39 results on '"Natascha Silva SANDY"'
Search Results
2. Management of children with functional constipation referred to tertiary care
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Giovanna Roberta Camargo de Campos, Natascha Silva Sandy, Elizete Aparecida Lomazi, and Maria Angela Bellomo-Brandao
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Constipation ,Child ,Functional gastrointestinal disorders ,Childcare ,Health care surveys ,Pediatrics ,RJ1-570 - Abstract
Objectives: To describe the management, to compare treatment at initial referral vs. during specialized follow-up, and to describe outcomes of children with functional constipation (FC) referred to a Brazilian tertiary care center. Methods: Retrospective study, including children (4–18 years) with FC followed at a single center from 2006 to 2019. Demographics, treatments, time of follow-up, and outcomes were analyzed. The management of FC followed an institutional protocol. Results: 104 patients were identified, 79 were eligible and included in the analysis: 59% male, mean age at referral was 6.4 years, and mean duration of symptoms was 4.4 years. There were significant changes in the therapy(ies) used at the time of referral compared to during follow-up, with a noticeable increase in the frequency of the use of polyethylene glycol, enemas, magnesium hydroxide, and bisacodyl; 5.1% received trans-anal irrigation, and 3.8% underwent surgery. Outcomes were favorable in more than half of the cases: 31% improved; 19.5% had complete resolution and 2.5% were transferred back to primary care. Symptoms remained unchanged in 30.4%, and no patients experienced worsening of symptoms. The mean duration of follow-up was 2.8 years. When comparing patients with favorable vs. unfavorable outcomes, the authors did not identify significant differences in gender, age, therapies used, duration of symptoms, or length of follow-up. Conclusions: Children with FC are often referred to specialized care not receiving optimal therapy. Many patients whose FC was labeled “refractory” may be treated successfully with a well-established plan of care, and do not truly present intractable constipation.
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- 2022
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3. FUNCTIONAL ABDOMINAL PAIN IS THE MAIN ETIOLOGY AMONG CHILDREN REFERRED TO TERTIARY CARE LEVEL FOR CHRONIC ABDOMINAL PAIN
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Gabriela Parússolo MARTINS, Natascha Silva SANDY, Lucas Rocha ALVARENGA, Elizete Aparecida LOMAZI, and Maria Angela BELLOMO-BRANDÃO
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Children ,adolescents ,abdominal pain ,functional disorders ,Rome IV ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
ABSTRACT Background Chronic abdominal pain (CAP) carries a significant burden of disease. The last edition of the Rome Criteria (Rome IV) allows the diagnosis of functional gastrointestinal disorders (FGIDs) according to symptoms-based criteria; however, patients continue to experience a delay in their diagnosis and to be submitted to different interventions before the establishment of a positive diagnosis. Objective We aimed to characterize etiology, clinical features, and interventions in a pediatric cohort of patients with CAP secondary to FGIDs, who were referred to our tertiary care university-affiliated hospital, in Brazil. Methods A retrospective descriptive study of children and adolescents (aged 20 years and younger) referred to our institution, from January/2013 to December/2018, for CAP, and who fulfilled criteria for FGIDs classified according to Rome IV criteria. Results Three hundred twenty-eight patients with CAP were screened, of which 67.9% (223 patients) fulfilled the criteria for FGIDs and were included in the study. Sixty percent were female, with a mean age of 8.3 years. At the time of referral, the mean duration of symptoms was 2.8 years. Length/height for age and weight for age mean z-scores were -0.08±1.87 and -0.38±1.62, respectively. Functional abdominal pain not otherwise specified was overall the most common diagnosis (70.4%). Before establishing the diagnosis of FGIDs, multiple pharmacological interventions were described, while after, the mainstay of therapy was education/reassurance and dietary interventions. Thirty-two percent of patients did not further require specialized follow-up. Conclusion Even at the tertiary care level, FGIDs were still the most common etiology of chronic abdominal pain, particularly functional abdominal pain not otherwise specified. Despite the relatively long duration of symptoms at referral, cessation of specialized care follow-up was possible in approximately a third of the cases.
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- 2022
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4. Dor abdominal crônica em pediatria e consumo excessivo de FODMAPS - relato de caso
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Bruna Caseri Marino, Natascha Silva Sandy, and Maria Angela Bellomo Brandão
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pediatrics ,abdominal pain ,carbohydrates ,Pediatrics ,RJ1-570 - Abstract
Abdominal pain affects up to 45% of children aged 4 to 18 years. Functional disorders rank as the main cause. There is no consensus over what constitutes “minimal” investigation for the management of pediatric patients with chronic abdominal pain. This case report presents and discusses a case of long-term chronic abdominal pain involving a child previously submitted to extensive complementary investigation, in which detailed clinical evaluation found the patient was in a high-FODMAP diet. The introduction of dietary interventions led to symptom remission. This report demonstrates how thorough anamnesis and careful physical examination are essential in elucidating the etiology of abdominal pain.
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- 2022
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5. Artrogripose renal colestática diagnosticada a partir de colestase neonatal: relato de caso
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Bruna Caseri Marino, Natascha Silva Sandy, Gabriel Hessel, and Maria Angela Bellomo Brandão
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arthrogryposis ,cholestasis ,fanconi syndrome ,Pediatrics ,RJ1-570 - Abstract
Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare autosomal recessive syndrome, with multisystemic manifestations and mainly characterized by arthrogriposis, renal dysfunction and cholestasis. The prognosis is poor and most patients die within the first year of life. This is a case report of a female infant, 37 days old, referred to a tertiary hospital due to neonatal cholestasis. Upon evaluation, she also presented with Fanconi syndrome, arthrogryposis, malnutrition, ichthyosis and agranular platelets, thus receiving the clinical diagnosis of ARC syndrome. The liver biopsy showed signs of neonatal hepatitis. Her admission was complicated by dehydration, worsening of metabolic acidosis and acute respiratory failure. The infant was discharged at the age of 3 months, in palliative care, and later died. As it is a very rare disease, knowledge of its characteristics is crucial for appropriated diagnostic evaluation and differential diagnosis with other causes of cholestasis, as well as adequate management.
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- 2022
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6. SYMPTOMATIC CHOLELITHIASIS AS THE PRESENTATION OF PEDIATRIC PRIMARY SCLEROSING CHOLANGITIS - CASE SERIES AND LITERATURE REVIEW
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Lucas Rocha ALVARENGA, Natascha Silva SANDY, Gabriela Souza GOMEZ, Gabriel HESSEL, Adriana Maria Alves DE TOMMASO, and Maria Ângela BELLOMO-BRANDÃO
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Cholelithiasis ,sclerosing cholangitis ,biliary tract ,child ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
ABSTRACT BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.
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- 2021
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7. LONG TERM MANAGEMENT OF GLYCOGEN STORAGE DISEASE TYPE 1B: A BRAZILIAN TERTIARY CENTER EXPERIENCE
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Marina Mayumi Vendrame TAKAO, Natascha Silva SANDY, Adriana Gut Lopes RICCETTO, and Adriana Maria Alves DE TOMMASO
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Glycogen storage disease type I ,Neutropenia ,Inflammatory bowel diseases ,Immune system diseases ,Granulcyte colony-stimulating fator ,Filgrastim ,Pediatrics ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
ABSTRACT BACKGROUND Glycogen storage disease (GSD) type 1b is a multisystemic disease in which immune and infectious complications are present, in addition to the well-known metabolic manifestations of GSD. Treatment with granulocyte-colony stimulating factor (G-CSF) is often indicated in the management of neutropenia and inflammatory bowel disease. OBJECTIVE To report on the demographics, genotype, clinical presentation, management, and complications of pediatric patients with glycogen storage disease type 1b (GSD 1b), with special attention to immune-related complications. METHODS Retrospective case series of seven patients with GSD 1b diagnosed and followed at a tertiary university hospital in Brazil, from July/2000 until July/2016. RESULTS Mean age at referral was fourteen months. Diagnosis of GSD 1b was based on clinical and laboratory findings and supported by genetic studies in five cases. All patients presented suffered from neutropenia, managed with G-CSF - specifically Filgrastim. Hospitalizations for infections were frequent. Two patients developed inflammatory bowel disease. Six patients remained alive, one died at age 14 years and 9 months. The mean age at the end of the follow-up was 11.5 years. Compliance to treatment was suboptimal: poor compliance to medications, starch and dietetic management of GSD were documented, and outpatient appointments were frequently missed. CONCLUSION Managing GSD 1b is challenging not only for the chronic and multisystemic nature of this disease, but also for the additional demands related dietary restrictions, use of multiple medications and the need for frequent follow-up visits; furthermore in Brazil, the difficulties are increased in a scenario where we frequently care for patients with unfavorable socioeconomic status and with irregular supply of medications in the public health system.
