Menstrual and obstetrical bleeding in women with inherited platelet receptor defects—A systematic review

Introduction: Women with inherited platelet receptor defects (IPRD) may have an increased risk of heavy menstrual bleeding (HMB) and postpartum haemorrhage (PPH). Aim: To present a systematic overview of the literature on the prevalence and management of menstrual and obstetrical bleeding in women with IPRD. Methods: Electronic databases were searched for original patient data on the prevalence and management of HMB and PPH in women with known IPRD or who were being investigated for IPRD. Results: Sixty-nine papers (61 case reports/series and 8 cohort studies) were included. Overall, studies were rated as ‘poor quality’. The included cohort studies reported HMB in 25% (13/52) of women with Bernard-Soulier syndrome and in 22.1% (34/154) of women with Glanzmann thrombasthenia. In total, 164 deliveries in women with IPRD were described. Excessive bleeding occurred in 16.9% (11/65) of deliveries described in the largest cohort. PPH occurred in 63.2% (55/87) of deliveries described in case reports/series. PPH occurred in 73.7% (14/19) of deliveries that were not covered by prophylaxis compared with 54.2% (32/59) of deliveries that were (OR = 2.36, 95% CI 0.75-7.40). Neonatal bleeding complications were reported in 10.0% (8/80) of deliveries. In all (6/6) deliveries with neonatal bleeding complications wherein the presence of alloantibodies was investigated, either antiplatelet or anti-HLA antibodies were detected. Discussion/Conclusion: Menstrual and particularly obstetrical bleeding problems frequently occur in women with IPRD, based on small case reports and series of poor quality. International collaboration, preferably on prospective studies, is needed to improve clinical management of women-specific bleeding in IPRD

Quality of intrapartum care:direct observations in a low-resource tertiary hospital

BACKGROUND: The majority of the world's perinatal deaths occur in low- and middle-income countries. A substantial proportion occurs intrapartum and is avoidable with better care. At a low-resource tertiary hospital, this study assessed the quality of intrapartum care and adherence to locally-tailored clinical guidelines. METHODS: A non-participatory, structured, direct observation study was held at Mnazi Mmoja Hospital, Zanzibar, Tanzania, between October and November 2016. Women in active labour were followed and structure, processes of labour care and outcomes of care systematically recorded. Descriptive analyses were performed on the labour observations and compared to local guidelines and supplemented by qualitative findings. A Poisson regression analysis assessed factors affecting foetal heart rate monitoring (FHRM) guidelines adherence. RESULTS: 161 labouring women were observed. The nurse/midwife-to-labouring-women ratio of 1:4, resulted in doctors providing a significant part of intrapartum monitoring. Care during labour and two-thirds of deliveries was provided in a one-room labour ward with shared beds. Screening for privacy and communication of examination findings were done in 50 and 34%, respectively. For the majority, there was delayed recognition of labour progress and insufficient support in second stage of labour. While FHRM was generally performed suboptimally with a median interval of 105 (interquartile range 57-160) minutes, occurrence of an intrapartum risk event (non-reassuring FHR, oxytocin use or poor progress) increased assessment frequency significantly (rate ratio 1.32 (CI 1.09-1.58)). CONCLUSIONS: Neither international nor locally-adapted standards of intrapartum routine care were optimally achieved. This was most likely due to a grossly inadequate capacity of birth attendants; without whom innovative interventions at birth are unlikely to succeed. This calls for international and local stakeholders to address the root causes of unsafe intrafacility care in low-resource settings, including the number of skilled birth attendants required for safe and respectful births

The experiences and attitudes of hemophilia carriers around pregnancy: A qualitative systematic review

