"An update on the approach to treatment of Sjogren's Disease in pregnancy".

BACKGROUND: Women with Sjögren's Disease are more likely to experience pregnancy complications compared to their counterparts without the disease. Attention to detail and familiarity with the most recent research and guidelines in this field are required to achieve optimal maternal and fetal outcomes. Such complications include pregnancy induced hypertension, fetal growth restriction, thromboembolic events, and preterm delivery. Among the most life-threatening sequela of maternal Sjogren's Disease is fetal autoimmune congenital heart block (ACHB), which has high potential to cause intrauterine fetal death, neonatal mortality, developmental delay, and other long-term pediatric complications. Currently, surveillance with weekly echocardiograms and obstetric sonograms in the second trimester are recommended to screen for ACHB with the goal of early detection and intervention before progression from first- or second- of heart block to complete heart block. OBJECTIVE: We describe a case of maternal Sjogren's Disease, which prompted us to raise questions regarding the optimal frequency of obtaining fetal echocardiograms, and the ideal management in case a prolonged PR interval was to be found. We use this case to provide a springboard for discussion on updated antenatal management strategies for ACHB prevention. METHODS: To conduct this analysis, we searched PubMed for articles published over the last 10 years, with attention focused on articles written since 2016. Additionally, updated guidelines by other specialties such as Rheumatology, Cardiology and Pediatrics on this issue were reviewed. RESULTS: Thorough search of the literature yielded several meta-analyses concurring that the mothers with Sjogren's Disease had increased rates of premature birth, pregnancy induced hypertension, increased risks of delivering infants with intrauterine growth restriction (IUGR), with the most life-threatening risk being that of congenital heart block. Literature supporting prophylactic hydroxychloroquine and the use of steroids to reverse or halt the progression of congenital heart block at the time of diagnoses appeared at the forefront of search results. CONCLUSION: Pregnant women with SS have an increased risk for complications such as intrauterine growth restriction, thromboembolic events, pregnancy-induced hypertension, preterm delivery, and cesarean delivery and should prioritize obtaining pre- or peri-conceptional counseling. In women with anti SSA/SSB antibodies, a medication regimen should be considered with the object of decreasing the concentration of these antibodies, and hence decrease the risks of ACHB. Current literature supports the inclusion of hydroxychloroquine for this purpose, even prior to conception. Although the most recent studies recommend against prophylactic use of steroids, their potential to prevent progression to complete block should be weighed against their potential negative effects. Short and long-term treatment with corticosteroids has been associated with increased maternal risk of infection, weight gain, osteonecrosis, hypertension and bone mineral density disorders. Intrauterine growth restriction, oligohydramnios, and adrenal suppression have been among the fetal risks associated with steroids while improved infant survival or decreased need for pacing have not been demonstrated. Management of these pregnancies is complex and should include a multidisciplinary approach involving a maternal-fetal medicine sub-specialist, a rheumatologist, a pediatrician, a neonatologist, and the patient herself with her family in a model of shared decision-making.

Are Systemic Inflammation Markers Reliable for Diagnosing Intrahepatic Cholestasis of Pregnancy? A Retrospective Cohort Study.

