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Abstract Background In both the US and Japan, the patient isolation policy for leprosy /Hansen's disease (HD) was preserved along with the isolation facilities, long after it had been proven to be scientifically unnecessary. This delayed policy termination caused a deprivation of civil liberties of the involuntarily confined patients, the fostering of social stigmas attached to the disease, and an inefficient use of health resources. This article seeks to elucidate the political process which hindered timely policy changes congruent with scientific advances. Methods Examination of historical materials, supplemented by personal interviews. The role that science played in the process of policy making was scrutinized with particular reference to the Garbage Can model. Results From the vantage of history, science remained instrumental in all period in the sense that it was not the primary objective for which policy change was discussed or intended, nor was it the principal driving force for policy change. When the argument arose, scientific arguments were employed to justify the patient isolation policy. However, in the early post-WWII period, issues were foregrounded and agendas were set as the inadvertent result of administrative reforms. Subsequently, scientific developments were more or less ignored due to concern about adverse policy outcomes. Finally, in the 1980s and 1990s, scientific arguments were used instrumentally to argue against isolation and for the termination of residential care. Conclusion Contrary to public expectations, health policy is not always rational and scientifically justified. In the process of policy making, the role of science can be limited and instrumental. Policy change may require the opening of policy windows, as a result of convergence of the problem, policy, and political streams, by effective exercise of leadership. Scientists and policymakers should be attentive enough to the political context of policies.

Passive infrared (IR) systems enable rapid detection of chemical vapors but are limited by size, weight, cost, and power. Previously, the authors reported a novel passive sensor that utilizes multiple IR filter/detector combinations to discriminate between different chemical vapors based on their unique IR absorption spectra in the same manner the human eye uses to generate colors. This approach enables a very small, compact, and low-power sensor system with the capability to discriminate between chemical vapors of interest and background chemicals. All previous work showed the capability of this sensor system in discriminating chemical vapors against a hot blackbody in a laboratory environment. Now the authors demonstrate the ability of this sensor system to discriminate between the chemical vapor agent simulant dimethyl methylphosphonate and ethanol against the cold sky in an outdoor environment., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Many optical applications, including free-space optical communications, lidar, and astronomical measurements, are impacted by the presence of light-scattering particles also known as obscurants. Scattering from particles consisting of sand, dust, dirt, and other substances can significantly degrade optical signals. For many obscurants, the index of refraction is dependent on the wavelength of light, and there exists a Christiansen wavelength (λ c ) at which scattering is at a minimum. At λ c the index of refraction of the scattering particles (n s ) matches that of the surrounding medium, in this case air (with refractive index n a ). This condition makes the scattering particulates almost invisible to the propagating light, minimizing scattering and increasing transmission at λ c . Previously, the authors showed a technique for measuring the index of refraction n (λ) and the extinction coefficient k (λ) using spectroscopic ellipsometry for various sand samples. Spectroscopic measurements on static sand samples demonstrated good agreement with the predicted spectral properties and highlighted the presence of a Christiansen feature near 8 µm. However, in outdoor environments, the scattering particles are never stationary but in a constant state of motion. In this work, spectroscopic measurements on dynamic sand samples (sand that is falling through the optical beam path) show two Christiansen features seen previously in predicted and observed static sand measurements. Additionally, we characterize, for the first time, transmission around a Christiansen feature using a tunable laser and show results consistent with other spectroscopic measurements., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

