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Objectives: Available evidence implies that unfavorable changes in the distribution of adipose tissue resulting from hormonal imbalance associated with ovarian insufficiency might influence bone mineral density (BMD). The purpose of our study was to verify if volumes of visceral (VAT), female (FAT) and android (AAT) body fat as determined by densitometry determined influence BMD in women with functional menstrual disorders, and if these correlates some endocrine factors. Material and methods: We examined 293 women (mean age 26.7 ± 4.4 years) who have had psychogenic type of functional hypothalamic secondary amenorrhea for at least three months (mean 5.82 ± 0.94). A variety of hormonal tests, determination of BMD and both distribution and volume of adipose tissue were performed. Results: Volume of adipose tissue in all analyzed body regions indicated a positive correlation with BMD in lumbar spine (VAT: R = 0.277, FAT: R = 0.345, AAT: R = 0.336) and entire skeleton (VAT: R = 0.453, FAT: R = 0.527, AAT: R = 0.529). BMD in both the lumbar spine and entire skeleton had positive correlation with body mass index (R = 0.380 and R = 0.599, respectively) and free androgen index values (R = 0.150 and R = 0.279). It showed a negative correlation with sex hormone-binding globulin (R = –0.191 and R = –0.326). We did not find a parameter that could be an independent predictor of BMD. Conclusions: Distribution of body fat is only one of numerous determinants of BMD in women with functional menstrual disorders and should not be treated as the only predictor for bone mass deficiency. Determination of adipose tissue distribution in these patients has probably minor clinical impact.

A mature teratoma is a germinal neoplasm that differentiates from embryonic multipotent cells into three germ layers. There may also be glandular tissue. The literature describes a total of 658 cases of ovarian neuroendocrine neoplasms, mainly in women over 40 years of age. The authors, together with a systemic review, present a case of a 16-year-old girl diagnosed with and treated for a neuroendocrine tumor. Case description: A 16-year-old girl visited the Paediatric Gynaecology Outpatient Clinic because of abdominal pains that intensified during menstruation. Standard painkillers and diastolic drugs were ineffective. An ultrasound examination revealed a large tumor with a heterogeneous structure in her right ovary. A sparing operation was carried out. During laparotomy, the lesion was enucleated, leaving healthy tissue. Histopathological examination revealed the typical features of teratoma, as well as the coexistence of a G1 neuroendocrine tumor. Immunohistochemical examination (IHC) showed the presence of markers characteristic for this type of tumor. The patient requires constant monitoring in the Endocrinology and Oncological Gynaecology Clinic. Conclusion: Tissue of neuroendocrine neoplasm within a teratoma is rare in this age group of patients; thus, there are currently no standards for long-term follow-up. This case adds to the body of evidence and demonstrates a possible good prognosis with non-aggressive behavior in G1 neuroendocrine tumors and teratomas in young patients.

Simpson-Golabi-Behmel syndrome (SGBS) is a rare genetic condition and is inherited in an X-linked recessive manner. The disease is caused by a change in the nucleotide sequence of an X-linked gene encoding glypican 3, a protein belonging to the heparan-sulfate membrane proteoglycan family. SGBS case studies are almost entirely restricted to the pediatric population. Scarce literature describing SGBS course in adults may be due to both the high mortality of SGBS patients in childhood and low rate of SGBS diagnosis in adults. We present a case of a 39-year-old man with an initial diagnosis of acromegaly. Genetic tests revealed a hitherto unreported deletion in the GPC3 gene. SGBS manifestations in our patient included tall stature, dysmorphic features, and central nervous system (CNS) anatomical pathology. MRI of the head visualized abnormalities of median line structures, a feature consistent with SGBS: an unclosed craniopharyngeal canal, a sellar-suprasellar cyst, dysmorphic pituitary gland, and a cyst of the septum pellucidum. Moreover, cardiomyopathy complicated by life-threatening paroxysmal ventricular tachycardia was diagnosed. Although various cardiac anomalies are often found in SGBS, their pathogenesis is unclear and may be multifactorial. We believe that the presented case contributes to a better understanding of SGBS and may help clinicians in introducing prophylaxis and treatment for its comorbidities.

