[Incidence of hypogammaglobulinaemia in children with steroid-dependent/frequently relapsing nephrotic syndrome treated with rituximab and its association with severe infections].

Objective: To investigate the incidence and influencing factors of hypogammaglobulinemia (HGG) in children with steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) treated with rituximab (RTX), and its relationship with the risk of severe infections. Methods: The clinical data of children with SDNS/FRNS treated with RTX at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University from December 2020 to January 2023 were retrospectively analyzed. RTX treatment was performed using a B-cell-guided regimen (a single dose of 375 mg/m2, a maximum of 500 mg/dose, and an additional one dose when reassessment of peripheral blood CD19+B cells≥1%). Patients were divided into HGG and non-HGG groups according to the presence or absence of HGG during the follow-up period. A multivariate logistic regression model was used to analyze the influencing factors of HGG, and the predictive value of each influencing factor on HGG was assessed by plotting the receiver operating characteristic (ROC) curve. Results: A total of 59 SDNS/FRNS children (48 males and 11 females) were included, and aged [M (Q1, Q3)] 9.4 (6.5, 12.2) years at the time of the first RTX treatment, with a median application of 3 (2, 4) doses of RTX. During the follow-up period of 15.5 (9.9, 22.8) months, the HGG was present in 16 (27.1%) children, of which seven persisted for more than 1 year. Compared with non-HGG group, HGG group had a shorter duration of the disease [3.3 (2.1, 3.6) vs 4.6 (2.4, 8.0) years, P=0.030], younger age at the time of the first RTX treatment [6.2 (5.6, 7.4) vs 11.3 (8.8, 13.3) years, P<0.001], and lower serum IgG levels [5.9 (4.9, 6.4) vs 7.5 (6.1, 8.2) g/L, P<0.001]. Multivariate logistic regression analysis showed that young age at the time of the first RTX treatment (OR=0.52, 95%CI: 0.35-0.78, P=0.002) was an influencing factor of HGG. The area under the curve (AUC) for age at first RTX treatment to predict HGG was 0.887 (95%CI: 0.778-0.955, P<0.001), with an optimal cut-off value of 8.3 years. During the follow-up period, six children (10.2%) developed severe infectious, and there was no statistically significant difference in the incidence of serious infections between the HGG and non-HGG groups [12.5% (2/16) vs 9.3% (4/43), P=1.000]. Conclusions: HGG is frequent in children with SDNS/FRNS treated with RTX, and nearly half of HGG persists for more than 1 year. The possibility of HGG is greater in those≤8.3 years at the first RTX treatment, but HGG does not increase the risk of severe infections in children.

[Transperineal targeted prostate puncture combined with rapid pathological examination in the diagnosis of prostate cancer].

Exploring the clinical value of multiparametric magnetic resonance (Mp-MRI)-cognitive fusion method of targeted transperineal prostate puncture combined with rapid pathological diagnosis. Patients with suspected prostate cancer admitted to our hospital from 2022.01 to 2023.05 were selected as the study subjects, and Mp-MRI was performed and the suspected lesions were scored by the Prostate Imaging Reporting and Data System (PI-RADS). The enrolled patients were randomly divided into the transperineal prostate targeted puncture plus rapid pathology group (experimental group) and the transperineal prostate systematic combined targeted puncture plus conventional pathology group (control group), and the positive puncture rate, pathological findings, and complications were analyzed to compare the differences between the two groups. A total of 100 patients were enrolled, 53 in the experimental group [age 55-89 years, (73.17±7.79) years; tPSA 7.01-100 µg/L, mean 21.34 (12.38, 44.42) µg/L]and 47 in the control group [age 60-87 years, (71.96±7.07) years; tPSA 6.11-98.82 µg/L, mean 18.77 (9.04, 38.09) µg/L], and there was no significant difference between the two groups in the diagnostic positivity rate of overall PCa and clinically significant PCa (P>0.05); there was no significant difference in the highest Gleason score of pathological tissues between the two groups (P>0.05); the number of cases of medically induced sarcoid hematuria in the experimental group were significantly reduced compared with the control group (P<0.05). In terms of biopsy pain score (VAS), patients in the experimental group experienced less pain than those in the control group (P<0.05). The Mp-MRI-cognitive fusion method of transperineal targeted prostate puncture combined with rapid frozen section pathological examination can provide rapid and accurate pathological results, reduce the chance of post-puncture complications, and alleviate the pain caused by puncture sampling, which has high clinical value.

