RESUMO
Primary intestinal lymphangiectasia is a rare disorder that may result in protein-losing enteropathy. We report a 21-year-old man with malabsorption syndrome, an unintentional weight loss of 30 kg over 10 months, lymphocytopenia, and hepatic aminotransferase elevation. His diagnosis was established by a combination of enteroscopy, histopathology, and secondary etiology exclusion. Institution of parenteral nutrition, followed by a low-fat diet, medium-chain triglycerides, and octreotide, resulted in the resolution of his symptoms and laboratory abnormalities and led to weight gain. Aminotransferase abnormalities are an atypical finding in primary intestinal lymphangiectasia and were most likely due to nonalcohol steatohepatitis after rapid weight loss. Primary intestinal lymphangiectasia should be considered in patients with protein-losing enteropathy and lymphocytopenia.
RESUMO
The rendering of proper care for the patient with intestinal failure requires the provider to have a functional understanding of digestion and absorption, nutrient requirements, and intestinal adaptation. Inherent in those concepts is that not only is nutritional absorption compromised, but medication absorption is as well. The principles of the management of home parenteral nutrition must be mastered and then proper and controlled weaning of parenteral nutrition may be commenced by use of dietary and pharmacologic means with appropriate clinical outcome measures followed. This complicated management requires a team experienced in both medical and surgical management of intestinal failure.