[Diagnosis and treatment of arrhythmogenic cardiomyopathy in children].

Objective: To summarize the clinical manifestations, experiences in diagnosis and treatment of arrhythmogenic cardiomyopathy (ACM) in children. Methods: A retrospective analysis of the clinical manifestations, laboratory tests, radiological features, treatment and follow-up results was conducted in 11 children diagnosed with ACM at the center of congenital heart disease, Beijing anzhen hospital from May 2010 to March 2022. Results: A total of 11 patients aged 2 to 16 years, including 5 males and 6 females were diagnosed with ACM. The clinical manifestations included decreased activity tolerance (7 patients), heart failure (4 patients), syncope or sudden death (3 patients), palpitation (3 patients), and chest tightness and pain (3 patients). Electrocardiogram showed right bundle branch block in 9 cases, paroxysmal ventricular tachycardia in 4 cases, frequent premature ventricular contraction in 4 cases, ventricular pre-excitation in 1 case, left bundle branch block in 1 case, and first degree atrioventricular block in 2 cases. Echocardiography showed enlargement of the right heart, widening of the right ventricular outflow tract, and thinning and bulging of the local wall of the right ventricle with reduced pulsation. Ventricular thrombosis was found in 2 cases. Six children underwent cardiac magnetic resonance imaging, which mainly showed severe enlargement of the right heart, thin free wall of the right ventricle, decreased right heart function, enhanced right ventricular myocardium, and formation of right ventricular aneurysm. Two children underwent myocardial biopsy examination and presented with typical pathological changes of ACM. Genetic tests in five patients revealed DSG2 gene mutation in 2 cases, PKP2 gene mutation in 2 cases, and MYH6 gene mutation in 1 case. All patients received anti heart failure treatment and antiarrhythmic drugs. Two children received anticoagulant treatment due to ventricular thrombosis. Radiofrequency ablation was performed in 2 patients. Glenn procedure was performed in 4 patients, and heart transplantation was performed in 1 patient due to progressive heart failure. The follow-up period ranged from 6 months to 12 years. Two cases died of right heart failure, 6 cases had different degrees of heart failure, 1 case had intermittent chest tightness and pain, and 2 cases were stable. Conclusions: ACM is a progressive genetic cardiomyopathy characterized by decreased activity tolerance, cardiac failure and arrhythmia in pediatric patients. The diagnosis is mainly based on clinical manifestations, electrocardiogram, cardiac imaging changes, and genetic testing. Early detection, diagnosis, and personalized treatment can improve the prognosis.

LncRNA LUCAT1 promotes rowth, migration, and invasion of oral squamous cell carcinoma by upregulating PCNA.

Since this article has been suspected of research misconduct and the corresponding authors did not respond to our request to prove originality of data and figures, "LncRNA LUCAT1 promotes rowth, migration, and invasion of oral squamous cell carcinoma by upregulating PCNA, by Y. Kong, Y. Feng, Y.-Y. Xiao, S.-C. Liu, X.-G. Li, Q.-L. Yang, W.-H. Chu, J.-G. Liu, published in Eur Rev Med Pharmacol Sci 2019; 23 (11): 4770-4776- DOI: 10.26355/eurrev_201906_18059-PMID: 31210306" has been withdrawn. The Publisher apologizes for any inconvenience this may cause. https://www.europeanreview.org/article/18059.

Minimal-access retroperitoneal pancreatic necrosectomy for infected necrotizing pancreatitis: a multicentre study of a step-up approach.

BACKGROUND: Various minimally invasive approaches have been described for infected necrotizing pancreatitis. This article describes a modified minimal-access retroperitoneal pancreatic necrosectomy (MARPN) procedure assisted by gas insufflation. METHODS: This retrospective, observational study documented patients who had undergone a step-up MARPN between 1 January 2010 and 31 December 2016. A minimum follow-up of 1 year was required for inclusion. The step-up approach involved percutaneous catheter drainage followed by the modified MARPN and necrosectomy. If more than one access site was needed it was categorized as complex MARPN. RESULTS: Of 212 patients with infected necrotizing pancreatitis, 164 (77·4 per cent) underwent a step-up approach. The median number of percutaneous catheter drains and MARPN procedures was 3 (range 1-7) and 1 (1-6) respectively. Ninety patients (54·9 per cent) underwent complex MARPN. For residual necrosis after MARPN, three patients (1·8 per cent) underwent sinus tract gastroscopy, and 11 (6·7 per cent) had sinography combined with a tube change. However, operations in 13 patients (7·9 per cent) required conversion to open surgery. Postoperative complications developed in 103 patients (62·8 per cent). The mortality rate was 6·1 per cent (10 deaths). CONCLUSION: A step-up approach using a modified MARPN for infected necrotizing pancreatitis is a reasonable option.