Membrane Potential-Dependent Uptake of Cationic Oligoimidazolium Mediates Bacterial DNA Damage and Death.

The treatment of bacterial infections is becoming increasingly challenging with the emergence of antimicrobial resistance. Thus, the development of antimicrobials with novel mechanisms of action is much needed. Previously, we designed several cationic main-chain imidazolium compounds and identified the polyimidazolium PIM1 as a potent antibacterial against a wide panel of multidrug-resistant nosocomial pathogens, and it had relatively low toxicity against mammalian epithelial cells. However, little is known about the mechanism of action of PIM1. Using an oligomeric version of PIM1 with precisely six repeating units (OIM1-6) to control for consistency, we showed that OIM1-6 relies on an intact membrane potential for entry into the bacterial cytoplasm, as resistant mutants to OIM1-6 have mutations in their electron transport chains. These mutants demonstrate reduced uptake of the compound, which can be circumvented through the addition of a sub-MIC dose of colistin. Once taken up intracellularly, OIM1-6 exerts double-stranded DNA breaks. Its potency and ability to kill represents a promising class of drugs that can be combined with membrane-penetrating drugs to potentiate activity and hedge against the rise of resistant mutants. In summary, we discovered that cationic antimicrobial OIM1-6 exhibits an antimicrobial property that is dissimilar to the conventional cationic antimicrobial compounds. Its killing mechanism does not involve membrane disruption but instead depends on the membrane potential for uptake into bacterial cells so that it can exert its antibacterial effect intracellularly.

Type IV collagen-derived angiogenesis inhibitor: canstatin low expressing in brain-invasive meningiomas using liquid chromatography-mass spectrometry (LC-MS/MS).

PURPOSE: Brain invasion in meningiomas is considered an indicator of more aggressive behavior and worse prognosis. But the precise definition and the prognostic role of brain invasion remains unsolved duo to lacking a standardized workflow of surgical sampling and the histopathological detection. Searching for molecular biomarker expression correlating with brain invasion, could contribute to establish a molecular pathological diagnosis without problems of subjective interobserver variation and deeply understand the mechanism of brain invasion and develop innovative therapeutic strategies. METHODS: We utilized liquid chromatography tandem mass spectrometry to quantify protein abundances between non-invasive meningiomas (n = 21) and brain-invasive meningiomas (n = 21) spanning World Health Organization grades I and III. After proteomic discrepancies were analyzed, the 14 most up-regulated or down-regulated proteins were recorded. Immunohistochemical staining for glial fibrillary acidic protein and most likely brain invasion-related proteins was performed in both groups. RESULTS: A total of 6498 unique proteins were identified in non-invasive and brain-invasive meningiomas. Canstatin expression in the non-invasive group was 2.1-fold that of the brain-invasive group. The immunohistochemical staining showed canstatin expressed in both groups, and the non-invasive group showed stronger staining for canstatin in the tumor mass (p = 0.0132) than the brain-invasive group, which showed moderate intensity. CONCLUSION: This study demonstrated the low expression of canstatin in meningiomas with brain invasion, a finding that provide a basis for understanding the mechanism of brain invasion of meningiomas and may contribute to establish molecular pathological diagnosis and identify novel therapeutic targets for personalized care.

Prenatal exome sequencing analysis in fetuses with central nervous system anomalies.

OBJECTIVE: To evaluate the utility of prenatal exome sequencing (pES) in fetuses with central nervous system (CNS) abnormalities. METHODS: This was a retrospective cohort study of fetuses identified to have CNS abnormality on prenatal ultrasound and/or magnetic resonance imaging. All fetuses were first analyzed by chromosomal microarray analysis (CMA). Fetuses with a confirmed aneuploidy or causal pathogenic copy-number variant (CNV) on CMA did not undergo pES analysis and were excluded, while those with a negative CMA result were offered pES testing. RESULTS: Of the 167 pregnancies included in the study, 42 (25.1%) were identified to have a pathogenic or likely pathogenic (P/LP) variant. The diagnostic rate was significantly higher in fetuses with a non-isolated CNS abnormality than in those with a single CNS abnormality (35.7% (20/56) vs 14.5% (8/55); P = 0.010). Moreover, when a fetus had three or more CNS abnormalities, the positive diagnostic rate increased to 42.9%. A total of 25/42 (59.5%) cases had de-novo mutations, while, in the remaining cases, mutations were inherited and carried a significant risk of recurrence. Families whose fetus carried a P/LP mutation were more likely to choose advanced pregnancy termination than those with a variant of uncertain significance, secondary/incidental finding or negative pES result (83.3% (25/30) vs 41.3% (38/92); P < 0.001). CONCLUSION: pES improved the identification of genetic disorders in fetuses with CNS anomalies without a chromosomal abnormality or CNV identified on CMA, regardless of the number of CNS anomalies and presence of extracranial abnormality. We also demonstrated that pES findings can significantly impact parental decision-making. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

