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Total estimated effective doses from radiologic imaging modalities of children with cancer:a single center experience

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World Journal of pediatrics 2017年第3期13卷 242-247页

作者： Derya Ozyorük Suna Emir HacιAhmet Demir Gülsah Bayram Kabacam Bahattin Tunc division of pediatric oncology Ankara Children's Hematology and Oncology Education and Research HospitalAnkaraTurkey division of Radiology Ankara Children's Hematology and Oncology Education and Research HospitalAnkaraTurkey

Background:Recently,awareness of the cumulative radiation exposure for pediatric oncology patients has been increasing,together with increased survival rates and longer life *** aim of our study was to quantify the amount of ionising radiation from imaging modalities of pediatric oncology ***:Eighty-eight patients who were diagnosed with childhood cancer and followed up for 5 years between 2004-2014 in our center were included in the ***' medical files were reviewed retrospectively for imaging history in the first 5 years after *** estimated effective doses from radiologic imaging modalities were ***,the basic demographic data,histologic type,stage,and outcomes of disease were collected for all ***:The individual total estimated effective doses ranged from 8.73 to 167 mSv,with a median of 62.92 *** tomography was the greatest contributor of total effective *** doses ranged 21.45-113.20 mSv (median:62.92 mSv) in Hodgkin lymphoma,12.53-167.10 mSv (median:52 mSv) in non-Hodgkin lymphoma,4.13-172.98 mSv (median:52 mSv) in neuroblastoma,31-149.89 mSv (median:63.10 mSv) in Wilms' tumor,11.50-73.72 mSv (median:36.90 mSv) in germ cell tumor,26.46-125.86 mSv (median:80.90 mSv) in other solid tumor and 0.02-13.31 mSv (5.25 mSv) in brain tumor ***-two children (25%) died with progressive disease during the 5-year follow-up ***:Similar to previous studies,the total estimated effective doses in children with cancer have been found various according to diagnosis,stage and clinical *** clarify the harmfull effects of radiation burden,prospective studies should be conducted in children with cancer.

关键词： children effective dose ionizing radiation

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Deciphering the role of hedgehog signaling in pancreatic cancer

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The Journal of Biomedical Research 2016年第5期30卷 353-360页

作者： Dongsheng Gu Kelly E Schlotman Jingwu Xie Wells Center for pediatric Research Division of Hematology and OncologyDepartment of PediatricsIndiana University Simon Cancer CenterIndiana University

Pancreatic cancer, mostly pancreatic ductal adenocarcinoma （PDAC）, is a leading cause of cancer-related death in the US, with a dismal median survival of 6 months. Thus, there is an urgent unmet need to identify ways to diagnose and to treat this deadly cancer. Although a number of genetic changes have been identified in pancreatic cancer, their mechanisms of action in tumor development, progression and metastasis are not completely understood. Hedgehog signaling, which plays a major role in embryonic development and stem cell regulation, is known to be activated in pancreatic cancer; however, specific inhibitors targeting the smoothened molecule failed to improve the condition of pancreatic cancer patients in clinical trials. Furthermore, results regarding the role of Hh signaling in pancreatic cancer are controversial with some reporting tumor promoting activities whereas others tumor suppressive actions. In this review, we will summarize what we know about hedgehog signaling in pancreatic cancer, and try to explain the contradicting roles of hedgehog signaling as well as the reason（s） behind the failed clinical trials. In addition to the canonical hedgehog signaling, we will also discuss several non-canonical hedgehog signaling mechanisms.

