Ageing successfully with haemophilia: A multidisciplinary programme.

INTRODUCTION: Persons with haemophilia (PWH) born before the middle 1970s have spent a substantial part of their lives without the benefits of replacement therapy, that became available on a relative large scale only during the 1970s. As a consequence, this group of PWH, although still relatively young, suffers from musculoskeletal and functional problems that are typical of old people. METHODS: We report herewith the short-term results of a project based upon a multidisciplinary training programme led by a physiotherapist and an occupational therapist, that was implemented over a period of 12 months in 40 patients with severe or moderate hemophilia A or B born before the middle 1970s and regularly followed-up at a comprehensive haemophilia treatment centre in Italy. The project was aimed to provide information and skills in order to empower the older PWH carrying physical handicaps and functional limitations that had resulted from the inadequate management in their early ages, and to enable them to cope more efficiently with their crippling disease and prevent further disabilities. RESULTS AND CONCLUSIONS: The comparison of the data obtained before and after the 12-month programme found marginal improvements, but the purpose of this programme was indeed to offer a blueprint for the future. In this respect, the level of satisfaction for the programme was very high and we expect that it will be implemented long-term by our older PWH.

Severe spontaneous properitoneal hemorrhage following ventral hernia repair caused by viral infection: report of a case.

The authors report a case of severe spontaneous hemorrhage in a patient who underwent surgical repair of an upper umbilical ventral hernia with placement of a polypropylene mesh. On the third postoperative day the patient experienced bleeding in the properitoneal space (above and below the mesh), which spread up to and along the retroperitoneal area, causing severe hypovolemic shock. Postoperative investigations finally identified a virus as the cause of the complication.

Triple-layer Mesh Plasty for Re-recurrent Ventral Hernia in a Liver Transplant Patient: A Case Report.

Ventral hernias often occur in transplanted patients because of weakness of the abdominal wall, poor muscle mass, and ascitis. In this report we describe the case of a re-recurrent ventral hernia seen emergently in a liver transplant recipient, who was treated using a singular 3-layer approach by placement of an intraperitoneal mesh, stressing technical aspects of the plasty as well as the importance of a sublay technique in the reinforcement of a previous prosthetic plasty.

Evidence of liver histological alterations in apparently healthy individuals evaluated for living donor liver transplantation.

BACKGROUND: Living donor liver transplantation (LDLT) represents an important therapeutic option for patients with end-stage liver disease (ESLD). It has been reported that steatosis may be a serious problem in patients who donate a part of their liver. Liver biopsy represents an accepted method to assess the rate of steatosis and the possible risk to the donor. Nonetheless, some histological abnormalities have been documented in the specimens from potential donors. The aim of this study was to evaluate the possible hepatic histological alterations among apparently healthy candidates for liver donation who did not show serological or ultrasound (US) evidence. MATERIALS AND METHODS: From January 1, 2005 until October 15, 2006, we performed virological, biochemical, and tumor marker evaluations and liver biopsies on 20 LDLT donor candidates. At histological evaluation we classified the evidence of steatosis (5%-10% or 10%-20%), fibrosis (absent or 1-3 portal space), inflammation, iron deposition, biliary neoductulation, and portal vein vascular alterations. RESULTS: Among the 20 subjects, serological markers did not show any pathological alterations. At liver biopsy we found: steatosis (5%-10%) in 6 individuals (about 30%) with 1 ranging from 10% to 20%; iron deposition in 4 (20%); biliary neoductulation in 3 (about 16%); fibrosis in 4 (20%); inflammation in 5 (25%); and portal vein dilatation in 10 (50%). CONCLUSIONS: Our data showed that apparently healthy individuals who did not display serological markers or US evidence of pathology had liver histological abnormalities. This result suggested that in absence of clinical or laboratory alterations, liver biopsy may represent a useful diagnostic tool for living donor candidates. Long-term follow-up results for the laboratory data among those patients should be performed even though they were not qualified for LDLT.

Surgical treatment with Ligasure Precise of schwannoma of brachial plexus: case report.

