC have one of the characteristic skin lesions which include hypopigmented macules (ash leaf spots), shagreen patches, angiofibromas, and a distinctive brown fibrous plaque on the forehead. Ocular findings
- astrocytic hamartoma s or phakomas which are grey-ish or yellowish-white lesion in the retina. Astrocytic hamartoma calcify and can be seen on a CT scan
- choroioretinal depigmentation
- coloboma
- angiofibromas of the eyelid
- papilledmea ( from brain tumors)
AD disroders
Resutls from one abnormal dominant gene. If an affected heterozygous man (Aa) and an affected heterozygous woman (Aa) have kids, the rolling phenotypes are possible
- 75% of offspring will have the condition
- the remaining 25% will NOT have the condition
AR disorders
Result from two abnormal recessive genes. If a man (aa) and a carrier heterozygous woman (Aa) have kids, the following phenotypes are possible in their offspring
- 50% of their kids will have the condition
- the remaining 50% of the kids will NOT
X-linked disorders
Defective recessive genes on the X chromosome. A man is affected i he has an abnormal recessive gene on his X chromosome. A woman is affected i both X chromosomes have the abnormal recessive gene. If an affected nan (XdY) marries a carrier women (XDXd), the following phonetypes are possible
- half of their sons will have the condition
- half od their daughters will have the condition
What systemic diseases are associated with keratoconnus
TDOME Turners Down OI Marfans Ehlers-Danlos
Down syndrome (trisomy 21)
- most common chromosomal disorder
- caused by an extra 21st chromosome
- mental retardation, flat facial profile, prominent epicanthal folds, congenital cataracts, glaucoma, strab, simian crease in hands, congenital heart disease, and early onset of Alzheimer’s, keratoconnus
Klinefelters syndrome
XXY
- girly men
- most common cause of male primary hypogonadism
- an extra X chromosome (XXY) results in testicular atrophy, long extremities, gynecomastia, female hair distribution, and hypogonadism
Turners syndrome
X0
- absent X chromosome
- manly girls
- the only sex chromosome aneuploidy with established ocular findings; common ones include keratoconus, strab, amblyopia, reduced accommodation, and convergence insuffiency (turners=eye turn)
- affects 1 in 3000 females and is characterized by short stature, dysgenesis, webbing of the neck, and coarctation of the aorta
- most common cause of primary amenorrhea
Which genetic gender disorder affects the binocular vision
Turners
-strab and Amblyopia
Autosomal dominant disorders result from
Abnormal structural genes. They affect many generations and occur equally in males and females. They often present after puberty; one must consider fam Hx in dx. Examples of AD disroders include
- VHL
- NF1
- marfans
- Huntington’s Chorea
- FAP
VHL
AD
Benign and malignant tumors
Retinal agiomas that can hemorrhage if left untreated, leading to retina detachment, glaucoma, and loss of vision
NF1
AD Tumor forming nerve cells 1:3000 50% have no fam Hx Triad -cafe au lait spots, neurofibromas, and lisch nodules on the iris Optic nerve gliomas (can squeeze ON) Congenital glaucoma
Marfans
AD
CT disease
Tall ,long extremeitis, subluxating joints, and long fingers and toes
Causes cardio effects such as aortic incompetence, dissecting aortic aneurysms, and floppy mitral valve
Ocular effects
-subluxation of the lens (ST), RD, kone
Number one cause of lens subluxation
Truama
Huntington’s chorea
AD
Chromosome 4
Gradual onset and progression of chorea and dementia
30-50 years of age, with a 15-20 year survival rate
Abnormal eye movements will occur and include a delay in pursuits, voluntary saccades, and refixation (“Hunters pursuit”)
FAP
AD
Chromosome 5
Hundreds of polyps on the colon post puberty
100% of these patients get colon cancer
Gardners syndrome
-variant of FAP characterized by multi-focal CHRPE (4 or more)
-refer for colonoscopy
AR diseases
Sickle cell
Tay-Sachs
PKU
AR disorders are usually
Seen in 1 generation, as 25% of offspring from 2 carrier parents are affects. The disease are often more severe than the dominant disorders and will often present in childhood
Sickle cell anemia
- patients have painful crises (ischemia) due to sickle CHRPEs RBCs causing blockages within arteriole vessels; ultimately leading to organ failure I
- the most common form of sickle cell anemia is caused by a single base pair mutation in the beta globin gener where VALINE is substituted with GLUTAMIC ACID
- 1 in 400 AA are afflicted. 8% of AA population are carriers
- can occlude retinal arterioles, leading to ischemia and subsequent retinal neo (proliferative). The blood vessels are often called sea fan retinopathy because of their shape.
Labs for sickle cell
Decreased hemoglobin
Increased bilirubin
Increased reticulocytes
Blood smear shows sickling
What diseases cause proliferative retinopathy
DRVOS
- DM
- ROP
- vein Occlusions
- OIS
- sickle cell
PKU
- AR
- mutations in the enzyme phenylalanine hydroxylase; this is used in the AA conversion of phenylalanine’ to tyrosine. If not treated, it will result in mental retardation. All newborns are tested for PKU. If the patient responds as a positive carrier, then treatment with a diet low in phenylalanine is initiated. Phenylalanine is found in milk products, aspartame, meat, and chicken.
- occurs in 1/10,000 people
Tay-Sachs disease
AR
Eastern European Jews
progressive destruction of the nervous system. Ocular findings
- cherry red spot
-atrophy of the ON
Build up of ganliosides in the ganglion cells (none in the fovea)
What are two conditions that can cause a cherry red spot
CRAO
Taysaches disease
X linked conditions
Fabrys
Duchenne muscular dystrophy
Fabrys diseas
X linked
Abnormal lipid depositions in blood vessel walls throughout the body. Deficiency of the enzyme alpha galactosidase A allows lipids to build up to harmful levels in the eyes, kidneys, ANS, and cardiovascular system. It affects adolescent boys and is characterized by excruciating pains in the extremeities and abdomen. Areas of telangiectasia on the umbilicus, growing, elbows, and knees are diagnostic
Ocular findings
-light colored, whorl K
Duchenne muscular dystrophy
- AR
- deletion within the gene coding for dystrophin
- 1/3000 male infants afflictedm symtoms by age 5
- condition is characterized by muscle weakness that begins in the pelvic girls and progresses superiority
- walking and getting tired
Which of the keratonnus systemic diseases has messed up type 1 collagen
OME of “TDOME”
OI
Brittle bone disease
-host of genetic defects giving rise to abnormal collagen synthesis. Characterized by multiple fractures occurring with minimal trauma. Ocular findings are blue sclera, keratoconnus, and megalocornea
Mitochondrial disorders
Transmitted only through mother’s (maternal inheritance). All offspring of affected females may show the disease. An important mitochondrial disorders with ocualr results manifestations is LHON
LHON
rare recessive disorder that resutls in bilateral, asymmetric primary optic neuropathy. 85% of affected patients are males. The average age is late teens or early 20s. The condition may spontaneously improve in 35% of cases
ocular findings:
-early: optic disc hyperemia, and telangiectatic vessles
-late: progressive optic disc pallor with resulting loss of central vision (BCVA 20/200 to CF)
My Mother Leber Lost My Eyes
Most common type of anemia
Iron deficiency
More than 50% of anemia’s fit into this category
Anemia’s with decreased MCV (small cells)
Iron deficiency
Thalassemia
All have decreased Hgb
Anemia’s with normal MCV
Aplastic
CKD
Sickle cell
All have decreased Hgb
Anemia’s with increased MCV
Vit B12 deficiency
Folic acid deficiency
Alcoholism
Iron deficiency anemia
- most common anemia
- adutls=GI blood loss
- may also occur with malabsorption or increased need with decreases intake, such as in childhood or pregnancy
- iron defiance impairs cellular function and can cause brittle hair, nail spooning, and pica (eating weird things)
- treat with oral iron
Aplastic anemia
Pancytopenia characterized by severer anemia, neutropenia, and thrombocytopenia
-destroyed bone marrow
Causes of aplastic anemia
Infectious agents (viruses) Radiation (chemo) Drugs -chloramphenicol -acetazolamide -trimethoprim -methotrexate -pyrimethamine
Anemia of chronic kidney disease