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- 2021
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8. PEUTZ-JEGHERS SYNDROME IN RESOURCE-LIMITED SCENARIO
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Natascha Silva SANDY, Elizete Aparecida LOMAZI, Maria de Fátima SERVIDONI, and Maria Angela BELLOMO-BRANDÃO
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Peutz-Jeghers syndrome ,Intussusception ,Demographic data ,Child ,Diseases of the digestive system. Gastroenterology ,RC799-869 - Abstract
ABSTRACT BACKGROUND: Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant disease, caused by deletions in the chromosome 19p33.3/ gene LKB1/STK11. These mutations inactivate a serine/threonine kinase and predispose to carcinogenesis. In PJS, tumors of the gastrointestinal, testicles, pulmonary, breast, pancreas, uterus and ovaries can be found. OBJECTIVE: To evaluate demographics, clinical presentation and complication/outcomes of pediatric patients presenting with Peutz-Jeghers syndrome (PJS), as well as to present and discuss management in the context of limited resources. METHODS: We conducted a retrospective chart review of a cohort of six patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/Brazil, between 2000 and 2018. Data analyzed included gender, age of presentation, age of diagnosis, family history, PJS complications. RESULTS: Median age of diagnosis of 6.7 years, with a mean time of follow-up of 8.1 years. Mucocutaneous pigmentation was universally present. Half of the patients had a known family history at the time of diagnosis. On follow up, intestinal intussusception was documented in four out of six patients, in most (three), in different locations and in multiple occasions. The active investigation of siblings and parents of the index case led to the diagnosis of three first-degree relatives in the present case series. CONCLUSION: In this first pediatric PJS Brazilian case series, we report a wide spectrum of PJS manifestations and complications. In a resource limited scenario, despite limitations for the surveillance of complications, the relative frequency of complications was not higher than historically reported.
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- 2020
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9. Elevated IgA and IL-10 levels in very-early-onset inflammatory bowel disease secondary to IL-10 receptor deficiency
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Natascha Silva Sandy, Lia Furlaneto Marega, Giane Dantas Bechara, Adriana Gut Lopes Riccetto, Carmen Bonfim, Maria Marluce dos Santos Vilela, Antonio Fernando Ribeiro, Maria De Fatima Servidoni, and Elizete Aparecida Lomazi
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Inflammatory bowel diseases ,Whole exome sequencing ,Genetic techniques ,Primary immunodeficiency diseases ,Child ,Pediatrics ,RJ1-570 - Abstract
ABSTRACT Objective: To report two patients with very-early-onset inflammatory bowel disease (VEOIBD) secondary to interleukin-10 receptor (IL-10R) mutations, explore immunophenotyping data and plasma cytokine profile on these cases compared to healthy controls, and describe the phenotype of IL-10/IL-10R mutations based on a literature review. Case description: We report on two female infants referred to our tertiary center at the age of ten months, with severe colonic and perianal disease, as well as significant malnutrition, who had shown limited response to usual inflammatory bowel disease (IBD) therapy agents. In the first case, whole-exome sequencing (WES) revealed a homozygous (c.537G>A/p.T179T) mutation in exon 4 of the IL-10RA gene, while in the second patient, compound heterozygosity was identified, also in the IL-10RA gene (chr11:117.859.199 variant A>G/p.Tyr57Cys and chr11: 117.860.335 variant G>T/p.Val123Leu). Both patients underwent hematopoietic cell transplantation (HCT). Immunological work-up of these patients revealed increased IL-10 plasma levels and increased IgA. Comments: Our case reports disclose novel findings on plasma cytokine profile in IL-10R deficiency, and we describe the severe phenotype of IL-10/IL-10R deficiency that should be recognized by physicians.
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- 2021
- Full Text
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10. Can the learning of laparoscopic skills be quantified by the measurements of skill parameters performed in a virtual reality simulator?
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Natascha Silva Sandy, Jose Arnaldo Shiomi da Cruz, Carlo Camargo Passerotti, Hiep Nguyen, Sabrina Thalita dos Reis, Eder Maxwell Gouveia, Ricardo Jordao Duarte, Homero Bruschini, and Miguel Srougi
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Virtual Reality Exposure Therapy ,Education ,Laparoscopy ,Diseases of the genitourinary system. Urology ,RC870-923 - Abstract
Purpose To ensure patient safety and surgical efficiency, much emphasis has been placed on the training of laparoscopic skills using virtual reality simulators. The purpose of this study was to determine whether laparoscopic skills can be objectively quantified by measuring specific skill parameters during training in a virtual reality surgical simulator (VRSS). Materials and Methods Ten medical students (with no laparoscopic experience) and ten urology residents (PGY3-5 with limited laparoscopic experience) were recruited to participate in a ten-week training course in basic laparoscopic skills (camera, cutting, peg transfer and clipping skills) on a VRSS. Data were collected from the training sessions. The time that individuals took to complete each task and the errors that they made were analyzed independently. Results The mean time that individuals took to complete tasks was significantly different between the groups (p < 0.05), with the residents being faster than the medical students. The residents' group also completed the tasks with fewer errors. The majority of the subjects in both groups exhibited a significant improvement in their task completion time and error rate. Conclusion The findings in this study demonstrate that laparoscopic skills can be objectively measured in a VRSS based on quantified skill parameters, including the time spent to complete skill tasks and the associated error rate. We conclude that a VRSS is a feasible tool for training and assessing basic laparoscopic skills.
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- 2013
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11. Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria
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Elizete Aparecida Lomazi, Marina Abib Stefano, Franziska Duckstein, Jochen G Mainz, Natascha Silva Sandy, Carlos Zagoya, and Antonio Fernando Ribeiro
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Constipation ,Cystic Fibrosis ,business.industry ,MEDLINE ,medicine.disease ,Cystic fibrosis ,Gastroenterology ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Humans ,Medicine ,In patient ,medicine.symptom ,business ,Intestinal Obstruction - Published
- 2022
12. Patients with treated autoimmune hepatitis and persistent suppression of plasmacytoid dendritic cells: A different point of view
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Irene P dos Santos, Mayra T de Assunção, Renan M Mauch, Natascha Silva Sandy, Marcos Tadeu Nolasco da Silva, Maria Angela Bellomo-Brandão, and Adriana Gut Lopes Riccetto
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Pharmacology ,Hepatitis, Autoimmune ,Cross-Sectional Studies ,immune system diseases ,Immunology ,Immunology and Allergy ,Humans ,hemic and immune systems ,Forkhead Transcription Factors ,Dendritic Cells ,T-Lymphocytes, Regulatory - Abstract
Objectives: Plasmacytoid dendritic cells (pDCs) have been shown to have a role in autoimmune diseases, but their role in Autoimmune Hepatitis (AIH) is not completely clear. In the present study, we assessed the frequency of pDCs in peripheral blood of AIH patients under long-term standard immunosuppressive therapy. Methods: This cross-sectional analysis enrolled 27 AIH patients and 27 healthy controls. We analyzed and compared their proportion of pDCs, CD4+, CD8+, γδ T cells, CD25+ regulatory T (Treg) cells, FoxP3+, Foxp3+CD39+ Treg cells, total B (CD19+) cells, and plasma cells (CD38+) in peripheral blood using flow cytometry immunophenotyping. Results: AIH patients had a lower percentage of pDCs (median frequencies of 0.2% vs. 0.4%; p = .002) and higher expression of CD8 T cells (32.5% vs 28.6%; p = 0.008) in peripheral blood, when compared to healthy controls. We did not find statistically significant differences between the groups regarding the other cell subtypes.Conclusion: Our data suggest a persistent suppression of pDCs in AIH patients, along with increased CD8 T cell activity, years after AIH diagnosis and despite of good clinical response to treatment, thus pointing to a role of pDCs in the AIH pathogenesis.
