Smit-Sibinga Neonatology and Blood Transfusion

1. Auflage 2010
ISBN: 978-0-387-23600-1
Verlag: Springer US
Format: PDF
Kopierschutz: 1 - PDF Watermark

E-Book, Englisch, Band 39, 294 Seiten

Reihe: Developments in Hematology and Immunology

Proceedings of the Twenty-Eighth International Symposium on Blood Transfusion, Groningen, NL, Organized by the Sanquin Division Blood Bank North-East, Groningen. It is in many ways fitting that the last of these international symposia on blood transfusion should end with neonatal blood transfusion. The most fragile, least well studied and most at risk population requires special care and concern. We need to expand our knowledge of their unique physiology, biochemical pathways and in planning treatment and interventions, always 'do no harm.' This proceedings of the last Groningen symposium presents a wealth of information on developmental immunology, the molecular basis of haematopoeisis, physiological basis of bleeding and thrombosis, transfusion risks and benefits and lastly, future therapies. Infants provide us with much to learn but in turn they will be the providers of (through cord blood) and the recipients of (through cellular engineering) the best that science can offer. Translational research, which has been the thrust of these presentations for 28 years, will benefit them in a way that no scientist could have ever predicted.

Autoren/Hrsg.

Smit-Sibinga, C. Th.

