Blue rubber-bleb nevus syndrome: report of a case associated with osteoid osteomas

Blue rubber-bleb nevus syndrome (BRBNS), or Bean's syndrome, is a rare angiomatosis characterized by multiple cavernous hemangiomas of the skin, mucosae and frequently of other sites. A 49-year-old male patient had been affected since birth by multiple angiomas localized in the skin, lips, oral cavity, cranial theca, and central nervous system; intrauterine rupture of angiomas in the right parietal lobe had caused partial hypotrophic paralysis of the left hemisoma. In addition to BRBNS, the patient was affected by three osteoid osteomas: this never-described clinical association is here discussed.     

Atopic dermatitis exclusively localized on nipples and areolas

We present an 11-year-old girl, with celiac disease, and a 9- month history of itchy and erythemato-edematous lesions with vesicles and exudation on her nipples and areolas. No other lesions or signs of scratching were present on her face or folds. She had no specific lesions of atopic dermatitis in typical sites nor in other body surface during her life. Patch tests showed a positive reaction to nickel and thimerosal that was not significantly related to the clinical appearance. This presentation documents the clinical relevance of atopic dermatitis minor diagnostic criteria. We discuss the importance of nipple eczema in AD and its differential diagnosis.     

COPD increases the risk of squamous histological subtype in smokers who develop non-small cell lung carcinoma

BACKGROUND: Squamous cell carcinoma has a stronger association with tobacco smoking than other non-small cell lung cancers (NSCLC). A study was undertaken to determine whether chronic obstructive pulmonary disease (COPD) is a risk factor for the squamous cell carcinoma histological subtype in smokers with surgically resectable NSCLC. METHODS: Using a case-control design, subjects with a surgically confirmed diagnosis of squamous cell carcinoma were enrolled from smokers undergoing lung resection for NSCLC in the District Hospital of Ferrara, Italy. Control subjects were smokers who underwent lung resection for NSCLC in the same hospital and had a surgically confirmed diagnosis of NSCLC of any histological type other than squamous cell. RESULTS: Eighty six cases and 54 controls (mainly adenocarcinoma, n = 50) were enrolled. The presence of COPD was found to increase the risk for the squamous cell histological subtype by more than four times. Conversely, the presence of chronic bronchitis was found to decrease the risk for this histological subtype by more than four times. Among patients with chronic bronchitis (n = 77), those with COPD had a 3.5 times higher risk of having the squamous cell histological subtype. CONCLUSIONS: These data suggest that, among smokers with surgically resectable NSCLC, COPD is a risk factor for the squamous cell histological subtype and chronic bronchitis, particularly when not associated with COPD, is a risk factor for the adenocarcinoma histological subtype.     

Corticospinal excitability during painful self-stimulation in humans: a transcranial magnetic stimulation study

We investigated changes in the corticospinal pattern of activity in healthy volunteers during sustained noxious and non-noxious mechanical stimulation of the first hand digit, resulting from active (self-stimulation) or passive (externally-induced) pressing against a sharp or blunted tip. The results indicate that, in order to press a finger onto a noxious stimulus with the same force generated to press onto a non-noxious one, the motor cortex adopts a peculiar strategy in terms of recruitment of motor units. This is reflected by an increase of corticospinal excitability (as revealed by motor potentials evoked by transcranial magnetic stimulation of the contralateral primary motor cortex) and EMG activity of agonist muscles, possibly related to an increase of motor unit synchronization.     

Case study: pachyonychia congenita: a mixed type II-type IV presentation

A 52-year-old woman in good health with a family history negative for dermatologic diseases presented to our department with thickening and dystrophy of all her fingernails and toenails that started when she was born. She also had hyperkeratosis on the palms of her hands and soles of her feet that was confined to sites of pressure and recurrent plantar blisters that began appearing at puberty. The patient reported marked pain while walking from such plantar involvement. Her medical history revealed a persistent hoarseness; palmoplantar hyperhidrosis; and the appearance of numerous cysts on her back, neck, and scalp since she was 20 years old. These latter lesions had been diagnosed as multiple steatocystoma on the basis of the histologic features. Upon examination, all of her fingernails and toenails appeared shortened, thickened,and dystrophic (Figures 1-3). In addition, they presented subungual keratosis and a yellowish-gray color. Hyperkeratosis and small ulcerations were present on the perionychium. Palmoplantar keratoderma was evident, especially on the soles,in association with superficial erosions (Figure 4). Keratosis pilaris was evident on the extensor surfaces of the forearms as well as on the anterior surfaces of the legs. Multiple nodules were detected on the patient's neck, trunk, and axillary regions(Figure 5). They consisted of multiplex steatocystoma and were characterized by a hemispheric shape, a normal-appearing skin color, and by an elastic consistency on palpation. Oral and dental changes were not detected, although hair anomalies were evident. Laboratory parameters disclosed eosinophilia and increased total IgE levels. The results of serum protein electrophoresis was normal, as were those concerning hepatic and renal functions. The ophthalmology examination showed neither corneal dyskeratosis nor cataracts. The neurologic-psychiatric visit revealed slight mental retardation.     