关键词： hedgehog pancreatic cancer Glil non-canonical signaling cancer metastasis

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Inflammasome links traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease

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Neural Regeneration Research 2025年第6期20卷 1644-1664页

作者： Gabriela Seplovich Yazan Bouchi Juan Pablo de Rivero Vaccari Jennifer C.Munoz Pareja Andrew Reisner Laura Blackwell Yehia Mechref Kevin K.Wang J.Adrian Tyndall Binu Tharakan Firas Kobeissy Department of Surgery Morehouse School of MedicineAtlantaGAUSA Department of neurobiology Center for NeurotraumaMultiomics&Biomarkers(CNMB)Neuroscience InstituteMorehouse School of MedicineAtlantaGAUSA Department of neurological Surgery and the Miami Project to Cure Paralysis University of Miami Miller School of MedicineMiamiFLUSA division of pediatric Critical Care Department of PediatricsUniversity of Miami Miller School of MedicineMiamiFLUSA Department of pediatrics Emory UniversityAtlantaGAUSA Department of neurosurgery Children’s Healthcare of AtlantaAtlantaGAUSA Department of Chemistry and Biochemistry Texas Tech UniversityLubbockTXUSA Morehouse School of Medicine AtlantaGAUSA

Traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease are three distinct neurological disorders that share common pathophysiological mechanisms involving neuroinflammation. One sequela of neuroinflammation includes the pathologic hyperphosphorylation of tau protein, an endogenous microtubule-associated protein that protects the integrity of neuronal cytoskeletons. Tau hyperphosphorylation results in protein misfolding and subsequent accumulation of tau tangles forming neurotoxic aggregates. These misfolded proteins are characteristic of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease and can lead to downstream neuroinflammatory processes, including assembly and activation of the inflammasome complex. Inflammasomes refer to a family of multimeric protein units that, upon activation, release a cascade of signaling molecules resulting in caspase-induced cell death and inflammation mediated by the release of interleukin-1β cytokine. One specific inflammasome, the NOD-like receptor protein 3, has been proposed to be a key regulator of tau phosphorylation where it has been shown that prolonged NOD-like receptor protein 3 activation acts as a causal factor in pathological tau accumulation and spreading. This review begins by describing the epidemiology and pathophysiology of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease. Next, we highlight neuroinflammation as an overriding theme and discuss the role of the NOD-like receptor protein 3 inflammasome in the formation of tau deposits and how such tauopathic entities spread throughout the brain. We then propose a novel framework linking traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease as inflammasomedependent pathologies that exist along a temporal continuum. Finally, we discuss potential therapeutic targets that may intercept this pathway and ultimately minimize long-term neurological decline.

关键词： Alzheimer's disease caspase-1 chronic traumatic encephalopathy inflammasomes neurodegeneration neuroinflammation NLRP1 NLRP3 pyroptosis tauopathy traumatic brain injury

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Recurrent lymphoma presenting as painless, chronic intussusception: A case report

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World Journal of Clinical Cases 2020年第2期8卷 306-312页

作者： Parker Giroux Anderson Collier Michael Nowicki Department of pediatrics University of Mississippi Medical CenterJacksonMS 39216United States division of pediatric Hematology and oncology University of Mississippi Medical CenterJacksonMS 39216United States division of pediatric Gastroenterology University of Mississippi Medical CenterJacksonMS 39216United States

BACKGROUND The clinical presentation of acute lymphoblastic lymphoma is highly *** prognosis is good, recurrence of disease can occur. Gastrointestinal relapse, including intussusception, is well-described but the absence of abdominal pain in this setting is *** SUMMARY We report a 13-year-old male with B-cell precursor acute lymphoblastic leukemia in remission presenting with anemia and weight loss. Examination was significant for absence of abdominal pain, but a stool sample was positive for occult blood. Pan-endoscopy was performed with colonoscopy revealing a mass filling the colonic lumen. Biopsy of the mass confirmed recurrence of recurrent Bcell lymphoma. Computed tomography scan revealed ileocolic intussusception resulting from the tumor. This case is unusual in that the patient had no abdominal pain despite the presence of *** While intestinal involvement with lymphoma has been well described in the literature, presentation as painless intussusception has not been reported. This case report highlights the wide spectrum of clinical manifestations of recurrent Bcell lymphoma involving the gastrointestinal tract, in particular the near absence of symptoms despite the finding of intussusception.

