Refractory multisystemic sarcoidosis, a diagnosis and treatment challenge: a case report.

BACKGROUND: Sarcoidosis is a multisystemic granulomatous disease of unknown origin. It is characterized by abnormal activation of lymphocytes and macrophages with the formation of granulomas. Most cases have asymptomatic pulmonary involvement. In case of symptoms, they have an excellent response to glucocorticoid therapy. We present a case of sarcoidosis with multi-organ involvement, refractory to multiple treatments including biological. Partial remission was achieved in it. CASE PRESENTATION: We report an interesting case of a 38-years-old Spanish woman treated by Heerfordt's syndrome (uveitis, parotiditis, fever and facial palsy) plus pulmonary hiliar adenopathy. A sarcoidosis diagnosis was confirmed by lung biopsy. She was initially treated with an 8 weeks course of medium dose oral glucocorticoids and tapered over 8 weeks with improvement. After the suspension of glucocorticoids a relapse occurs with severe ocular involvement and suspicion of neurological involvement. The patient received multiple lines of treatment with poor response. Finally, after the combination of cyclophosphamide with infliximab, the uveitis resolved, improving the neurological symptoms. CONCLUSIONS: Sarcoidosis is a benign disease in most cases. In a small percentage of cases behaves aggressively, requiring early diagnosis and immunosuppressive treatment to avoid sequelae. An adequate immunosuppressive therapy based on Anti TNF drugs should be started to minimize damage and improve the quality of life.The choice of treatment depends on the type and severity of the disease.

CdS/CdTe Heterostructures for Applications in Ultra-Thin Solar Cells.

The preparation of ultra-thin semi-transparent solar cells with potential applications in windows or transparent roofs entails several challenges due to the very small thickness of the layers involved. In particular, problems related to undesired inter-diffusion or inhomogeneities originated by incomplete coverage of the growing surfaces must be prevented. In this paper, undoped SnO2, CdS, and CdTe thin films with thickness suitable for use in ultra-thin solar cells were deposited with a radiofrequency (RF) magnetron sputtering technique onto conductive glass. Preparation conditions were found for depositing the individual layers with good surface coverage, absence of pin holes and with a relatively small growth rate adapted for the control of very small thickness. After a careful growth calibration procedure, heterostructured solar cells devices were fabricated. The influence of an additional undoped SnO2 buffer layer deposited between the conductive glass and the CdS window was studied. The incorporation of this layer led to an enhancement of both short circuit current and open circuit voltage (by 19 and 32%, respectively) without appreciable changes of other parameters. After the analysis of the cell parameters extracted from the current-voltage (I-V) curves, possible origins of these effects were found to be: Passivation effects of the SnO2/CdS interface, blocking of impurities diffusion or improvement of the band alignment.

Natural mortality estimates throughout the life history of the sea cucumber Isostichopus Badionotus (Holothuroidea: Aspidochirotida).

The Natural Mortality coefficient (M) is a key parameter for stock assessments. The need to establish age-specific natural mortality coefficients is widely recognized because M decreases rapidly over the early stages of the life cycle until it reaches a stable M value around the age-at-maturity. The aim of this study was to estimate M during the life cycle of the sea cucumber Isostichopus badionotus, a species under heavy fishing exploitation in the Caribbean Sea and the Gulf of Mexico. Coefficients M at age were estimated using two models: The Gnomonic Interval Model (GIM) and the Chen & Watanabe model. Two different scenarios were simulated considering early and late age-at-maturity. Estimated M values using the GIM model for the early maturity scenario were 2.15 to 2.35 year-1 (interquartile range) for the juvenile stage and 0.39 to 0.43 year-1 for the adult stage; for the late maturity scenario were 0.65 to 0.71 year-1 for the juvenile stage and 0.68 to 0.74 year-1 for the adult stage. The Chen & Watanabe model M estimates for juvenile stage were between 0.85 and 2.23 year-1 and 0.39 and 2.23 year-1 for the early and late maturity scenarios respectively; for adult stage were between 0.97 and 0.21 year-1 and 0.62 and 0.43 year-1 respectively. The GIM estimated high natural mortality rates during larval stages. These estimates provided a higher level of certainty for the population models to more effectively manage a fishery and improve stock assessments.

Annotated checklist of the coastal ichthyofauna from Michoacán State, Mexico.

