Twenty-four hour 17-hydroxyprogesterone response to adrenocorticotropine in adrenal incidentalomas: augmented response after adrenalectomy in two patients

The current study aimed to investigate the midterm (24 hour) response of 17-hydroxyprogesterone (17-OHP) and dehydroepiandrosterone sulphate (DHEA-S) to synthetic high-dose adrenocorticotropin (ACTH) in adrenal incidentalomas (Al). Seventeen patients with Al and 40 age- and sex-matched controls received synthetic ACTH (tetracosactide, 1000 microg, IM). Plasma, 17-OHP and DHEA-S were collected in basal conditions and after 1, 4, 6, 8 and 24 hours. (HPA) axis was also evaluated using circadian serum cortisol, urinary free cortisol and over-night 2 mg dexamethasone suppression. Basal plasma 17-OHP levels did not differ among the groups. However, the increment in plasma 17-OHP in patients both in terms of peak [13.76 +/- 2.52, 4.77 +/- 0.30ng/ml, mean +/- S.E.M, p < 0.001] and area under the curve [190 +/- 46, 96.75 +/- 32 ng/ml/h, p < 0.001] were significantly higher than that of the controls. Stimulated 17OH-P levels never reached 9.1 ng/ml in controls. Sixty-five (11/17) % of the patients were found to have exaggerated response. Three of the patients were found to have subclinical Cushing's syndrome and interestingly, two augmented their 17-OHP response to ACTH after unilateral adrenalectomy and normalisation of their HPA axis. Basal DHEA-S levels of the patients were significantly lower [99.21 +/- 45, 230.18 +/- 34 microg/dl, p < 0.01] and stayed persistently lower than that of the controls. Evidence of a heterozygous 21 hydroxylase deficiency, as indicated by the exaggerated 17-OHP response to ACTH, has been widely reported in Al patients. However, to our knowledge to date there is no report on augmented 17-OHP response to ACTH after adrenalectomy. Possible reasons for the augmentation were discussed.     

Blood pressure control and left ventricular hypertrophy in long-term CAPD and hemodialysis patients: a cross-sectional study.

BACKGROUND: It is still not clear whether hypertension and left ventricular hypertrophy (LVH) are more common in continuous ambulatory peritoneal dialysis (CAPD) than in hemodialysis (HD) patients. METHODS: To examine this subject, the indices of cardiac performance were compared between 50 HD and 34 CAPD patients. Patients were further divided into two subgroups [long-term (L) CAPD and L-HD] according to dialysis modality and duration of dialysis (more than 60 months' duration). RESULTS: The blood pressure and cardiothoracic index of CAPD patients did not differ from HD patients. On average, the left atrial index was 2 mm/m2 higher in HD patients than in CAPD patients. Left ventricular chamber sizes, wall thickness, and left ventricular mass index (LVMI) in patients on CAPD were similar to those of HD patients. Isovolumic relaxation time (IVRT) of CAPD patients was insignificantly less than that of HD patients (101 +/- 22 and 115 +/- 27 msec respectively). There was no significant difference between the two subgroups (L-HD and L-CAPD) in blood pressure, left atrial diameter, left ventricular chamber size, wall thickness, LVMI, ejection fraction, or IVRT. CONCLUSION: If normovolemia and normotension are obtained by strict volume control without using antihypertensive drugs, the effects of the two modalities of chronic dialysis treatment (HD and CAPD) on cardiac structure and function are not different from each other.     

Prevalence of metabolic syndrome and associated risk factors among Turkish adults: Trabzon MetS study

Objective In order to estimate the prevalence of metabolic syndrome (MetS) as defined by NCEP Adult Treatment Panel III (ATP III) criteria in the Trabzon Region and its associations with demographic factors (age, sex, marital status, reproductive history in women, and level of education), socioeconomic factors (household income and occupation), family history of selected medical conditions (diabetes, hypertension, and obesity), lifestyle factors (smoking habits, physical activity, and alcohol consumption) in the adult population. Research Methods and Procedures In this cross-sectional survey, a sample of households was systematically selected from the central province of Trabzon and its nine towns. A total of 4,809 adult subjects ≥20 years (2,601 women and 2,208 men) were included in the study. Blood pressure levels were measured for all subjects. The persons included in the questionnaire were invited to the local medical centers for blood examination between 08:00 and 10:00 following 12 h of fasting. Fasting serum glucose (FBG) levels and lipid profile were measured with autoanalyzer. MetS was defined according to guidelines from the NCEP ATP III diagnostic criteria. Results The prevalence of MetS was 26.9%: 31.3 in women and 21.7% in men. The prevalence increased with age, being highest in the 60–69-year-old age group (53.4%) but lower again in the ≥70 age group. MetS was associated positively with marital status, parity, cessation of cigarette smoking, and negatively with the level of education, alcohol consumption, current cigarette use, household income, and physical activity. Hypertension was found as the most common MetS component in our study (57.4%). Others in decreasing order were abdominal obesity (40.9%), low high-density lipoprotein-C (HDL-C) (31.8%), hypertriglyceridemia (30.7%), and high FBG levels (9.2%). Similarly, in the subjects diagnosed with MetS, HT had the highest prevalence (91.9%). This was followed by abdominal obesity (82.3%), hypertriglyceridemia (74%), low HDL-C (68.6%), and high fasting blood glucose levels (28.6%). Discussion MetS is moderately common and an important health problem in the adult population of Trabzon. In order to control MetS and its components, effective public health education and taking urgent steps are needed. These steps include serious education, providing a well-balanced diet and increasing physical activity.     

