Pedro A. de Alarcon

Iron Absorption in the Thalassemia Syndromes and Its Inhibition by Tea

To determine the hemoglobin concentration at which iron absorption is minimal, five subjects with thalassemia major and one with thalassemia intermedia underwent a series of iron-absorption studies. The effect of tea as an inhibitor of non-heme iron absorption was also tested. Iron absorption increased as the hemoglobin concentration decreased, although iron absorption was much higher at any given hemoglobin level in the subject with thalassemia intermedia. In the subjects with thalassemia major, iron absorption averaged 10 per cent at hemoglobin concentrations between 9 and 10 and 2.7 per cent between 11 and 13 g per deciliter. The percentage of iron absorbed could be accurately predicted from the nucleated red-cell count (r = 0.91, P less than 0.001). Tea produced a 41 to 95 per cent inhibition of iron absorption. Since patients with thalassemia intermedia may absorb a large percentage of dietary iron, inhibitors of iron absorption, such as tea, may be useful in their management.

Neuroblastoma-associated opsoclonus-myoclonus treated with intravenously administered immune globulin G

An 18-month-old black girl had progressive truncal ataxia, opsoclonus, and multifocal myoclonus associated with a nonresectable abdominal ganglioneuroblastoma. Before chemotherapy, she received intravenously administered IgG, 1 gm/kg, for 2 days; within 48 hours of the first dose, there was significant improvement of the opsoclonus-myoclonus and ataxia. She required a 1 gm/kg maintenance dose every 4 to 6 weeks for a total of 12 doses, but is now free of symptomatic after 2 years with no therapy.

Lithium therapy in childhood neutropenia

This case emphasizes the need for caution in planning a course of therapy for childhood lead poisoning. Initial and serial evaluations of renal function (urinalysis, BUN, and serum creat inine) are needed. The decision to include BAL in the therapy should also be guided by the presence or absence of G-6-PD deficiency, as BAL can cause intravascular hemolysis in the presence of such deficiency. REFERENCES 1. Pueschel SM, Kapilo L, Schwachman H: Children with an increased lead burden: A screening and follow-up. JAMA 222:462, 1972. 2. Chisolm JJ Jr: Amnioaciduria as a manifestation of renal tubular injury in lead intoxication and a comparison with pattern of amniaciduria seen in other diseases. J PED1ATR 60:117, 1962. 3. Chisolm JJ Jr: Increased lead absorption and lead poisoning (plumbism). In Vaughan VC, McKay R J, Behrman RE, editors: Nelson text of pediatrics, ed 11. Philadelphia. 1979, WB Saunders, p 2025. 4. Foreman H, Finnegan C, Lushaugh CC: Nephotoxic hazard from uncontrolled edathamil calcium-disodium therapy. JAMA 160:1042, 1956. 5. Reuber MD, Bradley JE: Acute versenate nephrosis occurring as a result of treatment of lead intoxication. JAMA 174:263, 1960. 6. Schrier RW: Acute renal failure. Kidney Int 15:205, 1979. 7. Ingelfinger JR, Avner E: Renal disorders. In Graef JW, Cone TE, editors: Manual of pediatric therapeutics. Boston, 1980, Little Brown p 205. 8. Chisolm JJ Jr: The use of chelating agents in the treatment of acute and chronic lead intoxication in childhood. J PEDIATR 73:1, 1968. 9. Smith HD: Pediatric lead poisoning. Arch Environ Health 8:68, 1964. 10. Henderson DA: A follow-up of cases of plumbism in children. Aust Ann Med 3:219, 1954. 11. Tepper LB: Renal function subsequent to childhood plumbism. Arch Environ Health 7:82, 1962.

