MIRELA COSTA DE MIRANDA

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Instituto Central, Hospital das Clínicas, Faculdade de Medicina
LIM/42 - Laboratório de Hormônios e Genética Molecular, Hospital das Clínicas, Faculdade de Medicina

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  • article 9 Citação(ões) na Scopus
    Adverse Outcomes and Economic Burden of Congenital Adrenal Hyperplasia Late Diagnosis in the Newborn Screening Absence
    (2020) MIRANDA, Mirela Costa de; HADDAD, Luciana Bertocco de Paiva; MADUREIRA, Guiomar; MENDONCA, Berenice Bilharinho de; BACHEGA, Tania A. S. S.
    Objective: To establish short- and long-term adverse outcome frequencies related to a late diagnosis of congenital adrenal hyperplasia (CAH) in the absence of newborn screening (NBS) and to determine respective treatment costs, which have never been reported. Design: A retrospective analysis of a CAH cohort diagnosed without NBS. Methods: We evaluated medical record data concerning 195 patients (141 females) diagnosed with CAH through clinical suspicion and confirmed using hormonal and CYP21A2 analysis, who were followed from 1980 to 2016 at Sao Paulo University. We measured mortality, dehydration, mental impairment frequencies, and hospitalization length outcomes in the salt-wasting form; the frequency of genetic females raised as males in both forms, frequency of depot GnRh analog (GnRha) and GH therapies in the simple virilizing form, and related outcome costs were calculated. Results: Mortality rates and associated costs, varying from 10% to 26% and from $2,239,744.76 to $10,271,591.25, respectively, were calculated using the Brazilian yearly live-births rate, estimated productive life years, and gross domestic product. In the salt-wasting form, 76% of patients were hospitalized, 8.6% were mentally impaired, and 3% of females were raised as males (total cost, $86,230/salt-wasting patient). GnRha and growth hormone were used for 28% and 14% of simple virilizing patients, respectively, and 18% of females were raised as males (preventable cost, $4232.74/simple virilizing patient). Conclusions: A late CAH diagnosis leads to high mortality and morbidity rates, notably increasing public health costs, and may result in physical and psychological damage that is not easily measurable. (C) Endocrine Society 2019.
  • article 3 Citação(ões) na Scopus
    Long-term cardiometabolic morbidity in young adults with classic 21-hydroxylase deficiency congenital adrenal hyperplasia
    (2023) RIGHI, Beatrice; ALI, Salma R.; BRYCE, Jillian; TOMLINSON, Jeremy W.; BONFIG, Walter; BARONIO, Federico; COSTA, Eduardo C.; GUARAGNA-FILHO, Guilherme; T'SJOEN, Guy; COOLS, Martine; MARKOSYAN, Renata; BACHEGA, Tania A. S. S.; MIRANDA, Mirela C.; IOTOVA, Violeta; FALHAMMAR, Henrik; CECCATO, Filippo; STANCAMPIANO, Marianna R.; RUSSO, Gianni; DANIEL, Eleni; AUCHUS, Richard J.; ROSS, Richard J.; AHMED, S. Faisal
    PurposeTo study the current practice for assessing comorbidity in adults with 21-hydroxylase CAH and to assess the prevalence of comorbidity in these adults.MethodsA structured questionnaire was sent to 46 expert centres managing adults with CAH. Information collected included current therapy and surveillance practice with a particular focus on osteoporosis/osteopaenia, hyperlipidaemia, type 2 diabetes/hyperinsulinaemia, hypertension, CV disease, obesity.ResultsOf the 31 (67%) centres from 15 countries that completed the survey, 30 (97%) screened for hypertension by measuring blood pressure, 30 (97%) screened for obesity, 26 (84%) screened for abnormal glucose homoeostasis mainly by using Hb1Ac (73%), 25 (81%) screened for osteoporosis mainly by DXA (92%), 20 (65%) screened for hyperlipidaemia and 6 (19%) screened for additional CV disease. Of the 31 centres, 13 provided further information on the six co-morbidities in 244 patients with a median age of 33 yrs (range 19, 94). Of these, 126 (52%) were females and 174 (71%) received fludrocortisone in addition to glucocorticoids. Of the 244 adults, 73 (30%) were treated for at least one comorbidity and 15 (21%) for more than 2 co-morbidities. Of 73, the patients who were treated for osteoporosis/osteopaenia, hyperlipidaemia, type 2 diabetes/hyperinsulinaemia, hypertension, CV disease, obesity were 43 (59%), 17 (23%), 16 (22%), 10 (14%), 8 (11), 3 (4%) respectively.ConclusionCardiometabolic and bone morbidities are not uncommon in adults with CAH. There is a need to standardise the screening for these morbidities from early adulthood and to explore optimal therapy through routine collection of standardised data.
