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Human adenovirus associated with severe cold agglutinin syndrome: a rare complication in Pediatrics syndrome
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Sion, Julia Loureiro

; Flores, Angelica Lucía Hidalgo

; Cardoso, Regina Aparecida

; Garanito, Marlene Pereira

Revista Paulista de Pediatria

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2024, Volume 42 elocation e2022174

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

RESUMO Objetivo: Descrever o primeiro caso pediátrico de síndrome da crioaglutinina desencadeado por adenovírus humano e revisar a literatura. Descrição do caso: Paciente do sexo feminino, dois anos e seis meses, previamente hígida e diagnosticada com adenovírus humano isolado em swab nasal. Após 72 horas da admissão, a paciente evoluiu com quadro de anemia grave (hemoglobina de 2,6 g/dL). Os achados laboratoriais foram compatíveis com síndrome da crioaglutinina. A paciente recebeu transfusão de concentrado de hemácias, suplementação vitamínica, hidratação adequada e proteção térmica. Em seu último retorno ambulatorial, um ano após a apresentação inicial, permanecia clinicamente bem, sem sinais de hemólise. Comentários: Enquanto a síndrome da crioaglutinina grave é extremamente incomum na emergência pediátrica, a infecção por adenovírus humano é um quadro comum na infância. Recentemente, o adenovírus tem sido associado a novas complicações, e pediatras e hematologistas devem ficar atentos à possibilidade de uma evolução incomum dessa infecção e dos sinais e sintomas que possam necessitar de atenção urgente. No caso apresentado, a suspeita da complicação hematológica foi a chave para o diagnóstico precoce e seu manejo adequado. Objetivo literatura feminino meses nasal 7 admissão hemoglobina 26 2 6 2, g/dL. gdL g/dL . g dL g/dL) hemácias vitamínica térmica ambulatorial inicial bem hemólise Comentários pediátrica infância Recentemente complicações urgente apresentado adequado

ABSTRACT Objective: The objectives of this study were to describe the first pediatric case of cold agglutinin syndrome (CAS) triggered by human adenovirus and review the literature. Case description: This case report involves a previously healthy, 2½-year-old female child with human adenovirus isolated in a nasal swab. At 72 h after admission, the patient progressed to a severe episode of anemia (hemoglobin level: 2.6 g/dL). The laboratory findings were consistent with CAS. The patient received blood transfusion, vitamin supplementation, adequate hydration, and thermal protection. At her last follow-up, 1 year after her initial presentation, she remains clinically well without signs of hemolysis. Comments: While severe CAS is extremely uncommon in the pediatric emergency department, human adenovirus infection is a common illness in pediatrics. Recently, the adenovirus has been associated with new complications (acute hepatitis and fulminant liver failure). Pediatric physicians and hematologists should be aware of unusual evolution, signs, and symptoms of this infection that warrant more urgent medical attention. In this case, the hematologic complication suspicion was the key to early diagnosis and adequate management. Objective (CAS literature description healthy 2½yearold yearold 2½ old swab 7 admission hemoglobin level 26 2 6 2. g/dL. gdL g/dL . g dL g/dL) transfusion supplementation hydration protection followup, followup follow up, up follow-up presentation hemolysis Comments department pediatrics Recently acute failure. failure failure) evolution attention management

https://doi.org/10.1590/1984-0462/2024/42/2022174

Coexistent sickle cell anemia and autoimmune hemolytic anemia in two adolescents
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Soares, Vinícius Reis

; Blanco, Bruna Paccola

; Dinardo, Carla Luana

; Garanito, Marlene Pereira

einstein (São Paulo)

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2024, Volume 22 elocation eRC1105

Resumo: En | Texto: En | PDF: En

ABSTRACT The development of alloantibodies or autoantibodies is a complication observed in sickle cell disease. Autoimmunization occurs in 7.6-12% of chronically or intermittently transfused patients with sickle cell disease; however, the clinical implications of autoAbs are unclear. Few studies have focused on pediatric sickle cell disease and autoimmune hemolytic anemia. Herein, we present the coexistence of sickle cell disease and autoimmune hemolytic anemia in two adolescent patients, focusing on their pathophysiology, diagnosis, clinical management, and outcomes. 7.612% 7612 7.6 12% 7 6 12 7.6-12 however unclear Herein pathophysiology diagnosis management outcomes 612 7.612 761 76 7. 1 7.6-1 61 7.61 7.6-

https://doi.org/10.31744/einstein_journal/2024rc1105

Pulmonary embolism in pediatrics: A 10-year experience from a tertiary center in Brazil pediatrics 10year year 10 1
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Lira, Liana Ariel de Siqueira