In order to model the propagation of light through a sand cloud, it is critical to have accurate data for the optical constants of the sand particles that comprise it. The same holds true for modeling propagation through particles of any type suspended in a medium. Few methods exist, however, to measure these quantities with high accuracy. In this paper, a characterization method based on spectroscopic ellipsometry (SE) that can be applied to a particulate material is presented. In this method, a polished disc of an adhesive compound is prepared, and its optical constants are measured. Next, a mixture of the adhesive and a sand sample is prepared and processed into a polished disc, and SE is performed. By treating the mixture as a Bruggeman effective medium, the optical constants of the particulate material are extracted. For verification of the proposed method, it is first applied to pure silica powder, demonstrating good agreement between measured optical constants and literature values. It is then applied to Arizona road dust, a standard reference material, as well as real desert sand samples. The resulting optical constant data is input into a rigorous scattering model to predict extinction coefficients for various types of sand. Modeling results are compared to spectroscopic measurements on static sand samples, demonstrating good agreement between predicted and measured spectral properties including the presence of a Christiansen feature near a wavelength of 8 µm., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Epidermolysis bullosa is characterized by cutaneous fragility and blistering. Historically, diagnosis is achieved by immunofluorescence mapping or transmission electron microscopy, both involving biopsy procedures. Genetic analysis, especially through next-generation sequencing, is an important tool for the diagnosis of this disease. In Brazil, access to diagnostic methods is limited, and consequently, most patients do not have an accurate diagnosis. Diagnosis allows the indication of prognosis and genetic counselling of the patient., Objectives: To evaluate the cost-effectiveness of a gene panel compared to immunofluorescence mapping and transmission electron microscopy by analyzing its benefits, limitations, and economic aspects., Methods: The gene panel included the 11 main genes associated with epidermolysis bullosa. The techniques were compared, assessing the average cost, advantages, and limitations, through a price survey and literature review., Results: Both immunofluorescence mapping and transmission electron microscopy require skin biopsy, are dependent on the investigator's expertise, and are subject to frequent inconclusive results. The gene panel is effective for the conclusive diagnosis of epidermolysis bullosa, presents high efficiency and accuracy, is economically feasible, and excludes the need for biopsy. The gene panel allows for prognosis, prenatal genetic diagnosis, and genetic counseling., Study Limitations: It was not possible to find laboratories that perform transmission electron microscopy for epidermolysis bullosa diagnosis in Brazil., Conclusion: This study supports the gene panel as the first-choice method for epidermolysis bullosa diagnosis., (Copyright © 2021 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. All rights reserved.)

Congenital absence of skin (CAS) is a clinical sign associated with the main types of epidermolysis bullosa (EB). Very few studies have investigated the genetic background that may influence the occurrence of this condition. Our objective was to investigate genotype-phenotype correlations on EB with CAS through a literature revision on the pathogenic variants previously reported. A total of 171 cases (49 EB simplex, EBS; 23 junctional EB, JEB; and 99 dystrophic EB, DEB), associated with 132 pathogenic variants in eight genes, were included in the genotype-phenotype analysis. In EBS, CAS showed to be a recurrent clinical sign in EBS with pyloric atresia (PA) and EBS associated with kelch-like protein 24; CAS was also described in patients with keratins 5/14 alterations, particularly involving severe phenotypes. In JEB, this is a common clinical sign in JEB with PA associated with premature termination codon variants and/or amino acid substitutions located in the extracellular domain of integrin α6β4 genes. In DEB with CAS, missense variants occurring close to non-collagenous interruptions of the triple-helix domain of collagen VII appear to influence this condition. This study is the largest review of patients with EB and CAS and expands the spectrum of known variants on this phenomenon., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We present a method of post-deposition tuning of the optical properties of thin film dielectric filters and mirrors containing chalcogenide glass (ChG) layers by thermally adjusting their refractive index. A common challenge associated with the use of ChG films in practical applications is that they suffer from slight run-to-run variations in optical properties resulting from hard-to-control changes in source material and deposition conditions. These variations lead to inconsistencies in optical constants, making the fabrication of devices with prescribed optical properties challenging. In this paper, we present new work that takes advantage of the large variation of a ChG films' refractive index as a function of annealing. We have carried out extensive characterization of the thermal index tuning and thickness change of arsenic selenide (As 2 Se 3 ) ChG thin films and observed refractive index changes larger than 0.1 in some cases. We show results for refractive index as a function of annealing time and temperature and propose a model to describe this behavior based on bond rearrangement. We apply thermal refractive index tuning to permanently shift the resonance of a Fabry-Perot filter and the cutoff wavelength of a Bragg reflector. The Bragg reflector, consisting of alternating As 2 Se 3 and CaF 2 layers, exhibits high reflectance across a ∼550 nm band with only five layers. Modeling results are compared with spectroscopic measurements, demonstrating good agreement.

