Renal: Pathology Flashcards by James Wren

Kidneys connected at the Lower Pole
Most common congenital Renal anomaly
Abnormally located in the Lower abdomen
Gets caught on the Inferior Mesenteric Artery root during Ascent from the Pelvis → Abdomen

Horseshoe Kidney

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Absent Kidney formation → Unilateral or Bilateral
Unilateral agenesis – hypertrophy of the existing Kidney and Hyperfiltration leads to increase risk of Renal failure later in life
Bilateral agenesis – Oligohydramnios w/ Lung Hypoplasia, Flat Face, and Low ears and developmental defects of extremities (Potter Sequence) = incompatible w/ Life

Renal Agenesis

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A condition in pregnancy characterized by a deficiency of amniotic fluid. It is the opposite of polyhydramnios
The breathing of the amniotic fluid helps stretches the babies lungs and helps to promote lung development – w/out the lungs fail to develop correctly

Oligohydramnios

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Noninherited, Congenital malformation of the Renal Parenchyma characterized by Cysts, and Abnormal tissue (e.g. cartilage)
Usually unilateral
Bilateral – MUST be distinguished from Inherited Polycystic Kidney disease

Dysplastic Kidney

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Inherited defect – Bilateral enlarged Kidneys
Cysts in the Renal Cortex and Medulla
Autosomal Recessive - PKHD1 (6q21-p23)– form presents in infants as worsening Renal failure and Hypertension
- Newborns present w/ Potter Sequence
  - Congenital Hepatic Fibrosis (Portal Hypertension)
  - Hepatic cysts – in the Liver and Kidney
Autosomal Dominant – Young Adults as Hypertension due to increased Renin, Hematuria, and Worsening Renal Failure
- Mutations APKD1 (16p13.3) or APKD2 (4q21) genes – cysts develop over time
- A/w Berry aneurysm, Hepatic cysts, Mitral valve prolapse

Polycystic Kidney Disease (PKD)

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Inherited disease causing Tubulointerstitial fibrosis and Progressive Renal Insufficiency w/ inability to concentrate urine
Autosomal dominant defect leading to Cysts in the Medullary collecting ducts
Parenchymal Fibrosis results in Shrunken Kidneys and woresening Renal Failure
Poor prognosis

Medullary Cystic Kidney Disease

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Acute, Severe decrease in Renal Function (develops w/in Days) as measured by GFR (Normal 115 - 125 mL/min
Hallmark is Azotemia (↑ BUN and ↑ Creatinine [Cr], often w/ Oliguria (Urine < 500 mL/day) or Anuria (Urine < 100mL/day)), Polyuria (> 3 L/day)
- Increase in nitrogenous waste production
Divided into PreRenal, PostRenal, and IntraRenal based on etiology

Acute Renal Failure (ARF)

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Decreased Blood flow to Kidneys (e.g. cardiac failure) → common cause ARF
↓ RBF → ↓ GFR – Azotemia and Oliguria - ↓ Urine production - Na⁺ / H₂O and Urine retained
Reasbsorption of Fluid and BUN ensues (serum BUN:Cr ratio > 15); Tubular function remains intact (Fractional excretion of Na [FENa] < 1% and Urine osmolality [osm] > 500 mOsm/kg – The Kidneys still have the ability to concentrate the Urine **Urine Na < 10 **(mEq/L) **

Prerenal Azotemia

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Due to Obstruction of Urinary Tract downstream from the Kidney (e.g. Ureters)
↓ GFR – Azotemia, and Oliguria
Early stage obstruction, increased Tubular pressure “forces” BUN into the blood - Urine Na⁺ < 20 mEq/L
- (serum BUN:Cr ratio > 20); Tubular function remains intact
- (FE_Na < 1% and Urine [osm] > 500 mOsm/kg – tubules still function
Long stage obstruction, Tubular damage ensues, resulting in decreased reabsorption of BUN
- (serum BUN:Cr ratio > 15)
- Decreased reabsorptoin of sodium (FENa > 4%) and Urine [osm] < 350 mOsm/kg, Urine Na > 40 mEq/L

