CAROLINE DE GOUVEIA BUFF PASSONE

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Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina

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Agora exibindo 1 - 10 de 28
  • conferenceObject
    Challenged Diagnosis on Hypoglycemia: Hirata Disease X Factitious Hypoglycemia
    (2015) JERONIMO, T.; PASSONE, C. Buff; FARIA JUNIOR, J.; SAVOLDELLI, R. Diaz; KUPERMAN, H.; MENEZES FILHO, H. Cabral de; STEINMETZ, L.; DITCHTCHEKENIAN, V; MANNA, T. Della; DAMIANI, D.
  • conferenceObject
    Central Precocious Puberty After Surgical Resection of Giant Craniopharyngioma in a Girl
    (2018) BEZERRA, Marilia; KLINK, Gabriela; BORNEA, Rondi; PASCHOAL, Fernanda; PASSONE, Caroline; STEINMETZ, Leandra; COMINATO, Louise; ROCHA, Ruth; MANNA, Thais; FILHO, Hamilton; DAMIANI, Durval
  • article 5 Citação(ões) na Scopus
    Translation and validation of Pediatric Quality of Life Inventory (TM) 3.0 Diabetes Module (PedsQL (TM) 3.0 Diabetes Module) in Brazil-Portuguese language
    (2018) GARCIA, Leila F. dos S.; MANNA, Thais Della; PASSONE, Caroline de Gouveia Buff; OLIVEIRA, Lygia Spassapan de
    Objective: The aim of the present study was to create a translated version of the Pediatric Quality of Life Inventory (TM) 3.0 Diabetes Module (PedsQL (TM) 3.0 Diabetes Module) in Brazilian Portuguese that was conceptually equivalent to the original American English version and to linguistically validate it in a Brazilian pediatric population with type 1 diabetes mellitus and their parents or caregivers. Methods: The instrument was translated, back-translated, and then administered to 83 children/adolescents (5-18 years) with type 1 diabetes mellitus and their family members and to 25 parents/caregivers of patients aged between 2 and 4 years. The final translated version was tested for reliability by analyzing internal consistency, intraobserver (test-retest) reliability, and concurrent validity. Results: Cronbach's alpha coefficient for the total score of the questionnaires of children/adolescents (alpha = 0.85) and their parents (alpha = 0.82) was above the recommended minimum of 0.70 for group comparisons. Intraobserver reliability and concurrent validity exhibited a significant positive correlation (p < 0.001), indicating the reliability of the translated instrument. A moderate but significant positive correlation (r = 0.40; p < 0.001) was demonstrated between the total scores of patient self-report and parent proxy-report scales. There was no significant correlation between glycated hemoglobin (HbA1c) levels and the respective scores in the questionnaires answered by patients and their parents/caregivers. Conclusion: The analysis of the translated version of the PedsQL (TM) 3.0 Diabetes Module revealed adequate psychometric characteristics with respect to reliability and validity following administration to a sample of Brazilian children/adolescents with type 1 diabetes mellitus and their caregivers. (C) 2018 Sociedade Brasileira de Pediatria.
  • article 17 Citação(ões) na Scopus
    Clinical application of ACMG-AMP guidelines in HNF1A and GCK variants in a cohort of MODY families
    (2017) SANTANA, L. S.; CAETANO, L. A.; COSTA-RIQUETTO, A. D.; QUEDAS, E. P. S.; NERY, M.; COLLETT-SOLBERG, P.; BOGUSZEWSKI, M. C. S.; VENDRAMINI, M. F.; CRISOSTOMO, L. G.; FLOH, F. O.; ZARABIA, Z. I.; KOHARA, S. K.; GUASTAPAGLIA, L.; PASSONE, C. G. B.; SEWAYBRICKER, L. E.; JORGE, A. A. L.; TELES, M. G.
    Maturity-onset diabetes of the young (MODY) is a form of monogenic diabetes with autosomal dominant inheritance. GCK-MODY and HNF1A-MODY are the prevalent subtypes. Currently, there is growing concern regarding the correct interpretation of molecular genetic findings. The American College of Medical Genetics and Genomics (ACMG) updated guidelines to interpret and classify molecular variants. This study aimed to determine the prevalence of MODY (GCK/HNF1A) in a large cohort of Brazilian families, to report variants related to phenotype, and to classify them according to ACMG guidelines. One hundred and nine probands were investigated, 45% with clinical suspicion of GCK-MODY and 55% with suspicion of HNF1A-MODY. Twenty-five different variants were identified in GCK gene (30 probands61% of ositivity), and 7 variants in HNF1A (10 probands17% of positivity). Fourteen of them werenovel (12GCK/2HNF1A). ACMG guidelines were able to classify a large portion of variants as athogenic (36%GCK/86%HNF1A) and likely pathogenic (44%GCK/14%HNF1A), with 16% (5/32) as uncertain significance. This allows us to determine the pathogenicity classification more efficiently, and also reinforces the suspected associations with the phenotype among novel variants.
