The challenge of kidney lesions in von Hippel-Lindau disease.
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Biomedical subjects
Publications and source records attributed to D Fontana.
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The authors report a rare case of littoral hemangioma of the spleen (LHS) accompanied by a revision of the literature on the argument. A male 65-year-old patient was referred to their attention with suspected ultrasonographic diagnosis of lymphoma with a splenic localisation. The complete CT diagnosis led to suspected splenic angioma. During surgery, anatomopathological analysis of the biopsy revealed LHS. The pathological anatomy showed lesions ranging in size from small foci to large nodules which almost completely replaced the splenic parenchyma. These areas were made up of vascular canals or axes that imitate splenic sinuses and have irregular lumen, often appearing as papillary projections and cyst-like spaces; they are bordered by high (cylindrical) endothelial cells that project into the vascular lumen and reveal hemophagocytosis; there is very little mitotic activity. The patient was discharged 7 days after surgery. The authors underline the extreme rarity of this neoplasm and the virtual absence of symptoms, although some cases report signs of hypersplenism, including platelet deficiency and anemia. The diagnostic iter must take care to exclude other pathologies affecting the spleen, including lymphoma, metastases and primary malignant splenic tumours. Lastly, a differential diagnosis must be made with the malignant variant, littoral hemangiosarcoma of the spleen.
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PURPOSE: Increasing evidences indicate that despite the osteoblastic nature of metastatic bone lesions due to prostate cancer osteolysis is a regular feature and may cause skeletal morbidity. This observation provides the rationale for the use of bisphosphonates for managing bone metastatic prostate cancer. MATERIALS AND METHODS: We reviewed the literature on the mechanisms by which prostate cancer affects bone cell function and disrupts physiological bone turnover. We also summarized the clinical results of bisphosphonate for treating bone pain in patients with prostate cancer. RESULTS: Metastatic prostate cancer in bone interferes with physiological bone remodeling by abnormal release of the hormones and paracrine factors physiologically involved in the modulation of osteoblastic and osteoclastic activity. Tumor induced bone formation and resorption develop within the same metastasis but excessive new bone is deposited away from bone resorption sites and does not contribute to bone strength. The increase in bone resorption may also be a generalized phenomenon that is most likely due to iatrogenic osteoporosis or related to hyperparathyroidism in response to the increased calcium demand. The bone resorption index in patients with bone metastatic prostate cancer correlates with bone pain and is an independent predictor of adverse skeletal events. However, small clinical studies of the efficacy of bisphosphonates for controlling bone pain in patients with prostate cancer show contradictory results. CONCLUSIONS: Improved understanding of the pathophysiology of prostate cancer induced metabolic bone disease implies that bisphosphonates may have a role in the treatment of this disorder. This issue is being addressed by large-scale ongoing randomized studies.
OBJECTIVES: To evaluate the effectiveness of medical therapy during watchful waiting in patients with distal ureter stones. METHODS: Ninety-six patients with radiopaque stones located in the distal tract of the ureter and with stone sizes of 1 cm or smaller were involved in the study. The patients were randomly divided into two groups. Group A (n = 48) received oral treatment with 30 mg of deflazacort daily (maximum 10 days) plus 30 mg of slow-release nifedipine daily (maximum 4 weeks). Group B (n = 48) underwent a wait-and-watch approach. Both groups of patients were allowed to use diclofenac on demand. Statistical analyses were carried out using Student's t test, the chi-square test, and Fisher's exact test. RESULTS: The average stone size was 5.8 +/- 1.8 mm for group A and 5. 5 +/- 1.4 mm for group B. No statistically significant difference was found in stone size. Stone expulsion was observed in 38 (79%) of 48 patients in group A and in 17 (35%) of 48 patients in group B. The average expulsion time was 7 days (range 2 to 10) for group A and 20 days (range 10 to 28) for group B. A statistically significant difference was observed in both the expulsion rate and the expulsion time (P <0.05). The mean amount of sodium diclofenac used was 15 mg per patient for group A and 105 mg per patient for group B (P <0.05). CONCLUSIONS: The medical treatment proved to be effective and safe, as demonstrated by the increased stone expulsion rate, decreased expulsion time, and reduced need for analgesic therapy.
