<p>This thesis is about HELLP Syndrome (hemolysis, elevated liver enzymes, low platelet count in pregnancy): a devastating maternal hypertensive complication that results in multi-system changes that can rapidly deteriorate into organ failure and death. Despite rapid advancesin medical technology and medical science this disease continues to take&nbsp / the lives of women and their infants. The only effective intervention for this disorder is immediate termination irrespective of the gestational stage of the pregnancy. The primary objective of this thesis was to explore the subjective experiences and meaningmaking processes of women in and through their high-risk pregnancies. This objective crystallised into the following aims: to facilitate and listen to the voices of women who were HELLP Syndrome survivors / to explore the reported bodily, psychological and&nbsp / emotional experiences of HELLP Syndrome survivors / to understand the role medical intervention and biomedical discourses play in these women&rsquo / s experiences and finally to explore the subjective experiences of HELLP Syndrome in the context of traditionallyheld notions of motherhood. The study was couched in a feminist poststructuralist&nbsp / epistemology. A material-discursive framework which comprised phenomenological and poststructuralist theorising was usedin an attempt to understand both the lived experiences as well as the discursively constructed nature of those subjective experiences. Thus the analysis encompassed both a broadly phenomenological framework to understand the lived experiences of HELLP Syndrome, and a discourse analysis to explore the meaning-making processes of participants in relation to larger social&nbsp / discourses, in particular the dominant biomedical and motherhood discourses. A qualitative approach using in depth semi-structured interviews was utilisedto gather data. Eleven participants from very diverse backgrounds consented to be part of thisstudy. The findings of the study highlighted the immense trauma, difficulties and challenges participants faced in these high-risk situations. What was evident from the analysis was that their experiences were so diverse and werecompletely shaped by the severity of the disorder and the gestational stage of the pregnancy. Some women ended up in the Intensive Care Units (ICU) and had near-death experiences, some had very premature babies, while some of the participants lost their babies during the process. With regards to the emotional, psychological and corporeal aspects of the disorder,participants described their situations as a disaster, painful and difficult. Due to the rapid deterioration of symptoms, they described the tempo of these events as a whirlwind in which they felt they had no control. Emotions ranged from shock, total disbelief and surprise to anger, helplessness and powerlessness. Lacking knowledge and access to appropriate&nbsp / information further compounded the situation for participants. Theparticipants who had premature babies found the Neonatal Intensive Care Unit experience (NICU) extremely challenging and stressful. A discourse analysis revealed that women&rsquo / s talk was shaped by the disciplinary frameworks oftechnocratic medicine and patriarchal notions of&nbsp / gender. Participants&rsquo / discourses about their encounters inthe medical context werelocated in, and shaped by, the structure of health care in our country. In this regard binaries&nbsp / (like private versus public health care, women versus men and nurses versus doctors) were evident. Furthermore their hospital stay reflected their experiences in the Intensive&nbsp / Care (ICU) and the Neonatal Intensive Care Units (NICU) both of which are highly technologically orientated and managed. Biomedical discourses that filtered through the&nbsp / participants&rsquo / talk were: medicine as indisputable truth / mechanistic model of the body as machine / medical doctors as gods and the foetus as &lsquo / super subject&rsquo / . Discourses of risk&nbsp / were inevitably taken up as participants tried to make sense of both their current pregnancies and the potential ones to follow. The passage into motherhood for these&nbsp / participants was dependent on whether they had live babies or not. For those who had live babies it was a difficult time as they had to contend with their own recovery as well as the prematurity of their infants. The NICU experience was described as tiring, trying and cumbersome. For mothers who lost their babies it was a time of profound sadness and&nbsp / loss coupled to the notion that motherhood itself was lost. This loss of their children symbolised broken dreams, severed connections and a powerful taboo. In addition, discourses in which motherhood was naturalised and normalised saturated their talk and framed their experience in a narrative of deficit and failure. The ideologies of mother&nbsp / blame and the &lsquo / all responsible&rsquo / mother were pervasive in their discussions. In conclusion, this high-risk situation represented a time of tremendous uncertainty and unpredictability for all participants and was powerfully shaped by dominant discourses about motherhood and the biomedical discursive and institutional framework in which&nbsp / participants were subjugated. The study thus highlights how the HELLP syndrome experience illuminates the erasure of women&rsquo / s subjectivities while the foetus/infants&rsquo / life&nbsp / takes precedence. This has significant implications for scholarship in general and feminist scholarship in particular and highlights the need for this type of engagement in an area that has remained on the periphery of feminist research.</p>

