The electrophysiological tests in the early detection of the visual pathway dysfunction in patients with microadenoma.

PURPOSE: To evaluate the validity of electrophysiological tests in the early diagnosis of a ganglion cells and/or optic nerve dysfunction in patients with pituitary microadenoma. METHODS: 66 eyes, from 33 patients with microadenoma with no evidence of the optic chiasm compression in magnetic resonance imaging (MRI) and the visual impairment in the routine ophthalmological examination, standard static perimetry (24-2 white on white) and optical coherence tomography (HD-OCT), were analysed. The pattern electroretinogram (PERG), standard pattern visual evoked potentials (PVEPs) and multichannel visual evoked potentials (mVEPs) (ISCEV standards) were performed. The results obtained from the electrophysiological tests were compared to the same number of age-matched healthy controls. RESULTS: Statistically significant differences between the patients with microadenoma and healthy controls were detected in all electrophysiological tests (p < 0.001). The most frequent abnormalities were observed in mVEPs (25/33 patients, 75.8%; 43/66 eyes, 65.2%). The most frequent features registered in this test were: (1°4')-an increase in the P100wave latency from uncrossed fibres (13/33 patients, 39.39%; 21/66 eyes, 31.8%) and (0°16')-an amplitude reduction of this wave from the crossed fibres (11/33 patients, 33.33%; 19/66 eyes, 28.8%). The changes in PVEPs (15/33 patients, 45.5%; 25/66 eyes, 37.9%) and PERG (10/33 patients, 30.3%; 15/66 eyes, 22.7%) were also registered. Of all the tests and parameters analysed in the study, the greatest diagnostic value in detecting the visual pathway dysfunction in this group of patients was the amplitude of P100 wave from the crossed fibres of the mVEPs (1°4') with a sensitivity of 60.6% and a specificity of 93.8%. These parameters suggest that this type of dysfunction is downstream to the chiasm and can also indicate the visual pathway dysfunction severity. CONCLUSIONS: In patients with microadenoma, the abnormalities in the electrophysiological tests are registered even without clinical evidence of visual impairment from the routine ophthalmological examination, SAP, OCT and chiasmal compression in MRI. The mVEPs have the most significant role in the diagnosis of the visual pathway dysfunction in patients with microadenoma.

Simpson-Golabi-Behmel syndrome in a 39-year-old male patient with suspected acromegaly-A case study.

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.

[Legal aspects in pediatric and adolescent gynecology]. / Wybrane aspekty prawne w ginekologii wieku rozwojowego.

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.

Assessment of real-world usage of lanreotide AUTOGEL 120 in Polish acromegalic patients - results from the prospective 12-month phase of Lanro-Study.

AIM OF THE STUDY: To assess resource utilization and costs of treatment with lanreotide AUTOGEL 120 mg (ATG120) administered as part of routine acromegaly care in Poland. MATERIAL AND METHODS: A multicentre, non-interventional, observational study on resource utilization in Polish acromegalic patients treated with ATG120 at 4 weeks or extended (> 4 weeks) dosing interval. The study recruited adult acromegalic patients treated medically for ≥ 1 year including at least 3 injections of ATG120. Data on dosing interval, aspects of administration, and resource utilization were collected prospectively during 12 months. Costs were calculated in PLN from the public health-care payer perspective for the year 2013. RESULTS: 139 patients were included in the analysis. Changes in dosing regimen were reported in 14 (9.4%) patients. Combined treatment was used in 11 (8%) patients. Seventy patients (50%) received ATG120 at an extended dosing interval; the mean number of days between injections was 35.56 (SD 8.4). ATG120 was predominantly administered in an out-patient setting (77%), by health-care professionals (94%). Mean time needed for preparation and administration was 4.33 and 1.58 min, respectively, mean product wastage - 0.13 mg. Patients were predominantly treated in an out-patient setting with 7.06 physician visits/patient/year. The most common control examinations were magnetic resonance imaging of brain and brain stem (1.36/patient/year), ultrasound of the neck (1.35/patient/year), GH (1.69/patient/year), glycaemia (1.12/patient/year), IGF-1 (0.84/patient/year), pituitary-thyroid axis hormone levels assessment (TSH-0.58/patient/year, T4-0.78/patient/year). There were 0.43 hospitalizations/patient/year. For direct medical costs estimated at PLN 50 692/patient/year the main item was the costs of ATG120 (PLN 4103.87/patient/month; 97%). The mean medical cost, excluding pharmacotherapy, was PLN 1445/patient/year (out-patient care - 49%, hospitalization - 23%, diagnostics/laboratory tests - 28%). CONCLUSIONS: These results represent the current use of ATG120 in the population of Polish acromegalic patients in a realistic clinical setting. Findings that 50% of patients could be treated with dose intervals of longer than 28 days support the potential of ATG120 to reduce the treatment burden.