English version We present a case of bulky schwannoma arising from the brachial plexus treated by a new surgical device. A 38-year-old man presented with a slow-growing left-sided supraclavicular mass and complained paresthesia of the third and forth fingers of the hand and forearm weakness. Physical examination revealed Tinel's sign. A CT-scan revealed a solid mass situated in the left profound supraclavicular fossa. The tumour was resected with the utilization of bipolar vessel sealing system (Ligasure Precise). This device is very useful in sutureless removal of masses localized in deep supraclavicular fossa. During the operation, care was taken to preserve the nerve function.

Breast surgery and sentinel node biopsy. Our experience.

Massive campaigns of screening of breast pathologies improved early diagnosis of breast cancers. Most of these cancers are small-sized (T1) and seldom show intraoperative nodal involvement. Sentinel node biopsy is the elective choice in the above mentioned cases because, if negative, it avoids axillary dissection. International literature reports rates of false negative sentinel node biopsy ranging among 4.5 and 12%; results in our experience account for almost 6%. As a consequence, there is the definite risk of potentially positive axilla that will not be dissected with subsequent risk of axillary recurrence within 24 months. The reason of that could be related to the fact that in Referral Centers this technique is performed in strictly selected patients, so as to gain a diagnostic accuracy of 98%. The rate of axillary recurrences does not justify the routine axillary dissection, since this is just a staging, not a therapeutic procedure. In case of doubt, it can be recommended a close and careful follow-up of the axilla.

[Thyroid microcarcinoma: our experience]. / Microcarcinoma tiroideo: nostra esperienza.

Differentiated thyroid cancer had an excellent prognosis; the diagnosis of sub-centimetric differentiated cancer (microcarcinoma) is more and more frequently. Clinical evolution of microcarcinoma could be various: microcarcinoma can represent an autoptic finding or can evolve with locoregional lymph node involvement or metastasis. Various clinical and genetic factors have been considered to predict the aggressiveness of this neoplasm. From 2001 to 2004, 74 patients with diagnosis of thyroid microcarcinoma underwent surgical treatment. By a retrospective survey we evaluated risk factors and the choice of surgical treatment. The aggressiveness appear to be related mainly to multifocality and size. Our therapeutic strategy is to perform total thyroidectomy for benign pathologies, total thyroidectomy with lymphadenectomy in cases with preoperative diagnosis of microcarcinoma.

[Male breast cancer. Five years experience and literature review]. / Il cancro della mammella maschile. La nostra esperienza di 5 anni e confronto con la letteratura internazionale.

Male breast cancer is an uncommon disease and its aetiology, clinical behaviour and treatment is not well-known . Retrospective studies show that age and stage-matched breast cancer in men and women are compared. Nevertheless, the poor mammary tissue in man allow a rapid local infiltration, late diagnosis and poor survival rate. The Authors report their five years experience and with a short literature review they summarise what is currently known about this uncommon neoplasm in terms of prognostic factors, therapy and survival.

Antifungal prophylaxis with liposomal amphotericin B and caspofungin in high-risk patients after liver transplantation: impact on fungal infections and immune system.

Antifungal prophylaxis may be required in high-risk patients undergoing liver transplantation and for that reason we aimed to verify its role and its related impact on the graft. From January 2006 throughout 2012, 250 liver transplants were evaluated and 54 patients identified as being at higher risk were randomly selected to undergo the following schedule: 28 patients received liposomal amphotericin B and 26 received caspofungin. We evaluated, throughout 12 months, renal and liver function tests, bacterial and fungal infection episodes, and intensive care unit (ICU) stay, as well as the Th1 and Th2 cytokine network. Differences were analyzed according to non-parametric tests (two-tailed p values). Neither of the groups showed episodes of invasive fungal infection during the 12 months follow-up; however, patients receiving prophylaxis with liposomal amphotericin B had reduced episodes of bacterial infections coupled with an improved immune system response compared with those receiving caspofungin. Finally, a reduced stay in the ICU was also observed. In conclusion, even if the results of liposomal amphotericin B and caspofungin prophylaxis strategies did not differ in terms of invasive fungal infection rate, patients receiving prophylaxis with liposomal amphotericin B had a reduced ICU stay and an improved Th2 status, as well as a reduced number of post-transplant bacterial infections. Further studies are required to better address and evaluate these findings.

Gastrointestinal tumours in elderly.

