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Children with sickle cell Disease in Northern Benin: Follow up of a Cohort at the Borgou/Alibori Branch of Integrated Medical Healthcare Center for Infants and Pregnant Women with sickle cell Disease from 2017 to 2022

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Open Journal of Pediatrics 2024年第3期14卷 445-458页

作者： Falilatou Agbeille Mohamed Alphonse Noudamadjo Médétinmè Gérard Kpanidja Yémalin Zinsou François Anikè Akinola Kpedio Clarisse Marie Chantal Bossa Julien Didier Adédémy Joseph Agossou Teaching and Research unit Department of Mother and Child Faculty of Medicine University of Parakou Parakou Benin Integrated Healthcare Center for Infants and Pregnant Women with sickle cell Disease (CPMI-NFED) Branch of Borgou/Alibori Parakou Benin

Introduction: sickle cell disease is a public health problem in sub-Saharan Africa. A national referral center for the management of infants and pregnant women with sickle cell disease (CPMI-NFED) was created three decades ago in Cotonou, in the south of Benin with two regional branches including that of Parakou in the North for better access of patients to specialized care. This work is a review of five years of activities in order to describe the epidemiological, clinical, hematological and evolutionary profiles of the children followed up in the said branch. Method: This was a descriptive and retrospective cross-sectional study on the medical records of children with sickle cell disease, followed up at the regional branch of CPMI-NFED in Borgou/Alibori from June 1, 2017 to May 31, 2022. The variables studied were epidemiological, clinical, biological and evolutionary. Results: A total of 101 children with sickle cell disease were included in the study, including 78 homozygous SS (77.2%) and 23 heterozygous SC (22.8%). Their mean age at inclusion was 51.2 ± 37.6 months [6 - 204]. The sex ratio was 1.4. Vaso-occlusive crises were the main diagnostic circumstances in 42.3% of homozygotes. More than half of the children (51.5%) had a regular follow-up. The average baseline level of hemoglobin (Hb) in homozygous children was 8.8 ± 1.4 g/dl [5.8 - 11.5];and the rate of Hb S varied between 61.9 and 94.7%. In heterozygous SC children, the mean baseline level of Hb was 10.7 ± 0.6 g/dl [9.7 - 11.5]. Acute complications observed during follow-up were dominated by pneumonia and vaso-occlusive crises in both phenotypes. The overall mortality was 3% and only affected homozygous patients. Conclusion: On average, three out of four children were homozygous in our cohort. The main acute complications were infectious and vaso-occlusive. The mortality only affected homozygous carriers. Specialized follow-up has contributed to improving the quality of life of children with sickle cel

关键词： sickle cell Disease Children CPMI-NFED Parakou Benin

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Hematological Malignancies in sickle cell Disease Patients: Report of Four Cases in Togo and Literature Review

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Open Journal of Blood Diseases 2024年第1期14卷 31-41页

作者： Padaro Essohana Guedenon M. Koffi Magnang Hèzouwè Womey M. C. Kodzovi Layibo Yao Kalaissi Mandjamana Agate R. Pikiliwè Department of Hematology Campus Teaching Hospital University of Lomé Lomé Togo Department of Pediatrics Sylvanus Olympio Teaching Hospital University of Lomé Lomé Togo National Center of Research and Treatement of sickle cell Disease Patients (CNRSD) Lomé Togo Department of Hematology Sylvanus Olympio Teaching Hospital University of Lomé Lomé Togo

Background: Hemopathies were rarely observed in major sickle cell disease patients some thirty years ago, probably due to the high mortality rate among the latter as a result of progressive complications. Thanks to advances in the management of sickle cell disease, patients' life expectancy has increased considerably, exposing them more frequently to neoplasia, including hematological malignancies. The increased risk of leukemogenesis is multifactorial and linked to the pathophysiological mechanisms of the clinical manifestations of sickle cell disease. Study Setting: The clinical haematology department of campus teaching hospital and the paediatric onco-haematology unit of Sylvanus Olympio teaching hospital in Lomé were used as study settings. Observations: Four hematologic malignancies were collected in a cohort of 5847 major sickle cell syndromes. The median age of the patients was 31.25 years (extremes: 14 and 58 years) and they were predominantly female (sex ratio M/F = 0.25). Two were on background therapy with hydroxyurea. Among the four patients, there were two cases of acute lymphocytic leukemia, including ALL3 in a 58-year-old SS woman and T-ALL2 in a 12-year-old SC. Then, a case of lymphocytic lymphoma in a 20-year-old SS man was reported and finally a case of chronic myelocytic leukemia in a 33-year-old woman of Sβ+ thalassaemia phenotype. Conclusion: To further report this coexistence, it is therefore essential to systematically consider hematological malignancies during major sickle cell syndromes even if there are similarities in the symptomatology of these two serious pathological situations.

