Maternal sepsis in the era of genomic medicine

Purpose

Maternal sepsis remains one of the leading causes of direct and indirect maternal mortality both in high- and low-income environments. In the last two decades, systems biology approaches, based on ‘-omics’ technologies, have started revolutionizing the diagnosis and management of the septic syndrome. The scope of this narrative review is to present an overview of the basic ‘-omics’ technologies, exemplified by cases relevant to maternal sepsis.

Methods

Narrative review of the new ‘-omics’ technologies based on a detailed review of the literature.

Results

After presenting the main ‘omics’ technologies, we discuss their limitations and the need for integrated approaches that encompass research efforts across multiple ‘-omics’ layers in the ‘-omics’ cascade between the genome and the phenome.

Conclusions

Systems biology approaches are revolutionizing the research landscape in maternal sepsis. There is a need for increased awareness, from the side of health practitioners, as a requirement for the effective implementation of the new technologies in the research and clinical practice in maternal sepsis.

     

Maternal mortality ratio in selected rural communities in Kebbi State,Northwest Nigeria

Background

Maternal mortality remains a topical issue in Nigeria. Dearth of data on vital events posed a huge challenge to policy formulation and design of interventions to address the scourge. This study estimated the lifetime risk (LTR) of maternal death and maternal mortality ratio (MMR) in rural areas of Kebbi State, northwest Nigeria, using the sisterhood method.

Methods

Using the sisterhood method, data was collected from 2917 women aged 15–49?years from randomly selected rural communities in 6 randomly selected local government area of Kebbi State. Retrospective cohort of their female siblings who had reached the childbearing age of 15?years was constructed. Using the most recent total fertility rate for Kebbi State, the lifetime risk and associated MMR were estimated.

Result

A total of 2917 women reported 8233 female siblings of whom 409 had died and of whom 204 (49.8%) were maternal deaths. This corresponds to an LTR of 6% (referring to 11?years before the study) and an estimated MMR of 890 deaths/100,000 live births (95% CI, 504–1281).

Conclusion

The findings provide baseline information on the MMR in rural areas of the State. It underscores the need to urgently address the bane of high maternity mortality, if Kebbi State and Nigeria in general, will achieve the health for all by year 2030 as stated in the Sustainable Development Goals (SDGs).

     

A global view of severe maternal morbidity: moving beyond maternal mortality

Background

Maternal mortality continues to be of great public health importance, however for each woman who dies as the direct or indirect result of pregnancy, many more women experience life-threatening complications. The global burden of severe maternal morbidity (SMM) is not known, but the World Bank estimates that it is increasing over time. Consistent with rates of maternal mortality, SMM rates are higher in low- and middle-income countries (LMICs) than in high-income countries (HICs).

Severe maternal morbidity in high-income countries

Since the WHO recommended that HICs with low maternal mortality ratios begin to examine SMM to identify systems failures and intervention priorities, researchers in many HICs have turned their attention to SMM. Where surveillance has been conducted, the most common etiologies of SMM have been major obstetric hemorrhage and hypertensive disorders. Of the countries that have conducted SMM reviews, the most common preventable factors were provider-related, specifically failure to identify “high risk” status, delays in diagnosis, and delays in treatment.

Severe maternal morbidity in low and middle income countries

The highest burden of SMM is in Sub-Saharan Africa, where estimates of SMM are as high as 198 per 1000 live births. Hemorrhage and hypertensive disorders are the leading conditions contributing to SMM across all regions. Case reviews are rare, but have revealed patterns of substandard maternal health care and suboptimal use of evidence-based strategies to prevent and treat morbidity.

Effects of SMM on delivery outcomes and infants

Severe maternal morbidity not only puts the woman’s life at risk, her fetus/neonate may suffer consequences of morbidity and mortality as well. Adverse delivery outcomes occur at a higher frequency among women with SMM. Reducing preventable severe maternal morbidity not only reduces the potential for maternal mortality but also improves the health and well-being of the newborn.

Conclusion

Increasing global maternal morbidity is a failure to achieve broad public health goals of improved women’s and infants’ health. It is incumbent upon all countries to implement surveillance initiatives to understand the burden of severe morbidity and to implement review processes for assessing potential preventability.

     

Factors associated with maternal near miss in childbirth and the postpartum period: findings from the birth in Brazil National Survey, 2011–2012

Background

Maternal near-miss (MNM) audits are considered a useful approach to improving maternal healthcare. The aim of this study was to evaluate the factors associated with maternal near-miss cases in childbirth and the postpartum period in Brazil.

