NYU Department of NYU Department of Medicine Grand Rounds Medicine Grand Rounds Clinical Vignette Clinical Vignette Elizabeth Haskins, PGY Elizabeth Haskins, PGY 3 3 February 25, 2009 February 25, 2009
Dec 15, 2015
NYU Department of Medicine NYU Department of Medicine Grand RoundsGrand Rounds
Clinical VignetteClinical Vignette
Elizabeth Haskins, PGY 3Elizabeth Haskins, PGY 3
February 25, 2009February 25, 2009
Medicine-Surgery ConferenceMedicine-Surgery ConferenceThursday, February 26th, at 5pmThursday, February 26th, at 5pm
Hepatocellular Carcinoma:Hepatocellular Carcinoma:Current Treatment OptionsCurrent Treatment Options
Speakers:Speakers:
Dr. Umut SarpelDr. Umut SarpelAssistant Professor of Surgery; Surgical OncologyAssistant Professor of Surgery; Surgical Oncology
Dr. Thomas DifloDr. Thomas DifloAssociate Professor of Surgery; Transplant SurgeryAssociate Professor of Surgery; Transplant Surgery
Farber Auditorium (Bellevue Atrium)Farber Auditorium (Bellevue Atrium)
Food will be servedFood will be served
Chief ComplaintChief Complaint
The patient is an 18 year old female who The patient is an 18 year old female who presents to clinic for routine follow-up. presents to clinic for routine follow-up.
History of Present IllnessHistory of Present Illness
At 8 months of age, the patient developed At 8 months of age, the patient developed hypoglycemic coma.hypoglycemic coma.Work-up at the time included a liver biopsy which Work-up at the time included a liver biopsy which made the diagnosis of Glycogen Storage Disease, made the diagnosis of Glycogen Storage Disease, type 1atype 1aThis was confirmed on mutation analysis.This was confirmed on mutation analysis.She was managed with frequent feeds during the day She was managed with frequent feeds during the day and nocturnal nasogastric feeds.and nocturnal nasogastric feeds.At the age of 16, uncooked corn starch replaced her At the age of 16, uncooked corn starch replaced her nocturnal feeds.nocturnal feeds.She had been doing well and one year prior to the She had been doing well and one year prior to the current visit, she was noted to have normal hematocrit, current visit, she was noted to have normal hematocrit, iron studies and a normal abdominal ultrasound.iron studies and a normal abdominal ultrasound.
Additional HistoryAdditional History
Past Medical History:Past Medical History:– Glycogen Storage Disease, type 1aGlycogen Storage Disease, type 1a
Past Surgical History:Past Surgical History:– NoneNone
Social History:Social History:– Denies any toxic habitsDenies any toxic habits
Family History:Family History:– No other known genetic disorders in her familyNo other known genetic disorders in her family
Allergies: No Known Drug AllergiesAllergies: No Known Drug AllergiesMedications: noneMedications: none
Physical ExamPhysical Exam
General: well appearing female in no General: well appearing female in no acute distressacute distress
Vital Signs: T: 98 BP: 105/65 HR: 70 Vital Signs: T: 98 BP: 105/65 HR: 70 RR: 12RR: 12
The remainder of the physical exam The remainder of the physical exam was normalwas normal
LaboratoryLaboratoryHemoglobin: 9.8 g/dLHemoglobin: 9.8 g/dLHematocrit: 29.4%Hematocrit: 29.4%MCV: 80.2 fLMCV: 80.2 fLRDW: 15.3%RDW: 15.3%
Iron: 20 mcg/dLIron: 20 mcg/dLTIBC: 301 mcg/dLTIBC: 301 mcg/dLSaturation: 6.6%Saturation: 6.6%
Ferritin: 67.2 ng/mLFerritin: 67.2 ng/mLSedimentation Rate: 100 mm/hrSedimentation Rate: 100 mm/hr
Working DiagnosisWorking Diagnosis
Microcytic AnemiaMicrocytic Anemia– Likely a result of chronic glucose-6-Likely a result of chronic glucose-6-
phosphatase deficiencyphosphatase deficiency
Hospital CourseHospital Course
An Abdominal Ultrasound was obtained, An Abdominal Ultrasound was obtained, revealing:revealing:– Single, large hepatic adenomaSingle, large hepatic adenoma
Oral iron was initiated, but her hematocrit Oral iron was initiated, but her hematocrit remained low.remained low.
Hospital CourseHospital Course Follow-up MRI 3 months later
demonstrated enlargement of the adenoma:
Hospital CourseHospital Course
6 months later, the patient underwent 6 months later, the patient underwent resection of the adenomaresection of the adenoma
6 weeks after her surgery, her labs were as 6 weeks after her surgery, her labs were as follows:follows:– Hematocrit 38%Hematocrit 38%– Iron 100 mcg/dLIron 100 mcg/dL– Ferritin 4 ng/mLFerritin 4 ng/mL– Erythrocyte Sedimentation Rate 10 mm/hrErythrocyte Sedimentation Rate 10 mm/hr