Search PubMed⌕ Search

Biomedical subjects

J B Bussel

Publications and source records attributed to J B Bussel.

At least 55 records · Page 3Linked to original sources
PubMed1996

Advantages of larger volume, less frequent intrauterine red blood cell transfusions for maternal red cell alloimmunization.

Larger volume intravascular transfusions to manage severe maternal red cell alloimmunization in pregnancy may prolong the interval between procedures without increasing maternal, fetal, or neonatal complications. A retrospective cohort study compared the management and outcome of 19 patients with severe red cell alloimmunization managed at two facilities with different intravascular transfusion protocols. The volume of blood transfused, pre- and post-transfusion fetal hematocrit, and interval (days) between intravascular transfusions were compared. The respective maternal, fetal, and neonatal results were compared. The red blood cell volume transfused per procedure and the post- but not pre-transfusion fetal hematocrits were higher at New York Hospital than at Westchester County Medical Center. The interval between transfusions at New York Hospital (25.2 +/- 8.65 days) was longer than at Westchester County Medical Center (13.5 +/- 6.0 days, p < 0.0001). Although larger volume transfusion was occasionally associated with transient fetal bradycardia, all red blood cell transfusions were completed without complication. The adverse outcomes, complication rates, and neonatal outcomes were otherwise similar in both management protocols. It is possible to significantly increase the interval between intravascular transfusions with larger transfusion volumes for the management of severe maternal red cell alloimmunization without undue risk. The overall risk for the fetus and mother may be reduced by performing fewer transfusions and avoiding additional blood product exposures.

Blood Transfusion, Intrauterine↗
PubMed1996

Fetal transfusion therapy.

Rapid advances are occurring in the diagnosis and treatment of the fetus with a red blood cell or platelet cytopenia. Noninvasive methods of monitoring the alloimmunized pregnancy, invasive methods such as amniocentesis and cordocentesis, and intrauterine transfusion therapy of both red cells and platelets, are being further refined to allow the prompt recognition and treatment of fetal cytopenias. Specialized centers have now accrued a large experience in the management of the fetus severely affected by alloimmunization. Advances in ultrasound, blood banking techniques, and genetic engineering technology have spurred the most recent advances. The indications for diagnosis, timing and frequency of invasive procedures for treatment, and technical considerations regarding preparation of blood products and volume of transfusion, are outlined in this review. Polymerase chain reaction (PCR) determination of fetal Rh(D) genotype by chorionic villus sampling or amniocentesis in the first or second trimesters is a recent clinically useful advance. The advent of hematopoietic stem cell transplantation and the potential for gene therapy are exciting advances in the treatment and prevention of hematopoietic diseases, including, but not limited, to the fetal cytopenias.

Anemia↗
PubMed1996

TTP following ITP in an HIV-positive boy.

PURPOSE: To report the previously undescribed development of thrombotic thrombocytopenic purpura (TTP) in a human immunodeficiency virus (HIV)-positive child and discuss the differential diagnosis. PATIENT AND METHODS: Our patient was a 9-year-old boy with vertically acquired HIV infection and a previous history of immune thrombocytopenic purpura (ITP). Initial presentation, difficulty in diagnosis, clinical course, and subsequent outcome are described. RESULTS: Rapid resolution of TTP following plasmapheresis was seen. CONCLUSIONS: Recognition of this treatable though potentially fatal complication in severely ill HIV-infected children requires a high degree of suspicion in view of its diverse clinical manifestations. The long-term outcome may be relatively good.

Child↗
PubMed1995

Alloimmune thrombocytopenia: fetal and neonatal losses related to cordocentesis.

OBJECTIVE: This report describes the increased risks of cordocentesis in fetuses affected with alloimmune thrombocytopenia. STUDY DESIGN: As part of a multicenter treatment study clinical and laboratory data from five pregnancies with alloimmune thrombocytopenia in which there was a fetal or neonatal loss associated with cordocentesis were reviewed. The fetal or neonatal deaths were all thought to be a result of exsanguination. These fetuses were compared with a group of 44 affected fetuses who underwent the same procedure but who survived. The data were analyzed by the Wilcoxon rank-sum test and the two-tailed Fisher's exact test. A p value < 0.05 was considered significant. RESULTS: The mean platelet count at cordocentesis was significantly lower in the cases than in the controls (5.8 vs 32.8 x 10(9)/L, p = 0.005). The incidence of antenatal intracranial hemorrhage in the untreated sibling of the prior affected pregnancy was significantly greater in the cases than in the controls (two of five vs one of 42, p = 0.02). CONCLUSION: Fetuses affected with alloimmune thrombocytopenia are at increased risk for fatal exsanguination associated with cordocentesis.

