Pharmacological and nutritional therapy of children and adults with chronic intestinal pseudo-obstruction

Nardo, Giovanni Di; Zenzeri, Letizia; Guarino, Matteo; Molfino, Alessio; Parisi, Pasquale; Barbara, Giovanni; Stanghellini, Vincenzo; Giorgio, Roberto De

doi:10.1080/17474124.2023.2193887

Cited by 3 publications

(7 citation statements)

References 155 publications

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“…The deterioration of bowel function caused by CIPO [1][2][3][4]6] could impair the absorption of 25-hydroxyvitamin D and lead to a vitamin D deficiency. Also, intestinal inflammation may play a role in the malabsorption of vitamin D; in fact, most patients with intestinal bowel disease have a 25-hydroxy vitamin D deficiency [13].…”

Section: Discussionmentioning

confidence: 99%

“…Idiopathic chronic intestinal pseudoobstruction (CIPO) syndrome is a rare disease characterized by derangement of gastrointestinal propulsive motility that causes intestinal failure with variable degree of severity [1][2][3]. The pathogenesis of idiopathic CIPO is not well defined [2,4,5]. This syndrome may be due to alterations of enteric and extrinsic nervous system, interstitial cells of Cajal and smooth muscle cells, causing neuropathy, mesenchymopathy, myopathy or mixed forms [4,6].…”

Section: Introductionmentioning

confidence: 99%

“…The association between idiopathic CIPO and serum levels of 25-hydroxy-vitamin D is not clear. The reduced intestinal motility, impairment of the intestinal environment and chronic inflammation due to slowed intestinal transit may cause malabsorption of various macro and micronutrients that could lead to serum 25-hydroxy-vitamin D deficiency [2][3][4]6]. On the other hand, 25-hydroxy-vitamin D deficiency may negatively affect the gut microbiome, the mucosal immunity and the intestinal permeability and motility, thus contributing to the severity of disease.…”

Section: Introductionmentioning

confidence: 99%

“…The pathogenesis of idiopathic CIPO is not well defined [ 2 , 4 , 5 ]. This syndrome may be due to alterations of enteric and extrinsic nervous system, interstitial cells of Cajal and smooth muscle cells, causing neuropathy, mesenchymopathy, myopathy or mixed forms [ 4 , 6 ]. There are three main phenotypes of idiopathic CIPO: predominant neuropathy (RAD21-related or SGO-1-related, with neuronal alterations to the interstitial cells of Cajal), myopathy (ACTG2-dependent) and neuro-myopathy caused by mitochondrial dysfunction (TYMP-, POLG-, and LIG3-related) [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

“…From a clinical point of view, patients with CIPO usually present with recurrent intestinal subocclusive episodes, significant weight loss and severe malnutrition. Patients are often unable to undertake oral nutrition, with serious consequences on morbidity and mortality, and therefore require parenteral nutrition [ 3 , 4 ]. Most often, idiopathic CIPO remains unrecognized for long time, so that patients almost invariably undergo repeated, useless and potentially dangerous surgical procedures.…”

Section: Introductionmentioning

confidence: 99%

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Idiopathic chronic intestinal pseudo-obstruction syndrome is strongly associated with low serum levels of vitamin D

Panarese,

Dajti,

Eusebi

et al. 2024

European Journal of Gastroenterology &Amp; Hepatology

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Idiopathic chronic intestinal pseudo-obstruction (CIPO) is associated with intestinal inflammation and malabsorption and may cause serum vitamin D deficiency. We aimed to assess whether there is an association between idiopathic CIPO and serum levels of 25-hydroxy-vitamin D. Consecutive patients with confirmed diagnosis of idiopathic CIPO were prospectively enrolled and matched with healthy controls by gender, age, and BMI. Median serum level of 25-hydroxy-vitamin D of patients with CIPO was compared with that of healthy subjects using the Wilcoxon signed-rank test for matched samples. A total of 35 patients with CIPO and 35 matched healthy subjects were enrolled. All patients with CIPO had a 25-hydroxy-vitamin D deficiency with serum levels <12 ng/ml. The median serum level of vitamin D was significantly lower in patients with CIPO than in healthy controls (5.7 vs. 29.7 ng/ml, P < 0.0001). Serum level of vitamin D was not associated with gender (P = 0.27), age (P = 0.22), BMI (P = 0.95), high (>10 000 × ml) WBC count (P = 0.08), or high (>5 mg/l) C-reactive protein (P = 0.87) among patients with CIPO. CIPO seems to be strongly associated with low serum levels of 25-hydroxy-vitamin D.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Idiopathic chronic intestinal pseudo-obstruction syndrome is strongly associated with low serum levels of vitamin D

Panarese,

Dajti,

Eusebi

et al. 2024

European Journal of Gastroenterology &Amp; Hepatology

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Chronic intestinal pseudo-obstruction: associations with gut microbiota and genes expression of intestinal serotonergic pathway

