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The clinical data of one child with metanephric stromal tumor (MST) and BRAF V600E gene mutation admitted to the First Affiliated Hospital of Zhengzhou University in June 2022 was analyzed retrospectively.Literature was reviewed.The patient, a 2-year-old girl, was diagnosed with a tumor in the left abdomen.The maximum diameter of the tumor was 10.5 cm.A radical nephrectomy was performed on the left kidney, and postoperative pathology revealed MST.Microscopically, the tumor had no envelope and exhibited expansive growth.The tumor cells were fusiform or stellate, and nuclear division was visible in the cell-rich region.Dysplastic blood vessels were seen inside the tumor.The tumor cells around the blood vessels and invaginated renal tubules were arranged like onion skin.CD34 was detected positive by immunohistochemical staining, and BRAF V600E mutation was also detected positive by fluorescent polymerase chain reaction.A total of 21 relevant case reports were retrieved, including 16 in English and 5 in Chinese.Fifty-eight MST patients, including the one in this report were analyzed.These patients were aged 2 days to 15 years, with a median age of 2 years.Except for 2 patients with unknown sex, the ratio of male to female was about 1.4∶1.0.Most MST patients were asymptomatic, with an average tumor size of 5.3 cm.The tumor cell CD34 showed positive expression in different degrees.Eight patients received the BRAF V600E mutation detection, and the results were all positive.Fifty-eight patients underwent nephrectomy and were followed up for 0-156 months, of which 7 patients were assisted with radiotherapy and chemotherapy.During the follow-up, 1 patient died, and 1 patient had a relapse.MST is a rare benign renal stromal tumor. BRAF V600E mutations are detected in a variety of malignancies.This paper is the first to report MST with BRAF V600E mutation in China and points out the importance of molecular detection of BRAF mutation for accurate diagnosis of MST.
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Cryptosporidium is an important intestinal parasite that is mainly transmitted through the fecal-oral route. Human infection may occur following ingestion of water and food contaminated by Cryptosporidium oocysts, and children and immunocompromised individuals are at a high risk of infections. The main symptoms of Cryptosporidium infections include diarrhea, vomiting, malnutrition, and even death. Because of high sensitivity and rapid procedures, molecular tests are helpful for the diagnosis of cryptosporidiosis and may reduce the public health risk of cryptosporidiosis. This review summarizes the advances in the latest prevalence and molecular detection of human Cryptosporidium infections during recent years.
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Objective:To explore the prognostic values of peripheral blood cell parameters: neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR) and mean platelet volume-to-lymphocyte ratio (MPVLR) in children under the age of 4 years with infection-associated hemophagocytic syndrome (IAHS).Methods:The clinical data of 70 children under the age of 4 years with IAHS treated in the First Affiliated Hospital of Zhengzhou University from January 2015 to October 2020 were analyzed retrospectively, including the changes in peripheral blood cell parameters at the time of diagnosis, and 2, 4 and 8 weeks after treatment. Mann- Whitney U test was used to compare the differences in peripheral blood cell parameters of IAHS children with different prognosis statues.The prognostic values of NLR, PLR and MPVLR were evaluated by plotting receiver operating characteristic (ROC) curves.The optimal cut-off value was determined by Youden index.Children were grouped according to the optimal cut-off values of NLR, PLR and MPVLR.The survival rate was estimated by Kaplan-Meier method, followed by Log- rank test for pairwise comparison. Results:(1)Compared with the survival group, NLR, PLR and MPVLR were significantly higher at the diagnosis in the death group(all P<0.001). (2)ROC curves showed that the area under the curve(AUC) of NLR, PLR and MPVLR to predict the prognosis of children with IAHS were 0.805, 0.815 and 0.772, respectively.When the optimal cut-off value of NLR, PLR and MPVLR were 0.73, 32.86 and 14.19, respectively, the sensitivity and specificity were 69.57% and 89.36%, 86.96% and 68.09%, and 60.87% and 89.36%, respectively.(3)According to the optimal cut-off value, the total survival time of children in NLR>0.73 group was significantly shorter than that in NLR ≤ 0.73 group, and that of children in PLR>32.86 group was significantly shorter than that in PLR ≤ 32.86 group; the total survival time of children in MPVLR>14.19 group was significantly shorter than that in MPVLR ≤ 14.19 group(all P<0.001). (4)After 2 weeks of treatment, the NLR and MPVLR in the death group were significantly higher than those in the survival group(all P<0.05). After 2, 4 and 8 weeks of treatment, NLR, PLR and MPVLR in the survival group were significantly improved as compared with those at the diagnosis(all P<0.05), but there was no significant change in the death group(all P>0.05). Conclusions:NLR, PLR and MPVLR at the time of diagnosis have a certain predictive value for the prognosis of children under the age of 4 years with IAHS.Monitoring their changes in the early stage of treatment is helpful to judge the prognosis.
