Long-term renal survival of γ3-heavy-chain deposition disease complicated by heart failure: A case report.

Key Clinical Message: Heavy-chain deposition disease (HCDD), a rare monoclonal immunoglobulin deposition disease, involves truncated heavy-chain deposition in kidneys. Limited long-term data exist. We report a case of renal and cardiac failure with favorable outcomes post bortezomib-based therapy. Stable renal function observed over 4 years suggests efficacy in HCDD with multisystem involvement. Abstract: Heavy-chain deposition disease (HCDD) is an extremely rare form of monoclonal immunoglobulin deposition disease (MIDD) that involves the deposition of truncated immunoglobulin heavy chains in the kidneys. Only a few cases of HCDD with a favorable long-term renal prognosis have been reported, resulting in limited long-term follow-up data for this patient population. In this report, we present the case of a 52-year-old patient with nephrotic syndrome who experienced renal failure and cardiac failure. Renal biopsy confirmed the presence of γ3-HCDD and monoclonal Immunoglobulin G (IgG)κ in the serum. The patient exhibited low voltage on electrocardiogram (ECG) and unexplained left ventricular hypertrophy on cardiac ultrasound. The patient underwent eight cycles of bortezomib-based chemotherapy, which led to hematological remission. After 4 years of follow-up, the patient's renal function remained stable, with serum creatinine levels ranging from 0.7 to 0.9 mg/dL and proteinuria of 0.3-0.5 g/24 h. Our findings suggest that bortezomib-based chemotherapy is equally effective in HCDD patients with combined multisystem damage.

Long-axis in-plane combined with short-axis out-of-plane technique in ultrasound-guided arterial catheterization in infants: A randomized controlled trial.

STUDY OBJECTIVE: To determine whether the long-axis in-plane (LAX-IP) combined with short-axis out-of-plane (SAX-OOP) technique is more suitable than modified dynamic needle tip positioning (MDNTP) technique for ultrasound-guided radial artery catheterization in infants. DESIGN: A randomized controlled trial. SETTING: Department of Anesthesiology, Children's Hospital of Chongqing Medical University. PATIENTS: Overall, 72 patients, aged 1-12 months old, who were primarily undergoing thoracic or cardiac surgery in the Children's Hospital of Chongqing Medical University between July 1, 2021, and March 31, 2022, were selected. These patients were randomly divided into two groups: i) the MDNTP group and ii) the LAX-IP combined with SAX-OOP group. INTERVENTIONS: Radial artery cannulation in the two groups was performed using ultrasound-guided MDNTP or LAX-IP combined with SAX-OOP technique. MEASUREMENTS: The primary outcome was first-time success rate, and the secondary outcomes included total success rate, cannulation time, and incidence of complications. MAIN RESULTS: In the LAX-IP combined with SAX-OOP group, the first-time success rate was 75.0% (n = 27), total success rate was 97.2% (n = 35), cannulation time was 91.39 ± 102.60 s, puncture attempts was 1.5 ± 1.3 times, and local hematoma was formed on the first day in one (2.8%) infant. In the MDNTP group, the first-time success rate was 36.1% (n = 13) (P = 0.001; RR, 2.08; 95% confidence interval, 1.29-3.34), total success rate was 91.7% (n = 33) (P = 0.303; RR, 1.06; 95% confidence interval, 0.95-1.19), cannulation time was 181.00 ± 146.72 s(P = 0.047; Median difference,-89.61; 95% confidence interval, -149.12 to -30.10), puncture attempts was 2.3 ± 1.6 times (P = 0.133; Median difference,-0.81), and local hematoma was formed on the first day in nine (25%) infants (P = 0.006; RR, 0.11; 95% confidence interval, 0.01-0.83). No thrombosis occurred in any group. CONCLUSIONS: The ultrasound-guided LAX-IP combined with SAX-OOP technique for radial arterial catheterization in infants, which was performed by anesthesia residents, exhibited an increased first-time success rate, reduced cannulation time, and lower incidence of complications than the MDNTP technique.

Outer Membrane Vesicles from Acinetobacter baumannii: Biogenesis, Functions, and Vaccine Application.

This review focuses on Acinetobacter baumannii, a Gram-negative bacterium that causes various infections and whose multidrug resistance has become a significant challenge in clinical practices. There are multiple bacterial mechanisms in A. baumannii that participate in bacterial colonization and immune responses. It is believed that outer membrane vesicles (OMVs) budding from the bacteria play a significant role in mediating bacterial survival and the subsequent attack against the host. Most OMVs originate from the bacterial membranes and molecules are enveloped in them. Elements similar to the pathogen endow OMVs with robust virulence, which provides a new direction for exploring the pathogenicity of A. baumannii and its therapeutic pathways. Although extensive research has been carried out on the feasibility of OMV-based vaccines against pathogens, no study has yet summarized the bioactive elements, biological activity, and vaccine applicability of A. baumannii OMVs. This review summarizes the components, biogenesis, and function of OMVs that contribute to their potential as vaccine candidates and the preparation methods and future directions for their development.