<p>This thesis is about HELLP Syndrome (hemolysis, elevated liver enzymes, low platelet count in pregnancy): a devastating maternal hypertensive complication that results in multi-system changes that can rapidly deteriorate into organ failure and death. Despite rapid advancesin medical technology and medical science this disease continues to take&nbsp / the lives of women and their infants. The only effective intervention for this disorder is immediate termination irrespective of the gestational stage of the pregnancy. The primary objective of this thesis was to explore the subjective experiences and meaningmaking processes of women in and through their high-risk pregnancies. This objective crystallised into the following aims: to facilitate and listen to the voices of women who were HELLP Syndrome survivors / to explore the reported bodily, psychological and&nbsp / emotional experiences of HELLP Syndrome survivors / to understand the role medical intervention and biomedical discourses play in these women&rsquo / s experiences and finally to explore the subjective experiences of HELLP Syndrome in the context of traditionallyheld notions of motherhood. The study was couched in a feminist poststructuralist&nbsp / epistemology. A material-discursive framework which comprised phenomenological and poststructuralist theorising was usedin an attempt to understand both the lived experiences as well as the discursively constructed nature of those subjective experiences. Thus the analysis encompassed both a broadly phenomenological framework to understand the lived experiences of HELLP Syndrome, and a discourse analysis to explore the meaning-making processes of participants in relation to larger social&nbsp / discourses, in particular the dominant biomedical and motherhood discourses. A qualitative approach using in depth semi-structured interviews was utilisedto gather data. Eleven participants from very diverse backgrounds consented to be part of thisstudy. The findings of the study highlighted the immense trauma, difficulties and challenges participants faced in these high-risk situations. What was evident from the analysis was that their experiences were so diverse and werecompletely shaped by the severity of the disorder and the gestational stage of the pregnancy. Some women ended up in the Intensive Care Units (ICU) and had near-death experiences, some had very premature babies, while some of the participants lost their babies during the process. With regards to the emotional, psychological and corporeal aspects of the disorder,participants described their situations as a disaster, painful and difficult. Due to the rapid deterioration of symptoms, they described the tempo of these events as a whirlwind in which they felt they had no control. Emotions ranged from shock, total disbelief and surprise to anger, helplessness and powerlessness. Lacking knowledge and access to appropriate&nbsp / information further compounded the situation for participants. Theparticipants who had premature babies found the Neonatal Intensive Care Unit experience (NICU) extremely challenging and stressful. A discourse analysis revealed that women&rsquo / s talk was shaped by the disciplinary frameworks oftechnocratic medicine and patriarchal notions of&nbsp / gender. Participants&rsquo / discourses about their encounters inthe medical context werelocated in, and shaped by, the structure of health care in our country. In this regard binaries&nbsp / (like private versus public health care, women versus men and nurses versus doctors) were evident. Furthermore their hospital stay reflected their experiences in the Intensive&nbsp / Care (ICU) and the Neonatal Intensive Care Units (NICU) both of which are highly technologically orientated and managed. Biomedical discourses that filtered through the&nbsp / participants&rsquo / talk were: medicine as indisputable truth / mechanistic model of the body as machine / medical doctors as gods and the foetus as &lsquo / super subject&rsquo / . Discourses of risk&nbsp / were inevitably taken up as participants tried to make sense of both their current pregnancies and the potential ones to follow. The passage into motherhood for these&nbsp / participants was dependent on whether they had live babies or not. For those who had live babies it was a difficult time as they had to contend with their own recovery as well as the prematurity of their infants. The NICU experience was described as tiring, trying and cumbersome. For mothers who lost their babies it was a time of profound sadness and&nbsp / loss coupled to the notion that motherhood itself was lost. This loss of their children symbolised broken dreams, severed connections and a powerful taboo. In addition, discourses in which motherhood was naturalised and normalised saturated their talk and framed their experience in a narrative of deficit and failure. The ideologies of mother&nbsp / blame and the &lsquo / all responsible&rsquo / mother were pervasive in their discussions. In conclusion, this high-risk situation represented a time of tremendous uncertainty and unpredictability for all participants and was powerfully shaped by dominant discourses about motherhood and the biomedical discursive and institutional framework in which&nbsp / participants were subjugated. The study thus highlights how the HELLP syndrome experience illuminates the erasure of women&rsquo / s subjectivities while the foetus/infants&rsquo / life&nbsp / takes precedence. This has significant implications for scholarship in general and feminist scholarship in particular and highlights the need for this type of engagement in an area that has remained on the periphery of feminist research.</p>

Proefschrift Universiteit van Amsterdam. / Met samenvatting in het Nederlands.

