Measuring refugees' capabilities: translation, adaptation, and valuation of the OxCAP-MH into Juba Arabic for use among South Sudanese male refugees in Uganda.

BACKGROUND: Forcibly displaced populations are highly vulnerable to psychosocial distress and mental disorders, including alcohol misuse. In an ongoing trial that seeks to develop a transdiagnostic intervention addressing psychological distress and alcohol use disorders among conflict-affected populations, we will carry out a cost-effectiveness evaluation using a capability-based Oxford Capabilities Mental Health (OxCAP-MH) measure. The OxCAP-MH is a 16-item questionnaire developed from the Capability Approach, that covers multiple domains of functioning and welfare. The aim of the current paper is to present the results of the translation, cultural adaptation and valuation of the OxCAP-MH into Juba Arabic for South Sudanese refugees living in Uganda. We adhered to the official Translation and Linguistic Validation process of the OxCAP-MH. To carry out the translation, the Concept Elaboration document, official English version of the OxCAP-MH, and the Back-Translation Review Template were used. Four independent translators were used for forward and back translations. The reconciled translated version was then piloted in two focus group discussions (N = 16) in Rhino refugee settlement. A most important to least important valuation of the sixteen capability domains covered in the OxCAP-MH was also done. RESULTS: The Juba Arabic version of the OxCAP-MH was finalized following a systematic iterative process. The content of the Juba Arabic version remained unchanged, but key concepts were adapted to ensure cultural acceptability, feasibility, and comprehension of the measure in the local context of Rhino refugee settlement. Most participants had low levels of literacy and required support with filling in the tool. Participants suggested an additional capability that is currently not reflected in the OxCAP-MH, namely access to food. Furthermore, discussions around the valuation exercise of the sixteen domains led to two separate importance scales, which showed relevant differences. CONCLUSIONS: In this context, the OxCAP-MH was considered culturally acceptable. The valuation exercise proved cognitively demanding. Participants voiced confusion over how to answer the questions on the OxCAP-MH instrument due to low levels of literacy. These concerns invite consideration for future research to consider how measures such as the OxCAP-MH can be made more accessible to individuals with low literacy rates in resource poor settings.

Multiscale static and dynamic mechanical study of the Turritella terebra and Turritellinella tricarinata seashells.

Marine shells are designed by nature to ensure mechanical protection from predators and shelter for molluscs living inside them. A large amount of work has been done to study the multiscale mechanical properties of their complex microstructure and to draw inspiration for the design of impact-resistant biomimetic materials. Less is known regarding the dynamic behaviour related to their structure at multiple scales. Here, we present a combined experimental and numerical study of the shells of two different species of gastropod sea snail belonging to the Turritellidae family, featuring a peculiar helicoconic shape with hierarchical spiral elements. The proposed procedure involves the use of micro-computed tomography scans for the accurate determination of geometry, atomic force microscopy and nanoindentation to evaluate local mechanical properties, surface morphology and heterogeneity, as well as resonant ultrasound spectroscopy coupled with finite element analysis simulations to determine global modal behaviour. Results indicate that the specific features of the considered shells, in particular their helicoconic and hierarchical structure, can also be linked to their vibration attenuation behaviour. Moreover, the proposed investigation method can be extended to the study of other natural systems, to determine their structure-related dynamic properties, ultimately aiding the design of bioinspired metamaterials and of structures with advanced vibration control.

Prevalence of Bartonella spp., haemotropic Mycoplasma spp. and others vector-borne pathogens in private-owned dogs and cats, Egypt.

