具体描述
This will be the fifth edition of successful clinical manual aimed at all practitioners treating children with cancer and blood diseases, such as leukemia, anemia, and bone marrow failure. As with previous editions, this edition will be a comprehensive book on patient management, replete with algorithms and flow diagrams on diagnosis and management. Its concise and easy-to-read format will enable readers to make accurate diagnoses and permit them to treat patients even if they have not had extensive previous hematologic or oncologic experience. It will contain a list of normal values at various ages in children, which is extremely useful as a reference in the management of patients. Since the previous edition was written five years ago, there have been considerable advances particularly in the treatment and management of oncologic diseases in children. These oncology sections (approximately 40% of the book) will be entirely rewritten to incorporate all current protocols. In addition, advances in many other areas will require that all hematology sections of the book be updated. The successful basic format and content of clinical manifestations, diagnosis and differential diagnosis will be maintained - as in previous editions, the editor will closely edit chapter content to maintain consistency in format, depth, and style. In addition to the core group of hematologists and oncologists from Schneider Children's Hospital, who contributed to the 4 th edition, the editor is inviting 15 new authors who are all experts from around the country - in order to keep up with the advances in management of hem/onc diseases. The book will reflect the 45+ years of practical experience by the editor as well as the extensive clinical experience and research expertise of the various contributors.
Feature Offers a concise, systematic approach to all pediatric hematologic and oncologic disorders in one reference manual Easy-to-read format: multiple tables, charts, and flow-diagrams for diagnosis and management of pediatric hematologic and oncologic disorders Clear presentation by practicing clinicians, who are also academic researchers, of recent developments in molecular genetics, cytogenetics, immunology, transplantation, and biochemistry to the extent that they have bearing on clinical practice Benefits Practitioners responsible for the care of children with hem/onc diseases will save time going to one concise, authoritative source for diagnosis and treatment options, without having to wade through all the details in larger tomes on hematology or oncology, or in thousands of journal articles Residents will use it as a guide to prepare for board examinations in pediatric hematology/oncology, pediatrics, hematology, and oncology Practicing pediatricians, hematologists, oncologists, fellows, and pediatric nurses will use it a quick source for a second opinion in diagnosis and disease management when they are unable to consult with a pediatric hem/onc specialist Practicing pediatric hem/onc specialists will use the book as a "refresher course" and will appreciate the systematic approach For all practitioners, correct diagnosis (and therefore correct treatment) of oncologic and hematologic diseases depends on a strong understanding of the molecular basis for the disease
Pediatric Nephrology: A Comprehensive Clinical Guide, Third Edition Description: Pediatric Nephrology: A Comprehensive Clinical Guide, Third Edition stands as the definitive, authoritative resource for the diagnosis, management, and ongoing care of infants, children, and adolescents with renal disorders. This meticulously updated edition, crafted by world-renowned experts in the field, synthesizes the latest advancements in molecular biology, genetics, diagnostic imaging, and therapeutic modalities, providing a practical framework grounded in the most current evidence-based practice. This textbook moves beyond basic descriptions to offer in-depth, clinically relevant coverage across the entire spectrum of pediatric nephrology. It is designed for busy pediatricians, nephrology fellows, pediatric residents, pediatric intensivists, and allied healthcare professionals who require immediate access to precise, actionable information concerning complex kidney diseases in the young population. Structure and Content Highlights: The Third Edition is structured logically, moving from foundational physiology to complex pathological states and specialized management protocols. Part I: Foundations of Pediatric Nephrology This section establishes the essential framework for understanding renal function in health and disease across developmental stages. Developmental Nephrogenesis and Maturation: A detailed exploration of the embryonic formation of the kidney, the establishment of nephron populations, and the physiological changes in renal function from the perinatal period through adolescence. Emphasis is placed on understanding the developmental trajectory and how insults during critical windows lead to lifelong susceptibility to disease. Renal Physiology in Health and Disease: Comprehensive review of glomerular filtration, tubular transport mechanisms, acid-base homeostasis, and electrolyte balance, including updated insights into the molecular pathways governing these processes. New chapters address the complexities of fluid balance in the critically ill neonate and the long-term implications of early renal insults on adult cardiovascular health. Diagnostic Modalities and Imaging: A thorough guide to the modern diagnostic armamentarium. This includes advanced interpretation of urinalysis, serum markers, radionuclide scans, and high-resolution ultrasound and magnetic resonance angiography (MRA) tailored for pediatric use. Protocols for safe contrast administration and management of nephrotoxicity in sensitive populations are