Sotelo-Cruz N, López-Cervantes G, González-Ramos LA, Ibarra-Silva R, Hurtado-Valenzuela JG, Fernández-Gámez A

Language: Spanish
References: 17
Page: 265-272
PDF size: 276.48 Kb.

ABSTRACT

Introduction. Cushing syndrome is associated with high levels of glucocorticoids in the circulation and is infrequently in infants. Case report. We present a case of congenital cortical adrenal adenoma-associated endogenous hypercortisolism in a 3-month-old infant. The patient manifested polyphagia, weight gain, and changes in sleep patterns. During physical examination we found a full-moon face, bulkiness in the cervico-dorsal “buffalo-hump” region, high blood pressure, and serum cortisol of 163 µg/dL. Abdominal ultrasound revealed left adrenal tumor. During hospitalization, the patient experienced respiratory difficulty and tachycardia, and thoracic X- ray revealed cardiomegaly. After a simple surgical procedure (venous dissection), the patient developed cardiogenic shock and died. At autopsy, adrenal tumor was found in addition to hypertrophic cardiomyopathy and intrapancreatic accessory spleen. Conclusion. The most common cause of Cushing syndrome in all age groups is exogenous hypercortisolism, except in infants. The associated pathologies described in this article are rare.

REFERENCES

1. Diez JJ, Rodríguez-Aranao M, Gómez-Pan A. Hiperfunción suprarrenal: síndrome de Cushing e hiperaldosteronismo primario. En: Pombo M, editor. Tratado de endocrinología pediátrica. 3ª ed. España, SAU: McGraw-Hill Interamericana; 2002. p. 1006-17.

2. Orth DN. Cushing’s syndrome. N Engl J Med. 1995; 332: 791-803.

3. Lafferty AR. Pituitary tumors in children and adolescents. J Clin Endocrinol Metab. 1999; 4317-23.

4. Chorousos GP. Cushing’s syndrome. En: Rosenfel RL, editor. A current review of pediatric endocrinology. San Diego, CA, USA: Serono Symposia Inc.; 1995. p. 123-38.

5. Trainer PJ, Grossman A. The diagnosis and differential diagnosis of Cushing syndrome. Clin Endocrinol. 1991; 34: 317-30.

6. Farol L, Masson P. Mollet V, Simeoni V. Cushing’s syndrome in pregnancy and neonatal hypertrophic obstructive cardiomiopathy. Acta Paediatr. 2004; 93: 1400-2.

7. Muesan ML, Lupia M, Salvetti M, Girigoletto C, Sonino N, Boscaro M, et al. Left ventricular structural and functional characteristics in Cushing’s syndrome. Am Coll Cardiol. 2003; 41: 2275-9.

8. Zevitz EM, Sander EG. Cardiomyopathy hypertophic. (serial on internet) 2006 jul, section 1-11. Available from: http//www.emedicine.com/med/topic 290 htm 126 k.

9. Sandrini R, Ribeiro RC, de la Cerda L. Childhood adrenocortical tumors. J Clin Endocrinol Metab. 1997; 82: 2027-31.

10. Gayer G, Zissin R, Apter S, Atar E, Portnoy O, Itzchak Y. Pictorial review. CT findings in congenital anomalies of the spleen. BJR. 2001; 74: 767-72.

11. Kanazowa H, Kamya J, Nagino M, Vesaka KI, Yuasa N, Odak A, et al. Epidermoid cyst in on intrapancreatic accessory spleen: A case report. J Hepatobiliary Pancreat Surg. 2004; 11: 61-3.

12. Michalkiewicz E, Sandrini R, Figuereido B, Miranda ECM, Caran E, Oliveira-Filho A, et al. Clinical and outcome characteristics of children with adrenocorticol tumors: A report from the international pediatric adrenocortical tumor registry. J Clin Oncol. 2004; 22: 838-45.

13. Forga L, Anda E, Martínez-de Esteban JP. Síndromes paraneoplásicos. An Sist Sanit Navar. 2005; 28: 213-26.

14. Diez JJ, Gómez-Pan A. Diagnóstico del síndrome de Cushing. En: Novaendo, editor. Barcelon: Ed. EDIKA Med.; 2002. p. 1-10.

15. Tzanela M, Zianni D, Stylianidou Ch, Karavitaki N, Tsagarakis S, Thalassinos C. Evaluation of GH reserve in patients with adrenal incidentalomas and biochemical evidence of subclinical autonomous glucocorticoid hypersecretion. Clin Endocrinol. 2005: 62: 597-602.

16. Lindholm J, Juul S, Jorgensen JO, Astrup J, Bjerre P, Feldt-Rasmussen V, et al. Incidence and late prognosis of Cushing’s syndrome: A population based study. J Clin Endocrinol Metab. 2001; 86: 117-24.

17. Fink AM, Kulkarni S, Crowley P, Crameri JA. Epidermoid cyst in a pancreatic accessory spleen mimicking and infected abdominal cyst in a child. AJR Am J Roentgenol. 2002; 79: 206-8.