The impact of the distance traveled between residence and gestational trophoblastic neoplasia reference center and clinical outcomes in Brazilian women.

OBJECTIVE: To relate the distance traveled from the patient's residence to the gestational trophoblastic neoplasia (GTN) reference center (RC) and the occurrence of unfavorable clinical outcomes, as well as to estimate the possible association between this distance and the risk of metastatic disease at presentation, the need for multiagent chemotherapy to achieve remission and loss to follow-up before remission. STUDY DESIGN: Retrospective historical cohort study of patients with GTN followed at 8 Brazilian GTN-RC, from January 1st, 2000 - December 31st, 2017. RESULTS: Evaluating 1055 cases of GTN, and using a receiver operating characteristic curve, we found a distance of 56 km (km) from the residence to the GTN-RC (sensitivity = 0.57, specificity = 0.61) best predicted the occurrence of at least one of the following outcomes: occurrence of metastatic disease, need for multiagent chemotherapy to achieve remission, or loss to follow-up during chemotherapy. Multivariate logistic regression adjusted by age, ethnicity, marital status and the reference center location showed that when the distance between residence and GTN-RC was ≥56 km, there was an increase in the occurrence of metastatic disease (relative risk - RR:3.27; 95%CI:2.20-4.85), need for multiagent chemotherapy (RR:1.36; 95%CI:1.05-1.76), loss to follow-up during chemotherapy (RR:4.52; 95CI:1.93-10.63), occurrence of chemoresistance (RR:4.61; 95%CI:3.07-6.93), relapse (RR:10.27; 95%CI:3.08-34.28) and death due to GTN (RR:3.62; 95%CI:1.51-8.67). CONCLUSIONS: The distance between the patient's residence and the GTN-RC is a risk factor for unfavorable outcomes, including death from this disease. It is crucial to guarantee these patients get prompt access to the GTN-RC and receive follow-up support.

Influence of COVID-19 pandemic on molar pregnancy and postmolar gestational trophoblastic neoplasia: An observational study.

OBJECTIVE: To assess whether the incidence and aggressiveness of molar pregnancy (MP) and postmolar gestational trophoblastic neoplasia (GTN) changed during the COVID-19 pandemic. DESIGN: Observational study with two separate designs: retrospective multicentre cohort of patients with MP/postmolar GTN and a cross-sectional analysis, with application of a questionnaire. SETTING: Six Brazilian Reference Centres on gestational trophoblastic disease. POPULATION: 2662 patients with MP/postmolar GTN treated from March-December/2015-2020 were retrospectively evaluated and 528 of these patients answered a questionnaire. METHODS: Longitudinal retrospective multicentre study of patients diagnosed with MP/ postmolar GTN at presentation and a cross-sectional analysis, with application of a questionnaire, exclusive to patients treated during the period of study, to assess living and health conditions during the COVID-19 pandemic compared with previous years. MAIN OUTCOME MEASURES: The incidence of MP/postmolar GTN. RESULTS: Compared with the last 5 pre-pandemic years, MP/postmolar GTN incidence remained stable during 2020 (COVID-19 pandemic). Multivariable logistic regression, adjusted for the patient age, showed that during 2020, presentation with MP was more likely to be >10 weeks of gestation (adjusted odds ratio [aOR] 2.50, 95% confidence interval [CI] 1.90-3.29, P < 0.001), have a pre-evacuation hCG level ≥100 000 iu/l (aOR 1.77, 95% CI 1.38-2.28, P < 0.001) and time to the initiation of chemotherapy ≥7 months (aOR 1.86, 95% CI 1.01-3.43, P = 0.047) when compared with 2015-2019. CONCLUSIONS: Although the incidence of MP/postmolar GTN remained stable during the COVID-19 pandemic in Brazil, the pandemic was associated with greater gestational age at MP diagnosis and more protracted delays in initiation of chemotherapy for postmolar GTN.

Perinatal outcomes of first pregnancy after chemotherapy for gestational trophoblastic neoplasia: a systematic review of observational studies and meta-analysis.