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- 2022
13. Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis
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José Dirceu Ribeiro, Elizete Aparecida Lomazi, Maria de Fátima Servidoni, Antonio Fernando Ribeiro, Natascha Silva Sandy, Aline Cristina Gonçalves, and Lilian Helena Polak Massabki
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medicine.medical_specialty ,Adolescent ,Cystic Fibrosis ,Meconium Ileus ,Criança ,Therapeutics ,Gastroenterology ,Cystic fibrosis ,03 medical and health sciences ,Terapêutica ,0302 clinical medicine ,Maintenance therapy ,030225 pediatrics ,Internal medicine ,Diagnosis ,Genotype ,medicine ,Humans ,Medical history ,030212 general & internal medicine ,Child ,Retrospective Studies ,business.industry ,Diagnóstico ,lcsh:RJ1-570 ,nutritional and metabolic diseases ,lcsh:Pediatrics ,South America ,medicine.disease ,Distal intestinal obstruction syndrome ,Obstrução intestinal ,Intestinal obstruction ,Pediatrics, Perinatology and Child Health ,Exocrine Pancreatic Insufficiency ,Fibrose cística ,Presentation (obstetrics) ,medicine.symptom ,business ,Body mass index ,Intestinal Obstruction - Abstract
Objective: To evaluate the demographics, genotype, and clinical presentation of pediatric patients presenting with distal intestinal obstruction syndrome (DIOS), and factors associated with DIOS recurrence. Methods: Case series of ten patients (median age 13.2 years), followed-up in a reference center, retrospectively assessed. Data analyzed included age, gender, cystic fibrosis genotype, meconium ileus at birth, hydration status, pulmonary exacerbation, Pseudomonas aeruginosa colonization, pancreatic insufficiency (PI), body mass index (BMI) at the episodes, clinical manifestations of DIOS, imaging studies performed, acute management of DIOS, maintenance therapy, and recurrence on follow-up. Results: All patients had two positive sweat chloride tests, and nine of ten also had genotype study. The most common genotype identified was homozygosis for the delta F508 mutation. In seven cases, a previous history of meconium ileus was reported. All patients had pancreatic insufficiency. Diagnosis of DIOS was based on clinical and imaging findings. Of the total number of episodes, 85% were successfully managed with oral osmotic laxatives and/or rectal therapy (glycerin enema or saline irrigation). Recurrence was observed in five of ten patients. Conclusion: In this first report of pediatric DIOS in South America, the presence of two risk factors for DIOS occurrence was universal: pancreatic insufficiency and severe genotype. Medical history of meconium ileus at birth was present in most patients, as well as in the subgroup with DIOS recurrence. The diagnosis relied mainly on the clinical presentation and on abdominal imaging. The practices in the management of episodes varied, likely reflecting changes in the management of this syndrome throughout time. Resumo: Objetivo: Avaliar os dados demográficos, o genótipo e o quadro clínico de pacientes pediátricos que apresentam síndrome da obstrução intestinal distal (DIOS) e os fatores associados à recidiva da DIOS. Métodos: Casuística de 10 pacientes (média de 13,2 anos) monitorados em um centro de referência e avaliados de forma retroativa. Os dados analisados incluíram idade, sexo, genótipo da fibrose cística, íleo meconial no nascimento, estado de hidratação, exacerbação pulmonar, colonização por Pseudomonas aeruginosa, insuficiência pancreática (IP), IMC nos episódios, manifestações clínicas da DIOS, estudos de diagnóstico por imagem realizados, manejo agudo da DIOS, terapia de manutenção e recidiva no acompanhamento. Resultados: Todos os pacientes apresentaram dois exames de cloreto no suor positivos e 09/10 também apresentaram estudo do genótipo. O genótipo mais comum identificado foi a homozigose da mutação delta F508. Em sete casos foi mencionado um histórico de íleo meconial. Todos os pacientes apresentaram insuficiência pancreática. O diagnóstico da DIOS teve como base achados clínicos e de imagem; 85% do número total de episódios foram tratados com sucesso com laxantes osmóticos orais e/ou terapia retal (enema de glicerina ou irrigação salina). A recidiva foi observada em 5 de 10 pacientes. Conclusão: Neste primeiro relatório da DIOS pediátrica na América do Sul, a presença de dois fatores de risco na ocorrência da DIOS foi universal: insuficiência pancreática e genótipo associado a doença grave. O histórico de íleo meconial no nascimento esteve presente na maioria dos pacientes, bem como no subgrupo com recidiva da DIOS. O diagnóstico dependeu principalmente do quadro clínico e do diagnóstico por imagem abdominal. As práticas de manejo de episódios variaram, provavelmente refletiram as mudanças no tratamento dessa síndrome ao longo do tempo.
- Published
- 2020
14. Manejo da síndrome de Peutz-Jeghers no contexto de recursos limitados
- Author
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Natascha Silva Sandy, Maria Angela Bellomo-Brandão, Maria de Fátima Servidoni, and Elizete Aparecida Lomazi
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Síndrome de Peutz-Jeghers ,Pediatrics ,medicine.medical_specialty ,congenital, hereditary, and neonatal diseases and abnormalities ,Peutz-Jeghers syndrome ,Mucocutaneous zone ,Dados demográficos ,Context (language use) ,Peutz–Jeghers syndrome ,Criança ,RC799-869 ,medicine ,Humans ,Family history ,skin and connective tissue diseases ,Child ,Index case ,Retrospective Studies ,business.industry ,Gastroenterology ,Autosomal dominant trait ,Retrospective cohort study ,Demographic data ,Diseases of the digestive system. Gastroenterology ,medicine.disease ,Cohort ,Mutation ,Female ,Intussuscepção ,business ,Intussusception ,Brazil - Abstract
BACKGROUND: Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant disease, caused by deletions in the chromosome 19p33.3/ gene LKB1/STK11. These mutations inactivate a serine/threonine kinase and predispose to carcinogenesis. In PJS, tumors of the gastrointestinal, testicles, pulmonary, breast, pancreas, uterus and ovaries can be found. OBJECTIVE: To evaluate demographics, clinical presentation and complication/outcomes of pediatric patients presenting with Peutz-Jeghers syndrome (PJS), as well as to present and discuss management in the context of limited resources. METHODS: We conducted a retrospective chart review of a cohort of six patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/Brazil, between 2000 and 2018. Data analyzed included gender, age of presentation, age of diagnosis, family history, PJS complications. RESULTS: Median age of diagnosis of 6.7 years, with a mean time of follow-up of 8.1 years. Mucocutaneous pigmentation was universally present. Half of the patients had a known family history at the time of diagnosis. On follow up, intestinal intussusception was documented in four out of six patients, in most (three), in different locations and in multiple occasions. The active investigation of siblings and parents of the index case led to the diagnosis of three first-degree relatives in the present case series. CONCLUSION: In this first pediatric PJS Brazilian case series, we report a wide spectrum of PJS manifestations and complications. In a resource limited scenario, despite limitations for the surveillance of complications, the relative frequency of complications was not higher than historically reported. RESUMO CONTEXTO: A síndrome de Peutz-Jeghers (SPJ) é uma doença autossômica dominante rara, causada por deleções no cromossomo 19p33.3/gene LKB1/STK11. Essas mutações inativam uma serina/treonina quinase e predispõem à carcinogênese. Na SPJ, podem ser encontrados tumores do trato gastrointestinal, testicular, pulmonar, de mama, de pâncreas, de útero e de ovários. OBJETIVO: Avaliar dados demográficos, apresentação clínica e complicações de pacientes pediátricos que se apresentam com SPJ, além de apresentar e discutir o manejo no contexto de recursos limitados. MÉTODOS: Realizamos uma revisão retrospectiva de prontuários de uma coorte de seis pacientes, diagnosticados e/ou acompanhados no Hospital das Clínicas da Universidade de Campinas - São Paulo, Brasil, entre 2000 e 2018. Os dados analisados incluíram sexo, idade de apresentação, idade do diagnóstico, história familiar, complicações da SPJ. RESULTADOS: Idade média de diagnóstico de 6,7 anos, com tempo médio de seguimento de 8,1 anos. A pigmentação mucocutânea estava universalmente presente. Metade dos pacientes tinha um histórico familiar conhecido no momento do diagnóstico. Intussuscepção intestinal foi observada em quatro dos seis pacientes durante o período de acompanhamento, sendo que em três ocorreram vários episódios em diferentes múltiplas localizações. A investigação ativa de irmãos e pais do caso-índice levou ao diagnóstico de três parentes de primeiro grau na presente série de casos. CONCLUSÃO: Nesta primeira série de casos brasileiros de SPJ pediátrica, relatamos um amplo espectro de manifestações e complicações da SPJ. Em um contexto de recursos limitados, apesar das limitações para a vigilância de complicações, a frequência relativa de complicações não foi maior do que o relatado historicamente.