Vector-borne pathogens have been increasingly investigated for their impact on dog and cat health and their zoonotic potential. The aim of this study was to investigate the prevalence estimates of selected vector-borne pathogens in client-owned pets from the Giza and Cairo governorates, Egypt.  Out of 200 dogs and 100 cats, 94 (47%) and 23 (23%) were positive for at least one of the tested pathogens (P<0.0001). In particular, 84 (42%) dogs and 3 (3%) cats tested PCR-positive for Bartonella spp. (P<0.0001). A significantly higher prevalence of Bartonella spp. was detected in dogs from the rural areas of the Giza governorate (60/77, 79.2%, P<0.0001) compared to those from Cairo governorate. Bartonella henselae was the dominant species infecting dogs (81/200, 40.5%) followed by Candidatus Bartonella merieuxii (3/200, 1.5%), while B. henselae (2/100, 2%) and B. clarridgeiae were rare in cats. Haemoplasma DNA was detected in 17% (34/200) of dogs and 20% (20/100) of cats with increased risk in dogs from Giza rural areas (21/77, 27.27%, P=0.002) and from both dogs (16/63, 25.40%, P=0.03) and cats (7/14, 50%, P<0.002) with anemia. Candidatus Mycoplasma haematoparvum (30/200, 15%) and Mycoplasma haemocanis (4/200, 2%) in dogs and Candidatus Mycoplasma haemominutum (18/100, 18%) and M. haemofelis (2/100, 2%) in cats were detected. Additionally, 2 dogs were positive for C. burnetii DNA. Coinfections were detected in dogs, with the majority (23/200, 11.5%) including B. henselae and C.M. haematoparvum, followed by Mycoplasma haemocanis and C.M. haematoparvum (2/200, 1%) and B. henselae, CMhp and C. burnetii (2/200, 1%). Haemoplasma infection was high in Egyptian dogs and cats with a high prevalence for zoonotic Bartonella spp. in dogs with anemia, highlighting the need to investigate these agents in the diagnostic algorithm of anemia and to adopt preventive measures to protect both animal and human health.

Risk of misclassification during diagnosis of malnutrition in retroperitoneal sarcoma patients.

BACKGROUND: Retroperitoneal sarcoma (RPS) is a rare tumour that can reach exceptionally large size at diagnosis and affects body weight with its volume. We investigated the risk of nutritional status misclassification based on the Global Leadership Initiative on Malnutrition (GLIM) criteria in RPS patients. METHODS: We retrospectively analyzed the baseline data of patients with primary RPS including anthropometry, Malnutrition Universal Screening Tool (MUST) score and skeletal muscle index (SMI) calculated on preoperative CT scan. Phenotypic GLIM criteria considered were non-volitional weight loss, low-BMI and low muscle mass. MUST score, being inclusive of weight loss, was chosen as its surrogate. Reduced muscle mass was defined with SMI cut-offs <38.5-52.4 cm2/m2 for female and male respectively. RESULTS: From 2018 to 2020, 100 consecutive patients (male/female: 48/52) were included. Median age was 61 (48-68) years, median BMI 24.6 (21.5-27.4) kg/m2. Seven patients (7%) reported MUST score ≥2 and 80 (80%) score 0. Five patients (5%) were underweight, 44 (44%) overweight and obese. Sixty patients (60%) presented low SMI, of those 56 (93%) were normal or overweight, and 46 (77%) not at nutritional risk according to the MUST score. Both underweight BMI and MUST score ≥2 were not correlated with low SMI (p = 0.406, p = 0.612, respectively). No relevant discrepancy was found in the postoperative course of patients with low or normal muscle mass, although SMI was able to better characterize nutritional risk in the proportion of patents with high-grade liposarcoma and predicted worst overall survival. CONCLUSION: GLIM criteria are a useful tool for diagnosis of malnutrition. However, our analysis suggests that phenotypic criteria are not interchangeable with each other. An altered body composition is often hidden in patients with retroperitoneal sarcoma and the use of weight-based classification does not seem to be appropriate, enhancing the role of mass muscle measurement to prevent misdetections of malnourished patient.

Higher testosterone is associated with increased inflammatory markers in women with SARS-CoV-2 pneumonia: preliminary results from an observational study.