关键词： B-cell acute lymphoblastic leukemia Tumor relapse Intussusception Case report Anemia

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Clinical implications of hedgehog signaling pathway inhibitors

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Chinese Journal of Cancer 2011年第1期30卷 13-26页

作者： Hailan Liu Dongsheng Gu Jingwu Xie Wells Center for pediatric Research Division of Hematology and Oncology Department of Pediatrics Indiana University Simon Cancer Center Indiana University Indianapolis Indiana 46202 USA.

Hedgehog was first described in Drosophila melanogaster by the Nobel laureates Eric Wieschaus and Christiane Nüsslein-Volhard. The hedgehog (Hh) pathway is a major regulator of cell differentiation, proliferation, tissue polarity, stem cell maintenance, and carcinogenesis. The first link of Hh signaling to cancer was established through studies of a rare familial disease, Gorlin syndrome, in 1996. Follow-up studies revealed activation of this pathway in basal cell carcinoma, medulloblastoma and, leukemia as well as in gastrointestinal, lung, ovarian, breast, and prostate cancer. Targeted inhibition of Hh signaling is now believed to be effective in the treatment and prevention of human cancer. The discovery and synthesis of specific inhibitors for this pathway are even more exciting. In this review, we summarize major advances in the understanding of Hh signaling pathway activation in human cancer, mouse models for studying Hh- mediated carcinogenesis, the roles of Hh signaling in tumor development and metastasis, antagonists for Hh signaling and their clinical implications.

关键词： Hedgehog 信号通路临床意义抑制剂癌症治疗细胞分化前列腺癌预防效果

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Fecal microbiota transplantation in allergic diseases

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World Journal of Methodology 2025年第2期15卷 83-91页

作者： Ece TüsüzÖnata ÖnerÖzdemir division of pediatric Allergy and Immunology Medical FacultySakarya UniversityAdapazarı54100SakaryaTürkiye

Microorganisms such as bacteria,fungi,viruses,parasites living in the human intestine constitute the human intestinal *** refers to composi-tional and quantitative changes that negatively affect healthy gut *** recent years,with the demonstration that many diseases are associated with dysbiosis,treatment strategies targeting the correction of dysbiosis in the treat-ment of these diseases have begun to be *** microbiota trans-plantation(FMT)is the process of transferring faeces from a healthy donor to another recipient in order to restore the gut microbiota and provide a therapeutic *** studies have gained popularity after probiotic,prebiotic,symbiotic studies in the treatment of dysbiosis and related *** has emerged as a potential new therapy in the treatment of allergic diseases as it is associated with the maintenance of intestinal microbiota and immunological balance(T helper 1/T helper 2 cells)and thus suppression of allergic *** this article,the definition,application,safety and use of FMT in allergic diseases will be discussed with current data.

关键词： Microbiota Dysbiosis Faecal microbiota transplantation Allergic diseases

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Etiology and long-term outcome of extrahepatic portal vein obstruction in children

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World Journal of Gastroenterology 2010年第39期16卷 4968-4972页

作者： Batia Weiss Eyal Shteyer Asaf Vivante Drora Berkowitz Shimon Reif Zvi Weizman Yoram Bujanover Rivka Shapiro division of pediatric Gastroenterology Hepatology and NutritionEdmond and Lily Safra Children's Hospital Sackler Faculty of Medicine Tel-Aviv University division of pediatric Gastroenterology Hepatology and NutritionHadassah Medical CenterThe Hebrew University School of Medicine division of pediatric Gastroenterology Hepatology and NutritionMeyer Children's HospitalRambam Medical Center Rappaport School of Medicine TechnionIsrael Institute of Technology division of pediatric Gastroenterology Hepatology and NutritionDana Children's HospitalSouraski Medical Center division of pediatric Gastroenterology Hepatology and NutritionSoroka Medical Center Faculty of Health Sciences Ben-Gurion University of the Negev Department of pediatric Gastroenterology and Nutrition Division of Pediatric TransplantationSchneider Children's Hospital