- Published
- 2020
15. Elevated IgA and IL-10 levels in very-early-onset inflammatory bowel disease secondary to IL-10 receptor deficiency
- Author
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Natascha Silva Sandy, Lia Furlaneto Marega, Giane Dantas Bechara, Adriana Gut Lopes Riccetto, Carmen Bonfim, Maria Marluce dos Santos Vilela, Antonio Fernando Ribeiro, Maria De Fatima Servidoni, and Elizete Aparecida Lomazi
- Subjects
medicine.medical_specialty ,Sequenciamento completo do exoma ,Técnicas genéticas ,Case Report ,Criança ,Doenças inflamatórias intestinais ,Inflammatory bowel diseases ,Compound heterozygosity ,Pediatrics ,Gastroenterology ,Inflammatory bowel disease ,RJ1-570 ,Exon ,Immunophenotyping ,Primary immunodeficiency diseases ,Internal medicine ,medicine ,Child ,Receptor ,Exome sequencing ,business.industry ,Whole exome sequencing ,medicine.disease ,Doenças da imunodeficiência primária ,Transplantation ,Interleukin 10 ,Pediatrics, Perinatology and Child Health ,Genetic techniques ,business - Abstract
Objective: To report two patients with very-early-onset inflammatory bowel disease (VEOIBD) secondary to interleukin-10 receptor (IL-10R) mutations, explore immunophenotyping data and plasma cytokine profile on these cases compared to healthy controls, and describe the phenotype of IL-10/IL-10R mutations based on a literature review. Case description: We report on two female infants referred to our tertiary center at the age of ten months, with severe colonic and perianal disease, as well as significant malnutrition, who had shown limited response to usual inflammatory bowel disease (IBD) therapy agents. In the first case, whole-exome sequencing (WES) revealed a homozygous (c.537G>A/p.T179T) mutation in exon 4 of the IL-10RA gene, while in the second patient, compound heterozygosity was identified, also in the IL-10RA gene (chr11:117.859.199 variant A>G/p.Tyr57Cys and chr11: 117.860.335 variant G>T/p.Val123Leu). Both patients underwent hematopoietic cell transplantation (HCT). Immunological work-up of these patients revealed increased IL-10 plasma levels and increased IgA. Comments: Our case reports disclose novel findings on plasma cytokine profile in IL-10R deficiency, and we describe the severe phenotype of IL-10/IL-10R deficiency that should be recognized by physicians.
- Published
- 2022
16. Colelitíase sintomática como apresentação de colangite esclerosante primária na infância - série de casos e revisão de literatura
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Lucas Rocha ALVARENGA, Natascha Silva SANDY, Gabriela Souza GOMEZ, Gabriel HESSEL, Adriana Maria Alves DE TOMMASO, and Maria Ângela BELLOMO-BRANDÃO
- Subjects
sclerosing cholangitis ,Pediatrics ,medicine.medical_specialty ,medicine.medical_treatment ,Hepatobiliary Disorder ,Cholangitis, Sclerosing ,RC799-869 ,Primary sclerosing cholangitis ,colangite esclerosante ,03 medical and health sciences ,0302 clinical medicine ,Colelitíase ,Cholelithiasis ,medicine ,Humans ,Multicenter Studies as Topic ,Child ,trato biliar ,Retrospective Studies ,criança ,Past medical history ,child ,business.industry ,Gastroenterology ,Retrospective cohort study ,Diseases of the digestive system. Gastroenterology ,medicine.disease ,Inflammatory Bowel Diseases ,Natural history ,030220 oncology & carcinogenesis ,Etiology ,biliary tract ,030211 gastroenterology & hepatology ,Cholecystectomy ,Presentation (obstetrics) ,business ,Brazil - Abstract
BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone. RESUMO CONTEXTO: A colangite esclerosante primária (CEP) é uma doença hepatobiliar rara, cuja etiologia ainda não está totalmente elucidada. Dada a raridade do CEP na infância, até a recente publicação de uma colaboração multicêntrica internacional, mesmo dados sobre suas características e história natural eram escassos. A colelitíase sintomática não foi relatada anteriormente como a apresentação inicial de CEP na infância. OBJETIVO: O objetivo deste estudo foi o diagnóstico de CEP após a apresentação inicial incomum com colelitíase sintomática, que seguiu um curso clínico atípico que não poderia ser explicado apenas pela colelitíase. Também foi realizada uma revisão da literatura. MÉTODOS: Foi realizada uma revisão retrospectiva dos prontuários de três pacientes, que foram diagnosticados e/ou acompanhados no Hospital das Clínicas da Universidade Estadual de Campinas - São Paulo / Brasil, entre 2014 e 2020. Os dados analisados incluíram sexo, idade de apresentação, história médica pregressa, achados de imagem, resultados laboratoriais, avaliação endoscópica, resposta à terapia médica e acompanhamento. RESULTADOS: A idade no momento da apresentação da colelitíase variou de 10 a 12 anos. Em dois dos casos relatados, um início mais subagudo dos sintomas precedeu o episódio de colelitíase. Dois pacientes foram tratados com colecistectomia, não seguida de qualquer complicação cirúrgica, e um paciente foi tratado de forma conservadora. Biópsia hepática percutânea foi realizada em todos os três casos, mostrando achados histológicos compatíveis com CEP. Doença inflamatória intestinal associada não foi observada em nenhum dos pacientes. Os pacientes foram acompanhados por um tempo médio de 3,4 anos. CONCLUSÃO: CEP e colelitíase são raras na população pediátrica. Este estudo relata a colelitíase sintomática como uma apresentação de CEP e levanta a importância da suspeita de doença hepatobiliar subjacente em crianças com colelitíase sem quaisquer fatores predisponentes conhecidos e/ou que seguem um curso clínico atípico.
