Manco-Johnson chemotherapy 90 Brain tumors of the posterior fossa, P. Langmuir; A. Meadows brain stem and visual pathway S. Bailey Monitoring for late effects in Malignant Disorders children with malignancies 92 Initial management of a child with A. Meadows; W. Hobbie 74 Assessment of a child with a tumor involving or near the suspected leukemia spinal cord Useful normal laboratory values P. Hann S.
Bailey R. Sills 76 Assessment of a mediastinal mass 94 Recognition and management of M. Pearce tumor lysis syndrome S. Bailey; R. The authors and the publisher have ex- All rights reserved. However, in view of ongoing research, changes in by any information storage and retrieval system, with- ISBN 3———0 spiral bound: alk.
Pediatric hematology. Cancer in children. Sills, Richard H. Hematologic Diseases — diagnosis — Child. Karger AG, P. Box, 2. Neoplasms — diagnosis — Child.
Decision Trees. This is particularly CH— Basel Switzerland 4. Diagnosis, Differential. WS P ] important when the recommended agent is a new www.
Printed in Switzerland on acid-free paper by Sills, MD C. Heeney, MD Anna T. I lem solving. In reading the final prod- individually. Decision trees prepared would still refer my patients to a spe- uct, written by some of the finest in advance have the disadvantage of cialist with many of the diagnoses, pediatric hematologist-oncologists in unacquaintedness with the individual symptoms and signs discussed here.
Yet, for the physician who is But, with the help of this outstanding Richard Sills, it is obvious that the less experienced with a given prob- book, I would refer them after an edu- spirit of the algorismus has been uti- lem, a prepared algorithm would pro- cated initial workup, and would be lized to its best. Each algo- and cost-effective fashion. Practical agement of conditions such as sickle rithm must be used in the context of Algorithms in Pediatric Hematology cell anemia, hemophilia and red blood the individual findings of each patient and Oncology uses this approach to cell transfusions.
The The clinician must always be aware malignancies. To provide a founded by concomitant disorders or problems on a daily basis as well as better sense of which diagnoses are complications, to render our aproach- residents and trainees in Pediatrics more likely, very common diagnoses es invalid.
In addition, advances in di- and Pediatric Hematology and Oncol- causing each problem are noted in agnosis and management can render ogy. In addressing oncologic problems, of common diagnoses in standard We hope you will find the book our goal is to efficiently determine font and rare diagnoses in italics. No helpful in managing the children un- whether children have malignant or algorithm can contain every possible der your care.
Details of specific not included while others may be list- therapeutic regimens for malignant ed in the algorithm but not the text. Richard H. Sills, MD disorders are not addressed because Cross-references to other algorithms they should be determined individual- make the book easier to use.
An ap- ly in consultation with a pediatric on- pendix of age-dependent normal val- cologist. Algorithms also address the ues and a convenient list of all abbre- My thanks to all the students, management of complications which viations used are also provided.
I dedicate this book to the memory of my father, Sidney Sills. Red Cell Disorders R. While some specific some cell lines may fall below the normal disorders can present with these hematologic diagnoses are discussed here, most will be range before others; however, if one cell line is findings. Specific serologic studies may be found in the seven other algorithms, which are severely affected, the others are usually ap- indicated.
The reticulocyte ANC obtained automatically with the Hb. With count is usually very low. It also affected. Anemia and platelet count are normal. The peripheral smear should be associated with either severe malnutrition or and usually thrombocytopenia. If blasts are in the peripheral blood, cytopenia. Thrombocytosis is a very thrombocytopenic, complicating DIC should Emedicine. Prima- cause of anemia with thrombocytopenia or of the complete blood count.
The abnormalities are more likely Pediatr Clin N Am ;— Br J Hosp Med ;— Consider HIV with positive risk factors, other symptoms and failure to resolve. Hb levels can be as high deposition in marrow erythroblasts. Protein and particularly in adolescent girls. An under- as However, of metabolic defects of Fe absorption and Smear reveals hypochromia, microcytosis, and small numbers of normoblasts may be seen in metabolism, but they are very rare.