Inhaltsverzeichnis

1;INTRODUCTION;7
2;Table of Contents;8
3;MODERATORS AND SPEAKERS;10
4;OPENING ADDRESS;12
5;I. FOETAL AND NEONATAL HAEMATOLOGY;15
5.1;REGULATION OF DEVELOPMENTAL HAEMATOPOIESIS BY GATA TRANSCRIPTION FACTORS1;16
5.1.1;Introduction;16
5.1.2;Biology of ‘haematopoietic’ GATA transcription factors;17
5.1.3;Developmental changes in haematopoiesis;20
5.1.4;GATA transcription factors in the switch from ‘primitive’ to ‘definitive’ haematopoiesis;20
5.1.5;GATA transcription factors in the switch of the primary site of haematopoiesis;21
5.1.6;GATA transcription factors in developmental changes of the proliferative activity of haematopoietic progenitor cells;23
5.1.7;Regulation of the Epo receptor by GATA-1 in haematopoietic progenitor cells;23
5.1.8;Role of GATA transcription factors in the switch of globin genes during development;24
5.1.9;GATA transcription factors in the regulation of the switch of primary site of erythropoietin production;25
5.1.10;GATA transcription factors in congenital or acquired disorders of haematopoiesis;26
5.1.11;Human disorders linked to cis-acting GATA sequences;28
5.1.12;Summary;28
5.1.13;Acknowledgement;29
5.1.14;References;29
5.2;DEVELOPMENT OF THE IMMUNE SYSTEM IN THE FOETAL AND PERINATAL PERIOD;37
5.2.1;References;41
5.3;FOETAL AND NEONATAL IMMUNOHAEMATOLOGICAL RESPONSES: CONSEQUENCES FOR PRACTICAL MANAGEMENT?;43
5.3.1;Introduction;43
5.3.2;Foetal and Neonatal Transfusions;44
5.3.3;Long-Term Effects of Intraperitoneal Transfusions with Non-Leukocyte Reduced, Non-Irradiated Red Cells;44
5.3.4;Effects of Maternally Administered High Dose Immunoglobulin (IVIG) and Platelet Transfusions (IUPT) in Infant Hood;46
5.3.5;Short Term Effects of Intrauterine Erythrocyte Transfusions (despite leukocyte reduction and gamma irradiation);47
5.3.6;Immediate Effects of IUET;50
5.3.7;Premature Infants;50
5.3.8;Conclusion;52
5.3.9;References;53
5.4;BIOLOGY OF THROMBOPOIETIN IN THE HUMAN FOETUS AND NEONATE;55
5.4.1;Introduction;55
5.4.2;Discovery and terminology of thrombopoietin and its receptor c-mpl;55
5.4.3;Cellular biology of thrombopoietin;55
5.4.4;Molecular biology of thrombopoietin;56
5.4.5;Molecular biology of c-mpl, the thrombopoietin receptor;57
5.4.6;Regulation of Tpo;58
5.4.7;Clinical biology of Tpo;59
5.4.8;Biology of thrombopoietin in inherited neonatal thrombocytopenias;60
5.4.9;Biology of thrombopoietin in acquired neonatal thrombocytopenias;62
5.4.10;Towards a rational to treat neonatal thrombocytopenia in sick preterm and term neonates;64
5.4.11;New aspects of the developmental biology of thrombopoietin;64
5.4.12;Acknowledgement;65
5.4.13;References;66
5.5;DISCUSSION;75
6;II. IMMUNOHAEMATOLOGY AND HAEMOSTASIS;81
6.1;MANAGEMENT OF RED CELL ALLOIMMUNIZATION IN PREGNANCY;82
6.1.1;History of Red Cell Alloimmunization;82
6.1.2;Prevention of Red Cell Immunisation;84
6.1.3;Maternal Serum Testing;86
6.1.4;Paternal Zygosity and Foetal Typing;87
6.1.5;Obstetric History;88
6.1.6;Plasmapheresis;88
6.1.7;Immunoglobulin in Red Cell Alloimmunization;89
6.1.8;Foetal monitoring;89
6.1.9;Intrauterine Transfusion;94
6.1.10;References;102
6.2;NEW TREATMENT OPTIONS IN NEONATAL HYPERBILIRUBINAEMIA;126
6.2.1;Introduction;126
6.2.2;Pathophysiology;127
6.2.3;Risk of Severe Hyperbilirubinaemia;128
6.2.4;Treatment of Unconjugated Hyperbilirubinaemia;129
6.2.5;Phototherapy;130
6.2.6;Mechanism of Action;130
6.2.7;Exchange Transfusion;134
6.2.8;Pharmacological Approach;135
6.2.9;Summary;135
6.2.10;References;136
6.3;CONSENSUS AND CONTROVERSY IN FOETAL AND NEONATAL ALLOIMMUNE THROMBOCYTOPENIA;140
6.3.1;Areas of Consensus;140
6.3.2;Antenatal Management;143
6.3.3;Maternally-administered Intravenous (IV) Gammaglobulin (IVIG);144
6.3.4;Antenatal Treatment;147
6.3.5;Foetal Platelet Transfusions;149
6.3.6;Management of the Neonate;151
6.3.7;Conclusion;152
6.3.8;References;152
6.4;THE BLEEDING INFANT;155
6.4.1;Introduction;155
6.4.2;Investigation of the Neonate;156
6.4.3;Well Infant;156
6.4.4;Sick Infant;157
6.4.5;Gastrointestinal Bleeding;157
6.4.6;Intracranial Haemorrhage;157
6.4.7;Vitamin K Deficiency;157
6.4.8;Congenital Hereditary Syndromes;158
6.4.9;References;158
6.5;DISCUSSION;160
7;III. BLOOD TRANSFUSION IN THE NEONATE;173
7.1;NEONATAL THROMBOSIS;174
7.1.1;Introduction;174
7.1.2;Developmental Haemostasis;174
7.1.3;Risk Factors;175
7.1.4;Venous TEs;176
7.1.5;Arterial TEs;176
7.1.6;Renal Vein Thrombosis;177
7.1.7;Diagnosis;177
7.1.8;Laboratory Testing;177
7.1.9;Treatment;178
7.1.10;Summary;179
7.1.11;References;179
7.2;CRITERIA FOR SELECTING A RED BLOOD CELL PRODUCT TO TRANSFUSE ANAEMIC INFANTS;181
7.2.1;Introduction;181
7.2.2;Concerns About and Rationale for Transfusing Stored RBCs;182
7.2.3;Increase in Extracellular Potassium;182
7.2.4;Decrease in 2,3-DPG;183
7.2.5;Safety of Additives in Pre1ervative Solutions;183
7.2.6;Clinical Experience Transfusing Stored RBCs;185
7.2.7;Conclusions and Recommendations;187
7.2.8;References;187
7.3;HAZARDS OF TRANSFUSION: GvHD;189
7.3.1;History;189
7.3.2;Pathogenesis;189
7.3.3;Clinical Manifestations;191
7.3.4;Diagnosis of TA-GvHD;191
7.3.5;Microchimerism and its Relationship to TA-GvHD;192
7.3.6;Groups at Risk;192
7.3.7;The Irradiation Process;193
7.3.8;Instrumentation for Irradiation;194
7.3.9;Components to be Irradiated;195
7.3.10;Storage of Red Cells and Platelets after Irradiation;197
7.3.11;Selection of Radiation Dose;199
7.3.12;Quality Assurance Measures;200
7.3.13;Confirming that Irradiation Occurred;200
7.3.14;New Methods in the Prevention of TA-GvHD;202
7.3.15;Treatment of TA-GvHD;203
7.3.16;References;203
7.4;NON-IMMUNE, NON-INFECTIOUS COMPLICATIONS OF TRANSFUSION;210
7.4.1;Introduction;210
7.4.2;Extended Storage/Additive Solutions;211
7.4.3;Metabolic Complications;213
7.4.4;Potential Toxicities of Transfusion;217
7.4.5;References;219
7.5;EXTRACORPOREAL MEMBRANE OXYGENATION IN THE NEONATE WITH RESPIRATORY FAILURE;222
7.5.1;Introduction;222
7.5.2;ECMO Criteria;224
7.5.3;Pre-ECMO Procedures;225
7.5.4;The ECMO Procedure;225
7.5.5;Equipment and Systems;227
7.5.6;Patient Management;228
7.5.7;Daily Medical Management (Figure 5);229
7.5.8;Outcome Data;231
7.5.9;Summary;232
7.5.10;References;232
7.6;DISCUSSION;236
8;IV. CELLULAR THERAPIES IN NEONATOLOGY;249
8.1;GENETIC ENGINEERING FOR THE FOETUS AND NEONATE;250
8.1.1;In Utero Haematopoietic Stem Cell Transplantation;250
8.1.2;Human IUHSCTx Clinical Trials;252
8.1.3;Gene Therapy for X-Linked Severe Combined Immune Deficiency;253
8.1.4;SCID Clinical Trials and Leukaemia;254
8.1.5;Future Directions;255
8.1.6;References;255
8.2;META-ANALYSIS AND EVIDENCE-BASED DECISION MAKING IN NEONATAL CARE;256
8.2.1;Introduction;256
8.2.2;Meta-analysis and Evidence-based Decisions in Neonatology;257
8.2.3;Pathophysiology of Erythropoietin in the Anaemia of Prematurity;258
8.2.4;Meta-Analysis of Clinical Trials Studying rHEPO in the Anaemia of Prematurity;259
8.2.5;Evidence-Based Medicine beyond Meta-Analysis for Making Medical Decisions;260
8.2.6;Conclusions;263
8.2.7;References;264
8.3;PLACENTAL BLOOD BANKING IN THE YEAR 2003;266
8.3.1;Introduction;266
8.3.2;The Milano Cord Blood Bank;268
8.3.3;Collection Centres;269
8.3.4;Donor Selection and Informed Consent;269
8.3.5;Exclusion Criteria;270
8.3.6;Cord Blood Collection, Transportation and Receipt at the Bank;270
8.3.7;Unit Processing, Characterisation and Quality Control;271
8.3.8;Cord Blood Cryopreservation;273
8.3.9;Cord Blood Unit Validation;274
8.3.10;Mother and Newborn Check Six Months after Delivery;275
8.3.11;Cord Blood Banking Process Monitoring;277
8.3.12;Conclusions;277
8.3.13;References;277
9;DISCUSSION;279
10;EPILOGUE;286
11;INDEX;287