Epidermolysis bullosa (EB) is a genodermatosis that encompasses a group of clinically and genetically heterogeneous disorders classified in four major types: EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB) and Kindler syndrome. Our aim was to characterize recurrent and novel mutations associated to EB in a sample of Brazilian patients. Eighty-seven patients (25 EBS, 4 JEB and 58 DEB) were studied. We performed a next-generation sequencing-based multigene panel through ion torrent technology including 11 genes: KRT5, KRT14, PLEC, TGM5, LAMA3, LAMB3, LAMC2, COL17A1, ITGB4, COL7A1, and FERMT1. A total of 72 different pathogenic or likely pathogenic variants were identified, 32 of them are novel. The causal variant was detected in 82 patients (efficiency of 94.3%). Pathogenic variants in the residue 125 of KRT14 were identified in 32% of all EBS patients. In DEB patients, four COL7A1 variants were quite frequent, some of them clustered in specific Brazilian regions. Our study extends the spectrum of known mutations in EB and describes, for the first time, the genetic profile of EB patients from Brazil., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We present recent advancements in structured, antireflective surfaces on optics, including crystals for high-energy lasers as well as windows for the infrared wavelength region. These structured surfaces have been characterized and show high transmission and laser damage thresholds, making them attractive for these applications. We also present successful tests of windows with antireflective surfaces that were exposed to simulated harsh environments for the application of these laser systems.

We report the case of an infant with Adams-Oliver syndrome, a rare disorder characterized by aplasia cutis congenita, defects of the limbs and extremities, and cutis marmorata telangiectatica. Other associated anomalies have been reported, such as facial dysmorphism, heart defects, and disorders of the central nervous system., (© 2014 Wiley Periodicals, Inc.)

We present a method of Cu(In,Ga)S2 (CIGS) thin film formation via conversion of layer-by-layer (LbL) assembled Cu-In-Ga oxide (CIGO) nanoparticles and polyelectrolytes. CIGO nanoparticles were created via a novel flame-spray pyrolysis method using metal nitrate precursors, subsequently coated with polyallylamine (PAH), and dispersed in aqueous solution. Multilayer films were assembled by alternately dipping quartz, Si, and/or Mo substrates into a solution of either polydopamine (PDA) or polystyrenesulfonate (PSS) and then in the CIGO-PAH dispersion to fabricate films as thick as 1-2 microns. PSS/CIGO-PAH films were found to be inadequate due to weak adhesion to the Si and Mo substrates, excessive particle diffusion during sulfurization, and mechanical softness ill-suited to further processing. PDA/CIGO-PAH films, in contrast, were more mechanically robust and more tolerant of high temperature processing. After LbL deposition, films were oxidized to remove polymer and sulfurized at high temperature under flowing hydrogen sulfide to convert CIGO to CIGS. Complete film conversion from the oxide to the sulfide is confirmed by X-ray diffraction characterization.

Barium copper sulfur fluoride (BaCuSF) is a p-type transparent conductor (p-TC) that, when doped with potassium, exhibits exceptionally high conductivity. The results of a detailed optical and electronic characterization of BaCuSF thin films deposited at a substrate temperature of 100 °C are presented. X-ray diffractometry shows the presence of a cubic BaCuSF phase. Spectroscopic measurements demonstrate that the films transmit from the visible through the mid-infrared with a band gap of 1.8 eV. Hall measurements indicate that the material is a degenerate semiconductor. As deposited, the films exhibit conductivity at room temperature of approximately 260 S/cm - among the highest reported room temperature conductivities for p-TCs. After post-deposition treatment in water, their conductivity increases to as high as 800 S/cm, and their band gap is reduced to 1.5 eV. The potential for low temperature deposition of p-type films with high conductivity and optical transmittance makes BaCuSF promising for several applications including flexible electronics and photovoltaics.