The 1 µg Synacthen stimulation test in the diagnosis of secondary adrenal insufficiency in patients with Rathke's cleft cyst and empty sella syndrome.

INTRODUCTION: Rathke's cleft cyst (RCC) and primary empty sella syndrome (PESS) are usually incidental findings on magnetic resonance imaging (MRI) scans. In most cases, these lesions do not cause mass effect symptoms and do not require surgical intervention. In patients with RCC or PESS, it is important to exclude secondary adrenal insufficiency (SAI), which may be a life-threatening condition. MATERIAL AND METHODS: The incidence of SAI was assessed in patients with RCC or PESS detected by MRI, using the 1 µg Synacthen stimulation test. A total of 38 patients were analysed. Test results were linked to clinical symptoms and the type of cystic lesion. RESULTS: Assuming that cortisol levels < 14.6 µg/dL in Synacthen test are the criterion of SAI diagnosis, SAI was diagnosed only in 2 patients (5%). Adopting the traditional criterion of cortisol levels < 18 µg/dL, SAI would be diagnosed in 7 patients (18.4 %). Dizziness (Chi2 = 3.89; p = 0.049) and apathy (Chi2 = 3.87; p = 0.049) were significantly more frequent in the PESS group than in the RCC group. CONCLUSIONS: The incidence of SAI in the general patient population with empty sella syndrome and Rathke's cleft cysts is low. The 1 µg Synacthen test seems to be a valuable tool in the diagnosis of SAI among patients with RCC and PESS. Further studies are necessary to determine the sensitivity and specificity of the 1 µg Synacthen test with the standardization of test protocol and considering the cortisol level at the 20-minute timepoint. PESS patients report dizziness and apathy more frequently than RCC patients, which does not result from the disturbance of the hypothalamic-pituitary-adrenal axis, but probably from the different pathogenesis of these cystic lesions.

Endocrine Autoimmunity in Pregnancy.

Human gestation leads to a number of physiological alterations which peak at the development of placentta known for, among many other functions, being a transient but highly potent endocrine organ. Hormonal activity of placenta is marked by its ability to continuously produce and secrete high levels of progesterone. Progesterone guards the well-being of the fetoplacental unit throughout the gestation and one of the proposed mechanisms of this principle involves the development of local and systemic immune tolerance mainly due to impediment of CD4+ lymphocyte activation. However, though these alterations are present and well-established, autoimmunity is not entirely rare and a wide spectrum of diseases can continue, or develop de novo, throughout the gestation or even after the delivery. Up-to-date data supports the existence of a relationship between the clinical course of chosen autoimmune diseases and levels of circulating sex steroids. The most common autoimmune endocrinopathies in pregnant women are Hashimoto's disease, Graves' disease, and, more rarely, primary adrenal insufficiency in the form of Addison's disease. Gestation can influence the clinical course of these endocrinopathies in patients who were diagnosed before conception. Multiple particles, like TSH-receptor stimulating antibodies, thyroid hormones, glucocorticoids, and anti-thyroid medications, can cross the placental barrier and evoke biological action in fetal tissues. Thyroid pathology in the form of postpartum thyroiditis is particularly prevalent in patients with positive anti-thyroperoxidase and anti-thyroglobulin antibodies. Certain populations are more at risk of developing numerous gestational complications and require regular follow-up. In our paper, we would like to address physiological, physiopathological, and clinical aspects of endocrine autoimmunity throughout human gestation, as well as special circumstances to consider in pregnant women.

