RENAL PATHOLOGY -3

Question 1

Name one condition associated with formation of the characteristic crystals shown in the attached image.

Answer 1

Hyperuricemia- Gout, Leukemia

The uric acid crystals are described as Diamond/ rhombic/ rosette forms

Question 2

What is the underlying reason for the typical physical appearance and assoc pulmonary hypoplasia in Potter sequence?

Answer 2

Oligohydramnios and compression while in utero

Question 3

Identify the type of crystal seen on microscopic exam of a urinalysis specimen

Answer 3

Calcium oxalate crystals: colorless octahedral or “envelope”- shaped crystals which look like small squares crossed by intersecting diagonal lines . Appears dumbbell shaped when viewed from the side.

Question 4

Define hydronephrosis

Answer 4

dilation of the renal pelvis and calyces associated with progressive atrophy of the kidney due to obstruction to the outflow of urine

Question 5

What are some ways in which urinary tract obstruction can compromise renal function?

Answer 5

1. High pressure in the renal pelvis-→ decreased renal blood flow-→increased renin-→hypertension
2. elevated intratubular pressure-→tubular dysfunction-→polyuria, nocturia
3. complete obstruction-→elevation in BUN, creatinine, oliguria, anuria i.e post renal azotemia

Question 6

electrolyte abnormalities associated with chronic kidney disease

Answer 6

hypervolemic hyponatremia

hyperkalemia - due to transcellular shift in acidosis and decreased renal excretion

hypocalcemia- due to decreased synthesis of 1-α-hydroxylase and binding of excess phosphorus with calcium

Question 7

Two important consequences of a posterior urethral valve abnormality

Answer 7

A. Chronic kidney disease

B. VUR

Question 8

Staghorn calculi are associated with infection with which specific group of micro-organisms?

Answer 8

Infections by urea-splitting bacteria that Convert urea to ammonia, alkalinizing urine e.g., Proteus

Question 9

One consequence of hydronephrosis on morphology of renal parenchyma

Answer 9

1. Cortical atrophy
2. decreased medullary blood flow

Question 10

Acid base abnormality associated with CKD

Answer 10

Metabolic acidosis

Question 11

Read the scenario below and deduce whether it would result in unilateral/ bilateral hydronephrosis:

Obstruction is above the level of the bladder

Answer 11

Unilateral hydronephrosis

Question 12

How does CKD lead to osteomalacia?

Answer 12

Hypovitaminosis D →hypocalcemia →decreased mineralization of osteoid

[bone biopsy shows exaggeration of osteoid seams]

Question 13

most common etiology of urinary tract obstruction in the newborn male.

Answer 13

Posterior Urethral Valve

Question 14

Top 3 causes of prevalence of end stage renal disease in the United States

Answer 14

1. Diabetes Mellitus
2. Hypertension
3. Glomerulonephritis

Question 15

Mineral disorders in chronic kidney disease

Answer 15

diminished production of 1,25-(OH)2D3 (calcitriol, the activated form of vitamin D),

decreased serum calcium

increased serum phosphorus.

increased PTH (secondary hyperparathyroidism)

Question 16

Simple or complex renal cyst?

Incidentally discovered

Asymptomatic

USG: Well defined, sharp margins, thin walls, anechoic

Answer 16

Simple renal cyst

Question 17

What is the genetic mutation associated with the condition described below?

Autosomal dominant

Bilateral enlarged kidneys

C/F: Proteinuria, polyuria, CKD

Answer 17

Condition described: Autosomal polycystic kidney disease

Every cell carries a germline mutant allele of either PKD1 or PKD2

Question 18

Identify the condition being described

congenital absence or shortening of the intravesical portion of the ureter→ ureter enters the bladder at right angles→reflux of urine into ureters during micturition

Answer 18

Vesicoureteral reflux

Question 19

Why does hypertension occur in chronic kidney disease?

Answer 19

Chronic injury→ nephron loss → decreased perfusion pressure→ release of renin→ increased Angiotensin II→ glomerular hypertension and hyperfiltration

Question 20

One consequence of hydronephrosis on tubular function

Answer 20

impaired concentrating ability

[obstruction to outflow-→high pressure in renal pelvis-→elevated intratubular pressure-→ tubular dysfunction]

Question 21

See the attached image. What’s your diagnosis?

Answer 21

Autosomal Dominant (Adult) Polycystic Kidney Disease

Question 22

List 2 key CVS abnormalities in CKD

Answer 22

1. Hypertension\Heart failure and pulmonary edema
2. Pericarditis

Question 23

Read the scenario described. Is it likely to result in a spastic/ flaccid neurogenic bladder?

