Hemoglobin Bart's hydrops fetalis, characterized by a deletion of all four a-globin genes is the most severe and lethal form of Thalassemia disease. Mortality rate usually ranges from 60-100% of cases. Given the poor overall prognosis, most countries resort to pregnancy termination or expectant management as the only options to offer affected pregnancies.
This paper presents a case of the successful management of a primigravid, diagnosed with hydrops fetalis at 29 4/7 weeks age of gestation. She delivered successfully to a live, preterm, baby boy who was later found out to have hydrops fetalis due to Hemoglobin Bart's disease, and currently, continues to thrive past eight months of age.
This report aims to improve the clinicians' knowledge regarding the work up and management of pregnant patients diagnosed with hydrops fetalis, and increase the clinician's awareness on the epidemiology, importance of targeted screening, and diagnosis of Alpha-Thalassemia in Filipino patients.

Human ; Female ; Adult ; Alpha-thalassemia ; Hydrops Fetalis ; Hemoglobin Bart's ; Hemoglobin, Sickle ; Anemia, Sickle Cell ; Prognosis

Introduction: Preeclampsia remains to be a major cause of both fetal and maternal morbidity and mortality, particularly in severe forms leading to preterm birth. There is a lack of consensus, however, on the preferred screening test for early diagnosis with the aim of reducing the prevalence and morbidity of the disease. Objective: To compare the performance of the comprehensive first trimester screening using maternal characteristics, ultrasonographic findings and serum biochemical markers, with the NICE and ACOG guidelines in predicting the development of preeclampsia. The study also aims to determine the compliance rate of clinicians in giving aspirin prophylaxis using the different screening tests. Methodology: This is a retrospective, analytical, cross sectional study of all pregnant patients between 11 to 13 6/7 weeks referred for comprehensive first trimester screening for preeclampsia from January 2014 to January 2018. Maternal factors were assessed to determine the risk of preeclampsia using NICE guidelines, ACOG guidelines and comprehensive first trimester screening. The compliance on aspirin administration for high-risk patients was also determined. The outcome measure was diagnosis of preeclampsia and the detection rate (DR) of the three screening tests were compared. Results: A total of 202 women were included in the analysis where 24 (11.9%), 11 (5.4%) and 13 (6.4%) developed preeclampsia, early-onset preeclampsia (EO-PE) and late-onset preeclampsia (LO-PE) respectively. The NICE and ACOG guidelines were able to detect preeclampsia with an accuracy of 76.73% (Sn 75%, Sp 77% PPV 30.5%) and 43.07% (Sn 83.3%, Sp 37.6% PPV 15.3%) respectively. The comprehensive first trimester screening was able to detect preeclampsia with an accuracy of 89.60% (Sn 83.3%, Sp 90.5% PPV 54.1%). EO-PE and LO-PE were detected with an accuracy of up to 97.2% using the comprehensive screening (Sn 90.9%, Sp 97.9% PPV 71.4%), compared with the NICE guideline (up to 74.26%, Sn 81.8%, Sp 73.8% PPV 15.3%) and the ACOG guideline (up to 39.6%, Sn 90.9%, Sp 36.6, PPV 7.63%). Compliance with the NICE and ACOG recommendation on aspirin administration was only 42.37% and 33.33%, respectively, and this increased to up to 62% when comprehensive first trimester screening was used. Conclusion This study confirmed that the performance of screening for PE, and therefore appropriate selection of the patients that would benefit from prophylactic use of aspirin and closer surveillance, is by far superior if the comprehensive first trimester screening is used than the method advocated by ACOG and NICE.
Pre-Eclampsia ; Pregnancy-Associated Plasma Protein-A