Transcript Block 8 Board Review

Block 8 Board Review
Renal Disorders
7Feb14
Chauncey D. Tarrant, M.D.
Chief of Residents 13-14
Pediatrics In Review Articles
QUIZ!!!
Nephrotic Syndrome
• What are the presenting signs and symptoms
of Minimal Change Nephrotic Syndrome?
• What are the presenting signs and symptoms
of Minimal Change Nephrotic Syndrome?
Heavy Proteinuria, Hypoalbuminemia,
Hyperlipidemia, Edema
-Anorexia, Irritability, Fatigue, Abdominal
discomfort, diarrhea
• What lab Findings might you find in a child
with MCNS?
• What lab Findings might you find in a child
with MCNS?
Hypoalbuminemia, hyperlipidemia, hyponatremia
(Why??), low serum calcium (iCa is normal), UPr/Cr
ratio >3
• What would the initial treatment for a child
with MCNS be??
• What would the initial treatment for a child
with MCNS be??
Prednisone
• When would you consider other agents
(alkylating agents)??
• When would you consider other agents
(alkylating agents)??
frequent relapsers
• What would be your differential diagnosis for
nephrotic syndrome?
• If hematuria is a factor???
• What would be your differential diagnosis for
nephrotic syndrome?
MCNS, FSGS, CGN
MPGN, Lupus Nephritis, IgA Nephritis
• What are the complications of Nephrotic
Syndrome?
• What are the complications of Nephrotic
Syndrome?
Thromboembolic state, peritonitis,
cellulitis, meningitis, pneumonitis, growth
stunting, reduced mineral bone density
A 4-year-old boy presents with swelling of the face and
extremities of 2 days’ duration. Physical
examination reveals an otherwise happy child who has
swelling of the face and pitting edema of all extremities.
Vital signs and the rest of the physical examination
findings are normal. Urinalysis shows 4+ proteinuria and
5 red blood cells per high-power field. Of the following,
the most likely abnormality on histologic examination of
this boy’s kidney is:
A. Deposition of immunoglobulin A in mesangium.
B. Diffuse thickening of glomerular capillary walls.
C. Fusion of epithelial foot processes only.
D. Mesangial cell proliferation and thickening of
Bowman capsule.
E. Scar tissue in segments of some glomeruli.
A 4-year-old boy presents with swelling of the face and
extremities of 2 days’ duration. Physical
examination reveals an otherwise happy child who has
swelling of the face and pitting edema of all extremities.
Vital signs and the rest of the physical examination
findings are normal. Urinalysis shows 4+ proteinuria and
5 red blood cells per high-power field. Of the following,
the most likely abnormality on histologic examination of
this boy’s kidney is:
A. Deposition of immunoglobulin A in mesangium.
B. Diffuse thickening of glomerular capillary walls.
C. Fusion of epithelial foot processes only.
D. Mesangial cell proliferation and thickening of
Bowman capsule.
E. Scar tissue in segments of some glomeruli.
A 4-year-old boy presents with swelling of the face and
extremities of 2 days’ duration. Physical examination
reveals an otherwise happy child who has swelling of
the face and pitting edema of all extremities. Vital signs
and the rest of the physical examination findings are
normal. Urinalysis shows 4+ proteinuria and 5 red blood
cells per high-power field. Of the following, the best
indicator of good outcome for this child is:
A. Normal C3 complement value.
B. Normal serum creatinine concentration.
C. Resolution of symptoms with prednisone treatment.
D. Serum cholesterol less than 500 mg/dL (13.0
mmol/L).
E. Urine protein:creatinine ratio less than 5.
A 4-year-old boy presents with swelling of the face and
extremities of 2 days’ duration. Physical examination
reveals an otherwise happy child who has swelling of
the face and pitting edema of all extremities. Vital signs
and the rest of the physical examination findings are
normal. Urinalysis shows 4+ proteinuria and 5 red blood
cells per high-power field. Of the following, the best
indicator of good outcome for this child is:
A. Normal C3 complement value.
B. Normal serum creatinine concentration.
C. Resolution of symptoms with prednisone treatment.
D. Serum cholesterol less than 500 mg/dL (13.0
mmol/L).
E. Urine protein:creatinine ratio less than 5.
You are treating a 9-year-old girl who has
nephrotic syndrome with prednisone. Which of
the following is the strongest indication for
performing renal biopsy?
A. Lack of response to therapy after 1 week.
B. Microscopic hematuria showing more than 5
red blood cells per high-power field in urine.
