Von Willebrand factor propeptide makes it easy to identify the shorter Von Willebrand factor survival in patients with type 1 and type Vicenza von Willebrand disease.

Reduced von Willebrand factor (VWF) half-life has been suggested as a new pathogenic mechanism in von Willebrand disease (VWD). The usefulness of VWF propeptide (VWFpp) in exploring VWF half-life was assessed in 22 type 1 and 14 type Vicenza VWD patients, and in 30 normal subjects, by comparing the findings on post-Desmopressin (DDAVP) VWF t(1/2) elimination (t(1/2el)). The VWFpp/VWF antigen ratio (VWFpp ratio) was dramatically increased in type Vicenza VWD (13.02 +/- 0.49) when compared to normal subjects (1.45 +/- 0.06), whereas it appeared to be normal in all type 1 VWD patients (1.56 +/- 0.7), except for the four carrying the C1130F mutation (4.69 +/- 0.67). A very short VWF t(1/2el) was found in type Vicenza VWD (1.3 +/- 0.2 h), while all type 1 VWD patients had a t(1/2el) similar to that of the controls (11.6 +/- 1.4 and 15.4 +/- 2.5 h respectively), except for the four patients carrying the C1130F mutation, who had a significantly shorter VWF survival (4.1 +/- 0.2 h). A significant inverse correlation emerged between VWFpp ratio and VWF t(1/2el) in both VWD patients and normal subjects. The VWFpp ratio thus seemed very useful for distinguishing between type 1 VWD cases with a normal and a reduced VWF survival, as well as for identifying type Vicenza VWD.

Identifying carriers of type 2N von Willebrand disease: procedures and significance.

The defective FVIII carrier function of von Willebrand factor (VWF) identifies type 2N von Willebrand disease (VWD), a variant with a pattern resembling hemophilia A. Type 2N characterization is based on the evaluation of the capacity of VWF to bind exogenous FVIII (VWF:FVIIIB). Here we report on a retrospective evaluation of hemostatic laboratory parameters most useful in detecting type 2N carriers. The diagnostic capacity of aPTT, FVIII, VWF:Ag, FVIII/VWF:Ag ratio, VWF:FVIIIB and VWF:FVIIIB/VWF:Ag ratio was evaluated in 21 type 2N VWD carriers. Twenty subjects were heterozygous for the R854Q mutation, one was heterozygous for the R760C missense mutation, which interferes with cleavage of the VWF propeptide. We found that prolongation of aPTT and decrease in FVIII and FVIII/VWF:Ag ratio were not frequent findings in type 2N carriers. The same was true for VWF:FVIIIB which was not always abnormal. On the contrary, VWF:FVIIIB/VWF:Ag ratio was always defective and its values were not related with FVIII and FVIII/VWF:Ag ratio or influenced by plasma VWF concentration. Given these results, we attribute the greatest significance to VWF:FVIIIB/VWF:Ag ratio in the diagnosis of type 2N defects, and only search for type 2N mutations, to validate the diagnosis, if the ratio proves abnormal.

Diagnosis and follow-up of thrombotic thrombocytopenic purpura by means of von Willebrand factor collagen binding assay.

Thrombotic thrombocytopenic purpura (TTP) is characterized by intravascular thrombosis leading to consumption of large or unusually large von Willebrand factor (VWF) multimers. The usefulness of VWF collagen binding (VWF:CB) assay was assessed in detecting the decrease/absence of large VWF multimers or the presence of abnormally large forms in patients with TTP. Nine patients with TTP were studied during the acute phase of the disorder and the absence of large VWF multimers was demonstrated by means of the VWF:CB assay. These findings were confirmed by VWF multimer pattern analysis; VWF:CB deficiency appeared to correlate with abnormalities in large VWF multimers. The diagnostic potency of VWF:CB was especially evident when the values were expressed as VWF:CB/VWF:Ag ratio. VWF:CB was also used during the follow-up of the disorder to document improvement or restoration of large VWF multimers. VWF:CB was able to detect the absence or decrease of large VWF multimers better than VWF ristocetin cofactor (VWF:RCo); in fact, VWF:CB was defective when large VWF multimers persisted to be decreased, in contrast with what observed with VWF:RCo. In conclusion, VWF:CB is a simple test that appears to be useful, together with clinical symptoms and reduced platelet count, for the diagnosis and follow-up of TTP.

