Inborn errors of metabolism in a cohort of pregnancies with non-immune hydrops fetalis: a single center experience.

OBJECTIVE: The purpose of this study was to determine the frequency of non-immune hydrops fetalis (NIHF) among all pregnancies referred for prenatal care at Sultan Qaboos University Hospital (SQUH) during the study period and to evaluate the underlying etiologies of NIH. STUDY DESIGN: All pregnancies referred to SQUH between February 2014 and December 2015 were identified, and all pregnancies meeting the diagnosis of NIHF were included in this study. All cases of NIHF referred to our center during this period underwent standard systematic diagnostic work-up that included biochemical and molecular studies in addition to the standard investigations for hydrops fetalis. Clinical characteristics and results of the diagnostic work-up were retrospectively reviewed. RESULTS: A total of 3234 pregnancies were referred for prenatal care at SQUH during the study period, and 12 pregnancies were affected by NIHF. An underlying diagnosis was established in nine cases, and the majority of cases (7/9) were caused by inborn errors of metabolism (IEM). These included a novel homozygous variant in the AARS2 gene (5/7) and two cases of galactosialidosis (2/7). CONCLUSION: IEM was a major cause of NIHF in this cohort. The AARS2 variant accounts for a significant number of cases with NIHF in this cohort of Omani patients.

Successful management of severe hemolytic disease of the fetus due to anti-Jsb using intrauterine transfusions with serial maternal blood donations: a case report and a review of the literature.

BACKGROUND: The management of pregnant women with anti-Jsb is challenging due to the paucity of antigen-negative blood for fetal and neonatal transfusion. CASE REPORT: A 29-year-old woman with anti-Jsb was referred for assessment of recurrent fetal losses. With the presence of the sister as a historically matched donor, she was planned for active surveillance for fetal anemia during pregnancy. STUDY DESIGN AND METHODS: The fetus remained well until 21 weeks of gestation when signs of fetal anemia and early hydrops fetalis were noted. Anti-Jsb titer was at 128. The sister's red blood cells (RBCs) were cross-match incompatible. Urgent intrauterine transfusion (IUT) was performed with washed irradiated maternal RBCs, donated after cessation of heparin. The mother was given intravenous iron (IV-Fe) and continued on weekly recombinant human erythropoietin (rHu-EPO). RESULTS: Repeated IUTs were needed every 1 to 3 weeks. Throughout a 7-week period, three maternal donations were performed with total donated whole blood volume of 1250 mL, supporting four IUTs. At 29 weeks of gestation, the procedure was complicated by umbilical cord hematoma necessitating urgent cesarean section. A male newborn was delivered, transfused at birth, and subsequently treated with phototherapy and five top-up transfusions. CONCLUSION: This case represents a successful example of managing hemolytic disease of the fetus due to a rare antibody using maternal blood. It also supports previous data on safety of maternal donations during pregnancy and the use of combination of rHu-EPO and IV-Fe as a supportive measure.

Maternal complications and the association with baseline variables in pregnant women with sickle cell disease.

Sickle cell disease is an inherited hemoglobinopathy with multi system complications. It has been associated with multiple maternal complications. A retrospective review of 68 consecutive pregnant women with sickle cell disease, followed in a tertiary center, was conducted over 5 years, to estimate the incidence of different maternal complications and the impact of baseline characteristics. Sixty-eight patients were analyzed (mean age 30 years). Sixty-two patients had a Hb SS genotype. The initial mean hemoglobin (Hb) level was 9.5 g/dL. Twelve patients delivered by Cesarean section. Sixty-five patients required admission for sickle cell disease/pregnancy-related complications [96.0%; 95% confidence interval (95% CI) 91-100]. Infection was seen in 17 patients (25.0%, 95% CI 14-36). Blood transfusions were given to 61 patients (90.0%, 95% CI 82-97). Eight patients had gestational hypertension (18.0%, 95% CI 4-20), while five patients (7.0%, 95% CI 1-14) had pre term labor. One patient developed eclampsia and one had a uterine rupture. One patient died due to post partum hemorrhage. The multi variable logistic regression model on the impact on the major maternal complications revealed none of the baseline factors to be statistically significant. Sickle cell disease patients have low mortality and pregnancy-related morbidity but high sickle cell disease-related morbidity. Prospective studies are needed to confirm these results.

Successful Management of Maternal Left Atrial Myxoma in Pregnancy.

A 29-year-old woman (gravida 3, para 2) presented at 28 weeks+2 days of gestation with a two-months history of dyspnea associated with orthopnea and occasional palpitations. On transthoracic echocardiography, she was diagnosed with a 3.2 × 2.7 cm left atrial myxoma. The patient underwent open surgical resection at 30 weeks of gestation. She had an uneventful postoperative recovery and was discharged on the ninth day. At 41 weeks of gestation, she gave birth by cesarean to a healthy baby of normal weight. Both the mother and the baby were discharged in stable condition.

