1/38. prenatal diagnosis and management of nonimmune hydrops fetalis.We examined the incidence, aetiological factors and outcome in 40 cases of nonimmune hydrops fetalis (NIH) and suggest a rational approach to management. The incidence of NIH was 1 in 830 deliveries during the last 10-year period. In spite of extensive antenatal and postnatal investigation no cause could be established in 14 (35%) cases. A probable aetiological factor was found in 65% of cases. These included viral infection (7), cardiovascular (6), twin-to-twin transfusion (3), chromosomal abnormality (3), other malformation syndromes (4), renal dysplasia (1), laryngeal atresia (1) and severe fetomaternal haemorrhage (1). Five of the 40 fetuses survived, 2 treated antenatally for tachyarrhythmia, 2 had spontaneous resolution and the fifth fetus had repeated intrauterine transfusions because of human parvovirus B19-induced anaemia. After diagnosis of nonimmune hydrops fetalis, early referral to a tertiary centre is to be encouraged for investigation and provision of intensive perinatal care. Investigation allows parents to be counselled appropriately that the mortality is no longer 100% and a steadily growing number may be amenable to some form of fetal therapy.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/38. In utero erythrocyte transfusion for fetal xerocytosis associated with severe anemia and non-immune hydrops fetalis.Hereditary xerocytosis is a rare hemolytic anemia occurring secondary to a defect in cell membrane potassium flux. We report a case of severe fetal anemia and non-immune hydrops secondary to hereditary xerocytosis that was managed successfully with in utero erythrocyte and albumin transfusion.- - - - - - - - - - ranking = 0.84844594724898keywords = hemolytic (Clic here for more details about this article) |
3/38. Hb H hydrops foetalis syndrome: a case report and review of literature.Haemoglobin H (Hb H) disease is caused by deletion or inactivation of three alpha-globin genes, leaving only one intact and active alpha-globin gene. People with Hb H disease usually have moderate anaemia, but are generally thought to be asymptomatic. Some Hb H disease patients require transfusions, and there are reports of fetuses with Hb H disease who have severe anaemia in utero resulting in fatal hydrops foetalis syndrome. We now report a case of Hb H hydrops foetalis syndrome, caused by the inheritance of a hitherto novel alpha-globin gene point mutation (codon 35 TCC-->CCC or serine-->proline) and an alpha-thalassaemia deletion of the Filipino type removing all zeta-alpha-globin genes on the other chromosome 16. The infant was delivered prematurely because of pericardial effusion and fetal distress, and was found to have severe anaemia and congenital anomalies. A review of the relevant literature on this syndrome is presented, and serves to underscore the phenotypic variations of Hb H disease and the need for surveillance for this condition among newborns and genetic counselling in communities with a high proportion of at-risk populations.- - - - - - - - - - ranking = 3keywords = anaemia (Clic here for more details about this article) |
4/38. Hb H hydrops fetalis syndrome associated with the interaction of two common determinants of alpha thalassaemia (--MED/(alpha)TSaudi(alpha)).To date, more than 35 single or oligonucleotide mutations of the alpha genes that cause alpha thalassaemia have been described. Their interactions give rise to widely variable clinical manifestations, from a mild hypochromic, microcytic anaemia to a lethal intrauterine anaemia associated with hydrops fetalis. Understanding the molecular genetics enables accurate genotyping, genetic counselling and prenatal testing for the most severe forms of alpha thalassaemia. Here we show for the first time that the interaction between two relatively common forms of alpha thalassaemia (--MED/(alpha)TSaudi(alpha)) may be associated with a clinically severe form of alpha thalassaemia, Hb H hydrops fetalis.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
5/38. Successful perinatal management of hydrops fetalis due to hemolytic disease associated with D-- maternal phenotype.We report the successful management of a case of hemolytic disease and hydrops fetalis secondary to anti Rh 17 antibodies in a woman with the rare D-- phenotype. We discuss the efficacy of intravenous immunoglobulins in treating hemolytic disease of the newborn infant.- - - - - - - - - - ranking = 5.0906756834939keywords = hemolytic (Clic here for more details about this article) |
6/38. Fatal hydrops fetalis caused by anti-D in a mother with partial D.BACKGROUND: Rare cases of hemolytic disease of the newborn occur in women with genetic variants of the D antigen. A partial D variant might be suspected when typing for the D antigen shows weaker-than-normal reactions (weak D). Historically, patients with a weak D phenotype have not been considered candidates for Rh immune globulin prophylaxis. CASE: A gravida 2, para 1 woman with A-positive blood type, delivered an infant who died from severe hemolytic disease of the newborn 6 days after birth. Anti-D, undetectable at the first prenatal visit, was identified (titer 1:64) at delivery. The mother's red cells were partial D(VI) phenotype. CONCLUSION: Although severe hemolytic disease in patients with partial D is rare, this neonatal death illustrates the need for a change in management of women with weak D.- - - - - - - - - - ranking = 2.5453378417469keywords = hemolytic (Clic here for more details about this article) |
