ABSTRACT
PURPOSE OF REVIEW: Pediatric intestinal failure is a complex condition requiring specialized care to prevent potential complications. In this article, we review the available evidence supporting recent advances in care for children with intestinal failure. RECENT FINDINGS: Multidisciplinary intestinal rehabilitation teams utilize medical and surgical management techniques to help patients achieve enteral autonomy (EA) while preventing and treating the complications associated with intestinal failure. Recent advances in lipid management strategies, minimization of intestinal failure associated liver disease, prevention of central line-associated blood stream infections, and loss of access, as well as development of promising new hormone analogue therapy have allowed promotion of intestinal adaptation. These advances have decreased the need for intestinal transplant. There have been recent advances in the care of children with intestinal failure decreasing morbidity, mortality, and need for intestinal transplantation. The most promising new therapies involve replacement of enteroendocrine hormones.
Subject(s)
Enteral Nutrition , Intestinal Diseases/therapy , Short Bowel Syndrome/therapy , Catheterization, Central Venous/adverse effects , Catheterization, Central Venous/methods , Child , Chronic Disease , Fat Emulsions, Intravenous/administration & dosage , Hormones/therapeutic use , Humans , Intestinal Diseases/diagnosis , Intestinal Diseases/etiology , Intestinal Diseases/rehabilitation , Intestinal Pseudo-Obstruction/diagnosis , Intestinal Pseudo-Obstruction/etiology , Intestinal Pseudo-Obstruction/rehabilitation , Intestinal Pseudo-Obstruction/therapy , Intestines/transplantation , Organ Transplantation , Parenteral Nutrition , Short Bowel Syndrome/diagnosis , Short Bowel Syndrome/etiology , Short Bowel Syndrome/rehabilitationABSTRACT
Abstract A 28-year-old woman with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE syndrome) undergoing evaluation for multichannel cochlear implantation is described. The case history, diagnosis of mitochondrial disease, and assessment of the benefits of cochlear implantation are documented. The hearing level with cochlear implant and speech recognition were improved significantly for this patient. MNGIE syndrome is a rare congenital disorder of mitochondrial DNA (mt-DNA). It is crucial for the otolaryngologist to have awareness of MNGIE syndrome and other mitochondrial encephalomyopathies when patients present with sensorineural hearing loss (SNHL). Cochlear implantation can be recommended to patients with MNGIE syndrome and satisfactory results can be achieved.
