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Retinoblastoma: Socio-Demographic, Clinical and Therapeutic Aspects in the Hemato-Oncology Unit of the Department of pediatrics of Donka National Hospital, Guinea

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Open Journal of pediatrics 2020年第3期10卷 561-569页

作者： Aïssata Barry Mamadou M. Diop Mamadou A. Doukoure Emmanuel Camara Saliou B. Diallo Narcisse V. Gateu Tadjom Fatoumata Barry Hasmiou Dia Boubacar Togo Mamadou P. Diallo Department of pediatrics National Hospital Donka Conakry Guinea Department of pediatrics Ignace Deen Hospital Conakry Guinea departmentof pediatrics Gabriel Toure Hospital Bamako Mali Medical and Health Counseling Center Conakry Guinea

Introduction: Retinoblastoma is one of the pediatric malignancies and its treatment is still challenging. The objective of this study was to highlight the sociodemographic, clinical, and therapeutic aspects of retinoblastoma at the Donka Paediatric Haematology/Oncology unit in Donka university hospital, Guinea, and thereby highlight the conditions of this malignancy in this country. Methods: This was a retrospective study over 3 years (January 1, 2017 to December 31, 2019). The study population was all retinoblastoma patients who were treated in this unit. Socio-demographic, clinical, and therapeutic data were analyzed. Results: Out of 210 pediatric patients followed up for cancer in this unit during the period, retinoblastoma accounted for 46 (22%). The patient age at diagnosis was 35.17 months (range: 3 - 132 months). At admission, metastases were observed in 16 patients (35%): 14 with brain metastases and 2 with lymph node metastases. Preoperative chemotherapy was employed in 38 (83%), tumor resection was performed in 20 patients (43%), and postoperative chemotherapy was performed in 19 patients. As for the prognosis, remission in 9, death in 14, and treatment discontinuation in 15. Only one patient had health insurance (2%). Conclusion: In this region of Guinea, retinoblastoma is the most common ocular tumor in children. Its diagnosis is easy: strabismus and leukocoria are the early signs of retinoblastoma. The prognosis depends on how early the diagnosis is made. Physicians, care-givers, and family members should be aware of the nature of this malignancy and social education/message as such is necessary.

关键词： Retinoblastoma Leukocoria Chemotherapy Enucleation

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

Prevalence and risk factors of stress-induced gastrointestinal bleeding in critically ill children

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World Journal of Gastroenterology 2005年第43期11卷 6839-6842页

作者： Chookhuan Nithiwathanapong Sanit Reungrongrat Nuthapong Ukarapol Department of pediatrics

AIM： To assess the frequency and the risk factors of stress-induced gastrointestinal （GI） bleeding in children admitted to a pediatric intensive care unit （PICU）. METHODS： The medical records of children aged between 1 month and 15 years admitted to the PICU between January 2002 and December 2002 were reviewed. Demographic data, indications for PICU admission, principle diagnosis, and basic laboratory investigations were recorded. Previously described factors for stress ulcer bleeding （mechanical ventilation, sepsis, acute respiratory distress syndrome, renal insufficiency, coagulopathy, thrombocytopenia, and intracranial pathology） were used as independent variables in a multivariate analysis. RESULTS： One hundred and seventy of two hundred and five medical records were eligible for review. The most common indication for PICU admission was respiratory failure （48.8%）. Twenty-five children received stress ulcer bleeding prophylaxis with ranitidine. The incidence of stress ulcer bleeding was 43.5%, in which 5.3% were clinically significant bleeding. Only mechanical ventilation and thrombocytopenia were significantly associated with stress ulcer bleeding using the univariate analysis. The odds ratio and 95% confidence intervals were 5.13 （1.86-14.12） and 2.26 （1.07-4.74）, respectively. However, the logistic regression analysis showed that mechanical ventilation was the only significant risk factor with the odds ratio of 14.1. CONCLUSION： The incidence of gastrointestinal bleeding was high in critically ill children. Mechanical ventilation was an important risk factor for gastrointestinal bleeding.