A dimorphic population severe anemia of any etiology. Blood ;— Pediatrics ;— If there is no response in E-thalassemia and sickle thalassemia are asso- Pediatr Clin N Am ;— The etiology of the Fe target erythrocytes. J Pediatr ;— Hb stability studies can relationships and evidence for a threshold.
Am J Publ Hlth ;80;— Pb poisoning itself; anemia is a late sign of Pb poisoning. A dimorphic trauma. Chronic GI blood loss may be occult often associated with severe normocytic population of normal and hypochromic RBCs and melena may not be recognized as signifi- anemia and an elevated RDW are usually evident and cant; stools should be examined for occult a trial of oral Fe is often diagnostic.
Typically, pallor is noted without common. Bilirubin should be normal except the child since it might be due to an unrecog- are still expected as is a high RDW reflecting in neonates with enclosed hemorrhage in nized viral illness. If the anemia is more severe marked anisocytosis variation in cell size.
Smear usually reveals or there are concerning clinical signs, a bone The potential irreversible neurologic damage polychromasia from the reticulocytosis, and marrow aspirate and biopsy should be done. A associated with B12 deficiency makes it critical a dimorphic population in early or partially concerning laboratory finding is myelophthisic to consider this diagnosis.
J Pediatr ; Other acquired etiolo- Abshire TC: Anemia of inflammation. Fluid overload in the absence of disorder. Drug-induced RBC aplasia occurs Blood ;— If the anemia is mild e. It findings include myelodysplastic syndromes, Congenital bone marrow failure syndromes. Curr Opin Pediatr ;— Warming the other etiologies of macrocytosis. Hypersegmented neutrophils or congenital dyserythropoietic anemia , or acquired myelo- sample normalizes the MCV.
In the absence of malnutrition, megaloblas- cations and folate levels should be obtained if an obvious imal anemia, can alter RBC size. Normal neonates are often tic anemia is uncommon in children; no cases of folate or B12 etiology for the macrocytosis is not identified. Drugs can cause isolated because of macrocytosis.
Serum B12 levels are usually diagnostic. In these in- anemia, but may also be due to ileal disease, malabsorption, stances, the RBCs have a larger surface area causing them to vegan diet, and fish tapeworm.
Most are sociated with an actual increase in measured volume MCV. Associated congenital malformations are common. TEC is not a Selected reading patients because of its potential irreversible neurologic macrocytic process unless recovery is occurring with a reticu- Bessman JD, Banks D: Spurious macrocytosis: damage. Combined deficiency occurs, so even if folate levels locytosis. A common clue to erythrocyte cold agglutinins. Treatment of Am J Clin Pathol ;— If the diagnosis is not certain, normal logic changes.
Marrow hypoplasia is volume and hematocrit caused by hyperglycemia. The most Lindenbaum J: Status of laboratory testing in the utilized diagnostic finding is enhanced chromosomal break- diagnosis of megaloblastic anemia.
Blood ; —— Folate deficiency can be due to drugs e. Ann Intern Med ; — The pathogenesis is not always clear, but it is likely due roblastic anemia. MDS is diagnosed on the basis of myelo- diseases and therapies. The drugs dysplastic changes in the marrow; the finding of a clonal 11 most commonly involved are chemotherapeutic agents e.
Dyserythropoietic changes suggest the rare di- Vitamin B12 deficiency in children and adolescents. Sideroblastic J Pediatr ;— This is usually due loblastic anemia.
The simplest initial screen for megaloblas- neutrophil count is decreased neutropenia , the patient to malignant replacement but is also caused by benign tic anemia in the macrocytic patient is to review the periph- should still be considered pancytopenic. Bone marrow examination patients with megaloblastic anemias. The combination of should be performed. Unless in which aplastic anemia develops later in the first decade. RBC folate are more accurate than serum levels. It may occur in isolation or in association with collagen vascular disorders, particularly SLE.
Examples drug-induced changes e. Splenomegaly is and Legionaires disease. It can also cause varying very rarely, PNH. Myelodysplasia is an acquired clonal disorder of the Schwartz CL, Cohen HT: Preleukemic syndromes Ham test should be replaced by specific flow cytometric bone marrow characterized by abnormal maturation of one and other syndromes predisposing to leukemia. Chromosomal analysis Pediatr Clin North Am ;— Leukoerythroblastosis myelophthisic anemia It is very rare in children but when it occurs it is usually in is less common in children and usually results from bone toddlers with trisomy 21 and represents a form of acute marrow invasion.
