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Microvillus Inclusion Disease Treatment & Management

  • Author: Stefano Guandalini, MD; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Jul 17, 2015
 

Medical Care

Several drugs have been tried to counteract the massive secretory diarrhea in patients with microvillus atrophy; however, none has proven effective. At present, the only available therapy is total parenteral nutrition (TPN). Agents tentatively given to induce a better growth of the intestinal mucosa (eg, epithelial growth factor, colostrum) are ineffective. Antisecretagogue agents (eg, somatostatin, octreotide, loperamide, chlorpromazine) can reduce the stool output, but the clinical significance of this effect is marginal.

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Surgical Care

Successful transplantation of the small intestine may allow for the patient's survival without TPN. Transplantation appears to be the only option for patients who do not fare well with long-term TPN (eg, because of sepsis, liver damage, lack of vascular access). Although only small series have been reported, evidence suggests that early small-bowel transplantation should be performed.

The analysis of 16 patients who underwent a small-bowel transplantation[18] shows anyway a lower death rate compared to those who did not (23% versus 37%) after an average 3.5 years observation period (but variable between 3 mo and 14 y). In all of the cases, apart from the first 2, the colon had been transplanted too.

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Diet

In most patients with early-onset MVID, no intake by mouth is possible. In the late-onset variant, minimal oral intake may be possible.

Except for rare, documented exceptions, no improvement of the condition is observed. Food intolerance remains complete in the overwhelming majority of patients described.

Long-term nutritional support is accomplished with TPN.

For patients in whom transplantation is successful, a gradual return to a normal diet is considered possible.

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Contributor Information and Disclosures
Author

Stefano Guandalini, MD Founder and Medical Director, Celiac Disease Center, Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medical Center; Professor, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, North American Society for the Study of Celiac Disease

Disclosure: Received consulting fee from AbbVie for consulting.

Coauthor(s)

Agostino Nocerino, MD, PhD Chief of Pediatric Oncology, Department of Pediatrics, University of Udine, Italy

Agostino Nocerino, MD, PhD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, Italian Society of Pediatric Hematology and Oncology, Italian Society of Pediatric Emergency and Urgent Care Medicine, Italian Society of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Additional Contributors

Chris A Liacouras, MD Director of Pediatric Endoscopy, Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania School of Medicine

Chris A Liacouras, MD is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

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