关键词： Gastrointestinal Hemorrhage Stress Risk factor Child

Molecular physiology of vesicular glutamate transporters in the digestive system

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World Journal of Gastroenterology 2005年第12期11卷 1731-1736页

作者： Fayez K.Ghishan Departments of pediatrics and Medicine

Glutamate is the major excitatory neurotransmitter in the mammalian central nervous system (CNS). Packaging and storage of glutamate into glutamatergic neuronal vesicles require ATP-dependent vesicular glutamate uptake systems, which utilize the electrochemical proton gradient as a driving force. Three vesicular glutamate transporters (VGLUT1-3) have been recently identified from neuronal tissue where they play a key role to maintain the vesicular glutamate level. Recently, it has been demonstrated that glutamate signaling is also functional in peripheral neuronal and non-neuronal tissues, and occurs in sites of pituitary, adrenal, pineal glands, bone, GI tract, pancreas,skin, and testis. The glutamate receptors and VGLUTs in digestivesystem have been found in both neuronal and endocrinal cells. The glutamate signaling in the digestive system may have significant relevance to diabetes and GI tract motility disorders. This review will focus on the most recent update of molecular physiology of digestive VGLUTs.

关键词： Glutamate Vesicular glutamate transporters Digestive system

Immunosurveillance function of human mast cell?

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World Journal of Gastroenterology 2005年第44期11卷 7054-7056页

作者： ner zdemir Department of pediatrics

Mast cell (MC) is so widely recognized as a critical effector in allergic disorders that it can be difficult to think of MC in any other context. Indeed, MCs are multifunctional and recently shown that MCs can also act as antigen presenters as well as effector elements of human immune system. First observations of their possible role as anti-tumor cells in peri- or intra-tumoral tissue were mentioned five decades ago and a high content of MCs is considered as a favorable prognosis,consistent with this study. Believers of this hypothesis assumed them to be inhibitors of tumor development through their pro-apoptotic and -necrolytic granules e.g.,granzymes and TNF-α. However, some still postulate them to be enhancers of tumor development through their effects on angiogenesis due to mostly *** are also some data suggesting increased MC density causes tumor development and indicates bad prognosis. Furthermore, since MC-associated mediators have shown to influence various aspects of tumor biology, the net effect of MCs on the development/progression of tumors has been difficult to evaluate. For instance, chymase induces apoptosis in targets; yet,tryptase, another MC protease, is a well-known *** with these various enzyme expression patterns may mediate different functions and the predominant MC type in tissues may be determined by the environmental needs. The coexistence of tryptase-expressing MCs(MCT) and chymase and tryptase-expressing MCs (MCTC)in physiological conditions reflects a naturally occurring balance that contributes to tissue homeostasis. We have recently discussed the role and relevance of MC serine proteases in different bone marrow diseases.

关键词： Mast cell Immunosurveillance Tryptasechymase Cytotoxicity Tumors

Intramuscular vs intradermal route for hepatitis B booster vaccine in celiac children

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World Journal of Gastroenterology 2012年第40期18卷 5729-5733页

作者： Salvatore Leonardi Andrea Domenico Praticò Elena Lionetti Massimo Spina Giovanna Vitaliti Mario La Rosa Department of pediatrics University of Catania

AIM： To compare intradermal （ID） and intramuscular （IM） booster doses, which have been used in healthy and high risk subjects, such as healthcare workers, haemodialysis patients, human immunodeficiency virus patients, and renal transplant recipients unresponsive to initial hepatitis B vaccination, in celiac individuals. METHODS： We conducted our study on 58 celiac pa- tients, vaccinated in the first year of life, whose blood analysis had showed the absence of protective hepati- tis B virus （HBV） antibodies. All patients had received the last vaccine injection at least one year before study enrolment and they had been on a gluten free diet for at least 1 year. In all patients we randomly performed an HBV vaccine booster dose by ID or IM route. Thirty celiac patients were revaccinated with recombinant hepatitis B vaccine （Engerix B） 2 μg by the ID route, while 28 celiac patients were revaccinated with Engerix B 10 μg by the IM route. Four weeks after every boost- er dose, the anti-hepatitis B surface （HBs） antibody titer was measured by an enzyme-linked immune- adsorbent assay. We performed a maximum of three booster doses in patients with no anti-HBs antibodies after the first or the second vaccine dose. The cut off value for a negative anti-HBs antibody titer was 10 IU/*** with values between 10 and 100 IU/L were considered ＂low responders＂ while patients with an antibody titer higher than 1000 IU/L were considered ＂high responders＂. RESULTS： No significant difference in age, gender, du- ration of illness, and years of gluten intake was found between the two groups. We found a high percent- age of ＂responders＂ after the first booster dose （ID = 76.7%, IM = 78.6%） and a greater increase after the third dose （ID = 90%, IM = 96.4%） of vaccine in both groups. Mloreover we found a significantly higher num- ber of high responders （with an anti-HBs antibody titer 〉 1000 IU/L） in the ID （40%） than in the IM （7.1%） group, and this difference was evident after the firs