Leseproben

"II. IMMUNOHAEMATOLOGY AND HAEMOSTASIS MANAGEMENT OF RED CELL ALLOIMMUNIZATION IN PREGNANCY (p. 68-67)

I.L. van Kamp, H.H.H. Kanhai

History of Red Cell Alloimmunization

Haemolytic disease of the foetus and newborn, also known as erythroblastosis foetalis, used to be one of the main causes of perinatal mortality for many centuries. Although the clinical picture of extremely hydropic and icteric stillborns was already described in the 17 century, the pathogenesis of the disease was not understood until the early 1940s.

Darrow published in 1934 an extensive overview on the clinical picture and etiologic considerations of neonatal haemolytic disease [1]. From the observation that the disease frequently occurred in the offspring in one family, Darrow hypothesized that the placenta may be the means of transmission of a destructive influence from mother to foetus [1]. Subsequently, Levine and Stetson managed to identify an unknown red-cell antigen in the blood of a woman who was delivered from a stillborn hydropic baby [2].

The woman had massive uterine bleeding and appeared to suffer from a life-threatening transfusion reaction, after being transfused with her husbands f blood. As husband and wife both had blood type 0, Levine and Stetson called this unknown phenomenon “intra-group agglutination” [2,3]. Simultaneously, Landsteiner and Weiner discovered an agglutinating factor in th serum of rodents, sensitised with blood from a Macacus Rhesus monkey [4]. As this factor caused agglutination of the blood of 85% of New York’s population, it was f initially assumed to be similar to the human antibody causing erythroblastosis foetalis.

Eventually, the antibody appeared comparable, though not identical to the human antibody, but by that time it had been erroneously called “Rhesus”. t After the discovery of the alloimmune origin of haemolytic disease, more insight and knowledge was gained on this pathological process by several studies [5]. In these years a beneficial effect of maternal and pater f nal ABO incompati- r bility on the severity of haemolytic disease was observed [6].

Neonatal exchange transfusion as a method of treatment of hyperbilirubinaemia was described by Wallerstein in 1946 [7]. This was the first impor tant step in the prevention of kernicterus, the most serious and feared complication of neonatal hyperbilirubinaemia, followed in 1958 by the introduction of fototherapy. However, until the 1960s severe foetal haemolytic disease could neither be diagnosed nor be treated. Elective preterm delivery of a foetus, assumed to be viable, was the policy in most pregnancies complicated by maternal Rhesus (D) alloimmunization, aiming to prevent foetal demise and to be able to start neonatal treatment."