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Objective:To summarize the clinical features, treatments and prognoses of aggressive natural killer cell leukemia (ANKL) in children.Methods:Clinical data and follow-up results were retrospectively reviewed for one hospitalized case of ANKL in June 2019.Through a literature search, the relevant items were retrieved from the databases of China National Knowledge Infrastructure, WanFang and PubMed using the Chinese and English keywords of "aggressive natural killer cell leukemia" and "children" up to December 2021.Results:This 8-year-old girl was diagnosed with ANKL by flow cytometric immunophenotype and immunohistochemical stain.Fever was the initial manifestation accompanied by sallow complexion, fatigue, enlargement of liver, spleen and lymph node and hematopenia of three lines.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) was performed after chemotherapy.As of April 2022, the child stayed in a disease-free survival state after follow-ups for over 2 years.The literature search finally yielded 7 eligible Chinese and 10 English reports with a total of 17 pediatric ANKLs.In this group, there were fever (n=15), rash (n=1), perineal mass (n=1) and diarrhea, vomiting and other digestive tract symptoms (n=1). Six cases were misdiagnosed during an early stage of disease.4 cases received chemotherapy alone, 3 cases received chemotherapy regimen for acute lymphoblastic leukemia, 1 child died and one death occurred after received chemotherapy regimen of "cisplatin + vincristine + doxorubicin + ifosfamide". Allo-HSCT was performed in 5 patients after remission with chemotherapy and one child died from multiple organ failure at 9 months after allo-HSCT.Nine cases gave up treatment.Conclusions:ANKL has a rapid disease progression, diverse clinical manifestations, easy misdiagnosis and poor prognosis.For suspected ANKL cases, clinicians perform multiple bone perforations at multiple sites and immunophenotype by flow cytometry as soon as possible to confirm the diagnosis.Currently allo-HSCT offers a long-term survival of ANKL patients.
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Objective:To investigate the clinical characteristics, diagnosis and treatment and prognosis of children with leukemia secondary to malignant solid tumor.Methods:From January 2012 to January 2022, a total of 2 275 children under 15 years of age with malignant solid tumor were admitted to the First Affiliated Hospital of Zhengzhou University.Six children diagnosed with secondary leukemia after follow-up to August 1, 2022 were selected as the study objects.Their clinical data were retrospectively analyzed and the literature was reviewed.Results:(1)A total of 2 275 children with malignant solid tumors included 1 369 males and 906 females.There were 6 children with secondary leukemia, with an incidence rate of 0.26%, including 4 males and 2 females.The age of onset of solid tumor was 5.5(2.8, 9.7)years, and the age of secondary leukemia was(9.1±3.9)years, and the interval between them was(26.2±17.3)months.(2)Malignant solid tumor types: according to the time of secondary leukemia, there were hip malignant rhabdomyoma in 1 case, intracranial medulloblastoma in 2 cases, intracranial and pelvic malignant germinoma in 1 case respectively, and pancreatic blastoma in 1 case.Intracranial lesion biopsy was performed in 1 case, and tumor resection was performed in the other 5 cases.Three patients with intracranial tumors underwent local tumor bed, whole brain and spinal radiotherapy.All the 6 children received chemotherapy, and the main chemotherapy drugs were doxorubicin, vincristine, cyclophosphamide, platinum, ifosfamide, etoposide, bleomycin, temozolomide, etc.Complete remission was achieved in 3 cases, partial remission in 1 case, stable disease in 1 case, and disease progression in 1 case.(3)The secondary leukemia types were as follows: acute myeloid leukemia(AML)M5 in 3 cases, M1 in 1 case, M2 in 1 case, and acute B-lymphoblastic leukemia(B-ALL)in 1 case.All six cases refused hematopoietic stem cell transplantation(HSCT).One case with B-ALL gave up after receiving hydroxyurea and dexamethasone.Five cases with AML received chemotherapy according to the AML-2006 Protocol of Hematology Group of Pediatrics Society of Chinese Medical Association.The outcome of the disease was as follows: 2 cases died early, 4 cases achieved complete remission after 1 ~ 2 courses of chemotherapy, among which 2 cases did not continue treatment after 3 courses of chemotherapy due to pulmonary infection, deep mycosis, osteomyelitis, etc, and then recurred and died.By the end of follow-up, 2 cases survived and continued treatment, of which 1 case relapsed.After the diagnosis of secondary leukemia, the 1-year overall survival rate of the 6 cases was(33±26)%.Conclusion:Leukemia secondary to malignant solid tumors in children is rare and mostly occurs in older children.The pathogenesis is related to genotoxic injury caused by exposure to chemotherapy or radiotherapy, and the prognosis is unfavourable.HSCT after chemotherapy combined with immunotherapy is the best treatment strategy.
