One Case Report of A Child With CD56 Negative Blastic Plasmacytoid Dendritic Cell Neoplasm(BPDCN)Lei Ma1, Yanyan Wang1, Haiyan Ye1*, Jinshen Wang1, Yueqin Han1, Daogang Qin11. Department of Pediatrics, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China.* Corresponding Author: Haiyan Ye. Email: 366115886@qq.com.The authors hereby declare that there is no competing interest with the research.Tables: 0Figures: 0Text Word Count: 753Brief Running Title: Child BPDCN Case ReportKey Words: BPDCN; Blastic Plasmacytoid Dendritic Cell Neoplasm; CD123 +, CD4 +, CD56 - hematopoietic tumor; child; Acute lymphoblastic leukemia protocol, Chemotherapy; misdiagnose.To the editor:BPDCN is a very rare and highly invasive hematological malignancy with an extremely poor prognosis[1,2].The etiology, pathogenesis, and prognostic factors of BPDCN are unknown, and it is easily misdiagnosed. No standard treatment regimen exists[1,2]. Fewer than 100 cases of BPDCN in children have been reported worldwide[3,4,5], and the number of children with CD56-negative BPDCN is even rarer[6]. We have diagnosed 1 child with CD56 negative BPDCN and report it as follows.A six-year-old boy was admitted to the hospital with fever and pain from a mass on the left side of his neck. Physical examination showed hepatosplenomegaly. Hematologic examination revealed a white cell count of 147.31×109/L, a hemoglobin concentration of 102g/L, and a platelet count of 92×109/L. Leukocyte classification of blood smear showed 90% abnormal cells. Bone marrow morphologic analysis showed primitive cells, accounting for 91% of nuclear cells. 43 fusion genes in bone marrow were negative. Gene analysis showed high CRLF2 expression. Bone marrow chromosome examination showed 46XY. Bone marrow immunotyping before treatment was consistent with BPDCN but did not express CD56 (these tumor cells were positive for CD7, HLA-DR, CD33, CD123, CD4, CD99, CD304, and CD303, but negative for TDT, CD34, CD5, CD8, CD3, CD10, CD19, CD20, cCD79a, and CD64). The patient received chemotherapy according to the CCLG-2018-Acute lymphoblastic leukemia (ALL) regimen from March 7, 2020. After treatment (day 5 of oral prednisone), the bone marrow immunotyping test at the second testing institution showed BPDCN, but no CD56 expression. From these findings, a diagnosis of BPDCN was made. The patient completed chemotherapy more than 7 months ago, and repeated bone marrow MRD tests were negative.In 1994, Adachi et al. reported the first case of BPDCN[7] .In 2008, BPDCN was officially classified as acute myeloid leukemia (AML) and related precursor cell tumors as a separate type in WHO Classification criteria for Hematopoietic and lymphoid tissue tumors[8]. BPDCN was officially listed as an independent disease in WHO classification criteria for Hematopoietic and lymphoid tissue tumors in 2016[9].BPDCN has a very poor prognosis in elderly patients, and relatively good prognosis in children and young patients [10,11]. Most BPDCN patients have skin lesions as the first symptom, and the skin manifestations lack specificity, so it is easy to misdiagnose clinically. BPDCN may involve skin, bone marrow, lymph nodes, spleen, and other sites [11,12]. In this case, cervical lymph node enlargement was the initial presentation, accompanied by bone marrow invasion.As BPDCN is a rare disease, its pathogenesis is still unclear[1,2]. BPDCN tumor cells were derived from plasmacytoid dendritic cell (pDC) precursors[1,2,3].The diagnosis was based on histopathological and immunohistochemical markers. Tumor cells did not express myeloid, lymphocyte, or NK cell specific markers, but expressed CD4, CD56, CD123, TCL1A, and CD303, which could be used to diagnose the disease; CD123 was considered a specific marker for the diagnosis of BPDCN[3,4,5,10]. In a few rare cases, plasmacytoid dendritic cell associated antigens CD123, TCL1A or CD303 were positive, and CD4 negative or CD56 negative[6,11,12]. In this case, the bone marrow immunotyping of the two institutions expressed CD123, CD4 and CD303, but did not express CD56, and both supported BPDCN (CD56 negative). Cytogenetic abnormalities can be seen in about 60% of BPDCN patients, but there is no specific change[13,14].Currently, there is no standard treatment regimen, and high-intensity chemotherapy combined with hematopoietic stem cell transplantation (HSCT) is mostly used for treatment; new, targeted therapy and immunotherapy drugs have been successively put into clinical use[15]. The chemotherapy regimen is mainly based on chemotherapy for lymphoma, ALL and AML. Currently, most studies[16] recommend high-risk ALL-like chemotherapy for pediatric patients, and those without skin involvement have a better prognosis than those with skin involvement[17]. Pediatric HSCT is recommended for recurrent/refractory children[19,20]. The application of targeted drugs provides a new option for the treatment of BPDCN. CD123 is a specific marker on the surface of BPDCN tumor cells, and Tagraxofusp (SL401) with CD123 as a target is used for the treatment of newly treated or refractory BPDCN patients (age ≥2 years) [18]. The patient received ALL chemotherapy and the treatment course concluded more than 9 months ago.Children with CD56 negative BPDCN are extremely rare. Compared with adults, BPDCN in children is less invasive and has a better prognosis. At present, high-risk ALL chemotherapy is the first choice. Allogeneic HSCT can significantly improve the prognosis, but the risk of transplantation is higher than that of chemotherapy. More basic research and clinical trials are needed to provide more valuable references for treatment selection of CD56-negative BPDCN in children.

