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Nonimmune Hydrops Fetalis
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Chelsea DeBolt, Katherine Connolly, Mary E. Norton, Joanne Stone
Fetomaternal hemorrhage may be significant enough to induce NIH and should be suspected after trauma or placental abruption. Even without a bleeding history, in one review NIH was the presenting sign of fetomaternal hemorrhage greater than 50 mL in 7.5% of cases [33]. A Kleihauer–Betke test or flow cytometry may be used to aid in diagnosis and to assess the magnitude of the transfusion. Management with delivery or intrauterine transfusion should be considered, depending on gestational age and results of fetal testing. If left untreated, fetuses subjected to severe anemia may develop cardiac failure, hydrops, hypovolemic shock, fetal or neonatal death, neurologic damage, cerebral palsy, or persistent pulmonary hypertension [33].
Obstetrics: Answers
Published in Euan Kevelighan, Jeremy Gasson, Makiya Ashraf, Get Through MRCOG Part 2: Short Answer Questions, 2020
Euan Kevelighan, Jeremy Gasson, Makiya Ashraf
Mild – follow-up with ultrasound.Severe – intrauterine transfusion.
Blood transfusion and rhesus disease
Published in Michael J. O’Dowd, The History of Medications for Women, 2020
Flint Porter and colleagues (1997) from the Department of Obstetrics and Gynecology, University of Utah School of Medicine, reported on the use of adjuvant therapy to suppress maternal Rh antibody production: ‘although intra-uterine fetal transfusion has improved dramatically perinatal outcome ... Intravenous immune globulin (IVIG) administration has been increasingly used to successfully treat a variety of immune-mediated diseases’. The paper reported a favorable outcome with IVIG treatment of a severe case of rhesus disease: ‘IVIG may have an adjunctive role in the treatment of severe Rh iso-immunisation’. The technique may be used in concert with intrauterine transfusion. Combined modes of therapy could help to conserve the affected pregnancy until the third trimester, and increase the prospects for fetal survival.
Trisomy 21 and hydrops fetalis: parvovirus B19 or transient abnormal myelopoiesis?
Published in Journal of Obstetrics and Gynaecology, 2019
Lee Na Tan, Ka Wang Cheung, Mark David Kilby
A 25-year-old, multiparous woman was referred at 13 weeks and 6 days of gestation with an enlarged nuchal translucency (4.8 millimetres). First trimester anatomy scan demonstrated no anomaly and prenatal chromosomal testing was declined. A fetal echocardiography at 20 weeks was normal. At 24 weeks, NIFH was diagnosed with an echogenic bowel, ventriculomegaly (ventricular height of atrium measuring 10 millimetres) and cardiomegaly. The middle cerebral artery peak systolic velocimetry (MCA PSV) was elevated at 49 – 50 centimetres/second (cm/s) (>1.5 multiples of median (MoM)) and there was a high clinical suspicion of fetal anaemia. Intrauterine transfusion (IUT) was performed by an intrahepatic vein puncture, with foetal blood sampling demonstrating a haemoglobin of 55 gram/litre (g/L). An uneventful IUT with packed cells to a haemoglobin of 100 g/L was done.
A Rare Hb H Hydrops Fetalis Syndrome Caused by the – –SEA Deletion in Combination with the Rare Hb Hirosaki Mutation in a Chinese Patient
Published in Hemoglobin, 2018
Qiang Li, Yihong Li, Mei Zhong, Victor Wei Zhang, Wangjie Jin, Shaoyuan Li, Liyan Li
However, the Hb level of the cord blood sample at 23 weeks’ gestation was 5.8 g/dL with 29.9% Hb Bart’s and 60.5% Hb F, indicating the severe anemia of the fetus. Due to the unknown causes for severe anemia, an intrauterine transfusion was given to the fetus. After birth, the infant still suffered from severe anemia and was transfusion-dependent. Some of the hematological indices at 2 months of age were as follows: Hb A 84.7%, Hb A2 1.7%, Hb F 9.1% and Hb Bart’s 4.5%. The routine α-thal examination showed that the neonate was heterozygous for the – –SEA deletion. Whole exome sequencing was performed on the fetus in search for other potential disease-causing variants. Consequently, we identified the rare Hb Hirosaki mutation. This mutation results in a Phe→Leu substitution at position 43 of the α2-globin gene, expressing an unstable protein. This mutation was inherited from her mother and was subsequently verified using Sanger sequencing. Our results indicated that the severe fetal anemia with hydrops fetalis was caused by the – –SEA deletion in combination with the Hb Hirosaki mutation.
Fetal Hemoglobin H Hydrops Fetalis: Another Three Case Reports
Published in Hemoglobin, 2023
Hai-Shen Tang, Yi Xiong, Dong-Zhi Li
In conclusion, this report highlights the importance of watchful observation of pregnant women with fetuses of nondeletional Hb H disease or homozygotes for Hb CS/CS. Due to a relatively high frequency of Hb CS and QS in South China, a workup for managing pregnancies at risk for nondeletional thalassemias is necessary. Ultrasound may play an important role in early detection of fetal hydropic changes which is occasionally associated with nondeletional thalassemias. If fetal hydrops was detected, intrauterine intervention might be a treatment choice. Successful outcome after intrauterine transfusion has been reported in such patients [7].