Focal segmental glomerulosclerosis is scattered (segmental) mesangial

sclerosis that begins in some but not all (focal) glomeruli and eventually
involves all glomeruli. It is most often idiopathic but may be secondary to use of
heroin or other drugs, HIV infection, obesity, sickle cell disease, atheroembolic
disease, or nephron loss (eg, in reflux nephropathy, subtotal nephrectomy, or
renal dysgenesis). It manifests mainly in adolescents but also in young and
middle-aged adults. Patients have insidious onset of proteinuria, mild
hematuria, hypertension, and azotemia. Diagnosis is confirmed by renal biopsy.
Treatment is with angiotensin inhibition and, for idiopathic disease,
corticosteroids and sometimes cytotoxic drugs.

Focal segmental glomerulosclerosis (FSGS) is now the most common cause of idiopathic (or
primary) nephrotic syndrome  among adults in the US. It is especially common in black men.
Though usually idiopathic, FSGS can occur in association with other factors (secondary FSGS),
including drugs (eg, heroin, lithium, interferon alfa, pamidronate, cyclosporine, or nonsteroidal anti-
inflammatory drugs [causing analgesic nephropathy ]), atheroembolic disease affecting the
kidneys, obesity, HIV infection (see HIV-associated nephropathy ), and disorders causing nephron
loss (eg, reflux nephropathy, subtotal nephrectomy, renal dysgenesis [eg, oligomeganephronia:
renal hypoplasia with a decreased number of nephrons]). Familial cases exist.
In FSGS, because charge as well as size ultrafiltration barriers are defective, proteinuria is
typically nonselective, affecting high molecular-weight proteins (eg, Igs) as well as albumin.
Kidneys tend to be small.

Patients with focal segmental glomerulosclerosis (FSGS) commonly present with heavy
proteinuria, hypertension, renal dysfunction, edema, or a combination. Sometimes the only sign is
asymptomatic proteinuria that is not in nephrotic range. Microscopic hematuria is occasionally
present.
Diagnosis is confirmed by renal biopsy , which shows focal and segmental hyalinization of the
glomeruli, often with immunostaining showing IgM and complement (C3) deposits in a nodular and
coarse granular pattern. Electron microscopy reveals diffuse effacement of podocyte foot
processes in idiopathic cases but may show patchy effacement in secondary cases. Global
sclerosis may be visible, along with secondary atrophic glomeruli. Biopsy may be falsely negative
if areas of focal abnormalities are not sampled.
FSGS may recur after kidney transplantation ; proteinuria sometimes returns within hours of
transplantation. Of patients whose transplant was for end-stage renal disease caused by FSGS,
about 8 to 30% lose their graft due to recurrent FSGS; risk is highest in young children, patients
who are not black, patients who develop renal failure < 3 years after disease onset, patients with
mesangial proliferation, and patients with repeat transplants when the diagnosis before the first
transplant was primary FSGS. Familial forms of FSGS rarely recur after transplantation.

Treatment
 Angiotensin inhibition

 Corticosteroids and sometimes cytotoxic drugs for idiopathic focal segmental

glomerulosclerosis (FSGS)

 Kidney transplantation for patients with end-stage renal disease

Treatment often is not effective. Patients with FSGS should be treated with angiotensin inhibition
(with an angiotensin-converting enzyme [ACE] inhibitor or an angiotensin II receptor blocker
[ARB]) unless contraindicated by angioedema or hyperkalemia. Patients with nephrotic syndrome
should be treated with a statin.
In idiopathic FSGS, a trial of immunosuppressive therapy is indicated if proteinuria reaches the
nephrotic range or if renal function worsens, especially if kidney biopsy reveals a tip lesion. In
contrast, patients with secondary FSGS, collapsing FSGS, or advanced tubulointerstitial fibrosis
on renal biopsy are generally not treated with immunosuppression because they tend to not
respond; instead, the primary disorder is treated.

Immunosuppressive therapy
Corticosteroids (eg, prednisone 1 mg/kg orally once a day or 2 mg/kg every other day) are
recommended for at least 2 months, although some experts recommend use for up to 9 months.
Response rates of 30 to 50% have been reported with prolonged therapy and vary by the
histologic classification of FSGS. After a 2-week remission of proteinuria, the corticosteroid is
slowly tapered over ≥ 2 months. Secondary and familial cases, collapsing FSGS, and advanced
tubulointerstitial fibrosis are more likely to be corticosteroid-resistant.
If only slight improvement or relapse occurs with corticosteroid therapy, remission may be induced
with cyclosporine 1.5 to 2 mg/kg orally twice a day for 6 months or,
alternatively, mycophenolate mofetil 750 to 1000 mg orally twice a day for 6 months in
patients >1.25 m2 BSA or 600 mg/ m2 BSA bid up to 1000 mg twice a day.
In patients with contraindications to high-dose corticosteroids (eg, diabetes,
osteoporosis), cyclosporine can be given along with a lower dose of corticosteroids
(eg, prednisone 0.15 mg/kg orally once a day).

