BACKGROUND:Osteogenesis imperfecta (OI) is a rare hereditary disorder of connective tissue. A Danish national registry study surprisingly identified digestive causes as one of the leading causes of OI mortality. However, there is a dearth of prospective data describing gastrointestinal symptoms in OI. Our aim was to assess common gastrointestinal symptoms in an OI patient population and compare it to the general U.S. POPULATION: METHODS:OI patients enrolled in a natural history protocol (NCT03575221) were prospectively evaluated for gastrointestinal symptoms and provided with PROMIS questionnaires for six gastrointestinal symptoms. The HMSS application was used to obtain T scores, score of 50 (and standard deviation of 10) representing the average of the general U.S. POPULATION: RESULTS:41 OI patients were included. Median age 28 years (IQR 18-26), median BMI 28 kg/m2 (IQR 21-34), and 54 % female. OI type IV 61 %, OI type III 20 %, and remaining patients had OI type VI, VII, XIV. The mean T score for the six gastrointestinal symptoms ranged from 46 to 49, within 1 SD from the general U.S. POPULATION:Subgroup analyses showed no differences based on age, mobility, BMI, type of OI and genetic mutations (COL1A1 vs COL1A2), except increased abdominal pain with age. Patients with severe scoliosis (>50°) reported increased nausea and vomiting, and diarrhea compared to patients with mild to moderate scoliosis. DISCUSSION:We report the largest cohort of OI patients evaluated prospectively and directly for gastrointestinal complaints. Study patients, which excluded type I OI, did not report gastrointestinal symptoms higher than the general population except for abdominal pain in older patients. OI patients should be carefully evaluated in the same way as any other patient presenting with gastrointestinal complaints.

An optically reconfigurable metasurface antenna for Future G beam steering applications is demonstrated experimentally. Optical reconfigurability is achieved by using photodiode arrays to provide a photovoltage to varactors, which in turn provide the required phase shift to enable beam-steering. Illumination of the photodiodes is provided over free space by using a galvo scanner to deflect a laser beam which is delivered from a high-power laser via a multicore fiber. In addition to delivering power over fiber, the same multicore fiber is also used to deliver a 64-QAM 5G NR signal to the metasurface antenna unit. Measured EVM values ranging from 3% to 5.7% for a beam-steering range of 58 degrees are obtained. The use of a galvo scanner for free space illumination of the metasurface antenna not only eliminates the requirement for on-antenna DC bias networks (thus reducing the system complexity), but also allows greater control over illumination of the photodiode arrays, resulting in continuous beam-steering over 58 degrees.

A dual layer metasurface antenna designed for 28 GHz gain and bandwidth enhancement has been integrated with a radio-over-fiber system to demonstrate successful transmission of a 16-QAM 5G NR signal. Measured results for the magnitude of the reflection coefficient and gain of the fabricated metasurface antenna are presented. Good matching along with gain and bandwidth enhancement has been achieved for the 28 GHz mm-wave band. Subsequently a 5G radio-overfiber demonstration is used to evaluate the impact of the improved antenna on EVM (error vector magnitude) performance. Inclusion of a metasurface superstrate reduces the measured EVM from 3.73% to 2.68%. By leveraging the strengths of both metasurfaces and radio-over-fiber, network operators can achieve high-capacity, reliable, and cost-effective communication infrastructures.

Abstract A reconfigurable inductorless negative‐refractive‐index transmission‐line (NRI‐TL) metamaterial phase shifter for sub‐6 GHz 5G antenna applications is proposed. The design consists of a microstrip transmission line loaded both in series and in parallel with varactor diodes to provide continuous tuning of the phase of the transmitted signal by varying the bias voltage applied to the varactors, without the need for variable inductor components. The total measured differential phase shift is 190° at 3.2 GHz, with a measured insertion loss of 1.5–2.7 dB in the frequency range of 3.2–3.6 GHz, when the biasing voltage of the varactors is varied between 0 and 7 V. The total size of the microstrip NRI‐TL phase shifter is 19.4 mm × 24 mm. The proposed reconfigurable phase shifter has been incorporated into the feeding network of a two‐element antenna array to demonstrate its use in beam‐steering applications. The measured results for the reconfigurable array show good impedance matching between 3 and 3.35 GHz for all biasing values, allowing continuous beam steering over a scanning range of 60°.

This paper presents a modified reconfigurable cactus-shaped planar monopole Ultra-wideband (UWB) antenna with 20 x 28 mm(2) dimensions. The three legs of the monopole enable a better command over the antenna matching parameter. The antenna operates in the UWB region ranging from 3.5 to 10.6 GHz. The reconfigurability is introduced by using three PIN diodes placed at the base of each leg. These diodes are turned ON and OFF simultaneously to manage the operational frequency of the monopole. The effortless control of the return loss extends its implementations for numerous UWB applications, particularly in intelligent sensing, intelligent surfaces (e.g. RISs, STAR-RIS) and signal filtering, where accuracy, high signal quality and noise reduction are essential for accurate and efficient data processing.