; Celeste, Daniele Martins

; Garanito, Marlene Pereira

; Carneiro, Jorge David Aivazoglou

Hematology, Transfusion and Cell Therapy

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Jul 2023, Volume 45 Supl. 2 Páginas S85 - S90

Resumo: En | Texto: En | PDF: En

ABSTRACT Introduction: Although still rare, pulmonary embolism (PE) in children has been increasing over the years. Data regarding this group of patients are still sparse, which contributes to the lack of standardized prophylaxis protocols and the misdiagnosis. This study aimed to determine the incidence of pediatric PE at a Brazilian tertiary hospital, describe clinical characteristics and identify possible risk factors. We also analyzed the diagnosis and management of PE. Methods: This was a retrospective review of tertiary Brazilian single-center data of all pediatric patients (0 – 18 years) with acute PE, diagnosed radiologically, from September 2009 to May 2019. Results: The incidence of PE was 3.3 cases per 10,000 hospitalized children. All the twenty-three cases had some risk factor identified and sixteen of them (69.5%) had more than one risk factor. The most important were central venous catheter (39.1%), malignancy (34.8%) and recent surgery (34.8%). Among the children with identifiable symptoms (69.5%), the most common was dyspnea (56.2%). Only one patient did not receive antithrombotic therapy because of the high bleeding risk and most patients (70.6%) were treated for 3 to 6 months. Among the nineteen patients alive at the end of the six-month follow-up, ten (52.6%) repeated the PE image control. Seven of them (70.0%) had complete or partial resolution of the thrombosis and none had worsening images. Conclusion: Our lower incidence than that of the current literature may reflect underdiagnosis due to low suspicion of PE. At least one risk factor was identified in all patients, which emphasizes the importance of increasing awareness of high-risk children. Introduction rare (PE years sparse misdiagnosis hospital factors Methods singlecenter single center 0 ( 1 radiologically 200 2019 Results 33 3. 10000 10 000 10,00 twentythree twenty three 69.5% 695 69 5 (69.5% 39.1%, 391 39.1% , 39 (39.1%) 34.8% 348 34 8 (34.8% 34.8%. . 69.5%, 56.2%. 562 56.2% 56 2 (56.2%) 70.6% 706 70 (70.6% months sixmonth six month followup, followup follow up, up follow-up 52.6% 526 52 (52.6% control 70.0% 700 (70.0% images Conclusion highrisk 20 201 1000 00 10,0 69.5 (69.5 39.1 (39.1% 34.8 (34.8 56.2 (56.2% 70.6 7 (70.6 52.6 (52.6 70.0 (70.0 100 10, 69. (69. 39. (39.1 34. (34. 56. (56.2 70. (70. 52. (52. (69 (39. (34 (56. (70 (52 (6 (39 (3 (56 (7 (5

https://doi.org/10.1016/j.htct.2022.02.005

Safety and efficacy of romiplostim in children and adolescents with Immune thrombocytopenia: A systematic review thrombocytopenia
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Oliveira, Fernanda Lubiana de

; Sequeira, Fernanda Silva

; Garanito, Marlene Pereira

Hematology, Transfusion and Cell Therapy

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Mar 2023, Volume 45 Nº 1 Páginas 83 - 89