ANTECEDENTES: Los procedimientos mínimamente invasivos se han convertido en los más frecuentes para el tratamiento de necrosis pancreáticas infectadas. El objetivo de este estudio fue presentar un procedimiento de necrosectomía pancreática retroperitoneal de acceso mínimo (minimal-access retroperitoneal pancreatic necrosectomy, MARPN) modificado y asistido mediante insuflación de gases, así como evaluar su seguridad y eficacia. MÉTODOS: Se realizó un análisis retrospectivo y observacional de los datos de un hospital desde el 1 de enero de 2010 hasta el 31 de diciembre de 2016. Se incluyeron en el análisis todos los pacientes en los que realizó un abordaje por etapas, que consistía en el drenaje percutáneo mediante la colocación de un catéter seguido de un procedimiento MARPN modificado, en los que se dispusiese de un seguimiento postoperatorio mínimo de 1 año. El MARPN en el lado derecho y la necrosectomía realizada a través de más de un acceso se clasificaron como MARPN complejo. Se evaluaron los resultados radiológicos y quirúrgicos. RESULTADOS: De 212 pacientes con necrosis pancreática infectada, en 164 (77,4%) se realizó un abordaje por etapas. La mediana del número de drenajes percutáneos y procedimientos MARPN fue 3 (rango, 1-7) y 1 (rango, 1-6), respectivamente. En 90 pacientes (54,9%) se realizó un MARPN complejo. Para la exéresis de necrosis residual después de un MARPN, en 3 pacientes (1,8%) se realizó mediante gastroscopia y en 11 pacientes (6,7%) con un recambio de drenaje bajo control radiológico. En 13 pacientes (7,9%) fue necesaria la reconversión a cirugía abierta. Hubo complicaciones postoperatorias en 103 pacientes (62,8%). La tasa de mortalidad fue del 6,1% (n = 10). CONCLUSIÓN: El abordaje por etapas con un MARPN modificado es seguro y efectivo en el tratamiento de la necrosis pancreática infectada.

LncRNA LUCAT1 promotes rowth, migration, and invasion of oral squamous cell carcinoma by upregulating PCNA.

OBJECTIVE: Recent studies have revealed the important role of long noncoding RNAs (lncRNAs) in the progression of tumorigenesis. This study aims to identify how lncRNA LUCAT1 functions in the progression of OSCC (oral squamous cell carcinoma). PATIENTS AND METHODS: Relative expression of lncRNA LUCAT1 in both OSCC cells and 50 paired tissue samples was detected by quantitative Real-time polymerase chain reaction (qRT-PCR). Moreover, biological function of LUCAT1 in OSCC was identified by performing transwell assay, wound healing assay and proliferation assay in vitro. The underlying mechanism was explored by qRT-PCR and Western blot. RESULTS: LUCAT1 expression was remarkably downregulated in OSCC tissues when compared with that in adjacent normal samples. Moreover, proliferation, invasion and migration of OSCC cells were inhibited after knockdown of LUCAT1 in vitro. Knockdown of LUCAT1 downregulated PCNA in OSCC cells at mRNA and protein level in vitro. Besides, PCNA expression in OSCC tissues was positively correlated with the expression of LUCAT1. CONCLUSIONS: Knockdown of LUCAT1 could inhibit migration, invasion and proliferation capacities of OSCC cells through downregulating PCNA, which may offer a new therapeutic intervention for OSCC patients.

[Clinical application of CT-guided radiofrequency ablation combined with biopsy synchronously to multiple small nodules of lung metastatic tumors].

