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
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)
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
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)
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
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)
Article
Obstetrics & Gynecology
Chang Liu, Ka U. Lio, Yiping Le, Chuan Wang, Xin Kang, Jianhua Lin, Yu Zhang, Ning Zhang
Summary: This article reported a case of acute intestinal pseudo-obstruction (IPO) associated with Sjogren's syndrome (SjS) in pregnancy, which was successfully treated with combined immunosuppressive therapy and resulted in an uneventful caesarean delivery.
BMC PREGNANCY AND CHILDBIRTH
(2023)
Article
Nutrition & Dietetics
Cristina Cuerda, Home Artificial Nutr Chronic Intestinal Failure Special Interest Grp ESPEN, Loris Pironi, Jann Arends, Federico Bozzetti, Lyn Gillanders, Palle Bekker Jeppesen, Francisca Joly, Darlene Kelly, Simon Lal, Michael Staun, Kinga Szczepanek, Andre Van Gossum, Geert Wanten, Stephane Michel Schneider, Stephan C. Bischoff
Summary: This practical guideline, based on the ESPEN Guidelines, offers simplified recommendations for the management and treatment of patients with chronic intestinal failure, providing guidance and support to healthcare professionals.
CLINICAL NUTRITION
(2021)
Review
Pediatrics
Marie-Catherine Turcotte, Christophe Faure
Summary: Chronic intestinal pseudo-obstruction is a rare and severe gastrointestinal disorder, with significant differences between adult and pediatric patients. This article aims to provide pediatric healthcare professionals with an up to date review of PIPO, including etiology, clinical features, diagnostic and management approaches.
FRONTIERS IN PEDIATRICS
(2022)
Article
Gastroenterology & Hepatology
Jong Woo Hahn, Soo Young Moon, Min Soo Kim, Min Hyung Woo, Min Ji Sohn, Hyun-Young Kim, Moon-Woo Seong, Sung Sup Park, Sung-Hye Park, Jin Soo Moon, Jae Sung Ko
Summary: This study examined the clinical features and ACTG2 variants in Korean patients with CIPO. The results showed that ACTG2 missense variants were found in 50% of the patients. Genetic testing of ACTG2 should be considered for CIPO patients with megacystis and abnormal prenatal ultrasonography.
JOURNAL OF NEUROGASTROENTEROLOGY AND MOTILITY
(2022)
Article
Endocrinology & Metabolism
Mikiko Okazaki-Hada, Izumi Fukuda, Ryuta Nagaoka, Mototsugu Nagao, Takehito Igarashi, Shunsuke Kobayashi, Takeshi Oba, Yuji Yamaguchi, Tomoko Nagamine, Iwao Sugitani, Hitoshi Sugihara
Summary: This case report presents a rare case of pheochromocytoma coexisting with a liver abscess. The clinical presentation of the patient was atypical, with constipation, intestinal pseudo-obstruction, and weight loss as the main symptoms. The findings of this case provide valuable insight into the impact of catecholamine excess on the intestinal tract and body weight.
THERAPEUTIC ADVANCES IN ENDOCRINOLOGY AND METABOLISM
(2022)
Article
Gastroenterology & Hepatology
Zhiliang Wei, Lina Lu, Youjie Zheng, Weihui Yan, Yijng Tao, Yongtao Xiao, Wei Cai, Ying Wang
Summary: This study identified that variants affecting codon 257 of ACTG2 protein sequence were common in Chinese PIPO patients and discovered 4 novel variants in ACTG2 related to Chinese PIPO.
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
(2021)
Article
Multidisciplinary Sciences
Yang Cao, Weixin Wang, Jiong Chen, Bo Jiang, Sugai Liang, Xianyu Chen, Hui Dong
Summary: This article reports a patient with comorbid schizophrenic catatonia and CIPO, and describes the complete remission with lorazepam, which may contribute to the understanding and treatment of this comorbidity.
Article
Biochemistry & Molecular Biology
Keqiang Liu, Lina Lu, Shanshan Chen, Beilin Gu, Hui Cai, Ying Wang, Wei Cai
Summary: This study describes a male infant with LMOD1 mutations presenting pediatric intestinal pseudo-obstruction (PIPO) symptoms but without megacystis and microcolon. The mutations result in significantly reduced LMOD1 protein levels and impair LMOD1-mediated actin nucleation. Molecular modeling shows the structural changes caused by the mutations. These findings provide further evidence of LMOD1 as a pathogenic gene and highlight the critical role of the ABS2 domain in LMOD1-mediated actin nucleation and protein stability.