[Risk factors of systemic allergic reactions caused by subcutaneous allergen immunotherapy].

Objective: To investigate the incidence and risk factors of systemic allergic reactions induced by subcutaneous immunotherapy (SCIT) in patients undergoing SCIT injections in Peking Union Medical College Hospital (PUMCH). Methods: This is a single center retrospective cohort study. Using the outpatient information system of PUMCH, the demographic information and injection-related reaction data of patients undergoing SCIT injection in Allergy Department of PUMCH from December 2018 to December 2022 were retrospectively analyzed to count the incidence and risk factors of systemic allergic reactions caused by SCIT. Mann-Whitney nonparametric test or chi-square test was used for single-factor analysis, and multiple logistic regression was used for multiple-factor analysis. Results: A total of 2 897 patients received 18 070 SCIT injections in Allergy Department during the four years, and 40 systemic allergic reactions occurred, with the overall incidence rate of 0.22%. The incidence of systemic allergic reaction was 0.37% when using imported dust mite preparation and 0.15% when using domestic multi-component allergen preparation. The risk factors significantly related with SCIT-induced systemic allergic reactions in patients using imported dust mite preparation were age less than 18 years old (OR=3.186,95%CI: 1.255-8.085), highest injection concentration (OR value could not be calculated because all patients with systemic reactions were injected with highest concentration), and large local reaction in previous injection (OR=22.264,95%CI: 8.205-60.411). The risk factors for SCIT-induced systemic allergic reactions in patients using domestic allergen preparation were 5 or more types of allergens (OR=3.455,95%CI: 1.147-10.402), highest injection concentration (OR=3.794,95%CI: 1.226-11.740) and large local reaction in previous injection (OR=63.577,95%CI: 22.248-181.683). However, SCIT injection in pollen allergic patients during the pollen season did not show a correlation with systemic allergic reaction. Conclusion: The incidence of SCIT-induced systemic allergic reactions was low in the Chinese patient population of this study. Patients with one or more risk factors, such as multiple allergen injection, highest injection concentration, large local reaction in previous injection, should be given high attention and vigilance against systemic allergic reactions.

[Strategies for immunotherapy-related hepatotoxicity in liver cancer].

The successful market availability of immune checkpoint inhibitors (ICIs) has brought revolutionary changes to the treatment of hepatocellular carcinoma (HCC). With the widespread application of ICIs in HCC patients, the impact or even termination of antitumor therapy due to ICIs hepatotoxicity is a clinical problem that must be faced. However, it is currently unclear whether there are differences in the occurrence and risk factors of ICI hepatotoxicity between HCC patients and other tumors. At the same time, the chronic liver disease that often accompanies HCC patients also poses great challenges to their diagnosis and management. Therefore, clinical physicians need to understand the coping strategies for ICIs hepatotoxicity so as to improve the benefit of immunotherapy for patients.

[Genetic susceptibility to drug-induced liver injury].

Drug-induced liver injury (DILI) risk prediction, diagnosis establishment, clinical management, and all other aspects are facing great challenges. Although the current understanding of its pathogenesis is still incomplete, research over the past 20 years has shown that genetic susceptibility may play an important role in the occurrence and development of DILI. In recent years, pharmacogenomics studies have further revealed the association between human leukocyte antigen (HLA) genes, some non-HLA genes, and hepatotoxicity from certain drugs. However, due to the lack of well-designed, prospective, large-sample cohort validation and low positive predictive values, there may still be some way to go before the current results can be truly translated into clinical practice for precise prediction and prevention of DILI risk.