Philosophiae Doctor - PhD / This thesis is about HELLP Syndrome (hemolysis, elevated liver enzymes, low platelet count in pregnancy): a devastating maternal hypertensive complication that results in multi-system changes that can rapidly deteriorate into organ failure and death. Despite rapid advancesin medical technology and medical science this disease continues to take the lives of women and their infants. The only effective intervention for this disorder is immediate termination irrespective of the gestational stage of the pregnancy. The primary objective of this thesis was to explore the subjective experiences and meaningmaking processes of women in and through their high-risk pregnancies. This objective crystallised into the following aims: to facilitate and listen to the voices of women who were HELLP Syndrome survivors; to explore the reported bodily, psychological and emotional experiences of HELLP Syndrome survivors; to understand the role medical intervention and biomedical discourses play in these women’s experiences and finally to explore the subjective experiences of HELLP Syndrome in the context of traditionallyheld notions of motherhood. The study was couched in a feminist poststructuralist epistemology. A material-discursive framework which comprised phenomenological and poststructuralist theorising was usedin an attempt to understand both the lived experiences as well as the discursively constructed nature of those subjective experiences. Thus the analysis encompassed both a broadly phenomenological framework to understand the lived experiences of HELLP Syndrome, and a discourse analysis to explore the meaning-making processes of participants in relation to larger social discourses, in particular the dominant biomedical and motherhood discourses. A qualitative approach using in depth semi-structured interviews was utilisedto gather data. Eleven participants from very diverse backgrounds consented to be part of thisstudy. The findings of the study highlighted the immense trauma, difficulties and challenges participants faced in these high-risk situations. What was evident from the analysis was that their experiences were so diverse and werecompletely shaped by the severity of the disorder and the gestational stage of the pregnancy. Some women ended up in the Intensive Care Units (ICU) and had near-death experiences, some had very premature babies, while some of the participants lost their babies during the process. With regards to the emotional, psychological and corporeal aspects of the disorder,participants described their situations as a disaster, painful and difficult. Due to the rapid deterioration of symptoms, they described the tempo of these events as a whirlwind in which they felt they had no control. Emotions ranged from shock, total disbelief and surprise to anger, helplessness and powerlessness. Lacking knowledge and access to appropriate information further compounded the situation for participants. Theparticipants who had premature babies found the Neonatal Intensive Care Unit experience (NICU) extremely challenging and stressful. A discourse analysis revealed that women’s talk was shaped by the disciplinary frameworks oftechnocratic medicine and patriarchal notions of gender. Participants’ discourses about their encounters inthe medical context werelocated in, and shaped by, the structure of health care in our country. In this regard binaries (like private versus public health care, women versus men and nurses versus doctors) were evident. Furthermore their hospital stay reflected their experiences in the Intensive Care (ICU) and the Neonatal Intensive Care Units (NICU) both of which are highly technologically orientated and managed. Biomedical discourses that filtered through the participants’ talk were: medicine as indisputable truth;mechanistic model of the body as machine; medical doctors as gods and the foetus as ‘super subject’. Discourses of risk were inevitably taken up as participants tried to make sense of both their current pregnancies and the potential ones to follow. The passage into motherhood for these participants was dependent on whether they had live babies or not. For those who had live babies it was a difficult time as they had to contend with their own recovery as well as the prematurity of their infants. The NICU experience was described as tiring, trying and cumbersome. For mothers who lost their babies it was a time of profound sadness and loss coupled to the notion that motherhood itself was lost. This loss of their children symbolised broken dreams, severed connections and a powerful taboo. In