1. NYU Department of Medicine Grand Rounds Clinical Vignette Elizabeth Haskins, PGY 3 February 25, 2009

2. Medicine-Surgery Conference Thursday, February 26th, at 5pm Hepatocellular Carcinoma: Hepatocellular Carcinoma: Current Treatment Options Speakers: Dr. Umut Sarpel Assistant Professor of Surgery; Surgical Oncology Dr. Thomas Diflo Dr. Thomas Diflo Associate Professor of Surgery; Transplant Surgery Farber Auditorium (Bellevue Atrium) Food will be served

3. Chief Complaint The patient is an 18 year old female who presents to clinic for routine follow-up.

4. History of Present Illness At 8 months of age, the patient developed hypoglycemic coma. Work-up at the time included a liver biopsy which made the diagnosis of Glycogen Storage Disease, type 1a This was confirmed on mutation analysis. She was managed with frequent feeds during the day and nocturnal nasogastric feeds. At the age of 16, uncooked corn starch replaced her nocturnal feeds. She had been doing well and one year prior to the current visit, she was noted to have normal hematocrit, iron studies and a normal abdominal ultrasound.

5. Additional History Past Medical History: –Glycogen Storage Disease, type 1a Past Surgical History: –None Social History: –Denies any toxic habits Family History: –No other known genetic disorders in her family Allergies: No Known Drug Allergies Medications: none

6. Physical Exam General: well appearing female in no acute distress Vital Signs: T: 98 BP: 105/65 HR: 70 RR: 12 The remainder of the physical exam was normal

7. Laboratory Hemoglobin: 9.8 g/dL Hematocrit: 29.4% MCV: 80.2 fL RDW: 15.3% Iron: 20 mcg/dL TIBC: 301 mcg/dL Saturation: 6.6% Ferritin: 67.2 ng/mL Sedimentation Rate: 100 mm/hr

8. Working Diagnosis Microcytic Anemia –Likely a result of chronic glucose-6- phosphatase deficiency

9. Hospital Course An Abdominal Ultrasound was obtained, revealing: –Single, large hepatic adenoma Oral iron was initiated, but her hematocrit remained low.

10. Hospital Course Follow-up MRI 3 months later demonstrated enlargement of the adenoma:

11. Hospital Course 6 months later, the patient underwent resection of the adenoma 6 weeks after her surgery, her labs were as follows: –Hematocrit 38% –Iron 100 mcg/dL –Ferritin 4 ng/mL –Erythrocyte Sedimentation Rate 10 mm/hr

12. Final Diagnosis Hepatic adenoma Microcytic anemia