rigorously detailed. Part II: Glomerular Diseases This major section offers unparalleled depth in the evaluation and management of primary and secondary glomerulopathies. Congenital Nephrotic Syndrome and Steroid Resistance: Comprehensive review of genetic causes, including nuances in diagnosing NPHS1, NPHS2, and other gene mutations. Management strategies emphasize individualized immunosuppressive regimens and the role of potent non-steroid agents in refractory cases. Acute Post-infectious Glomerulonephritis (APIGN) and Progressive Nephropathies: Detailed clinical pathways for managing post-infectious syndromes, including atypical presentations. Focus is given to rapidly progressive glomerulonephritis (RPGN), including the appropriate timing and dosing for aggressive immunosuppression (e.g., cyclophosphamide, mycophenolate mofetil) and plasma exchange protocols. Hereditary Nephropathies: Exhaustive coverage of Alport Syndrome, including the latest understanding of COL4A3/4/5 mutations, and their phenotypic variability. Management protocols stress monitoring for cochlear and ocular manifestations and the role of RAAS blockade in preserving renal function. IgA nephropathy and thin basement membrane disease are addressed with pragmatic guidelines for biopsy thresholds and treatment initiation based on proteinuria levels and histology. Part III: Disorders of Tubular Function and Electrolyte Imbalances This critical section provides step-by-step guidance for managing complex tubular acidosis and salt-wasting disorders. Renal Tubular Acidosis (RTA): Detailed differentiation between Type 1 (Distal), Type 2 (Proximal), and Type 4 (Hyperkalemic) RTA, including the specific challenges of managing associated nephrocalcinosis and bone disease. Practical tables detail bicarbonate replacement protocols tailored to the child's weight and severity of acidosis. Salt-Wasting Syndromes: Definitive management protocols for Bartter Syndrome, Gitelman Syndrome, and Congenital Chloride Diarrhea. The text provides clinical algorithms for distinguishing these entities based on urine electrolyte profiles and discusses the long-term use of NSAIDs or potassium-sparing diuretics where appropriate. Disorders of Water Homeostasis: In-depth examination of Diabetes Insipidus (central vs. nephrogenic) and the Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH). Management algorithms emphasize titrated vasopressin receptor agonists (vaptans) for controlled water diuresis in select populations, underscoring safety precautions in pediatric critical care. Part IV: Pediatric Acute Kidney Injury (AKI) This section is entirely reorganized to align with the KDIGO criteria adapted for pediatrics. Prerenal, Intrinsic, and Postrenal Causes: Detailed etiology and recognition of AKI across neonatal, infant, and older child populations. Particular attention is paid to volume status assessment in septic shock, the management of AKI secondary to congenital heart disease, and the pitfalls of relying solely on serum creatinine in high-output states. Management of Volume Overload and Hyperkalemia: Practical, evidence-based approaches to diuresis, ultrafiltration, and the immediate medical management of life-threatening hyperkalemia using calcium gluconate, insulin/dextrose, and cation-exchange resins. Renal Replacement Therapy (RRT) in Pediatrics: An extensive review of indications, timing, and modality selection for RRT in the pediatric intensive care unit. This includes detailed schematics for initiating Continuous Renal Replacement Therapy (CRRT)—including CVVHD and CAVHDF—and specifying appropriate anticoagulation strategies (e.g., citrate vs. heparin) for small-volume circuits. Part V: Chronic Kidney Disease (CKD) and Transplantation This final section addresses the long-term care continuum. Etiology and Progression of CKD: Focuses on obstructive uropathy, reflux nephropathy, and chronic glomerulonephritis as leading causes. Emphasis is placed on optimizing blood pressure control using advanced RAAS blockade combinations and managing anemia with erythropoiesis-stimulating agents (ESAs) and iron supplementation guided by modern guidelines. Nutritional Management and Bone/Mineral Disorder (CKD-MBD): Comprehensive dietary guidelines for protein restriction, phosphate control, and Vitamin D metabolite management in CKD stages 3–5. Detailed charts outline appropriate calcium/phosphate binders, including the proper titration of sevelamer and lanthanum carbonate in children. Renal Transplantation in Children: A state-of-the-art overview covering donor evaluation, surgical principles, induction and maintenance immunosuppressive protocols (including mTOR inhibitors and costimulation blockade), and the management of long-term complications such as polyomavirus BK nephropathy and de novo donor-specific antibodies (DSA). Protocols for managing obesity and long-term cardiovascular risk in pediatric transplant recipients are highlighted. Key Features of the Third Edition: Genetics Integration: Incorporated genetic testing algorithms for hereditary nephropathies and congenital anomalies of the kidney and urinary tract (CAKUT). Consensus Guidelines: Direct incorporation of current major society guidelines (e.g., KDIGO, ESPN/ERA-EDTA) into clinical pathways. Procedural Guidance: High-quality illustrations and procedural descriptions for renal biopsy interpretation and central venous access for CRRT. Focus on Transition: Dedicated chapters addressing the transition of care from pediatric subspecialists to adult nephrology services, addressing issues specific to young adults with chronic conditions. Pediatric Nephrology: A Comprehensive Clinical Guide, Third Edition ensures that clinicians possess the most current knowledge base required to deliver optimal, evidence-informed care to every child facing a kidney disorder.
