Multidisciplinary Care for Cystic Fibrosis Liver Disease: Where Does the Adult Hepatologist Fit In?

Ayoub, Fares; Li, Hechu; Blay, C.; Trillo-Alvarez, Cesar; Lascano, Jorge; Morelli, Giuseppe

doi:10.1002/cld.845

Cited by 2 publications

(2 citation statements)

References 15 publications

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“…Cystic Fibrosis (CF) is one of the most common inherited genetic disorder in Caucasians. A mutation in Cystic Fibrosis Transmembrane Conductance Regulator (CTFR) gene is the cause of the disease [1]. The CTFR gene encodes for the chloride ion channel, belonging to adenosine triphosphate's family.…”

Section: Introductionmentioning

confidence: 99%

Surveillance of Colorectal Cancer (CRC) in Cystic Fibrosis (CF) Patients

Ingravalle

Casella²,

Ingravalle

et al. 2021

Gastrointestinal Disorders

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Cystic Fibrosis (CF) is the commonest inherited genetic disorder in Caucasians due to a mutation in the gene CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), and it should be considered as an Inherited Colorectal Cancer (CRC) Syndrome. In the United States, physicians of CF Foundation established the “Developing Innovative Gastroenterology Speciality Training Program” to increase the research on CF in gastrointestinal and hepatobiliary diseases. The risk to develop a CRC is 5–10 times higher in CF patients than in the general population and even greater in CF patients receiving immunosuppressive therapy due to organ transplantation (30-fold increased risk relative to the general population). Colonoscopy should be considered the best screening for CRC in CF patients. The screening colonoscopy should be started at the age of 40 in CF patients and, if negative, a new colonoscopy should be performed every 5 years and every 3 years if adenomas are detected. For transplanted CF patients, the screening colonoscopy could be started at the age of 35, in transplanted patients at the age of 30 and, if before, at the age of 30. CF transplanted patients, between the age of 35 and 55, must repeat colonoscopy every 3 years. Our review draws attention towards the clinically relevant development of CRC in CF patients, and it may pave the way for further screenings and studies.

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Section: Introductionmentioning

confidence: 99%

Surveillance of Colorectal Cancer (CRC) in Cystic Fibrosis (CF) Patients

Ingravalle

Casella²,

Ingravalle

et al. 2021

Gastrointestinal Disorders

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“…Numerous studies have shown that multidisciplinary clinics improve outcomes [10] , [11] , [12] , reduce mortality [13] , [14] , and are a cost-effective approach to patient care [4] , [5] . However, limited studies were identified which assess the utilization and efficacy of automatic referrals in a multidisciplinary CF clinic [15] .…”

Section: Introductionmentioning

confidence: 99%

Automatic referrals within a cystic fibrosis multidisciplinary clinic improve patient evaluation and management

Darukhanavala

Kremer

2021

Journal of Clinical & Translational Endocrinology

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AUTOMATIC REFERRALS WITHIN A CYSTIC FIBROSIS MULTIDISCIPLINARY CLINIC IMPROVE PATIENT EVALUATION AND MANAGEMENT. Background Cystic fibrosis (CF) affects multiple systems beyond the pulmonary system, including the gastrointestinal and endocrine systems. Many CF clinics focus on pulmonary effects, initiating referrals to other specialties only when a condition has been identified by the primary pulmonary team. Unfortunately, many extrapulmonary manifestations of cystic fibrosis may be overlooked. Thus, implementing a multidisciplinary clinic with automatic referrals to designated subspecialists may improve patient care. Methods This retrospective review of medical records examined the effects of integrating a pediatric endocrinologist into the University of Massachusetts Memorial Medical Center Pediatric CF Clinic in March 2017. In this new CF/Endocrinology clinic, all patients scheduled to see a pulmonologist were automatically referred to pediatric endocrinology. We compared rates of referrals to pediatric endocrinology, oral glucose tolerance tests (OGTTs), and bone density (DEXA) scans before (2013–2016) and after (2017–2020) implementation of this clinic. We also recorded endocrine disorders being evaluated and/or treated after implementation. Results The rate of referral to pediatric endocrinology increased from before (4%) to after (82%) (p < 0.0001) implementation of the CF/Endocrinology Clinic. OGTT and DEXA scan screening rates also increased from 7% to 65% (p < 0.0001) and from 6% to 63% (p = 0.0011), respectively. Before implementation, patients were evaluated by endocrinology primarily for CF-related diabetes. After implementation, the diversity of endocrine conditions under evaluation and/or management increased substantially; the most common were vitamin D insufficiency/deficiency (37.2% of clinic patients), glycemic dysregulation (36.8%), and poor weight gain/failure to thrive (17.5%). Conclusion Implementing a multidisciplinary CF clinic with automatic referrals to pediatric endocrinology improves patient care by promoting early detection and management of endocrine concerns that may have been overlooked and by increasing OGTT and DEXA screening rates.

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Multidisciplinary Care for Cystic Fibrosis Liver Disease: Where Does the Adult Hepatologist Fit In?

Abstract: http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/14-5-reading-ayoub a video presentation of this article

Cited by 2 publications

References 15 publications

Surveillance of Colorectal Cancer (CRC) in Cystic Fibrosis (CF) Patients

Surveillance of Colorectal Cancer (CRC) in Cystic Fibrosis (CF) Patients

Automatic referrals within a cystic fibrosis multidisciplinary clinic improve patient evaluation and management

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