7/38. middle cerebral artery Doppler velocimetric assessment in two cases of hydrops fetalis without fetal anaemia.OBJECTIVES: Raised middle cerebral artery (MCA) Doppler velocity has been shown to be highly predictive of moderate to severe fetal anaemia. We present two false-positive cases of raised MCA Doppler velocity in non-immune hydropic fetuses. methods: In both cases, routine investigations for fetal hydrops, as well as detailed ultrasound scanning and MCA peak-systolic velocity Doppler assessment (maximum velocity taken at zero degrees to the vessel), were performed. fetal blood sampling was carried out at the same visit. RESULTS: MCA peak-systolic velocity values greater than 1.50 MoM for gestation were found in both cases. However, both fetuses had normal haemoglobin values and haematocrits. Both fetuses died in utero soon after diagnosis. CONCLUSIONS: A raised MCA velocity in a hydropic fetus may not always be due to fetal anaemia, rather it may indicate a fetus in poor condition perhaps due to cardiovascular decompensation and redistribution of blood to the fetal brain.- - - - - - - - - - ranking = 6keywords = anaemia (Clic here for more details about this article) |
8/38. hydrops fetalis associated with homozygosity for hemoglobin Taybe (alpha 38/39 THR deletion) in newborn triplets.Hemoglobin Taybe is an unstable alpha-chain hemoglobin variant caused by a deletion of a threonine residue at codon 38 or 39 of the alpha-1 globin chain. We describe preterm infant triplets born with hydrops fetalis and anemia who were found by dna analysis to be homozygous for hemoglobin Taybe. All three infants developed intrauterine hemolytic anemia, which subsequently led to hydrops fetalis. To the best of our knowledge, this is the first description of hydrops fetalis associated with this hemoglobinopathy. We suggest that hemoglobin Taybe be considered in the differential diagnosis of hydrops fetalis and that known affected fetuses be carefully followed both antenatally and postnatally.- - - - - - - - - - ranking = 0.84844594724898keywords = hemolytic (Clic here for more details about this article) |
9/38. Maternal ABO-mismatched blood for intrauterine transfusion of severe hemolytic disease of the newborn due to anti-Rh17.BACKGROUND: Clinically significant antibodies to high-incident antigens present a challenge in hemolytic disease of the newborn. Antigen-negative blood may be difficult to obtain for intrauterine transfusion (IUT). In these instances, maternal blood is de facto compatible regardless of an ABO mismatch. CASE REPORT: A group B/D-- woman with a history of hemolytic disease of the newborn due to anti-Rh17 (titer 256) presented to the obstetrical clinic at 12 weeks gestation for management of her third pregnancy. She consented to donate blood for possible IUT. STUDY DESIGN AND methods: Washed maternal packed cells were suspended in saline to 75 percent Hct and irradiated before transfusion. The fetus was transfused via the intrahepatic vein. RESULTS: Ultrasound examination at 19 weeks indicated a hydropic fetus. The fetal blood group was O Rh , direct antiglobulin test 4 , and hemoglobin 22 g per L. A total of 368 mL of maternal blood was transfused during seven procedures. Labor was induced at 38 weeks, and a 2560-g male infant was delivered by Caesarian-section due to fetal distress. The infant grouped as B Rh , direct antiglobulin test negative. No group O red blood cells were detected. The hemoglobin level was 143 g per L rising to 209 g per L at discharge 3 days later. The indirect bilirubin was 55 micromol/L and remained stable during the hospital stay. phototherapy was discontinued after 1 day, and the infant was discharged without an exchange or top-up transfusion. CONCLUSIONS: Maternal ABO-mismatched blood is an alternate source for IUT in instances when antigen-compatible allogenic blood is unavailable.- - - - - - - - - - ranking = 5.0906756834939keywords = hemolytic (Clic here for more details about this article) |
10/38. Nonimmune hydrops fetalis due to congenital xerocytosis.Hereditary xerocytosis is a rare hemolytic anemia in which erythrocytes are dehydrated due to a loss of potassium and water through their cell wall membrane. In adults, this condition leads to a mild-to-moderate hemolysis. We report a case of hydrops fetalis secondary to hereditary xerocytosis. Management with intrauterine erythrocyte and albumin transfusions resulted in a favorable postnatal course.- - - - - - - - - - ranking = 0.84844594724898keywords = hemolytic (Clic here for more details about this article) |
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