BACKGROUND: Noradrenergic vascular hyper-responsiveness is a hallmark of essential hypertension. To evaluate whether nitric oxide plays a role in the enhanced vascular response to norepinephrine in hypertension, we examined 32 hypertensives and 28 normotensives who were distributed in 3 experimental series. METHODS AND RESULTS: In the first series, we measured the forearm blood flow (FBF) response to a norepinephrine infusion under control conditions and during the infusion of L-N-monomethylarginine (L-NMMA). Norepinephrine evoked dose-dependent vasoconstriction that was greater in hypertensives than in normotensives (maximum FBF, -61+/-1 versus -51+/-1%; P<0.01). During L-NMMA infusion, norepinephrine vasoconstriction was not modified in hypertensives; however, it was potentiated in normotensives (maximum FBF, -64+/-2%; P<0.01). In the second series, we tested whether norepinephrine vasoconstriction could be affected by an antioxidant such as ascorbic acid. Norepinephrine vasoconstriction was blunted by ascorbic acid administration only in hypertensives (maximum FBF, -49+/-3 versus -63+/-2%; P<0.01); the vasoconstriction became similar to that observed in normotensives. During ascorbic acid plus L-NMMA administration, the vascular response to norepinephrine increased to a similar extent in both study groups. To rule out the possibility that the effect of ascorbic acid on norepinephrine vasoconstriction could depend on adrenergic receptor-induced nitric oxide release, in the last series we inhibited endogenous nitric oxide and replaced it with an exogenous nitric oxide donor (sodium nitroprusside). Even in these conditions, ascorbic acid attenuated norepinephrine vasoconstriction only in hypertensives (maximum FBF, -50+/-2 versus -62+/-1%; P<0.01). CONCLUSIONS: Our data demonstrate that noradrenergic vascular hyper-responsiveness in hypertension is dependent on an impairment of nitric oxide activity that is realized through norepinephrine-induced oxygen free radical production.
Solvent extraction is a proven technology for the selective removal and recovery of metal ions from aqueous solutions: the use of aqueous biphase systems can be attractive for many separation processes. These systems have usually been used previously at temperatures around 25 degrees C; however it is possible that better separations may be achieved at other temperatures: phase diagrams have been determined for a polyethylene glycol (AMW 3350)-ammonium sulphate-water system over the temperature range 269-343 K and the effect of temperature on partitioning has been determined for 1,10-phenanthroline-copper(II) sulphate complex.
BACKGROUND: Circulating neuroendocrine markers were measured in patients with prostate carcinoma (PC), prostatic intraepithelial neoplasia (PIN), and benign prostatic hypertrophy (BPH) with the goal to: 1) evaluate the differences in the expression of these markers in patients with benign, premalignant, and primary or metastatic PC; 2) evaluate their prognostic significance; 3) compare values in patients with hormone-naive and hormone-refractory disease; and 4) assess changes after androgen deprivation or chemotherapy. METHODS: Serum neuron specific enolase (NSE) (immunoradiometric assay) and plasma chromogranin A (CgA) (enzyme-linked immunoadsorbent assay) were evaluated in 141 patients with BPH, 54 patients with PIN, and 159 patients with PC; 119 patients were bearing hormone-naive disease and 40 were bearing hormone-refractory disease. CgA was monitored in 31 patients submitted to androgen deprivation and in 24 patients receiving chemotherapy. RESULTS: Supranormal CgA was observed more frequently in patients with American Urologic Association (AUA) Stage D2 disease (45.5%) compared with those with Stage D1 disease (33.3%), Stage C disease (16.7%), Stage A/B disease (18.8%), PIN (25.9%), and BPH (17.0%) (P < 0.02). Supranormal NSE did not change in any of the patient subgroups. Elevated CgA was observed in 36.0% of patients with metastases who had hormone-naive disease and in 45.0% of patients with hormone-refractory disease (P value not significant). Supranormal NSE and CgA values were predictors for poor prognosis in patients with hormone-refractory disease. Elevated baseline CgA values decreased > 50% in 1 of 12 patients who received luteinizing hormone-releasing hormone analogs and in 2 of 12 patients who underwent chemotherapy. CONCLUSIONS: CgA appears to reflect the neuroendocrine activity of PC better than NSE. Elevated CgA values correlate with poor prognosis and are scarcely influenced by either endocrine therapy or chemotherapy.