- Published
- 2021
17. Increased Prevalence of Left Lobe Atrophy in Long-standing Extrahepatic Portal Vein Obstruction
- Author
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Gabriel Hessel, Roberta Vacari de Alcantara, Adriana Maria Alves De Tommaso, Roberto Massao Yamada, Juliana C.C. Barreto, Natascha Silva Sandy, and Maria Angela Bellomo-Brandão
- Subjects
medicine.medical_specialty ,Context (language use) ,Esophageal and Gastric Varices ,Gastroenterology ,Esophageal varices ,Atrophy ,Internal medicine ,Hypertension, Portal ,medicine ,Prevalence ,Humans ,Child ,Past medical history ,Neonatal sepsis ,business.industry ,Portal Vein ,Infant, Newborn ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Cohort ,Portal hypertension ,Upper gastrointestinal bleeding ,business ,Gastrointestinal Hemorrhage - Abstract
OBJECTIVES The aim of this study was to describe long-term changes in standard blood tests and ultrasound (US) findings in pediatric patients with Extrahepatic Portal Vein Obstruction (EHPVO) who have not undergone Meso-Rex Bypass (MRB) surgery. METHODS US, laboratory, and endoscopic data of 77 patients were analyzed and compared at 2 different points in time: at initial workup and at most recent follow-up. Differences were assessed using McNemar and Wilcoxon tests, while correlations were evaluated using generalized estimating equations. RESULTS Upper gastrointestinal bleeding was the most frequent initial manifestation of EHPVO, in 45.4% of subjects. The mean age at diagnosis was 4.3 years. Seventy-four percentage had a previous history of umbilical catheterization and/or neonatal sepsis. Over time, there was a significant increase in the prevalence of leukopenia, thrombocytopenia, and, interestingly, of left lobe atrophy (LLA), even though the number of esophageal varices and the need for endoscopic interventions reduced. A significant correlation was found between history of umbilical catheterization and LLA, splenomegaly and LLA, and gallbladder wall thickening and LLA. Overall, the number of patients with cholelithiasis at initial workup was low (and therefore, not analyzed with inferential statistics); however, we observed a relative increase over time. CONCLUSIONS In this cohort of patients with EHPVO followed over more than 8 years without treatment with MRB, we report the novel finding of a significant increase in the prevalence of LLA over time. LLA correlated with past medical history of umbilical catheterization, and findings of splenomegaly and gallbladder wall thickening. We propose that that LLA should be further explored as a marker of portal hypertension, particularly in the context of a history of umbilical catheterization.
- Published
- 2021
18. Major Complications of Pediatric Percutaneous Liver Biopsy Do Not Differ Among Physicians With Different Degrees of Training
- Author
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Gabriel Hessel, Natascha Silva Sandy, and Maria Angela Bellomo-Brandão
- Subjects
Image-Guided Biopsy ,Male ,medicine.medical_specialty ,Hepatitis, Viral, Human ,Postoperative Hemorrhage ,03 medical and health sciences ,Postoperative Complications ,0302 clinical medicine ,Hemoglobin drop ,Internal medicine ,Biopsy ,Medical Staff, Hospital ,medicine ,Humans ,Major complication ,Fellowships and Scholarships ,Child ,Pediatric gastroenterology ,Ultrasonography ,Hepatitis ,Cholestasis ,Hepatology ,medicine.diagnostic_test ,business.industry ,Biopsy, Needle ,Gastroenterology ,Infant ,medicine.disease ,Hepatitis, Autoimmune ,Liver ,Child, Preschool ,030220 oncology & carcinogenesis ,Percutaneous liver biopsy ,Female ,030211 gastroenterology & hepatology ,business ,Liver pathology ,Metabolism, Inborn Errors - Abstract
Introduction The objective was to compare safety of pediatric percutaneous liver biopsy (PLB) performed by fellows or staff physicians. Methods Outcomes of 212 PLB completed by first-year pediatric gastroenterology fellows or by staff physicians over 8 years were analyzed and compared. Results Approximately 81.5% of the biopsies were completed by trainees. No significant differences were found between groups (fellows vs staff) regarding number of punctures (median of 1.7 for both), nonrepresentative biopsies (4.2% vs 2.6%), and hemoglobin drop (median of 0.7 vs 0.5 g/L). Discussion Complications of pediatric PLB are uncommon and did not differ among physicians with different training levels.
- Published
- 2019
19. Management of children with functional constipation referred to tertiary care
- Author
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Elizete Aparecida Lomazi, Giovanna Roberta Camargo de Campos, Natascha Silva Sandy, and Maria Angela Bellomo-Brandão
- Subjects
Male ,Pediatrics ,medicine.medical_specialty ,Constipation ,Referral ,Functional gastrointestinal disorders ,Enema ,Single Center ,Tertiary care ,Refractory ,medicine ,Humans ,Bisacodyl ,Child ,Referral and Consultation ,Retrospective Studies ,business.industry ,Childcare ,Tertiary Healthcare ,Retrospective cohort study ,Health care surveys ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Functional constipation ,Female ,medicine.symptom ,business ,medicine.drug - Abstract
Objectives To describe the management, to compare treatment at initial referral vs. during specialized follow-up, and to describe outcomes of children with functional constipation (FC) referred to a Brazilian tertiary care center. Methods Retrospective study, including children (4–18 years) with FC followed at a single center from 2006 to 2019. Demographics, treatments, time of follow-up, and outcomes were analyzed. The management of FC followed an institutional protocol. Results 104 patients were identified, 79 were eligible and included in the analysis: 59% male, mean age at referral was 6.4 years, and mean duration of symptoms was 4.4 years. There were significant changes in the therapy(ies) used at the time of referral compared to during follow-up, with a noticeable increase in the frequency of the use of polyethylene glycol, enemas, magnesium hydroxide, and bisacodyl; 5.1% received trans-anal irrigation, and 3.8% underwent surgery. Outcomes were favorable in more than half of the cases: 31% improved; 19.5% had complete resolution and 2.5% were transferred back to primary care. Symptoms remained unchanged in 30.4%, and no patients experienced worsening of symptoms. The mean duration of follow-up was 2.8 years. When comparing patients with favorable vs. unfavorable outcomes, the authors did not identify significant differences in gender, age, therapies used, duration of symptoms, or length of follow-up. Conclusions Children with FC are often referred to specialized care not receiving optimal therapy. Many patients whose FC was labeled “refractory” may be treated successfully with a well-established plan of care, and do not truly present intractable constipation.
- Published
- 2021
20. Juvenile Polyposis of Infancy Presenting as Protein-Losing Enteropathy
- Author
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Silvia Regina Cardoso, Maria Angela Bellomo-Brandão, Natascha Silva Sandy, and Marcia Alessandra C. P. Cavalaro-Silva
- Subjects
medicine.medical_specialty ,business.industry ,Protein losing enteropathy ,MEDLINE ,medicine ,Image ,General Medicine ,Juvenile Polyposis ,business ,medicine.disease ,Dermatology ,Pediatrics - Published