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Objective:To summarize and analyze the clinical characteristics, treatment and prognosis of superior vena cava syndrome (SVCS) with malignant tumors in children, and to improve the understanding of its clinical management.Methods:Clinical data of 50 children with SVCS combined with malignant tumors treated in the First Affiliated Hospital of Zhengzhou University from November 2010 to May 2022 were analyzed retrospectively.The pathological types, clinical manifestations, imaging examination, treatment and prognosis were summarized.The overall survival (OS) rate and event-free survival (EFS) rate were evaluated by Kaplan-Meier method.Results:Among the 50 cases, 38 were males and 12 were females, with a male/female ratio of 3.2∶1.0.The median onset was 12.5 (8.0, 14.5) years, and the most common onset occurred in adolescence (66.0%, 33/50). Cough (80.0%, 40/50) was the most common clinical manifestation, followed by face and neck edema (66.0%, 33/50), chest tightness (56.0%, 28/50) and dyspnea (50.0%, 25/50). All the 50 cases were confirmed by histopathological examination, 39 cases(78.0%) were diagnosed as non-Hodgkin′s lymphoma (NHL). NHL was the most common malignant tumor, of which T-lymphoblastic lymphoma (T-LBL) accounted for 74.4%(29/39). All the 50 cases were examined by CT examination, involving 42 cases (84.0%) detected with mediastinal masses.Pleural effusion (86.0%, 43/50) and pericardial effusion (70.0%, 35/50) were common imaging findings.The 3-year OS rate and EFS rate of them were 59.7% and 57.9%, respectively.The 3-year OS rate and EFS rate of the 39 children with NHL were 62.9% and 60.9%, respectively.Conclusions:Children with malignant tumors complicated with SVCS are featured by the acute onset, rapid progress and poor prognosis.NHL is the most common cause, especially T-LBL.Cough, edema of face and neck, chest tightness and dyspnea are common clinical manifestations.Early detection and treatment contribute to save children′s lives.
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Objective:To investigate the clinical efficacy, safety and compliance of Voriconazole suspension formula on the prevention and treatment of invasive fungal infection (IFI) in children with allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:Clinical data of 25 children treated Voriconazole suspension formula for the prevention and treatment of IFI during the period of allo-HSCT in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from August 1, 2020 to April 30, 2021 were retrospectively analyzed.The plasma trough concentration of Voriconazole was detected by high-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS), and the genotype of CYP2C19 was detected by polymerase chain reaction-restriction fragment length polymorphism (RFLP). The effect of CYP2C19 genotype on Voriconazole trough concentration was analyzed by rank-sum test, and Fisher′ s accurate test was used to analyze the influence of severity of gastrointestinal mucositis on serum trough concentration of Voriconazole in children with allo-HSCT. Results:A total of 25 children, including 18 males and 7 females were recruited.The median age at allo-HSCT was 6 (2-13) years.After initial administration of conventional dose of Voriconazole suspension formula during transplantation, plasma trough concentration of Voriconazole was intermittently monitored.Only 13 cases (52.0%) reached the target plasma trough concentration, 11 cases(44.0%) reached the target plasma trough concentration after adjusting the dose according to the plasma concentration, and 1 cases(4.0%) failed to reach it after increasing the dose twice.Genotype detection of CYP2C19 was performed in 20 children, involving 4 cases of poor metabolizers (PM), 9 cases of intermediate metabolizers (IM), 6 cases of extensive metabolizers (EM), and 1 case of ultra extensive metabolizer (UEM). A significant difference in plasma trough concentration was detected among all groups ( F=24.012, P<0.01). During the transplantation, 12 cases developed mild to moderate gastrointestinal mucositis, and 7 cases had severe gastrointestinal mucositis.The stan-dard rate of plasma trough concentration in children with severe gastrointestinal mucositis (1/7 cases, 14.3%)was significantly lower than those with mild to moderate gastrointestinal mucositis (9/12 cases, 75.0%) ( P=0.02). Five children (71.4%) with severe gastrointestinal mucositis could reach the target trough concentration after increasing the drug dose, suggesting that severe gastrointestinal mucositis had a great influence on the plasma concentration of Vorico-nazole suspension.The incidence of IFI in 25 children with allo-HSCT was 0, and the compliance of children taking Voriconazole dry suspension was 100.0%.The incidence of adverse reactions was 24.0% and all adverse reactions were relieved after symptomatic treatment. Conclusions:The plasma concentration of Voriconazole varies greatly among children and in different states of the same patient.Therefore, it is necessary to monitor the trough concentration of the drug and adjust the drug dose.The use of Voriconazole suspension formula for the prevention and treatment of fungal infection during allo-HSCT in children is clinically safe and effective, with a good compliance in children.