Neonatal Hematology: Pathogenesis, Diagnosis, and Management of Hematologic Problems

This is the second edition of a very useful and comprehensive review of the key aspects of neonatal hematology. Neonatal hematology has distinguished itself as a unique and important area from both clinical and research aspects. The book comprises 22 chapters organized by disorders of the varying hematologic systems written by experts in hematology and neonatology. In addition, the authors provide a discussion of disorders of the fetomaternal unit, neonatal oncology, and reference ranges in neonatal hematology. The book is targeted to both hematologists and neonatologists and should prove to be useful in daily practice. The chapters are succinctly written. The figures, tables, and images illustrate and complement key points in the text. This book begins with an introductory chapter that provides a fascinating historical review of hematology. This review demonstrates how far the field has come from its inception 2000 years ago when “Ancient concepts of the blood were described by Hippocrates and Galen 2000 years ago in their doctrine of ‘humors’” and demonstrates how 3 key hematologic diseases (erythroblastosis fetalis, hemorrhagic disease of the newborn, and physiologic anemia of infancy) stimulated research into mechanisms of neonatal hematologic physiology. The authors begin the remainder of the book with 2 chapters on developmental hematology with a focus on hematopoiesis and the immune system. These chapters provide a thorough understanding of the developmental changes that occur in the hematologic system of neonates. A comprehensive understanding of these changes is necessary to distinguish normal from disease, thereby directing appropriate treatment. The editors should be applauded for including a comprehensive practical review of disorders commonly encountered in the neonatal intensive care unit, including hemolysis, thrombocytopenia, and coagulation disorders (thrombosis and bleeding). These abnormalities are common reasons for inpatient hematologic consultation. Two chapters are focused on neonatal hemolysis and include a discussion of common and rare, hereditary and acquired disorders. The chapters focused on platelets include immune, acquired, and congenital causes of thrombocytopenia with discussion about both workup and management. Two very helpful tables displaying causes of thrombocytopenia based on time of onset (fetal, <72 h or >72h) and disease severity are included. Finally, the chapters focused on neonatal thrombosis and bleeding provide practical guides to the management and workup. The inclusion of a table outlining dosing of antithrombotic agents in the neonate is very useful clinically. The chapter focused on bleeding disorders comprehensively reviews congenital and acquired bleeding disorders in the context of normal developmental hemostasis. The chapter on Inherited Bone Marrow Failure Syndromes includes a comprehensive summary of the initial evaluation and differential diagnosis of an infant with a suspected bone marrow failure syndrome. The tables in this chapter comparing the hematologic abnormalities and congenital anomalies for these syndromes are comprehensive and very clinically useful for distinguishing between syndromes that have overlapping features. This book also includes 2 chapters on leukocyte disorders that are focused on both quantitative and qualitative conditions that can be either congenital or acquired. Decision trees outlining “consistent approaches” to the evaluation and treatment of neonates with leukocyte disorders are included that should prove to be useful for clinicians. The book includes a single chapter on neonatal oncology that is a concise overview of the most common tumors affecting neonates. Although this chapter provides useful information, it does appear to be beyond the scope of the book as it discusses tumors other than those that involve the hematologic system. The book concludes with a chapter discussing hematologic reference ranges in neonates. This chapter is crucial to a thorough understanding of neonatal hematology as results must be considered in the context of the age of the neonate. This chapter might have been better placed toward the beginning of the book. In conclusion, this book provides a foundation for the evaluation and treatment of the most common and important hematologic disorders of the neonatal period and is a practical reference for clinical practice. The chapters are concise and are well balanced in terms of pathophysiology, diagnostic workup, treatment recommendations, and research findings. I recommend this book to both practicing hematologists/oncologists and neonatologists and those who are training in these fields.

On Dr. Louis K. Diamond's 1932 Article and Subsequent Contributions to Erythroblastosis Fetalis