Postrenal Azotemia

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Injury and Necrosis of Tubular Epithelial cells
Most common cause of Acute Renal Failure (ARF) (intrarenal azotemia) may have Oliguria and Azotemia
A/w Rhabdomyolysis and Crush injury
Necrosis cells plug Tubules; Obstruction decreases GFR
Muddy-brown, Granular, Casts are seen in the Urine
Dysfunctional Tubular Epithelium results in Decreased Reabsorption of BUN (serum BUN:Cr ratio < 15)
Decreased reabsorption of sodium (FENA > 2%)
Inability to concentrate Urine (Urine [osm] < 500 mOsm/kg)

Acute Tubular Necrosis

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Decreased blood supply results in Necrosis of Tubules (decreased ATP leads to ischemia)
A/w rupture of the Basement membrane (Tubulorrhexis)
1^st: Often Preceded by Prerenal Azotemia
Proximal Tubule and Medullary segment of the Thick Ascending Limb are particularly Susceptible to Ischemic Damage

Ischemic Acute Tubular Necrosis

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Toxic agents result in Necrosis of Tubules
Proximal tubule is particularly susceptible
2^nd: Causes include Aminoglycosides (most common), Heavy metals, Myoglobinuria (crush inj.), Ethylene glycol (a/w oxalate crystals in urine), Radiocontrast dye, Urate, Tumor lysis syndrome
Hydration and Allopurinol are used prior to initiation of Chemotherapy to Decrease risk of Urate-induced ATN
Dx: Oliguria (2-3 weeks before recovery, regen.) w/ ‘Muddy’ Brown Granular casts
Elevated BUN and Creatinine
Hyperkalemia w/ Metabolic acidosis – decreases excreating of organic acids – Anion gap
Tx: Reversible but requires Supportive Dialysis since Electrolyte imbalance – Fatal (Oliguric phase)

Nephrotoxic Acute Tubular Necrosis

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Drug-induced Hypersensitivity involving the Interstitium and Tubules - acting as Haptens
Results in Acute Renal Failure (intrarenal azotemia)
Caused by NSAIDS, Penicillin, and Diuretics
Presents w/ Oliguria, Fever, and Rash days to weeks after starting a drug; Eosinophils and Azotemia may be seen in Urine
WBC casts
Resolves w/ cessation of drug
May progress to Renal papillary necrosis

Acute Interstitial Nephritis

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Necrosis of Renal papillae ‘sloughing’
Presents w/ Gross Hematuria and Flank Pain
Polyuria, Rust-colored urine, ARF, Sediment, Casts
CXR - Ring of calcification (neprocalcinosis)
Causes include:
- Chronic Analgesic abuse (long term Phenacetin or Aspirin use) (Acetaminophen is a derivative)
- Diabetes mellitus
- Sickle cell traits or Disease
- Severe Acute Pyelonephritis

Renal Papillary Necrosis

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Glomerular disorders characterized by Proteinuria (> 3.5 g/day) resulting in:
- Hypoalbuminemia – pitting edema
- Hypogammaglobulinemia – increased risk of infection – protein in blood
- Hypercoagulable state – due to loss of Antithrombin III – loss of anti-coagulant protein – breaking up of thrombi and prevention of thrombi anti-coagulet
- Hyperlipidemia and Hypercholesterolemia – may result in Fatty Casts in urine – thin blood – Liver adds Fat to Thicken the blood

Basics of Nephrotic Syndrome

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Most common cause of Nephrotic syndrome in Children (idopathic) (80%)
Usually Idiopathic; may be a/w Hodkin lymphoma (cytokine-mediated damage)
Normal glomeruli on H&E stain; Lipid may be seen in Proximal tubule cells - “Lipoid nephrosis” - ‘Foamy’
Effacement of Foot processes on electron microscopy
No immune complex deposits; negative immunofluorescence – not deposit mediated
Selective Proteinuria (Loss of Albumin, but not immunoglobulin)
Excellent response to Corticosteroids (knock out cytokine production) (damage is mediated by cytokines from T cells)
- Hodgekins Lymphoma – overproduction of cytokines by Reed-Sternberg cells – Fever, Chills, Night sweats