Methods

The study is based on data from a nationwide hospital-based survey of 23,894 women conducted in 2011–2012. The data are from interviews with mothers during the postpartum period and from hospital medical files. Univariate and multivariable logistic regressions were performed to analyze factors associated with MNM, including estimation of crude and adjusted odds ratios and their respective 95 % confidence intervals (95 % CI).

Results

The estimated incidence of MNM was 10.2/1,000 live births (95 % CI: 7.5–13.7). In the adjusted analyses, MNM was associated with the absence of antenatal care (OR: 4.65; 95 % CI: 1.51–14.31), search for two or more services before admission to delivery care (OR: 4.49; 95 % CI: 2.12–9.52), obstetric complications (OR: 9.29; 95 % CI: 6.69–12.90), and type of birth: elective C-section (OR: 2.54; 95 % CI: 1.67–3.88) and forceps (OR: 9.37; 95 % CI: 4.01–21.91). Social and demographic maternal characteristics were not associated with MNM, although women who self-reported as white and women with higher schooling had better access to antenatal and maternity care services.

Conclusion

The high proportion of elective C-sections performed among women in better social and economic situations in Brazil is likely attenuating the benefits that could be realized from improved prenatal care and greater access to maternity services. Strategies for reducing the rate of MNM in Brazil should focus on: 1) increasing access to prenatal care and delivery care, particularly among women who are at greater social and economic risk and 2) reducing the rate of elective cesarean section, particularly among women who receive services at private maternity facilities, where C-section rates reach 90 % of births.

     

A Study of Hepatitis E in Pregnancy: Maternal and Fetal Outcome

Purpose

HEV infection, a major public health concern, is known to cause large-scale epidemic and sporadic cases of acute viral hepatitis in developing countries. The infection occurs primarily in young adults and is generally mild and self-limiting; however, the case fatality rate is reportedly higher among women, especially during the second or third trimesters of pregnancy.

Methods

This study, a prospective observational study, was conducted at the Dr. D. Y. Patil Medical College Hospital and Apple Saraswati Multispeciality hospital, in Kolhapur for over a period of 3 years (Jan 2010 to Jan 2013) to find out the prevalence and clinical outcome in a series of HEV-infected pregnant women.

Results

A total of fifty-five symptomatic Anti-HEV IgM-positive women were included, and the maternal-fetal outcome was analyzed. The maternal mortality was 5 % including one antenatal death. Prematurity (80 %) and PROM (11 %) were the commonest fetal complications noted with a vertical transmission rate of 28 %.

Conclusion

Variations in maternal morbidity and mortality between different studies indicate a need to subtype the viral genotype according to its virulence and morbidity.

     

Validation of Canadian mothers’ recall of events in labour and delivery with electronic health records

Background

Maternal report of events that occur during labour and delivery are used extensively in epidemiological research; however, the validity of these data are rarely confirmed. This study aimed to validate maternal self-report of events that occurred in labour and delivery with data found in electronic health records in a Canadian setting.

Methods

Data from the All Our Babies study, a prospective community-based cohort of women’s experiences during pregnancy, were linked to electronic health records to assess the validity of maternal recall at four months post-partum of events that occurred during labour and delivery. Sensitivity, specificity and kappa scores were calculated. Results were stratified by maternal age, gravidity and educational attainment.

Results

Maternal recall at four months post-partum was excellent for infant characteristics (gender, birth weight, gestational age, multiple births) and variables related to labour and delivery (mode of delivery, epidural, labour induction) (sensitivity and specificity >85%). Women who had completed a university degree had significantly better recall of labour induction and use of an epidural.

Conclusion

Maternal recall of infant characteristics and events that occurred during labour and delivery is excellent at four months post-partum and is a valid source of information for research purposes.

     

First-Trimester Uterine Artery Pulsatility Index and Maternal Serum PAPP-A and PlGF in Prediction of Preeclampsia in Primigravida

Background

Preeclampsia is a heterogeneous disorder affecting different body systems and frequently associated with morbidity and mortality. Early preeclampsia prediction will reduce this associated morbidity and mortality as it will give the chance for frequent maternal and fetal surveillance and application of prophylactic procedures.

Objective

The aim of this work is to evaluate the role of mean pulsatility index (PI) of the uterine arteries and maternal serum concentrations of pregnancy-associated plasma protein A (PAPP-A) and placental growth factor (PlGF) in early preeclampsia prediction in primigravida.