Chronically damage kidneys synthesize inadequate amounts of erythropoietin (EPO), a hormone that normally stimulates RBC production in the bone marrow
Sickle cell anemia
Can cause proliferative retinopathy due to the crescent or sickle shaped cells occluding the retinal vessels
Vitamin B12 deficiency
Due to inadequate intake or malabsorption of vitamin B12; malabsorption is often caused by pernicious anemia
Pernicious anemia
Characterized by autoAB directed against parietal cells of the stomach, causing decreased production of intrinsic factors
Intrinsic factor and B 12
Intrinsic factors helps absorb B12. Decreased=decreased B12
Folic acid deficiency
- dietary deficiency is the leading cause and use especially common in alcoholics
- may also be drug induced (chemo, methotrexate), or from malabsorption syndromes
- folic acid deficiency in pregnancy increases the risk of neural tube defects (spina bifida)
Multiple myeloma
Neoplastic disorder of plasma cells
Proliferation of a malignant clone of plasma cells in the bone marrow. This often results in extensive skeletal bone destruction, unexplained anemia, hypercalcemia, and acute renal failure
Neoplastic disorders of the blood cells
Leukemia and lymphoma are malignant proliferative diseases involving leukocytes. Early cell growth and maturation are inhibited, causing “malignant clones” of these cells to accumulate. Death results because of substantial loss of normal cells, and also due to poor organ function owing to the increase in malignant cells
Lymphoma-lymph tumor
Proliferation of malignant lymphoid cells in solid tissues such as lymph nodes, spleen, and the GI tract. Initally the tumor is localized, but it may subsequently spread. There are two types of lymphomas
- Hodgkin
- Non-Hodgkins
Hodgkin’s lymphoma
40% of lymphoma
- tow peak age groups: 15-30 and then >50
- commonly presents with enlarged lymph nodes, fever, night sweats, and itching
- characterized by Reed-Sternberg cells (owl eye nucleus)
- prognosis is good is dx early enough
- 50% of cases are associated with EBV
Non-Hodgkins lymphoma
- 60% of lymphomas
- enlarged lymph nodes and GI tumors (with abdominal pain)
- heterogenous group of malignancies with variable prognoses depending on the type
- bone marrow biopsy is performed to determine T or B cell ropes
Reed sternberg cells
Hodgkin lymphomas
Which type of lymphoma is most common
Non-Hodgkin
Acute leukemia
This disease can affect all ages, but usually occurs in younger patients. The predominate cell type is Blast cells: >30% of marrow cells are blasts (immature cells). There are two major types of acute leukemia
- Acute myeloblastic leukemia (AML)
- Acute lymphocytic leukemia (ALL)
Acute myeloblastic leukemia (AML)
- infants and middle aged or older
- characterized by normal WBC count with excessive myeloblasts
- Auer Rods may be seen within leukemic cells in the blood
Auer rods
AML
Acute lymphoblastic leukemia (ALL)
- peak age 2-10
- normal WBC count with excessive lymphoblastic
- with treament, 75% of kids remain disease free > 5 years
ALL kids live long
A retinal hemorrhage with a white spot in the middle
Roth spot
Characteristic of leukemia and endocarditis
Chronic leukemia
Usually affects older adults. The predominant cells are mature cells of the bone marrow. Patients are often asymptomatic and may have anemia. There are two types
- Chronic myelocytic leukemia (CML)
- Chronic lymphocytic Leukemia (CLL)
Chronic myelocytic leukemia
- age of onset 25-60 years old. Poor prognosis, only 3 year survival rate
- characterized by WBC count of 50,000 to 300,000 with increased granulocytes in all states of maturation
- 90% have the Philadelphia chromosome
“Cut my life”
Philadelphia chromosome
CML
Chronic lymphocytic leukemia (CLL)
- onset >50
- male/female 2:1
- WBC count of 20,000-200,000 with a predominance of mature small lymphocytes
- 5-10 year survival rate
“Could live long”
Leukopenia
A decrease in the number of WBC
Due to bone marrow injury, bone marrow inactivation, drugs, or chemical suppression
Leukocytosis
Increase in the absolute number of WBCs
Can occur after surgery, or result from infections, illness, stress, or pregnancy
Neutrophilia
Increase in he absolute number of neutrophils
Typically reuslts from stress, exercise, pain, fear, or pathological infections
Thrombocytosis
Elevated platelet count
Causes include inflammation, kidney disease, or spleen removal
Pancytopenia
Decrease in the number of RBCs and WBCs and platelets
Thrombocytopenia
Decrease in platelets
Causes include infection, live failure, and bone marrow disorders
Edema
Increased intestinal fluid that can be non-inflammtory (yielding protein poor transudate) and inflammatory (yielding protein rich exudate) in etiology. Edema can be recognized on exam by increased swelling of the length due to subcutaneous edema, or shortness of breath due to pulmonary edema. Treatment includes diuretics and compression stockings
Non-inflammatory causes of edema
- Increased organ pressure: seen in CHF, liver cirrhosis, and venous obstruction or compression
- Reduced plasma osmotic pressure: protein-losing glomerulo-pathologies and malnutrition
- Lymphatic obstruction-post surgical or neoplastic
- Sodium retention: excessive salt intake with renal insuffiency
diabetic macular edema
Caused by macroaneurysms with dilation of capillary walls. Pericytes, endothelial foot-plates that surround the vessels, are damaged, allowing leakage of blood and fluid with resulting edema
Inflammatory causes of edema
Acute and chronic inflammation
Angiogenesis (after injury)
Hemorrhage
Leakage of blood due to a vessel injury that may be uncontained or enclosed within a tissue. Hemorrhage within a tissue are referred to as hematomas. All are grouped according to size
Petechiae
1-2mm hemorrhages on the skin
Purpura
Greater than 3mm hemorrhagehs associated with trauma, local vascular inflammation, and low platelet count
Ecchymoses
Greater than 1-2cm and include subcutaneous hematomas or bruises
Hemothorax/hemopericardium/hemoperitoneum
These are large accumulations of blood within body cavities
Thrombosis
Resutls from an inappropriate activation of blood clotting in an uninjured vein, or an occlusion of a vessel after a relatively minor injury. These can form anywhere in the circulatory system, may be arterial or venous, and may be non-occlusive. The most common location of thrombus formation is the deep venous system of the legs
Virchows triad
A major theory delineating the cause of a venous thromboembolism (VTE). It proposes that VTE occurs as a result of
- Alterations in blood flow STASIS
- Vascular endothelial INJURY
- Alterations in the constituents of the blood through an inherited or acquired HYPERCOAGULABLE STATE
Inherited hypercoagulable states
Factor V laden
Antothrombin 3 deficiency
Protein C and S deficiency
Prothrombin gene mutation
Acquired hypercoagulable states
BCP
Preg
Smoking
Malignancy
Venous thrombosis
Commonly occurs in either the superficial or deep legs of the veins. Those in the deep leg veins (typically above the knee) are mote likely to embolism to the heart or lungs
Leg to lungs=pulmonary embolism
Arterial thrombosis
These are commonly formed from atherosclerosis or a MI. They usually travel (embolism) to the brain, kidneys, and spleen
Causes of CRVO and BRVO
Systemic HTN and DM are associated with an increased risk for the development of central abd branch retinal vein occlusions, conditions that most commonly result from thrombus formation
CRVO in young female
BCP
CRVO in young male
Inherited state of hypercoagulability
Difference between a thrombus and embolus
A thrombus is a clot that remains where it was formed. An embolism is a clot that has dislodged from where it was formed
Embolism
Refers to any intravascular solid, liquid, or gas mass carried by the blood to a distant site from its point of origin: 99% arise from thrombi
What are the types of emboli?
FATBAT
- fat
- air
- thrombus
- bacterial
- amniotic
- tumor
Fat embolism
Long bone fractures and lipsuction
Air embolisms
Result from gas bubbles after a chest wall injury or from an obstetric procedure
Thrombus
Most from DVTs go to the pulmonary system
What is the number one type of embolism
Thrombus
Bacterial and tumor embolism
Cause tissue destruction and pain
Amniotic fluid emboli
Can lead to DIC, especially postpartum
Shock
Decreased blood perfusion resulting from a reduction of either cardiac output or circulating blood volume.
