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- Chaya N. Murali, MD*,†
- Rebecca Ganetzky, MD‡,§
- *Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX
- †Texas Children’s Hospital, Houston, TX
- ‡Division of Genetics, Section of Metabolic Disease, Children’s Hospital of Philadelphia, Philadelphia, PA
- §University of Pennsylvania Perelman School of Medicine, Philadelphia, PA
AUTHOR DISCLOSURE
Drs Murali and Ganetzky have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.
Presentation
An 18-month-old girl with juvenile myelomonocytic leukemia develops lactic acidosis. She was diagnosed with juvenile myelomonocytic leukemia at 14 months old and received a bone marrow transplant (BMT) at 17 months of age.
The hospital course is complicated by severe anorexia, and enteral intake is limited to minimal human milk. On day 1 after BMT, she begins on total parenteral nutrition (TPN) with intravenous (IV) multivitamins and intralipids (IL). On day 11 of TPN/IL, she develops hives and emesis coinciding with start of the TPN/IL infusion, concerning for an allergy. The next day, TPN alone is administered and tolerated. When IL is reintroduced, she again develops hives. Allergy consultants review the case, and diagnose an allergy to IL. Exclusive TPN is continued, without multivitamins/IL, for 4 weeks.
On day 20 post-BMT, she begins to take an oral multivitamin. Multiple attempts at enteral feeds have occurred, but she has emesis, food refusal, and gut graft-versus-host disease. On day 46, she develops tachycardia and tachypnea. Laboratory testing reveals metabolic acidosis with a bicarbonate level of 14 mmol/L with anion gap 25, and an elevated lactate level of 11.5 mmol/L. Pyruvate is also elevated, at 0.45 mmol/L (normal 0.05 to 0.14 mmol/L). She is treated empirically for sepsis, but cultures …
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