Radocchia,

Marazzato,

Harbi

et al. 2024

BMC Microbiol

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Background Pediatric chronic intestinal pseudo-obstruction (PIPO) is a rare disease characterized by symptoms and radiological signs suggestive of intestinal obstruction, in the absence of lumen-occluding lesions. It results from an extremely severe impairment of propulsive motility. The intestinal endocrine system (IES) jointly with the enteric nervous system (ENS) regulates secreto-motor functions via different hormones and bioactive messengers/neurotransmitters. The neurotransmitter 5-hydroxytryptamine (5-HT) (or serotonin) is linked to intestinal peristalsis and secretory reflexes. Gut microbiota and its interplay with ENS affect 5-HT synthesis, release, and the subsequent serotonin receptor activation. To date, the interplay between 5-HT and gut microbiota in PIPO remains largely unclear. This study aimed to assess correlations between mucosa associated microbiota (MAM), intestinal serotonin-related genes expression in PIPO. To this purpose, biopsies of the colon, ileum and duodenum have been collected from 7 PIPO patients, and 7 age-/sex-matched healthy controls. After DNA extraction, the MAM was assessed by next generation sequencing (NGS) of the V3-V4 region of the bacterial RNA 16 S, on an Illumina Miseq platform. The expression of genes implicated in serotoninergic pathway (TPH1, SLC6A4, 5-HTR3 and 5-HTR4) was established by qPCR, and correlations with MAM and clinical parameters of PIPO have been evaluated. Results Our results revealed that PIPO patients exhibit a MAM with a different composition and with dysbiosis, i.e. with a lower biodiversity and fewer less connected species with a greater number of non-synergistic relationships, compared to controls. qPCR results revealed modifications in the expression of serotonin-related intestinal genes in PIPO patients, when compared to controls. Correlation analysis do not reveal any kind of connection. Conclusions For the first time, we report in PIPO patients a specific MAM associated to underlying pathology and an altered intestinal serotonin pathway. A possible dysfunction of the serotonin pathway, possibly related to or triggered by an altered microbiota, may contribute to dysmotility in PIPO patients. The results of our pilot study provide the basis for new biomarkers and innovative therapies targeting the microbiota or serotonin pathways in PIPO patients.

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Pseudo-obstrucción intestinal crónica, reporte de un caso y revisión de la bibliografia

Francisco,

Estefanía,

Moreira

et al. 2023

Braz. J. Hea. Rev.

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Introducción: La pseudo-obstrución intestinal crónica es un trastorno en la motilidad intestinal, que afecta al intestino delgado y/o colón, tiene una prevalencia en adultos es de 0,80 a 1,00 e incidencia de 0,21 a 0,24 por cada 100.000 personas alrededor del mundo, con la edad media de diagnóstico de 59,2 a 63,1 años de edad en hombres y mujeres respectivamente. Caso clínico: Se trata de un paciente masculino de 72 años, con antecedente de episodio similar hace 6 meses, clínica de 48 horas de evolución de dolor abdominal difuso, continuo y de moderada intensidad que se intensificó a gran intensidad 10/10 en la escala de EVA, acompañado de distensión abdominal y náuseas. Al examen físico temperatura de 37°, abdomen distendido, doloroso a la palpación profunda de manera difusa y ruidos hidroaéreos ausentes. Se solicitó laboratorio con presencia de leucocitosis y neutrofilis, radiografía de abdomen evidenciando importante distensión de asas intestinales, con niveles hidroaéreos, sin neumoperitoneo, por lo que, se manejó de forma expectante. A las 24 horas de no mostrar mejoría, se solicitó una TAC con evidencia de dilatación de asas intestinales, sin causa de obstrucción intestinal. Se procedió a la laparoscopia diagnóstica y durante la reversión anestésica se utilizó neostigmina como dosis máxima 2.5 mg, con lo que, el paciente evoluciona favorablemente en el posquirúrgico. Conclusión: Se trata de una patología muy controvertida por la diversidad de tratamiento farmacológico para reducir los síntomas; sin embargo, estudios actuales muestran que el uso de neostigmina en estos pacientes tiene efectos eficaces.

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Pharmacological and nutritional therapy of children and adults with chronic intestinal pseudo-obstruction

Cited by 3 publications

References 155 publications

Idiopathic chronic intestinal pseudo-obstruction syndrome is strongly associated with low serum levels of vitamin D

Idiopathic chronic intestinal pseudo-obstruction syndrome is strongly associated with low serum levels of vitamin D

Chronic intestinal pseudo-obstruction: associations with gut microbiota and genes expression of intestinal serotonergic pathway

Pseudo-obstrucción intestinal crónica, reporte de un caso y revisión de la bibliografia

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