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Board Review 2009

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Title: Board Review 2009


1
Board Review 2009
  • Hypo/Hyper natremia
  • mixed acid/base disturbances
  • Nephrotic Syndrome
  • Glomerulonephitis
  • RTAs
  • Calcium/Phosphate homeostasis
  • renal vasculitis
  • pictures of urine sediment
  • ATN
  • AIN
  • CKD staging and principles of management
  • Indications for renal transplant
  •  
  • Below is a list of topics that our residents did
    not do well on during the In-Training exam
  • diagnosis of primary hyperaldosteronism in
    metabolic alkalosis
  • diagnose type 4 rta
  • diagnose psychogenic polydipsia
  • diagnose mixed acid/base disturbances

2
Question 1
  • A 65 y.o. male is referred for evaluation of
    edema and proteinuria. He complains of fatigue,
    but otherwise is asymptomatic. On exam the BP is
    150/80. There is 1 ankle edema. Labs show
    hemoglobin 10 (MCV 74, RDW 20) urine
    proteincreatinine ratio is 4.4 mg/gm, serum
    creatinine is 1 mg/dL, and cholesterol is 320
    mg/dL. Serum complement levels are normal.
    Urinalysis shows 3 protein, hyalofatty casts and
    oval fat bodies.
  • Which of the following is the most likely cause
    of this patients renal symptoms?
  • Minimal change glomerulopathy
  • FSGS
  • Membranous glomerulopathy
  • IgA nephropathy
  • ANCA-associated GN

3
Question 2
  • A 19 y.o. female is evaluated for sudden onset
    periorbital and pretibial edema. 3 weeks ago she
    was diagnosed with an URI that has since
    resolved. On PE the BP is 150/100. A soft S3
    gallop is present. There are crackles at both
    lung bases. The liver is enlarged and tender.
    There is bilateral pitting pretibial edema. There
    is no rash. Labs show creatinine 1.5, albumin
    3.8. C3 and C4 are low. Urinalysis shows rare
    dysmorphic red cells and trace protein.
  • Which of the following is the most likely
    diagnosis?
  • IgA nephropathy
  • Goodpasture's syndrome
  • ANCA vasculitis
  • Postinfectious GN
  • SLE nephritis

4
Question 3
  • A 19 y.o. female presents with a several month
    history of symmetric arthralgias, Raynauds
    phenomenon, and a Coombs positive hemolytic
    anemia. On physical exam she has a malar rash
    that crosses the nasal labial folds. The heart
    and pulmonary exams are unremarkable. Her abdomen
    is benign. There is 1 leg edema. Urinalysis
    shows red cell casts and 2 protein. A kidney
    biopsy shows immune complex focal proliferative
    glomerulonephritis.
  • Which if the following tests provides the most
    additional diagnostic information?
  • Low C 3 and C4
  • Positive ANA
  • Positive ss-DNA
  • Positive anti-Smith antibody
  • Positive ds-DNA

5
Question 4
  • A 51-year-old man with a history of chronic
    lymphocytic leukemia with transformation to
    prolymphocytic leukemia is hospitalized for
    chemotherapy with R-CHOP (cyclophosphamide
    doxorubicin vincristine prednisone rituximab).
  • On physical examination, he is afebrile, pulse
    rate is 98/min, respiratory rate is 16/min, and
    blood pressure is 134/78 mm Hg. There is
    lymphadenopathy involving the cervical and
    submental chains and supraclavicular areas
    bilaterally, as well as bulky axillary and
    inguinal lymphadenopathy. Cardiac and pulmonary
    examinations are normal. The spleen is palpable
    approximately 3 cm to 4 cm below the left costal
    margin, and there is no hepatomegaly. There is no
    edema, cyanosis, or clubbing of the extremities.
  • Labs show hematocrit 22, leukocyte count
    110,000/µL, platelet count 19,000/µL, BUN 63
    mg/dL, uric acid 19 mg/dL, creatinine 1.3 mg/dL,
    potassium 5.5 meq/L, bicarbonate 17 meq/L,
    albumin 4.2 g/dL, calcium 7.5 mg/dL, phosphorus 5
    mg/dL, urinalysis pH 5, numerous finely granular
    casts/hpf, no uric acid crystals
  • In addition to hospitalization for intravenous
    hydration, which of the following is the most
    appropriate next step in this patients
    management?
  • A. Furosemide
  • B. Rasburicase
  • C. Allopurinol
  • D. Probenecid

6
Tumor Lysis Syndrome
  • Acute oliguric renal failure associated with
    urate levels gt 15 mg/dl and hyperphosphatemia
  • Associated with overproduction and excretion of
    urate and cell lysis resulting in increased
    release of potassium and phosphorus in patients
    undergoing chemotherapy or with a heavy tumor
    burden
  • Urine urate/creatinine gt 1
  • Prevention allopurinol 600-900 mg/d NS (uo gt
    2.5 l/d)
  • Urinary alkalinization may worsen calcium
    phosphate precipitation and NS is as effective as
    urinary alkalinization alone
  • Early dialysis indicated for oliguric ARF to
    decrease urate burden