Zulassung: Die Kinder- und Jugendgynakologie befasst sich mit angeborenen und entwicklungsbedingten gynakologischen Storungen im Kindes- und Jugendalter. Eine der wichtigsten-spezifischen Fragen der Gynakologie des Entwicklungsalters sind die psychologische-etische Probleme, sehr oft Probleme, die mit einer vorzeitigen sexuellen Initiation zusammenhangen. Zwischen mehreren gynekologischen somatischen Problemen der jugend Patientinen sind auch psychologische-etische Probleme und Probleme, die mit einer vorzeitigen sexuellen Initiation zusammenhangen. Zielsatzung : Prasentation der Bedeutung der psychologischen-etischen und sexuologischen Probleme in der Gynakologie der Pubertat. Methode: Analyse der Literatur der Problematik der Pubertatsgynakologie in Bezug auf Sexuologie und Psychologie. Schlussfolgerung: Die kurze Uberprufung der gewahlten sexuologischen Themen in Bezug auf die Kinder- und Jugendgynakologie weist auf die Bedeutung der sexuologischen Ausbildung, der Gesundheitspromotion, inklusiv sexuelles Wohlstandes hin.

The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions. The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe-sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke's cleft cyst, which became possible with the use of ¹¹C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke's cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess. The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig-ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.

Introduction: The most common clinical and neurological signs and symptoms of pituitary macroadenomas include headache, vision impairment and cranial nerve palsy. Case report: The patient presented in this article was admitted to the Intensive Care Unit at regional hospital; at admission, the patient was unconscious, he had convulsions and spasms, and a 3­‍-day history of headache and body temperature up to 41.5°C. The patient with suspected neuroinfection was transferred to the Department of Infectious Diseases of the Pomeranian Medical University in Szczecin (PMU), where cerebrospinal meningitis of bacterial etiology was established based on cerebrospinal fluid investigations and the presence of pituitary abscess was suggested based on magnetic resonance imaging (MRI). Magnetic resonance imaging findings included an extensive pathological lesion with the diameter of 27 × 28 × 38 mm located in the sellar­‍-suprasellar region, with intensive peripheral contrast enhancement. The lesion protrudes into the sphenoid sinus through the lowered bottom of sella turcica and the fluid content has also been visualized in the sphenoid sinus. After 10­‍-day antibiotic therapy, the patient was transferred to neurosurgery ward for surgical treatment. The pathological lesion was partially evacuated during right frontotemporal craniotomy. The patient’s general condition after the surgery was moderately severe; the patient was conscious, able to follow simple commands, presenting hemiparesis of the left side of the body, particularly affecting left lower limb and with speech disturbances. The signs of hypopituitarism affecting all hormonal axes were also observed and the patient was transferred to the Department of Endocrinology of the PMU for further treatment. Follow­‍-up MRI scan continued to show the presence of pathological mass in the sellar­‍-suprasellar region, which penetrated into the sphenoid sinus through damaged sellar bottom. After correction of reduced hormone levels and several weeks of antibiotic therapy, the patient was transferred to the Department of Neurosurgery of the PMU for further surgical treatment. Transsphenoidal resection of the sellar­‍-suprasellar tumor and sphenoid sinus reconstruction were performed. Histopathology report confirmed the diagnosis of pituitary adenoma. The patient in relatively good condition, with partial hemiparesis on the left side of the body, able to stand with support, not able to walk, with speech disturbances and able to follow commands was transferred to the rehabilitation center. One year later, follow­‍-up MRI scan showed deepened sella turcica, filled with a mass corresponding to postoperative material. No evidence of disease progression has been found. Conclusion: Neuroinfection may be the first manifestation of pituitary macroadenoma.

