MARCHWEEK 1 QUIZCR

							MARCH WEEK 1 QUIZ CRITIQUE
Question 1
A 14-year-old girl presents with a 2-month history of joint pain that is responding poorly to over-the-counter
anti-inflammatory medications. She reports some sores in her mouth and mild swelling around her eyes and
ankles. On physical examination, her temperature is 37.0°C, heart rate is 76 beats/min, respiratory rate is
14 breaths/min, and blood pressure is 130/86 mm Hg. She has oral ulcers, mild periorbital and pretibial
edema, and mild swelling of her wrists and knee joints. Laboratory findings include:


       Sodium, 136 mEq/L (136 mmol/L)
       Potassium, 4.8 mEq/L (4.8 mmol/L)
       Chloride, 100 mEq/L (100 mmol/L)
       Bicarbonate, 22 mEq/L (22 mmol/L)
       Blood urea nitrogen, 24.0 mg/dL (8.6 mmol/L)
       Creatinine, 1.3 mg/dL (114.9 mcmol/L)
       Albumin, 2.5 g/dL (25.0 g/L)
       Hemoglobin, 10.1 g/dL (101.0 g/L)
       White blood cell count, 3.0x103/mcL (3.0x109/L)
       Platelet count, 190x103/mcL (190x109/L)
       Urinalysis: 3+ blood, 3+ protein, with 20 to 50 red blood cells/high-power field
       Antinuclear antibody titer: 1:1,280
       Anti-double-stranded DNA titer: 1:640


Of the following, the next BEST step in management is to

A             admit the patient for intravenous cyclophosphamide treatment
B             initiate treatment with ibuprofen
C             order a 24-hour urine for protein collection
D             refer the patient for a renal biopsy
E             refer the patient for bone marrow aspiration




Critique D

The adolescent girl in the vignette meets the diagnostic criteria for systemic lupus erythematosus
(SLE). Her renal involvement necessitates an aggressive approach to diagnosis and treatment, but the
severity of renal involvement must be determined before aggressive treatment is initiated.

Renal disease in patients who have SLE usually manifests as an immune complex-mediated
glomerulonephritis (GN), often associated with hypocomplementemia and positive serologic testing for
antinuclear antibody (ANA) and anti-double-stranded (ds) DNA. A recent observational study from
Toronto demonstrated that 37% of children have nephritis at diagnosis, 46% within 1 year of
diagnosis, and 55% in long-term follow-up. The clinical manifestations of lupus nephritis are those
typically seen with GN and may include one or more of the following: hematuria, proteinuria, azotemia,
hypertension, and edema. Lupus nephritis is categorized further by histologic criteria into the World
Health Organization classification system: class I (normal), class II (mesangial proliferative GN), class
III (focal proliferative GN), class IV (diffuse proliferative GN), and class V (membranous GN). Because
of the need to classify the form of nephritis prior to the institution of corticosteroids, the standard of
care is to obtain a renal biopsy prior to treatment. Some forms of lupus nephritis, including diffuse
proliferative nephritis, are treated with cyclophosphamide as an adjunctive agent, but this medication
should not be used for renal indications without a kidney biopsy. Results of the renal biopsy can
provide both prognostic and treatment information.
Once the renal disease is classified histologically and initial treatment is instituted, the patient can be
monitored by periodic assessment of urinary protein excretion as well as measurement of serologic
markers such as complement components and anti-ds DNA titers. Patients who exhibit worsening
proteinuria, decreasing concentrations of complement components, or rising anti-ds DNA titers require
assessment for a disease flare, which may necessitate increasing immunosuppressive therapy
(including corticosteroids).

Because nonsteroidal anti-inflammatory drugs such as ibuprofen have potential nephrotoxicity, they
usually are not administered to children who have lupus nephritis. The 24-hour urine collection
typically is not used for quantitating urine protein excretion, which can be assessed accurately with a
spot urine protein and creatinine measurement. There is no indication for a bone marrow aspiration in
this patient.

Question 2
A 16-year-old girl comes to your office for a follow-up visit from the emergency department, where she went
for the acute onset of knee pain and swelling. The emergency department physician had ordered an
antinuclear antibody test, which was positive at 1:320. Further history reveals that she has had intermittent
joint pains for several weeks and dark-colored urine. Findings on her physical examination are normal
except for an effusion in her right knee. You decide that further evaluation for systemic lupus erythematosus
(SLE) is warranted.

