Surgical approach to a rare case of Beckwith Wiedemann syndrome with left thigh hyperplasia.

Thigh lift surgery is generally performed in patients with severe weight loss outcomes, particularly those undergoing bariatric surgery. However, there are other congenital malformation conditions that may require the same treatment, such as Beckwith Wideman syndrome.

Properties of balanced flows with bottlenecks: Common stylized facts in finance and vibration-driven vehicles.

We study experimentally the properties of the flow of mechanical vibration-driven vehicles confined in two chambers connected through a narrow opening. We report that the density of particles around the opening presents critical behavior and scaling properties. By mapping this density to the financial market price, we document that the main stylized facts observed in financial systems have their counterparts in the mechanical system. The experimental model accurately reproduces financial properties such as scaling of the price fluctuation, volatility clustering, and multiscaling.

Unexpected Stretching of Entangled Ring Macromolecules.

In the melt state at equilibrium, entangled nonconcatenated ring macromolecules adapt more compact conformations compared to their linear analogs and do not form an entanglement network. We show here that, when subjected to uniaxial stretching, they exhibit a unique response, which sets them apart from any other polymer. Remarkably, whereas both linear and ring polymers strain-harden, the viscosity of the rings increases dramatically (the melt thickens) at very low stretch rates due to the unraveling of their conformations along the stretching direction. At high rates, stretching leads to viscosity thinning similar to that of entangled linear polymers, albeit with subtle differences.

Direct Assessment of Tube Dilation in Entangled Polymers.

A key ingredient within theories focusing on the rheology of entangled polymers is the way how the topological constraints of an entangled chain are lifted by unconstrained segments, i.e., how the constraining tube is dilated. This important question has been addressed by directly measuring the tube diameter d at the scale of the tube by neutron spin echo spectroscopy. The tube diameter d and plateau modulus G_{N}^{0} of highly entangled polyethylene oxide (PEO) chains of volume fraction c that are diluted by low molecular PEO show a concentration dependence d∝c^{a/2} and G_{N}^{0}∝c^{1+a} with an exponent a close to 4/3. This result allows the clear discrimination between different theoretical models that predict 4/3 or other values between 1 and 2 and provides an important ingredient to tube model theories.

A rare case of Brooke-Spiegler syndrome: integrated surgical treatment of multiple giant eccrine spiradenomas of the head and neck in a young girl.

INTRODUCTION: The authors present a "four-step" integrated surgical protocol to treat a rare case of multiple giant eccrine spiradenoma (ES) of the head and neck in a young patient. PRESENTATION OF CASE: An 18-year-old female patient presented with multiple swellings in the head and neck regions. The patient had a severe psychological trauma with a negative impact on her social life. Physical examination revealed multiple papulo-nodular swellings measuring between 5 cm × 8 cm and up to 10 cm × 20 cm in size with cerebriform aspect and soft consistency. Major lesions were located in the scalp, frontal area, neck, occipitotemporal, and retroauricular regions. Tissue biopsy found a benign composite adnexal neoplasm consisted in ES, trichoepithelioma, and cylindroma, a typical feature of Brooke-Spiegler Syndrome. A staged excision was planned, and available reconstructive options were considered. Scalp reconstruction included tissue expansions, advancement flaps, skin grafts, and dermal regeneration template (Integra®). All treatments were successful, and no recurrence was observed. The patient returned to a normal social life, and a radical excision with satisfying aesthetic results was achieved. DISCUSSION: Although adnexal tumors are benign in most of the cases, these lesions are prone to arise in the craniofacial region, thereby causing aesthetic discomfort associated with pain, hemorrhage, and infection to the patient every day. Furthermore, there is a potential risk of malignant transformation. These concerns demonstrate the need to establish a surgical protocol for the treatment of adnexal tumors. CONCLUSIONS: Our integrated surgical approach showed excellent aesthetic and functional results with benefits to the patient's life and complete oncological excision.

The role of lipofilling in reconstructions with dermal regeneration template: clinical and histological assessment.