Identification of cerebrospinal fluid metabolites as biomarkers for neurobrucellosis by liquid chromatography-mass spectrometry approach.

Neurobrucellosis is the most morbid form in brucellosis disease. Metabolomics is an emerging method which intends to explore the global alterations of various metabolites in samples. We aimed to identify metabolites in cerebrospinal fluid (CSF) as biomarkers that were potentially unique for neurobrucellosis. CSF samples from 25 neurobrucellosis patients and 25 normal controls (uninfected patients with hydrocephalus) were collected for metabolite detection using liquid chromatography-mass spectrometry (LC-MS) approach. Inflammatory cytokines in CSF were measured with Enzyme-linked immunosorbent assay (ELISA). The base peak chromatogram in CSF samples showed that small-molecule metabolites were well separated. Principal Component Analysis (PCA) analysis exhibited the examined samples were arranged in two main clusters in accordance with their group. Projection to Latent Structures Discriminant Analysis (PLS-DA) revealed there was a noticeable separation between neurobrucellosis and normal groups. Orthogonal Partial Least-Squares-Discriminant Analysis (OPLS-DA) could responsibly illuminate the differences between neurobrucellosis and normal controls. Neurobrucellosis showed a total of 155 differentiated metabolites. Prominent potential biomarkers including 30 metabolites were then selected out, regarded as more capable of distinguishing neurobrucellosis. TNF-α and IL-6 in CSF were remarkably increased in neurobrucellosis. We presented the heatmaps and correlation analyses among the identified 30 potential biomarkers. In conclusion, this study showed that CSF metabolomics based on LC-MS could distinguish neurobrucellosis patients from normal controls. Our data offered perspectives for diagnosis and treatment for neurobrucellosis.

BHBA regulates the expressions of lipid synthesis and oxidation genes in sheep hepatocytes through the AMPK pathway.

Pregnancy toxemia (PT) is the most frequent metabolic disease of sheep during late pregnancy, which can lead to enormous economic losses in sheep farm industry. However, the underlying mechanism of PT in sheep has not been fully elucidated. High levels of ß-hydroxy butyric acid (BHBA) exist in PT sheep. The AMP-activated protein kinase (AMPK) pathway plays a major role in regulating liver function. The aim of this study was to explore the effects of gradient concentrations of BHBA on lipid metabolism of sheep hepatocytes and the underlying molecular mechanism in vitro. The results showed that 0.6, 1.2 mmol/L BHBA could activate AMPKα, promoted the expressions of peroxisome proliferator-activated receptor alpha (PPARα) and its target genes, and inhibited the expressions of sterol regulatory element binding protein-1c (SREBP-1c) as well as its downstream genes. When the concentration of BHBA was beyond 1.2 mmol/L, the expressions of the above-mentioned proteins and genes were just the opposite. However, the expressions of adipose triglyceride lipase (ATGL) and hormone-sensitive lipase (HSL) did not change significantly. The levels of very low density lipoprotein (VLDL), triglyceride (TG) and cholesterol (T-CHOL) showed a gradually increasing trend with the increase of BHBA concentration. According to the results above, it demonstrates that high levels of BHBA can inhibit the expression of the AMPK pathway and cause lipid metabolism disorders in sheep hepatocytes, which may lead to the occurrence of PT.

Optimal insertion depth of subclavian vein catheterization via the right supraclavicular approach in children.

BACKGROUND: Methods to determine the optimal insertion depth of ultrasound-guided supraclavicular approach to the subclavian vein (SCV) catheterization, alternatively used for central venous access, are debatable in children. AIM: We investigated the applicability and reliability of the modified formula for determining the depth of SCV catheterization using an ultrasound-guided supraclavicular approach in children. METHODS: This prospective observational study included 36 children (age <6 years; weight ≥5 kg) scheduled to undergo congenital heart disease surgery. After intubation, ultrasound-guided supraclavicular approach to the SCV catheterization was performed. Actual insertion depth was determined by real-time transesophageal echocardiography. Insertion depth was calculated by subtracting 1 cm from the sum of the distance from the insertion point to the sternal head of the right clavicle and that from the latter point to the midpoint of a perpendicular line drawn from the sternal head of the right clavicle to the line connecting the nipples. RESULTS: Insertion depth calculated with the modified formula and actual insertion depth of the SCV catheter correlated strongly (r = .806, 95% confidence interval [CI]: 0.658-0.908; p < .001). Bland-Altman analysis showed a mean bias and precision of 0.36 and 0.65 cm, respectively (95% CI: 0.14-0.58, 95% limits of agreement: -0.92, 1.64). All plots were above the -1.0 line, indicating no catheter tip insertion into the right atrium. CONCLUSIONS: Optimal insertion depth for an ultrasound-guided supraclavicular approach to the SCV catheterization can be calculated using modification of a surface landmark formula in children younger than 6 years and weight heavier than 5 kg.