OBJECTIVE: To assess perinatal outcomes of first pregnancy after remission from gestational trophoblastic neoplasia and the impact of the time between the end of chemotherapy and the subsequent pregnancy. DATA SOURCES: The Medical Subject Headings related to perinatal outcomes, chemotherapy, and gestational trophoblastic neoplasia were used alone or in combination to retrieve relevant articles. We searched all references registered until April, 2019 in Embase, LILACS, MEDLINE, the Cochrane Central Register of Controlled Trials, and Web of Science. STUDY ELIGIBILITY CRITERIA: We included any observational or interventional studies that evaluated perinatal outcomes of first pregnancy after chemotherapy for gestational trophoblastic neoplasia. Animal studies, narrative reviews, expert opinions, and previous treatments with potential risks for future perinatal outcomes which may introduce confounding bias were excluded. STUDY APPRAISAL AND SYNTHESIS METHODS: Two reviewers independently screened all identified references for eligibility and data extraction. Methodological quality and bias of included studies were assessed using the Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies from the National Institutes of Health. For the meta-analysis, the measures of association were calculated using bivariate random-effects models. Statistical heterogeneity was evaluated with I2 statistics and explored through sensitivity analysis. Publication bias was assessed by visual inspection of the funnel plot or Egger's test, according to the number of articles included. For all analyses, a P value of <.05 indicated statistical significance. This study was registered on PROSPERO (CRD42018116513). RESULTS: A total of 763 studies were identified after literature search and 23 original studies were included in the systematic review and in the meta-analysis. The combined data from the subgroup meta-analysis (outcome vs time after chemotherapy) showed an incidence of spontaneous abortion of 15.28% (95% confidence interval, 12.37-18.74; I2=73%), 3.30% of malformation (95% confidence interval, 2.27-4.79; I2=31%), 6.19% of prematurity (95% confidence interval, 5.03-7.59; I2=0), and 1.73% of stillbirth (95% confidence interval, 1.17-2.55; I2=0%). These results were not influenced by the time between the end of chemotherapy and the subsequent pregnancy in most of the studied outcomes, including malformation (P=.14, I2=31%), prematurity (P=.46, I2=0), and stillbirth (P=.66, I2=0). However, there was a higher occurrence of spontaneous abortion (P<.01, I2=73%) in pregnancies that occurred ≤6 months after chemotherapy. CONCLUSION: Chemotherapy for gestational trophoblastic neoplasia does not appear to increase the chance of unfavorable perinatal outcomes, except for the higher occurrence of spontaneous abortion in pregnancies occurring ≤6 months after chemotherapy.

Gestational trophoblastic neoplasia lethality among Brazilian women: A retrospective national cohort study.

OBJECTIVE: To investigate GTN lethality among Brazilian women comparing cases of death by GTN with those who survived, thereby identifying factors associated with GTN lethality. METHODS: We retrospectively reviewed medical records of women with GTN treated at ten Brazilian GTN Reference Centers, from January 1960 to December 2017. We evaluated factors associated with death from GTN and used Cox proportional hazards regression models to identify independent variables with significant influence on the risk of death. RESULTS: From 2186 patients with GTN included in this study, 2092 (95.7%) survived and 89 (4%) died due to GTN. When analyzing the relative risk (RR), adjusted for WHO/FIGO score, patients with low risk disease had a significantly higher risk of death if they had choriocarcinoma (RR: 12.40), metastatic disease (RR: 12.57), chemoresistance (RR: 3.18) or initial treatment outside the Reference Center (RR: 12.22). In relation to patients with high-risk GTN, these factors were significantly associated with death due to GTN: the time between the end of antecedent pregnancy and the initiation of chemotherapy (RR: 4.10), metastatic disease (RR: 14.66), especially in brain (RR: 8.73) and liver (RR: 5.76); absence of chemotherapy or initial treatment with single agent chemotherapy (RR: 10.58 and RR: 1.81, respectively), chemoresistance (RR: 3.20) and the initial treatment outside the Reference Center (RR: 28.30). CONCLUSION: The risk of mortality from low and high-risk GTN can be reduced by referral of these patients to a Reference Center or, if not possible, to involve clinicians in a Reference Center with consultation regarding management.

Is chemotherapy always necessary for patients with nonmetastatic gestational trophoblastic neoplasia with histopathological diagnosis of choriocarcinoma?

OBJECTIVE: To evaluate expectant management versus immediate chemotherapy following pathological diagnosis of gestational choriocarcinoma (GCC) in patients with nonmetastatic disease. METHODS: Multicenter retrospective cohort that included patients with histological diagnosis of GCC with nonmetastatic disease followed at one of thirteen Brazilian referral centers for gestational trophoblastic disease from January 2000 to December 2016. RESULTS: Among 3191 patients with gestational trophoblastic neoplasia, 199 patients with nonmetastatic GCC were identified. Chemotherapy was initiated immediately in 152 (76.4%) patients per FIGO 2000 guideline, while 47 (23.6%) were managed expectantly. Both groups presented with similar characteristics and outcomes. All patients (n=12) who had normal human chorionic gonadotropin (hCG) in the first 2-3weeks of expectant management achieved complete sustained remission with no chemotherapy. Only 44.7% (21 patients) of patients who were expectantly managed needed to receive chemotherapy due to plateauing or rising hCG level in the first 2-3weeks of follow up. The outcome of patients receiving chemotherapy after initial expectant management was similar to those who received chemotherapy immediately after the diagnosis in terms of need for multi-agent chemotherapy or number of cycles of chemotherapy. There was no case of relapse or death in either group. Logistic regression analysis showed that age≥40years and hCG≥92,428IU/L at GCC diagnosis were risk factors for needing chemotherapy after initial expectant management of nonmetastatic GCC. CONCLUSION: In order to avoid exposing patients unnecessarily to chemotherapy, close surveillance of women with pathological diagnosis of nonmetastatic GCC seems to be a safe practice, particularly for those who have a normal hCG at the time of diagnosis. If confirmed by other studies, the FIGO guidelines may need to be revised.

