Bone health and disease in cystic fibrosis

Marquette, Malcolm; Haworth, Charles

doi:10.1016/j.prrv.2016.06.003

Cited by 24 publications

(33 citation statements)

References 21 publications

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“…Follow-up DEXA depends on results of the baseline study (117). Decreased BMD in CF, similar to that seen in adults with CP, is more likely in adolescents and adults and is probably secondary to both systemic inflammation resulting in bone reabsorption and nutritional deficiencies secondary to EPI (118).…”

Section: Methodsmentioning

confidence: 99%

Nutritional Considerations in Pediatric Pancreatitis

Abu‐El‐Haija

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Werlin

et al. 2018

J. pediatr. gastroenterol. nutr.

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This document presents the first authoritative recommendations on nutritional considerations in pediatric pancreatitis. Future research should address the gaps in knowledge particularly relating to optimal nutrition for AP in children, role of diet or dietary supplements on recurrent attacks of pancreatitis and pain episodes, monitoring practices to detect early growth and nutritional deficiencies in CP and identifying risk factors that predispose children to these deficiencies.

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Section: Methodsmentioning

confidence: 99%

Nutritional Considerations in Pediatric Pancreatitis

Abu‐El‐Haija

Uç

Werlin

et al. 2018

J. pediatr. gastroenterol. nutr.

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“…Bone health has not specifically been examined in individuals with CFLD, but given the potential of poor nutritional status and lean body mass deficits, worse bone health is expected before transplant . Guidelines for bone health in CF are available and should inform the approach to its evaluation and management …”

Section: The Evaluation For Lt In Patients With Cfldmentioning

confidence: 99%

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

et al. 2019

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Approximately 5%‐10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD. Liver transplantation (LT) in patients with CFLD with portal hypertension confers a significant survival advantage over those who do not receive LT, although the timing in which to optimize this benefit is unclear. Despite the value and efficacy of LT in selected patients with CFLD, established clinical criteria outlining indications and timing for LT as well as disease‐specific transplant considerations are notably absent. The goal of this comprehensive and multidisciplinary report is to present recommendations on the unique CF‐specific pre‐ and post‐LT management issues clinicians should consider and will face.

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“…91,92 Other complications related to CF include combination of CFTR dysfunction and inflammation and CF-related arthropathy, which is poorly understood, and CF-associated osteopenia, which is a common occurrence in chronic systemic inflammatory diseases and, in part, related to CFTR dysfunction. 10,[93][94][95] In addition to conditions related to CF with increasing age, degenerative conditions and cancers are also emerging problems in care delivery. 1,96 There is some evidence to suggest that chronic inflammation accelerates aging, and there are some anecdotal reports of cardiovascular events and some biomarkers that are indicators of increased cardiovascular risk factors.…”

Section: Other Organ Diseasesmentioning

confidence: 99%

Adult Care in Cystic Fibrosis

Elborn

2019

Semin Respir Crit Care Med

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Cystic fibrosis (CF) is now more common in adults than children in countries with well-developed health care systems. The number of adults continues to increase and will further increase if the new cystic fibrosis transmembrane conductance regulator (CFTR) modulators are disease modifying. Most of the complex morbidity and almost all the mortality of CF occur in adults and will increasingly follow this pattern even with new effective modulator therapies. Maintaining good quality of life including social functioning and maximizing survival for adults are the key priorities. This requires a highly knowledgeable and adaptable multidisciplinary team, which, though focused on maintaining lung health, requires an increasing range of other disciplines and specialties to maximize well-being. Changes in health care systems will require current models of care to adapt to provide care for the large number of adult patients. With increasing survival and age, many are likely to have both CF morbidities and additional diseases of aging. New models are needed for health care delivery for this expanding population with complex medical conditions.

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Bone health and disease in cystic fibrosis

Cited by 24 publications

References 21 publications

Nutritional Considerations in Pediatric Pancreatitis

Nutritional Considerations in Pediatric Pancreatitis

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

Adult Care in Cystic Fibrosis

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