Transsternal approach to closure of bronchopleural fistulas after pneumonectomy. A fifteen cases report.

A treatment method for main bronchus fistula after pneumonectomy via median sternotomy was described by P. Abruzzini in 1961. This operation is performed in an area not involved with infection. Fifteen patients underwent the procedure in our surgical department; one of them died of myocardial infarction while all the others survived for different periods of time, closely associated with the original disease; seven were long-term survivors. The transmediastinal approach seems an effective means of managing such a difficult complication.

Inguinal hernioplasty: the gold standard of hernia repair.

On the basis of a critical review of 936 inguinal hernioplasties performed in 8 years, the authors present their good long-term results with tension-free techniques including the original Lichtenstein technique, Lichtenstein with a sutured mesh and annulorrhaphy of the deep inguinal ring, and Lichtenstein with plug. The only two recurrences in this case series occurred with the original technique. Suturing of the mesh and deep inguinal ring annulorrhaphy proved to be reliable and inexpensive. Results were equivalent with the use of the plug, despite the presence of an additional foreign body. In conclusion, the results obtained with the three variants are practically equivalent, and the choice of technique may depend on the preference and experience of the surgeon.

Acute idiopathic pancreatitis: clinical and diagnostic contribution.

Acute idiopathic pancreatitis is a term used when no underlying cause can be identified on routine investigations. However more specialized investigations, such as endoscopic, endoscopic retrocolangio pancreopathy (CPRE), may detect aetiological factors, particularly biliary sludge and pancreatic duct abnormalities. The authors, reviewing the current literature, report their caseload of idiopathic pancreatitis. They conclude that CPRE is indicated if ultrasonography shows a calibre of the common duct at the superior limits of normal values and in each idiopathic recurrent AP.

Neurinomas of the brachial plexus: case report.

Neurinomas, also referred to as neurilemmomas and schwannomas, are rare benign tumours of the peripheral nerves, a low proportion of which arise from the brachial plexus. Authors report a case of an ancient schwannoma arising from the brachial plexus. The tumour, usually asymptomatic, may cause sensory radicular symptoms, or rarely motor deficits in the involved arm. Enucleation of the tumour from the nerve without damage to any of the fascicles is the correct treatment.

Malignant tumours of the small intestine: a case of jejunal adenocarcinoma.

The Authors analyse the main problems concerning malignant tumours of the small intestine. They report a case of jejunal adenocarcinoma recently observed. They emphasise the importance of surgery, both diagnostic and therapeutic, even in advanced stages, for the treatment of this neoplasm.

Papillomatosis and breast cancer: a case report and a review of the literature.

Papillomatosis is a relatively common (22%) benign microscopic lesion in the breast and rarely seen in women less than 30 years old. It is a papillary proliferation of the ductal epithelium which partly fills up smaller ducts and to degree distends them. The histological classification of this entity is controversial because similar or identical lesions have been classified using different terms such as epitheliosis and epithelial hyperplasia, and interpretation of published series has been difficult due to imprecise definition of this term. Clinical, radiological and histological patterns of this entity are often sufficient to raise concern as to possible malignancy. Moderate or florid hyperplasia without atypia is considered to carry slight (1,5-2 times) increase in risk of later developing cancer, while in the atypical hyperplasia the risk is four to five times that of the general population. The authors describe a case of papillonlatosis recentely observed in a 67 years old female patient and, confirmed the importance to establish an accurate preoperative diagnosis. It is important that the surgeon works with the pathologist to produce clear descriptive report of epithelial changes from normal through hyperplasia to atypias in order to establish a precise surgical indication.

