WBR1007

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Author [[PageAuthor::Vidit Bhargava, M.B.B.S [1]]]
Exam Type ExamType::USMLE Step 2 CK
Main Category MainCategory::Internal medicine
Sub Category SubCategory::Dermatology, SubCategory::Hepatology
Prompt [[Prompt::A 35 year old female presents with a 2 week history of sensitivity to sunlight. Whenever she steps outside unprotected she develops blistering, scarring, pigmentation on the back of her hands and face. She has also noticed increased hair growth at unusual places during these past 2 weeks. She is sexually active and takes oral contraceptives regularly. The physician orders the following labs:

Complete blood count:

  • Hemoglobin 12.0 mg/dL
  • Hematocrit 45%
  • RBC's 4 million/cc
  • WBC's 8000 /cc
  • Platelets 3 lac/cc

LFT's

  • AST 100 IU/L
  • ALT 90 IU/L
  • Prothrombin time 20 secs
  • Bilirubin 2.0

Urinalysis:

  • Color - pale
  • pH – 5
  • Specific gravity – 1.010
  • Ketones – None
  • Nitrates – Negative
  • Leukocyte esterase – Negative
  • WBCs - 3 WBCs/hpf
  • Casts – 0-5 hyaline casts/lpf
  • Crystals – none
  • Uroporphyrin Elevated
  • Porphobilinogen Normal

USG: Normal liver architecture

The patient is diagnosed with an enzymatic defect in heme metabolism, which of the following steps must be taken to treat this condition?

]]

Answer A AnswerA::Stop oral contraceptives
Answer A Explanation AnswerAExp::
Answer B AnswerB::Use barrier sun protection
Answer B Explanation AnswerBExp::
Answer C AnswerC::Phlebotomy or Deferoxamine to remove iron
Answer C Explanation AnswerCExp::
Answer D AnswerD::Chloroquine as it increases the excretion of porphyrins
Answer D Explanation AnswerDExp::
Answer E AnswerE::All of the above are true
Answer E Explanation AnswerEExp::
Right Answer RightAnswer::E
Explanation [[Explanation::

Educational Objective:
References: ]]

Approved Approved::No
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