Skin and soft tissue reconstruction represents one of the most debated issues of plastic surgery. The advent of regenerative medicine has shown new pathways with the use of lipofilling and dermal regeneration templates. The aim of this study was to investigate the histological and clinical modifications occurring after lipofilling in the areas previously reconstructed with Integra® and an autologous thin dermal-epidermal graft. Histological and immunohistochemical analysis were performed on nine patients to compare skin before and after lipofilling. Pre- and post-operative examinations (POSAS, VAS scale) were carried out as well as taking clinical photographs. The authors detected an overall clinical and histological improvement in all cases. Data obtained from POSAS and VAS scale showed a statistically significant (p less than 0.05) improvement concerning all variables investigated before surgery. The biopsies revealed qualitative modifications with hematoxylin-eosin and Masson trichrome stain. Immunohistochemistry with CD31 antibody also demonstrated quantitative changes with an increased number of vessels. The photographs enabled to compare the clinical situation before and after lipofilling with better aesthetic outcomes. Lipofilling gave good functional and aesthetic results in the areas treated with Integra® and autologous thin dermal-epidermal grafts.

Clogging Transition of Vibration-Driven Vehicles Passing through Constrictions.

We report experimental results on the competitive passage of elongated self-propelled vehicles rushing through a constriction. For the chosen experimental conditions, we observe the emergence of intermittencies similar to those reported previously for active matter passing through narrow doors. Noteworthy, we find that, when the number of individuals crowding in front of the bottleneck increases, there is a transition from an unclogged to a clogged state characterized by a lack of convergence of the mean clog duration as the measuring time increases. It is demonstrated that this transition-which was reported previously only for externally vibrated systems such as colloids or granulars-appears also for self-propelled agents. This suggests that the transition should also occur for the flow through constrictions of living agents (e.g., humans and sheep), an issue that has been elusive so far in experiments due to safety risks.

Pedestrian collective motion in competitive room evacuation.

When a sizable number of people evacuate a room, if the door is not large enough, an accumulation of pedestrians in front of the exit may take place. This is the cause of emerging collective phenomena where the density is believed to be the key variable determining the pedestrian dynamics. Here, we show that when sustained contact among the individuals exists, density is not enough to describe the evacuation, and propose that at least another variable -such as the kinetic stress- is required. We recorded evacuation drills with different degrees of competitiveness where the individuals are allowed to moderately push each other in their way out. We obtain the density, velocity and kinetic stress fields over time, showing that competitiveness strongly affects them and evidencing patterns which have been never observed in previous (low pressure) evacuation experiments. For the highest competitiveness scenario, we detect the development of sudden collective motions. These movements are related to a notable increase of the kinetic stress and a reduction of the velocity towards the door, but do not depend on the density.

Simulating competitive egress of noncircular pedestrians.

We present a numerical framework to simulate pedestrian dynamics in highly competitive conditions by means of a force-based model implemented with spherocylindrical particles instead of the traditional, symmetric disks. This modification of the individuals' shape allows one to naturally reproduce recent experimental findings of room evacuations through narrow doors in situations where the contact pressure among the pedestrians was rather large. In particular, we obtain a power-law tail distribution of the time lapses between the passage of consecutive individuals. In addition, we show that this improvement leads to new features where the particles' rotation acquires great significance.

Adipose-derived mesenchymal stromal (stem) cells differentiate to osteoblast and chondroblast lineages upon incubation with conditioned media from dental pulp stem cell-derived osteoblasts and auricle cartilage chondrocytes.

The potential of adipose-derived mesenchymal stromal (stem) cells (ADSCs) to differentiate into either osteoblasts or chondrocytes is controversial. In this study we investigated the multicapacity potential of ADSCs to differentiate towards adipocyte, osteoblast, and chondrocyte lineages when cells are seeded onto plastic in comparison with incubation with conditioned media (CM) obtained from differentiated cell types.ADSCs, obtained from liposuctions, were characterized for mesenchymal and hematopoietic markers by cytofluorimetry. Their differentiation capacity towards adipocytes, osteoblasts, and chondrocytes was investigated by histochemistry methods (Oil-Red-O staining, Safranin O and Alizarin Red staining, respectively). Dental pulp stem cells (DPSCs) and dedifferentiated auricle derived-chondrocytes were differentiated towards osteoblastic and chondrocytic lineages respectively, and the CM obtained from these cultures was used to induce differentiation of ADSCs. ADSCs were positive for mesenchymal markers (CD29, CD105, CD73, CD44), but not for hematopoietic lineage markers (CD14, CD34, CD45) and this behavior was conserved from the isolation up to the fifth passage. While ADSCs were readily differentiated in adipocytes, they were not towards chondrocytes and osteoblastic lineages, a behavior different from that of bone marrow-derived MSCs that differentiated into the three lineages at two weeks post-induction. Only ADSCs treated with CM from cultured chondrocytes and DPSCs, produced glycosaminoglycans and mineralized matrix. These results indicate that ADSCs need growth/morphogenic factor supplementation from the tissue environment to be appropriately differentiated to mesodermic lineages.