Rare clinical manifestation of multiple endocrine neoplasia type 1.

OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is a rare disorder characterized by tumors in various endocrine glands. It is caused by a mutation in the MEN1 gene. This gene encodes menin, a protein that regulates cell proliferation. The clinical manifestation of the syndrome most commonly involves hyperparathyroidism and pancreatic, pituitary gland, and adrenocortical adenomas. Although the first symptoms of the disease usually occur in patients under the age of 20, the data on MEN1 in children is scarce. Here, we report a case study of a familial MEN1 syndrome with a central nervous system ganglioglioma, a manifestation that has not been characterized so far. CASE REPORT: The diagnosis of a 17-year-old boy with hypoglycemia of unknown origin revealed the presence of a pancreatic tumor. As kidney stone disease and acute pancreatitis were reported in his father, and his asymptomatic sister was initially diagnosed with a pancreatic tumor, a familial MEN1 syndrome was suspected. Indeed, a pathogenic mutation within the MEN1 gene was detected. Further diagnosis revealed primary hyperparathyroidism in both children and their father, which is typical of MEN1. The girl also presented with hydrocephalus caused by ganglioglioma of the central nervous system. Surgical treatment was successfully conducted in both children. CONCLUSIONS: The reported family case provides evidence of the diagnostic and therapeutic difficulties related to the MEN1 syndrome. In children, the benefits of an early surgery should be considered in relation to the risks of possible surgical complications and consequences of a loss of endocrine gland function.

Thyroid diseases and fertility disorders - Guidelines of the Polish Society of Endocrinology [Choroby tarczycy a zaburzenia plodnosci - rekomendacje Polskiego Towarzystwa Endokrynologicznego].

Thyroid hormones influence female fertility, directly stimulating oocyte maturation and regulating prolactin and sex hormone binding globulin (SHBG) concentrations. Hyperthyroidism affects 1-2%, overt hypothyroidism 0.3%, and subclinical hypothyroidism up to 15% of women of childbearing age. Approximately 10% of euthyroid women have elevated concentrations of anti-thyroid peroxidase antibodies (aTPO) and/or anti-thyroglobulin (aTg) antibodies. Hypothyroidism can cause menstrual and ovulation disorders, and impact fertility. Studies carried out to date have not conclusively demonstrated that subclinical hypothyroidism or elevated aTPO/aTg concentrations make it harder to conceive, but they do increase the risk of pregnancy loss. Subclinical hypothyroidism and elevated aTPO/aTg concentrations without thyroid disorders are more common in polycystic ovary syndrome, premature ovarian insufficiency, and idiopathic infertility. Fertility problems are therefore an indication for screening for thyroid diseases (in females as well as in some males). A thyroid disorder diagnosed in subfertile couples should be treated appropriately, especially before attempting assisted reproductive techniques. These recommendations are intended as a guide for the management of thyroid diseases associated with infertility.

Association between ER-α polymorphisms and bone mineral density in patients with Turner syndrome subjected to estroprogestagen treatment--a pilot study.

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 < 0.001) and 4 (p < 0.001) years of treatment. In conclusion, genotypes xx and pp were shown to be prognostic markers of a good response to EP treatment, whereas the XXPP haplotype carriers were revealed to have the risk factors for insufficient responsiveness against EP treatment in BMD control.

Laparoscopic adrenalectomy for pheochromocytoma during pregnancy.

Not required for Clinical Vignette.

Quality of Life and Sleep in Patients with Pituitary Adenoma in Relation to Tumor Type and Compression of the Optic Chiasm.

