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Erythroblastosis Fetalis [keywords]
- Anti-D administration after spontaneous miscarriage for preventing Rhesus alloimmunisation. [Journal Article, Research Support, Non-U.S. Gov't]
- Cochrane Database Syst Rev 2013.:CD009617.
During pregnancy, a Rhesus-negative (Rh-negative) woman may develop antibodies if her fetus is Rh-positive, which can cause fetal morbidity or mortality in following pregnancies, if untreated.To assess the effects of administering anti-D immunoglobulin (Ig) after spontaneous miscarriage in a Rh-negative woman, with no anti-D antibodies.We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 December 2012).Randomised controlled trials (RCT) in Rh-negative women without antibodies who were given anti-D Ig following spontaneous miscarriage compared with no treatment or placebo treatment following spontaneous miscarriage as control.Two review authors independently assessed trials for inclusion and trial quality. Two review authors extracted data and checked it for accuracy.We included one RCT, involving 48 women who had a miscarriage between eight to 24 weeks of gestation. Of the 19 women in the treatment group, 14 had therapeutic dilatation & curettage (D&C) and five had spontaneous miscarriage; of the 29 women in the control group, 25 had therapeutic D&C and four had spontaneous miscarriage. The treatment group received 300 µg anti-D Ig intramuscular injection and were compared with a control group who received 1 cc homogenous gamma globulin placebo.This review's primary outcomes (development of a positive Kleihauer Betke test (a test that detects fetal cells in the maternal blood; and development of RhD alloimmunisation in a subsequent pregnancy) were not reported in the included study.Similarly, none of the review's secondary outcomes were reported in the included study: the need for increased surveillance for suspected fetal blood sampling and fetal transfusions in subsequent pregnancies, neonatal morbidity such as neonatal anaemia, jaundice, bilirubin encephalopathy, erythroblastosis, prematurity, hypoglycaemia (low blood sugar) in subsequent pregnancies, maternal adverse events of anti-D administration including anaphylactic reaction and blood-borne infections.The included study did report subsequent Rh-positive pregnancies in three women in the treatment group and six women in the control group. However, due to the small sample size, the study failed to show any difference in maternal sensitisation or development of Rh alloimmunisation in the subsequent pregnancies.There are insufficient data available to evaluate the practice of anti-D administration in an unsensitised Rh-negative mother after spontaneous miscarriage. Thus, until high-quality evidence becomes available, the practice of anti-D Immunoglobulin prophylaxis after spontaneous miscarriage for preventing Rh alloimmunisation cannot be generalised and should be based on the standard practice guidelines of each country.
- Xerocytosis is caused by mutations that alter the kinetics of the mechanosensitive channel PIEZO1. [Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S.]
- Proc Natl Acad Sci U S A 2013 Mar 19; 110(12):E1162-8.
Familial xerocytosis (HX) in humans is an autosomal disease that causes dehydration of red blood cells resulting in hemolytic anemia which has been traced to two individual mutations in the mechanosensitive ion channel, PIEZO1. Each mutation alters channel kinetics in ways that can explain the clinical presentation. Both mutations slowed inactivation and introduced a pronounced latency for activation. A conservative substitution of lysine for arginine (R2456K) eliminated inactivation and also slowed deactivation, indicating that this mutant's loss of charge is not responsible for HX. Fitting the current vs. pressure data to Boltzmann distributions showed that the half-activation pressure, P1/2, for M2225R was similar to that of WT, whereas mutations at position 2456 were left shifted. The absolute stress sensitivity was calibrated by cotransfection and comparison with MscL, a well-characterized mechanosensitive channel from bacteria that is driven by bilayer tension. The slope sensitivity of WT and mutant human PIEZO1 (hPIEZO1) was similar to that of MscL implying that the in-plane area increased markedly, by ∼6-20 nm(2) during opening. In addition to the behavior of individual channels, groups of hPIEZO1 channels could undergo simultaneous changes in kinetics including a loss of inactivation and a long (∼200 ms), silent latency for activation. These observations suggest that hPIEZO1 exists in spatial domains whose global properties can modify channel gating. The mutations that create HX affect cation fluxes in two ways: slow inactivation increases the cation flux, and the latency decreases it. These data provide a direct link between pathology and mechanosensitive channel dysfunction in nonsensory cells.
- Anti-S antibodies: an unusual cause of haemolytic disease of the fetus and newborn (HDFN). [Case Reports, Journal Article]
- BMJ Case Rep 2013.
We report a case of haemolytic disease of the fetus and newborn due to anti-S antibodies. Baby G was born by emergency caesarean section at 35 weeks due to reduced fetal movement. Prior to delivery, antenatal screening revealed the mother's blood group was AB rhesus positive with anti-S antibody titres. The baby was pale but non-hydropic at birth with hepatosplenomegaly. Haemoglobin at birth was 5.23 g/dl and serum bilirubin 138 µmol/l. The baby required phototherapy, γ-globulin infusion and exchange transfusion with post-transfusion complications.
- [Hydrops foetalis caused by a mediastinal teratoma]. [Case Reports, English Abstract, Journal Article]
- Ugeskr Laeger 2012 Nov 19; 174(47):2944-5.