EGR2-related mixed demyelinating and axonal Charcot-Marie-Tooth disease: An electrodiagnostic, nerve imaging, and histological study.

BACKGROUND AND AIMS: The early growth response 2 gene (EGR2) mutations are associated with a group of hereditary neuropathy, including axonal neuropathy and hypomyelinating neuropathy or Charcot-Marie-Tooth disease (CMT) type 1D. We aim to perform an electrodiagnostic, nerve imaging, and histological study of EGR2-associated neuropathy. MATERIALS AND METHODS: We performed a retrospective analysis of two patients with EGR2-related neurology at our hospital. The neuropathy was confirmed by the nerve conduction study. Nerve imaging and sural biopsies were performed in two patients. RESULTS: Two unrelated boys exhibited early-onset length-dependent neuropathy. Next generation sequencing identified EGR2 gene with previously described E412K mutation in the third zine finger domain in patient 1 and a previously undescribed variant D355N mutation in the first zinc finger domain in patient 2. The magnetic resonance imaging of the lumbosacral plexus showed no abnormalities in patient 1 and thickened lumbosacral plexuses in patient 2. Electrophysiology and nerve biopsies showed a prominent axonal neuropathy, accompanied with demyelinating involvement. CONCLUSION: Therefore, it seemed that the EGR2 mutations could cause not only the known demyelinating type and axonal type but also mixed-type CMT. Our findings expanded the phenotypic heterogeneities of EGR2-associated neuropathy.

Improved cancer detection in Waldeyer's tonsillar ring by 68Ga-FAPI PET/CT imaging.

PURPOSE: In cancer of unknown primary (CUP), positron emission tomography/computed tomography (PET/CT) with the glucose analog [18F]FDG represents the standard imaging approach for localization of the malignant primary. Frequently, however, [18F]FDG PET/CT cannot precisely distinguish between small occult tumors and chronic inflammation, especially in Waldeyer's tonsillar ring. To improve the accuracy for detecting primary tumors in the Waldeyer's tonsillar ring, the novel PET tracer [68Ga]Ga-FAPI-4 for specific imaging of fibroblast activation protein (FAP) expression was used as a more specific target for cancer imaging. METHODS: Eight patients with suspicion of a malignant tumor in Waldeyer's tonsillar ring or a CUP syndrome were examined. PET/CT scans with [18F]-FDG and [68Ga]Ga-FAPI-4 were performed for pre-operative tumor localization. After surgical resection, histopathological and immunohistochemical results were compared to PET/CT findings. RESULTS: Histopathology revealed a palatine or lingual tonsil carcinoma in all patients. In case of lymph node metastases smaller than 7 mm in size, the [18F]FDG PET/CT detection rate of cervical lymph node metastases was higher than that of [68Ga]FAPI PET/CT, while both tracers identified the primary tumors in all eight cases. The size of the primary and the lymph node metastases was directly correlated to the respective FAP expression, as detected by immunohistochemistry. The mean SUVmax for the primary tumors was 21.29 ± 7.97 for 18F-FDG and 16.06 ± 6.29 for 68Ga-FAPI, respectively (p = 0.2). The mean SUVmax for the healthy contralateral tonsils was 8.38 ± 2.45 for [18F]FDG and 3.55 ± 0.47 for [68Ga]FAPI (p < 0.001). The SUVmax ratio of [68Ga]FAPI was significantly different from [18F] FDG (p = 0.03). Mean TBRmax for the [68Ga]Ga-FAPI-4 tracer was markedly higher in comparison to [18F]FDG (10.90 vs. 4.11). CONCLUSION: Non-invasive imaging of FAP expression by [68Ga]FAPI PET/CT resulted in a better visual detection of the malignant primary in CUP, as compared to [18F]FDG imaging. However, the detection rate of lymph node metastases was inferior, presumably due to low FAP expression in small metastases. Nevertheless, by offering a detection method for primary tumors with the potential of lower false positive rates and thus avoiding biopsies, patients with CUP syndrome may benefit from [68Ga]FAPI PET/CT imaging.

[Clinical analysis of robot-assisted laparoscopic transabdominal preperitoneal inguinal hernia repair].