Complete agenesis of the permanent canines in the maxilla and the mandible is extremely rare and has only been reported twice. Studies have shown that the incidence of missing canines is more in females. This report describes a sporadic case of congenital absence of maxillary (bilateral) and mandibular (bilateral) canines.

The subfamily Typhlocybinae is a ubiquitous, highly diverse group of mostly tiny, delicate leafhoppers. The tribal classification has long been controversial and phylogenetic methods have only recently begun to test the phylogenetic status and relationships of tribes. To shed light on the evolution of Typhlocybinae, we performed phylogenetic analyses based on 28 newly sequenced and 19 previously sequenced mitochondrial genomes representing all currently recognized tribes. The results support the monophyly of the subfamily and its sister group relationship to Mileewinae. The tribe Zyginellini is polyphyletic with some included genera derived independently within Typhlocybini. Ancestral character state reconstruction suggests that some morphological characters traditionally considered important for diagnosing tribes (presence/absence of ocelli, development of hind wing submarginal vein) are homoplastic. Divergence time estimates indicate that the subfamily arose during the Middle Cretaceous and that the extant tribes arose during the Late Cretaceous. Phylogenetic results support establishment of a new genus, Subtilissimia Yan & Yang gen. nov., with two new species, Subtilissimia fulva Yan & Yang sp. nov. and Subtilissimia pellicula Yan & Yang sp. nov. (Typhlocybinae: Typhlocybini); but indicate that two previously recognized species of Farynala distinguished only by the direction of curvature of the processes of the aedeagus are synonyms, i.e., Farynala dextra Yan & Yang, 2017 equals Farynala sinistra Yan & Yang, 2017 syn. nov. A key to tribes of Typhlocybinae is provided.

A female of 60 years old who suffered from 2-month intermittent upper abdominal pains, was adopted by the hospital. She once went through radical gastrectomy (BillrothⅡ) oriented with gastric antral cancer. CE-CT and abdominal ultrasound displayed a primary tumor on the neck of pancreas. Pathological examination exhibited that the lesion is a Pancreatic Ductal Adenocarcinoma. According to the results of the imaging, the open approach radiofrequency ablation therapy was selected to be the treatment of the primary tumor. A local recurrent tumor was discovered in the follow-up CE-CT after 8 months with the performance of another open RFA. In spite of the evidence that RFA for the recurrence of other cancers like hepatocellular carcinoma might lengthen the survival of the patients, whether repeated RFA of local recurrence of pancreatic cancer is feasible is still unclear. The patient still enjoys her 9-year existence following the first RFA. It is promising that some cases still reveal the attainment of long-term survival based on repeated open RFA.