Resumo: En | Texto: En | PDF: En

Abstract Objective To evaluate the efficacy and safety of romiplostim (thrombopoietin-receptor agonist) in the treatment of pediatric immune thrombocytopenia (ITP). Methods Searches were conducted in MEDLINE, EMBASE, LILACS, Cochrane Central Register of Controlled Trials and ClinicalTrials.gov (from January 2011 to August 2021). Randomized controlled trials (RCTs), double-blind, comparing romiplostim with a placebo in pediatric persistent or chronic ITP were included. The primary outcome was the overall response rate (platelets ≥ 50 × 109/L) in the absence of rescue therapy for at least two consecutive weeks. The secondary endpoints were the minimization of clinically significant bleeding and the necessity for rescue treatments and the maximization of safety (incidence of overall adverse events) and durable response (maintaining platelet counts for at least twelve weeks). Results Two double-blind randomized placebo-controlled trials (84 participants) were included in this systematic review. Our data showed that, compared to the placebo group, the proportion of patients achieving durable platelet response was significantly higher in the romiplostim group (p= 0.003, RR = 6.34, 95%CI = 1.89 - 21.23), as was the overall response in the romiplostim group (p= 0.002, RR = 3.62, 95%CI = 1.63 - 8.03). Significant bleeding incidents (p= 0.49), overall adverse events (p= 0.71) and the need for rescue treatment (p= 0.13) were not statistically different between the romiplostim and placebo groups. Conclusions Romiplostim might improve both durable and overall platelet response in children and adolescents with ITP, compared to a placebo. More clinical trials are needed to evaluate the efficacy and safety of romiplostim and to compare it with other second-line treatments that are being used in pediatric ITP. thrombopoietinreceptor thrombopoietin receptor agonist . (ITP) MEDLINE EMBASE LILACS ClinicalTrialsgov ClinicalTrials gov from 201 2021. 2021 2021) RCTs, RCTs , (RCTs) doubleblind, doubleblind double blind, blind platelets 5 109/L 109L L 109 weeks incidence maintaining weeks) placebocontrolled 84 (8 participants review p= p (p 0003 0 003 0.003 634 6 34 6.34 95CI CI 95 189 1 89 1.8 21.23, 2123 21.23 21 23 21.23) 0002 002 0.002 362 3 62 3.62 163 63 1.6 8.03. 803 8.03 8 03 8.03) 0.49, 049 0.49 49 0.49) 0.71 071 71 0.13 013 13 groups secondline second line (ITP 20 202 (RCTs 10 ( 000 00 0.00 6.3 9 18 1. 212 21.2 2 36 3.6 16 80 8.0 04 0.4 4 0.7 07 7 0.1 01 0.0 6. 21. 3. 8. 0.

https://doi.org/10.1016/j.htct.2022.09.1275

Pediatric Evans Syndrome: A 20-year experience from a tertiary center in Brazil Syndrome 20year year 20 2
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Blanco, Bruna Paccola

; Garanito, Marlene Pereira

Hematology, Transfusion and Cell Therapy

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2023, Volume 45 Nº 2 Páginas 196 - 203

Resumo: En | Texto: En | PDF: En

Abstract Introduction The Evans syndrome (ES) is a rare, often chronic, relapsing and treatment-refractory hematological disorder. We described the clinical features, diagnostic workup, treatment and outcome in patients with ES. Method We performed a retrospective chart review of patients aged < 18 years with ES admitted to a tertiary center in Brazil from 2001 to 2021. The analysis of the data was primarily descriptive, using median, interquartile range and categorical variables presented in absolute frequencies. Main results Twenty patients (12 female, 8 male) were evaluated in this study. The median age at the initial cytopenia was 4.98 years (1.30-12.57). The ES was secondary in nine cases (45%), of which six patients (30%) showed autoimmune disease (AID) or primary immunodeficiencies (PID) and one presented a spontaneous recovery. Steroids and intravenous immunoglobulin were first-line therapy in 19 cases. Twelve patients (63%) required second-line treatments (rituximab, cyclosporine, splenectomy, sirolimus, cyclophosphamide, mycophenolate mofetil, azathioprine and eltrombopag). The median follow-up period was 2.41 years (1.4 -7.52). One patient (5%) died of underlying neuroblastoma, one case (5%) was lost to follow-up and four patients (20%) received a medical discharge. The median age for the 14 remaining cases was 12.6 years. Twelve patients (85.7%) were in complete response (CR) with no therapies. Two patients (14.3%) were in CR with chronic therapy. Conclusion As ES may be a symptom of AID and PID, a thorough rheumatological, immunologic and genetic workup and a careful follow-up are essential. The second-line treatment remains a dilemma. Further prospective studies are needed to address the optimal therapeutic combinations, morbidity and mortality in this disorder. (ES rare treatmentrefractory refractory disorder features 1 200 2021 descriptive frequencies 12 (1 female male study 498 4 98 4.9 1.3012.57. 1301257 1.30 12.57 . 30 57 (1.30-12.57) 45%, 45 45% , (45%) 30% (30% (AID PID (PID recovery firstline first line 63% 63 (63% secondline second rituximab, rituximab (rituximab cyclosporine splenectomy sirolimus cyclophosphamide mofetil eltrombopag. eltrombopag eltrombopag) followup follow up 241 2 41 2.4 1.4 (1. 7.52. 752 7.52 7 52 -7.52) 5% 5 (5% neuroblastoma 20% 20 (20% discharge 126 6 12. 85.7% 857 85 (85.7% (CR therapies 14.3% 143 3 (14.3% rheumatological essential dilemma combinations 202 ( 49 9 4. 3012 1.3012.57 130125 130 1.3 1257 12.5 (1.30-12.57 (45% (30 (63 24 2. 1. 75 7.5 -7.52 (5 (20 85.7 (85.7 14.3 (14.3 301 1.3012.5 13012 13 125 (1.30-12.5 (45 (3 (6 7. -7.5 (2 85. (85. 14. (14. 1.3012. 1301 (1.30-12. (4 -7. (85 (14 1.3012 (1.30-12 -7 (8 1.301 (1.30-1 - (1.30- (1.30 (1.3