PURPOSE: Objective of this study was to assess the association between testosterone (T) levels and biochemical markers in a cohort of female patients admitted for SARS-CoV-2 infection in a respiratory intensive care unit (RICU). METHODS: A consecutive series of 17 women affected by SARSCoV-2 pneumonia and recovered in the RICU of the Hospital of Mantua were analyzed. Biochemical inflammatory markers as well as total testosterone (TT), calculated free T (cFT), sex hormone-binding globulin (SHBG), and luteinizing hormone (LH) were determined. RESULTS: TT and cFT were significantly and positively associated with PCT, CRP, and fibrinogen as well as with a worse hospital course. We did not observe any significant association between TT and cFT with LH; conversely, both TT and cFT showed a positive correlation with cortisol. By LOWESS analysis, a linear relationship could be assumed for CRP and fibrinogen, while a threshold effect was apparent in the relationship between TT and procalcitonin, LDH and ferritin. When the TT threshold value of 1 nmol/L was used, significant associations between TT and PCT, LDH or ferritin were observed for values above this value. For LDH and ferritin, this was confirmed also in an age-adjusted model. Similar results were found for the association of cFT with the inflammatory markers with a threshold effect towards LDH and ferritin with increased LDH and ferritin levels for values above cFT 5 pmol/L. Cortisol is associated with serum inflammatory markers with similar trends observed for TT; conversely, the relationship between LH and inflammatory markers had different trends. CONCLUSION: Opposite to men, in women with SARS-CoV-2 pneumonia, higher TT and cFT are associated with a stronger inflammatory status, probably related to adrenal cortex hyperactivity.

Early childhood vaccination coverage and timeliness by macro-area of origin in children born to foreign women residing in Italy.

OBJECTIVES: Country of origin might affect vaccine uptake in children born to immigrants. We aimed to evaluate differences in childhood vaccination coverage (VC) and timeliness by macro-area of origin of foreign mothers residing in Italy. STUDY DESIGN: Multicentre retrospective birth cohorts. METHODS: We analysed data of 23,287 children born in 2009-2014 to foreign women in the cities of Rome, Turin and Treviso. We retrieved data through record-linkage of the population, vaccination and birth registries. We estimated VCs at different ages for vaccines against tetanus, measles and meningococcal group-C, using the Kaplan-Meier method. Factors associated with vaccine uptake were evaluated using multilevel Poisson models. RESULTS: Estimates of VC at any age and for all antigens were significantly lower in children born to women from Asia and higher in children born to women from Africa, as compared to other macro-areas. Similar differences by area of origin were observed for timeliness; independently of mother's sociodemographic characteristics and neonatal outcomes, the probability of delay vaccination after 2 years of age for each antigen was highest in children born to women from Asia. The risk of missed vaccination for all antigens was significantly higher in children born to younger and unemployed women. CONCLUSIONS: Factors related to area of origin (e.g., cultural habits, language skills) are likely to affect parents' decision to vaccinate their children. These factors, as well as sociodemographic characteristics, should be adequately investigated and addressed to increase vaccine uptake in foreign children, especially those born to Asian women.

Topographically guided hierarchical mineralization.

Material platforms based on interaction between organic and inorganic phases offer enormous potential to develop materials that can recreate the structural and functional properties of biological systems. However, the capability of organic-mediated mineralizing strategies to guide mineralization with spatial control remains a major limitation. Here, we report on the integration of a protein-based mineralizing matrix with surface topographies to grow spatially guided mineralized structures. We reveal how well-defined geometrical spaces defined within the organic matrix by the surface topographies can trigger subtle changes in single nanocrystal co-alignment, which are then translated to drastic changes in mineralization at the microscale and macroscale. Furthermore, through systematic modifications of the surface topographies, we demonstrate the possibility of selectively guiding the growth of hierarchically mineralized structures. We foresee that the capacity to direct the anisotropic growth of such structures would have important implications in the design of biomineralizing synthetic materials to repair or regenerate hard tissues.

Nanoscale structural and electrical properties of graphene grown on AlGaN by catalyst-free chemical vapor deposition.

