Abstract
Diencephalic syndrome DS is a rarely-seen early childhood syndrome, characterized by diffuse emaciation, loss of subcutaneous fat tissue, hyperactivity, euphoria and hyperalertness. Usually the syndrome is accompanied by space-occupying neoplastic lesions in hypothalamic-optic chiasmatic region. Tumor size is inversely correlated with weight gain.In this case series, we aimed to present three infants with hypothalamic-optic chiasmatic gliomas treated with chemotherapy in our clinic. All three patients had normal linear growth but excessive weight loss. Diencephalic syndrome is further discussed and it is pointed out that excessive weight loss might be the rare but important outcome of this syndrome
References
- Russell A. A diencephalic syndrome of emaciation in infancy and childhood. Arch Dis Child 1951;26:274.
- Shuper A, Bloch I, Kornreich L, Horev G, Michowitz S, Zaizov R, et al. Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence. Pediatr Hematol Oncol 1996;13:443-9.
- Huber J, Sovinz P, Lackner H, Mokry M, Eder H, Urban C. Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive. Klin Pediatr 2007;219:91-4.
- Waga S, Shimizu T, Sakakura M. Diencephalic syndrome of emaciation (Russell syndrome). Surg Neurol 1982;17:141-6.
- Pimstone BL, Sobel J, Meyer E, Eale D. Secretion of growth hormone in the diencephalic syndrome of childhood. J Pediatr 1970;76:886-9.
- Gianotti L, Pincelli AI, Scacchi M, Rolla M, Bellitti D, Arvat E, et al. Effects of recombinant human insulin-like growth factor I administration on spontaneous and growth hormone-releasing hormone-stimulated growth hormone secretion in anorexia nervosa. J Clin Endocrinol Metabol 2000;85:2805-9.
- 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;115:742-8.
- Drop SL, Guyda HJ, Colle E. Inappropriate growth hormone release in diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up. Clin Endocrinol 1980;13:181-7.
- Nagaya N, Kojima M, Uematsu M, Yamagishi M, Hosoda H, Oya H et al. Hemodynamic and hormonal effects of human ghrelin in healthy volunteers. Am J Physiol Regul Integr Comp Physiol. 2001;280:1483-7.
- Brauner R, Trivin C, Zerah M, Souberbielle JC, Doz F, Kalifa C et al. Diencephalic syndrome due to hypotalamic tumor: a model of the relationship between weight and puberty onset. J Clin Endocrinol Metab. 2006;91:2467-73.
Abstract
Diensefalik sendrom, daha çok erken çocukluk döneminde görülen, yaygın kilo kaybı ve cilt altı yağ dokusu kaybı, aşırı hareketlilik, öfori ve aşırı uyanıklık durumu ile karakterize bir sendromdur. Genellikle hipotalamik-optik kiazmatik bölgenin yer kaplayıcı neoplastik oluşumlarına eşlik eder. Kitle boyutu ile kilo alımı arasında ters orantı mevcuttur. Bu olgu sunumunda kliniğimizde hipotalamik-optik kiazmatik gliom tanısı ile tedavi alan ve boy uzaması devam ettiği halde aşırı kilo kaybı olan ve tartıları -2 standart sapmanın altında izlenen 1 yaş altı 3 olgu, endokrinolojik incelemeleri ile birlikte literatür bilgileri ışığında değerlendirilerek sunulmuştur. Kilo alımında duraklamanın ender de olsa önemli nedenlerinden birinin diensefalik sendrom olabileceğine dikkat çekilmiştir
References
- Russell A. A diencephalic syndrome of emaciation in infancy and childhood. Arch Dis Child 1951;26:274.
- Shuper A, Bloch I, Kornreich L, Horev G, Michowitz S, Zaizov R, et al. Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence. Pediatr Hematol Oncol 1996;13:443-9.
- Huber J, Sovinz P, Lackner H, Mokry M, Eder H, Urban C. Diencephalic syndrome: a frequently delayed diagnosis in failure to thrive. Klin Pediatr 2007;219:91-4.
- Waga S, Shimizu T, Sakakura M. Diencephalic syndrome of emaciation (Russell syndrome). Surg Neurol 1982;17:141-6.
- Pimstone BL, Sobel J, Meyer E, Eale D. Secretion of growth hormone in the diencephalic syndrome of childhood. J Pediatr 1970;76:886-9.
- Gianotti L, Pincelli AI, Scacchi M, Rolla M, Bellitti D, Arvat E, et al. Effects of recombinant human insulin-like growth factor I administration on spontaneous and growth hormone-releasing hormone-stimulated growth hormone secretion in anorexia nervosa. J Clin Endocrinol Metabol 2000;85:2805-9.
- 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;115:742-8.
- Drop SL, Guyda HJ, Colle E. Inappropriate growth hormone release in diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up. Clin Endocrinol 1980;13:181-7.
- Nagaya N, Kojima M, Uematsu M, Yamagishi M, Hosoda H, Oya H et al. Hemodynamic and hormonal effects of human ghrelin in healthy volunteers. Am J Physiol Regul Integr Comp Physiol. 2001;280:1483-7.
- Brauner R, Trivin C, Zerah M, Souberbielle JC, Doz F, Kalifa C et al. Diencephalic syndrome due to hypotalamic tumor: a model of the relationship between weight and puberty onset. J Clin Endocrinol Metab. 2006;91:2467-73.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | March 1, 2017 |
| Published in Issue | Year 2017Issue: 1 |