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Revista Espanola de Enfermedades... Mar 2022We propose an algorithm for the early detection of cancer from a case of paraneoplastic syndrome.
We propose an algorithm for the early detection of cancer from a case of paraneoplastic syndrome.
Topics: Antibodies, Antinuclear; Chronic Disease; Humans; Intestinal Pseudo-Obstruction; Paraneoplastic Syndromes; Prognosis
PubMed: 34991323
DOI: 10.17235/reed.2021.8521/2021 -
Journal of Pediatric Gastroenterology... Jul 2023Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study...
Pediatric Intestinal Pseudo-Obstruction: An International Survey on Diagnostic and Management Strategies in the European Reference Network for Rare Inherited and Congenital Anomalies Intestinal Failure Teams.
OBJECTIVES
Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines.
METHODS
An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams.
RESULTS
In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse.
CONCLUSIONS
Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed.
Topics: Child; Humans; Intestinal Failure; Intestinal Pseudo-Obstruction; Parenteral Nutrition; Surveys and Questionnaires
PubMed: 37027146
DOI: 10.1097/MPG.0000000000003788 -
British Medical Journal (Clinical... May 1986
Topics: Aged; Colonic Diseases; Humans; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Male
PubMed: 3087479
DOI: 10.1136/bmj.292.6533.1463 -
Singapore Medical Journal Aug 2015Phaeochromocytomas are rare neuroendocrine tumours with variable clinical signs and symptoms. Hypertension, tachycardia, sweating and headaches are cardinal...
Phaeochromocytomas are rare neuroendocrine tumours with variable clinical signs and symptoms. Hypertension, tachycardia, sweating and headaches are cardinal manifestations. Although nausea and abdominal pain are the more common gastrointestinal features, rare gastrointestinal spectrums have been reported that can mimic abdominal emergencies. Metabolic effects of hypercatecholaminaemia are vast and one such rare presentation is lactic acidosis. We describe a case of phaeochromocytoma presenting with both intestinal pseudo-obstruction as well as lactic acidosis. This case report highlights the importance of having a high index of suspicion for and early recognition of the gastrointestinal and metabolic manifestations of phaeochromocytomas.
Topics: Abdominal Pain; Acidosis, Lactic; Adrenal Gland Neoplasms; Diabetes Mellitus, Type 2; Dyslipidemias; Female; Humans; Hypertension; Intestinal Pseudo-Obstruction; Middle Aged; Neuroendocrine Tumors; Pheochromocytoma; Radiography, Abdominal
PubMed: 26311913
DOI: 10.11622/smedj.2015126 -
Rheumatology (Oxford, England) Sep 2023SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal...
OBJECTIVES
SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal pseudo-obstruction (IPO) are often refractory to conventional intervention and can result in longer in-hospital stay or even increased mortality. We aimed to summarize the insights to date regarding the efficacy of IVIG against GI symptoms of SSc to unveil what we should focus on in future studies.
METHODS
Herein we report the response of GI symptoms in three cases with SSc-myositis overlap who received IVIG administration. We also conducted a systematic literature review to summarize previous reports regarding the efficacy of IVIG upon the GI manifestations of SSc, according to the PRISMA 2020 guideline.
RESULTS
The case series demonstrated remarkable and rapid improvement of GI symptoms, including IPO, after IVIG administration. The literature review revealed that previous reports also support the efficacy and safety of IVIG against GI manifestations of SSc. However, they were all retrospective studies and lacking description of the short-term outcome after IVIG administration with objective and quantitative metrics.
CONCLUSION
IVIG seems to be a promising therapeutic option for the management of GI symptoms in SSc, including IPO. Investigators should focus more on short-term outcomes to properly assess the therapeutic benefit of IVIG, ideally using reliable quantitative measures in a multicentre randomized placebo-controlled setting.