Minimal Change Disease (MCD)

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Most common cause of Nephrotic Syndrome in Hispanics and African Americans
Usually Idiopathic; may be a/w HIV, Heroin use, and Sickle cell disease, Massive obesity, INF treatment
**Focal accumulation of Hyaline material (<50%) **(some glomeruli) and Segmental Sclerosis (involving only part of the glomerulus) – sclerosis on H&E stain
Effacement of Foot processes on EM
No immune complex deposists; Negative IF
Poor response to steroids -> Cyclophosphamide and Cyclosporine -> Progresses to Chronic Renal Failure

Focal Segmental Glomerulosclerosis (FSGS)

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Most common cause of Nephrotic syndrome in Caucasian Adults
Usually idiopathic; may be a/w Hepatitis B and C, Solid tumors, SLE, Rheumatoid arthritis, Syphilis, Schistosomiasis, Malaria, Leprosy, **Phospholipase A₂ receptor, **Drugs (NSAIDs and Penicillamine)
GBM Thickening on H&E w/ ‘Spike and dome’
Due to Immune Complex Deposition (granular IF; subepithelial deposits), and Nephrotic SLE
Poor response to Steroids progresses to Chronic Renal Failure

Diffuse Membranous Glomerulopathy

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Thick Glomerular basement membrane on H&E, often w/ ‘TRAM-Track’ appearance - Hypercellular glomeruli
Immune Complex Deposition (granular IF)
Dividied into (2) Types based on Location of Deposits
- Type I – Subendothelial (**HBV, **HCV, Cryoglobulinemia) (Type III Hypersensitivity)
- Type II – “dense deposit disease” – Intramembranous; a/w C3 Nephritic Factor (auto-Ab that Stabalizes C3 Convertase, leading to Overactivation of Complement, inflammation, and Low Levels of circulating C3), No IgG present
Poor response to Steroids; progresses to Chronic Renal Failure

Membranoproliferative Glomerulonephritis

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High Serum glucose leads to Nonenzymatic glycosylation of the Vascular basement membrane resulting in Hyaline arteriolosclerosis (basement membrane becomes Leaky – leaking protein – Hyaline prolif.) -> mesangial expansion
Glomerular Efferent >> Glomerular Afferent in the Arteriole – leading to High Glomerular Filtration process
- Hyperfiltration injury leads to Microalbuminuria
Eventually progresses to Nephrotic Syndrome
- Characterized by Sclerosis of the Mesangium w/ formation of Kimmelstiel-Wilson nodules
ACE inhibitors slow progression of Hyperfiltration-induced damage

Diabetic Nephropathy

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Kidney is the most commonly involved organ in Systemic Amyloidosis
Amyloid deposists in the Mesangium, resulting in Nephrotic Syndrome
Characterized by Apple-green Birefringence under Polarized light after straining w/ Congo red

Systemic Amyloidosis

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Glomerular Inflammation and Bleeding -> Hematuria
Mild Proteinuria (< 3.5 g/day)
Oliguria (< 400 mL/day) and Azotemia (↑ BUN and Creatinine) – decrease in urine and increase in nitrogenous waste products w/in the blood
RAAS w/ Periorbital / Pitting Edema and Hypertension
RBC casts and Dysmorphic RBCs in urine
Biopsy reveals Hypercellular, Inflamed glomeruli
Immune-complex deposition activates complement; C5a attracts Neutrophils – which Mediate damage – Hypercellularity
**C3 levels, anti-GBM titer, **and ANCA titer

Nephritic Syndrome

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Nephritic syndrome w/ Group A β-hemolytic Streptococcal Infection of the skin (impetigo) or pharynx - ‘**Starry-sky’ **appearance - Type III Hyper.
W/ Nephritogenic strains – Carry **M protein Virulence Factor - Serum chemistry -> Antitreptolysin-O **and anti-DNAase B are elevated, ANCA and anti-GBM neg.
After Infection w/ Nonstreptococcal organisms as well
Presents 2 -3 weeks after infection as Hematuria (cola-colored urine), Oliguria, Hypertension, and Periorbital edema -> “Smokey-brown” or “Cola-colored” urine
Usually seen in Children but may occur in Adults
Hypercellular, inflamed Glomeruli on H&E
Mediated by immune complex deposition (Granular IF); Subepithelial ‘humps or lumpy-bumpy’ on EM
Tx: Children rearely (1%) profress to Renal Failure
- Some Adults develop Rapidly Progressive Glomerulnephritis (RPGN) (wks – mths)

