Title: Integrating Consistent Individualized Carbohydrate-Controlled Anti- Inflammatory Nutritional Plan (C-ICAN) in the Management of Prader-Willi Syndrome: A Case Report. Prader–Willi syndrome (PWS) is a rare genetic disorder caused by a loss of function of specific genes on chromosome 15. Patients with this disease present unique challenges in management, particularly regarding obesity and nutritional regulation as the disease symptoms change depending on the age of the patient and the phase of the disease. These challenges pose critical stressors to caregivers and their families. We present a case report of a 5-year-old Caucasian male diagnosed with PWS, exhibiting failure to thrive and uncontrolled weight gain. His caregiver was his elderly grandmother who, by her own admission, was ill-equipped to deal with the patient’s physical symptoms and his behavior in response to dietary restrictions. Through a multidisciplinary approach involving medical nutrition therapy (MNT) involving the implementation of a Consistent Individualized Carbohydrate-Controlled Anti-Inflammatory Nutritional plan (C-ICAN), growth hormone supplementation, and behavioral interventions patient markedly improved physically and emotionally.
References
[1]
Butler, M.G. (1990) Prader‐Willi Syndrome: Current Understanding of Cause and Diagnosis. AmericanJournalofMedicalGenetics, 35, 319-332. https://doi.org/10.1002/ajmg.1320350306
[2]
Bohonowych, J., Miller, J., McCandless, S.E. and Strong, T.V. (2019) The Global Prader-Willi Syndrome Registry: Development, Launch, and Early Demographics. Genes, 10, Article 713. https://doi.org/10.3390/genes10090713
[3]
Angulo, M.A., Butler, M.G. and Cataletto, M.E. (2015) Prader-Willi Syndrome: A Review of Clinical, Genetic, and Endocrine Findings. JournalofEndocrinologicalInvestigation, 38, 1249-1263. https://doi.org/10.1007/s40618-015-0312-9
[4]
Tauber, M. and Hoybye, C. (2021) Endocrine Disorders in Prader-Willi Syndrome: A Model to Understand and Treat Hypothalamic Dysfunction. TheLancetDiabetes&Endocrinology, 9, 235-246. https://doi.org/10.1016/s2213-8587(21)00002-4
Bekx, M.T., Carrel, A.L., Shriver, T.C., Li, Z. and Allen, D.B. (2003) Decreased Energy Expenditure Is Caused by Abnormal Body Composition in Infants with Prader-Willi Syndrome. TheJournalofPediatrics, 143, 372-376. https://doi.org/10.1067/s0022-3476(03)00386-x
[7]
Couto-Rosende, Y., Garcia-Tirado, D., Palacio-Marco, M., Caixàs, A. and Corripio, R. (2023) A Personalized Approach to Determining the Caloric Needs of Children with Prader-Willi Syndrome Treated with Growth Hormone. JournalofClinicalMedicine, 12, Article 967. https://doi.org/10.3390/jcm12123967
[8]
Knott, B., Kocher, M.A., Paz, H.A., Hamm, S.E., Fink, W., Mason, J., etal. (2022) Dietary Conjugated Linoleic Acid Reduces Body Weight and Fat in Snord116m+/p- and Snord116m-/p- Mouse Models of Prader-Willi Syndrome. Nutrients, 14, Article 860. https://doi.org/10.3390/nu14040860
[9]
Butler, M.G., Miller, J.L. and Forster, J.L. (2019) Prader-Willi Syndrome—Clinical Genetics, Diagnosis and Treatment Approaches: An Update. CurrentPediatricReviews, 15, 207-244. https://doi.org/10.2174/1573396315666190716120925
[10]
Duis, J., van Wattum, P.J., Scheimann, A., Salehi, P., Brokamp, E., Fairbrother, L., etal. (2019) A Multidisciplinary Approach to the Clinical Management of Prader-Willi Syndrome. MolecularGenetics&GenomicMedicine, 7, e514. https://doi.org/10.1002/mgg3.514
[11]
Barrea, L., Vetrani, C., Fintini, D., de Alteriis, G., Panfili, F.M., Bocchini, S., etal. (2022) Prader-Willi Syndrome in Adults: An Update on Nutritional Treatment and Pharmacological Approach. CurrentObesityReports, 11, 263-276. https://doi.org/10.1007/s13679-022-00478-w