https://doi.org/10.1016/j.htct.2022.01.011

Prevalence and outcomes of thrombotic and hemorrhagic complications in pediatric acute promyelocytic leukemia in a tertiary Brazilian center
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Antunes, Alexandre de Albuquerque

; Breviglieri, Carla Nolasco Monteiro

; Celeste, Daniele Martins

; Garanito, Marlene Pereira

; Cristofani, Lilian Maria

; Carneiro, Jorge David Aivazoglou

Hematology, Transfusion and Cell Therapy

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Set 2021, Volume 43 Nº 3 Páginas 309 - 312

Resumo: En | Texto: En | PDF: En

Abstract Introduction: Little attention is given to thrombosis associated with pediatric acute promyelocytic leukemia (APL). This study describes the thrombotic and hemorrhagic manifestations of APL in pediatric patients and evaluates their hemostasis, based on coagulation tests. Methods: Inclusion criteria were age 0–18 years and APL diagnosis between April 2005 and November 2017. Patients who had received blood transfusion prior to coagulation tests were excluded. Baseline coagulation tests, hematologic counts, and hemorrhagic/thrombotic manifestations were evaluated. Results: Median age was 10.7 years (1–15 years). The initial coagulation tests revealed a median Hgb of 8.3 g/dL (4.7–12.9 g/dL), median leucocyte count of 10.9 × 109/L (1.1–95.8 × 109/L), median platelet count of 31.8 × 109/L (2.0–109.0 × 109/L), median activated partial thromboplastin time (aPTT) of 31.7 s (23.0–50.4 s), median aPTT ratio of 1.0 (0.78–1.6), median thromboplastin time (PT) of 17.5 s (13.8–27.7 s), median PT activity of 62% (25–95 %), and median fibrinogen of 157.7 mg/dL (60.0–281.0 mg/dL). Three patients (13%) had thrombosis. At diagnosis, 21 patients (91.3%) had bruising, one patient (4.3%) had splenic vein and artery thrombosis and one patient (4.3%) presented without thrombohemorrhagic manifestations. During treatment, two patients (8.6%) had thrombosis. Conclusion: Knowledge of thrombosis in pediatric APL is important to determine its risk factors and the best way to treat and prevent this complication.

https://doi.org/10.1016/j.htct.2020.06.017 17 downloads

Differences in children and adolescents with SARS-CoV-2 infection: a cohort study in a Brazilian tertiary referral hospital
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Marques, Heloisa Helena de Sousa