Background and purpose: Postoperative pain occurs in as many as 70% of the over 230 million surgeries performed annually worldwide. Postoperative pain management still relies on opioids despite their negative consequences, resulting in a public health crisis. Therefore, it is of utmost importance to develop alternative therapies to treat chronic pain. Natural products derived from medicinal plants are potential sources of novel and are potential sources biologically active compounds for development of novel analgesics safe analgesics. Experimental approach: Hence, in this study, we screened a library of natural products to identify small molecules that target the activity of voltage-gated sodium and calcium channels due to their important roles in nociceptive sensory processing. Key Results: We found that fractions derived from the Native American medicinal plant, Parthenium incanum, inhibited depolarization-evoked calcium influx in rat dorsal root ganglion (DRG) neurons. Further separation of these fractions yielded a cycloartane-type triterpene identified as argentatin C which blocked the activity of both voltage-gated sodium and calcium channels in calcium imaging assays. Docking analysis predicted that argentatin C may bind to NaV1.7-1.9 and CaV3.1-3.3 channels. Furthermore, voltage and current clamp electrophysiology experiments showed that argentatin C decreased Na+ and T-type Ca2+ currents as well as excitability in rat and macaque DRG neurons. Consistent with these observations, argentatin C treatment reversed mechanical allodynia in a mouse model of postsurgical pain. Conclusions & Implications: The dual effect of argentatin C on voltage-gated sodium and calcium channels supports its potential as a novel treatment for painful conditions.

Introduction Anomalous aortic origin of coronary artery (AAOCA) is the second leading cause of sudden cardiac death in children and young adults. Intramural-interarterial course is the most frequent anatomic variation and coronary unroofing is widest adopted for surgical management. Symptoms recurrence is described regardless of the technique used. This study aims to describe how an anatomic patient-centered approach aimed to restore a normal coronary artery take-off is associated with symptoms resolution. Methods From 2008 to 2021, 25 patients were operated on for an AAOCA at a median age of 20 years. Nineteen patients had a right AAOCA and six had left AAOCA. Intramural course was present in 18 patients. Seventy-six percent were symptomatic. No episodes of aborted sudden cardiac death before surgery was described in the population. Surgical technique used were coronary unroofing in 18 patients, coronary neo-ostioplasty in 3, coronary re-implantation in 3 and main pulmonary artery re-location in one. Results No hospital mortality or re-operation was observed in our experience as well as major complications related to surgery. Mean hospital length of stay was 8.5 days. None of patients reported symptoms recurrence at follow-up. Young athletes returned to play competitive sport. Postoperative computed-tomography scan evaluation showed a general improvement of the take-off angle. Conclusions AAOCA requires a patient anatomic-based surgical management. There is not a single surgical technique that can fits all anatomic subtype of AAOCA. Surgical techniques may be selected on the base of the preoperative images and intraoperative findings. In our experience this policy is associated with no symptoms recurrence.

Background and Aim: To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re-interventions, for the rare instances of transposition physiology with concordant ventriculo-arterial connections. Methods: We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo-arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results: Among reported cases, just over two-thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four-fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one-fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multi-staged. Overall mortality was 25%, with one-fifth of patients requiring pacemakers for surgically-induced heart block. The majority of survivors were in good functional state. Conclusions: The flow patterns produced by discordant atrioventricular and concordant ventriculo-arterial connections remain an important, albeit rare, indication for atrial redirection. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long-term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.

A document by Cheima Khennaoui. Click on the document to view its contents.

Chemical engineering is a broad field in terms of the scope of practice but the discipline has been united by a few intellectually coherent principles. Among them, thermodynamics, reaction kinetics and transport phenomena are often considered as the cornerstones, providing support for design and operation of diverse chemical processes for power generation and production of numerous industrial goods including plastics, gasoline and ammonia. Traditionally, these processes use fossil fuels as the raw material and/or energy input and thus are responsible for massive greenhouse gas emissions. As fossil-energy-based processes are deemed phasing out due to environmental concerns, development of alternative routes with renewable energy and sustainable feedstock is calling for the expansion of the knowledge base such that eco-friendly processes can be quantified, controlled and optimized with high precision. This article presents some perspectives on possible engineering developments to accelerate the ongoing paradigm shift from fossil fuels to renewable energy.

The fluid mixing plays a crucial role in continuous flow chemical process. To enhance the mixing efficiency, a novel mixer was designed and constructed by adding ellipsoidal baffle in large scale Y-shaped mixer. The mixing performance in the mixers were studied by computational fluid dynamics (CFD) simulations and experimentally verified by the Villermaux/Dushman method. Furthermore, the mixer structure was optimized, and the flow characteristics of the fluid in the optimal mixer were investigated. The results indicated that the mixer can improve the mixing performance and reduce the pressure drop on the premise of ensuring high throughput by the secondary flow disturbance of ellipsoidal baffle. All the findings reported provide useful references for enhancement of mixing efficiency and reduction of pressure drop in a large scale mixer.