PROBLEM: This study aims to evaluate the effectiveness of inflammation indexes (systemic immune-inflammation index [SII], systemic inflammation response index [SIRI], pan-immune inflammation value [PIV], and neutrophil-to-lymphocyte ratio [NLR]) in the diagnosis of intrahepatic cholestasis of pregnancy (ICP). METHOD OF STUDY: A retrospective study was conducted, reviewing medical records of patients diagnosed with ICP who delivered between October 1, 2022, and May 31, 2023, at the Perinatology clinic of Etlik City Hospital, Ankara. A control group of healthy pregnant women with uncomplicated pregnancies was also included. Demographic data, clinical characteristics, and laboratory results, including systemic inflammation indices and liver enzyme levels, were collected and analyzed. RESULTS: A total of 242 participants were included, with 121 ICP patients and 121 controls. White blood cell count, neutrophil count, and monocyte count showed significant differences between the two groups (p = 0.011, p = 0.004, and p = 0.039, respectively). Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were significantly elevated in the ICP group (p < 0.001 for both). SII and NLR were higher in the ICP group compared to controls (p = 0.032 and p = 0.010, respectively). Receiver operating characteristic (ROC) analysis revealed moderate predictive values for SII (area under the curve [AUC] = 0.581, p = 0.030) and NLR (AUC = 0.598, p = 0.009), with no significant difference in their predictive power (p = 0.502). CONCLUSIONS: Systemic inflammation indices such as SII and NLR offer a cost-effective and rapid means of diagnosing ICP, potentially complementing or surpassing traditional biomarkers like bile acid levels and liver function tests (LFTs). These indices can be easily integrated into routine clinical practice, providing timely intervention to improve maternal and fetal outcomes. Further research is warranted to confirm these findings and establish standardized protocols for their use in ICP management.

Primary hyperparathyroidism-induced acute pancreatitis in pregnancy: A systematic review with a diagnostic-treatment algorithm.

BACKGROUND: Primary hyperparathyroidism (PHPT)-induced acute pancreatitis (AP) during pregnancy has rarely been described. Due to this rarity, there are no diagnostic or treatment algorithms for pregnant patients. AIM: To determine appropriate diagnostic methods, therapeutic options, and factors related to maternal and fetal outcomes for PHPT-induced AP in pregnancy. METHODS: A literature search of articles in English, Japanese, German, Spanish, and Italian was performed using PubMed (1946-2023), PubMed Central (1900-2023), and Google Scholar. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) protocol was followed. The search terms included "pancreatite acuta," "iperparatiroidismo primario," "gravidanza," "travaglio," "puerperio," "postpartum," "akute pankreatitis," "primärer hyperparathyreoidismus," "Schwangerschaft," "Wehen," "Wochenbett," "pancreatitis aguda," "hiperparatiroidismo primario," "embarazo," "parto," "puerperio," "posparto," "acute pancreatitis," "primary hyperparathyroidism," "pregnancy," "labor," "puerperium," and "postpartum." Additional studies were identified by reviewing the reference lists of retrieved studies. Demographic, imaging, surgical, obstetric, and outcome data were obtained. RESULTS: Fifty-four cases were collected from the 51 studies. The median maternal age was 29 years. PHPT-induced AP starts at the 20th gestational week; higher gestational weeks were seen in mothers who died (mean gestational week 28). Median values of amylase (1399, Q1-Q3 = 519-2072), lipase (2072, Q1-Q3 = 893-2804), serum calcium (3.5, Q1-Q3 = 3.1-3.9), and parathormone (PTH) (384, Q1-Q3 = 123-910) were reported. In 46 cases, adenoma was the cause of PHPT, followed by 2 cases of carcinoma and 1 case of hyperplasia. In the remaining 5 cases, the diagnosis was not reported. Neck ultrasound was positive in 34 cases, whereas sestamibi was performed in 3 cases, and neck computed tomography or magnetic resonance imaging was performed in 9 cases (the enlarged parathyroid gland was not localized in 3 cases). Surgery was the preferred treatment during pregnancy in 33 cases (median week of gestation 25, Q1-Q3 = 20-30) and postpartum in 12 cases. The timing was not reported in the remaining 9 cases, or surgery was not performed. AP was managed surgically in 11 cases and conservatively in 43 (79.6%) cases. Maternal and fetal mortality was 9.3% (5 cases). Surgery was more common in deceased mothers (60.0% vs 16.3%; P = 0.052), and PTH values tended to be higher in this group (910 pg/mL vs 302 pg/mL; P = 0.059). Maternal mortality was higher with higher serum lipase levels and earlier delivery week. Higher calcium (4.1 mmol/L vs 3.3 mmol/L; P = 0.009) and PTH (1914 pg/mL vs 302 pg/mL; P = 0.003) values increased fetal/child mortality, as well as abortions (40.0% vs 0.0%; P = 0.007) and complex deliveries (60.0% vs 8.2%; P = 0.01). CONCLUSION: If serum calcium is not tested during admission, definitive diagnosis of PHPT-induced AP in pregnancy is delayed, while early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes.

