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Evaluation of Glomerular Hyperfiltration and Albuminuria in Sickle Cell Disease Adolescents: Cross-Sectional Retrospective Study

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open Journal of Nephrology 2021年第3期11卷 321-334页

作者： Nosakhare Joyce Iduoriyekemwen Caroline Booth Marilyn McDougall Psalm Duniya Baba Inusa Advance paediatric Masters Programme King’s College London London UK paediatric Nephrology Unit Department of Child Health University of Benin Teaching Hospital Benin City Nigeria Department of paediatric Nephrology Evelina Children Hospital London UK Department of paediatric Critical Care Evelina Children Hospital London UK Department of paediatric haematology Evelina Children Hospital London UK

Background: Sickle Cell Disease (SCD) renal abnormalities commence early in childhood. The glomerular abnormalities, glomerular hyperfiltration and albuminuria are the most prevalent. However, these SCD glomerulopathies have not been considered exclusively in the adolescent age group. objective: To determine the prevalence of glomerular hyperfiltration and albuminuria as well as identify the determinants for glomerular hyperfiltration in adolescents with SCD. Patients and Methods: The electronic patient records of 153 adolescents with SCD aged 10 - paediatrics haematology Clinic at Evelina London Children’s Hospital, United Kingdom, were reviewed from the 10th to 23rd June 2019. Clinical information and laboratory parameters were obtained. The glomerular filtration rate was derived using the Bedside Schwartz’s method. Grouping of the adolescents was based on the presence and absence of glomerular hyperfiltration, which was defined as glomerular filtration rate > 140 ml/min/m2. The presence of albuminuria was defined as urine albumin-to-creatinine ratio > 3 mg/mmol or protein-to-creatinine ratio of >15 mg/mmol. The clinical and laboratory determinants of glomerular hyperfiltration in the total study population were investigated. Result: Prevalence of glomerular hyperfiltration was 33.3% in the adolescents studied, and that of albuminuria was 15.7% amongst the SCD adolescents studied, of which 13.7% of those with glomerular hyperfiltration also had albuminuria. on univariable analysis, the SCD adolescents with glomerular hyperfiltration had significantly lower weight (48.0 ± 18.0 versus 54.8 ± 17.0 kg;p = 0.02), height (155.1 ± 13.1 versus 160.6 ± 13.1 cm;p = 0.01), body mass index (19.4 ± 5.0 versus 21.0 ± 4.3;p = 0.04), haemoglobin level (88.7 ± 13.3 versus 98.1 ± 21.7 g/L;p = 0.001), and serum creatinine level (0.4 ± 0.1 versus 0.6 ± 0.2 mg/dl;p = 0.0001) as compared to tho

关键词： Glomerular Hyperfiltration Albuminuria Sickle Cell Disease Adolescent

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Management of opioid Use Disorder in Sickle Cell Anaemia amidst Growing Menace in the General Population

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open Journal of Pediatrics 2023年第6期13卷 807-820页

作者： Abimbola Aboluwarin Ayobola ojuawo oluwatobi Akanbi Lateef Quadri Hannah Elukpo Adebayo oloko Kolade Ernest paediatric haematology Unit Department of Paediatrics University of Ilorin Teaching Hospital Ilorin Nigeria Institute of Medical Research and Training University of Ilorin Ilorin Nigeria

Nigeria has a very high number of sickle cell disease (SCD) population with addition of 150,000 babies born annually with the disease. Early infant diagnosis and good care make many of these babies survive to adulthood. Severe pain requiring moderately strong or very strong analgesics is a common presentation of patients with Sickle Cell Anaemia. paediatricians find ready usefulness of opioids which are very useful for the painful episodes among these patients. Therefore, the chances of abuse and addiction to these medications become very high and constitute additional burden on the deficient manpower in the health sector. opioid Use Disorder among Sickle Cell Disease patients has subtle presentation, so a high index of suspicion is required to make both the diagnosis and referral to treatment centres. In this review, the epidemiology, pain pathophysiology, behavioural and pharmacologic therapy have been re-examined.

