Estado Hiperosmolar Hiperglucémico en Pediatría: Revisión Actualizada y Consideraciones en el Síndrome de Prader-Willi
Resumen
Introducción: El estado hiperosmolar hiperglucémico representa una complicación metabólica grave y poco frecuente en pediatría, cuya incidencia ha mostrado un ascenso vinculado a la obesidad. En pacientes con Síndrome de Prader-Willi, esta condición adquiere una relevancia crítica debido a los desafíos diagnósticos y al elevado riesgo de morbimortalidad asociado a su perfil genético y endocrino. Objetivo: Sintetizar la evidencia científica sobre el estado hiperosmolar hiperglucémico en el síndrome de Prader Willi, sus implicaciones fisiopatológicas y ajustes terapéuticos para reducir complicaciones iatrogénicas. Metodología: Se realizó una revisión bibliográfica exhaustiva (48 referencias), centrada en la osmorregulación, composición corporal y sensibilidad a la insulina durante crisis hiperosmolares. Resultados: La evidencia destaca tres factores críticos en el síndrome de Prader Willi: hipodipsia central, sarcopenia relativa y sensibilidad insulínica paradójica. Además, protocolos estándar basados en el peso total pueden inducir sobrecarga hídrica y edema cerebral. Conclusión: El manejo en el síndrome de Prader Willi exige precisión para garantizar la seguridad clínica, es imperativo calcular la reposición hídrica basada en la masa magra y no en el peso total, conjuntamente con dosis bajas de insulina (0.025- 0.05 U/kg/h) y establecer un control ambiental estricto de alimentos.
Descargas
Citas
Alves, C., & Franco, R. R. (2020). Prader-Willi syndrome: endocrine manifestations and management. Archives of endocrinology and metabolism, 64(3), 223–234. https://doi.org/10.20945/2359-3997000000248
American Diabetes Association Professional Practice Committee (2025). 2. Diagnosis and Classification of Diabetes: Standards of Care in Diabetes-2025. Diabetes care, 48(1 Suppl 1), S27–S49. https://doi.org/10.2337/dc25-S002
Angulo, M. A., Butler, M. G., & Cataletto, M. E. (2015). Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. Journal of endocrinological investigation, 38(12), 1249–1263. https://doi.org/10.1007/s40618-015-0312-9
Bellis, S. A., Kuhn, I., Adams, S., Mullarkey, L., & Holland, A. (2022). The consequences of hyperphagia in people with Prader-Willi Syndrome: A systematic review of studies of morbidity and mortality. European journal of medical genetics, 65(1), 104379. https://doi.org/10.1016/j.ejmg.2021.104379
Boboc, A. A., Ionescu, M. I., Tataranu, E., Boboc, C., & Galos, F. (2024). Exploring the Diagnostic Complexity of Diabetes Subtypes in Pediatric Obesity: A Case Report of an Adolescent With Prader-Willi Phenotype and Literature Review. Cureus, 16(8), e66456. https://doi.org/10.7759/cureus.66456
Brown, S. S. G., Manning, K. E., Fletcher, P., & Holland, A. (2022). In vivo neuroimaging evidence of hypothalamic alteration in Prader-Willi syndrome. Brain communications, 4(5), fcac229. https://doi.org/10.1093/braincomms/fcac229
Calcaterra, V., Magenes, V. C., Destro, F., Baldassarre, P., Silvestro, G. S., Tricella, C., Visioli, A., Verduci, E., Pelizzo, G., & Zuccotti, G. (2023). Prader-Willi Syndrome and Weight Gain Control: From Prevention to Surgery-A Narrative Review. Children (Basel, Switzerland), 10(3), 564. https://doi.org/10.3390/children10030564
Choi, Y. J., & Yang, A. (2025). Long-term impact of growth hormone therapy on mortality and type 2 diabetes in Prader-Willi syndrome: a nationwide cohort study. Frontiers in endocrinology, 16, 1642129. https://doi.org/10.3389/fendo.2025.1642129
Clerc, A., Coupaye, M., Mosbah, H., Pinto, G., Laurier, V., Mourre, F., Merrien, C., Diene, G., Poitou, C., & Tauber, M. (2021). Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients. Journal of clinical medicine, 10(22), 5310. https://doi.org/10.3390/jcm10225310
Couto-Rosende, Y., Garcia-Tirado, D., Palacio-Marco, M., Caixàs, A., & Corripio, R. (2023). A Personalized Approach to Determining the Caloric Needs of Children with Prader-Willi Syndrome Treated with Growth Hormone. Journal of clinical medicine, 12(12), 3967. https://doi.org/10.3390/jcm12123967
Crinò, A., Fintini, D., Bocchini, S., & Grugni, G. (2018). Obesity management in Prader-Willi syndrome: current perspectives. Diabetes, metabolic syndrome and obesity : targets and therapy, 11, 579–593. https://doi.org/10.2147/DMSO.S141352
Crinò, A., & Grugni, G. (2020). Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome. Current diabetes reports, 20(2), 7. https://doi.org/10.1007/s11892-020-1284-5
Daley, S., Fermin, M., & Mendez, M. D. (2025). Prader-Willi Syndrome. In StatPearls. StatPearls Publishing.