The integration of graphene (Gr) with nitride semiconductors is highly interesting for applications in high-power/high-frequency electronics and optoelectronics. In this work, we demonstrated the direct growth of Gr on Al0.5Ga0.5N/sapphire templates by propane (C3H8) chemical vapor deposition at a temperature of 1350 °C. After optimization of the C3H8 flow rate, a uniform and conformal Gr coverage was achieved, which proved beneficial to prevent degradation of AlGaN morphology. X-ray photoemission spectroscopy revealed Ga loss and partial oxidation of Al in the near-surface AlGaN region. Such chemical modification of a ∼2 nm thick AlGaN surface region was confirmed by cross-sectional scanning transmission electron microscopy combined with electron energy loss spectroscopy, which also showed the presence of a bilayer of Gr with partial sp2/sp3 hybridization. Raman spectra indicated that the deposited Gr is nanocrystalline (with domain size ∼7 nm) and compressively strained. A Gr sheet resistance of ∼15.8 kΩ sq-1 was evaluated by four-point-probe measurements, consistently with the nanocrystalline nature of these films. Furthermore, nanoscale resolution current mapping by conductive atomic force microscopy indicated local variations of the Gr carrier density at a mesoscopic scale, which can be ascribed to changes in the charge transfer from the substrate due to local oxidation of AlGaN or to the presence of Gr wrinkles.

Nutritional status and body composition assessment in patients with a new diagnosis of advanced solid tumour: Exploratory comparison of computed tomography and bioelectrical impedance analysis.

OBJECTIVE: Low skeletal muscle is a common characteristic of cancer-related malnutrition and a predictor of poorer prognosis in oncological patients. In this study we evaluated nutritional status and altered body composition using computed tomography (CT) and bioelectrical impedance analysis (BIA) in newly diagnosed patients. Our purpose was to compare the results of two available techniques to assess body composition suggested by the guidelines and some diagnostic criteria to identify malnutrition. METHODS: In a prospective study, patients with a new diagnosis of advanced solid tumour were enrolled and evaluated before starting first-line chemotherapy. Anthropometric, body composition and systemic inflammation measurements were collected and cut-off points from literature data were used for results classification. Malnutrition was expressed as weight loss (WL) in the previous 6 months >10% and underweight body mass index (BMI). Altered body composition was assessed as low index both skeletal muscle (SMI) derived by CT and fat-free mass by BIA (FFMI). Descriptive statistic was presented. Several statistical correlation analyses were performed. RESULTS: 67 patients were assessed: 40M/27F; average age 59 ± 13 years and BMI 23 ± 4; 43 (64%) upper gastrointestinal, 12 lung, 9 colorectal, 3 other cancers. Fourty-five (67%) were malnourished with WL criteria but only 8 (12%) resulted underweight. From analysis of CT images and BIA, 49 (73%) and 10 (15%) patients respectively reported lower cut-off point. Overall, 35 (52%) had both sarcopenia and WL > 10%. CONCLUSIONS: Our results suggest that prevalence data of malnutrition expressed as WL are more in agreement with those of sarcopenia recognised using CT than BIA method. Further studies are required to confirm these findings and to identify the best and easiest methods for monitoring BC during nutritional intervention and oncological therapies.

Glial-fibrillary-acidic-protein (GFAP) biomarker detection in serum-matrix: Functionalization strategies and detection by an ultra-high-frequency surface-acoustic-wave (UHF-SAW) lab-on-chip.

Glial-fibrillary-acidic-protein (GFAP) has recently drawn significant attention from the clinical environment as a promising biomarker. The pathologies which can be linked to the presence of GFAP in blood severely affect the human central nervous system. These pathologies are glioblastoma multiforme (GBM), traumatic brain injuries (TBIs), multiple sclerosis (MS), intracerebral hemorrhage (ICH), and neuromyelitis optica (NMO). Here, we develop three different detection strategies for GFAP, among the most popular in the biosensing field and never examined side by side within the experimental frame. We compare their capability of detecting GFAP in a clean-buffer and serum-matrix by using gold-coated quartz-crystal-microbalance (QCM) sensors. All the three detection strategies are based on antibodies, and each of them focuses on a key aspect of the biosensing process. The first is based on a polyethylene glycol (PEG) chain for antifouling, the second on a protein-G linker for controlling antibody-orientation, and the third on antibody-splitting and direct surface immobilization for high-surface coverage. Then, we select the best-performing protocol and validate its detection performance with an ultra-high-frequency (UHF) surface-acoustic-wave (SAW) based lab-on-chip (LoC). GFAP successful detection is demonstrated in a clean-buffer and serum-matrix at a concentration of 35 pM. This GFAP level is compatible with clinical diagnostics. This result suggests the use of our technology for the realization of a point-of-care biosensing platform for the detection of multiple brain-pathology biomarkers.