7
Risk Stratification for TLS
Type of cancer Risk Risk Risk
Type of cancer High Intermediate Low
NHL Burkitt's, lymphoblastic, B-ALL DLBCL Indolent NHL
ALL WBC 100,000/microL WBC 50,000-100,000/microL WBC 50,000/microL
AML WBC 50,000/microL, monoblastic WBC 10,000-50,000/microL WBC 10,000/microL
CLL   WBC 10,000-100,000/microL treated with fludarabine WBC 10,000/microL
Other hematologic malignancies (including CML and multiple myeloma) and solid tumors   Rapid proliferation with expected rapid response to therapy Remainder of patients
From Coiffier B, Altman A, Pui CH, Younes A,
Cairo MS. Guidelines for the management of
pediatric and adult tumor lysis syndrome an
evidence-based review. J Clin Oncol. 2008
262767-78.
8
Prevention of TLS
  • If there is a concern about tumor lysis, as
    predicted by an elevated serum LDH, serum uric
    acid, or heavy tumor burden, the patient should
    be admitted for hydration and close monitoring of
    kidney function, serum potassium, phosphorus and
    uric acid.
  • Patients with a preexisting reduction in GFR,
    oliguria and/or acidic urine, and volume
    depletion should also be hospitalized for
    hydration and observation.
  • High-risk patients should be hospitalized for
    aggressive intravenous hydration and prophylactic
    rasburicase.
  • Intermediate risk patients should receive
    allopurinol rather than rasburicase for
    prophylaxis in the absence of pretreatment
    hyperuricemia.
  • Patients at low risk for TLS should receive
    hydration, but do not require hypouricemic
    therapy.

9
Question 5
  • A 64-year-old woman is evaluated for progressive
    weakness, nausea, dyspnea, and acute renal
    failure of several weeks duration. Her
    creatinine level is 4.6 mg/dL (last creatinine is
    1.3 mg/dL 2 months ago). She has a history of
    type 2 diabetes mellitus and hypertension treated
    with metoprolol and hydrochlorothiazide. Other
    medications include aspirin and glipizide.
  • On physical examination, the blood pressure is
    110/70 mm Hg. Fundoscopic examination reveals
    arteriovenous nicking but no evidence of diabetic
    retinopathy. Cardiac examination shows a
    laterally displaced PMI and a grade I
    holosystolic murmur at the apex. On pulmonary
    examination, the lungs are clear to auscultation.
    There is no pedal edema.
  • Hemoglobin 12.8 g/dL, leukocyte count 8000/µL,
    platelet count 311,000/µL, BUN 48 mg/dL, uric
    acid 11.6 mg/dL, creatinine 4.6 mg/dL, sodium 140
    meq/L, potassium 4.3 meq/L, chloride 110 meq/L,
    bicarbonate 26 meq/L, total protein 8.4 g/dL,
    albumin 3.8 g/dL, calcium 10.5 mg/dL, phosphorus
    5.6 mg/dL, lactate dehydrogenase 634 U/L
    Urinalysis pH 6.5, trace protein, trace blood, 2
    leukocytes/hpf, amorphous crystals. Urine sodium
    60 meq/L, urine creatinine 90 mg/dL, urine
    proteincreatinine ratio 3 mg/mg
  • Renal ultrasound shows enlarged hyperechoic
    kidneys bilaterally measuring 14 cm in length.
    Chest radiograph reveals mild cardiomegaly but is
    otherwise unremarkable.
  • Which of the following is the most likely
    diagnosis?
  • A. Uric acid nephropathy
  • B. Thiazide-induced acute renal failure
  • C. Primary amyloidosis
  • D. Myeloma cast nephropathy
  • E. Lymphomatous infiltration of the kidneys

10
Question 6
  • A 38 y.o. man with a history of chronic liver
    disease secondary to hepatitis C is treated with
    a 24-wk course of pegalated IFN combined with
    ribavarin. Four weeks after completing treatment
    he complains of proximal muscle weakness. On PE
    the BP is 120/80, pulse 110, RR 18. His general
    exam is unremarkable. On neurologic exam he has
    symmetric proximal weakness 3/5. Labs show Na
    142, K 2.1, Cl 104, HCO3 20, creat 1. Urine Na
    100, urine K 10, urine Cl 110, urine osm 585,
    urine pH 5.3.
  • Which of the following is the most likely
    diagnosis?
  • Distal renal tubular acidosis (type I)
  • Vomiting
  • Diuretic abuse
  • IFN complication
  • Hypomagnesemia
  • Diarrhea

