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Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants
PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific cl...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Pediatric Society
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4342778/ https://www.ncbi.nlm.nih.gov/pubmed/25729396 http://dx.doi.org/10.3345/kjp.2015.58.1.28 |
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author | Kim, Ahlee Moon, Jin Soo Yang, Hye Ran Chang, Ju Young Ko, Jae Sung Seo, Jeong Kee |
author_facet | Kim, Ahlee Moon, Jin Soo Yang, Hye Ran Chang, Ju Young Ko, Jae Sung Seo, Jeong Kee |
author_sort | Kim, Ahlee |
collection | PubMed |
description | PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18±10.5 months, and diagnosis after symptom onset was made at the mean age of 11±9.7 months. The mean z score was -3.15±1.14 for weight, -0.12±1.05 for height, 1.01±1.58 for head circumference, and -1.76±1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis. |
format | Online Article Text |
id | pubmed-4342778 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | The Korean Pediatric Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-43427782015-02-27 Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants Kim, Ahlee Moon, Jin Soo Yang, Hye Ran Chang, Ju Young Ko, Jae Sung Seo, Jeong Kee Korean J Pediatr Original Article PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18±10.5 months, and diagnosis after symptom onset was made at the mean age of 11±9.7 months. The mean z score was -3.15±1.14 for weight, -0.12±1.05 for height, 1.01±1.58 for head circumference, and -1.76±1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis. The Korean Pediatric Society 2015-01 2015-01-31 /pmc/articles/PMC4342778/ /pubmed/25729396 http://dx.doi.org/10.3345/kjp.2015.58.1.28 Text en Copyright © 2015 by The Korean Pediatric Society http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Kim, Ahlee Moon, Jin Soo Yang, Hye Ran Chang, Ju Young Ko, Jae Sung Seo, Jeong Kee Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title | Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title_full | Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title_fullStr | Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title_full_unstemmed | Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title_short | Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
title_sort | diencephalic syndrome: a frequently neglected cause of failure to thrive in infants |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4342778/ https://www.ncbi.nlm.nih.gov/pubmed/25729396 http://dx.doi.org/10.3345/kjp.2015.58.1.28 |
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