Waveguide amplifiers fabricated in Er3+-doped gallium lanthanum sulfide (GLS) glass are demonstrated. GLS is deposited onto fused silica substrates by RF magnetron sputtering, and waveguides are patterned by use of the lift-off technique. The waveguides exhibit a total internal gain of 6.7 dB (2.8 dB/cm) for a signal with a wavelength of 1.55 mum. This experiment is, to the best of our knowledge, the first demonstration of gain in an Er3+-doped chalcogenide glass waveguide. The fabrication methods we apply, if used with other rare earth dopants, could potentially be employed to produce sources operating in the mid-IR.

Bend loss effects can be a significant concern in the design and performance of diffused, buried waveguide devices. Since diffused, buried waveguides typically do not have analytical mode solutions, the bend mode must be expressed as an expansion of straight waveguide modes. For the case of buried ion-exchanged waveguides, the bend loss is affected by bend radius, the duration of the ion exchange and burial processes, as well as the size of the mask opening used to create the waveguides and applied field during burial. The bend loss effects for each of these variables are explored under typical fabrication conditions.

A model describing the angular selectivity of noise gratings in volume holographic recording materials is presented. The noise grating is treated as an ensemble of superimposed, statistically distributed planar gratings. Rigorous coupled-wave analysis is used to treat reconstruction with various polarization states. The model accounts for material properties such as thickness change, absorption, and the angular distribution of scattered light within the recording medium. Results show good agreement with noise gratings that are experimentally formed in a thick cationic ring-opening photopolymer material.

As a part of our effort to explore various aspects of ferrokinetics in infancy, the present study was designed to determine the timing of entry of an orally ingested iron isotope into circulating erythrocytes, and the effect of the level of dietary iron [0.3 mg/100 kcal (418.4 kJ) vs. 1.8 mg/100 kcal] after isotope administration on erythrocyte incorporation of the isotope. We administered the stable isotope, (58)Fe, orally to 56-d-old and 168-d-old infants. All infants were fed a low-iron formula (LF) before and until 5 h after isotope administration. Thereafter, half the infants were fed a formula high in iron (HF group) while the remaining infants continued to receive the LF (LF group) for an additional 28 d. The quantity of (58)Fe in circulating erythrocytes increased from 14 to 28 d after isotope administration was nearly constant from 28 through 84 d of age (plateau value) and decreased between 84 and 112 d. Erythrocyte incorporation of (58)Fe was greater by the 168-d-old infants than by the 56-d-old infants, presumably because of the lesser iron stores of the older infants. In the 56-d-old infants, erythrocyte incorporation of (58)Fe was greater by the LF than by the HF group, but this difference was not significant in the 168-d-old infants. Thus, at least in younger infants, the level of iron intake after administration of an iron isotope affects erythrocyte incorporation of the isotope. The fact that less isotope was present in erythrocytes 112 d than 84 d after administration indicates that the life span of erythrocytes of infants, even beyond the immediate newborn period, is less than the 120-d life span of erythrocytes in the adult.

Erythrocyte incorporation of an administered iron isotope has been used as a surrogate for iron retention on the assumption (validated in normal and iron-deficient adults) that 80-100% of the retained isotope is promptly incorporated into circulating erythrocytes. This assumption has not been validated in infants or children. The purpose of our study was to determine concurrently in normal infants absorption and erythrocyte incorporation of the stable isotope, (58)Fe. In a preliminary study (Study 1), we demonstrated that fecal excretion of ingested isotope occurs predominantly during the first 4 d after administration but continues beyond 7 d after ingestion, that is, beyond the point at which isotope in feces can be explained either by excretion of isotope that failed to enter enterocytes or by exfoliation of isotope-enriched enterocytes. In Study 2, we administered (58)Fe to nine younger (age 20-69 d) and nine older (age 165-215 d) term infants and collected feces for 11 d. Geometric mean retention of (58)Fe by the younger infants was 31.2% of intake at 4 d and 26.9% at 11 d, and by the older infants, 35.0% at 4 d and 32.5% at 11 d. Erythrocyte incorporation of (58)Fe 14 d after ingestion was 5.2% of the dose by the younger infants and 12.5% by the older infants. Utilization of retained (11 d) isotope thus was 19.8% by the younger infants and 38.3% by the older infants. We conclude that far less than 80% of retained isotope is promptly incorporated into erythrocytes (utilized) by infants.