Drugs
First-line drugs for hypertension during pregnancy include

 Methyldopa
 Beta-blockers
 Calcium channel blockers

Initial methyldopa dose is 250 mg po twice a day, increased as needed to a total of 2 g a day

unless excessive somnolence, depression, or symptomatic orthostatic hypotension occurs.
The most commonly used beta-blocker is labetalol (a beta-blocker with some alpha-1 blocking
effects), which can be used alone or with methyldopa when the maximum daily dose
of methyldopa has been reached. Usual dose of labetalol is 100 mg twice or 3 times a day,
increased as needed to a total maximum daily dose of 2400 mg. Adverse effects of beta-blockers
include increased risk of fetal growth restriction, decreased maternal energy levels, and maternal
depression.
Extended-release nifedipine, a calcium channel blocker, may be preferred because it is given
once/day (initial dose of 30 mg; maximum daily dose of 120 mg); adverse effects include
headaches and pretibial edema. Thiazide diuretics are only used to treat chronic hypertension
during pregnancy if the potential benefit outweighs the potential risk to the fetus. Dose may be
adjusted to minimize adverse effects such as hypokalemia.
Several classes of antihypertensives are usually avoided during pregnancy:

 ACE inhibitors are contraindicated because risk of fetal urinary tract abnormalities is

increased.
 ARBs are contraindicated because they increase risk of fetal renal dysfunction, lung
hypoplasia, skeletal malformations, and death.
 Aldosterone antagonists (spironolactone and eplerenone) should be avoided because
they may cause feminization of a male fetus.

 Primary: Excessive secretion of PTH due to a disorder of the parathyroid glands

 Secondary: Hypocalcemia due to non-parathyroid disorders leads to chronic PTH
hypersecretion
 Tertiary: Autonomous secretion of PTH unrelated to serum calcium concentration in
patients with long-standing secondary hyperparathyroidism

Primary hyperparathyroidism
Primary hyperparathyroidism is a generalized disorder resulting from excessive secretion of
parathyroid hormone by one or more parathyroid glands. It probably is the most common cause
of hypercalcemia, particularly among patients who are not hospitalized. Incidence increases with
age and is higher in postmenopausal women. It also occurs in high frequency ≥ 3 decades after
neck irradiation.
Primary hyperparathyroidism causes hypercalcemia, hypophosphatemia , and excessive bone
resorption (leading to osteoporosis ). Although asymptomatic hypercalcemia is the most frequent
presentation, nephrolithiasis  is also common, particularly when hypercalciuria occurs due to long-
standing hypercalcemia.
Histologic examination shows a parathyroid adenoma in about 85% of patients with primary
hyperparathyroidism, although it is sometimes difficult to distinguish an adenoma from a normal
gland. About 15% of cases are due to hyperplasia of ≥ 2 glands. Parathyroid cancer occurs in <
1% of cases.

Secondary hyperparathyroidism
Secondary hyperparathyroidism occurs most commonly in advanced chronic kidney disease  when
decreased formation of active vitamin D in the kidneys and other factors lead to hypocalcemia  and
chronic stimulation of PTH secretion. Hyperphosphatemia that develops in response to chronic
kidney disease also contributes.
Other less common causes of secondary hyperparathyroidism include

 Decreased calcium intake

 Poor calcium absorption in the intestine due to vitamin D deficiency
 Excessive renal calcium loss due to loop diuretic use
  Inhibition of bone resorption due to bisphosphonate use

Once the hyperparathyroidism is established, hypercalcemia or normocalcemia may occur. The

sensitivity of the parathyroid glands to calcium may be diminished because of pronounced
glandular hyperplasia and elevation of the calcium set point (ie, the amount of calcium necessary
to reduce secretion of PTH).

Tertiary hyperparathyroidism
Tertiary hyperparathyroidism results when PTH secretion becomes autonomous of serum calcium
concentration. Tertiary hyperparathyroidism generally occurs in patients with long-standing
secondary hyperparathyroidism, as in patients with end-stage renal disease  of several years’
duration.

Hyperparathyroidism in renal failure

Although primary hyperparathyroidism can occur, hyperparathyroidism in patients with renal failure
is usually secondary. Secondary hyperparathyroidism in patients with renal failure can result in a
number of symptoms, including

 Osteitis fibrosa cystica with arthritis, bone pain, and pathologic fractures
 Spontaneous tendon rupture
 Proximal muscle weakness
 Extraskeletal calcifications, including soft tissue and vascular calcification
 Pruritis

Secondary hyperparathyroidism can develop in patients with moderate chronic kidney disease
(estimated glomerular filtration rate < 60 mL/minute), but it is usually more advanced in patients
who require long-term dialysis. The interplay between phosphate retention, hypocalcemia,
decreased active vitamin D concentration, and increased fibroblast growth factor-23 stimulates
parathyroid secretion in patients with renal failure.

