LEADER 03156nam 2200577Ia 450 001 9910454123703321 005 20200520144314.0 010 $a1-281-93082-2 010 $a9786611930820 010 $a0-19-156796-5 035 $a(CKB)1000000000707170 035 $a(EBL)415950 035 $a(OCoLC)437096379 035 $a(SSID)ssj0000223897 035 $a(PQKBManifestationID)11185384 035 $a(PQKBTitleCode)TC0000223897 035 $a(PQKBWorkID)10204923 035 $a(PQKB)10158273 035 $a(MiAaPQ)EBC415950 035 $a(Au-PeEL)EBL415950 035 $a(CaPaEBR)ebr10273017 035 $a(CaONFJC)MIL193082 035 $a(EXLCZ)991000000000707170 100 $a20080926d2009 uy 0 101 0 $aeng 135 $aur|n|---||||| 181 $ctxt 182 $cc 183 $acr 200 04$aThe politics of aid$b[electronic resource] $eAfrican strategies for dealing with donors /$fedited by Lindsay Whitfield 210 $aOxford ;$aNew York $cOxford University Press$d2009 215 $a1 online resource (422 p.) 300 $aDescription based upon print version of record. 311 $a0-19-956017-X 320 $aIncludes bibliographical references and index. 327 $aContents; Foreword; Preface; List of Abbreviations; Notes on Contributors; Introduction: Aid and Sovereignty; 1. Negotiating Aid; 2. Aid-Recipient Sovereignty in Historical Context; 3. Understanding Contemporary Aid Relationships; 4. Botswana: The African Success Story; 5. Ethiopia: Retaining Sovereignty in Aid Relations; 6. Rwanda: Milking the Cow. Creating Policy Space in Spite of Aid Dependence; 7. Ghana: Breaking Out of Aid Dependence? Economic and Political Barriers to Ownership; 8. Mali: Patterns and Limits of Donor-Driven Ownership 327 $a9. Mozambique: Contested Sovereignty? The Dilemmas of Aid Dependence10. Tanzania: A Genuine Case of Recipient Leadership in the Aid System?; 11. Zambia: Back to the Future?; 12. Aid and Power: A Comparative Analysis of the Country Studies; Conclusion: Changing Conditions?; Index 330 $aThe volume examines negotiations between rich countries and African governments over what should happen with money given as aid. Describing the history of aid talks the volume presents eight studies of the strategies of negotiation tried by particular African countries. - ;This book presents an original approach to understanding the relationship between official aid agencies and aid-receiving African governments. The first part provides a challenge to the hazy official claims of aid donors that they have stopped trying to force African governments to do what 'we' think is best for 'them' and i 606 $aEconomic assistance$xPolitical aspects$zAfrica 607 $aAfrica$xForeign economic relations 608 $aElectronic books. 615 0$aEconomic assistance$xPolitical aspects 676 $a338.91096 701 $aWhitfield$b Lindsay$f1978-$0991738 801 0$bMiAaPQ 801 1$bMiAaPQ 801 2$bMiAaPQ 906 $aBOOK 912 $a9910454123703321 996 $aThe politics of aid$92269694 997 $aUNINA LEADER 08705oam 22004691 450 001 9910794013203321 005 20230814234319.0 010 $a1-315-11403-8 010 $a1-138-19660-6 010 $a1-138-19662-2 024 7 $a10.1201/9781315114033 035 $a(CKB)4100000011324053 035 $a(MiAaPQ)EBC6236323 035 $a(OCoLC)1173825014$z(OCoLC)1164374776 035 $a(OCoLC-P)1173825014 035 $a(FlBoTFG)9781315114033 035 $a(EXLCZ)994100000011324053 100 $a20180119d2018 uy 0 101 0 $aeng 135 $aur||||||||||| 181 $ctxt$2rdacontent 182 $cc$2rdamedia 183 $acr$2rdacarrier 200 10$aAtlas of inherited metabolic diseases 205 $aFourth edition. 210 1$aBoca Raton :$cCRC Press,$d2018. 215 $a1 online resource (871 pages) 311 $a1-138-19659-2 327 $aPART 1: ORGANIC ACIDEMIAS. Introduction to the organic acidemias. Propionic academia. Methylmalonic academia. Cobalamin C, D, F, G diseases; methylmalonic aciduria and homocystinuria. The methylmalonic aciduria of deficiency of AcylCoA synthetase (ACSF3). Multiple carboxylase deficiency/holocarboxylase synthetase deficiency. Multiple carboxylase deficiency/biotinidase deficiency. Isovaleric academia. Glutaric aciduria (type I). 