Anemia may be accompanied by several megakaryoblastic leukemia. Pallor is usu- mia in sick neonates. The nization. Infection is of- are icteric and some may require exchange transfusion. One-half of the Anemia is frequent in the neonate but does not predict with jaundice. When assessing whether a neonate is ane- newborns with toxoplasmosis have anemia, which may be disease severity later in life. The family history will be nega- mic, consider that capillary samples can average 3.
Hb also varies ciated with mild anemia. Bacterial infection, whether com- with age and with gestational age. In the first few days of life, nucleated erythrocytes Shock, regardless of etiology, can trigger DIC.
Progressive hemolytic anemia is common sults are common in mild variants during a reticulocytosis. Ensure that the peat the screen once the reticulocyte is normal. Maternal blood type should is not continuing. The anti- immediately, the MCV will be normal and a reticulocytosis populations. Maternal nal blood. Frequent spherocytes suggest hereditary spherocytosis pulmonary hemorrhage. Iatrogenic blood loss phlebotomy, or ABO incompatibility. Hereditary elliptocytosis and hered- accidents with catheters should be considered.
The Apt itary stomatocytosis are easily recognized by a large num- Selected reading test differentiates neonatal gastrointestinal hemorrhage ber of these cells in peripheral blood. Bleeding disorders, such locytosis presents with microcytosis and bizarrely shaped, 15 newborn infants. Clin Perinatol ;— The lower limit of in the neonate. It is most often caused by Selected reading normal in cord blood at term is 98, 95 in the neuroblastoma and congenital leukemia, but is Blanchette VS, Zipurskey A: Assessement of first 3 days from capillary samples and 88 at also seen with Langerhans cell histiocytosis anemia in newborn infants.
Clin Perinatol 1 week of age. Philadelphia, Saunders, Note that hydrops fetalis which will be clinical- in life. Clin Perinatol ; seen in people of African descent. Bone marrow aspirate reveals recombinant erythropoietin as treatment for cytosis.
It is most often due to feto-maternal virtual absence of erythroid precursors. Other the anemia of bronchopulmonary dysplasia. The reticulocyte count is often rare and include drug-induced RBC aplasia, decreased because iron deficiency inhibits Aase syndrome associated with skeletal ano- Quirolo K, Foote D, Vichinsky EP: Changing reticulocyte production.
Most often these are ill neonates mia. Initially, macrocytosis, then anemia, and and reticulopenia. This is particularly common finally pancytopenia develops.
B12 deficiency in infants with bronchopulmonary dysplasia. A number of rare metabolic defects, including transcobalamin II deficiency and orotic acid- uria, can cause megaloblastic anemia at birth or soon after. Macrocytosis is often not evident because of the relatively high normal MCV in newborns. Possible pre- present on red cells.
Past medical history should inquire ing paroxysmal cold hemoglobinuria. Know- about jaundice as a neonate or later. Attempt to find compatible angiopathic hemolytic process.
Family history should include an- possible. Most cases of autoimmune hemolytic diagnoses. Concomitant cardiac prosthesis as a cause of hemolysis. Hereditary spherocyto- uolization, visible bacteria. Note fever as a sign for intravascular hemoly- sis and elliptocytosis are usually autosomal- sis, acute infection or autoimmune disease.
Splenomegaly for splenomegaly. In hereditary spherocytosis, milder variants during a reticulocytosis. An increased reticulocyte count ideal- in autosomal-recessive diseases. Enhanced os- stem cell, characterized by a membrane pro- ly corrected for variation in RBC count sug- motic fragility usually confirms the diagnosis tein defect that renders red blood cells suscep- gests hemolysis, but a low or normal reticulo- of hereditary spherocytosis.
Membrane protein tible to damage by serum complement. Diag- cyte count occurs when a hypoplastic crisis studies are helpful in selected cases. Coombs nosis can be made by flow cytometry for GPI- complicates hemolytic anemia. Beutler E, Luzzatto L: Hemolytic anemia. Direct Coombs test detects antibodies on Semin Hematol ;36 suppl 7 — Philadelphia, serum. Coombs negative autoimmune he- Saunders, Red Cell Disorders A.