; Pereira, Maria Fernanda Badue

; Santos, Angélica Carreira dos

; Fink, Thais Toledo

; Paula, Camila Sanson Yoshino de

; Litvinov, Nadia

; Schvartsman, Claudio

; Delgado, Artur Figueiredo

; Gibelli, Maria Augusta Bento Cicaroni

; Carvalho, Werther Brunow de

; Odone Filho, Vicente

; Tannuri, Uenis

; Carneiro-Sampaio, Magda

; Grisi, Sandra

; Duarte, Alberto José da Silva

; Antonangelo, Leila

; Francisco, Rossana Pucineli Vieira

; Okay, Thelma Suely

; Batisttella, Linamara Rizzo

; Carvalho, Carlos Roberto Ribeiro de

; Brentani, Alexandra Valéria Maria

; Silva, Clovis Artur

; Eisencraft, Adriana Pasmanik

; Rossi Junior, Alfio

; Fante, Alice Lima

; Cora, Aline Pivetta

; Reis, Amelia Gorete A. de Costa

; Ferrer, Ana Paula Scoleze

; Andrade, Anarella Penha Meirelles de

; Watanabe, Andreia

; Gonçalves, Angelina Maria Freire

; Waetge, Aurora Rosaria Pagliara

; Silva, Camila Altenfelder

; Ceneviva, Carina

; Lazari, Carolina dos Santos

; Abellan, Deipara Monteiro

; Santos, Emilly Henrique dos

; Sabino, Ester Cerdeira

; Bianchini, Fabíola Roberta Marim

; Alcantara, Flávio Ferraz de Paes

; Ramos, Gabriel Frizzo

; Leal, Gabriela Nunes

; Rodriguez, Isadora Souza

; Pinho, João Renato Rebello

; Carneiro, Jorge David Avaizoglou

; Paz, Jose Albino

; Ferreira, Juliana Carvalho

; Ferranti, Juliana Ferreira

; Ferreira, Juliana de Oliveira Achili

; Framil, Juliana Valéria de Souza

; Silva, Katia Regina da

; Kanunfre, Kelly Aparecida

; Bastos, Karina Lucio de Medeiros

; Galleti, Karine Vusberg

; Cristofani, Lilian Maria

; Suzuki, Lisa

; Campos, Lucia Maria Arruda

; Perondi, Maria Beatriz de Moliterno

; Diniz, Maria de Fatima Rodrigues

; Fonseca, Maria Fernanda Mota

; Cordon, Mariana Nutti de Almeida

; Pissolato, Mariana

; Peres, Marina Silva

; Garanito, Marlene Pereira

; Imamura, Marta

; Dorna, Mayra de Barros

; Luglio, Michele

; Rocha, Mussya Cisotto

; Aikawa, Nadia Emi

; Degaspare, Natalia Viu

; Sakita, Neusa Keico

; Udsen, Nicole Lee

; Scudeller, Paula Gobi

; Gaiolla, Paula Vieira de Vincenzi

; Severini, Rafael da Silva Giannasi

; Rodrigues, Regina Maria

; Toma, Ricardo Katsuya

; Paula, Ricardo Iunis Citrangulo de

; Palmeira, Patricia

; Forsait, Silvana

; Farhat, Sylvia Costa Lima

; Sakano, Tânia Miyuki Shimoda

; Koch, Vera Hermina Kalika

; Cobello Junior, Vilson

Clinics

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2021, Volume 76 elocation e3488

Resumo: En | Texto: En | PDF: En

OBJECTIVES: To compare demographic/clinical/laboratory/treatments and outcomes among children and adolescents with laboratory-confirmed coronavirus disease 2019 (COVID-19). METHODS: This was a cross-sectional study that included patients diagnosed with pediatric COVID-19 (aged <18 years) between April 11, 2020 and April 22, 2021. During this period, 102/5,951 (1.7%) of all admissions occurred in neonates, children, and adolescents. Furthermore, 3,962 severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) detection samples were processed in patients aged <18 years, and laboratory-confirmed COVID-19 occurred in 155 (4%) inpatients and outpatients. Six/155 pediatric patients were excluded from the study. Therefore, the final group included 149 children and adolescents (n=97 inpatients and 52 outpatients) with positive SARS-CoV-2 results. RESULTS: The frequencies of sore throat, anosmia, dysgeusia, headache, myalgia, nausea, lymphopenia, pre-existing chronic conditions, immunosuppressive conditions, and autoimmune diseases were significantly reduced in children and adolescents (p<0.05). Likewise, the frequencies of enoxaparin use (p=0.037), current immunosuppressant use (p=0.008), vasoactive agents (p=0.045), arterial hypotension (p<0.001), and shock (p=0.024) were significantly lower in children than in adolescents. Logistic regression analysis showed that adolescents with laboratory-confirmed COVID-19 had increased odds ratios (ORs) for sore throat (OR 13.054; 95% confidence interval [CI] 2.750-61.977; p=0.001), nausea (OR 8.875; 95% CI 1.660-47.446; p=0.011), and lymphopenia (OR 3.575; 95% CI 1.355-9.430; p=0.010), but also had less hospitalizations (OR 0.355; 95% CI 0.138-0.916; p=0.032). The additional logistic regression analysis on patients with preexisting chronic conditions (n=108) showed that death as an outcome was significantly associated with pediatric severe acute respiratory syndrome (SARS) (OR 22.300; 95% CI 2.341-212.421; p=0.007) and multisystem inflammatory syndrome in children (MIS-C) (OR 11.261; 95% CI 1.189-106. 581; p=0.035). CONCLUSIONS: Half of the laboratory-confirmed COVID-19 cases occurred in adolescents. Individuals belonging to this age group had an acute systemic involvement of SARS-CoV-2 infection. Pediatric SARS and MIS-C were the most important factors associated with the mortality rate in pediatric chronic conditions with COVID-19.

