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Purpuric phototherapy-induced eruption in transfused neonates: relation to transient porphyrinemia.

OBJECTIVE: Blue light phototherapy is commonly administered to neonates as treatment of indirect hyperbilirubinemia, often in conjunction with blood transfusions to treat hemolytic anemia. We observed a distinctive cutaneous complication of phototherapy in six neonates with hyperbilirubinemia. METHODOLOGY: We studied the clinical and histologic characteristics of the eruption, as well as the porphyrin levels in affected neonates. Five of the patients had erythroblastosis fetalis; the other had profound anemia from twin-twin transfusion. All of the neonates developed purpuric patches at sites of maximal exposure to the phototherapy lights, with dramatic sparing at shielded sites within 24 hours after initiation of the phototherapy. On discontinuation of phototherapy, all eruptions cleared within 1 week. Examination of skin biopsy sections showed purpura without significant inflammation or keratinocyte necrosis. Plasma porphyrins (copro- and proto-) were elevated in the two patients in which they were assessed. CONCLUSIONS: The distribution of the eruption in areas exposed to light and presence of circulating porphyrins suggest that porphyrinemia may underlie the light-induced purpuric eruption. Additional studies will be required to determine definitively the mechanisms of both the purpuric phototherapy-induced eruption and the development of increased blood porphyrin levels in these transfused neonates.

Anemia↗

Racial difference in incidence of ABO hemolytic disease.

In this review of 7,464 consecutive infants born at North Carolina Memorial Hospital, hemolytic disease from ABO incompatibility was found to be two to three times as common in black infants as in white infants. The statistical significance of the difference remained high as more restrictive criteria for ABO hemolytic disease were applied. ABO disease, serious enough to cause an indirect serum bilirubin of 15 mg/100ml or higher, had an incidence in black newborns as great as the incidence of Rh hemolytic disease in whites. In contrast, the general prevalence and severity of hyperbilirubinemia was not found to be higher in black newborns than in white infants. The difference cannot be attributed to differences in the prevalence of ABO blood groups between the two races. Policies of early discharge of newborns could be affected by the finding that ABO erythroblastosis is two to three times as common in black infants as in white infants.

ABO Blood-Group System↗

Amniotic fluid bilirubin as a prognostic indicator in rhesus isoimmunization.

The bilirubin concentration in 654 amniotic fluids obtained by amniocentesis of 227 consecutive rhesus isoimmunized pregnancies is determined by the chloroform extraction method. The material is divided in 3 classes reflecting the severity of erythroblastosis. It is found, by means of graphical analysis that corresponding fractiles of the amniotic fluid concentrations of bilirubin in different gestational weeks imply an almost uniform prognosis. In terms of logarithm the various bilirubin fractiles seem to move parallel to and linear with gestational time. A prediction diagram is constructed, making it possible to estimate fetal prognosis from the concentration of amniotic fluid bilirubin.

Amniotic Fluid↗

Intra-uterine transfusion in the management of pregnant women with severe rhesus isoimmunization. I. Indications, technique, and complications.

Intra-uterine transfusion may be a life-saving procedure in severe cases of rhesus isoimmunization. Yet it may be technically difficult and poses a real hazard to the fetus. This material consists of 51 intra-uterine transfusions performed in 34 severe rhesus (anti-D) isoimmunized pregnant women. The selection of patients was based upon an evaluation of the severity of fetal erythroblastosis (past obstetric history, serological and amniotic fluid examinations). The major criterion for selection was the concentration of amniotic fluid bilirubin. The technique employed was based upon a fluoroscopic television control system. The most serious complications were blood-stained amniotic samples in 15% of the amniocenteses and vaginal leaking of amniotic fluid and preterm labour after the performance of the intra-uterine transfusions in 33%.

Amniocentesis↗

Alloimmunization during pregnancy treated with high dose intravenous immunoglobulin. Effects on fetal hemoglobin concentration and anti-D concentrations in the mother and fetus.

BACKGROUND: High dose intravenous immunoglobulin has been reported to be advantageous in the treatment of alloimmunization during pregnancy. The mode of action is unknown. METHOD: High dose intravenous immunoglobulin was used as the sole prenatal treatment in six severely rhesus(D) sensitized pregnant women. Maternal and fetal anti-D concentrations as well as fetal hemoglobin concentrations were studied. Seven pregnancies in rhesus(D) sensitized women served as controls. They received no treatment because they had milder forms of erythroblastosis fetalis or, in one case, a rhesus(D) negative fetus. RESULT: No obvious inhibitory effect of the treatment on maternal anti-D production and transplacental anti-D passage to the fetus was found. The fetal hemoglobin concentrations remained stable at about 80 g/L (hematocrit 27%) in five of six treated patients while there was a significant decrease in the control group. CONCLUSION: High dose intravenous immunoglobulin treatment seems to act mainly on fetal red cell destruction rate, possibly by blocking Fc receptor mediated macrophage phagocytosis. We claim that the treatment can successfully be used to prevent further deterioration of fetal anemia in rhesus(D) immunizations if started before severe fetal anemia (hemoglobin concentration < 70 g/L, hematocrit < 23%) and imminent hydrops fetalis arises.