11
Question 7
  • A 34 year old woman who underwent elective
    laparoscopic cholecystectomy develops severe
    headache and nausea the next morning. During the
    surgery, D5½NS was started and continued
    postoperatively at 125 mL/hr. She remained in
    recovery until late afternoon because she was too
    sedated to be discharged. Intravenous meperidine
    is administered with adequate relief of her pain.
    On physical exam the blood pressure is 130/80.
    She is afebrile. The heart and lung exams are
    normal. There is no peripheral edema. Neurologic
    exam is remarkable only for lethargy.
  • Laboratory studies show sodium 128, potassium
    3.4, chloride 86, bicarbonate 28, BUN 10,
    creatinine 0.8, glucose 86. Urine sodium 46,
    urine osmolality 453.
  • Which of the following should be done next?
  • NS 200 mL/hr furosemide
  • 3 saline at 1 ml/kg/hr furosemide
  • Emergent head CT
  • Naloxone
  • Observation

12
Symptomatic Hyponatremia Recommendations for
Management
  • 3 saline at average rate of 1 mL/kg/hr over
    first 24 hours furosemide if urine osm gt 300
    mOsm/kg or risk of CHF
  • Stop therapy when symptoms resolve
  • Measure the serum sodium every 1-2 hours for the
    first 6 hours and then every 4 hours
  • Do not exceed 10 mEq/L in 24 hours or 18 mEq/L in
    48 hours in a symptomatic patient with chronic
    hyponatremia

13
Hyponatremia Recommendations for Management
  • Do not use fluid restriction alone in the
    symptomatic patient
  • If the serum sodium increases too rapidly,
    interrupt the increase by starting hypotonic
    fluids and/or dDAVP 4 micrograms s.c.
  • Do not use 0.9 NaCl for symptomatic patients
    with SIADH, especially when the urine osm is gt
    300 mOsm/kg
  • Do not use vaptan therapy for symptomatic patients

14
Question 8
  • A 46 y.o. male is hospitalized for severe
    necrotizing pancreatitis. He is placed on NG
    suction and over the first 24 hours of
    hospitalization he receives 6 liters of NS and
    then NS at 100 mL/hour. Over the next 24 hours
    his urine output increases to gt 3 liters per day
    and his plasma sodium concentration rises from
    145 meq/L on admission to 153 meq/L.
  • On exam the blood pressure is 140/90. Chest is
    clear . There is no edema.
  • Labs show sodium 153, potassium 3, chloride 112,
    bicarbonate 24, BUN 49, creatinine 1.1, urine
    sodium 50, urine potassium 20, urine osmolality
    500 mosm/kg.
  • Which of the following is the most likely cause
    of this patients polyuria?
  • Central diabetes insipidus
  • Nephrogenic diabetes insipidus
  • Post obstructive diuresis
  • Solute diuresis

15
Polyuria
  • Urine output exceeding 3 L per day
  • Etiology
  • Water diuresis
  • diabetes insipidus
  • central
  • nephrogenic
  • primary polydipsia
  • Solute diuresis

16
Evaluation of Polyuria
Urine Osmolality
lt 250 mosm/kg
gt 300 mosm/kg
Water Diuresis
Solute Diuresis
17
Urine and Plasma Osmolality in Disorders of Water
Balance
Normal
1000
Water Deprivation
dDAVP
800
Primary polydipsia
Uosm(mosm/kg)
600
Central DI
400
Nephrogenic DI
200
280
285
290
295
300
Posm(mosm/kg)
18
Question 9
  • A 58-year-old woman with a history of chronic
    alcohol abuse is admitted to the hospital for
    evaluation of abdominal pain and vomiting. A
    diagnosis of recurrent pancreatitis is made on
    the basis of the history of alcoholism, the
    presence of diffuse abdominal tenderness and
    decreased bowel sounds, and elevated serum
    amylase and lipase levels.
  • Therapy is begun with intravenous fluids (0.9
    saline and 5 dextrose in water at 75 mL/hr) and
    nasogastric drainage, which produces copious
    amounts of fluid. After five days of therapy, the
    patient's symptoms resolve and the following
    laboratory studies are obtained
  • BUN 21 mg/dL, serum creatinine 1.4 mg/dL, plasma
    glucose180 mg/dL, serum sodium 140 mEq/L,
    potassium 2.6 mEq/L, chloride 86 mEq/L,
    bicarbonate 38 mEq/L, urine pH 7.0, urine sodium
    50 mEq/L, urine chloride 5 mEq/L
  • Which of the following best explains the
    hypokalemia in this patient?
  • (A) Renal potassium losses derived from
    decreased proximal tubule reabsorption
  • (B) Potassium loss in the gastric aspirate
  • (C) Intracellular redistribution of potassium
    is a major determinant of the hypokalemia
  • (D) Increased aldosterone and distal nephron
    bicarbonate delivery causing renal potassium
    losses