[Organization and management of clinical-care pathways at the Regina Margherita Hospital in Turin during Covid-19 emergency]. / L'emergenza COVID-19 in ambito pediatrico: esperienze di gestione ed organizzazione dei percorsi clinico-assistenziali dell'Ospedale Infantile Regina Margherita di Torino.

The Covid-19 pandemic significantly increased the workload for the Italian Health Service. There is few information in the literature on the pediatric population and on the management of pediatric hospitals. The aim of this article is to describe the management of healthcare services during Covid-19 emergency in Regina Margherita Children's Hospital. The Regina Margherita Children's Hospital is specialized in the prevention, diagnosis and treatment of pediatric diseases. About 1000 health worker work in this Hospital and 278 hospitalization places are available.

An overview on periodontal changes and dental movements.

Only in recent times has been enhanced the importance of gingival crevicular fluid in periodontal health and in particular in maintaining the integrity of periodontium during application of orthodontic forces. The aim of this short review is to evaluate the importance of substances as valid biomarkers of periodontal health during orthodontic movements. A search on PubMed and Cochrane database was performed considering the literature from 2003 to 2014, using the following key words: gingival crevicular fluid, biomarkers of periodontal tissue, orthodontic movements. After abstracts screening, the full-texts of selected papers were analyzed and the papers found from the reference lists were also considered. The search focused on clinical applications documented in studies in the English language: levels of evidence included in the literature analysis were I, II and III. Literature analysis showed 28 papers that fulfilled the inclusion criteria. The conclusion is that GCF is a powerful vehicle for clinical diagnostics, since it contains different biochemical and cellular arrays in relation to different clinical situations indicative of the state of periodontal health during orthodontic treatment.

Current concepts on cleft lip and palate etiology.

Nonsyndromic cleft lip with or without cleft palate is the most common craniofacial anomaly affecting around 1 in 700 live births worldwide. Clefts of the human face can be classified anatomically as cleft palate only (CPO), cleft lip only (CLO), cleft lip and palate (CLP) or a combined group of cleft lip with or without cleft palate (CL/P), based on different in embryologic development. These malformations have some genetic origin, in fact several association studies have been performed to obtain important information about the candidate genes; but more important are gene-environment interactions that play an increasing role in its etiology. Epidemiological studies have shown how environmental factors (alcohol, smoking, drugs), as well as possible gene-environment interactions, play an important role in the onset of the malformation. On the contrary, folic acid intake seems to have a protective effect. In this review, we analyze the role of environmental factors related to onset of cleft.

Efficacy of a new chemical device to minimize microbial contamination along implant-abutment connection: an in vitro study.