Types of shock
Cardiogenic
Hypovolemic
Septic
Anaphylactic
Cardiogenic shock
Etiologies include MI, ventricular rupture, arrhythmias, and pulmonary embolism. The heart fails to pump due to cardiac cell damage, extrinsic pressure, or outflow obstruction
Hypoveolemic shock
Hemorrhage and fluid loss from vomiting, diarrhea, burns, or trauma
Septic shock
Overwhelming microbial infections or toxic shock gram positive septicemia. The main mechanism includes peripheral vasodilation, leading to pooling of blood
Anaphylactic shock
Histamine cause cause the following during anaphylactic shock
- hypotension and tachycardia
- sneezing, itching, hoarseness, and stridor
- bronchospasm
- nausea, vomiting, abdominal pain, and diarrhea
- HA, syncope, and seizure
Aneurysms
Localized abnormal dilation of blood vessels. A true aneurysm is enclosed by complete arterial wall components. The most common aneurysms occur in the abdominal aorta, iliac, and other large arteries
False aneurysm
Pseudoaneurysm
-form with parts of the arterial wall missing
Death from aneurysm
May result from rupture, pressure upon adjacent structures, occlusion of the proximal vessels, or embolism from mural thrombosis
Pupil involving CN III palsies
Most likely a result of an aneurysm
Abdominal aortic aneurysm
Typically found in men over 50
Risk of aneurysm rupture
Increases with the maximal diameter of the aneurysm bulge
Atherosclerosis
Slowly progressing disease of the arteries marked by elevated fibrosis and fatty intimal plaques. Theses A.R. formed by fat deposits, smooth muscle cell proliferation, and synthesis of an extracellular matrix in the intima. Elastic arteries are involved, principally in the abdominal aorta, coronary arteries, popliteal arteries, descending thoracic artery, and ICAs. Patients are asympatomic for decade and can eventually experience shortness of breath and chest pain
Endothelial cell injuries and heart disease
These may lead to atherosclerosis. Monocytes and leukocytes circulating in the bloodstream adhere to the injured vessel endothelium. They then transform into macrophages, which attract lipids and become foamy cells. This causes lipoproteins to migrate into the vessel wall at the enter of the endothelial injury. The accumulation of macrophages and the adhesion of platelets to the injured areas cause a mass ICA plaque or clot to form. This can lead to reduced blood glow to the heart, ultimately causing MI
When does atherosclerosis being
Childhood
Usually appears in middle aged
Risk factors for atherosclerosis
Older than 50, family Hx, HTN, smoking, hypercholestermia, and diabetes
Lab results and atherosclerosis
Increased total cholesterol, decreased HDL, increased LDL, and increased CRP
Normal total cholesterol numbers
<200
Normal HDL levels
> 40
Normal LDL levels
<130
Normal triglyceride levels
<150
Disease prevention of atherosclerosis
Low fat diet, exercise, BP monitoring, and smoking cessation
Treatment for atherosclerosis
Aggressive lipid lowering therapy (statins), diet and exercise. Stenting of arteries also serves as a secondary treatment for patients who demonstrate symptoms of CAD
What do statins do for lipids
Inhibit HMG-CoA reductase, the rate limiting step in cholesterol synthesis
The most commonly encountered peripheral corneal opacity
Arcus senilis
It is a sign that the patient has or has had high cholesterol. It does not effect vision
CAD
Characterized by atherosclerosis within the coronary arteries supplying blood to the heart. Patients can be asymptomatic or have chest pain (angina) and dyspnea (difficulty breathing). If not treated by diet, exercise, or stenting of the artery, the patient may suffer a MI. If a patient experiences MI, 325mg of aspirin should be given immediately
What drugs should be given to someone with chest pain
MONA Morphine Oxygen Nitroglycerin 325 mg aspirin
If someone is having an MI, what should be given to them immediately
325 mg aspirin
CPR
No pulse or respiratory efforts
Chest compressions are the most important element.
Interruptions in CPR Results in declines in coronary and cerebral perfusion pressure
100 compressions/min
Compress chest at least 5cm with each down stroke
Allow chest to recoil completely between
30 compressions to 2 ventilations
Continue compressions with an advanced airway
Carotid artery disease
Atherosclerosis of the carotid arteries. 25% of strokes are due to build up of plaque causing stenosis of the carotid arteries. These patients present with sensory loss, dizziness, amaurosis fugax, or complete loss of vision in the ipsilateral eye. They may have a high pitched bruit over the artery
HTN
The most common primary diagnosis in America. More than 50% of people 60-69 years old, and appx 75% of those 70 years of age and older are affected. The rise in systolic pressure continues throughout life, in contrast to diastolic presure which increases until age 50, and then tends to level off. HTN leads to increased risks of heart attack, heart failure, stroke, and kidney disease
Prehypertension
120-139/80-89
Hypertension Dx
BP of 140/90 or higher
New HTN guidelines
120-129/<80=elevated BP
>130-139/>80-89=HTN stage 1
>140/>90= HTN stage 2
>180/>120= HTN urgency
Lifestyle and HTN
AHA recommends health lifestyle changes for patietns with elevated BP
Recommendations for stage 2 HTN
Healthy life style changed and 2 BP lowering meds in different classes
The risk of HTN increases with
Age, family Hx, race, obesity, diabetes, smoking, and excessive Na intake. AA have the highest probability of HTN, with Asians maintaining the lowest risk
Treaments for HTN
Anti hypertensive meds, exercise, weight loss, low Na diet
Taking blood pressure
When taking BP reading, the patient should be seated quietly for at least 5 minutes in a chair, with feet on the floor, and arm supported at heart level. Smoking, caffeine, and excessive should be avoided for 30m prior to BP measurement. Appx the size of the cuff should be used to ensure accuracy. At least two measurements should be made and the average recorded. SBP is the point at which the first of two Kotokoff sounds are heard; and the disappearance of the Korotkoff sound is used to determine the DBP
HTN retinopathy/choroidopathy
Narrowing of the retinal arterioles. In acute or advanced HTN, the retinal vasculature may be injured enough to cause occlusion of leakage. These changes may be seen as RNFL infarcts ( soft exudates or CWS), extravascular edema (hard exudates), intraretinal hemorrhages, and retinal arterial macroaneurysms. HTN choroidopathy is often found in younger patients with acute HTN. These findings can include Elschnig spots and Siegrist Streaks. Severe HTN can cause optic neuropathy and may present with flame hemorrhages, venous congestion, and macular exudates. Papilledema is found in stage 4 HTN retinopathy (malignant HTN)
CHF is a chronic disease that is defined by the hearts
- Inability to fill with blood (diastolic heart failure) or
2. Inability to pump efficient amounts of blood through the body (systolic heart failure)
Left sided heart failure
- When the left side of the heart fails, blood backs up into the lungs, causing them to fill with fluid (pulmonary edema)
- the most common cause of left sided CHF is Ischemic cardiomyopathy resulting from coronary artery disease (MI)
- the primary symptom is shortness ofbreak
Right sided heart failure
- Inability to pump blood into the lungs, causing blood to back up in the abdomen (ascites) and legs (lower extremity edema)
- symptoms: edema, chest discomfort, and shortness of breath
- the smog common cause of right sided CHF is left sided CHF. Other causes include pulmonary HTN, COPD, and right sided MI
Risk factors for CHF
CHD, cigarette smoking, HTN, obesity, DM, and valvular heart disease
Diagnostic findings of CHF
Elevated BNP
Cardiomegaly on chest X ray
Changes on echocardiogram
Treatment of CHF
BBlockers, ACE inhibitors, diuretics, and correction of any underlying causes
Rheumatic fever
Usually ages 5-15; results from untreated pharyngeal infections with group A strep beta hemolytic streptococci (strep pyogenes). This disease can alter the shape of the heart valves. In 75-80% of cases, the mitral valve is attacked, often requiring valve replacement later in life. Common signs and symptoms include fever, elevated ESR, red-hot joints, and endocarditis
Bacterial endocarditis
Bacteria may infect the inner lignin of the heart, especially the valves.
- risk factors: prosthetic heart valves, IV drug use, and age
- present with fever and may have symptoms from arterial emboli to heart failure, depending on the severity of valvular damage
- the echocaridogram (US of heart) will show “vegetations” on heart valves (mixture of bacteria nad thrombus) or abscesses within the heart tissue
Heart palpitations
Symptoms a patient experiences when the heart has an atypical rhythm. Most are benign, irregular heartbeats, with the most common being PVCs. These can increased with dehydration, stress, decreased sleep, exercise, or pregnancy
TIAs
TEMPORARY neurological deficits (always less than 24 hours, usually less than 15m) due to inadequate perfusion; as perfusion is restored, patients are left with no symptoms. The most common cause is an embolism.