Functional hypothalamic amenorrhoea (FHA) is associated with functional inhibition of the hypothalamic-pituitary-ovarian axis. Causes of FHA can be classified into the three groups: 1) stress-related factors, 2) consequences of weight loss and/or underweight, and 3) consequences of physical exercise or practicing sports. Diagnosis of FHA should be based on a history of menstrual disorders. During physical examination, patients with FHA present with secondary and tertiary sex characteristics specific for the pubertal stage preceding development of the condition and with the signs of hypoestrogenism. Laboratory results determine further management of patients with amenorrhea, and thus their correct interpretation is vital for making appropriate therapeutic decisions. Treatment of chronic anovulation, menstrual disorders, and secondary amenorrhea resulting from hypothalamic disorders should be aimed at the elimination of the primary cause, i.e. a decrease in psycho-emotional strain, avoidance of chronic stressors, reduction of physical exercise level, or optimisation of BMI in patients who lose weight. If menses do not resume after a period of six months or primary causative treatment is not possible, neutralisation of hypoestrogenism consequences, especially unfavourable effects on bone metabolism, become the main issue. Previous studies have shown that oestroprogestagen therapy is useful in both the treatment of menstrual disorders and normalisation of bone mineral density. Hormonal preparations should be introduced into therapeutic protocol on an individualised basis. (Endokrynol Pol 2015; 66 (3): 252–268)

Introduction: Pituitary gland adenomas producing prolactin are one of the commonest hormonally active tumours. Pharmacological treatment using of dopamine receptors agonists is the therapy of choice in a case of prolactinoma. Bromocriptine, which causes numerous side‑effects is the most commonly used drug. Recently, good results of therapy have been achieved with cabergoline – a selective dopamine receptor agonist with prolonged time of action. The aim of the study was to evaluate therapy with cabergoline of men with macroprolactinoma based on clinical, hormonal and radiological examinations. Material and methods: Ten men aged 18–65 (mean 41.9 ±15.01 years) with the presence of a pathological mass in the pituitary gland sized between 16.7 and 40.5 mm (mean 29.8 ±9.38 mm) and an elevated prolactin (PRL) level of between 37.3 and 4700 ng/mL (mean 1608.2 ±1771.6 ng/mL) were included in the study. The PRL and other trophic hormones levels were evaluated after 1, 3, 6 and 12 months, and tumour size was evaluated by magnetic resonance imaging examination after 12 months of therapy with cabergoline. Results: Therapy with cabergoline led to remission of headaches, visual acuity correction, and a significant improvement in libido and erection in all patients. In 90% of patients, PRL normalisation was achieved, just the initial months of therapy. The mean PRL serum concentrations were before, and after 1, 3, 6 and 12 months of therapy respectively, 1608.2 ±1771.6 ng/mL and 263.4 ±223.4, 136.1 ±244.7, 91.31 ±105.5 and 27.5 ±57.7 ng/mL. A significant tumour size reduction was observed: from 29.8 ±9.4 mm to 23.2 ±9.4 mm, a mean reduction of about 6 mm, or 25.1% (from 4–48.5%). No significant correlation between the mean tumour size and PRL level was observed before or during the treatment. A decreased testosterone level before the therapy was proven, and its gradual increase during the treatment was observed, but after 12 months no normal mean testosterone concentration was achieved. Conclusions: 1. The administration of cabergoline to patients with macroprolactinoma is effective in reaching PRL level normalisation as well as in tumour size reduction. 2. Therapy with cabergoline significantly decreases the clinical symptoms of hyperprolactinemia and neurological and ophtalmological changes associated with the presence of a pathological lesion in the pituitary gland. 3. Tumour size is not a predictive factor for the effectiveness of therapy with cabergoline.