OBJECTIVE: To compare the effects of rilmenidine with those of amlodipine on blood pressure, glucose metabolism, plasma lipid concentration and fibrinolysis parameters. DESIGN: A four-month randomized double-blind, parallel group study. PATIENTS AND METHODS: Obese hypertensive patients with hypertriglyceridaemia (> or = 2.3 mmol/l) and impaired glucose tolerance (OMS-ADA) were included (n = 52). A placebo run-in period of 2 weeks was followed by 4 months of double-blind treatment with either rilmenidine or amlodipine. Blood pressure was recorded using a mercury sphygmomanometer. Glucose metabolism was evaluated by an oral glucose tolerance test RESULTS: Of the 52 patients recruited, 47 (21 rilmenidine and 26 amlodipine) completed the 4-month treatment period. The intention-to-treat analysis showed a comparable reduction in systolic and diastolic blood pressure (SBP, DBP) with the two anti-hypertensive treatments (rilmenidine -13.9/-13.5 mmHg; amlodipine - 17.6/-15.0 mmHg). Insulin concentrations under basal conditions and 2 h after a standard oral glucose load did not change significantly after treatment in both groups. Plasma glucose under basal conditions and 2 h after a standard oral glucose load as well as the area under the plasma glucose concentration curve tended to decrease in the rilmenidine group and to increase in the amlodipine group so that the changes in these parameters were significantly different between the two study groups (P= 0.041, P = 0.042 and P = 0.015, respectively). Plasminogen activator inhibitor type 1 (PAI-1) antigen and PAI-1 activity were only decreased in the rilmenidine group (not statistically significant). CONCLUSION: Our results demonstrate that rilmenidine and amlodipine have a comparable anti-hypertensive effect but only rilmenidine is able to improve glucose metabolism.
PURPOSE: We evaluated the incidence of skeletal complications in patients with bone metastatic prostate cancer and hormone refractory disease. We also assessed the predictive role of bone turnover markers determined at baseline. MATERIALS AND METHODS: A total of 112 patients were consecutively enrolled in our study from July 1990 to July 1998 and followed until death or the last followup. Bone pain, disease extent in bone, serum prostate specific antigen, hemoglobin, and a panel of bone formation and resorption markers were assessed at baseline before any second line treatment. RESULTS: Skeletal complications in 34 patients (30.3%, estimated yearly incidence 12.3%) involved vertebral deformity or collapse requiring spinal orthosis in 20 (17.9%), spinal cord compression in 7 (6.2%), pathological bone fracture in 10 (8.9%), symptomatic hypercalcemia in 1 (0.9%) and symptomatic hypocalcemia in 1 (0.9%). Median time to the evidence of the initial skeletal complication was 9.5 months. These adverse events did not influence overall survival. At baseline patients with eventual skeletal complications had greater bone pain (p = 0.02), a heavier tumor load in bone (p = 0.005), lower performance status (p = 0.05), and higher serum alkaline phosphatase (p <0.02) and urinary deoxypyridoline (p <0.05) than their counterparts. Multivariate analysis revealed that only urinary deoxypyridinoline was independently associated with the onset of these events (p <0.02). The scatterplot of urinary deoxypyridinoline values in patients with and without skeletal complications enabled us to detect a cutoff of 38 pM./mM. for predicting 51% of skeletal events with only an 8% false-positive rate. CONCLUSIONS: Skeletal complications are common in patients with prostate cancer and hormone refractory disease. Bone loss is the major cause of onset. Baseline deoxypyridinoline at the cutoff point noted had moderate sensitivity but high specificity for predicting these adverse skeletal events.