Types of embolism
- Carotid artery TIA with contralateral hand/arm weakness or sensory loss and face and leg symptoms. Patients can also have ipsilateral visual symptoms (amaurosis fugax) or aphasia
- Vertebrobasilar TIA can result in diplopia, ataxia, vertigo, dysarthria, and either unilateral or bilateral visual loss
Hollenhurst plaque
Embolism found on the retinal vasculature. These patients often have TIAs and should be referred immediately
Stroke
Characterized by sudden onset of neurological deficits reflecting the arteries involved and the brain structures they supply
Risk factors for stroke
HTN DM HLD Tobacco use Fam HX
Early diagnosis of stroke
Auscultation of the subclavian and carotid arteries for a bruit
strokes and CT scans
Used to detmermine the size and the location of strokes
Ocular manifestations of strokes
Depend on the vasculature affected and whether the macula is invovled
Macular sparing homonymous hemianopsia
Stroke at the occipital lobe that has affected either the MCA or the PCA but not both
Macula only homonymous hemianopsia
Almost never in a stroke
-most commonly occurs from a tumor that has compressed both blood supplies to the macualr cortex
Ischemic stroke
Accounting for 80% of all strokes, they resutls from occlusion of an artery leading to the brain; embolism of an atherosclerotic plaque is the most common cause. Treatment involves modification of stroke risk factors
Hemorrhagic stroke
Occurs as a result of accumulation of blood within the brain due to bleeding from brain tissue, truama, and/or vascular malformations. The smog common type of hemorrhagic stroke is a subarachnoid hemorrhage
Subarachnoid hemorrhage
- sudden severe HA, pupil involving CN III palsy, and nuchal rigidity
- 30% have changes in consciousness
- aside from head trauma, the smog common cause of a subarachnoid hemorrhage is rupture of an intracranial aneurysm. Tend to occur at arterial bifurcations and are usually asymptomatic until they rupture, causing a hemorrhage.
- those that cause CN III palsies tend to occur in the circle of Willis, between the junction of the posteiror communicaitng and ICA
Where is the most common cause of hemorrhagic stroke
Junction of ICA and posterior communicating arteries
CN III pupil involving
The circle of Willis
Meeting loop for the basilar artery, the ICA, and the anterior and posterior communicating arteries, which are small arteries bridging the basilar and ICA. The CoW forms an arteriolar circle beneath the Crain and allows for a system of redundancy for the flow of the blood to intracranial tissue
ACA location
Extends upward and forward from the ICA
Area the ACA supplies
Frontal lobe
MCA location
Last and largest branch of the ICA
What does the MCA supply
Frontal lobe, lateral surface of the temporal and parietal lobe, and the occipital lobe.
Location of PCA
Stems from the basilar artery
Supply of the PCA
Temporal and occipital lobe
Defects in the ACA will effect
Logical thought, personality, and voluntary movement
MCA infarct will affect
Primary motor and sensory areas of the face, throat, hand, and arm, and areas for speech and vision
PCA infarct will affect
Contralateral hemiplegia, hemianopsia, color blindness, verbal dyslexia, and opposite VF defects
Seizures
These single events may be caused by fevers, HA, stress, illness, or neurological deficits
- epilepsy is a condition with recurrent seizures. Status epilepticus is a prolonged seizure lasting longer than 5m-medical emergency
- treatment includes antiepitleptics (phenytoin, phenobarbital, carbamazepine, valproic acid). Absence seizures are treated with Klonopin
Syncope
Abrupt, transient loss of consciousness lasting a few seconds to a few minutes due to decreased blood flow to the brain. Prompt recovery to full consciousness follows the syncopal episode. Causes include cardiac abnormalities, vascular problems, or neurological deficits. Specific causes can be hard to diagnose and are usually benign
Cluster HA
- men 30-50 years old
- orbital or temporal; usually unilateral
- smokers and drinkers
- HA wake patients up at night or early in the AM
- generally 1-2 attacks per day, each lasting less than an hour
- often present with red eyes and/or nasal stuffiness and can cause transient or permanent ipsilateral horners syndrome
What kind of HA can cause an ipsilateral horners syndrome
Cluster HA
Tension HA
- all ages, females
- worse with stress, and may precede a migraine
- band like distribution and varying intensities
Migraine HA
- women, certain triggers
- ages 20-30, rarely after age 50
- usually present with or without an aura, nausea, or photosensitivity
- migraines typically last anywhere from 4-72 hours and are aggravated by physical activity
Brain tumor HA
- also cause HA and can occur in all ages and sexes
- interrupt sleep
- associated with nausea, vomiting, and visual changes, and steadily increase in severity with time. Brain tumor causing HA tend to be worse in the AM
- a child with a brain tumor is likely to have 1 or mroe other physical symptoms (other than HA) and 1 or more other neurological deficits. Less than 1% of patients with brain tumors have HA as their only symptom
Meningitis
Inflammation of the meninges caused by various infectious agents such as viruses, bacteria, fungi, or chemical agents. Viral meningitis is common and extremely difficult to treat
- 3 out of 100,000 people
- often preceded by an URTI
- fever, HA, and neck stiffness. May also have nausea, vomiting, sweats, weakness, myalgias, papilledema, and photophobia.
What is the most common primary malignant brain tumor
Glioblastoma multiforme
Glioblastoma multiforme
- the most common primary malignant brain tumor
- cerebral hemispheres and can cross the corpus callosum
- grave prognosis, less than 1 year life expectancy
The most common brain cancer in adutls
Is from metastasis
-lung and breast are the most common primary tumor sites
Meningioma
- arachnoid cells
- most common benign tumor of the brain although it can be malignant
- usually in middle aged women
Most common primary benign brain tumor
Meningioma
Schwannoma
- tumor of Schwann cells. Can cause gradual onset of painless, progressive proptosis
- often localized to the CN VIII (acoustic Schwannoma)
- rare, young to middle aged adutls
Pituitary adenoma
- can be functional or non functional. Prolactin secreting tumor is the most common functional pituitary adenoma
- bitemporal hemianopsia or junctional scotoma VF loss
MS
AI disease marked by recurrent inflamamtion of the CNS that ultimately reuslts in demyelination. Typically affects females more than males, with onset 20-40yo. most common in caucasians living in northern latitudes and has genetic components
Diagnosis of MS
Two separate CNS lesions (separated in space) on two or more occasions (spearted in time). These lesion must include involvement of the white matter
2 lesions
2 MRIs
White matter
Systemic symtoms of MS
Numbness, tremors, lack of coordination, vertigo, bowel and bladder dysfunction, and fatigue. Symptoms may occur abruptly and typically last weeks to months
Most common presenting symptom of MS
Optic neuritis
Ocular findgins of MS
Optic neuritis Pain on eye movement APD INO Diplopia
MS with optic neuritis
Classically present with a sudden, non-progressive onset of monocular vision loss. Can also report decreased acuity after increase in body temp (Uhtoff’s phenomenon) (exercise or hot shower)
INO
Lesion of the MLF
Loss of ipsilateral adduction and contralteral nystagmus
Guillain-Barré syndrome
inflammation and demyelination of peripheral nerves and motor fibers of the ventral roots. Classically presents with symmetric ascending muscle weakness that begins in the distal lower extremities. Ocular findings include
- ADies tonic pupil, diplopia, facial diplopia
- elevated protein in the CSF can cause papilledema
MG
AI disease with autoAb directed against ACH receptros in the NMJ. This condition may affect all ages, but is more common among younger women and older men. It is assocaited with a thymic tumor (thymoma), RA, and lupus. Systemic symtoms include respiratory weakness and weakness of the jaw muscles. Ocular findings include
- ptosis and diplopia
- SYMPTOMS WORSE AT THE END OF THE DAY
Alzheimer’s disease
Unknown reasons is develops
Develop deposits (plaques) of a protein called beta amyloid, and disorganized masses of protein fibers within the brain cells known as neurofibrillary tangles.
-this is the most common form of dementia affecting up to 10% of Americans over the age of 65
-more prevalent in women, people with head injuries, Down syndrome, prolonged exposure to chemicals, lower educational levels, and epilepsy
-no cure, but donepizil helps slow progression (ACHase inhibitor)
Vascular dementia
- damaged areas of brain tissue secondary to reduced cerebral blood flow, due to vessels having blood clots or fatty deposits (cerebral microinfarcts)
- more prevalent among people who are at risk for strokes, especially those with longstanding high BP and DM. It can occur together with Alzheimer’s disease
Parkinson’s disease
- deficiency of dopamine in the striatum due to degeneration of the neurons in the substantia Nigra
- classic symptoms include tremor at rest, rigidity, Akinesia, and postural instability (TRAP). Slowness of moments if often the first sign of Parkinson’s disease, along with cogwheel rigidity and pill rolling tremor.
