Bronchioloalveolar carcinoma arising in a congenital pulmonary airway malformation in a child: case report with an update of this association

An 8-year-old girl was evaluated for a mass in the left pulmonary lobe. Her clinical history was remarkable for an intermittent dry cough since the age of 2, with recurrent episodes of purulent sputum and fever. She underwent left lower lobectomy, which was found to be a bronchioloalveolar carcinoma arising in a type 1 congenital pulmonary airway malformation at pathologic examination. No additional therapies were undertaken. Two years after resection, imaging studies showed 5 contralateral pulmonary nodules suggestive of disseminated disease with a poor outcome. A review of presentation, treatment, and outcome of this association was made.     

Motor cortex stimulation for ALS: A double blind placebo-controlled study

Preliminary data suggest that repetitive transcranial magnetic stimulation (rTMS) of the brain may produce a modest slowing of disease progression in amyotrophic lateral sclerosis (ALS). The present study was designed to test the hypothesis that rTMS given as continuous theta burst stimulation (cTBS), repeated monthly for one year, would affect ALS progression. We performed a double blind, placebo-controlled trial. Twenty patients with ALS were randomly allocated to blinded real or placebo stimulation. cTBS of the motor cortex was performed for five consecutive days every month for one year. Primary outcome was the rate of decline as evaluated with the revised ALS functional rating scale (ALSFRS-R). Treatment was well tolerated. There was no significant difference in the ALSFRS-R score deterioration between patients treated with real or placebo stimulation. ALSFRS-R mean scores declined from 32.0 (SD 7.1) at study entry to 23.1 (SD 6.3) at 12 months in patients receiving real cTBS and from 31.3 (SD 6.9) to 21.2 (SD 6.0) in those receiving placebo stimulation. Although cTBS proved a safe procedure, on the basis of the present findings a larger randomized confirmatory trial seems unjustified in ALS patients, at least in advanced stage of the disease.     

Cutaneous distribution of plasmacytoid dendritic cells in lupus erythematosus. Selective tropism at the site of epithelial apoptotic damage

Recent evidences suggest a significant role of Plasmacytoid dendritic cells (PDC) role in the pathogenesis of lupus erythematosus (LE) via production of type I IFN. Taking advantage on the availability of multiple reagents (CD123, BDCA2, and CD2ap) specifically recognizing PDC on fixed tissues, we investigated the occurrence of PDC in a cohort of 74 LE patients. The large majority of LE biopsies (67/74; 90.5%) showed cutaneous infiltration of PDC. PDC were more frequently observed (96.4 vs 72.2) and numerous in cutaneous LE compared to systemic LE (SLE) and correlated with the density of the inflammatory infiltrate (r=0.40; p<0.001). PDC reduction in SLE might be related to a broader tissue distribution of this cellular population, as indicated by their occurrence in kidneys in 11 out of 24 (45.8%) cases studied. The distribution of cutaneous PDC showed two distinct patterns. More commonly, PDC were observed within perivascular inflammatory nodules in the dermis, associated with CD208+ mature DC and T-bet+ cells [D-PDC]. A second component was observed along the dermal-epithelial junction [J-PDC], in association with cytotoxic T-cells in areas of severe epithelial damage. Notably, chemerin reactivity was observed in 64% of LE biopsies on endothelial cells and in the granular layer keratinocytes. Cutaneous PDC in LE strongly produced type I IFN, as indicated by the diffuse MxA expression, and the cytotoxic molecule granzyme B.This study confirms cutaneous PDC infiltration as hallmark of LE. The topographical segregation in D-PDC and J-PDC suggests a novel view of the role of these cells in skin autoimmunity.     

Uninfected Para-Anastomotic Aneurysms after Infrarenal Aortic Grafting

Purpose

This single-institution retrospective review examines the management of uninfected para-anastomotic aneurysms of the abdominal aorta (PAAA), developed after infrarenal grafting.

Materials and Methods

From October 1979 to November 2005, 31 PAAA were observed in our Department. Twenty-six uninfected PAAA of degenerative etiology, including 24 false and 2 true aneurysms, were candidates for intervention and retrospectively included in our database for management and outcome evaluation. Six (23%) patients were treated as emergencies. Surgery included tube graft interposition (n = 12), new reconstruction (n = 8), and graft removal with extra-anatomic bypass (n = 3). Endovascular management (n = 3) consisted of free-flow tube endografts.

Results

The mortality rate among the elective and emergency cases was 5% and 66.6%, respectively (p = 0.005). The morbidity rate in elective cases was 57.8%, whereas 75% in emergency cases (p = 0.99). The survival rate during the follow-up was significantly higher for elective cases than for emergency cases.

Conclusion

Uninfected PAAA is a late complication of aortic grafting, tends to evolve silently and is difficult to diagnose. The prevalence is underestimated and increases with time since surgery. The mortality rate is higher among patients treated as an emergency than among patients who undergo elective surgery, therefore, elective treatment and aggressive management in the case of pseudoaneurysm are the keys to obtain a good outcome. Endovascular treatment could reduce mortality. Patients who undergo infrarenal aortic grafting require life-long surveillance after surgery.     