Pelvic lymphoceles, occurred in patients undergone radical retropubic prostatectomy, are believed to increase the risk of deep venous thrombosis and so thromboembolic complications. The authors' aim is to evaluate the usefulness of pelvic ultrasonography in the diagnosis and in the possible early treatment of pelvic lymphoceles. Fifty-eight patients undergone radical prostatectomy and pelvic lymphadenectomy, between January '98 and December '99, underwent a pelvic ultrasonography in VII post-operative day. When a symptomatic or large (over 5 cm) lymphocele was found, it was treated with ultrasound (US) guided drainage. In the study, the following parameters were considered: lymphocele occurrence, size, location and treatment and thromboembolic complication. Statistical analysis was carried out with Fisher's exact test. Pelvic US showed a lymphocele in 23 out of 58 (40%) patients. Mean size was 5 cm. Deep venous thrombosis occurred in 4 patients, all with pelvic lymphoceles. No thromboembolic complications were recorded. In our experience, pelvic ultrasound has shown to be an easy e reliable tool for diagnosis and percutaneous treatment of pelvic lymphoceles.
OBJECTIVES: This study sought to show the effectiveness and safety of three repeated alcohol injections for the treatment of simple large renal cysts. METHODS: From September 1991 to December 1997 we treated 72 renal cysts. The cyst was drained with an 8F mono J stent. Ninety-five percent sterile ethanol was injected into the cyst and left in place for 20 minutes. Two repeat alcohol injections were performed every 24 hours. After the third alcohol injection, the catheter was removed. To avoid pain in the last 39 patients, 20 mL of 2% lidocaine hydrochloride was injected into the cyst for 15 minutes before the alcohol injections. RESULTS: The follow-up period ranged from 8 to 83 months (mean 48). One patient underwent surgical intervention because of considerable bleeding in the cystic cavity that occurred after percutaneous drainage. In 1 patient in whom the cystic cavity communicated with the urinary tract, no alcohol injections were performed. After the repeated alcohol injections, the cystic cavity completely disappeared in 68 of the 70 treated cysts at first ultrasound examination. This result, observed at the first ultrasound control observation, remained unchanged during follow-up. CONCLUSIONS: In our experience, percutaneous drainage with three repeated alcohol injections offers a high rate of success without the cost and morbidity associated with other procedures, such as operation or laparoscopy.
OBJECTIVES: The incidental discovery of an adrenal mass has become a frequent finding because of the increased use and technical improvement of computed tomography (CT) and magnetic resonance imaging. The approach to the investigation of these masses is ill-defined, and unequivocal guidelines for their management are lacking. The first problem concerns the distinction between malignant masses requiring surgery and the more frequent benign masses. In the case of a benign mass, an additional problem is the method of follow-up, considering cost, discomfort to the patient, and the consequences of false-positive results. The aim of this study was to evaluate the possible role of ultrasonography (US) in the follow-up of incidentally discovered adrenal masses. METHODS: Two hundred eight cases of adrenal incidentalomas consecutively diagnosed in Piedmont, Italy from 1989 to 1996 and collected for a retrospective multicenter analysis were used. The US and CT characteristics of the adrenal masses were compared in patients who underwent surgery. RESULTS: Most patients were 50 to 69 years of age; women were predominantly affected. CT was more reliable than US in detecting the malignant nature of an adrenal lesion. The mass diameter measured by US and CT was clearly correlated. CONCLUSIONS: At diagnosis, US was not sufficiently reliable in evaluating adrenal mass characteristics. Considering the high correlation between US and CT size estimation, in the case of a presumably benign lesion, US could be considered a simple, economic, and effective method of follow-up, with CT limited to evaluating masses growing over time (CT remains mandatory at diagnosis).
