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Cystic Fibrosis and Vitamin D review with 2012 Guidelines - Nov 2019

Vitamin D deficiency and its treatment in cystic fibrosis

Journal of Cystic Fibrosis

VitaminDWiki

Tanicia Daleya'*, Kara Hughanb, Maria Rayasc, Andrea Kellyd, Vin Tangprichae,f

  • a Division of Pediatric Endocrinology and Metabolism, Emory + Children's Pediatric Institute, Department of Pediatrics, Emory University, School of Medicine, 1400 Tullie Road, Atlanta, GA 30329, USA
  • b Division of Pediatric Endocrinology and Diabetes, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, 4401 Penn Avenue, Pittsburgh, PA 15224, USA
  • c Division of Pediatric Endocrinology and Diabetes, University of Texas Health San Antonio, 7703 Floyd Curl, San Antonio, TX 78229, USA
  • d Division of Pediatric Endocrinology and Diabetes, Children's Hospital of Philadelphia, Perelman School of Medicine of University of Pennsylvania, 2716 South Street, Philadelphia, PA 19146, USA
  • e Atlanta VA Medical Center, 1670 Clairmont Road NE, Decatur, GA 30300, USA

Vitamin D deficiency is a common finding in individuals with cystic fibrosis (CF), despite routine supplementation. Hypovitaminosis D is often the result of fat malabsorption, but other contributors include increased latitude, poor nutritional intake, decreased sun exposure, impaired hydroxylation of vitamin D, and nonadherence to the prescribed vitamin D regimen. Vitamin D is critical for calcium homeostasis and optimal skeletal health, and vitamin D deficiency in CF can lead to skeletal complications of osteopenia and osteoporosis. Over time, our understanding of treatment regimens for vitamin D deficiency in CF has evolved, leading to recommendations for higher doses of vitamin D to achieve target levels of circulating 25-hydroxyvitamin D. There is also some evidence that vitamin D deficiency may have non-skeletal consequences such as an increase in pulmonary exacerbations. The exact mechanisms involved in the non-skeletal complications of vitamin D deficiency are not clearly understood, but may involve the innate immune system. Future clinical studies are needed to help address whether vitamin D has a role in CF beyond skeletal health.
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Table 2 Adapted Cystic Fibrosis Foundation Guidelines for Screening, Diagnosis and Treatment of Vitamin D Deficiency in Cystic Fibrosis Recommendations 2012?

4A. Assessment of Vitamin D Status, Target Treatment Goals and Strategy

  • Annually assess vitamin D status, preferably at the end of winter
  • Treat with vitamin D3 (cholecalciferol) to achieve and maintain serum 25(OH)D levels of at least 30 ng/ml (>75 nmol/liter).
  • Check serum 25(OH)D levels 3 months after the dose of vitamin D3 has been changed.
  • Avoid using 1a25(OH)2D as a marker of vitamin D status
  • Take once-daily vitamin D3 therapy or its weekly equivalent to maintain serum 25(OH)D levels of at least 30 ng/ml (>75 nmol/liter).
  • Difficult to treat vitamin D deficiency can be treated with calcitriol, doxercalciferol, or paricalcitol only in consultation with a specialist with expertise in vitamin D therapy.

4B. Treatment of Infants Birth to 12 months

  • 1. Initial dose of 400-500IU vitamin D3 per day.
  • 2. 25(OH)D levels less than 10 ng/ml ( <25 nmol/liter) assess for rickets and manage urgently in consultation with a specialist with expertise in vitamin D therapy.
  • 3. 25(OH)D levels that are at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml ( < 75 nmol/liter), and adherence to the prescribed regimen confirmed, increase the dose of vitamin D3 to 800-1,000 IU per day.
  • 4. 25(OH)D levels less than 20 ng/ml ( <50 nmol/liter) or persistent serum 25(OH)D levels of at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml (<75 nmol/liter), and there is confirmed adherence, increase vitamin D3 to a maximum of 2,000 IU per day.
  • 5. If unable to achieve a serum 25(OH)D level of at least 30 ng/ml (>75 nmol/liter) after treatment with 2,000 IU vitamin D3 per day, and confirmed adherence, consult a specialist with expertise in vitamin D therapy.

4C. Treatment of Children Older than 12 Months to 10 yr of age

  • Initial dose of 800-1,000 IU vitamin D3 per day.
  • Serum 25(OH)D levels that are at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml ( <75 nmol/liter), and confirmed adherence, adjust vitamin D3 to 1,600-3,000 IU per day.
  • Serum 25(OH)D levels less than 20 ng/ml ( <50 nmol/liter) or the patient has a persistent serum 25(OH)D level of at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml ( <75 nmol/liter), and confirmed adherence, increase vitamin D3 to a maximum of 4,000 IU per day.
  • If unable to achieve a serum 25(OH)D level of at least 30 ng/ml (>75 nmol/liter) after treatment with 4,000 IU vitamin D3 per day, and confirmed adherence, consult a specialist with expertise in vitamin D therapy.

4D. Treatment of Children Above 10 yr of Age and Adults

  • 1. Initial dose of 800-2,000 IU vitamin D3 per day.
  • 2. 25(OH)D levels that are at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml ( < 75 nmol/liter), and confirmed adherence, increase vitamin D3 to 1,600-6,000 IU per day.
  • 3 Serum 25(OH)D levels less than 20 ng/ml ( <50 nmol/liter) or persistent serum 25(OH)D level of at least 20 ng/ml (>50 nmol/liter) but less than 30 ng/ml (<75 nmol/liter), and confirmed adherence, increase vitamin D3 to a maximum of 10,000 IU per day.
  • 4 If unable to achieve a serum 25(OH)D level of at least 30 ng/ml (>75 nmol/liter) after treatment with 10,000 IU vitamin D3 per day, and confirmed adherence, consulta specialist with expertise in vitamin D therapy.

4E. Use of UV lamps

1. There are no recommendations for or against the use of UV lamps in the management of vitamin D deficiency.

Guidelines adapted from: Tangpricha, V., et al., An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab, 2012. 97(4): p. 1082-93. The strength of evidence is predominantly described as "Low - Consensus recommendation”.

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