Is chemotherapy necessary for patients with molar pregnancy and human chorionic gonadotropin serum levels raised but falling at 6months after uterine evacuation?

OBJECTIVE: To compare the outcomes of Brazilian patients with molar pregnancy who continue human chorionic gonadotropin (hCG) surveillance with those treated with chemotherapy when hCG was still positive, but falling at 6months after uterine evacuation. METHODS: Retrospective chart review of 12,526 patients with hydatidiform mole treated at one of nine Brazilian reference centers from January 1990 to May 2016. RESULTS: At 6months from uterine evacuation, 96 (0.8%) patients had hCG levels raised but falling. In 15/96 (15.6%) patients, chemotherapy was initiated immediately per FIGO 2000 criteria, while 81/96 (84.4%) patients were managed expectantly. Among the latter, 65/81 (80.2%) achieved spontaneous remission and 16 (19.8%) developed postmolar gestational trophoblastic neoplasia (GTN). Patients who received chemotherapy following expectant management required more time for remission (11 versus 8months; p=0.001), had a greater interval between uterine evacuation and initiating chemotherapy (8 versus 6months; p<0.001), and presented with a median WHO/FIGO risk score higher than women treated according to FIGO 2000 criteria (4 versus 2, p=0.04), but there were no significant differences in the need for multiagent treatment regimens (1/15 versus 3/16 patients, p=0.60). None of the women relapsed, and no deaths occurred in either group. CONCLUSION: In order to avoid unnecessary exposure of women to chemotherapy, we no longer follow the FIGO 2000 recommendation to treat all patients with molar pregnancy and hCG raised but falling at 6months after evacuation. Instead, we pursue close hormonal and radiological surveillance as the best strategy for these patients.

Quality of life and adherence to antiretroviral therapy in Southern Brazil.

The absence of or limited adherence to treatment is the main cause for the failure of Highly Active Antiretroviral Therapy (HAART). In Brazil, adherence to antiretroviral therapy has been lower than the recommended levels. Although HAART may produce adverse reactions, failure to comply with it may aggravate patients' health status and impair Quality of Life (QoL). The QoL of individuals living chronically with HIV and AIDS has been considered one of the main treatment outcomes. This study is part of a 225-day prospective trial in which participants were enrolled in two different modalities of follow-up: the usual model medical follow-up or an intervention based on the Medication Adherence Training Instrument (MATI). The WHOQOL-HIV BREF questionnaire was used to evaluate QoL of research participants in both groups on the 15th and 225th days of follow-up after the baseline assessment. The result of this study revealed no significant differences of WHOQOL-HIV BREF scores between participants allocated to MATI and non-MATI groups in the first assessment. However, there was a significant difference between the scores obtained on the 15th and 225th days in the domain related to spirituality and personal beliefs irrespective of the modality of follow-up. Other domains of the WHOQOL-HOV BREF remained unchanged. These results indicate that, in this sample, personal beliefs and spirituality may be relevant subjects to explain sustained levels of adherence to HAART.

Epidemiological report on the treatment of patients with gestational trophoblastic disease in 10 Brazilian referral centers: results after 12 years since International FIGO 2000 Consensus.

OBJECTIVE: To evaluate treatment of Brazilian patients with gestational trophoblastic disease (GTD). STUDY DESIGN: A retrospective cohort study with analysis of medical reports performed in 10 Brazilian referral centers from January 2000 to December 2011. RESULTS: Of 5,250 patients 3 died (0.06%) at the time of uterine evacuation. Spontaneous remission of GTD (group G1) was observed in 4,103 cases, and 1,144 (21.8%) progressed to gestational trophoblastic neoplasia (GTN) (G2). In G1 2,716 (66.2%) had complete hydatidiform mole (HM) and 1,210, partial HM (29.5%); 3,772 patients (92.7%) recovered as noted in December 2012. In G2, of 1,118 patients treated, initial histopathological results of previous gestation were complete HM (77.5% [n = 886]), partial HM (8.8% [n = 100]), and choriocarcinoma (8.0% [n = 92]); 930 (81.3%) were low-risk, 200 (17.5%) were high-risk GTN, and 14 had placental site trophoblastic tumor (PSTT) (1.2%); cure was achieved in 1,078 cases (96.4%), but 26 patients (2.3%) died (4 low-risk [0.4%], 19 high-risk [9.5%], and 3 PSTT [21.4%]). CONCLUSION: The highest death rates were due to high-risk GTN and PSTT. Patients with molar pregnancy should be referred to a referral center for an early diagnosis and prompt treatment of GTN in order to reduce the morbidity and mortality found in advanced stages.

Immunotherapy in the treatment of chemoresistant gestational trophoblastic neoplasia - systematic review with a presentation of the first 4 Brazilian cases.