Patients and Methods

Three hundred primigravida attending the antenatal care clinic in Zagazig University Hospitals were included in the study. The mean PI of the uterine arteries was calculated. Maternal serum levels of PAPP-A and PIGF were analyzed by specific immunoassay.

Results

Three hundred women were included in the final analysis, of them 30 patients (10%) suffered from preeclampsia. There was a significant difference between preeclamptic and normal women as regards the mean PI of the uterine arteries and levels of PAPP-A and PIGF at 11–13 weeks. When combining the cutoff levels of the three methods, mean PI of the uterine arteries ≥1.69, PAPP-A assay <0.96 multiple of median (MoM) and PlGF assay <0.91 MoM, the sensitivity, specificity, positive predictive value, negative predictive value and overall accuracy were 56.7, 99.3, 89.5, 95.4 and 67%, respectively.

Conclusion

The combined measurement of maternal serum PAPP-A and PlGF concentrations and mean PI of the uterine arteries at 11–13 weeks of pregnancy may help to predict preeclampsia in primigravida when other parameters of preeclampsia prediction are normal. However, we need more studies on larger and variable populations to evaluate the use of those combined methods in preeclampsia prediction.

     

Maternal ascites an independent prognostic factor in severe preeclampsia: a matched cohort study

Background

Preeclampsia is a multi-systemic, multi-organ dysfunction associated with increased maternal and perinatal complications. The presence of maternal ascites, a manifestation of endothelial dysfunction and increased capillary permeability, is shown to be associated with adverse outcomes. We aim to investigate the impact of maternal ascites on pregnancy outcome in women with severe preeclampsia.

Methods

A matched cohort study was conducted in a tertiary care teaching hospital in South India between March 2014 and March 2015. One hundred and twenty-one severe preeclamptic women with ascites formed the study cohort while age-, parity-, and gestational age-matched group of 121 severe preeclamptic women without ascites formed the control. Primary outcome was the composite maternal adverse outcome defined as the development of any of eclampsia, pulmonary edema, renal failure, or disseminated intravascular coagulation (DIC). Secondary outcome was the composite perinatal outcome defined as the occurrence of any of still birth, hypoxic ischemic encephalopathy or early neonatal death.

Results

Four maternal deaths occurred in the study group. The rates of pregnancies with composite maternal adverse outcome [42 vs 9% RR 4.6 (95% CI 2.5–8.4)] and composite perinatal adverse outcome [36 vs 17% RR 2.1, (95% CI 1.3–3.3)] were significantly more in ascites group than in control group. After adjusting for other confounding variables, ascites was independently associated with adverse maternal events [adjusted OR 16.40 (95% CI 2.88–93.31)] but not adverse perinatal outcome.

Conclusion

In women with severe preeclampsia, maternal ascites is an independent risk factor for adverse maternal outcome.

     

Les déterminants maternels associés au petit poids pour l’âge gestationnel à la maternité de l’hôpital Donka de Conakry

Objective

To identify the maternal determinants of small for gestational age (SGA) newborns in order to propose preventive measures to reduce infant morbidity and mortality.

Material and methods

A six-month case-control study (January 1 to June 30, 2015) was conducted at the maternity ward of Donka Hospital in Conakry. Included were records of women who gave birth to a living child. The cases consisted of women who gave birth to a child whose birth weight was between 500 g and 2,499 g. The composition of the controls was made by matching the case of a woman who gave birth to a child of normal weight (2,500 g to 3,999 g) in each case. The socio-demographic, obstetric, and maternal pathologies were analyzed and compared. A univariate analysis comparing the cases to the controls and a multivariate analysis seeking an association between the maternal determinants were carried out.

Results

The prevalence of SGA births was 7.33 %. Maternal determinants significantly associated with SGA infants in multivariate analysis were low socio-economic status (odd ratio = 1.89), leanness (OR = 3.35), poor prenatal followup (OR = 1, 75) and anemia (OR = 2.37), arterial hypertension (OR = 5.69), malaria (OR = 2.34) and genital hemorrhage (OR = 3.48).

Conclusion

Prevention of SGA births involves improving the socioeconomic status of women, improving nutrition, improving prenatal care, and managing pregnancy-related pathologies.

     

Feto-maternal Outcomes in Pregnancies Complicated by Thermal Burns

Background

Burns in pregnancy can be a potentially life-threatening condition for both mother and baby. Human physiology is altered during pregnancy and burns add further stress leading to diminished maternal reserves. Very few studies have been reported for management of such patients.

