Dirty Face

https://digitalcommons.library.umaine.edu/mmb-vp/1304/thumbnail.jp

Visual pathway function and structure in Wolfram syndrome: Patient age, variation and progression

Background/aimsTo report alterations in visual acuity and visual pathway structure over an interval of 1–3 years in a cohort of children, adolescents and young adults who have Wolfram syndrome (WFS) and to describe the range of disease severity evident in patients with WFS whose ages differed by as much as 20 years at first examination.MethodsAnnual, prospective ophthalmological examinations were performed in conjunction with retinal nerve fibre layer (RNFL) analysis. Diffusion tensor MRI-derived fractional anisotropy was used to assess the microstructural integrity of the optic radiations (OR FA).ResultsMean age of the 23 patients with WFS in the study was 13.8 years (range 5–25 years). Mean log minimum angle resolution visual acuity was 0.66 (20/91). RNFL thickness was subnormal in even the youngest patients with WFS. Average RNFL thickness in patients with WFS was 57±8 µ or ~40% thinner than that measured in normal (94±10 µ) children and adolescents (P&lt;0.01). Lower OR FA correlated with worse visual acuity (P=0.006). Subsequent examinations showed declines (P&lt;0.05) in visual acuity, RNFL thickness and OR FA at follow-up intervals of 12–36 months. However, a wide range of disease severity was evident across ages: some of the youngest patients at their first examination had deficits more severe than the oldest patients.ConclusionThe genetic mutation of WFS causes damage to both pregeniculate and postgeniculate regions of the visual pathway. The damage is progressive. The decline in visual pathway structure is accompanied by declines of visual function. Disease severity differs widely in individual patients and cannot be predicted from their age.</jats:sec

Economic Analysis of a Brackish Water Photovoltaic-Operated (BWRO-PV) Desalination System

The photovoltaic (PV)-powered reverse-osmosis (RO) desalination system is considered one of the most promising technologies in producing fresh water from both brackish and sea water, especially for small systems located in remote areas. We analyze the economic viability of a small PV-operated RO system with a capacity of 5 m3/day used to desalinate brackish water of 4000 ppm total dissolve solids, which is proposed to be installed in a remote area of the Babylon governorate in the middle of Iraq; this area possesses excellent insolation throughout the year. Our analysis predicts very good economic and environmental benefits of using this system. The lowest cost of fresh water achieved from using this system is US $3.98/ m3, which is very reasonable compared with the water cost reported by small-sized desalination plants installed in rural areas in other parts of the world. Our analysis shows that using this small system will prevent the release annually of 8,170 kg of CO2, 20.2 kg of CO, 2.23 kg of CH, 1.52 kg of particulate matter, 16.41 kg of SO2, and 180 kg of NOx

Analysis of post-transplant lymphoproliferative disorder (PTLD) outcomes with Epstein-Barr virus (EBV) assessments-a single tertiary referral center experience and review of literature

Post-transplant lymphoproliferative disorders (PTLDs) are lymphoid or plasmacytic proliferations ranging from polyclonal reactive proliferations to overt lymphomas that develop as consequence of immunosuppression in recipients of solid organ transplantation (SOT) or allogeneic bone marrow/hematopoietic stem cell transplantation. Immunosuppression and Epstein-Barr virus (EBV) infection are known risk factors for PTLD. Patients with documented histopathologic diagnosis of primary PTLD at our institution between January 2000 and October 2019 were studied. Sixty-six patients with PTLD following SOT were followed for a median of 9.0 years. The overall median time from transplant to PTLD diagnosis was 5.5 years, with infant transplants showing the longest time to diagnosis at 12.0 years, compared to pediatric and adolescent transplants at 4.0 years and adult transplants at 4.5 years. The median overall survival (OS) was 19.0 years. In the monomorphic diffuse large B-cell (M-DLBCL-PTLD) subtype, median OS was 10.7 years, while median OS for polymorphic subtype was not yet reached. There was no significant difference in OS in patients with M-DLBCL-PTLD stratified by quantitative EBV viral load over and under 100,000 copies/mL at time of diagnosis, although there was a trend towards worse prognosis in those with higher copies