Objective: To explore the clinical value of robot-assisted laparoscopic transabdominal preperitoneal (TAPP) inguinal hernia repair. Methods: We performed a retrospective review of 23 consecutive patients who underwent robotic inguinal hernia repair from January 2018 to December 2019 at Beijing United Family hospital. The patients were all male with mean age 48.8 (17-72) years and BMI 25.8 (21.2-32.6)kg/m(2). Data examined included perioperative bleeding, operative times, length of stay, pain scale ratings and postoperative complication. Results: There were no major complications during the surgeries including no significant intraoperative bleeding, injury to the vas deferens or major vascular structures. There were no conversions to open. No patients received prophylactic antibiotics according to our hospital policy. No surgical site infections were found. All patients were discharged home within the 24-hour postoperative period. The mean operating time of 13 cases of unilateral hernia was 121 (82-165) min, and that of 10 cases of bilateral hernia was 166 (100-315) min. The mean intraoperative blood loss was 13.3 ml (5-25 ml). The average pain score in recovery was 0.96 (0-3). The total length of stay was 28.4 (24.2-37.5) h. During a follow-up period of 3-18 months, none of the patients experienced a recurrent hernia. None experienced chronic pain or discomfort in the operative field. Conclusions: The robotic surgical platform facilitates a new safe, minimally invasive approach to groin hernia. Because of improved ergonomics, visualization and wristed instrumentation, the robotic approach enabled creation of larger peritoneal flaps and had the potential for less injuries. The major advantage to the patient is a shorter hospital stay, and more rapid postoperative recovery and decreased postoperative pain, lower complications and recurrences.

[Design and clinical application of goal-oriented retroperitoneoscopic adrenalectomy].

Objective: To investigate the goal-oriented retroperitoneoscopic adrenalectomy and report the initial experiment. Methods: A total of 102 patients were selected to our clinic experiment, and performed retroperitoneoscopic adrenalectomy with the new method. including adrenal cortex adenoma 76 cases, phaochromocytoma 12 cases, adrenal cyst 6 cases, myelolipoma 4 cases, gangliocytoma 1 case and corticohyperplassia 3 cases. The mean diameter of the tumors was 2.8 cm (0.5-5.8 cm). The operative procedure was briefly described as such, with ultrasound guiding, a needle was punched percutaneously up to the adrenal mass or the renal upper pole from lateral to posterior axillary line just below the inferior border of the 12th rib. labeled the pathway of the needle with methylene blue. Along the way of the needle, a 12 mm port was introduced into the retroperitoneal space with closed method, and the laparoscope with a working tunnel was introduced to make a tunnel along the label up to the adrenal for finally removing it. Additional port should be used when it was needed in the procedure. Results: The procedures of all patients were successful, and 10 patients were performed with only one port, 81 patients with two ports, 11 patients with three ports. The operative duration was 49 (31-115) min, the average blood loss was 38 (0-260) ml. There was no transition to open surgery and no perioperative complications. The length of postoperative hospital stay was 4.1 d (2-7 d). 98 patients were available for follow-up of 16.5 months (1-38 months), no complication was found. Conclusions: The new method of retroperitoneoscopic adrenalectomy is feasible and safe for renal masses, and compared to the conventional method, it may be less trauma to the abdominal wall and retropertoneal tissue, and it was also better on cosmetics.

Biomarker of long-chain n-3 fatty acid intake and breast cancer: Accumulative evidence from an updated meta-analysis of epidemiological studies.

Objective: We aimed to summarize the up-to-date epidemiology evidence on biomarkers of long-chain (LC) n-3 fatty acid (FA) intake in relation to breast cancer (BC).Methods: Epidemiology studies determining FA levels in biospecimen (circulating blood or adipose tissue (AT)) were identified from PubMed, EMBASE, and Cochrane Library databases until March 2018. Multivariate-adjusted risk ratios (RRs) with 95% confidence intervals (CIs) were pooled using a random-effect model. Difference in biospecimen proportions of LC n-3 FA between BC cases and non-cases were analyzed as a standardized mean difference (SMD).Results: Thirteen cohort and eleven case-control studies were eligible for the present meta-analysis. The estimated SMD was -0.14 (95% CI: -0.27, -0.11) for LC n-3 FA and -0.27 (95% CI: -0.42, -0.11) for LC n-3/n-6 FA ratio. When comparing the top tertiles with the bottom baseline levels, circulating LC n-3 FA was significantly associated with a lower risk of BC (RR: 0.84, 95% CI: 0.74, 0.96), but not AT (RR: 1.02, 95% CI: 0.70, 1.48). Significant inverse dose-response associations were observed for each 1% increment of circulating 20:5n-3 and 22:6n-3.Conclusion: This meta-analysis highlights that circulating LC n-3 FA as a biomarker of intake may be an independent predictive factor for BC, especially 20:5n-3 and 22:6n-3.