Poststreptococcal Glomerulonephritis (PSGN)

Acute Proliferative Glomerulonephritis

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Malignant form of Nephritic syndrome that progresses to Renal Failure in Weeks to Months
Characterized by Crescents in Bowman space (of Glomeruli) on H&E stain
Crescents are comprised of Fibrin and Macrophages
Clinical picture and IF help resolve etiology
(3) Distinct Types:
1. Goodpasture syndrome - Type II, ANCA-negative
2. Poststrep GN, SLE, IgA neph. Henoch-Schonlein purpura - ANCA-negative - Type III
3. Wegener granulo. or ideopathic - PR3-ANCA / c-ANCA - positive

Rapidly Progressive Glomerulonephritis

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* IgA immune complex deposition in **Mesangium of Glomeruli** * Most common nephropathy worldwide * Presents during **Childhood** as **Episodic Gross** or **Microscopic Hematuria** w/ RBC casts * Usually following **Mucosal Infection** – IgA production in increased during Infection, a/w **Hnoch-Schonlein purp.** * IgA immune complex deposition in the **Mesangium** is seen on IF – glomerular bleeding * Every episodic infection you get bleeding from the Kidney **a/w Celiac disease** * Slow progress to Renal failure

IgA Nephropathy (Berger Disease)

* **Hereditary Glomerular injury**, defect in **Type IV collagen** * Most commonly **X-linked --\> Mostly males** * Results in **Thinning** and **Splitting** of the Glomerular basement membrane '**Foamy' **changes * Presents as **Gross Hematuria (males) **and **Sensory Hearing Loss** and **Ocular distribution –** damage to basement membrane (**nerve deafness, lens dislocation, cataracts**) -\> Females are carriers (mild symptoms) * Presented w/in Family History * ANCA and anti-GGM both negative, C3 lvls normal

Alport Syndrome (Hereditary Nephritis)

* Infection of Urethra, Bladder, or Kidney * Most commonly arises due to **Ascending Infection**, Increased Incidence in **Females** * Risk factors include **Sexual Intercourse**, **Urinary stasis**, and **Catheters**

Basics of Urinary Tract Infection

* Infection / Inflammation of the Bladder * Presents as Dysuria, Urinary frequency, Urgency, and Suprapubic pain, Systemic signs (Fever) are usually absent - "**Honeymoon cystitis**", indwelling catheters * Lab: * **Urinalysis** – cloudy urine w/ \> 10 WBC/high power field * **Neg**. **Culture – Urethritis** – *Chlamydia trachomatis* and *Neisseria gonorrhoeare* * **Dipstick** – Positive leukocyte esterase (due to Pyuria) and **Nitrites** (bacteria convert Nitates to Nitrites) * **Culture** – greater than 100,000 colony forming units (GOLD-Standard) * Etiology: ***E. coli*** (80%), ***Stapjylococcus saprophyticus*** (young sexually active women), ***Klebsiella pneumoniae,** **Proteus mirabilis*** – **Alkaline** urine w/ **Ammonia scent**, *Enteroccus faecalis, **Adenovirus***

Cystitis

* Infection of the Kidney - Cortex * Usually due to **Ascending infection** – increased Risk w/ **Vesicoureteral reflux** * Fever, Flank pain, **WBC casts**, and Leukocytosis, in addition to symptoms of **Cystitis** - 'striated parenchymal enhancement' * Most commonly: Tx: ABX * ***E. coli* (90%)** * ***Enterococcus faecalis* (species)** * *Klebsiella* species