19
Metabolic Alkalosis
BP Volume status
BP Normal or Low ? Effective circulating volume
BP High ? ECF
Loss of HCl Loss of Volume (Na/H20)
Primary Hyperaldosteronism
Loss of Gastric secretions (vomiting, NG
suction) Diuretics Bartter syndrome Gitelman
syndrome
20
Metabolic Alkalosis Loss of Gastric
Secretions-Generation Phase
Loss of HCl from Stomach
Generation of NaHCO3
Kidney
1. Elimination of chloride from the urine 2.
Excretion of NaHCO3 and KHCO3 in Urine
21
Metabolic Alkalosis Loss of Gastric
Secretions-Maintenance Phase
Volume Contraction
Reabsorption of sodium, chloride, and
bicarbonate along the nephron
Secondary increase in aldosterone
Metabolic alkalosis Paradoxical aciduria
Elimination of sodium, chloride, and
bicarbonate from the urine
Increased H Excretion
22
Question 10
  • A 42 y.o. female is evaluated for minimal edema
    and a urinary protein excretion of 5 gm/24 hours.
    As a child she had frequent urinary tract
    infections and underwent a surgical procedure to
    reimplant the ureters to prevent reflux. On PE
    the BP is 140/95. There is trace peripheral
    edema. Labs show creatinine 1.5, albumin 3.4,
    Urinalysis shows 3 protein and oval fat bodies.
  • Chest x-ray is normal. Renal US shows a normal
    left kidney and the right kidney small and
    difficult to visualize.
  • Which of the following is the most likely cause
    of the proteinuria?
  • Minimal change disease
  • Membranous nephropathy
  • FSGS
  • Membranoproliferative GN

23
Focal Segmental Glomerulosclerosis (FSGS)
  • Most common cause for nephrotic syndrome in
    African-Americans
  • Occurs as a primary (idiopathic) glomerular
    disease and also secondary to recognized causes
    (such as obesity, HIV infection, and inherited
    genetic defects)
  • This is a pathologically and clinically
    heterogeneous category of disease that includes
    multiple structural variants with different
    demographics, clinical presentations and outcomes

24
Different Structural Variants of FSGS
25
PRIMARY (IDIOPATHIC) FSGS SECONDARY
FSGS VIRUS-ASSOCIATED HIV-1 (HIV-associated
nephropathy) Parvovirus B-19 FAMILIAL
FSGS Mutations in a-actinin 4 gene Mutations
in NPHS2 gene for podocin Mutations in TRPC6
gene for a cation channel DRUG
TOXICITY Heroin (Heroin nephropathy) Pamidro
nate Interferon-a MEDIATED BY ADAPTIVE
STRUCTURAL RESPONSES Reduced renal mass
Obesity Cyanotic congenital heart
disease Sickle cell anemia
26
Question 11
  • A 44 y.o. female with cirrhosis is admitted with
    fever and abdominal pain. Medications include
    spironolactone, furosemide, and lactulose. On
    exam the BP is 74/55, HR 72, T 38.3, RR 24. She
    is cachectic. The abdomen is tense and diffusely
    tender. There is 1 leg edema.
  • Labs show Serum sodium 128, potassium 5.1,
    chloride 104, bicarbonate 12, BUN 20, creatinine
    1.3, glucose 84, albumin 1.4. ABG pH 7.25, pCO2
    28, pO2 78.
  • Which best describes the acid-base status of this
    patient?
  • Mixed anion gap metabolic acidosis and
    respiratory alkalosis
  • Mixed anion gap metabolic acidosis and
    respiratory acidosis
  • Anion gap metabolic acidosis
  • Hyperchloremic acidosis
  • Mixed anion gap metabolic acidosis and
    hyperchloremic acidosis

27
Question 11 Analysis
1. What is the overriding disorder? Metabolic
acidosis 2. What is the anion gap? 128-104-12
12, ?increased AG met acidosis (Normal anion
gap is 5.5 in this case due to the
hypoalbuminemia- For every 1 gm/dL fall in the
albumin from 4 gm/dL, the anion gap expected
anion gap decreases 2.5 mEq/L). 3. What is the
expected bicarbonate with pure ?AG metabolic
acidosis? 25-(12-5.5) 18.5 Meas bicarb is 12,
? concurrent hyperchloremic metabolic acidosis 4.
What is the expected pCO2 with normal respiratory
compensation? Expect PCO2 12 15 27. Meas
PCO2 is 28, ?normal resp comp Answer Mixed ?AG
met acid, hyperchloremic met acidosis
28
Question 12
  • 23-year-old Caucasian female referred for further
    evaluation of hypokalemic acidosis. She was in
    her usual state of excellent health with normal
    growth and development until her second month of
    pregnancy. She had a spontaneous miscarriage, and
    was found to have a serum potassium of 3.2 mEq/L
    and a bicarbonate level of 19 mEq/L during a
    hospitalization for a D and C. She was treated
    with oral potassium and bicarbonate supplements
    and then weaned these off after 4 months of
    therapy. Six weeks later, she developed myalgias
    and collapsed due to profound weakness. She was
    found to have a serum bicarbonate level of 14
    mEq/L with a serum potassium of 1.9 mEq/L.
  • 140 114 13 Calcium 9.1
  • 1.9 14 1 Phosphorus 3.5
  • ABG-pH 7.29, PCO2 30, pO2 100
  • Urine K 46 Urine Na 36 Urine Cl 42 Urine Osm
    580
  • UA ph 6.8 trace protein No casts 10-15 white
    cells per high power field
  • Which of the following is the correct diagnosis?
  • Type IV RTA
  • Diarrhea
  • Type I RTA
  • Renal tubular alkalosis
  • Proximal RTA