Hyperphosphatemia  should be prevented or treated if present. Treatment combines dietary

phosphate restriction and phosphate-binding agents, such as calcium carbonate, calcium
acetate, lanthanum, or sevelamer. Despite the use of phosphate binders, dietary restriction of
phosphate is also needed. Aluminum-containing compounds have been used to limit phosphate
concentration in the past, but they should be avoided, especially in patients receiving long-term
dialysis, to prevent aluminum accumulation in bone resulting in severe osteomalacia.

Thrombotic thrombocytopenic purpura (TTP) is an acute, fulminant disorder

characterized by thrombocytopenia and microangiopathic hemolytic anemia.
Other manifestations may include alterations in level of consciousness and
sometime kidney failure. Diagnosis requires demonstrating characteristic
laboratory test abnormalities, including direct antiglobulin test–negative
hemolytic anemia and reduced levels of ADAMTS13. Treatment is plasma
exchange, corticosteroids, rituximab, and rarely caplacizumab.
Dialysis
Common toxins that may require dialysis or hemoperfusion include
 Ethylene glycol
 Lithium
 Methanol
 Salicylates
 Theophylline


 Renal atheroembolism usually results from manipulation of the aorta during
vascular surgery, angioplasty, or arteriography, and not from spontaneous
atherosclerotic embolization.
 Suspect the diagnosis if renal function deteriorates after the aorta or another large
artery is manipulated.
 Confirm the diagnosis based on clinical findings and occasionally with
percutaneous renal biopsy.
 Treat supportively, correcting modifiable risk factors and, when possible treating the
embolic source; however, the overall prognosis remains poor.

 Patients with SIADH are euvolemic and have low serum osmolality but
inappropriately high urine osmolality.
 Despite the name, not all patients with SIADH have excessive vasopressin.
 Causes include central nervous system disorders, lung disorders (particularly
infections), certain cancers (particularly lung cancer) and certain drugs.
 Water restriction and treatment of cause may be adequate.
 Some patients will also require a vasopressin receptor antagonist or hypertonic
saline.

 Urinary calculi are solid particles in the urinary system. They may cause
pain, nausea, vomiting, hematuria, and, possibly, chills and fever due to
secondary infection. Diagnosis is based on urinalysis and radiologic
imaging, usually noncontrast helical CT. Treatment is with analgesics,
antibiotics for infection, medical expulsive therapy, and, sometimes,
shock wave lithotripsy or endoscopic procedures.

 Hypocitruria (urinary citrate < 350 mg/day [1820 micromol/day]), present in about 40 to
50% of calcium calculi–formers, promotes calcium calculi formation because citrate
normally binds urinary calcium and inhibits the crystallization of calcium salts.
Dialysis
Dialysis is usually initiated at the onset of either of the following:

 Uremic symptoms (eg, anorexia, nausea, vomiting, weight loss, pericarditis, pleuritis)
 Difficulty controlling fluid overload, hyperkalemia, or acidosis with drugs and lifestyle
interventions

These problems typically occur when the estimated GFR reaches ≤ 10 mL/min in a patient without
diabetes or ≤ 15 mL/min in a patient with diabetes; patients whose estimated GFR values are near
these values should be closely monitored so that these signs and symptoms are recognized early.
Dialysis is best anticipated so that preparations can be made and urgent insertion of a
hemodialysis catheter can be avoided. Such preparations usually begin when the patient is in
early to mid stage 4 CKD; preparation allows time for patient education, selection of the type of
dialysis, and timely creation of an arteriovenous fistula  or placement of a peritoneal dialysis
catheter. (For dialysis preparation, see Hemodialysis.)

Systemic sclerosis is a rare chronic disease of unknown cause characterized by

diffuse fibrosis and vascular abnormalities in the skin, joints, and internal
organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and
kidneys). Common symptoms include Raynaud phenomenon, polyarthralgia,
dysphagia, heartburn, and swelling and eventually skin tightening and
contractures of the fingers. Lung, heart, and kidney involvement accounts for
most deaths. Diagnosis is clinical, but laboratory tests support the diagnosis
and aid in prognostication. Specific treatment is difficult, and emphasis is often
on treatment of complications.