C. Reduced serum concentration of C3
complement.
D. Serum albumin less than 1.5 g/dL (15 g/L).
E. Urine protein:creatinine ratio of 1 at
presentation.
You are treating a 9-year-old girl who has
nephrotic syndrome with prednisone. Which of
the following is the strongest indication for
performing renal biopsy?
A. Lack of response to therapy after 1 week.
B. Microscopic hematuria showing more than 5
red blood cells per high-power field in urine.
C. Reduced serum concentration of C3
complement.
D. Serum albumin less than 1.5 g/dL (15 g/L).
E. Urine protein:creatinine ratio of 1 at
presentation.
A 6-year-old girl is admitted for swelling of her
face and extremities. Findings on her physical
examination and vital signs are normal except for
generalized anasarca. Urinalysis shows 4+ protein
with no casts or red blood cells. Serum albumin is
1.3 g/dL (13 g/L), cholesterol is 550 mg/dL (14.2
mmol/L), and creatinine is 0.4 mg/dL (35.4
mcmol/L). This patient is at greatest risk for:
A. Centrilobular hepatic necrosis.
B. Cerebral edema.
C. Congestive heart failure.
D. Myoglobinuric renal failure.
E. Peritonitis.
A 6-year-old girl is admitted for swelling of her
face and extremities. Findings on her physical
examination and vital signs are normal except for
generalized anasarca. Urinalysis shows 4+ protein
with no casts or red blood cells. Serum albumin is
1.3 g/dL (13 g/L), cholesterol is 550 mg/dL (14.2
mmol/L), and creatinine is 0.4 mg/dL (35.4
mcmol/L). This patient is at greatest risk for:
A. Centrilobular hepatic necrosis.
B. Cerebral edema.
C. Congestive heart failure.
D. Myoglobinuric renal failure.
E. Peritonitis.
A 4-year-old boy is seen in the emergency
department because of recurrent facial swelling.
The mother reports that the boy has been
evaluated by her pediatrician on several occasions
with a similar complaint. Each time the boy was
treated with 3- to 5-day courses of an
antihistamine or oral steroid. The mother
maintains full adherence with these treatment
recommendations. Physical examination shows a
healthy-appearing boy who has normal growth
parameters. He is afebrile with a respiratory rate
of 18 breaths/min, heart rate of 84 beats/min, and
blood pressure of 90/60 mm Hg. The only finding
of significance is facial puffiness and periorbital
edema (Item Q232).
Of the following, the MOST appropriate next step
is to
A. obtain C1 esterase concentration
B. obtain a specimen for urinalysis
C. prescribe a 5-day course of diphenhydramine
and prednisone
D. reassure the mother and discharge the patient
home
E. refer the patient for an allergy evaluation
Of the following, the MOST appropriate next step
is to
A. obtain C1 esterase concentration
B. obtain a specimen for urinalysis
C. prescribe a 5-day course of diphenhydramine
and prednisone
D. reassure the mother and discharge the patient
home
E. refer the patient for an allergy evaluation
Renal Dysplasia
• Neonate with unilateral flank mass…..
• Neonate with unilateral flank mass…..
– Think Multicystic Dysplastic Kidney
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Associated with Hypertension
Associated with Intracranial Aneurysms
Ultrasound is the preferred diagnostic method
3rd to 5th decades
Autosomal Dominant Polycystic
Kidney Disease
• Neonates/Children/Adolescents
• Associated with Portal Hypertension
• Bilaterally enlarged kidneys
Autosomal Recessive Polycystic
Kidney
Disease
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Polydypsia
Polyuria
Anemia
Growth Failure
Retinal Disease
Juvenile Nephronopthisis
Syndromes/Associations
Potter’s Sequence
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Imperforate Anus
Vertebral Abnormalities
TE fistula
Renal Abnormalities
Limb abnormalities (radial agenesis)
Cardiac Defects (VSD, ASD, ToF)
VATER/VACTERL Association
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Male Predominance
Hydronephrosis
Undescended Testes
Absence of Abdominal Wall Muscles
Prune Belly Syndrome
• Congenital Nephropathy
• Wilms Tumor
• Gonadal Dysgenesis
Denys Drash
• Congenital Nephropathy
• Wilms Tumor
• Gonadal Dysgenesis
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GU abnormalities
Wilm’s Tumor
Chromosome 11 affected
Aniridia
“Retardation”
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Wilm’s Tumor
Aniridia
GU abnormalities
Retardation
PREP
A 14-year-old boy is brought to the emergency
department after being struck by a car. On arrival, he is
unresponsive and hypotensive. You intubate him
endotracheally, place two large-bore intravenous lines,
and infuse 3 L of 0.9% saline. Following these measures,
his heart rate is 100 beats/min and blood pressure is
100/60 mm Hg. On secondary survey, you find a large
swelling on the back of his head, a distended abdomen,
blood at the urethral meatus, guaiac-positive stool, and a
right femur fracture.
