Analysis of risk factors for complications and adverse obstetrical outcomes in women with Takayasu arteritis: a French retrospective study and literature review.

OBJECTIVE: Takayasu arteritis (TAK) is a large vessel vasculitis affecting young women of childbearing age. The outcome of pregnancies in TAK patients, factors associated with maternal and foetal complications and adverse outcomes were analysed. METHODS: All pregnancies in women with a TAK diagnosis were retrospectively included from 20 French hospitals providing care for TAK, until August 2015. RESULTS: The study consisted of 43 pregnancies in 33 women, including 29 with a pre-existing TAK diagnosis and 4 diagnosed during pregnancy. Complications were observed in 20 pregnancies (47%), including 35% with arterial hypertension (n = 15), 9% with pre-eclampsia (n = 4), 2% with HELLP syndrome (n = 1) and 14% with intrauterine growth restriction (IUGR, n = 6, leading in one case to a medically indicated termination of pregnancy). There were 42 live births (98%) at a median term of 38 [27-42] weeks gestation including 9 before 37 weeks (21%). The median birth weight was 2940 [610-4310] grams. Five children (12%) required transfer to a neonatal intensive care unit. One premature boy (27 weeks gestation) died after 2 days. Treatment during pregnancy included steroids (n = 25/43; 58%), azathioprine (n = 9/43; 21%) and infliximab (n = 1/43; 2%). The risk of developing arterial hypertension during pregnancy was associated with previous chronic arterial hypertension and with an infra-diaphragmatic vasculitis injury (P = 0.01 and P = 0.04, respectively). No correlation was reported between TAK activity and any of the obstetrical complications described in the study. CONCLUSION: This study showed a high rate of adverse obstetrical complications without significant impact on live birth rates. Pregnancy did not appear to influence TAK disease activity. Key Points • We observed a high rate of adverse obstetrical complications in women with Takayasu arteritis; however, the rate of live births was high. Pregnancy did not appear to influence TA disease activity.

[Management of Gitelman syndrome during pregnancy reporting 12 cases]. / Prise en charge de la grossesse chez des patientes atteintes de syndrome de Gitelman : série de 12 cas.

INTRODUCTION: Gitelman syndrome is a rare hereditary renal tubulopathy, responsable of hypokalemia and hypomagnesaemia-related ionic disorders, which management is poorly codified during pregnancy. We report 12 cases of pregnancies with Gitelman syndrome and we compare our data with those of literature. MATERIAL AND METHODS: It is a report of 12 pregnancies in 5 patients with Gitelman syndrome between 2002 and 2016. Follow up and outcome of pregnancy, delivery modalities and maternal-fetal prognosis have been collected. RESULTS: In our serie, maximum kaliemie observed was 3.4mmol/L, with an average potassium, over all pregnancies of 2.3mmol/L. Oral potassium and magnesium supplementation at the end of pregnancy were 8900mg/day and 460mg/day, respectively. There were no serious maternal complications. Two pregnancies were complicated by intrauterine growth retardation in a context of preeclampsia. There is a large disparity in the methods of anesthetic management of these patients. Materno-fetal prognosis at 1 month post-partum is good. CONCLUSION: Gitelman syndrome is a rare pathology where there is a lack of homogeneity in management of pregnancy. Monitoring of monthly ionogram is necessary. The goal is to obtain stable, non-symptomatic kaliemias, which will never be standardized even in increasing treatment. The most important is to inform and detect situations at risk of decompensation, including vomiting or the use of certain anesthetics. In agreement with literature data, monitoring of fetal growth and the amount of amniotic fluid in the third trimester is still warranted. These pregnancies require the development of a common care in multidisciplinary consultation meeting.

Pregnancy and myeloproliferative neoplasms : A retrospective monocentric cohort.