The Authors report their experience concerning 129 cases of gastrointestinal neoplasms (gastric, colonic, anorectal), recorded during the last years among patients aged between 70 and 81 years, who underwent radical surgery. The main issues evaluated were: anaesthesiological risk, stage, post-operative mortality and morbidity. Elderly seems not to be a contraindication, nor a limit for surgery if the patient is correctly and strictly managed pre and post-operatively.

[Bile leakage after laparoscopic cholecystectomy: report of three cases in our experience]. / Coleperitoneo dopo colecistectomia laparoscopica: descrizione di tre casi nella nostra esperienza.

Laparoscopic cholecystectomy (LC) is associated with an higher incidence of bile duct injury than open cholecystectomy. There has been concern and the reason why bile leakages are more frequent with LC is yet unclear. Bile collections can occurr because of major about (75%) or minor (about 25%) injuries. Anyway, external biliary decompression is a key factor in the treatment, in order to avoid an emergency intervention. The Authors report their experience concerning 3 cases of bile leakages among a series of 458 LC. Only in one of the three cases the subhepatic space was not drained and an emergency laparotomy was performed. The Authors, on the basis of their own experience and of the data reported in literature, suggest the routinary intraoperative placement of a drainage into the subhepatic space, to be removed, if no complications occur, one day after surgery.

Which therapy to prevent post-thyroidectomy hypocalcemia?

Hypocalcemia is one of the most frequent complications after total extracapsular thyroidectomy (TET). In most of cases it is a transient phenomenon. The aim of this study is to evaluate if and how the oral administration of calcium or calcium combined with D-vitamin could effectively prevent post-thyroidectomy hypocalcemia. A randomized prospective study was performed, recruiting 120 patients who underwent total thyroidectomy. The patients in our series were randomly assigned to one of two groups: group A--patients who received calcium lactogluconate/calcium carbonate (mg 300 per day); group B--patients who received calcium carbonate/cholecalciferol therapy (calcium carbonate: 1500 mg per day; cholecalciferol 400 UI per day). The groups were well matched for age, sex and pathologies. Patients of both A and B groups were divided in two subgroups: those operated on for benign thyroid diseases (A1 and B1) and those operated on for malignancy (A2, B2). Serum calcium assays, performed 24, 48 and 72 hours after surgery, showed mean values of calcemia higher in patients of the B1 and B2 group. Statistical analysis was performed using a Student's t test. Mean serum calcium concentrations on post-operative day one, two and three were higher in patients of the group B (p<<0.01). Early and combined oral administration of both calcium and vitamin D seemed to prove major efficacy in preventing and treating post-operative hypocalcemia, showing mean serum calcium levels higher than those of patients who received only oral calcium administration. Nevertheless, further studies are necessary to validate these data.

Major complications in thyroid surgery: utility of bipolar vessel sealing (Ligasure Precise).

In the present study the Authors tried and assess the advantages of the standard sutureless thyroidectomy performed by the Ligasure Vessel Sealing System, thanks to the use of the dedicated Precise handle. The Authors compared the efficacy of haemostasis and the economical impact of the device, in terms of drug administration and costs. The Authors comparatively analyzed 120 total extracapsular thyroidectomies (TET) performed by the standard operative technique (Group A, control) and 70 TET achieved by the "sutureless technique" (Group B, case). There was a statistically significant decrease of transient postoperative hypocalcemia (5.71% vs 7.5%) and also of mean operative time (about 20 minutes) in patients of the group B. Non significant decrease of other kind of complications (postoperative hemorrhage, transient and permanent inferior laryngeal palsy, stupor of the superior laryngeal nerve, seromas) were also observed. The use of the Ligasure Precise resulted easy, safe and efficient in the Authors' experience. It allowed the decrease of postoperative haemorrhages and mean operative time.

[Interstitial laser photocoagulation in liver tumours: our experience]. / Laserterapia interstiziale nell'epatocarcinoma su cirrosi: nostra esperienza.