Penile length is a very important factor for cosmesis, function and psychosexual development in patients affected by hypospadias: Results from a long-term longitudinal cohort study.

Few studies of long-term outcome of hypospadias treatment in terms of voiding, surgical complications, sexual functioning, intimate relationships and cosmetic results have been investigated and contrasting results have been obtained so far. The aim of our study is to investigate the long-term outcome of urinary and sexual function, cosmesis and the quality of intimate relationships in a series of hypospadias. In this study, 42 patients who underwent surgery for hypospadias were prospectively followed for 15 years. Medical records provided the hypospadias data, the number of reconstructive operations and the reconstruction technique that was used. Patients underwent physical examination, including penile length measurement and completed International Prostatic Symptoms Score (I-PSS), International Index Of Erectile Function (IIEF 15) and the Penile Perception Score questionnaire (PPPS). Twenty patients agreed to participate in the study. At the enrolment, the median value of HOSE was 13, as regards PPPS, 18/20 (90%) were satisfied, while in 1998 only 80% were satisfied. No significant statistical difference has been reported from the results obtained at enrolment and those obtained at follow-up, in terms of PPPS (P = 0.81), IPSS and IIEF-15. Penile length was 6.5 cm flaccid and 10.5 cm stretched. Our data show how cosmesis, function and psychosexual development for these patients are highly connected to surgical outcome, which is understood to be a decrease in penile size.

Continuous-wave Pr³âº:BaY2F8 and Pr³âº:LiYF4 lasers in the cyan-blue spectral region.

We report on the first, to the best of our knowledge, continuous-wave quasi three-level lasers emitting in the cyan-blue spectral range in praseodymium-doped crystalline materials. Applying Pr(3+):BaY2F8 as an active medium, up to 201 mW of output power at 495 nm could be obtained with a slope efficiency of 27% under pumping with an optically pumped semiconductor laser (2ω-OPSL) at 480 nm. In the same pumping scheme using Pr(3+):LiYF4, output powers up to 70 mW were realized at 491 and 500 nm, respectively. With Pr(3+):BaY2F8, diode-pumped laser operation with up to 11% slope efficiency and 44 mW output power was also achieved. In the latter case, detailed investigations on the temperature dependency of the laser output were conducted. Moreover, comparative experiments were carried out for the first time, to the best of our knowledge, with green-emitting Pr(3+):BaY2F8 lasers at 524 and 553 nm both under diode and 2ω-OPSL excitation.

Pre-clinical evaluation of a new coral-based bone scaffold.

Coral is used worldwide for bone reconstruction. The favorable characteristics that make this material desirable for implantation are (i) osteoinduction, (ii) and osteoconduction. These proprieties have been demonstrated by in vivo studies with animal models and clinical trials over a twenty-year period. Also poly(2-hydroxyethylmethacrylate) [poly(HEMA)] is a widely used biomaterial. By using coral and poly(HEMA), a scaffold for bone reconstruction application has been recently synthesized. Cytological, histological and genetic analyses were performed to characterize this new alloplastic material. Four samples were analyzed: (a) white coral (WC), (b) red coral (RC), (c) WC plus polymer (WCP) and (d) RC plus polymer (RCP). Quantification of mitochondrial dehydrogenase activity by MTT assay was performed as indirect detector of cytotoxicity. In vivo effects were revealed by implanting corals and coral-based polymers in rabbit tibia. Samples were collected after 4 weeks and subjected to histological analysis. To evaluate the genetic response of cells to corals and coral-derived polymers an osteoblastlike cell line (i.e. MG63) was cultured in wells containing (a) medium, (b) medium plus corals and (c) medium plus two types of scaffolds (RCP or WCP). RNAs extracted from cells were retro-transcribed and hybridized on DNA 19.2K microarrays. No cytotoxicity was detected in corals and coral-based biopolymers. No inflammation or adverse effect was revealed by histological examination. By microarray analysis 154 clones were differentially expressed between RC and WC (81 up and 73 down regulated) whereas only 15 clones were repressed by the polymer. Histological evaluation not only confirmed that coral is a biocompatible material, but also that the polymer has no adverse effect. Microarray results were in agreement with cytological and histological analyses and provided further data regarding the genetic effects of RC, WC and the new polymer.

