Pulm answers

Normal lung:

Fluid accumulation within the pleural space = _______________________ (effusion)

Transudates are caused by __________________________________ (starling forces) and 3 examples of causes of transudates in the pleural space are:

1.

2.

3.

CHF/cirrhosis/Nephrotic syndrome

If you collect fluid from the pleural space and it turns out to be an exudate what would you expect the fluid to be composed of? Name 3 causes of exudates:

1.

2.

3.

increase cellularity, protein concentration, LDH, cholesterol

infection, malignancy, rheumatologic

Obstructive lung diease

How is the severity of COPD assessed?

FEV1/FVC ratio and FEV1 decrease

What major diseases are within the category of COPD?

Chronic bronchitis, emphysea, bronchiectasis, asthma

All of the following are associated with COPD mortality except:

·         R-sided heart failure

·         Pneumothoraces

·         Infectious exacerbations

·         Respiratory alkalosis

Respiratory acidosis (not alkalosis) is associated with morbidity/mortality. Due to the decreased ability to exhale CO2 there is a build up in the body (H+ HCO2---H20 + CO2) which causes an accumulation of acid (left shift).

Why do people with emphysema have difficulty breathing?

decreased surface area for GE: airway wall destruction leads to enlargement of airspaces and a simplification of lung parenchyma

Which parts of the lung are involved in centriacinar emphysema?

Initially: proximal acinus, resp bronchiole (later may extend to alveolus)

·         Typically found in upper lobes of spokers

·         Lypmphoplasmcytic inflammatory infiltrate

Panlobulaar emphysema is associated with a deficiency in _________. The inheritance pattern is _____.  It is typically found in (upper/lower/all lobes). Patients may have associated illness in (name organ)_________.

·         Alpha-1-antitrypsin deficiency leads to low protease inhibitor

·         AR

·         Typically involves lower lobe (note: centriacinar involves upper)

·         Associated with hepatitis/cirrhosis of the liver

Paraseptal emphysema predisposes a patient to what complication (and why).

·         Paraseptal = distal destruction enlargement of alveoli

·         Usually upper lobe subpleural

·         Damage to this area predisposes to spontaneous pneumothorax

Why is smoking associated with emphysema?

·         Smoking increased PMN/macrophages activity  and increases  free radicalsà inhibit anti-protease activity

Clinically – how is chronic bronchitis defined?

·         Persistent cough + sputum that continues for 3+ months/yr over a 2+yr span

What is the pathophysiology of chronic bronchitis? Is there alveolar involvement as seen in emphysema

·         Proximal airway involvement w/o parenchymal injury (loss of GE) as seen in emphysema

·         Particulates/noxious chemicals inflame airways (many with mucous metaplasia) leading to mucopurulent exudates

What measurement is used to determine if there is increased mucus gland components seen with a histologic specimen of chronic bronchitis?

·         Reid index

·         Normal < 0.4

·         In chronic bronchitis can see 0.8

Why are people with chronic bronchitis prone to infections?

Mucus plugs create stasis – whenever there is stasis bacterial infections are more likely to occur (harder for immune cells to get to them, and they are not being cleared from the body as they normally would so they build up)

What changes to the airways are seen with bronchial asthma?

·         Hyper-responsiveness

·         Goblet cell metaplasia

·         Mucous gland hyperplasia

·         Airway muscular hypertrophy

What is the difference beyween extrinsic and intrinsic asthma?

·         Extrinsic (T1 HST to a defined allergen)

·         Intrinsic: reactive non-immune condition

If a patient is suspected of having type 1 asthma what type of testing maybe done to determine which allergen is responsible?

·         Skin testing to identify “wheal-and-flare- rxn

Bronchoconstriction is mediated by _______

·         Leukotrienes

Abnormally dilated airway due to rolonged detructive inflammation/infection og the airway + supporting structures is known as:

Bronchiectasis

This disorder is commonly seen in patients with what other diseases/disorders?