Acute Pyelonephritis

* **Interstitial fibrosis** / **scarring **and **Atrophy** of Tubules due to **Multiple** bouts of **Acute Pyelonephritis** * **Vesicourecteral reflux** (VUR) (**Children**) or **Obstruction** (Benign prostati hypertrophy BPH or Cervical carcinoma) - due to predisposition to infections * Leads to **Cortical scarring** w/ **Blunted calyces** * Low-grade Fever, Flank pain, Nausea / Vomiting, Failure to thrive, HTN, Renal insufficiency, Protenuria * Atrophic tubules containing **Eosinophilic proteinaceous material** resemble **Thyroid follicles** (**‘Thyroidization’ of the Kidney**, waxy casts may be seen in Urine)

Chronic Pyelonephritis

* Precipitation of a **Urinary solute** as a **Stone** * Risk factors include High concentration of **Solute** in the **Urinary filtrate** and **Low Urine Volume** * Presents as a **Colicky Pain** w/ **Hematuria** and **Unilateral Flank Tenderness** * Stone is usually passed w/in Hours; if not, Surgical Intervention may be required

Nephrolithiasis

* **Radiopaque** **Stone** type, Adults, **Colorless Octahedron** * Calcium **Absorption** of the gut **\>** **Excretion** in the Urine or Primary Renal defect of **Calcium re-absorption** * Most common cause is Idiopathic **Hypercalciuria, 2^ndHyperparathyroidism, Vit. D intoxication, Sarcoidosis**; **Hypercalcemia** and its related causes must be excluded * Can also form from Ethylene glycol (**antifreeze**) * a/w **Crohn disease** – small bowel damage – increased abs. Oxolate – binds Ca²⁺ - stone * Tx: **Hydrochlorothiazide** (calcium-sparing diuretic)

Calcium Oxalate stones (↓ pH) Calcium Phosphate stones (↑ pH)

* 2^nd most common **Stone** type * **Radioopaque**, **Rectangular prism**, like **'Coffin-lids'** * Most common cause is **Infection w/ Urea-positive organisms** (*Proteus vulgaris, Staphylococci,* *Klebsiella, and Psuedomonas,* but **NOT *E. coli***); **Alkaline urine** leads to formation of stone + ABX * Results in **Staghorn calculi cast (kidney stone)** in **Renal Calyces** – act as a **Nidus** for Urinary tract infections * Tx: Surgical removal of stone (size) and Eradication of Pathogen, Carbonic anhydrase inhibitors (Acetazolamide)

Ammonium Magnesium Phosphate (AMP stone) (↑ pH) 'Stuvite'

* Third most common stone (5%) * **Radiolucent on CXR** * **Yellow** or **Red-brown, Diamond or Rhombus** * Hot, **Arid climates**, Low urine volume, **Acidic Urine pH** * Most common stone seen in pts. w/ **Gout** * **Hyperuricemia** (in **Leukemia** or **Myeloproliferative disorders**) increases risk * Tx: Hydration and Alkalinization of Urine (potassium bicarbonate); Allopurinol is also administered in pts. w/ Gout

Uric acid (↓ pH)

* Rare cause of Nephrolithiasis; most commonly see in **Children** * A/w **Cystinuria** (a genetic defect of tubules that results in decreased reabsorption of Cysteine, Ornithine, Lysine, Arginine), more likely in **Acidic Urine pH** * **Fairly opaque** w/ **Ground-glass appearance CXR** * Sodium nitroprusside test * **Flat, Yellow, Hexagonal** * May form **Staghorn calculi** * Tx: **Hydration** and **Alkalinization of Urine**

Cystine Stones (↓ pH)

* End-stage Kidney Failure – may result from **Glomerular, Tubular, Inflammatory**, or **Vascular insults** * Most common causes are **Diabetes mellitus, HTN,** and **Glomerular disease (GFR \< 60 mL/min)** * Uremia, Azotemia, Nausea, Anorexia, Pericarditis, Platelet dysfunction, Encephalopathy w/ Asterixis, Deposition of Urea crystals in skin * **Salt and Water retention** w/ resultant Hypertension * **Hyperkalemia** w/ Metabolic acidosis – cannot get rid of organic acids * **Anemia** due to **decreased Erythropoietin** production by Renal Peritubular Interstitial cells * **Hypocalcemia** due to decreased 1-alpha-hydroxylation of Vit. D by Proximal Renal tubule cells and **Hyperphosphatemia** – cannot excrete PO₄ bind to Ca²⁺ - low [Ca²⁺] * **Renal Osteodystrophy** – due to secondary Hyperparathyroidism osteomalacia and osteoporosis * Tx: Dialysis or Renal Transplant w/ cysts in shrunken end-stage Kidneys on dialysis