addition, discourses in which motherhood was naturalised and normalised saturated their talk and framed their experience in a narrative of deficit and failure. The ideologies of mother blame and the ‘all responsible’ mother were pervasive in their discussions. In conclusion, this high-risk situation represented a time of tremendous uncertainty and unpredictability for all participants and was powerfully shaped by dominant discourses about motherhood and the biomedical discursive and institutional framework in which participants were subjugated. The study thus highlights how the HELLP syndrome experience illuminates the erasure of women’s subjectivities while the foetus/infants’ life takes precedence. This has significant implications for scholarship in general and feminist scholarship in particular and highlights the need for this type of engagement in an area that has remained on the periphery of feminist research. / South Africa

Orientador: Mary Angela Parpinelli / Texto em português e inglês / Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências Médicas / Made available in DSpace on 2018-11-27T12:55:48Z (GMT). No. of bitstreams: 1 Zanette_ElviraAmeliadeOliveira_M.pdf: 12724973 bytes, checksum: b98c418079a875c5a37d346111e35a77 (MD5) Previous issue date: 2012 / Resumo: Introdução: Estima-se que os distúrbios hipertensivos (DH) na gravidez causem 50.000 mortes maternas (MM) a cada ano e que a imensa maioria ocorra em países de baixa ou média renda, além de aumentarem de 3 a 25 vezes o risco de complicações graves. Nas duas últimas décadas, tem sido crescente o interesse em estudar a morbidade materna grave (MMG)/near miss (NM) como método complementar às auditorias e inquéritos sobre MM. A investigação do NM é capaz de fornecer mais detalhes sobre fatores que contribuem para ambos, mortalidade e morbidade grave e uma referência para avaliação da qualidade do cuidado obstérico. Objetivo: identificar a prevalência e os fatores associados ao NM/MM em uma população de mulheres com DH graves (pré-eclâmpsia grave, eclâmpsia, hipertensão arterial grave e síndrome HELLP). Método: estudo multicêntrico, envolvendo 27 maternidades de referência, participantes da Rede Nacional de Vigilância da Morbidade Materna Grave, localizadas nas cinco regiões do Brasil. Realizou-se vigilância prospectiva com dados coletados após o desfecho final do caso de todas as mulheres admitidas por MMG e selecionados para o estudo os casos de MMG por DH grave, no período de junho de 2009 a julho de 2010. Os dados foram coletados em formulários específicos e digitados na plataforma OpenClinics®. Foram estudadas variáveis maternas sociodemográficas, obstétricas, clínicas, resultados perinatais e tipos de demora no cuidado obstétrico. Os casos foram classificados segundo os critérios da OMS em condição potencialmente ameaçadora da vida (CPAV) e NM/MM. Foi realizada análise bivariada pelo cálculo das razões de prevalência (RP) e seus respectivos intervalos de confiança (IC) de 95% ajustados pelo efeito conglomerado e análise múltipla por regressão de Poisson. O nível de significância adotado foi de 0,05%. Resultados: os DH graves foram a principal causa de MMG (6706/9555); a prevalência de NM foi 4,2 casos por 1000 (NV), a relação caso/fatalidade foi de 8,3 NM para 1 MM. A manifestação precoce da doença e a hemorragia pós-parto foram variáveis independentes associadas ao desfecho em NM/MM, além do edema agudo de pulmão, cardiopatia prévia e demoras no cuidado de segundo e terceiro tipos. Conclusão: o estudo do near miss identificou dentre as mulheres com DH graves situações independentemente associadas ao pior desfecho, e que pode ser modificado por intervenções no cuidado obstétrico direto a estas mulheres e no sistema de saúde. O estudo mostrou ainda a factibilidade de um sistema de vigilância hospitalar de MMG capaz de contribuir para a redução da MM / Abstract: Introduction: It has been estimated that hypertensive disorders (HD) in pregnancy may cause 50.000 maternal deaths (MD) annually and the large majority occurs in low-income or middle-income countries. Furthermore, these disorders increase the risk of severe complications by 3 to 25 times. In the last two decades, there has been increased interest in the study of severe maternal morbidity (SMM)/near miss (NM) as a supplementary method to audits and enquiries about MD. The investigation of NM is able to offer more details about factors that may contribute to both mortality and severe morbidity and may be used as a reference for evaluating quality of obstetric care. Objective: to identify the prevalence and factors associated