·         CF

·         Bronchial obstruction

·         Immunodeficiency disease

·         Necrotizing bronchoPNA

·         **damaged airways are permanent and result from congenital/hereditary condition—these enlarged airways are at risk for mucosal plugging and further destruction

Restrictive lung disease:

Occupational/environmental disease in which an inhalent leads to interstitial fibrosis is called__________________

Pneumoconiosis

If a patient presented with an intersitial lung disease what would you expect to see on PFTs?

Restrictive pattern: inflammatory and fibrosing processes lead to collagen deposition and thus scarring which leads to a shrunken hardened lung.

FEV1/FVC >80 but a decrease in both values

What causes the scarring seen in interstitial fibrosis?

·         Damage to alveoli/ducts causes an accumulation of inflammatory cells which secrete cytokines and inflammatory mediators which damage the alveolar epithelium and induce collagen deposition/fibrinogenesis

·         Interstitium expands from the vast cellularity

Match AIP, UIP, NSIP, RBILD, DIP for each of the following

·         Patchy interstitial fibrosis (UIP)

·         Best Rx is to quit smoking + steroids (RBILD)

·         AKA hamman-rich syndrome (AIP)

·         Homogenous chronic interstitial PNA (NSIP)

·         Which has the best outcome (NSIP)

·         Diffuse interstitial infiltrates on xray leading to pulmonary fibrosis, systic changes, diffuse scarring (AIP)

·         Respiratory failure occurs over 2-5 years and ultimately progresses to honeycomb lung (UIP)

·         Involvement of alveolar units with desquamted alveolar pneumocytes and macrophages (DIP)

All of the following are true concerning sarcoidosis except:

a.       Non-caseating granulomas can be found in different organ systems including the heart, lung, brain, skin

b.      Pulmonary involvement starts with the lung then progresses to mediastinal and hilar LAD

c.       CD4 t cells activate macrophages to become epithelioid histioytes (T4 HST)

d.      Increased CD4:CD8 ratio

b. LAD proceeds lung involvement

match the following: silicosis, asbestosis, berylliosis

·         Birefringent (silica)

·         May present with pleural effusion/plaques (asbestos)

·         Interstitial granulomatous reaction (berylliosis – presents like sarcoidosis)

·         Interstitial fibrosis (asbestosis)

·         Sclerotic nodule (silicosis)

·         Commonly seen in brake mechanics (asbestosis)

Farmers lung is an example of what type of  pneumonitis?

HST – type ¾

Vascular lung disease:

Transudates result from _______________________ starling forces

3 causes of transudates are _____________________ _____________________ ________________

CHF/nephritic/cirrhosis

Describe what would be found in a chest tube that drained an exudate? Elevated cellularity, proteins, cholesterol, LDH

3 causes: ____________ _________________ ______________ cancer, infection, rheumatoid

What causes respiratory failure in ARDS (pathogenesis)?

Acute injury to alveolar/capillary unit leads to increased permeability and exudation of plasma protein/inflammatory cells which form an exudate  hyaline fibrous membrane

Can you treat ARDS with supplemental O2?

No – due to vascular shunting, V/Q mismatch, and increased lung stiffness

Pulmonary infections:

What 3 patterns of PNA may be seen on x-ray? Describe each:

·         Broncho-pneumonia – most common

o   Patchy consilidation

·         Lobar

o   Bacterial infection of confluent area or entire lobe

o   Most common cause = strep pneumo

·         Interstital

o   Atypicals

What are the bodies defense mechanisms for the following:

·         >several microns: trapped in nasal and tracheobronchial clearance

·         approx micron: phagocytosis by macrophages in respiratory bronchiole/alveola duct pathways

·         <micron: remain in air current = exhale

Name 3+ complications of PNA

·         lung abscess

·         empyema: pus in pleural sace

·         bacteremia

·         sepsis

·         fibrinoblastic organzation w/ scarring

Which types of PNA are being described below?