Five years and 4 months of recurrence-free survival in hepatic angiosarcoma

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 × 42 × 35?mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.     

Congenital adrenal hyperplasia: a rare cause of renal failure and a successful renal transplantation

Congenital adrenal hyperplasia belongs to a group of autosomal recessive disorders affecting steroid biosynthesis; a rare disease with a prevalence of 1 case per 16,000 population. A 30-year-old phenotypically male patient had been diagnosed with 11-β hydroxylase deficiency at the age of 16; presenting with ambiguous genitalia, growth retardation, presence of menstrual cycles, severe hypertension, hypokalemia and renal dysfunction. He developed endstage renal disease due to hypertension and was treated with hemodialysis for 3 y. After careful evaluation, he was approved to undergo renal transplantation. The patient has now finished 6th month after transplantation and is currently under follow-up at our outpatient clinic, having no problems related to the transplant. While early treatment to prevent hypertension is mandatory in patients with congenital adrenal hyperplasia, once renal failure occurs, renal transplantation may the best choice of treatment. In this study, we describe the first report of a successful renal transplantation in an adrenal hyperplasia.     

Identification of the parental origin of polysomy in two 49,XXXXY cases

The parental origin and mechanism of formation of polysomy X were studied in two polysomic cases, using four X-linked restriction fragment length polymorphisms, three (CA)_n dinucleotide repeat sequences and one variable number tandem repeat (VNTR) locus as genetic markers. A nonradioactive technique based on the hybridization of the polymerase chain reaction (PCR) product was developed for the analysis of dinucleotide repeats. Segregation analysis using different nonradioactive approaches based on the PCR, revealed that all four X chromosomes were of maternal origin. These data provide additional evidence of an identical mechanism of successive nondisjunctions in maternal meiosis I and II.     

Aspiration cytology of the collagenized variant of mammary myofibroblastoma: a case report with review of the literature

Myofibroblastoma of the breast is a rare benign stromal neoplasm, which occurs primarily in men. Classical myofibroblastoma is a circumscribed, nonencapsulated tumor comprised of bipolar fusiform cells arranged randomly, or in fascicles alternating with broad collagenous bands. Additional histologic variants (the cellular, collagenized, infiltrative, and epitheloid types) have been described. Several case reports describe the cytopathologic features of the classical and cellular variants. We report on a 70-yr-old woman, who presented with a circumscribed mass in her left breast. Aspiration biopsy showed paucicellular smears with singly distributed atypical spindle-shaped cells and rare fragments of collagenized stroma, raising suspicion of a phyllodes tumor. Histologic examination revealed spindle-shaped cells distributed in a diffusely collagenized stroma. Some nuclear atypia was present. To the best of our knowledge, this is the first case reporting the cytologic features of the collagenized variant of myofibroblastoma. Although we believe a specific diagnosis of myofibroblastoma can be rendered in a male based on the typical cytologic and clinical findings in the classical type, the variant forms are difficult to classify accurately and require excision for a definitive diagnosis.     

Spectrum of head and neck lesions diagnosed by fine‐needle aspiration cytology in the pediatric population

Fine‐needle aspiration cytology (FNAC) of the head and neck region is well accepted as a diagnostic procedure in the adult population. FNAC in the pediatric population is gaining acceptance as clinicians add this technique to the diagnostic armamentarium. An experience with FNAC of the head and neck region in the pediatric population is described from 2 large inner‐city hospitals. Eighty‐five cases were retrieved from patients age <18 years. In 52 cases, clinical or surgical follow‐up was obtained and among these cases the specificity and sensitivity of FNA was 93% and 100%, respectively. The high specificity of FNAC allows the clinician to be confident of malignancy in a clinically suspicious lesion of the head and neck in a pediatric patient. Cancer (Cancer Cytopathol) 2007. © 2007 American Cancer Society.