Prognostic Analysis of Preoperative Inflammatory Biomarkers in Patients With Laryngeal Squamous Cell Carcinoma.

OBJECTIVE: The purpose of this study was to demonstrate the prognostic role of inflammatory biomarkers in patients with laryngeal squamous cell carcinoma. METHODS: For this study, we enrolled 151 patients who had undergone surgery for laryngeal squamous cell carcinoma. We assessed the preoperative neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), monocyte to lymphocyte ratio (MLR), mean platelet volume, red cell distribution width, and alkaline phosphatase. The chi-square test, Kaplan-Meier survival analysis, and Cox proportional hazards model were conducted on overall survival, progression-free survival, locoregional recurrence-free survival, and distant metastasis-free survival of patients with laryngeal squamous cell carcinoma. RESULTS: Both Kaplan-Meier analysis and univariate analysis showed significant prognostic differences with age, laryngectomy methods, Tumor Node Metastasis (TNM) staging, tumor location, NLR, PLR, MLR, and mean platelet volume. Multivariate analysis indicated that NLR (overall survival: hazard ratio [HR] = 3.02, 95% confidence interval [CI]: 1.28-7.10, P = .011), PLR (overall survival: HR = 0.33, 95% CI: 0.14-0.78, P = .011; progression-free survival: HR = 0.016,95% CI: 0.10-0.79, P = .016), and MLR (overall survival: HR = 0.29, 95% CI: 0.11-0.76, P = .012) were independent prognostic factors for 5-year survival. However, red cell distribution width and alkaline phosphatase had no significant difference in overall survival and progression-free survival. CONCLUSIONS: Preoperative high NLR, PLR, and MLR were associated with poor prognosis. They were found to be effective and reliable inflammatory biomarkers for patients with laryngeal squamous cell carcinoma.

Ex vivo liver resection and autotransplantation with cardiopulmonary bypass for hepatoblastoma in children: A case report.

Some primary and secondary liver carcinomas cannot be resected using a conventional approach due to their size, location, or major vascular invasion. The aim of this study was to explore the application of ex vivo liver resection and autotransplantation for complicated HB in children. A 1.5-year-old girl with HB after repeated chemotherapy was analyzed. In this patient, tumor invasion includes the IV, V, and VIII liver segments, and thrombosis formed in the inferior vena cava and right atrium. It is difficult to obtain complete tumor resection using conventional hepatectomy. The patient was treated via ex vivo liver resection and autotransplantation, and tumor thrombus in the inferior vena cava and right atrium was removed via cardiopulmonary bypass. Operative methods and precautions were analyzed during and after the operation. The operation was completed successfully within 8 hours, and the liver's cold ischemia time was 190 minutes. The patient recuperated successfully, and the liver's function and AFP levels gradually tended to normalize 2 weeks after the operation. Ultrasonic examination revealed that the blood flow velocity of the hepatic vein, portal vein, and hepatic artery was good. The patient recovered and was discharged 3 weeks after the operation. Ex vivo liver resection and autotransplantation have great application value for complicated HB in children that is not suitable for conventional hepatic lobectomy.

Annular pancreas in China: 9 years' experience from a single center.

To summarize the clinical characteristics, diagnosis, treatment and prognosis among 152 children with annular pancreas (AP). A retrospective review of 152 patients with AP who were treated with surgical repair between January 2009 and August 2017 was performed at our pediatric surgical units. Presenting symptoms, birth weight, radiological findings, associated anomalies, the type of surgery performed were analyzed. (1) 152 patients were identified, out of which 82 were males, and 70 were females; (2) the average birth weight of children with AP was less than that of healthy newborns. The birth weights of 5.4% premature infants were less than 1500 g; the birth weight of 17% full-term infants, 69% premature infants and 50% post-term infants were less than 2500 g. (3) 100 (66%) patients presented symptoms during neonatal period and 43 (28%) patients had duodenal obstruction diagnosed by prenatal ultrasound scan. (4) All cases were managed surgically by open laparotomy, and all patients were duly discharged. AP most commonly presents symptoms in early neonatal period. Infants with AP are associated with a higher rate of low birth weight, and it was because swallowed amniotic fluid could not be absorbed and impaired insulin secretion caused by abnormal pancreas. Ultrasonography, abdominal plain film and upper gastrointestinal series (UGI) are helpful, but cannot make the diagnosis, and surgery is the only effective way to diagnose and treat AP.

