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

Tang, Powei; Lu, Lina; Yan, Wei; Tao, Y; Feng, Haixia; Cai, Wei; Wang, Ying

doi:10.1002/ncp.10929

Cited by 3 publications

(2 citation statements)

References 28 publications

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“…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%

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: Nutritional Assessmentmentioning

confidence: 99%

Optimizing nutrition in pediatric intestinal pseudo‐obstruction syndrome

Pescarin

Day

Thapar

et al. 2023

Neurogastroenterology Motil

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“…Visceral myopathies (VMs), including chronic intestinal pseudo-obstruction, pediatric-onset intestinal pseudo-obstruction, and megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), are a group of rare diseases that often cause death in childhood ( 1 – 3 ). VM arises from smooth muscle dysfunction in the bowel, bladder, and uterus ( 1 ).…”

Section: Introductionmentioning

confidence: 99%

Molecular mechanisms linking missense ACTG2 mutations to visceral myopathy

Ceron,

Báez-Cruz,

Palmer

et al. 2024

Sci. Adv.

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Visceral myopathy is a life-threatening disease characterized by muscle weakness in the bowel, bladder, and uterus. Mutations in smooth muscle γ-actin (ACTG2) are the most common cause of the disease, but the mechanisms by which the mutations alter muscle function are unknown. Here, we examined four prevalent ACTG2 mutations (R40C, R148C, R178C, and R257C) that cause different disease severity and are spread throughout the actin fold. R178C displayed premature degradation, R148C disrupted interactions with actin-binding proteins, R40C inhibited polymerization, and R257C destabilized filaments. Because these mutations are heterozygous, we also analyzed 50/50 mixtures with wild-type (WT) ACTG2. The WT/R40C mixture impaired filament nucleation by leiomodin 1, and WT/R257C produced filaments that were easily fragmented by smooth muscle myosin. Smooth muscle tropomyosin isoform Tpm1.4 partially rescued the defects of R40C and R257C. Cryo–electron microscopy structures of filaments formed by R40C and R257C revealed disrupted intersubunit contacts. The biochemical and structural properties of the mutants correlate with their genotype-specific disease severity.

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Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo‐obstruction in the Netherlands: A 20‐year retrospective cohort study

Demirok,

Nagelkerke,

Veldt

et al. 2024

J. pediatr. gastroenterol. nutr.

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ObjectivesTo describe incidence, clinical course, diagnostic and therapeutic management and long‐term follow‐up of paediatric intestinal pseudo‐obstruction (PIPO) in the Netherlands between 2000 and 2020.MethodsMulticenter, national, retrospective, observational study including patients aged <18 years diagnosed with PIPO and treated between 2000 and 2020 in Dutch academic medical centres. Outcomes included demographics, incidence, symptoms, diagnostic‐ and treatment methods used during follow‐up, number of hospital admissions and mortality.ResultsBetween 2000 and 2020, 43 children (median age 120 months, range 13 – 301, 54% female) were diagnosed with PIPO in the Netherlands. Mean incidence was 0.008/100,000/years (range 0/100 000–0.029/100 000). Twenty‐six patients developed PIPO in the neonatal period. Initial symptoms were vomiting (n = 21/35, 60%) and abdominal distension (n = 14/35, 40%). Diagnostic strategies included imaging, manometry, histopathology, metabolic‐ and genetic screening, endoscopy and exploratory surgery. Treatment was divided in nutritional support, pharmacotherapy, colonic irrigation and surgical interventions, of which nutrition and surgery were the cornerstones for care. During the observed study period, the median number of hospital admissions was 22.5 (range 1–176) with a median of 157.5 days (range 3–840) during 20‐year follow‐up. Two patients (6%) died: one from sepsis and one due to a severe underlying neurological disease. Heterogeneity in diagnostic‐ en treatment methods existed between patients.ConclusionsPIPO is a rare, long‐lasting complex disease requiring a high number of diagnostic and therapeutic interventions and hospital admissions. However, mortality rate is relatively low. Based on our results, we recommend centralization and standardization of care for this complex rare disease.

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

Cited by 3 publications

References 28 publications

Optimizing nutrition in pediatric intestinal pseudo‐obstruction syndrome

Optimizing nutrition in pediatric intestinal pseudo‐obstruction syndrome

Molecular mechanisms linking missense ACTG2 mutations to visceral myopathy

Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo‐obstruction in the Netherlands: A 20‐year retrospective cohort study

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