Article
Clinical Neurology
Satoshi Yamashita, Tomohiro Chiyonobu, Michiko Yoshida, Hiroshi Maeda, Masashi Zuiki, Satoshi Kidowaki, Kenichi Isoda, Masafumi Morimoto, Mitsuhiro Kato, Hirotomo Saitsu, Naomichi Matsumoto, Tatsutoshi Nakahata, Megumu K. Saito, Hajime Hosoi
Article
Neurosciences
Masashi Zuiki, Tomohiro Chiyonobu, Michiko Yoshida, Hiroshi Maeda, Satoshi Yamashita, Satoshi Kidowaki, Tatsuji Hasegawa, Hitoshi Gotoh, Tadashi Nomura, Katsuhiko Ono, Hajime Hosoi, Masafumi Morimoto
NEUROSCIENCE LETTERS
(2017)
Article
Genetics & Heredity
N. Tsuchida, M. Nakashima, M. Kato, E. Heyman, T. Inui, K. Haginoya, S. Watanabe, T. Chiyonobu, M. Morimoto, M. Ohta, A. Kumakura, M. Kubota, Y. Kumagai, S. -I. Hamano, C. M. Lourenco, N. A. Yahaya, G. -S. Ch'ng, L. -H. Ngu, A. Fattal-Valevski, M. Weisz Hubshman, N. Orenstein, D. Marom, L. Cohen, H. Goldberg-Stern, Y. Uchiyama, E. Imagawa, T. Mizuguchi, A. Takata, N. Miyake, H. Nakajima, H. Saitsu, S. Miyatake, N. Matsumoto
Editorial Material
Hematology
Masaya Suematsu, Toshihiko Imamura, Tomohiro Chiyonobu, Shinya Osone, Hajime Hosoi
INTERNATIONAL JOURNAL OF HEMATOLOGY
(2018)
Article
Clinical Neurology
Takenori Tozawa, Akira Nishimura, Tamaki Ueno, Daisuke Kaneda, Yuri Miyanomae, Tomohiro Chiyonobu, Masafumi Morimoto, Hajime Hosoi
Article
Clinical Neurology
Tatsuji Hasegawa, Kei Yamada, Takenori Tozawa, Tomohiro Chiyonobu, Sachiko Tokuda, Akira Nishimura, Hajime Hosoi, Masafumi Morimoto
BRAIN & DEVELOPMENT
(2018)
Article
Multidisciplinary Sciences
Takashi Kasai, Harutsugu Tatebe, Masaki Kondo, Ryotaro Ishii, Takuma Ohmichi, Wing Tung Esther Yeung, Masafumi Morimoto, Tomohiro Chiyonobu, Naoto Terada, David Allsop, Masanori Nakagawa, Toshiki Mizuno, Takahiko Tokuda
Article
Clinical Neurology
Yui Zen, Tomohiro Chiyonobu, Mariko Yuge, Isao Yokota, Masafumi Morimoto, Satoshi Teramukai, Hajime Hosoi
BRAIN & DEVELOPMENT
(2019)
Article
Multidisciplinary Sciences
Makiko Shinomoto, Takashi Kasai, Harutsugu Tatebe, Masaki Kondo, Takuma Ohmichi, Masafumi Morimoto, Tomohiro Chiyonobu, Naoto Terada, David Allsop, Isao Yokota, Toshiki Mizuno, Takahiko Tokuda
Article
Clinical Neurology
Hiroshi Maeda, Ryotaro Ishii, Susumu Kusunoki, Tomohiro Chiyonobu
BRAIN & DEVELOPMENT
(2020)
Article
Clinical Neurology
Rei Takada, Takenori Tozawa, Hidehito Kondo, Zenro Kizaki, Yoshihito Kishita, Yasushi Okazaki, Kei Murayama, Akira Ohtake, Tomohiro Chiyonobu
BRAIN & DEVELOPMENT
(2020)
Article
Clinical Neurology
Takenori Tozawa, Takashi Kasai, Harutsugu Tatebe, Kozue Shiomi, Hisahide Nishio, Takahiko Tokuda, Tomohiro Chiyonobu
BRAIN & DEVELOPMENT
(2020)
Article
Oncology
Ibuki Ueoka, Akari Takai, Mizuki Yamaguchi, Tomohiro Chiyonobu, Hideki Yoshida, Masamitsu Yamaguchi
EXPERIMENTAL CELL RESEARCH
(2020)
Article
Genetics & Heredity
M. Yoshida, M. Nakashima, T. Okanishi, S. Kanai, A. Fujimoto, K. Itomi, M. Morimoto, H. Saitsu, M. Kato, N. Matsumoto, T. Chiyonobu
Article
Clinical Neurology
Satoshi Kidowaki, Masafumi Morimoto, Kei Yamada, Koji Sakai, Masashi Zuiki, Hiroshi Maeda, Satoshi Yamashita, Takashi Morita, Tatsuji Hasegawa, Tomohiro Chiyonobu, Sachiko Tokuda, Hajime Hosoi
BRAIN & DEVELOPMENT
(2017)