Manuela Wally Ossola

Eculizumab for atypical hemolytic uremic syndrome in pregnancy.

BACKGROUND: Atypical hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy often caused by mutations in complement genes. During pregnancy, disease outcome is poor both for mother and fetus. Since 2009, the humanized monoclonal antibody eculizumab has been successfully used in the treatment of atypical HUS in nonpregnant patients. CASE: A 26-year-old woman with a homozygous mutation in complement factor H developed a relapse of atypical HUS at 17 weeks of gestation in her first pregnancy. Because the disease remained active despite multiple plasma exchanges, eculizumab was started at 26 weeks of gestation. It was well tolerated and has led to remission and to the delivery of a healthy neonate. CONCLUSION: Eculizumab may be useful for the treatment of atypical HUS during pregnancy.

Chorioamnionitis and neonatal outcome in preterm infants: a clinical overview.

Abstract The term chorioamnionitis is used to refer to an intrauterine infection/inflammation occurring between the maternal tissues and the fetal membranes (choriodecidual space) or in the fetal annexes (chorioamniotic membranes, amniotic fluid, umbilical cord). Histological examination of the placenta is the gold standard for diagnosis. However, clinical, biochemical and microbiological criteria are also used to define the disease. The literature contains a large body of evidence showing that chorioamnionitis is the leading cause of very preterm birth and, therefore, contributes significantly to neonatal morbidity and mortality. In recent decades, numerous studies have attempted to establish whether, and to what extent, intrauterine infection/inflammation might negatively affect the short- and long-term outcome of preterm infants. The question is still unanswered. The discrepancy observed across studies can be attributed largely to the use of different inclusion and exclusion criteria, diagnostic criteria and methods, and to whether or not potential confounding factors, such as gestational age were considered. Anyhow, the association between chorioamnionitis and severe prematurity requires serious efforts by researchers to clarify the mechanisms linking intrauterine infection/inflammation with preterm birth, and thus to identify strategies that may guide clinicians’ diagnostic and therapeutic choices, with regard to both mothers and infants.

Predictive factors for neonatal survival in women with periviable preterm rupture of the membranes

Abstract Objective: To identify clinical, hematological or instrumental factors available at the time of the diagnosis that may predict neonatal survival in periviable preterm premature rupture of the membranes (PROM). Methods: We report on a cohort (n = 85) of women with periviable PROM (14–23.6 weeks’ gestation) occurring over a 10-year period in a single institution. The main outcome chosen was the survival rate beyond the neonatal period. Variables considered were those available at 24 h after admission. Results: The overall survival rate was 49%. In the multivariate analysis, significant contributions for the prediction of neonatal survival were provided by four variables: genetic amniocentesis-related cause of PROM (p < 0.001), gestational age at PROM (p = 0.019), CRP > 1 mg/dl within 24 h after admission (p = 0.042) and oligohydramnios (largest vertical pocket ≤2 cm) (p = 0.041). The corresponding adjusted odds ratio (OR)s were 73.9 (95% CI: 7.9–694.7), 1.5 (95% CI: 1.1–2.0) per week, 0.26 (95% CI: 0.07–0.95) and 0.20 (95% CI: 0.04–0.93), respectively. Conclusions: Genetic amniocentesis-related cause of PROM, gestational age at PROM, C-reactive protein >1 mg/dl and oligohydramnios are significantly associated with survival in women with periviable PROM. The evaluation of these few and easily available variables may help physicians and patients in the decision-making process of this demanding condition.

Risk factors for emergency postpartum hysterectomy : the neglected role of previous surgically induced abortions

The objective of this retrospective case–control study was to identify clinical factors associated with emergency peripartum hysterectomy. Deliveries from January 2003 through October 2009 in this tertiary care obstetrics hospital were reviewed. Cases were women who underwent emergency peripartum hysterectomy. Controls were those who delivered immediately after the cases but in whom hysterectomy was not needed. They were matched to cases in a 5:1 ratio. Thirty‐eight cases and 190 controls were selected. Variables found to be significantly associated with emergency postpartum hysterectomy were a stage III–IV placenta previa (p<0.001), previous surgical abortions (p=0.001) and number of fetuses (p=0.039). The corresponding adjusted odds ratios were 40.2 (95% confidence interval 5.6–287.0), 6.0 (95% confidence interval 2.1–17.2) and 7.8 (95% confidence interval 1.1–55.0), respectively. The study confirms the detrimental role of major placenta previa in influencing the risk of postpartum hysterectomy, but also suggests multiple pregnancy and surgical abortion as potential additional risk factors.

Fetoscopic Endoluminal Tracheal Occlusion in Fetuses with Severe Diaphragmatic Hernia: A Three-Year Single-Center Experience.

Objective: To report on our experience in the prenatal treatment of severe congenital diaphragmatic hernia (CDH) by fetoscopic endoluminal tracheal occlusion (FETO). Methods: Between 2012 and 2014, FETO was performed at our center in 21 cases of CDH considered to be severe based on sonographic measurement of observed/expected lung-to-head ratio (O/E LHR) and side of the defect. We reported pre- and postoperative ultrasound findings, procedure-related complications, pregnancy outcome and survival at 1-3 years of age. Results: The median gestational age (GA) at balloon insertion was 28.1 weeks (range 26.0-31.1) and the median GA at delivery 34.7 weeks (range 31.6-39.0); delivery before 32 and 34 weeks occurred in 2 (9.5%) and 7 (33.3%) cases, respectively. Postnatal survival at 1-3 years of age in the 17 cases with isolated unilateral CDH was 47.1%. The percentage difference between pre-balloon removal O/E LHR and pre-FETO O/E LHR was significantly higher in survivors compared to neonates who died (40.8 vs. 21.2%, respectively; p < 0.05). Conclusions: In this study, FETO was associated with an infant survival of 47% in cases with isolated unilateral severe CDH. The post-FETO increase in O/E LHR was higher in fetuses that survived compared to those who died.

Conservative treatment of giant uterine artery pseudoaneurysm

A pseudoaneurysm is an extraluminal collection of arterial blood, with turbulent flow, that results from blood flowing through a defect in an arterial wall. Uterine artery pseudoaneurysm (UAP) is a rare but life-threatening complication of uterine surgery such as Cesarean section1. It may be asymptomatic, may thrombose or may rupture, resulting in secondary postpartum hemorrhage (PPH)1–4. The incidence of ruptured UAP has been estimated to be about 3% of all patients presenting with PPH5. Diagnosis of UAP generally involves color Doppler sonography and confirmation by angiography1,2. It can be treated with arterial embolization or hysterectomy, depending on clinical status2. We describe here our findings in a case of a giant UAP treated with a conservative approach.

Prepartum Eculizumab for prevention of atypical hemolytic uremic syndrome: A case report

Background: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) often caused by complement disregulation for which pregnancy and delivery are common triggers. The disease is associated with a poor prognosis both for the mother and the fetus. Case: A 30-year-old women with a membrane cofactor protein (MCP) gene mutation and two previous aHUS episodes, presented at 23 weeks of pregnancy with no signs of active TMA. Pregnancy proceeded uneventfully and a single dose of Eculizumab was given 24 hours prior to delivery. The patient gave birth to a healthy baby, did not develop TMA and had no side effects. Conclusion: A single prepartum Eculizumab dose may be a cost-effective and safe strategy to face the high risk of relapse in pregnancy-associated aHUS. Correspondence to: Gianluigi ARDISSINO, Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Via Commenda 9, 20122 Milano, Italy, Phone: +39 0255032300; Fax: +39 0255032451; E-mail: ardissino@italkid.org