·         phagocytosing macrophages + giant cells: Aspiration PNA

·         patchy, neutrophilic fibrinopurulent exudate in distal airways/alveoli: BronchoPNA

·         goes through stages (congestion à red hepatization à grey hepatization à resolution): lobar PNA

List some of the unique pulmonary infections AIDS patients are suseptable to due to their low T cell count:

·         PCP

·         CMV

·         Fungal: histoplasmosis, cociodiomycosis, cryptococcosis

All of the following about TB is true except

·         Caseating granulomas

·         Ghon lesion is a localized infection that drains to hilar LN (Ghon complex)

·         Tuberculoma is an upper lobe cavitation

·         If a patient becomes immunocomprosed they can have a reactivation = secondary TB

Tuberculoma is a fibro-caseous/calcific mass

Post pneumonic abscesses commonly result from which 2 organisms?

Staph aureus + klebsiella

Foul smelling purulent material is associated with what type of pneumonia? What type of bacteria would be seen in their abscess?

Aspiration pneumonia *mixed anaerobes/aerobes

Fungal disease: match the following: histoplasmosis, coccidoides, cryptococcous, aspergillus, zucor

·         Found in ohio river valley: histoplasma

·         Found in san jauquin area of SW: coccidioides

·         Budding yeast with prominent polysaccharide coat: Cryptococcus

·         Endospores: coccidiodes

·         Right angle branching: zucor

·         Maybe associated with hypersensitivity allergic pattern: aspergillis

·         Identified as budding yeast on silver stain: histo

·         Maybe found in TB cavitary lesion: aspergillis

Lung cancer:

Most lung cancers are bronchogenic carcinoma: what are the major subtypes (most commonà least)?

·         Adenocarcinoma

·         Squamous cell

·         Small cell

·         Large cell

What type of bronchogenic carcinoma is being described below?

·         Cytoplasmic keratin + intercellular brideges are seen histologically – sq.

·         Can be associated with hyponatremia – small cell (ADH paraneoplastc)

·         Arise peripherally and produce a desmoplastic stromal response: adenocarcinoma

·         Maybe associated with hypercalcemia (Sq – Parathyroid related hormone)

·         Arise peripherally and often has malignant giant cells: large cell

Tumors may produce local injury and tissue damage. Name 3+ examples:

·         Pleural effusion

·         SVC syndrome

·         Nerve damageà hoarseness

·         Airway obstruction à PNA/abscess

·         Horners

·         Pancoast syndrome

How are bronchogenic lung cancers staged?

All use TNM staging except Small cell undifferentiated which commonly presents with advanced disease/distant mets so it is staged as “localized or disseminated”

Which form of bronchogenic lung cancer is not treated surgically

Small cell

Renal Answers

Developmental defects:

Oligohydramnios maybe a symptom of which congenital renal defect? What abnormalities would the child be born with?

·         Bilateral agenesis

·         Kidneys make amniotic fluid: the decreased amount of fluid  à increased pressure exerted on the fetus by the uterus

·         Depressed/flattened facies (Potters), low set ears, deformed lower extremities, pulmonary hypoplasia

If a child has unilateral renal agenesis will he be symptomatic (if so, what symptoms will be present) and what will the other kidney look like?

·         Typically asymptomatic – only one kidney is needed for renal function

·         Adaptive hypertrophy of remaining kidney

·         Women may have mullerian duct abnormalities à infertility

·         Adults may have HTN

How can a true hypoplastic kidney be differentiated from an acquired atrophic kidney at autopsy?

·         Very few will be true hypoplastic – most due to acquired scarring

·         True hypoplastic kidney will have no scars and a reduced number of renal lobes/pyramids (>6)

Why might a patient with horseshoe or ectopic kidneys be prone to UTIs?

·         Horseshoe: ureters have to cross over the central connecting area of the horseshoe àin some causes leading to an increased risk for obstruction and thus bacterial infection

·         Ectopic kidney: the abnormal position may lead to tortuosity of ureters à obstruction of flow and thus risk for bacterial infection

Cystic Diseases of the kidney:

Match the following:

A.       Cystic renal dysplasia

B.       ADPCKD

C.        ARPCKD

D.       Medullary sponge kidney

E.        Nephronophthisis

F.        Acquired cystic disease

G.       Simple cyst

         I.            Most common genetic cause of end stage renal disease in kids/young adults – E

        II.             Caused by abnormal gene on chromosome 16 –B

      III.            Caused by a mutation in PKHD1 gene (which encodes fibrocystin)- C

      IV.            Cysts in the medulla are typically concentrated at the corticomedullary junction - E

       V.            Failure in kidney development despite normally formed precursors- A

      VI.            Likely caused by obstruction to drainage -A

    VII.            Cysts contain clear fluid and often calcium oxalate crystals –F

   VIII.            Cysts are translucent and lined by single layer of cuboidal epithelium – these are a common postmortem finding -G

      IX.            Multiple cystic dilations of the collecting ducts in the medulla-D

       X.            Silent until adulthood when they classily present as: hematuria, HTN, abdominal mass - B

      XI.            Affected children present first with polyuria/polydypsia with terminal renal failure following in 5-10 years - E

    XII.            One of the variants of this disorder is associated with ocular lesions – E

   XIII.            Cortical and medullary cysts seen in patients following prolonged dialysis -F

  XIV.            This disease affects the kidneys/liver in inverse proportions – C

    XV.            bilateral and symmetric enlargement but otherwise looks normal externally- but upon cutting the specimen there are multiple radially arranged cysts involving only the collecting ducts – C

  XVI.            initially involves distal tubules with BM disruption which is followed by chronic and progressive tubular atrophy (medulla + cortex) à interstitial fibrosis à renal insufficiency - E

 XVII.            Kidneys are massively enlarged with multiple variably sized cysts which may affect any area of the nephron - B

XVIII.            Histologically shows: arrested development with simple ducts and often cartilage –A

  XIX.            Juvenile form is associated with NPH1,2,3 - E

    XX.            20% of patients with this disease develop cerebral artery aneurysms – B

Urolithiasis:

Most common (75-85%) form of stone is made of _________________

·         Calcium with the most common being calcium oxalate (1/3 of stones)

Which type(s) of stones are radiolucent? ________________uric acid

How are stones composed of magnesium ammonium phosphate are formed?

·         Post-infection with ura-splitting bacteria (proteus) which alkalizes the urine causing precipitation of magnesium ammonium phosphate

·         Largest stones = staghorn

This type of stones may be seen in a patient with gout or tumor lysis syndrome________________ uric acid

A patient with squamous cell carcinoma of the lung may be at risk for which type of stone?___________

·         Calcium containing stone due to paraneoplastic syndrome which secretes PTHr

Diseases of tubules/interstitium:

What is the most common cause of acute renal failure? ____________________ ATN

What are the two major causes of ATN? ___________+____________

·         Ischemic: decreased renal perfusion (many associated with hypovolemia and shock) – typically follow prolonged period of pre-renal azotemia

·         Toxic

Define Oliguria

·         Production of <400 mL of urine/day

What would you expect the pertinent labs to say in a patient suffering from ATN (UA/urine chemistry)?

·         When the tubular cells are no longer functioning properly

o    Decreased reabsorption of Na = >2% FENa

o    BUN is normally reabsorbed in the PT while Cr is not reabsorbed and just lost in urine (BUN:Cr~15)

§  Conditions that decrease GFR = increased reabsoption and thus BUN:Cr >15

§  Example: pre/post-renal azotemia (there maybe tubular damage in late stages of both of these conditions **remember that pre-renal azotemia is a common cause of ischemic ATN)

§  When there is tubular damage BUN will also be lost in the urine resulting in a BUN:Cr <15***

o    Tubular casts**

T/F: widespread necrosis of the tubules is present in ATN

·         F: necrosis of individual tubular cells with irregular spacing of epithelial cells and dropout of tubule cells = characteristic ATN

Toxic or Ischemic?

·         Which type of ATN is more common? – I

·         The straight segment of PCT in primarily affected (+thick ascending limb of loop of henle) and appears dilated with flattening of individual epithelial cells and loss of brush border (distilazation) –I

·         Typically affects entire proximal portion of nephron with patchy involvement of ascending limb (loop of henle) +/- distal tubule – T (toxins such as mercuric chloride are more severe and will also involve the distal tubule)

How is ATN treated? Is it permanent?