BACKGROUND: The most frequent myeloproliferative neoplasms are essential thrombocythemia and chronic myelogenous leukemia, which usually manifests with thrombocytosis. Only essential thrombocythemia is associated with morbidity during pregnancy (recurrent miscarriages, intrauterine fetal death, small for gestational age and preeclampsia). The aim of this paper is to describe outcomes of pregnancy in women with myeloproliferative neoplasms seen at a single academic institution. METHODS: Data were collected retrospectively from 2002 to 2015. Descriptive analyses were performed. RESULTS: Eighteen pregnancies in 13 patients and 17 births were identified. One patient had recurrent miscarriages. There were two intrauterine fetal deaths, three small for gestational age linked to vascular placenta pathology and one preeclampsia. All of these mothers harbored JAK2V617F mutation. Two out of three patients with small for gestational age developed a venous thrombosis in the two years following delivery. CONCLUSION: Thrombocytosis associated with myeloproliferative neoplasms should be considered as a risk factor for maternal and fetal complications.

High-Dose Methylprednisolone to Prevent Platelet Decline in Preeclampsia: A Randomized Controlled Trial.

OBJECTIVE: To evaluate whether early administration of high-dose methylprednisolone limits the fall of platelets in preeclampsia. METHODS: A randomized trial of 180 mg methylprednisolone or placebo administered in divided doses over 36 hours was conducted in women admitted for preeclampsia and platelet counts below 150×10/L in four French academic centers. Patients were not included when platelet counts were below 50×10/L or when immediate delivery was required. The primary study outcome was the proportion of patients with platelet counts above 100×10/L 36 hours after the first dose of study medication. The total sample size needed to detect a 23% difference in the rate of this outcome between groups with a one-tailed α of 0.05 and 90% power was 94 patients. RESULTS: Thirty-six patients were randomly assigned to receive methylprednisolone and 34 placebo between October 2007 and May 2011. Platelet counts above 100×10/L at 36 hours after the first dose of study medication were recorded in 30 (83%) in the active group and 29 (85%) in the placebo group (relative risk 0.98, 95% confidence interval 0.80-1.20; P=.82). The only adverse potentially study-related event was hyperglycemia in one woman allocated to methylprednisolone. CONCLUSION: In women with preeclampsia and platelet counts under 150×10/L, methylprednisolone was not effective in maintaining platelet counts above 100×10/L. CLINICAL TRIAL REGISTRATION: EU Clinical Trials Register, http://clinicaltrialsregister.eu, EudraCT 2006-004881-15-FR.

Successful angioplasty during pregnancy for renal artery stenosis.

Renal artery stenosis can be diagnosed during pregnancy and treated at the same time. A 30-year-old woman had a sudden, severe but asymptomatic hypertensive crisis at 21 weeks of gestation. The diagnosis of renal artery stenosis suspected on Doppler ultrasonography was confirmed and treated by renal angioplasty, which reduced her blood pressure. At 27 weeks of gestation, her blood pressure increased again, associated with significant proteinuria, suggesting pre-eclampsia. A cesarean section was performed giving birth to a healthy 940-g child. Renal artery stenosis should be considered when sudden and early-onset hypertension appears during pregnancy.

Effect of pregnancy on the course of immune thrombocytopenia: a retrospective study of 118 pregnancies in 82 women.

In women with pre-existing immune thrombocytopenic purpura (ITP), the effect of pregnancy on the course of the disease is poorly known. We performed a dual-centre retrospective cohort study of 118 pregnancies in 82 women with primary ITP. In early pregnancy, the platelet count was <100 × 10(9) /l in 35·6% of pregnancies. During pregnancy the median platelet count nadir was 66 × 10(9) /l (25th-75th percentile: 42-117), with platelet count <30 × 10(9) /l for 26 pregnancies (22%). In 49% of pregnancies, a significant decrease of the platelet count required treatment at least transiently in preparation for delivery. At the time of delivery, the median platelet count was 110 × 10(9) /l (77-155). Compared to before pregnancy, at 3 months post-partum, only 11% of pregnancies [95% confidence interval (95% CI): 6·8-20·2] showed disease worsening. Previous splenectomy was the only factor significantly associated with ITP worsening after pregnancy (53·9% vs. 10·3%, P < 0·001). For 8·3% of the pregnancies (95% CI: 3·8-15·1), neonatal thrombocytopenia required treatment, especially in case of previous maternal splenectomy (adjusted odds ratio 16·7, 95% CI: 2·61-106). The overall risk of exacerbation of ITP and severe thrombocytopenia during pregnancy is acceptable.