Materials and Methods

This was a prospective based study carried out in Department of Burns and Plastic Surgery in collaboration with Department of Obstetrics and Gynaecology and Department of Pediatrics for a period of 20 months from December 2011 to July 2013. Pregnant women with thermal injuries more than 15% TBSA were included in the study. Patients with coexisting obstetrics complications and burns other than thermal were excluded.

Results

Out of 3397 female patients of burns admitted, 1382 patients were in reproductive groups, 1116 were married and 67 were pregnant; these were enrolled. Maternal and fetal outcome is inversely linked with the TBSA of the mother (p < 0.001). In TBSA group 15–30%, there was no maternal and fetal mortality, but in TBSA >30–50% maternal mortality was 44%, and in 50–70% maternal mortality was 83% and no mother survived in >70%. In TBSA 30–50%, fetal mortality was 72%. Only one baby survived in 50–70% TBSA group and one in >70% TBSA group after intensive care in NICU for prematurity. Fetal survival was also dependent on gestational age, and there are better outcomes in late trimesters.

Conclusion

Maternal and fetal outcome are directly related to TBSA of mother, and best care can be offered to such patients with a multidisciplinary team-based approach.

     

Brought in Dead: An Avoidable Delay in Maternal Deaths

Purpose

Maternal brought in dead are the patient who dies in the need of adequate medical care. These deaths are often not analyzed sincerely as they are not institutional deaths. Our aim is to find out actual life threatening cause of delay leading to death.

Method

Patients brought dead to casualty were seen by the doctors on duty in Department of Obstetrics and Gynaecology,Gandhi Medical College, Bhopal round the clock. Cause of death was analyzed by verbal autopsy of attendants and referral letter from the institute. In this analytical study a complete evaluation of brought deaths from January 2011 to Decmeber 2014 was done.

Results

A total of 64 brought in deaths were reported in this 4 year duration. Most common cause of death was postpartum hemorrhage (54.68 %) followed by hypertension (15.62 %) and the most common cause of delay was delay in getting adequate treatment (56.25 %).

Conclusion

The brought in dead are the indicator of the three delays in getting health care. Challenges appear to be enormous to be tackled. Timely management proves to be critical in preventing maternal death. Thus it appears that community education about pregnancy and its complications, EmOC training at FRU and strict adherence to referral protocol may help us to reduce the brought dead burden.

     

A Review of Outcomes in Pregnant Women with Portal Hypertension

Background

The course of pregnancy in a woman with portal hypertension is a difficult one as it is associated with complications like variceal bleeding, splenic artery rupture and coagulopathy. All these pose a threat to a woman’s life. Although this condition is rare, every obstetrician should have a high index of suspicion when an antenatal mother presents with splenomegaly, thrombocytopenia or hematemesis. Hence, we aimed to review maternal and fetal outcomes in pregnant women with portal hypertension.

Methods

In a retrospective observational study, 41 women and 47 pregnancies were evaluated, from January 2000–December 2015 at Fernandez Hospital, a tertiary referral perinatal center. Maternal outcomes studied were variceal bleed during pregnancy, surgical procedures, morbidity and mortality. Neonatal variables were gestational age at delivery, birth weight and morbidities.

Results

Mean maternal age was 26.4 years. Average gestational age at delivery was 36.5 weeks. Mean birth weight was 2507.5 g. There were three maternal deaths out of 47 deliveries, the cause of death was massive variceal bleed in one, the second one was due to cardiac arrest on MRI table, and the third death was due to splenic hilar vessel bleed. There was one stillbirth, and no neonatal deaths.

Conclusion

A multidisciplinary approach is essential to improve perinatal outcomes in pregnancy complicated by portal hypertension. Surgical measures to reduce portal venous pressure done before pregnancy or beta blockers during pregnancy might help reduce sudden variceal bleeds.

     

Correlation of Maternal Autoantibodies with Fetal Congenital Heart Block

Background

Autoimmune fetal congenital heart block (CHB) is the most severe manifestation of neonatal lupus, and it is seen when maternal autoimmune antibodies cross the placenta and damage the AV node of the fetus. CHB is mainly associated with maternal SLE with anti-Ro/SSA- and anti-La/SSB-positive status, and incidence of CHB increases when both the antibodies are present. This study was conducted to know the incidence of fetal CHB in patients of SLE who had ANA, anti-Ro/SSA and anti-La/SSB positivity.

Methods

A prospective study was conducted in a tertiary-care teaching hospital of Indian Armed Forces between Jan 2012 to Sep 2014 where 13 cases of SLE were studied. All these patients were tested for ANA, anti-Ro/SSA and anti-La/SSB antibodies and fetal heart abnormalities. Fetuses with CHB were treated with steroids.

