Intestinal transplantation in children with chronic intestinal pseudo-obstruction. 1999

L Sigurdsson, and J Reyes, and S A Kocoshis, and G Mazariegos, and K M Abu-Elmagd, and J Bueno, and C Di Lorenzo
Department of Paediatric Gastroenterology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213-2583, USA.

BACKGROUND Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN) which puts them at risk of liver failure and recurrent line infections. Intestinal transplantation has become a therapeutic option for TPN dependent children with intestinal failure who are failing management with TPN. OBJECTIVE To investigate the outcome of children with CIPO referred for intestinal transplantation. METHODS A retrospective review was carried out of records and diagnostic studies from 27 patients with CIPO referred for intestinal transplantation. RESULTS Five children were not listed for transplantation: two because of parental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependent and awaiting transplantation. Eight children died awaiting transplantation. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a median follow up of 2.6 years (range two months to six years). All transplanted children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, narcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in children with CIPO. Early referral and thorough pretransplant evaluation are keys to successful transplantation.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007418 Intestinal Pseudo-Obstruction A type of ILEUS, a functional not mechanical obstruction of the INTESTINES. This syndrome is caused by a large number of disorders involving the smooth muscles (MUSCLE, SMOOTH) or the NERVOUS SYSTEM. CIPO,Chronic Idiopathic Intestinal Pseudo-Obstruction,Paralytic Ileus,Pseudo-Obstruction, Intestinal,Visceral Myopathy,Congenital Short Bowel Syndrome,Enteric Neuropathy,Intestinal Pseudo-Obstruction, Idiopathic,Intestinal Pseudoobstruction,Pseudointestinal Obstruction Syndrome,Pseudoobstructive Syndrome,Chronic Idiopathic Intestinal Pseudo Obstruction,Enteric Neuropathies,Idiopathic Intestinal Pseudo-Obstruction,Idiopathic Intestinal Pseudo-Obstructions,Intestinal Pseudo Obstruction,Intestinal Pseudo Obstruction, Idiopathic,Intestinal Pseudo-Obstructions,Intestinal Pseudo-Obstructions, Idiopathic,Intestinal Pseudoobstructions,Myopathy, Visceral,Neuropathy, Enteric,Obstruction Syndrome, Pseudointestinal,Pseudo Obstruction, Intestinal,Pseudointestinal Obstruction Syndromes,Pseudoobstruction, Intestinal,Pseudoobstructive Syndromes,Syndrome, Pseudointestinal Obstruction,Syndrome, Pseudoobstructive,Visceral Myopathies
D007422 Intestines The section of the alimentary canal from the STOMACH to the ANAL CANAL. It includes the LARGE INTESTINE and SMALL INTESTINE. Intestine
D008297 Male Males
D010289 Parenteral Nutrition, Total The delivery of nutrients for assimilation and utilization by a patient whose sole source of nutrients is via solutions administered intravenously, subcutaneously, or by some other non-alimentary route. The basic components of TPN solutions are protein hydrolysates or free amino acid mixtures, monosaccharides, and electrolytes. Components are selected for their ability to reverse catabolism, promote anabolism, and build structural proteins. Hyperalimentation, Parenteral,Intravenous Hyperalimentation,Nutrition, Total Parenteral,Parenteral Hyperalimentation,Total Parenteral Nutrition,Hyperalimentation, Intravenous
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D005260 Female Females

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