with NM/MD in a female population suffering from severe HD (severe preeclampsia, eclampsia, severe arterial hypertension and HELLP syndrome). Method: A multicenter study, involving 27 referral maternity hospitals, participating in the National Surveillance Network for Severe Maternal Morbidity, located in five Brazilian regions. A prospective surveillance was performed with data collected after final case outcome in all women admitted to hospital for SMM and selected for the study of SMM cases due to severe HD, from June 2009 to July 2010. Data were collected in specific forms and entered into the OpenClinics® platform. Variables studied were maternal sociodemographic characteristics, obstetric and clinical history, perinatal results and types of delay in obtaining obstetric care. Cases were classified according to the WHO criteria: potentially life-threatening conditions (PLTC) and NM/MD. Bivariate analysis was performed by estimation of prevalence ratios (PR) and their respective 95% confidence intervals (CI) adjusted by the conglomerate effect and Poisson multiple regression analysis. The significance level adopted was 0.05%. Results: severe HD was the main cause of SMM (6706/9555); the prevalence of NM was 4.2 cases per 1000 (LB), the case/fatality rate was 8.3 NM to 1 MD and the MD index was 10.7%. Early manifestation of disease and postpartum hemorrhage were independent variables associated with outcome in NM/MD, in addition to acute pulmonary edema, previous heart disease and delays in receiving secondary and tertiary care. Conclusion: the near miss study identified severe conditions that were independently associated with a worse outcome among women with HD that could be modified by interventions in direct obstetric care of these women and in the healthcare system. The study also showed that a hospital surveillance system for SMM is feasible and can contribute to a reduction in MD / Mestrado / Saúde Materna e Perinatal / Mestra em Ciências da Saúde

Bakgrund: Det finns begränsad kunskap om hur mammor upplever att drabbas av HELLP syndrom och samtidigt mista sitt väntade barn. Ytterligare kunskap kan bidra till ökad förståelse och leda till bättre vård. Syfte: Att beskriva mammors upplevelser av att drabbas av HELLP syndrom och samtidigt mista sitt väntade barn. Metod: En kvalitativ fallstudie med djupintervjuer som analyserades genom kvalitativ innehållsanalys. Resultat: Studien påvisade det kaos och den overklighetskänsla mammorna upplevde då insjuknandet och händelserna skedde i snabb takt. Det fanns ingen tid för reflektion, tillståndet var livshotande och en katastrofkänsla uppstod. Temat overkligt kaos formades. De svårt sjuka mammorna klarade inte att delta vid första omvårdnaden av sitt döda barn men kunde under vårdtiden vara tillsammans med barnet vid flera tillfällen vilket gav känslor av både glädje och sorg. Mammorna upplevde restsymtom av HELLP syndrom ett halvår efter händelsen. Slutsatser: Det snabba insjuknandet och händelseförloppet resulterade i ett overkligt kaos för mammorna. Det är viktigt att vårdpersonalen är tydliga i sin kommunikation och hela tiden närvarande hos mamman. Barnmorskan har en central roll i att hjälpa mammorna att möta och vara nära sitt döda barn så mycket som möjligt.

Orientadores: João Luiz de Carvalho Pinto e Silva, Melania Ramos de Amorim / Tese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas / Made available in DSpace on 2018-08-08T21:54:32Z (GMT). No. of bitstreams: 1 Katz_Leila_D.pdf: 3055222 bytes, checksum: 91a25842ad35d99d90d5f1db2c72177b (MD5) Previous issue date: 2007 / Resumo: Objetivo: o objetivo desse estudo foi determinar a efetividade de dexametasona pós-parto em pacientes com síndrome HELLP. Desenho do Estudo: um estudo prospectivo, randomizado, duplo-cego foi conduzido envolvendo 105 mulheres com síndrome HELLP, randomicamente alocadas para utilizar dexametasona (n=56) ou placebo (n=49), após o parto. O tratamento (dexametasona 10mg ou placebo) foi administrado a cada 12 horas, intravenoso por quatro dias, realizando-se uma análise de acordo com a intenção de tratar. As variáveis analisadas foram duração da hospitalização, morbidade materna e comportamento de parâmetros clínicos e laboratoriais. Os dados foram analisados utilizando-se o X2 ou teste exato de Fisher, sendo considerado significativo um valor de p<0,05. O comportamento das variáveis clínicas durante e após o tratamento foi avaliado por ajuste de modelos lineares. Resultados: os grupos foram homogêneos não havendo diferença significativa entre suas características biológicas e clínicas e laboratoriais ao