Systemic sclerosis is about 4 times more common among women than men. It is most common
among people aged 20 to 50 and is rare in children.
Diagnosis of Systemic Sclerosis
 Clinical criteria
 Antibody testing

Systemic sclerosis should be considered in patients with Raynaud phenomenon, typical

musculoskeletal or skin manifestations, or unexplained dysphagia, malabsorption, pulmonary
fibrosis, pulmonary hypertension, cardiomyopathies, or conduction disturbances. Diagnosis of
systemic sclerosis sine scleroderma should be considered in patients who have unexplained
visceral findings (eg, pulmonary hypertension). Diagnosis of systemic sclerosis can be obvious in
patients with combinations of classic manifestations, such as Raynaud phenomenon (with
abnormal nail-fold capillary findings), dysphagia, and tight skin. However, in some patients, the
diagnosis cannot be made clinically, and confirmatory laboratory tests can increase the probability
of disease but their absence does not exclude it.
Antinuclear antibodies (ANA) are present in ≥ 90% of patients, often with an antinucleolar pattern.
Rheumatoid factor is positive in one third of patients. Antibody to centromeric protein
(anticentromere antibody) occurs in the serum of a high proportion of patients with limited disease.
Patients with generalized (diffuse) systemic sclerosis are more likely than those with limited
disease to have anti-Scl-70 (topoisomerase I) antibodies. RNA polymerase III is associated with
generalized systemic sclerosis, scleroderma renal crisis, and cancer. U3 RNP (fibrillarin) antibody
is also associated with diffuse disease. The most cost-effective way to test for antibodies is to test
for ANA, anti-Scl-70, and anticentromere antibodies first; if results are negative, testing for other
antibodies should be considered based on clinical manifestations.
LAB TEST
Antinuclear Antibody (ANA)

To help establish the diagnosis, clinicians can also consult the American College of Rheumatology
(ACR)/European League Against Rheumatism's (EULAR) classification criteria for systemic
sclerosis.
ACR/EULAR criteria for systemic sclerosis include the following features:

 Skin thickening of the fingers of both hands

 Fingertip lesions (eg, ulcers, pitting scars)
 Telangiectasia
 Abnormal nail-fold capillaries (eg, ectatic blood vessels, dropout areas) on capillaroscopy
examination (eg, seen with an ophthalmoscope or dissecting microscope)
 Pulmonary arterial hypertension and/or interstitial lung disease
 Raynaud phenomenon
 Systemic sclerosis–related autoantibodies (anticentromere, anti–Scl-70, anti–RNA
polymerase III)

These criteria are weighted, in some cases according to subcriteria, and added to generate a
score. Scores above a certain threshold are classified as definite systemic sclerosis.

If lung involvement is suspected, pulmonary function testing, chest CT, and echocardiography can
begin to define its severity. Acute alveolitis is often detected by high-resolution chest CT.

 Key findings in systemic sclerosis include skin and joint changes, Raynaud
phenomenon, and esophageal changes, but life-threatening effects may involve
organs such as the lungs, heart, or kidneys.
 Consider the diagnosis if patients have Raynaud phenomenon, typical
musculoskeletal or skin manifestations, or unexplained dysphagia, malabsorption,
interstitial lung disease and pulmonary fibrosis, pulmonary hypertension,
cardiomyopathies, or conduction disturbances.
 Test for ANA, Scl-70 (topoisomerase I), and anticentromere antibodies.
  Because there is no clear disease-modifying therapy, direct treatment at involved
organs.

A 44-year-old male comes to the clinic 6 months post-renal transplantation. He

complains of fatigue and chills started 2 weeks ago. His temperature is 100 F,
heart rate is 78/min, blood pressure 110/78 mm Hg. On examination, there is a 2
cm nodule on his lower lip that has an irregular border with central ulceration.
What is the most common malignancy in postoperative renal transplant
patients? 

1.B cell lymphomas

2.Skin cancer
3.Cervical cancer
4.T cell lymphomas
Well done! You answered successfully
Teaching Points 

 Transplant patients have a three to fivefold risk of cancer when compared to controls.
Because of immunosuppression, their immune system is unable to stop the abnormal
proliferation of cells as compared to the people with normal immunity.
 The most common malignancies are of the skin with lymphomas being second.
 Cancers in these patients are usually more aggressive.
 Risk factors for malignancy in this group include long term immunosuppression, age, sun
exposure, and smoking.
The majority of post renal transplant cancers have been linked to which virus? 

1.Cytomegalovirus
2.Epstein Barr virus
3.Herpes virus
4.HIV
Well done! You answered successfully
Teaching Points 

 Epstein-Barr virus (EBV) is associated with lymphomas, especially in patients who have
had renal transplants.
 Infection with Hepatitis B and C are risk factors for hepatocellular carcinoma. Human
herpesvirus 8 is linked to Kaposi sarcoma and lymphomas.
 Human papillomaviruses are linked to cervical, penile, and head and neck cancers.
 Antiviral medications are often given to renal transplant patients to reduce infections.
A 65-year-old male presents to the office with complaints of painless gross
hematuria, flank pain, and low-grade fever. The patient says his symptoms began
8 months ago, but he is concerned due to the increased blood in his urine. He has
lost 8 kilograms of weight in the last 4 months. A CT scan shows a 6x6 cm mass
in the right kidney, with focal areas of necrosis and calcifications. After a detailed
evaluation, the patient is diagnosed with renal cell carcinoma. He undergoes a
nephrectomy. After 4 months, he undergoes a renal transplant and is started on
immunosuppressive drugs. The patient is at increased risk of developing which
of the following conditions after a few years? 

