Introduction

Basic science results suggest that abnormalities of the endocrine pancreas in cystic fibrosis (CF) occur earlier than hitherto estimated (1). All stages of the abnormal glucose tolerance (AGT) spectrum are associated with declining pulmonary function and increased mortality which could be reduced by early recognition and treatment (2-4). Despite, screening for AGT is recommended only from 10 years of age (yoa) according to the current guidelines (5,6). Accurate knowledge of the prevalence of AGT and its subtypes is essential to develop a better screening strategy, therefore we conducted a systematic review and meta-analysis in the topic.

Aims & Methods

Our systematic review and meta-analysis (PROSPERO number: CRD42021282516) was conducted on studies that reported data on the prevalence of AGT or its subtypes in CF populations. Literature search was conducted in MEDLINE (via PubMed), Embase and Cochrane Register of Controlled Trials (CENTRAL). General and superselected populations (e.g.: pancreas exocrine insufficient, ΔF508 homozygous) were separately analyzed. Pooled proportions, risk and odds ratios with 95% confidence intervals (CI) were calculated in at least three age subgroups (pediatric, adult, mixed/unknown). One-stage dose-response random effect meta-analysis was used to assess the effect of age on CF- related diabetes (CFRD).

Results

The quantitative analysis included 457 studies and data from 520,544 patients. More than one third of children with CF (chwCF) were affected by AGT (0.31 [95% CI 0.25-0.37]), even under 10 yoa 0.33 [95% CI 0.23-0.44], and half of the adults with CF (awCF) had AGT (0.51 [95% CI 0.45-0.57]). The prevalence of prediabetes remained unchanged (impaired glucose tolerance in chwCF: 0.14 [95% CI 0.10-0.18]) vs. awCF: 0.19 [95% CI 0.14-0.25]), while the proportion of CFRD increased by age (<5 yoa: 0.005 [95% CI 0.0001-0.15]; 5-10 yoa: 0.05 [95% CI 0.01-0.27]; 10-18 yoa: 0.11 [95% CI 0.08-0.14]; >18 yoa: 0.27 [95% CI 0.24-0.30]).

Conclusion

CF-related AGT is common under 10 years of age. Reconsideration of the current guidelines and better awareness are needed for screening and treating AGT in CF, especially under 10 yoa, to delay the disease progression and maintain a better life quality.

References

1. Olivier AK, Yi Y, Sun X, Sui H, Liang B, Hu S, et al. Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets. J Clin Invest. 2012;122(10):3755-68.
2. Ode KL, Moran A. New insights into cystic fibrosis-related diabetes in children. Lancet Diabetes Endocrinol. 2013;1(1):52-8.
3. Lewis C, Blackman SM, Nelson A, Oberdorfer E, Wells D, Dunitz J, et al. Diabetes-related mortality in adults with cystic fibrosis. Role of genotype and sex. Am J Respir Crit Care Med. 2015;191(2):194-200.
4. Moran A, Pekow P, Grover P, Zorn M, Slovis B, Pilewski J, et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009;32(10):1783-8.
5. Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33(12):2697-708.
6. Balfour-Lynn I. Clinical Guidelines: Care of Children with Cystic Fibrosis. UK: Royal Brompton and Harefield Hospital, NHS Trust Foundation; 2023.

Introduction

Video capsule endoscopy is a diagnostic method for examination of the small intestine. It is completely non-invasive and painless method that ensures complete examination of the small intestine because the video capsule allows a visual examination of the entire small intestine including parts that are difficult to reach with other endoscopic methods. Capsule provide high-quality images of the inner walls of the small intestine. This allows a better detection of pathological changes such as polyps, inflammation or bleeding. In a certain population of patients there is a risk that capsule "get stuck" in the intestine. This especially applies to patients with previous operations in the small intestine, in those who are on long-term therapy with nonsteroidal antirheumatic drugs, in patients who have had radiation to the pelvic region, or in patients where there is a suspicion of stenosis of the small intestine which is possible in patients with Crohn's disease.

Results & Findings

Patient underwent a small bowel video capsule examination. After the patient did not notice that the capsule had come out of the large intestine he come to our department. We performed a radiological evaluation which determined that capsule had remained in the small intestine. It was suggested to try to remove it through a single balloon enteroscope in order to avoid surgery. He was prepared for single balloon enteroscopy under general anesthesia. The endoscope was advanced through the small intestine to the site where the capsule had remained (middle part of jejunum) and with the help of a net loop the capsule was grasped and safely removed.

Summary & Discussion

One of the common risks of the procedure is capsule retention which is defined as a capsule remaining in the gastrointestinal system for longer than two weeks. Capsule retrieval is not emergent in asymptomatic retainers. In this case, the patient had no symptoms but was concerned because the capsule did not come out as expected. Considering the passage of time since installation and the type of capsule itself it was not possible to see online where the capsule is located. We used a radiological methods to detect capsule retention. Because we gave the patient clear instructions on what to expect after the capsule was placed so the retention condition was recognized in time and the extraction procedure was performed which ultimately resulted in avoiding patient surgery.

Conclusion

In order to avoid potential complications in the form of retention of the capsule in the small intestine in patients with Crohn's disease, before the actual placement of the capsule, the patient can be given a capsule to check the patency of the small intestine. A successful patency test minimizes the risk of retention and allows the safe administration of a capsule. On the other hand single baloon enteroscopy provides us possibility of therapeutic action in the small intestine and using of this endoscopic method spare patient from other invasive solutions.

References

1. Boysen M and Ritter M, Small Bowel Obstruction from Capsule Endoscopy, West J Emerg Med. 2010 Feb;11(1):71–73.
2. Mitselos IV, Katsanos KH, Tsianos, Eliakim R, Christodoulou DK, Clinical Use of Patency Capsule: A Comprehensive Review of the Literature, Inflammatory Bowel Diseases, Volume 24, Issue 11, November 2018, Pages 2339–2347