A novel cystic fibrosis-pregnancy service: Two-year review after introduction of elexacaftor/tezacaftor/ivacaftor

Background: As a quality service improvement response since elexacaftor/ tezacaftor/ivacaftor (ELX/TEZ/IVA) became available and the yearly average number of cystic fibrosis (CF) pregnancies (n = 7 pre-2020, n = 33 in 2021) increased significantly at an adult CF center (~600 people with CF), a monthly multidisciplinary CF-maternal health virtual clinic was established with antenatal virtual CF exercise classes dedicated to providing adaptive, specialist support to this cohort, aswell as outreach guidance and education to local obstetric teams. Method(s): This was a single-center retrospective reviewof Royal Brompton Hospital CF-Maternal Health multidisciplinary team clinic records and a patient survey from March 2020 to March 2022. Result(s): Of 47 pregnancies in 41 women (median age 30;) eligible for ELX/ TEZ/IVA at start of pregnancy, 40% (n = 19) were unplanned, and 19% (n = 9) used assisted conception. Three women with a history of infertility conceived naturally, having required assisted conception for previous pregnancies, and five women had multiple pregnancies during the study period. ELX/TEZ/IVA was continued in 60% (n = 28), delayed in 28% (n = 13), and stopped in 13% (n = 6) of pregnancies through maternal choice and careful clinical counselling. Pre-pregnancy pulmonary status was poorer in women who continued than in those who delayed or stopped (Table 1). Of those who stopped, 85% (n = 5) restarted because of pulmonary deterioration by the third trimester. Prenatal CF complications included at least one episode of minor hemoptysis in 21% (n = 9/41) of women, at least one infective exacerbation in 55% of pregnancies (n = 26/47), and noninvasive ventilation in one woman. Other pregnancy-associated complications included one case of ovarian hyperstimulation syndrome, one case of sub-segmental pulmonary embolism, and two cases of pregnancy-induced hypertension. Excluding 10 first trimester terminations, 10 current pregnancies, and one patient relocation, obstetric outcomes available for 26 pregnancies confirmed a live birth rate of 85% (n = 22/26) and a 15% first-trimester miscarriage rate (n = 4). Obstetric complications included preterm delivery rate of 23% (n = 6/26), including two cases of COVID infection resulting in two neonatal intensive care unit admissions, one case of endometritis after cesarean section, and a fourthdegree perineal tear. There were no ectopic pregnancies, maternal or neonatal deaths, or reports of infant cataracts or congenital malformations. Median gestational age was 37/40 weeks (range 29-40). Mode of delivery was via cesarean section in 45% (n = 10/22, of which twowere emergency) and vaginal in 55% (n = 12/22), of which 83% (n = 10/12) were via induction of labor for diabetes (CF or gestational) indication. Deliveries were supported and occurred equally at local obstetric units and in tertiarycare obstetric hospital settings (50%, n = 11/22). Patient-experience survey responses cited high levels of confidence in health optimization and prioritization during pregnancy and praised excellent inter-health care provider communication and peer-to-peer emotional support provided among expectant mothers in the virtual prenatal exercise groups. Table 1. Baseline demographic and clinical characteristics of elexacaftor/tezacaftor/ivacaftoreligible expectant mothers according to therapeutic decision (Table Presented) Conclusion(s): In the absence of clinical trial safety data, the novel approach of a dedicated CF-maternal health multidisciplinary team clinic with local obstetric outreach support has ensured regular specialist clinical and emotional peer-to-peer support for this cohort of women eligible for ELX/ TEZ/IVA to ensure optimal outcomes and experiences of their pregnancies, where appropriate, close to home.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved

Analysis of pathogenic bacteria, their drug resistance, and cytokines in 60 patients with a puerperal infection after a Cesarian section

Objectives: To investigate pathogenic bacteria, their drug resistance, and changes in levels of cytokines in patients with a puerperal infection after a Cesarean section.

