Article
Cell Biology
Almira Zada, Laura E. Kuil, Bianca M. de Graaf, Naomi Kakiailatu, Jonathan D. Windster, Alice S. Brooks, Marjon van Slegtenhorst, Barbara de Koning, Rene M. H. Wijnen, Veerle Melotte, Robert M. W. Hofstra, Erwin Brosens, Maria M. Alves
Summary: Whole exome sequencing identified a de novo heterozygous deletion in TFAP2B associated with Pediatric Intestinal Pseudo-obstruction (PIPO), causing reduced neuronal numbers and gastrointestinal dysmotility.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Medicine, General & Internal
Emanuele Sinagra, Gaia Pellegatta, Marcello Maida, Francesca Rossi, Giuseppe Conoscenti, Socrate Pallio, Rita Alloro, Dario Raimondo, Andrea Anderloni
Summary: CIIPO is a disease characterized by symptoms of small bowel obstruction in the absence of mechanical obstruction, with a hypothesis that various viruses localized in the intestine may play a role in its pathogenesis. This review aims to summarize new perspectives in the etiology and pathophysiology of CIIPO.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Microbiology
Giulia Radocchia, Bruna Neroni, Massimiliano Marazzato, Elena Capuzzo, Simone Zuccari, Fabrizio Pantanella, Letizia Zenzeri, Melania Evangelisti, Francesca Vassallo, Pasquale Parisi, Giovanni Di Nardo, Serena Schippa
Summary: Chronic intestinal pseudo-obstruction (CIPO) is a rare clinical syndrome characterized by severe impairment of gastrointestinal motility, posing a significant challenge for diagnosis and therapy. This review aims to explore the relationship between CIPO patients and intestinal microbiota, with a focus on understanding the causes of gut motility dysfunction in these patients. Further studies on the role of the enteric nervous system and the intestinal endocrine system are essential for a deeper understanding of CIPO.
Article
Gastroenterology & Hepatology
Kitzia Colliard, Samuel Nurko, Alejandro Flores, Leonel Rodriguez
Summary: This study retrospectively reviewed children undergoing ileal manometry and found that ileal motility patterns were abnormal in children with chronic intestinal pseudo-obstruction (CIPO) and normal in children with defecation disorders. There was moderate agreement between ileal manometry and antroduodenal manometry, suggesting that ileal manometry could be used as a surrogate for small bowel motility.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2023)
Review
Surgery
L. Billiauws, M. Cohen, D. Cazals-Hatem, F. Joly
Summary: Chronic intestinal pseudo-obstruction (CIPO) is a rare condition characterized by chronic or recurrent obstructive symptoms and intestinal dilation without mechanical obstruction. Diagnosis is complex and based on clinical evaluation, imaging tests, and intestinal manometry. Treatment requires multidisciplinary care in expert centers, focusing on symptom management, nutritional support, and psychological support.
JOURNAL OF VISCERAL SURGERY
(2022)
Article
Cell Biology
Delphine Martire, Sarah Garnier, Sebastien Sagnol, Annick Bourret, Stephane Marchal, Norbert Chauvet, Amandine Guerin, Dominique Forgues, Dominique Berrebi, Christophe Chardot, Marc Bellaiche, John Rendu, Nicolas Kalfa, Sandrine Faure, Pascal de Santa Barbara
Summary: Smooth Muscle Cells (SMC) exhibit a unique ability to modulate their phenotype, switching between a quiescent contractile state and a highly proliferative and migratory state. In a study on infants with chronic intestinal pseudo-obstruction (CIPO), it was found that CIPO-SMCs have decreased levels of contractile markers and increased expression of PDGFRA, suggesting a phenotypic switch towards an undifferentiated stage.
JOURNAL OF CELLULAR AND MOLECULAR MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Francesca Bianco, Giulia Lattanzio, Luca Lorenzini, Maurizio Mazzoni, Paolo Clavenzani, Laura Calza, Luciana Giardino, Catia Sternini, Anna Costanzini, Elena Bonora, Roberto De Giorgio
Summary: CIPO is a typical clinical phenotype of severe gut dysmotility characterized by changes in neuro-enteric innervation, interstitial cells of Cajal, and smooth muscle cells. Genetic studies have identified several genes associated with CIPO, paving the way for potential targeted therapies for gut dysmotility.
