pediatric case study primary ciliary dyskinesia n.
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Pediatric Case Study Primary Ciliary Dyskinesia
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  1. Pediatric Case StudyPrimary CiliaryDyskinesia Marion Viglietta UMD College Park Dietetic Intern

  2. Objectives Overview of Primary CiliaryDyskinesia Assessment of Patient PES Statement and Needs Plans & Goals Evaluation and Follow-Up

  3. Primary CiliaryDyskinesia • Autosomal recessive genetic disorder • Often seen in siblings • Immotile cilia • Chronic recurrent lung infections • Treatment much like CF • Nebulizers, bronchodilators, nasal steroids, etc. • No pancreatic insufficiency (PI) • Near normal life expectancy

  4. Initial Assessment • 14 y/o F admitted with Primary CiliaryDyskinesia (PCD) exacerbation and 6.9 kg weight loss since 06/2011 admission • PMH: PCD, FTT, pHTN, Brconchiectasis, Left Lower Lobectomy, Reflux • Social Hx: Family from Algeria

  5. PES Statement • PES: Inadequate oral food/beverage intake related to high calorie needs from PCD exacerbation and severely decreased lung function as evidenced by 6.9 kg weight loss in 9.5 months and patient BMI < 3rd percentile

  6. Growth Chart

  7. Labs & Medications

  8. Needs

  9. Plans/Goals

  10. Nutritional Evaluation Follow Up Plans… • Monitor/encourage PO intake • Monitor weight gain Patient with 2.7 kg weight gain in 4 days PO intake increasing Megace effective

  11. Health of the Patient PCD Improper management of care FTT Left Lower Lobectomy Bronchiectasis

  12. References Block, R.W., Krebs, N.F, et al (2005) Failure to Thrive as a Manifestation of Child Neglect Pediatrics Official Journal of the American Academy of Pediatrics 116;1234 DOI: 10.1542/peds.2005-2032 Frequently Asked Questions Primary CiliaryDyskenesia Foundation website 2011 • Mackerness, K. J., Jose, P. J., & Bush, A. A. (2009). Differences in airway inflammation in cystic fibrosis and primary ciliarydyskinesia. Pediatric Asthma Allergy & Immunology, 22(4), 163-168. doi:10.1089/pai.2009.0022

  13. Questions?