Long-Term Parenteral Nutrition in Pediatric Autoimmune Enteropathies

Gambarara, M.; Bracci, F.; Diamanti, Antonella; Ambrosini, M; Pietrobattista, Andrea; Knafelz, D.; Ferretti, Francesca; Castro, M.

doi:10.1016/j.transproceed.2005.03.063

Cited by 11 publications

(6 citation statements)

References 7 publications

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“…However, some intestinal failure patients, notably if they have a short remaining bowel, a congenital epithelium disease, or extended aganglionosis, cannot achieve satisfactory intestinal autonomy and become definitively dependent on PN (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). These patients, dependent on PN for years, are considered to have permanent or irreversible intestinal failure and are candidates for intestinal transplantation.…”

mentioning

confidence: 99%

Irreversible Intestinal Failure: Prevalence and Prognostic Factors

Diamanti¹,

Basso²,

Castro³

et al. 2008

J. pediatr. gastroenterol. nutr.

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mentioning

confidence: 99%

Irreversible Intestinal Failure: Prevalence and Prognostic Factors

Diamanti¹,

Basso²,

Castro³

et al. 2008

J. pediatr. gastroenterol. nutr.

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“…Only one paper described total parenteral nutrition (PN) in 10 pediatric AIE patients. The results showed five of them were weaned off PN, three died of sepsis after a mean period of 18 months; one underwent total colectomy and one patient is still dependent on TPN for 24 months 6. Our paper describes our experience with glutamine-supplemented PN in managing adult patients with AIE and its related malnutrition.…”

Section: Introductionmentioning

confidence: 94%

Glutamine-Supplemented Parenteral Nutrition and Probiotics in Four Adult Autoimmune Enteropathy Patients

Yan

Zhou

et al. 2014

Gut Liver

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To evaluate the effects of glutamine-supplemented parenteral nutrition (PN) and probiotics in adult autoimmune enteropathy (AIE) patients. Four adult AIE patients were identified from April 2006 to January 2012. Clinical and nutritional data were obtained from the patients' medical records. Glutamine-supplemented PN started immediately when the AIE diagnosis was confirmed. The total PN duration was 351 days. According to the PN prescription, the average caloric intake ranged from 20 to 25 kcal/kg/day, and the protein intake ranged from 1.2 to 1.5 g/kg/day. Alanyl-glutamine (20 g/day) was administered to AIE patients for 4 weeks followed by a 2-week break, and this treatment schedule was repeated when PN lasted for more than 6 weeks. Body weight gain and an increased serum albumin level were achieved after PN, and defecation frequency and quality also improved. Each patient received oral supplements, 250 mL of Ensure and two probiotics capsules (each capsule containing 0.5×108 colonies) three times a day when enteral nutrition started. Three AIE patients were successfully weaned off PN, and one patient died of pneumonia. Glutamine-supplemented PN and probiotics show promise in managing patients with AIE and related malnutrition.

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“…However, advancements in medicine over the past three decades since the discovery of this condition have likely improved long-term survival. Multiple factors determine long-term outcomes, including severity of symptoms, age of presentation, and need for parenteral nutrition [5,81]. Although the prognosis of autoimmune enteropathy has improved with the use of immunosuppressive medications and aggressive nutritional support, close monitoring for complications is critical.…”

Section: Prognosis and Long-term Outcomesmentioning

confidence: 99%

A Review of Autoimmune Enteropathy and Its Associated Syndromes

et al. 2020

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Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. Affected individuals frequently present with other signs of autoimmunity. The diagnosis is made based on a characteristic combination of clinical symptoms, laboratory studies, and histological features on small bowel biopsy. Autoimmune enteropathy is associated with a number of other conditions and syndromes, most notably immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome and autoimmune polyglandular syndrome type 1 (APS-1). Diagnosis and treatment is challenging, and further research is needed to better understand the pathogenesis, disease progression, and long-term outcomes of these conditions.

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Long-Term Parenteral Nutrition in Pediatric Autoimmune Enteropathies

Cited by 11 publications

References 7 publications

Irreversible Intestinal Failure: Prevalence and Prognostic Factors

Irreversible Intestinal Failure: Prevalence and Prognostic Factors

Glutamine-Supplemented Parenteral Nutrition and Probiotics in Four Adult Autoimmune Enteropathy Patients

A Review of Autoimmune Enteropathy and Its Associated Syndromes

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