Diagnosis and treatment of endocrine comorbidities in patients with cystic fibrosis

Siwamogsatham, Oranan; Alvarez, Jessica A.; Tangpricha, Vin

doi:10.1097/med.0000000000000096

Cited by 28 publications

(36 citation statements)

References 105 publications

(147 reference statements)

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“…13 Because the median age of survival in patients with CF is now closer to 40 years, 13,14 there has been an increase in prevalence of multiple comorbidities, including CF-related bone disease (CFBD). Known risk factors for CFBD include poor nutrition, chronic lung infection and inflammation, pancreatic insufficiency, delayed puberty, decreased physical activity, and medications, including glucocorticoids and transplant-related therapies.…”

Section: Pediatric Bone Health: Cystic Fibrosismentioning

confidence: 99%

“…Known risk factors for CFBD include poor nutrition, chronic lung infection and inflammation, pancreatic insufficiency, delayed puberty, decreased physical activity, and medications, including glucocorticoids and transplant-related therapies. 15,16 The presence of impaired glucose metabolism in CF-related diabetes and possible direct effect of the CFTR on osteoclast activation 12,13 may also be responsible for the bone disease reported in both children and adults.…”

Section: Pediatric Bone Health: Cystic Fibrosismentioning

confidence: 99%

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Update on Bone Health in Pediatric Chronic Disease

Williams

2016

Endocrinology and Metabolism Clinics of North America

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Section: Pediatric Bone Health: Cystic Fibrosismentioning

confidence: 99%

Section: Pediatric Bone Health: Cystic Fibrosismentioning

confidence: 99%

Update on Bone Health in Pediatric Chronic Disease

Williams

2016

Endocrinology and Metabolism Clinics of North America

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“…In the pancreas of patients with CF, stasis of mucous, reduction of pancreatic fluid flow and increased concentration of protein results in pancreatic fibrosis, destruction and eventual insufficiency, leading to malabsorption of fat and fat-soluble vitamins, including vitamin D(5, 6). Other endocrine abnormalities can occur in patients with CF(7). Cystic fibrosis related diabetes (CFRD), delayed puberty and impaired fertility are also commonly occurring endocrine co-morbidities in patients with CF(7).…”

Section: Introductionmentioning

confidence: 99%

“…Other endocrine abnormalities can occur in patients with CF(7). Cystic fibrosis related diabetes (CFRD), delayed puberty and impaired fertility are also commonly occurring endocrine co-morbidities in patients with CF(7). …”

Section: Introductionmentioning

confidence: 99%

Treatment of vitamin D deficiency in cystic fibrosis

Chesdachai

Tangpricha

2016

The Journal of Steroid Biochemistry and Molecular Biology

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Vitamin D deficiency occurs frequently in patients with cystic fibrosis (CF). Vitamin D is important for optimal mineralization of bone and may be important for other comorbidities commonly occurring in patients with CF. Vitamin D deficiency in patients with CF can arise from various causes including pancreatic exocrine insufficiency, lack of outdoor activity, and alterations of vitamin D metabolism. Due to fat malabsorption stemming from pancreatic insufficiency, higher oral doses of vitamin D are necessary to correct and maintain optimal vitamin D status in patients with CF. Recent studies have demonstrated that higher vitamin D status is associated with better lung function and that vitamin D therapy may help recovery from pulmonary exacerbations of CF. The mechanisms by which vitamin D may exert its beneficial actions in CF are unclear but likely related to the role vitamin D has in modulating the adaptive and innate immune response. Large randomized clinical studies to evaluate the potential role of vitamin D as adjunctive therapy in CF that goes beyond bone are necessary.

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“…With iodine-containing expectorant no longer in routine use, the prevalence of thyroid dysfunction in CF patients is now unknown (11). A recent small study by Volta et al showed that there was no significant thyroid dysfunction observed in 17 CF patients compared to controls, and concluded that this may be related to improved nutritional status and lack of iodinecontaining medication use (12).…”

Section: Introductionmentioning

confidence: 99%

Thyroid Function in Patients with Cystic Fibrosis: No Longer a Concern?

Lee

Chesdachai

Lee

et al. 2016

Thyroid

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Background: Development of goiter and hypothyroidism has been reported in patients with cystic fibrosis (CF) since the 1970s, especially when treated with iodine-based expectorants. With iodine-containing expectorants no longer in routine use, the prevalence of thyroid dysfunction in CF patients is unknown. This cross-sectional study assessed thyroid function status in a large cohort of CF patients. Methods: Sera from ambulatory subjects were obtained from an Institutional Review Board (IRB)-approved biorepository of patients seen at the Emory CF Center between January 1, 2011, and December 31, 2014. Sera from hospitalized subjects were obtained from banked specimens from an IRB-approved inpatient clinical trial. Demographics, forced expiratory volume in one second (FEV1), and medication use were assessed from medical records. Thyroid function tests were measured from the stored sera. Multivariate regression models assessed associations between covariates and thyrotropin (TSH), free thyroxine (fT4), and thyroid dysfunction risk. Results: A total of 89 subjects (54% male, 91% white, M age = 24.4 years, median FEV1 63%) were included in the analyses. One subject was on thyroid hormone replacement, 93% were on pancreatic enzyme replacement, and 68% received antibiotics within six months. None had computed tomography scans with intravenous contrast within six months. One patient had positive thyroid peroxidase (TPO) antibodies. Of the 87 subjects with measured TSH values, seven (8%) had abnormal levels (range 0.2-7.6 lIU/mL; one overt, four subclinical hypothyroidism, and two subclinical hyperthyroidism). Of the 56 subjects with measured fT4 values, 19 (34%) had slightly low levels (range 0.49-0.79 ng/dL; 17 isolated mild hypothyroxinemia). A positive correlation between age and body mass index (BMI; p < 0.001) and a negative correlation between age and FEV1 (p = 0.041) were seen. Age, sex, race/ethnicity, BMI, FEV1, hospitalization status, use of pancreatic enzyme or thyroid hormone replacement, recent antibiotic use, and TPO antibody positivity were not predictive of TSH, fT4, or thyroid dysfunction risk. Stratified analyses by hospitalization did not predict TSH or fT4. Conclusions: Although 24 (27%) of the patients had abnormal serum thyroid function tests, overt thyroid dysfunction was rare in this cohort of 89 patients with CF. The degree of hypothyroxinemia was marginal, likely due to nonthyroidal illness. There were no significant predictors of thyroid dysfunction.

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Diagnosis and treatment of endocrine comorbidities in patients with cystic fibrosis

Cited by 28 publications

References 105 publications

Update on Bone Health in Pediatric Chronic Disease

Update on Bone Health in Pediatric Chronic Disease

Treatment of vitamin D deficiency in cystic fibrosis

Thyroid Function in Patients with Cystic Fibrosis: No Longer a Concern?

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