The following could be in- Nephrology, ed 4. Baltimore, for malaria. Prior transfusion fol- globin portion of Hb with acidic mucoproteins. Philadelphia, lowed by abdominal pain and hemoglobinuria 2 Renal ischemia due to concomitant release Saunders, Consider he- of vasoconstrictive substances. Blood Rev ;— Note fever as a sign of intravascular he- anuria from acute renal failure, peritoneal or management and unusual presenting molysis or acute infection.
Clin Lab Haematol ;— This class of will be positive for all three. In myoglobinuria and activated and hemolysis induced. PCH should hemoglobinuria, spun urine remains red. Classically, patients have intermittent episodes of dark urine, most commonly in the morning. This phenome- 21 non has been termed march hemoglobinuria and is caused by physical injury sustained by RBC in the affected blood vessels.
Rare unstable hemoglobins may be is usually noted within 3—7 days and an in- tion. Hb stability studies are diagnostic. TS is the percentage of saturation correction. Losses are most commonly due to gastro- with an underlying acute or chronic illness. Micro- Gross SJ, et al: Malabsorption of appropriate. Hb rarely iron in children with iron deficiency.
The quanti- the detection of iron in stools. Fe therapy does not result in Am J Med ;— Br J Haematol ;— This may not be valid for more severe forms of thalassemia. Moderate to severe Barts hydrops fetalis syndrome.
The occurs only in early embryonic development. These patients may develop tachypnea and extramedullary erythropoiesis e. Clinical severity is dependent on genotype. Age of diagnosis, usually in patosplenomegaly. Target cells, anisocytosis and lassemias depends on ethnic origin. In people of African de- sult in secondary hemosiderosis leading to dilatative car- poikilocytosis are seen in the peripheral blood smear. Therefore, iron chelation therapy with parenterally ad- ciency.
See the algorithms on 1 microcytic anemia, and 2 paired with a normal gene on each chromosome, more se- ministered deferoxamine is important for increased life ex- presumed iron deficiency anemia that fails to respond to vere disease HbH disease and Hb Barts hydrops fetalis syn- pectancy.
Deferriprone, which can be given orally, is used in iron. In patients who fail to comply with deferoxamine. Matched re- Southeast Asia, all genetic variants are found.
DNA analysis may be prenatal diagnosis when appropriate. These children are asympto- Lucarelli G et al: Bone marrow transplantation ence of Hb Barts, but in later childhood Hb analysis is usu- matic but have a mild microcytic anemia.
Mild elevations of in thalassemia: The experience of Pesaro. Childhood, ed 5. N Engl J Med ;— Patients of Hemoglobin. Other unusual phenotypes may require the using capillary blood blotted onto filter paper, and identifies a thy identified. Screening jor which will require intensive intervention , the more benign DPunjab, E or OArab have similar but often less severe clinical prob- methods vary in sensitivity and accuracy.
Sickle solubility tests entities of hereditary persistence of fetal hemoglobin HPFH ei- lems. Targeted screening of high- lulose acetate electrophoresis is inexpensive, and widely used not yet be detectable. Practitioners automation, speed, accuracy, reproducibility and the ability to ac- post-natally, with elevated HbF throughout life without signifi- should be familiar with the screening programs in their area.
HbFSA indicates diagnostic precision and genetic counseling. At a minimum it therapeutic. HbF, HbC, significantly different implications for treatment and follow-up. HbS, etc. Acta Paediat Suppl ; —6. Certain atypical results may require further elucidation with the size parental education, symptom recognition, and preventive J Pediatr ;— D, E, G.
Southeast Asian populations. These phenotypes can jor and are usually transfusion dependent. Accessed via www pathies. The lowed intermittently with blood counts. In high risk groups, this may represent Hb H dis- Revised January 9, There are no rapidly available children in the first 2 years of life. Most studies show administered immediately after obtaining the blood count laboratory tests on hand that can rule out all bacterial that the WBC count is not reliable for predicting sepsis in and the blood culture, but before taking the radiograph and infections such as pneumococcal bacteremia.
All febrile an individual child with SCD. However, among children waiting for the laboratory results. Lumbar puncture should children with SCD must be evaluated for serious bacterial 24 months of age or younger who have the highest inci- be performed on toxic children and those with signs of infection.
