Supra-ampullar cystectomy and ileal neobladder

OBJECTIVE: This article describes both the open and laparoscopic operative techniques of supra-ampullar cystectomy (SAC). METHODS: Both open (photographs and drawings) and laparoscopic (attached DVD) SAC are explained step by step. RESULTS: Between May 1984 and December 2005, 31 patients with bladder tumour underwent SAC with ileal orthotopic neobladder (2 Camey I, 26 Camey II, and 3 Y). Three patients underwent laparoscopy. Preoperatively, 26 patients had superficial high-risk transitional cell carcinoma (TCC). Median follow-up was 95.0 mo (range: 5-260 mo). The 10-yr cause-specific survival rate was 76.7%. Two patients had local recurrence. Potency was preserved in 28 patients (90.3%); 15 patients (48.3%) also maintained antegrade ejaculation, allowing procreation in 3 cases. In one patient the Camey I neobladder was converted into an ileal conduit (high postvoid residual, recurrent pyelonephritis). None of the remaining patients had daytime incontinence, eight had nightime urinary incontinence, and six performed intermittent self-catheterisation. CONCLUSION: SAC with detubularised ileal orthotopic neobladder allows preservation of sexual function and maintenance of urinary continence in most patients, without compromising oncologic outcome. The key element is the very strict and careful preoperative selection of the patients.     

Diagnostic-therapeutic pathways as instruments of clinical management: experience at the S. Maria Hospital in Terni

Diagnostic-therapeutic itineraries and treatment profiles are instruments of clinical management. The authors report on their experience with the experimental creation of such itineraries in thyroid nodular pathology. These are the fruit of collaboration between the management team, endocrinological surgeons, and the hospital computer staff. The drawing-up of guidelines in the hospital setting allows the systematic organisation of clinical activities to be accomplished in the health-care facility, quantifying costs for all diseases in order to be able to plan and implement changes in resources and staff utilisation. Application of the method, in addition, helps to develop a common language among hospital doctors and nurses, facilitates proper communication with patients, and ensures adequate patient information regarding the clinical itinerary the patient will have to take for his or her condition.     

Epidemiological aspects of ovarian malignancies in North Sardinia in the period 1992-2001

Malignant ovarian tumors have been continuously increasing in Western countries and represent the leading cause of death for gynecological cancer. In fact, the mortality for malignant ovarian tumors remains very high with a low percentage of 5-year survival in the advanced stage of disease. The aim of this study was to evaluate the incidence trend and epidemiological characteristics of malignant ovarian tumors in the Province of Sassari, Sardinia (Italy) in the period 1992-2001 and to report the variations in comparison to the 1974-1985 period. The analysis of our data regarding the period 1992-2001, if compared with those of the period 1974-85, showed an increase of malignant ovarian tumors which triplicated achieving an incidence of 11.99/100,000 vs 4.27/100,000. The analysis of our epidemiologic data showed an increase of the age of first diagnosis (mean 60.9 years for epithelial ovarian tumors), the occurrence of the cancer in women at low socio-economic levels and a family history of cancer among the patients with malignant ovarian tumors. These data suggest that both local environmental factors combined with genetic characteristics play a role in the pathogenesis of ovarian tumors. The genetic characteristics could be of particular interest because Sardinia has been through the centuries a geographical area with little population migration. The marked increase in the incidence of ovarian tumors in the last several years points out the need to organize systematic screening by ultrasonography in our population.     

Unusual Pattern of Inheritance of Haemoglobin-H Disease

A 35-year-old Sardinian woman with haemoglobin-H disease was found to have normal parents. This case with an unusual inheritance pattern is described, and its possible relevance for the current concepts of haemoglobin-H disease genetics is discussed.     

Tooth wear in the mixed dentition: a comparative study between children born in the 1950s and the 1990s

The aim of this study was to evaluate the degree of tooth wear in posterior deciduous teeth of 100 subjects in the second phase of mixed dentition who were born in the 1950s (50sG) and 100 subjects born in the 1990s (90sG). The degree of abrasion for each posterior deciduous tooth was scored ranging from 0 to 3. The comparison of the degree of abrasion showed significant differences between the two groups for all examined teeth (upper and lower deciduous canines and first and second primary molars) all of which appeared to be more abraded in the 50sG. The findings of the present study indicate that subjects who were born in the 1950s exhibited a significantly greater degree of tooth wear on the posterior deciduous teeth when compared with contemporary subjects, along with a well-recognized lower prevalence of malocclusions. Changes in dietary habits and a diet based on processed foods may be postulated as factors involved with a decrease of dental attrition in contemporary populations. An increase in the prevalence of mouth breathing (tested in the present study) appears to be associated with a decrease in tooth wear in the contemporary population as well.     