Validation of the Turkish version of the Modified Early Obstetric Warning System (MEOWS) chart.

BACKGROUND: The Modified Early Obstetric Warning System (MEOWS) is a score-based or color-coded system that detects changes in physiological parameters and enables earlier diagnosis and care of worsening obstetric patients. The aim of this study is to evaluate the tool's performance and contribute to its use in Türkiye by translating MEOWS into Turkish. METHODS: This prospective and descriptive study, approved by the local ethics committee, included 350 obstetric in-patients who gave birth at Samsun Training and Research Hospital, Gynecology and Children's Hospital between April and August 2022. The study involved patients with a gestational week greater than 28 weeks and up to six weeks postpartum. RESULTS: The average age of the patients was 28.9±5.9 (18-40) years, with trigger values occurring in 34.6% (n=121) and morbidity occurring in 30.9% (n=108) of the cases. The most common trigger among the individual physiological indicators was high systolic blood pressure (28.3%). When the performance of MEOWS was evaluated, a statistically significant correlation was found between trigger and morbidity (Kappa=0.605; p<0.001). The sensitivity of MEOWS in estimating morbidity was 77.78% (95% confidence interval [CI]: 68.76-85.21%), specificity was 84.71% (95% CI: 79.55-89.00%), Positive Predictive Value (PPV) was 69.42% (95% CI: 62.40-75.64%), Negative Predictive Value (NPV) was 89.52% (95% CI: 85.67-92.43%), and accuracy was 82.57% (95% CI: 78.18-86.40%). CONCLUSION: MEOWS was found to be an effective screening tool for predicting morbidity in this study and performs well in Turkish with sufficient sensitivity, specificity, and accuracy. However, the inclusion of long-term results would provide a more comprehensive understanding of the effectiveness of MEOWS.

Choledochal Cyst Diagnosed during Pregnancy and Treated by Postpartum Laparoscopic Choledochal Cyst Excision: Case Report.

We report a rare case of choledochal cyst with acute cholangitis that was diagnosed at 37 weeks' gestation and treated by laparoscopic choledochal resection and biliary reconstruction after delivery. A 31-year-old Japanese primigravida at 37 weeks' gestation presented with right upper quadrant pain. The patient was diagnosed as having acute cholangitis due to a type-Ia choledochal cyst, according to the Todani classification, with pancreaticobiliary maljunction. Acute cholangitis improved with conservative treatment, the fetus was delivered by Cesarean section at 38 weeks' gestation, and the patient was treated by laparoscopic choledochal cyst excision and biliary reconstruction at 47 days postpartum. Total operation time was 579 minutes and intraoperative body fluid loss was 100 mL. The patient is now healthy with normal liver function 7 years after the operation. To ensure good outcomes for the mother and fetus, treatment decisions for choledochal cyst diagnosed during pregnancy must be carefully considered.

Misdiagnosed case of acute angle closure caused severe visual field defects.

In this case report, a 33-year-old pregnant woman with migraine and visual aura complained of headache, nausea, and blurred vision. Clinically, she presented with bilateral dilated and fixed pupils, ptosis, and tearing. She was erroneously diagnosed as having a migraine attack, but after referral to an ophthalmological department she was diagnosed with bilateral acute angle closure. At one-year follow-up her visual acuity was normalized but the severe visual field defects affected her daily activities and prevented her from having a driving licence.

Urethral diverticulum in pregnancy: Rare case report and brief literature review.