Objectives: Young infants commonly show occult intestinal blood loss when fed cow's milk, but in older infants blood loss may be less common. This study examined intestinal blood loss in response to cow's milk feeding in normal 7(1/2)-month-old and 12-month-old infants., Study Design: Infants (n = 62) were fed formula for 1 month and then pasteurized cow's milk for 2 months. Stools were collected for quantitative determination of hemoglobin. Iron nutritional status was assessed., Results: Infants fed cow's milk from 7(1/2) months of age showed a significant increase in guaiac-positive stools and in stool hemoglobin concentration. These effects were largely limited to those infants who had been breast fed early in life. Infants fed cow's milk from 12 months of age at baseline had greater stool hemoglobin concentrations than 7(1/2)-month-old infants, but cow's milk produced no significant increase. In neither age group did cow's milk affect iron nutritional status., Conclusion: The response to cow's milk is attenuated in infants aged 7(1/2) months compared with younger infants. By 12 months of age, the response has disappeared entirely. We conclude that the gastrointestinal tract of healthy infants gradually loses its responsiveness to cow's milk.

Background: An adequate protein-energy ratio of infant formulas has been defined as one that permits growth similar to that of infants fed relatively generous protein-energy ratios, and serum concentrations of albumin and urea nitrogen no less than those observed in breast-fed infants. A safe ratio has been defined as one with no detectable adverse effects. The hypothesis was that a protein-energy ratio of 1.7 g/100 kcal is adequate and safe., Methods: Healthy male infants were fed Formula 1.7, a milk-based formula, as the sole source of energy from the 8th to the 112th day of life. Weight, length, and energy intake were measured; serum albumin and urea nitrogen were determined; and the results were compared with data from appropriate reference groups of infants., Results: Energy intake from 8 through 55 days was significantly higher than that of infants in the formula-fed reference group. Gain in weight was significantly more than that of the formula-fed reference group or of a breast-fed reference group, whereas gain in length was similar to that of the formula-fed reference group. Body mass index was significantly higher than that of either reference group, suggesting more fat accumulation in infants fed Formula 1.7. Plasma concentrations of albumin and urea nitrogen were similar to those of the breast-fed reference group., Conclusion: Infants fed Formula 1.7 received adequate intakes of protein. Because of the possibility that ad libitum feeding of diets with moderately inadequate protein-energy ratios is associated with increased food intake leading to excess weight gain, it is not possible to conclude that a protein-energy ratio of 1.7 g/100 kcal is safe.