1.Arthritis
2.Lymphoma
3.Nephropathy
4.Gastritis
Well done! You answered successfully
Teaching Points 

 Chronic suppression of the immune system is a risk for the development of malignancy.
 Routine cancer surveillance is mandatory in these patients to ensure rapid diagnosis and
treatment.
 Lymphomas are common after 5 to 10 years of immune suppression.
 Transplant patients are at risk for many types of other cancers, including skin, stomach,
liver, vulva, oropharynx, and penis. The risk is diminished for prostate, breast, and
testicular cancers.


A 40-year-old male presents to the emergency department after being involved in

a fight. The patient was shot twice in the right lumbar region. The patient is
bleeding profusely, and his condition is deteriorating. His blood pressure is 80/50
mmHg, and his pulse rate is 120/min. After initial resuscitative measures, an
ultrasound reveals extensive damage to the right kidney. The right renal artery is
damaged, and the kidney sustained a grade 5 renal injury. The patient undergoes
a renal transplant. Which of the following viral pathogens can increase the risk of
complications in this patient? 

1.Adenovirus
2.Retrovirus
3.Polyoma
4.Poxvirus
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Teaching Points 

 Polyomavirus infections are now being recognized with increased frequency as a cause of
graft rejection.
 Most people have been exposed to this virus by adulthood via the respiratory tract.
However, healthy people do not have symptoms.
 In patients with a renal transplant, the polyomavirus is increasingly isolated, but often
graft function is not compromised.
 Widespread use of immunosuppressive agents is thought to cause reactivation of this
virus and result in polyomavirus-associated nephropathy (PVAN).
A 16-year-old male presents to the emergency department following a high-speed
motor vehicle collision. The patient was drunk while driving and was not wearing
a seat belt. He was thrown out of the car through the windshield. The patient is
bleeding profusely from a deep laceration involving his right lumbar region. His
blood pressure is 75/40 mmHg, and his pulse rate is 130/min. After initial
resuscitative measures, an ultrasound reveals extensive damage to the right
kidney. The right renal artery is damaged, and the kidney sustained a grade 5
renal injury. The patient undergoes a renal transplant. Which of the following
need to be monitored regularly to detect organ rejection in this patient? 

1.CD4 count
2.Microalbuminuria
3.Creatinine and potassium
4.Urine output
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Teaching Points 

 The first indication of the rejection of a renal graft can present as elevated creatinine
levels.
 In many cases, the elevation in creatinine is delayed in acute rejection. These patients
may have other features like fever, flank pain, and graft swelling.
 The final diagnosis of graft rejection can only be made with a biopsy. Acute graft
rejection is usually treated with pulse steroids.
 Patients will generally present with a decrease in urine output, fever, or mild elevations in
leucocytes.
A 48-year-old male patient is brought to the emergency department after being hit
by a motorbike while coming back from his office. Initial assessment and
evaluation reveal renal trauma, and thus, a CT scan is done, after which renal
transplant is planned. After the surgery is done, which went uneventful, the
patient is shifted back to the ward. After a few hours, the patient complains of
abdominal pain. Which of the following is the most likely to cause abdominal pain
in this patient? 

1.Ureteral obstruction
2.Inguinal hernia
3.Graft perfusion
4.Malignancy
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Teaching Points 

 Ureteral obstruction can cause pain over the allografted kidney and in the low abdomen.
 Urinary symptoms include decreased urine output with increased BUN and creatinine.
 Ureteral obstruction occurs in 2% to 10% of renal transplant patients post-operatively,
usually presenting within the first few weeks after transplantation but is still relatively
common for the first year after surgery.
 Ureteric ischemia is the most common cause accounting for around 90% of cases.
A 65-year-old male presents with complaints of gross hematuria and flank pain.
The patient says his symptoms began six months ago, but he is concerned due to
the increased blood in his urine. He has lost 8 kilograms of weight in the last four
months. A CT scan shows a 5x6 cm mass in the right kidney, with focal areas of
necrosis and calcifications. After a detailed evaluation, the patient is diagnosed
with renal cell carcinoma. He undergoes a nephrectomy. After two months, he
undergoes a renal transplant and is started on immunosuppressive drugs. On
discharge, patient education should be emphasized on which of the following? 