Objective: To investigate the therapeutic efficacy and safety of CT-guided radiofrequency ablation(RFA)combined with biopsy synchronously to multiple small nodules of metastatic tumors in lung. Method: From January 2016 to December 2016, a total of 86 patients in the General Hospital of People's Liberation Army with 144 lesions were divide into two groups(all the lesions were less than 1 cm). Group A with 51 cases located in the lung periphery underwent biopsy prior to RFA.Group B with 35 cases located in the middle and inner side of lung adjacent to the vasculatures contrarily underwent RFA first.The changes of these lesions during the 1 to 12-month were followed up. Results: All the procedures were completed successfully.The intra operative CT scanning showed the ablation zones were completely covered by the indicative "halo sign" respectively.The P value was 0.818, 0.155 and 0.452 respectively, in the number of nodules, pathological results positive rate and complications in the rank and inspection for A, B two groups, which were all higher than 0.05.Though different strategies according to different location of the nodes, the two groups can achieve safe and effective treatments.All patients in two groups had high density ablation zones on their 1-month post operative CT without enhancement.3-and 6-post operative CT illustrated a decrease of lesions, 12-month post operative CT showed the lesions turned to fibrous stripes. Conclusion: The CT-guided RFA combined with biopsy synchronously to multiple small nodules of metastatic tumors in lung is safe and effective, for the lesions located in the middle or inner side of lung, RFA prior to the biopsy can avoid the massive hemoptysis.

[Experience of minimal-access video-assisted retroperitoneal debridement in treatment of infected pancreatic necrosis].

Objective: To explore the experience of minimal-access video-assisted retroperitoneal debridement in treatment of infected pancreatic necrosis(IPN). Methods: A retrospective review was performed on 12 patients with IPN who underwent minimal-access video-assisted retroperitoneal debridement between June 2008 and June 2013 in People's Liberation Army General Hospital and First Affiliated Hospital of People's Liberation Army General Hospital, respectively.There were 10 male patients and 2 female patients aging from 33 to 55 years with mean age of(43±8)years.Pancreas infective necrosis, the serious complications of severe acute pancreatitis occurred in all of the patients among which there were 2 patients with infection after percutaneous catheter drainage(PCD)in early phase of disease, and 12 patients with spontaneous during the late phase. The technical strategies of the minimally invasive treatment mainly included PCD, minimal-access video-assisted retroperitoneal debridement, and irrigation. Results: Ten patients received PCD and the median time from onset of acute necrotizing pancreatitis to PCD was mean of 24 days(range 8-86 days). Minimal-access video-assisted retroperitoneal debridement was performed after 18 days(range 3-29 days) after PCD.Three patients died after surgery.Five patients had hemorrhage complication and 3 had colonic fistula.Pancreatic fistula occurred in 2 patients. Conclusions: The technique of minimal-access video-assisted retroperitoneal debridement has advantage and some minor disadvantage.Delayed minimal-access video-assisted retroperitoneal debridement is recommended.

Identification of LCK mutation in a family with atypical epidermodysplasia verruciformis with T-cell defects and virus-induced squamous cell carcinoma.

BACKGROUND: Inherited epidermodysplasia verruciformis (EV) is a rare skin disorder characterized by susceptibility to specific types of human papilloma virus (HPV) and is strongly associated with skin carcinomas. Inactivating mutations in EVER1/EVER2 account for most cases of EV. However, more phenotypes related to but distinct from EV have been reported with an immunodeficiency state but without EVER1/EVER2 mutation, and the genetic basis for these atypical EV cases is poorly understood. OBJECTIVES: To identify the causative gene responsible for three siblings affected by atypical EV but without EVER1/EVER2 mutation. METHODS: Whole-exome sequencing followed by Sanger sequencing was performed to identify the gene responsible for the patients with atypical EV enrolled in our study. RESULTS: A homozygous splicing mutation was detected in LCK (c.188-2A>G). This mutation resulted in an exon 3 deletion T lymphocyte-specific protein tyrosine kinase isoform, which further led to frameshift mutation and subsequent mRNA decay. CONCLUSIONS: We demonstrate a novel mutation in LCK in a family affected by atypical EV with T-cell defects, HPV infection and virus-induced malignancy, providing new clues in the understanding of host defences against HPV and better genetic counselling of patients with the EV phenotype.