Hydrops foetalis (HF) is defined as an accumulation of fluid in at least two compartments of the body, e.g. the subcutis, the pleural cavities, the pericardial cavity and the abdomen. In present time, non-immune causes, including tumours, predominate. We present a case of severe HF, caused by a mass which was clinically interpreted as a hernia of the diaphragm at gestational week 19 + 6. An abortion was granted and performed. Autopsy revealed an immature teratoma in the mediastinum compressing the heart and airway. The prognosis and treatment of mediastinal teratoma and HF is discussed.
- Fetal lung lesions: can we start to breathe easier? [Journal Article]
- Am J Obstet Gynecol 2013 Feb; 208(2):151.e1-7.
The purpose of this study was to develop a simple and accurate approach for risk stratification of fetal lung lesions that are associated with respiratory compromise at birth.We conducted a retrospective review of 64 prenatal lung lesions that were managed at a single fetal care referral center (2001-2011). Sonographic data were analyzed and correlated with perinatal outcomes.Hydrops occurred in only 4 cases (6.3%). Among fetuses without hydrops, the congenital pulmonary airway malformation volume ratio (CVR) was the only variable that was associated significantly with respiratory compromise and the need for lung resection at birth (P < .01). Based on a maximum CVR >1.0, the sensitivity, specificity, positive predictive value, and negative predictive value for respiratory morbidity were 90%, 93%, 75%, and 98%, respectively.Nonhydropic fetuses with a maximum CVR >1.0 are a subgroup of patients who are at increased risk for respiratory morbidity and the need for surgical intervention. These patients should be delivered at a tertiary care center with pediatric surgery expertise to ensure optimal clinical outcomes.
- Mediastinal masses: a case of fetal teratoma and literature review. [Case Reports, Journal Article, Review]
- Clin Exp Obstet Gynecol 2012; 39(3):384-7.
Fetal mediastinal masses are rare congenital formations that could complicate pregnancy. They are usually discovered as space occupying lesions in the fetal chest during routine ultrasound scan. The most important prognostic factors of mediastinal masses are mass location, compressing effect causing pulmonary hypoplasia and/or heart failure, and the presence or absence of hydrops. We report a case of fetal mediastinal teratoma and a review of the literature. A 32-year-old woman carrying a fetus with hydrops due to a mediastinal mass underwent cesarean section at 32 1/7 weeks' gestation. A well encapsulated tumor was excised by surgery at one day of life. The baby is now eight months old without respiratory difficulty. To our knowledge, this is the fourth case report of a mediastinal teratoma associated with nonimmune hydrops in a fetus that survived the neonatal period. Fetal mediastinal teratoma requires close surveillance and multidisciplinary management by obstetricians, neonatologists, and pediatric surgeons.
- Are alanine, cysteine, glycine and valine amino acids the cause of non-immune hydrops fetalis? [Journal Article]
- Clin Exp Obstet Gynecol 2012; 39(3):341-2.
Our objective was to measure amniotic fluid amino acid concentrations in pregnant women diagnosed as having fetuses with non immune hydrops fetalis in the second trimester of pregnancy. Twenty-three pregnant women who had fetuses with non immune hydrops fetalis detected by ultrasonography (non immune hydrops fetalis group) in the second trimester and 19 women who had healthy fetuses (control group) were enrolled in the study. Amniotic fluid was obtained by amniocentesis. The chromosomal analysis of the study and control groups was normal. Levels of free amino acids were measured in amniotic fluid samples using EZ: fast kits (EZ: fast GC/FID free (physiological) amino acid kit) by gas chromatography (Focus GC Al 3000 Thermo Finnigan analyzer). The mean levels of alanine, cysteine, glycine and valine amino acids were found to be significantly higher in fetuses with non immune hydrops fetalis than in the control group (p<0.05). The detection of significantly higher amino acid concentrations in the amniotic fluid of fetuses with a non immune hydrops fetalis in healthy fetuses suggests loss of amino acids from the fetus through capillary permeability or/and the lymphatic system through the amniotic fluid may contribute to the etiology of non-immune hydrops fetalis.
- Management of neonates with hyperbilirubinemia: improving timeliness of care using a clinical pathway. [Comparative Study, Journal Article]
- Pediatrics 2012 Dec; 130(6):e1688-94.
Neonatal hyperbilirubinemia is a common reason for neonates to present to the emergency department (ED). Although clinical practice guidelines provide recommendations for evaluation and therapy, few studies have evaluated ways to apply them effectively in the ED setting. The primary objective of this study was to compare time to phototherapy in neonates presenting to the ED with jaundice before and after implementation of a nursing-initiated clinical pathway. Secondary outcomes included time to bilirubin result and ED length of stay in neonates.We performed a retrospective historical control study comparing neonates presenting to the ED with jaundice during 9-month periods before and after initiation of the pathway. Charts were abstracted for times of assessment and treatment and final disposition.Three hundred neonates were included in this study: 149 before and 151 after pathway implementation. Median time to phototherapy (historical control: 128 minutes vs postintervention group: 52 minutes; P < .001), median time to bilirubin result (157 vs 99; P < .001), and median ED length of stay (268 minutes vs 195 minutes; P < .001) were shorter for neonates treated after the implementation of the clinical pathway. No complications were reported during the study period.After implementation of a clinical pathway for the management of neonates with jaundice in the ED, we observed a reduction in time to phototherapy, time to bilirubin measurement, and overall length of stay.