- treatment: levodopa, bromocriptine, carbidopa, and sinemet
symptom of Parkinson’s
TRAP
- tremor at rest
- rigidity
- akinesia
- postural instability
Epidural hematoma
After a closed head trauma like a fall or blow to the head, blood collects between the skull and the dura. The middle meningeal artery is often affected due to herniation of the temporal region of the skull. In general, the injury has three phases: an initial loss of consciousness, a period of lucidity, and another loss of consiouscness
Subdural hematoma
Venous blood collects netween the dura and the arachnoid space. Patients may or may not experience symptoms depending in the severity of the causative trauma. In the elderly, this si commonly seen from a minor head injury, epically if on Coumadin. Symptoms vary, though altered mental state and HA are common
Symptoms to watch with any head trauam
Changes in pupillary size (a blow pupil may signal impending uncal herniation)
Nausea or vomiting
Loss of consciousness
Horners Syndrome
Lesion in the sympathetic pathway from the hypothalamus to the eye. Classified as preganglionic central, preganglionic, or postganglionic
Preganglionic central horners lesions
Between the hypothalamus and the cilispinal center of Budge (C8-T2)
-CVA, demyelinating disease, cluster HA, tumor, neck truama)
Preganglionic horners leision
Between the cilispinal center of budge and the SCG
-pancoast tumor, thyroid mass, neck trauma, history of thyroid or neck surgery
Postganglionic lesion of horners syndrome
Between the SGC and the iris dilator muscle (carotid artery dissesction, ICA aneurysm, cavernous sinus fistula, cavernous sinus syndrome, head trauma, cluster HA)
Neck trauma is most likely to cause horners in what lcaotion
Preganglionic central
Pancoast tumor is most likely to cause horners syndrome where
Preganglionic
Carotid artery dissection and ICA aneurysm is most likely to cause horners syndrome where
Postganglionic
Signs and symptoms of horners syndrome
Ptosis, miosis, anhydrisis
Patients with carotid artery diessction can also have neck and shoulder pain
Anhydrosis and horners syndrome
Most prominent in preganglionic central or pregnagnlion lesions. A postgagnlionic lesion will only result in anhydrosis of the ipsilateral forehead as most sweat fibers diverge away from the sympathetic fibers near the SCG
Congenital horners
Less than 5% of cases and maybe due to birth truama causing a brachial plexus injury or thoracic or cervical neuroblastomas (life threatening tumor). Infants will present with heterochromia
Bell’s palsy
- idiopathic condition Dx by excision after factoring out other causes if CN VII palsy
- sudden deficit on the lower motor neuron in CN VII, causing facial muscle paralysis on the entire side of the face with ectropion and exposure keratopathy
- treatment: patching or closing of the eyelid to decreased exposure K and corneal dryness. Short course of oral steroids or antivirals has been shown to increase the probability and speed of recovery, although 71% will have complete recovery in one year without treatment
Type 1 DM (juvenile DM)
- 10-20% of diabetic patients
- Younger
- Polyuria, polydipsia, and polyphagia (and weight loss)
- Most are immune mediated and have positive HLA association
- caused by a complete lack of insulin production due to beta cell destruction which can be triggered by environmental insult, virus, or genetics. Treatment includes insulin injections, and careful glucose and diet monitoring
- complications: diabetic ketoacidosis, microangiopathy (retinopathy, nephropahy, neuropathy), cataracts and glaucoma
Type II DM
Insulin resistance or abnornal beta cell secretion of insulin. Obesity is associated with 80% of cases of type II diabetes, and most patients are over the age of 40. Most patients re asymptaomtic at the time of dx. Treatment includes diet, exercise, oral glycemic agents, and occasionally insulin
Diabetes lab tests
FGL (should be les than 126mg/dL)
HgA1c (should be less than 7%)
Which is more common, type I and II DM
II
Insulin therapy need: type I vs type II
Always needed in type I, not always in type II
Obesity and type I vs type II Dm
Type II
HLA associateions and DM I vs II
Type I has HLA DR 3 and 4 association
Genetic predisposition for type I vs type II DM
Stronger for type II
Islet cells in type I vs II DM
Severe B cell depletion in type I
Mild B cell depletion in type II
Ketoacidosis: type I vs type II
Much more common in type I
Glucose intolerance: type I vs type II
Severe in type I
Mild in type II
When should diabetics have eye exams
Type I within 5 years of diagnosis
Type II upon diagnosis
In both cases, yearly exams are indicated yields retinal findings indicate more frequent care. A significant increase in blood glucose is most likely to induce a myopic shift in the refractive error
Diabetes insipidus
Extreme thirst and polyuria resulting from lack of ADh or deficiency in renal repsosne to ADH. Treatment options consists of increased fluid intake, intranasal desmopressin (ADH analog), indomethacin, or HCTZ for the nephrogenic causes
Hypothyroidism
Abnormality that hinders the appropriate production of thyroid hormones
-cold intolerance, hypoactivity, weight gain, fatigue, lethargy, decreased appetite, constipation, weakness, myxedema, dry cool skin, coarse hair
Hashimotos
AI mediated condition that attacks the thyroid gland. This is the most common cause of hypothyroidism in iodine sufficiency areas of the world. Common ages are 45-65. Females mroe liekly
Hyperthyroidism
85% caused by Graves’ disease, an AI condition caused by TSH autoAb; this reuslts in constant thyroid stimulation and excessive production of T3 and T4
- middle aged pateitns, female
- 1% will have MG
- common complaints: heart palpitations, weight loss, heat intolerance, and/or hair loss
- testing reveals elevated T4 and decreased TSH
Main ocular side effects of graves
Proptosis and upper eyelid retraction
SLK can occur
Hyperparathyroidism
Primary Hyperparathyroidism caused by hypercalcemia. This disease is caused by over secretion of parathyroid hormone. Symptoms include bone pain, pathologic fractures, renal stones, constipation, mental changes, and fatigue. May also have band keratopathy. Treatment is parathyporiedcomy
Hypoparathyroidism
- most commonly seen following a parathyroidectomy. Symptoms include muscles cramps, tetany, irritability, capopedal spasms, convulsions, and mental retardation. Decreased serum calcium more prominent evidence
- ocular findings: cataracts and uveitits, blurry vision
Addison’s disease
-adrenocortical deficiency that is a result of AI atrophy of the adrenal glands. Symptoms include weakness, fatigue, weight loss, nausea and vomiting, diarrhea, abdominal pain, muscle and joint pains, and amenorrhea. Treatment includes replacement therapy with a combo of glucocorticoids and mineralocorticoids
Cushing’s syndrome
Hypercortisolism
-effects of excessive corticosteroids on the body. Most are a result of chronic, prescribed corticosteroid medications. Usually present with central obesity, mood face, buffalo hump. May also have osteoporosis, HTN, poor wound healing, hyperglycemia, and elevated serum cortisol levels
Pheochromocytoma
Tumor secreting excessive amounts of NE and Epi. Located on one or both adrenal glands or anywhere along the sympathetic nervous chain
- any age, usually 40-60 years old
- elevated BP, papilledema, severe HA, perspiration, heart palpitations, and anxiety with a sense of impending doom
- treatment is removing the tumor
- should be suspected in patients with HTN accompanied by HA (pain), palpitations, pallor, and perspiration
Acute renal failure
Abrupt decline in renal functions with a decrease in GFR. Blood tests measuring kidney function include createnine and BUN
-decreased GFR and increased createnine and BUN
Chronic renal failure
Kidneys fail to make urine and excrete nitrogenous wastes. The main causes are DM and HTN
Glomerular Disease
Inflammation of the glomerulus, two types
- Nephritic syndrome
- Nephrotic syndrome
Nephrotic syndrome
Inflamamtion of the glomerulus
-associated with HTN, edema, and active urine sediment with hematuria, blood casts, and proteinuria
Nephrotic syndrome
Glomerular inflammation
-greater than 3.5 grams of protein in the urine. Accompanied by hypoalbumineria, HLD, and edema
Post strep glomerulonephritis
- Renal infection caused by groupie A beta hemolytic streptococci (pyogenes)
- 7-21 days after respiratory or skin infection (5-15yo)
- abrupt onset of hematurira, edema, HTN, and RBC casts in the urine
Differnece betwee UTI and plynephritis
UTI is urethra to the bladder
Pyelonephritis is bladder to the kidneys
Pyelonephritis
Bacterial infection of the kidneys.