  • conferenceObject
    Prader-Willi Syndrome - A General Picture of 51 Cases
    (2015) ITO, S.; JERONIMO, T.; FARIA JUNIOR, J.; PASSONE, C. de Gouveia Buff; FRANCO, R. Rocha; STEINMETZ, L.; KUPERMAN, H.; DAMIANI, D.
  • article 37 Citação(ões) na Scopus
    Adjusting insulin doses in patients with type 1 diabetes who use insulin pump and continuous glucose monitoring: Variations among countries and physicians
    (2018) NIMRI, Revital; DASSAU, Eyal; SEGALL, Tomer; MULLER, Ido; BRATINA, Natasa; KORDONOURI, Olga; BELLO, Rachel; BIESTER, Torben; DOVC, Klemen; TENENBAUM, Ariel; BRENER, Avivit; SIMUNOVIC, Marko; SAKKA, Sophia D.; SHENKER, Michal Nevo; PASSONE, Caroline G. B.; RUTIGLIANO, Irene; TINTI, Davide; BONURA, Clara; CAIULO, Silvana; RUSZALA, Anna; PICCINI, Barbara; GIRI, Dinesh; STEIN, Ronnie; RABBONE, Ivana; BRUZZI, Patrizia; OMLADIC, Jasna Suput; STEELE, Caroline; BECCUTI, Guglielmo; YACKOBOVITCH-GAVAN, Michal; BATTELINO, Tadej; DANNE, Thomas; ATLAS, Eran; PHILLIP, Moshe
    Aims: To evaluate physicians' adjustments of insulin pump settings based on continuous glucose monitoring (CGM) for patients with type 1 diabetes and to compare these to automated insulin dose adjustments. Methods: A total of 26 physicians from 16 centres in Europe, Israel and South America participated in the study. All were asked to adjust insulin dosing based on insulin pump, CGM and glucometer downloads of 15 patients (mean age 16.2 +/- 4.3 years, six female, mean glycated haemoglobin 8.3 +/- 0.9% [66.8 +/- 7.3 mmol/mol]) gathered over a 3-week period. Recommendations were compared for the relative changes in the basal, carbohydrate to insulin ratio (CR) and correction factor (CF) plans among physicians and among centres and also between the physicians and an automated algorithm, the Advisor Pro (DreaMed Diabetes Ltd, Petah Tikva, Israel). Study endpoints were the percentage of comparison points for which there was full agreement on the trend of insulin dose adjustments (same trend), partial agreement (increase/decrease vs no change) and full disagreement (opposite trend). Results: The percentages for full agreement between physicians on the trend of insulin adjustments of the basal, CR and CF plans were 41 +/- 9%, 45 +/- 11% and 45.5 +/- 13%, and for complete disagreement they were 12 +/- 7%, 9.5 +/- 7% and 10 +/- 8%, respectively. Significantly similar results were found between the physicians and the automated algorithm. The algorithm magnitude of insulin dose change was at least equal to or less than that proposed by the physicians. Conclusions: Physicians provide different insulin dose recommendations based on the same datasets. The automated advice of the Advisor Pro did not differ significantly from the advice given by the physicians in the direction or magnitude of the insulin dosing.