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Objective:To investigate the clinical characteristics and treatment of bronchiolitis obliterans syndrome (BOS) after hematopoietic stem cell transplantation (HSCT) in children.Methods:Clinical data of 7 patients with BOS after HSCT in the Department of Hematology and Oncology, Children′s Hospital, the First Affiliated Hospital of Zhengzhou University from September 2015 to June 2019, who had a survival of longer than 100 days were retrospectively analyzed.Results:At the last follow-up visit, the incidence of BOS was 4.6%(7/152 cases), including 5 males and 2 females.The median time from HSCT to the diagnosis of BOS was 15 (9-27) months.Among the 7 cases, 5 cases had dry cough and shortness of breath after activity, and 2 cases had no obvious clinical symptoms.Pulmonary function was moderate in 5 cases and severe in 2 cases of obstructive ventilatory disorder.High-resolution CT showed mosaic sign in 5 cases and bronchial wall thickening in 4 cases.Bronchoalveolar lavage (BAL) was performed in 4 cases, and flocculent secretion was found in the bronchus.Membranous substance was formed in the bronchus in 3 cases, and some lumens were completely occluded and dredged by foreign body forceps.After treatment with Fluticasone, Azithromycin and Montelukast sodium (FAM regimen), the pulmonary function of 5 cases(71.4%) was significantly improved, but ineffective in 2 cases.Conclusions:BOS after HSCT in children mainly begins with dry cough and shortness of breath after activity.Regular screening of pulmonary function is beneficial to identify asymptomatic children.BAL can clear inflammatory cytokines, which is conductive to the following drug treatment.If necessary, foreign forceps should be used to dredge the occluded bronchus to relieve symptoms quickly.FAM regimen is an effective treatment method, and timely adjustment of treatment according to the disease situation can improve the prognosis.
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Acute pancreatitis (AP) is one of the common acute abdominal diseases of the digestive system, and early treatment to avoid aggravation to severe pancreatitis (SAP) is the key to guaranteeing prognosis. AP with acute kidney injury (AKI) can significantly increase the mortality rate of pancreatitis. Early diagnosis of AP with AKI is a top priority to reduce mortality rate. This article reviews the current studies on the early predictors for AKI in AP and briefly describes commonly used indicators (neutrophil-to-lymphocyte ratio, cystatin C, renal vascular resistance index, and neutrophil gelatinase-associated lipocalin) and other valuable indicators. It is pointed out that a combination of various markers based on their sensitivity and specificity has a promising future in the diagnosis of AKI in AP.
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Natural medicinal chemistry is one of the important courses for students in pharmacy majors. Its experimental teaching focuses on fostering comprehensive experimental skills and innovative abilities of undergraduates. Liaoning University has explored ways to promote the experimental teaching of natural drug chemistry based on the graduate employment and practical teaching experience in the past decade. These explorations include three aspects, such as synchronizing experimental teaching with theoretical teaching, fostering students' awareness of experimental safety, and improving experimental teaching methods in natural drug chemistry experiments. The practices showed that the reform has achieved a good effect. A teaching system that can achieve the three expected aspects has been established, which improved the teaching effect and quality of natural medicinal chemistry experimental courses for undergraduates. Furthermore, these explorations may facilitate fostering pharmacy specialists who can meet the opportunities of developing Chinese medicine and natural drug research and meet the requirements of employment.