- 2021
21. Pediatric Intestine Transplantation: Are We Ready for the Prime Time?
- Author
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Natascha Silva Sandy and Yaron Avitzur
- Subjects
medicine.medical_specialty ,Intestine transplantation ,business.industry ,General surgery ,Gastroenterology ,MEDLINE ,Infant ,Intestines ,Prime time ,Text mining ,Pediatrics, Perinatology and Child Health ,medicine ,Humans ,business ,Child - Published
- 2020
22. An Infant with Acute Onset of Nonbilious Emesis
- Author
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Peter C Church, Natascha Silva Sandy, and Tanja Gonska
- Subjects
medicine.medical_specialty ,Hepatology ,business.industry ,Duodenum ,Vomiting ,Gastroenterology ,Infant ,Bezoars ,Acute onset ,Internal medicine ,Phytobezoar ,medicine ,Humans ,Female ,business ,Intestinal Obstruction ,Gastrointestinal endoscopy - Published
- 2020
23. International Latin American Survey on Pediatric Intestinal Failure Team
- Author
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Jose Vicente Spolidoro, Gabriela Jimenez-Arguedas, Roberta Serra, Mikaelle S M Mateus, Daniela Gattini, Natascha Silva Sandy, María Noel Tanzi, Veronica Busoni, Carlos Cuadros-Mendonza, Colomba Cofre, Maria Del Carmen Padilla, Nelson E Ramirez, Juan Rivera-Medina, Lidia P Valdivieso, Clara Plata, Beatriz J Dos Santos, Carola Saure, Mirella Cristiane de Souza, and Helena Ayako Sueno Goldani
- Subjects
Male ,medicine.medical_specialty ,Latin Americans ,Adolescent ,education ,Pharmacist ,Vascular access ,Intestinal rehabilitation ,intestinal rehabilitation ,Pediatrics ,Article ,home parenteral nutrition ,intestinal failure ,Surveys and Questionnaires ,Intestinal failure ,medicine ,Humans ,survey ,TX341-641 ,Practice Patterns, Physicians' ,Child ,Patient Care Team ,Nutrition and Dietetics ,Nutrition. Foods and food supply ,business.industry ,Gastroenterology ,Infant, Newborn ,Infant ,Pediatric Surgeon ,Micronutrient ,Intestinal Diseases ,Latin America ,Parenteral nutrition ,Caribbean Region ,Child, Preschool ,Family medicine ,Female ,Parenteral Nutrition, Home ,business ,Food Science - Abstract
There is little data on the experience of managing pediatric Intestinal Failure (IF) in Latin America. This study aimed to identify and describe the current organization and practices of the IF teams in Latin America and the Caribbean. An online survey was sent to inquire about the existence of IF teams that managed children on home parenteral nutrition (HPN). Our questionnaire was based on a previously published European study with a similar goal. Twenty-four centers with pediatric IF teams in eight countries completed the survey, representing a total number of 316 children on HPN. The median number of children on parenteral nutrition (PN) at home per team was 5.5 (range 1–50). Teams consisted of the following members: pediatric gastroenterologist and a pediatric surgeon in all teams, dietician (95.8%), nurse (91.7%), social worker (79.2%), pharmacist (70.8%), oral therapist (62.5%), psychologist (58.3%), and physiotherapist (45.8%). The majority of the centers followed international standards of care on vascular access, parenteral and enteral nutrition, and IF medical and surgical management, but a significant percentage reported inability to monitor micronutrients, like vitamins A (37.5%), E (41.7%), B1 (66.7%), B2 (62.5%), B6 (62.5%), active B12 (58.3%), and trace elements—including zinc (29.2%), aluminum (75%), copper (37.5%), chromium (58.3%), selenium (58.3%), and manganese (58.3%). Conclusion: There is wide variation in how IF teams are structured in Latin America—while many countries have well-established Intestinal rehabilitation programs, a few do not follow international standards. Many countries did not report having an IF team managing pediatric patients on HPN.
- Published
- 2021
24. O-15: The Noticeable Diversity in Phenotype of ACTG2-Related Disorders – A Report of 4 Cases and Systematic Review of the Literature
- Author
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Alqhatani S, Natascha Silva Sandy, Paul W. Wales, Huysentruyt K, Aleixo M. Muise, Yaron Avitzur, Daniel J. Mulder, and Warner N
- Subjects
Transplantation ,Evolutionary biology ,media_common.quotation_subject ,Biology ,Phenotype ,Diversity (politics) ,media_common - Published
- 2021
25. Nutritional Treatment of a Young Infant with Cystic Fibrosis Presenting with Severe Kwashiorkor Dermatosis
- Author
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Natascha Silva Sandy and Roberto José Negrão Nogueira
- Subjects
Vitamin ,Male ,Pediatrics ,medicine.medical_specialty ,Parenteral Nutrition ,Protein–energy malnutrition ,Cystic Fibrosis ,030204 cardiovascular system & hematology ,Cystic fibrosis ,Skin Diseases ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Fibrosis ,Edema ,medicine ,Humans ,030212 general & internal medicine ,business.industry ,Kwashiorkor ,Infant ,medicine.disease ,Micronutrient ,Infant Formula ,Malnutrition ,Infectious Diseases ,chemistry ,Pediatrics, Perinatology and Child Health ,Dietary Supplements ,medicine.symptom ,business - Abstract
Kwashiorkor in infancy is typically associated to an underlying disease. Edema, a striking feature of this type of malnutrition, can be difficult to assess in this age group. The typical dermatosis of Kwashiorkor is not fully explained the deficiency of one isolated vitamin or micronutrient. This article presents an infant with cystic fibrosis, who developed Kwashiorkor in the third month of life with extensive cutaneous manifestations. An early, individualized and aggressive nutritional intervention with optimized supplementationof sulfur amino acids, vitamins and micronutrients was established, with impressively recovery of overall nutrition and skin manifestations in a relatively short period of time.
- Published
- 2019
26. Incomplete Distal Intestinal Obstruction Syndrome Complicated by Oligosymptomatic Intussusception
- Author
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Natascha Silva Sandy and Elizete Aparecida Lomazi
- Subjects
medicine.medical_specialty ,endocrine system ,business.industry ,Colon ,nutritional and metabolic diseases ,Case Report ,General Medicine ,medicine.disease ,Cystic fibrosis ,Distal intestinal obstruction syndrome ,03 medical and health sciences ,0302 clinical medicine ,030225 pediatrics ,Intussusception (medical disorder) ,medicine ,030211 gastroenterology & hepatology ,Radiology ,medicine.symptom ,Complication ,business ,hormones, hormone substitutes, and hormone antagonists - Abstract
Distal intestinal obstruction syndrome (DIOS) is a relatively common intestinal complication in cystic fibrosis (CF), and it can eventually lead to intussusception, a less frequent complication in CF. Intussusception is classically associated with gastrointestinal symptoms that overlap those of DIOS. We describe a young woman with oligosymptomatic intussusception diagnosed after resolution of DIOS symptoms.
- Published
- 2018
27. Does Training Laparoscopic Skills in a Virtual Reality Simulator Improve Surgical Performance?
- Author
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Natascha Silva Sandy, Ricardo Jordão Duarte, Carlo C. Passerotti, Sabrina T. Reis, Alberto A. Antunes, Marcos F. Dall'Oglio, Hiep T. Nguyen, José Arnaldo Shiomi da Cruz, and Miguel Srougi
- Subjects
Laparoscopic surgery ,medicine.medical_specialty ,Students, Medical ,Time Factors ,Virtual reality simulator ,Urology ,medicine.medical_treatment ,Teaching method ,Sus scrofa ,education ,Blood Loss, Surgical ,Virtual reality ,User-Computer Interface ,medicine ,Animals ,Humans ,Computer Simulation ,Medical physics ,Laparoscopy ,Intraoperative Care ,medicine.diagnostic_test ,business.industry ,Gallbladder ,Clipping (medicine) ,Endoscopy ,Cholecystectomy ,Clinical Competence ,business - Abstract
Background and purpose Several different methods of teaching laparoscopic skills have been advocated, with virtual reality surgical simulation (VRSS) being the most popular. Its effectiveness in improving surgical performance is not a consensus yet, however. The purpose of this study was to determine whether practicing surgical skills in a virtual reality simulator results in improved surgical performance. Materials and methods Fifteen medical students recruited for the study were divided into three groups. Group I (control) did not receive any VRSS training. For 10 weeks, group II trained basic laparoscopic skills (camera handling, cutting skill, peg transfer skill, and clipping skill) in a VRSS laparoscopic skills simulator. Group III practiced the same skills and, in addition, performed a simulated cholecystectomy. All students then performed a cholecystectomy in a swine model. Their performance was reviewed by two experienced surgeons. The following parameters were evaluated: Gallbladder pedicle dissection time, clipping time, time for cutting the pedicle, gallbladder removal time, total procedure time, and blood loss. Results With practice, there was improvement in most of the evaluated parameters by each of the individuals. There were no statistical differences in any of evaluated parameters between those who did and did not undergo VRSS training, however. Conclusion VRSS training is assumed to be an effective tool for learning and practicing laparoscopic skills. In this study, we could not demonstrate that VRSS training resulted in improved surgical performance. It may be useful, however, in familiarizing surgeons with laparoscopic surgery. More effective methods of teaching laparoscopic skills should be evaluated to help in improving surgical performance.