  • article 21 Citação(ões) na Scopus
    International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry
    (2021) BACILA, Irina; FREEMAN, Nicole; DANIEL, Eleni; SANDRK, Marija; BRYCE, Jillian; ALI, Salma Rashid; ABALI, Zehra Yavas; ATAPATTU, Navoda; BACHEGA, Tania A.; BALSAMO, Antonio; BIRKEBAEK, Niels; BLANKENSTEIN, Oliver; BONFIG, Walter; COOLS, Martine; COSTA, Eduardo Correa; DARENDELILER, Feyza; EINAUDI, Silvia; ELSEDFY, Heba Hassan; FINKEN, Martijn; GEVERS, Evelien; GRINTEN, Hedi L. Claahsen-van der; GURAN, Tulay; GUVEN, Ayla; HANNEMA, Sabine E.; HIGHAM, Claire E.; IOTOVA, Violeta; KAMP, Hetty J. van der; KORBONITS, Marta; KRONE, Ruth E.; LICHIARDOPOL, Corina; LUCZAY, Andrea; MENDONCA, Berenice Bilharinho; MILENKOVIC, Tatjana; MIRANDA, Mirela C.; MOHNIKE, Klaus; NEUMANN, Uta; ORTOLANO, Rita; POYRAZOGLU, Sukran; THANKAMONY, Ajay; TOMLINSON, Jeremy W.; VIEITES, Ana; VRIES, Liat de; AHMED, S. Faisal; ROSS, Richard J.; KRONE, Nils P.
    Objective: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids in CAH. Design: This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International CAH Registry. Methods: Data were collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 and 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement were analyzed from 4174 patient visits. Results: The most frequently used glucocorticoid was hydrocortisone (87.6%). Overall, there were significant differences between age groups with regards to daily hydrocortisone-equivalent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0-14.5) mg/m(2)/day at age 1-8 years and the highest dose of 14.0 (11.6-17.4) mg/m(2)/day at age 12-18 years. Glucocorticoid doses decreased after 2010 in patients 0-8 years (P < 0.001) and remained unchanged in patients aged 8-18 years. Fludrocortisone was used in 92% of patients, with relative doses decreasing with age. A wide variation was observed among countries with regards to all aspects of steroid hormone replacement. Conclusions: Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.
  • article 6 Citação(ões) na Scopus
    Real-world impact of glucocorticoid replacement therapy on bone mineral density: retrospective experience of a large single-center CAH cohort spanning 24 years
    (2020) IERVOLINO, L. L.; FERRAZ-DE-SOUZA, B.; MARTIN, R. M.; COSTA, F. C.; MIRANDA, M. C.; MENDONCA, B. B.; BACHEGA, T. S.
    The congenital adrenal hyperplasia population seems to have an intrinsic tendency to a high frequency of low bone mass. However in this single-center and long-term evaluated cohort, the simplified corticoid regimen, with exclusive dexamethasone single dose reposition during adulthood, did not represent a risk factor for decrease in bone health. Introduction The impact of long-term and supposedly physiological doses of gluco and mineralocorticoid (GC/MC) on bone mineral density (BMD) in congenital adrenal hyperplasia (CAH) remains discordant among studies, which contain different clinical forms and corticoid regimens. Our aim was to evaluate the BMD in CAH adults receiving similar GC regimen since childhood and to correlate it with GC/MC cumulative doses. Methods Only patients with good compliance, who used cortisone acetate (CA) during childhood and dexamethasone after the final height achievement. Cumulative GC/MC doses were calculated from diagnosis until last evaluation. BMD was analyzed by the first and last energy X-ray absorptiometry (DXA) scans performed. Results Twenty simple virilizing (SV) and 14 salt wasting (WS) whose mean age was 26 +/- 6 years, mean CA, dexamethasone, and fludrocortisone cumulative doses were 63,813 +/- 32,767, 812 +/- 558, and 319 +/- 325 mg/m(2), respectively. Based on the last DXA, low BMD was observed in 11% of patients, total hip Z-score was lower in the SW than SV form (p = 0.04). Cumulative CA dose had an inverse correlation with femoral neck Z-score (p < 0.01). Total cumulative GC and MC doses had an inverse correlation with total hip Z-score (p < 0.01). In the analysis of sequential BMD during dexamethasone therapy, no association was observed among cumulative GC/MC doses, clinical forms, sex, and lumbar Z-score delta. Conclusions Even though a low CA regimen during growth periods in addition to MC replacement appears to have an influence on BMD at femoral sites, interestingly a low dexamethasone one does not seem to be deleterious for bone health in adulthood.