OBJECTIVE: To determine the effect of transsphenoidal surgery on quality of life and sleep in patients with pituitary adenomas depending on tumor type and compression of the optic chiasm. METHODS: In this prospective study, patients with pituitary adenomas who were scheduled for transsphenoidal surgery completed the Short Form 36 Questionnaire, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale preoperatively and 7.5 (±1.5) months after surgery. Patients were analyzed based on tumor type and compression of the optic chiasm. RESULTS: Significant improvements with large effect sizes were seen for patients with Cushing's disease in general health (Z = -2.37; p = 0.018), vitality (Z = -2.05; p = 0.041), and mental health (Z = -2.06; p = 0.040). A significant deterioration with large effect size occurred in physical functioning (Z = -2.02; p = 0.043) in patients with acromegaly. A significant improvement with medium effect size was seen in subjective sleep quality, (Z = -2.24; p = 0.025), sleep duration (Z = -2.11; p = 0.035), and habitual sleep efficiency (Z = -2.26; p = 0.024) after decompression of the optic chiasm. Multiple significant correlations were observed between sleep parameters and Short Form 36 subscales before and after treatment. CONCLUSIONS: Changes in quality of life during the follow-up period depend on tumor type. Circadian rhythm disturbances may resolve promptly after decompression of the optic chiasm. Quality of life in pituitary adenoma patients is associated with quality of sleep in many dimensions, thus implying that developing strategies to improve sleep quality could increase overall well-being and everyday functioning in pituitary adenoma patients.

Thyroid diseases in pregnancy: guidelines of the Polish Society of Endocrinology [Choroby tarczycy w ciazy: zalecenia postepowania Polskiego Towarzystwa Endokrynologicznego].

Appropriate care of pregnant women with coexisting thyroid dysfunction is still a subject of much controversy. In recent years, there has been a dynamic increase in the number of scientific reports on the diagnosis and treatment of thyroid diseases in women planning pregnancy, pregnant women, and women in the postpartum period. These mainly concern the management of hypothyroidism, autoimmune thyroid diseases, and fertility disorders. Therefore, the Polish Society of Endocrinology deemed it necessary to update the guidelines on principles of diagnostic and therapeutic management in this group of patients, previously published in 2011. The recommendations were prepared by Polish experts according to evidence based medicine principles, if such data were available.

From open to laparoscopic adrenalectomy: thirty years' experience of one medical centre.

INTRODUCTION: Laparoscopic adrenalectomy (LA) has become the standardized treatment of benign adrenal lesions over the last two decades, making the indications to open adrenalectomy (OA) limited. The purpose of this study was to show the thirty years of experience in open (OA) and laparoscopic adrenalectomy (LA) gained in one medical centre as well as to compare the results of OA and LA performed for benign adrenal lesions. MATERIAL AND METHODS: Three hundred patients underwent 127 open and 173 laparoscopic adrenalectomies between 1979 and 2009 at M. Curie Hospital in Szczecin, Poland. Analyzed factors included patients demographic data, ASA score, indication for surgery, tumour size and side, characteristics of the removed tumours, intraoperative and postoperative outcome of LA and OA, postoperative pain sensation, intraoperative and postoperative complications, and conversion rate from LA to OA. Tumours with diameter exceeding 8 cm were excluded. RESULTS: There were no significant differences regarding the analyzed preoperative data in both groups of patients. The mean operative time was longer in the LA group (137 v. 82 min., p < 0.0001) and the blood loss was lower in LA group (110 v. 254 mL, p < 0.0001). The mean time until resumption of normal diet was shorter after LA (22 v. 44 h), as was the mean time until ambulation (17 v. 36 h), mean length of the hospital stay (4.6 v. 6.8 days), and mean time until return to normal activities (14 v. 23 days, p < 0.0001 for each difference). The analgesic requirement on the first and the second day postoperatively was lower in the LA group (p < 0.0001). The incidence of intraoperative and postoperative complications did not differ significantly between both analyzed groups. The rate of the conversion from LA to OA was 16%. The histopathological diagnosis was adenoma of the adrenal gland in the majority of cases. CONCLUSIONS: This study shows that LA is a safe, effective, and well-tolerated procedure. It may be recommended as a "gold standard" surgery in a case of benign functioning or non-functioning adrenal tumours with diameter less than 8 cm. (Pol J Endocrinol 2010; 61 (1): 94-101).

