Post-pneumonectomy broncho-pleural fistula successfully closed by open-window thoracostomy associated with V.A.C. therapy

Broncho-pleural fistula (BPF), is a dramatic complication that may occur after lung resection. The treatment is challenging due to its high rate of morbidity and mortality. Herein, a case of BPF associated with empyema, occurred in an elderly patient who had undergone to left pneumonectomy for non-small cell lung cancer (NSCLC), is reported. After various treatments including chest drainage and endoscopic procedures, BPF was successfully closed by open-window thoracotomy associated with vacuum assisted closure (V.A.C.) device therapy. The authors conclude that V.A.C. is a convenient and safe measure in the management of empyema with BPF. Moreover, in similar clinical contexts, V.A.C. may be the only option available that may assure the survival of the patient and the avoiding any later-phases of residual cavity

Migration of surgical clips through a right lobectomy stump mimicking an asthmatic syndrome

The mechanical stapler is routinely used in thoracic surgery practice to attend resection of bronchus and vessels. Herein, we reported a very rare complication as the migration of a titanium surgical clip through a right lobectomy stump. One year after the procedure, the patient complained of persistent cough. A misdiagnosis of asthma was made and she treated for 6 months with bronchodilators, corticosteroid and antihistaminic without success. Thus, patient re-referred of our unit. No clinical signs of infection as fewer, productive cough, dyspnea were present. The laboratory exams were within normal value including white cells. CT scan revealed no abnormalities. Bronchoscopy demonstrated a healed upper bronchus stump without evidence of an actual, open bronchopleural fistula but with clips apparently working their way into the airway, with approximately half of the clip visible within the lumen. The side of the clips that would be open before closure by the surgeon formed the leading edge of the clips visible in the lumen. The clips were successfully removed during flexible bronchoscopy with a forceps usually used for biopsy. After the procedure, the cough disappeared. The endoscopy check after 3 months showed a normal bronchial stump without evidence of fistula

Surgical management of cervico-mediastinal goiters: Our experience and review of the literature

We analyze and discuss the clinical presentation, the diagnostic procedures and the surgical technique in relation to post-operative complications and results in cervico-mediastinal thyroid masses admitted in Thoracic Surgery Unit of AOU Second University of Naples from 1991 to 2006 and in Thoracic Surgery Unit of AOU "S. Giovanni di Dio & Ruggi D'Aragona" of Salerno over a period of 3 years (2011-2014)

MRX87 family with Aristaless X dup24bp mutation and implication for polyAlanine expansions

<p>Abstract</p> <p>Background</p> <p>Cognitive impairments are heterogeneous conditions, and it is estimated that 10% may be caused by a defect of mental function genes on the X chromosome. One of those genes is <it>Aristaless related homeobox </it>(<it>ARX</it>) encoding a polyA-rich homeobox transcription factor essential for cerebral patterning and its mutations cause different neurologic disorders. We reported on the clinical and genetic analysis of an Italian family with X-linked mental retardation (XLMR) and intra-familial heterogeneity, and provided insight into its molecular defect.</p> <p>Methods</p> <p>We carried out on linkage-candidate gene studies in a new MRX family (MRX87). All coding regions and exon-intron boundaries of ARX gene were analysed by direct sequencing.</p> <p>Results</p> <p>MRX87 patients had moderate to profound cognition impairment and a combination of minor congenital anomalies. The disease locus, MRX87, was mapped between DXS7104 and DXS1214, placing it in Xp22-p21 interval, a hot spot region for mental handicap. An in frame duplication of 24 bp (ARXdup24) in the second polyAlanine tract (polyA_II) in ARX was identified.</p> <p>Conclusion</p> <p>Our study underlines the role of ARXdup24 as a critical mutational site causing mental retardation linked to Xp22. Phenotypic heterogeneity of MRX87 patients represents a new observation relevant to the functional consequences of polyAlanine expansions enriching the puzzling complexity of ARXdup24-linked diseases.</p

Mutations in the nuclear localization sequence of the Aristaless related homeobox; sequestration of mutant ARX with IPO13 disrupts normal subcellular distribution of the transcription factor and retards cell division