Treatment Efficacy and Risk Factors of Neurobrucellosis.

BACKGROUND: This study aimed to analyze the risk factors and treatment efficacy of neurobrucellosis. MATERIAL/METHODS: A cross-sectional epidemiologic survey was carried out in 557 patients with brucellosis by specially trained neurologic clinicians. Sixty-six patients with neurobrucellosis were treated with doxycycline, rifampicin, and ceftriaxone sodium as standard medication and evaluated for efficacy on a regular basis. RESULTS: (1) Symptoms improved in most patients after 6 weeks of treatment, which demonstrated a favorable efficacy. (2) Cross-sectional epidemiologic survey suggested that sex, nationality, and regional distribution were not related to nervous system damage in patients with brucellosis (P>0.05), whereas age and duration of disease were related factors. Increased age as well as a prolonged duration of disease were risk factors for nervous system damage in patients with brucellosis (P<0.05). CONCLUSIONS: (1) Doxycycline, rifampicin, and third-generation cephalosporins should be considered both standard and first-choice medications for neurobrucellosis. Treatment should last for at least 6 weeks. Standardized, sufficient, and combined medication is recommended for better efficacy and prognosis. (2) Age and duration of disease are risk factors for neurobrucellosis, whereas sex, nationality, and regional distribution are not. Older patients with a prolonged duration of disease are more likely to develop neurobrucellosis.

Hypoxia Enhances Direct Reprogramming of Mouse Fibroblasts to Cardiomyocyte-Like Cells.

Recent work has shown that mouse and human fibroblasts can be reprogrammed to cardiomyocyte-like cells with a combination of transcription factors. Current research has focused on improving the efficiency and mechanisms for fibroblast reprogramming. Previously, it has been reported that hypoxia enhances fibroblast cell reprogramming to pluripotent stem cells. In this study, we observed that 6 h of hypoxic conditions (2% oxygen) on newborn mouse dermal fibroblasts can improve the efficiency of reprogramming to cardiomyocyte-like cells. Expression of cardiac-related genes and proteins increased at 4 weeks after transfer of three transcription factors (Gata4/Mef2c/Tbx5 [GMT]). However, beating cardiomyocyte cells were not detected. The epigenetic mechanism of hypoxia-induced fibroblast reprogramming to cardiomyocyte cells requires further study.

[Effect of shexiang baoxin pill on the function of vascular endothelium in patients with diabetes mellitus type 2 complicated with angina pectoris].

OBJECTIVE: To study the effect of Shexiang Baoxin pill (SBP) on the vascular endothelial function in patients with diabetes mellitus type 2 (DM2) complicated with angina pectoris. METHODS: Two weeks after runin, according to the randomizing table, 111 patients were divided into two groups, the XBP group (56 patients) and the control group (55 patients, treated with delayed-released isosorbide mononitrate, DRIM), they were treated for 6 months. In the treatment period, the episodes of angina attack and condition of rescue medication were recorded in the daily card, and brachial arterial changes of endothelium-dependent relaxing function before and after treatment were measured by B-ultrasonography. RESULTS: Comparison between the two groups in episodes of angina attack and rescue medication were insignificantly different. In the control group, the basal value of brachial arterial inner diameter before and after treatment was 3.68 +/- 0.56 mm and 3.70 +/- 0.58 mm respectively, those before and after responsive congestion was 5.44 +/- 0.81% vs 5.68 +/- 0.83%, and those before and after taking nitroglycerin was 19.8 +/- 4.9% vs 20. +/- 5.2%, all showed insignificant difference (P > 0.05). In the SBP group, the corresponding basal value was 3.73 +/- 0.62 mm vs 3.71 +/- 0.59 mm, and those after taking nitroglycerin 18.8 +/- 4.5 % vs 19.2 +/- 5.8%, also showed insignificant difference, but those before and after responsive congestion (5.69 +/- 0.79 % vs 9.56 +/- 3.82 %) did show significant difference (P < 0.01). CONCLUSION: XBP could improve the vascular endothelial function in patients with DM2 complicated with angina pectoris.