Background: Hemophilia carriers (HCs) face specific psychosocial challenges related to pregnancy, caused by their inherited bleeding disorder. Optimal support from healthcare providers can only be realized by exploring medical and psychological healthcare requirements. Objective: To review all published evidence on the experiences and attitudes of HCs regarding reproductive decision-making, prenatal diagnosis, pregnancy, childbirth, and puerperium to provide an accessible overview of this information for health care providers. Study selection: Cochrane library, PubMed/MEDLINE, EMBASE, CINAHL, and PsycINFO were searched for original qualitative data. Two authors performed study selection, risk-of-bias assessment, data extraction, and data analysis through meta-summary. The extracted themes were discussed within the research team. Findings: Fifteen studies with an overall moderate quality were included. The following findings were identified: (a) Quality of life of family members with hemophilia influences reproductive decision-making; (b) Genetic counselling is generally considered useful; (c) The development of a specialized carrier clinic is considered valuable; (d) HCs describe prenatal diagnosis as beneficial yet psychosocially challenging; and (e) noninvasive prenatal diagnosis and preimplantation genetic diagnosis are predominantly considered beneficial. These findings are limited by the overall moderate quality of included studies and the possibly partly outdated results in the current era of hemophilia treatment. Conclusions: Available qualitative literature on HCs around pregnancy focuses on genetic counselling and prenatal diagnosis. Future studies are needed on the experiences and needs of HCs through pregnancy and puerperium as well as in light of emerging hemophilia diagnosis and treatment options

Menstrual problems in chronic immune thrombocytopenia: A monthly challenge - a cohort study and review

Immune thrombocytopenia (ITP) may cause menstrual problems. This cross-sectional study assessed menstrual problems in premenopausal chronic ITP women by several questionnaires, including the pictorial bleeding assessment calendar (PBAC; score ≥100 indicates heavy menstrual bleeding [HMB]), and the menorrhagia multiattribute scale (MMAS). Spearman was used for assessing correlations. A literature review was performed in Pubmed. The cohort comprised 37 women (mean age 31 ± 9). A total of 29/37 (78%) had experienced clinical menstrual problems in the present or past. Of the 33 patients who returned the PBAC, 13 (39%) had a score of ≥100. The median MMAS score was 79 (IQR 60-95). The PBAC scores correlated with the MMAS. Both questionnaires were unrelated to the platelet count. Patients with a levonorgestrel intrauterine device (LNG-IUD) had lower PBAC scores than patients with other or no hormonal therapy. MMAS scores were correlated with fatigue. The review identified 14 papers. HMB occurred in 6%-55% at ITP diagnosis and 17%-79% during disease. Menstrual symptoms influenced the quality of life, particularly in patients with a low platelet count. This explorative study suggested that HMB is frequent in women with chronic ITP despite management and platelet counts >50 *10 9 /l. An LNG-IUD seemed to reduce blood loss significantly

Platelet count and indices as postpartum hemorrhage risk factors: a retrospective cohort study

Background: Severe postpartum hemorrhage (SPPH) is the leading cause of maternal mortality and morbidity worldwide. Platelet anomalies frequently occur during pregnancy. However, their role in the etiology of SPPH is largely unknown. Objective: To study the relation between platelet parameters and SPPH. Methods: This retrospective single-center cohort included deliveries between 2009 and 2017. SPPH was defined as ≥1000 ml blood loss within 24 h after delivery. Platelet parameters were measured within 72 h before delivery. Multiple imputation was performed for missing data. Odds ratios were adjusted (aORs) for maternal age, multiple gestation, macrosomia, induction of labor, preeclampsia, and hemolysis, elevated liver enzymes, and low platelets syndrome. Results: A total of 23 205 deliveries were included. Of the 2402 (10.4%) women with thrombocytopenia (<150 × 10 9/L), 10.3% developed SPPH, compared with 7.6% of women with a normal platelet count (aOR: 1.34, 95% CI: 1.14–1.59). Women with a platelet count of <50 × 10 9/L were most at risk (aOR of 2.24 [1.01–4.94]) compared with the reference group with normal platelet counts; the aOR was 1.22 (0.77–1.93) for the 50–99 × 10 9/L platelet count group and 1.31 (1.10–1.56) for the 100–149 × 10 9/L platelet count group. Plateletcrit was associated with SPPH (aOR 1.15 [1.08–1.21] per 0.05% decrease), and, although rarely present, a platelet distribution width (PDW) ≥23% (n = 22) also increased the odds of SPPH (aOR 6.05 [2.29–16.20]). Conclusion: Different degrees of thrombocytopenia were independently associated with the occurrence of SPPH. Despite their relation to SPPH, plateletcrit and a PDW of ≥23% have limited additional value in addition to platelet count

Prevalence, burden and treatment effects of vaginal bleeding in women with (suspected) congenital platelet disorders throughout life: a cross-sectional study

