Women with Polycystic Ovary Syndrome and Risk of Cardiovascular Disease.

BACKGROUND: Polycystic ovary syndrome (PCOS) is associated with reproductive and metabolic abnormalities. The aim of this study was to analyse risk of cardiovascular disease (CVD) in PCOS, to define individual risk factors and assess their ability to predict risk. METHODS: Fifty-four young women with PCOS (22 obese and 32 normal weight) were compared to 46 respective controls (17 obese and 29 normal weight). Anthropometric parameters, lipid status parameters, inflammation markers, concentrations of glucose, transaminases, sex and anterior pituitary hormones, sex hormone binding globulin (SHBG) and androgens were measured. Cardiovascular Risk Score (CVRS), indices for identifying Non-Alcoholic Fatty Liver Disease (NAFLD) and the Index of Central Obesity (ICO) were calculated. RESULTS: Significantly higher CVRS values (p<0.05) were found in obese PCOS women compared to normal weight control and normal weight PCOS groups. Anthropometric parameters, lipid status parameters and fibrinogen (p<0.001, p<0.01) were higher in women with higher CVRS. The most significant CVRS predictors in all PCOS women were SHBG, androstenedione, follicle-stimulating hormone (FSH) and dehydroepiandrosterone sulphate (DHEAS). ICO and all NAFLD indices exhibited significant positive correlation with CVRS and a model consisting of these indices provided good diagnostic accuracy (AUC>0.8) in identifying patients with increased cardiovascular risk (CVR). CONCLUSIONS: Obesity is a higher risk for developing CVD than PCOS alone. Anthropometric parameters, lipid parameters, fibrinogen, NAFLD indices and ICO increase CVR in PCOS women. For the prediction of CVR in PCOS, we suggest a combination of NAFLD indices and ICO.

Coexisting diseases modifying each other's presentation - lack of growth failure in Turner syndrome due to the associated pituitary gigantism.

Introduction: Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH) secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceleration, although these patients also suffer from hypogonadism and soft tissue hypertrophy. Case report: We presented a girl with mosaic Turner syndrome, delayed puberty and normal linear growth for the sex and age, due to the simultaneous GH hypersecretion by pituitary tumor. In the presented case all the typical phenotypic stigmata related to Turner syndrome were missing. Due to excessive pituitary GH secretion during the period while the epiphyseal growth plates of the long bones are still open, characteristic stagnation in longitudinal growth has not been demonstrated. The patient presented with delayed puberty and primary amenorrhea along with a sudden appearance of clinical signs of hypersomatotropinism, which were the reasons for seeking medical help at the age of 16. Conclusion: Physical examination of children presenting with delayed puberty but without growth arrest must include an overall hormonal and genetic testing even in the cases when typical clinical presentations of genetic disorder are absent. To the best of our knowledge, this is the first reported case of simultaneous presence of Turner syndrome and gigantism in the literature.

Myelodysplastic syndromes in adults aged less than 50 years: incidence and clinicopathological data.

PURPOSE: Myelodysplastic syndrome (MDS) is rarely seen in patients younger than 50 years, but rapidly increases with advancing age. Data on MDS biology in young patients are yet scarce but more than necessary. The purpose of this study was to estimate the proportion of MDS patients <50 years of age and to compare the clinicopathological data between younger and older patients. METHODS: Of our total MDS cases comprising 587 adult patients we studied 83 adults (14.14%) aged < 50 years with primary MDS. RESULTS: MDS patients were classified in those aged < 50 years and those aged ≥ 50 years. Younger MDS patients were characterized by female preponderance (p<0.001), better performance status (p=0.0035), less severe anaemia (p=0.008), better preserved kidney function (p=0.037), less often blast infiltration in bone marrow (p=0.015), more cases of RA (p<0.001) and RCUD (p=0.0066), lower MD Anderson score (p<0.001), longer overall survival (OS) (p<0.001), but similar progression rate (p=0.591). Median OS of young MDS patients was 39.7 months and 19 months of patients >50 years (p<0.001). In this group, 24 patients (28.92%) progressed to acute myeloid leukaemia (AML) vs 111 (22.02%) patients>50 years (p=0.402). Multivariate analysis identified platelet count (p=0.008) and percent of blasts in bone marrow (p=0.024) to be predictive for shorter OS in patients < 50 years of age; the same factors (p<0.001) together with IPSS-R cytogenetic risk group (p<0.001) were identified in patients >50 years of age. Platelet count (p=0.003) and percent of blasts in bone marrow (p=0.001) were predictive for higher risk of transformation to AML in patients <50 years, and bone marrow infiltration (p=0.022) and IPSS-R cytogenetic risk group (p=0.027) for patients >50 years of age. CONCLUSION: Presenting features in young MDS patients may identify subjects at higher risk for unfavorable outcome.