FAVORABLE MATERNAL AND PERINATAL OUTCOMES OF PREGNANCY IN A COVID-19 PATIENT WITH TOTAL LUNG DAMAGE

Background: The course and prognosis of the novel coronavirus infection (SARS-CoV-2) in pregnant women are unpredictable;at the same time, the clinical presentation can be vague and non-specific;a critical condition can suddenly develop in the presence of the stable course of the disease. In the literature, there are data on survival rates with 100% lung damage caused by SARS-CoV-2 delta variant;however, the experience of managing pregnant women with total lung damage is not presented. Case report: A female patient was admitted to the hospital at 33 weeks of gestation on day 7 of the novel coronavirus infection (SARS-CoV-2). On the 4th day of inpatient treatment, there was a progressive deterioration in the condition of the pregnant woman with a complication as severe bilateral polysegmental interstitial pneumonia. She received combined antibacterial, glucocorticoid, hepatoprotective, infusion, anticoagulant therapy and non-invasive ventilation with the postpartum transition to mechanical ventilation. Taking into consideration the signs of progressive fetal hypoxia, her baby was delivered by cesarean section. The boy was alive premature who had signs of morphofunctional immaturity. After 88 days of inpatient treatment (49 days after 100% lung disease), the female patient was discharged with respiratory support with humidified O2 using a portable concentrator. The patient had been in the intensive care unit for 65 days, of which 46 days on mechanical ventilation. The newborn was discharged home in a satisfactory condition. The clinical case demonstrates favorable gestational and perinatal outcomes with 100% lung involvement, as evidenced by computed tomography. Management of a near-miss pregnant or puerperant woman required an interdisciplinary approach. Specialists used the possibilities of telemedicine for consulting the patients. Conclusion: The accumulation of knowledge and clinical experience in the management of pregnant and puerperant women with SARS-CoV-2 and total lung tissue damage will undoubtedly be able to optimize medical strategies and to prevent maternal and perinatal critical conditions and deaths.

Infections in the structure of comorbid pathology in patients with inflammatory diseases of the joints

Objective: To evaluate the significance of infections in the structure of comorbid pathology in patients with inflammatory diseases of the joint (IDJ) during a single-stage retrospective study. Methods: The study included 437 patients with IDJ (259 women, 178 men, mean age 45 y): 172 -rheumatoid arthritis, 169 -ankylosing spondylitis, 74 -psoriatic arthritis, 22 -undifferentiated spondylarthritis. The majority of patients (n=343) received immunosuppressive therapy (glucocorticoids, methotrexate, leflunomide, biological drugs). The patients were interviewed by a research doctor with the completion of a unified questionnaire. Additional information was obtained from medical records. Results: The following comorbid pathology was documented in patients with IDJ: cardiovascular diseases -30.1%, gastrointestinal diseases -27.2%, respiratory diseases -12.5%, endocrine system diseases -10.9%, urogenital diseases -9.1%, skin diseases, except for psoriasis -4.2%. 653 cases of respiratory tract infections (RTI) and ENT organs and 537 cases of infections of other localization were diagnosed. RTI and ENT organs included acute nasopharyngitis (n=273), tonsillitis (n=110), pneumonia (n=69, including 29 caused by the SARS-CoV2 virus), acute bronchitis (n=54), sinusitis (n=52), influenza (n=47), otitis (n=41), tuberculosis (n=7). Infections of other localizations were represented by herpes-viral infections (n=184), mycoses (n=121), urinary tract infections (n=84), conjunctivitis and blepharitis (n=63), skin infections (n=26), intestinal infections (n=25), genital infections (n=22), osteomyelitis, purulent arthritis, nervous system infections (2 cases each), chronic hepatitis A, B and C, rubella, measles, HIV infection (1 case each). After the debut of IDJ, an increase in the frequency of acute nasopharyngitis, acute bronchitis, sinusitis, herpes-viral infections, and mycoses was noted. Serious infections requiring hospitalization and/or intravenous administration of antibiotics were diagnosed in 78 patients. of these, 64%of cases were caused by RTJ and ENT organs (pneumonia, including those caused by the SARS-CoV2 virus, acute bronchitis, sinusitis, purulent otitis), 36% -by other infections (intestinal infections, purulent paraproctitis, acute salpingitis, purulent endometritis). Conclusion: The problem of infections in patients with IDJ still remains relevant. Further studies are needed on large samples of patients with the aim of studying the prevalence of infections depending on the therapy (primarily, biological drugs), as well as the search for significant risk factors.