Cushing's Syndrome in a Patient With Rathke's Cleft Cyst and ACTH Cell Hyperplasia Detected by 11C-Methionine PET Imaging-A Case Presentation.

Background: Adrenocorticotropic Hormone (ACTH)-dependent Cushing's Syndrome (CS) is most often caused by a pituitary adenoma. Although rarely, it can also result from pituitary corticotroph cell hyperplasia (CH). Reports on concomitant pituitary lesions including ACTH-producing adenomas and Rathke's cleft cysts (RCCs) have been published. Positron emission tomography (PET), using 11C-labelled-methionine (MET) as a tracer and co-registered with magnetic resonance imaging (MRI) has been shown to be useful in the diagnosis of pituitary collision lesions, however, its role is still under investigation. In this work we present the case of a patient in whom CS was caused by non-adenomatous CH within the wall of an RCC. Case Summary: In 2015 a patient with signs and symptoms of CS was referred to our Department. Biochemical studies repeatedly showed elevated midnight serum cortisol and ACTH levels. Magnetic resonance imaging of the sellar region revealed an RCC and MET-PET/MR showed heterogeneous labelled-methionine metabolism in the vicinity of the cyst's wall. Transsphenoidal surgery resulted in rapid, complete and lasting relief of symptoms. Histopathological examination demonstrated an RCC and CH. Conclusions: Concomitance of pituitary focal lesions is a rare phenomenon. Methionine-labelled PET/MR may be useful in the diagnosis of collision sellar lesions, including CH. Corticotroph cell hyperplasia can present as mild and fluctuating hypercortisolaemia.

The Neuroendocrine Neoplasms of the Digestive Tract: Diagnosis, Treatment and Nutrition.

Nuroendocrine neoplasms (NENs) are a group of rare neoplasms originating from dispersed neuroendocrine cells, mainly of the digestive and respiratory tract, showing characteristic histology and immunoprofile contributing to classification of NENs. Some NENs have the ability to produce biogenic amines and peptide hormones, which may be associated with clinical syndromes like, e.g., the carcinoid syndrome caused by unmetabolized overproduced serotonin, hypoglycemic syndrome in case of insulinoma, or Zollinger-Ellison syndrome accompanying gastrinoma. Diagnostics for these include ultrasound with endoscopic ultrasound (EUS), computed tomography (CT), magnetic resonance imaging (MRI), and positron-emission tomography/computed tomography (PET/CT). Different nuclear medicine procedures can also be used, like somatostatin analogues scintigraphy (SRS) and 68Ga-Dota-Peptide PET/CT, as well as biochemical methods to determine the level of general neuroendocrine markers, such as chromogranin A (CgA), 5-hydroxyindolacetic acid (5-HIAA), synaptopfysin and cell type-specific peptide hormones, and neurotransmitters like gastrin, insulin, serotonin, and histamine. NENs influence the whole organism by modulating metabolism. The treatment options for neuroendocrine neoplasms include surgery, somatostatin analogue therapy, radionuclide therapy, chemotherapy, molecular targeted therapies, alpha-interferon therapy, and inhibitors of serotonin production. In the case of hypersensitivity to biogenic amines, a diet that limits the main sources of amines should be used. The symptoms are usually connected with histamine, tyramine and putrescine. Exogenic sources of histamine are products that take a long time to mature and ferment. Patients with a genetic insufficiency of the diamine oxidase enzyme (DAO), and those that take medicine belonging to the group of monoamine oxidases (MAO), are particularly susceptible to the negative effects of amines. Diet plays an important role in the initiation, promotion, and progression of cancers. As a result of the illness, the consumption of some nutrients can be reduced, leading to nutritional deficiencies and resulting in malnutrition. Changes in metabolism may lead to cachexia in some patients suffering from NENs. The aim of this narrative review was to advance the knowledge in this area, and to determine possibilities related to dietary support. The authors also paid attention to role of biogenic amines in the treatment of patients with NENs. We can use this information to better understand nutritional issues faced by patients with gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs), and to help inform the development of screening tools and clinical practice guidelines.