Long-term follow-up in infantile-onset lambert-eaton myasthenic syndrome.

Lambert-Eaton myasthenic syndrome is a neuromuscular junction disorder characterized by proximal limb muscle weakness, fatigability, decreased deep-tendon reflexes, and autonomic symptoms. There are 2 forms of Lambert-Eaton myasthenic syndrome: one most frequently associated with small-cell lung cancer (P-Lambert-Eaton myasthenic syndrome) and the other that is a pure autoimmune form (NP-Lambert-Eaton myasthenic syndrome). Lambert-Eaton myasthenic syndrome is a very rare disorder in children younger than age 12 years. Herein, we report a 25-year-old man with NP-Lambert-Eaton myasthenic syndrome, which onset was at the age of 10 years. To date, this is the most long-term follow-up of NP-Lambert-Eaton myasthenic syndrome in childhood. In our patient, the only symptomatic treatment with 3,4-diaminopyridine phosphate has been sufficient to guarantee him a good quality of life. Our data remind physicians to keep in mind the diagnosis of Lambert-Eaton myasthenic syndrome in children with a proximal myopathic pattern and they confirm the specificity of compound muscle action potential incremental pattern after brief maximal effort in Lambert-Eaton myasthenic syndrome.

ANT1 is reduced in sporadic inclusion body myositis.

To investigate ANT1 and NF-κB expression in inclusion body myositis (IBM) muscle and to verify their possible roles in the pathogenesis of the disease, we collected muscle samples from five patients with IBM, polimyositis (PM) and controls. p65 form of NF-κB was analyzed using immunocytochemistry, Western blot and EMSA. Western blot of ANT1 was performed and confirmed by gene expression study. Mann-Whitney test was used for groups comparisons. NF-κB (p65) was found over-expressed both with western blot and EMSA, either in IBM or PM patients versus controls (p < 0.01). Expression of ANT1 were lower in IBM samples versus both PM and controls (p < 0.01). ANT1 reduction and NF-κB over-expression in IBM muscle could explain the lack of apoptosis in such disease. Normal ANT1 expression in PM could be related to the scarcity of mitochondrial abnormalities in the disease, but it could also suggest that these two conditions diverge in activating different anti-apoptotic pathways.

Epidemiology of pulmonary embolism in Apulia from analysis of current data.

BACKGROUND AND AIMS: Pulmonary embolism (PE) is a relatively common cardiovascular emergency: present evidence suggests that PE is the third most acute cardiovascular disease after cardiac ischemic syndromes and stroke. The aim of this study is to evaluate hospital admissions for PE in the Apulia Region of Italy in the period 2001-2007 through an analysis of the Apulia Region hospital patient discharge database. METHODS: Patients were selected on the basis of admissions between 01/01/2001 and 31/12/2007 with ICD-9-CM code of 415.11 (Iatrogenic pulmonary embolism and infarction) or 415.19 (Other pulmonary embolism and infarction) as principal or secondary diagnosis. RESULTS: The number of patients selected from the database was 4,303. The raw annual admission data shows an increasing trend from 13.9x100,000 residents in 2001 to 18.9 x 100,000 residents in 2007. The average patient age was 68.7 years and 59% were females and 41%. There were 470 deaths in hospital (10.9% of patients). CONCLUSIONS: PE is associated with much health care and a substantial economic burden, yet many PE and general venous thromboembolism (VTE) events are preventable. It remains the responsibility of individual hospitals to identify specific areas in which they can improve their VTE prophylaxis rates to obtain positive results from the reporting initiatives and incentive programs.