AIM:To study the management and outcome of children with extrahepatic portal vein obstruction(EHPVO) in a whole country ***:A nationwide multicenter retrospective case series of children with EHPVO was *** on demographics,radiographic studies,laboratory workup,endoscopic and surgical procedures,growth and development,were extracted from the patients' *** of clinical presentation,etiology of EHPVO,management and outcome were ***:Thirty patients,13 males and 17 females,19(63.3%) Israeli and 11(36.7%) Palestinians,were included in the *** at presentation was 4.8± 4.6 years,and mean follow-up was 4.9±4.3 *** anomalies were found in 4 *** incidence of EHPVO in Israeli children aged 0-14 years was 0.72/*** factors for EHPVO were detected in 13(43.3%)patients,including 9 patients(30%) with perinatal risk factors,and 4 patients(13.3%) with prothrombotic states:two had low levels of protein S and C,one had lupus anticoagulant,and one was homozygous for methyltetrahydrofolate reductase *** 56.6% of patients,no predisposing factors were *** most common presenting symptoms were an incidental finding of splenomegaly(43.3%),and upper gastrointestinal bleeding(40%).No differences were found between Israeli and Palestinian children with regard to age at presentation,etiology and clinical *** occurred in 18 patients(60%),at a median age of 3 *** or esophageal banding was performed in 20 *** sclerotherapy complications were *** shunts were performed in 11 patients(36.6%),at a median age of 11(range 3-17)years:splenorenal in 9,mesocaval in 1,and a meso-Rex shunt in 1 *** patient underwent splenectomy due to severe *** were followed up for a median of 3(range 0.5-15)*** patient died aged 3 years due to mucopolysaccharidase deficiency type Ⅲ.None of the patients died due to gastrointestin

关键词： Children Extrahepatic Obstruction Outcome Portal Vein

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Age-adjusted NT-proBNP could help in the early identification and follow-up of children at risk for severe multisystem inflammatory syndrome associated with COVID-19(MIS-C)

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World Journal of Clinical Cases 2022年第29期10卷 10435-10450页

作者： Moises Rodriguez-Gonzalez Ana Castellano-Martinez division of pediatric Cardiology Puerta del Mar University HospitalCadiz 11009Spain division of pediatric Nephrology Puerta del Mar University HospitalCadiz 11009Spain

BACKGROUND Multisystem inflammatory syndrome in children(MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular *** To determine whether the age-adjusted N-terminal pro-brain natriuretic peptide(NT-proBNP) value(Z-log-NT-proBNP) is associated with severe MIS-C and myocardial *** A retrospective study was conducted which included children with MIS-C managed at our institution between April 1,2020,and February 28,*** divided the population into groups depending on severity based on pediatric intensive care unit(PICU) *** compared Z-log-NT-proBNP values across these groups and analyzed Z-log-NT-proBNP dynamics during the onemonth *** We included 17 participants [median age 3(2-9) years] and seven(41%) required PICU ***(100%) of these cases presented very high(Z-log > 4) levels of NT-proBNP at the time of admission compared to only 5(50%) patients with non-severe MIS-C(P = 0.025).NT-proBNP was significantly correlated with highsensitive Troponin I levels(P = 0.045),Ross modified score(P = 0.003) and left ventricle ejection fraction(P = 0.021).CONCLUSION Raised NT-proBNP,specifically very high values(Z-log-NT-proBNP > 4) could help in the early identification of MIS-C patients with myocardial dysfunction requiring inotropic support and PICU admission.

