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Adrenal Insufficiency by Adrenoleukodystrophy

Health 2020年第1期12卷 1-13页

作者： Elda Cristinne Mattos botelho eduardo romero sampaio botelho Luciana Correa de Souza Rodrigues Tatiana Prado Wanderley Fernanda Navarini Queiroz Pollyana Cirino Gomes Borges Sabrina Arrais Rolim Aragao Ximenes Cruz Janete Pacheco da Silva Larissa Bianca Cunha de Sa Alberto Krayyem Arbex IPEMED Medical School AFYA EducationalBrasíliaBrazil Medical Clinic in Endocrinology and Diabetology Schleswig-FlensburgGermany

The X-linked adrenoleukodystrophy (ALD) is a severe neurodegenerative disorder due to mutations in the ABCD1 gene. Objective: To report a case of a 19-year-old man with adrenal insufficiency due to adrenoleukodystrophy. Method: Case report and literature review. Result: A previously healthy 19-year-old male patient was admitted to the emergency room with nausea and vomiting for 5 days, who progressed to abdominal pain, severe asthenia, and fever (38.5°C). He referred progressive darkening of the skin, oral mucosa, tongue and nail bed of the hands and feet, observed in the last 6 years. Emergency laboratory evaluation showed severe hyponatremia and hyperkalemia, which, together with decreased plasma cortisol, directed the investigation to causes of adrenal insufficiency. High ACTH (Adrenocorticotropic hormone) and very long chain fatty acid levels closed the diagnosis. Discussion: ALD is characterized by progressive demyelination in the central and peripheral nervous system and adrenal insufficiency consequence to the accumulation of very long chain fatty acids (VLCFA) in the adrenal. The overall incidence of ALD is 1:17,000. Adrenal insufficiency may be the first symptom of ALD in boys and adults. The diagnosis is based on the measurement of VLCFA plasma levels. Allogeneic bone marrow transplantation is the only treatment that provides a permanent cure when the procedure is performed at an early stage of brain demyelination, i.e. when patients are asymptomatic, although brain magnetic resonance imaging (MRI) is abnormal. Treatment of Addison’s disease is obligatory, but does not change the course of neurological symptoms.

关键词： Adrenoleukodystrophy Adrenomyeloneuropathy Adrenal Insufficiency Addison’s Disease

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Delivery of essential pediatric congenital surgical care within Brazil’s universal health coverage system:a national survey of pediatric surgeons

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World Journal of Pediatric Surgery 2023年第3期6卷 136-146页

作者： Paul R Truche Abbie E Naus Fabio botelho Julia Ferreira Alexis Bowder Luke Caddell Kathrin Zimmerman Isabella Maria de Freitas Faria Bellisa Caldas Lopes eduardo Corrêa Costa Fernanda Lage Lima Dantas Augusto J S A Cavalcante Carlos A L B Carvalho Simone Abib David P Mooney Nivaldo Alonso Program in Global Surgery and Social Change Harvard Medical SchoolBostonMassachusettsUSA Department of Surgery Rutgers Robert Wood Johnson Medical SchoolNew BrunswickNew JerseyUSA Department of Pediatric Surgery McGill UniversityMontrealQuébecCanada Department of Surgery Medical College of WisconsinMilwaukeeWisconsinUSA Division of Otolaryngology-Head and Neck Surgery Department of SurgeryUniversity of Wisconsin-Madison School of Medicine and Public HealthMadisonWisconsinUSA School of Medicine UFMGBelo HorizonteMinas GeraisBrazil Department of Pediatric Surgery Instituto de Medicina Integral Professor Fernando FigueiraRecifeBrazil Department of Pediatric Surgery Hospital de Clinicas de Porto AlegrePorto AlegreBrazil Department of Pediatric Surgery Federal University of AcreRio BrancoBrazil Department of Pediatric Surgery Hospital Santa Casa de Belo HorizonteBelo HorizonteBrazil Department of Pediatric Surgery Federal University of Mato GrossoCuiabaBrazil Department of Pediatric Surgery UNIFESPSao PauloBrazil Department of Pediatric Surgery Boston Children's HospitalBostonMassachusettsUSA Department of Plastic Surgery University of São Paulo Institute of Biomedical SciencesSao PauloBrazil

