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1.
Article in English | MEDLINE | ID: mdl-39026475

ABSTRACT

INTRODUCTION: Adrenocortical carcinoma (ACC) is rare and an aggressive tumour. Mitotane is the mainstay adjuvant drug in treating ACC. The study aimed to describe patients diagnosed with precocious puberty (PP) and other endocrinological complications during mitotane therapy. MATERIAL AND METHODS: This retrospective study enrolled 4 patients with ACC treated with mitotane therapy complicated by PP. We analysed clinical manifestations, radiological, histopathological findings, and hormonal results. RESULTS: The median age at the diagnosis of ACC was 1.5 years. All patients were treated with surgery and mitotane, accompanied by chemotherapy regimens in 2 cases. The median time from surgery to the initiation of mitotane therapy was 26 days. During mitotane treatment, PP was confirmed based on symptoms, and hormonal and imaging tests. In one patient, incomplete peripheral PP was followed by central PP. The median time from the therapy initiation to the first manifestations of PP was 4 months. Additionally, due to mitotane-induced adrenal insufficiency, patients required a supraphysiological dose of hydrocortisone (HC), and in one patient, mineralocorticoid (MC) replacement with fludrocortisone was necessary. In 2 patients, hypothyroidism was diagnosed. All patients presented neurological symptoms of varying expression, which were more severe in younger children. CONCLUSIONS: The side effects of using mitotane should be recognized quickly and adequately treated. In prepubertal children, PP could be a complication of therapy. The need to use supraphysiological doses of HC, sometimes with MC, should be highlighted. Some patients require levothyroxine replacement therapy. The neurotoxicity of mitotane is a significant clinical problem.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Antineoplastic Agents, Hormonal , Mitotane , Puberty, Precocious , Humans , Puberty, Precocious/drug therapy , Puberty, Precocious/chemically induced , Mitotane/therapeutic use , Mitotane/adverse effects , Female , Adrenocortical Carcinoma/drug therapy , Adrenal Cortex Neoplasms/drug therapy , Retrospective Studies , Male , Child, Preschool , Antineoplastic Agents, Hormonal/therapeutic use , Antineoplastic Agents, Hormonal/adverse effects , Infant , Child , Endocrine System Diseases/chemically induced
2.
Article in English | MEDLINE | ID: mdl-32651988

ABSTRACT

Objectives The main cause of hyperandrogenism in children is congenital adrenal hyperplasia, adrenal and gonadal tumors, polycystic ovary syndrome (PCOs) and Cushing's disease. In the last 20 years several descriptions of girls with hyperandrogenism and venous porto-systemic shunts appeared in literature. Case presentation First case is an eleven and a half-year-old girl, was admitted to Department of Endocrinology because of symptoms of hyperandrogenism. Laboratory tests revealed high serum testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEAS). The ammonia concentration was also increased. In the abdominal angio-CT scans persistent umbilical vein which connected portal and femoral vein was found. The second case was a seven-year-old boy with symptoms of precocious puberty. Blood tests also revealed high concentration of testosterone, androstenedione, DHEAS and ammonia. Imaging studies showed persistent ductus venosus. Conclusion Although pathophysiological relation is not clear, porto-systemic shunts should be considered as a cause of hyperandrogenism of unknown origin in children.

3.
Clin Case Rep ; 6(3): 484-489, 2018 03.
Article in English | MEDLINE | ID: mdl-29531723

ABSTRACT

Virginal breast hypertrophy is a multidisciplinary condition including surgical, pediatric, and endocrine/gynecological disciplines, and its successful diagnosis and management requires complex, team approach.

4.
J Clin Endocrinol Metab ; 95(7): 3133-40, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20444919

ABSTRACT

CONTEXT: Nijmegen breakage syndrome (NBS) is a severe chromosomal instability disorder characterized by microcephaly, growth retardation, immune deficiency, and predisposition for malignancy. It is caused by hypomorphic mutations in the NBN gene, which product belongs to the protein complex critical for processing DNA double-strand breaks during mitotic and meiotic recombination. Data on gonadal function in patients with NBS are limited. OBJECTIVE: Growth and sexual development, along with hormonal assays, were evaluated in girls and young women with NBS homozygous for c.657_661del5 mutation. STUDY DESIGN AND PATIENTS: The group comprised 37 girls and young women with NBS (ages, 0.17-24.25 yr), followed between 1993 and 2008. Patients were divided into three age groups: 1) 1-3 yr; 2) 4-9 yr; and 3) 10 yr and older. Growth, puberty, concentrations of gonadotropins and 17-beta-estradiol, bone age, and pelvic ultrasound were assessed. RESULTS: None of the patients presented a typical growth spurt; the adult height ranged between the 3rd and 25th centiles. Median bone age was delayed by 4.05 yr. Pubarche reached stadium P2 in eight patients and P3 in two patients. In all but one girl, thelarche did not exceed Th2, with low 17beta-estradiol levels. Gonadotropin levels showed a biphasic pattern, with median FSH values of 55.0, 10.9, and 81.9 IU/liter, and LH of 3.2, 0.8, and 21.0 IU/liter in consecutive age groups. Ultrasound visualized small ovaries or solid streaks and the hypoplastic uterus. CONCLUSIONS: Primary ovarian insufficiency and the associated hypergonadotropic hypogonadism are hallmark manifestations in girls and young women with NBS. Our findings emphasize the need for long-term endocrinological and interdisciplinary supervision of these patients.


Subject(s)
Hypogonadism/epidemiology , Nijmegen Breakage Syndrome/complications , Primary Ovarian Insufficiency/epidemiology , Puberty, Delayed/epidemiology , Adolescent , Analysis of Variance , Body Height , Child , Child, Preschool , Estradiol/blood , Female , Follicle Stimulating Hormone/blood , Humans , Hypogonadism/blood , Hypogonadism/complications , Infant , Longitudinal Studies , Luteinizing Hormone/blood , Nijmegen Breakage Syndrome/blood , Prevalence , Primary Ovarian Insufficiency/blood , Primary Ovarian Insufficiency/complications , Puberty, Delayed/blood , Puberty, Delayed/complications , Statistics, Nonparametric , Young Adult
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