Double-hit primary unilateral adrenal lymphoma with good outcome.

INTRODUCTION: Primary adrenal non-Hodgkin's lymphoma (NHL) is a rare neoplasm with poor prognosis. On the other side, double-hit lymphomas with BCL2 and MYC translocation are characterized by advanced disease stage, extranodal and central nervous system involvements at presentation or disease progression. CASE REPORT: We reported a 73-year-old male patient with double-hit primary adrenal lymphoma and preserved adrenal function, showing a favorable clinical course. Computed tomography of abdomen showed a 9 x 7 cm mass of the left adrenal gland. Laparatomy with left adrenalectomy was done and histological examination revealed diagnosis of a diffuse large B-cell NHL (DLBCL), non-GCB subtype. The patient was treated with 6 cycles of R-CHOP chemotherapy with reduced doses of doxorubicin because of the decreased left verticle ejection fraction. The patient was followed up regularly for 20 months with no evidence of tumor recurrence despite the inherently poor prognostic profile and double-hit phenotype of the disease. CONCLUSION: R-CHOP chemotherapy in combination with adrenalectomy can be an effective first-line regimen for primary adrenal DLBCL, despite the inherently poor prognostic profile (non-GCB subtype, bulky disease, elevated lactate dehydrogenase and double-hit phenotype of the disease).

Uric acid and gamma-glutamyl transferase activity are associated with left ventricular remodeling indices in patients with chronic heart failure.

INTRODUCTION AND OBJECTIVES: Uric acid and gamma-glutamyl transferase are prognostic indicators in chronic heart failure. Nevertheless, the mechanism underlying the association between uric acid, gamma-glutamyl transferase, and chronic heart failure progression and prognosis remains largely unknown. METHODS: The association of uric acid and gamma-glutamyl transferase with flow-mediated dilation and echocardiographic indices of cardiac remodeling was addressed in 120 patients with chronic ischemic heart failure. To determine the independent contribution of uric acid and gamma-glutamyl transferase to the flow-mediated dilation and echocardiographic indices of remodeling, a series of multiple linear regression models, based on traditional and nontraditional risk factors impacting upon these parameters, were constructed. RESULTS: Uric acid, but not gamma-glutamyl transferase, was an independent predictor of flow-mediated dilation. Uric acid was associated with all the echocardiographic indices of left ventricular dysfunction tested in 3 multiple-regression models. Uric acid correlated with left ventricular end-systolic diameter, left ventricular end-diastolic diameter, left ventricular end-systolic volume, and left ventricular end-diastolic volume (r = 0.337; r = 0.340; r = 0.321; r = 0.294; P = .001, respectively). Gamma-glutamyl transferase was an independent predictor of left ventricular end-systolic volume and left ventricular end-diastolic volume, after adjustment for all variables. Gamma-glutamyl transferase correlated with left ventricular end-systolic diameter, left ventricular end-diastolic diameter, left ventricular end-systolic volume, and left ventricular end-diastolic volume (r = 0.238, P = .009; r = 0.219, P = .016; r = 0.359, P < .001; r = 0.369, P = .001, respectively). CONCLUSIONS: Serum uric acid and gamma-glutamyl transferase levels are associated with left ventricular remodeling in patients with chronic ischemic heart failure.