Hemolytic disease of the fetus and newborn (HDFN) resulting from Rh incompatibility is uncommon today, thanks to the successful implementation in 1968 of routine administration of Rh immunoglobulin to prevent Rh sensitization, and because most women previously sensitized are now past childbearing age. Affected neonates are still seen, mainly those born to women who have sustained large fetomaternal hemorrhages and those who have not received anti-D prophylaxis. The latter is a problem among women who have poor access to care. For this reason, physicians caring for neonates with anemia and jaundice need to remain alert to the possibility of HDFN, whether from Rh or other blood group incompatibilities. Before 1968, Rh HDFN was a common cause of death and neonatal complications, particularly neurologic handicap among survivors of kernicterus. At that time, the disease was generally referred to as erythroblastosis fetalis. Those of us in training during the 1950s and 1960s remember the sleepless nights caring for affected neonates: following serial bilirubin levels, checking with the blood bank to make sure there was compatible fresh blood available, performing exchange transfusions, and, in most cases, reassuring anxious parents that “all went well.” Since then, there have been many advances in diagnosis (amniotic fluid spectrophotometry, ultrasound, periumbilical blood sampling) and treatment (intrauterine intraperitoneal and intravascular transfusion, phototherapy) that have eased the burden on pediatricians and improved the outlook for infants born to sensitized mothers. These advances all began with the groundbreaking study by Drs. Diamond, Blackfan, and Baty published in 1932 (1). Diamond was the “designated” pediatric hematologist. Before that, the literature was replete with reports of neonates with apparently unrelated and unexplained syndromes characterized by varying degrees of severe jaundice (“icterus gravis neonatorum”), generalized edema (“universal edema of the fetus”), or severe anemia (“congenital anemia of the newborn”). Faced with similar cases from their clinical experience in Boston, Diamond and his colleagues performed a critical analysis of the literature, integrating this with data from their own more detailed case studies. They concluded that these syndromes were likely manifestations of the same disease process. This paved the way for future investigators whose discovery of the Rh factor and other key observations helped define the pathogenesis of the disease we know today as HDFN. Their accomplishment is remarkable on several accounts. The literature search and review were performed manually, using what was then known as the Quarterly Cumulative Index Medicus, and must have been tedious. Of the 159 references, more than half were in languages other than English. Because machines for reproducing the articles had not yet been invented, obtaining translations must have been a formidable challenge. A familiarity with German, French,

Neonatal Hematology: Contributors

This is the second edition of a very useful and comprehensive review of the key aspects of neonatal hematology. Neonatal hematology has distinguished itself as a unique and important area from both clinical and research aspects. The book comprises 22 chapters organized by disorders of the varying hematologic systems written by experts in hematology and neonatology. In addition, the authors provide a discussion of disorders of the fetomaternal unit, neonatal oncology, and reference ranges in neonatal hematology. The book is targeted to both hematologists and neonatologists and should prove to be useful in daily practice. The chapters are succinctly written. The figures, tables, and images illustrate and complement key points in the text. This book begins with an introductory chapter that provides a fascinating historical review of hematology. This review demonstrates how far the field has come from its inception 2000 years ago when “Ancient concepts of the blood were described by Hippocrates and Galen 2000 years ago in their doctrine of ‘humors’” and demonstrates how 3 key hematologic diseases (erythroblastosis fetalis, hemorrhagic disease of the newborn, and physiologic anemia of infancy) stimulated research into mechanisms of neonatal hematologic physiology. The authors begin the remainder of the book with 2 chapters on developmental hematology with a focus on hematopoiesis and the immune system. These chapters provide a thorough understanding of the developmental changes that occur in the hematologic system of neonates. A comprehensive understanding of these changes is necessary to distinguish normal from disease, thereby directing appropriate treatment. The editors should be applauded for including a comprehensive practical review of disorders commonly encountered in the neonatal intensive care unit, including hemolysis, thrombocytopenia, and coagulation disorders (thrombosis and bleeding). These abnormalities are common reasons for inpatient hematologic consultation. Two chapters are focused on neonatal hemolysis and include a discussion of common and rare, hereditary and acquired disorders. The chapters focused on platelets include immune, acquired, and congenital causes of thrombocytopenia with discussion about both workup and management. Two very helpful tables displaying causes of thrombocytopenia based on time of onset (fetal, <72 h or >72h) and disease severity are included. Finally, the chapters focused on neonatal thrombosis and bleeding provide practical guides to the management and workup. The inclusion of a table outlining dosing of antithrombotic agents in the neonate is very useful clinically. The chapter focused on bleeding disorders comprehensively reviews congenital and acquired bleeding disorders in the context of normal developmental hemostasis. The chapter on Inherited Bone Marrow Failure Syndromes includes a comprehensive summary of the initial evaluation and differential diagnosis of an infant with a suspected bone marrow failure syndrome. The tables in this chapter comparing the hematologic abnormalities and congenital anomalies for these syndromes are comprehensive and very clinically useful for distinguishing between syndromes that have overlapping features. This book also includes 2 chapters on leukocyte disorders that are focused on both quantitative and qualitative conditions that can be either congenital or acquired. Decision trees outlining “consistent approaches” to the evaluation and treatment of neonates with leukocyte disorders are included that should prove to be useful for clinicians. The book includes a single chapter on neonatal oncology that is a concise overview of the most common tumors affecting neonates. Although this chapter provides useful information, it does appear to be beyond the scope of the book as it discusses tumors other than those that involve the hematologic system. The book concludes with a chapter discussing hematologic reference ranges in neonates. This chapter is crucial to a thorough understanding of neonatal hematology as results must be considered in the context of the age of the neonate. This chapter might have been better placed toward the beginning of the book. In conclusion, this book provides a foundation for the evaluation and treatment of the most common and important hematologic disorders of the neonatal period and is a practical reference for clinical practice. The chapters are concise and are well balanced in terms of pathophysiology, diagnostic workup, treatment recommendations, and research findings. I recommend this book to both practicing hematologists/oncologists and neonatologists and those who are training in these fields.