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Humanos , Química Farmacêutica/educação , Estudantes , UniversidadesRESUMO
Background@#We hypothesized that specific amino acids or acylcarnitines would have benefits for the differential diagnosis of diabetes. Thus, a targeted metabolomics for amino acids and acylcarnitines in patients with diabetes and its complications was carried out. @*Methods@#A cohort of 54 normal individuals and 156 patients with type 2 diabetes mellitus and/or diabetic complications enrolled from the First Affiliated Hospital of Jinzhou Medical University was studied. The subjects were divided into five main groups: normal individuals, impaired fasting glucose, overt diabetes, diabetic microvascular complications, and diabetic peripheral vascular disease. The technique of tandem mass spectrometry was applied to obtain the plasma metabolite profiles. Metabolomics multivariate statistics were applied for the metabolic data analysis and the differential metabolites determination. @*Results@#A total of 10 cross-comparisons within diabetes and its complications were designed to explore the differential metabolites. The results demonstrated that eight comparisons existed and yielded significant metabolic differences. A total number of 24 differential metabolites were determined from six selected comparisons, including up-regulated amino acids, down-regulated medium-chain and long-chain acylcarnitines. Altered differential metabolites provided six panels of biomarkers, which were helpful in distinguishing diabetic patients. @*Conclusion@#Our results demonstrated that the biomarker panels consisted of specific amino acids and acylcarnitines which could reflect the metabolic variations among the different stages of diabetes and might be useful for the differential diagnosis of prediabetes, overt diabetes and diabetic complications.
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Objective:To raise awareness of acute myeloid leukemia (AML) combined with lymphoblastic lymphoma (LBL) in children.Methods:The clinical characteristics, diagnostic methods, treatment plans and prognosis of a child with AML combined with LBL who was admitted to the First Affiliated Hospital of Zhengzhou University in April 2016 were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient was an 11-year-old boy with fever, abnormal hemogram and cervical lymph node enlargement as clinical manifestations. A biopsy of the cervical lymph node was performed and the patient was diagnosed as T-lymphoblastic lymphoma (T-LBL). After the bone marrow morphology, immunology, cytogenetics and molecular biology (MICM) classification examination, the diagnosis was AML. The patient met the diagnostic criteria of two diseases at the same time, and thus he was confirmed as AML combined with T-LBL. AML chemotherapy regimens were given, and the patient achieved complete remission and disease-free survived.Conclusions:AML with LBL is extremely rare in children. The diagnosis mainly depends on MICM classification examination of bone marrow and pathological examination of lymph nodes. There is currently no standard treatment scheme, and the prognosis is extremely poor. AML chemotherapy followed by bridging hematopoietic stem cell transplantation is the best treatment option for these patients.
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Objective:To investigate the mechanism of programmed death 1(PD-1)/ programmed death ligand 1(PD-L1) signaling pathway and its feasibility as a potential therapeutic target and prognostic predictor by detecting the expressions, of PD-1 and PD-L1 in bone marrow mononuclear cells of children with acute lymphoblastic leukemia (ALL), and to provide new ideas for the diagnosis and treatment of ALL as well.Methods:Bone marrow samples were collected from 59 children with ALL in the First Affiliated Hospital of Zhengzhou University from September 2018 to July 2019.Flow cytometry was applied to detect the expression of PD-1 and PD-L1 in bone marrow mononuclear cells in 59 ALL patients, including 47 newly-diagnosed ALL patients and 12 relapsed ALL patients, respectively, at initial diagnosis, after induction therapy and early intensive treatment.Their relevant clinical data were collected and compared with the bone marrow specimens of 12 children suffering from non-malignant blood diseases as the control group of the same hospital during the same period.Results:There was no significant difference in the expression of PD-1 in the bone marrow mononuclear cells of the primary diagnosis group, recurrence group and control group ( H=2.402, P>0.05). The expression of PD-L1 in the relapsed and refractory group [(7.32±3.60)%] and the newly diagnosed group [(3.18±2.37)%] was higher than that in the control group [(0.84±0.39)%], and the differences were statistically significant ( H= 28.048, P<0.05). In the initial treatment group, the expression of PD-L1 in the bone marrow mononuclear cells was the strongest expression before treatment ( B=1.293), followed by after induction treatment ( B=0.036) and after early intensive treatment ( B=0.000), suggesting that there was a downward trend as the continued treatment.The expression of PD-L1 was the weakest expression in the low-risk group ( B=-3.912) than in the medium-risk group ( B=-3.595) and high-risk group ( B=0.000), revealing that the expression of PD-L1 is related to the risk grades of ALL.The higher the risk rating is, the higher the PD-L1 protein expression is. Conclusions:The high expression of PD-L1 may be involved in the pathogenesis and be used as an adverse predictor of ALL childhood and an evaluation index of chemotherapy efficacy.PD-1 / PD-L1 signaling pathway may be a potential therapeutic target of ALL childhood.