Of the following, the procedure that is
CONTRAINDICATED in this patient is
A. diagnostic peritoneal lavage
B. femoral traction splint placement
C. orogastric tube placement
D. retrograde urethrography
E. urethral catheter placement
A. diagnostic peritoneal lavage
B. femoral traction splint placement
C. orogastric tube placement
D. retrograde urethrography
E. urethral catheter placement
A 17-year-old sexually active girl presents for a
follow-up evaluation after her third episode of a
urinary tract infection. She is currently
asymptomatic. The results of renal
ultrasonography and voiding cystourethrography
are negative. She asks you how to prevent
further episodes.
Of the following, you are MOST likely to advise
her to
A. drink cranberry juice frequently
B. increase her daily water intake
C. make sure to void after intercourse
D. self-medicate with antibiotics for 3 days when
symptomatic
E. use single-dose postcoital antibiotic prophylaxis
A. drink cranberry juice frequently
B. increase her daily water intake
C. make sure to void after intercourse
D. self-medicate with antibiotics for 3 days when
symptomatic
E. use single-dose postcoital antibiotic prophylaxis
A 5-year-old girl presents with mild flank pain. She has no
history of fever, trauma, gross hematuria, frequency,
urgency, or dysuria. Physical examination of the afebrile
child reveals a heart rate of 90 beats/min, respiratory rate
of 20 breaths/min, blood pressure of 106/62 mm Hg, and
normal growth parameters. There are no other findings of
note. A dipstick urinalysis reveals a specific gravity of
1.020; pH of 8.5; 2+ protein; and negative for blood,
leukocyte esterase, and nitrite. A urine protein-tocreatinine ratio performed on this specimen is
subsequently reported as 0.02.
Of the following, the MOST likely explanation for this
girl’s urinary findings is
A. alkaline urine
B. minimal change disease
C. orthostatic proteinuria
D. urinary tract infection
E. urolithiasis
A. alkaline urine
B. minimal change disease
C. orthostatic proteinuria
D. urinary tract infection
E. urolithiasis
You are examining a 1-day-old term male infant
during rounds in the newborn nursery and palpate
a right upper quadrant abdominal mass. The
remainder of the examination findings are normal.
The infant’s mother is an otherwise healthy 22year-old gravida 1, para 1, woman who received no
prenatal care. You note on the chart that the infant
voided at 6 hours of age.
Of the following, the MOST likely diagnosis in this
infant is
A. posterior urethral valves
B. ureterocele
C. ureteropelvic junction obstruction
D. urolithiasis
E. Wilms tumor
A. posterior urethral valves
B. ureterocele
C. ureteropelvic junction obstruction
D. urolithiasis
E. Wilms tumor
You are examining a newborn who has wrinkling
of the abdominal wall skin. His mother recalls
her obstetrician mentioning that her “fluid was
low.” The infant was born at 37 weeks’
gestation, and his birthweight was appropriate
for gestational age.
Of the following, the MOST likely additional
findings expected in this infant are
cryptorchidism and
A. bilateral hydronephrosis
B. hypospadias
C. nephrocalcinosis
D. polycystic kidney disease
E. unilateral renal agenesis
A. bilateral hydronephrosis
B. hypospadias
C. nephrocalcinosis
D. polycystic kidney disease
E. unilateral renal agenesis
A 3-year-old boy presents with difficulty voiding. His mother states
that he appears fussy and seems to strain when trying to void. He is
otherwise well and has no history of fever, vomiting, difficulty feeding,
swelling, or gross hematuria. He does have a history of acute
pyelonephritis at 10 months of age. Ultrasonography and voiding
cystourethrography (VCUG) results were reported as normal. His
mother noted that he had some visible blood in his urine immediately
after undergoing his VCUG, which cleared several hours later. The boy
was toilet trained at 2½ years of age. He has had no recent urinary
tract infections. Physical examination reveals an uncircumcised male
who has easily retractable foreskin and no sacral dimples or hair tufts
on the back. Urinalysis results include a specific gravity of 1.020; pH of
6; and no blood, protein, leukocyte esterase, or nitrite. Microscopy is
negative.