·         Remove causative event (ex: remove aminoglycoside Rx)

·         Supportive +/- peritoneal dialysis/hemodialysis

·         Renal failure typically lasts 1-2 weeks (likelihood of recovery diminishes >4-6wks)

What causes acute vs chronic pyelonephritis? What would you look for in the urine to support your diagnosis?

·         Acute: ascending bacterial infection

o    Bacteria introcusded to bladder will multiply in the presence of stasis (due to dysfunction of bladder/obstruction)

·         Chronic: repeated UTI/obstruction/vesico-ureteral reflux à scarring

·         WBC casts

What bacteria is the most common cause of UTIs?

·         E. coli (gram negative bacilli from intestinal tract – 85%)

Match the following:

A.       Acute pyelonephritis

B.       Chronic pyelonephritis

C.        Papillary necrosis

D.       Pyonephrosis

E.        Perinephric abscess

         I.            Irregularly scarred kidneys corticomedullary scar overlying dilated/deformed calyx – B

        II.            Patchy interstitial suppurative inflammation which begins as limited to the interstitim à destroys tubulesà extends along nephrons/Collecting tubules: A

      III.            Following complete obstruction a suppurative exudates fills the pelvicalyceal system - D

      IV.            A complication of acute pyelonphritis that is commonly seen in diabetic and urinary tract obstruction – C (usually bilatyeral, coagulative infarct with leykocytic response at junction of viable tissue)

       V.            Presents as pain at costovertebral angle, bladder symptoms, and evidence of infection - A

      VI.            Forms due to suppurative inflammation extending through the renal capsule à perinephric tissue – E

What are the two forms of chronic pyelonephritis?

·         Chronic obstructive

·         Chronic reflux

Tubulointerstitial nephritis can be induced by drugs/toxins: name 2+ ways injury is induced:

         I.             Interstitial immunologic rxn

        II.             Acute renal failure

      III.             Subtle cumulative injury to tubules (ex: chronic analgesic use)

Which drugs maybe associated with drug-induced interstitial nephritis?

·         Sulfonamides

·         Synthetic antibiotics

·         Penicillin

·         NSAID

Symptoms present 2 weeks post drug exposure and are associated with eosinophilia, rash, hematuria/proteinuria/leucocyturia

·         50% will have a rising Cr or acute renal failure

·         Interstitial edema, PMN, eosinophils, mononuclear infiltrate +/- granulomas/tubular necrosis

·         Rx: withdraw drug!

Neoplasms of Urinary Tract:

What is the number of layers of urothelium found in a “normal” bladder?

·         5-7: according to degree of distention

·         If urothelium is made of >7 layers = abnormal

All of the following are true except:

A.       Von brunns nests contain a large central lumen with colloid material

B.       Cystitis glandularis is considered a normal variant of urothelium

C.        Von brunns nest are an example of reactive proliferation of urothelium

D.       Cystitis glandularis contain a columnar, mucus secreting cell lining

A: Von Brunns nest are compact groups of urothelial cells in the LP of the bladder. When they contain a central lumen with colloid accumulation it is called “Cystitis cystic” and when the lumen is enlarged further and there are columnar/mucus secreting cells lining the space- then it is cystitis glandularis. These are all normal variants of urothelium which have undergone reactive proliferation due to noxious stimuli.

An Egyptian woman presents with squamous cell carcinoma of the bladder. What infectious agent are you suspicious she was exposed to?

·         Schistosoma hematobium

What is the most common bladder sarcoma in adults vs kids?

·         Adults: leiomyosarcoma

·         Rhabdomyosarcoma: kids

Bladder cancer may occur due to direct extension from ___________+__________+_________

·         Rectum

·         Cervix

·         Prostate

Fill in the chart:

	description	Hyperplasia	Pleomorphism	chromatin	mitoses
Grade1
Grade2
Grade3

	description	Hyperplasia	Pleomorphism	chromatin	mitoses
Grade1	Well differentiated +/- atypia	Cell layers maybe slightly increased	None	Fine/regular	Uncommon
Grade2	Still recognizable as originating from urothelial but more disorganized	Variable increased in cell layers	Greater loss of polarity Greater variability in size/shape	Fine/regular	Common
Grade3	Difficult to recognize as transitional epithelial	Prominent increase	Extreme!	Coarse	prominent