关键词： Sickle Cell Disease opioid Use Disorder Global Menace Treatment Centres True Addiction and Pseudo-Addiction

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CLAG-M chemotherapy followed by umbilical cord blood stem cell transplantation for primary refractory acute myeloid leukaemia in a child:A case report

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World Journal of Clinical Cases 2020年第22期8卷 5603-5610页

作者： Jie Huang Xiao-Yun Yang Liu-Cheng Rong Yao Xue Jun Zhu Yong-Jun Fang Department of haematology and oncology Children’s Hospital of Nanjing Medical UniversityNanjing 210008Jiangsu ProvinceChina

BACKGRoUND The prognosis of paediatric primary refractory/relapsed acute myeloid leukaemia(R/R AML)remains *** therapy is typically used as salvage treatment for those with R/R *** data are currently available about the use of the CLAG-M protocol as salvage therapy in paediatric patients with R/R *** SUMMARY An 8-year-old patient was diagnosed with acute myeloid leukaemia by bone marrow morphology and *** patient showed poor response to two cycles of induction therapy with 60%blast cells in the bone marrow after the second induction *** patient achieved complete remission after being treated with the CLAG-M protocol as salvage therapy before undergoing umbilical cord blood stem cell *** complete remission with haematological recovery has hitherto been maintained over 4 *** gene mutations detected at diagnosis were undetectable after haematopoietic stem cell *** Here we present a paediatric patient with primary refractory acute myeloid leukaemia who was successfully treated with the CLAG-M *** the positive results of the presented patient,large-scale clinical studies are required to assess the role of the CLAG-M protocol in the salvage treatment of refractory or relapsed AML in childhood.

关键词： Acute myeloid leukaemia CLAG-M protocol Salvage therapy Refractory child Case report

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Prevalence of Infectious Complications in Children with Cancer

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Journal of Cancer Therapy 2019年第12期10卷 938-947页

作者： Zaheer Ullah Khan Nagi Amin Gaafar Ahmed Mustafa Mohammad Sanad Tarmoom Mohammad Yousef AlGhar Anwaar Saud Aldahir Dayel AlShahrani Department of paediatric haematology and oncology King Fahad Medical City Riyadh Kingdom of Saudi Arabia Department of Infectious Diseases King Fahad Medical City Riyadh Kingdom of Saudi Arabia Department of General paediatric King Fahad Medical City Riyadh Kingdom of Saudi Arabia

Background:?Infections are the most common complications during chemotherapy. The trends have been changing over time due to use of multi-agent intensive chemotherapy.?Procedure:?We looked?over?our data to see what complications we get in our patients. The data was collected on patients treated in King Fahad Medical City from July 2009 to Dec 2015.?Results:?We?found that 86 patients had one episode only while 92 had 2 or more episodes reaching up to 11 episodes in 1 patient. We found positive cultures in 17.3% of episodes with staphylococcus as common gram positive and Klebsiella pneumoniae as common gram negative bacteria, respiratory viral infections in 13.8% and GI infections in 9%. We found Candida albicans as the most common fungus while other yeasts followed. Acute lymphoblastic leukaemia was the most common diagnosis. The episodes were associated with neutropenia of 7 days in 1st?episode and 56.8% in cases with repeated episodes. We had 2 deaths one from recurrent infections with CNS (Central Nervous System) damage and one from Pulmonary infections causing pulmonary haemorrhage. We had one with persistent neurological sequele from prolonged fungal infection.?Conclusions:Neutropenia of 7 days came out to be significant risk factors for infection across all diseases. We have an acceptable level of morbidity and mortality due to good supportive services.?We need prospective studies to get an up-to-date picture of our practice.?We need prophylactic antibiotics and antifungals for high-risk patients like AML.