Spinal lesions at/above T12 (traumatic spinal cord injuries, multiple sclerosis).

Answer 23

Spastic neurogenic Bladder

Question 24

Which malignancy in women is associated with bilateral hydronephrosis and subsequent renal failure?

Answer 24

Invasive cervical cancer

Question 25

Identify the congenital anomaly shown in the attached image

Answer 25

Horseshoe kidneys

Question 26

Give one term that describes the gross morphology shown in the attached image

Answer 26

Hydronephrosis

Question 27

What would be the morphology of urine crystals associated with a condition wherein there is a genetic defect in the renal reabsorption of amino acids, including cystine?

Answer 27

colorless, refractile, hexagonal plates

Question 28

Most likely etiologic factor for development of calcium oxalate stones

Answer 28

Hypercalcemia, hypercalciuria

Question 29

Name one marker of renal dysfunction that is less dependent on age, sex, race, and muscle mass than serum creatinine

Answer 29

**Serum cystatin C** - Cysteine protease inhibitor that is produced by all nucleated cells. Filtered by the glomerulus but is *not* secreted.

Question 30

List 2 radiopaque renal calculi

Answer 30

Calcium oxalate Magnesium ammonium phosphate (Triple phosphate)

Question 31

Identify the type of crystal see on microscopic exam of a urinalysis specimen

Answer 31

Magnesium ammonium phosphate stones - coffin lid appearance colorless prisms with from three to six sides that frequently have oblique ends

Question 32

**Name this pathologic lesion** Mimics a renal neoplasm when unilateral Composed of islands of undifferentiated mesenchyme, often with cartilage, and immature collecting ducts

Answer 32

Multicystic renal dysplasia

Question 33

Diagnostic method of choice to identify a VUR

Answer 33

Voiding cystourethrography

Question 34

**Read the scenario below and deduce whether it would result in unilateral/ bilateral hydronephrosis:** Obstructive lesions at /below the level of the bladder

Answer 34

Bilateral hydronephrosis

Question 35

Morphology of Bone change due to secondary hyperparathyroidism in CKD

Answer 35

Osteitis fibrosa cystica [increased bone cell activity + peritrabecular fibrosis + cystic brown tumors]

Question 36

Most common type of renal calculi

Answer 36

Calcium oxalate stones

Question 37

Most common extra renal congenital anomaly associated with ADPKD

Answer 37

Liver cysts

Question 38

Type of casts seen on urinalysis in CKD

Answer 38

Waxy casts

Question 39

Type of anemia seen in CKD and why?

Answer 39

§Normochromic and normocytic anemia- due to decreased production of erythropoietin

Question 40

List 3 possible causes of death in patients with ADPKD?

Answer 40

1. 40%-coronary or hypertensive heart disease 2. 25% - infection 3. 15% - ruptured berry aneurysm or hypertensive intracerebral hemorrhage

Question 41

List 2 radiologic hallmarks of CKD

Answer 41

Rugger jersey spine - sclerosis of the vertebral endplates at multiple contiguous levels, producing an alternating dense-lucent-dense appearance , resembling bands of a rugby jersey Pepper pot skull- multiple tiny well-defined lucencies in the calvaria caused by resorption of trabecular bone in [hyperparathyroidism](https://radiopaedia.org/articles/hyperparathyroidism?lang=us)

Question 42

Mutation in the PKHD1 gene Gross appearance of kidney: Smooth surface, Cysts with dilated elongated channels present at right angles to the cortical surface **What protein is coded for by this gene?**

Answer 42

This is ARPKD. It is assoc with mutations in the PKHD1 gene which codes for **fibrocystin**

Question 43

Most common extra renal congenital anomaly associated with ADPKD

Answer 43

Liver cysts

Question 44

3 common causes of death in ADPKD

Answer 44

1. Coronary or hypertensive heart disease 2. Infection 3. Ruptured berry aneurysm or hypertensive intracerebral hemorrhage

Question 45

**What is the diagnosis?** Mutation in the PKHD1 gene Gross: Smooth surface, Cysts with _dilated elongated channels present at right angles to the cortical_ surface

Answer 45

Autosomal Recessive Polycystic Kidney Disease

Question 46

Most likely effect on hydrostatic pressure in outflow obstruction due to BPH

Answer 46

Increased hydrostatic pressure proximal to obstruction Decreased GFR