Antigens, Human Platelet↗
PubMed1995

Fetal and neonatal thrombocytopenia.

Thrombocytopenia is defined as platelet count less than 150,000 plat/mm3. Etiologic factors involved include immunological (NAIT and ITP), fetal infectious disease, chromosomal and nonchromosomal, and miscellaneous causes. While the understanding of fetal thrombocytopenia is driven by reason to do fetal blood sampling, discovery of neonatal thrombocytopenia is driven by blood counts performed because of the risk of infections. The most serious consequence of thrombocytopenia in the fetus/neonate is intracranial hemorrhage which can occur in utero as early as 18 weeks gestation. The key factor in perinatal prevention of intracranial hemorrhage is early diagnosis and treatment, possibly in utero. Cordocentesis under direct ultrasound guidance and platelet transfusions have played a major role in the management of fetal/neonatal thrombocytopenia. Ongoing studies and high resolution ultrasound will continue to explore and hopefully clarify fetal and neonatal thrombocytopenia and facilitate recognition of primary and secondary thrombocytopenias.

Fetal Blood↗
PubMed1995

Neonatal thrombocytopenia.

The workup and management of neonatal thrombocytopenia is a problem commonly encountered by pediatric hematologists and neonatologists. The incidence of this problem has been estimated to range as high as 1% or more for healthy term infants to 20% of newborns admitted to a neonatal intensive care unit. An expeditious approach to this problem and timely intervention when necessary can prevent or attenuate the potentially serious consequences of this problem, most notably that of intracranial hemorrhage. In this article we discuss the incidence and causes of this problem, and present an algorithm for an approach to the neonate with thrombocytopenia. This will include consideration of fetal thrombocytopenia and its management, that is, in repeated pregnancies in women with alloimmune thrombocytopenia. Case discussions will be used to illustrate the algorithm.

Algorithms↗
PubMed1994

Lactic acidosis and renal enlargement at diagnosis and relapse of acute lymphoblastic leukemia.

Bilateral renal enlargement was noted on ultrasonography during an extensive renal evaluation for severe hypokalemic metabolic acidosis with an increased anion gap in a 12-year-old Hispanic boy who had normal results of a physical examination and complete blood count. The patient was found to have acute lymphoblastic leukemia. Resolution of the lactic acidosis and bilateral renal enlargement occurred with initiation of chemotherapy and recurred with each subsequent relapse.

Acidosis, Lactic↗
PubMed1994

Accurate intrapartum estimation of fetal platelet count by fetal scalp samples smear.

Fetal scalp sampling has been used to determine fetal platelet count in early labor. Because platelet clumping may lead to falsely low platelet counts, this pilot study was carried out to evaluate an improved method of scanning an intrapartum fetal scalp smear for platelet estimation. We report 5 years' experience with the use of scanning of the smear for platelet aggregates instead of direct measurement of platelets. In 22 cases, intrapartum examination of the scalp blood smear at low power revealed the great majority of high-power, dry microscopic fields devoid of platelets, whereas isolated fields contained aggregates of more than 10 platelets. In one case, an adequate number of platelets were evenly distributed. Intrapartum fetal platelet estimation by scanning of the smear (using these platelet aggregates) correctly identified an adequate neonatal platelet count in 23 cases (mean, 264,600 +/- 15,000; range, 133,000 to 396,000). Cesarean section was avoided in 82.6% of these cases. In this pilot study, platelet estimation on fetal scalp sample were reliably performed by scanning of the smear for platelet aggregates. When aggregates were seen, platelet adequacy was found in all cases.

Blood Specimen Collection↗
PubMed1994

Antenatal management of the thrombocytopenias.

Thrombocytopenia, the most common hemostatic disease of the newborn, can now be safely identified and treated antenatally to prevent life-threatening fetal and neonatal hemorrhage. This article focuses on the fetal thrombocytopenias with emphasis on the most common causes, the immune thrombocytopenias, including alloimmune and autoimmune mechanisms. The clinical presentation and differential diagnosis are discussed briefly.

Diagnosis, Differential↗
PubMed1993

Efficacy of intravenous immunoglobulin in the treatment of autoimmune hemolytic anemia: results in 73 patients.