29
Practical Approach (Hyperchloremic metabolic
acidosis)
Urinary Anion Gap Negative Positive Type 2
RTA Diarrhea DKA/Toluene HCl (Hyperalimentation)
Urine pH and Plasma K Urine pH lt 5.5, K ?
Urine pH gt 5.5, K nl/low Urine pH gt 5.5, K
? Type 4 Type 1 (secretory
defect Type 1 (voltage)
or back-leak)
30
Type I Classic Distal RTA-Mechanism 1
Tubular lumen
Na
Peritubular Capillary
Na
3Na
ATPase
(-)
2K
(-)
H Retention
K
R-Aldo
K Wasting Urine pH gt 5.5 Ca-P stones
Cl-
ATPase
3Na
H
ATPase
H2O
2K
(-)
T
HCO3-
OH- CO2
K
Cl-
ATPase
(-)
H
31
Type I Classic Distal RTA-Mechanism 2
Tubular lumen
Na
Peritubular Capillary
Na
3Na
ATPase
(-)
2K
(-)
H Retention
K
R-Aldo
K Wasting Urine pH gt 5.5 Ca-P stones
Cl-
ATPase
3Na
H
ATPase
H2O
2K
(-)
T
?HCO3-
OH- CO2
K
Cl-
ATPase
(-)
H
32
Type I Distal RTA-Mechanism 3
Tubular lumen
Na
Peritubular Capillary
Na
3Na
ATPase
(-)
2K
(-)
K
R-Aldo
K Wasting Urine pH gt 5.5 Ca-P stones
Cl-
Backleak of H
ATPase
3Na
H
H
ATPase
H2O
H Retention
2K
(-)
T
HCO3-
OH- CO2
K
Cl-
ATPase
(-)
H
33
Question 13
  • A 32 y.o. male presents with paresthesias,
    perioral numbness, and generalized weakness. He
    is not on any medications. The BP is 120/88, and
    the physical exam is remarkable for dental
    caries. Earlier in the day he had attended a
    birthday party for his nephew. Labs show Na 139,
    potassium 2.8, chloride 90, bicarbonate 38. Urine
    sodium 28, urine potassium 38, urine chloride lt
    10, urine pH 6.2, urine calciumcreatinine ratio
    0.2 (mmol/mmol).
  • Is this
  • Barrter syndrome
  • Vomiting
  • Gitelman syndrome
  • Hypokalemia periodic paralysis
  • Licorice ingestion

34
Urine Na and Cl- in the Differential Diagnosis
of Metabolic Alkalosis and Hypokalemia
Urine Electrolytes Na Cl- Condition
(meq/L)
Vomiting Alkaline urine gt15 lt15 Acidic
urine lt15 lt15 Diuretic Drug active gt15 gt15 R
emote use lt15 lt15 Hyperaldosteronism gt15 gt15
35
Question 14
  • You are asked to evaluate a 42 year old woman
    because of hyponatremia. She has a history of
    schizophrenia and is currently hospitalized
    because of suicidal and homicidal ideation. Her
    admission laboratory studies obtained during a
    psychiatric hospitalization were normal. Current
    medications are haloperidol and benztropine
    mesylate.
  • Physical exam shows pulse 92 supine, 100
    standing, BP 112/82 supine and 108/88 standing.
    Occasional involuntary movements of the tongue
    and lips are noted. There is no edema.
  • Labs show sodium 120 mEq/L, potassium 4.2 mEq/L,
    chloride 85 mEq/L, bicarb 27 mEq/L, BUN 8 mg/dL,
    creatinine 0.8 mg/dL, serum osmolality 250
    mOsm/kg, TSH 3.8 microunits/mL, uric acid 3.2
    mg/dL Urine sodium 12, urine potassium 3, urine
    chloride 10, urine osmolality 55.
  • Which of the following is the most likely cause
    of this patients hyponatremia?
  • Primary polydipsia
  • Mineralocorticoid deficiency
  • Reset osmostat
  • Diuretic abuse
  • SIADH

36
Question 15
  • A 49 y.o. female is admitted to the hospital
    because of severe right sided abdominal pain
    requiring administration of narcotic analgesics.
    The patient is unable to provide a complete
    medical history, but reports that she has had
    seizures for as long as she can remember.
  • Physical exam reveals papular skin lesions in the
    malar area. Bilateral flank masses are noted.
    There is a 2-cm periungual nodular lesion on the
    right great toe.
  • Hematocrit is 25. Serum creatinine is 5.5 mg/dL.
    CT of the abdomen without contrast reveals
    enlarged kidneys with bilateral renal cysts of
    varying size in the cortex and the medulla
    several variably sized masses with densities
    identical to perinephric fat are also detected in
    areas not involved with cysts.
  • Which if the following is the most likely
    diagnosis?
  • ADPCKD
  • Von Hippel-Lindau disease
  • Medullary cystic kidney disease
  • Tuberous sclerosis
  • Bilateral renal dysplasia