关键词： Infections Cancer Children

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对儿童时期曾患嗜铬细胞瘤患者进行进一步诊断性检查具有重要意义

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世界核心医学期刊文摘（儿科学分册） 2005年第5期1卷 12-12页

作者： Beck o. Fassbender W. J. Beyer P. 李开 paediatric haematology and o ncology Johann Wolfgang Goethe-University Frankfurt Germany

We report on our experience with two patients with pheochromocytoma. one patie nt underwent surgery of pheochromocytoma at the age of 30 y; 18 y later, medulla ry thyroid carcinoma (MTC) was detected in his son. Subsequently, multiple endoc rine neoplasia (MEN) type 2A was diagnosed by genetic examination in both father and son. Further diagnostic procedures also revealed an MTC in the father. The other patient suffered from bifocal pheochromocytoma of the left suprarenal glan d. Diagnostic work-up revealed papillary thyroid carcinoma, which was also dete cted in the mother 8 mo later. Whereas a point mutation in SDHB gene was found i n the son, no genetic abnormality was detected in the mother. Conclusion: Every pheochromocytoma in childhood warrants further diagnostic work-up, including ge netic examination. In addition, clinical data of patients suffering from pheochr omocytoma and papillary thyroid carcinoma should be collected by an internationa l registry, and a joint effort should be undertaken in order to define possible underlying mutated genes in these patients.

关键词：嗜铬细胞瘤诊断性检查左侧肾上腺乳头状甲状腺癌甲状腺髓样癌临床病例资料基因异常基因点突变基因突变基因检查

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Management of neuroblastoma in limited-resource settings

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World Journal of Clinical oncology 2020年第8期11卷 629-643页

作者： Jaques van Heerden Mariana Kruger Department of paediatrics and Child Health Faculty of Medicine and Health SciencesStellenbosch UniversityCape Town 8000South Africa Department of paediatric haematology and oncology Antwerp University HospitalEdegem 2650Belgium

BACKGRoUND Neuroblastoma(NB)is a heterogeneous disease with variable outcomes among *** is known about NB in low-and middle-income countries(LMICs).AIM The aim of this review was to evaluate regional management protocols and challenges in treating NB in paediatric oncology units in LMICs compared to high-income countries(HICs).METHoDS PubMed,Global Health,Embase,SciELo,African Index Medicus and Google Scholar were searched for publications with keywords pertaining to NB,LMICs and *** English language manuscripts and abstracts were included.A descriptive review was done,and tables illustrating the findings were *** Limited information beyond single-institution experiences regarding NB outcomes in LMICs was *** disease characteristics varied among countries for the following variables:sex,age at presentation,MYCN amplification,stage and *** were found to be burdened with a higher percentage of stage 4 and high-risk NB compared to *** of evidence-based treatment protocols was still a barrier to *** socioeconomic variables also influenced the diagnosis,management and followup of patients with *** Patients presented at a later age with more advanced disease in *** was limited by the lack of resources and genetic studies for improved NB *** research is needed to develop modified diagnostic and treatment protocols for LMICs in the face of limited resources.

关键词： Neuroblastoma Limited resources Management outcomes Low-and middle-income countries

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Human parvo-virus B19 infection among children with sickle cell anaemia in Jos,North Central Nigeria

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海南医学院学报 2010年第10期16卷 1263-1265,1268页

作者： Girei AI Alao oo Joseph DE Damulak Do Banwat EB Nwadioha SI Jombo GTA Department of haematology Federal Medical CentreGombe Department of haematology College of Health SciencesBenue State UniversityMakurdi Department of haematology Jos University Teaching HospitalJos Department of Microbiology Jos University Teaching HospitalJos Department of Microbiology College of Health SciencesBenue State UniversityMakurdi

