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Gorlin-Goltz syndrome is mainly characterised by the development of numerous multicentric and relapsing cutaneous basal cell carcinomas (BCCs). A major problem for patients with Gorlin-Goltz syndrome is the large amount of BCCs that can invade the deep underlying structures, especially the face. Here, we describe the case of a 23-year-old male affected by Gorlin-Goltz syndrome. He had recurrent BCCs on a hairless scalp and dorsum since he was 17 years old and underwent four surgical procedures to excise BCCs, including a reconstruction with anteromedial thigh perforator flap. For each of the surgical procedures, a phenotypic study on peripheral blood mononuclear cells using flow cytometry was performed on the same day of surgery, and on days 7, 14 and 21 after surgery. The role of the tumour-specific cytolytic immune response as a potential future treatment of syndromic BCCs and its trend in relation to surgical ablation of large portions of tumour tissue was examined, and the cosmetic and therapeutic results are shown. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Post-mastectomy radiotherapy (PMRT) is well known in the plastic surgery community for having a negative impact on expander-implant based immediate breast reconstruction (IBBR), although recently some technical improvements allow better results. Very recent papers would suggest that there is no difference in postoperative complications in patients receiving post-mastectomy radiotherapy using modern techniques. However, study results are often biased by small groups of patients and by heterogeneity of radiotherapy timing, different surgical techniques and measured outcomes. MATERIALS AND METHODS: We have conducted a MEDLINE search to summarize the latest data (2012-2014) on the topic. Search was conducted using the following parameters: breast reconstruction AND implant AND expander AND post-mastectomy radiotherapy. RESULTS: The MEDLINE search showed 53 reports, demonstrating a great interest on this topic; among these 37 dealed specifically with post-mastectomy radiotherapy after breast reconstruction. In particular, 15 were amenable to plastic surgeons, 6 to breast surgeons, 9 to radiotherapists and 7 to oncologists. Papers amenable to plastic surgeons highlighted the highest rate of undesired results, although with recent advances such as delayed-immediate reconstruction or protective lipofilling. CONCLUSIONS: PMRT remains an undesired event when pursuing an implant-based breast reconstruction, although it does not represent an absolute contraindication. The higher rate of complications reported by plastic surgeons and not by other specialists can be explained with the greater attention to aesthetic details, such as capsular contractures, that our community has. Technical strategies to prevent complications described in this community now allow better results, should be well known and improved if possible in the future. [ABSTRACT FROM AUTHOR]

This paper updates the calibration of the cosmic distance scale by Cepheid variable stars making reference to a ‘geometric’ Baade–Wesselink (BW) distance indicator which adopts pulsation linear radii and photometric angular diameters from period–radius and surface brightness relations, respectively, calibrated by fundamental data of Galactic Cepheids. The large number of fundamental-like BW distances (pulsation parallaxes) predictable for Cepheids by using the period and the (V, I) intensity mean magnitudes from HIPP photometry are then applied to calibrate the Galactic period–luminosity relations and the reddening-free Wesenheit relation in the (V, I) passbands well suited to obtain metallicity-corrected distance moduli μO = (18.486 ± 0.047) mag or D = (49.80 ± 1.10) kpc from Large Magellanic Cloud Cepheids of the Optical Gravitational Lensing Experiment map and μO = (29.281 ± 0.051) mag or D = (7.18 ± 0.17) Mpc from Hubble Space Telescope (HST) Cepheids in the inner field of the maser host galaxy NGC 4258, this value resulting in fair good agreement with the high-weight geometric water maser distance modulus. The Cepheid distance moduli of galaxies from the HST SN Ia Calibration Programme are found to be affected by significant systematic errors. Removing these errors leads to an upward revision of the Hubble constant at the value HO = (72.4 ± 6.0) km s−1 Mpc−1, in fair good agreement now with the result of HO = (72 ± 8) km s−1 Mpc−1 originally derived in the HST Key Project Programme as well as with the most recent accurate determination of HO = (73.8 ± 2.4) km s−1 Mpc−1 obtained by HST near-infrared observations of Cepheids. [ABSTRACT FROM AUTHOR]

I report on the implementation of the surface brightness (SB) technique aimed at deriving reliable Cepheid angular diameters at optical wavelengths and then Baade–Wesselink (BW) distances using spectroscopic linear radii. The technique is applied to calibrate the Galactic distance scale and the distance to the Large Magellanic Cloud (LMC) based on this Galactic calibration. The LMC distance benefits from the large number of Cepheids with high-precision photometry and reddening data available by the Optical Gravitational Lensing Experiment of Udalski et al. and from improvements and refinements, including (i) a least-squares (LS) fitting approach to the individual distances to recover the distance modulus unbiased by the period distribution of target Cepheids and (ii) a better understanding of the metallicity effects on the period–luminosity relations and SB scales. The resulting true distance modulus of the LMC is . The SB technique is also applied to confidently determine the Cepheid distance to the maser host galaxy NGC 4258. Cepheids in the inner region with near solar metallicity yield a true distance modulus of , in excellent agreement, at the 0.01 mag level, with the high-weight geometric water maser distance modulus. It is shown that the current data-reduction approach of averaging individual Cepheid distances can introduce significant systematic errors in the distance scale calibration. From a small sample of Hubble Space Telescope (HST) based galaxies, I derive an overall error of (12 ± 5) per cent, which would suggest a downward revision of the Freedman et al. Hubble constant to the value , other things being equal. [ABSTRACT FROM AUTHOR]