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Objective:To investigate the clinical experience about the transconjunctival lower eyelid blepharoplasty with correction of the eyelid orbital sulcus deformity.Methods:This group received 67 cases from June 2016 to July 2018, including 3 males and 64 females. The average age was 22 years. All the cases were diagnosed as lower eyelid bag with different degrees of fat herniation after orbital septum, with no or slight lower eyelid skin relaxation. Orbital sulcus of different degree appeared with outline of bony orbital margin, or accompanied by underdevelopment of the suborbital region. Through a transconjunctival incision or with short external canthal incision, the adipose mass was fully exposed behind the anterior wall of lower eyelid, the arcuate edge was released, and the pedicled orbital fat was transferred to the gap between the orbital eyelid sulcus and fixed in the lower orbital margin. If there was insufficient fat behind the orbital septum, a retroauricular fascia graft was used to make up.Results:No postoperative complications occured in all cases. After followed up for more than 3-month, 3 of 67 cases showed mild fat bulging pouch deformity in bilateral eyelids and 1 case was in unilateral eyelid. In other cases, deformity of the pouch and the eyelid orbital sulcus were improved significantly, and dynamic expression (orbicularis muscle contraction) showed no local uplift in lower eyelid morphology.Conclusion:The transconjunctival lower eyelid blepharoplasty with correction of the eyelid orbital sulcus deformity can obtain good clinical results in lower eyelid area.
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Background@#We hypothesized that specific amino acids or acylcarnitines would have benefits for the differential diagnosis of diabetes. Thus, a targeted metabolomics for amino acids and acylcarnitines in patients with diabetes and its complications was carried out. @*Methods@#A cohort of 54 normal individuals and 156 patients with type 2 diabetes mellitus and/or diabetic complications enrolled from the First Affiliated Hospital of Jinzhou Medical University was studied. The subjects were divided into five main groups: normal individuals, impaired fasting glucose, overt diabetes, diabetic microvascular complications, and diabetic peripheral vascular disease. The technique of tandem mass spectrometry was applied to obtain the plasma metabolite profiles. Metabolomics multivariate statistics were applied for the metabolic data analysis and the differential metabolites determination. @*Results@#A total of 10 cross-comparisons within diabetes and its complications were designed to explore the differential metabolites. The results demonstrated that eight comparisons existed and yielded significant metabolic differences. A total number of 24 differential metabolites were determined from six selected comparisons, including up-regulated amino acids, down-regulated medium-chain and long-chain acylcarnitines. Altered differential metabolites provided six panels of biomarkers, which were helpful in distinguishing diabetic patients. @*Conclusion@#Our results demonstrated that the biomarker panels consisted of specific amino acids and acylcarnitines which could reflect the metabolic variations among the different stages of diabetes and might be useful for the differential diagnosis of prediabetes, overt diabetes and diabetic complications.