Benign breast diseases in breast cancer screening programs in Italy (2000-2001)

AIMS AND BACKGROUND: Screening mammography has been shown to be effective in reducing breast cancer mortality in several randomized clinical trials. One major side effect of screening is the diagnosis of benign breast disease (BBD), which is considered as a nonprogressive lesion, except for a small percentage of lesions considered at high risk. We present data referring to service screening programs active in Italy in 2000 and 2001 and participating in the national survey carried out by the Italian Group for Mammography Screening (GISMa). METHODS: To all centers participating in the GISMa National Survey, we submitted a questionnaire regarding the service screening protocol and main indicators of performance of the local program in the years 2000 and 2001. RESULTS: A total of 657 detected BBD cases, registered by 23 Italian breast cancer screening centers in women 50 to 69 years of age, are included in this study. The BBD detection rate was 2.5 per 1000 at the first screening test and 1.05 per 1000 at repeated tests. The benign/malignant ratio was 0.34 at the first and 0.22 at the repeated test. CONCLUSIONS: Detection of BBD occurred frequently in breast screening programs, and prognostic implications should be further investigated. Women should be individually informed at screening, and with greater detail at the moment of the recall for assessment, of the implications of BBD detection and receive the necessary, also psychological, counseling to avoid the possible harm related to breast cancer screening.     

Monitoring high-dose heparin levels by ACT and HMT during extracorporeal circulation: diagnostic accuracy of three compact monitors

The correct monitoring of heparin therapy and its reversal determines the successful conduct of cardiovascular surgery with extracorporeal circulation (ECC). The activated coagulation time (ACT) and the heparin management test (HMT) are the most frequently used tests in the operating room. Three compact monitors for ACT or HMT are here evaluated. Forty samples were obtained, at 10-min intervals, from eight patients during ECC. The ACT or HMT was immediately performed using: Hemochron juniors ACT, CoaguCeck Pro (ACT) and Rapid Point Coag (HMT). Data were compared between them and with the heparin levels, measured as anti-Xa. The simple least squares linear regression among, respectively, Hemochron Junior ACT, CoaguCeck Pro ACT, Rapid Point Coag HMT and anti-Xa activity were i=452.3, s=15.2, Sy/x=37.5, r=0.18; i=411.9, s=22.1, Sy/x=48.7, r=0.21 and i=479.4, s=9.0, Sy/x=9.3; r=0.41. CoaguCeck Pro ACT results were above the upper detection limit (500 s) in 37 of 40 determinations. The comparison between ACT Hemocron and HMT Rapid Point Coag shows i=35.7, s=0.9, Sy/x=35.4, r=0.68, with a bias of 29.0 s (CI: 17.9-40.1), 95% of agreement between -41.5 s (CI: -60.7 to -22.3) and 99.5 s (CI: 80.4-118.7). Taking a concentration of 2.0 U/ml of heparin to discriminate between high- and low-risk conditions, receiver-operator characteristic (ROC) curve was used to rank the performance of the methods. Areas under the ROC curve+/-SE for Hemochron Junior ACT and Rapid Point Coag HMT were 0.629+/-0.097 and 0.543+/-0.096. The results obtained by HMT appear similar to those obtained by the ACT for monitoring high-dose heparin therapy in patients undergoing ECC. HMT appeared to perform better than ACT in measuring the heparin effect, while the ROC analysis gives a little more accuracy for ACT. Neither of the two methods is able to achieve enough evidence of diagnostic accuracy. Since these tests are widely used, and there are no laboratory alternatives, a real comparison with the outcome of the patients should be helpful for an evidence-based evaluation of these point-of-care tests.     

Postoperative aortic fistulas into the airways: etiology,pathogenesis, presentation,diagnosis, and management

Postoperative aortobronchial and aortopulmonary fistulas are rare and late complications of cardiac surgery. They mostly complicate descending thoracic aortic procedures. Hemoptysis is the main symptom, and may be massive or intermittent. The reported interval between the time of operation and the onset of hemoptysis ranges from 3 weeks to 25 years. Diagnostic examinations are often unable to directly visualize a fistula. Indication for surgical or endovascular repair mostly relies on clinical suspicion and nonspecific diagnostic features. Urgent treatment is based on the association of the following elements: (1) hemoptysis, (2) history of previous cardiac or aortic operation, (3) presence of lung infiltrates on the chest roentgenogram, (4) lung hemorrage on the computed tomographic scan, and (5) and visualization of a pseudoaneurysm. Aortobronchopulmonary fistulas are uniformly fatal if untreated. The overall surgical mortality rate is 15.3%. There is no procedure-related mortality after endovascular stent grafting. A review of the English-language literature from 1947 to October 2002 is presented.