ingressar no estudo. A média de tempo de internação (10 dias) foi semelhante entre os dois grupos, bem como as morbidades maternas tanto quando analisadas de forma global, como para cada complicação de forma isolada. O esquema de resgate foi usado em 12,5% das pacientes que receberam dexametasona e em 18,8% das que usaram placebo. Não houve diferença estatisticamente significativa entre os grupos em relação ao uso de hemoderivados. Modelos de ajuste linear foram usados, não havendo diferença significativa entre os grupos, considerando o padrão de recuperação da contagem plaquetária (p = 0,84), AST (p = 0,59), DHL (p = 0,647), hemoglobina (p=0,43) e diurese (p = 0,31). Conclusão: os resultados desse ECR não dão suporte ao uso de dexametasona no puerpério para recuperação de pacientes com síndrome HELLP / Abstract: Objective: The purpose of this study was to determine the effectivity of postpartum dexamethasone in patients with HELLP syndrome. Study Design: A prospective, randomized, double-blind trial was conducted including 105 women with HELLP syndrome who were assigned randomly to treatment (n=56) or placebo (n=49) groups, after delivery. The treatment (dexamethasone 10mg or placebo) was administered every 12 hours for four days and an intention to treat analysis was carried out. The analysis variables were duration of hospitalization, maternal morbidity, and the behavior of laboratorial and clinical parameters. Data were analyzed by X2 or Fisher's exact tests, considering significant a value of p<0.05. Behavior of laboratorial and clinical variables during and after treatment was evaluated by linear model adjustments. Results: Before treatment, no significant difference was observed between the two groups. Mean duration of hospitalization (10 days) was the same in both groups. Maternal morbidity did not differ between groups, considering each complication or when analyzed globally. Rescue scheme was used in 12.5% of the dexamethasone and 18.8% in the placebo group. The use of blood products was not statistically different between groups. Linear model adjustments were used and there was no significant difference between groups considering the pattern of recovery of platelet count (p = 0.84), AST (p = 0.59), LDH (p = 0.647), hemoglobin (p=0.43) and diuresis (p = 0.31). Conclusions: The results of this RCT do not support the use of dexamethasone in the puerperium for recovery of patients with HELLP syndrome / Doutorado / Tocoginecologia / Doutor em Tocoginecologia

Objective: To evaluate the association between preeclampsia (PE) and eclampsia (E) on subsequent metabolic and biochemical outcomes. Methods: Systematic review and meta-analysis of observational studies. We searched five engines until November 2018 for studies evaluating the effects of PE/E on metabolic and biochemical outcomes after delivery. PE was defined as presence of hypertension and proteinuria at >20 weeks of pregnancy; controls did not have PE/E. Primary outcomes were blood pressure (BP), body mass index (BMI), metabolic syndrome (MetS), blood lipids and glucose levels. Random effects models were used for meta-analyses, and effects reported as risk difference (RD) or mean difference (MD) and their 95% confidence interval (CI). Subgroup analyses by time of follow up, publication year, and confounder adjustment were performed. Results: We evaluated 41 cohorts including 3300 PE/E and 13,967 normotensive controls. Women were followed up from 3 months after delivery up to 32 years postpartum. In comparison to controls, PE/E significantly increased systolic BP (MD = 8.3 mmHg, 95%CI 6.8 to 9.7), diastolic BP (MD = 6.8 mmHg, 95%CI 5.6 to 8.0), BMI (MD = 2.0 kg/m2; 95%CI 1.6 to 2.4), waist (MD = 4.3 cm, 95%CI 3.1 to 5.5), waist-to-hip ratio (MD = 0.02, 95%CI 0.01 to 0.03), weight (MD = 5.1 kg, 95%CI 2.2 to 7.9), total cholesterol (MD = 4.6 mg/dL, CI 1.5 to 7.7), LDL (MD = 4.6 mg/dL; 95%CI 0.2 to 8.9), triglycerides (MD = 7.7 mg/dL, 95%CI 3.6 to 11.7), glucose (MD = 2.6 mg/dL, 95%CI 1.2 to 4.0), insulin (MD = 19.1 pmol/L, 95%CI 11.9 to 26.2), HOMA-IR index (MD = 0.7, 95%CI 0.2 to 1.2), C reactive protein (MD = 0.05 mg/dL, 95%CI 0.01 to 0.09), and the risks of hypertension (RD = 0.24, 95%CI 0.15 to 0.33) and MetS (RD = 0.11, 95%CI 0.08 to 0.15). Also, PE/E reduced HDL levels (MD = –2.15 mg/dL, 95%CI –3.46 to −0.85). Heterogeneity of effects was high for most outcomes. Risk of bias was moderate across studies. Subgroup analyses showed similar effects as main analyses. Conclusion: Women who had PE/E have worse metabolic and biochemical profile than those without PE/E in an intermediate to long term follow up period. © / Revisión por pares

