WBR1006

Author	[[PageAuthor::Vidit Bhargava, M.B.B.S [1]]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pharmacology
Sub Category	SubCategory::Dermatology
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 indicates the disease and the associated enzymatic defect? ]]
Answer A	AnswerA::Congenital erythropoietic porphyria; uroporphyrinogen III synthase
Answer A Explanation	AnswerAExp::
Answer B	AnswerB::Porphyria cutanea tarda; uroporphyrinogen III decarboxylase
Answer B Explanation	AnswerBExp::
Answer C	AnswerC::Acute intermittent porphyria; porphobilinogen deaminase
Answer C Explanation	AnswerCExp::
Answer D	AnswerD::Variegate porphyria; protoporphyrinogen oxidase
Answer D Explanation	AnswerDExp::
Answer E	AnswerE::Acute porphyria; ALA dehydrogenase deficiency
Answer E Explanation	AnswerEExp::
Right Answer	RightAnswer::B
Explanation	[[Explanation:: Educational Objective: References: ]]
Approved	Approved::No
Keyword
Linked Question	Linked::
Order in Linked Questions	LinkedOrder::