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Objective:To explore the clinical efficacy and safety of Ruxolitinib, a Janus kinase inhibitor, in combination with Methylprednisolone as a bridge to allogeneic hematopoietic stem cell transplantation (allo-HSCT) for relapsed/refractory Epstein-Barr virus-associated hemophagocytic syndrome (EBV-AHS) in pediatric patients.Methods:The clinical data of 4 patients with relapsed/refractory EBV-AHS treated with Ruxolitinib in combination with Methylprednisolone as a bridge to allo-HSCT at the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from August 2018 to February 2020 were retrospectively analyzed, and the disease characteristics, diagnosis and treatment process, clinical experience and related research progress were analyzed and summarized.Results:Among 4 patients with relapsed/refractory EBV-AHS, 2 patients were treated with low-dose Ruxolitinb in combination with Methylprednisolone for 6-10 weeks after partial remission.The disease did not progress, and they survived after being bridged to allo-HSCT.One patient was treated with large-dose Ruxolitinib in combination with Methylprednisolone due to the intolerance to chemotherapy, with the biochemical indicators of hemophagocytic syndrome significantly improved, and then the bridging to allo-HSCT was performed 2 months ago and this patient survived.One patient with EBV-AHS relapsed was relieved by chemotherapy again, then was given maintenance therapy with Ruxolitinib and Methylprednisolone, but the condition still progressed and the treatment was ineffective.This patient underwent allo-HSCT for salvage treatment more than 1 year ago and survived.Except that 1 patient developed mild anemia, the other 3 patients had no significant Ruxolitinib-related toxicities.Conclusions:Ruxolitinib in combination with Methylprednisolone can be safely employed as a salvage treatment for pediatric patients with relapsed/refractory EBV-AHS and a bridge to allo-HSCT, which has favorable safety, efficacy and tolerance in clinical practice.
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Objective:To investigate the clinical features and long-term prognosis of pediatric acute lymphoblastic leukemia (ALL) with renal involvement as the initial manifestation, thus enhancing the diagnostic and therapeutic efficacy.Methods:Twenty-four cases of pediatric ALL with renal involvement as the initial manifestation treated in the First Affiliated Hospital of Zhengzhou University from March 2013 to March 2019 were analyzed retrospectively, and their clinical characteristics were analyzed.According to renal imaging examination findings, they were divided into abnormal group and normal group.The differences in clinical features between the two groups were compared, and the cumulative survival rate was evaluated by Kaplan-Meier method.Results:Among 1 030 newly treated cases of pediatric ALL, 24 cases(2.33%) had renal involvement as the initial manifestation, involving 20 males and 4 females, with a male/female ratio of 5∶1 and the median age of 4.3 years (1.3-14.0 years). There were 16 cases of superficial lymph node enlargement and 21 cases of hepatosplenomegaly.Immature cells in peripheral blood were found in 15 cases.Nine cases were examined with abnormal renal imaging, involving 8 cases returned normal after chemotherapy, and 1 died of renal failure.At the end of follow-up on August 1, 2020, there were 9 cases of bone marrow relapse, 11 survival cases, 10 death cases and 3 cases of loss to follow-up.There were no significant differences in the sex, age, immunophenotype, organ infiltration and urinary protein between the two groups (all P>0.05). The proportion of high creatinine level and intramedullary recurrence rate in the abnormal group were significantly higher than those in the normal group [55.6%(5/9 cases) vs.0(0/15 cases), P=0.003; 66.7%(6/9 cases) vs.20.0%(3/15 cases), P=0.036]. The survival analysis indicated that the 3-year cumulative survival in the abnormal group was significantly lower than that of normal group (17.3% vs.72.7%, χ2=4.047, P< 0.05). Conclusions:For children with unexplained renal involvement as the initial manifestation, clinicians should consider the possibility of leukemic renal infiltration or nephrogenic lymphoma.Physical examinations of the liver, spleen and lymph nodes, morphological analysis of peripheral blood cells, bone marrow examination and renal biopsy are important to make a definite diagnosis in time.Children with imaging abnormalities caused by leukemic renal infiltration are more likely to relapse and have a lower survival rate, which may be a poor prognostic factor for ALL.
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Melanoma is a highly aggressive cancer, attracting increasing attention worldwide. The 5-year survival rate of patients with metastatic melanoma is low. Therefore, it is critical to identify potential effective biomarkers for diagnosis of melanoma metastasis. In the present study, the melanoma cohort and immune genes were obtained from the Cancer Genome Atlas (TCGA) database and the ImmPort database, respectively. Then, we constructed the immune risk score (IRS) using univariate and multivariate logistic analysis. The area under the curve (AUC) of IRS in sequencing samples and the initial diagnosis patients was 0.90 and 0.80, respectively. Besides, IRS could add benefits for metastasis diagnosis. For sequencing samples, IRS (OR = 16.35, 95% CI = 8.74-30.59) increased the odds for melanoma metastasis. Similar results were obtained in the initial diagnosis patients (OR = 8.93, 95% CI = 3.53-22.61). A composite nomogram was built based on IRS and clinical information with well-fitted calibration curves. We further used other independent melanoma cohorts from Gene Expression Omnibus (GEO) databases to confirm the reliability and validity of the IRS (AUC > 0.75, OR > 1.04, and P value < 0.01 in all cohorts). In conclusion, IRS is significantly associated with melanoma metastasis and can be a novel effective signature for predicting the metastasis risk.