Interstitial lasertherapy (ILT) is one of the percutaneous thermal procedures allowing the ablation of primary or secondary hepatic lesions. This technique can be performed without general anaesthesia, since the patient is completely painless, in spite of the high temperatures reached, because there are no sensitive fibers around the lesion. This study includes patients with small hepatocarcinomas (< 2 cm) non peripherically sited, far from the gallbladder and the hepatic hilum. Twenty patients suffering from 24 cirrhotic hepatocarcinomas (two patients showed two nodules) have been enrolled for the present study. Mean diameter of lesions was 1.8 cm (1.3-2 cm). One or more 21 G (length 20 cm) Chiba's needles were inserted under sonographic view into the lesions. The laser energy was switched on at 4 Watts for 4-6 minutes, until an amount of energy of 900-1800 Joule was reached. All the patients were discharged 48 hours after the procedure. Triphasic CT-scan with contrast were performed 7 to 14 days after ILT, in order to check its efficacy. CT-scan showed complete necrosis in all the nodules ranging from 1.5 to 3 cm. All the patients received only one IL T session, whose longest duration was 20 minutes. No major complications occurred. ILT of small hepatocarcinomas is a safe, efficient procedure, also feasible without anaesthesia. Differently from percutaneous radiofrequency, in fact, it shows the advantage of less pain, that can avoid the need for general anaesthesia.

Tropospheric ozone: respiratory effects and Australian air quality goals.

OBJECTIVE: To review the health effects of tropospheric ozone and discuss the implications for public health policy. DESIGN: Literature review and consultation with scientists in Australia and overseas. Papers in English or with English language abstracts were identified by Medline search from the international peer reviewed published reports. Those from the period 1980-93 were read systematically but selected earlier papers were also considered. Reports on ozone exposures were obtained from environmental agencies in the region. RESULTS: Exposure to ozone at concentrations below the current Australian air quality goal (0.12 ppm averaged over one hour) may cause impaired respiratory function. Inflammatory changes in the small airways and respiratory symptoms result from moderate to heavy exercise in the presence of ozone at levels of 0.08-0.12 ppm. The changes in respiratory function due to ozone are short lived, vary with the duration of exposure, may be modified by levels of other pollutants (such as sulphur dioxide and particulates), and differ appreciably between individuals. Bronchial lavage studies indicate that inflammation and other pathological changes may occur in the airways before reductions in air flow are detectable, and persist after respiratory function has returned to normal. It is not known whether exposures to ozone at low levels (0.08-0.12 ppm) cause lasting damage to the lung or, if such damage does occur, whether it is functionally significant. At present, it is not possible to identify confidently population subgroups with heightened susceptibility to ozone. People with asthma may be more susceptible to the effects of ozone than the general population but the evidence is not consistent. Recent reports suggest that ozone increases airway reactivity on subsequent challenge with allergens and other irritants. Animal studies are consistent with the findings in human populations. CONCLUSION: A new one hour air quality ozone goal of 0.08 ppm for Australia, and the introduction of a four hour goal of 0.06 ppm are recommended on health grounds.

Peripheral cholinesterase inhibition by occupational chlorpyrifos exposure in Australian termiticide applicators.

Occupational exposure to organophosphorus insecticides (OPs), such as chlorpyrifos, may be monitored by the measurement of the activity of peripheral cholinesterase (ChE) enzymes, including erythrocyte acetylcholinesterase (EAChE) and serum cholinesterase (SChE). Lymphocyte neuropathy target esterase (NTE) is thought to have potential as a predictor of organophosphate-induced delayed neuropathy (OPIDN). This paper describes work performed in 39 Australian pest control operators (PCOs) exposed to a termiticide containing chlorpyrifos, and 34 unexposed control subjects. EAChE activities in PCOs did not differ from those of unexposed control workers. Mean NTE activity was slightly higher in PCOs than in controls and mean SChE was 52% of control activity. These results indicate that exposure of Australian PCOs to termiticides containing chlorpyrifos may be monitored using SChE but not EAChE or NTE, and that workers in this industry have sufficiently high OP exposure to significantly depress SChE activity. SChE inhibition of 70-80% may be associated with symptoms. Although no current symptoms were reported to be associated with occupational OP exposure, these workers may be at increased risk of acute effects following inadvertent spills or self-contamination due to their background level of exposure to OPs. While it is preferable to compare ChE enzyme activities between pre- and post-exposure periods to evaluate OP-related effects in individuals, in some cases there is an absence of pre-exposure data. The results of this study suggest that a screening value for SChE of 550 nmol/min/ml in a single blood sample may be useful to identify potentially OP-exposed individuals in the Australian population. Australian control subjects were similar with respect to EAChE, but displayed activities of NTE and SChE approximately 50 and 23% lower than an unexposed UK reference group. While these comparisons are presently speculative, they suggest that there may be differences in SChE and NTE activities in control populations of the two countries. The routine treatment of Australian homes with termiticides containing OPs, or differences in the availability and use of domestic OP-containing insecticides may explain these population differences. Further work is required to examine whether these differences are real, and if so their likely cause.

