LECTURE 2 Flashcards
IgA Nephropathy aka
Berger’s Disease
What is the MC disease worldwide?
IgA Nephropathy (aka Berger’s Disease)
What is the Most common cause of gross hematuria (brown urine)?
IgA Nephropathy. (Aka Berger’s Disease)
When does IgA nephropathy aka Berger’s Disease develop?
Within 1-2 days of an acute upper respiratory infection (common cold) OR
Acute gastrointestinal/urinary infection
Urination lasts for a while and then _______ in IgA Nephropathy (Berger’s)
Stops spontaneously
What is IgA nephropathy (Berger’s) characterized by?
By development of loin pain (low back/butt)
What is Increased in IgA Nephropathy/ Berger’s?
Increased concentration of IgA immunoglobulin in the blood circulation as well as increased concentration of IgA
Deposition of IgA complexes into mesangium** QUIZ
What is the role of IgA, and w
Protects mucus membranes during infection
What type of rash is seen with IgA nephropathy / Berger’s?
Henoch-Schonlein Purpura ** QUIZ
What is the prognosis of IgA Nephropathy aka Berger’s?
Different/varietal 10-20% will have kidney pathology -> kidney failure
Buerger’s disease aka
Thromboangiitis Obliterans
What is Buerger’s / Thromboangiitis Obliterans characterized by?
Acute/Chronic thrombizing inflammation of the middle small arteries (VASCULITIS)
ALSO with inflammation of VEINS (PHLEBITIS) and NERVES (vasonevorum)
1 arteries
2 veins
3 nerves
What is the biggest distinguishing factor about Buerger’s Disease aka Thromboangiitis Obliterans?
Only disease in the cardiovascular system with VASCULITIS involving arteries and veins **
Is Buerger’s associated with kidney pathology?
NO, it is a CARDIOVASCULAR system disease
What is Buerger’s Disease associated with?
Young < 35 yo smoking people, typically in younger females with heavy tobacco use
What does smoking do in Buerger’s disease?
Initiates and maintains the disease (quit smoking and the disease goes away)
What are the 3 manifestations of Buerger’s Disease?
1) . Instep Claudication
2) . Raynauds Phenomenon
3) . Thrombophlebitis
What is Instep Claudication?
Periodic spasm of artery associated with exertion
- Patient experiences severe pain in the calf due to ischemia or neuropathy -> as you use the muscle, blood supply is needed, increased use cannot be supplied with enough blood/oxygen
What is Raynaud’s Phenomenon?
Vascular disease of the fingers -> Vasospastic reaction resulting in arteries, veins (WHITE, BLUE, RED)
What is Thrombophelbitis?
Inflammation of veins -> Formation of thrombytes -> can kill patient
If a patient continues to smoke with Buerger’s disease what will eventually happen?
They will develop gangrene due to narrowing of the arteries (usually vertebral, radial, and ulnar arteries)
- Amputation is common
What is the best definition of Buerger’s Disease?
Nonarterosclerotic , segmental, inflammatory disease affecting small and medium sized arteries and veins of upper and lower extremities
Where does Buerger’s Disease occur?
Japan, Israel, and India
- Predisposing factors are still unknown
Hereditary Glomerulonephritis aka
Alport Syndrome
What is Hereditary GMN aka Alport Syndrome?
Abnormal production of type 4 collagen (poor quality collagen/not stable)
Hereditary GMN (Alport) develops _____
In boy with impairment of type 4 collagen production (collagen in glands in basement membrane of capillaries; and early childhood (5-20 yo) first decade of life
What are the symptoms of Hereditary GMN aka Alport Syndrome?
- Nerve deafness
- Lens dislocation (lens made of collagen type 4)
- Posterior cataracts
- Corneal dystrophy (deaf and blind)
- Usually become chronic and develop renal failure (20-25 yo) death by 20 yo
What is Nephrotic Syndrome clinically manifested by?
Dramatically increased permeability of proteins (albumin molecular cites) in glomeruli
How does the damage happen in Nephrotic Syndrome?
Damage to the cell membrane allowing proteins through, NOT water (due to the protein leaving, it takes water with it)
What are the 4 main symptoms of Nephrotic Syndrome?
1) . Massive (heavy) proteinuria (>3.5g/24hr)
2) . Hypoalbuminemia (<3.0 g/dL)
3) . Generalized edema
4) . Hyperlipidemia and lipiduria
In Nephrotic syndrome, what is Massive (heavy) proteinuria characterized by?