Dysuria, frequency, urgency fever, chills, flank pain, nausea/vomiting
Renal cell carcinoma
Usually asymptomatic, but if symptoms are present, they include the classic triad of flank pain, hematuria, and abdominal renal mass. The first diagnostic test is an abdominal US
Most common bacterial STD
Chlamydia
Chlamydia serotypes D-K
AIC
- chronic follicular conjunctivitis and papillae inferior
- some mucopurulent
DicK
Chlamydia serotypes A-C
Trachoma
- follicular and papillary conj
- artls line
- Herberts pits
Treatment of chlamydia
Azithromycin (1g single dose, makes people sick)
Doxy
Gonorrhea
STD that is caused by direct contact with neisseria gonorrhea. 85% of infected female patients are asymptomatic. The incubation period od 3-5 days following infection
Signs and symptoms of gonorrhea
Range from none, to vaginal discharge, pelvic pain, urethritis, and dysuria or urinary frequency. Males are most commonly asymptomatic or will hav burning upon urination
Ocular gonorrhea findings
Severe, hyperacute, purulent discharge
Treatment of gonorrhea
Single dose of ceftriaxone 250mg IM plus doxycycline 100mg p.o. BID x 7 days
HSV
Type I and II. Dx is confirmed by multinucleated giant cells on a Wright-Giemsa stain. Type I is a mucosal lesion on the lips. Type II is associated with STD and is found on the mucosa of the male and female sex organs. Both types can be found in the eye
- prodromal symtoms that include tingling, irritation, itching
- treatment=acyclovir or famcyclovir orally
Syphilis
STD caused by spirochete treponema pallidum
Primary phase of syphilis
Chancre (painless ulcer on gentitals)
Secondary phase of syphilis
Lesions involve the eye,kidney, mucous membranes, skin, CNS or liver
Tertiary phase of syphilis
Nervous system and ophthalmic lesions including argyll Robertson’s pupil
Ocular manifestations of syphilis that can occur at any stage
- Interstitial keratitis, retinitis, and retinal vasculitis
- Salt and pepper fundus and flame shaped hemorrhages
- Uveitis-less than 1% of uveitis cases are from this
- CN and optic neuropathies
- Acute multifocal chorioretinitis and vitritis (panuveitis)
- Argyll Robertson pupil
Why is syphilis called the great mimic
Because of its variable presentation. Regardless of uveitis presentation, syphilis should always be considered in the list of diff
What tests are used to Dx syphilis
RPR VDRL FTA-ABS TPPA MHA-TP
What indicates an active syphilis infection in the bloodstream
Positive RPR and VDRL
FTA-ABS and syphilis testing
Detmeins whether patient has active or latent disease. Will be positive for life if the patient has ever had syphilis
Past infection of syphilis will yield what lab results
+ FTA-ABS
- PRP/VDRL
Active infection of syphilis will reveal what lab results
+ FTA-ABS
+ PRP/VDRL
What are the new recommendations for lab testing for syphilis
EIA and MFI
-specific to treponema antigens and therefore result in less false positives.
Most tests screen for syphillis IgG Ab
If syphilis IgG is negative
Does not have syphilis
If syphilis IgG is positive
PRP is performed, start back at the top
- if the PRP is postive, the patient likely has an untreated active syphilis infection in the blood steam. No other testing is performed
- if PRP is negative, FTA-ABs or TPPA is performed. If they are negative, the positive IgG test result was likely a false positive and the patients does not have syphilis. If the FTA-ABS or TPPA are postive, the patients has a pssible active and early syphilis infection (<6 weeks) or the patient was previously treated for syphilis and no longer has an active infection
BPH
Males over age of 50
- increased urinary frequency, urgency, nocturia, a weak stream, hesitancy, and dribbling
- treatment includes conservative measures with A1 blockers (terazosin) which relaxes the prostate muscle. Surgical intervention is often necessary
Prostate cancer
2nd most common cause of cancer deaths in males
- risk: over age 50, positive fam Hx, AA males
- rectal exam reveals firm, rock-hard, non-tender localized area
- elevated PSA test may indicate prostate cancer
Signs and symptoms of pregnancy
Amenorrhea, nausea and vomiting, breast tenderness, and weight gain
Lab tests and pregnancy
Beta HCG levels
Trimesters of pregnancy
0-12 weeks
12-26 weeks
27-40 weeks
Common tests during pregnancy
Triple screen to detect Down syndrome, STD testing, US, diabetic screening, Rhogram injection for Rh negative women (28 weeks), and group B strep test
Spontaneous abortion
Less than 20 weeks
Still birth
After 20 weeks
Ectopic pregnancy
Pregnancy within the Fallopian tube or anywhere outside of the uterus
Pre-eclampsia
Triad of BP greater than 140, protein in the urine, and swelling in the lower legs
Can result in still birth and maternal death
Eclampsia
Preeclampsia + seizures
Can result in still birth and maternal death
Breast feeding
Oxytocin from the post pituitary allows milk ejection. Prolactin from the anteiror pituitary allows for milk production. Benefits include child mother bonding, and a transfer of immunoglobulins
Cervical cancer
Common but treatable cancer in women that begins as cervical dysplasia and progresses over time to malignancy
- risk: early sex, multiple sex partners, HPC
- diagnostic tests: Pap Smear
Most common female cancer
Breast
Ductal carcinoma
Starts in the tubes (ducts) that move milk from the breast to the nipple
What is the most common cancer that causes death in women
Lung
Lobular carcinoma
Starts in parts of the breast, called lobules that produce milk
What causes breast cancer
In many breast cancers, estrogen causes the breast cancer tumor to grow. This is called estoppel receptor positive cancer or ER-positive cancer. Risk factors include age, fam Hx of breast cancer, or defects in the BRCA1 and BRCA2 genes
Most common cancers in me n
Prostate
Lung
colon
Most common case of cancer death in men
Lung
Prostate
Colon
Neural tube defects
Spina bifida
Anencephaly
Spina bifida
Reduced folic acid supplementation
Anencephaly
100% fatal
Congenital heart disease: right to left shunt
Shunting of deoxy blood from the right atrium or ventricle to the left atrium of ventricle. Results in blue (cyanotic) babies because the oxygen deficit is immediately problematic. Example if tetralogy or Fallot.
Worse or the two
Congenital heart disease: left to right shunt
Oxygenated blood from the left heart to the right heart
-blue (cyanotic) babies after exertion (playing sports)
Less problematic
-VSD, ASD, PDA
Fetal alcohol syndrome
Number one cause of congenital malformations
Cerebral palsy
Generic term that includes non-progressive varied infections, toxins, and congenital malformations in infants. Signs and symptoms include mental slowness or retardation, impaired function of voluntary muscles, seizures, and speech and sensory defects
Rubella
Mothers transfer this to the fetus. Side effects are microphthalmia, glaucoma, cataracts.
Screened early in pregnancy
Microphthlamos
Small malformed globe
Congenital syphilis
Causes mucous membrane lesions on the fetus. If untreated, the baby can develop interstitial keratitis and CNS disorders. This is screened for early in pregnancy
Congenital toxoplasmosis
Can cause stillbirth
Most will develop brain or eye problems, including retinochoroiditis
Infant cataracts
Should raise suspicion for rubella or galactosemia
GERD
Movement of gastric juice from the stomach into the esophagus due to gastroesophageal junction incompetence; this results in heartburn. Risk factors include alcohol use; hiatal hernias, obesity, pregnancy, scleroderma, and smoking
Peptic ulcer disease
Affects 20% of adults and is due to helicobacter pylori, chronic NSAID use, food intolerance and smoking. Treatment includes H2 blockers, PPIs, and combination therapy (H2 blocker and PPI plus 2 abx) for H pylori. Diagnostic studies include an upper endoscopy and barium swallow studies.
