Browsing by Author "Vorgučin, Ivana (24924004500)"

Introduction. Langerhans Cell Histiocytosis (LCH) is a rare chronic granulomatous, usually multisystem disease of elusive etiology, with peak incidence in early childhood and slow progressing course. Isolated brain histiocytosis is a very rare condition and neurological finding does not correlate with the extent of space-occupying anatomical lesions and degenerative changes. Case report. A girl, age 2.5 years was presented with diabetes insipidus and nearly fatal full spectrum isolated brain histiocytosis. Brain magnetic resonance imaging (MRI) showed multiple nodules with perifocal edema, the most prominent in the projection of the hypothalamus/pituitary and the stalk and in the region of the pineal gland. Identical nodules were present in both caudate nucleus and putamen, left insular subcortex, both temporal lobes, tegmental area of the midbrain, central part of pons and medulla, both cerebellar hemispheres and leptomeningeal membranes. The pattern resembled snow balls and flakes. Biopsy showed positivity for vimentin, S-100, CD-68 and CD1a markers. Treatment protocol LCH-III was not successful and a salvage treatment was refused by parents. She appeared again at the age of 7 with growth deceleration and fully developed precocious puberty. The control MRI of the brain revealed similar nodules in certain regression. Due to central precocious puberty, treatment with luteinizing hormone–releasing hormone (LH-RH) analogue was introduced. School performance was mediocre with cocktail-party effect behavior and slower speech. Conclusion. Brain histiocytosis is potentially fatal disease with chronic, variable, slowly progressive course and unpredictable responses to treatment protocols. © 2020 Inst. Sci. inf., Univ. Defence in Belgrade. All rights reserved.

Introduction Primary hyperparathyroidism is rare pathology in children (2–5:100,000). In more than 85% of patients, a single adenoma is present, and its extirpation is usually the only treatment a patient requires. In approximately 15–80% of cases, ectopic mediastinal parathyroid tissue can be found inside the thymus. Case outline Our patient was a 13-year-old boy, who presented with multiple bone fractures in the previous period of time, and fatigue. Parathyroid hormone levels preoperatively were extremely high (1320 pg/ml – more than 19 times higher than normal). Serum calcium was also elevated (total 3.55 mmol/l; ionized 1.41 mmol/l). He was examined and diagnosed as primary hyperparathyroidism by a pediatric endocri-nologist. Imaging procedures for the preoperative localization of parathyroid adenomas were done (99mTc sestamibi scintigraphy and magnetic resonance imaging suggested ectopic mediastinal parathyroid adenoma). The patient underwent video-assisted thoracoscopic surgery procedure. After exploration of the mediastinum and chest, no ectopic parathyroid tissue was found, so total thoracoscopic thymectomy was performed. Final pathological section confirmed parathyroid adenoma inside the thymus. Conclusion We believe that if no parathyroid tissue is found during surgical exploration of mediastinum, in a child with preoperatively detected parathyroid adenoma in anterior mediastinum, recommendation is to think about possible intrathymic localization and consider removing the thymus. Greater sample size is necessary for higher reliability of this statement. © 2021, Serbia Medical Society. All rights reserved.