https://doi.org/10.6061/clinics/2021/e3488

ADOLESCENT PATIENTS AND THE CLINICAL DECISION ABOUT THEIR HEALTH
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Garanito, Marlene Pereira

; Zaher-Rutherford, Vera Lucia

Revista Paulista de Pediatria

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Dez 2019, Volume 37 Nº 4 Páginas 503 - 509

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

RESUMO Objetivo: Realizar uma revisão da literatura sobre a participação do adolescente na tomada de decisão sobre a sua saúde. Fonte de dados: Revisão nos bancos Scientific Electronic Library Online (SciELO), Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS) e PubMed. Consideramos artigos científicos e livros entre 1966 e 2017. Palavras-chave: adolescência, autonomia, bioética e adolescência, autonomia, ética, em línguas inglesa, portuguesa e espanhola. Critérios de inclusão: artigos científicos, livros e dissertações que contemplassem a tomada de decisão clínica pelo paciente adolescente. Critérios de exclusão: relatos de caso e artigos que não abordavam a questão norteada nesta pesquisa. Do total de 1.590 resumos, 78 foram lidos na íntegra, e 36, utilizados neste manuscrito. Síntese dos dados: A idade em que o indivíduo é capaz para tomar decisões é motivo de debate na literatura. O desenvolvimento de um sistema cognitivo e psicossocial é um processo demorado, e faz-se fundamental a integração da investigação psicológica, neuropsicológica e neurobiológica na adolescência. A capacidade de reflexão madura não é determinada pela idade cronológica; em teoria, um menor maduro seria capaz de consentir ou recusar um tratamento. A tomada de decisão exige análise cuidadosa e reflexiva dos principais fatores associados, e a abordagem desse problema deve ocorrer por meio do reconhecimento da maturidade e da autonomia que existe no adolescente. Para tanto, é necessário “deliberar” com ele. Conclusões: Diretrizes internacionais recomendam que os adolescentes participem de discussões sobre sua doença, tratamento e tomada de decisão, entretanto não há nenhum consenso universalmente aceito sobre como avaliar a capacidade de decisão desses pacientes. Apesar disso, quando possível, o adolescente deve ser incluído em um processo sério, honesto, respeitoso e sincero de deliberação.

ABSTRACT Objective: To carry out a review of the literature on adolescents’ participation in decision making for their own health. Data sources: Review in the Scientific Electronic Library Online (SciELO), Latin American and Caribbean Health Sciences Literature (LILACS) and PubMed databases. We consider scientific articles and books between 1966 and 2017. Keywords: adolescence, autonomy, bioethics and adolescence, autonomy, ethics, in variants in the English, Portuguese and Spanish languages. Inclusion criteria: scientific articles, books and theses on clinical decision making by the adolescent patient. Exclusion criteria: case reports and articles that did not address the issue. Among 1,590 abstracts, 78 were read in full and 32 were used in this manuscript. Data synthesis: The age at which the individual is able to make decisions is a matter of debate in the literature. The development of a cognitive and psychosocial system is a time-consuming process and the integration of psychological, neuropsychological and neurobiological research in adolescence is fundamental. The ability to mature reflection is not determined by chronological age; in theory, a mature child is able to consent or refuse treatment. Decision-making requires careful and reflective analysis of the main associated factors, and the approach of this problem must occur through the recognition of the maturity and autonomy that exists in the adolescents. To do so, it is necessary to “deliberate” with them. Conclusions: International guidelines recommend that adolescents participate in discussions about their illness, treatment and decision-making. However, there is no universally accepted consensus on how to assess the decision-making ability of these patients. Despite this, when possible, the adolescent should be included in a serious, honest, respectful and sincere process of deliberation.

