LEADER 13929nam 22009135 450 001 9910484302503321 005 20200702131549.0 010 $a1-280-38450-6 010 $a9786613562425 010 $a3-540-76341-4 024 7 $a10.1007/978-3-540-76341-3 035 $a(CKB)1000000000772837 035 $a(SSID)ssj0000319375 035 $a(PQKBManifestationID)11253347 035 $a(PQKBTitleCode)TC0000319375 035 $a(PQKBWorkID)10338519 035 $a(PQKB)10845919 035 $a(DE-He213)978-3-540-76341-3 035 $a(MiAaPQ)EBC3064428 035 $a(MiAaPQ)EBC6314981 035 $a(MiAaPQ)EBC5576591 035 $a(Au-PeEL)EBL5576591 035 $a(OCoLC)1066191362 035 $a(PPN)139950028 035 $a(EXLCZ)991000000000772837 100 $a20100301d2009 u| 0 101 0 $aeng 135 $aurnn|008mamaa 181 $ctxt 182 $cc 183 $acr 200 10$aPediatric Nephrology$b[electronic resource] /$fedited by Ellis D. Avner, William E. Harmon, Patrick Niaudet, Norishige Yoshikawa 205 $a6th ed. 2009. 210 1$aBerlin, Heidelberg :$cSpringer Berlin Heidelberg :$cImprint: Springer,$d2009. 215 $a1 online resource (eReference.) 300 $aPrevious ed.: Philadelphia, Pa.: Lippincott Williams & Wilkins, 2003. 300 $aTwo volumes combined as one. 311 $a3-540-76344-9 311 $a3-540-76327-9 320 $aIncludes bibliographical references and index. 327 $aIntro -- Preface -- Table of Contents -- Embryology -- Introduction -- Anatomy of Kidney Development -- Overview -- Pronephros and Mesonephros -- Metanephros -- The Ureter and Urinary Bladder -- Methods Used to Study the Biology of Nephrogenesis -- Descriptive Studies of Gene Expression -- Functional Studies In Vitro -- Functional Studies In Vivo -- Cell Biology of Nephrogenesis -- Cell Proliferation and Cell Death -- Differentiation -- Morphogenesis -- Molecular Control of Nephrogenesis -- Overview -- Transcription Factors -- Growth Factors -- Adhesion Molecules -- Conversion of Metanephric Mesenchyme into Nephron Epithelia -- Uninduced Metanephric Mesenchyme Is Preprogrammed to Form Nephrons -- Uninduced Metanephric Mesenchyme Is Preprogrammed to Die -- Condensation of Renal Mesenchyme -- Morphogenesis into Nephron Tubules -- Formation of the Glomerulus -- Branching Morphogenesis of the Ureteric Bud -- Growth Factor Control of Ureteric Bud Growth -- Transcription Factors and Ureteric Bud Growth -- Cell Adhesion Molecules and Ureteric Bud Growth -- Further Maturation of the Collecting Ducts -- Metanephric Stromal Cells -- Lower Urinary Tract Development -- References -- Glomerular Circulation and Function -- Renal Blood Flow -- Development of Renal Blood Flow -- Prenatal Renal Blood Flow -- Postnatal Renal Blood Flow -- Measurement of Renal Blood Flow -- Indirect Assessment of Renal Blood Flow -- Glomerular Filtration -- Theoretical Considerations of Glomerular Filtration -- Effect of Perturbation in the Determinants of SNGFR -- Mean Glomerular Transcapillary Hydraulic Pressure Difference, deltaP -- Systemic Plasma Colloid Osmotic Pressure, piA -- Glomerular Plasma Flow Rate, QA -- Glomerular Capillary Ultrafiltration Coefficient, Kf -- Defense of Glomerular Filtration Rate -- Myogenic Reflex -- Tubuloglomerular Feedback (TGF). 327 $aDevelopment of Glomerular Filtration Rate -- Ontogenic Development of Glomerular Capillaries -- Prenatal GFR -- Postnatal GFR -- Impaired Fetal Glomerular Development and Adult Disease -- Low Birth Weight -- Extreme Prematurity -- Maternal Factors -- Clinical Assessment of Glomerular Filtration Rate -- Inulin Clearance -- Modifications of the Standard Clearance Method -- Creatinine as a Marker of GFR -- Cystatin C as a Marker of GFR -- Other Endogenous Markers of GFR -- Glomerular Sieving of Macromolecules -- Role of Endothelial Cells -- Role of Glomerular Basement Membrane -- Role of Podocytes -- Role of the Renin Angiotensin System -- References -- Renal Tubular Development -- Organization of the Nephron -- Principals of Membrane Transport -- Maturation of Na+-K+-ATPase along the Nephron -- Proximal Tubule Transport -- Glucose Transport -- Amino Acid Transport -- Organic Acid Transport -- Phosphate Transport -- Proximal Tubule Acidification -- Proximal Tubule NaCl Transport -- Proximal Tubule Water Transport -- Thick Ascending Limb -- Distal Convoluted Tubule -- Urinary Concentration and Dilution -- Distal Tubule Acidification -- Cortical Collecting Tubule Sodium Transport -- Potassium Transport -- References -- Perinatal Urology -- Diagnosis -- Diagnostic Accuracy -- Ureteropelvic Junction Obstruction -- Cystic Kidneys -- Ureterovesical Junction Obstruction -- Duplication Anomalies -- Vesicoureteral Reflux -- Posterior Urethral Valves -- Rationale for Prenatal Intervention -- Clinical Experience with Intervention for Prenatal Hydronephrosis -- Postnatal Management of Infants with Prenatally Diagnosed Urologic Renal Abnormality -- Unilateral Hydronephrosis -- Bilateral Hydronephrosis -- Renal Agenesis, Renal Ectopia and Unilateral Multicystic Dysplastic Kidney -- Summary -- Acknowledgment -- References -- Renal Dysplasia/Hypoplasia -- Introduction. 327 $aRenal Agenesis -- Bilateral Renal Agenesis (BRA) -- Unilateral Renal Agenesis (URA) -- Multicystic/Dysplastic Kidney (MCDK) -- Abnormalities of Kidney Position and Patterning -- Primary Renal Hypoplasia -- Renal Coloboma Syndrome -- Branchio-Oto-Renal Syndrome -- Renal Tubular Dysgenesis (RTD) -- Renal Dysplasia/Hypoplasia and Vesico-Ureteral Reflux -- Renal Hypoplasia/Dysplasia and Fetal Urinary Tract Obstruction -- Syndromic Renal Hypoplasia/Dysplasia -- Genetic and Environmental Causes of Subtle Renal Hypoplasia -- References -- Syndromes and Malformations of the Urinary Tract -- Introduction -- Prevalence of Urinary Tract Anomalies -- Approach to the Child with a Urinary Tract Anomaly -- Overview of Normal Embryogenesis of the Urinary System -- Anomalies Involving the Urinary Tract -- Kidney Defects -- Renal Agenesis -- Ectopic Kidney -- Horseshoe Kidney -- Dysplasia and Polycystic Kidney -- Obstruction and Hydronephrosis -- Ureter Defects -- Duplication -- Hydroureter -- Bladder Defects -- Bladder Exstrophy -- Urethral Defects -- Agenesis and Atresia -- Duplication -- Posterior Urethral Valves -- Associations and Sequences Involving the Urinary Tract -- VATER Association -- CHARGE Syndrome (CHARGE Association) -- MURCS Association -- Oligohydramnios Sequence -- Urethral Obstruction Sequence -- Sirenomelia Sequence -- References -- Sodium and Water -- Introduction -- Body Fluid Compartments and their Composition -- Total Body Water and its Compartments -- Composition of Body Water Compartments -- Maintenance Sodium and Water Requirements -- Sodium -- Water -- Distinct Roles of Sodium and Water in Body Fluid Homeostasis -- Sensor Mechanisms: Sodium and Water -- Sodium -- Atrial Receptors -- Hepatic Receptors -- Pulmonary Receptors -- Carotid Arch Receptors -- Cerebral Receptors -- Water -- AVP -- Efferent Mechanisms: Sodium and Water -- Sodium. 327 $aRenin-Angiotensin-Aldosterone Axis -- Endothelin -- Renal Nerves -- Atrial Natriuretic Peptide -- Prostaglandins -- Kinins -- Nitric Oxide -- Adrenomedullin -- Water -- AVP -- Angiotensin -- Thirst -- Effector Mechanisms: Sodium and Water -- Sodium -- GFR -- Proximal Tubule -- Distal Nephron Including Collecting Duct -- Water -- AVP -- Countercurrent Mechanism -- Osmoprotective Molecule (Compatible Osmolytes) -- Laboratory Assessment of Sodium and Water Balance -- Sodium -- Water -- Overview of the Evaluation of Fluid and Water Abnormalities -- Sodium Balance Disturbances: Deficit and Excess -- Sodium Deficit -- Diagnosis and Evaluation -- Treatment -- Sodium Excess -- Diagnosis and Evaluation -- Therapy -- Water Balance Disturbances: Deficit and Excess -- Hyponatremia -- Diagnosis and Evaluation -- Treatment -- Hypernatremia -- Diagnosis and Evaluation -- Treatment -- Acknowledgements -- References -- Potassium -- Potassium Homeostasis -- Regulation of Internal Potassium Balance -- Plasma Potassium Concentration -- Hormones -- Acid-Base