CIS 1

Question 1

1. Which of the following is an accurate description of a kidney glomerulus? It is an
   
   1. Arteriole-venous shunt
   2. Vascular epithelial organ
   3. Capillary plexus
   4. Endothelial fenestrated structure

Answer 1

Vascular epithelial organ

Question 2

1. Erythropoeitin is exhaggerated where?

Answer 2

• the interstitial cells

Question 3

Erythropoeitin is exhaggerated in the interstitial cells.

What is the primary site of production before birth and after birth?

Answer 3

• BEfore: Liver
• After: Kidney

Question 4

What part of the kidneys are the major sites of production of erthryopoietin?

Answer 4

• peritubular capillary endothelial cells
• peritubular fibroblasts

Question 5

• The term diffuse proliferative glomerulonephritis means

Answer 5

Inflammatory infiltration of the glomerulus

Question 6

1. What is the difference between acute/diffuse/focal proliferative glomerulonephritis?

Answer 6

1. Acute and diffuse are the same
2. Focal: occuring in some glomeruli, not all

Question 7

Generally, the etiology of GN can be ascribed to what?

Answer 7

Immunologic phenomena

Question 8

Benz jones proteins is seen in _____________

Answer 8

• Benz jones proteins is seen in plasma cell dysplasia (tubular damage)

Question 9

• Localized edema => _____ syndrome

Answer 9

nephritic

Question 10

Generalized edema => ____ syndrome

Answer 10

nephrotic

Question 11

1. Gross hematuria => _____ syndrome

Answer 11

nephritic

Question 12

1. _____ on UA => nephritic syndrome

Answer 12

less than 4

Question 13

1. membranoproliferative GN (type 2) occurs most commonly in who and is associated with what?

Answer 13

• Adults
• Chronic antigenemia

Question 14

Patients with:

1. Hep C with cryoglobinemia
2. Malignancies
3. Chronic immune complex disorders

are most likely to have?

Answer 14

Membranoproliferative GN Type 2

Question 15

Membranoproliferative GN (type 2) is characteritized by what type of deposits

Answer 15

Dense electron deposits of C3 (not C1q or C4) in BM

Question 16

What is the prognosis of MPGN Type 2?

Answer 16

Poor prognosis.

Question 17

If we see something that looks like nephrotic syndrome, we think of 3 things

Answer 17

KIDS

• MCD

Adults:

• 1. FSGD (more common)
• 2. Memnranous GN

Question 18

1. 52 YO male has a BP of 140/84, mild periorbital edema, moderate pitting edema of the LE and significant proteinuria on dipstick testing. His CBC shows a white count of 10,000. A 24 hour urine collection shows 3.7gm of protein, primary albumin. PMH indicates that he was a previously healthy except for mild HTN for which he takes HCTZ. The protein in his urine is most likely due to

Answer 18

1. Glomerular disorder (nephrotic syndrome)
   
   1. Bc it is primarily albumin, we are selecting for SMALL proteins

Question 19

1. A 4YO M presents with a CC of sore thorast and dry cough. He has a 4gm of protein reflected in 24 hour urine collection and discernible periorbital edema. You intend to begin steroid therepy but first meet with his parents to consel them. Nased on the most likelyy diagnosis you indicate, tx and prognosis

Answer 19

1. Nephrotic => MCD => tx with steroids => good prognosis

Question 20

Hyposthenuria is what

Answer 20

1. inability to concentrate URINE

Question 21

MCD will show what on EM

Answer 21

• Foot process fusion

Question 22

Mesangial interposition occurs in what

Answer 22

MPGN

Question 23

Sclerotic nodules and capillary loops occurs in

Answer 23

DB

Question 24

MPGN Type 1 shows what on IF?

Answer 24

IgG, C3, C1q and C4 in subendothelium d/t alternative and classical activation

Question 25

In **MCD**, proximal tubules will most like exhibit what? * A. Necrosis * B. Ben Jones Proteins * C. Regengeration * D. Lipid deposition

Answer 25

D: * The cells of the PT are typically laden with **lipids**, reflecting tubular **reabsorption of lipoproteins** (after passing through damaged glomeruli): lipoid nephrosis

Question 26

When can we see regeneration of glomeruli

Answer 26

**as long as BM is intact**

Question 27

* Necrosis occurs in settings of \_\_\_\_\_\_\_\_\_

Answer 27

**acute kidney injury**

Question 28

* Children with **MCD** will have _____ renal function and is assx with \_\_\_\_\_\_\_\_\_\_\_\_\_\_ * ____________ occurs in adults

Answer 28

* Children with MCD will have **normal** renal function and is assx with **exposure to allergens or immunizations** * **Acute renal failure** occurs in adults

Question 29

1. A 7YO child has 3.2 grams of protein in a 24 hour urine collection and lipiduria. His serum albumin is 3.1 gm/dL. He has generalized edema and a sore throat. He fails to respond to steroid thereapy. His BP is not remarkable abut he has a temp of 100.4. Of the following, it is most likely the patient has 1. RPGN type 1 2. Diffuse proliferative (post-infectious) GN 3. Acute Kidney Injury 4. FSGS 5. IgA Npehopathy

Answer 29

1. **FSGS** 1. **​generalized edema is most likely to be nephrotic**

Question 30

* If we think its post-strep, what test could we run that would favor this result?

Answer 30

**ASO titers**

Question 31

* IF is positive for **IgM + C3**. EM shoes **mesengial sclerosi**s and **foot process fusion**. Best diagnosis?

Answer 31

**FSGS**

Question 32

* IF shows **IgM & C3** What is the diagnosis?