Dhatariya K, Mustafa O, Stathi D. (2025). Hyperglycemic Crises. Endotext [Internet]. Disponible en: https://www.ncbi.nlm.nih.gov/books/NBK279052/ ( 2026, 20 de enero)
Erhardt, É., & Molnár, D. (2022). Prader-Willi Syndrome: Possibilities of Weight Gain Prevention and Treatment. Nutrients, 14(9), 1950. https://doi.org/10.3390/nu14091950
Everett, E. M., Copeland, T., Wisk, L. E., & Chao, L. C. (2023). Risk Factors for Hyperosmolar Hyperglycemic State in Pediatric Type 2 Diabetes. Pediatric diabetes, 2023, 1318136. https://doi.org/10.1155/2023/1318136
Feria Díaz, G. E., Sagué Larrea, J., Leyva Proenza, C. A., & Hernández Batista, S. de la C. (2025). Estado hiperosmolar hiperglucémico, revisión bibliográfica. Correo Científico Médico, 29, e5307. Recuperado a partir de https://revcocmed.sld.cu/index.php/cocmed/article/view/5307
Glaser, N., Fritsch, M., Priyambada, L., et al. (2022). Cetoacidosis diabética y estado hiperglucémico hiperosmolar. ISPAD Clinical Practice Consensus Guidelines 2022 [Internet]. Berlín: International Society for Pediatric and Adolescent Diabetes. Disponible en: https://www.ispad.org/resources/ispad-clinical-practice-consensus-guidelines/2022-ispad-clinical-practice-consensus-guidelines/ispad-cpcg-2022-spanish.html; (2026, 21 enero).
Grugni, G., Lupi, F., Bonetti, M., Bocchini, S., Bucolo, C., Corica, D., Crinò, A., Faienza, M. F., Fintini, D., Licenziati, M. R., Maghnie, M., Mozzillo, E., Pajno, R., Zampino, G., Sartorio, A., Radetti, G., & Genetic Obesity Group of the Italian Society of Pediatric Endocrinology and Diabetology (2025). Assessing Metabolic Syndrome Risk in Children and Adolescents with Prader-Willi Syndrome: A Comparison of Index Performance. Journal of clinical medicine, 14(13), 4716. https://doi.org/10.3390/jcm14134716
Höybye, C., & Petersson, M. (2025). Neuropeptides and the Autonomic Nervous System in Prader-Willi Syndrome. International journal of molecular sciences, 27(1), 352. https://doi.org/10.3390/ijms27010352
Howarth, A., Carroll, C., Battersby, M., Brown, S. & DiMucci-Ward, J. (2024). Prader-Willi Syndrome and the Use of Medical Nutrition Therapy. Case Reports in Clinical Medicine, 13(8):339-344. https://doi.org/10.4236/crcm.2024.138041
Hu, C. X., Xia, F. L., Qin, Y. F., Chao, Y. Q., Ruan, Y. L., Li, J. W., Dong, G. P., & Zou, C. C. (2025). Food responsiveness, addiction, and hyperphagia in Prader-Willi syndrome: a cross-sectional study of 210 Chinese patients. Frontiers in endocrinology, 16, 1665040. https://doi.org/10.3389/fendo.2025.1665040
Hwang, J., & Cho, S. Y. (2023). Management of Hyperphagia and Obesity in Prader-Willi Syndrome. Ewha medical journal, 46(Suppl 1), e32. https://doi.org/10.12771/emj.2023.e32
Injeyan, M., Baron, S., Lauzier, B., Gaillard-Le Roux, B., & Denis, M. (2023). Hyperglycaemic hyperosmolar state and cerebral thrombophlebitis in paediatrics: A case report. Endocrinology, diabetes & metabolism, 6(2), e389. https://doi.org/10.1002/edm2.389
Kahane, A., Metivier, E., Bahm, A., & Hamilton, J. (2025). Late Diagnosis of Prader-Willi Syndrome in an Adolescent With Significant Complications of Type 2 Diabetes. JCEM case reports, 3(11), luaf221. https://doi.org/10.1210/jcemcr/luaf221
Kim, S. J., Cho, S. Y., & Jin, D. K. (2021). Prader-Willi syndrome: an update on obesity and endocrine problems. Annals of pediatric endocrinology & metabolism, 26(4), 227–236. https://doi.org/10.6065/apem.2142164.082