Balance -- Other Factors -- Regulation of External Potassium Balance -- Renal Contribution -- Sites of Potassium Transport along the Nephron -- Proximal Tubule -- Thick Ascending Limb of the Loop of Henle (TALH) -- Distal Nephron -- Potassium Secretion -- Potassium Absorption -- Developmental Regulation of Distal Potassium Transport -- Luminal and Peritubular Factors Affecting Potassium Transport -- Sodium Delivery and Absorption -- Tubular Flow Rate -- Potassium Intake and Cellular Potassium Content -- Hormones -- Acid-Base Balance -- Contribution of the Gastrointestinal Tract -- References -- Acid-Base Homeostasis -- Introduction -- Buffering Systems -- Renal Acidification -- Proximal Mechanisms -- Na+/H+ Exchanger (NHE) -- Na+/K+ ATPase -- Carbonic Anhydrase (CA) -- Na+/HCO3-Cotransporter (NBC). 327 $aDistal Mechanisms -- Loop of Henle and Thick Ascending Loop (TAL) -- Collecting Duct -- Genetics of Renal Tubular Acidosis (RTA) -- Inherited Distal Renal Tubular Acidosis -- Inherited Proximal Renal Tubular Acidosis -- Genetics of Metabolic Alkalosis -- Physiologic Response to Nonvolatile Acid Loads -- Physiologic Response to Alkaline Loads -- Acid-Base Map -- Physiologic Response to Changes in Carbon Dioxide Tension -- Molecular and Cellular Renal Regulation in Acid-Base Disorders -- Concept of Serum Anion Gap -- Concept of Urine Anion Gap -- Nutrition and Acid-Base Balance -- Acid-Base Balance in Neonates, Infants and Children -- Mechanisms of Growth Retardation in Chronic Acidosis -- Effect of Acidosis on Bone and Calcium Homeostasis -- References -- Calcium and Phosphorus -- Calcium -- Calcium Homeostasis -- Calcium Distribution in the Body -- Calcium Chemistry -- Extracellular Calcium Homeostasis -- Calcium-Sensing Receptor -- Vitamin D -- Transepithelial Calcium Transport -- Calcium Entry -- Transcytoplasmic Calcium Movement -- Calcium Exit -- Renal Calcium Transport -- Physiology and Tubular Localization -- Regulation of Renal Calcium Transport -- Phosphorus -- Phosphorus Homeostasis -- Phosphorus Distribution in the Body -- Phosphate Chemistry -- Extracellular Phosphate Homeostasis -- Intestinal Phosphate Absorption -- Cellular Aspects -- Regulation -- Molecular Mechanisms -- Renal Phosphate Transport -- Physiology and Tubular Localization -- Cellular and Molecular Aspects -- Regulation of Renal Phosphate Transport -- References -- Genetic Disorders of Calcium and Phosphate Homeostasis -- Introduction -- Regulators of Calcium and Phosphate Homeostasis -- Parathyroid Hormone (PTH) and PTH-related Peptide (PTHrP) -- Vitamin D and Its Metabolites -- Fibroblast Growth Factor 23 (FGF-23) and Other Proteins with Phosphaturic Properties. 327 $aHypercalcemia and Hypophosphatemia due to Increased Parathyroid Gland Activity. 330 $aThrough 5 editions, Pediatric Nephrology has become the standard reference text for students, trainees, practicing physicians (Pediatricians, Nephrologists, Internists, and Urologists), sub-specialists, and allied health professionals seeking information about Children?s Kidney Diseases. It is global in perspective and reflects the international group of editors, who are well-recognized world?s experts in Pediatric Nephrology. In a single text, the development of kidney structure and function is followed by detailed and comprehensive chapters on all childhood kidney diseases. These chapters, grouped by major disease categories, utilize molecular and cellular pathophysiology of disease to provide unique and comprehensive information of the current state of the art on all known childhood kidney diseases. Each chapter makes new genetic information easily understandable for the practitioner, and uses many algorithms and diagrams to describe appropriate clinical evaluation of symptoms, differential diagnosis, specific diagnostics, and currently available therapies. 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