OBJECTIVE: Female urethral diverticulum (UD), an evagination of the urethral mucosa into the surrounding connective tissue, is extremely rare in pregnancy. No clear guidelines on the optimal management of UD have been established, except for a common conservative approach. Here, we discuss how to manage UD with pregnancy. CASE REPORT: A 39-year-old gravida 4, para 0, abortion 3 (G4P0A3) woman at 34+0 gestational weeks (GW) visited our outpatient department with a 6-cm septate vaginal mass. Transvaginal ultrasound sonography (TVUS) revealed a 5.5 x 4.9-cm multicystic mass, which was confirmed as UD with pelvic MRI. She was admitted because of preterm labor. A cesarean section was performed at 36+5 GW due to a previous myomectomy, and a healthy male baby was born. UD was still observed in the patient two months after delivery. Periurethral diverticulectomy was performed, and pathological analysis revealed UD with chronic inflammation and edema. CONCLUSION: Previous reports and our case report show that UD can develop during pregnancy and that pelvic MRI is suitable for its accurate diagnosis. Vaginal delivery is possible in pregnant women with the small size of the UD. UD aspiration can permit vaginal delivery in a few cases; however, pus can occur at the aspirated site after the operation. If UD is still observed after delivery, urethral diverticulectomy is recommended.

Maternal and Fetal Outcomes in Disseminated Intravascular Coagulation Cases Associated with Pregnancy Complications.

BACKGROUND: The relationship between the pregnancy modified DIC score, which is applied in obstetric conditions where the risk of disseminated intravascular coagulation is high, and underlying disease, as well as its effect on the prognosis, was investigated. METHODS: Those with a DIC score ≥ 26 from obstetric conditions, such as obstetric bleeding, placental abruption, or preeclampsia/HELLP syndrome, which are at high risk of developing DIC, were included in the study. These patients were compared in terms of laboratory results, maternal morbidity/mortality, and neonatal outcomes, according to the underlying disease. RESULTS: The DIC score was ≥ 26 in 224 of 154,233 deliveries in our center, and the incidence was 0.14%. In the preeclampsia/HELLP syndrome group, the platelet count and prothrombin time were lower, and the fibrinogen level was higher than those of the obstetric hemorrhage and placental abruption groups. In addition, the rates of blood transfusion and hysterectomy were lower in women who developed DIC due to pre-eclampsia/HELLP syndrome than in those with obstetric hemorrhage. CONCLUSIONS: Considering the underlying disease is an important factor in predicting prognosis, when using the new pregnancy modified diagnostic scores for DIC diagnosis.

Predominant approaches to measuring pregnancy-related anxiety in Sub-saharan Africa: a scoping review.

BACKGROUND: Pregnancy-related anxiety significantly impacts maternal and fetal health in low- and middle-income countries (LMICs), including those within Sub-Saharan Africa (SSA). Most studies conducted to evaluate pregnancy-related anxiety in LMICs have utilized scales developed in high-income countries, despite significant variations in pregnancy-related anxiety due to socioeconomic and cultural contexts. This review surveyed existing literature in order to identify which scales have been used to measure pregnancy-related anxiety in SSA. METHODS: A systematic search was conducted in PubMed, Health and Psychosocial Instruments, and APA PsycNet for relevant studies published in the English language up to March 22, 2023. Eligible studies focused on anxiety in pregnant populations within SSA, using validated scales or tools. Screening followed PRIMSA guidelines, with blinded review at the abstract/title level and subsequent full-text review. Data was extracted and analyzed to identify trends and characteristics of the screening tools used. RESULTS: From 271 articles, 37 met inclusion criteria, identifying 24 different tools used to measure anxiety in pregnant women in SSA. The most common tools were the Generalized Anxiety Disorder 7-item scale (seven uses), State-Trait Anxiety Inventory (five uses), and the Self-Reporting Questionnaire 20 (five uses). Seven tools were pregnancy-specific, with only two designed specifically for SSA: the Risk Factor Assessment (RFA), and the 4-Item Screening Tool. Studies were most frequently conducted in South Africa, followed by Tanzania, Ethiopia, Nigeria, and Ghana. CONCLUSIONS: This scoping review illustrates that only two tools (the RFA and 4-item Screening Tool) were created to assess pregnancy-related anxiety specifically in SSA. This highlights the need for more culturally sensitive tools tailored to the specific contexts of pregnant populations in SSA.