关键词： Hematological Malignancies sickle cell Disease Lomé-Togo

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Influence of Hemoglobin S Haplotypes on the Responses to Hydroxyurea Treatment in Children with sickle cell Disease in Abidjan, Côte d’Ivoire

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Open Journal of Genetics 2024年第1期14卷 1-12页

作者： Mireille Aye-Yayo Vincent Yapo Boidy Kouakou Missa Louis Adjé Adia Eusèbe Adjambri Ebah Hermance Kassi Taïratou Kamagate Duni Sawadogo Hematology unit Central Laboratory of the Teaching Hospital of Yogoupon Abidjan C&#244te d’Ivoire Hematology and cell Biology unit Laboratory of Biological Sciences Department of Pharmacy Felix Houphouet Boigny University Abidjan C&#244te d’Ivoire Molecular Biology unit Center for Diagnostic and Research on AIDS and Other Infectious Diseases (CeDReS) Teaching Hospital of Treichville Abidjan C&#244te d’Ivoire Clinical Hematology unit Teaching Hospital of Yogoupon Abidjan C&#244te d’Ivoire

Background: In Côte d’Ivoire so far, the circulating haplotypes have been inferred on the phenotypic profiling of SCD patients. The impact of the circulating haplotypes on the use of Hydroxyurea has not been assessed yet. Therefore the objective of this study is to identify in Abidjan the HbS haplotypes that modulate HU treatment responses. Methods: In a cross-sectional descriptive and analytical study, children aged 5 to 15 years with SCD, and carrying the hemoglobin phenotypes SSFA2 and SFA2, were recruited into a HU treatment cohort. Various parameters on the haplotypes and the outcomes of the treatment were analyzed. Results: Thirty nine children with SCD were included. The phenotypic profile of the cohort was 86.6% of SSFA2 and 15.4% of SFA2. Three haplotypes were found, the Benin haplotype, the Senegal haplotype, and an atypical one. The participants belonged to three genotypes, Benin/atypical (64.1%), Benin/Senegal (33.3%) and Senegal/Senegal (2.6%). Overall, HU treatment was successful in all haplotypes with 12 out of 39 patients failing treatment after 12 months in the Benin haplotype group. The association between HU treatment success and the Benin haplotype was found in terms of the decrease in the number of white blood cells and the students missing class. Conclusion: The study revealed that inferring haplotype based on the phenotypic profile could be inaccurate. The proportion of atypical haplotype that were not previously described in Côte d’Ivoire was high. All the haplotypes seemed to be associated with HU treatment success but some patients with Benin haplotype did not respond well.

关键词： sickle cell Disease Children Haplotype Hydroxyurea Côte d’Ivoire

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APPLICATION OF HIGH PRESSURE LIQUID CHROMATOGRAPHY AND MICROSEQUENCING METHODOLOGY IN THE STRUCTURAL ANALYSIS OF HUMAN HEMOGLOBIN VARIANTS

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Science China Chemistry 1983年第08期26卷 836-849页

作者：曾溢滔,黄淑帧,A.REYNOLDS,H.LAM,B.B.WEBBER,J.B.WILSON,T.H.J.HUISMAN Laboratory of Medical Genetics Shanghai Children's Hospital Shanghai People's Republic of China Laboratory of Medical Genetics Shanghai Children's Hospital Shanghai People's Republic of China Laboratory of Protein Chemistry Comprehensive sickle cell Center Department of cell and Molecular Biology Medical Collge of Georgia Augusta Ga 30912 U. S. A. Laboratory of Protein Chemistry Comprehensive sickle cell Center Department of cell and Molecular Biology Medical Collge of Georgia Augusta Ga 30912 U. S. A. Laboratory of Protein Chemistry Comprehensive sickle cell Center Department of cell and Molecular Biology Medical Collge of Georgia Augusta Ga 30912 U. S. A. Laboratory of Protein Chemistry Comprehensive sickle cell Center Department of cell and Molecular Biology Medical Collge of Georgia Augusta Ga 30912 U. S. A. Laboratory of Protein Chemistry Comprehensive sickle cell Center Department of cell and Molecular Biology Medical Collge of Georgia Augusta Ga 30912 U. S. A.

