Novel uses of laser therapy in Goltz syndrome.

Goltz syndrome is an X-linked dominant, multisystem birth defect due to PORCN mutation. The skin findings follow Blaschko's lines and often show epidermal atrophy and herniation of subcutaneous fatty tissue. Regarding treatment, light sources can offer a good therapeutic option for some manifestations of this rare disease and improve the aesthetic appearance of the skin lesions. We report two new cases of Goltz syndrome in which the cutaneous findings remarkably improved with pulsed dye laser and carbon dioxide laser.

Development and validation of a questionnaire to measure empowerment in adult patients with atopic dermatitis. The DATEMP questionnaire.

BACKGROUND AND OBJECTIVES: In recent years, the hypothesis that increasing the empowerment of patients can help to improve health outcomes and rationalize the use of health services has become relevant, especially for chronic diseases. Developing validated measurement tools is necessary to achieve this goal. In the field of dermatology, there are few studies related to empowerment of the patient. The aim of the present research is to develop and validate a self-administered questionnaire for adult patients diagnosed with atopic dermatitis (AD) in order to measure their level of empowerment. METHODS: After a literature review and the establishment of consulting groups of patients and dermatologists, a large battery of 35 items was generated, based on the dimensions included in previous empowerment descriptions. A multidisciplinary group selected the 20 most suitable items to include in the questionnaire. A cross-sectional study was carried out with the items included in the questionnaire as well as demographic and clinical characteristics. To reduce the number of items and measure the construct validity, an exploratory factorial analysis (EFA) of the primary components and varimax rotation were used. Cronbach's α was used to measure the reliability of the individual scales and the global questionnaire. RESULTS: 242 valid questionnaires were included. After completion of the EFA, the final Dermatitis Atópica EMPoderamiento (DATEMP) questionnaire was composed of 17 items that converged on four factors. The four scales had adequate reliability: "Knowledge" (Cronbach's alpha = 0.808), "Abilities" (Cronbach's alpha = 0.744), "Intention to change" (Cronbach's alpha = 0.798) and "Coping skills" (Cronbach's alpha = 0.772). The overall Cronbach's α of the questionnaire was 0.764. CONCLUSIONS: The DATEMP questionnaire is the first specific empowerment measurement instrument developed for patients with AD that has demonstrated adequate levels of reliability and construct validity. It is a self-administered questionnaire that is simple and quick to answer.

Effect of Flywheel Resistance Training on Balance Performance in Older Adults. A Randomized Controlled Trial.

This study aimed to assess the effects of flywheel resistance exercise training on postural stability and mobility in older adults and to investigate whether changes in power are related to improvements in balance. Thirty-six participants were randomly allocated to either a flywheel resistance exercise training group (ETG; n = 18) who underwent 6-weeks of training (2 to 3 days per week) or a control group (CON; n = 18). The average power and Mean Propulsive Velocity (MPV) were computed. Timed up-and-go test (TUG) and postural balance (anterior-posterior (AP) and medial-lateral (ML) center of pressure (COP) excursions) in different tasks were also assessed. Within-group analyses showed a significantly better performance in mobility (TUG, p < 0.01) and COPAP with open eyes (p < 0.05) for ETG. Between-groups analyses showed significant improvements in TUG (-0.68 [-1.25 to -0.98]) and in COPAP (-2.90 [-4.82 to -0.99]) in ETG compared with CON. Mean power also increased in ETG and the changes were related to those observed in stability (COP; r = -0.378, p < 0.05). In conclusion flywheel resistance exercise training improved balance and mobility in older adults as well as muscle power.

[Venolymphatic malformation with bone erosion. An unusual affectation in these lesions]. / Malformación venolinfática con erosión ósea. Una afectación rara en estas lesiones.

A 30 year old male presented with a cutaneous lesion on the distal area of the 5th toe with involvement of the outer edges. Macroscopically, it was a 1cm violaceous and keratotic lesion. Radiography showed an increase in soft tissue, possibly due to a vascular lesion. MRI showed a hyper-intense signal with erosion of the distal phalanx compatible with a low-flow vascular malformation. The distal phalanx was amputated. Histopathology revealed a lesion formed by venous and D2-40 positive lymphatic vessels. This case highlights the fact that even minimal skin involvement in vascular malformations may conceal an important deeper lesion, such as erosion of the cortical bone.

Scaling laws and bulk-boundary decoupling in heat flow.

When driven out of equilibrium by a temperature gradient, fluids respond by developing a nontrivial, inhomogeneous structure according to the governing macroscopic laws. Here we show that such structure obeys strikingly simple scaling laws arbitrarily far from equilibrium, provided that both macroscopic local equilibrium and Fourier's law hold. Extensive simulations of hard disk fluids confirm the scaling laws even under strong temperature gradients, implying that Fourier's law remains valid in this highly nonlinear regime, with putative corrections absorbed into a nonlinear conductivity functional. In addition, our results show that the scaling laws are robust in the presence of strong finite-size effects, hinting at a subtle bulk-boundary decoupling mechanism which enforces the macroscopic laws on the bulk of the finite-sized fluid. This allows one to measure the marginal anomaly of the heat conductivity predicted for hard disks.