Introduction: Childhood obesity has been associated with the development of insulin resistance, potentially leading to several metabolic disorders. Osteocalcin has been reported to contribute to the regulation of glucose tolerance and insulin sensitivity. The purpose of this study was to examine the relationship between serum osteocalcin and metabolic risk factors in obese children and adolescents. Material and methods: Age, gender, pubertal stage, adiposity markers (standard deviation score of body mass index: BMI-SDS, percentage of body fat, waist circumference), blood pressure, serum osteocalcin (OC), fasting plasma glucose and insulin, glycated haemoglobin level (HbA1c), insulin resistance estimated by homeostasis model assessment (HOMA-IR), lipid profile, C-reactive protein (CRP), fibrinogen (FB), white blood cell count (WBC) and 25-hydroxyvitamin D (25-OH-D) were evaluated in 142 obese children and adolescents. Stepwise multiple regression analysis was used to determine the relationship between serum osteocalcin and metabolic risk parameters. Results: Mean serum osteocalcin level was 72.0 ± 20.5 μg/L (range: 16.8–181.5 µg/L). After adjustment for multiple potential confounders, serum osteocalcin concentration was inversely associated with adiposity markers as well as HOMA-IR, HbA1c, triglycerides, CRP, FB and positively with 25-OH-D and HDL-cholesterol. In stepwise multiple linear regression analysis adjusted for age, gender and pubertal stage, osteocalcin was significantly negatively related to HOMA-IR, triglycerides and waist circumference. Conclusions: Serum osteocalcin concentration is associated with blood markers of dysmetabolic phenotype and measures of adiposity, suggesting that osteocalcin is important not only for bones but also for glucose and fat metabolism as early as during childhood. (Endokrynol Pol 2013; 64 (5): 346–352)

Introduction The aim of this study was the long-term prospective evaluation of the effects of estroprogestagen (EP) therapy on the bone mineral density (BMD) of girls with functional hypothalamic amenorrhea (FHA) carrying various PvuII and XbaI polymorphisms of ER-α. Material and methods Prospective observation included 84 FHA girls and 50 controls. The FHA patients were subjected to 4-year sequential therapy with 17β estradiol (2 mg from the 2nd to 25th day of the menstrual cycle) and dydrogesterone (10 mg from the 16th to the 25th day). Hormonal parameters, serum concentration of the bone fraction of alkaline phosphatase (BALP), urine concentration of cross-linked n-telopeptide of type I collagen (Ntx) and BMD were determined before and after the treatment. Results Six-month treatment resulted in a marked increase in estradiol (p = 0.001), testosterone and prolactin levels (p = 0.01 both) and a significant decrease in BALP and Ntx (p = 0.001 both). Patients with the PP polymorphism had significantly lower baseline BMD compared to carriers of other polymorphic variants of PvuII (p = 0.003). A significant increase in BMD was observed throughout the entire therapy period, with no significant differences in the yearly dynamics of BMD changes observed amongst various polymorphic variants and haplotypes of ER-α. Conclusions The EP therapy is effective in the treatment of BMD disorders associated with FHA, and treatment results do not depend on PvuII and XbaI polymorphisms of ER-α.

Reduced bone mineral density (BMD) is present in many women with Turner syndrome (TS), and hypo-estrogenism is known to play a vital role in bone mineralization disturbances. It has been suggested that genetic factors play an important role in the regulation of BMD. The aim of this study was to analyze the association between Pvu II and XbaI ER-α polymorphisms and BMD in TS patients subjected to estroprogestagen (EP) treatment. Thirty-two TS patients aged 17–38 (mean age 22.7 ± 8.2) along with 82 healthy controls were the subjects for this study. Baseline values of hormonal parameters, BMD and bone density markers were measured in the subjects. Subsequently, TS patients underwent 4 years of EP therapy. The results of laboratory parameters and BMD were analyzed in regard to PvuII and XbaI polymorphic variants of the ER-α gene. The increase in BMD of TS subjects was the highest in the 1st (7.5%, p = 0.013) and 2nd (6.6%, p = 0.008) years of treatment. Four years of EP therapy was reflected by a significant increase in BMD z-scores in patients with xx and Xx genotypes of the XbaI gene and in those with with the pp and Pp genotypes of PvuII. In patients with haplotypes other than XXPP, BMD z-scores were significantly higher compared to their baseline after 2 (p = 0.002), 3 (p