Therapeutic effect of presurgical treatment with longacting octreotide (Sandostatin® LAR®) in patients with acromegaly.

INTRODUCTION: The aim of this study was to assess the therapeutic effect and the safety of pre-surgical treatment with long-acting octreotide in patients with acromegaly. MATERIAL AND METHODS: This project was conducted in 25 centres across Poland as a non-interventional, multicentre, observational study in patients with acromegaly, in which long-acting octreotide Sandostatin® LAR®) was administered before surgery. They were 148 patients included into the study: 88 females and 60 males aged 18-86 years (51.3 ± 13.4). RESULTS: Eighty patients completed the study (underwent tumour surgery). The CRF included: baseline visit, four follow-up visits every three months before surgery, and two follow-up visits every three months after surgery. Sandostatin® LAR® was administered every four weeks. The efficacy measures were as follows: change of growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels, number of patients fulfilling criteria of cure, and change of adenoma (micro- and macroadenomas) size during the treatment. Normalisation of GH and IGF-1 concentrations were obtained in 42.4 and 49.1% of patients at the end of medical therapy, respectively. Normalisation of GH and IGF-1 concentrations were obtained in 77.9 and 83.8% of patients after surgery, respectively. Reduction of microadenoma size was documented in 58.8% of patients, and in 70% of patients with macroadenomas at the end of medical therapy. In 74.0% of patients no pituitary tumour was shown on MRI after surgery. CONCLUSION: We have shown good surgical outcome in patients with acromegaly after pre-treatment with somatostatin analogue, and good tolerance and safety of the therapy, supporting the national recommendation for pre-surgical treatment with long-acting somatostatin analogues in acromegaly patients.

Quality of life assessment in patients with Graves' disease and progressive infiltrative ophthalmopathy during combined treatment with methylprednisolone and orbital radiotherapy.

INTRODUCTION: The aim of the study was to assess quality of life (QoL) in patients with infiltrative form of Graves' ophthalmopathy (GO) during the combined pulse treatment with methylprednisolone and orbital radiotherapy, and also to search for the relation between the results of ophthalmopathy treatment and changes in QoL. MATERIAL AND METHODS: The study involved 29 patients aged 25-74 (the mean age: 52 +/- 6 years) with infiltrative form of GO. They were classified for ophthalmopathy treatment on the basis of the following factors: the obtained euthyreosis, progressive character of eye changes, the level of eye changes determined on the basis of NO SPECS classification (at least class 3c), ophthalmopathy index (OI) according to Donaldson >or= 4. GO was diagnosed as active if CAS (clinical activity score) >or= 4. During the treatment, the patients received 6 cycles of methylprednisolone sodium succinate in doses of 1,0 g/24 h given as one-hour-long intravenous infusions for three successive days in a week. Between the 2nd and 4th cycle of Solu-Medrol, orbital radiotherapy with 10 MeV X-rays was performed. The control group was made up of healthy volunteers selected with regard to sex, age, educational background and nicotine addiction so as they corresponded with the study group. It involved 53 individuals aged 21-75 (the mean age: 52,4 +/- 14 years). QoL was assessed by means of the MOS SF-36 questionnaire. RESULTS: Patients with GO evaluated their QoL lower than healthy individuals, which referred to physical functioning, physical and emotional role functioning, general health, vitality, social functioning, mental health and bodily pain. No correlation was found between quality of life and such factors as age, sex, or duration time of Graves disease and ophthalmopathy. Analogically, no relation was observed between the activity and stage of clinical development of eye changes and QoL. The use of the combined GO therapy contributed to a considerable decrease in the development of eye changes and the disease activity. After treatment, the patients' QoL improved which referred to physical role functioning, bodily pain, and vitality. Other QoL parameters did not statistically significantly differ. CONCLUSIONS: GO causes a considerable worsening of QoL. The stage of clinical development and activity of GO find no reflection in QoL. Effectiveness of treatment for GO cannot be evaluated on the basis of changes in QoL.