OBJECTIVE: To evaluate the efficacy of immunotherapy for GTN treatment after methotrexate-resistance or in cases of multiresistant disease, through a systematic review, as well as to present the first 4 Brazilian cases of immunotherapy for GTN treatment. METHODS: Three independent researchers searched five electronic databases (EMBASE, LILACS, Medline, CENTRAL and Web of Science), for relevant articles up to February/2023 (PROSPERO CRD42023401453). The quality assessment was performed using the Newcastle Ottawa scale for case series and case reports. The primary outcome of this study was the occurrence of complete remission. The presentation of the case reports was approved by the Institutional Review Board. RESULTS: Of the 4 cases presented, the first was a low-risk GTN with methotrexate resistance unsuccessfully treated with avelumab, which achieved remission with sequential multiagent chemotherapy. The remaining 3 cases were high-risk multiagent-resistant GTN that were successfully treated with pembrolizumab, among which there were two subsequent gestations, one of them with normal pregnancy and healthy conceptus. Regarding the systematic review, 12 studies were included, only one of them on avelumab, showing a 46.7% complete remission rate. The remaining 11 studies were on pembrolizumab, showing an 86.7% complete remission rate, regardless of tumor histology. Both immunotherapies showed good tolerability, with two healthy pregnancies being recorded: one after avelumb and another after pembrolizumab. CONCLUSION: Immunotherapy showed effectiveness for GTN treatment and may be especially useful in cases of high-risk disease, where pembrolizumab achieves a high therapeutic response, regardless of the histological type, and despite prior chemoresistance to multiple lines of treatment.

Epidemiological profile of congenital hypothyroidism at a southern Brazilian state.

Objective: To determine the incidence of congenital hypothyroidism (CH) over a 10-year period at the Reference Service in Neonatal Screening of the state of Rio Grande do Sul (RSNS-RS). Subjects and methods: Historical cohort study including all newborns screened for CH by the RSNS-RS from January 2008 until December 2017. Data of all newborns with neonatal TSH (neoTSH; heel prick test) values ≥ 9 mIU/L were collected. According to neoTSH values, the newborns were allocated into two groups: Group 1 (G1), comprising newborns with neoTSH ≥ 9 mIU/L and serum TSH (sTSH) < 10 mIU/L, and Group 2 (G2), comprising those with neoTSH ≥ 9 mIU/L and sTSH ≥ 10 mIU/L. Results: Of 1,043,565 newborns screened, 829 (0.08%) had neoTSH values ≥ 9 mIU/L. Of these, 284 (39.3%) had sTSH values < 10 mIU/L and were allocated to the G1 group, while 439 (60.7%) had sTSH ≥ 10 mIU/L and were allocated to the G2 group, and 106 (12.7%) were considered missing data. The overall incidence of CH was 42.1 per 100,000 newborns screened (95% confidence interval [CI] 38.5-45.7/100,000) or 1:2377 screened newborns. The sensibility and specificity of neoTSH ≥ 9 mIU/L were 97% and 11%; of neoTSH 12.6 mUI/L, 73% and 85% respectively. Conclusion: In this population, the incidence of permanent and transitory CH was 1:2377 screened newborns. The neoTSH cutoff value adopted during the study period showed excellent sensibility, which matters for a screening test.

Continuous glucose monitoring in obese pregnant women with no hyperglycemia on glucose tolerance test.

OBJECTIVE: The objective of the present study was to compare 24-hour glycemic levels between obese pregnant women with normal glucose tolerance and non-obese pregnant women. METHODS: In the present observational, longitudinal study, continuous glucose monitoring was performed in obese pregnant women with normal oral glucose tolerance test with 75 g of glucose between the 24th and the 28th gestational weeks. The control group (CG) consisted of pregnant women with normal weight who were selected by matching the maternal age and parity with the same characteristics of the obese group (OG). Glucose measurements were obtained during 72 hours. RESULTS: Both the groups were balanced in terms of baseline characteristics (age: 33.5 [28.7-36.0] vs. 32.0 [26.0-34.5] years, p = 0.5 and length of pregnancy: 25.0 [24.0-25.0] vs. 25.5 [24.0-28.0] weeks, p = 0.6 in the CG and in the OG, respectively). Pre-breakfast glycemic levels were 77.77 ± 10.55 mg/dL in the CG and 82.02 ± 11.06 mg/dL in the OG (p<0.01). Glycemic levels at 2 hours after breakfast were 87.31 ± 13.10 mg/dL in the CG and 93.48 ± 18.74 mg/dL in the OG (p<0.001). Daytime blood glucose levels were 87.6 ± 15.4 vs. 93.1 ± 18.3 mg/dL (p<0.001) and nighttime blood glucose levels were 79.3 ± 15.8 vs. 84.7 ± 16.3 mg/dL (p<0.001) in the CG and in the OG, respectively. The 24-hour, daytime, and nighttime values of the area under the curve were higher in the OG when compared with the CG (85.1 ± 0.16 vs. 87.9 ± 0.12, 65.6 ± 0.14 vs. 67.5 ± 0.10, 19.5 ± 0.07 vs. 20.4 ± 0.05, respectively; p<0.001). CONCLUSION: The results of the present study showed that obesity in pregnancy was associated with higher glycemic levels even in the presence of normal findings on glucose tolerance test.