Objective: The study tested the hypothesis that inclusion of palm olein (45% of fat) in the fat blend of a milk-based infant formula decreases the absorption of fat and calcium., Methods: Formula PO contained palm olein (45%) in addition to soy, coconut and high-oleic sunflower oils (20%, 20%, and 15%, respectively); Formula HOS contained high-oleic safflower oil (42%) in addition to coconut and soy oils (30% and 28%, respectively) and no palm olein. Fat and calcium levels in the two formulas were similar. In a balanced crossover design, fat and calcium absorption were determined in 10 normal infants ranging in age from 22 to 192 days. In three infants metabolic balance studies with complete separation of urine and feces were performed, whereas in seven infants excreta were in part collected at home, resulting in incomplete separation of urine and feces., Results: Mean (+/- SD) fecal excretion of fat was higher when Formula PO was fed than when Formula HOS was fed (0.55 +/- 0.29 vs. 0.09 +/- 0.04 g/kg/day; p < 0.001). Hence % fat absorption was lower with PO than with HOS (90.0 +/- 6.4 vs. 98.5 +/- 0.6% of intake; p < 0.01). The difference in percent fat absorption was explained by significantly (p < 0.05) lower % absorption of palmitic (16:0) and stearic (18:0) acids when Formula PO was fed than when Formula HOS was fed. Fecal excretion of calcium was higher with Formula PO than with Formula HOS (53.4 +/- 12.0 vs. 37.4 +/- 14.9 mg/kg/day; p < 0.01), and hence % calcium absorption was lower with Formula PO than with Formula HOS (37.5 +/- 11.5 vs. 57.4 +/- 14.9%; p < 0.001)., Conclusion: Absorption of fat and calcium by normal infants is lower when palm olein provides a substantial proportion of formula fat than when formula does not contain palm olein.

Although feeding of formulas with iron concentration of 215 mumol/L (12 mg/L) is a reliable means of preventing iron deficiency, high intakes of iron may adversely affect absorption of copper and zinc. Because data are not available to establish whether fortification at a lower level would result in equivalent iron absorption, we tested the hypothesis that iron absorption is greater by infants fed formulas with an iron concentration of 215 mumol/L (12 mg/L) than by those fed formulas with an iron concentration of 143 mumol/L (8 mg/L). Fifty-two normal infants entered the study at 112 +/- 4 d of age, and 46 of these were successfully studied until 196 d of age. Using the stable isotope 58Fe, we determined erythrocyte incorporation of iron by infants fed Formula 8 [iron approximately 143 mumol/L (8 mg/L)] and by infants fed Similac with Iron [iron approximately 215 mumol/L (12 mg/L)]. On each of three test days beginning at 154 d of age, a major portion of the formula was labeled with 58Fe. Geometric mean erythrocyte incorporation of iron adjusted for plasma ferritin concentration at 168 d of age was 4.82 mumol/d (0.269 mg/d) by infants fed Formula 8 and 5.21 mumol/d (0.291 mg/d) by infants fed Similac with Iron. Corresponding values at 196 d of age were 5.12 and 5.41 mumol/d (0.286 and 0.302 mg/d). The differences in quantity of iron incorporated into erythrocytes by infants fed Formula 8 and Similac with Iron were not statistically significant (P = 0.66 at 168 d of age, P = 0.75 at 196 d of age) and were judged to be nutritionally trivial. Because we were unable to provide support for our hypothesis that iron absorption is greater by infants fed formulas providing 215 mumol (12 mg) of iron per liter than by those fed formulas providing 143 mumol (8 mg) of iron per liter, we conclude that, pending the results of further studies, It is reasonable to decrease the iron concentration of iron-fortified infant formulas.

In an effort to obtain information about absorption of supplemental iron by breast-fed infants during the early months of life, we determined erythrocyte incorporation of a stable iron isotope, administered to 56-d-old breast-fed infants in the form of a 58Fe-labeled vitamin-iron supplement. Infants of similar age fed a milk-based formula low in iron (approximately 4 mg/L) were also studied. The 58Fe-labeled vitamin-iron supplement was given between feedings. Fourteen days after administration of 58Fe, mean erythrocyte incorporation of the isotope was 7.8% of the dose by breast-fed infants and 4.4% of the dose by formula-fed infants. The feeding-related difference was statistically significant, probably reflecting the greater quantities of inhibitors of iron absorption in the intestines of formula-fed infants. With mean iron intake from the 58Fe-labeled vitamin-iron supplement of 7.99 mg for the breast-fed infants, erythrocyte incorporation of 7.8% of the dose corresponded to 0.62 mg, a value in the range of the estimated requirement for absorbed iron. We conclude that 2-mo-old breast-fed infants are able to absorb nutritionally significant amounts of iron from an iron supplement.