1.Signs and symptoms of infection

2.Monitoring urine input and output
3.Measuring abdominal girth daily
4.Exercise and lifestyle modification
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Teaching Points 

 Transplant teaching should contain information about their medications, signs of

infection, rejection, daily monitoring weight, blood pressure, necessary diet, and follow
up medical instructions.
 The most important is that the patient should be familiar with the signs and symptoms of
infection. As early detection and presentation result in decreased complications and an
improved prognosis.
 Transplant rejection usually presents with nonspecific symptoms.
 Most transplanted kidneys last about 10 to 15 years.
A 65-year-old male presents with complaints of gross hematuria and flank pain.
The patient says his symptoms began six months ago and has not visited his
provider regularly. The patient has worked in a dye factory for 20 years. He has
lost 8 kilograms of weight in the last four months. A CT scan shows a 5x6 cm
mass in the right kidney, with focal areas of necrosis and calcifications. After a
detailed evaluation, the patient is diagnosed with renal cell carcinoma. He
undergoes a nephrectomy. The patient is started on immunosuppressive therapy.
He presents after 1-month complaining of fatigue and anorexia. An ultrasound of
the remaining kidney shows compensatory renal parenchymal hypertrophy. The
patient has a decreased glomerular filtration rate. After much evaluation, it is
decided that he needs a kidney transplant. The patient's daughter wants to
donate her kidney. Which kidney will be used as a graft for renal transplant, and
why? 

1.Left kidney as it is larger in size

2.Right kidney as it is larger in size
3.Left kidney as it has a longer renal artery
4.Left kidney as it has a longer renal vein
Well done! You answered successfully
Teaching Points 

 For transplants, the left kidney is usually selected as a graft as it has a longer length of the
vein. In most cases, the left gonadal vein drains into the left renal vein inferiorly.
  The left renal vein is a few centimeters longer than the right vein as it has to cross the
midline from the left side to reach the inferior vena cava.
 The left renal vein passes just below the origin of the superior mesenteric artery to reach
the inferior vena cava.
 On the right side, the gonadal and renal veins separately drain into the inferior vena cava.

Five years following a cadaveric renal transplant, a 56-year-old white male on

azathioprine and daily prednisone presents for his regular six-month full-body skin
exam. Since his kidney transplantation, he has been treated for more than ten
squamous cell carcinomas (SCC) over his chest, neck, and upper extremities. In
the interest of decreasing the patient's risk for future cutaneous carcinoma, a
discussion ensues about altering his immunosuppression regimen. Which of the
following would be the best recommendation?

1.Discontinue azathioprine, start cyclosporine

2.Add voriconazole to his current regimen
3.Add mycophenolate mofetil to his current regimen
4.Discontinue azathioprine, start sirolimus
Well done! You answered successfully
Teaching Points 

 The incidence of squamous cell carcinomas (SCC) in solid organ transplant recipients is
correlated with higher levels of immunosuppressive medications. 
 These cancers tend to be more aggressive and are associated with greater tumor depth, a
higher probability of recurrence, and a greater incidence of perineural or lymphatic
invasion.
 Randomized controlled trials comparing sirolimus to other immunosuppressive regimens,
including azathioprine, cyclosporine, and prednisone, have shown a lower incidence of
SCC. However, it is not without serious adverse events. 
 The most common adverse events of sirolimus are edema, acneiform eruption, aphthous
ulcers, and proteinuria, causing some patients to discontinue it.

A patient undergoes a renal transplant. During the procedure, the surgeon uses a
vessel to attach the recipient’s renal artery to the transplanted kidney. This vessel is
derived at the level of the sacroiliac joint. Which of the following vessels had been
used in this procedure?
1.Common iliac artery
2.External iliac artery
3.Internal iliac artery
4.Inferior epigastric artery
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Teaching Points 

 External iliac artery bifurcates from the common iliac artery at the level of the sacroiliac
joint.
 The external iliac artery travels anteroinferior with the psoas major muscle.
 The branches of the external iliac artery are the inferior epigastric artery, deep circumflex
iliac artery, and the femoral artery.
 Below the inguinal ligament, the external iliac artery continues as the femoral artery.

A 16-year-old male deceased donor renal transplant (DDRT) recipient presents to

the office 6 months after transplant complaining of constitutional symptoms
including cyclical fevers, night sweats, and malaise. His EBV viral load is
40,000/mL. What is the most likely diagnosis?

1.Burkitt cell lymphoma

2.Peripheral T-cell lymphoma
3.Diffuse large B-cell lymphoma
4.Plasma cell lymphoma
Well done! You answered successfully
Teaching Points 

 PTLD is most commonly associated with EBV viremia as EBV transforms B-cells.
 More than 80% occur in the first year after transplant.
 Those most at risk include young patients, those that are more heavily
immunosuppressed, and those who were seronegative pre-transplant and received a
seropositive donor allograft. Diffuse large B-cell lymphoma is the most common B-cell
lymphoma seen in monomorphic PTLD.
 T-cell lymphomas and NK-cell lymphomas are rare.