  • conferenceObject
    DEFINING THE DOSE, TYPE AND TIMING OF GLUCOCORTICOID AND MINERALOCORTICOID REPLACEMENT IN 256 CHILDREN AND ADULTS WITH CONGENITAL ADRENAL HYPERPLASIA (CAH) IN THE I-CAH REGISTRY
    (2017) DANIEL, Eleni; SANDRK, Marija; BLANKENSTEIN, Oliver; NEUMANN, Uta; GRINTEN, Hedi Claahsen-Van der; LINDE, Annelieke Van der; DARENDELILER, Feyza; PYRAZOGLU, Sukran; MENDONCA, Berenice B.; BACHEGA, Tania S. S.; MIRANDA, Mirela C.; ACERINI, Carlo; GURAN, Tulay; BIRKEBAEK, Niels H.; COOLS, Martine; MILENKOVIC, Tatjana; BONFIG, Walter; TOMLINSON, Jeremy W.; AHMED, Syed Faisal; ELSEDFY, Heba; BALSAMO, Antonio; HANNEMA, Sabine E.; HIGHAM, Claire; ATAPATTU, Navoda; LICHIARDOPOL, Corina; KRONE, Ruth E.; MOHNIKE, Klaus; KRONE, Nils
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    Long-Term Cardiometabolic Morbidity in Young Adults with Classic 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia
    (2021) RIGHI, Beatrice; ALI, Salma R.; BRYCE, Jillian; TOMLINSON, Jeremy W.; BONFIG, Walter; BARONIO, Federico; COSTA, Eduardo C.; GUARAGNA FILHO, Guilherme; T'SJOEN, Guy; COOLS, Martine; MARKOSYAN, Renata; BACHEGA, Tania A. S. S.; MIRANDA, Mirela C.; IOTOVA, Violeta; FALHAMMAR, Henrik; CECCATO, Filippo; STANCAMPIANO, Marianna R.; RUSSO, Gianni; VUKOVIC, Rade; GIORDANO, Roberta; MAZEN, Inas; GUVEN, Ayla; DARENDELILER, Feyza; POYRAZOGLU, Sukran; VRIES, Liat de; ELLAITHI, Mona; DANIEL, Eleni; JOHNSTON, Colin; HUNTER, Steven J.; CARROLL, Paul V.; ADAM, Safwaan; PERRY, Colin G.; KEARNEY, Tara; ABRAHAM, Prakash; REES, D. Aled; LEESE, Graham P.; REISCH, Nicole; STIKKELBROECK, Nike M. M. L.; AUCHUS, Richard J.; ROSS, Richard J.; AHMED, S. Faisal
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    Molecular Confirmatory Test Improves the Accuracy of Congenital Adrenal Hyperplasia Diagnosis in Newborn Screening Program
    (2016) MIRANDA, Mirela; SANTOS, Eliane dos; CARVALHO, Daniel de; RODRIGUES, Andressa; NADER, Ivana; AMELIO JUNIOR, Joao; MENDONCA, Berenice; BACHEGA, Tania
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    Global Practice of Glucocorticoid and Mineralocorticoid Treatment in Children and Adults with Congenital Adrenal Hyperplasia - Insights from the I-CAH Registry
    (2019) BACILA, Irina-Alexandra; BLANKENSTEIN, Oliver; NEUMANN, Uta; GRINTEN, Hedi L. Claahsen-van der; KRONE, Ruth; ACERINI, Carlo; BACHEGA, Tania S. S.; MIRANDA, Mirella C.; MENDONCA, Berenice; BIRKEBAEK, Niels H.; COOLS, Martine; MILENKOVIC, Tatjana; BONFIG, Walter; TOMLINSON, Jeremy; ELSEDFY, Heba; BALSAMO, Antonio; HANNEMA, Sabine; HIGHAM, Claire; ATAPATTU, Navoda; LICHIARDOPOL, Corina; GURAN, Tulay; ABALI, Zehra; MOHNIKE, Klaus; FINKEN, Martijn J. J.; VIEITES, Ana; DARENDELILER, Feyza; GUVEN, Ayla; KORBONITS, Marta; VRIE, Liat De; COSTA, Eduardo; EINAUDI, Silvia; KAMP, Hetty van der; IOTOVA, Violeta; ROSS, Richard; AHMED, S. Faisal; KRONE, Nils P.
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    Dramatic Size Reduction with Primary Pharmacological Therapy of a Giant Pituitary Macroadenoma in an Acromegalic Patient
    (2014) MIRANDA, Mirela Costa de; NAKAGUMA, Marilena; DUARTE, Felipe H. G.; JALLAD, Raquel S.; BRONSTEIN, Marcello D.
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    An International Study of the Association between Local Health Care Resources and Acute Adrenal Insufficiency Events in Children with Congenital Adrenal Hyperplasia
    (2022) TSERETOPOULOU, Xanthippi; ALI, Salma R.; BRYCE, Jillian; NAVODA, Atapattu; BIRKEBAEK, Niels H.; BARONIO, Federico; BONFIG, Walter; GRINTEN, Hedi L. Claahsen-van der; COOLS, Martine; DARENDELILER, Feyza; POYRAZOGLU, Sukran; SANCTIS, Luisa de; ELSEDFY, Heba; FINKEN, Martijn J. J.; FLUCK, Christa E.; GEVERS, Evelien; KORBONITS, Marta; GURAN, Tulay; GUVEN, Ayla; HUGHES, Ieuan A.; TADOKORO-CUCCARO, Rieko; THANKAMONY, Ajay; IOTOVA, Violeta; KRONE, Ruth; LICHIARDOPOL, Corina; MENDONCA, Berenice B.; BACHEGA, Tania A. S. S.; MIRANDA, Mirela C.; MILENKOVIC, Tatjana; MOHNIKE, Klaus; NORDENSTROM, Anna; KAMP, Hetty J. van der; AHMED, Syed Faisal