This paper reports the results of the analyses of 17 different Hb variants which were observed in 37 families. High pressure liquid chromatography (HPLC) was used to separate tryptic and thermolytic peptides of globin chains. The double coupling microsequencing technique can successfully be used for sequencing small or larger peptides.

关键词： APPLICATION OF HIGH PRESSURE LIQUID CHROMATOGRAPHY AND MICROSEQUENCING METHODOLOGY IN THE STRUCTURAL ANALYSIS OF HUMAN HEMOGLOBIN VARIANTS HPLC

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A Review of Endocrine Disorders in thalassaemia

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Open Journal of Endocrine and Metabolic Diseases 2014年第2期4卷 25-34页

作者： Parijat De Radhika Mistry Christine Wright Shivan Pancham Wyn Burbridge Kalyan Gangopadhayay Terence Pang Gautam Das Department of Diabetes and Endocrinology Southern General Hospital Glasgow UK Department of Diabetes Endocrinology and Lipid Metabolism City Hospital Birmingham UK sickle cell and thalassaemia unit City Hospital Birmingham UK

Endocrine dysfunction in thalassaemia is amongst the most common complication and is principally attributed to excessive iron overload and suboptimal chelation. The prevalence is quite high particularly in multiethnic populations but determining the prevalence is often difficult due to the widespread heterogeneity of the population and timing of exposure to chelation therapy. Disturbances in growth, pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes and disorderly bone growth are commonly encountered. Early detection and institution of appropriate transfusion regimen and chelation therapy and treatment of complications are the keys to managing this population including regular follow. In this article, we review the literature in relation to the various endocrine complications encountered in thalassaemia.

关键词： thalassaemia Chelation Endocrinopathies Diabetes Hypothyroidism

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Primary gastric non-Hodgkin lymphomas:Recent advances regarding disease pathogenesis and treatment

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World Journal of Gastroenterology 2021年第35期27卷 5932-5945页

作者： Michael D Diamantidis Maria Papaioannou Evdoxia Hatjiharissi Department of Hematology Thalassemia and Sickle Cell Disease UnitGeneral Hospital of LarissaLarissa 41221ThessalyGreece Division of Hematology First Department of Internal MedicineAHEPA General HospitalAristotle University of ThessalonikiThessaloniki 54636Greece

Primary gastric lymphomas(PGLs)are distinct lymphoproliferative neoplasms described as heterogeneous entities clinically and *** main histological types are diffuse large B-cell lymphoma(DLBCL)or mucosaassociated lymphoma *** has been one of the main fields of clinical research of our group in recent *** gastric DLBCLs are frequent,sufficient data to guide optimal care are *** today,a multidisciplinary approach has been applied,including chemotherapy,surgery,radiotherapy or a combination of these *** this minireview article,we provide an overview of the clinical manifestations,diagnosis and staging of these diseases,along with their molecular pathogenesis and the most important related clinical published *** then discuss the scientific gaps,perils and pitfalls that exist regarding the aforementioned studies,in parallel with the unmet need for future research and comment on the proper methodology for such retrospective *** to fill this gap,we retrospectively evaluated the trends in clinical presentation,management and outcome among 165 patients with DLBCL PGL who were seen in our institutions in *** study cohort was divided into two subgroups,comparing the main 2 therapeutic options[cyclophosphamide doxorubicin vincristine prednisone(CHOP)vs rituximab-CHOP(R-CHOP)].A better outcome with immunochemotherapy(R-CHOP)was *** the next 2 mo,we will present the update of our study with the same basic conclusion.