Proteins in the urine, there is a huge loss of proteins during filtration of blood in the kidney
- Presence of protein in the urine indicates a decrease of albumin from the circulation
What is Hypoalbuminemia characterized by?
Inversion of albumin to globulin ratio (<1) leaving less proteins in the body
What is “Electrophoresis” associated with?
Hypoalbuminemia in Nephrotic Syndrome
What is Electrophoresis?
Albumin peak normally much higher than all others, peak decreases in size blood circulation, contains proteins
What is the normal protein ratio in the blood?
Albumin 55%
Globulin 40-45%
Fibrinogen 5-7%
What is the ratio that makes “Hypoalbuminemia?”
Normal - Divide (albumin/globulin) = > 1
- Abnormal: When you lose albumin in the blood, ratio switches to <1 (inversion)
What happens if you do not have enough albumin in the tissue?
Albumins maintain oncotic pressure, if you don’t have enough -> the tissues will have a high oncotic pressure and pull fluid from the vessels into tissues
What does Hypoalbuminemia lead to?
Generalized edema
What is generalized edema characterized by?
Swelling of the face, particularly under the eyes:
- Adrenal cortex is stimulated to increase aldosterone secretion, which causes increased absorption and pulling of fluid from the primary urine (anterior/medial portion of face, ankle low back, low abdomen (abdominal, pleural and heart cavity)
If there is escape of albumin in the blood ________
Fluid will exit the vessels into the tissue (osmosis)
What are the signs of Hypoalbuminemia?
- Ascites
- Pleural Effusion
- Pericardial Effusion
- Muehrcke’s lines aka M’s nails aka leukonychia striata
What is Ascites?
Accumulation of transudate in the abdominal cavity
What is Pleural effusion?
Accumulation of transudate fluid in the pleural cavity
What is Pericardial Effusion?
Accumulation of transudate fluid in the pericardial cavity
What are Muehrcke’s lines aka?
Muehrcke’s nails aka Leukonychia striata
What are Muehrcke’s lines?
An appearance of horizontal lines beneath the nail and specific swelling of the nail bed
- See lines in the nails parallel to lunula
Where would you see Muehrcke’s lines?
With Nephrotic syndrome, especially Hypoalbuminemia
In generalized edema during Nephrotic system, is transudate or exudate escaped?
Transudate
What is Transudate?
Perfusion of the fluid portion of the blood which develops in edema (formation does not allow for escape of WBC’s, very little protein in the transudate fluid in tissues)
What is (4) Hyperlipidemia and Lipiduria?
Albumin is produced in the liver (to meet the demand) as glycoproteins OR lipoprotein to increase the concentration of lipids in the blood -> loss of protein component
What is the cause of (4) Hyperlipidemia and Lipiduria?
- Lipid goes through the damaged glomerular membrane
- Casts are formed with fat globules
- It manifests and accumulates fluid in the pleural and abdominal cavity
What will you see with Diabetic Nephropathy?
1) Glomerular lesions
2) Renal Vascular lesions
3) Pyelonephritis often with Necrotizing Papillitis
What are Glomerular lesions associated with Diabetic Neuropathy?
- Glomerular basement membrane thickening
- Diffuse glomerulosclerosis (diffuse increase of mesangial cell proliferation)
- Nodular Glomerulosclerosis (characterized by Kimmelstiel-Wilson lesions - ball like deposits of a laminar matrix within the masangium
What are Renal Vascular lesions associated with Diabetic Nephropathy?
- Hyaline arteriosclerosis of both afferent and efferent arterioles
- Atherosclerosis
What Primary Glomerular Diseases are associated with Nephrotic Syndrome?
1) Minimal change disease (lipoid Nephrosis) - droplets
2) Membranous GMN
3) Focal Glomerulosclerosis**** Major cause of nephrotic disease that leads to chronic GMN
4) . Membranoproliferative Glomerulonephritis (GMN)
What are the Systemic Diseases associated with Glomerulonephritis?
1) . Diabetes Mellitus
2) . Amyloidosis
3) . SLE systemic lupus erthematosus
What is Amyloidosis associated with Nephrotic syndrome?
Deposition of amyloid
- Amyloid: The generic term for a variety of proteins does material that are abnormally deposited in tissue interstitium in a spectrum of clinical disorders
What are the 2 types of Amyloidosis?
1) Amyloid AA
2) . Amyloid BB
What does Amyloidosis lead to?
Amyloidosis is Idiopathic, incurable and leads to pressure atrophy
What happens to functional tissue during Amyloidosis?
Normal functional tissue, in the KIDNEYS, BRAIN, SKIN, AND LIVER* is destroyed by intercellular deposition of amyloid
What normally accompanies Amyloidosis?