·         NOTE: all transitional cell carcinomas have a tendency to recur post excision (may recur at higher stage)

·         High correlation between grade/stage = infiltrating tumors tend to be grade 3

Match the following: answers maybe reused/multiple

A.       Renal papillary adenoma

B.       Renal oncocytoma

C.        Angiomyolipoma

D.       Renal fibroma

                                 I.            Epithelial neoplasm in which cells have abundant eosinophilic cytoplasm and small round nuclei. They have a high concentration of mitochondria – B

                                II.            Typically are very small and present within the renal medulla and have no malignant potential. Neoplasm shows fibroblast proliferation. – D

                              III.            Benign – A,B,C,D

                              IV.            Irregular and admixed proliferation of blood vessels, smooth muscle, fat – C

                               V.            Small (<1cm) – if larger than this generally considered RCC – A

                              VI.            Grossly: homogenous and brown with a central scar –B

                            VII.            Do not misdiagnose a granular RCC or chromophobe RCC as this! –B

                           VIII.            Composed of branching papillary fronds with fibrovascular cores – A

                              IX.            25% patients with tuberous sclerosis have this neoplasm – C

All of the following are true about RCC except:

A.       85% of all renal cancers are RCC

B.       There is a male predominance

C.        Tumors typically occur 40-50’s

D.       Unopposed estrogen therapy is a risk factor

E.        Most cases are sporadic

C: most occur between 60-70

The three classic diagnostic deatures of RCC are: ___________+_____________+_________

·         Costovertebral pain

·         Palpable mass

·         Hematuria

·         Fq asymptomatic until large!

·         May also see signs of paraneoplastic syndromes such as:

o    Polycythemia

o    Hypercalcemia

o    HTN

o    Hepatic dysfunction

o    Feminization/msaculinization

o    Cushings

o    Amyloid

Match the type of RCC with the description:

A.       Clear cell

B.       Papillary

C.        Chromophobe

D.       Collecting duct

         I.            Maybe associated with sickle cell trait – if so it is termed “medullary carcinoma” and is rapidly fatal - D

        II.            80% of all RCC- A

      III.            composed of pale eosinophilic cells with distinct cell borders and perinuclear clearing - C

      IV.            May see psammoma bodies when these tan tumors are examined histologically - B

       V.            These bright yellow tumors have a high propensity for venous invasion – A

RCC  is associated with  a loss of part of ______ chromosome in 98% of tumors. What syndrome is associated with this loss (resulting in a high co-incidence).

·         Chromosome 3 – VHL gene (TSG)

·         Von hippel landau syndrome

All of the following concerning urothelial carcinoma of the renal pelvis are true except:

A.       These tumors become symptomatic late in their course

B.       Patients may have gross hematuria and renal colic

C.        Up to 50% of patients have a pre-existing/concomitant bladder cancer

D.       Poorer prognosis than bladder carcinoma

A: these tumors sit right in the collecting system so they are more likely to present with hematuria when they fragment à prompting earlier discovery. These tumors typically infiltrate the pelvic/calcyceal walls so therefore have a poorer prognosis than bladder cancer

What is the most common renal neoplasm of childhood? Wilms = nephroblastoma

This tumor has been linked to abnormalities with a TSG located on chromosome ___ 11

This tumor is (histologically) composed of three main components

1.         

2.         

3.         

                                                               i.       Blastema

                                                              ii.       Epithelial (primitive tubules/glomerular structures/rosettes-rare)

                                                            iii.       stromal

Glomerular renal disease:

Although albumin is small enough to fit through the fenestrated epithelium of the glomeruli capillaries it is not found in high concentration in the urine of a patient with a functioning kidney. Why is this?