Topics: Humans; Immunoglobulins, Intravenous; Retrospective Studies; Scleroderma, Systemic; Gastrointestinal Diseases; Intestinal Pseudo-Obstruction
PubMed: 36825818
DOI: 10.1093/rheumatology/kead093 -
Frontiers in Immunology 2019Mutations in the tetratricopeptide repeat domain 7A (TTC7A) gene cause very early onset inflammatory bowel diseases (VOIBD) or multiple intestinal atresia associated...
Mutations in the tetratricopeptide repeat domain 7A (TTC7A) gene cause very early onset inflammatory bowel diseases (VOIBD) or multiple intestinal atresia associated with immune deficiency of various severities, ranging from combined immune deficiency to mild lymphopenia. In this manuscript, we report the clinical, biological and molecular features of a patient born from consanguineous parents, presenting with recurrent lymphoproliferative syndrome and pan-hypergammaglobulinemia associated with chronic intestinal pseudo obstruction (CIPO). Genetic screening revealed the novel c.974G>A (p.R325Q) mutation in homozygosity in the gene. The patient's phenotype differs significantly from that previously associated with TTC7A deficiency in humans. It becomes closer to the one reported in the ttc7a-deficient mice that invariably develop a proliferative lymphoid and myeloid disorder. Functional studies showed that the extreme variability in the clinical phenotype couldn't be explained by the cellular phenotype. Indeed, the patient's TTC7A mutation, as well as the murine-ttc7 mutant, have the same functional impact on protein expression, DNA instability and chromatin compaction, as the other mutations that lead to classical TTC7A-associated phenotypes. Co-inheritance of genetic variants may also contribute to the unique nature of the patient's phenotype. The present case report shows that the clinical spectrum of TTC7A deficiency is much broader than previously suspected. Our findings should alert the physicians to consider screening of mutations in patients with lymphoproliferative syndrome and hypergammaglobulinemia and/or chronic intestinal pseudo-obstruction.
Topics: Animals; Cells, Cultured; Chronic Disease; Consanguinity; Female; Humans; Infant; Intestinal Pseudo-Obstruction; Lymphoproliferative Disorders; Male; Mice; Protein Deficiency; Proteins
PubMed: 31787977
DOI: 10.3389/fimmu.2019.02592 -
Indian Journal of Pathology &... 2023Chronic intestinal pseudo-obstruction (CIPO) is caused by a plethora of primary and secondary causes, dominantly involving the neuromuscular tissue, interstitial cells...
Chronic intestinal pseudo-obstruction (CIPO) is caused by a plethora of primary and secondary causes, dominantly involving the neuromuscular tissue, interstitial cells of Cajal, or the connective tissue framework. The lack of the connective tissue framework, known as desmosis, is evaluated by Masson's trichrome (MT) or picrosirius red stains, both of which are recommended in the London classification. We evaluated the orcein stain in detecting desmosis in comparison to the MT stain. We performed both orcein and MT stains in six previously published cases of complete or partial desmosis along with six age-matched controls. Our results showed comparable results of the orcein stain as compared to the MT stain. Additional advantages of lower cost and a clearer background in orcein stain were noteworthy, whereas MT stain can be used for the detection of additional pathology. We believe that orcein stain can be used as a cheap alternative in resource-limited settings.
Topics: Humans; Staining and Labeling; Methyl Green; Intestinal Pseudo-Obstruction; Chronic Disease
PubMed: 37077096
DOI: 10.4103/ijpm.ijpm_539_21 -
JA Clinical Reports May 2019Intestinal pseudo-obstruction, which is a rare complication of pheochromocytoma, can be caused by hypersecreted catecholamines.
BACKGROUND
Intestinal pseudo-obstruction, which is a rare complication of pheochromocytoma, can be caused by hypersecreted catecholamines.
CASE PRESENTATION
A 45-year-old woman was admitted for local recurrence of pheochromocytoma complicated by intestinal pseudo-obstruction. The intestinal pseudo-obstruction showed poor response to α-adrenergic receptor blocker and she was scheduled for surgical resection of pheochromocytoma. The surgery was uneventfully accomplished with general anesthesia combined with epidural anesthesia. The latter was performed with the aim of not only perioperative pain management but also of promoting intestinal peristalsis. The anticipated effect for intestinal peristalsis was not apparent in the early postoperative phase. The abdominal symptoms were gradually relieved over the course of about 1 month.