37
Question 16
  • A 66-year-old man comes for a follow-up
    examination for elevated blood pressure. He has
    a history of chronic kidney disease and
    hypertension well controlled with
    hydrochlorothiazide. One week ago, he was
    evaluated in the office after obtaining several
    home blood pressure measurements averaging 145/90
    mm Hg. Enalapril was added at that time. He has
    felt well and has no history of cough,
    lower-extremity edema, or dyspnea. He also takes
    low-dose aspirin.
  • On physical examination today, temperature is
    normal, blood pressure is 126/70 mm Hg,
    respiration rate is 18/min, and pulse rate is
    78/min and regular. On cardiac examination, the
    point of maximal impulse is laterally displaced
    and an S4 gallop is heard. There is no edema.

1 week ago Today
Potassium 4.5 meq/L 5.2 meq/L
Creatinine 1.2 mg/dL 1.5 mg/dL
Urine albumincreatinine ratio 200 mg/g
  • In addition to dietary potassium restriction,
    which of the following is the most appropriate
    next step in this patients management?
  • Add diltiazem
  • Discontinue enalapril switch to metoprolol
  • Repeat creatinine and potassium measurement in 1
    week
  • Kidney arteriography

38
Question 17
  • A 55 y.o. male with stage 3 chronic kidney
    disease presents for routine follow up.
    Laboratory studies show calcium 9.2 mg/dL,
    phosphorus 2.8 mg/dL, PTH 215 pg/mL, 25-OH
    vitamin D 10 ng/mL, 1,25-(OH)2-vitamin D 19.
  • Which one of the following would you recommend?
  • Restrict dietary phosphorus to 600 mg daily
  • Start sevelamer 800 mg three time daily with
    meals
  • Start calcitriol 0.25 mcg once daily
  • Start ergocalciferol 50,000 units once weekly

39
Time Course of Physiologic Alteration in Calcium,
Phosphorus, Vitamin D and PTH in CKD
Stage 1
Stage 2
Stage 3
Stage 4
Stage 5
? 25-OH-vitamin D
? 1,25-(OH)2-vitamin D
Transient Post-prandial Hypocalcemia
Hypocalcemia
Hyperphosphatemia
Increased PTH secretion
90 80 70 60 50
40 30 20 lt15
GFR (mL/min/1.73 m2)
40
Question 18
  • A 59 y.o. female presents with 2 weeks of R hip
    pain. The past history is significant for end
    stage renal disease due to stone disease
    complicating type I distal renal tubular acidosis
    now on peritoneal dialysis, s/p bilateral
    nephrectomy, s/p living donor kidney transplant 5
    years ago with loss of allograft function after 3
    years from chronic rejection, gout, and
    hypothyroidism. Medications include levothyroxine
    0.15 mg p.o. daily, allopurinol 150 mg p.o.
    daily, aspirin 81 mg p.o. daily, erythropoietin
    10,000 units subcutaneously weekly, colchicine
    0.6 mg p.o. daily, sevelamer 800 mg three times
    daily with meals, multivitamin one daily, and
    calcitriol 0.25 mcg once daily. There is no
    history of exposure to aluminum containing
    medications.

41
Question 18
  • On physical exam there is tenderness over the
    lateral trochanteric bursa and pain with internal
    and external rotation of the hip. Labs show
    calcium 9.1 mg/dL, phosphorus 5.6 mg/dL, intact
    PTH 21 pg/mL, 1,25-dihydroxy-vitamin D 52 pg/mL
    (reference range 15-75) 25-hydroxy-vitamin D 25
    ng/mL (reference range 15-57), alkaline
    phosphatase 86 U/L (reference range 38-126). TSH
    is normal at 2.5 microIU/mL (reference range
    0.6-3.3). Plain films of the right hip show
    diffuse osteopenia and an area of lucency along
    the medial aspect of the femoral neck on the
    right consistent with stress fracture.

42
Question 18
  • Which of the following is the most likely
    diagnosis?
  • A. Adynamic bone disease
  • B. Beta-2 microglobulin associated amyloidosis
  • C. Osteomalacia
  • D. Osteitis fibrosa cystica

43
Disorder Turnover Mineralization Clinical Features
Adynamic bone disease Low Decreased Fractures Bone pain Osteopenia Low PTH Hypercalcemia with calcium loading
Osteitis fibrosa cystica High ?? Resorption ? Formation Fractures Mixed sclerosis and osteopenia Bone pain Proximal myopathy High PTH Elevated alkaline phosphatase Subperiosteal reabsorption Brown tumors (rare)
Osteomalacia Low Low Fractures Osteopenia Bone pain Proximal myopathy Looser zones on x-ray High PTH related to secondary hyperparathyroidism Usually related to aluminum accumulation
Mixed uremic osteodystrophy Mixed Variable PTH and bone alkaline phosphatase variable
Osteoporosis Low Decreased Osteopenia CKD specific risk factors heparin steroids Hypogonadism Poor nutrition Vitamin D deficiency Metabolic acidosis
Amyloid Variable Variable Due to accumulation of beta-2 microglobulin Bone pain Cystic bone lesions Transplant ameliorates bone pain
44
Question 19
  • A 64-year-old woman is referred for evaluation of
    a rising serum creatinine level. She had been
    recently diagnosed with severe reflux esophagitis
    for which she was treated with omeprazole. Over
    the past several weeks she had noted onset of
    generalized malaise, fatigue, and anorexia.
    Screening laboratory studies revealed that the
    serum creatinine level had risen to 2.5 mg/dL
    (0.221 mmol/L) compared with a previous value of
    1.2 mg/dL (0.106 mmol/L) 6 weeks ago.