An optically reconfigurable phase shifter based on negative-refractive-index transmission line (NRI-TL) metamaterials is presented; the design is suitable for 5G antenna beam steering. Continuous tuning of the transmitted signal's phase is achieved by using varactor diodes for which the biasing is provided via an array of photodiodes placed on the back of the substrate. These are illuminated by a remote optical source whose wavelength can be tuned in the visible range, thus enabling indirect control of the varactor capacitance (and hence the phase shift) while also eliminating the need for a complex biasing network. Three different phases are achieved by illuminating the photodiode array with three different wavelengths (blue, green, and red). The compact size along with simplified biasing network introduced by the inclusion of the optical wavelength tunability has numerous applications in antenna and phased array technology, making the design a suitable candidate for 5G beam steering applications.

INTRODUCTION: Splenic stiffness (SS) measurement (SSM) is an evolving noninvasive assessment to evaluate portal hypertension. Studies with respect to SSM in patients with alcohol use disorder are limited. METHODS: We studied patients seeking treatment for alcohol use disorder in an inpatient treatment protocol at the National Institutes of Health and parsed SSM into 3 groups based on degree of change. RESULTS: The improved SS group had statistically higher initial SSM and a nonstatistically increased liver stiffness measurement compared with others. DISCUSSION: SS is dynamic in a subset of patients immediately after alcohol cessation, and improved SS is associated with a normalization of platelet count.

SummaryBackground and AimsLiver involvement is an increasingly recognised complication of common variable immunodeficiency (CVID). Nodular regenerative hyperplasia (NRH), a subgroup of porto‐sinusoidal vascular disorder, and manifestations of portal hypertension (PH) unrelated to cirrhosis are the most common findings. Nonetheless, the evolution of liver disease over time remains unknown.MethodsRetrospective review of patients followed at the National Institutes of Health with CVID‐related liver disease and liver biopsy from 1990 to 2020. Clinical, imaging and histological follow‐up were recorded as part of clinical research protocols.ResultsForty patients were included, with a median age of 37.5 years at initial biopsy, 73% presenting with clear evidence of NRH, and a median fibrosis stage of 1. At biopsy, median platelet count was 100 × 109/L, spleen size 19.5 cm, hepatic venous pressure gradient 9.5 mmHg and 37.5% of patients had signs of PH. Cumulative incidence of PH was 65% at 5 years. In a subgroup of 16 patients, a follow‐up liver biopsy, performed at a median time of 3 years after the index biopsy, revealed an increase in fibrosis by ≥2 stages in 31% of cases and an increase to an overall stage of 2.2 (p = 0.001). No clinical or histological factors were associated with progression of fibrosis.ConclusionsIn this CVID cohort, NRH is the most common initial histological finding; however, unexpectedly fibrosis progresses over time in a subgroup of patients. A better understanding of the underlying causal process of liver disease CVID might lead to improved outcomes.

BACKGROUND:Alcohol cessation is the only intervention that both prevents and halts the progressions of alcohol-associated liver disease. The aim of this study was to assess the relationship between a return to alcohol use and consultation with hepatology in treatment-seeking patients with alcohol use disorder (AUD). METHODS:Two hundred forty-two patients with AUD were enrolled in an inpatient treatment program, with hepatology consultation provided for 143 (59%) patients at the request of the primary team. Patients not seen by hepatology served as controls. The primary outcome was any alcohol use after discharge assessed using AUDIT-C at 26 weeks after discharge. RESULTS:For the primary endpoint, AUDIT at week 26, 61% of the hepatology group and 28% of the controls completed the questionnaire (p=0.07). For the secondary endpoint at week 52, these numbers were 22% and 11% (p = 0.6). At week 26, 39 (45%) patients in the hepatology group versus 31 (70%) controls (p = 0.006) returned to alcohol use. Patients evaluated by hepatology had decreased rates of hazardous alcohol use compared to controls, with 36 (41%) versus 29 (66%) (p = 0.008) of the patients, respectively, reporting hazardous use. There were no significant differences in baseline characteristics between groups and no difference in rates of prescribing AUD therapy. There was no difference in outcomes at 52 weeks. CONCLUSIONS:Patients evaluated by hepatology had significantly lower rates of return to alcohol use and lower rates of hazardous drinking at 26 weeks but not at 52 weeks. These findings suggest that hepatology evaluation during inpatient treatment of AUD may lead to decreased rates of early return to alcohol use.