- Published
- 2010
28. Fatores determinantes para a escolha da especialidade médica no Brasil
- Author
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Éder Maxwell Gouveia, Carlo C. Passerotti, Natascha Silva Sandy, Miguel Srougi, Homero Bruschini, Tiago Ribeiro Vannucchi, and José Arnaldo Shiomi da Cruz
- Subjects
General Medicine - Abstract
Fundamento. A escolha da especialidade médica é complexa e multifatorial. A importância dos diferentes fatores varia mundialmente e está associada a diferentes valores e sistemas de educação. O objetivo do estudo é identificar os fatores que determinam essa escolha no Brasil. Métodos. Um questionário foi enviado, via internet (eletronic mail), para graduandos dos seis anos do curso de medicina, solicitando informações demográficas e a avaliação de fatores que influenciam na escolha da especialidade médica. Resultados. A idade média foi de 22 anos. Quanto à influência de familiares, 42,5% dos alunos têm pelo menos um médico como parente próximo, sendo as especialidades mais comuns desse parente: Pediatria, Ginecologia e Obstetrícia e Oftalmologia. A quantidade de respondentes que declararam certeza da carreira a ser seguida ao entrar na faculdade foi 19%, enquanto essa certeza foi de 22,2% na ocasião da pesquisa. Afinidade pela especialidade e estilo de vida foram fatores avaliados como importantes ou muito importantes por mais de 95% dos respondentes. Urgência em ganhar dinheiro rápido, tempo curto de residência, alto rendimento inicial e influência familiar foram os fatores avaliados como menos importantes pelos respondentes na escolha de sua especialidade. Quase metade dos respondentes (49,7%) descartaram uma especialidade que cogitaram fortemente, as principais razões para isso foram ter descoberto não ter afinidade pela especialidade pela qualidade de vida. Conclusões. Afinidade e estilo de vida são os fatores determinantes da escolha e da rejeição de especialidade médica em nosso meio.
- Published
- 2010
29. INGESTÃO E LESÕES CÁUSTICAS GASTROINTESTINAIS – CASUÍSTICA DE UM CENTRO TERCIÁRIO
- Author
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Gabriela Gomez, Natascha Silva Sandy, Ciro Garcia Montes, M.F. Servidoni, Elizete Aparecida Lomazi, and Príscila Pereira
- Subjects
General Medicine - Published
- 2017
30. DOENÇA INFLAMATÓRIA INTESTINAL EM PACIENTES PEDIÁTRICOS COM GLICOGENOSE TIPO IB – CASUÍSTICA DE UM SERVIÇO TERCIÁRIO
- Author
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Flavia Justo, Gabriela Gomez, Elizete Aparecida Lomazi, Adriana Maria Alves De Tommaso, Julia Barreto, and Natascha Silva Sandy
- Subjects
General Medicine - Published
- 2017
31. ASPECTOS CLÍNICOS E ENDOSCÓPICOS DE PACIENTES PEDIÁTRICOS PORTADORES DE SÍNDROME DE PEUTZ-JEGHERS – SÉRIE DE CASOS
- Author
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Maria Brandao, Juliana C.C. Barreto, Natascha Silva Sandy, Gabriela Gomez, and Flavia Justo
- Subjects
General Medicine - Published
- 2017
32. RELATO DE CASO – DOENÇA DE CROHN DE DIFÍCIL CONTROLE NA INFÂNCIA COMPLICADA PSORÍASE COMO REAÇÃO PARADOXAL A IMUNOBIÓLOGICOS E POR INFECÇÃO POR CLOSTRIDIUM DIFFICILE
- Author
-
Elizete Aparecida Lomazi, M.F. Servidoni, Gabriela Gomez, and Natascha Silva Sandy
- Subjects
General Medicine - Published
- 2017
33. Can the learning of laparoscopic skills be quantified by the measurements of skill parameters performed in a virtual reality simulator?
- Author
-
Miguel Srougi, Natascha Silva Sandy, José Arnaldo Shiomi da Cruz, Homero Bruschini, Hiep T. Nguyen, Ricardo Jordão Duarte, Éder Maxwell Gouveia, Carlo C. Passerotti, and Sabrina T. Reis
- Subjects
Male ,medicine.medical_specialty ,Virtual reality simulator ,Students, Medical ,Time Factors ,Training course ,Urology ,education ,Virtual reality ,Task completion ,lcsh:RC870-923 ,behavioral disciplines and activities ,Task (project management) ,Education ,Virtual Reality Exposure Therapy ,Patient safety ,User-Computer Interface ,Task Performance and Analysis ,Medicine ,Humans ,Medical physics ,Computer Simulation ,Surgical simulator ,Prospective Studies ,business.industry ,Internship and Residency ,Reproducibility of Results ,lcsh:Diseases of the genitourinary system. Urology ,Surgery ,Female ,Laparoscopy ,Clinical Competence ,business ,Learning Curve - Abstract
Purpose To ensure patient safety and surgical efficiency, much emphasis has been placed on the training of laparoscopic skills using virtual reality simulators. The purpose of this study was to determine whether laparoscopic skills can be objectively quantified by measuring specific skill parameters during training in a virtual reality surgical simulator (VRSS). Materials and Methods Ten medical students (with no laparoscopic experience) and ten urology residents (PGY3-5 with limited laparoscopic experience) were recruited to participate in a ten-week training course in basic laparoscopic skills (camera, cutting, peg transfer and clipping skills) on a VRSS. Data were collected from the training sessions. The time that individuals took to complete each task and the errors that they made were analyzed independently. Results The mean time that individuals took to complete tasks was significantly different between the groups (p < 0.05), with the residents being faster than the medical students. The residents' group also completed the tasks with fewer errors. The majority of the subjects in both groups exhibited a significant improvement in their task completion time and error rate. Conclusion The findings in this study demonstrate that laparoscopic skills can be objectively measured in a VRSS based on quantified skill parameters, including the time spent to complete skill tasks and the associated error rate. We conclude that a VRSS is a feasible tool for training and assessing basic laparoscopic skills.
- Published
- 2013
34. Assessment of parental satisfaction in children undergoing voiding cystourethrography without sedation
- Author
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Jeanne S. Chow, Angela Franceschi, Frank J. Penna, Brian J. Minnillo, Hiep T. Nguyen, Natascha Silva Sandy, and Sonja I. Ziniel
- Subjects
Male ,medicine.medical_specialty ,Pediatrics ,Urology ,Sedation ,media_common.quotation_subject ,Urinary Bladder ,Conscious Sedation ,Directive Counseling ,Pain ,Anxiety ,Vesicoureteral reflux ,Urination ,Patient satisfaction ,Urethra ,Surveys and Questionnaires ,medicine ,Humans ,Parental Consent ,Parent-Child Relations ,Child ,media_common ,Pain Measurement ,Vesico-Ureteral Reflux ,Analysis of Variance ,business.industry ,Toilet Training ,Infant ,Urography ,medicine.disease ,Urination Disorders ,Surgery ,Distress ,Patient Satisfaction ,Child, Preschool ,Female ,medicine.symptom ,Parental consent ,business ,Child life specialist - Abstract
Approximately 50,000 children undergo voiding cystourethrography annually. There is a recent trend toward using sedation or delaying voiding cystourethrography due to the anticipated distress to the patient. We hypothesized that with adequate preparation and proper techniques to minimize anxiety, voiding cystourethrography can be performed without sedation. We assessed parental satisfaction associated with patient and parent experience of voiding cystourethrography without sedation.We used a 33-question survey to evaluate parental satisfaction with patient and parent experience of voiding cystourethrography without sedation. Children were divided into 3 groups according to toilet training status. Statistical analysis was performed using Stata®.A total of 200 surveys were completed. Of the children 54% were not toilet trained. Of the parents 90% reported adequate preparation. More than half of parents classified the experience of voiding cystourethrography as equivalent to or better than a physical examination, immunization, ultrasound and prior catheterization. Most parents were satisfied with the ability of the child to tolerate the procedure and considered the experience better than expected. Children in the process of toilet training had the most difficulty with the procedure, correlating with lower levels of parental satisfaction.Voiding cystourethrography performed with adequate preparation and support can be tolerated without sedation. Children in the process of toilet training and females tolerate the procedure least.