A 65-year-old woman presents to the emergency department 4 months following

kidney transplant with diminished urine output. Ultrasound of the transplanted
kidney demonstrates moderate-severe hydronephrosis without any evidence of
peri-nephric collections. She currently takes tacrolimus and prednisone. Post void
residual is negligible. What is the most likely diagnosis?

1.Dehydration
2.Tacrolimus toxicity
3.Ureteral stricture
4.Rejection
Well done! You answered successfully
Teaching Points 

 Urinary obstruction may be the result of urinary bladder dysfunction (particularly in

patients with diabetes and autonomic neuropathy) or BPH in men. It may also result from
ureteral scarring most commonly secondary to ischemia or kinking, but can also be
caused by BK virus.
 With a negligible PVR in the setting of moderate-severe hydronephrosis, the diagnosis of
ureteral stricture or obstruction becomes more likely particularly following stent removal
~ 4-6 weeks post-transplant.
 The incidence of ureteral stricture following a kidney transplant is quoted as anywhere
between 1-6.5%.
 Ultrasound shows hydronephrosis without signs of rejection involving the graft
parenchyma or defects in its blood flow.

A 65-year-old woman presents to the clinic for follow up. She underwent a renal
transplant six months ago but has been noticed to have a diminishing GFR.
Transplant allograft biopsy is suggestive of tacrolimus toxicity. A decision is made to
switch tacrolimus to belatacept. Which of the following is the most appropriate test
to perform prior to this medication change in this patient?

1.CMV status
2.EBV status
3.BK viral load
4.Respiratory viral panel
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Teaching Points 

 Belatacept is contraindicated in patients who are EBV-seronegative pre-transplant as it

increases the risk of EBV infection and subsequent EBV-associated PTLD in these
patients.
  Belatacept is a T-cell costimulatory blocker.
 It is often considered in patients with calcineurin inhibitor (CNI) toxicity on tacrolimus
and/or those with proteinuria/glomerular injury on sirolimus.
 The other tests are unlikely to affect the decision.

A 39-year-old woman has volunteered to donate a kidney for transplantation. She is

otherwise healthy with no medical history or kidney disease. A CT angiogram is
performed to delineate the anatomy of her kidneys. It reveals a left kidney 12 cm in
length and a right kidney 8 cm in length. There are no contraindications to donation
based on her other work-up. Which of the following is the next most appropriate
course of action?

1.Cancel the transplant based on anatomy

2.Use the 12 cm kidney for donation
3.Use the 8 cm kidney for donation
4.Order a function nuclear medicine scan
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Teaching Points 

 Differences in size between bilateral kidneys are normal and not a reason to cancel
surgery.
 In the case of a discrepancy in size between the potential donor kidneys, the smaller of
the two kidneys would be chosen preserving the most nephron mass for the donor.
 The chief concern in living donor kidney transplant is to avoid unnecessary harm or risk
in the donor. In the case of a discrepancy in size between the potential donor kidneys, the
smaller of the two kidneys would be chosen, preserving the most nephron mass for the
donor.
 Further imaging is not indicated.

A 56-year-old man with end-stage renal disease is being evaluated for deceased
donor renal transplantation. The etiology of his end-stage renal disease is hemolytic
uremic syndrome. Which of the following is the most appropriate medication to
mitigate recurrence in this patient?

1.Eculizumab
2.Rituximab
3.Alemtuzumab
4.Basiliximab
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Teaching Points 

 Eculizumab is a terminal complement inhibitor that largely preserves the disease-

preventing functions of the proximal complement system while impeding properties of
C5 which promote inflammation and cell destruction.
 It is often used peri-operatively for varying course lengths to protect against recurrence in
the recipient as HUS leads to uncontrolled, excessive complement activation.
 Meningococcal disease is a common adverse effect of eculizumab.
 The other listed medications are not known to help prevent the recurrence of HUS.

A 45-year-old woman who recently underwent kidney transplantation is admitted to

the hospital 2 weeks post-operatively with subjective fevers and chills. She has a
fever of 101.5 F. Her physical exam is otherwise unremarkable. What is the most
likely source of fever in this patient?

1.CMV infection
2.Urinary tract infection
3.Valley fever (coccidioidomycosis)
4.Adenovirus
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Teaching Points 

 Within the first month of transplant, common nosocomial, bacterial infections remain the
most prevalent in the transplant population.
 Beyond 6 months, community-acquired and/or persistent infections predominant.
 Unconventional or opportunistic infections become more common in the months that
follow and can include Listeria, pneumocystis, CMV, VZV, adenovirus, or fungal
infections.
 Suspicion for CMV retinitis or colitis should also remain high.