3-MethylcrotonylCoA carboxylase deficiency/3-methylcrotonylglycinuria. D-2-hydroxyglutaric aciduria. L-2-hydroxyglutaric aciduria. 4-Hydroxybutyric aciduria. PART 2: DISORDERS OF AMINO ACID METABOLOISM. Alkaptonuria. Phenylketonuria. Hyperphenylalaninemia and defective metabolism of tetrahydrobiopterin. Biogenic amines. Homocystinuria. Maple syrup urine disease (branched-chain oxoaciduria). Branched chain keto acid dehydrogenase kinase (BCKDK) deficiency. Oculocutaneous tyrosinemia/tyrosine aminotransferase deficiency. Hepatorenal tyrosinemia/fumarylacetoacetate hydrolase deficiency. Nonketotic hyperglycinemia. Serine deficiencies. PART 3: HYPERAMMONEMIA AND DISORDERS OF THE UREA CYCLE. Introduction to hyperammonemia and disorders of the urea cycle. Ornithine transcarbamylase deficiency. Carbamylphosphate synthetase deficiency. Citrullinemia type I. Argininosuccinic aciduria. Argininemia. Hyperornithinemia, hyperammonemia, homocitrullinuria syndrome. Lysinuric protein intolerance. Glutamine synthetase deficiency. PART 4: DISORDERS OF FATTY ACID OXIDATION. Introduction to disorders of fatty acid oxidation. Carnitine transporter deficiency. Carnitine-ayclcarnitine translocase deficiency. Carnitine palmitoyl transferase I deficiency. Carnitine palmitoyl transferase II deficiency, lethal neonatal. Medium-chain acyl CoA dehydrogenase deficiency. Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency. Long chain L-3-hydroxyacl CoA dehydrogenase deficiency -- (trifunctional protein) deficiency. Short-chain acyl CoA dehydrogenase (SCAD) deficiency. Short-chain 3-hydroxyacylCoA dehydrogenase (SCHAD) deficiency. Short/branched-chain acyl-CoA dehydrogenase (2-methylbutyrylCoA dehydrogenase) deficiency. Multiple acyl CoA dehydrogenase deficiency/glutaric aciduria, type II ethylmalonic-adipic aciduria. 3-Hydroxy-3-methylglutarylCoA lyase deficiency. PART 5: THE LACTIC ACIDEMIAS AND MITOCHONDRIAL DISEASE. Introduction to the lactic acidemias. Pyruvate carboxylase deficiency. Fructose-1,6-diphosphatase deficiency. Deficiency of the pyruvate dehydrogenase complex. Mitochondrial encephalomyelopathy, lactic acidosis, and stroke-like episodes (MELAS). Myoclonic epilepsy and ragged red fiber (MERRF) disease. Neurodegeneration, ataxia and retinitis pigmentosa (NARP). Kearns-Sayre syndrome. Pearson syndrome. The mitochondrial DNA depletion syndromes: mitochondrial DNA polymerase deficiency. PART 6: DISORDERS OF CARBOHYDRATE METABOLISM. Galactosemia. Glycogen storage disease: introduction. Glycogenosis type I -- von Gierke disease. Glycogenosis type II/Pompe/lysosomal [alpha]-glucosidase deficiency. Glycogenosis type III/amylo-1,6-glucosidase (debrancher) deficiency. PART 7: PEROXISOMAL DISORDERS. Adrenoleukodystrophy. Neonatal adrenoleukodystrophy/disorders of peroxisomal biogenesis. PART 8: DISORDERS OF PURINE AND PYRIDINE METABOLISM. Introduction to the disorders of purine and pyrimidine deficiencies. Lesch-Nyhan disease and variants. Adenine phosphoribosyltransferase (APRT) deficiency. Phosphoribosylpyrophosphate synthetase and its abnormalities. Adenosine deaminase deficiency. Adenosine kinase deficiency. Purine nucleoside phosphorylase deficiency. Adenylosuccinate lyase deficiency. Santhinuria, xanthine oxidase deficiency. Orotic aciduria. Molybdenum cofactor deficiency. PART 9: MUCOPOLYSACCHARIDOSES. Introduction to mucopolysaccharidoses. Hurler disease/mucopolysaccharidosis type IH (MPSIH)/[alpha]-L-iduronidase deficiency. Scheie and Hurler-Scheie diseases/mucopolysaccharidosis IS and IHS/[alpha]-iduronidase deficiency. Hunter