ESPE2022 Poster Category 2 Adrenals and HPA Axis (27 abstracts)
The first case of pediatric Cushing disease in Serbia
Sanja Panic Zaric 1 , Rade Vukovic 1,2 , Tatjana Milenkovic 1 , Katarina Mitrovic 1,2 , Sladjana Todorovic 1 , Snezana Ristic 1 , Natasa Stajic 1,2 , Jovana Putnik 1,2 , Aleksandra Paripovic 1,2 , Vladislav Vukomanovic 1,2 , Sanja Ninic 1 , Sergej Prijic 1,2 , Sasa Popovic 1 , Ivana Cerovic 1 , Ivana Kitic 1,2 , Srdjan Pasic 1,2 , Predrag Minic 1,2 , Milan Rodic 1 , Milos Kuzmanovic 1,2 , Milena Jovic 1 , Maja Djordjevic Milosevic 1,2 , Sandra Pekic Djurdjevic 3,2 , Milan Petakov 3,2 , Mihajo Milicevic 4,2 & Milan Jovanovic 5,2
1Mother and Child Health Care Institute of Serbia "Dr Vukan Cupic", Belgrade, Serbia; 2School of Medicine, University of Belgrade, Belgrade, Serbia; 3Department of Endocrinology, Diabetes and Metabolic Diseases, University Clinical Centre of Serbia, Belgrade, Serbia; 4Clinic for Neurosurgery, University Clinical Centre of Serbia, Belgrade, Serbia; 5Center for Endocrine Surgery, University Clinical Centre of Serbia, Belgrade, Serbia
Background: The definition of Cushing disease (CD) is hypercortisolism caused by excess adrenocorticotropic hormone (ACTH) secretion by a pituitary corticotrope adenoma. It is extremely rare in the pediatric population and requires rapid diagnosis and early management.
Case presentation: A 14-year old boy was hospitalized because of sudden onset of chest pain, loss of consciousness, elevated cardio-specific enzymes, and suspected cardiomyopathy. On admission, he was short stature (-3 SD), obese (BMI 32,9 kg/m2, at +3 SD), with wide and long purple stretch marks on the abdomen and in the axillary region, hypertrichosis of the back, round face, buffalo hump neck, hypertensive (95-99th percentile), tachycardic (HR 109/min) with systolic murmur 3/6, Tanner stage A3, P4, testicles volume 2-3 ml. In the last few years, he has started to gain weight abruptly. His mother noticed that he is shorter than his peers. Easier got bruises and stretch marks on the abdomen have been noticed during the last 6-12 months. Also, the last two years, he withdrew and distanced himself from society, felt depressed, and took antidepressants for a while. In blood analyses, he had metabolic alkalosis, severe hypokalemia, hypophosphatemia, low level of serum immunoglobulins, high blood sugar, and hypercortisolism without circa dial variation. Ultrasound of the heart showed hypertrophic cardiomyopathy with obstruction in LVOT and mild mitral regurgitation. CT and ultrasound of the abdomen displayed hyperplasia of both adrenal glands without pathological masses. We did dexamethasone suppression tests-overnight and prolonged. We didn’t obtain suppression of cortisol. After all, we got the result of elevated ACTH. Pituitary MRI described microadenoma of the right half of the adenohypophysis. We consulted an adult endocrinologist and start metyrapone (11β hydroxylase inhibitor). After 2 days of metyrapone therapy, the boy had clinical deterioration due to sepsis caused by Coagulase negative staphylococcus followed by pulmonary edema. We started therapy immediately in the intensive care unit (antibiotics, diuretics, intravenous immunoglobulins, fraxiparine, aminophylline, oxygen therapy) and after 9 days he got recovered. In a month transsphenoidal surgery of the pituitary gland was performed. The tumor was completely removed. The morphological finding, supplemented by immunohistochemistry, corresponds to a neuroendocrine pituitary tumor of corticotropic adenoma type, densely granulated subtype.
Conclusion: CD is a serious disorder, which could be life-threatening, where pediatric endocrinologists have limited experience. A multidisciplinary approach is a key to success in timely diagnosis and therapy.
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