Subacute thyroiditis: a rare presentation in pregnancy.

Not required for Clinical Vignettes.

Neonatal pemphigus vulgaris.

A neonate presented to a tertiary clinic with blisters and erosions over his trunk and extremities. The mother had multiple erosions with crusts affecting the scalp, oral cavity and trunk present since the first trimester and worse since delivery. Skin biopsy for histopathology and direct immunofluorescence confirmed pemphigus vulgaris (PV) in the mother. Indirect immunofluorescence assays for serum antibodies against desmogleins 1 and 3 were positive in both mother and baby confirming a diagnosis of neonatal PV. The baby's lesions healed spontaneously within 2 weeks, and the mother was clear at 2 months following treatment with rituximab.

The screening accuracy of the Edinburgh Postnatal Depression Scale (EPDS) to detect perinatal depression with and without the self-harm item in pregnant and postpartum women.

BACKGROUND: This study aims to examine whether the Edinburgh Postnatal Depression Scale (EPDS), excluding the self-harm item (EPDS-9), performs as effectively as the full EPDS in identifying depression among perinatal women. METHODS: A total of 3571 pregnant women and 3850 postpartum women participated in this observational study. Participants who scored ≥ 9 on the EPDS underwent further diagnostic evaluations by a clinical psychologist and/or psychiatrist. RESULTS: The EPDS-9 and full EPDS demonstrated a near-perfect correlation in both the antepartum (r = 0.996) and postpartum (r = 0.998) cohorts. EPDS-9 showed exceptional precision in identifying depression as screened by the full EPDS at cutoff points ranging 9-14, with areas under the curve ≥0.998. The sensitivity of EPDS-9 and full EPDS to detect depression that requires psychotropic medications was poor. The highest accuracy for both versions was at a cutoff score of 9: sensitivity of 0.579 for the full EPDS and 0.526 for the EPDS-9. At the cutoff point of 9, EPDS-9 performed adequately in predicting the response of the participants to the self-harm item. CONCLUSION: The EPDS-9 represents a solid and effective replacement for the full EPDS in clinical settings. If the presence of suicidal thoughts needs to be assessed, specialized scales should be used.

Reproductive Health in RA, Lupus, and APS.

ABSTRACT: Systemic lupus erythematosus, antiphospholipid syndrome, and rheumatoid arthritis are chronic autoimmune diseases affecting women of childbearing age. These diseases may impair fertility and fecundity, as well as complicate pregnancy and the puerperium in these patients including disease flare and obstetric complications on both the maternal and fetal side. For each patient, an appropriate preconceptional counseling with risk stratification is required, including assessment of disease activity, organ involvement, serological profile, and comorbidities.In cases of pregnancy, the aims of treatment are to prevent disease activity, to treat disease activity in cases of flare, and to prevent maternal and fetal complications such as preeclampsia or fetal loss. In all patients with these diseases, close clinical monitoring during pregnancy and puerperium is mandatory. This review aims to summarize the fertility issues in patients with systemic lupus erythematosus, antiphospholipid syndrome, and rheumatoid arthritis and to provide an update on pregnancy management and outcomes in these patients.

Reproductive Health in Scleroderma, Vasculitis, and Sjögren Syndrome.