Chronic Renal Failure

* Hamartoma comprised of (3) components * **Blood vessels** * **Smooth muscle** * **Adipose tissue** * Increased frequency in **Tuberous sclerosis**

Angiomyolipoma

* **Malignant epithelial tumor** from **Proximal tubules** * Classic Triad (3) **Hematuria** (most common)**, Palpable mass**, and **Flank pain, **Polygonal clear cells * **Fever, Weight loss,** or **Paraneoplastic syndrome** (EPO – reactive polycythemic??, Renin – HTN, PTHrP – Hyper Ca²⁺, ACTH – Coshney’s symptom??) * A/w **Left-sided Variocele** – left spermatic vein involvement, Men - **50 - 70 years-old** * Yellow-Mass w/ clear cytoplasm * Loss of *VHL* (3p) tumor supressor gene – increased IGF-1 (promotes growth) – increase HIF transcription factor – increases VEGF and PDGF * **Sporadic –** adult makes (60 y.o.) single tumor in Upper pole of Kidney * **Hereditary tumors** – young adults, Bilateral tumor, **Von Hippel-Lindau disease** (*VHL* gene)

Renal cell Carcinoma

* Malignant Kidney tumor comprised of **Blastemal** (immature kidney mesenchyme), Primitive **Embryonic **Glomeruli and Tubules, and Stromal cells * Common in **Children**, avg. 2 - 4 y.o. * Large, Unilateral Flank mass w/ Hematuria and Hypertension (Renin secretion) * (3) Sporadic (90%) tumors w/ syndromic tumors 1. _WAGR syndrome_ – **W**ilms tumor, **A**niridia, **G**enital abnormalities, and Mental and Motor **R**etardation a/w *deletion* of ***WT1*** **tumor suppressor gene** (**11p13**) 2. _Denys-Drash syndrome_ – Wilms tumor, progressive Renal (glomerular) disease, and male Pseudohermaphroditism, a/w *mutation* of ***WT1*** 3. **_Beckwith-Wiedemann syndrome_** – Wilms tumor, **neonatal hypoglycemia**, muscular **hemihypertrophy**, and **organomegaly** (including tongue); a/w ***WT2* gene cluster** (**11p15.5** ) particularly **IFG-2**

Wilms Tumor

* Maliginant tumor; Urothelial lining of **Renal pelvis**, **Ureter**, **Bladder**, or **Urethra** * Most common type of Lower Urinary tract cancer (Bladder) (No casts) * **P**ee **SAAC-N - P**henacetin**, S**moking**, A**niline**/A**zo dyes**, ** Long - term **C**yclophosphamide and **N**aphthylamine * Older adults; w/ Painless Hematuria * (2) Pathways 1. _Flat_ – High-grade tumor then Invades, a/w **early P53 mutation** 2. _Papillary_ – low-grade papillary tumor then High-grade then Invades, **NOT a/w P53 mut.** * Multifocal and Recur (‘Field defect’ = entire endothelium has been exposed to carcinogen exp.)

Urothelial (Transitional cell) Carcinoma

* Maliginant tumor, Squamous cells, usually w/in Bladder * In background of **Squamous metaplasia** (normal bladder does not have squamous epithelium) * 'Warty' or Ulcerated lesions * **Chronic cystitis** (older woman), ***Schistosoma hematobium*** infection (Middle Eastern and Egyptian males), and **Long-standing Nephrolithiasis** (chronic infection / Cystitis in young males)

Squamous Cell Carcinoma

* Maliginant proliferation of **Glands**, usually involving the Bladder * Arises from a **Urachal Remnant** (@ the **Dome of the Bladder**) * **Urachus** is a duct that connect the Bladder into the Yolk sac – remnant becomes Adenocarcinoma * **Cystitis glandularis** (columnar metaplasia) * **Exstrophy** (congenital failure to form the Caudal portion of the Anterior abdominal wall and Bladder walls)

Adenocarcinoma

Diseases associated w/ Red Blood Cell casts?