Introdução: A enzima 3-hidroxiacil CoA desidrogenase de cadeia longa (LCHAD) é uma das enzimas envolvidas na beta-oxidação mitocondrial de ácidos graxos e faz parte do complexo enzimático chamado proteína trifuncional. Sua deficiência segue um modelo de herança autossômica recessiva com uma mortalidade maior que 70%, porém um tratamento dietoterápico adequado reduz substancialmente a morbimortalidade. Estudos recentes descreveram que gestantes de fetos homozigóticos para defeitos de LCHAD apresentam grandes chances de desenvolver síndrome HELLP e doença hepática aguda da gestação, com risco de morte materna, fetal e do recém-nascido. A síndrome HELLP é caracterizada por plaquetopenia, enzimas hepáticas elevadas e hemólise, podendo se apresentar na forma completa ou parcial. Estudos mostram que investigar a relação entre síndrome HELLP e defeitos de LCHAD, possibilita a prevenção de futuras gestações de risco por meio de aconselhamento genético e o diagnóstico precoce deste erro inato do metabolismo. Objetivos: Verificar a associação entre gestantes com síndrome HELLP e lactentes com defeitos LCHAD e identificar problemas de saúde gerados pela doença nesses conceptos. Metodologia: Análise de prontuários, necropsias e desfecho atual dos conceptos de 42 gestantes com síndrome HELLP e 84 controles. Resultados: Entre as gestantes que compunham os casos, a maioria apresentou proteinúria; além de sintomas como vômitos e epigastralgia. O parto cesárea foi realizado em 93% das gestantes. Quase metade das puérperas apresentou algum tipo de complicação materna. Quanto ao desfecho perinatal, 90% dos conceptos apresentaram baixo peso ao nascer e 23,8% evoluíram para óbito. Entre esses 10 óbitos, resgatamos 7 imagens histopatológicas com esteatose hepática. Inferimos doença metabólica nesses casos, que levou a uma associação de 11% com a síndrome HELLP. Entre o grupo controle, 46,2% das mulheres já haviam sofrido pelo menos um aborto. Na atual gestação 6,4% desenvolveram pré-eclampsia; entre outras complicações. Encontramos gravidezes subsequentes das gestantes do grupo Caso com recorrência de HELLP e óbito. Conclusão: Os resultados reforçam a importância do diagnóstico precoce de síndrome HELLP, além da investigação da associação do defeito de LCHAD e HELLP mesmo post morten afim de evitar futuras gestações de risco e diminuir a morbimortalidade materna e neonatal. / Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) is one of the enzymes involved in the mitochondrial fatty acids beta-oxidation and part of the enzymatic complex called trifunctional protein. Its deficiency follows an autosomal recessive model with a higher mortality, but an adequate dietary treatment reduces its morbimortality. Recent studies reported that mothers of fetuses homozygous for LCHAD deficiency have higher chances of developing HELLP and acute fatty liver of pregnancy with risk of maternal, infant and fetal death. Thrombocytopenia, elevated liver enzymes and hemolysis characterize HELLP syndrome, which can be diagnosed as complete or partial. Studies demonstrate that investigate the association between HELLP syndrome and LCHAD defects can prevent future risk pregnancies through genetic counseling and early diagnosis of this inborn error of metabolism. Objectives: To investigate the association between pregnant women and concepts with LCHAD deficiency and identify health problems caused by the disease in these fetuses. Methodology: Analysis of medical records, autopsy reports and current outcome of fetuses of 42 pregnant women with HELLP syndrome and 84 controls. Results: In case group, most patients presented proteinuria; as well as symptoms as vomiting and epigastric pain. The cesarean delivery was performed in 93% of pregnant women. Almost half of women presented maternal complications. In perinatal outcome, 90% of fetuses has low weight at birth and 23.8% died. Among these 10 deaths, we rescued 7 hystopathological images with hepatic steatosys. We could infer metabolic disease in these cases, which led to an association of 11% to the HELLP syndrome. Among the control group, 46.2% of women had at least one abortion before this pregnancy. During the pregnancy 6.4% developed pre-eclampsia among other complications. In control group, we find HELLP syndrome recurrence and death in subsequent pregnancy. Conclusion: The results reinforce the importance of early diagnosis of HELLP syndrome, as well as research LCHAD and HELLP association even post mortem to avoid future risk pregnancies and reduce maternal and neonatal morbidity and mortality.