A technology-assisted learning setup as assessment supplement for three persons with a diagnosis of post-coma vegetative state and pervasive motor impairment

Post-coma persons in an apparent condition of vegetative state and pervasive motor impairment pose serious problems in terms of assessment and intervention options. A technology-based learning assessment procedure might serve for them as a diagnostic supplement with possible implications for rehabilitation intervention. The learning assessment procedure adopted in this study relied on hand-closure and eye-blinking responses and on microswitch technology to detect such responses and to present stimuli. Three participants were involved in the study. The technology consisted of a touch/pressure sensor fixed on the hand or an optic sensor mounted on an eyeglasses’ frame, which were combined with a control system linked to stimulus sources. The study adopted an ABABCB sequence, in which A represented baseline periods, B intervention periods with stimuli contingent on the responses, and C a control condition with stimuli presented non-contingently. Data showed that the level of responding during the B phases was significantly higher than the levels observed during the A phases as well as the C phase for two of the three participants (i.e., indicating clear signs of learning by them). Learning might be deemed to represent basic levels of knowledge/consciousness. Thus, detecting signs of learning might help one revise a previous diagnosis of vegetative state with wide implications for rehabilitation perspectives.     

The Italian version of the Response Evaluation Measure-71

Objective

The aim of the present study was to analyze the psychometric properties of the Italian version of the Response Evaluation Measure (REM-71), a 71-item self-report measure previously developed for the assessment of defenses in adults and adolescents. The authors also examined the differences in the use of defenses based on sex and age (early adolescence, late adolescence, and early adulthood), and the association between defenses, psychosocial health, and psychologic distress in a large community sample.

Method

The Italian version of REM-71 was obtained through back-translation and administered to 1648 (1020 female subjects, mean age = 19.5 years, SD = ±5.77) community subjects, aged between 13 and 68 years, voluntarily recruited among high school and university students in Milan, Italy, and the surrounding area. All subjects completed a self-report measure to assess demographic variables and satisfaction with life. A subsample (n = 1197) completed the Italian version of the Symptom Checklist-90-Revised to assess symptoms of psychologic distress.

Results

Results were highly consistent with those obtained in the original English version of the REM-71 and included satisfactory internal consistency of the measure. Factor analyses yielded 2 principal factors that showed overall stability across age and sex subgroups. Factor 1 and factor 2 defenses were significantly correlated, in line with theoretical expectations, with positive and negative aspects of various domains of life.

Conclusions

Results provide further support for the structure and validity of the REM-71 as a useful instrument for the assessment of defenses in adolescents and adults and suggest no major cross-cultural differences in the organization of these defenses.     

JAK2 inhibitors: are they the solution?

The discovery of the JAK2V617F mutation in patients with Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPN) started the era of targeted therapy for these diseases. Until now, patients had few treatment options available, which usually were restricted to hydroxyurea, interferon preparations, and chemotherapy in more aggressive cases. JAK2 inhibitors have been developed over the past 5 years, and the results of the first clinical trials with JAK2 inhibitors for patients with myelofibrosis were recently published. Current research results suggest that JAK2 inhibitors have a potential to decrease disease burden and its activity, as manifested by a decrease in splenomegaly and improvement in systemic disease-related symptoms, but they do not seem to be able to eradicate the malignant clone. However, JAK2 inhibitors help patients regardless of their mutation status, because patients without JAK2V617F mutation benefit to the same extent as patients with JAK2V617F mutation. A greater understanding of the pathophysiology of MPNs is needed before we can cure myelofibrosis with drug therapy. Currently, several new JAK2 inhibitors are in clinical trials for patients with myelofibrosis, and clinical trials for patients with polycythemia vera and essential thrombocythemia have also started. We review recent data on JAK2 inhibitors for the management of patients with Ph-negative MPNs.     

Evaluation of antioxidant activity and antiproliferative effect of fruit juices enriched with Pycnogenol® in colon carcinoma cells. The effect of in vitro gastrointestinal digestion

The aim of this study was to examine the effect of in vitro gastrointestinal digestion on the antioxidant and antiproliferative effect of fruit juices enriched with Pycnogenol® (0.5 g/L) on a colon carcinoma cell line (Caco‐2). The total phenolic concentration (TPC), antioxidant activity and inhibition cell growth were studied in fresh and digested pineapple juice and red fruits juice (both enriched with pine bark extract and not). After in vitro digestion the level of detectable phenolic compounds (expressed as gallic acid equivalent) was higher in both pineapple and red fruits juices enriched with Pycnogenol® than in non‐enriched commercial juices (155.6 mg/100 mL vs 94.6 mg/100 mL and 478.5 mg/100 mL vs 406.9 mg/100 mL, respectively). Increased antioxidant activity (measured by 2,2'‐azino‐bis(3‐ethylbenzothiazoline‐6‐sulphonic acid) (ABTS) and oxygen radical absorbance capacity assay (ORAC) methods) was observed in digested enriched juices with respect to the same samples before digestion. Pycnogenol® enrichment led to a high antiproliferative effect between 24 and 72 h of incubation with undigested pineapple juice compared with the non‐enriched juice. It can be concluded that enrichment of fruit juices with Pycnogenol® provides a source of phenolic compounds with high stability to in vitro gastrointestinal conditions; however, the antioxidant properties of fruit juices were affected to a different extent. Copyright © 2011 John Wiley & Sons, Ltd.     

Radiopharmaceuticals: when and how to use them to treat metastatic bone pain

Bone pain due to skeletal metastases constitutes the most common type of cancer-related pain. The management of bone pain remains challenging and is not standardized. In patients with multifocal osteoblastic metastases, systemic radiopharmaceuticals should be the preferred adjunctive therapy for pain palliation. The lack of general knowledge about radiopharmaceuticals, their clinical utility and safety profiles, constitutes the major cause for their underutilization. Our goal is to review the indications, selection criteria, efficacy, and toxicities of two approved radiopharmaceuticals for bone pain palliation: strontium-89 and samarium-153. Finally, a brief review of the data on combination therapy with bisphosphonates or chemotherapy is included.