objective:Human Parvovirus B19 is known to cause significant morbidity and mortality and among diverse patient population *** patients with sickle all disease,who have high red cell turn over due to chronic haemolysis,infection with parvovirus B19 can cause severe life threatening transient aplastic *** study was conducted to determine the Seroprevalence of parvovirus B19 infection and to provide basic epidemiological data on parvovirus B19 infection among children with sickle cell anaemia in Jos,north central ***: In a hospital based cross sectional survey,200 children aged 1-18 years confirmed to have sickle cell anaemia attending the paediatric sickle cell clinic of Jos university teaching hospital were studied.A questionnaire was designed to obtain basic socio-demographic information,& other relevant aspect of patients′ *** samples were taken for anti parvo-virus B19 ***: The over all prevalence rates of parvo-virus B19 immunoglobulin(IgG) and immunoglobulin(IgM) antibodies were 39.5% and 3.5% respectively,Conclusion: This study confirms that parvo-virus B19 infection is prevalent among children with sickle cell anaemia in Jos,North Central *** is a need for further studies to fully evaluate the clinical impact of the infection on our sickle cell anaemia patients.

关键词：传染病贫血病儿童红细胞

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Risk and benefit from clinical trials in minors:Making the case for transparent and consistent publications

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open Journal of Pediatrics 2013年第2期3卷 151-164页

作者： Andreas Dirk Henschel Lillian Geza Rothenberger Dominik Schrey Joachim Gerβ Georg Hempel Joachim Boos Department of Pharmaceutical and Medical Chemistry—Clinical Pharmacy University of MünsterMünsterGermany Department of paediatric haematology and oncology University Children’s Hospital MünsterMünsterGermany Institute of Ethics and History in Medicine Center for MedicineSociety and PreventionUniversity of TuebingenGermany The Royal Marsden Hospital Department of Paediatric OncologySurreyUK Institute for Biostatistics and Clinical Research University of MuensterGermany Department of Pediatric Hematology and oncology University Children’s HospitalMuenster Albert-Schweitzer-CampusMuensterGermany

Rationale, aim and objectives: The European (EU) regulation on medicinal products for pediatric use (EC 1901/2006), which became effective in 2007, aimed to stimulate the clinical testing of medications in minors in order to reduce off-label use. In consequence, the number of minors taking part in randomized controlled clinical trials (RCTs) is likely to increase. Clinical trials in minors require a complex methodological design, a careful consideration of risks and benefits and a high level of ethical reflection. Unfortunately, as to the quality of clinical trials and their publications in minors little is known. Therefore, we assessed published reports of randomized, controlled clinical trials in minors, focusing on a common disease (asthma) and a defined spectrum of lifethreatening diseases (malignant diseases). Method: In an exploratory design, we scanned the publications for methodological aspects as well as indicators of ethical soundness, e.g., statements that informed consent had been obtained before the start of the trial or that a Data and Safety Monitoring Board ensured the patients’ safety during the trial. We also looked for passages reflecting the debate on equipoise or other forms of weighing risks and benefits. Results: We found that many of these aspects, which according to the scientific literature and generally acknowledged guidelines are essential to ensure good-quality trials and trial reports, were not considered in the publications analyzed. Conclusion: Therefore, we call for a more transparent and consistent presentation of the trials, especially of safety aspects, relying on a more critical and transparent ethical reflection.

关键词： Clinical Trials Minors SIGN CoNSoRT Ethics

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Pathogens causing diarrhoea among Bangladeshi children with malignancy: Results from two pilot studies

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World Journal of Clinical Cases 2020年第2期8卷 276-283页