Patients don’t live more than 2 years, and it is usually accompanied by AS and other autoimmune diseases
What is Chronic GMN?
Typically the end stage of GMN that has slow progression - glomeruli are obliterated and replaced by CT - later the kidney will decrease in size and become granulated
What does Chronic GMN lead to?
Slow inflammation which leads to loss of renal tissue and chronic renal failure
What happens to the kidney in Chronic GMN?
The kidney becomes CONTRACTED* due to CT (becomes small, half of normal size, yellow, pale, and coarsely granular)
What are the Symptoms of Chronic GMN in the GI system?
Waste products remain in the body, waste products being excreted = thin layer of frost
What are the symptoms of Chronic Renal failure in the Circulatory system?
Fibrous structures like pericardium pericardium and pleura attempt to filtrate the products and cause an accumulation of crystals of toxic products and can cause the manifestation of bruits when auscultation
= FUNERAL BRUIT ***
- Deposition of crystals into the pericardial cavity
What happens to the Nervous System during Chronic Renal Failure?
Psychosis, paranoia hallucinations
When would you develop Hematopoiesis?
With Chronic Renal Failure
- non production of erythropoietin by the kidney (smell of urine in the mouth)
What is the treatment for Chronic Renal failure?
Kidney Replacement: Patient dies without it
- HEMODIALYSIS** : Used to prolong life when kidney isn’t functioning, lasts 8 hrs every other day
What is the #1 cause of death in the US?
Cardiovascular problems
Rheumatic fever aka
Migratory Arthritis
Where is Rheumatic fever common in?
In the past that affected young people (teens)
What does Rheumatic fever effect?
Joints, heart, Associated with strep throat** -> acute tonsillitis -> follicular strep throat beginning not cause
What is the beginning of the of follicular strep throat?
Beta hemolytic streptococcus group A (pyogenesis)
What is Molecular Mimicry associated with Rheumatic Fever?
After repeated strep throat episodes , antibodies created to fight the strep throat infection attach to the heart and destroy the tissue
What is the pathogenesis of Rheumatic fever?
Tonsils (too cold water)
Infection: Beta hemolytic Step group A (same as GMN)
Repeated strep throat episodes
What is the treatment for Rheumatic Fever?
Lacunar Tonsilits: Gargle with salt water
Follicular Tonsillitis: Must take antibiotics or tonsillectomy (but labile tissue, so might grow back)
Strep Throat aka
Acute Tonsillitis
What is strep throat aka acute tonsillitis ?
Common disease behind common cold
- Inflammation of the pharynx and acute tonsillitis of the palatine tonsils (formation of pus) which eventually leads to the development of rheumatic fever
Define Molecular Mimicry:
Pyogenes infection causes immune responses characterized by formation of antibodies against the antigens of the streptococcal wall - tissue of the heart has the same configuration as the streptococcal wall antigen — antibodies bind to both the S. Pyogenes and heart tissue - heart DESTROYED in phagocytic process
Tonsillitis will eventually lead to ____
Development of RF - If you have relapse twice a year, must be removed -> removing the tonsils does not mean you will have more infections in the future
- If not all removed = can regenerate
If tonsils are not completely removed what do they become?
They can regenerate and become hypertrophic or hyper plastic
WHat are the major provocative factors that lead to tonsillitis ?
Sudden changes in temperature: Exposure to cold (drinking cold water after overheated)
What are the 2 types of tonsillitis?
1) Lacunar
2) FOllicular
Define Lacunar Tonsillitis:
With regular inflammation of the tonsils, it becomes structure allows for pockets and spaces to be filled with bacteria
- Grows in normal temp, pain is caused by swelling, infection in cracks, and forms pus (looks white)
How do you remove white pus in Lacunar Tonsilitis?
Gargle salt water
What is FOllicular Tonsillitis ?
The glands within the tonsils are infected, pus filled spots (cannot be removed mechanically) — inflammation makes it difficult to swallow (fever > 105) very dangerous
How do you treat FOllicular Tonsillitis?
Antibiotic treatment is necessary, if don’t treat properly (with antibiotics) can develop chronic tonsillitis or repetition of tonsillitis -> GMN or RF
What is Retrotonsillar abscess?
Development of suppurative inflammation (formation of pus behind the tonsils) which can go DOWN between layers of mediastinum
What can Retrotonsillar abscess lead to?
Very dangerous (can lead to death) - if you see bulging of the posterior wall of the pharynx , this requires immediate action (cut off and pus drained)