A VERY COMPLICATED COURSE after VAGINAL DELIVERY in TYPE 3 von WILLEBRAND DISEASE with ALLOANTIBODIES to von WILLEBRAND FACTOR and FACTOR VIII

Introduction: Delivery in type 3 VWD with alloantibodies, a rare clinical entity with few treatment options, is a very high-risk situation. Methods: Case report Results: A 28 yo patient with type 3 VWD and alloantibodies to VWF and FVIII became pregnant after extensive preconceptional counselling. Previous ITI was unsuccessful and complicated by anaphylaxis. The pregnancy was complicated by a mild COVID-19 infection in the 2nd trimester, but otherwise uncomplicated. Delivery was induced at 38 4/7 weeks with prostaglandin and rFVIIa (NovoSeven®) started when in active labor. After a rapid vaginal delivery and afterbirth, manual placental removal was performed and a Bakri balloon inserted for ongoing bleeding despite rFVIIa 90μg/kg every 2h. As bleeding still continued, plasma-derived VWF was infused with initial excellent recovery and successful embolization of the aa uterinae was performed. Another infusion of VWF to prevent rebleeding resulted in minimal recovery and an allergic reaction despite prednisolone and clemastine. Rebleeding did not occur and patient was discharged at day 8. At day 12 she was readmitted because of endometritis followed by vaginal bleeding unresponsive to rFVIIa. Re-embolization was performed and off label emicizumab started to prevent rebleeding. A loading dose of 6mg/kg on day 1 and 3mg/kg on day 2 was given, followed by 3mg/kg EOW from the 2nd week onwards. As the infection was uncontrolled by broadspectrum antibiotics, hysterectomy was performed at dag 29, again complicated by diffuse bleeding requiring direct intra-abdominal packing and rFVIIa 90μg/kg every 2 hours in addition to emicizumab. A week after unpacking, asymptomatic pulmonary embolisms and thrombosis of the left v iliaca were discovered on CT. rFVIIa was stopped, prophylactic LMWH started and a third embolization performed when bleeding reoccurred. Two months after delivery she was discharged with low dose LMWH, emicizumab and antibiotics because of an intra-abdominal abcess. Discussion/Conclusion: Delivery in patients with severe bleeding disorders in the presence of alloantibodies is a high-risk situation. Emicizumab was partially helpful in maintaining hemostatic control. Besides bleeding, postpartum patients receiving intensive correction of coagulation and especially with additional risk factors like surgery and infection, are also at risk for thrombotic events.

Acute postpartum care utilization after expedited postpartum discharge

Objective: During the COVID-19 pandemic, many hospitals provided expedited postpartum discharge (EPD) for patients wishing to leave the hospital soon after birth. This study was performed to assess whether acute postpartum care utilization increased after EPD during COVID-19. Study Design: Birth hospitalization data from a single teaching hospital from two 6-week periods (3/22-4/30/19 and 3/22-4/30/20) were used for this retrospective cohort study. EPD, defined as discharge on postpartum day 1 or 2 following vaginal or cesarean birth, respectively, was the primary exposure. The primary outcome was acute postpartum care utilization defined as emergency or obstetrical triage unit visits within 6 weeks of delivery. Secondary outcomes included 6-week postpartum readmission. We fit logistic regression models to assess the risk for the primary and secondary outcomes. Categorical comparisons were made with the chi square test. Results: Of 1,358 deliveries in the study, 5.0% of deliveries in 2019 (n=36) compared to 60.3% of deliveries in 2020 (n=388) underwent EPD (p< 0.01). Rates of acute postpartum care utilization were 8.8% and 5.6% for 2019 and 2020, respectively (Figure 1). In 2020, patients with hypertensive disorders of pregnancy (HDP), chorioamnionitis/endometritis, or a positive COVID test were less likely to be discharged early (Table 1). There were no significant differences in rates of acute postpartum care utilization (OR 0.9, 95% CI 0.5, 1.8) or readmissions (OR 1.3, 95% CI 0.5, 3.6) between patients with EPD as compared to routine discharge. Among patients with HDP, readmission risk was significantly higher among those who had early discharge as compared to routine discharge (OR 6.1, 95% CI 2.1, 17.3). Conclusion: Rates of EPD were significantly higher in 2020 compared to 2019 with no impact on acute postpartum care utilization or readmission rates. Among patients with hypertensive disorders of pregnancy, expedited discharge was associated with higher risk of readmission. EPD discharge does not appear to be associated with increased acute postpartum care utilization among low-risk patients. [Formula presented] [Formula presented]