作者： Sabina Karim Ferdousi Begum Afiqul Islam Monowar Ahmad Tarafdar Mamtaz Begum Md Johirul Islam Bushra Malik Md Shamim Ahsan Ameneh Khatami Harunor Rashid Department of paediatric haematology and oncology National Institute of Cancer Research and HospitalMohakhaliDhaka 1212Bangladesh Department of Pediatric Hematology and oncology Bangabandhu Sheikh Mujib Medical UniversityDhaka 1212Bangladesh Department of Community Medicine Z.H.Sikder Women’s Medical CollegeDhaka 1212Bangladesh Department of Cancer Epidemiology National Institute of Cancer Research and HospitalMohakhaliDhaka 1212Bangladesh National Centre for Immunisation Research and Surveillance The Children’s Hospital at WestmeadWestmeadNSW 2145Australia Medical Services Rangpur CantonmentRangpur 5400Bangladesh Department of Infectious Diseases and Microbiology The Children’s Hospital at WestmeadNSW 2145Australia Discipline of Child and Adolescent Health Faculty of Medicine and HealthUniversity of SydneyNSW 2145Australia

BACKGRoUND Diarrhoea is a frequent symptom in children with cancer, and occurs due to a composite effect of underlying disease and immunosuppression consequent to therapy, malnutrition, and non-infective aetiologies such as mucositis. In a large proportion of cases, the aetiology of diarrhoea remains unknown but is often attributed to multiple pathogens including *** To identify and describe the pathogens causing diarrhoea in Bangladeshi children with *** Two cross-sectional pilot studies were conducted involving paediatric oncology patients with diarrhoea. Stool samples were collected from children who were hospitalised with or without being treated with chemotherapy during the study period, and had diarrhoea at any stage during their admission. In the first study,stool samples were tested by conventional microbiological methods and by polymerase chain reaction for parasites, and by immunoassays for Clostridium difficile. In the second study, conventional microbiology was conducted for bacteria and parasites including an enzyme-linked immunosorbent assay for Cryptosporidium antigen, and in a subset, immunoassays for Clostridium *** In the first study Giardia lamblia was detected in 68.5% of samples, Entamoeba histolytica in 13%, Cryptosporidium in 5.6%, non-toxigenic C. difficile in 22.4%, and other bacteria in 5.2%. In the second study, E. histolytica was detected in 10% of samples, Cryptosporidium in 4.3%, G. lamblia in 1.4%, C. difficile in 5.1%, and other bacteria in 5.7% of *** These pilot data suggest that parasites are important aetiologies of diarrhoea in Bangladeshi children with malignancy. While molecular diagnostic tools detect an array of stool pathogens with greater sensitivity, conventional diagnostic methods are also useful.

关键词： Bangladesh Cancer Child Cryptosporidium Gastroenteritis Parasite

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paediatric Cancer Care in Abakaliki South-East Nigeria: Challenges and outcome

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Journal of Pharmacy and Pharmacology 2022年第3期10卷 97-104页

作者： Udechukwu Ngozi Patricia Ukoh Uchechukwu Chukwuebuka Nwagha Theresa Ukamaka Nwokoye Ikenna Chidiebele Department of paediatrics Alex Ekwueme Federal University Teaching Hospital Abakaliki(AEFUTHA)Ebonyi StateNigeria Department of haematology College of Medicine University of NigeriaItuku-Ozalla CampusNigeria

The care of children with malignancy is almost always a feat and is even more difficult in resource-poor settings such as our country Nigeria where several factors present as challenges as seen in other countries of sub-Saharan *** this study,we present our experience in AEFUTHA,over a six-year *** study aimed at finding out the current prevalence,outcomes and challenging issues leading to poor *** retrospective study spanned from January 2012 to December *** was extracted from the patients’case notes using a structured data *** aged 17 years and below who satisfied the inclusion and exclusion criteria were studied and the sample size was *** our study,the prevalence of paediatric malignancy is 2.0%.The outcome was generally poor with a mortality rate of 30.9%,and 60.3%having abandoned treatment at various stages of *** top-ranking challenges noted included diagnostic problems and late presentation accounting for 19.8%and 17.3%respectively and,poverty and lack of effective treatment and drugs representing 16.0%*** conclude that there is a need to adopt a multifaceted approach to tackling these challenges to improve outcomes.

关键词： paediatric malignancy challenges outcome resource-poor-setting

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