Article
Gastroenterology & Hepatology
Luis G. Alcala-Gonzalez, Anna Accarino, Ramon Marti, Daniel Sanchez-Tejerina, Arnau Llaurado, Fernando Azpiroz, Carolina Malagelada
Summary: This study aims to describe gastrointestinal motor dysfunction in MNGIE patients using advanced techniques and evaluate the relationship between motor abnormalities and symptoms. The results show that MNGIE patients have characteristic motor dysfunction in the small bowel, even in the presence of mild digestive symptoms. Early investigation is necessary.
NEUROGASTROENTEROLOGY AND MOTILITY
(2023)
Review
Pharmacology & Pharmacy
Ramasatyaveni Geesala, You-Min Lin, Ke Zhang, Xuan-Zheng Shi
Summary: Mechano-transcription is a process where mechanical stress alters gene expression, which can lead to pathophysiological changes in gastrointestinal disorders like inflammation, pain, and fibrosis. Studies have shown that persistent mechanical stress in the gut may result in visceral hypersensitivity, inflammation, and fibrosis development.
FRONTIERS IN PHARMACOLOGY
(2021)
Review
Pediatrics
Susan Nham, Alexander T. M. Nguyen, Andrew J. A. Holland
Summary: Paediatric intestinal pseudo-obstruction (PIPO) is a rare and challenging disorder to diagnose and treat, with management mainly relying on parenteral nutrition and intestinal transplantation, which may lead to serious complications in the long term.
EUROPEAN JOURNAL OF PEDIATRICS
(2022)
Article
Gastroenterology & Hepatology
Atchariya Chanpong, Osvaldo Borrelli, Nikhil Thapar
Summary: Hirschsprung disease and Paediatric Intestinal Pseudo-obstruction are recognized as severe disorders of gastrointestinal motility. Histopathology is considered the gold standard for diagnosis, but other diagnostic modalities have seen advancements. Multidisciplinary management in specialist referral centers is optimal for Paediatric Intestinal Pseudo-obstruction. Surgery remains the only viable treatment for Hirschsprung disease, while novel therapies such as neural stem cell transplants show promise for the future.
BEST PRACTICE & RESEARCH CLINICAL GASTROENTEROLOGY
(2022)
Article
Medicine, General & Internal
Dayoung Ko, Hee-Beom Yang, Joong Youn, Hyun-Young Kim
Summary: A study of 66 pediatric CIPO patients revealed a higher proportion of early-onset CIPO and patients with urologic symptoms, as well as a majority of cases with generalized involvement. Factors such as duration of PN, PN use, and home management were found to impact outcomes, while mortality was associated with pathologic type.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Gastroenterology & Hepatology
Atchariya Chanpong, Hannah Cronin, Dyanne Rampling, Michael Ashworth, Simon Eaton, Anna Rybak, Efstratios Saliakellis, Keith J. Lindley, Osvaldo Borrelli, Nikhil Thapar
Summary: The newly developed enhanced ADM analysis and GLASS score can effectively differentiate between PIPO and non-PIPO patients, as well as different histopathological pathologies, providing a more accurate diagnostic tool in pediatric intestinal pseudo-obstruction.
NEUROGASTROENTEROLOGY AND MOTILITY
(2022)
Article
Gastroenterology & Hepatology
Muhammad Zarrar Khan, Ruishen Lyu, John McMichael, Scott Gabbard
Summary: The study aims to explore the association and prevalence of hydrogen and methane subtypes of SIBO in patients with CIP. A retrospective chart review was conducted for 494 patients who underwent glucose breath tests (GBT) in 2019. The prevalence of methane-positive GBT was higher in CIP patients than in patients without CIP, suggesting a facilitative relationship between motility disorders and methanogen overgrowth.
DIGESTIVE DISEASES AND SCIENCES
(2022)
Article
Gastroenterology & Hepatology
Xiangqin He, Kunzhe Dong, Jian Shen, Guoqing Hu, James D. Mintz, Reem T. Atawia, Juanjuan Zhao, Xiuxu Chen, Robert W. Caldwell, Meixiang Xiang, David W. Stepp, David J. Fulton, Jiliang Zhou
Summary: This study reveals the critical role of Carmn, a specific long noncoding RNA (lncRNA), in regulating the contractility and phenotype of gastrointestinal smooth muscle cells (SMCs). Loss of Carmn function leads to impaired gastrointestinal motility and disease, indicating the essential role of Carmn in maintaining the function of SMCs in the gut.