https://doi.org/10.1590/1984-0462/;2019;37;4;00011 1294 downloads

A espiritualidade na prática pediátrica
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Garanito, Marlene Pereira

; Cury, Marina Rachel Graminha

Revista Bioética

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Abr 2016, Volume 24 Nº 1 Páginas 49 - 53

Resumo: Pt En Es | Texto: Pt En Es | PDF: Pt | PDF: En | PDF: Es

Resumo O tema dos cuidados paliativos na prática pediátrica ocupa relevante espaço de discussão no contexto da bioética. Nesse aspecto, destacamos que a dimensão espiritual é parte integrante do cuidado ao paciente pediátrico e que, portanto, a equipe de saúde precisa estar apta a acolher esse movimento de transcendência e atenta às necessidades espirituais desses pacientes, bem como de seus responsáveis.

Abstract The theme of palliative care in pediatric practice represents a relevant topic for discussion in the context of bioethics. In this respect, we seek to emphasize that the spiritual dimension is an integral part of the care of pediatric patients, and therefore health teams need to be able to embrace this transcendent movement and be attentive to the spiritual needs of pediatric patients, as well as their caregivers.

Resumen El tema de los cuidados paliativos en la práctica pediátrica ocupa un espacio relevante de discusión en el contexto de la bioética y, en este sentido, hacemos hincapié en que la dimensión espiritual es una parte integrante de la atención a los pacientes pediátricos y por lo tanto el equipo de salud debe ser capaz de acoger este movimiento de trascendencia y permanecer atento a las necesidades espirituales de los pacientes pediátricos, así como a las de sus padres.

https://doi.org/10.1590/1983-80422016241105 7796 downloads

10.

Outcome of children with severe acquired aplastic anemia treated with rabbit antithymocyte globulin and cyclosporine A,
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Garanito, Marlene Pereira

; Carneiro, Jorge David Aivazoglou

; Odone Filho, Vicente

; Scheinberg, Phillip

Jornal de Pediatria

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Out 2014, Volume 90 Nº 5 Páginas 523 - 527

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Objetivo:Avaliar o resultado de crianças com anemia aplástica grave adquirida tratadas com globulina antitimocítica de coelho e ciclosporina como tratamento inicial em nosso instituto.Métodos:Análise retrospectiva de 26 pacientes pediátricos com anemia aplástica tratados entre 1996 e 2011 com globulina antitimocítica de coelho e ciclosporina.Resultados:A taxa de resposta geral em seis meses foi de 34,6% (9/26), e a incidência acumulada de recorrência foi de 26,5% (intervalo de confiança [IC] de 95%,1,4%-66%) em cinco anos. A incidência acumulada de evolução clonal após a terapia imunossupressora foi de 8,3% (IC 95%, 0,001%-53,7%) em cinco anos, com ambas as evoluções clonais em pacientes sem resposta que adquiriram o cariótipo com monossomia 7. A sobrevida geral em cinco anos foi de 73,6% (IC 95%, 49,2%-87,5%).Conclusões:Nossos resultados confirmam a baixa taxa de resposta com globulina antitimocítica de coelho como terapia inicial em pacientes pediátricos, da mesma forma como relatado para pacientes de todas as idades. Essa confirmação é problemática em nosso país devido à falta de globulina antitimocítica de cavalo em muitos mercados fora dos Estados Unidos, incluindo o Brasil.