Introduction:Prolidase deficiency is a rare autosomal recessive disorder caused by variants in the PEPD gene. Patients usually have multi-organ involvement and a wide range of clinical features including recurrent skin ulcers, dysmorphic facial features, recurrent infections, intellectual disability, and splenomegaly. Studies have shown that patients with prolidase deficiency may have hepatic manifestations including hepatomegaly and abnormal liver enzymes. However, there is no detailed description of liver disease in this patient population.Case Presentation:Here, we present 3 patients with prolidase deficiency with varying extents of hepatic involvement.Conclusion:Prolidase deficiency patients with liver disease should be followed up long term to understand more about the pathophysiology and the impact of liver disease on long-term outcomes.

BACKGROUND: Intravenous vancomycin therapy can cause liver injury as well as "drug reaction with eosinophilia and systemic symptoms" (DRESS) syndrome. This study aimed to better define the clinical features and HLA associations of vancomycin-induced liver injury. OBJECTIVE: To describe clinical, biochemical, and temporal characteristics of vancomycin-induced liver injury. METHODS: Cases of liver injury with recent exposure to vancomycin who were enrolled in the US Drug-induced Liver Injury Network between 2004 and 2020 were assessed. Sequencing of HLA alleles was performed on stored blood samples. RESULTS: Among 1697 cases of drug-induced liver injury identified between 2004 and 2021, 9 (0.5%) were attributed to intravenous vancomycin. The 9 cases included 6 men, median age 60 years (range, 23-85 days), and treatment for 26 days (range, 1-34 days). The clinical presentation was DRESS syndrome in 8 patients, of whom 6 received corticosteroids. Liver injury varied from hepatocellular to cholestatic and from mild (n = 5) to fatal (n = 1). In survivors, liver injury and DRESS syndrome ultimately resolved. HLA typing demonstrated the HLA-A*32:01 allele in 7 vancomycin cases (78%, all with DRESS syndrome), versus 1 of 81 cases (1.2%) exposed but not attributed to vancomycin, and 113 of 1708 cases (6.6%) without vancomycin exposure. The allele frequency in vancomycin cases was 0.44 compared with less than 0.04 in US populations. CONCLUSIONS: Vancomycin-induced liver injury is commonly associated with DRESS syndrome and linked to HLA-A*32:01. HLA-A*32:01 testing could be considered early to risk -stratify patients using long-term intravenous vancomycin therapy. Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology (J Allergy Clin Immunol Pract 2024;12:168-74)

This paper presents the results of a wireless power transfer (WPT) rectifier connected to an antenna (rectenna), suitable to bias the front-end of a 5G receiver at FR1 frequency band. A patch antenna has been designed to receive the RF power at 3.45 GHz (Sub 6 GHz band), while the rectification process is based on a full wave voltage doubler rectifier. The matching circuit between the patch antenna and the rectifier is tuned for maximum power transfer with a varying load between 1kOhm to 5kOhm. When the antenna receives a power of - 10dBm, the rectifier produces a corresponding DC voltage of one Volt with an RF to DC power conversion efficiency of 38 The rectenna circuit is studied and designed using the PathWave Advanced Design System (ADS) tool. The proposed design fully covers the power needs of an RF front-end receiver at FR1 and paves the way for other practical implementations in various wireless energy harvesting applications.

Introduction Prophylactic total gastrectomy (PTG) can eliminate gastric cancer risk and is recommended in carriers of a germline CDH1 pathogenic variant. PTG has established risks and potential life-long morbidity. Decision-making regarding PTG is complex and not well-understood. Methods Individuals with germline CDH1 pathogenic or likely pathogenic variants who underwent surveillance endoscopy and recommended for PTG were evaluated. Factors associated with decision to pursue PTG (PTG pos ) or not (PTG neg ) were queried. A decision-regret survey was administered to patients who elected PTG. Results Decision-making was assessed in 120 patients. PTG pos patients (63%, 76/120) were younger than PTG neg (median 45 vs 58 years) and more often had a strong family history of gastric cancer (80.3% vs 34.1%). PTG pos patients reported decision-making based on family history more often and decided soon after diagnosis (8 vs 27 months) compared with PTG neg . Negative endoscopic surveillance results were more common among PTG neg patients. Age >60 years, male sex and longer time to decision were associated with deferring PTG. Strong family history, a family member who died of gastric cancer and carcinoma on endoscopic biopsies were associated with decision to pursue PTG. In the PTG pos group, 30 patients (43%) reported regret which was associated with occurrence of a postoperative complication and no carcinoma detected on final pathology. Conclusion The decision to undergo PTG is influenced by family cancer history and surveillance endoscopy results. Regret is associated with surgical complications and pathological absence of cancer. Individual cancer-risk assessment is necessary to improve pre-operative counselling and inform the decision-making process. Trial registration number NCT03030404 .