The Portuguese Version of the Schedule of Racist Events.

OBJECTIVE: There is a lack of research tools in Portuguese to evaluate racial discrimination. The purpose of this study was to psychometrically assess the Portuguese version of the Schedule of Racist Events (SRE) in a sample of individuals enrolled in a research trial with antiretroviral medications in southern Brazil. METHODS: Sample of 147 individuals living with HIV and/or AIDS. Research participants completed the Schedule of Racist Events and the WHOQOL-HIV BREF questionnaires. RESULTS: The SRE scores of non-white participants were significantly higher than the scores of white-participants. The Crombach's alpha coefficients, for the three subscales of the Portuguese version of the SRE, were high and significant. There were significant correlations between all subscales of the SRE and relevant domains of the WHOQOL-HIV BREF. CONCLUSION: The original English version of the SRE was successfully adapted to Portuguese. The Portuguese version of SRE constitutes a valid research instrument for evaluating racial discrimination.

Gestational Diabetes in the Population Served by Brazilian Public Health Care. Prevalence and Risk Factors.

OBJECTIVE: To assess the prevalence of gestational diabetes mellitus and the main associated risk factors in the population served by the Brazilian Unified Health System in the city of Caxias do Sul, state of Rio Grande do Sul. MATERIALS AND METHODS: A descriptive, cross-sectional and retrospective study was conducted. Maternal variables were collected from the medical records of all pregnant women treated at the basic health units in 2016. Hyperglycemia during pregnancy (pregestational diabetes, overt diabetes and gestational diabetes mellitus) was identified by analyzing the results of a 75-g oral glucose tolerance test, as recommended by the Brazilian Ministry of Health. Based on the data, the women were allocated into two groups: the gestational diabetes group and the no gestational diabetes group. RESULTS: The estimated prevalence of gestational diabetes among 2,313 pregnant women was of 5.4% (95% confidence interval [95%CI]: 4.56-6.45). Pregnant women with 3 or more pregnancies had twice the odds of having gestational diabetes compared with primiparous women (odds ratio [OR] = 2.19; 95%CI: 1.42-3.37; p < 0.001). Pregnant women aged 35 years or older had three times the odds of having gestational diabetes when compared with younger women (OR = 3.01; 95%CI: 1.97-4.61; p < 0.001). Overweight pregnant women were 84% more likely to develop gestational diabetes than those with a body mass index lower than 25 kg/m2 (OR = 1.84; 95%CI: 1.25-2.71; p = 0.002). A multivariable regression analysis showed that being overweight and being 35 years old or older were independent variables. CONCLUSION: In this population, the prevalence of gestational diabetes mellitus was of 5.4%. Age and being overweight were predictive factors for gestational diabetes.

OBJETIVO: Avaliar a prevalência de diabetes mellitus gestacional, e dos principais fatores de risco associados, em população usuária do Sistema Único de Saúde em Caxias do Sul-RS. MéTODOS: Um estudo descritivo, transversal e retrospectivo foi feito. As variáveis maternas foram coletadas de registros de prontuários de todas gestantes atendidas nas Unidades Básicas de Saúde do município em 2016. A identificação de hiperglicemia na gestação (diabetes pré-gestacional, diabetes identificado durante a gestação e diabetes mellitus gestacional) foi feita pela avaliação dos resultados do teste oral de tolerância com 75 g glicose, conforme preconizado pelo Ministério da Saúde. Com base nesses dados, as gestantes foram separadas em dois grupos: o grupo com diabetes gestacional e o grupo sem diabetes gestacional. RESULTADOS: A prevalência estimada de diabetes gestacional em 2.313 gestantes foi de 5,4% (intervalo de confiança de 95% [IC95%]: 4,56­6,45). Gestantes com 3 ou mais gestações apresentaram chance 2 vezes maior para a ocorrência de diabetes gestacional, quando comparadas às primigestas (razão de possibilidades [RP] = 2,19; IC95%: 1,42- 3,37; p < 0,001). Gestantes com idade de 35 anos ou mais apresentaram chance três vezes maior do que as mais jovens (RP = 3,01; IC95%: 1,97­4,61; p < 0,001). A chance de desenvolver diabetes gestacional em gestantes com sobrepeso foi 84% maior do que a das com índice de massa corporal inferior a 25 kg/m2 (RP = 1,84; IC95%: 1,25­2,71; p = 0,002). A análise de regressão multivariada mostrou sobrepeso e idade materna como variáveis com associação independente. CONCLUSãO: Nesta população, a prevalência de diabetes mellitus gestacional foi de 5,4%. Idade materna e sobrepeso pré-gestacional foram fatores preditivos para diabetes gestacional.

Ectopia Cordis Associated with Pentalogy of Cantrell-A Case Report.

Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by changes in the mesodermal median structures and congenital heart disease, often with a poor prognosis. In 1958, Cantrell et al2 defined the full spectrum of the syndrome with the following anomalies: defects of the anterior diaphragm, of the lower part of the sternum, of the supraumbilical region and the abdominal wall, of the diaphragmatic pericardium, and various intracardiac congenital abnormalities. The present report describes a case of ectopia cordis associated with PC and the importance of the participation of a multidisciplinary team in the treatment of this condition.

A pentalogia de Cantrell (PC) é uma rara anomalia congênita caracterizada por alterações nas estruturas medianas mesodérmicas e doenças cardíacas congênitas, cursando muitas vezes com um mau prognóstico. Em 1958, Cantrell et al2 definiram o espectro completo da síndrome com as seguintes anomalias: defeitos do diafragma anterior, da parte inferior do esterno, da região supraumbilical e parede abdominal, do pericárdio diafragmático, e várias anormalidades congênitas intracardíacas. O presente relato relaciona-se a um caso de ectopia cordis associado à PC e à importância da participação de uma equipe multidisciplinar no acompanhamento da doença.

Análise do desejo de manter ou interromper gravidezes decorrentes de violência sexual / Analysis of the desire to maintain or interrupt pregnancies resulting from sexual assault

Objetivo: Avaliar o desejo de gestantes, vítimas de violência sexual, em manter ou interromper a gravidez. Métodos: Estudo transversal retrospectivo que avaliou o desejo da gestante vítima de violência sexual pela manutenção ou interrupção da gravidez, no Programa de Atendimento às Vítimas de Violência Sexual, no período de janeiro de 2019 a setembro de 2022. Resultados: Foram acolhidas 67 gestantes vítimas de violência sexual que procuraram atendimento com vistas a orientação, manutenção ou interrupção da gestação. Entre elas, 56 (83,6%) pacientes solici- taram a interrupção da gestação e para 32 (57,2%) a solicitação foi aceita; 9 (16%) não tiveram a solicitação de interrupção autorizada por equipe multidisciplinar e mantiveram a gestação; 11 (16,4%) não solicitaram a interrupção e também optaram pela manutenção da gestação. A média das idades foi de 26 anos. Em sua maio- ria, eram solteiras, brancas e procedentes de munícipios do entorno de Caxias do Sul. O agressor era quase sempre desconhecido, e a agressão teria ocorrido pre- dominantemente na residência da vítima ou do agressor. Conclusão: No período estudado, foram avaliadas 67 gestações decorrentes de violência sexual. Dessas, 56 pacientes solicitaram a interrupção da gestação e 32 tiveram a solicitação aceita; nove não tiveram a solicitação de interrupção autorizada e mantiveram a gestação; 11 não solicitaram a interrupção e também optaram pela manutenção da gestação. Não cabe ao médico julgar a decisão da vítima, mas, sim, acolher e ofertar o seu direito de escolha.

Objective: To evaluate the desire of pregnant women, victims of sexual violence, to maintain or terminate pregnancy. Methods: Retrospective cross-sectional study that evaluated the desire of pregnant women victims of sexual violence for the mainte- nance or interruption of pregnancy in Program to Assist Victims of Sexual Assault, from January 2019 to September 2022. Results: Sixty-seven pregnant women who were victim of sexual assault and who sought care for maintenance or interruption of pregnancy were received. Among these, 56 (83.6%) patients requested the interruption of pregnancy and in 32 (57.2%) cases the request was accepted; 9 (16%) didn't have the interruption request authorized by the multidisciplinary team and have kept the pregnancy; 11 (16.4%) didn't request the interruption and have chosen to keep the pregnancy. The average age was 26 years. They were single, white, and mostly from cities around Caxias do Sul. The aggressor was almost always unknown, and the assault occurred mostly at the victim's or aggressor's home. Conclusion: Sixty-seven pregnancies resulting from sexual assault were evaluated during the period of the study. Fifty-sixth patients of those have requested pregnancy termination and 32 had their request accepted; nine didn't have their request for termination authorized and have kept their pregnancy; eleven didn't request termination and have chosen to keep their pregnancy. It isn't up to the physician to judge the victim's decision, but to welcome and offer her the right to choose.

Epidemiological profile of congenital hypothyroidism at a southern Brazilian state

ABSTRACT Objective: To determine the incidence of congenital hypothyroidism (CH) over a 10-year period at the Reference Service in Neonatal Screening of the state of Rio Grande do Sul (RSNS-RS). Subjects and methods: Historical cohort study including all newborns screened for CH by the RSNS-RS from January 2008 until December 2017. Data of all newborns with neonatal TSH (neoTSH; heel prick test) values ≥ 9 mIU/L were collected. According to neoTSH values, the newborns were allocated into two groups: Group 1 (G1), comprising newborns with neoTSH ≥ 9 mIU/L and serum TSH (sTSH) < 10 mIU/L, and Group 2 (G2), comprising those with neoTSH ≥ 9 mIU/L and sTSH ≥ 10 mIU/L. Results: Of 1,043,565 newborns screened, 829 (0.08%) had neoTSH values ≥ 9 mIU/L. Of these, 284 (39.3%) had sTSH values < 10 mIU/L and were allocated to the G1 group, while 439 (60.7%) had sTSH ≥ 10 mIU/L and were allocated to the G2 group, and 106 (12.7%) were considered missing data. The overall incidence of CH was 42.1 per 100,000 newborns screened (95% confidence interval [CI] 38.5-45.7/100,000) or 1:2377 screened newborns. The sensibility and specificity of neoTSH ≥ 9 mIU/L were 97% and 11%; of neoTSH 12.6 mUI/L, 73% and 85% respectively. Conclusion: In this population, the incidence of permanent and transitory CH was 1:2377 screened newborns. The neoTSH cutoff value adopted during the study period showed excellent sensibility, which matters for a screening test.