Efficient in-band pumped Ho:LuLiF(4) 2 microm laser.

An efficient Ho:LuLiF(4) laser in-band pumped by a cladding-pumped Tm-doped silica fiber laser operating at 1937 nm is reported. At low-cavity output coupling, the Ho:LuLiF(4) laser yielded 5.1 W of output at a wavelength of 2066 nm for 8.0 W of absorbed pump power with a slope efficiency of 70%. At high-cavity output coupling, the lasing wavelength shifted to 2053 nm and the laser produced an output power of 5.4 W with a slope efficiency of 76%. The beam propagation factor (M(2)) was measured to be approximately 1.1 at the maximum output power confirming fundamental transverse mode (TEM(oo)) operation. The influence of resonator design on laser performance is discussed, along with prospects for further power scaling and improvement of the laser efficiency.

Diode-pumped single-frequency Tm:LiLuF(4) ring laser.

We report a room-temperature single-frequency Tm:LiLuF(4) laser with a maximum output power of 120 mW, a slope efficiency of 13%, and a wavelength tunability range from 1875 to 1895 nm. Both frequency and relative intensity noise have been investigated, showing an emission linewidth of approximately 300 kHz over 1 ms observation time and an intensity noise spectrum limited by quantum noise for Fourier frequencies larger than 5 MHz.

Morphostructural changes in transferred fasciocutaneous free flaps: a preliminary clinical and immunohistochemical report.

Many local factors, yet to be investigated, can promote changes in tissue transferred by microsurgical technique into the recipient site. Several studies have attempted to assess the nature of modifications that occur in the vascular network of such a flap after transfer. Although these investigations have interesting conclusions, the majority of them were based only on indirect evaluations. The aim of this study was to detect, by histological and statistical analysis, the morphostructural changes that occurred in fasciocutaneous free flaps transferred to the cephalic region or to the lower limb. Patients were enrolled in this study only when neither local inflammatory reactions nor systemic diseases were observed at the time of biopsy. Six patients consented to undergo biopsy at both the donor and the recipient area of a previously transferred fasciocutaneous free flap. Three flaps were used for facial reconstruction, and three others for lower limb reconstruction. Standard staining and immunohistochemical investigations were performed. The sections were also analysed by specific software. Statistical analysis of the data was performed using the student's t-test and Fisher's test. In five out of six transferred flaps (83%), there was increased microvascularity compared to the donor area. It was correlated to the neoangiogenesis in the dermal layer of the flaps. In five recipient sites there were more new vessels. In particular, a higher score of angiogenesis was observed both in the cheek (one flap) and in the non weight-bearing area of the foot (two flaps) (P<0.001). Some differences in microvascularity between the donor and the recipient site in the same flap were related to the specific recipient site. This represents the first demonstration of adaptation of fasciocutaneous free flaps to the recipient area, as well as to their new function, at both the macroscopic and microscopic level.

Microsurgical treatment of injury to peripheral nerves in upper and lower limbs: a critical review of the last 8 years.

Nerve injuries to the upper and inferior limbs represent a common event due both to home and working accidents. Minor traumas can lead to severe disabilities if a wrong treatment is carried out. From 1997 to 2005, 920 patients were observed with a total of 1,200 major or minor nerves injuries operated. In 852 cases acute lesions treated. In 68 patients we reviewed old injuries. In 707 patients direct nerve suture and in 145 patients nerve grafts were carried out. One to 8 years follow up observed. Different protocols were adopted according to the injury: simple cut, complex lesion or surgical revision. Results were evaluated during the follow up period with different models: motor evaluation in six levels (BMRC), sensitive evaluation in five levels (HIGHET), and global evaluation with four grades (from the worst to the best result). Nerve healing is a complex biological phenomenon influenced by many parameters related both to the patient characteristics and nerve lesion. Functional and esthetical outcomes vary also according to: age, cultural and economic condition, health status, and smoking. Post operative rehabilitation is mandatory to obtain an acceptable functional result. Nerve injuries to the upper and inferior limbs represent a challenge for the microsurgeon. Ultimate success in nerve surgery is judged by functional as well as cosmetic parameters. Only patients with the appropriate indication should be operated and always by a skilled surgeon.