The dynamics and breakup of bubbles in swirl-venturi bubble generator (SVBG) are explored in this work. The three-dimensional movement process and breakup phenomena of bubbles are captured by one high-speed camera system with two cameras while the distribution of swirling flow field are recorded through Particle Image Velocimetry technology. It is revealed that bubbles have two motion trajectories, which are deeply related to bubble breakup. One trajectory is that mother bubble moves upward in an axial direction of the SVBG to the diverging section, and the other trajectory is that mother bubble rotates obliquely upward to another side-wall along the radial direction. Meanwhile, binary breakup, shear-off-induced breakup, static erosive breakup and dynamic erosive breakup are observed. For relatively high liquid Reynolds number, vortex flow regions are extended and the bubble size is reduced. Furthermore, it is worth noting that the number of microbubbles increases significantly for intensive swirling flow.

OBJECTIVE Assess whether antenatal corticosteroids for fetal lung maturation are associated with hypoglycaemia in neonates born at term. DESIGN Cohort study of term singleton deliveries over a 3-year period. SETTING Tertiary UK hospital. POPULATION The cohort includes neonates not exposed to corticosteroids; those exposed before 34 weeks because of suspected preterm birth but delivered at term (group 1); those exposed after 34 weeks because of anticipated late preterm birth (group 2); and - included in the latter - a subgroup of neonates exposed within 7 days of their actual delivery (group 2a). METHODS Retrospective analysis of the association between exposure and neonatal outcomes using multivariate regression to adjust for confounders. MAIN OUTCOME MEASURES Severe neonatal hypoglycaemia requiring admission to NNU; and need for ventilatory support. RESULTS Amongst 20102 eligible pregnancies, 143 women received corticosteroids before 34 weeks; and 187 after 34 weeks, of which 106 were within 7 days of delivery. Severe hypoglycaemia occurred in 227 neonates. Univariate predictors of hypoglycaemia were maternal BMI, nulliparity, hypertension, diabetes, gestation at birth, birthweight<10 centile and corticosteroid exposure. Following adjustment for covariates, corticosteroid exposure was independently associated with hypoglycaemia in all exposed groups: group 1 adjusted odds ratio (aOR) 3.26 (1.38-7.73); group 2 aOR 4.56 (2.47-8.42); and group 2a aOR 5.70 (2.49-13.03). Ventilatory support was not significantly different in any of the exposed groups. CONCLUSION There is increased risk of hypoglycaemia in neonates exposed to antenatal corticosteroids who are born at term. The risk of hypoglycaemia is higher with decreasing corticosteroid-to-birth interval.

Background: Childhood cancer survivors are 8.8 times more likely to die of pulmonary causes when compared to general population: an aspect of concern. Pulmonary dysfunction is the third leading cause of non recurrence related cause of death among Hodgkin lymphoma survivors. Methods: A cross section study on Hodgkin lymphoma survivors in complete remission, who completed treatment within last 5 years was done. All children were subjected to detail history including drugs, past history of respiratory illnesses, physical and respiratory system examination followed by spirometry and three minute step test under supervision. Pulmonary dysfunction was determined as presence of obstructive, restrictive or mixed pattern on spirometry or abnormality in three minute step test. Subclinical pulmonary dysfunction was determined as patients who were clinically asymptomatic but had pulmonary dysfunction Results: A total of 60 children were enrolled (Mean age of 11.3 years and 53 were boys) Abnormal pulmonary function tests were documented in 11 (18.3%) of HL survivors at a median time of 2 years (IQR 1,3) from treatment completion. Restrictive pattern was documented in 10 (16.67%) and obstructive pattern in only one patient (1.67%), mostly mild in severity. Older age at start of chemotherapy and radiotherapy and past history of respiratory illness were found to be significantly associated with pulmonary dysfunction. Conclusion: Majority of Hodgkin lymphoma survivors had subclinical pulmonary dysfunction at median follow up of 2 years from treatment completion. Hodgkin lymphoma survivors require long term follow up for timely detection of pulmonary dysfunction and improve quality of life.