Effects of dietary isomaltooligosaccharide and Bacillus spp. supplementation during perinatal period on lactational performance, blood metabolites, and milk composition of sows.

BACKGROUND: In this study, we evaluated the effects of isomaltooligosaccharide (IMO) and Bacillus spp. in perinatal diets on lactational performance, blood metabolites, and milk composition of sows. Multiparous gestating sows (N = 130) with similar body conditions were fed diets without containing IMO (control, CON group), IMO (IMO group), IMO and Bacillus subtilis (IMO + S group), IMO and Bacillus licheniformis (IMO + L group), and IMO and both B. subtilis and B. licheniformis (IMO + S + L group). RESULTS: The average daily feed intake of lactating sows in the IMO + S group (P < 0.05) and the average litter gain and total milk yield in the IMO + S + L group (P < 0.01) were significantly higher than those of the sows in the other treatment groups. In addition, the serum alanine aminotransferase and alkaline phosphatase concentrations in all treatment groups were lower than those in the CON group (P < 0.05). Furthermore, the highest proportion of fat in colostrum (0.05 < P < 0.1) and lowest proportion of lactose in milk were observed in the IMO + L group (P < 0.05). The concentrations of growth hormone and immunoglobulins A and G in milk were higher in sows supplemented with IMO, IMO + S, IMO + L, or IMO + S + L (P < 0.05). CONCLUSION: IMO and Bacillus spp. supplementation during the perinatal period improved the sows' lactational performance by affecting their metabolism and milk quality. Sows in the IMO + S and IMO + S + L treatment groups exhibited the best performance. © 2019 Society of Chemical Industry.

Abnormality of visual neuromagnetic activation in female migraineurs without aura between attacks.

OBJECTIVE: The present study aimed to preliminary explore the abnormal neuromagnetic activation in female migraine patients between attacks using magnetoencephalography (MEG) and pattern reversed visual evoked magnetic fields (PR-VEFs). METHODS: A total of 17 female migraine subjects during the headache-free phase and 17 healthy controls (HC) were studied using a 275-channel magnetoencephalography (MEG) system. In this study, visual evoked magnetic fields (VEFs) were generated by a pattern-reversal check as the visual stimulus. The average of 100 VEFs was evolved by different half patterns were averaged and used to analyze waveform, spectrum, and source location within two frequency ranges (5-100 and 100-1000 Hz), respectively. RESULTS: In migraine subjects, the latency of second peak of VEFs (VIIs) showed significant prolongations when compared with HC. On the sensor level, the cortical spectral power in migraine subjects was similar to that of HC in the 5-100 Hz range and was lower in the 1000-1000 Hz range. There was a decrement of source strength in the visual cortex in migraine patients when compared to HC in both the 5-100 and 100-1000 Hz frequency range. Moreover, there was a similar odds of activation in 5-100 and 100-1000 Hz frequency ranges in the area beyond the primary visual cortex between the two groups. In addition, no correlation was observed between clinical data (intensity of headache, headache-history duration, the frequency of headaches) and MEG results. CONCLUSIONS: The findings presented in the current study, suggested that interictal cortical activation following a visual stimulus was low in female migraine patients. The low pre-activation was detected in the visual cortex using VEF and MEG in both low and high-frequency band. Our results add to the existing evidence that cortical interictal excitability change may be relative to the pain-module mechanism in migraine brains. Thus, our data improved the apprehension of the cortical disorder of migraine in the high-frequency domain.

The international WAO/EAACI guideline for the management of hereditary angioedema-The 2017 revision and update.

Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE-1/2 be defined and classified?, (2) How should HAE-1/2 be diagnosed?, (3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, (4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures?

Optimization of Growth Temperature of ß-Ga2O3 Thin Films for Solar-Blind Photodetectors.