Of the following, the MOST likely explanation for this boy’s difficulty
voiding is
A. bladder stone
B. cystitis
C. neurogenic bladder
D. posterior urethral valves
E. urethral stricture
A. bladder stone
B. cystitis
C. neurogenic bladder
D. posterior urethral valves
E. urethral stricture
A 7-year-old girl complains of 1 day of dysuria, vaginal
itching, and perineal pain without fever or vomiting. She
does not have urgency, frequency, or enuresis, but she
does report a light yellow discharge. She denies any type
of trauma, including sexual abuse. Her vital signs and
abdominal examination findings are normal, and she has
no flank tenderness. Perineal inspection shows a minimal
amount of mucoid vaginal discharge and moderate
erythema of the vestibule. There is no evidence of
trauma. A urine dipstick evaluation is negative except for
trace leukocyte esterase.
Of the following, the MOST appropriate next step in the
evaluation/treatment of this patient is
A. a course of oral antibiotics for urinary tract infection
B. application of bland emollients as needed for symptom
relief
C. topical anticandidal cream for 7 days
D.urine testing for gonorrhea and chlamydia
E. vaginoscopy
A. a course of oral antibiotics for urinary tract infection
B. application of bland emollients as needed for
symptom relief
C. topical anticandidal cream for 7 days
D.urine testing for gonorrhea and chlamydia
E. vaginoscopy
An 8-year-old boy presents with cola-colored urine without blood clots. He was well
until 2 days ago, when he developed a sore throat with upper respiratory tract
infection symptoms. He denies any dysuria, frequency, urgency, flank pain, or trauma.
On physical examination, his temperature is 37.8°C, heart rate is 84 beats/min,
respiratory rate is 18 breaths/min, and blood pressure is 118/78 mm Hg. He has no
costovertebral tenderness, abdominal tenderness, or edema. The urinalysis reveals:
Specific gravity, 1.025
pH, 6.0
3+ blood
3+ protein
1+ leukocyte esterase
Nitrite, negative
Microscopy shows more than 100 red blood cells/high-power field (hpf) and 5 to 10
white blood cells/hpf. Other laboratory findings include:
Blood urea nitrogen, 24 mg/dL (8.6 mmol/L)
Creatinine, 0.9 mg/dL (79.6 mcmol/L)
Complement component 3 (C3), 140 mg/dL (normal, 80 to 200 mg/dL)
Complement component 4 (C4), 30 mg/dL (normal, 16 to 40 mg/dL)
Antinuclear antibody, negative
Of the following, the MOST likely diagnosis is
A. acute pyelonephritis
B. immunoglobulin A glomerulonephritis
C. postinfectious glomerulonephritis
D. urolithiasis
E. viral cystitis
A. acute pyelonephritis
B. immunoglobulin A glomerulonephritis
C. postinfectious glomerulonephritis
D. urolithiasis
E. viral cystitis
You recently diagnosed Burkitt lymphoma in one of your
patients. He is an 8-year-old boy, and his pediatric oncologist
is planning to treat him with CODOX-M (cyclophosphamide,
doxorubicin, vincristine, methotrexate intravenously and
cytarabine and methotrexate intrathecally) for cycles 1 and 3
and IVAC (ifosfamide, etoposide, cytarabine intravenously and
methotrexate intrathecally) for cycles 2 and 4. As her son’s
longstanding pediatrician, the boy’s mother asks you about
the adverse effects of the therapy. Because her cousin is
undergoing dialysis, she asks you specifically which
medication is most likely to damage her son’s kidneys.
Of the following, the MOST nephrotoxic medication being
prescribed is
A. cyclophosphamide
B. cytarabine
C. doxorubicin
D. etoposide
E. ifosfamide
A. cyclophosphamide
B. cytarabine
C. doxorubicin
D. etoposide
E. ifosfamide
A 15-year-old girl presents with gross hematuria (bright red blood with clots)
accompanied by sharp left-sided back pain. She denies fever, dysuria, frequency,
urgency, or trauma. On physical examination, her temperature is 37.3°C, heart rate is
90 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 116/72 mm
Hg. You note no costovertebral angle tenderness, suprapubic tenderness, or edema.