Objective In contrast to the well-recognized benefit of trans-obturator tape (TOT) to urine continence, if the TOT-enhanced urethra resistance results in modified voiding functions in stress urinary incontinence (SUI) patients is not adequately investigated. Design and setting Urodynamic investigations were retrograde analyzed in a tertiary referral hospital. Population A total of 26 female SUI patients Methods The area enclosed by the loop trajectory (Apv) in a pressure-volume analysis (PVA) which represent the work performed in each voiding cycle was retrospectively analyzed Main outcome measures The Apv of female SUI patients before and after the TOT were analyzed. Results When compared with the pre-operative control, the Apv was significantly increased post-operatively. The TOT-enhanced Apv was accompanied by increments in the mean urethra resistance (Rv) and mean voiding pressure (Pv) but unaffected voided volume (Vv) and voiding time (Tv). Moreover, the TOT-induced Apv increase (ΔApv) displayed moderate correlations with the changes in Rv and Pv (ΔRv and ΔPv, respectively) but no correlation with the changes in Vv or Tv (ΔVv and ΔTv, respectively). Notably, the TOT-induced ΔRv displayed a strong correlation with the ΔPv. Conclusions: The thermodynamic efficacy of the bladder was enhanced after the TOT; that could be attributed to that bladder develops an elevated pressure gradient in response to the TOT-enhanced outlet resistance during urine emission. Keywords: mid-urethra sling, trans-obturator tape, stress urinary incontinence, urethra resistance, thermodynamics, pressure-volume analysis Tweetable abstract The bladder developed an elevated pressure gradient to the TOT-enhanced outlet resistance; and herein enhanced the voiding efficacy.

Objective: To investigate whether an association exists between deceleration and acceleration areas on continuous fetal cardiotocography (CTG) and neonatal encephalopathy (NE). Design: A retrospective case-control study. Setting: A single tertiary medical center with over 15,000 births a year. Population: All deliveries complicated by neonatal encephalopathy in our center during the study period, with two controls for every case, matched by gestational age and cord blood pH. Methods: We compared CTG characteristics of low-risk pregnancies (35 weeks gestation or more), complicated by moderate to severe NE with matched controls. We analyzed the intrapartum CTG recordings by calculation of the deceleration and acceleration areas. Main outcome measure: Deceleration and acceleration areas and the ratio between the two. Results: During the period between 2013 and 2019, we identified 95 cases of low-risk pregnancies that were complicated by moderate to severe NE in our center. Thirty-three (34.7%) deliveries were excluded, mostly due to an insufficient duration of the CTG recordings. The remaining 62 cases were matched with 123 controls. We found that NE was significantly associated with an increased total deceleration area, a decreased total acceleration area and a lower acceleration-to-deceleration ratio. Conclusions: In our population, NE was significantly associated with increased total deceleration area, decreased total acceleration area and a lower acceleration-to-deceleration ratio, irrespective of cord blood pH. Development of a computerized real-time analysis of fetal heart rate tracings may contribute to making these measurements a more valid clinical tool.

Copepods provide a rich organic microenvironment allowing the settlement and proliferation of microorganisms, forming dynamic microbial hotspots in the oceans. Such symbiotic associations in the plankton were previously hypothesized to be especially developed in warm oligotrophic seas, as they have a potential role in enhancing nutrient availability in biologically-poor waters. Aiming to better understand how copepod microbiomes are shaped in an oligotrophic sea, we characterized microbiota associated with three dominant coastal epipelagic copepod species in the ultra-oligotrophic Eastern Mediterranean Sea using amplicon sequencing of the 16S rRNA gene. Our results show that copepod-associated microbial communities were host-specific rather than determined by seasonal environmental changes. In the filter-feeding copepod with a tendency to herbivory, Temora stylifera, microbial diversity was low and relatively stable throughout the year. In contrast, omnivorous copepods, the ambush-feeding Oithona nana and the mixed-feeding Centropages ponticus, harbored more diverse microbiomes dominated by transient taxa. We suggest that filter-feeding strategy and narrow food spectrum can limit copepod-microbe interactions, while the ambush and mixed feeding strategies combined with omnivory confer higher microbial diversity. Filter feeders may reduce the recruitment of opportunistic microbes by maintaining high fidelity associations, as indicated by the large number of core taxa in T. stylifera. We underline the importance of the copepod-microbe associations in nutrient-impoverished ecosystems, based on predicted enrichment of nitrogen metabolism in the core microbiome, mostly during summer when the shallow coastal waters are nitrogen-depleted.

We present a case of immunocompetent boy with brain abscess (BA) complicating hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD), which is rarely reported. During the recovery period of BA, he had another fever accompanied by enlarged lymph nodes, eye conjunctival congestion, strawberry tongue, swelling on hands and feet, and exanthema. Simultaneously, further investigations revealed bicytopenia, liver dysfunction, hyperferritinemia, coagulopathy and hemophagocytosis. The patient was treated with antibiotics, intravenous immunoglobulin (IVIG) and steroid that led to rapid resolution. Our report highlights the importance of early detection and treatment of HLH, and possible resolution without using immunosuppressive therapy in infectious patients.