Osseointegrated dental implants showed elevated success rates on the long-term treatment in the last ten years. However, the risk of peri-implantitis and implant failure is the main complication of implantology. The presence of a micro gap at the implant-abutment connection (IAC) allows microorganisms to penetrate and colonize the inner part of the implant leading to biofilm accumulation and consequently to peri-implantitis development. Some chemical devices (CD) has been studied to reduce bacterial penetration at IAC level but no one have been demonstrated to be effective for this purpose. Aim of the present study is to evaluate the effectiveness of a new chemical formulation STCX-1, placed in the internal part of dental implants for killing bacteria present in the IAC. To identify the antibacterial power of SXTC-1 at interface between implant-abutment connection, the passage of genetically modified Escherichia coli across IAC was evaluated. A total of eight implants were used (Edierre Implant System, Edierre SpA, Genova, Italy). The inner side of four out of the eight implants were firstly contaminated with few microliters of pure bacteria, subsequently were treated with SXTC-1 for few second and finally, the antibacterial was replaced with Lysogeny Broth (LB) and antibiotics without bacteria. The remaining four implants were not treated with SXTC-1 and just filled with LB with antibiotics. Bacteria viability was determined by measuring their Optical Density (OD) at 600nm. The analysis revealed that, in untreated implants, bacteria grew (internally and externally) for the first 48 hours, but subsequently they started to dye. In treated implants, instead, bacteria grew just in the space surrounding the device suggesting that, even if bacteria were able to get into, they immediately died thanks to the presence of SXTC-1. The STCX-1 liquid formulation have been demonstrated to be an adjuvant CD effective for prevention of of bacterial colonization at IAC level.

Occurrence and risk factors of Coxiella burnetii in domestic ruminants in Lebanon.

Coxiella burnetii causes diseases in humans (Q fever) and animals, domestic ruminants playing a major role in the epidemiology of the infection. Information on C. burnetii infection in Lebanon is scanty. In order to assess the prevalence of C. burnetii infection in ruminants, a cross-sectional study was undertaken in 2014. A total of 1633 sera from ruminants (865 cattle, 384 sheep and 384 goats) from 429 farms (173 cattle, 128 sheep and 128 goats), in seven provinces of Lebanon were randomly selected and assayed for the presence of antibodies. 39.86% of farms (95% CI: 35.23-44.56) resulted positive. The seroprevalence was 30.63% in Cattle-farms, 46.88% in sheep-farms and 45.31% in goat-farms. Milk samples collected from 282 seropositive animals (86 cows, 93 sheep and 103 goats) from 171 positive farms were tested by a high sensitive Real-Time PCR targeted to the IS1111 transposon of C. burnetii. The overall prevalence in farms was estimated to be 14.04%. Cattle-, sheep- and goat farm prevalence rates were 15.09%, 10% and 17.24%, respectively. The findings of the study show that C. burnetii prevalence in Lebanese domestic ruminants is related to animal species and farming practices. Indeed, the mixed herds with sheep (p < 0.01), the presence of common lambing/kidding areas (p < 0.001) in farms where the use of disinfectants was not a routine practice (p < 0.05) were identified as important risk factors. The results of the study provide baseline information for setting up herd management and public health measures for the prevention and control of Q fever in Lebanon.

Quasi-3D morphology and modulation of focal adhesions of human adult stem cells through combinatorial concave elastomeric surfaces with varied stiffness.

In vivo cell niches are complex architectures that provide a wide range of biochemical and mechanical stimuli to control cell behavior and fate. With the aim to provide in vitro microenvironments mimicking physiological niches, microstructured substrates have been exploited to support cell adhesion and to control cell shape as well as three dimensional morphology. At variance with previous methods, we propose a simple and rapid protein subtractive soft lithographic method to obtain microstructured polydimethylsiloxane substrates for studying stem cell adhesion and growth. The shape of adult renal stem cells and nuclei is found to depend predominantly on micropatterning of elastomeric surfaces and only weakly on the substrate mechanical properties. Differently, focal adhesions in their shape and density but not in their alignment mainly depend on the elastomer stiffness almost regardless of microscale topography. Local surface topography with concave microgeometry enhancing adhesion drives stem cells in a quasi-three dimensional configuration where stiffness might significantly steer mechanosensing as highlighted by focal adhesion properties.

Diagnostic accuracy of percutaneous biopsy in retroperitoneal sarcoma.