Monoclinic gallium oxide thin films were deposited on c-plane sapphire substrates at various substrate temperatures ranging from 450 °C to 700 °C by radio frequency magnetron sputtering technology. X-ray diffraction results showed that the deposited ß-Ga2O3 films were oriented at ( 2 ¯ 01) direction. As the substrate temperature increased, the intensity of ß-Ga2O3 peaks increased and bandgap decreased accordingly. Metal/semiconductor/metal structured solar-blind photodetectors based on ß-Ga2O3 thin films growing at various substrate temperatures had been fabricated. The growth temperatures of thin films had no obvious influence on dark current and response to 365 nm light illuminations. The photoelectric properties such as responsivity and response speed of the thin films to 254 nm light illuminations were growth temperature dependent. At an applied bias of 50 V, the photodetectors prepared with 450 °C grown film had the highest responsivity of 2.18 A/W, and the photodetectors prepared with 700 °C grown film had the shortest rising time of 0.95 s under 254 nm light illuminations.

Virtual Planning and 3D printing modeling for mandibular reconstruction with fibula free flap.

BACKGROUND: This study was to evaluate the use of virtual planning and 3D printing modeling in mandibular reconstruction and compare the operation time and surgical outcome of this technique with conventional method. MATERIAL AND METHODS: Between 2014 and 2017, 15 patients underwent vascularized fibula flap mandibular reconstruction using virtual planning and 3D printing modeling. Titanium plates were pre-bent using the models and cutting guides were used for osteotomies. 15 patients who underwent mandibular reconstruction using fibula flap without aid of virtual planning and 3D printing models were selected as control group. The operation time was recorded and compared in two groups. Accuracy of reconstruction was measured by superimposing the preoperative image onto the postoperative image of mandible. The selected bony landmark, distance and angle were measured. RESULTS: The mean total operation time and reconstruction time were 1.60±0.37 and 5.54±0.50 hours in computer-assisted group, respectively; These were 2.58±0.45 and 6.54±0.70 hours in conventional group, respectively. Both operation time and reconstruction time were shorter in computer-assisted group. The difference between the preoperative and postoperative intercondylar distances, intergonial angle distances, anteroposterior distances and gonial angles were 2.92±1.15 and 4.48±1.41mm, 2.93±1.19 and 4.79±1.48mm, 4.31±1.24 and 5.61±1.41mm, 3.85±1.68° and 5.88±2.12° in the computer-assisted and conventional group, respectively. The differences between the preoperative and postoperative mandible is smaller in the computer-assisted group. CONCLUSIONS: Virtual planning and 3D printing modeling have the potential to increase mandibular reconstruction accuracy and reduce operation time. we believe that this technology for mandibular reconstruction in selected patients will become a used method and improve the quality of reconstruction.

[Virtual planning and 3D printing modeling for mandibular reconstruction with fibula free flap].

Objective: To evaluate the use of virtual planning and 3D printing modeling in mandibular reconstruction and compare the operation time and surgical outcome of this technique with conventional method. Methods: Between June 2013 and June 2017, A total of 18 patients underwent the mandibular reconstruction with fibula free flap in the Affiliated Hospital of Qingdao University.Among 18 patients, there were 11 males and 7 females with an average age of 36.5 years (21-73 years). Nine patients underwent vascularized fibula flap mandibular reconstruction using virtual planning and 3D printing modeling.Titanium plates were pre-bent using the models and cutting guides which were used for osteotomies.Another 9 patients who underwent mandibular reconstruction using fibula flap without aid of virtual planning and 3D printing models were selected as control group. The operation time was recorded and compared in two groups. Accuracy of reconstruction was measured by superimposing the preoperative image onto the postoperative image of mandible. The selected bony landmark, distance and angle were measured. Results: The mean total operation time were 4.7-6.2(5.5±0.5) h in computer-assisted group and 5.6-7.5(6.6±0.7) h in conventional group, respectively. The operation time was shorter in computer-assisted group. The difference between the preoperative and postoperative intercondylar distances, intergonial angle distances, anteroposterior distances were(2.6±1.4)vs(4.4±1.6)mm, (2.9±1.2)vs(4.7±1.7)mm, (4.2±1.4) vs(5.9±1.8)mm in the computer-assisted and conventional group, respectively. The differences between the preoperative and postoperative mandible were smaller in the computer-assisted group. Conclusions: Virtual planning and 3D printing modeling have the potential to increase mandibular reconstruction accuracy and reduce operation time. We believe that this technology for mandibular reconstruction in selected patients can significantly improve the quality of reconstruction.