·         The job of the glomerulus: keep proteins in and allow water/solute to pass out

·         The glomerulus is composed of:

o     capillaries (fenestrated epithelium)

o     basement membrane (major barrier to protein passage because it contains strong negative charge which repels the proteins)

o     podocytes (exact function is not known – appear to trap proteins and have pinocytotic functions)

o    mesangial cells SMC which support capillary loops – these are highly responsive to hormones + have the ability to regulate glomerular blood flow via contraction /expansion

Match the following – there maybe multiple :

A.       nephrotic syndrome

B.       nephritic syndrome

C.        low grade proteinuria

         I.            Associated with red cell casts – B

        II.            minimal basement membrane damage that results in a urine protein excretion of <150 mg and normal serum protein concentration because the liver is able to compensate: C

      III.            mild-moderate diffuse glomerular inflammation that is associated with a drop in GFR-B

      IV.            moderate to severe basement membrane injury resulting in protein loss that exceeds the livers ability to compensate à drop in serum albumin à drop in oncotic pressure and thus edema: A

       V.            Associated with hypercholesterolemia A

What causes crescent formation in RPGN?

·         This is the most severe form of glomerular inflammation

·         Capillary loops are disruptedà cytokines/chemokines leak into bowmans space à activation/proliferation of parietal epithelial cells with associated inflammatory cells à crescent formation à if untreated - sclerosis

·         The excess cellularity may compress the glomeruslus

What will happen to C3 + C4 in the following scenarios:

·         Activation of classic complement pathway: decrease C3+ C4

·         Activation of the alternative pathway: decrease C3 only (ex: membranoproliferative GN)

What does the term “membranous” refer to?

·         Involvement of the capillary basement membrane leading to an increase in loop thickness

What causes a “granular” appearance on IF?

·         Antigen-antibody complexes àgranular

·         Antibodies that bind directly to antigen (ex: goodpastures: anti-GBM) à linear

What is EM used for when evaluating glomerular diseases?

·         Localize immune complex location

·         Provides extreme detail of BM structure

In adults, MCD may occur secondary to _______ or _________

·         NSAIDs

·         Hodgkin lymphoma

Match the following:

A.       Minimal change disease

B.       Membranous nephropathy

C.        Diabetic glomerular damage

D.       Amylodosis

E.        FSGN

F.        Membranoproliferative GN

         I.            Corticosteroid response generally good: A

        II.            Capillary loops are pushed to the edge of tufts creating kimmelsteil-wilson – C

      III.            Subepithelial deposits seen on EM create “spike and dome” pattern: B

      IV.            Subendothelial deposits are seen with this condition- F (T1)

       V.            C3 convertase leads to overactivation of complement à low C3 circulating – F(T2)

      VI.            Associated with HIV - E

    VII.            Widespread podocyte effacement with normal LM – A

   VIII.            Most common cause of nephritic syndrome in kids: A

      IX.            Associated with sickle cell – E

       X.            Congo red with apple green birefringence - D

      XI.            Thick glomerular basement membrane appears as “tram tracks” because divided by mesangium - F

    XII.            Earliest evidence is microscopic albuminuria: C

   XIII.            IF: thickened mesangium, no immune complexes, thickened capillary loops: C

  XIV.             Most common cause of nephrotic syndrome in Caucasian adults: B

    XV.            Maybe seen in patients with multiple myeloma: D

  XVI.            Associated with HBV : B

 XVII.            Most common nephrotic syndrome in African Americans - E

XVIII.            Granular deposits of IgG and C3 seen in even line along GBM on IF: B

  XIX.            Circulating serum proteins are deposited in the tissues as B-pleated sheets: D

Postinfectious glomerulonephritis: Which of the following are not true?

·         Typically follows a skin or less commonly a throat infection with group B strep

o    F: group A

·         Infection seen >1-2 weeks after infection as coca-cola colored urine, periorbital edema, HTN, oliguria

·         LM: increase PMN in the glomerulus and tubulointerstitial compartment (F: TI spared!)

·         Biopsies are only preformed for persistent proteiuria

·         May progress to RPGN

·         Better Px in kids

IgA in Berger disease deposits in which part of the glomerulus? Mesangium - #1 nephropathy worldwide!

What disease presents as hematuria, sensory hearing loss and ocular disturbances? Alports (IV collagen defect – X linked)