disease/mucopolysaccharidosis type II/iduronate sulfatase deficiency. Sanfilippo disease/mucopolysaccharidosis type III. Morquio syndrome/mucopolysaccharidosis type IV/keratan sulfaturia. Maroteaux-Lamy disease/mucopolysaccharidosis VI/N-acetylgalactosamine-4-sulfatase deficiency. Sly disease/[beta]-glucuronidase deficiency/mucopolysaccharidosis VII. PART 10: MUCOLIPIDOSIS. Mucolipidosis II and III/ (I-cell and pseudo-Hurler polydystrophy N-acetyl-glucosaminyl-l-phosphotransferase deficiency. PART 11: DISORDERS OF CHOLESTEROL AND NEUTRAL LIPID METABOLISM. Familial hypercholesterolemia. Mevalonic aciduria. Lipoprotein lipase deficiency/type I hyperlipoproteinemia. PART 12: LIPID STORAGE DISORDERS. Fabry disease. Tay-Sachs disease/hexosaminidase A deficiency. Sandhoff disease/GM2 gangliosidosis/deficiency of Hex A and Hex B subunit deficiency. Gaucher disease. Niemann-Pick disease. Niemann-Pick type C disease/cholesterol-processing abnormality. Krabbe disease/galactosylceramide lipidosis/globoid cell leukodystrophy. Lysosomal acid lipase deficiency: Wolman disease/cholesteryl ester storage disease. Fucosidosis. [alpha]-Mannosidosis ([beta]-Mannosidosis). Galactosialidosis. Metachromatic leukodystrophy. Multiple sulfatase deficiency. PART 13: MISCELLANEOUS. Disorders of vitamin B6 metabolism. PMM2-CDG (Congenital disorders of glycosylation, type Ia). Ethylmalonic encephalopathy. Disorders of creatine metabolism. GLUT1 deficiency. Hypophosphatasia. NBAS/RALF deficiency. [alpha]1-Antitrypsin deficiency. 330 $aIn a field where even experts may find that years have elapsed since they last encountered a child with a given disorder, it is essential for the clinician to have a comprehensive source of practical and highly illustrated information covering the whole spectrum of metabolic disease to refer to. The content is divided into sections of related disorders, including disorders of amino acid metabolism, lipid storage disorders, and mitochondrial diseases for ease of reference, with an introductory outline where appropriate summarizing the biochemical features and general management issues. Within the sections, each chapter deals with an individual disease, opening with a useful summary of major phenotypic expression including clear and helpful biochemical pathways, identifying for the reader exactly where the defect occurs. Throughout the book, plentiful photographs, often showing extremely rare disorders, are an invaluable aid to diagnosis. Key Features Fully updated to incorporate all new developments in the field Brand new chapters cover methylmalonic aciduria of ACSF3 deficiency, branched chain keto acid dehydrogenase deficiency, serine deficiencies, purine nucleoside phosphorylase deficiency, antiquitin deficiency, and others Excellent and detailed clinical descriptions, with numerous valuable hints and suggestions for management Helpful explanatory algorithms and decision trees, and high-quality illustrative material including biochemical pathways and an unrivaled photographic collection, which enhance clinical applicability The fourth edition of this highly regarded book, authored by two of the foremost authorities in pediatric metabolic medicine, continues to provide incomparable insight into the problems associated with metabolic diseases and remains invaluable to pediatricians, geneticists, and general clinicians worldwide. 606 $aMetabolism$xDisorders$vAtlases 615 0$aMetabolism$xDisorders 676 $a616.39 700 $aNyhan$b William L.$f1926-$01537200 702 $aHoffman$b Georg 702 $aBarshop$b Bruce Allen 801 0$bOCoLC-P 801 1$bOCoLC-P 906 $aBOOK 912 $a9910794013203321 996 $aAtlas of inherited metabolic diseases$93786371 997 $aUNINA