[Bone mineral density as an indicator of hormonal and metabolic disturbance in girls in developmental period]. / Gestosc mineralna kosci jako wskaznik zaburzen hormonalnych i metabolicznych u dziewczat w okresie rozwojowym.

PURPOSE OF THE WORK: To assess bone mineral density (BMD) in girls with menstruation disorders in the first years after menarche and to demonstrate that BMD value may be one of the indicators confirming an early diagnosis of hypothalamic hypogonadism or androgenisation complex. MATERIAL AND METHODS: The research included 155 girls at the ages of 16-17, observed due to rare menstruation or secondary amenorrhea. In case of 71 girls functional, hypothalamic disorders were found (Group A), in 35 girls increasing androgenisation features were diagnosed additionally (Group B), and 49 healthy girls represented Group C--control group. BMI was determined, as well as FSH, LH, T, E2, PRL concentrations and the BMD test of lumbar spine section was made in all of the cases. RESEARCH RESULTS: The average values of BMI and BMD in Group A were the lowest compared to Group B (p<0.001) and Group C (p<0.001). E2 concentrations in Group A was the lowest and in Group B--the highest (p<0.001). T concentrations in Group B girls was the highest and SHBG--the lowest compared to Groups A and C (p<0.001; p<0.01). FSH concentrations in Group A were the lowest compared to Groups B and C (p<0.01), while LH in Group B was the highest (p<0.001; p<0.01). Basic PRL concentrations were not essentially different. In groups (A + B + C) together, a statistically significant correlation between BMD and BMI values was found (r=0.67; p<0.001), E2 (r=0.38; p<0.01) and T (r=0.56; p<0.001). CONCLUSIONS: Low bone mineral density value compared to the age value may signify long-term hypoestrogenism, and high value - a complex of metabolic disorders with high concentration of androgens. Therefore, bone mineral density may be a reliable and highly sensitive metabolic and hormonal indicator of menstruation disorders of hypothalamic, functional type or androgenisation complex.

[Under-age girl as a patient of pediatric and adolescent ginecology outpatient clinic]. / Nieletnia jako pacjent w poradni ginekologii wieku rozwojowego.

The research carried out in Poland reflected that sexual initiation before 18 years of age is a common phenomenon and refers to roughly 80% of teenagers. In Poland there is no uniform standing of medical and legal environments with regard to dealing with a juvenile patient who has become sexually active and expects the advice of a gynaecologist, gynaecologic examination and often asks for prescribing contraceptives. The procedures must take into account the fact that in Poland, until 18 years of age, a juvenile functions under the parental or tutelary authority, while a consent for medical service requires beside of the consent of legal guardian also the consent of a juvenile who is 16 years of age and becomes a full-right patient. According to the Act on Health Care Institutions, a patient has the right to self-decisions, the respect of physical and mental integrity and the respect of privacy, while the participation of a statutory representative post 16th year of age refers practically to co-deciding on a medical service provision. Therefore, the information received from such juvenile patient in subjective and objective examination does not have to be passed to the statutory representative, if the juvenile patient requires confidentiality and if this does not affect the patient's health and the planned medical procedures (e.g. the necessity of making an operation). The knowledge of conduct procedures with regard to a juvenile patient as a carrier of rights shall enable doctors to make aware choices of conduct and provide services or, in most cases, only advice, without the necessity to breach the laws of Poland.

Cystic lesions of the sellar-suprasellar region - diagnosis and treatment.

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.