PURPOSE: Previous in vitro investigations recorded an inhibition of cell proliferation by BCG when added to different cell cultures. The induction of apoptosis by BCG is controversial. Our study aimed to evaluate the influence of BCG on the expression of tumor suppressing proteins p53 and p21Waf1-Cip1 and apoptosis of the urothelial cells in vivo. MATERIALS AND METHODS: Twenty-one cases of superficial bladder cancer, treated with TUR and subsequent intravesical BCG, were studied retrospectively. The assays evaluated the expression of p53 and p21Waf1-Cip1 by immunochemistry (IHC), and the presence of apoptosis by TUNEL assay. The estimates were performed, in each case, on the following specimens: one tumor sample and one non-neoplastic sample collected during the TUR which preceded the administration of BCG; one non-neoplastic sample collected 3 months after the diagnosis; and one non-neoplastic sample collected in the first 2 weeks after the completion of the treatment. Samples of 6 cancer recurrences detected during BCG were examined too. RESULTS: As usual for non-neoplastic urothelium, the pre-BCG samples displayed poor p53 and p21Waf1-Cip1 immunoreactivity. By contrast, the samples collected during and in the aftermath of BCG showed an overall increase of the expression of both proteins. The rare occurrence of apoptosis proved to be chronologically unrelated to the BCG treatment. DISCUSSION: The relationship between changes of the IHC features and BCG suggests that BCG, at least under some circumstances, can induce the activation of wild type p53 and p21Waf1-Cip1 in the urothelium. The mechanism of the BCG-p53 status interaction and its role in the antitumor activity of BCG remain to be clarified.
The current investigation aimed to check the effectiveness of transvaginal ultrasonography in the diagnosis of organic urethral diseases, comparing its results with those of conventional examinations (physical examination, voiding cystourethrography, pelvic ultrasonography, cystourethroscopy). Transvaginal ultrasonography was performed in 560 female patients with recurrent cystitis, dysuria, or palpable masses and diagnosed the following urethral diseases: 25 diverticula, seven stenoses, three carcinomas, two leiomyomas of periurethral tissue, and one incomplete duplex urethra. In our study transvaginal ultrasonography proved to be the most reliable diagnostic tool among imaging methods used.
PURPOSE: To provide preliminary data on whether the diagnostic role of serum prostate specific antigen (PSA) in assessing the response to treatment is improved by concomitant free PSA evaluation both markers were evaluated in 42 patients with advanced prostate cancer who received hormonal therapy and 57 with hormone refractory disease who received chemotherapy. MATERIALS AND METHODS: PSA was assessed at baseline and every 3 months during treatment. Free PSA was assessed in stored serum samples obtained at baseline and at maximum PSA decrease. Free PSA was not measurable in 17 patients who received androgen deprivation (40.5%) and 2 who received chemotherapy (3.5%) because it was less than 1.5 ng./ml. RESULTS: Of the 21 patients with greater than 50% PSA decrease after hormonal therapy free-to-total PSA increased in 12 (57.2%) and decreased in 9 (42.9%). Of the 20 patients with PSA response after chemotherapy free-to-total PSA increased in 18 (90.0%) and decreased in 2 (10.0%). Free-to-total PSA increased in 12 of the 20 patients (60.0%) with PSA stabilization after chemotherapy. Patients with an increase in free-to-total PSA after chemotherapy had greater survival compared to those with a decrease or no change (19.8 versus 15.5 months, respectively, p <0.03). CONCLUSIONS: These data suggest that an effective cytotoxic regimen mainly affects the protein bound PSA fraction. The absence of a clear predominant pattern of free-to-total PSA in patients with PSA response to hormonal therapy and the high percentage of hormone sensitive patients in whom free PSA was not assessable at maximum PSA decrease suggest that free PSA evaluation is less useful in prostate cancer patients undergoing androgen deprivation.
We describe a congenital bilateral absence of the vas deferens (CBAVD) patient with a compound heterozygosity in the cystic fibrosis transmembrane regulator (CFTR) gene for a stop mutation W1282X and a new missense mutation P499A. The P499A is interpreted as a mild mutation whose phenotypic effects, in this case limited to the development of wolffian duct derivatives, are revealed only in combination with a severe CFTR mutation.
The rising interest on male genital aesthetics increased the number of patients complaining of short penis. therefore, the andrologist must be able to perform a correct diagnostic assessment and to make the correct therapeutical choice as well, even if there's no standard yet. The aim of this paper is to discuss some diagnostic troubles about penile shortness and, in particular, to explain the basic surgical strategies suitable for the treatment of "hidden penis".