ABSTRACT: Women with systemic chronic inflammatory disease, such as those with scleroderma, systemic vasculitis, and Sjögren syndrome, need preconception evaluation by a multidisciplinary team. Counseling and pregnancy management should be tailored to patients' needs, considering specific disease features, organ involvement, treatment options, and risk factors to minimize risks of maternal-fetal complications during pregnancy.Additionally, considerations regarding fertility, assisted reproductive techniques, and contraception also need to be addressed for these women.In this narrative review, we integrate the current published literature with our expert opinion to address the issues faced by patients with the aforementioned inflammatory conditions.

Torsion of Tubo-Ovarian Complex during Pregnancy: A Case Report.

Introduction: Adnexal torsion is characterised by the rotation of the ovary and, occasionally, the fallopian tube around their supporting ligaments by more than 45 degrees. It predominantly occurs during the first and second trimesters of pregnancy, with an incidence of up to 0.1% in the third trimester. Dermoid and functional ovarian cysts, most commonly associated with benign serous cystadenomas, are frequently identified among the torted adnexal masses. Case Presentation: We report the case of a 32-year-old primigravida with a known ovarian cyst diagnosed in the first trimester, which was managed conservatively throughout the pregnancy. At 36 weeks of gestation, she presented with abdominal pain and was subsequently managed with an emergency caesarean section at 37 weeks due to the development of an acute surgical abdomen. During the procedure, a torted left tubo-ovarian complex was excised, with partial preservation of the healthy-appearing ovarian tissue. Histopathological examination identified the mass as a benign serous cystadenoma. Conclusions: Ovarian torsion during pregnancy poses a significant diagnostic challenge. The decision between conservative management and surgical intervention is primarily guided by a high index of suspicion for torsion.

Early detection of perinatal depression in couples: a single-center prospective study.

OBJECTIVE: This prospective study aimed to assess couples' psychological status during the perinatal period to identify those at risk for postpartum depression. METHODS: Conducted at Lyon University Hospital from March to July 2022, the study enrolled pregnant women without progressive psychiatric disorders or obstetric risk factors, and their partners. Participants completed the Edinburgh Postnatal Depression Scale (EPDS) at three points: during the 9th month of pregnancy, immediate postpartum, and 6-8 weeks after delivery. A score ≥10 on the EPDS indicated depression risk. A score ≥10 on the EPDS indicate depression risk. The primary endpoint was EPDS scores throughout the perinatal period. RESULTS: Ninety-five couples participated; 96% of patients and 68% of partners completed pre-delivery questionnaires, 81% and 71% during maternity stay, and 64% and 46% postpartum, respectively. Overall, 15% of patients and 1% of partners had EPDS scores >10 in the postpartum period. Psychiatric history and emergency cesarean sections were associated with higher immediate postpartum EPDS scores in patients [Beta 3.7 points, 95% CI 0.91; 6.4 and Beta 5.2 points, 2.2; 8.1, respectively]. Episiotomy was associated with higher EPDS scores in partners. No significant association between the different factors studied and the EPDS score was found at 6-8 weeks postpartum in patients nor their partners. CONCLUSIONS: While specific risk factors for persistent perinatal depression in couples were not identified, a notable proportion of patients exhibited high EPDS scores. Screening all couples during prepartum and postpartum periods is crucial, regardless of identified risk factors.

Bruck syndrome in pregnancy.

Bruck syndrome is a rare, autosomal-recessive condition associated with features of both arthrogryposis and osteogenesis imperfecta. It is characterised by congenital large joint contractures with pterygia and bone fragility, leading to fractures and deformities, along with a short stature caused by progressive skeletal deformities. There are fewer than 50 described cases of Bruck syndrome in the literature, with no reported cases in pregnancy. We describe a case of a successful pregnancy in a woman with Bruck syndrome.In pregnant women with Bruck syndrome, we recommend a multidisciplinary approach including input from obstetric and fetal medicine specialists, midwives, anaesthetists, geneticists, occupational therapists and physiotherapists.