Lee, S., Yoo, S., Yoon, J. Y., Cheon, C. K., & Kim, Y. A. (2023). Pediatric management challenges of hyperglycemic hyperosmolar state: case series of Korean adolescents with type 2 diabetes. Annals of pediatric endocrinology & metabolism, 28(1), 61–66. https://doi.org/10.6065/apem.2142108.054
Lukman, L., Darma, I., & Arimbawa, I. (2022). Hyperglycemic hyperosmolar state in children: a case series. Intisari Sains Medis, 13(2):431–435. 10.15562/ism.v13i2.1333
Madeo, S. F., Zagaroli, L., Vandelli, S., Calcaterra, V., Crinò, A., De Sanctis, L., Faienza, M. F., Fintini, D., Guazzarotti, L., Licenziati, M. R., Mozzillo, E., Pajno, R., Scarano, E., Street, M. E., Wasniewska, M., Bocchini, S., Bucolo, C., Buganza, R., Chiarito, M., Corica, D., … Delvecchio, M. (2024). Endocrine features of Prader-Willi syndrome: a narrative review focusing on genotype-phenotype correlation. Frontiers in endocrinology, 15, 1382583. https://doi.org/10.3389/fendo.2024.1382583
Mager, D. R., MacDonald, K., Duke, R. L., Avedzi, H. M., Deehan, E. C., Yap, J., Siminoski, K., & Haqq, A. M. (2022). Comparison of Body Composition, Muscle Strength and Cardiometabolic Profile in Children with Prader-Willi Syndrome and Non-Alcoholic Fatty Liver Disease: A Pilot Study. International journal of molecular sciences, 23(23), 15115. https://doi.org/10.3390/ijms232315115
Mazur, A., Zachurzok, A., Baran, J., Dereń, K., Łuszczki, E., Weres, A., Wyszyńska, J., Dylczyk, J., Szczudlik, E., Drożdż, D., Metelska, P., Brzeziński, M., Kozioł-Kozakowska, A., Matusik, P., Socha, P., Olszanecka-Glinianowicz, M., Jackowska, T., Walczak, M., Peregud-Pogorzelski, J., ... Wójcik, M. (2022). Childhood Obesity: Position Statement of Polish Society of Pediatrics, Polish Society for Pediatric Obesity, Polish Society of Pediatric Endocrinology and Diabetes, the College of Family Physicians in Poland and Polish Association for Study on Obesity. Nutrients, 14(18), 3806. https://doi.org/10.3390/nu14183806
Mohn, A., Polidori, N., Castorani, V., Comegna, L., Giannini, C., Chiarelli, F., & Blasetti, A. (2021). Hyperglycaemic hyperosmolar state in an obese prepubertal girl with type 2 diabetes: case report and critical approach to diagnosis and therapy. Italian journal of pediatrics, 47(1), 38. https://doi.org/10.1186/s13052-021-00983-z
Muscogiuri, G., Barrea, L., Faggiano, F., Maiorino, M. I., Parrillo, M., Pugliese, G., Ruggeri, R. M., Scarano, E., Savastano, S., Colao, A., & RESTARE (2021). Obesity in Prader-Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches. Journal of endocrinological investigation, 44(10), 2057–2070. https://doi.org/10.1007/s40618-021-01574-9
NCD Risk Factor Collaboration (NCD-RisC) (2024). Worldwide trends in underweight and obesity from 1990 to 2022: a pooled analysis of 3663 population-representative studies with 222 million children, adolescents, and adults. Lancet (London, England), 403(10431), 1027–1050. https://doi.org/10.1016/S0140-6736(23)02750-2
Noordam, C., Stephan, A., & Eiholzer, U. (2023). Lean Body Mass in Boys With Prader-Willi Syndrome Increases Normally During Spontaneous and Induced Puberty. The Journal of clinical endocrinology and metabolism, 108(9), 2299–2306. https://doi.org/10.1210/clinem/dgad101
Paprocki, E., Eickhoff, P., Sweeney, B., & Halpin, K. (2022). ODP383 Clinical Presentation and Management of Diabetes Mellitus in Youth with Prader-Willi Syndrome. Journal of the Endocrine Society, 6(Suppl 1), A601–A602. https://doi.org/10.1210/jendso/bvac150.1248