European registry of babies born to mothers with antiphospholipid syndrome.

OBJECTIVES: This study aimed to describe the long-term outcome and immunological status of children born to mothers with antiphospholipid syndrome, to determine the factors responsible for childhood abnormalities, and to correlate the child's immunological profile with their mothers. METHODS: A prospective follow-up of a European multicentre cohort was conducted. The follow-up consisted of clinical examination, growth data, neurodevelopmental milestones and antiphospholipid antibodies (APL) screening. Children were examined at 3, 9, 24 months and 5 years. RESULTS: 134 children were analysed (female sex in 65 cases, birth weight 3000±500 g, height 48±3 cm). Sixteen per cent had a preterm birth (<37 weeks; n=22), and 14% weighted less than 2500 g at birth (n=19). Neonatal complications were noted in 18 cases (13%), with five infections (4%). During the 5-year follow-up, no thrombosis or systemic lupus erythematosus (SLE) was noted. Four children displayed behavioural abnormalities, which consisted of autism, hyperactive behaviour, feeding disorder with language delay and axial hypotony with psychomotor delay. At birth lupus anticoagulant was present in four (4%), anticardiolipin antibodies (ACL) IgG in 18 (16%), anti-ß(2) glycoprotein-I (anti-ß2GPI) IgG/M in 16 (15%) and three (3%), respectively. ACL IgG and anti-ß2GPI disappeared at 6 months in nine (17%) and nine (18%), whereas APL persisted in 10% of children. ACL and anti-ß2GPI IgG were correlated with the same mother's antibodies before 6 months of age (p<0.05). CONCLUSION: Despite the presence of APL in children, thrombosis or SLE were not observed. The presence of neurodevelopmental abnormalities seems to be more important in these children, and could justify long-term follow-up.

[Prevention of heel pressure sores with a foam body-support device. A randomized controlled trial in a medical intensive care unit]. / Efficacité de supports anatomiques en mousse pour la prévention des escarres de talons. Etude contrôlée randomisée en réanimation médicale.

BACKGROUND: To assess in a prospective controlled study the efficacy and safety of a specific foam body-support device designed as to prevent heel pressure ulcers. METHODS: A randomization table was used to allocate 70 patients into 2 groups. The control group was treated with our standard pressure sore prevention protocol (half-seated position, water-mattress and preventive massages 6 times a day); the experimental group was treated with the same standard protocol as well as with the foam body-support device being evaluated. Patients were included if their Waterlow score was >10, indicating a high risk of developing pressure ulcers and if they had no skin lesion on the heels. Foam devices, covered with jersey, were constructed for the legs and allowed the heels to be free of any contact with the bed; another foam block was arranged perpendicularly to the first, in contact with the soles, to prevent ankles from assuming an equinus position (to prevent a dropfoot condition). The principal criterion for efficacy was the number of irreversible skin lesions on the heel (that is, beyond the stage of blanching hyperemia, reversible after finger pressure); these lesions were assessed every day until the end of the study (up to 30 days). FINDINGS: The number of irreversible heel pressure ulcers was lower in the experimental (3 patients, 8.6%) than in the control group (19 patients, 55.4%) (p<0.0001). Mean time without any pressure ulcer was higher in the experimental group (5.6 days, compared with 2.8 days, p=0.01). The groups did not differ in the number of pressure sores on the sacrum and leg. CONCLUSION: An anatomical foam body-support is effective in preventing heel pressure ulcers in patients on a medical intensive care unit and is well tolerated.