BACKGROUND: Percutaneous biopsy is recommended before surgery for suspected retroperitoneal sarcoma (RPS) to confirm the histological diagnosis and guide surgical strategy. The present study aimed to establish the diagnostic accuracy of percutaneous core biopsy with respect to histological diagnosis and tumour grade. METHODS: Data on patients with suspected RPS who underwent percutaneous biopsy followed by surgical resection between 2005 and 2016 at one of two tertiary European sarcoma units were reviewed. Histological tumour type and tumour grade on biopsy were correlated with postoperative histology to evaluate diagnostic accuracy. RESULTS: A total of 239 patients underwent percutaneous core biopsy followed by surgical resection in Milan (163, 68·2 per cent) or Birmingham (76, 31·8 per cent). Diagnostic accuracy varied with histological diagnosis (P < 0·001), but demonstrated overall concordance with final pathology following resection in 67·2 per cent of biopsies (κ = 0·606). The majority of discrepancies occurred in dedifferentiated liposarcoma (DDLPS), owing to under-recognition of dedifferentiation in this group. Concordance between pathology on biopsy and resection improved to 81·1 per cent when DDLPS and well differentiated liposarcoma were grouped together as liposarcoma. Grade on biopsy was concordant with grade on resection specimen in 60·4 per cent of tumours (κ = 0·640). Diagnosis of high-grade tumours on biopsy had a high specificity (98 per cent), and moderate positive predictive value (85 per cent) and negative predictive value (78 per cent). CONCLUSION: A diagnosis of DDLPS or leiomyosarcoma on percutaneous biopsy is highly reliable. High-grade sarcomas can be identified with high specificity, which opens the door to a study on neoadjuvant therapy in these patients.

Wearable, Fiber-less, Multi-Channel System for Continuous Wave Functional Near Infrared Spectroscopy Based on Silicon Photomultipliers Detectors and Lock-In Amplification.

Development and in-vivo validation of a Continuous Wave (CW) functional Near Infrared Spectroscopy (fNIRS) system is presented. The system is wearable, fiber-less, multi-channel (16×16, 256 channels) and expandable and it relies on silicon photomultipliers (SiPMs) for light detection. SiPMs are inexpensive, low voltage and resilient semiconductor light detectors, whose performances are analogous to photomultiplier tubes (PMTs). The advantage of SiPMs with respect to PMTs is that they allow direct contact with the scalp and avoidance of optical fibers. In fact, the coupling of SiPMs and light emitting diodes (LEDs) allows the transfer of the analog signals to and from the scalp through thin electric cables that greatly increase the system flexibility. Moreover, the optical probes, mechanically resembling electroencephalographic electrodes, are robust against motion artifacts. In order to increase the signal-to-noise-ratio (SNR) of the fNIRS acquisition and to decrease ambient noise contamination, a digital lock-in technique was implemented through LEDs modulation and SiPMs signal processing chain. In-vivo validation proved the system capabilities of detecting functional brain activity in the sensorimotor cortices. When compared to other state-of-the-art wearable fNIRS systems, the single photon sensitivity and dynamic range of SiPMs can exploit the long and variable interoptode distances needed for estimation of brain functional hemodynamics using CW-fNIRS.

Late-Onset Pompe Disease with Nemaline Bodies.

Pompe disease is an autosomal recessive disorder characterized by deficiency of alpha-glucosidase, a lysosomal enzyme, which can lead to glycogen accumulation in skeletal muscle, heart, and nervous system. Clinical presentation is highly variable, with infantile and late-onset (LOPED) forms. Although muscle biopsy findings are rather stereotyped, atypical features have been described. A 52-year-old man without a family history of muscle disorders presented with slowly progressing upper and lower limb girdle weakness and hyperCKemia. At needle EMG, a diffuse neurogenic pattern was detected. Muscle biopsy showed a selective type 1 fiber atrophy with vacuoles of various sizes, filled with PAS and acid phosphatase positive material, confirmed to be glycogen by electron microscopy (EM). Many atrophic fibers contained foci of myofibrillar material recognized as nemaline bodies (NBs) at EM. Low level of alpha-glucosidase activity in blood and molecular genetic testing confirmed the diagnosis of late-onset Pompe disease (LOPED). Major causes of hereditary and acquired NB myopathy were ruled out. In conclusion, NBs represent a novel histological finding in LOPED and characterize the atypical presentation of our case.