  • bookPart
    Puberdade atrasada
    (2016) STEINMETZ, Leandra; PASSONE, Caroline de Gouveia Buff; PAULINO, Mariana da Costa Rose; MANNA, Thais Della
  • article 3 Citação(ões) na Scopus
    Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata's disease) in a hypoglycemic child: a case report and review of the literature
    (2019) SANTOS, Tiago Jeronimo Dos; PASSONE, Caroline Gouvea Buff; YBARRA, Marina; ITO, Simone Sakura; TELES, Milena Gurgel; MANNA, Thais Della; DAMIANI, Durval
    Background: Insulin autoimmune syndrome (IAS) is a rare cause of hyperinsulinemic hypoglycemia (HH) not addressed as a potential differential diagnosis in current pediatric guidelines. We present a case of IAS in a child with no previous history of autoimmune disease, no previous intake of triggering medications and absence of genetic predisposition. Case presentation: A 6-year-old boy presented with recurrent HH (blood glucose of 26 mg/dL [1.4 mmol/L] and insulin of 686 mu U/mL). Abdominal imaging was normal. After multiple therapeutic failures, we hypothesized misuse of exogenous insulin and factitious hypoglycemia. Council of Guardianship had the child separated from his mother, but insulin levels remained high. A chromatography test was then performed which showed high titers of endogenous insulin autoantibody (IAA) with early dissociation from the insulin molecule. The human leukocyte antigen (HLA) test showed a DRB1 *13:01/*08:02 genotype. The patient was advised to control food intake and physical activity routines. During a 5-year follow-up, hypoglycemic episodes were sparse, despite high insulin levels. Conclusions: Misdiagnosis of IAS with factitious hypoglycemia may happen if IAS is not considered as a differential diagnosis, leading to potential traumatic consequences. Further efforts should be made to increase awareness of IAS as a differential diagnosis of hypoglycemia and to include it in pediatric guidelines.
  • article 3 Citação(ões) na Scopus
    Translation and validation of diabetes self-management profile (DSMP) into Brazilian Portuguese language: first instrument to assess type 1 diabetes self-management in a pediatric population
    (2017) PASSONE, Caroline Gouveia Buff; ESTEVES, Lygia Spassapan Oliveira; SAVOLDELLI, Roberta Dias; HARRIS, Michael A.; DAMIANI, Durval; MANNA, Thais Della
    Objective: To translate and validate the instrument Diabetes Self-Management Profile (DSMP)-Conventional and Flexible Regimens into Brazilian Portuguese language in order to evaluate the quality of diabetes self-management in children and adolescents with type 1 diabetes and their caregivers. Methods: DSMP was submitted to forward and back translation method and validated in a group of type 1 diabetes youths between 6 and 18 years (n = 102), and their families. Analysis of DSMP internal consistency, intra and interobserver reliability and concurrent correlation with HbA1c were done. Results: DSMP total scores demonstrated adequate internal consistency (Cronbach's alpha = 0.79), 3-month test-retest reliability (rho = 0.53; p < 0.001), inter-interviewer agreement (rho = 0.55; p < 0.001). DSMP total score was significantly correlated to HbA1c (rho = -0.54, p < 0.001). Conclusion: DSMP-translated version is a reliable and valid tool to assess diabetes self-management.
  • article 7 Citação(ões) na Scopus
    Cardiac autonomic control during non-REM and REM sleep stages in paediatric patients with Prader-Willi syndrome
    (2021) BRITO, Leandro C.; QUEIROGA, Thereza; FRANCO, Ruth R.; PASSONE, Caroline G. B.; LOPES, Maria-Cecilia; SHEA, Steven A.; BUENO, Clarissa; SOSTER, Leticia M. S. F. A.
    Cardiac death is the second most prevalent cause in Prader-Willi syndrome (PWS). Paediatric patients with PWS often present cardiac autonomic dysfunction during wakefulness, obesity and sleep-disordered breathing. However, the extent of cardiac autonomic modulation during sleep in PWS has not been documented. The objective of this study was to assess alterations in cardiac autonomic modulation of paediatric patients with PWS during different sleep stages. Thirty-nine participants in three groups: 14 PWS, 13 sex and age-matched lean controls (LG) and 12 obese-matched controls (OB). All participants underwent overnight polysomnography, including continuous electrocardiogram recordings. Heart rate variability (HRV) was analysed during representative periods of each sleep stage through time and frequency domains calculated across 5-min periods. Between-within ANOVAs were employed (p < .05). The results show that total HRV was lower in PWS than OB and LG during slow-wave sleep (SWS) (standard deviation of all NN intervals [SDNN] ms,p = .006). Parasympathetic modulation assessed by time-domain analysis was lower during SWS in PWS compared to both OB and LG (square root of the mean of the sum of the squares of differences between adjacent NN intervals [RMSSD] ms,p = .004; SDSD, standard deviation of differences between adjacent NN intervals [SDSD] ms,p = .02; number of adjacent NN intervals differing by >50 ms [NN50] ms,p = .03; proportion of adjacent NN intervals differing by >50 ms [pNN50] ms,p = .01). Sympathovagal balance assessed by frequency-domain analysis was lower during both N2 and SWS than during the rapid eye movement (REM) sleep stage, but not different among groups. In conclusion, this group of paediatric patients with PWS had impaired cardiac autonomic balance due to reduced parasympathetic modulation during SWS. This result could imply an underlying increased cardiovascular risk in PWS even during early age and independent of obesity.