Objective In this study,we assess the delivery of congenital pediatric surgical care under Brazil’s system of universal health coverage and evaluate differences in delivery between public and private *** A cross-sectional national survey of pediatric surgeons in Brazil was *** were asked which of 23 interventions identified through the Disease Control Priorities 3(Surgical Interventions for Congenital Anomalies)they perform and to report barriers faced while providing surgical *** were weighted by state and stratified by sector(public vs private).Results A sample of 352 responses was obtained and weighted to represent 1378 practicing pediatric surgeons registered in Brazil during the survey time.73%spend the majority of their time working in the public sector(‘SistemaÚnico de Saúde’and Foundation hospitals),and most of them also work in the private ***,Brazilian pediatric surgeons have the expertise to provide thoracic,abdominal,and urologic *** working mostly in the public sector were more likely to report a lack of access to essential medications(25%vs 9%,p<0.01)and a lack of access to hospital beds for surgical patients(52%vs 32%,p<0.01).Conclusions Brazilian pediatric surgeons routinely perform thoracic,abdominal,and urologic *** working in government-financed hospitals face barriers related to infrastructure,which may impact Brazilians who rely on Brazil’s universal health coverage *** that support pediatric surgeons working in the public sector may promote the workforce available to provide congenital pediatric surgical care.

关键词： pediatric survey Brazil

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Low-Cost Sensors Calibration for Monitoring Air Quality in the Federal District—Brazil

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Journal of Environmental Protection 2015年第2期6卷 173-189页

作者： Erick Frederico Kill Aguiar Henrique Llacer Roig Luís Henrique Mancini eduardo Neiva Caetano botelho de Carvalho Institute of Geosciences (IG) University of Brasilia Brasilia Brazil

Critical situations that cannot be solved by conventional approaches (traditional air quality monitoring networks), have the possibility of being managed quickly by a wide network of portable systems with sensors. The purpose of this research was to calibrate and validate low-cost sensors. Pilot indoor and outdoor areas, in the central area of Brasilia (Brazil’s capital city) were chosen for corporative performance evaluation of the sensors. The CO at 99.999% volumetric injection method has been used in a gas test box, among two MiCS-5521 (CO/VOC) sensors, one being new and the other one with a short useful life. The number of injections adopted to each volume (from 1 ml to 6 ml) was 10, rising each sensor’s confidence interval mean. A increase of the injected volume (ml) of CO resulted in significant decrease in a resistance (Ohms), as shown by a good inverse relationship on the interaction of these two variables (r = 0.88), with good measurement accuracy, when compared to the manufacturer’s reference datasheet. Finally, a geospatial management system was built for the pollution data measured by the low-cost sensors.

关键词： Low-Cost Sensors Monitoring Air Quality Calibration of Micro-Sensors MiCS-5521 (CO/VOC) WebGIS

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Triple-modal imaging of stem-cells labeled with multimodal nanoparticles, applied in a stroke model

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World Journal of Stem Cells 2019年第2期11卷 100-123页

作者： Helio Rodrigues da Silva Javier Bustamante Mamani Mariana Penteado Nucci Leopoldo Penteado Nucci Andrea Tiemi Kondo Daianne Maciely Carvalho Fantacini Lucas eduardo botelho de Souza Virginia Picanço-Castro Dimas Tadeu Covas José Mauro Kutner Fernando Anselmo de Oliveira Nelson Hamerschlak Lionel Fernel Gamarra Hospital Israelita Albert Einstein LIM44 Hospital das Clinicas HCFMUSP Faculdade de MedicinaUniversidade de São Paulo Centro Universitário do Planalto Central Faculdade de Medicina de Ribeirão Preto Universidade de São Paulo