Maternal, Perinatal, and Neonatal Outcomes of COVID-19 Severity in Pregnant Women: A Retrospective Study from Oman.

Objectives: To evaluate the effect of COVID-19 severity on maternal, perinatal, and neonatal outcomes in pregnant women infected with SARS-CoV-2. We also aimed to assess the effect of medical comorbidities on the severity of COVID-19. Methods: A retrospective cohort study was conducted on women who became infected with SARS-CoV-2 during pregnancy and delivered at Sultan Qaboos University Hospital, Oman, from 1 March 2020 to 31 December 2021. Results: A total of 118 pregnant women with COVID-19 and their 118 newborns were included in the study. Mean maternal age was 32.0 years, with 60.2% of women infected in the third trimester. The majority of the participants had mild symptoms. Eleven (9.3%) women had moderate infection needing inpatient care. Six (5.1%) with severe infection were admitted to intensive care unit. The chronic diseases among the participants were hypothyroidism, obesity, sickle cell disease, epilepsy, and diabetes. The mean gestational age at delivery was 37.0 weeks with 20.9% of women delivering by cesarean section out of whom 37.5% had moderate-to-severe COVID-19. The most common complications associated with COVID-19 severity were preterm labor (p =0.002), preeclampsia (p =0.002), and intrauterine fetal death (p =0.089). Of the total 118 newborns, 111 were singletons and six were twins. One fetus died and three singletons were lost to miscarriage. Placental histopathology conducted in 64 patients had no COVID-specific findings in most cases. Conclusions: Most pregnant women with COVID-19 infections had mild symptoms. The majority of women with moderate-to-severe infection were admitted for COVID-19 pneumonia. There was no direct effect of COVID-19 severity in neonatal outcomes or placental histopathology changes.

Histopathological Results and the Outcome of Women Who Underwent Postpartum Evacuation and Pelvic Ultrasound Scan.

Objectives: This study aimed to assess the proportion of women who underwent postpartum evacuation and were histopathologically confirmed to have retained products of conception (RPOC), compare the reliability of histopathology and ultrasound (US) in determining the presence of RPOC, and assess the maternal complications associated with postpartum evacuation. Methods: A retrospective cross-sectional study was conducted on all women who delivered and had postpartum evacuation at a tertiary teaching hospital in Oman over 11 years from May 2009 to May 2020. The participants were divided into two groups based on their histopathology results. McNemar test was used to compare the sonographic results with the histopathological findings. Results: A total of 151 women were included in this study. The diagnosis of RPOC was confirmed in histopathological reports of 64 (42.4%) women (group 1) but not in 87 (57.6%) women (group 2). There was no significant difference between the two groups in maternal characteristics. Parameters of clinical presentation including fever and abdominal pain were significantly different between the two groups (p =0.026 and p =0.028, respectively). Vaginal bleeding was not significantly different between the groups (p =0.255). Pelvic US detected RPOC in 135 (89.4%) women whereas the histopathology confirmed it in 64 (42.4%) women (p < 0.001). The sensitivity of US compared to histopathology in diagnosing RPOC was 98.4% (95% CI: 91.60-99.96) and the specificity was 17.2% (95% CI: 9.98-26.84). The overall diagnostic accuracy of US in detecting RPOC was 51.7%. Two (1.3%) women had hysterectomy as a result of the evacuation. Histopathology showed smooth muscle in 20 (13.2%) women. Significant bleeding during surgery occurred in 17 (11.3%) cases. Conclusions: Diagnosis of postpartum RPOC is challenging. Our results highlighted the complexity of diagnosing RPOC. Special training is needed for doctors to diagnose RPOC from transvaginal scans. A multicenter study in Oman with a larger sample size is recommended to confirm our findings.

Is Forced Coughing Effective in Reducing Pain During Cervical Biopsy?: A systematic review and meta-analysis.

This review aimed to compare the potential analgesic effect of forced coughing (FC) with that of local anaesthetics (LA) or placebo during cervical biopsy. A total of 5 electronic databases-Scopus, PubMed, Web of Science, Cochrane Library and Google Scholar-were systematically searched from inception till March 2021. Data were extracted from 6 randomised controlled trials and analysed. During cervical biopsy, the overall effect favoured LA over FC (mean difference [MD] = 1.06, 95% confidence interval [CI]: 0.58 to 1.54; P <0.0001). Compared to no pain management, pooled data were comparable between the two groups (MD = -1.2, 95% CI: -3.35 to 0.94; P = 0.27). Procedure duration was significantly longer in the LA group than in the FC group (MD = -1.94, 95% CI: -2.47 to -1.41; P <0.00001). FC and LA are both useful pain-lowering modalities during cervical biopsy, depending on the setting and their availability.