45
Question 19
  • On physical exam the blood pressure was 120/60 mm
    Hg. There was no rash or edema.
  • Laboratory studies showed sodium 138 mmol/L,
    potassium 4.7 mmol/L, chloride 103 mmol/L, total
    carbon dioxide 17 mmol/L, BUN 43 mg/dL (15.3
    mmol/L), creatinine 2.5 mg/dL (0.221 mmol/L),
    hemoglobin 11 gm/dL (110 gm/L), white count 8,200
    mm3 without eosinophilia. Serologic studies
    including ANA and ENA all returned negative.
  • Urinalysis revealed trace protein and 35 white
    blood cells per high-power field without casts.

46
Question 19
  • What is the most likely diagnosis?
  • Ischemic acute tubular necrosis
  • Acute interstitial nephritis
  • Myeloma cast nephropathy
  • Pyelonephritis
  • Obstruction due to kidney stones

47
Etiology of Acute Interstitial Nephritis (AIN)
Etiology Frequency Drugs 71 Penicillins
and cephalosporins NSAIDs, including COX-2
inhibitors Rifampin Sulfonamides
Quinolones Allopurinol Proton pump
inhibitors Indinavir 5-aminosalicylates
(e.g. mesalamine) H-2 blockers Infection
15 Tubulointerstitial 5 nephritis and
uveitis Sarcoidosis 1
Medication Frequency
Antibiotics Cephalosporins Quinolones Penicillins 56 15/34 12/34 7/34
NSAIDs 37
Other Allopurinol Omeprazole Ranitidine Pimozide 7
González E, et al. Kidney Int 2008 73 940946.
Baker RJ and Pusey CD. Nephrol Dial Transplant
2004198-11.
48
Clinical Features of AIN
Clinical Feature Frequency
Leukocyturia 82
Microhematuria 67
Fever 42
Eosinophilia 34
Rash Oliguria 23 23
González E, et al. Kidney Int 2008 73 940946.
49
Corticosteroid Treatment for AIN
Plt0.05
No steroids
Steroid-treated
  • Chronic dialysis in 44 with no steroids vs 3.8
  • with steroids (Small study-52 patients treated
    with
  • steroids, 9 managed with drug withdrawal alone)
  • Trend toward more fibrosis on biopsy when
    steroids
  • withheld and when started later (NS)

González E, et al. Kidney Int 2008 73 940946.
50
Proton Pump Inhibitors (PPI) and AIN
  • 95 million prescriptions written for PPI in the
    United States in 2005
  • PPIs are the most common cause of drug induced
    interstitial nephritis (32) in an adverse drug
    reaction registry in New Zealand (Simpson et al.
    Nephrology 200611381-385).

51
Clinical Features of PPI-associated AIN
Finding Frequency
Pyuria 72
Fatigue and nausea 39
Eosinophilia 33
Weakness 22
Fever 10
Rash lt10
Geevasinga N, et al. Clin Gastroenterol Hepatol
20064597-604.
52
Question 20
  • A 56 y.o. male with a history of hypertension and
    gout is seen for routine follow up by his primary
    care physician. Medications include metoprolol,
    colchicine, aspirin and meloxicam.
  • On PE the BP is 130/80, HR 86, RR 16, T 36.1. The
    heart exam shows an S4 gallop. The remainder of
    the exam is benign.

53
Question 20 Labs
Serum Serum
Sodium 140
Potassium 5.9
Chloride 110
Bicarbonate 18
BUN 18
Creatinine 1.1
Glucose 76
ABG ABG
pH 7.35
pCO2 35
pO2 106
Urine Urine
Sodium 26
Potassium 22
Chloride 28
Urinalysis shows pH 5.2, (-) glucose, (-) blood,
no casts. Urine P/C 0.052.
54
Which of the following is the most likely cause
for the laboratory abnormalities?
  1. Increase potassium intake
  2. Rhabdomyolysis
  3. Proximal RTA
  4. Adverse effect of meloxicam
  5. Adverse effect of colchicine