To determine whether warm-antibody autoimmune hemolytic anemia (AIHA) responds to treatment with intravenous gammaglobulin (IVGG), we conducted separate pilot studies at three institutions enrolling a total of 37 patients. We combined these results with a review of 36 cases of AIHA treated with IVGG reported in the literature. Sixteen clinical variables were examined to determine associations with response to IVGG. Overall, 29 of 73 patients (39.7%) responded to IVGG therapy. Two variables were strongly related to a good response to IVIG: the presence of hepatomegaly (with and without splenomegaly) and a low pre-treatment hemoglobin. A trend towards a better response was observed in the 11 children. Overall, IVGG provided acute benefit in only 1/3 of patients and therefore cannot be recommended as standard therapy for AIHA. It may, however, be useful as adjunctive treatment in selected cases, such as in those with a pre-treatment hemoglobin < 6-7 gm/dl or those with hepatomegaly, and in clinical settings where the toxicity of other treatments may be an important consideration.

Adult↗
PubMed1993

Coagulation disorders in children.

Many new developments have taken place in the field of coagulation during the past year. We focus on the interesting new controversies surrounding vitamin K prophylaxis of the newborn infant, on the increasing information regarding the growth factors responsible for thrombopoiesis, and on the role of protein C in the genesis of thrombosis.

Bleeding Time↗
PubMed1992

Antenatal treatment of alloimmune thrombocytopenia.

OBJECTIVE: Neonatal alloimmune thrombocytopenia is caused by platelet antigen incompatibility between the mother and fetus. Affected fetuses may have severe thrombocytopenia leading to intracranial hemorrhage before or at birth. We sought to treat this condition in utero to prevent these hemorrhages. METHODS: Eighteen women who had previously delivered infants with severe alloimmune thrombocytopenia were treated with weekly infusions of intravenous gamma globulin from the diagnosis of fetal thrombocytopenia until birth; nine were also treated with corticosteroids. RESULTS: There were no intracranial hemorrhages in the treated fetuses, compared with ten cases among the 21 untreated siblings (48%). Only three treated fetuses, compared with 16 of 20 untreated siblings, had platelet counts of less than 30,000/microL, with no bleeding complications. CONCLUSION: Antenatal treatment of alloimmune thrombocytopenia with weekly gamma globulin effectively improves the fetal platelet count and prevents intracranial hemorrhage.

Adrenal Cortex Hormones↗
PubMed1991

Prenatal diagnosis of neonatal alloimmune thrombocytopenia using allele-specific oligonucleotide probes.

The prediction of neonatal alloimmune thrombocytopenia (NATP) in affected families has, in the past, been based on information about gene frequencies of the antigen systems involved, parental phenotyping, and fetal platelet counts. We explored the feasibility of allele-specific oligonucleotide probe typing for PIA antigens to determine the risk of second or subsequent fetuses in families where one infant had a diagnosis of anti-PIA1-mediated NATP. A total of eight families at risk for delivering an affected fetus were studied with both serologic and oligonucleotide typing. The correlation between serologic and oligonucleotide PIA types was 100%. Similarly, in an additional eight families not at risk for PIA1-mediated NATP, serologic and oligonucleotide typing maintained a perfect correlation. DNA isolated from fetal leukocytes as well as fetal amniocytes was successfully typed using this technology. Oligonucleotide-based typing of fetuses at risk for NATP whose fathers are heterozygous for the PIA antigens allows early recognition of affected fetuses so that prenatal therapy of mothers can be instituted if necessary. When fetuses are found to be unaffected, invasive, and/or expensive, prenatal interventions can be avoided.

Adult↗
PubMed1991

Intravenous anti-D treatment of immune thrombocytopenic purpura: analysis of efficacy, toxicity, and mechanism of effect.

The efficacy, toxicity, and mechanism of effect of intravenous Anti-D (Winrho) were studied in 43 Rh+ patients with immune thrombocytopenia purpura (ITP) who had not undergone splenectomy and in three already splenectomized patients. The mean platelet increase for the 43 nonsplenectomized patients was 95,000/microL (median 43,000/microL). Children had greater acute platelet responses than did adults. Human immunodeficiency virus status and duration of thrombocytopenia did not affect response. Maintenance treatment was given to patients as needed: the average interval between infusions was 24 days. The three splenectomized patients had no platelet response whatsoever. Toxicity was minimal; infusions were completed in less than 5 minutes. The generally accepted mechanism of effect of Anti-D has been Fc receptor blockade by substitution of antibody-coated red blood cells for antibody-coated platelets. Evidence is presented suggesting that the effect of IV Anti-D is not limited to Fc receptor blockade, including: (1) no correlation of parameters of hemolysis with platelet increase; (2) a 48- to 72-hour delay before platelet increase; (3) a tendency of the change in monocyte Fc receptor I expression to correlate with platelet increase; and (4) increased in vitro production of antibodies to sheep red blood cells following IV Anti-D infusion.

Adult↗