Neonatal Hematology: Contents

This is the second edition of a very useful and comprehensive review of the key aspects of neonatal hematology. Neonatal hematology has distinguished itself as a unique and important area from both clinical and research aspects. The book comprises 22 chapters organized by disorders of the varying hematologic systems written by experts in hematology and neonatology. In addition, the authors provide a discussion of disorders of the fetomaternal unit, neonatal oncology, and reference ranges in neonatal hematology. The book is targeted to both hematologists and neonatologists and should prove to be useful in daily practice. The chapters are succinctly written. The figures, tables, and images illustrate and complement key points in the text. This book begins with an introductory chapter that provides a fascinating historical review of hematology. This review demonstrates how far the field has come from its inception 2000 years ago when “Ancient concepts of the blood were described by Hippocrates and Galen 2000 years ago in their doctrine of ‘humors’” and demonstrates how 3 key hematologic diseases (erythroblastosis fetalis, hemorrhagic disease of the newborn, and physiologic anemia of infancy) stimulated research into mechanisms of neonatal hematologic physiology. The authors begin the remainder of the book with 2 chapters on developmental hematology with a focus on hematopoiesis and the immune system. These chapters provide a thorough understanding of the developmental changes that occur in the hematologic system of neonates. A comprehensive understanding of these changes is necessary to distinguish normal from disease, thereby directing appropriate treatment. The editors should be applauded for including a comprehensive practical review of disorders commonly encountered in the neonatal intensive care unit, including hemolysis, thrombocytopenia, and coagulation disorders (thrombosis and bleeding). These abnormalities are common reasons for inpatient hematologic consultation. Two chapters are focused on neonatal hemolysis and include a discussion of common and rare, hereditary and acquired disorders. The chapters focused on platelets include immune, acquired, and congenital causes of thrombocytopenia with discussion about both workup and management. Two very helpful tables displaying causes of thrombocytopenia based on time of onset (fetal, <72 h or >72h) and disease severity are included. Finally, the chapters focused on neonatal thrombosis and bleeding provide practical guides to the management and workup. The inclusion of a table outlining dosing of antithrombotic agents in the neonate is very useful clinically. The chapter focused on bleeding disorders comprehensively reviews congenital and acquired bleeding disorders in the context of normal developmental hemostasis. The chapter on Inherited Bone Marrow Failure Syndromes includes a comprehensive summary of the initial evaluation and differential diagnosis of an infant with a suspected bone marrow failure syndrome. The tables in this chapter comparing the hematologic abnormalities and congenital anomalies for these syndromes are comprehensive and very clinically useful for distinguishing between syndromes that have overlapping features. This book also includes 2 chapters on leukocyte disorders that are focused on both quantitative and qualitative conditions that can be either congenital or acquired. Decision trees outlining “consistent approaches” to the evaluation and treatment of neonates with leukocyte disorders are included that should prove to be useful for clinicians. The book includes a single chapter on neonatal oncology that is a concise overview of the most common tumors affecting neonates. Although this chapter provides useful information, it does appear to be beyond the scope of the book as it discusses tumors other than those that involve the hematologic system. The book concludes with a chapter discussing hematologic reference ranges in neonates. This chapter is crucial to a thorough understanding of neonatal hematology as results must be considered in the context of the age of the neonate. This chapter might have been better placed toward the beginning of the book. In conclusion, this book provides a foundation for the evaluation and treatment of the most common and important hematologic disorders of the neonatal period and is a practical reference for clinical practice. The chapters are concise and are well balanced in terms of pathophysiology, diagnostic workup, treatment recommendations, and research findings. I recommend this book to both practicing hematologists/oncologists and neonatologists and those who are training in these fields.