Protective effects of morphine preconditioning in delayed phase on myocardial ischemia-reperfusion injury in rabbits.

The aim of this study was to investigate the protective mechanisms of delayed-phase morphine preconditioning on myocardial ischemia-reperfusion injury. Thirty healthy male New Zealand white rabbits were randomly divided into three groups: a sham operation group (C), ischemia-reperfusion group (I/R), and delayed-phase morphine preconditioning group (M) (N = 10/group). Rabbits in the C group received thoracotomy for 160 min. Rabbits in the I/R group received left artery blockage for 40 min and reperfusion for 120 min. Rabbits in the M group received 1.0 mg/kg intravenous morphine 24 h prior to the identical treatment as the rabbits in the I/R group. In each group, the interleukin (IL)-10 and tumor necrosis factor (TNF)-α levels were detected at five time points: 20 min before the left coronary artery blockage (T1), 20 and 40 min after the left coronary artery blockage (T2 and T3, respectively), and 1 and 2 h after the myocardial reperfusion (T4 and T5, respectively). After reperfusion, the infarction size was measured with Evans blue and 2,3,5-triphenyltetrazolium chloride (TTC) staining. Compared with the C group, serum IL-10 and TNF-α concentrations increased in the I/R and M groups; the difference was significant (P < 0.05). When compared with the I/R group, the IL-10 concentrations in the M group were significantly increased (P < 0.05), but the infarction size and TNF-α concentrations were significantly decreased (P < 0.05). These results suggested that delayed-phase morphine preconditioning might achieve myocardial protection through the regulation and balance of inflammatory cytokines.

Effects of isoflurane preconditioning in the delayed phase on myocardial tumor necrosis factor alpha levels and caspase-3 protein expression in a rabbit model of ischemia-reperfusion injury.

This study aimed to investigate the protective effects and the mechanisms underlying these effects of isoflurane preconditioning in the delayed phase of myocardial ischemia-reperfusion injury. We randomly divided 30 healthy male New Zealand white rabbits into three groups with 10 rabbits in each group as follows: sham operation group (C group), ischemia-reperfusion group (I/R group), and 2.0% isoflurane preconditioning group (S group). Rabbits in the C group received thoracotomy for 160 min. Rabbits in the I/R group underwent left coronary artery occlusion for 40 min and reperfusion for 120 min. Rabbits in the S group received inhalation of 2.0% isoflurane and 100% oxygen for 2 h; after 24 h, rabbits in this group received the same treatment as that administered to rabbits in the I/R group. We examined the tumor necrosis factor alpha (TNF-α) levels in each group 20 min before occlusion of the left coronary, 20 and 40 min after occlusion of the left coronary artery, and 1 and 2 h after myocardial reperfusion. After reperfusion, immunoblotting was used to measure the myocardial caspase-3 expression levels, and the infarct size was measured using Evans blue and tetrazolium chloride staining. The levels of TNF-α and caspase-3 were lower in the S group than in the I/R group, and the myocardial infarct size decreased in the S group. Thus, isoflurane preconditioning in the delayed phase exerted protective effects by decreasing the myocardial caspase-3 expression and TNF-α production in a rabbit model of ischemia-reperfusion injury.

CT-guided percutaneous chemoablation using an ethanol-ethiodol-doxorubicin emulsion for the treatment of metastatic lymph node carcinoma: a comparative study.

