Severe Lactic Acidosis in a Parenteral Nutrition-Dependent Teenager with Ulcerative Colitis.

A 16-year-old male with previously diagnosed ulcerative colitis (UC) was initially admitted to an outside hospital because of bloody diarrhea. Total parenteral nutrition (TPN) was started after admission, but after failure of medical management of his UC with steroids and infliximab, he underwent a total colectomy with an end ileostomy. His postoperative course was complicated by pancreatitis and ileus, requiring prolonged nothing-bymouth status with TPN. On hospital day 32, he developed ataxia, tremors, and auditory hallucinations. Over the next 2 days, his serum bicarbonate concentration decreased to a low level of 11 mmol/L, and his serum lactate concentration rose to 16 mmol/L. Although his physicians were clinically suspicious of bowel ischemia or abdominal infection, an abdominal computed tomography (CT) scan was reported to show no evidence of either. A course of empiric broad-spectrum antibiotics was started; 3 days later, the patient developed hypotension, requiring vasopressor therapy and prophylactic intubation for impending respiratory failure. The patient then was transferred to our pediatric intensive care unit (PICU) for escalation of care. Despite multiple doses of sodium bicarbonate administered while en route to our hospital, the patient’s plasma pH was 6.8 upon arrival. He was immediately taken for exploratory laparotomy, which revealed a well-perfused bowel without any abnormalities. He was returned to the PICU where he required extensive support including IV insulin, the transfusion of multiple units of blood products, and continuous renal replacement therapy (CRRT) for refractory acidosis with a serum lactate[30 mmol/L. His ammonia had risen to 608 lmol/L and his serum creatinine to 2.3 mg/dL. There was suspicion for sepsis in the presence of an indwelling intravenous catheter, but the patient remained afebrile, had no leukocytosis, and had no elevation of C-reactive protein (C-RP) (2.0 mg/L). Noting the severity of the systemic acidosis, an astute PICU fellow located multiple case reports of patients with similar clinical features as a result of thiamine deficiency (beriberi) [1–10]. A call to the transferring facility confirmed the patient had been receiving TPN without IV multivitamins (MVIs) for the past 31 days. Two hours after IV administration of 400 mg thiamine, the patient’s pH rose from 6.78 to 7.17 and his serum bicarbonate concentration rose from 3.6 to 12.1 mmol/L. He was subsequently dosed with thiamine every 4 h starting 2 h after the initial dose. Within 6 h, his serum pH and bicarbonate concentrations normalized, and his vasopressors and insulin therapies were stopped; within 12 h, CRRT was discontinued; within 24 h, he was extubated, sedation was stopped, and his mental status improved. A brain MRI 3 days after his clinical decompensation revealed no abnormality, with none of the stigmata associated with Wernicke’s encephalopathy noted. The patient’s renal function did not completely recover; his creatinine prior to discharge was 1.4 mg/dL, which was attributed to renal hypoperfusion that occurred during the initial clinical deterioration. Three days later, the serum thiamine concentration from a sample obtained prior to his first dose was 7 nmol/L (normal 8–30 nmol/L). The transferring facility disclosed that they had no available supply of adult MVIs. A memorandum was sent notifying their providers. & John Kerner john.kerner@stanford.edu