Results

Incidence of SLE was 0.14 %, 92 % of SLE patients were positive for ANA, and 46 % had anti-Ro/SSA- and anti-La/SSB-positive status. Two fetuses had congenital heart block, and one fetus required pacemaker placement 5 months after delivery.

Conclusion

All the fetal congenital heart blocks are associated with maternal anti-Ro/SSA and anti-La/SSB and ANA antibodies. Treatment by steroids may improve the outcome in early stages of fetal CHB, and delivery with follow-up should be planned in a tertiary-care center where pacemaker placement facility is available.

     

Comparison of maternal and fetal complications in elective and emergency cesarean section: a systematic review and meta-analysis

Background

Though the same types of complication were found in both elective cesarean section (ElCS) and emergence cesarean section (EmCS), the aim of this study is to compare the rates of maternal and fetal morbidity and mortality between ElCS and EmCS.

Methods

Full-text articles involved in the maternal and fetal complications and outcomes of ElCS and EmCS were searched in multiple database. Review Manager 5.0 was adopted for meta-analysis, sensitivity analysis, and bias analysis. Funnel plots and Egger’s tests were also applied with STATA 10.0 software to assess possible publication bias.

Results

Totally nine articles were included in this study. Among these articles, seven, three, and four studies were involved in the maternal complication, fetal complication, and fetal outcomes, respectively. The combined analyses showed that both rates of maternal complication and fetal complication in EmCS were higher than those in ElCS. The rates of infection, fever, UTI (urinary tract infection), wound dehiscence, DIC (disseminated intravascular coagulation), and reoperation of postpartum women with EmCS were much higher than those with ElCS. Larger infant mortality rate of EmCS was also observed.

Conclusion

Emergency cesarean sections showed significantly more maternal and fetal complications and mortality than elective cesarean sections in this study. Certain plans should be worked out by obstetric practitioners to avoid the post-operative complications.

     

Comparison of perinatal outcomes following frozen embryo transfer cycles using autologous versus donor oocytes in women 40 to 43 years old: analysis of SART CORS data

Objective

To study the differences in perinatal outcomes after frozen embryo transfer cycles using autologous or donor oocytes in women of advanced maternal age.

Design

Historical cohort study.

Setting

US national database from the Society of Assisted Reproductive Technology Clinic Outcome Reporting System (SART CORS) from 2009 to 2013.

Patient(s)

Women at 40–43 years of age undergoing autologous frozen embryo transfers (a-FET) or donor oocyte frozen embryo transfers (d-FET) resulting in singleton pregnancies that were entered in the SART CORS database from 2009 to 2013.

Results

a-FET resulted in 4402 singleton live births whereas d-FET resulted in 2703 singleton live births. d-FET resulted in a higher risk of preterm births (<?37 weeks), with adjusted odds ratio (aOR) 1.33 (95% CI 1.02–1.75), but similar risk of small for gestational age (SGA), with aOR 1.75 (95% CI 0.85–3.7), when compared to a-FET. However, when only single blastocyst transfer cycles are considered, d-FET and a-FET showed no difference in preterm births or other adverse perinatal outcomes.

Conclusions

Singletons resulting from d-FET are at increased risk for perinatal morbidity. However, the risk was diminished in single blastocyst transfer cycles. Our study supports the current American Society for Reproductive Medicine (ASRM) guidelines of transferring a single blastocyst in d-FET cycles.

     

Factors associated with late fetal mortality

Purpose

Perinatal mortality has been decreasing in Europe thanks to a reduction in neonatal mortality. The causes of fetal mortality remain poorly studied. The objective was to determine the late fetal mortality rate in Spain in 2015 and the associated factors.

Methods

A cross-sectional study was performed using data regarding births in 2015 in Spain extracted from the National Institute of Statistics. Single births at 28 or more weeks of pregnancy were included. The sample comprised 340,371 births. Sociodemographic, obstetrical and neonatal variables were analyzed using univariate and multivariate logistic regression (MLR), with the fetal mortality from 28 weeks of pregnancy as the dependent variable.

Results

The total number of late fetal deaths was 884 (2.6 × 1000). The MLR model showed that the following factors were associated with late fetal mortality: birth before 37 weeks of pregnancy (OR 13.1); weight of the newborn < 2500 g (OR 3.22) and ≥ 4000 g (OR 3.36); low training level (OR 2.28); and others, such as African origin, maternal age ≥ 35 years, primiparity and mothers who were single.