Introdução: A enzima 3-hidroxiacil CoA desidrogenase de cadeia longa (LCHAD) é uma das enzimas envolvidas na beta-oxidação mitocondrial de ácidos graxos e faz parte do complexo enzimático chamado proteína trifuncional. Sua deficiência segue um modelo de herança autossômica recessiva com uma mortalidade maior que 70%, porém um tratamento dietoterápico adequado reduz substancialmente a morbimortalidade. Estudos recentes descreveram que gestantes de fetos homozigóticos para defeitos de LCHAD apresentam grandes chances de desenvolver síndrome HELLP e doença hepática aguda da gestação, com risco de morte materna, fetal e do recém-nascido. A síndrome HELLP é caracterizada por plaquetopenia, enzimas hepáticas elevadas e hemólise, podendo se apresentar na forma completa ou parcial. Estudos mostram que investigar a relação entre síndrome HELLP e defeitos de LCHAD, possibilita a prevenção de futuras gestações de risco por meio de aconselhamento genético e o diagnóstico precoce deste erro inato do metabolismo. Objetivos: Verificar a associação entre gestantes com síndrome HELLP e lactentes com defeitos LCHAD e identificar problemas de saúde gerados pela doença nesses conceptos. Metodologia: Análise de prontuários, necropsias e desfecho atual dos conceptos de 42 gestantes com síndrome HELLP e 84 controles. Resultados: Entre as gestantes que compunham os casos, a maioria apresentou proteinúria; além de sintomas como vômitos e epigastralgia. O parto cesárea foi realizado em 93% das gestantes. Quase metade das puérperas apresentou algum tipo de complicação materna. Quanto ao desfecho perinatal, 90% dos conceptos apresentaram baixo peso ao nascer e 23,8% evoluíram para óbito. Entre esses 10 óbitos, resgatamos 7 imagens histopatológicas com esteatose hepática. Inferimos doença metabólica nesses casos, que levou a uma associação de 11% com a síndrome HELLP. Entre o grupo controle, 46,2% das mulheres já haviam sofrido pelo menos um aborto. Na atual gestação 6,4% desenvolveram pré-eclampsia; entre outras complicações. Encontramos gravidezes subsequentes das gestantes do grupo Caso com recorrência de HELLP e óbito. Conclusão: Os resultados reforçam a importância do diagnóstico precoce de síndrome HELLP, além da investigação da associação do defeito de LCHAD e HELLP mesmo post morten afim de evitar futuras gestações de risco e diminuir a morbimortalidade materna e neonatal. / Long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) is one of the enzymes involved in the mitochondrial fatty acids beta-oxidation and part of the enzymatic complex called trifunctional protein. Its deficiency follows an autosomal recessive model with a higher mortality, but an adequate dietary treatment reduces its morbimortality. Recent studies reported that mothers of fetuses homozygous for LCHAD deficiency have higher chances of developing HELLP and acute fatty liver of pregnancy with risk of maternal, infant and fetal death. Thrombocytopenia, elevated liver enzymes and hemolysis characterize HELLP syndrome, which can be diagnosed as complete or partial. Studies demonstrate that investigate the association between HELLP syndrome and LCHAD defects can prevent future risk pregnancies through genetic counseling and early diagnosis of this inborn error of metabolism. Objectives: To investigate the association between pregnant women and concepts with LCHAD deficiency and identify health problems caused by the disease in these fetuses. Methodology: Analysis of medical records, autopsy reports and current outcome of fetuses of 42 pregnant women with HELLP syndrome and 84 controls. Results: In case group, most patients presented proteinuria; as well as symptoms as vomiting and epigastric pain. The cesarean delivery was performed in 93% of pregnant women. Almost half of women presented maternal complications. In perinatal outcome, 90% of fetuses has low weight at birth and 23.8% died. Among these 10 deaths, we rescued 7 hystopathological images with hepatic steatosys. We could infer metabolic disease in these cases, which led to an association of 11% to the HELLP syndrome. Among the control group, 46.2% of women had at least one abortion before this pregnancy. During the pregnancy 6.4% developed pre-eclampsia among other complications. In control group, we find HELLP syndrome recurrence and death in subsequent pregnancy. Conclusion: The results reinforce the importance of early diagnosis of HELLP syndrome, as well as research LCHAD and HELLP association even post mortem to avoid future risk pregnancies and reduce maternal and neonatal morbidity and mortality.