Congenital platelet disorders (CPDs) are rare bleeding disorders that are associated with mucocutaneous bleeds. However, data on vaginal bleeding in women with CPDs are scarce. A set of generic and bleeding-specific questionnaires were used to evaluate the prevalence of vaginal bleeding, its impact on quality of life (QoL) and sexual functioning and the consequences for pregnancy, miscarriage and delivery in a cohort of women who were referred for diagnostic evaluation for CPDs. A total of 78 women included in the study were either diagnosed with a CPD (n = 35) or were clinically suspected of a CPD (n = 43). Heavy menstrual bleeding (HMB) was reported by a large proportion of women, which mainly started at menarche. In all, 76% of women received any kind of HMB treatment, often leading to surgical prodecures. HMB was shown to have a high impact on QoL, which improved upon treatment. Even though women reported that vaginal bleeding affects sexuality, this topic is not frequently discussed with physicians. Heavy blood loss frequently occurred after miscarriage/delivery, often requiring treatment. Women with (suspected) CPDs frequently encounter HMB, negatively impacting daily life and sexual functioning. Together with peripartum bleeding, these data highlight the burden of vaginal bleeding in CPDs and importance of adequate treatment

The ISTH-BAT score and outcomes after endometrial ablation in women with heavy menstrual bleeding

BACKGROUND: The International Society on Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT), is used during the diagnostic workup of bleeding disorders. Data on ISTH-BAT scores in women with heavy menstrual bleeding (HMB) undergoing endometrial ablation (EA) could be essential in optimizing HMB counselling. OBJECTIVE: To investigate the postsurgical incidence of amenorrhea, dysmenorrhea, quality of life, re-intervention after EA, and ISTH-BAT score. METHODS: This study included women who have undergone EA because of HMB. During a follow-up of 2 to 5 years, ISTH-BAT, pictorial blood assessment chart (PBAC), and Short Form-36 survey (SF-36) were administered. At 10 years of follow-up surgical re-interventions were evaluated. RESULTS: Seventy-one women were included of whom 77% (n = 55) had an ISTH-BAT score &lt; 6, versus 23% (n = 16) ISTH-BAT score =6 (mean age 46.3 versus 42.3, p = 0.004). In the ISTH-BAT =6 group versus &lt; 6 group, amenorrhea occurred in 63% (10/16) versus 82% (45/55) (p = 0.111), dysmenorrhea in 38% (6/16) versus 18% (10/55) (p = 0.111), and surgical re-intervention in 19% (3/16) versus 25% (14/55) (p = 0.582). SF-36 item (Bodily) pain was lower in the ISTH-BAT =6 group versus &lt; 6 (median score 58.7 vs. 80.0, p = 0.104). CONCLUSIONS: An ISTH-BAT score =6 may be related to a lower amenorrhea incidence and higher dysmenorrhea rate after EA

Reliability of IMU-Based Gait Assessment in Clinical Stroke Rehabilitation

Background: Gait is often impaired in people after stroke, restricting personal independence and affecting quality of life. During stroke rehabilitation, walking capacity is conventionally assessed by measuring walking distance and speed. Gait features, such as asymmetry and variability, are not routinely determined, but may provide more specific insights into the patient’s walking capacity. Inertial measurement units offer a feasible and promising tool to determine these gait features. Objective: We examined the test–retest reliability of inertial measurement units-based gait features measured in a two-minute walking assessment in people after stroke and while in clinical rehabilitation. Method: Thirty-one people after stroke performed two assessments with a test–retest interval of 24 h. Each assessment consisted of a two-minute walking test on a 14-m walking path. Participants were equipped with three inertial measurement units, placed at both feet and at the low back. In total, 166 gait features were calculated for each assessment, consisting of spatio-temporal (56), frequency (26), complexity (63), and asymmetry (14) features. The reliability was determined using the intraclass correlation coefficient. Additionally, the minimal detectable change and the relative minimal detectable change were computed. Results: Overall, 107 gait features had good–excellent reliability, consisting of 50 spatio-temporal, 8 frequency, 36 complexity, and 13 symmetry features. The relative minimal detectable change of these features ranged between 0.5 and 1.5 standard deviations. Conclusion: Gait can reliably be assessed in people after stroke in clinical stroke rehabilitation using three inertial measurement units