Urinalysis reveals:
Specific gravity, 1.020
pH, 6
3+ blood
Trace protein
Leukocyte esterase, negative
Nitrite, negative
Microscopy documents 20 to 50 red blood cells/high-power field (hpf) and 5 to 10
squamous epithelial cells/hpf. Abdominal ultrasonography shows mild dilation of the
collecting system on the left, with some debris in the bladder. You suspect urolithiasis.
Of the following, the study that is MOST likely to establish the diagnosis is
A. abdominal computed tomography scan
B. diethylene-triamine-penta-acetic acid (DTPA)
furosemide renal scan
C. magnetic resonance urography
D. random urine sample for calcium and creatinine
E. 24-hour urine collection for calcium and creatinine
A. abdominal computed tomography scan
B. diethylene-triamine-penta-acetic acid (DTPA)
furosemide renal scan
C. magnetic resonance urography
D. random urine sample for calcium and creatinine
E. 24-hour urine collection for calcium and creatinine
A 3-week-old term infant presents with vomiting, increased fussiness,
and poor weight gain. In answer to your questioning, his mother states
he has a vigorous suck, decreased tearing, and several wet diapers per
day. The infant is formula-fed. On physical examination, he is fussy and
difficult to console. His weight is 2.9 kg (birthweight was 3.2 kg),
temperature is 37.3°C, heart rate is 180 beats/min, respiratory rate is
40 breaths/min, and blood pressure is 76/36 mm Hg. His anterior
fontanelle is flat and mucous membranes are tacky. His chest is clear
to auscultation, he has tachycardia without murmurs, and he has no
abdominal masses. Skin evaluation reveals reduced turgor, and
capillary refill time is approximately 3 to 4 seconds. Laboratory results
include: sodium of 168 mEq/L (168 mmol/L), potassium of 3.5 mEq/L
(3.5 mmol/L), chloride of 130 mEq/L (130 mmol/L), and bicarbonate of
20 mEq/L (20 mmol/L). Urinalysis shows a specific gravity of 1.002, pH
of 5.5, and otherwise negative results.
Of the following, the MOST likely diagnosis for this patient is
A. Bartter syndrome
B. diabetes insipidus
C. Fanconi syndrome
D. Gitelman syndrome
E. improper formula preparation
A. Bartter syndrome
B. diabetes insipidus
C. Fanconi syndrome
D. Gitelman syndrome
E. improper formula preparation
You are called to attend the delivery of a
newborn male who had been monitored
carefully prenatally for moderate
oligohydramnios. Prenatal ultrasonography
suggested bilateral cystic dysplastic kidneys,
with no other congenital anomalies identified.
Of the following, the MOST likely immediate lifethreatening problem in this infant is
A. bladder outlet obstruction
B. cardiac arrhythmias
C. liver failure
D. renal failure
E. respiratory failure
A. bladder outlet obstruction
B. cardiac arrhythmias
C. liver failure
D. renal failure
E. respiratory failure
A 6-year-old girl presents for a health supervision visit that was scheduled as
a follow-up appointment after she had an elevated blood pressure at an
urgent care facility during an evaluation for abdominal pain. Her abdominal
pain has resolved. Her mother recalls the blood pressure in the urgent care
center as 135/90 mm Hg. The girl has had two urinary tract infections with
fever in the past, and her father had hypertension diagnosed at age 45 years.
On physical examination, the girl’s temperature is 37.3°C, heart rate is 90
beats/min, respiratory rate is 20 breaths/min, and blood pressure is 146/86
mm Hg. A repeat blood pressure reading is 142/88 mm Hg. The four limb
blood pressures are: 142/88 mm Hg in the right arm, 144/84 mm Hg in the
left arm, 156/100 mm Hg in the right leg, and 160/96 mm Hg in the left leg.
You find no cardiac murmurs, abdominal bruits, or edema. Femoral pulses are
2+ and symmetrical bilaterally. Renal ultrasonography shows the left kidney to
be 8.5 cm with normal corticomedullary differentiation and the right kidney
to be 5.5 cm with increased echogenicity.
Of the following, the MOST likely cause for this patient’s elevated blood
pressure is
A. coarctation of the aorta
B. essential hypertension
C. renal artery stenosis
D. renal hypoplasia/dysplasia
E. renal scarring from prior pyelonephritis
A. coarctation of the aorta
B. essential hypertension
C. renal artery stenosis
D. renal hypoplasia/dysplasia
E. renal scarring from prior pyelonephritis