Immunotherapy in the treatment of chemoresistant gestational trophoblastic neoplasia - systematic review with a presentation of the first 4 Brazilian cases

Abstract Objective To evaluate the efficacy of immunotherapy for GTN treatment after methotrexate-resistance or in cases of multiresistant disease, through a systematic review, as well as to present the first 4 Brazilian cases of immunotherapy for GTN treatment. Methods Three independent researchers searched five electronic databases (EMBASE, LILACS, Medline, CENTRAL and Web of Science), for relevant articles up to February/2023 (PROSPERO CRD42023401453). The quality assessment was performed using the Newcastle Ottawa scale for case series and case reports. The primary outcome of this study was the occurrence of complete remission. The presentation of the case reports was approved by the Institutional Review Board. Results Of the 4 cases presented, the first was a low-risk GTN with methotrexate resistance unsuccessfully treated with avelumab, which achieved remission with sequential multiagent chemotherapy. The remaining 3 cases were high-risk multiagent-resistant GTN that were successfully treated with pembrolizumab, among which there were two subsequent gestations, one of them with normal pregnancy and healthy conceptus. Regarding the systematic review, 12 studies were included, only one of them on avelumab, showing a 46.7% complete remission rate. The remaining 11 studies were on pembrolizumab, showing an 86.7% complete remission rate, regardless of tumor histology. Both immunotherapies showed good tolerability, with two healthy pregnancies being recorded: one after avelumb and another after pembrolizumab. Conclusion Immunotherapy showed effectiveness for GTN treatment and may be especially useful in cases of high-risk disease, where pembrolizumab achieves a high therapeutic response, regardless of the histological type, and despite prior chemoresistance to multiple lines of treatment.

Group B Streptococcus detection in pregnant women via culture and PCR methods.

INTRODUCTION:: Group B Streptococcus (GBS), a source of neonatal infection, colonizes the gastrointestinal and genitourinary tracts of pregnant women. Routine screening for maternal GBS in late pregnancy and consequent intrapartum antibiotic prophylaxis have reduced the incidence of early-onset GBS neonatal infection. The aim of this study was to evaluate the performance of PCR, compared to culture (gold standard), in GBS colonization screening of pregnant women, and to establish the prevalence of GBS colonization among this population. METHODS:: Vaginal introitus and perianal samples were collected from 204 pregnant women, between the 35th and 37th weeks of pregnancy, at the Obstetrics and Gynecology Unit of the University of Caxias do Sul General Hospital between June 2008 and September 2009. All samples were cultured after enrichment in a selective medium and then assayed by culture and PCR methods. RESULTS:: The culture and PCR methods yielded detection rates of vaginal/perianal GBS colonization of 22.5% and 26%, respectively (sensitivity 100%; specificity 95.6%; positive and negative predictive values 86.8% and 100%, respectively). A higher prevalence of GBS colonization was detected in the combined vaginal and perianal samples by both culture and PCR assay analyses. CONCLUSIONS:: PCR is a faster and more efficient method for GBS screening, allowing for optimal identification of women who should receive intrapartum antibiotic prophylaxis to prevent newborn infection.

Effect of Obesity on Gestational and Perinatal Outcomes.

Purpose To assess the impact of pre-pregnancy obesity (body mass index [BMI] ≥ 30 kg/m2) on the gestational and perinatal outcomes. Methods Retrospective cohort study of 731 pregnant women with a BMI ≥ 30 kg/m2 at the first prenatal care visit, comparing them with 3,161 women with a BMI between 18.5 kg/m2 and 24.9 kg/m2. Maternal and neonatal variables were assessed. Statistical analyses reporting the demographic features of the pregnant women (obese and normal) were performed with descriptive statistics followed by two-sided independent Student's t tests for the continuous variables, and the chi-squared (χ2) test, or Fisher's exact test, for the categorical variables. We performed a multiple linear regression analysis of newborn body weight based on the mother's BMI, adjusted by maternal age, hyperglycemic disorders, hypertensive disorders, and cesarean deliveries to analyze the relationships among these variables. All analyses were performed with the R (R Foundation for Statistical Computing, Vienna, Austria) for Windows software, version 3.1.0. A value of p < 0.05 was considered statistically significant. Results Obesity was associated with older age [OR 9.8 (7.8-12.2); p < 0.01], hyperglycemic disorders [OR 6.5 (4.8-8.9); p < 0.01], hypertensive disorders [OR 7.6 (6.1-9.5); p < 0.01], caesarean deliveries [OR 2.5 (2.1-3.0); p < 0.01], fetal macrosomia [OR 2.9 (2.3-3.6); p < 0.01] and umbilical cord pH [OR 2.1 (1.4-2.9); p < 0.01). Conversely, no association was observed with the duration of labor, bleeding during labor, Apgar scores at 1 and 5 minutes after birth, gestational age, stillbirth and early neonatal mortality, congenital malformations, and maternal and fetal injury. Conclusion We observed that pre-pregnancy obesity was associated with maternal age, hyperglycemic disorders, hypertension syndrome, cesarean deliveries, fetal macrosomia, and fetal acidosis.