Dose-Response Relationship, Drug↗

Perivascular demyelinization of cochlear neurons in two neonates.

Localized perivascular demyelinization of the peripheral processes of cochlear neurons was observed in surface preparations of the osseous spiral lamina in two neonates, one a premature infant with erythroblastosis fetalis, the other a full-term infant with cyanotic congenital heart disease. In both cases the cause of the demyelinization was unknown. Both infants had severe edema as a result of congestive heart failure and anoxia. The serum bilirubin level in the erythroblastotic child appeared too low to have caused injury to the nerve fibers. The demyelinization was most striking in the lower half of the basal turn. It was observed only in the immediate vicinity of meandering stretches of capillaries as they crossed the nerve fibers. In the premature infant there was hair cell loss throughout the cochlea; in the other there was no hair cell loss, but a malformed labyrinth of the Mondini type.

Cochlear Nerve↗

Electron microscopic identification of parvovirus virions in erythroid-line cells in fatal hydrops fetalis.

Intrauterine infection with parvovirus B19 may lead to fatal hydrops fetalis. Intranuclear particles, consistent with parvovirus virions, within erythroid cells were readily identified on transmission electron microscopy of formalin-preserved material obtained at necropsy from a neonate and two fetuses in whom clinical and light microscopic criteria for parvovirus B19 infection were met. No such particles were seen in similar material from a neonate and two fetuses with erythroblastosis fetalis due to alpha-thalassemia, maternofetal Rh incompatibility, and an erythrocyte membrane protein defect. When other means of investigation are impracticable transmission electron microscopy is widely available and easily performed and may be of value in establishing a diagnosis of parvovirus B19 infection.

Adult↗

Triplet pregnancy and Rhesus isoimmunization.

A case of Rh isoimmunization complicating triplet pregnancy is reported and the rarity of the combination is noted. The management of Rh immunized pregnancy is review with particular reference to problems associated with plural pregnancy. The findings confirm earlier reports that fetal or placental influences as well as antibody titres determine the severity of erythroblastosis.

Adult↗

[Irregular blood group antibodies during pregnancy: screening is mandatory].

During pregnancy irregular blood group antibodies, originating either from earlier pregnancies or from blood transfusions, may severely jeopardize both mother and child. Three patients are described with pregnancy-associated blood group incompatibility. In one case of Kell antagonism a previous child had reportedly died of cot death, but in retrospect it had most probably suffered from erythroblastosis fetalis as a result of anti Kell antibodies. In the second case, a twin pregnancy, the diagnosis of neonatal haemolytic anaemia on the basis of blood group incompatibility with a very rare antibody (anti-Kpb) had been established in the previous child. No precautions had been taken during this pregnancy, putting both mother and children at risk. All three children recovered, the twins after repeated transfusion of Kpb-free erythrocytes. The described cases emphasize the importance of being informed about the presence of antibodies during pregnancy. Such information can only be obtained by assessing the antibody status during pregnancy. In the Netherlands, the screening of all pregnant women for the presence of irregular antibodies was introduced last year.

Adult↗

Fetal lung and kidney maturation in abnormal pregnancies.

A variety of gestational factors have been reported to increase or decrease the incidence of hyaline membrane disease in newborn infants. The purpose of the current study was to determine if some of these factors influence the rate at which fetal lung structures mature. Histologic measures of lung maturation were retarded in some infants with Rh erythroblastosis and in some whose mothers had diabetes mellitus. By contrast, such maturation was accelerated in offspring of some toxemic gestations and in those whose membranes had ruptured before the onset of labor. Congenital bacterial pneumonia with intact membranes did not accelerate maturation. None of the factors that altered lung maturity influenced renal maturation.

Abruptio Placentae↗

Management of isoimmunization in pregnancy. ACOG Technical Bulletin number 148--October 1990.

Isoimmunization is diagnosed by a positive antibody screen and requires identification of the specific antigen responsible, titration of the level of antibody response, and classification of the antigen into either a clinically significant or benign group. The paternal antigen status and zygosity should be determined whenever possible. A significant antibody response to an antigen associated with erythroblastosis should be monitored at regular intervals by obtaining serial titers, and repetitive amniocentesis or fetal blood sampling may be required to adequately monitor the fetal condition. Early and continued consultation with a perinatologist and a neonatologist who are experts in the management of this condition is critical in developing an appropriate therapeutic plan that includes proper management at delivery and optimal neonatal support. New technologies and expertise now allow better outcome for severely affected fetuses.

Blood Group Antigens↗

Th Rh factor in rural practice; responsibility of the general practitioner.

As the incidence of erythroblastosis fetalis is as high in rural as in urban areas, it behooves physicians in rural areas to anticipate the disease, to make special antepartum studies to determine the Rh status of obstetrical patients, and to be prepared to treat the affected baby if the aid of a specialist and special facilities are not obtainable.Exchange transfusions have been carried out, by means described, in a small rural hospital.