The kinetics of the Beckmann rearrangement of cyclohexanone oxime (CHO) catalyzed by trifluoroacetic anhydride (TFAA) and trifluoroacetic acid (TFA) is proposed in this work. The effects of experimental parameters on the reaction rate are studied in microreactors, including CHO concentration, TFAA concentration, temperature, and caprolactam (CPL) concentration. The reaction is verified as first-order and the reaction rate shows a linear relationship with TFAA concentration and decreases with the increasing of CPL concentration. The in-situ FTIR is adopted to study the reaction mechanism and quantitative equilibrium relationship of CPL with TFAA. Based on the experimental results, a reaction mechanism is proposed and a kinetics model is established, which is in good agreement with the experimental data. The TFAA/TFA catalytic system allows high conversion and reaction rate compared with other organic acids.

The hydrodynamic performance of the three mixers (single shaft central mixer, single shaft off-centred mixer, dual shaft off-centred mixer, i.e. SSC, SSO, DSO), was investigated in the mixing of yield-pseudoplastic fluids (xanthan gum solutions) in the laminar regime. Computational Fluid Dynamics was employed to simulation the apparent viscosity distribution, mixing time and the flow pattern in the stirred tank. The influence mechanism of the rotational speed and fluid rheology on the cavern evolution was explored deeply. The results verified that the faster the rotating speed, the greater influence of the fluid rheology was obtained on the cavern evolution. Moreover, the Nt95 of DSO was nearly 42.8% and 6.1% shorter than that of SSC and SSO at the same Re*, respectively. These data also revealed that the DSO was more efficient than SSC and SSO in mixing of yield pseudoplastic fluids with regard to the mixing time and power drawn.

Imerslund-Grasbeck syndrome is a rare autosomal recessive disease mainly characterized by megaloblastic anemia and proteinuria. Herein, we reported two cases presenting with anemia and growth retardation. Especially, case 1 manifested proteinuria and case 2 was diagnosed with α-thalassemia. The whole gene sequencing confirmed that they harbored the same homozygous mutation in the AMN gene (c.C742T). Both of them showed a good hematological and neurological improvement to intramuscular injection of cobalamin. However, proteinuria still existed for case 1 and was unknown for case 2.

A 27 year old man with an history of persistent hyperthyroidism, had been referred to surgeon for total thyroidectomy, but the procedure was delayed during the COVID-19 pandemic. He developed a nonpitting oedema on the pretibial region of both legs leading to diagnosis of thyroid dermopathy.

Objective This study aimed to analyse the efficacy of autologous peripheral blood stem cell transplantation for high-risk neuroblastoma in China. Methods The data of 90 high-risk neuroblastoma patients treated with the CCCG-NB 2015 regimen at the Tianjin Medical University Cancer Institute & Hospital were reviewed. The baseline clinicopathological characteristics and prognosis of were analysed and compared. In addition, the prognoses of tandem autologous stem cell transplantation and single autologous stem cell transplantation groups were compared. Results The results of survival analysis showed that autologous peripheral blood stem cell transplantation based on this pretreatment regimen significantly improved the prognosis of children in the high-risk group. The 3-year event-free survival (EFS) and overall survival (OS) rates for the transplantation group and the nontransplantation group were 65.5% vs. 41.3% (P=0.023) and 77.1% vs. 57.9% (P=0.03), respectively. There was no difference in the distribution of baseline clinical case characteristics between the single transplantation group and the tandem transplantation group (P>0.05), and there was no significant difference in EFS and OS between the two groups (P>0.05). Conclusion Based on this pretreatment programme, autologous peripheral blood stem cell transplantation is safe and tolerable and significantly improves the prognosis of children in the high-risk group. The value of tandem autologous stem cell transplantation is worthy of further discussion, which should consider various aspects such as the transplantation plan and the patient’s state.

Considering the difficulty in the treatment of cerebrospinal fluid leakage （CSFL） caused by intrathecal catheterization, we report here a case of successful treatment of CSFL after Intrathecal drug delivery system implantation with an epidural blood patch combined with fibrin glue.

Pseudoachalasia is an uncommon disorder, mainly caused by malignancy. To our knowledge, no cases of a signet-ring cell carcinoma of the non-ampullary duodenum revealed by pseudoachalasia were previously reported. Early identification of malignancy-associated pseudoachalasia is crucial to avoid delaying adequate treatment.