Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood with Cystic Fibrosis

Aris, Robert M.; Renner, Jordan B.; Winders, Andrew D.; Buell, Hope; Riggs, Debra B.; Lester, Gayle E.; Ontjes, David A.

doi:10.7326/0003-4819-128-3-199802010-00004

Cited by 277 publications

(210 citation statements)

References 27 publications

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“…However, chronically ill young adults with a wide variety of disorders, when compared to their cohort in the general population, have lesser academic and employment achievement, less vocational education and less permanent employment [45], are more likely to be single [47] and have delayed independence [3]. Cohorts have not been followed long enough to establish whether this group "catches up" developmentally at a later point in adulthood, which might allow for survivors to make the transition from dependence to independence in their relationships and work.…”

Section: Psychosocial Considerationsmentioning

confidence: 99%

“…It is important that linear growth be maximized through medical and nutritional intervention [44]. Bone mineralization is decreased and rates of osteoporosis are significantly increased in patients with CF as they age; adults with late stage CF are at high risk for fractures and severe kyphosis [45].…”

Section: Impaired Growth and Development And Impact Of Chronic Steroimentioning

confidence: 99%

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Late Consequences of Chronic Pediatric Illness

Turkel

Pao

2007

Psychiatric Clinics of North America

118

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Section: Psychosocial Considerationsmentioning

confidence: 99%

Section: Impaired Growth and Development And Impact Of Chronic Steroimentioning

confidence: 99%

Late Consequences of Chronic Pediatric Illness

Turkel

Pao

2007

Psychiatric Clinics of North America

118

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“…Zato se zmanjša pljučna funkcija in sposobnost izkašljevanja ter čiščenja dihalnih poti, kar lahko privede do poslabšanja pljučne bolezni. 22 Presejanje z DXA je potrebno opraviti pri odraslih bolnikih s CF in otrocih, starejših od 8 let, pri bolnikih s telesno težo, manjšo od 90 % idealne, vrednost FEV 1, nižja od 50 % pričakovane vrednosti, kadar je predvideno zdravljenje s kortikosteroidi v odmerku ekvivalentnemu 5 mg pronisona dnevno za obdobje treh mesecev na leto, pri bolnikih z zapoznelo puberteto in/ali pri podatku o zlomih. DXA ponavljamo na določene intervale od enega do pet let, odvisno od izhodiščne vrednosti kostne gostote, od katere so odvisni tudi priporočeni ukrepi.…”

Section: Novosti V O Bolezni Pre Zapletov Pr Fibroziunclassified

Novosti v Obravnavi Bolezni Prebavil in Zapletov Pri Cistični Fibrozi Ter Organizacija Prehoda Iz Pediatričnega Centra v Center Za Cistično Fibrozo Za Odrasle

et al. 2015

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Cystic fibrosis (CF) is the most common autosomal recessive lethal hereditary disorder among whites. Survival of patients with CF has progressively improved over the last four decades.As life expectancy has been greatly extended, complications like osteopenia and CF-related diabetes mellitus (CFRDM) occur. In our article, a new management of these two complications is described.Gastrointestinal tract, exocrine pancreas and liver are also affected in CF.Maintaining good nutrition while treating pancreatic insufficiency, treating gastroesophageal reflux disease and treating liver disease are very important issues in the treatment gastrointestinal tract disease in CF.As the CF patient population median survival increases, a growing number of adolescents require care in the adult health care system which must be capable of responding appropriately to their needs.We present a model of transition from paediatric to adult medical care in our CF center.

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“…Most patients with severe CF-related lung disease have reduced bone mass (115,116) . Recent reports suggest that defective bone mineralisation occurs in early childhood in individuals with CF (51,117) .…”

Section: Bone Healthmentioning

confidence: 99%

Symposium 6: Young people, artificial nutrition and transitional care The nutritional challenges of the young adult with cystic fibrosis: transition

Morton

2009

Proc. Nutr. Soc.

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Cystic fibrosis (CF) is a complex multisystem disorder affecting mainly the gastrointestinal tract and respiratory system. Intestinal malabsorption occurs in approximately 90 % of patients. In the past, malnutrition was an inevitable consequence of disease progression, leading to poor growth, impaired respiratory muscle function, decreased exercise tolerance and immunological impairment. A positive association between body weight and height and survival has been widely reported. The energy requirements of patients with CF vary widely and generally increase with age and disease severity. For many young adults requirements will be 120-150% of the age-related estimated average requirement. To meet these energy needs patients are encouraged to eat a high-fat high-energy diet with appropriate pancreatic enzyme supplements. Many patients are unable to achieve an adequate intake as a result of a variety of factors including chronic poor appetite, infection-related anorexia, gastro-oesophageal reflux and abdominal pain. Oral energy supplements and enteral tube feeding are widely used. Nutritional support has been shown to improve nutritional status and stabilise or slow the rate of decline in lung function. With such emphasis on nutritional intake and nutritional status throughout life, poor adherence to therapies and issues relating to body image are emerging. The median survival of patients with CF is increasing. CF is now considered a life-limiting disease of adulthood rather than a terminal childhood illness. With increased longevity new challenges are emerging that include the transition of young adults with CF to adult services, CF-related diabetes, disordered eating, osteoporosis, liver disease and transplantation.

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Increased Rate of Fractures and Severe Kyphosis: Sequelae of Living into Adulthood with Cystic Fibrosis

Abstract: Osteoporosis is universal in adults with late-stage cystic fibrosis, and its complications include increased fracture rates and severe kyphosis.

Cited by 277 publications

References 27 publications

Late Consequences of Chronic Pediatric Illness

Late Consequences of Chronic Pediatric Illness

Novosti v Obravnavi Bolezni Prebavil in Zapletov Pri Cistični Fibrozi Ter Organizacija Prehoda Iz Pediatričnega Centra v Center Za Cistično Fibrozo Za Odrasle

Symposium 6: Young people, artificial nutrition and transitional care The nutritional challenges of the young adult with cystic fibrosis: transition

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