关键词： Multisystem inflammatory syndrome associated with COVID-19 COVID-19 SARS-CoV-2 NT-proBNP Echocardiography Myocardial dysfunction Children

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Thioperamide treats neonatal hypoxic-ischemic encephalopathy by postsynaptic H1 receptors

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Neural Regeneration Research 2013年第19期8卷 1814-1822页

作者： Feiyong Jia Lin Du Yunpeng Hao Shicheng Liu Ning Li Huiyi Jiang division of pediatric neurorehabilitation Department of Pediatrics Second Part of First Hospital of Jilin University

Thioperamide, a selective histamine H3 receptor antagonist, can increase histamine content in the brain, improve brain edema, and exert a neuroprotective effect. This study aimed to examine the mechanism of action of thioperamide during brain edema in a rat model of neonatal hypoxic ischemic encephalopathy. Our results showed that thioperamide significantly decreased brain water content and malondialdehyde levels, while significantly increased histamine levels and superoxide dismutase activity in the hippocampus. This evidence demonstrates that thioperamide could pre vent oxidative damage and attenuate brain edema following neonatal hypoxicischemic encepha Iopathy. We further observed that changes in the above indexes occurred after combined treatment of thioperamide with the H1 receptor antagonist, pyrilamine, and the H2 receptor antagonist, ci metidine. Experimental findings indicated that pyrilamine reversed the effects of thioperamide; however, cimetidine had no significant influence on the effects of thioperamide. Our present findings suggest that thioperamide can increase brain histamine content and attenuate brain edema and oxidative damage by acting in combination with postsynaptic H1 receptors in a rat model of neo natal hypoxicischemic encephalopathy.

关键词： neural regeneration thioperamide histamine histamine receptor antagonist cimetidine pyrilamineneonatal hypoxic-ischemic encephalopathy brain edema hippocampus malondialdehyde super-oxide dismutase grants-supported paper neuroregeneration

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Plasmodium falciparum found in the bone marrow of a child in Manado City,East Indonesia:A case report

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Asian Pacific Journal of Tropical Medicine 2017年第10期10卷 1015-1017页

作者： Suryadi N.N.Tatura Stefanus Gunawan Janno Bernadus Sianne Sandjoto Indonesian National Expert Committee of Malaria Ministry of Health Republic of IndonesiaIndonesia division of pediatric Infection and Tropical Medicine Department of PediatricFaculty of MedicineSam Ratulangi UniversityManadoIndonesia division of pediatric Infection and Tropical Medicine Department of PediatricProf.R.D.Kandou General HospitalManadoIndonesia Estella pediatric Cancer Center Prof.Dr.R.D.Kandou General HospitalManadoIndonesia division of Hematology oncology Department of PediatricFaculty of MedicineSam Ratulangi UniversityManadoIndonesia Department of Parasitology Faculty of MedicineSam Ratulangi UniversityManadoIndonesia Department of pediatric Faculty of MedicineSam Ratulangi UniversityManadoIndonesia

In Indonesia, there are at least 1.3 million cases of malaria each year and Plasmodium falciparum appears to be the most common *** finding of Plasmodium is important for the diagnosis and management of *** is a case of a 4-year-and-9-month-old male who lived in Manado, East *** presented with a prolonged fever, was pale in appearance, and was easily fatigued over the last 3 *** was found on the initial physical *** laboratory findings found pancytopenia and severe *** he was referred to our hospital, at the primary health center, the initial work-up was negative for Plasmodium with the serial Rapid Diagnostic Test and microscopic peripheral blood *** there were signs and symptoms mimicking malignancy, the patient was referred to our hospital for further malignancy work-up.A bone marrow puncture was done and we incidentally found Plasmodium falciparum in a microscopic bone marrow *** was a rare case because Plasmodium was not initially found in the preliminary work-up(Rapid Diagnostic Test and Microscopic) and qPCR is not a routine work-up for Plasmodium suspected *** the mortality rate of malaria is high, this condition can be treated if the clinician was aware of the clinical signs and symptoms in the early onset and prompt medical treatment is *** a severe case with an unclear etiology of fever and with signs and symptoms mimicking malignancy, qPCR is ***, a bone marrow puncture can also be considered to exclude the possibility of a malaria infection.

关键词： Malaria Plasmodium falciparum Peripheral blood Bone marrow

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