Seat-belt injuries to the supra-aortic trunks.

Five seat-belt related injuries to the supra-aortic and cervical vessels occurring in four patients are reported. All patients but one were symptomatic, none presented with stroke, and in one case arterial lesion was revealed by delayed onset of symptoms. Outcome of surgical treatment was overall good. Systematic search of such injuries and surgical repair of those without major neurological signs might be advisable.

Biofragmentable anastomosis ring (BAR) in intestinal surgery: review of 21 cases and evaluation of postoperative morbidity.

The authors report their experience with the use of biofragmentable anastomosis ring in intestinal anastomosis: 21 patients underwent intestinal resection and anastomosis by BAR. There was no operative mortality. One patient developed an anastomotic leak and three patients had symptoms of intestinal obstruction. The endoscopic follow up showed no late complications. Technical factors regarding the BAR are discussed. Advantages appear to be a safe, rapid and easy alternative to hand and stapled intraperitoneal large bowel anastomosis.

Local anaesthesia with eutectic cream of lidocaine and prilocaine for treatment of cicatrizial phimosis in outpatients.

The authors report their experience in ambulatory treatment of cicatrizial phimosis in outpatients, by using local anaesthesia with eutectic cream of lidocaine and prilocaine. This preliminary study shows that preparation seems to be effective and well tolerated. To the aim of getting a good analgesic result the most important aspects are a careful application of the cream all over the concerned area and an occlusive bandage for 60-90 minutes at least.

Metastasis to the breast of a renal carcinoma: a clinical case.

The breast is an unusual site for metastatic disease, and generally the lesion appears same times after the diagnosis of the primary malignancy. Authors report a case of a breast metastasis from a hypernephroma, six years after nefrectomy.

Carcinoid to cecal bowel localization: a case report and literature review.

The authors report a case of cecal malignant carcinoid, diagnosticated in a 68 years old male patient. Carcinoid tumours are rare neoplasm and their behaviour seems to change with the localization. Cecal carcinoid represents only 2-3% of gastrointestinal carcinoid. Some times it is discovered incidentally at autopsy, surgery or endoscopy. The aim of this paper has been to review, in literature, the frequency, treatment and prognosis of this neoplasm.

[Pulmonary carcinoids. Analysis of 53 cases]. / Carcinoidi polmonari. Analisi di 53 casi.

We have performed a retrospective analysis of 53 cases of bronchial carcinoids using our own patient data from more than 4700 lung tumors and 1632 resections. The male/female ratio was 1:12 (28/25) and the age range 13 to 75 years (mean 52.2). Fifty-three tumors resections of varying extent were performed, including one radical pneumonectomy in a patient who had previously undergone a lobectomy, and one limited resection of the main left bronchus; there was no intraoperative mortality. After histological examination, 44 tumors (83%) were found to be typical carcinoids and nine (17%) atypical carcinoids. The median follow-up period was 4.56 years, with a range from 1 to 10 years. Only one patient with an atypical carcinoid tumor had a relapse and died three years after, while another patient underwent surgery of the contralateral lung for a second primary lung cancer (SPLC). On the basis of these observations we would like to underline the importance of an accurate histopathological classification for both therapeutic and prognostic purposes; given the higher aggressiveness of atypical carcinoids, these tumors would be eligible for a therapeutic approach analogous to that adopted for bronchogenic carcinoma.

[Current indications for sublobar resection in non-small-cell bronchogenic carcinoma]. / Indicazioni attuali alle resezioni sublobari nel carcinoma broncogeno non microcitoma.