关键词： Hepatitis B virus Non responders Intradermal route Intramuscular route Celiac disease

Crohn's disease and recurrent appendicitis: A case report

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World Journal of Gastroenterology 2005年第43期11卷 6891-6893页

作者： Ron Shaoul Yosi Rimar Aurora Toubi Jorge Mogilner Reuven Polak Michael Jaffe Department of pediatrics Department of Radiology

The clinical diagnosis of classic Crohn's disease (CD)of the small bowel is based on a typical history, tender right lower quadrant fullness or mass, and characteristic radiographic findings of the terminal ileum. Appendicitis may as well present with chronic or recurrent symptoms and this presentation may be confused with CD. We herein describe the case of a young teenage girl with a presumptive diagnosis of CD, who was ultimately diagnosed as having chronic nongranulomatous appendicitis. The literature on the subject is reviewed.

关键词： Chronic appendicitis Crohn's disease Diagnosis Terminal ileitis Adolescent

Molecular docking studies of human MCT8 protein with soy isoflavones in Allan-Herndon-Dudley syndrome(AHDS)

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Journal of Pharmaceutical Analysis 2018年第5期8卷 318-323页

作者： Divya Shaji Department of pediatrics Aichi Medical University

Monocarboxylate transporter-8 （MCT8） is a specific thyroid hormone transporter, essential for the uptake of thyroid hormone into target tissues. Mutations in the MCT8 gene have been identified as the cause of Allan-Herndon-Dudley syndrome （AHDS）. It has been reported that soy isoflavones influence thyroid hormone system and can interact with thyroid hormone transporter proteins. Therefore, the present study aimed to find out whether soy isoflavones （genistein, daidzein and glycitein） can be used as a natural inhibitor to target MCT8 in AHDS. Docking studies were performed for soy isoflavones in order to evaluate their binding affinity to MCT8 protein using AutoDock4 （version 4.2.6） and AutoDock Vina. After docking, the ligands were ranked according to their binding energy and the best lead compound was selected based on the least binding energy. The docking results indicated that daidzein possesses the lowest binding energy against MCT8. Moreover, it was found that the residues PRO-338, HIS-341, and GLU-348 were involved in hydrogen bond interactions with genistein and daidzein. This study suggests that daidzein is a promising natural inhibitor to target MCT8 in AHDS.

关键词： Molecular docking MCT8 AHDS Soy isoflavones Daidzein Genistein

Safety and effi cacy of hepatitis A vaccine in children with chronic liver diseas

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World Journal of Gastroenterology 2006年第45期12卷 7337-7340页

作者： Hanaa Mostafa El-Karaksy Manal Ismail El-Hawary Nehal Mohammad El-Koofy Rokaya El-Sayed Mona Al-Saeed El-Raziky Samah Asaad Mansour Gamal Mohammad Taha Fatma El-Mougy Department of pediatrics Cairo University Egypt Department of pediatrics Fayoum University Egypt Department of pediatrics Beni-Swaif University Egypt Department of Chemical Pathology Cairo University Egypt

AIM： To study the safety and efficacy of hepatitis A vaccine （HAV） in children with chronic liver disease of various etiologies. METHODS： Eleven children with chronic liver disease and thirteen age- and sex-matched controls negative for HAV antibodies were vaccinated against hepatitis A after they gave their informed consent. Children with uncontrolled coagulopathy or signs of hepatic decompensation were excluded. The vaccine （Havrix： 720 ELISA units in 0.5 mL, from GlaxoSmithKline Biologicals） was given intramuscularly in the deltoid in 2 doses 6 mo apart. Children were tested for HAV antibodies one and six months after the ist dose and one month after the 2^nd dose. Total serum bilirubin, alanine aminotransferase （ALT）, and aspartate aminotransferase （AST） were determined immediately before and after one month of the 1st dose of the vaccine. RESULTS： Only 7 out of the 11 patients were positive for HAV antibodies after the 1^st dose of the vaccine, as compared to 100% of the controls. One month after the 2nd dose, all patients tested were positive for HAV antibodies. No deterioration in liver functions of patients was noted after vaccination. No adverse events, immediate or late, were reported by the mothers after each dose of the vaccine. CONCLUSION： Hepatitis A vaccine is both safe and effective in this small studied group of children with chronic liver disease. Given the high seroconversion rate, post-vaccination testing for HAV antibodies is not needed.

关键词： Children Chronic liver disease Hepatitis A Hepatitis A vaccine