BACKGROUND Mesenchymal stem cells(MSCs) have been widely tested for their therapeutic efficacy in the ischemic brain and have been shown to provide several benefits. A major obstacle to the clinical translation of these therapies has been the inability to noninvasively monitor the best route, cell doses, and collateral effects while ensuring the survival and effective biological functioning of the transplanted stem cells. Technological advances in multimodal imaging have allowed in vivo monitoring of the biodistribution and viability of transplanted stem cells due to a combination of imaging technologies associated with multimodal nanoparticles(MNPs) using new labels and covers to achieve low toxicity and longtime residence in *** To evaluate the sensitivity of triple-modal imaging of stem cells labeled with MNPs and applied in a stroke *** After the isolation and immunophenotypic characterization of human bonemarrow MSCs(hBM-MSCs), our team carried out lentiviral transduction of these cells for the evaluation of bioluminescent images(BLIs) in vitro and in vivo. In addition, MNPs that were previously characterized(regarding hydrodynamic size, zeta potential, and optical properties), and were used to label these cells,analyze cell viability via the 3-[4,5-dimethylthiazol-2-yl]-2,5 diphenyl tetrazolium bromide assay and BLI analysis, and quantify the internalization process and iron load in different concentrations of MNPs via magnetic resonance imaging(MRI),near-infrared fluorescence(NIRF), and inductively coupled plasma-mass spectrometry(ICP-MS). In in vivo analyses, the same labeled cells were implanted in a sham group and a stroke group at different times and under different MNP concentrations(after 4 h or 6 d of cell implantation) to evaluate the sensitivity of triple-modal *** hBM-MSC collection and isolation after immunophenotypic characterization were demonstrated to be adequate in hBM samples. After transduction of these cells with

关键词： Multimodal nanoparticles Human bone marrow mesenchymal stem cells Near-infrared fluorescence image Magnetic resonance image Bioluminescence Stroke

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Feasibility and safety of autologous bone marrow mononuclear cell transplantation in patients with advanced chronic liver disease

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World Journal of Gastroenterology 2007年第7期13卷 1067-1073页

作者： Andre Castro lyra Milena botelho Pereira Soares luiz Flavio Maia da Silva Marcos Fraga Fortes André Goyanna Pinheiro Silva Augusto César de Andrade Mota Sheilla A Oliveira eduardo lorens Braga Wilson Andrade de Carvalho Bernd Genser Ricardo Ribeiro dos Santos luiz Guilherme Costa lyra Hos-pital Sao Rafael Salvador Bahia Brazil Centro de Pesquisas Goncalo Moniz Fundacao Oswaldo Cruz Salvador Bahia Brazil BGStats Consulting Graz Austria

AIM: To evaluate the safety and feasibility of bone marrow cell (BMC) transplantation in patients with chronic liver disease on the waiting list for liver transplantation. METHODS: Ten patients (eight males) with chronic liver disease were enrolled to receive infusion of autologous bone marrow-derived cells. Seven patients were classified as Child-Pugh B and three as Child-Pugh C. Baseline assessment included complete clinical and laboratory evaluation and abdominal MRI. Approximately 50 ml of bone marrow aspirate was prepared by centrifugation in a ficoll-hypaque gradient. At least of 100 millions of mononuclear-enriched BMCs were infused into the hepatic artery using the routine technique for arterial chemoembolization for liver tumors. Patients were followed up for adverse events up to 4 mo. RESULTS: The median age of the patients was 52 years (range 24-70 years). All patients were discharged 48 h after BMC infusion. Two patients complained ofmild pain at the bone marrow needle puncture site. No other complications or specific side effects related to the procedure were observed. Bilirubin levels were lower at 1 (2.19 ± 0.9) and 4 mo (2.10 ± 1.0) after cell transplantation that baseline levels (2.78 ± 1.2). Albumin levels 4 mo after BMC infusion (3.73 ± 0.5) were higher than baseline levels (3.47 ± 0.5). International normalized ratio (INR) decreased from 1.48 (SD = 0.23) to 1.43 (SD = 0.23) one month after cell transplantation. CONCLUSION: BMC infusion into hepatic artery of patients with advanced chronic liver disease is safe and feasible. In addition, a decrease in mean serum bilirubin and INR levels and an increase in albumin levels are observed. Our data warrant further studies in order to evaluate the effect of BMC transplantation in patients with advanced chronic liver disease.

关键词：可行性分析安全性能自体骨髓干细胞细胞移植慢性肝疾病

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