Answer 32

**FSGS**

Question 33

1. A 35 YO HIV+ male presents to your San Fran Clinic with nephrotic sundrome and microscopic hematuria. His T4 count is \>500. What is the most likely glomerular disease pattern is most constistnt with

Answer 33

1. **FSGS**

Question 34

What are the secondary causes of **FSGS**?

Answer 34

* **1. HIV** * **2. Heroine** * **3. SS Disease** * **4. Massive obesity**

Question 35

1. A 47YO white male presents to aspen clinic/spa complaing of apainful subpurn across his upper face. He reutls 48 hours with periorgbital edema and grossly discernible hematuria. Sent for evalualtion in Denver, his dx progress and he is diagnosed with nephrotic syndrome. Mostly likely dx is? * SLE * FSGS * MCD * MPGN, Type II * DM

Answer 35

FSGS: **This is the most common nephrotic syndrome in adults in the US**

Question 36

1. **Most nephrotic syndromes are PRIMARY or SECONDARY?**

Answer 36

**PRIMARY**

Question 37

1. A 49 YO male presents with lower extremity pitting edema and oliguria. He has 3.8 gm of proteins on a 24 hour urine collection. His BP is 145/90 mmHg and oral temp of 99.5F. UA is positive for (1/4) for microscopic red cells. PMH is unremakle. Upon biopsy, which is most common?

Answer 37

1. **Membranous GN**

Question 38

1. A 52 YO previously well white male presents to your Ohio clinic with nephrotic syndrome. A renal biopsy is perfomed and has membrananous GN. What is the pt most at risk for? 1. Renal vein thrombosis 2. Acute kidney injury 3. RPGN

Answer 38

1. **Renal vein thrombosis**

Question 39

1. _______ infections are more likely at increased risk infection and coagulation

Answer 39

**NEPHROTIC**

Question 40

1. A 7YO white girl presents to the ED with a fever and gross hematuria. She has distinct periorbital edema nad pitting edema of the forehead. Her mother indicates she has had a facial rash recently. Folloywing adimission a 24 hour urine collection is positive for 1.4 gm of protrein- most of which is albumin. There are fragmented red cells and RBC casts in the urine. She is oliguric. Most likey dx? * Lupus nephritis * Acute proliferative GN * RPGN * FSGS * Interstitial nephritis

Answer 40

1. Acute proliferative GN

Question 41

Why wasnt the last answer NOT **lupus nephritis?**

Answer 41

1. We will typically not make a dx of lupus, **based on a renal biopsy.**

Question 42

1. Acute proliferative GN is treated with what?

Answer 42

**fluid and electrolyte management (95% recovery)**

Question 43

1. 34 YO F is an insulin dependent DB first diagnosed at 5 years of age. She now presents with confusion, increased BUN, creased creating clearance, fibrinous pericarditis , anemia and oliguria. If the patient was subjected to renal biopsy, which glomerular findings are most likely?

Answer 43

1. **Uremia** bc chronic renal failure

Question 44

1. 24 hour urine collection shows 150mg of albumin. What is her urine consistency?

Answer 44

1. **Albuminemia**

Question 45

**Microalbuminuria** is defined as?

Answer 45

urinary excretion of **30-300mg/24** **hours** of albumin in urine

Question 46

**Microalbuminema** is a early sign of what?

Answer 46

* **renal damage in DM**

Question 47

Pt has a GFR of 5mL/min. What stage of renal dysfunction is the patient in

Answer 47

**ESRD** because 5% of normal GFR (100mL/min)

Question 48

1. What can be treated with plasmaprhoresis + immunosuppressive therapy?

Answer 48

**RPGN Type 1;** NOT 2

Question 49

1. 37 YO male presents with HTN, gross hematuria, high fever, N, oliguria, and azotemia. His GFR continues to deteriorate rapidly. Pattern?

Answer 49

* **RPGN**

Question 50

**RPGN** is most likely that this pt will evolve into?

Answer 50

1. **Chronic glomerulosclerosis**

Question 51

* **NL** urine volume is \_\_\_\_\_\_\_

Answer 51

1. NL urine volume is **800-2000mL**

Question 52

1. 52YO AA has a long history of insulin dependent DM (well controlled) and diff to control essential HTN. +3 edema of LE and SOB. His BP is 155/110. Anemia and chronic kidney disease. There is significant proteinuria and urinary volume of 220 ml on 24 hour collection. Imaging shows an increased cardiac silhouette consistent with concentric L ventricular hypertrophy. Other studies indicate several small renal cortical cysts bilaterally. Most likely dx? 1. Npehrotic syndrome 2. Adult polycystic kidnery disease 3. Cystic, sporadic renal cell carcinoma 4. Ischemic cardio/renal vascular disease 5. Primary glomerulopathy

Answer 52

1. **Ischemic cardio/renal vascular disease**

Question 53

1. 47YO white female has a BMI of 29. Alopecia, increased TSH and painless, symmetric and enlargement of thyroid gland. Autoab is consistent with hashimoto throidisits. Lab reports small quantities of intract RBC and 4.2 grams of protein in 24 hour urine collection. Urine protein constituency is due to? Most likely diagnosis?

Answer 53

1. Secondary glomerular disease 2. --\> membranous glomerulopathy

Question 54

**HLA DRB1** is assx with what?

Answer 54

**RPGN Type 1**

Question 55

**HLA DQA1** is associated with what?

Answer 55

**Primary Membranous Glomerupathy**

Question 56

If someone has **solid tumor,** **hep C**, **lymphoma** and develop **glomerulopathies**, what is the MCC?

Answer 56

1. **Membranous glomerulonephropathy** 2. **MPGN Type 1 - secondary**