For children with HbSS younger than 5 years of dence of sepsis, the presence of leukopenia or extreme meningitis. Work-up and should be admitted and treated with parenteral antibiotics. The presence of a focus of into the CSF. Toxic patients or patients suspected of having must always be obtained. Chest X-ray should be included in infection e. If the patient is known circulating immature neutrophils.
Only if or suspected to have allergy to cephalosporin, clindamycin is mandatory for all infants, and older children with urinary the family is reliable can the patient be discharged home can be substituted. Documented sepsis should be treated symptoms, but therapy should not be delayed while await- with specific plan for out-patient follow-up.
The oncology topics emphasize diagnostic approach and management of complications, and not the details of chemotherapy. All algorithms are presented with a page of explanatory notes and a list of selected reading. Pediatricians, family physicians and general practitioners will find this book very helpful in managing problems in their practices. Series Editor: Z. Hochberg Author : Z. A Karger 'Publishing Highlights ' title Algorithms provide a logical, concise and cost-effective approach to medical reasoning: utilizing a concise, step-by-step approach based upon clues from the history, physical examination and laboratory studies, algorithms help avoid excessive unnecessary procedures and testing.
The 2nd, revised edition of Practical Algorithms in Pediatric Endocrinology deals with practical issues of child growth, puberty, diseases of the endocrine glands, sexual differentiation, as well as aberrations of water, electrolyte, mineral and carbohydrate metabolism.
Fifty clinical issues are covered by an algorithmic approach, breaking down long lists and tables of differential diagnosis into smaller, more manageable ones. Medical textbooks are mainly oriented by body systems, disease or diagnosis, yet practicing physicians are confronted with patients' complaints in the form of symptoms, physical signs or laboratory abnormalities, from which they are expected to reach a diagnosis and proceed with treatment.
This book is meant as a pragmatic text for use at the patient's bedside. It classifies common clinical symptoms and signs, laboratory abnormalities and issues of management as they present themselves in daily practice.
Special emphasis is given to new knowledge that has accumulated on the molecular pathophysiology and molecular genetics of various kidney diseases in order to deepen and strengthen the practical approach to common problems in pediatric nephrology.
Aimed at an audience of general and family practitioners, pediatricians and trainees who are not exposed on a day-to-day basis to pediatric nephrology problems, it provides a logical, concise and cost-effective approach from which they can profit and acquire medical reasoning.
Hochberg Author : R. Unlike adult gastroenterology, pediatric gastroenterology is characterized by developmental disorders; the approach to the same disease condition may therefore be widely different, and there is an increasing need from pediatric gastroenterologists and pediatricians for easy diagnostic tools. Algorithms provide a logical, concise and cost-effective approach to medical reasoning and help avoid excessive unnecessary procedures and testing.
Written by leading experts in the field of pediatric gastroenterology and surrounding fields, this book is aimed at an audience of general and family practitioners, pediatricians and trainees who are not exposed on a day-to-day basis to pediatric gastroenterology problems.
A state-of-the-art and concise guide to the clinical management of pediatric endocrine disorders, the second edition of the highly regarded Pediatric Endocrinology: A Practical Clinical Guide covers the most common and challenging conditions seen by practicing endocrinologists and primary care physicians, including growth, hypothalamic, pituitary, adrenal, thyroid, calcium and bone, and reproductive disorders, as well as metabolic syndromes.
Your enhanced eBook allows you to access all of the text, figures, and references from the book. Score: 4. Pediatric Endocrinology Author : M. Pediatric endocrinology expert Dr. Mark Sperling teams up with world-renowned authors to bring you up to date with the latest key developments in every area of the field, providing invaluable guidance on how your clinical decision making will be affected by today's technological and scientific advances.
The user-friendly text is enhanced by all-new online access to the complete contents of the book and more. Determine the best possible course for every patient with easy-to-follow algorithms in every clinical chapter. Stay up to date with today's hottest topics, including neonatal diabetes mellitus, Type II childhood diabetes, molecular endocrinology, and genetics.
Explore the impact of today's advances and challenges, including explosive growth in molecular biology, sophisticated imaging techniques, and an increase in both pediatric diabetes and obesity.
