Congenital erythropoietic porphyria associated with myelodysplasia presenting in a 72-year-old man: report of a case and review of the literature

Congenital erythropoietic porphyria (CEP) is a rare autosomal recessive disease owing to the deficient activity of uroporphyrinogen III synthase, the fourth enzyme in the porphyrin–haem synthetic pathway. Of the porphyrias, it is the most mutilating type, usually presenting early in life. To date, 12 documented cases of adult onset CEP have been reported. We report the second oldest documented patient with late onset CEP with incidental findings of thrombocytopenia and myelodysplasia with bone-marrow sideroblasts. We further discuss several current and future treatment options for this therapeutically challenging disease.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/73760/1/j.1365-2133.2003.05040.x.pd

The effect of ultraviolet C radiation against different N95 respirators inoculated with SARS-CoV-2

OBJECTIVES: There are currently no studies that have examined whether one dosage can be uniformly applied to different respirator types to effectively decontaminate SARS-CoV-2 on N95 filtering facepiece respirators (FFRs). Health care workers have been using this disinfection method during the pandemic. Our objective was to determine the effect of UVC on SARS-CoV-2 inoculated N95 respirators and whether this was respirator material/model type dependent. METHODS: Four different locations (facepiece and strap) on five different N95 FFR models (3M 1860, 8210, 8511, 9211; Moldex 1511) were inoculated with a 10 μL drop of SARS-CoV-2 viral stock (8 × 10 RESULTS: UVC delivered using a dose of 1.5 J/cm(2), to each side, was an effective method of decontamination for the facepieces of 3 M 1860 and Moldex 1511, and for the straps of 3 M 8210 and the Moldex 1511. CONCLUSION: This dose is an appropriate decontamination method to facilitate the reuse of respirators for healthcare personnel when applied to specific models/materials. Also, some straps may require additional disinfection to maximize the safety of frontline workers. Implementation of widespread UVC decontamination methods requires careful consideration of model, material type, design, and fit-testing following irradiation

CATS Near Real Time Data Products: Applications for Assimilation Into the NASA GEOS-5 AGCM

From February 2015 through October 2017, the NASA Cloud-Aerosol Transport System (CATS) backscatter lidar operated on the International Space Station (ISS) as a technology demonstration for future Earth Science Missions, providing vertical measurements of cloud and aerosols properties. Owing to its location on the ISS, a cornerstone technology demonstration of CATS was the capability to acquire, process, and disseminate near-real time (NRT) data within 6 hours of observation time. CATS NRT data has several applications, including providing notification of hazardous events for air traffic control and air quality advisories, field campaign flight planning, as well as for constraining cloud and aerosol distributions in via data assimilation in aerosol transport models. Recent developments in aerosol data assimilation techniques have permitted the assimilation of aerosol optical thickness (AOT), a 2-dimensional column integrated quantity that is reflective of the simulated aerosol loading in aerosol transport models. While this capability has greatly improved simulated AOT forecasts, the vertical position, a key control on aerosol transport, is often not impacted when 2-D AOT is assimilated. Here, we present preliminary efforts to assimilate CATS aerosol observations into the NASA Goddard Earth Observing System version 5 (GEOS-5) atmospheric general circulation model and assimilation system using a 1-D Variational (1-D VAR) ensemble approach, demonstrating the utility of CATS for future Earth Science Missions

CATS Version 2 Aerosol Feature Detection and Applications for Data Assimilation

The Cloud Aerosol Transport System (CATS) lidar has been operating onboard the International Space Station (ISS) since February 2015 and provides vertical observations of clouds and aerosols using total attenuated backscatter and depolarization measurements. From February March 2015, CATS operated in Mode 1, providing backscatter and depolarization measurements at 532 and 1064 nm. CATS began operation in Mode 2 in March 2015, providing backscatter and depolarization measurements at 1064 nm and has continued to operate to the present in this mode. CATS level 2 products are derived from these measurements, including feature detection, cloud aerosol discrimination, cloud and aerosol typing, and optical properties of cloud and aerosol layers. Here, we present changes to our level 2 algorithms, which were aimed at reducing several biases in our version 1 level 2 data products. These changes will be incorporated into our upcoming version 2 level 2 data release in summer 2017. Additionally, owing to the near real time (NRT) data downlinking capabilities of the ISS, CATS provides expedited NRT data products within 6 hours of observation time. This capability provides a unique opportunity for supporting field campaigns and for developing data assimilation techniques to improve simulated cloud and aerosol vertical distributions in models. We additionally present preliminary work toward assimilating CATS observations into the NASA Goddard Earth Observing System version 5 (GEOS-5) global atmospheric model and data assimilation system

Use of Ablative Fractional CO2 Laser for Scars in Skin Type IV-VI

Background: Fractional 10,600 nm CO2laser’s use is increasing for scar treatment. Studies analyzing the use of ablative fractional laser in skin type IV–VI is scarce. Our objective is to present the use of fractionated CO2laser for scars in skin types IV–VI. Study: Eight patients with skin types IV–VI were treated at Henry Ford Hospital Scar Clinic for acne scars, surgical scars and traumatic scars. An ablative fractional CO2 laser, a superficial and a deep setting, was used alone or in combination for different scars. Clinical improvement and adverse effects were monitored with photography and patient assessment. Results: Three patients were treated for acne scars. For superficial rolling and boxcar scars, a setting of 80 mJ fluence and 3% density was used. For ice pick scars, a deep laser setting was used with 15 mJ fluence & 10–15% density. One patient also received post treatment filler after the laser treatment. Two patients with surgical scars and two patients with traumatic scars were treated with the deep laser setting of 50 mJ fluence &5% density. One patient received post-laser 5-fluorouracil and triamcinolone 10 mg/mL. One of the traumatic scar patients was treated with both the superficial (225 mJ fluence, 5% density) and deep (15 mJ fluence, 15% density) setting. Minor pinpoint bleeding and erythema was noted in all patients. Moderate to marked improvement was noted in scar appearance or texture. While transient PIH was noted, long term PIH was not evident. Conclusion: Studies that discuss the use of lasers in dark skin types include pigment specific, vascular and non-ablative fractional lasers, but not fractionated CO2laser. This study finds the use of ablative fractional CO2laser to be safe in skin types IV VI with a low risk of PIH. Limitations of the study include small sample size and adjunctive treatments

Polymer-derived Biosilicate®-like glass-ceramics: Engineering of formulations and additive manufacturing of three-dimensional scaffolds

Silicone resins, filled with phosphates and other oxide fillers, yield upon firing in air at 1100◦C, a product resembling Biosilicate® glass-ceramics, one of the most promising systems for tissue engineering applications. The process requires no preliminary synthesis of parent glass, and the polymer route enables the application of direct ink writing (DIW) of silicone-based mixtures, for the manufacturing of reticulated scaffolds at room temperature. The thermal treatment is later applied for the conversion into ceramic scaffolds. The present paper further elucidates the flexibility of the approach. Changes in the reference silicone and firing atmosphere (from air to nitrogen) were studied to obtain functional composite biomaterials featuring a carbon phase embedded in a Biosilicate®-like matrix. The microstructure was further modified either through a controlled gas release at a low temperature, or by the revision of the adopted additive manufacturing technology (from DIW to digital light processing)