Importance of revealing a rare case of breast cancer in a female to male transsexual after bilateral mastectomy.

The incidence of breast carcinoma following prophylactic mastectomy is probably less than 2%. We present a 43-year-old female to male transsexual who developed breast cancer 1 year after bilateral nipple- sparing subcutaneous mastectomy as part of female to male gender reassignment surgery. In addition to gender reassignment surgery, total abdominal hysterectomy with bilateral salpingo-oophorectomy (to avoid the patient from entering menopause and to eliminate any subsequent risk of iatrogenic endometrial carcinoma), colpocleisys, metoidioplasty, phalloplasty, urethroplasty together with scrotoplasty/placement of testicular prosthesis and perineoplasty were also performed. Before the sex change surgery, the following diagnostic procedures were performed: breast ultrasound and mammography (which were normal), lung radiography (also normal) together with abdominal ultrasound examination, biochemical analysis of the blood and hormonal status.According to medical literature, in the last 50 years only three papers have been published with four cases of breast cancer in transsexual female to male patients. All hormonal pathways included in this complex hormonal and surgical procedure of transgender surgery have important implications for women undergoing prophylactic mastectomy because of a high risk of possible breast cancer.

[Autoimmune processes in diseases of the thyroid gland]. / Automuni procesi u bolestima stitaste zlezde.

The previous knowledge related to auto-immune occurrences in thyroid gland disease are described in the review, showing main antigens, auto-antibodies, the inclusion of humoral and cellular immunity in the most frequent thyroid gland diseases. In spite of many learnings, which are broadened every day, the exact sequence of events and roles of external factors in genetically predisposed persons is still unknown. An identification of auto-antibodies gives direct evidence about the auto-immune process, as they are present among the majority of patients with Graves' hyperthyroid and Hashimoto's thyroiditis. Immuno globulins, created by plasma cells, linked with cytokinins from lymphocyte CD4 T, engage the cell auto-immune reaction in these diseases. Being acquainted with all events in the auto-immune process, we can understand better why these frequent diseases occur, why do they have chronic and recidivous course, why they are more frequent with women and within some families, and why they enable us to make a better diagnosis and treatment of these patients.

[Adrenocortical hypertension]. / Adrenokortikalna hipertenzija.

INTRODUCTION: Endocrine hypertension is almost exclusively adrenal hypertension which includes syndromes of mineralocorticoid, glucocorticoid and amine excess. ALDOSTERONISM: Although primary aldosteronism accounts for less than 1% of hypertensive population, it is the most common form of endocrine hypertension and the second most common form of curable hypertension. It is possible that load in type 2 diabetes mellitus and cardiovascular fibrosis is related to aldosteronism. DIAGNOSIS OF PRIMARY ALDOSTERONISM: The conventional approach to low plasma potassium level in hypertensive patients suggests mineralocorticoid excess, whereas high aldosterone level and chronically suppressed plasma renin activity (PRA) are basic for the diagnosis of primary aldosteronism. However, recent studies use the ratio of plasma aldosterone to plasma renin activity (ARR) in plasma. DIFFERENTIAL DIAGNOSIS: Differential diagnosis between idiopathic aldosteronism (IHA) and aldosterone--producing adenoma (APA) can be performed using postural testing, 18-hydro- xycorticosterone levels, adrenal computed tomograms, iodocholesterol scans and adrenal venous sampling. GLUCOCORTICOID HYPERTENSION: However, association between glucocorticoid excess and hypertension is complex and still poorly understood. Hypothesized mechanisms include: increased hepatic angiotensinogen synthesis, inhibition of vasodilator and stimulation of vasoconstrictor systems in vascular tissue, enhanced glucocorticoid-mediated vascular reactivity to noradrenaline, shifting of sodium and fluid from intracellular to extracellular compartment, and increase of local endothelial growth factor activity.