Calciphylaxis: a report of six cases and review of literature.

Calciphylaxis is usually a fatal condition that develops in a few chronic renal failure patients, and it is characterized by calcifications in subcutaneous arteries, infarcts in skin, and the neighboring subcutis. Calciphylaxis, once considered as a rare condition, has been reported to have an annual incidence of 1% and a prevalence of 4% in dialysis patients. We describe our clinical experience in six end-stage renal disease patients on dialysis that presented with calciphylaxis and died due to sepsis, and review the pathogenesis, epidemiology, clinical and histopathologic features, and treatment of calciphylaxis. Physicians should initially consider the possibility of calciphylaxis in case of development of skin lesions in chronic renal failure patients with impaired calcium, phosphorus, and parathyroid hormone levels. The most important cause of mortality in this condition is infection. Therefore, differential diagnosis of these lesions from systemic vasculitis in their early stages and withdrawal of immunosuppressive therapy that increases the tendency to infections are essential.

Obesity might not be a risk factor for female sexual dysfunction.

OBJECTIVE: To evaluate female sexual dysfunction (FSD) in obese women in comparison with age-matched control group, emphasising their hormonal and psychological status. PATIENTS, SUBJECTS AND METHODS: Sixty-four sexually active obese premenauposal women were compared with a control group of 27 age-matched healthy volunteers with a normal body mass index (BMI). The obese group was evaluated in three categories according to BMI (kg/m(2)), as group 1 (30-34.9), group 2 (35-39.9) and group 3 (> 40). All women were evaluated with Female Sexual Function Index (FSFI) questionnaire and Beck Depression Inventory (BDI). In addition, serum levels of follicle-stimulating hormone, luteinizing hormone, prolactin, dehydroepiandrosterone-SO(4) , free testosterone, 17α-hydroxyprogestrone, androstenedione, oestradiol, free thyroxine and thyrotropin were determined. RESULTS: The mean FSFI scores were not statistically significant between control and obese patients (P = 0.29). FSD was diagnosed in 50% (32/64) and 41% (11/27) of the patients in the obese and control groups, respectively (P = 0.34). There were no differences between total FSFI and FSFI domain scores among BMI categories. BDI scores were significantly higher in the obese groups than in healthy controls, and negatively correlated with total FSFI and all FSFI domain scores. Among hormonal analyses, only free testosterone levels were negatively correlated with total FSFI scores. CONCLUSION: This study showed that obesity has no significant relationship with FSD, but obese patients were found to be in a more depressive mood than age-matched normal counterparts.

Fungal peritonitis in peritoneal dialysis: risk factors and prognosis.

Although less common than bacterial peritonitis, fungal peritonitis is associated with much higher morbidity and mortality. In this study, we aimed to determine the risk factors for fungal peritonitis in peritoneal dialysis patients. The records of 109 peritoneal dialysis patients were analyzed. A total of 86 episodes of peritonitis attacks were recorded. Nine (10.5%) of these attacks were fungal peritonitis attacks. The fungal peritonitis attack rate of the population was 1 attack per 480.1 patient months. In order to determine predisposing factors for fungal peritonitis patients, patients with bacterial peritonitis and with no peritonitis admitted immediately before and after those with fungal peritonitis were used as controls. There was no statistically significant difference between the bacterial and fungal peritonitis groups with respect to symptoms and signs. Obligatory peritoneal dialysis treatment due to access or other medical problems (p = 0.04) and serum albumin levels (p = 0.01) were found to be significantly related with fungal peritonitis (p = 0.04). The mortality rate was 11.1%. When compared with the mortality rate of bacterial peritonitis (1.8%) during the same period, it was found to be significantly higher (p = 0.0001). The catheter removal was performed within 2-7 days (mean = 5.2 days) of the fungal peritonitis attacks. In conclusion, we decided that because fungal peritonitis attacks end up with high morbidity and mortality, prompt diagnosis and removal of the catheter is mandatory. Diagnosis highly depends on culture results, as signs and symptoms usually do not differ from that of bacterial peritonitis. Low serum albumin levels and obligatory peritoneal dialysis treatment are found to be the risk factors for fungal peritonitis.

Vocal cord paralysis during the treatment of mantle cell lymphoma with vincristine.

CASE DESCRIPTION: We present a case of a seventy-eight year-old man who developed vocal cord paralysis without any sign of peripheral neuropathy during the treatment of Mantle Cell Lymphoma. He first presented in 2008 with a few bilateral small inguinal lymph nodes. Inguinal lymph node biopsy demonstrated Mantle Cell Lymphoma. Flow cytometry studies of peripheral blood and bone marrow cells were compatible with Mantle Cell Lymphoma. R-CHOP chemotherapy triweekly (Rituximab; Cyclophosphamide; Adriamycin; Vincristine; and Methylprednisolone) was planned. At the end of the second cycle, the patient complained of hoarseness without any symptoms of dysphagia or odynophagia. Direct flexible laryngoscopy showed bilateral vocal cord paralysis. Vincristine was discontinued and the patient's voice gradually resolved in about 4 months. CONCLUSION: Vincristine may cause peripheral, autonomic and cranial neuropathies. However cranial nerve involvement is quite uncommon.

