Chronic Intestinal Pseudo-Obstruction (Cipo) Syndrome. Results of a Multicentric Survey by Members of the French-Speaking Group of Pediatric Gastroenterology.

Faure, C; Goulet, O.; Ategbo, S; Breton, A; Girardet, Jean-Philippe; Giniès, J.L.; Scaillon, Michèle; Maurage, Claude‐Alain; Paquot, Isabelle; Hermier, M; Napoli, Sara; Digeon, B.; Dabadie, A; Huet, F.; Baudon, Jean-Jacques; Larchet, M

doi:10.1097/00005176-199704000-00047

Cited by 7 publications

(47 citation statements)

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“…PIPO is a chronic, debilitating disease that can be progressive in some patients 17,18 . The risk factors related to worsening enteral feed tolerance include onset of symptoms at birth, acute onset, presence of urinary tract involvement (e.g., megacystis, microcolon, and hypoperistalsis syndrome), gut malrotation, long‐term PN dependency, myopathic changes on histology, and requirement of surgery 13,97 . Moreover, as described before, low motility index during the phase III of migrating motor complex, documented via an antroduodenal manometry, is a predictor of poor clinical outcome, dependence on parenteral nutrition and death 102 …”

Section: Long‐term Outcomes Of Pipomentioning

confidence: 83%

“…Poor nutritional state, inability to achieve normal growth and maintain a normal body weight are observed in up to 72% of PIPO patients and are associated with longer hospital admissions and worse clinical outcome 5,13,14 . As mentioned earlier, the severity of digestive symptoms triggered by food ingestion, abnormal intestinal absorption, and the side effects of medications represent the main contributing factors to undernutrition in PIPO 15 .…”

Section: Nutritional Assessmentmentioning

confidence: 99%

“…Approximately one third of PIPO patients are able to tolerate oral feeds, one third require enteral nutrition and the final third need partial or total parenteral nutrition (PN) support (range 38–70%) 2,9,13,26,49,97 . Faure et al 13 reported that, in a cohort of 105 patients diagnosed with PIPO, 83% of children needed nutritional support, of which 42% were on total PN, 33% needed mixed enteral feeding and PN, and 9% received only enteral nutrition. A recent Italian epidemiological study revealed that 21% of total pediatric patients with chronic IF on home PN had motility disorders; of these patients, 64% had a diagnosis of PIPO 98 .…”

Section: Parenteral Nutritionmentioning

confidence: 99%

“…2,9,13,26,49,97 Faure et al13 reported that, in a cohort of 105 Diamanti et al26 showed 20% of patients developed irreversible IF requiring life-long PN. In the same study, comparison between neonatal and late onset PIPO showed that the latter was associated with a reduced requirement of PN and surgery.…”

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confidence: 99%

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Optimizing nutrition in pediatric intestinal pseudo‐obstruction syndrome

Pescarin

Day

Thapar

et al. 2023

Neurogastroenterology Motil

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Background Pediatric intestinal pseudo‐obstruction (PIPO) encompasses a variety of rare, heterogeneous, and disabling disorders that severely affect gastrointestinal motility and are associated with high morbidity and mortality. PIPO management is complex and focuses on maintaining an optimal nutritional status, improving gut function, relieving symptoms, and treating complications. Nutritional issues prevail, and PIPO patients often experience severe undernutrition and faltering growth. Thus, nutritional management plays a pivotal role for achieving the most favorable clinical outcomes. The calorie and nutrient intake of each patient needs to be tailored to age, extent and severity of gut involvement and nutritional needs to support an optimal nutritional status. After defining the extent and severity of gut dysmotility, an experienced team should perform a careful nutritional assessment. An oral diet should always be encouraged and might include bite and dissolve solids, liquid diet or simple oral stimulation. If oral caloric intake is inadequate, liquid gastric feeds should provide the subsequent step. In the presence of severe gastric dysmotility, continuous post‐pyloric feeding represents a viable option. In the most severe cases, parenteral nutrition (PN) is required to meet appropriate nutritional requirements. Purpose Pediatric data on this topic are scarce and mainly extrapolated from adult studies. In this review, we discuss current evidence and knowledge regarding nutritional options, implications of the use of different feed types, including a blended diet, and the use of PN. Moreover, based on our experience and the evidence from the literature, we propose a flow chart to guide the nutritional management of PIPO patients.

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Section: Long‐term Outcomes Of Pipomentioning

confidence: 83%

Section: Nutritional Assessmentmentioning

confidence: 99%

Section: Parenteral Nutritionmentioning

confidence: 99%

mentioning

confidence: 99%

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Optimizing nutrition in pediatric intestinal pseudo‐obstruction syndrome

Pescarin

Day

Thapar

et al. 2023

Neurogastroenterology Motil

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“…The mortality rate in children range from 10% to 40%. [1][2][3][4] The patients usually have malabsorption and insufficient oral intake due to severe GI motility disorders, leading to growth and developmental decline. Up to twothirds of patients with PIPO develop nutrition problems or specific nutrient deficiencies.…”

Section: Introductionmentioning

confidence: 99%

Long‐term follow‐up for pediatric intestinal pseudo‐obstruction patients in China

Tang

Yan

et al. 2022

Nut in Clin Prac

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Background: Pediatric intestinal pseudo-obstruction (PIPO) is a heterogeneous and severe group of disorders with a high mortality rate. Patients with PIPO often develop malnutrition and need long-term nutrition support. This study aimed to determine the nutrition status, particularly micronutrients, during the long-term follow-up of patients with PIPO.Methods: Fifty-eight patients with PIPO were followed up for at least 6 months between January 2008 and December 2020 in our hospital. PIPO was diagnosed based on the European society for pediatric gastroenterology, hepatology, and nutrition consensus. Data on clinical characteristics, medical and surgical management, nutrition support, serum vitamins, and mineral concentrations were collected. The patients were divided into the early-onset PIPO (EO-PIPO; neonatal-onset) and late-onset PIPO (LO-PIPO; infant-or child-onset) groups. Results: The mean follow-up was 29.5 months (6-153 months). The overall survival rate was 63.8% (37 out of 58 participants) (EO-PIPO, 48.6% [17 out of 35 participants]; LO-PIPO, 87.0% [20 out of 23 participants]). Mortality in the EO-PIPO group was higher than in the LO-PIPO group (P = 0.002). Twentyone patients died, of which 18 (85.7%) patients had EO-PIPO and 14 (66.7%) patients died under 1 year of age. Infection was the major cause of death.Severe malnutrition was observed at baseline and during follow-up in 25 (43.1%) and 6 (16.2%) patients, respectively. At baseline and during follow-up, the zinc deficiency rates were 29.6% and 26.3%, and those of vitamin D were 26.9% and 52.6%, respectively. Conclusions: Zinc and vitamin D deficiencies are common in patients with PIPO during follow-up. Therefore, additional supplements should be recommended.

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