This study is the first to complete an intensive and comprehensive list of the ichthyofauna of nearly all ecosystems of the Michoacán coast, Mexico. The resulting systematic checklist, supplemented with information from the literature and scientific collections, comprises 440 species belonging to two classes, 31 orders, 104 families, and 264 genera. The families with the highest number of species were Sciaenidae (30 spp.), Carangidae (26), Haemulidae (24), Serranidae (21), Paralichthyidae, and Gobiidae (13). Of the total species list, 134 represent first records for the Michoacán State, and one is a first record for Mexico. The results expand the number of known fish species of the Michoacán coast by almost one third and will help to develop conservation and management plans for this coastal zone.

The Effect of Bi-Sr and Ca-Sr Interactions on the Microstructure and Tensile Properties of Al-Si-Based Alloys.

The effect of bismuth and calcium additions on the microstructural characteristics and the tensile properties of the modified and grain-refined Al-Si based B319 alloys were studied in this paper. Based on the results obtained, it has been concluded that Bi reacts with both Sr and Mg, leading to severe demodification of the eutectic Si at Bi levels of 0.15%-0.6% Bi. Bismuth causes a decrease of the yield and tensile strengths for the as-cast and artificially aged conditions and an increase of yield strength in the solution heat-treated condition. The elongation increases with the Bi in the solution heat-treated condition. Based on this, Bi is found to be an efficient solid-solution strengthening element for these alloys. Thus, solution heat treatment, rather than the artificial aging, may be recommended for alloys containing about 1.0% Bi. Calcium has no significant demodification effect on the Sr-modified Si particles at 100-400 ppm Ca, and has a modifying effect at ~600 ppm Ca. The elongation increases with the Ca level at all conditions (as-cast, solution heat-treated, and artificially aged). A slight increase of the tensile strength in the heat-treated conditions was also observed. The lowest tensile properties either in the as-cast or the heat-treated conditions correspond to the most demodified-Si condition obtained at 408 ppm Ca. Calcium is, therefore, not as detrimental to the tensile properties as Bi.

Molecular identification of venous progenitors in the dorsal aorta reveals an aortic origin for the cardinal vein in mammals.

Coordinated arterial-venous differentiation is crucial for vascular development and function. The origin of the cardinal vein (CV) in mammals is unknown, while conflicting theories have been reported in chick and zebrafish. Here, we provide the first molecular characterization of endothelial cells (ECs) expressing venous molecular markers, or venous-fated ECs, within the emergent dorsal aorta (DA). These ECs, expressing the venous molecular markers Coup-TFII and EphB4, cohabited the early DA with ECs expressing the arterial molecular markers ephrin B2, Notch and connexin 40. These mixed ECs in the early DA expressed either the arterial or venous molecular marker, but rarely both. Subsequently, the DA exhibited uniform arterial markers. Real-time imaging of mouse embryos revealed EC movement from the DA to the CV during the stage when venous-fated ECs occupied the DA. We analyzed mutants for EphB4, which encodes a receptor tyrosine kinase for the ephrin B2 ligand, as we hypothesized that ephrin B2/EphB4 signaling may mediate the repulsion of venous-fated ECs from the DA to the CV. Using an EC quantification approach, we discovered that venous-fated ECs increased in the DA and decreased in the CV in the mutants, whereas the rest of the ECs in each vessel were unaffected. This result suggests that the venous-fated ECs were retained in the DA and missing in the CV in the EphB4 mutant, and thus that ephrin B2/EphB4 signaling normally functions to clear venous-fated ECs from the DA to the CV by cell repulsion. Therefore, our cellular and molecular evidence suggests that the DA harbors venous progenitors that move to participate in CV formation, and that ephrin B2/EphB4 signaling regulates this aortic contribution to the mammalian CV.

Magnamosis III: delivery of a magnetic compression anastomosis device using minimally invasive endoscopic techniques.

PURPOSE: Magnamosis creates a secure sutureless anastomosis through magnetic compression. In this study, we further develop and test delivery devices capable of creating a secure duodeno-colonic anastomosis using available minimally invasive endoscopic techniques. METHODS: Eight pigs underwent general anesthesia. Colonoscopy was used to deliver 1 magnetic ring to the hepatic flexure. Simultaneously, upper endoscopy delivered the other magnetic ring into the duodenum using a variety of techniques. The 2 magnetic rings were brought into magnetic proximity under laparoscopic guidance. The pigs were recovered and examined daily followed by sacrificing at 1, 2, 4, and 6 weeks. RESULTS: The device designed to deliver and release each magnetic ring evolved from using a guide wire and balloon devices to redesigning the magnetic ring casing with a groove to accommodate an endoscopic snare. Laparoscopic visualization assured safe magnet mating of intestinal segments. The duodeno-colonic anastomoses created with the snare yielded widely patent anastomoses. In vitro testing revealed excellent burst pressure. Histology revealed complete healing as early as 1 week. CONCLUSION: We redesigned the magnamosis device to facilitate delivery by endoscopic techniques. The snare technique allows endoscopic positioning and controlled release of the magnetic rings for a secure side-to-side duodeno-colonic anastomosis.