关键词： Primary gastric lymphoma Extranodal non-Hodgkin’s lymphoma Diffuse large B-cell lymphoma Mucosa-associated lymphoid tissue Immunochemotherapy Rituximab-cyclophosphamide doxorubicin vincristine prednisone

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“FISH VERTEBRA” about 3 sickle cell Patients Followed at Laquintinie Hospital, Douala

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Open Journal of Rheumatology and Autoimmune Diseases 2024年第1期14卷 20-25页

作者： Same Bebey Francine Mbono Betoko Ritha Eloundou Onomo Paul Mantho Fopa Pauline Eposse Ekoube Charlotte Megne Tamo Estelle Ebene Mbende Romain Singwe Ngandeu Madeleine Rheumatology unit Laquintinie Hospital Douala Douala Cameroon Paediatric unit Laquintinie Hospital Douala Douala Cameroon La Cite Verte Hospital Yaounde Yaounde Cameroon Paediatric Surgery unit Laquintinie Hospital Douala Douala Cameroon La Cite des palmiers Hospital Douala Douala Cameroon Rheumatology unit Central Hospital Yaounde Yaounde Cameroon

Vertebral involvement in particular is common in sickle cell patients. We report 3 cases of “fish vertebra” fractures in sickle cell patients aged 16, 18, and 24 years old respectively at Laquintinie Hospital, Douala. When the vertebral fractures were diagnosed, the 3 patients had back pain and kyphosis deformities of the dorsal spine. Treatment with an infusion of biphosphonates (zoledronic acid at a dose of 0.5 mg·per·kg) was offered to all three patients. Two out of three patients received treatment with biphosphonates with a successful outcome. Profound vitamin D deficiency is associated with increased bone remodeling and a history of fractures. In sickle cell anemia, vertebral fractures may also result from bone fragility, which is often overlooked as aseptic osteonecrosis and osteomyelitis, which are very often suspected.

关键词： Vertebral Fractures sickle cell Anemia Vertebral Bone Fragility

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Pathological and Etiological Aspects of Nephrotic Syndrome at the Niamey General Reference Hospital

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Open Journal of Nephrology 2024年第3期14卷 340-349页

作者： Djibrilla Gani Salamatou Amadou Niaouro Oumarou Ali Diallo Nephrology-Dialysis Department Niamey General Reference Hospital Niamey Niger Environmental Health and Climate Epidemiology unit (UESEC)-CERMES Niamey Niger

Introduction: Studies have been conducted on nephrotic syndrome in Niger. The study aimed to determine the histological and etiological aspects of nephrotic syndrome. Patients and Method: This was a retrospective study from February 1st, 2018 to January 31st, 2024. All patients with nephrotic syndrome who underwent renal biopsy were included. Samples were analyzed at the anatomy-cytology pathology laboratory of the Faculty of Medicine in Dakar (Senegal). The variables studied included clinical, biological, histological and etiological characteristics. Data were analyzed using Excel 2013 and Epi-info 7.2.0 software. Results: The study included 119 patients with nephrotic syndrome. Prevalence of nephrotic syndrome was 11.24%. The male-to-female ratio was 2.25:1. The mean age at diagnosis was between 34.5 ± 18.84 years. Edema was the reason for admission in 40.34% of cases. The nephrotic syndrome was impure in 63.86% of cases. Nine histological lesions were identified. Focal and segmental glomerulosclerosis (40.09%), minimal change disease (23.53%), membranous nephropathy (13.45%), diabetic nephropathy (10.92%), membranous proliferative glomerulonephritis (3.36%), acute glomerulonephritis (3.36%), glomerular thrombotic microangiopathy (2.52%), non-IgA mesengial proliferative glomerulonephritis (1.68%) and amyloidosis (0.84%). Nephrotic syndrome was primary in 57.98% of cases. Secondary etiologies were dominated by diabetes (11.76%), followed by hepatitis B virus (9.24%), lupus, lymphoma, malaria, syphilis, cryoglobulinemia, sickle cell disease and HIV. Conclusion: Future studies should investigate the causes of glomerulopathy secondary to chronic tubulointerstitial lesions.

关键词： Nephrotic Syndrome Anatomopathology Niamey General Reference Hospital

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Antiretroviral therapy related adverse effects:Can sub-Saharan Africa cope with the new“test and treat”policy of the World Health Organization?

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Infectious Diseases of Poverty 2017年第1期6卷 212-216页

作者： Jobert Richie N.Nansseu Jean Joel R.Bigna Department of Public Health Faculty of Medicine and Biomedical Sciences of the University of YaoundeIPO Box 1364YaoundeCameroon sickle cell Disease unit Mother and Child Centre of the Chantal Biya FoundationYaoundeCameroon Department of Epidemiology and Public Health Centre Pasteur of CameroonYaoundeCameroon Faculty of Medicine University of Paris Sud XILe Kremlin BicêtreParisFrance

