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Revista Cubana de Pediatría

ISSN 1561-3119 (Electronic)
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2013, Number 1

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Rev Cubana Pediatr 2013; 85 (1)

Diencephalic syndrome as a cause of severe malnutrition

Báez SL, Jiménez GR, Piñeiro FE, Pérez ME, Sánchez FI, Sánchez MO, Valle GL
Full text How to cite this article

Language: Spanish
References: 10
Page: 120-129
PDF size: 335.38 Kb.


Key words:

diencephalic syndrome, malnutrition, cachexia-anorexia.

ABSTRACT

Diencephalic syndrome is a set of symptoms and signs caused by dysfunction in this area of the encephalon and characterized by marked malnutrition despite adequate intake of calories. Two cases were reported in this paper. The first one was a 13-years old girl with a history of medro failure since her 3rd month of life, who was admitted to this service for the study of her severe protein/energy malnutrition. In the course of her hospitalization, she presented with unstable appetite and suffered a paroxistic event one month after being hospitalized. The infant girl was then performed magnetic resonance imaging test which revealed a 3 cm x 3cm rounded hypodense image in the suprasellar region. She was operated on twice, the tumor was excised and the anatomic and pathological analysis yielded the presence of low grade pilocytic astrocytoma. The second patient was an small infant who was admitted to the service with a confirmed diagnosis of intracranial tumor for study, very similar to the case previously presented. He suffered an acute protein/energy malnutrition, so he was performed a computerized tomography which showed a broad tumoral mass in the suprasellar region with dilated ventricular system. During the hospitalization, he presented with marked anorexia, progressive loss of weight and he finally underwent gastrotomy, but he died 59 days after admission as a result of pancytopenia. The result of necropsy was low grade pilocytic astrocytoma.


REFERENCES

  1. Russell AA. A diencephalic syndrome of emanciation in infancy and childhood. Arch Dis Child. 1951;26:266-74.

  2. Villares JM, Fernández Carrión F, Fernández Gallego Ma E, Muñoz González A, Manzanares López-Manzanares J, Rodrigo Alfageme M. Síndrome Diencefálico: Una causa poco común de malnutrición. An Esp Pediatr. 2002;56:466-71.

  3. Poussaint TY, Barnes PD, King N, Douglas CA, Cohen L, Goumnerova L. Dyencephalic syndrome: Clinical Feautures and Imaging Fndings. Am J Neuradiol. 1997;18:1499-505.

  4. Fleischman A, Brue C, Poussaint TY, Kieran M, Pomeroy SL, Goumnerova L, et al. Diencephalic syndrome: a cause of failure to thrive and a model of partial growth hormone resistance. Pediatrics. 2005;115 (6):e742-8.

  5. Jiménez García R, Cabana Armadas R, Nodarse Fernández R, Chagues Leiva O, González Carrasco MC, Peón Bertot L, et al. Caracterización nutricional del síndrome anorexia-caquexia en el paciente oncológico pediátrico. Rev Cubana Pediatr. 2011;83(4):337-45.

  6. Huber J, Sovinz P, Lackner H, Mokry M, Eder H, Urban C. Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive. Klin Padiatr. 2007;219(2):91-4.

  7. Densupsoontorn N, Jirapinyo P, Likasitwattanakul S, Sanmaneechai O, Sanpaki K, Surachatkumtonekul T, et al. Diencephalic syndrome due to astrocytoma in three infants with failure to thrive. Pediatric International. 2011;1:784-7.

  8. Argiles JM, Busquets S, Garcia-Martinez C, Lopez-Soriano FJ. Mediators involved in the cancer anorexia-cachexia syndrome: past, present and future. Nutrition. 2005;21:977-85.

  9. Packer RJ. Brain tumors in children. Curr Opin Pediatr. 1995;7:64-72.

  10. Vlachopapadopoulou E, Tracey KJ, Capella M, Gilker C, Matthews DE. Increased energy expenditure in a patient with diencephalic syndrome. J Pediatr. 1993;122(6):922-4.




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Rev Cubana Pediatr. 2013;85