1/22. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura.Upshaw-Schulman syndrome (USS) is a congenital bleeding disorder characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond to infusions of fresh frozen plasma. Inheritance of USS has been thought to be autosomal recessive, because 2 siblings in the same family are often affected but their parents are asymptomatic. Recently, chronic relapsing thrombotic thrombocytopenic purpura (CR-TTP), reported almost exclusively in adults, was shown to be caused by inherited or acquired deficiency in the activity of a plasma von willebrand factor-cleaving protease (vWF-CPase). The pathogenesis of USS is unknown, and a relationship between CR-YEP and USS has not been reported. We studied 3 unrelated USS patients (ST, SY, and KI) who presented with severe indirect neonatal hyperbilirubinemia. All 3 patients had undetectable vWF-CPase activity, and the inhibitors to vWF-CPase were all negative. In their parents with no clinical symptoms, vWF-CPase activities as a percentage of control samples (mother/father) were 17/20 for ST, 60/45 for SY, and 36/5.6 for KI. Thus, USS and vWF-CPase activity appear to be coinherited as autosomal recessive traits. Transfusion of fresh frozen plasma in 2 patients (ST and SY) resulted in the expected maximal increment of approximately 7% to 8% in vWF-CPase activity at 1 to 4 hours, but the levels became less than 3% within 2 days. After this decrease, platelet counts increased, plateaued in the normal range at 10 to 12 days, and declined thereafter. Thus, the 2 to 3 weeks of therapeutic benefit from plasma infusions will be discussed in relation to the intravascular lifetime of vWF-CPase.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/22. Low glucose-6-phosphate dehydrogenase enzyme activity level at the time of hemolysis in a male neonate with the African type of deficiency.glucose-6-phosphate dehydrogenase (G6PD) levels are not usually drawn in the evaluation of black neonates with hyperbilirubinemia because of the oft-stated opinion that the levels may be normal at the time of hemolysis and thus will be misleading. In fact, this opinion is not applicable to newborns as many studies have shown that deficiency in the conjugating ability of the liver, not hemolysis, is the main cause of neonatal jaundice associated with G6PD deficiency. We present a case report of a neonate with brisk hemolysis and hyperbilirubinemia in whom the G6PD level was abnormally low at the time of the hemolytic episode. dna analysis showed him to have the A-(202A,376G) variant and, as well, the UGT1A1 promoter repeat polymorphism associated with Gilbert's disease. This case, as well as a review of the literature, indicates that enzyme levels are not normal in patients with G6PD A- who are undergoing hemolysis.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
3/22. Hyporegenerative anemia associated with Rh hemolytic disease: treatment failure of recombinant erythropoietin.A postnatal hyporegenerative anemia may complicate Rh hemolytic disease. Intramedullary hemolysis, bone marrow suppression, and erythropoietin deficiency have been implicated etiologically. Treatment with recombinant erythropoietin (r-EPO) has yielded encouraging preliminary results. The authors describe an infant with rh isoimmunization who developed severe hyporegenerative anemia unresponsive to a 5-week course of r-EPO. Two additional doses at 12 weeks resulted in brisk reticulocytosis, coinciding with a 16-fold decline in the anti-Rh(D) antibody titer. Thus, treatment with r-EPO may be ineffective when anti-Rh(D) antibody titers are high. The authors also show that erythropoietin deficiency in hyporegenerative anemia is not as frequent and severe as originally thought.- - - - - - - - - - ranking = 5keywords = hemolytic (Clic here for more details about this article) |
4/22. Tin-mesoporphyrin in the treatment of severe hyperbilirubinemia in a very-low-birth-weight infant.Tin-mesoporphyrin (SnMP) is a competitive inhibitor of microsomal heme oxygenase (the rate-limiting enzyme in the heme catabolic pathway). It was administered as a single intramuscular dose at 46 hours of life to a very-low-birth-weight infant with severe hemolytic hyperbilirubinemia who had been awaiting an exchange transfusion. This case documents the effective elimination of the need for an exchange transfusion in a very-low-birth-weight infant and is a confirmation of the experience of others for the use of SnMP in reducing bilirubin production.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
5/22. 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 = 6keywords = hemolytic (Clic here for more details about this article) |
6/22. ABO incompatibility due to immunoglobulin g anti-B antibodies presenting with severe fetal anaemia.ABO incompatibility is a common haematological problem affecting the newborn. The haemolysis is widely accepted to follow a relatively benign course rarely causing the escalating levels of hyperbilirubinaemia and significant anaemia associated with Rh haemolytic disease of the newborn. case reports of fetal hydrops secondary to ABO incompatibility are particularly rare. We describe two cases, first that of a twin pregnancy with both fetuses developing severe anaemia at 20 weeks gestation, and then a second case of a preterm baby demonstrating aggressive haemolysis and anaemia within hours of delivery. Both mothers were of black Africian origin and both were identified to have elevated titres of IgG anti-B antibodies.- - - - - - - - - - ranking = 0.18407383313206keywords = anaemia (Clic here for more details about this article) |
7/22. Neonatal jaundice and splenic hematoma.In the newborn period, unconjugated hyperbilirubinemia (UHB) is common, multifactoral, and associated with a variety of physiologic and pathologic conditions. The most commonly identified pathologic cause leading to hyperbilirubinemia is hemolytic disease of the newborn. We report a five-days-old female infant with neonatal jaundice secondary to splenic hematoma.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
8/22. Hemolytic disease of the newborn due to anti-Di: a case study and review of the literature.BACKGROUND: The severity of hemolytic disease of the newborn (HDN) due to Diego(b) (Di(b)) mismatch ranges from no symptoms to severe jaundice that requires exchange transfusion (ET). The clinical significance of anti-Di(b) is incompletely recognized. CASE REPORT: A male newborn, referred with jaundice, was revealed to have HDN due to Di(b) mismatch and was treated successfully with phototherapy and high-dose intravenous gamma globulin (IVGG). STUDY DESIGN AND methods: The literature of HDN caused by Di(b) mismatch was reviewed. The cases were classified into three groups according to their severity: the mildest needed no therapy (NO), the moderate group received phototherapy alone (PHOTO), and the most severe was treated with ET and/or high-dose IVGG therapy plus phototherapy (ET/IVGG). RESULTS: Among 27 cases of HDN due to Di(b) reported to date, 10, 6, and 11 cases required NO, PHOTO, and ET/IVGG, respectively. A significant correlation (p < 0.01) was found between the maternal anti-Di(b) titer and the severity of the disease when the ET/IVGG group was compared with the NO group. All mothers of the group that needed ET/IVGG had an anti-Di(b) titer of 64 or greater. CONCLUSION: A maternal high titer (> or =64) of anti-Di(b) is associated with a higher risk of severe hyperbilirubinemia for mismatched newborns.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
9/22. ABO blood group incompatibility and primary tooth discoloration.A case report of discolored anterior primary teeth is presented. Medical history and clinical findings suggest an etiology of hemolytic anemia and jaundice secondary to ABO blood group incompatibility. There are no previous reports of tooth discoloration resulting from ABO blood group incompatibility.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
10/22. hyperbilirubinemia in otherwise healthy infants.Treatment of hyperbilirubinemia in otherwise healthy infants is controversial, since hyperbilirubinemia in the absence of hemolytic disease, hepatobiliary disease or prematurity is not clearly associated with kernicterus or other long-term morbidity. phototherapy, exchange transfusion and the withholding of breast milk are the only treatments that have proved effective. Home phototherapy may be useful in treating infants for whom hospitalization is otherwise unnecessary.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
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