Background:Recent studies have shown that early antiretroviral therapy(ART)initiation results in significant HIV transmission *** is the rationale behind the“test and treat”policy of the World Health Organization(WHO).Implementation of this policy will lead to an increased incidence of ART-related adverse effects,especially in sub-Saharan Africa(SSA).Is the region yet ready to cope with such a challenging issue?Main body:The introduction and widespread use of ART have drastically changed the natural history of HIV/AIDS,but exposure to ART leads to serious medication-related adverse effects mainly explained by mitochondrial toxicities,and the situation will get worse in the near ***,ART is associated with an increased risk of developing cardiovascular disease,lipodystrophy,prediabetes and overt diabetes,insulin resistance and hyperlactatemia/lactic *** prevalence of these disorders is already high in SSA,and the situation will be exacerbated by the implementation of the new WHO *** SSA countries are characterized by(extreme)poverty,very weak health systems,inadequate and low quality of health services,inaccessibility to existing health facilities,lack of(qualified)health personnel,lack of adequate equipment,inaccessibility and unaffordability of medicines,and heavy workload in a context of a double burden of ***,there is dearth of data on the incidence and predictive factors of ART-related adverse effects in SSA,to anticipate on strategies that should be put in place to prevent the occurrence of these conditions or properly estimate the upcoming burden and prepare an adequate response *** are required if we are to anticipate and effectively prevent this upcoming ***:While SSA would be the first region to experience the huge benefits of implementing the“test and treat”policy of the WHO,the region is not yet prepared to manage the consequential increased burden of ART-related toxic and

关键词： HIV/AIDS Test and treat 90-90-90 Antiretroviral therapy Sub-Saharan Africa

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Pregnancy Preferences in Females of Reproductive Age with sickle cell Disease at Laquintinie Hospital: A Cross-Sectional Analysis

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Open Journal of Obstetrics and Gynecology 2024年第5期14卷 744-757页

作者： Eposse Ekoube Charlotte Jeannette Disso Massako Mangala Fulbert Nkwele Mandeng Ma Linwa Edgar Puepi Djike Yolande Merlin Boten Bounyom Erero F. Njiengwe Épée Patricia Hassanatou Iyawa Dora Mbonjo Bitsie Emmanuel Heles Nsang Soumaiyatou Abba Ngo Linwa Esther Eleonore Ndolo Kondo Astrid Grâce Tocki Toutou Moustapha Bilkissou Essome Henri Faculty of Medicine and Pharmaceutical Sciences University of Douala Douala Cameroon Paediatric unit Douala Laquintinie Hospital Douala Cameroon Faculty of Health Sciences University of Bamenda Bamenda Cameroon

Introduction: Females with sickle cell disease (SCD), despite having a delayed pubertal development, are subject to many worries relating to their abilities to conceive, their capacity to maintain a maternofetal-risk-free pregnancy till term and give birth to healthy children without sickle cell disease. Knowing that unplanned pregnancies are more likely to increase maternofetal morbidity and mortality, we sought out to explore the pregnancy preferences in female patients with sickle cell disease to promote healthy conception and childbirth in this vulnerable population. Methodology: We conducted a cross-sectional study involving female patients of child-bearing age with sickle cell disease followed at Laquintinie Hospital Douala or who were members of a local sickle cell association. Pregnancy preferences were determined using the Desire to Avoid Pregnancy (DAP) scale. Factors associated with pregnancy preferences were determined using multivariable linear regression model. Threshold for significance was set at p Results: Seventy-seven patients were included with a mean age of 24.71 ± 5.53 years. Forty patients (51.95%) had one sexual partner and thirteen women (32.5%) used contraceptive methods. Most patients (46.0%) responded “Strongly Agree” or “Agree” to the statement “it would be a good thing for me if I became pregnant in the next 3 months”. The mean DAP score was 1.63 ± 0.91 [1.28 ± 0.82 in those who had a sexual partner and 1.99 ± 0.86 in those who had no sexual partner]. Factors that were positively associated with DAP score were students (b = 0.32, 95% CI [0.21, 0.95], p = 0.003), monthly income ≥ 100,000 FCFA (b = 0.24, 95% CI [0.07, 0.81], p = 0.022), and not having a sexual partner (b = 0.26, 95% CI [0.11, 0.85], p = 0.012). Conclusion: Most female patients with SCD have a low desire to avoid pregnancy. This is especially true for patients who are not students, have a partner and have a low monthly income.

关键词： Pregnancy Preferences sickle cell Disease Laquintinie Hospital Cameroun

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