- Published
- 2010
35. 911 DEVELOPMENT OF A PROFICIENCY-BASED SKILLS TRAINING CURRICULUM FOR ROBOTIC ASSISTED LAPAROSCOPIC SURGERY
- Author
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Brian Minnillo, Fernando Carvas, Hiep T. Nguyen, Alexandre Derevianko, Andres Silva Waissbluth, Natascha Silva Sandy, and Alan B. Retik
- Subjects
Laparoscopic surgery ,Medical education ,Skills training ,Robotic assisted ,business.industry ,Urology ,medicine.medical_treatment ,medicine ,business ,Curriculum - Published
- 2010
36. The concordance of testicular anatomic location in bilateral cryptorchidism
- Author
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Natascha Silva Sandy, Hiep T. Nguyen, David A. Diamond, Caleb P. Nelson, Frank J. Penna, and Carlo C. Passerotti
- Subjects
Male ,medicine.medical_specialty ,Gonad ,Adolescent ,Urology ,medicine.medical_treatment ,Concordance ,Anatomic Site ,Young Adult ,Bilateral Cryptorchidism ,Cryptorchidism ,Testis ,Medicine ,Humans ,Orchiopexy ,Young adult ,Anatomic Location ,Child ,Anatomical location ,business.industry ,Infant ,Anatomy ,medicine.anatomical_structure ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Radiology ,business - Abstract
Objective To determine the frequency of concordance of testicular anatomical location between both undescended testes based on surgical findings. Background Cryptorchidism occurs in approximately 1% of boys over one year of age, and in 10–20% of these cases, the condition is bilateral. We report a series of 168 patients with a preoperative diagnosis of bilateral undescended testes who underwent surgical exploration and in whom two testes were surgically identified. Methods The testicular location of both testes was stratified according to six anatomic regions. For each patient the anatomic site of each testis, both palpable and impalpable, and the surgical procedure required for orchidopexy were recorded. Results The mean age was 59.3 months (2.5 months–21 years). In 90% of the cases the surgical technique was identical for both sides. Approximately two thirds of the patients were found to have both testes in concordant anatomical locations and in 88% the location of one testis was predictive within one anatomic “step” of the location of the contralateral gonad. Conclusion These findings may have significant implications for counseling families and anticipating the necessary surgical approach required when only one undescended testis has been localized preoperatively.
- Published
- 2009
37. CAN THE LEARNING OF LAPAROSCOPIC SKILLS BE QUANTIFIED BY THE MEASUREMENTS OF SKILL PARAMETERS PERFORMED IN A VIRTUAL REALITY SIMULATOR?
- Author
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Carlo C. Passerotti, José Arnaldo Shiomi da Cruz, Rafael Coelho, Ricardo Jordão Duarte, Miguel Srougi, Natascha Silva Sandy, Hiep T. Nguyen, and Enrico F m Andrade
- Subjects
Virtual Reality Exposure Therapy ,Virtual reality simulator ,business.industry ,Human–computer interaction ,Urology ,Medicine ,business - Published
- 2009
38. DOES TRAINING LAPAROSCOPIC SKILLS IN A VIRTUAL REALITY SIMULATOR IMPROVES ACTUAL SURGICAL PERFORMANCE?
- Author
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Carlo C. Passerotti, José Arnaldo Shiomi da Cruz, Natascha Silva Sandy, Hiep T. Nguyen, Ricardo Jordão Duarte, and Miguel Srougi
- Subjects
medicine.medical_specialty ,Virtual reality simulator ,business.industry ,Urology ,medicine.medical_treatment ,Teaching method ,education ,Group ii ,Virtual reality ,behavioral disciplines and activities ,Surgery ,medicine ,Surgical skills ,Medical physics ,Cholecystectomy ,Surgical simulation ,business - Abstract
Background and Purpose: Several different methods of teaching laparoscopic skills have been advocated, with virtual reality surgical simulation (VRSS) being the most popular. Its effectiveness in improving surgical performance is not a consensus yet, however. The purpose of this study was to determine whether practicing surgical skills in a virtual reality simulator results in improved surgical performance. Materials and Methods: Fifteen medical students recruited for the study were divided into three groups. Group I (control) did not receive any VRSS training. For 10 weeks, group II trained basic laparoscopic skills (camera handling, cutting skill, peg transfer skill, and clipping skill) in a VRSS laparoscopic skills simulator. Group III practiced the same skills and, in addition, performed a simulated cholecystectomy. All students then performed a cholecystectomy in a swine model. Their performance was reviewed by two experienced surgeons. The following parameters were evaluated: Gallbladder pe...
- Published
- 2009
39. Does Training Laparoscopic Skills in a Virtual Reality Simulator Improve Surgical Performance?
- Author
-
José Arnaldo Shiomi da Cruz, Natascha Silva Sandy, Carlo Camargo Passerotti, Hiep Nguyen, Alberto Azoubel Antunes, Sabrina Thalita dos Reis, Marcos Francisco Dall'Oglio, Ricardo Jordao Duarte, and Miguel Srougi
- Subjects
- *
LAPAROSCOPIC surgery , *VIRTUAL reality in education , *SIMULATION methods in medical education , *CLINICAL competence , *TRAINING of medical students , *CHOLECYSTECTOMY , *LABORATORY swine , *GALLBLADDER surgery , *EDUCATION - Abstract
AbstractBackground and Purpose:Several different methods of teaching laparoscopic skills have been advocated, with virtual reality surgical simulation (VRSS) being the most popular. Its effectiveness in improving surgical performance is not a consensus yet, however. The purpose of this study was to determine whether practicing surgical skills in a virtual reality simulator results in improved surgical performance.Materials and Methods:Fifteen medical students recruited for the study were divided into three groups. Group I (control) did not receive any VRSS training. For 10 weeks, group II trained basic laparoscopic skills (camera handling, cutting skill, peg transfer skill, and clipping skill) in a VRSS laparoscopic skills simulator. Group III practiced the same skills and, in addition, performed a simulated cholecystectomy. All students then performed a cholecystectomy in a swine model. Their performance was reviewed by two experienced surgeons. The following parameters were evaluated: Gallbladder pedicle dissection time, clipping time, time for cutting the pedicle, gallbladder removal time, total procedure time, and blood loss.Results:With practice, there was improvement in most of the evaluated parameters by each of the individuals. There were no statistical differences in any of evaluated parameters between those who did and did not undergo VRSS training, however.Conclusion:VRSS training is assumed to be an effective tool for learning and practicing laparoscopic skills. In this study, we could not demonstrate that VRSS training resulted in improved surgical performance. It may be useful, however, in familiarizing surgeons with laparoscopic surgery. More effective methods of teaching laparoscopic skills should be evaluated to help in improving surgical performance. [ABSTRACT FROM AUTHOR]
- Published
- 2010
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