An immunohistochemical study of verrucous hemangiomas.

BACKGROUND: According to the International Society for the study of vascular Anomalies, vascular anomalies are classified as vascular neoplasms and vascular malformations. In some vascular lesions, categorization as a neoplasm or malformation has not been established with confidence so far. In order to further clarify the nosology of verrucous hemangioma, we studied 13 cases. OBJECTIVE: To analyze immunohistochemical characteristics of verrucous hemangiomas in order to gain further insight in its histogenesis. METHODS: We carried out a retrospective review. Immunohistochemical expression for Wilms tumor 1 (WT1), Glut-1 and D2-40 was performed in 13 cases. RESULTS: Immunohistochemistry performed with Glut-1 and WT1 showed positive staining in all lesions. All verrucous hemangiomas lacked D2-40 immunostaining. CONCLUSIONS: This is the first report in the literature investigating WT1 in verrucous hemangioma in order to further clarify the nosology of this vascular anomaly. Despite the clinical features of verrucous hemangioma, which are similar to those seen in vascular malformations, verrucous hemangioma exhibited an immunoprofile similar to vascular neoplasms, according to WT1 and Glut-1 positivity.

Estudio comparativo de disgregación de diferentes formulaciones de olanzapina / Comparative study on the disintegration of different olanzapine formulations

Las formas de dosificación bucodispersables son productos farmacéuticos que se disgregan y disuelven rápidamente en la saliva cuando se colocan en la boca. Hay distintos tipos de formulaciones y procesos de fabricación que pueden emplearse para su elaboración. En este trabajo, se estudia las diferencias entre los genéricos bucodispersables con 10 mg de olanzapina comercialmente disponibles en España y Zyprexa Velotab reconocido como innovación galénica. Se han analizado distintos aspectos, tales como forma y tamaño, formulación cualitativa, tiempo de disgregación in vitro en saliva artificial y material de acondicionamiento. Entre todos los productos analizados, Zyprexa Velotab fue el que tuvo una ultrarrápida disgregación en 3 segundos y el menor residuo de dispersión (AU)

Orally dispersable dosage forms are pharmaceutical products that disintegrate and dissolve rapidly in saliva when placed in the mouth. There are several types of formulations and manufacturing technologies, which may be used to produce orodispersibles. This work investigates different generic 10 mg olanzapine orodispersible products commercially available in Spain and the innovative Zyprexa Velotab manufactured by Eli Lilly & Co. Several aspects such as the shape and size, qualitative formulation, in vitro disintegration time in artificial saliva and packaging are analyzed. Among all the products tested Zyprexa Velotab showed an ultra fast disintegration in 3 seconds and the lowest dispersión (AU)

Leukonychia totalis associated with multiple pilar cysts: report of a five-generation family: FLOTCH syndrome?

The association of familial totalis leukonychia with multiple pilar cysts is a rare condition that could represent a separate syndromic entity. Since Bauer described a family with totalis leukonychia and sebaceous cysts in 1920, only four new affected families have been reported. We report a five-generation family with a total leukonychia and multiple pilar cysts on the scalp. The hypothesis of a deficiency of a gene regulating the structure of keratin has been postulated but the exact genetic mechanism has not been yet determined. In our family, no other keratinizing structures were involved.

Symmetries in fluctuations far from equilibrium.

Fluctuations arise universally in nature as a reflection of the discrete microscopic world at the macroscopic level. Despite their apparent noisy origin, fluctuations encode fundamental aspects of the physics of the system at hand, crucial to understand irreversibility and nonequilibrium behavior. To sustain a given fluctuation, a system traverses a precise optimal path in phase space. Here we show that by demanding invariance of optimal paths under symmetry transformations, new and general fluctuation relations valid arbitrarily far from equilibrium are unveiled. This opens an unexplored route toward a deeper understanding of nonequilibrium physics by bringing symmetry principles to the realm of fluctuations. We illustrate this concept studying symmetries of the current distribution out of equilibrium. In particular we derive an isometric fluctuation relation that links in a strikingly simple manner the probabilities of any pair of isometric current fluctuations. This relation, which results from the time-reversibility of the dynamics, includes as a particular instance the Gallavotti-Cohen fluctuation theorem in this context but adds a completely new perspective on the high level of symmetry imposed by time-reversibility on the statistics of nonequilibrium fluctuations. The new symmetry implies remarkable hierarchies of equations for the current cumulants and the nonlinear response coefficients, going far beyond Onsager's reciprocity relations and Green-Kubo formulas. We confirm the validity of the new symmetry relation in extensive numerical simulations, and suggest that the idea of symmetry in fluctuations as invariance of optimal paths has far-reaching consequences in diverse fields.

