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

Final DiagnosisFinal Diagnosis

Hepatic adenomaHepatic adenoma

Microcytic anemiaMicrocytic anemia