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Objective:To explore the problem of repairing postoperative tissue defect of double eyelid with the retro-orbicularis oculus fat and orbital septum flap.Methods:From March 2016 to July 2018, a total of 23 female patients (18-41 years old, average 26.3 years) were treated in the Department of Plastic Surgery, Jiangsu Hospital of Traditional Chinese Medicine. They all had abnormal morphology and severe tissue deficiency in the incision area after blepharoplasty. It was even accompanied by different degrees of iatrogenic levator dysfunction, and they were all repaired by the retro-orbicularis oculus fat and orbital septum flap; if the fat of the posterior orbicularis oculi muscle and orbital septal layer was too " weak" during the operation, the layer was used as the carrier for the granular adipose tissue. Follow-up was conducted for more than 3 months after operation to evaluate the effect.Results:All incisions in this group were healed in one stage after operation, without hematoma and infection. Two cases with severe tissue deficiency and severe mechanical blepharoptosis in the pre-operation still had different degrees of ptosis after operation. Five patients had different degrees of ptosis after operation. Five patients had postoperative shape of " step" in the incision when they closed their eyes. The shape of double eyelid was improved in different degree in other beauty seekers.Conclusions:Loosing and releasing the retro-orbicularis oculus fat and orbital septum flap can effectively prevent the bad double eyelid line from adhesion, and it has a satisfactory effect for the reconstruction of the serious tissue defect after double eyelid surgery.
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Objective:To discuss whether platelet distribution width (PDW) can effectively predict the prognosis of neuroblastoma (NB).Methods:The clinical data of 67 NB patients in the First Affiliated Hospital of Zhengzhou University between January 2014 and January 2018 were retrospectively analyzed.They were divided into low PDW group and high PDW group according to the PDW level, and the differences in clinical indicators between the 2 groups were compared.The prognostic effects of PDW were assessed by using the Kaplan- Meier method and Cox regression model. Results:Among the 67 patients, 41 cases were male, 26 cases were female, with the ratio of male to female being 1.58∶1.00, and the average age was 44 months (2-156 months). Five cases were in stage Ⅰ, 1 case in stage Ⅱ, 15 cases in stage Ⅲ and 46 cases in stage Ⅳ.At the first time of diagnosis, there were 14 cases with age ≤ 18 months, 53 cases with age > 18 months, 47 cases with neuron specific enolase (NSE) level ≥ 100 μg/L, 20 cases with NSE level<100 μg/L.The median follow-up time was 20.4 months.At the end of follow-up, 35 cases died and 32 cases survived.There was no statistical difference in age, gender, primary site of tumor, tumor stage and mean platelet volume between the low PDW group and the high PDW group (all P>0.05). The proportion of high-risk patients, the level of NSE, bone marrow metastasis rate, MYCN gene amplification rate and the red blood cell distribution width in the high PDW group were significantly higher than those in the low PDW group, but the high PDW group had a lower level of thrombocytocrit than the low PDW group, and the differences were statistically significant(all P<0.05). Survival analysis revealed that the 2-year overall survival of the low PDW group was significantly higher than that of the high PDW group (69.8% vs.25.3%, χ2=15.761, P<0.05). Univariate analysis showed that NSE ( HR=6.606, 95% CI: 2.018-21.620), MYCN gene ( HR=1.977, 95% CI: 0.794-4.919), tumor risk stratification ( HR=5.926, 95% CI: 1.416-24.794), PDW ( HR=4.036, 95% CI: 1.957-8.322), and red blood cell distribution width ( HR=1.120, 95% CI: 1.005-1.249) were the adverse factors affecting the overall survival, and thrombocytocrit was a protective factor for the prognosis of NB.Multivariate analysis indicated that PDW was an independent risk factor of NB ( HR=2.524, 95% CI: 1.017-6.264, P=0.046). Conclusions:There is a good consistency between the increase of PDW and the known prognostic risk factors, elevated tumor markers and bone marrow metastasis.Increased PDW is associated with poor prognosis in NB patients, and PDW is an independent risk factor for the poor prognosis of NB.