Over the past 30 years, there has been considerable controversy regarding the role of segmental and wedge resections in the management of stage I (T1-T2N0M0) non-small-cell lung cancer. Recently, a prospective randomized trial (Lung Cancer Study Group, 1995) revealed unfavorable results after limited resection, which, in early stage lung cancer, remains a reasonable option for patients with compromised pulmonary reserve, especially those in whom a previous contralateral resection has been performed. The following report describes the role of limited resection in the management of patients with T1-T2N0 non-small-cell lung cancer and presents a retrospective review of our series of 125 limited resections out of 1356 resections performed for lung cancer. In particular, long term survival and the frequency of local/regional recurrence were noted in 92 cases operated on with a curative intent. 26.6% vs 12.5% local/regional recurrence rates were observed among patients undergoing limited resections for T2 and T1 lung cancer, respectively. The five year survival in the limited resection group was 13.5% for T1 and 60% for T2 vs 51% and 72% in the standard procedure group, respectively. The lobectomy results were superior to those of sublobar resection. The latter should be reserved for patients in poor general condition contraindicating a standard lobectomy.

Surgical treatment of T3 lung cancer invading the chest wall.

Lung tumors invading the chest wall are classed as belonging to the T3 group and are considered potentially resectable. Their management, however, is controversial, and extrapleural resection, when possible, is preferred to en bloc resection which is regarded as a far more invasive and dangerous operation. Five year survival rates for completely resected cases range in the literature from 25 to 35%, but survival rates are much worse if lymph node metastases are present. These poor outcomes have prompted the development of combined surgical approaches: preoperative radiation therapy, with or without chemotherapy, has been used with an improvement in resectability rates, but only modest results in terms of median survival; in a number of case series, increased operative morbidity and mortality have been reported with this approach. The present report relates to 122 patients treated by en bloc (20 cases) or extrapleural (102 cases) resection, 31 of whom also received neoadjuvant treatment. The operative mortality was 4.6%. Median survival was 17 months after en bloc resection and 19 months after extrapleural resection. Though no statistically significant difference was found, extrapleural resection would appear to yield better results than the en bloc procedure.

[The surgery of tumors and "limited situations"]. / Chirurgia dei tumori e "situazioni--limite".

The term "limit" applied to cancer surgery, denotes the ideological moment beyond which one cannot and should not propose any aggressive treatment. Such limits may concern the operability of a patient and may be represented by some general characteristics independent of the patient's current disease status (e.g. very old age, poor performance status, poor cardiac, respiratory, renal hepatic or mental conditions). They may concern the neoplastic involvement of the organ affected by the tumor: if undertaken, surgery should guarantee a reasonable duration of life, and a quality of life that makes it worth living. Other factors to be taken in consideration are the possibility the tumor spread to local or distant sites, as well as certain extreme conditions such as cancer, cachexia, liver/kidney failure, irreversible septic-toxic shock, ect. Moreover, there may be limits related to the structural conditions of the establishment where the operation is to be carried out (facilities, equipment, pharmacological supplies, medical and paramedical personnel) and to the social environment and the economic situation of the patient, in view of the assistance required following surgery. Lastly, a severe assessment of one's own fitness to perform any specific task should be part of the daily preparation of any surgeon.

[Major surgery in thoracic injuries]. / La chirurgia maggiore nei traumi del torace.

Chest injuries have a high and steadily increasing incidence in western countries, but only some of the most common problems they create require an emergency thoracotomy or surgical video thoracoscopy. Flail chest, persistent pneumothorax, massive haemothorax, mediastinal emphysema, cardiac tamponade and intrathoracic foreign bodies can be identified as major surgical problems. Some of such patients (i.e. those with flail chest or foreign bodies) would be immediately candidates for major intervention. Other require fast but diagnostic procedures, because the choice of a therapy is dependent upon a precise identification of the damage. Injuries of trachea and primary bronchi, oesophagus, diaphragma, vena cava, great lung vessels, heart and aorta may represent important surgical emergencies; some leading rapidly to death. Fortunately, major surgical procedures are not really frequent in the management of thoracic traumas. Only 42 (3.5%) of nearly 2,000 patients with non-penetrating thoracic injuries had a thoracotomy or an surgical video thoracoscopy. The figure is far different for penetrating wounds; in fact 12 patients (41%) of 29 underwent mayor surgery.