We report the case of a 55-year-old man with long-standing Crohn's disease with multiple fistulas and intestinal resections due to poor response to medical treatment.
After the last surgical intervention intestinal exclusion with discharge gastrostomy was performed and parenteral nutrition was initiated.
He was diagnosed with chronic liver disease not associated with portal vein thrombosis, esophageal varices and mild pancitretin.
The patient had a weight at discharge of 50 kg, height of 170 cm and BMI of 17.3 kg/m2.
At discharge, the patient was started on daily parenteral nutrition therapy consisting of 35 kcal/kg/day (14g nitrogen, 225 g glucose, 50 g lipid MCT/LCT).
During the first months, the evolution was favorable, with improvement in the general condition, pancytis, nutritional parameters and liver function.
At 6 months there was a deterioration of liver function with stasis and worsening of pancytis; therefore the intake of fats, trace elements and liposoluble vitamins decreased.
Subsequently, the general condition of the patient with fever and intense agitation worsened, requiring hospitalization.
Blood analysis at admission showed a significant increase in bilirubin, coagulopathy, severe thrombocytopenia, and decreased levels of albumin with ferritin in normal values.
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Abdominal ultrasound showed chronic liver disease with no data of biliary obstruction, portal thrombosis and stenosis.
The liver biopsy showed a maintained architecture, portal spaces with inflammatory infiltrates and biliary stasis predominantly centrolobular with hepatocyte necrosis phenomena.
Bone marrow aspiration showed preserved medullary cellularity with accumulation of histiocytes with lipofuccinic material PAS+ (marine blue erythrocytes) which constituted at least 20% of total cellularity.
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Three doses of anti-TNFα were administered as treatment for their underlying disease in addition to s-adenosylmethionine, with total elimination of fats in parenteral nutrition.
The evolution was unfavorable presenting hepatocellular failure, hepatic encephalopathy, gastrointestinal bleeding and nosocomial pneumonia, progressing to multiple organ failure and death.