Shaikh, M. G., Barrett, T. G., Bridges, N., Chung, R., Gevers, E. F., Goldstone, A. P., Holland, A., Kanumakala, S., Krone, R., Kyriakou, A., Livesey, E. A., Lucas-Herald, A. K., Meade, C., Passmore, S., Roche, E., Smith, C., & Soni, S. (2024). Prader-Willi syndrome: guidance for children and transition into adulthood. Endocrine connections, 13(8), e240091. https://doi.org/10.1530/EC-24-0091
Szabadi, S., Sila, Z., Dewey, J., Rowland, D., Penugonda, M., & Ergun-Longmire, B. (2022). A Review of Prader–Willi Syndrome. Endocrines, 3(2), 329-348. https://doi.org/10.3390/endocrines3020027
Silva, A. C. S. C., Barcelos, G. P., Guimarães, M. A., Kanashiro, A. L. B., Grapegia, D. Z., Arruda, M. C., Silva, D. M., de Rosso, A. R. D., D’Amico, M. M., Silva, F. C., Venturini, M. R. Z. T., Paiva, I. G., Figueredo, M. R., Leão, A. S., Barbosa, C. I. C., Sanches, M. K., & de Oliveira, K. C. (2023). Síndrome de Prader-Willi: uma abordagem diagnóstica, evolução clínica e revisão. Brazilian Journal of Health Review, 6(5), 21932–21940. https://doi.org/10.34119/bjhrv6n5-226
Simunovic, M., Kumric, M., Rusic, D., Paradzik Simunovic, M., & Bozic, J. (2024). Continuous Glucose Monitoring-New Diagnostic Tool in Complex Pathophysiological Disorder of Glucose Metabolism in Children and Adolescents with Obesity. Diagnostics (Basel, Switzerland), 14(24), 2801. https://doi.org/10.3390/diagnostics14242801
Sohn, Y. B., Moon, J. E., Jung, Y. J., & Yu, Y. A. (2023). Updates on Obesity in Prader-Willi Syndrome: From Genetics to Management. Ewha medical journal, 46(Suppl 1), e33. https://doi.org/10.12771/emj.2023.e33
Qian, Y., Xia, F., Zuo, Y., Zhong, M., Yang, L., Jiang, Y., & Zou, C. (2022). Do patients with Prader-Willi syndrome have favorable glucose metabolism?. Orphanet journal of rare diseases, 17(1), 187. https://doi.org/10.1186/s13023-022-02344-3
Ryan, P. M., Sellers, E. A. C., Amed, S., & Hamilton, J. K. (2023). Hyperglycaemic hyperosmolar state: No longer an endocrine crisis exclusive to adulthood. Paediatrics & child health, 29(2), 81–83. https://doi.org/10.1093/pch/pxad073
Shahramian, I., Ostad Rahimi, P., & Radvar, S. (2022). Hyperosmolar hyperglycemic state in children: case report and review of the literature. Journal fur klinische Endokrinologie und Stoffwechsel, 15(2), 60–62. https://doi.org/10.1007/s41969-022-00164-1
Tachibana, M., Oto, Y., Kashimada, K., Ishii, T., Takahashi, Y., Muroya, K., Aoki, Y., Kurosawa, K., Ogata, T., & Kawai, M. (2025). A questionnaire-based survey on hyperphagia in individuals with Prader-Willi syndrome in Japan. Endocrine journal, 72(11), 1217–1225. https://doi.org/10.1507/endocrj.EJ25-0039
Tauber, M., & Hoybye, C. (2021). Endocrine disorders in Prader-Willi syndrome: a model to understand and treat hypothalamic dysfunction. The lancet. Diabetes & endocrinology, 9(4), 235–246. https://doi.org/10.1016/S2213-8587(21)00002-4
Van Eyck, A., Eerens, S., Trouet, D., Lauwers, E., Wouters, K., De Winter, B. Y., van der Lee, J. H., Van Hoeck, K., & Ledeganck, K. J. (2021). Body composition monitoring in children and adolescents: reproducibility and reference values. European journal of pediatrics, 180(6), 1721–1732. https://doi.org/10.1007/s00431-021-03936-0
Zahran, N. A., & Jadidi, S. (2023). Pediatric Hyperglycemic Hyperosmolar Syndrome: A Comprehensive Approach to Diagnosis, Management, and Complications Utilizing Novel Summarizing Acronyms. Children (Basel, Switzerland), 10(11), 1773. https://doi.org/10.3390/children10111773
Derechos de autor 2026 Gabriela Fernanda Samaniego Urrego , Jéssica Priscila Chacón Molina
Esta obra está bajo licencia internacional Creative Commons Reconocimiento 4.0.
.png)
.png)
1.png)