The electronic version of this article is the complete one and can be found online at: http://www.pathogeneticsjournal.com/content/3/1/1Background: Aristaless related homeobox (ARX) is a paired-type homeobox gene. ARX function is frequently affected by naturally occurring mutations. Nonsense mutations, polyalanine tract expansions and missense mutations in ARX cause a range of intellectual disability and epilepsy phenotypes with or without additional features including hand dystonia, lissencephaly, autism or dysarthria. Severe malformation phenotypes, such as X-linked lissencephaly with ambiguous genitalia (XLAG), are frequently observed in individuals with protein truncating or missense mutations clustered in the highly conserved paired-type homeodomain. Results: We have identified two novel point mutations in the R379 residue of the ARX homeodomain; c.1135C>A, p.R379S in a patient with infantile spasms and intellectual disability and c.1136G>T, p.R379L in a patient with XLAG. We investigated these and other missense mutations (R332P, R332H, R332C, T333N: associated with XLAG and Proud syndrome) predicted to affect the nuclear localisation sequences (NLS) flanking either end of the ARX homeodomain. The NLS regions are required for correct nuclear import facilitated by Importin 13 (IPO13). We demonstrate that missense mutations in either the N- or C-terminal NLS regions of the homeodomain cause significant disruption to nuclear localisation of the ARX protein in vitro. Surprisingly, none of these mutations abolished the binding of ARX to IPO13. This was confirmed by co-immunoprecipitation and immmuno fluorescence studies. Instead, tagged and endogenous IPO13 remained bound to the mutant ARX proteins, even in the RanGTP rich nuclear environment. We also identify the microtubule protein TUBA1A as a novel interacting protein for ARX and show cells expressing mutant ARX protein accumulate in mitosis, indicating normal cell division may be disrupted. Conclusions: We show that the most likely, common pathogenic mechanism of the missense mutations in NLS regions of the ARX homeodomain is inadequate accumulation and distribution of the ARX transcription factor within the nucleus due to sequestration of ARX with IPO13.Cheryl Shoubridge, May Huey Tan, Tod Fullston, Desiree Cloosterman, David Coman, George McGillivray, Grazia M Mancini, Tjitske Kleefstra and Jozef Géc

Models of classroom assessment for course-based research experiences

Course-based research pedagogy involves positioning students as contributors to authentic research projects as part of an engaging educational experience that promotes their learning and persistence in science. To develop a model for assessing and grading students engaged in this type of learning experience, the assessment aims and practices of a community of experienced course-based research instructors were collected and analyzed. This approach defines four aims of course-based research assessment—(1) Assessing Laboratory Work and Scientific Thinking; (2) Evaluating Mastery of Concepts, Quantitative Thinking and Skills; (3) Appraising Forms of Scientific Communication; and (4) Metacognition of Learning—along with a set of practices for each aim. These aims and practices of assessment were then integrated with previously developed models of course-based research instruction to reveal an assessment program in which instructors provide extensive feedback to support productive student engagement in research while grading those aspects of research that are necessary for the student to succeed. Assessment conducted in this way delicately balances the need to facilitate students’ ongoing research with the requirement of a final grade without undercutting the important aims of a CRE education

The use of LigaSure for preservation of a previous coronary artery bypass graft by using the left internal thoracic artery in a left upper lobectomy.

In a 63-year-old man, an opacity localized in the left upper pulmonary lobe was incidentally discovered on chest radiographic analysis. He had undergone CABG 8 years before with the LITA on the left anterior descending coronary artery. Clinical and laboratory evaluation showed no abnormalities. Spirometric results were normal. Computed tomographic analysis demonstrated a peripheral mass in the left upper lobe, and there was no significant mediastinal adenopathy. Bronchoscopy did not demonstrate abnormalities of the left upper bronchus, and percutaneous needle biopsy showed a non–small cell lung cancer (NSCLC). A standard posterolateral thoracotomy incision was performed through the fifth intercostal space. A 6-cm tumor was found in the left upper lobe (Figure 1). The dissection of the lobe from the pericardium was possible, but dense adhesions were identified between the LITA and the apex of the upper lobe. The challenge was to do an upper lobectomy while avoiding injury to the LITA. Therefore the part of parenchyma adjacent to the LITA was separated from the upper lobe by using LigaSure system (Figure 2). After this maneuver, we performed an anatomic upper lobectomy, leaving a small strip of parenchyma adjacent to the LITA; we tested the arterial graft manually, and a good pulsation was found. Anatomopathologic studies showed a 6–cm stage IB squamous cell carcinoma, according to TNM classification.2 The patient’s postoperative course was unremarkable, and he was discharged on the seventh postoperative day

The use of LigaSure for preservation of a previous coronary artery bypass graft by using the left internal thoracic artery in a left upper lobectomy.

In a 63-year-old man, an opacity localized in the left upper pulmonary lobe was incidentally discovered on chest radiographic analysis. He had undergone CABG 8 years before with the LITA on the left anterior descending coronary artery. Clinical and laboratory evaluation showed no abnormalities. Spirometric results were normal. Computed tomographic analysis demonstrated a peripheral mass in the left upper lobe, and there was no significant mediastinal adenopathy. Bronchoscopy did not demonstrate abnormalities of the left upper bronchus, and percutaneous needle biopsy showed a non–small cell lung cancer (NSCLC). A standard posterolateral thoracotomy incision was performed through the fifth intercostal space. A 6-cm tumor was found in the left upper lobe (Figure 1). The dissection of the lobe from the pericardium was possible, but dense adhesions were identified between the LITA and the apex of the upper lobe. The challenge was to do an upper lobectomy while avoiding injury to the LITA. Therefore the part of parenchyma adjacent to the LITA was separated from the upper lobe by using LigaSure system (Figure 2). After this maneuver, we performed an anatomic upper lobectomy, leaving a small strip of parenchyma adjacent to the LITA; we tested the arterial graft manually, and a good pulsation was found. Anatomopathologic studies showed a 6–cm stage IB squamous cell carcinoma, according to TNM classification.2 The patient’s postoperative course was unremarkable, and he was discharged on the seventh postoperative day