We report a tricky case of a 63-year-old man with previous coronary artery bypass graft surgery in whom transoesophageal echocardiography revealed a voluminous echolucent cavity simulating aortic ectasia but that proved to be of nephrogenic origin. [ABSTRACT FROM AUTHOR]

There is increasing interest in the effects of adult smoking on the health of infants and children. Although passive smoking is important in many childhood disorders, most attention has been paid to its effects on the respira tory tract and on infant mortality. Several studies have reported an increased risk of sudden infant death syndrome and respiratory illness in infants of mothers who had smoked during pregnancy. Post-natal exposure to passive smoking has been found responsible for an increased risk of acute respiratory illness morbidity and an increased occurrence of chronic respiratory symptoms. Maternal cigarette smoking aggravates asthma symptoms and bronchial responsive ness in children with an established diagnosis of the disease, and the possibility that passive smoking has a causal role in the aetiology of asthma is currently a matter of growing interest. In addition, several studies have shown small but significant reductions in lung function values of children exposed to passive smoking. [ABSTRACT FROM PUBLISHER]

Study design:Marjolin's ulcer is a squamous cell carcinoma that develops in posttraumatic scars and chronic wounds. Suspicion of such lesions should be raised in chronic wounds demonstrating characteristic changes. We have reported the peculiar phenomenon of malignant transformation of chronic pressure sores that occurred in a paraplegic patient.Objectives:The aim of this study was to cover the extensive defects by a last resort reconstructive option.Setting:Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy.Methods and results:A 40-year-old paraplegic man, with multiple hemangioblastomas of the brain and spinal cord due to Von Hippel Lindau syndrome developed pressure ulcers with unstable healing over the sacral, trochanteric, bilateral, and ischiatic areas after 15 years from neurosurgery. The biopsy result showed an invasive squamous carcinoma. Carcinomas in pressure sores are highly aggressive, and they need to be treated more radically. In our case we opted for a demolitive surgical treatment including musculocutaneous rotational flap harvested from total left thigh to cover the extensive defects. The limb was previously disarticulated.Conclusion:In Marjolin's ulcer, multiple biopsies are the first-line modality for the early diagnosis as they are a safe method with high rate of accuracy. First-line treatment is surgery consisting of radical excision with lymph node dissection, if they are involved. Adjuvant radiation therapy may be used in selected patients. Management of massive pelvic defects can be a challenging problem. The pedicled lower limb flap offers a technique that can be considered as a last resort procedure for extensive defects where other options are insufficient or not available anymore. In our case the patient is disease-free after 2 years of follow-up. [ABSTRACT FROM AUTHOR]

Purpose: Sympathetic ganglia neurons (SGNs) innervate the myocardium and tune heart rate and contractility. Development and viability of cardiac neurons depend on neurotrophins that are released in low amounts by the myocardium. This study aims to understand whether the direct cell-to-cell contact plays a role in NGF-mediated signaling between cardiomyocytes (CMs) and SGNs.Methods and results: Electron microscopy and immunofluorescence on mouse heart slices and rat SGN/CM co-cultures showed a close association between SGNs and CMs, neurotransmitter vesicle accumulation, increased membrane protein density and enrichment of the NGF receptor (TrkA) at the contact site. These data support that specialized and locally organized signaling domains exist (neuro-cardiac junction, NCJ, hereafter).We tested the functional role of the NCJ in NGF-mediated prosurvival signaling. NGF expression by CMs was assessed by western blot analysis and its silencing in co-cultures caused a 66% decrease of neuronal density, suggesting that neurons depend on NGF released by CMs. NGF binding to its receptor triggers TrkA retrograde transport to neuronal soma that was monitored using fluorescently labeled TrkA. TrkA-RFP retrograde movements were faster in processes contacting CMs than other cardiac cells, further supporting that neuronal NGF signaling is activated by the coupled CMs. SGNs cultured on NGF-silenced CMs showed a 20% decrease in the NCJ area when compared to those on wild type CMs of the same culture. Moreover, NGF uptake was observed only in processes contacting NGF overexpressing CMs, supporting that neurotrophin mediated signaling is involved at the SGN/CM interaction. Consistently, cultured SGNs in contact with CMs survived NGF withdrawal, whereas neurons alone treated with CM-conditioned medium did not survive because of the very low NGF concentration (<5pg/mL).An anti-NGF antibody, a TrkA antagonist (c(92-96)) and inhibitor (k252a) were used to antagonize receptor activation by NGF. Only the small membrane permeable k252a reduced neuronal density, suggesting that the NCJ is an isolated microdomain. K252a was used to estimate NGF concentration at the contact site, which resulted about 1.5nM, four orders of magnitude higher than that in CM-conditioned medium, supporting that the NCJ allows amplification of intercellular NGF signaling.Conclusions: Taken together, our results suggest that the NGF-dependent pro-survival signal to the SGN needs a direct interaction with the CM that facilitates NGF activation of TrkA thanks to the development of an isolated microdomain characterized by a high NGF concentration. [ABSTRACT FROM AUTHOR]