Incidence, Risk Factors, Maternal and Neonatal Outcomes of Peripartum Cardiomyopathy (PPCM) in Oman.

Background: Peripartum cardiomyopathy (PPCM) is an idiopathic life-threatening condition occurring towards the end of pregnancy or in the first few months following delivery that might affect the maternal and neonatal outcomes. Objectives: To assess the incidence and to evaluate the antenatal risk factors and the maternal and neonatal outcomes in Omani women diagnosed with PPCM. Methods: A retrospective cohort study was conducted at two tertiary institutions in Oman between the 1st of January 2010 to the 31st of December 2018. All cases fitting the standard definition of PPCM were included in the analysis. Patients with pre-existing dilated cardiomyopathy, chronic obstructive pulmonary disease and significant valvular heart disease have been excluded. Results: A total of 113,104 deliveries were screened during the study period. PPCM was confirmed in 116 cases with an incidence of 1.02 per 1000 deliveries. Independent predictors for the development of PPCM were age; especially women at the mid reproductive age (26-35 years), singleton pregnancy and gestational hypertension. In general, maternal outcomes were favorable, with full recovery of left ventricular ejection fraction in 56.0%, recurrence of 9.2%, and an overall mortality rate of 3.4%. The most common maternal complication was pulmonary edema (16.3%). The neonatal mortality rate was 4.3% and the preterm birth rate was 35.7%. Neonatal outcomes included 94.3% live births, out of which 64.3% were term with Apgar scores of more than 7 at five minutes in 91.5% of the neonates. Conclusion: Our study resulted in an overall incidence of PCCM in Oman of 1.02 in 1000 deliveries. Given the significance of maternal and neonatal complications, establishing a national PPCM database and local practice guidelines, and emphasizing their implementations in all regional hospitals, are fundamental for early recognition of the disease, timely referral, and application of therapy. Future studies, with a clearly defined control group, are highly recommended to appraise the significance of antenatal comorbidities in PPCM compared to non-PPCM cases.

COVID-19 and Pregnancy: A narrative review of maternal and perinatal outcomes.

As of August 11, 2021, approximately 206 million people worldwide had been infected with SARS-CoV-2. However, limited data are available regarding the effects of COVID-19 infection on pregnancy and maternal and perinatal outcomes. This review aimed to resolve this gap in literature. The MEDLINE®, SCOPUS and Cumulative Index to Nursing and Allied Health Literature databases were searched to identify relevant English-language articles published between January 2020 and February 2021. A total of 17 articles describing the outcomes of 762 pregnancies were identified. There were 613 babies born, including 16 sets of twins. Within the cases studied, 12 (1.6%) maternal deaths and eight (1.3%) stillbirths were reported. A small proportion of mothers (3.9%) required admission to the intensive care unit, usually due to associated comorbidities. Rates of caesarean and preterm delivery ranged from 27-100% and 4-50%, respectively. Further research is necessary to determine the effect of COVID-19 infection on early pregnancy.

Uptake of prenatal genetic diagnosis and termination of pregnancy by Omani Muslim families at risk of genetic disorders: experience over a 9-year period.

Studies on the acceptance of prenatal diagnosis and termination of pregnancy for single gene disorders within Islamic societies in the Middle East are limited. A few have examined the attitudes toward pregnancy termination for fetal indications, but a dearth of published data exists on actual behavior and uptake. This study reports on all prenatal diagnosis requests for single gene disorders, from the Sultanate of Oman, over 9 years. A retrospective study was conducted during which the medical records of all women who performed prenatal diagnoses for single gene disorders were reviewed. A total of 148 invasive procedures were performed for 114 families. The total number of yearly requests for prenatal diagnosis increased exponentially from three in 2012 to 21 in 2020. Sixty-four different diagnoses were tested for with the majority being autosomal recessive in nature. Seventy-one percent (28/39) of cases where an affected pregnancy was identified were terminated. Fifty-two of the 114 women (45.6%) repeated prenatal diagnosis in a future pregnancy. Seventy-two couples (63%) were consanguineous parents related as second cousins or closer. The majority of tests performed were for couples from Muscat (27%), Albatinah (27%), and Alsharqiya (20.3%) governorates in Oman. The findings of this study provide evidence that prenatal diagnosis is an acceptable reproductive option to prevent the occurrence of genetic disorders that meet termination eligibility criteria as outlined by the Islamic Jurisprudence (Fiqh) Council Fatwa, among Omani Muslim couples.