A 1-bit reconfigurable phase shifter for 28 GHz mm-wave beam steering antennas is proposed. The design uses two beam-lead GaAs PIN diodes loaded on a microstrip transmission line in parallel, to provide two discrete phases for the transmitted signal. When the diodes are switched OFF, no shift in the phase of the transmitted signal is observed while a phase shift of ~90° is achieved when the diodes are switched ON. The total length of the phase shifter is $\sim\lambda /2$ . The compact size, low cost, reduced complexity and easy adaptation in antenna and phased array technology makes it a suitable candidate for 5G mm-wave beam steering applications.

Background Loss of function variants in CDH1 are the most frequent cause of hereditary diffuse gastric cancer. Endoscopy is regarded as insufficient for early detection due to the infiltrative phenotype of diffuse-type cancers. Microscopic foci of invasive signet ring cells are pathognomonic of CDH1 and precede development of diffuse gastric cancer. We aimed to assess the safety and effectiveness of endoscopy for cancer interception in individuals with germline CDH1 variants, particularly in those who declined prophylactic total gastrectomy. Methods In this prospective cohort study, we included asymptomatic patients aged 2 years or older with pathogenic or likely pathogenic germline CDH1 variants who underwent endoscopic screening and surveillance at the National Institutes of Health (Bethesda, MD, USA) as part of a natural history study of hereditary gastric cancers (NCT03030404). Endoscopy was done with non-targeted biopsies and one or more targeted biopsy and assessment of focal lesions. Endoscopy findings, pathological data, personal and family cancer history, and demographics were recorded. Procedural morbidity, gastric cancer detection by endoscopy and gastrectomy, and cancer-specific events were assessed. Screening was defined as the initial endoscopy and all subsequent endoscopies were considered surveillance; follow-up endoscopy was at 6 to 12 months. The primary aim was to determine effectiveness of endoscopic surveillance for detection of gastric signet ring cell carcinoma. Findings Between Jan 25, 2017, and Dec 12, 2021, 270 patients (median age 46 center dot 6 years [IQR 36 center dot 5-59 center dot 8], 173 [64%] female participants, 97 [36%] male participants; 250 [93%] were non-Hispanic White, eight [3%] were multiracial, four [2%] were non-Hispanic Black, three [1%] were Hispanic, two [1%] were Asian, and one [<1%] was American Indian or Alaskan Native) with germline CDH1 variants were screened, in whom 467 endoscopies were done as of data cutoff (April 30, 2022). 213 (79%) of 270 patients had a family history of gastric cancer, and 176 (65%) reported a family history of breast cancer. Median follow-up was 31 center dot 1 months (IQR 17 center dot 1-42 center dot 1). 38 803 total gastric biopsy samples were obtained, of which 1163 (3%) were positive for invasive signet ring cell carcinoma. Signet ring cell carcinoma was detected in 76 (63%) of 120 patients who had two or more surveillance endoscopies, of whom 74 had occult cancer detected; the remaining two individuals developed focal ulcerations each corresponding to pT3N0 stage carcinoma. 98 (36%) of 270 patients proceeded to prophylactic total gastrectomy. Among patients who had a prophylactic total gastrectomy after an endoscopy with biopsy samples negative for cancer (42 [43%] of 98), multifocal stage IA gastric carcinoma was detected in 39 (93%). Two (1%) participants died during follow-up, one due to metastatic lobular breast cancer and the other due to underlying cerebrovascular disease, and no participants were diagnosed with advanced stage (III or IV) cancer during follow-up. Interpretation In our cohort, endoscopic cancer surveillance was an acceptable alternative to surgery in individuals with CDH1 variants who declined total gastrectomy. The low rate of incident tumours (>T1a) suggests that surveillance might be a rational alternative to surgery in individuals with CDH1 variants.

A 5G-NR radio-over-fiber system (RoF) that also incorporates power-over-fiber (PoF) via a multicore fiber (MCF) link is used to support a reconfigurable metasurface (MSF) antenna for rapid beam steering. Optical signals for RoF-PoF are generated in a central office (CO), and transmitted to the remote antenna unit (RAU) via MCF. A reconfigurable MSF antenna has been designed to perform continuous steering of the radiated beam using indirect optical control of varactor diodes. Subsequently, a proof-of-concept 5G-NR system has been demonstrated for the sub-6 GHz band. The PoF part of the system was able to provide 0–5 V to reconfigure the MSF antenna and thus achieve total beam steering of 66°. For the RoF segment, the measured EVM values for a 64-QAM scheme vary between 2.6%–6.9% for all states.