Cannabinoid CB(1) antagonist SR 141716A attenuates reinstatement of heroin self-administration in heroin-abstinent rats

Rats with a previous history of heroin self-administration were studied to assess interactions occurring between cannabinoids and opioids in an animal model of reinstatement of heroin-seeking behaviour. Rats were trained to self-administer heroin and after a long-term extinction were primed with one of the following non-contingent non-reinforced drug administrations: saline (or vehicle), heroin, synthetic cannabinoid CB₁ receptor agonists (WIN 55,212-2 or CP 55,940), opioid antagonist (naloxone) or CB₁ antagonist (SR 141716A), alone or in combination. After primings, lever-pressing activity was recorded and compared to those observed during previous phases of training and extinction. Results of this study showed that (i) priming injections of heroin (0.1 mg/kg) as well as CB₁ agonists WIN 55,212-2 (0.15 or 0.30 mg/kg) and CP 55,940 (0.05 or 0.1 mg/kg) completely restore heroin-seeking behaviour; (ii) primings of naloxone (1 mg/kg) and SR 141716A (0.3 mg/kg) had no effect when administered alone; (iii) heroin-induced reinstatement was fully prevented by pre-treatment with either naloxone or SR 141716A; (iv) pre-treatment with SR 141716A significantly reduced WIN 55,212-2 and CP 55,940 priming effects. These results suggest that cannabinoid CB₁ receptors play an important role in the mechanisms underlying relapse to heroin-seeking and depict CB₁ antagonists as possible therapeutic agents for use in the prevention of relapse to heroin abuse.     

Tabletted microspheres containing Cynara scolymus (var. Spinoso sardo) extract for the preparation of controlled release nutraceutical matrices

Controlled release dosage forms based on tabletted microspheres containing fresh artichoke Cynara scolymus extract were performed for the oral administration of a nutritional supplement. Microspheres were prepared using a spray-drying technique; lactose or hypromellose have been chosen as excipients. Microspheres were characterized in terms of encapsulated extract content, size and morphology. Qualitative and quantitative composition of the extract before and after the spray process was determined. Compressed matrices (tablets) were prepared by direct compression of the spray-dried microspheres. In vitro release tests of microparticles and tablets prepared were carried out in both acidic and neutral media. Spray-drying is a good method to prepare microspheres containing the artichoke extract. The microspheres encapsulate an amount of extract close to the theoretical value. Particle size analyses indicate that the microparticles have dvs of approximately 6-7 microm. Electronic microscopy observations reveal that particles based on lactose have spherical shape and particles containing hypromellose are almost collapsed. The hydroalcoholic extract is stable to the microsphere production process: its polyphenolic composition (qualitative and quantitative) did not change after spraying. In vitro release studies show that microparticles characterized by a quick polyphenolic release both in acidic and neutral media due to the high water solubility of the carrier lactose. On the contrary, microspheres based hypromellose release only 20% of the loaded extract at pH 1.2 in 2 h and the total amount of polyphenols is released only after about further 6 h at pH 6.8. Matrices prepared tabletting lactose microspheres and hypromellose microparticles in the weight ratio 1:1 show a slow release rate, that lasts approximately 24 h. This one-a-day sustained release formulation containing Cynara scolymus extract could be proposed as a nutraceutical controlled release dosage form for oral administration.     

Genetics of SCID

Human SCID (Severe Combined Immunodeficiency) is a prenatal disorder of T lymphocyte development, that depends on the expression of numerous genes. The knowledge of the genetic basis of SCID is essential for diagnosis (e.g., clinical phenotype, lymphocyte profile) and treatment (e.g., use and type of pre-hematopoietic stem cell transplant conditioning). Over the last years novel genetic defects causing SCID have been discovered, and the molecular and immunological mechanisms of SCID have been better characterized. Distinct forms of SCID show both common and peculiar (e.g., absence or presence of nonimmunological features) aspects, and they are currently classified into six groups according to prevalent pathophysiological mechanisms: impaired cytokine-mediated signaling; pre-T cell receptor defects; increased lymphocyte apoptosis; defects in thymus embryogenesis; impaired calcium flux; other mechanisms. This review is the updated, extended and largely modified translation of the article "Cossu F: Le basi genetiche delle SCID ", originally published in Italian language in the journal " Prospettive in Pediatria " 2009, 156: 228-238.