Treatment for H pylori peptic ulcer disease
H blocker
PPI
2 Abx
Two main locations for ulcers
Duodenal ulcer
Gastric ucler
Duodenal ulcer
Symptoms include burning or hunger like pain, primarily in the epigastric area. Pain may occur or worsen when the stomach is empty and is worse in the middle of the night when acid secretion is greatest
Gastric ulcer
Main symptoms is pain soon after eating
Barrett’s esophagus
- normal esophageal lining is replaced by metaplastic columnar cells; occurs as a result of squamous changing to columnar cells
- diagnosed via upper ednoscopay with biopsy to confirm cell change
- can lead to esophageal adenocarcinoma, which requires surgical resection of the esophagus
- treatment includes long-term PPIs
What are the two main components of IBD
Crohn’s disease
Ulcerative colitis
Etiology of Crohn’s disease
Infectious
Etiology of Ulcerative colitis
AI
Location of Crohn’s disease
Any portion of the GI tract. Usually affects the terminal ileum, small intestine and colon. Rectal sparing. Has SKIP LESIONS
Location of ulcerative colitis
Colon
Continuous lesions
Rectal involvement
Morphology of Crohn’s disease
Transmural inflammation
Cobblestone mucosa, creeping fat, bowel wall thickening, ulcers and fistula
Morphology of ulcerative colitis
Mucosal inflammation
Friable pseudopolyps, crypt abscesses, colorectal carcinoma
Complications of Crohn’s disease
Strictures, fistula, perinatal disease, malabsorption, and nutritional depletion
Complications of ulcerative colitis
Severe stenosis, toxic megacolon, colorectal carcinoma
Ocular findings of Crohn’s disease
Non-granulomatous uveitis
1-10%
Ulcerative colitis ocular findings
Non-granulomatous uveitis
1-5%
Something to help remember things about Crohn’s disease
Think of a fat old crone skipping down a cobblestone road
Colon cancer
Their leading cancer in both males and females in terms of incidence and cancer mortality
- low fiber, high fat diets, exposure to toxins.
- age, fam Hx of colon or breast cancer, and FAP
- screening is colonoscopy
Wilson’s disease
- disease of the liver
- failure of copper to enter circulation in the form of ceruloplasmin. This leads to copper accumulation that is most concentrated in the liver, brain, and cornea (Kayser-Fleischer rings). Some manifestations include a sunflower cataract, cirrhosis of the liver, basal ganglia degeneration, and dementia
Drug used to treat Wilson’s disease
Penicillamine
- chelating agent that used to treat Wilson’s
- ocular side effects include ocular myasthenia, ocular pemphigoid, and optic neuritis
Alcoholic hepatitis
This causes swollen, necrotic liver due to alcohol consumption. Mallory bodies (hyaline), fatty changes, and sclerosis around the central vein are also present, with an increase in AST and ALT liver enzymes
ALT and AST enzymes
Enzymes contained within liver cells that are released into the blood in increased concentrations in cases of hepatitis, regardless of etiology
Viral inflammatory condition of the liver parenchyma that leads to necrosis
Hepatitis
Hepatitis A
Vaccine available
Spread via fecal oral route, limited infection
Hep B
Vaccine available (0, 1, 6mos), spread via blood, IV drugs, and sex. Active infection typically has brief course
Hep C
No vaccine
Spreads like hep B with the same affects. Chronic hepatitis develops in appx 80% of patietns with acute hep C infection
Hep D
Carried only with Hep B as a superinfection. This increases morbidity and mortality
D=Dwarf; only in presence of B
Hep E
No vaccine
Fecal oral
Which Heps are spread fecal oral
Vowels to the bowels
A and E
Which hepatitis has vaccines
A and B
Which hep is chronic
C
Cirrhosis
Presence of fibrosis with creation of excessive extracellular matrix. Normal blood flow through the liver is hindered and nutrient and metabolite enhance are thus reduces. The liver initally enlarges, firm nodules are formed, and then the liver shrinks. The most frequent causes are alcohol and viral hepatitis; however, drugs, toxins, CHF, biliary obstruction, and AI complexes may also play a role. Complications include portal HTN, GI hemorrhage, and liver failure; the latter frequently results in death
Jaundice
Bilirubin pathway is obstructed in the liver or bilirubin is hemolyxed in the blood and cannot be excreted in the urine due to some other pathology. The build up of urobilinogen and bilirubin in the systemic blood stream causes a yellowing of the skin and conjunctiva. This is commonly seen in cirrhosis, hemolytic disorders, or obstructive gallbladder disorders
Cholecystitis
Occurs both acutely and chronically as a result of inflammation of the gallbladder lining secondary to obstructive cholesterol stones, sludge, or infection. Most common in overweight females of childbearing age. Patients have pain in the RUQ and a postive Murphys sign. Treatment involves surgery to remove the gallbladder
Acute pancreatitis
Occurs secondary to alcohol abuse or gallstones in 70-80% of cases, other causes include trauma, drugs, HLD, infection, or tumor. Patients present with pain around the umbilicus with possible radiation to the back. Lab studies show an increase in serum amylase and lipase
Chronic pancreatitis
90% of cases are due to chronic alcohol abuse; most of these patients have a history of recurrent episodes of acute pancreatitis, periodic or contentious peri-umbilical pain radiates through to the back. Weight loss, steatorrhea, and DM are also common
Hearing loss
Can be caused by loss of conduction, sensory loss and/or neural deficits. Loss of condition is the most common corm of hearing loss and is usually a result of cerumen impaction or trauma. Sensory hearing loss is due to deterioration of the cochlea, while neural hearing loss is due to damage of CN8. Two clinical tests, the Weber test and Rinne test, are used to determine which is the culprit
What is the most common form of hearing loss
Loss of conduction
Otitis media
Characterized by pain in the ear. Caused by bacterial infection from strep pneumoniae and H influenzae. Treatment is amoxicillin or other gram + agents. Very common in infants and chidlren
Vertigo
Disease of the vestibular ear system causing dizziness and nystagmus. True vertigo is always associated with nystagmus. A condition that can cause vertigo is Meniere’s disease, which is assocaited with episodic and often severe vertigo, hearing loss, and tinnitus
Sinusitis
Inflammation of the paranasal sinuses due to viral, bacterial, or fungal infections. Patients may have anterior and or posterior mucopurulent drainage, nasal obstruction, facial pain, pressure and/or fullness, and decreased sense of smell
Allergic rhinitis
Characterized by symptoms of runny nose, sneezing, conjunctival hyperemia, and watery eyes. Treatment includes histamine receptor blockers, nasal sprays, and eye drops such as pataday for ophthalmic symptoms
Pharyngitis
Signs and symptoms include enlarged cervical nodes, sore throat, and fever. Occurs as a result of viral or bacterial agents. Group A strep infections need to be treated with ABx such as penicillin or other gram + agents
Acute bacterial sialadenitis
Commonly located in the parotid or submandibular gland-swelling and pain of the gland will be present. Often caused by S aureus and treated with Abx. IV nafcilin is often used
Salivary gland tumors
80% of these will involve the parotid gland and only 50-60% will be benign. These present as an asymptomatic mass in the gland and can extend deep into the facial nerve area. MRI and CT scans are used to diagnose the tumor. Treatment involves removal of the tumor
TMJ
Commonly a result of acute and chronic inflammation secondary to arthritis, dislocation, developmental anomalies, and other factors. Patietns will often complain of HA, facial pain, and jaw pain
Obstructive sleep apnea
Breathing disorder during sleep. Inadequate muscle tone of the tongue or airway dilator muscles resutls in obstruction of the upper airway which causes the patient to frequently stop breathing during sleep. OSA affected appx 3-7% of men and 2-5% of woman in the US. Appx 41% of people who are defined as obese based on BMI have OSA.
Signs and symptoms of obstructive sleep apnea
Disruptive snoring
Daytime sleepiness
Obesity (BMI 30 or greater)
Large neck circumference (>42cm in men)
Obstructive sleep apnea is assocaited with the development of
HTN, fatal and non fatal cardiovascular events, stroke, epilepsy, and DM. Ocular manifestations include floppy eyelid syndrome, NTG, and NAION
COPD
Obstruction of air flow, causing air to be trapped in the lungs. The leading cause of COPD is smoking.