Conclusions

The rate of late fetal mortality in Spain has not decreased and has remained at the same level as in 2010. This result is related to prematurity, low birth weight, macrosomia and sociodemographic factors, such as low maternal preparation, mothers of African origin, age ≥ 35 years and mothers who are single. It is necessary to improve the quality and accessibility of prenatal care and the early detection of risk factors.

     

Morbidly Adherent Placenta: Its Management and Maternal and Perinatal Outcome

Objectives

The aim of the study was to identify the risk factors predisposing to morbidly adherent placenta and to study the different modes of management and the obstetric and neonatal outcome of these patients.

Methods

This was a retrospective cum prospective observational study conducted in the Department of Obstetrics and Gynaecology in a tertiary care referral hospital in Mumbai from January 2012 to November 2014.

Results

The incidence of morbidly adherent placenta was 1.32 per 1000 pregnancies with patient profile comprising second gravida in the age group 26–28 years; 90 % of the patients in this study had previous Caesarean section and co-existing placenta praevia was diagnosed in 63 %. Fifty-three per cent of the women delivered between 35 and 38 weeks and 40 % had elective deliveries. Caesarean section was the mode of delivery in 90 % of the patients. Prophylactic balloon placement in the internal iliac artery followed by classical Caesarean section, uterine artery embolization and post-operative methotrexate was done in 27 % which preserved the uterus and was associated the blood loss of 1000–2000 mL.

Conclusion

Antenatal diagnosis of morbidly adherent placenta allows for multidisciplinary planning in an attempt to minimize potential maternal or neonatal morbidity and mortality.

     

Perinatal and maternal outcomes at term in low-risk pregnancies according to NICE criteria: comparison between a tertiary obstetrical hospital and midwife-attended units

Purpose

The aim of this study was to evaluate the perinatal and maternal outcomes at term at a single tertiary, university hospital in women with low-risk pregnancies.

Patients and methods

We performed a retrospective cohort study of women with low-risk pregnancies, who delivered at University Women’s Hospital Magdeburg between January 2010 and December 2014. Data were compared with data published by Brocklehurst et al. 2011.

Results

Of the 6052 women investigated, 2014 were classified as low risk according to the NICE criteria and were eligible for analysis. In 94.8%, a spontaneous vertex birth was observed. There were only 2 (0.1%) perinatal complications and 52 (2.5%) maternal complications. Ventouse delivery, forceps delivery, and caesarean sections were performed in 2.5, 1, and 3.1% of the cases, respectively. Episiotomy was performed in 37.7% of women. The third and fourth degree perineal trauma were observed in 0.3% of births investigated. Complications during the third stage of labour and blood transfusions were observed in 0.25 and 0.2%, respectively. In comparison with the births at home, we had lower rate of fetal complications for nulliparous women, but not for multiparous women, lower rate for blood transfusions, third and fourth degree perineal trauma and forceps delivery, and higher rate of spontaneous vertex birth, epidural analgesia, and episiotomy. The rate of vacuum extractions and caesarean sections were similar between both the places of birth.

Conclusions

The tertiary-level obstetric unit is safe place of birth for women with low-risk pregnancies.

     

Neonatal and maternal risk in Hermansky–Pudlak syndrome: peripartum management—brief report and review of literature

Introduction

Challenges of modern medicine are peripartum bleeding complications as one of the most frequent cases of emergency in obstetrics with a prevalence of 0.5–5.0 %, meaning the main cause of maternal morbidity and mortality. In this context, inherited diseases such as Hermansky–Pudlak syndrome (HPS) should be recognized. HPS is a rare disease and belongs to a heterogeneous group of autosomal recessive disorders characterized by the triad of partial oculocutaneous albinism, disorder of “ceroid” metabolism and platelet storage pool deficiency with bleeding disorder.

Materials and methods

We report on a 30-year-old primipara, to show the peripartum obstetrical and anaesthesiological management. The patient presented with contractions in our outpatient department in the 39th gestational week. In previous operations there were bleeding complications due to HPS.

Discussion

Therefore, to minimize bleeding complications we aimed a vaginal birth, advised against the peridural anesthesia and optimized the coagulation parameters. The subsequent delivery was performed as vacuum extraction without complications. Patient and newborn could be discharged from the hospital without complications.

Conclusion

In conclusion, decisive factor for patients with bleeding disorders is a close interdisciplinary cooperation between obstetrician and anesthesiologist.