* Glomerulonephritis * IgA nephropathy * Poststreptococcal glomerulonephritis * Goodpasture syndrome (rapidly progressive glomneph.) * Malignant HTN * Vasculitis * Renal ischemia

What diseases are a/w White Blood Cell casts?

* Acute Pyelonephritis * Interstitial nephritis * Tubulointerstitial inflammation * Lupus Nephritis * Transplant rejection * WBCs in Urine indicate UTI

What diseases are a/w Granular 'Muddy-brown' casts?

* Acute Tubular Necrosis (ATN) * Chronic Renal failure * Nephrotic Syndrome * Fatty casts are also seen in Nephrotic syndrome

What diseases are a/w Epithelial cell casts?

* Acute tubular necrosis (ATN) * Ethylene glycol toxicity * Heavy-metal poisoning * Acute rejection of Transplant graft

What diseases are a/w Hyaline casts?

* Often seen in Normal Urine * Concentrated urine samples * Pyelonephritis

* Deposition of **Fibrous**, **Insoluble proteins in β-pleated** configuration deposits in the Kidney * '**Light-chain deposition disease**' * Proteinuria, Severe-Edema, Renal insufficiency, also a/w secondary diseases (**Multiple Myeloma, TB, RA,** etc.) * Congo red stain -\> Apple-green birefringence = amyloidosis * Tx: Melphalan and Prednisone

Renal Amyloidosis

* A/w both Nephrotic and/or Nephritic syndromes * Weight gain, High BP, Darker 'foamy' urine, Swelling around the eyes, legs, ankles, or fingers * A/w 5 classes of involvement

Lupus Nephritis * **Class I** - No evidence of disease * **Class II** - Mesangial involvement * **Class III** - Focal proliferative nephritis * **Class IV** - Diffuse proliferative nephritis (most common type in SLE) * **Class V** - Membranous nephritis, characterized by extreme edema and protein loss

* antibodies against proteins in Globular Basement Membrane (anti-GBM) * Can be seen in Lung and Kidnes due to cross-reactivity of antigens (eg. **α₃ chain of collagen type IV**) common to both alveolar and GBM * **Type II Hypersensitivity** * Hematuia, Proteinuria, and Rapidly Progressive Glomerulonephritis (RPGN), **Hymoptysis**, Dyspnea * Serum anti-GBM Ab, ANCA typically neg. C3 is normal * **RBC casts** and mile proteinuria, **Linear ribbon-like IgG**

Goodpasture syndrome

* **Focal / Segmental Necrotizing vasculitis** and **Necrotizing granulomas** in Upper and Lower respiratory tract (Lungs) w/ occasional **Crescent Formation** * Nonspecific symptoms, Fever, Arthralgias, Lethargy, Malaise, **Chronic sinusitis**, **Hemoptysis**, and **Hematuria** * Nephritic symptoms and mild Proteinuria * Cytoplasmic staining ANCA **(c-ANCA**) - **positive** * **NO IgG or anti-GBM**, and **Complement are normal** * Tx: **Corticosteroids** and **Cyclophosphamide** w/ Dialysis and Renal Transplantation

Wegener Granulomatosis

* Develops as a result of '**patchy**' **Infarction of the Cortices** of the Kidney secondary to Ischemia -\> can progress to Acute Renal Failure (ARF) - **Bilateral** * A/w **_Abruptio placentae_, Eclampsisa / Preeclampsia, _Septic shock_, Hemolytic-uremic syndrome** (in children) * Sepsis, Disseminated Intravacular Coagulation (DIC), Obstetric complications, Anuria, evidence of ARF, Flank pain, and FEVER

Diffuse Cortical Necrosis

Nephritic Syndrome Diseases?