Childhood and adolescent gynecology is an emerging specialty at the intersection of pediatrics, pediatric endocrinology gynecology pediatric surgery dermatology psychiatry, public health medicine and genetics, and in fact addresses many legal issues. Poland lacks a uniform standing of medical and legal environments on how to deal with a juvenile patient who has become sexually active and seeks the advice of a gynecologist, gynecologic examination and requests to be prescribed contraceptives. It needs to be taken into account that in Poland a parent or a legal guardian has legal guardianship, custody and control of a child until 18 years of age but once a juvenile reaches the chronological age of 16 years, and is given full rights of a patient, both parties need to consent to medical care. According to the Act on Health Care Institutions, a patient has the right to self-determination, respect for physical and mental integrity as well as privacy whereas, after the patient reaches the age of 16 years, the legal representative becomes in practice a mere co-decision maker to have medical services performed. Therefore, information obtained from a juvenile patient during physical test and medical interview does not have to be revealed to a legal representative, if the patient requests confidentiality and on condition it does not affect patient health and/or the planned medical procedures (e.g. the need to perform an operation). Knowledge about procedures for juvenile patients shall enable doctors to make conscious choices about conduct and care or in most cases, only advice, without the risk of breaching the Polish law.

The aim of this study was to evaluate the effects of 4-year estroprogestagen therapy (EP) on the bone mineral density (BMD) of 16- to 17-year-old girls with functional hypothalamic amenorrhea (FHA, n = 78). Baseline values of hormonal parameters, bone fraction of alkaline phosphatase (BALP), and cross-linked n-telopeptide of type I collagen (Ntx) were taken along with BMD measurements. Follow-up measurements of laboratory parameters were performed after 6 months of EP treatment. BMD was measured on a yearly basis. Six-month treatment resulted in a marked increase in estradiol levels and a significant decrease in BALP and Ntx. The relative increase in BMD was highest after the second year of treatment. Based on the dynamics of BMD changes during the first year of treatment, we identified a subgroup with no or insignificant reactions to the treatment. It was characterized by significantly higher baseline BMD and markedly lower baseline Ntx compared to the patients who responded to 1-year therapy well or extremely well. Further follow-up proved, however, that this subgroup did not differ significantly in terms of the long-term prognosis for BMD normalization. In conclusion, this study showed that EP therapy is effective in the treatment of BMD disorders associated with FHA.

The article presents a case of 57-year-old woman with the infiltration of rare small lymphocytic B cell lymphoma in the thyroid gland. Initially, the patient was followed-up due to chronic lymphocytic B-cell leukemia diagnosed on the basis of histopathological examination of cervical lymph node. Eight months later, general symptoms occurred along with lymphocytosis and exacerbation of lesions in lymph nodes, and therefore, chemotherapy was started according to COP regimen. After four chemotherapy cycles, further progression of the disease was observed during chemotherapy. Computed tomography (CT) performed at that time showed generalized lymphadenopathy and the presence of an irregular area in left thyroid lobe. On palpation, the thyroid was asymmetrical, with enlarged left lobe and palpable lymph node packages on the left side of the neck. The levels of thyroid hormones and anti-thyroid antibodies were normal. Ultrasound examination of the thyroid gland showed non-homogeneous hypoechogenic structure of the left lobe and complete focal remodeling. Cytological examination of left-lobe lesion obtained during fine needle aspiration biopsy showed multiple small lymphoid cells, suggestive of small lymphocytic lymphoma. To confirm this diagnosis, flow cytometry of the biopsy material sampled from the left lobe was performed showing B cellimmunophenotype: CD19+/CD20+/CD22 dim/FMC-7, CD23+/CD5+, sCD79b-+, CD38-, CD10-, kappa and lambda-/weak reaction. The results of flow cytometry of the thyroid bioptate and blood were nearly identical, confirming leukemic nature of the infiltration in left thyroid lobe. Cytogenetic findings included the presence of 17p deletion (TP53 gene). The patient received immunochemotherapy with alemtuzumab. The progression of the disease occurred in the sixth week of therapy. The treatment was discontinued after 8 weeks due to worsening of patient’s general status. The patient died 15 months after the diagnosis.