A 38-year-old man is brought to the emergency department after being involved in

a bar fight. The patient was shot twice from a close range. His blood pressure is
90/50 mmHg, and his pulse rate is 120/min. On examination, the patient has two
bullet entry wounds in his right flank. An ultrasound reveals a grade V kidney injury.
After a detailed evaluation, the patient undergoes a right renal transplant. During the
procedure, as the surgeon makes an anastomosis between the graft and the
recipient's blood vessels, the kidney rapidly becomes cyanotic and mottled. If a
biopsy of this kidney is performed at this point, which of the following changes is
most likely to be seen?

1.Intense lymphocytic infiltration

2.Vasculitis and fibrin thrombi within the glomeruli
3.Arterial obliterative intimal fibrosis
4.Tubular necrosis and atrophy
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Teaching Points 

 Hyperacute transplant rejection has occurred in this patient.

 Antigen-antibody complexes target the vascular endothelium, resulting in thrombotic
occlusion of the capillaries. This further results in fibrinoid necrosis and deposition of
fibrin within the glomeruli.
 Hyperacute rejection occurs due to the presence of preformed antibodies in the recipient
against the donor organ.
 Thrombosis is fortunately rare but is associated with a high risk of graft loss.

In a patient with a renal transplant, a fluid collection is seen around the graft. What
will be the investigation of choice in such a case?

1.Ultrasound
2.X-ray
3.Scintigraphy
4.Aspiration biopsy
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Teaching Points 

 Aspiration biopsy provides a definite diagnosis in the case of perigraft fluid collection.
 Perigraft mass or fluid collection can be due to a number of causes.
 Hematoma and abscess masses look similar on ultrasound.
 Lymphocele and urinoma are fluid collections that look alike on ultrasound and can be
differentiated only after the biopsy.

Vascular examination for the renal transplant includes which of the following
vessels?
1.Iliac artery, renal artery, and portal vein.
2.Iliac artery, intrarenal arteries, and renal vein.
3.Renal veins, iliac veins, and inferior vena cava.
4.Inferior vena cava, intrarenal arteries, and portal vein.
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Teaching Points 

 The vascular examination is carried out before renal transplant.

 It includes an examination of the iliac artery, renal artery, intrarenal arteries, renal vein,
and iliac vein.
 Perfusion study with power Doppler is also done sometimes.
 Inferior vena cava and portal vein are not included in the examination.

Where are transplanted kidneys placed in the body?

1.Renal fossa
2.Pelvis
3.Morisson pouch
4.Anterior to the bladder
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Teaching Points 

 The donor's kidney is usually placed in the pelvis.

 It is placed anterior to the psoas muscle and along the iliopsoas margin.
 The ureter of the donor's kidney is anastomosed to the bladder.
 Transplanted kidneys are assessed within 48 hours postoperatively.
A 25-year-old male presents to the emergency department for mild epigastric
pain, nausea, vomiting, and gross hematuria for 24 hours. He denies any
significant past medical history or intake of prescription medications. He is
overall well-appearing, vitals are; heart rate 104 beats per minute, blood pressure
96/54 mmHg, respiratory rate 13 breaths per minute, oxygen saturation of 99
percent at room air, temperature 98.7 degrees Fahrenheit. Examination reveals
some mild bilateral flank tenderness and mild epigastric tenderness without
guarding or rebound. The initial medical workup reveals creatinine of 2.2 mg/dL,
potassium of 4.1 mEq/L, anion gap of 22 mEq/L. On reevaluation, the patient
states he has a leg injury and has been taking 800mg of ibuprofen every 4 hours
for a week to continue working out. Which of the following is the cause of the
hematuria in this patient? 
1.NSAID overdose can cause bleeding, this is causing bleeding from his bladder
2.NSAIDs can cause kidney stones explaining the flank pain and hematuria
3.Renal papillary necrosis can be caused by NSAID overdose
4.NSAIDs can cause prostatitis leading to hematuria
Well done! You answered successfully
Teaching Points 

 Renal papillary necrosis can be seen in NSAID overdoses in the setting of renal failure.
Classical complaints are flank pain and hematuria.
 Renal papillary necrosis presentation is due to the passage of necrotic renal tissue.
 The primary cause of renal injury is believed to be due to the inhibition of prostaglandins.
This causes vasoconstriction, decreasing renal perfusion. 
 Overdoses do not need to be from one grand gesture from suicidal ideation or other.
Inquire about the frequency use of over the counter medications. This is particularly
important in salicylate and acetaminophen ingestions where chronic overuse can lead to
overdose sequelae, but laboratory values do not help with the diagnosis.