Access the fully searchable contents of the book and new self-assessment questions online at Expert Consult. Quickly access the information you need with a new, streamlined organization Concepts, Endocrine Disorders of the Newborn, Endocrine Disorders of Childhood and Adolescence, and Laboratory Tests and Imaging. Divided into seven sections, it covers all major endocrine problems in infants, children and adolescents, including growth, puberty, obesity, metabolic syndrome, type 1 and type 2 diabetes, fluid and electrolyte balance, hypoglycaemia, calcium metabolism, and sexual development disorders.
The final section discusses radiological and nuclear imaging. Written by experienced paediatric endocrinologists from Asia, USA, UK and South Africa, each topic features algorithms for evaluation and management, as well as diagrams and tables, to enhance learning. In a single, convenient volume, Pediatric Endocrinology offers complete coverage of all aspects of basic science and clinical practice, ideal for both pediatricians and endocrinologists.
Pediatric endocrinology expert Dr. Mark Sperling teams up with world-renowned authors to bring you up to date with the latest key developments in every area of the field, providing invaluable guidance on how your clinical decision making will be affected by today's technological and scientific advances. The user-friendly text is enhanced by all-new online access to the complete contents of the book and more. Determine the best possible course for every patient with easy-to-follow algorithms in every clinical chapter.
Stay up to date with today's hottest topics, including neonatal diabetes mellitus, Type II childhood diabetes, molecular endocrinology, and genetics. Explore the impact of today's advances and challenges, including explosive growth in molecular biology, sophisticated imaging techniques, and an increase in both pediatric diabetes and obesity.
Access the fully searchable contents of the book and new self-assessment questions online at Expert Consult. Quickly access the information you need with a new, streamlined organization Concepts, Endocrine Disorders of the Newborn, Endocrine Disorders of Childhood and Adolescence, and Laboratory Tests and Imaging. Focuses on the major endocrine disorders found in clinical practice. Each chapter begins with a discussion of normal physiology, providing normative clinical standards and normal lab values for each disorder.
Separate sections present information on disorders of hormone deficiency, hormone resistance, and hormone excess. Tables in subchapters inform the reader about diagnosis, laboratory and radiologic studies, therapies, and when to refer to a specialist. Key points at the end of each chapter highlight vital information. An ideal resource for both pediatricians and endocrinologists, Sperling's Pediatric Endocrinology, 5th Edition, brings you fully up to date with accelerating research; new discoveries in metabolic, biochemical and molecular mechanisms; and the resulting advances in today's clinical care.
The editorial team of world-renowned pediatric endocrinologists led by Dr. Mark Sperling, as well as expert contributing authors, cover comprehensive and current aspects of both basic science and clinical practice. Whether you're preparing for certification or have extensive clinical experience, this detailed, authoritative reference helps you increase your knowledge and determine the best possible course for every patient. Delivers trusted guidance in every area of the field: including Endocrine Disorders of the Newborn, Endocrine Disorders of Childhood and Adolescence, and Laboratory Tests.
Features new topics such as transgender issues in children and adolescents and endocrinology of pregnancy, the fetus and the placenta.
Offers expert coverage of hot topics such as disorders of sexual development, molecular basis of endocrine disorders, hypoglycemia in newborns and infants; neonatal and other monogenic forms of diabetes; Type I and Type II diabetes and their treatment with new insulins together with the progress in an artificial pancreas and new medications for T2DM in adolescents; the obesity epidemic and role of bariatric surgery; and advances toward personalized medicine.
Includes easy-to-follow algorithms and numerous quick-reference tables and boxes in every clinical chapter, plus interactive questions online for self-assessment. Offers state-of-the-art information and fresh perspectives from new and award-winning authors in such areas as disorders of growth, multiple endocrine tumors, and puberty and its disorders in girls and boys.
Enhanced eBook version included with purchase. Your enhanced eBook allows you to access all of the text, figures, and references from the book on a variety of devices. The body of knowledge in most medical specialties is rapidly expanding, making it virtually impossible to follow all advances in clinical and basic sciences that are relevant to a given field. This is particularly true in pediatric endocrinology, at the cross-road of pediatrics, endocrinology, development and genetics.
0コメント