TAC3 and TACR3 mutations in familial hypogonadotropic hypogonadism reveal a key role for Neurokinin B in the central control of reproduction.

The timely secretion of gonadal sex steroids is essential for the initiation of puberty, the postpubertal maintenance of secondary sexual characteristics and the normal perinatal development of male external genitalia. Normal gonadal steroid production requires the actions of the pituitary-derived gonadotropins, luteinizing hormone and follicle-stimulating hormone. We report four human pedigrees with severe congenital gonadotropin deficiency and pubertal failure in which all affected individuals are homozygous for loss-of-function mutations in TAC3 (encoding Neurokinin B) or its receptor TACR3 (encoding NK3R). Neurokinin B, a member of the substance P-related tachykinin family, is known to be highly expressed in hypothalamic neurons that also express kisspeptin, a recently identified regulator of gonadotropin-releasing hormone secretion. These findings implicate Neurokinin B as a critical central regulator of human gonadal function and suggest new approaches to the pharmacological control of human reproduction and sex hormone-related diseases.

Forty month follow-up of persistent and difficultly controlled acromegalic patients treated with depot long acting somatostatin analog octreotide.

The objective of the present study was to investigate the effects of octreotide long acting release (S-LAR) preparation on GH and IGF-1 serum concentrations and pituitary tumor size in patients with persistent and difficultly controlled acromegaly even after adjuvant irradiation and/or dopamine agonists. Thirty-three patients with active acromegaly (26 female and 7 male, mean age; 43.94 +/- 14.01 SD years) were included in this study. Patients were evaluated at baseline and at 6, 12, 30 and 40 months for GH, IGF-1, and GH response to OGTT and biliary ultrasonography. Sella MRI was performed at initial and at 40 months. All patients received 20 mg S-LAR. Afterwards, the dosage was titrated to improve individual GH response and reduction of IGF-1 into normal ranges. Basal serum IGF-1 levels decreased from median: 530 microg/l [IQR: 420-600] to 340 microg/l [IQR: 230-460] at 6 months (p = 0.01), to 400 microg/l [IQR: 222.4-600] at 12 months (p = 0.48), to 396 microg/l [IQR: 318-468] at 30 months (p = 0.49), to 482 microg/l [308-580] at 40 months (p = 0.47). Nadir GH levels in OGTT fell from 2.70 ng/ml [IQR: 1.35-6.90] to 1.60 ng/ml [IQR: 0.36-4.10] at 6 months (p = 0.03), to 0.31 ng/ml [IQR: 0.18-0.65] at 12 months (p<0.0001), to 1.50 ng/ml [IQR: 0.83-4.00] at 30 months (p = 0.398) and to 0.89 ng/ml [IQR: 0.58-1.35] at 40 months (p<0.0001). Initially, pituitary adenoma volume was median: 1.18 ml [IQR: 0.08-3.50] and it shrank to 0.21 ml [IQR: 0-2.1] at 40 months (p = 0.08). Gallstones were detected in 12 patients and six of them underwent cholecystectomy. S-LAR is an effective treatment regimen in reducing GH and IGF-1 concentrations and as well as in shrinking tumor volume in persistent and difficultly controlled acromegalic patients.

Sexual dysfunction in women with hyperprolactinemia: a pilot study report.

PURPOSE: Hyperprolactinemia is a common hormonal disorder in women that may affect the phases of female sexual function (FSD). We investigated sexual function in patients with hyperprolactinemia. MATERIAL AND METHODS: A total of 25 women with primary hyperprolactinemia and 16 age matched voluntary healthy women who served as the as control group were evaluated with a detailed medical and sexual history, including a female sexual function index (FSFI) questionnaire and the Beck Depression Inventory. Serum prolactin, dehydroepiandrosterone sulfate, free testosterone, androstenedione, 17alpha-hydroxyprogesterone, estradiol, free thyroxin and thyrotropin were measured. These variables were compared statistically between the 2 groups. RESULTS: Except for prolactin serum hormone levels in women with hyperprolactinemia were not different from those in the control group. The median total FSFI score was 23.40 (IQR 17.70 to 27.30) in the hyperprolactinemic group, whereas healthy women had a median total FSFI score of 31.10 (IQR 27.55 to 32.88, p < 0.0001). FSD was diagnosed in 22 of 25 patients (88%), while 4 of 16 healthy women (25%) had FSD (p = 0.03). Desire (p = 0.001), arousal (p < 0.0001), lubrication (p = 0.001), orgasm (p = 0.001), satisfaction (p = 0.07) and pain (p = 0.003) domain scores were also significantly lower in women with hyperprolactinemia. Total FSFI (p = 0.009, r = -0.405), desire (p = 0.001, r = -0.512), arousal (p = 0.002, r = -0.466), orgasm (p = 0.026, r = 0.348) and satisfaction (p = 0.041, r = -0.320) scores negatively correlated with mean prolactin but not with the other hormones measured. CONCLUSIONS: A significant percent of women with hyperprolactinemia whom we evaluated had sexual dysfunction. No hormonal changes other than prolactin and no depression was found as a cause of FSD.