Current therapies for metastatic lymph node (LN) are a major burden on health-care systems. Alternative such as percutaneous chemoablation using interstitial injection of ethanol or ethanol-ethiodol-drug(s) mixture (EEM) has been successfully applied to solid tumor ablation of the liver, adrenal glands, lymph nodes and others. However, EEM chemoablation efficacy on two most frequent clinical lymph node presentation, isolated or confluent, has not yet been determined. This study was designed to compare the therapeutic effects of CT-guided percutaneous EEM injection (PEEMI) on patients presenting with single or multiple confluent metastatic LN localizations from various carcinoma. Thirty six patients with metastatic LN carcinoma at various anatomical regions were enrolled from 2009 to 2010 over a nine month period. They were separated into two groups: group A includes 24 single isolated metastatic lymph nodes and group B includes 12 multiple and confluent nodes. The primary end point was the ablative efficacy of PEEMI. The intratumoral (IT) EEM distribution and the occurrence of reflux were recorded. Adjuvant systemic chemotherapy was administered after the procedure. Contrast-enhanced CT scans were performed during procedure and follow-up. Six patients were tested for Fluorodeoxyglucose (FDG) fixation before and after the procedure. For group A the EEM IT distribution-to-tumor ratio was 100% vs. 50% to 80% for group B (t = 11.5, p < 0.05). The reflux frequency was 80% for group A versus 30% for group B. For group A, a complete response (CR) rates of 45.8%, 70.8%, 91.7% and a partial response (PR) rates of 54.2%, 29.2%, 8.3% were obtained at 3, 6 and 12 months after therapy. Regarding group B, a CR rate of 0%, 0%, 0% and PR rate of 16.7%, 50%, 58.3% were observed at similar period of time. Standardized rate by direct method was performed and the CR rate (74.5%) for group A was higher than for group B. The tumor FDG uptake was lower 6 months after PEEMII compared with the preoperative images. Five patients presenting with concomitant lung metastasis, had a good local response-node size reduction on postoperative CT scanning, but no response on lung nodules that were progressing. No serious adverse events were observed. A few patients had mild pain during the procedure, which resolved with peritumor injection of local anesthetic. No needle tract seeding or infection occurred. CT-guided PEEMI treatment is a simple, fast and predictable procedure that has better effectiveness on single, well circumscribed metastatic lymph node than on multiple and confluent ones. Technical improvements are expected to bring better results on large nodes that should be confirmed on larger group of patients.

Preoperative platelet count in predicting lymph node metastasis and prognosis in patients with non-small cell lung cancer.

Recent studies have shown an indirect link between platelet count and blood vessel metastasis, but this association with lymphatic vessels metastasis has not been established in NSCLC. So we investigated whether an association exists between preoperative platelet count and lymph node metastasis in NSCLC patients. Between January 2001 and January 2011, platelet counts were obtained from 883 NSCLC patients who were resistant to chemotherapy, radiotherapy, and surgery. The preoperative platelet counts, tumor metastasis, and overall survival of NSCLC patients were analyzed for correlations via statistical analysis. Upon considering patients according to their TNM lymph node metastasis stage (N0-N3), multiple comparison analyses revealed that the mean preoperative platelet count of the N0 group was significantly lower than that of the N1-N3. Analysis of variance showed that the preoperative platelet count of patients in stage I was significantly lower than that of those in stages II, III, and IV, with no significant difference among the latter three stages. According to the Kaplan-Meier survival analysis, the overall survival of patients with platelet counts <214.5 × 109/L was significantly longer than that of those with platelet counts ≥214.5 × 109/L. Cox regression analysis indicated that, besides preoperative platelet count, patient age, gender, and TNM stage were independent prognostic factors. In conclusion, preoperative platelet count was significantly associated with metastasis of lymph nodes in NSCLC patients. Preoperative platelet count may be a reliable biomarker of lymph node metastasis possibility and an independent prognostic factor of overall survival in patients with NSCLC.

Functional magnetic resonance mapping of motor cortex in patients with mass lesions near primary motor and sensory cortices.

PURPOSE: To study motor cortex mapping in patients with mass lesions near primary motor and sensory cortices with BOLD-fMRI. MATERIAL AND METHODS: 18 patients with mass lesions near primary motor and sensory cortices, and 8 healthy volunteers were investigated with fMRI using a 1.5 T GE Signa scanner. The specific task was repetitive selfpaced index finger to thumb opposition with a frequency of approximately 2 Hz. Each task paradigm consisted of twelve 20-second blocks alternating between rest and activation. All functional data was sent to SUN GE Advanced Workstation 4.0 for post processing. RESULTS: 14 patients showed functional activation near mass lesion, 4 patients failed to show, and one patient with meningeoma was excluded because of heavy head movement. Of 14 patients, the functional activation of eloquent cortex was different between the group with declination of muscle force and the group with normal muscle force, generally the activated areas of the former were more scattered, dislocated, relative smaller than that of the latter. CONCLUSION: fMRI is a valuable method for pre-operative evaluation of neurosurgical patients and probably can evaluate the muscle force pre- and post-operation approximately.