Erythema Nodosum↗

[The "Eilbek capillary"--a new immune hematologic technic].

Studying RBC sedimentation in hematocrit capillaries filled with a variety of separation media (Sephacryl S-300, Ultrogel AcA22 and others) and spinned down for a short time we found some striking advantages in comparison to standard hemagglutination techniques. The scope of diagnostic procedures may be improved and broadened as far as anemias caused by autoantibodies and infections as well as hemolytic disease of the newborn--especially AB0-erythroblastosis--and pretransfusion testing are concerned.

ABO Blood-Group System↗

Fertility, pregnancies and offspring complications after bone marrow transplantation.

Hormonal assessment including luteotropic hormone (LH), follicle stimulating hormone (FSH), testosterone (T), oestradiol and sex hormone binding globulin was performed 1-6 years after allogeneic, syngeneic and autologous bone marrow transplantation in nine patients with severe aplastic anaemia (SAA) and 14 patients with haematological malignancy (HM). Among nine allografted SAA patients, seven aged less than 26 years showed normal gonadal function while two women above 26 years had premature ovarian failure. All eight female patients transplanted for HM had premature ovarian failure. Among six male patients transplanted for HM, four have elevated FSH but normal LH and T levels, while two have normal LH, FSH and T levels or elevated LH and FSH levels but decreased T levels, respectively. Three patients transplanted for SAA (two female, one male), but none of those transplanted for HM, parented four healthy children. A high incidence of offspring complications was noticed, including (i) persistence of fetal circulation syndrome, (ii) erythroblastosis fetalis, and (iii) prolonged newborn icterus. One female patient transplanted for SAA had an abortion in the 25th week of gestation. These observations confirm previous reports on recovery of fertility, mainly in younger patients transplanted for SAA; there was, however, an unexpectedly high incidence of pre-, peri- and postpartum complications in the offspring of bone marrow graft recipients.

Adolescent↗

[Studies on manifestations of Rh-related hemolytic disease of the newborn in the years 1974-1989 in the Erfurt district].

In the course of 13 years (1977-1989) 223 of 223,121 liveborn infants in the district of Erfurt suffered from haemolytic disease due to Rh-isoimmunization, 0.1 per cent died. An initial drop of morbidity from 1.6 to 0.6 of 1000 newborns was followed by stagnation and further increase during the last years. More than 50% of the affected babies were delivered by women who had to be protected from sensibilization by immune prophylaxis. The cause for the immunization were deliveries (75%), miscarriages and interruptions (23%). The most important reason for the insufficient decrease of the Rh-morbidity is the failure of immune prophylaxis in the case of ABO-incongruence between mother and child. Our results support the demands for a general immune prophylaxis without taking into consideration the main blood groups and the number and result of the pregnancy. The quantity of severe erythroblastosis (7 stillborn and 5 hydropic liveborn) demonstrates the necessity to improve the prenatal management of these babies.

Abortion, Induced↗

[Pathology of the placenta. IV. Maturation disorders of the placenta under special clinical conditions].

In Part IV of this review of placental pathology, reference is made to impaired maturation of the placenta under special clinical conditions. Foetal erythroblastosis is only of minor importance, in that context, today, while similar placental alterations may result from foetal hydrops of different genesis, including immunological causes. A detailed account is given of diabetic impairment of maturation together with possible placental diagnosis of diabetes. Cases of diabetes mellitus with concomitant EPH gestosis were found to be more strongly determined for placental differentiation by EPH gestosis. Reference, finally, is made to disturbed placental differentiation under conditions of EPH gestosis with or without concomitant impairment of intervillous circulation.

Erythroblastosis, Fetal↗

Plasma exchange in the management of severe rhesus haemolytic disease.

Over a ten year period from 1977 to 1987, 30 mothers with severe rhesus haemolytic disease and expected fetal loss were treated by plasma exchange using a blood cell separator. All patients had at least on previous stillborn or neonatal death due to haemolytic disease of the newborn. Of the 30 patients, 19 pregnant women were successfully treated. Overall, 53% (16 babies) survived intact. Three of the deaths were due to causes other than erythroblastosis foetalis.

Adult↗

Ultrasonography versus amniotic fluid spectral analysis: are they sensitive enough to predict neonatal complications associated with isoimmunization?

The modern management of pregnancy complicated by erythroblastosis fetalis is based on serial amniocenteses followed by either intrauterine transfusions or early delivery of affected infants, depending on the gestational age. A retrospective study was undertaken involving 92 isoimmunized pregnancies in which ultrasonography and amniotic fluid analyses were used to assess the relative predictive values for neonatal complications. Our data demonstrated that ultrasonography and optical density analyses were comparable in the evaluation of the isoimmunized pregnancy. The combined information from both these modalities did not increase the predictive value over that observed with either one alone. Both instruments were found to have high negative predictive values. However, neither method, used singly or in combination, was highly predictive of neonatal complications. In light of the low positive predictive values, caution should be exercised in using the results of amniotic fluid spectral analyses and/or ultrasound examinations for predicting perinatal complications.

Amniotic Fluid↗