[Efficacy of somatostatin analogs in the treatment of neuroendocrine tumours and paraneoplastic syndromes]. / Efficacia degli analoghi della somatostatina nel trattamento dei tumori neuroendocrini e delle sindromi paraneoplastiche.

BACKGROUND: The authors report their experience with octreotide LAR in the adjuvant treatment of 10 patients suffering from neuroendocrine tumours and paraneoplastic syndromes. METHODS: All patients were studied preoperatively using traditional radiology and scintigraphy using octreoscan. RESULTS: Octreoscan was positive in all cases for somatostatin receptors. All patients with neuroendocrine tumours underwent surgery of the primary neoplasm; treatment with somatostatin was commenced at a later stage. CONCLUSIONS: The authors' experience with octreotide LAR proved to be an efficacious means of controlling symptoms and stabilising disease without collateral effects. They conclude by affirming that octreotide LAR represents a valid therapeutic choice in the symptomatic treatment of neuroendocrine tumours.

[Carotid glomus tumours. Personal experience in the surgical treatment of chemodectoma]. / Tumori dei glomi carotidei. Nostra esperienza nel trattamento chirurgico dei chemodectomi.

The authors present 6 cases of carotid glomus tumours referred to their attention over the past 10 years. Their experience shows that colour ultrasonography (using both morphological and functional evaluation) is the diagnostic key for defining the treatment of paraganglioma. More recent experience highlights the role of 111In Pentreotride using a multidisciplinary therapeutic approach.

Laparoscopic repair of congenital diaphragmatic hernia with prosthesis: a case report.

We report here our experience in the treatment of a large congenital diaphragmatic hernia, an uncommon pathology, approachable by laparoscopy. The patient was a 33-year-old woman with trisomy 21 syndrome, who only complained of colicky abdominal pain and a cough for 7 months before the hospitalization. Thoracic and abdominal CT scans showed a large anteromedial diaphragmatic hernia with slippage of the colon into the mediastinum and posterior displacement of the cardiovascular structures. The patient underwent laparoscopic repair of the hernia. The colon was put back in the abdomen; the defect (8x4 cm) was repaired by a Composix mesh (PTFE-polypropylene), fixed to the diaphragm by nonabsorbable stitches and staples. The patient was discharged on the third postoperative day. The postoperative course was uneventful. Follow-up at 18 months didn't show any complications or recurrence. We believe laparoscopic repair of diaphragmatic hernia to be the elective surgical choice, because of its technical feasibility and certain intra- and postoperative advantages.

[Total implantable venous systems in day surgery]. / Sistemi venosi totalmente impiantabili in day surgery.

UNLABELLED: Total implantable venous system (port-cath)in day surgery. AIMS: Most of patients underwent to a total implantable venous system (port-cath) can be treated in day surgery. METHODS: Since January 2000 to March 2003 we have implanted 31 port-a-cath, preferring access in subclavia v. We've treated all patients in day surgery and we've performed a Rx-control with a medium follow up of two hour. RESULTS: We have not reported any early complication, only 3 late complications: a device malfunction, a "kinking" reservoir and an infection of the subcutaneous pocket. The last two complications have required the explantation of the device. In difficult catheterizations can be helpful to use a pediatric kit. The rules of an aseptic and antiseptic operative procedure and the utilization of a short term antibiotic prophylaxis can avoid infective complications. We have proposed, after the device implantation, cardioaspirina like preventive therapy of septic and no septic thrombosis. In the thrombotic occlusion of the catheter we propose the use of activase. In malfunction's prevention we propose a lateral placement (to the skin incision) of the subcutaneous pocket and a 45 degrees reservoir rotation. CONCLUSIONS: The total venous system (port-cath) implantation can be mostly performed in day surgery, improving the compliance of the patients with a low cost procedure.