Diastolic Cardiac Pathology and Clinical Twin-Twin Transfusion Syndrome in Monochorionic/Diamniotic Twins.

OBJECTIVE: to identify differences in echocardiographic profiles of monochorionic/diamniotic pregnancies with early or mild twin-twin transfusion syndrome (TTTS), compared to monochorionic/diamniotic twins affected only by discordant growth or discordant fluid. STUDY DESIGN: retrospective evaluation of sonograms and echocardiograms of twin pregnancies referred for suspected TTTS. RESULTS: 112 monochorionic/diamniotic pairs were studied. 41 did not have/develop TTTS, 61 had Stage I/II TTTS. Ten developed TTTS after initially not meeting criteria. TTTS recipients had a higher rate of venous Doppler or tricuspid inflow abnormalities than purported "recipients" in non-TTTS pregnancies (86% vs. 37%, P<0.001). TTTS recipients had shorter tricuspid inflow duration/RR intervals than non-TTTS fetuses (32+/-6% versus 37+/-4%, P<0.001). Logistic regression and recursive partitioning identified shorter tricuspid inflow duration, longer isovolumic relaxation, and ductus venosus abnormality associated with TTTS. CONCLUSION: Diastolic pathology, specifically shorter tricuspid inflow duration, may be considered a hallmark of TTTS distinguishing these pregnancies from other monochorionic/diamniotic twin complications.

Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with significant neonatal morbidity and mortality. Although prenatal complete tracheal occlusion (cTO) causes hypoplastic CDH lungs to enlarge, improved lung function has not been demonstrated. Furthermore, cTO interferes with the dynamic pressure change and fluid flow associated with fetal breathing. PURPOSE: The purpose of the study was to assess a novel dynamic tracheal occlusion (dTO) device that preserves pressure changes and fluid flow. METHODS: In this pilot study, CDH was created in fetal lambs at 65 days of gestational age (GA). At 110 days GA, a cTO device (n = 3) or a dTO device (n = 4) was placed in the fetal trachea. At 135 days GA, lambs were delivered and resuscitated. Unoperated lamb co-twins (n = 5), sham thoracotomy lambs (n = 2), and untreated CDH lambs (n = 3) served as controls. RESULTS: Tracheal opening pressure, lung volume, lung fluid total protein, and phospholipid were significantly higher in the cTO group than in the dTO and unoperated control groups. Maximal oxygenation and lung compliance were significantly lower in the cTO group when compared with the unoperated control and dTO groups. CONCLUSION: Preliminary results suggest that in the fetal lamb CDH model, dTO restores normal lung morphometrics and function, whereas cTO leads to enlarged but less functional lungs.

Perinatal outcome of conservative management versus fetal intervention for twin reversed arterial perfusion sequence with a small acardiac twin.

OBJECTIVE: To examine the outcomes of patients with twin reversed arterial perfusion (TRAP) sequence in which the acardiac twin was

Artery and vein size is balanced by Notch and ephrin B2/EphB4 during angiogenesis.

A mutual coordination of size between developing arteries and veins is essential for establishing proper connections between these vessels and, ultimately, a functional vasculature; however, the cellular and molecular regulation of this parity is not understood. Here, we demonstrate that the size of the developing dorsal aorta and cardinal vein is reciprocally balanced. Mouse embryos carrying gain-of-function Notch alleles show enlarged aortae and underdeveloped cardinal veins, whereas those with loss-of-function mutations show small aortae and large cardinal veins. Notch does not affect the overall number of endothelial cells but balances the proportion of arterial to venous endothelial cells, thereby modulating the relative sizes of both vessel types. Loss of ephrin B2 or its receptor EphB4 also leads to enlarged aortae and underdeveloped cardinal veins; however, endothelial cells with venous identity are mislocalized in the aorta, suggesting that ephrin B2/EphB4 signaling functions distinctly from Notch by sorting arterial and venous endothelial cells into their respective vessels. Our findings provide mechanistic insight into the processes underlying artery and vein size equilibration during angiogenesis.