Capillary-lymphatic malformation, kaposiform hemangioendothelioma and delayed Kasabach-Merritt phenomenon.

According to International Society for the Study of Vascular Anomalies classification, vascular anomalies are mainly divided into two groups: vascular tumors and vascular malformations. Nevertheless, a small group of patients present clinical and/or histopathologic overlapping features. We report a case of a 4-month-old boy that presented a vascular lesion on his right buttock with involvement of abdominal wall muscles, abdominal cavity and drainage to primitive iliac by a tortuous drainage vein. Surgery was performed and histopathology demonstrated a combined vascular malformation. Six months later he developed a thrombocytopenia and repeat magnetic resonance imaging revealed a new solid mass involving the majority of the abdominal cavity. At 18 months of age the patient developed a Kasabach-Merrit phenomenon and treatment with vincristine, interferon and then acetyl-salicilic acid and dypiridamol was started. No response in platelet counts was obtained and one more surgery was perfomed. On this occasion a histopathologic study revealed vascular malformation areas intermingled with areas of kaposiform hemangioendothelioma. This patient demonstrates the Kasabach-Merritt phenomenon with kaposiform hemangioendothelioma arising within a pre-existing combined vascular malformation.

Arteriovenous haemangioma in liver disease. Treatment with carbon dioxide laser vaporization in five cases.

BACKGROUND: Arteriovenous haemangioma (AVH) is considered a rare, benign, acquired, cutaneous tumour of vascular origin. Recently, a variant associated with chronic hepatic disease has been described. The usual treatment is surgical resection but no other treatments have been reported. OBJECTIVE: To evaluate the results obtained in the treatment of AVH with carbon dioxide laser vaporization. METHODS: Every patient with AVH related to chronic hepatic disease treated in the laser unit was reviewed. Five patients were treated with carbon dioxide laser vaporization. The first pass of treatment was performed in defocused mode at 2 W/cm(2). After this, several passes were performed in order to clear the entire lesion. One session of treatment was necessary for three patients, and the other two patients needed two sessions. RESULTS: The tumoral mass as well as the pulse disappeared in all lesions; total clearance was obtained in four of the five cases. In the postoperative time, no bleeding or haemorrhage were observed. No significant secondary effects of treatment were present. The cosmetic outcome was excellent in all cases. CONCLUSION: Carbon dioxide laser vaporization may be an alternative treatment for cases of AVH in chronic hepatic disease: the procedure is easy and with good cosmetic outcome.

Actinic cheilitis: evolution to squamous cell carcinoma after carbon dioxide laser vaporization. A study of 43 cases.

BACKGROUND: Actinic cheilitis (AC) is a precancerous lesion of the lip. Treatment of AC is indicated for prevention of squamous cell carcinoma (SCC), although the exact transition rate of AC to SCC is unknown. Carbon dioxide (CO(2)) laser vaporization seems to be an adequate therapy for AC, but there are no references about the evolution rate of AC to SCC after this treatment. OBJECTIVE: To evaluate the results obtained after treatment of AC by CO(2) laser vaporization in comparison with other treatment modalities and the evolution rate of AC to SCC after CO(2) laser treatment. METHODS: A retrospective review identified 43 patients with AC treated with CO(2) laser vaporization at our hospital from 2002 to 2006. Clinical and therapeutic information was evaluated. All patients were followed for more than 15 months after treatment. RESULTS: After a mean follow-up period of 29.4 months, 3/43 treated AC showed local recurrence. Another two patients developed SCC in the treated field. A residual scar was clinically evident in two patients. CONCLUSIONS: CO(2) laser vaporization with an adequate postoperative follow-up is an effective treatment for chronic AC. Nevertheless, some patients (4,6%) went on to develop lip SCC. The follow-up of these patients is mandatory.

Venous malformations with lip involvement: palliative treatment with carbon dioxide laser vaporization in five cases.

BACKGROUND: The treatment of venous malformations remains controversial. Traditional surgical excision is only possible in a few cases. Numerous sclerosing agents have been used, but none of them are ideal or absolutely safe for the treatment of venous disorders. In isolated cases, an expectative control would be a good option. OBJECTIVE: To report our experience with CO(2) laser vaporization as palliative treatment in five cases of head and neck venous malformations with lip involvement. METHODS: Five patients were treated with a CO(2) laser in superpulsed, focused mode at 2 W/cm(2), with several passes in each session. Patients were aged from 16 to 49 years old (mean: 36.6 years). Three sessions of treatment were performed in two patients while only one session was used in the rest. The follow-up was 6-36 months (mean: 22.8 months). RESULTS: Significant cosmetic improvement with flattening of the lip surface was achieved in all patients. CONCLUSION: Carbon dioxide laser vaporization can be considered as one method of choice for an effective palliative treatment of lip involvement in venous malformations.