55
Question 20 Analysis
1. What is the overriding disorder? Metabolic
acidosis 2. What is the anion gap?
140-110-18 12, ?Hyperchloremic metabolic
acidosis 3. What is the expected pCO2 with normal
respiratory compensation? Expect PCO2 18
15 33. Meas PCO2 is 35, ?normal resp comp 4.
Net urine charge 2622-28 20 Answer
Hyperchloremic met acidosis, distal RTA, type IV
56
Aldosterone Deficiency or Resistance(Type IV RTA)
Na
Tubular lumen
Peritubular Capillary
Na
3Na
Na
ATPase
2K
Na
K
? Aldo
R-Aldo
Na
Cl-
K retention H retention
ATPase
3Na
Urine pH lt 5.5 Defect in H excretion is mild
H
ATPase
H2O
2K
T
HCO3-
OH- CO2
K
NH4
ATPase
Cl-
H
NH3
H
NH3
57
Causes of Type IV Distal RTA
  • Diabetic nephropathy
  • Tubulointerstial disease
  • Cyclosporine and tacrolimus
  • Transplant rejection
  • Adrenocorticoid insufficiency
  • Drugs
  • NSAIDS, Cox 2 inhibitors
  • ACE inhibitors
  • Heparin
  • Obstructive uropathy
  • Sickle cell nephropathy

Probably due to voltage dependent type I RTA
which has a similar clinical picture (hyperkalemi
a, non-gap acidosis)
58
Inhibitors of the Renin-Angiotensin-Aldosterone
System
59
Question 21
  • A 26 year old female presents with a history of
    intermittent tea-colored urine, often becoming
    apparent a day or two after onset on upper
    respiratory tract infections. On exam the blood
    pressure is 140/90 mmHg, heart and lungs normal,
    and there is no peripheral edema. There is no
    rash or synovitis.
  • Urinalysis reveals trace protein and 5-10
    dysmorphic red cells per high power field. The
    serum creatinine concentration is 0.6 mg/dL.
    Anti-nuclear antibodies and anti-neutrophil
    antibodies return negative. Serum complement
    levels are normal.
  • Which one of the following represents the most
    likely diagnosis?
  • A. membranoproliferative glomerulonephritis
  • B. membranous nephropathy
  • C. IgA nephropathy
  • D. post-infectious glomerulonephritis

60
Question 22
  • A 35-year-old woman who is pregnant with her
    third child is evaluated for right upper quadrant
    abdominal pain. She has no significant medical
    history. Her father died of cerebral aneurysm.
    She takes no medication.
  • On physical examination, temperature is normal,
    pulse rate is 88/min, and blood pressure is
    140/90 mm Hg. Cardiac and pulmonary examinations
    are normal. Abdominal examination is
    unremarkable. There is no edema. Creatinine level
    is 0.8 mg/dL (70.74 µmol/L).
  • On abdominal ultrasound, the right kidney is 14
    cm and the left kidney is 13 cm. There are
    multiple cysts in both kidneys and the liver. No
    hydronephrosis, solid masses, or stones are
    present.

61
Question 22
  • Which of the following is the most likely
    diagnosis in this patient?
  • A. Autosomal recessive polycystic kidney
    disease
  • B. Acquired cystic kidney disease
  • C. Autosomal dominant polycystic kidney disease
  • D. Nephronophthisis

62
Question 23
  • 46 y.o. female with a history of alcohol abuse
    presents with progressive nausea, confusion and
    labored breathing over 5 days. The past medical
    history is significant for chronic migraines and
    hypertension. Medications include enalapril 10 mg
    daily, Premarin 0.625 mg daily and Darvocet N-100
    as needed for pain. She was a vegetarian.
  • Physical exam showed respiratory distress and
    postural hypotension.

63
Question 23 Labs
Serum Serum
Sodium 131
Potassium 4.0
Chloride 90
Bicarbonate 8
BUN 40
Creatinine 2.0
Glucose 106
AST 2200
ALT 900
GGT 1100
CK 159
ABG ABG
pH 6.88
pCO2 28
pO2 145
CBC CBC
White count 20
Hemoglobin 10.1
Platelets 230
64
Question 23 Labs
Toxicology Toxicology
Serum Ketones Negative
Urine opioids Positive
Urine cocaine Negative
Serum salicylate lt 3 mg/dL
Serum acetaminophen 6.8 mcg/mL
Alcohol screen Negative
Lactic acid 15 mmol/L
65
From http//www.merck.com/mmpe/sec21/ch326/ch326c.
html
66
Question 23
  • Treatment with intravenous fluids and supportive
    care produced overall improvement and resolution
    of the lactic acidosis. However, the anion gap
    remained high at 30 mmol/L over the next 3 days
    and the metabolic acidosis persisted.
  • What is your diagnosis?

67
5-Oxoproline (Pyroglutamic Acid) Related Acidosis
  • Due to disruption of the gamma-glutamyl cycle
  • Inherited defect
  • Acquired
  • Chronic acetaminophen use (decreases cysteine)
  • Malnutrition
  • Pregnancy
  • Vegetarian diet (decreased glycine)
  • Liver disease (depleted glutathione stores)

68
The Gamma-Glutamyl Cycle
Fenves, A. Z. et al. Clin J Am Soc Nephrol
20061441-447
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