Of the following, the MOST specific test in helping you make the diagnosis of SLE is

A             anticardiolipin antibody measurement
B             anti-double-stranded DNA antibody measurement
C             anti-Ro antibody measurement
D             complement measurement
E             Venereal Disease Research Laboratory (VDRL) test

Critique B

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that involves multiple organ
systems. The diagnostic criteria are well established and include the presence of characteristic
laboratory findings as well as clinical manifestations. Four of the following 11 criteria must be met for
the diagnosis to made:


       Malar rash
       Discoid rash
       Oral ulcers
       Photosensitivity
       Arthritis
       Serositis
       Hematologic manifestations
       Central nervous system manifestations
       Nephritis
       Immunologic manifestations (positive anti-double-stranded DNA [anti-ds DNA] or anti-Smith antibody,
        false-positive test for syphilis, elevated antiphospholipid antibodies)
       Elevation of antinuclear antibody (ANA)



A good screening test for the presence of SLE is the ANA test because almost all patients who have
SLE have positive results. However, many people who do not have SLE also may have a positive ANA
test result, including those who have no disease and those who have other conditions such as juvenile
idiopathic arthritis, dermatomyositis, thyroid disease, or recent infections. When a patient's laboratory
evaluation reveals a positive ANA and there are other potential clinical manifestations of SLE, such as
those described for the girl in the vignette, more specific antibodies, such as anti-ds DNA, anti-Smith,
anti-Ro, and anti-cardiolipin antibodies, should be obtained. Of these, the anti-ds DNA is the most
specific. Anti-Ro is seen most commonly in neonatal lupus erythematosus. Complement values may be
low in patients who have SLE, but this finding is not sensitive or specific for SLE. A false-positive
Venereal Disease Research Laboratory (VDRL) test occurs commonly among patients who have SLE,
making it one of the laboratory criteria for the disease; however, the anti-ds DNA antibody test has
greater diagnostic specificity.



Question 3
A 16-year-old girl comes to your office complaining of irregular menstrual periods. She had menarche at 11
years of age and experiences menstrual periods every 2 weeks to 3 months. She has noticed increased
acne, facial hair growth, and a 20-lb weight gain over the past year.

Of the following, the MOST likely diagnosis is

A             Cushing syndrome
B             hypothyroidism
C             Noonan syndrome
D             ovarian tumor
E             polycystic ovary syndrome



Critique E

Signs of androgen excess (especially hirsutism and acne) combined with oligomenorrhea or
anovulatory bleeding, as described for the adolescent girl in the vignette, should alert the clinician to
the possibility of polycystic ovary syndrome (PCOS). PCOS is the most common endocrinopathy in
premenopausal women, but its clinical criteria for diagnosis, pathophysiology, and treatment remain
controversial. Definite or probable criteria for diagnosis include laboratory or clinical
hyperandrogenism, menstrual dysfunction, and exclusion of congenital adrenal hyperplasia. Other
criteria often used to support a diagnosis include insulin resistance, perimenarcheal onset, elevated
luteinizing hormone-to-follicle-stimulating hormone ratio, and ultrasonographic abnormalities.
Abnormalities observed in patients who have PCOS occur in four key areas: 1) increase in luteinizing
hormone secretion, 2) increase in adrenal androgen production, 3) increase in body mass, and 4)
onset of adult patterns of insulin resistance. Some girls have a transient period of hyperandrogenism
during the first 3 years after menarche; others have persistent PCOS symptoms. Patients who have
PCOS are at increased risk for diabetes mellitus, obesity, insulin resistance, infertility, and impaired
quality of life.

Cushing syndrome is less likely in the absence of other stigmata of the syndrome, including weakness,
spontaneous ecchymoses, large purple striae (Item C73A), hypokalemia, and osteoporosis. A rapid
onset of hirsutism with virilization is characteristic of ovarian tumors. Hypothyroidism can cause
menstrual irregularities and weight gain, but is not associated with androgen excess. Patients who
have Noonan syndrome (Item C73B) may present with delayed puberty and associated amenorrhea,
but they do not exhibit androgen excess and weight gain.



Question 4
Therapies for DM2 include which of the following?
    A. Insulin
    B. Diet and exercise
    C. Sulfonyureas
    D. Metformin
    E. All of the answers are correct
Critique E


Question 5
The first line approach to the treatment of a hemorrhagic episode in a patient with hemophilia A complicated
by an inhibitor is?

    A.   Fresh frozen plasma
    B.   Cryoprecipitate
    C.   Factor VIII concentrate
    D.   Factor VIIa
    E.   Factor IX concentrate



Critique D