CONCLUSIONS
For intestinal pseudo-obstruction induced by pheochromocytoma, although inhibition of the sympathetic system by epidural infusion of local anesthetics may be promising, short-term usage of epidural local anesthetics infusion did not provide a quick recovery after pheochromocytoma removal surgery.
PubMed: 32026115
DOI: 10.1186/s40981-019-0255-9 -
Gut Jan 1987We report the clinical and intestinal manometric findings in a group of 42 patients with chronic idiopathic intestinal pseudo-obstruction evaluated at the Mayo Clinic....
We report the clinical and intestinal manometric findings in a group of 42 patients with chronic idiopathic intestinal pseudo-obstruction evaluated at the Mayo Clinic. The main clinical manifestations in these patients were nausea and vomiting (83%), abdominal pain (74%), distension (57%), constipation (36%), diarrhoea (29%), and urinary symptoms (17%). These symptoms preceded surgery in all patients. Air fluid levels or distended bowel loops occurred in 57% and a dilated bladder or urinary excretory pathway in 17%. All patients showed intestinal manometric abnormalities none of which are seen in healthy individuals: aberrant configuration or propagation of interdigestive motor complexes in 25 patients; bursts (greater than 2 min duration) of non-propagated phasic pressure activity in fasting and/or fed state in 30 patients; sustained incoordinated fasting pressure activity in 15 patients; and inability of an ingested meal to convert fasting into fed pattern in 28 patients. We conclude that qualitative analysis of intestinal manometry provides evidence of gut dysmotility in patients with the clinical syndrome of chronic intestinal pseudo-obstruction. These abnormalities of motility can help to establish the correct diagnosis.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Duodenum; Eating; Fasting; Female; Gastrointestinal Motility; Humans; Intestinal Pseudo-Obstruction; Jejunum; Male; Manometry; Middle Aged; Pressure
PubMed: 3817584
DOI: 10.1136/gut.28.1.5 -
Journal of Epidemiology 2013We estimated the prevalence and incidence of chronic intestinal pseudo-obstruction (CIPO) in Japan, investigated the patterns of hospital visits among those with CIPO,...
BACKGROUND
We estimated the prevalence and incidence of chronic intestinal pseudo-obstruction (CIPO) in Japan, investigated the patterns of hospital visits among those with CIPO, and examined present knowledge of CIPO among medical professionals.
METHODS
A self-administered questionnaire survey was distributed to targeted hospitals throughout Japan, which were selected using stratified random sampling. The questionnaire asked about the number of patients receiving treatment for CIPO, the frequency of their hospital visits, and overall clinical knowledge of CIPO among medical professionals.
RESULTS
CIPO prevalence was estimated to be 1.00 and 0.80 cases per 100 000 males and females, respectively. Incidence was 0.21 and 0.24 cases per 100 000 males and females, respectively. Prevalence and incidence did not significantly differ males and females. Mean age of patients was 63.1 years for males and 59.2 for females. Accurate diagnosis of CIPO sometimes required more than 3 months after initial presentation. Most medical professionals were unaware of or poorly understood CIPO.
CONCLUSIONS
We estimated the prevalence and incidence of CIPO in Japan, using data from a nationwide survey. The findings suggest that knowledge of CIPO should be further disseminated so that the disease is not overlooked and is diagnosed without delay.
Topics: Adult; Age Distribution; Aged; Chronic Disease; Clinical Competence; Female; Hospitals; Humans; Incidence; Intestinal Pseudo-Obstruction; Japan; Male; Middle Aged; Prevalence; Sex Distribution; Surveys and Questionnaires
PubMed: 23831693
DOI: 10.2188/jea.je20120173