* Acute poststreptococcal glomerulonephritis * Rapidly progressive glomerulonephritis * IgA glomerulonephropathy (Berger disease) * Alport Syndrome

Diseases that are both Nephritic and Nephrotic?

* Diffuse proliferative glomerulonephritis * Membranoproliferative glomerunephrtis

Disease of Nephrotic Syndrome?

* Focal segmental glomerulsclerosis * Membranous nephropathy * Minimal Change Disease * Amyloidosis * Diabetic glomerulonephropathy

Consequences of Renal Failure?

* MAD HUNGER * **M**etabolic **A**cidosis**** * **D**yslipidemia (esp. ↑ Triglycerides) * **H**yperkalemia * **U**remia - marked by Azotemia * **N**a⁺/H₂O retention * **G**rowth retardation and developmental delay * **E**rythropoietin Failure (anemia) * **R**enal osteodystrophy

* **Defect** in ability of **α intercalated cells** to **secrete H⁺** * New HCO₃^- is not generated -\> **Metabolic Acidosis** * A/w **Hypokalemia** -\> ↑ Risk for **Ca-PO₄ Kidney Stones** due to ↑ Urine pH and ↑ Bone turnover * Causes: Amphotericin B toxicity, Analgesis nephropathy, Multiple myeloma (light chains), and Congential anomalies (Obstruction) of the Urinary tract

Type I - Renal Tubular Acidosis | (Distal Tubule, pH \> 5.5)

* Defect in Proximal Tublue **HCO₃^- reabsorption** * Results in ↑ **excretion of HCO₃^-** in Urine and subsequent **Metabolic Acidosis** * Urine is acidificed by α intercalated cells in Collecting Tubule * A/w **Hypokalemia**, ↑ Risk of **Hypophosphatemic Rickets** -\> Fixed acid lost in Urine * Causes: **Fanconi Syndrome** (Wilson disease), Chemicals toxic to Proximal tubule (Lead, **Aminoglycosides**), and **Carbonic Anhydrase Inhibitors**

Type II - Renal Tubular Acidosis | (Proximal, pH \< 5.5)

* **Hypoaldosteronism** * Aldosterone resistance or K⁺ - sparing diuretics * Results in **HyperKalemia** -\> impairs **Ammoniagenesis in the Proximal Tublue** - \> ↓ Buffering capacity - \> ↓ H⁺ excretion into Urine

Type IV - Renal Tubular Acidosis | (HyperKalemic, pH \< 5.5)

* **Reabsorptive defect in PCT** * A/w ↑ Excretion of nearly all **Amino acids, Glucose, HCO₃^-, and PO₄^3-** * May result in **Metaboic Acidosis** (Proximal Renal Tubular Acidosis) -\> Hyper**K**alemia * Causes: Hereditary defects (Wilson disease), Ischemia, and Nephrotoxins / drugs * The Kidneys put out **FAB**ulous **G**littering **L**iquid

Fanconi Syndrome * **FAB**ulous **G**littering **L**iquid * **FA**nconi syndrome in the 1st defect of PCT * **B**artter syndrome is next (Thick ascending loop of Henle) * **G**itelman syndrome is after Bartter (DCT) * **L**iddle syndrome is last (Collecting tubule)

* **Reabsorptive defect in Thick Ascending Loop of Henle** * Autosomal recessive * Affects Na⁺ / K⁺ / 2Cl^- Cotransporter * Results in **HypoK**alemia and **Metabolic Alkalosis** w/ Hyper**Ca**lciuria

Bartter Syndrome

* **Reabsorptive defect of NaCl in DCT** * Autosomal recessive * Less severe than Bartter syndrome * Leads to **HypoK**alemia and Metabolic alkalosis, but **w/out HyperCa**lcuria

Gitelman Syndrome

* **↑ Na⁺ reabsorption in Distal and Colecting tubules** (↑ activity of Epithelial Na⁺ channel) * Autosomal dominant * Results in **HTN**, **HypoK**alemia, Metabolic alkalosis, ↓ Aldosterone * Tx: **Amiloride**

Liddle Syndrome

Renal: Pathology Flashcards

(63 cards)