Background and objectives: XX male syndrome is part of the disorders of sex development (DSD). The patients generally have normal external genitalia and discover their pathology in adulthood because of infertility. There are no guidelines regarding XX male syndrome, so the aim of our study was to evaluate the literature evidence in order to guide the physicians in the management of these type of patients. Materials and Methods: We performed a systematic review of the available literature in September 2018, using MEDLINE, Web of Science, Embase and Google Scholar database to search for all published studies regarding XX male syndrome according to PRISMA guidelines. The following search terms were used: “46 XX male”, “DSD”, “infertility”, “hypogonadism”. Results: After appropriate screening we selected 37 papers. Mean (SD) age was 33.14 (11.4) years. Hair distribution was normal in 29/39 patients (74.3%), gynecomastia was absent in 22/39 cases (56.4%), normal testes volume was reported in 0/14, penis size was normal in 26/32 cases (81.2%), pubic hair had a normal development in 6/7 patients (85.7%), normal erectile function was present in 27/30 cases (90%) and libido was preserved in 20/20 patients (100%). The data revealed the common presence of hypergonadotropic hypogonadism. All patients had a 46,XX karyotype. The sex-determining region Y (SRY) gene was detected in 51/57 cases. The position of the SRY was on the Xp in the 97% of the cases. Conclusions: An appropriate physical examination should include the evaluation of genitalia to detect cryptorchidism, hypospadias, penis size, and gynecomastia; it is important to use a validated questionnaire to evaluate erectile dysfunction, such as the International Index of Erectile Function (IIEF). Semen analysis is mandatory and so is the karyotype test. Abdominal ultrasound is useful in order to exclude residual Müllerian structures. Genetic and endocrine consultations are necessary to assess a possible hypergonadotropic hypogonadism. Testicular sperm extraction is not recommended, and adoption or in vitro fertilization with a sperm donor are fertility options.
A 46-year-old Caucasian male has been transferred to our urology department with a history of septic fever, uncompensated diabetes, pain, and scrotal swelling. On clinical examination, the left inguinal and scrotal area was swollen, tender, and painful; scrotal MR had been performed, showing the catheter tip in scrotal cavity and presence of gas. The case was diagnosed as scrotal abscess with urethroscrotal fistula. He was successfully treated with scrotal incision, drainage, catheter repositioning under fluoroscopic control, antibiotics, and insulin. This patient developed an infection of scrotum, which led to subcutaneous abscess getting worse by a poorly controlled glycemia. In this case, an iatrogenic fistula, caused by wrong catheterization, stops the evolving to a Fournier's Gangrene. Early detection and intervention provide opportunities to improve outcome of this disease.
Intravesical chemotherapy may cause chemical cystitis and related lower urinary tract symptoms (LUTS). The aims of this study were to evaluate the efficacy and safety of an oral preparation of hyaluronic acid (HA), chondroitin sulfate (CS), curcumin, and quercetin (Ialuril® Soft Gels) to reduce the severity of LUTS in patients with a history of bladder cancer (BCa) undergoing intravesical chemotherapy. We designed a monocentric, randomized, double-blind, placebo-controlled pilot trial. Patients referred to our institute between November 2016 and March 2018 were enrolled. All subjects had non-muscle-invasive BCa and received intravesical chemotherapy with mitomycin C (MMC). Patients were randomized 1:1 in two groups (intervention vs. control). All subjects underwent oral administration (Ialuril® Soft Gels or placebo) starting one week before the first weekly instillation and ending 30 days after the last one, subsequently starting one week before each monthly instillation and ending 14 days after it. International prostate symptom score (IPSS) and 0-100 visual analogue scale (VAS) were used to assess the efficacy of the treatment. Adverse events were also described. Patients were evaluated at baseline and after 1, 4, 7, and 13 months of intravesical chemotherapy. A total of 34 patients were enrolled. The median IPSS score was significantly lower in the intervention group compared to the control group at 4 (13 vs. 17 points; p = 0.038), 7 (10 vs. 18 points; p < 0.001), and 13 (10 vs. 17 points; p = 0.002) months. The median VAS score was significantly lower in the intervention group compared to the control group at 7 (22 vs. 37 points; p = 0.021) and 13 (20 vs. 35 points; p = 0.024) months. No AE specifically related to supplement or placebo was recorded. Oral formulation of HA, CS, quercetin, and curcumin could be an effective and safe supportive therapy against chemical cystitis in patients receiving intravesical chemotherapy for BCa.
No study has yet been done to evaluate topical alprostadil as a less invasive alternative vasoactive agent for Penile Dynamic Duplex Ultrasonography (PDDU) in the diagnosis of erectile dysfunction. The main aim of our study was to evaluate the usability and reliability of topical alprostadil for PDDU compared with standard intracavernous injection. A further objective was to determine the patients' preference between these two different approaches. During session A, patients received injection while during session B, they received topical alprostadil. Each patient underwent both sessions, 1 week apart from the other. A total of 80 patients were enrolled. After 20 min from drug administration, no significant difference was found between the two procedures in terms of peak systolic velocity and end‐diastolic velocity, while Erection Hardness Score was significantly higher with injection. Patients reported less pain/discomfort during the procedure in case of topical alprostadil use and an overall preference towards this examination modality. Topical alprostadil could represent a usable and reliable alternative to intracavernous injection for PDDU, with less discomfort and greater preference by patients.
Nephrolithiasis: how the laboratory of clinical pathology can play a pivotal role in the prevention of lithiasic events We here describe our 15-year experience and report both on methodological problems (proposing a new datasheet and biochemical techniques) and on organizational ones (we discuss our patient approach and useful reporting for the physician).
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