Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype

Fraquelli, Mirella; Baccarin, Alessandra; Corti, Fabiola; Conti, Clara Benedetta; Russo, Maria Chiara; Valle, S. Della; Pozzi, Roberta; Cressoni, Massimo; Conte, Dario; Colombo, Carla

doi:10.1016/j.dld.2015.09.010

Cited by 16 publications

(15 citation statements)

References 43 publications

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“…A high prevalence rate of GI symptoms in 60% of 70 CF patients has also been reported in a recent survey by Fraquelli et al [36]. This study, however, considered a lower number of items for the evaluation of the GI symptoms (7 items, compared to 17 items in the JenAbdomen-CF Score 1.0) what might explain the difference in the prevalence rates.…”

Section: Discussionmentioning

confidence: 64%

Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

et al. 2017

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Background & aimsAbdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history.MethodsA total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1.0) on abdominal pain and non-pain symptoms, disorders of appetite, eating, and bowel movements as well as symptom-related quality of life. Results were metrically dimensioned and related to abdominal manifestations, history of surgery, P. aeruginosa and S. aureus colonization, genotype, liver enzymes, antibiotic therapy, lung function, and nutritional status.ResultsAS during the preceding 3 months were reported by all of our patients. Most common were lack of appetite (130/131) and loss of taste (119/131) followed by abdominal pain (104/131), flatulence (102/131), and distention (83/131). Significantly increased AS were found in patients with history of rectal prolapse (p = 0.013), distal intestinal obstruction syndrome (p = 0.013), laparotomy (p = 0.022), meconium ileus (p = 0.037), pancreas insufficiency (p = 0.042), or small bowel resection (p = 0.048) as well as in patients who have been intermittently colonized with P. aeruginosa (p = 0.006) compared to patients without history of these events. In contrast, no statistically significant associations were found to CF-associated liver disease, chronic pathogen colonization, lung function, CF-related diabetes, and nutritional status.ConclusionAs the complex abdominal involvement in CF is still not fully understood, the assessment of the common AS is of major interest. In this regard, symptom questionnaires like the herein presented are meaningful and practical tools facilitating a wider understanding of the abdominal symptoms in CF. Furthermore, they render to evaluate possible abdominal effects of novel modulators of the underlying cystic fibrosis transmembrane (conductance) regulator (CFTR) defect.

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Section: Discussionmentioning

confidence: 64%

Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

et al. 2017

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“…Intussusception is a differential diagnosis for DIOS, easily identified using GIUS [47] as thickened wall bowel with hardened content believed to serve as a lead point. Asymptomatic intussusception is however also quite frequent in CF [42,48]. Appendicitis is less frequent in patients with CF than in the general population [49] and an appendicular diameter >6 mm, is found incidentally in more than half of the CF patients, with Celiac sprue.…”

Section: Cystic Fibrosis (Cf) (Mucoviscidosis)mentioning

confidence: 99%

EFSUMB Gastrointestinal Ultrasound (GIUS) Task Force Group: Celiac sprue and other rare gastrointestinal diseases ultrasound features

et al. 2019

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Transabdominal gastrointestinal ultrasound (GIUS) is unique in its capacity to examine the bowel non-invasively and in its physiological condition, including extra-intestinal features such as the splanchnic vessels, mesentery, omentum and lymph nodes- even at the bedside. Despite this, and its extensive documentation for its usefulness, it has only been fully implemented in a few European countries and expert centres. Therefore, the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) established a GIUS Task Force Group in 2014 consisting of international experts from 9 European countries with the objectives to standardize and promote the use of GIUS in a clinical setting. This is achieved by publishing clinical guidelines and recommendations on indications and use of GIUS and so far,4 guidelines have been published: first on “examination techniques and normal findings”, second on “inflammatory bowel disease”, third on “acute appendicitis and diverticulitis” and fourth on “transrectal and perineal ultrasound”.This paper describes the ultrasound features of miscellaneous disorders such as celiac disease, cystic fibrosis, omental infarction, Meckel’s diverticle, endometriosis, intestinal neoplasia, mucocele, amyloidosis, GVHD, foreign bodies, vasculitis, and pneumatosis cystoides intestinalis. Bowel ultrasound can be indicated in most of these conditions to investigate intestinal symptoms but in other cases the alterations of the bowel can be also an incidental finding that suggest other examinations which finally help to discover an unknown pathological condition.

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“…I-IV). Patients with mutant alleles of classes I, II and III were categorized as having severe genotype, while patients with mutations of classes IV, V or other were categorized as having mild genotype [19,20].…”

Section: Genotype Classificationmentioning

confidence: 99%

Variation of glucose tolerance in adult patients with cystic fibrosis: What is the potential contribution of insulin sensitivity?

Boudreau

Coriati

Hammana

et al. 2016

Journal of Cystic Fibrosis

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Bowel ultrasound imaging in patients with cystic fibrosis: Relationship with clinical symptoms and CFTR genotype

Cited by 16 publications

References 43 publications

Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

EFSUMB Gastrointestinal Ultrasound (GIUS) Task Force Group: Celiac sprue and other rare gastrointestinal diseases ultrasound features

Variation of glucose tolerance in adult patients with cystic fibrosis: What is the potential contribution of insulin sensitivity?

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