Categories of COPD
Emphysema
Chronic bronchitis
Emphysema
Pink puffers
Enlargement of air spaces and decrease in recoiling because of destruction of the alveolar walls. Shortness of breath, decreased breath sounds, and tachycardia
Chronic bronchitis
Blue bloaters
Productive cough for 3 consecutive months in 2 or more years due to hyeortrophy of mucous secreting glands in the bronchioles. Common symptoms include cyanosis of the fingers and toes and wheezing/crackling of the lungs
Asthma
- Acute reversible bronchocosntriction: triggered by infection, allergens, stress. Symptoms include coughing, wheezing, dyspnea, and tachycardia. Treated with bronchodilators
- Chronic inflammation of the airways. Treated with inhaled or oral steroids
Pneumoconiosis
Restrictive lung disease secondary to occupational inhalation of dust. The disease i given different names depending on the particular inhaled such as: Coal workers pneumoconiosis, Asbestosis, silicosis, and berylliosis
- restrictive lung disease can cause shortness of breath, and decreased lung volumes and lung compliance
- chest X-ray will show diffuse infiltrates with a ground glass appearance
Remember to always ask about underlying lung disease before treating your glaucoma patietns with
BBclokers
TB
Caused by airborne droplets of mycobacterium TB. The primary symptoms of TB include fever, cough, night sweats. All patients thought to have TB need to have a chest X ray
What is the number one symptom of TB
Night sweats
Latent TB
Positive test results but negative chest X ray
Active TB
Positive test reuslts and positive chest X ray
Treatment of TB
RIPE
- rifampin
- isoniazid
- pyrazinamide
- ethambutol
Ocular findgins of TB
- conjunctival granulomas
- phlyctenuels
- episcleritis
- scleritis
- keratitis
- BILATERAL CHRONIC GRANULOMATOUS UVEITIS
- CME ( determines acuity in most patietns)
- unilateral optic disc edema or papilledema
What are the two main causes of bilateral anterior chronic granulomatous uveitis
TB and sarcoidosis
PPD test
15mm or more=postive test in healthy person with normal immune system
10mm or more=postive test in healthcare working
5mm or more-positive in patients who are immunocompromised
Quantiferon TB gold in tube test (QFT-GIT)
Aid in the diagnosis of latent and active TB
- positive result (TB infection likely): Nil<8, TB response >0.35IU/mL and >25% of Nil, and any mitogen resposne
- negative results (TB not likely): Nil < 8, TB response <0.25 IU/mL or <25% Nil, and mitogen respsone >0.5
- indeterminate (uncertain likelihood of TB): Nil <8, TB response <0.25 or <25% of Nil, and mitogen respsone <0.5, OR Nil >8,any TB response, and any mitogen respsone
Routine testing with and IGRA + TB skin test is generally not recommended except for special cirmustances. Note that QFT-GIT does NOT differentiated between active and latent TB
Bacterial pneumonia
Infection of the lungs usually caused by bacteria or viruses. The most common cause is streptococcus pneuamoniae. Symptoms include productive cough, shortness of breath and fever. A less common form of pneumonia is “walking pneumonia” caused by agents such as mycoplasma pneumoniae
Influenza
Acute respiratory illness caused by influnza A or B viruses. Symptoms include fever, cough, rhinitis, HA, and myalgias
Lung cancer
Main cause of cancer deaths in both men and women. Adenocarcinoma of the lungs it he smot common lung cancer. Cigarette smoking is the leading caused, although secondary hand smoke can also caused lung cancer.
Tumor of the apex of the lung
Pancost tumor
Horners syndrome can ensue
Neoplasia
Cancers reuslt from a host of environmental and genetic factors that damage normal cells. The damaged cells are mutated and give rise to new populations of mutated cells. Typically cellular defense mechanisms such as apoptosis and suppressor genes are often inactivated in premalignant cells and are therefore not useful in halting abnormal cell growth. Further more, growth promoting oncogenes facilitate and encourage cancer mutation and growth
Behavior of neoplasms
Benign
Borderline
Malignant
Degree of differentiation of neoplasms
Well differentiated or poor differentiated
Embryological origin of neoplasms
Epithelial (adenocarincoma, SCC), lymphoproliferative (leukemia, lymphoma), or mesenchyamal (Sarcoma)
Gross appearance of neoplasms
Well circumscribed or infiltrative
Benign tumors
Usually slow growing and well circumscribed
- adenomas: benign epithelial Tumors arising in glandular patterns
- cystadenomas: adenomas producing large cystic masses seen in the fat and ovaries
- papilloaoms: epithelial tumors that form microscopic or macroscopic finger like projections
- polyps: tumor that extends from the mucosa into the lumen of a hollow organ
Malignant tumors
Often aggressive with invasion of adjacent structures and have metastatic potential
Metastatic
Cancer that shifts from its origins to another body site
Carcinomas
Arises from epithelial cells (SCC)
Sarcomas
Arise from mesechymasl (connective) tissues
Bones, vessels, heart
Rhabdomyosarcoma
CT cancer that causes bone destruction; it is the most common primary orbital malignancy
Dysplasia
Abnormal growth of epithelial cells caused by a disruption in cell maturation
Low grade dysplasia
Concerned the earliest form of a pre cancerous lesion that is recognizable in a biopsy by a pathologist
High grade dysplasia
Carcinoma in situ (CIN). Transformation of cancer is high, but treatment often still effective
Invasive carincoma (cancer)
Final step in the process of dysplasia. Growth penetrates the epithelial BM to invade the tissue
Metaplasia
Refers to change from one mature cell type to another as an adaptive response from chronic irritation or a pathogen or carincogen. Treatment aims to reverse cell damage. If left alone, dysplasia and center can result. SCC, the 2nd most common form of skin cancer, results from metaplasia
Barrett’s esophagus is also metaplasia
Neoplasia
Abnormal, disorganized “new growth” in a tissue OT organ that leads to a formation of a growth or mass (neoplasm). Can be benign or malignant. Melanocytic nevi are among the most common benign neoplasms
Oncogenes
Protein encoding gene that has the potential to cause cancer. Activated oncogenes allows mutated cells, which normally die secondary to apoptosis, to survive and proliferate into a cancer. Multiple oncogenes, such as the “ras” oncogene have been found to play a role in specific cancers
Tumor suppressor genes
Either have a repressive effect on the regulation of cell cycle and/or promote apoptosis. They prevent DNA damage and regulate cellular activities. Loss of tumor suppressor genes, such as the BRCA gene in breast cancer, can lead to cancer
Anorexia nervosa
Distorted view of their body image and an overwhelming fear of being fat. They have decreased body weight (less than 85% of ideal body weight), and absence of at least three menstrual cycles. It affects females more than males
Bulimia nervosa
Episodes of binge eating two times a week for at least 3 months. They compensate for the binge eating by vomitting, over exercising, or by utilizing laxatives
Kwashiorkor
Protein malnutrion
Very swollen belly
Marasmus
Decreased protein and decreased calories
Wasting appearance
Alcoholism
Addiction to alcohol. Withdrawals cause tremo, tachycardia, HTN, nausea, seizures, delirium tremens, and hallucinations. More prone to gallstones, alcoholic hepatitis, and cirrhosis of the liver
Alcoholic cirrhosis
Due to long term alcohol abuse, causing micronodular cirrhosis. Presenting symptoms include jaundice, hypoalbuminemia, coagulation factor deficiencies, and portal HTN
Wernicke-Korsakoff Syndrome
Assoacited with vit B1 (thiamine) deficiency
What can alsoholsim lead to
Toxic optic neuropathy that leads to bitemporal optic nerve pallor
Macular fibers
For a patient to be diagnosed with mental illness and behavior disroders
Physical disease and drug abuse must be elimated
Lab work and brain scans
Diagnosed used DSM-IV
Major depression
30% of patients have this
-thought to be caused by inappropriate NT involvement, metabolic abnormalities, or psychological factors. There is also a strong genetic predispostion
Anxiety
More in women
Early adulthood
Treatment: benzodiazepines, antidepressants, and counseling
Schizophrenia
Increased dopamine in the Mesolithic region
Massive disruption inf behavior, mood, and thinking for at least 6 months
Treatment=antipsychotic meds
Positive schizo
Delusions
Thought disorders
Inappropriate affect
Increase in motor functions
Negative Shizo
Lack of speech and thought
Loss of emotional response
No effect
Social isolation
Bipolar disorder
Severe depression alternating with periods of mania
- treatment is the same as severe depression
- manic episodes are elevated mood, decreased need for sleep, flighty thought processes, decreased reonsing skills, increase activity, and aggressive behavior. Mania is often followed by periods of severe depression
Suicide
White middle aged and elderly men most common
Hospitalization
Treatment of depression
Substance abuse
Triad of compulsive drug use which includes a psychological dependence, physiological dependence, and drug tolerance