Objective:To evaluate the outcome of children with severe acquired aplastic anemia treated with rabbit antithymocyte globulin and cyclosporine as first-line treatment at this institution.Methods:Retrospective analysis of 26 pediatric patients with aplastic anemia, treated between 1996 and 2011 with rabbit antithymocyte globulin plus cyclosporine.Results:The overall response rate at six months was 34.6% (9/26), and the cumulative incidence of relapse was 26.5% (95% confidence interval [CI]: 1.4%-66%) at 5 years. The cumulative incidence of clonal evolution after immunosuppressive therapy was 8.3% (95% CI: 0.001%-53.7%) at five years with both clonal evolutions in non-responders who acquired monosomy 7 karyotype. The overall survival at five years was 73.6% (95% CI: 49.2%-87.5%).Conclusions:The present results confirm the poor response rate with rabbit antithymocyte globulin as first therapy in pediatrics patients, similar to what has been reported for patients of all ages. This confirmation is problematic in Brazil, given the lack of horse antithymocyte globulin in many markets outside the United States.

https://doi.org/10.1016/j.jped.2014.02.004 3266 downloads

11.

Essential thrombocythemia: a rare disease in childhood
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Beatrice, Julia Maimone

; Garanito, Marlene Pereira

Revista Brasileira de Hematologia e Hemoterapia

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2013, Volume 35 Nº 4 Páginas 287 - 289

Resumo: En | Texto: En | PDF: En

Essential thrombocythemia is an acquired myeloproliferative disorder characterized by the proliferation of megakaryocytes in bone marrow, leading to a persistent increase in the number of circulating platelets and thus increasing the risk for thrombotic and hemorrhagic events. The disease features leukocytosis, splenomegaly, vascular occlusive events, hemorrhages and vasomotor disorders. The intricate mechanisms underlying the molecular pathogenesis of this disorder are not completely understood and are still a matter of discussion. Essential thrombocythemia is an extremely rare disorder during childhood. We report on a case of essential thrombocythemia in a child and discuss the diagnostic approach and treatment strategy.

https://doi.org/10.5581/1516-8484.20130059 2100 downloads Citado 1 vez em SciELO

12.

Valproato de sódio: efeitos colaterais em crianças
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Garanito, Marlene Pereira

; Rothschild, Cynthia

; Camlofski, Fernanda Glaucia L.

; Cabral, Edi

Revista Paulista de Pediatria

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Dez 2009, Volume 27 Nº 4 Páginas 456 - 460

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

OBJETIVO: O valproato de sódio é um fármaco anticonvulsivante usado com frequência no manejo dos quadros epilépticos refratários na infância e habitualmente apresenta boas taxas de resposta e toxicidade aceitável. O objetivo deste artigo é destacar os efeitos colaterais secundários ao uso prolongado de valproato de sódio em uma criança e alertar para a importância do acompanhamento dos pacientes em uso crônico de anticonvulsivantes. DESCRIÇÃO DO CASO: Criança do sexo masculino, com quatro anos e oito meses, portadora de síndrome de West, em uso de valproato de sódio desde os seis meses de idade, admitida no hospital com anemia, macrocitose, plaquetopenia, deficiência de vitamina B12, hipoalbuminemia e hiponatremia. Durante a internação e o acompanhamento ambulatorial, relacionou-se o quadro clínico ao uso do valproato de sódio. COMENTÁRIOS: A toxicidade hematológica do valproato de sódio é bem conhecida e comum, podendo variar em relação à época de instalação e gravidade. Os achados mais frequentes são a plaquetopenia e a macrocitose. Além das alterações hematológicas, a literatura relata outros efeitos colaterais relacionados ao uso do fármaco. Destaca-se a importância do conhecimento e monitorização dos eventos adversos nos pacientes submetidos a esse tipo de terapia, para que eles possam ser detectados e tratados o mais precocemente.

OBJECTIVE: Valproic acid is an anticonvulsant frequently used in the management of childhood refractory epilepsy with good clinical responses and acceptable toxicity. The objective of this case report is to describe side effects associated with long-term valproate therapy in a child and to warn about the importance of monitoring patients in chronic use of anticonvulsants. CASE DESCRIPTION: This male child, four years and eight months old, with West syndrome, had been using sodium valproate since six months of age. The patient was admitted with anemia, macrocytosis, thrombocytopenia, deficiency of vitamin B12, hypoalbuminemia and hyponatremia. The clinical signs were associated to chronic sodium valproate use. COMMENTS: Sodium valproate hematologic toxicity is frequent, varying in onset and severity. The most common findings are thrombocytopenia and macrocytosis. In addition to the hematological toxicity, literature reports other side effects associated with valproic acid therapy. It is important to know and to monitor adverse effects in patients undergoing sodium valproate therapy in order to detect and treat them as early as possible.

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