Objetivo Avaliar o impacto da obesidade pré-gestacional (índice de massa corpórea [IMC] ≥ 30 kg/m2) sobre os resultados gestacionais e perinatais. Métodos Estudo transversal retrospectivo, com 731 gestantes que apresentaram IMC ≥ 30 kg/m2 na primeira consulta de pré-natal, comparando-as a 3.161 gestantes com IMC entre 18,5 kg/m2 e 24,9 kg/m2. Foram avaliadas variáveis maternas e neonatais. A análise estatística baseou-se nas características demográficas das gestantes (obesas e com peso normal), e foi realizada com estatísticas descritivas seguidas de testes t de Student independentes bicaudais para variáveis contínuas, e teste de qui-quadrado (χ2) ou exato de Fisher para as variáveis categóricas. Foi realizada uma regressão linear múltipla do peso do recém-nascido sobre o IMC materno, ajustado por idade materna, síndromes hiperglicêmicas, síndromes hipertensivas hipertensivas e operações cesarianas, a fim de analisar a relação entre essas variáveis. Todas as análises foram realizadas com o uso de R (R Foundation for Statistical Computing, Viena, Áustria) para Windows, versão 3.1.0. Um valor de p < 0,05 foi considerado estatisticamente significante. Resultados A obesidade associou-se à idade materna [OR 9,8 (7,8­12,2); p < 0,01], distúrbios hiperglicêmicos [OR 6.5 (4,8­8,9); p < 0,01], distúrbios hipertensivos (RP: 7,6 [6,1­9,5]; p < 0,01), maior taxa de operação cesariana [OR 2,5 (2,1­3,0); p < 0,01], macrossomia fetal [OR 2,9 (2,3­3,6); p < 0,01] e baixo pH na artéria umbilical [OR 2,1 (1,4­2,9); p < 0,01]. Não foi observada associação com tempo de trabalho de parto, sangramento durante o trabalho de parto, índice de Apgar no 1° e 5° minutos, idade gestacional, natimortalidade e mortalidade neonatal precoce, malformações congênitas e tocotraumatismo materno e fetal. Conclusões O estudo mostrou que a obesidade pré-gestacional associou-se com idade materna mais elevada, distúrbios hiperglicêmicos e hipertensivos, taxas mais altas de operação cesariana, macrossomia e acidose fetal.

Accuracy of p57KIP2 compared with genotyping for the diagnosis of complete hydatidiform mole: protocol for a systematic review and meta-analysis.

BACKGROUND: Distinguishing hydatidiform moles (HMs) from non-molar specimens and the subclassification of HM are important because complete hydatidiform mole (CHM) is associated with an increased risk of gestational trophoblastic neoplasia. However, diagnosis based solely on morphology has poor interobserver reproducibility. Recent studies have demonstrated that the use of p57KIP2 immunostaining improves diagnostic accuracy for CHM. METHODS: We will conduct a systematic review of prospective and retrospective studies to evaluate the accuracy of p57KIP2 immunostaining compared with molecular genotyping for the diagnosis of CHM. A high-sensitivity search strategy will be employed in MEDLINE, EMBASE, LILACS, The Grey Literature Report, OpenGrey, OAIster, and Cochrane CENTRAL. Two reviewers will independently screen all identified references for eligibility and extract data. The methodological quality and bias of the included studies will be assessed according to the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool, and the overall quality of evidence will be assessed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. If a meta-analysis is possible, pooled estimates of sensitivity, specificity, and positive and negative likelihood ratios will be calculated using bivariate random-effects models. Statistical heterogeneity will be evaluated with I 2 statistics and explored through sensitivity analysis. DISCUSSION: There is considerable overlap between the histological features of molar and non-molar pregnancies and between complete and partial HMs, which results in significant interobserver variability in the diagnosis of CHM and its mimics. Therefore, molecular techniques are used to correctly diagnosis and treat CHM. However, these molecular diagnostic methods are technically difficult to perform, relatively costly, and unavailable in most pathology laboratories. According to our results, p57KIP2 immunostaining appears to be a practical and accurate adjunct for the diagnosis of CHM and its mimics because this technique is relatively simple, reliable, cost-efficient, and rapid. This systematic review will help to determine whether p57KIP2 immunostaining is an adequate alternative diagnostic test for CHM. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42015024181.