A 29 year-old male presents for an annual physical exam and is found to have proteinuria. He was diagnosed with “proteinuria” in February 2015. During an exam in July 2015, he was found to have lower extremity swelling and frothy urine, which was evaluated by PMD and referred to nephrology. A renal biopsy performed in December 2015. He was seen for an initial consultation February 2016.

His family history was negative for kidney disease. He denies tobacco or illicit drug use. He drinks alcohol occasionally twice per week. Medications include losartan 25 mg once daily. There are no known drug allergies. Review of systems found occasional blurred vision, mild lower extremity swelling at the end of the day, but otherwise negative.

Physical Exam is as follows: BP 130/75, P 74, T 98.4F, Ht 72 in, Wt 192 lbs, BMI 26 kg/m2. Gen NAD. HEENT NC/AT PERRL EOMI op clear. Neck supple no thyromegaly, no LAD. CV RRR S1 S2 heard, no m/g/r, no JVP. Lungs CTAB. Abd soft nd +bs. Skin no rashes. Musculoskeletal normal tone, no joint effusions. Ext no edema. Neuro nonfocal.

Labs are as follows: Hgb 13.9, wbc 6.0, plts 239. creat 1.0, eGFR 111 ml/min, BUN 10. Na 139, K 4.2, TCO2 26, Alb 3.9. UA:  pH 5.5/s.g., 1.025/3+ protein/neg heme/neg, nitrite/neg LE. 0-2 rbc/hpf, 0-5 wbc/hpf, no casts visualized. Urine protein-creatinine 1875 mg/g create.

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Renal Biopsy is as follows:

Renal Biopsy is as follows:

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* 1. What is the next best step in management?

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* 2. What is your level of confidence that your choice is correct?

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* 3. You are visiting a family with newborn twins, a girl and a boy. The father has just been diagnosed with Fabry disease. They are seeking advice for their children involvement by the disease.

Which one of the following is true:

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* 4. What is your level of confidence that your choice is correct?

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