Neonatal Hematology: Pathogenesis, diagnosis, and management of hematologic problems, Second edition

This is the second edition of a very useful and comprehensive review of the key aspects of neonatal hematology. Neonatal hematology has distinguished itself as a unique and important area from both clinical and research aspects. The book comprises 22 chapters organized by disorders of the varying hematologic systems written by experts in hematology and neonatology. In addition, the authors provide a discussion of disorders of the fetomaternal unit, neonatal oncology, and reference ranges in neonatal hematology. The book is targeted to both hematologists and neonatologists and should prove to be useful in daily practice. The chapters are succinctly written. The figures, tables, and images illustrate and complement key points in the text. This book begins with an introductory chapter that provides a fascinating historical review of hematology. This review demonstrates how far the field has come from its inception 2000 years ago when “Ancient concepts of the blood were described by Hippocrates and Galen 2000 years ago in their doctrine of ‘humors’” and demonstrates how 3 key hematologic diseases (erythroblastosis fetalis, hemorrhagic disease of the newborn, and physiologic anemia of infancy) stimulated research into mechanisms of neonatal hematologic physiology. The authors begin the remainder of the book with 2 chapters on developmental hematology with a focus on hematopoiesis and the immune system. These chapters provide a thorough understanding of the developmental changes that occur in the hematologic system of neonates. A comprehensive understanding of these changes is necessary to distinguish normal from disease, thereby directing appropriate treatment. The editors should be applauded for including a comprehensive practical review of disorders commonly encountered in the neonatal intensive care unit, including hemolysis, thrombocytopenia, and coagulation disorders (thrombosis and bleeding). These abnormalities are common reasons for inpatient hematologic consultation. Two chapters are focused on neonatal hemolysis and include a discussion of common and rare, hereditary and acquired disorders. The chapters focused on platelets include immune, acquired, and congenital causes of thrombocytopenia with discussion about both workup and management. Two very helpful tables displaying causes of thrombocytopenia based on time of onset (fetal, <72 h or >72h) and disease severity are included. Finally, the chapters focused on neonatal thrombosis and bleeding provide practical guides to the management and workup. The inclusion of a table outlining dosing of antithrombotic agents in the neonate is very useful clinically. The chapter focused on bleeding disorders comprehensively reviews congenital and acquired bleeding disorders in the context of normal developmental hemostasis. The chapter on Inherited Bone Marrow Failure Syndromes includes a comprehensive summary of the initial evaluation and differential diagnosis of an infant with a suspected bone marrow failure syndrome. The tables in this chapter comparing the hematologic abnormalities and congenital anomalies for these syndromes are comprehensive and very clinically useful for distinguishing between syndromes that have overlapping features. This book also includes 2 chapters on leukocyte disorders that are focused on both quantitative and qualitative conditions that can be either congenital or acquired. Decision trees outlining “consistent approaches” to the evaluation and treatment of neonates with leukocyte disorders are included that should prove to be useful for clinicians. The book includes a single chapter on neonatal oncology that is a concise overview of the most common tumors affecting neonates. Although this chapter provides useful information, it does appear to be beyond the scope of the book as it discusses tumors other than those that involve the hematologic system. The book concludes with a chapter discussing hematologic reference ranges in neonates. This chapter is crucial to a thorough understanding of neonatal hematology as results must be considered in the context of the age of the neonate. This chapter might have been better placed toward the beginning of the book. In conclusion, this book provides a foundation for the evaluation and treatment of the most common and important hematologic disorders of the neonatal period and is a practical reference for clinical practice. The chapters are concise and are well balanced in terms of pathophysiology, diagnostic workup, treatment recommendations, and research findings. I recommend this book to both practicing hematologists/oncologists and neonatologists and those who are training in these fields.