Molecular Basis of Pulmonary Disease: Insights from Rare Lung Disorders - Rilegato

Rilegato

ISBN 10: 1588299635 ISBN 13: 9781588299635

Casa editrice: Humana Pr Inc, 2010

Riassunto

theRareLungDiseaseConsortium,anetworkof13USandinter- tionalsitesthatiscurrentlyconductingclinicaltrialsandstudiesinLAM,alphaone antitrypsin de ciency, pediatric interstitial lung disease, and PAP. It has been a rare privilegetoworkonsuchfascinatingdiseaseswithsuchcapableinvestigatorsfromall overtheworldoverthepast6years. v vi Preface Theformatforthisvolumeisunique. Mostchaptershavebeenauthoredbyacli- cianandabasicscientistwhoareexpertinthediseasetopicandunderlyingmolecular defect,respectively. Theirchargewastofocusonthegeneticbasisandmolecularpat- genesisofdisease,animalmodels,clinicalfeatures,diagnosticapproach,conventional managementandtreatment,andfuturetherapeutictargetsanddirections. Theintentwas nottoprovideabroadoverview,butrathertoshedlightonthemolecularmechanisms thatevoketheclinicalpresentationandengendertreatmentstrategiesforeachdisease. Wehopethatthisapproachwillproveusefulforpulmonarycliniciansandscientists alike. Wethankourwives,Holly,Jean,andVicky,fortheirsupportandindulgencewith latenightemailsandwork- lledweekends,Dr. Roundsfortheinvitationtowritethe book,andalloftheauthorswhocontributed. FrancisMcCormack,MD RalphPanos,MD BruceTrapnell,MD Contents Preface. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v Contributors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix 1 AClinicalApproachtoRareLungDiseases. . . . . . . . . . . . . . . . 1 RalphJ. Panos 2 ClinicalTrialsforRareLungDiseases. . . . . . . . . . . . . . . . . . . 31 JeffreyKrischer 3 IdiopathicandFamilialPulmonaryArterialHypertension . . . . . . . . 39 JeanM. Elwing,GailH. Deutsch,WilliamC. Nichols, andTimothyD. LeCras 4 Lymphangioleiomyomatosis. . . . . . . . . . . . . . . . . . . . . . . . 85 ElizabethP. HenskeandFrancisX. McCormack 5 AutoimmunePulmonaryAlveolarProteinosis. . . . . . . . . . . . . . . 111 BruceC. Trapnell,KohNakata,andYoshikazuInoue 6 MutationsinSurfactantProteinCandInterstitialLungDisease . . . . . 133 RalphJ. PanosandJamesP. Bridges 7 HereditaryHaemorrhagicTelangiectasia . . . . . . . . . . . . . . . . . 167 ClaireShovlinandS. PaulOh 8 Hermansky–PudlakSyndrome. . . . . . . . . . . . . . . . . . . . . . . 189 LisaR. YoungandWilliamA. Gahl 9 Alpha-1AntitrypsinDe ciency . . . . . . . . . . . . . . . . . . . . . . 209 CharlieStrangeandSabinaJanciauskiene vii viii Contents 10 TheMarfanSyndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 225 AmareshNathandEnidR. Neptune 11 SurfactantDe ciencyDisorders:SP-BandABCA3. . . . . . . . . . . . 247 LawrenceM. Nogee 12 PulmonaryCapillaryHemangiomatosis . . . . . . . . . . . . . . . . . . 267 EdwardD. Chan,KathrynChmura,andAndrewSullivan 13 Anti-glomerularBasementDisease:Goodpasture’sSyndrome. . . . . . 275 GangadharTaduri,RaghuKalluri,andRalphJ. Panos 14 PrimaryCiliaryDyskinesia. . . . . . . . . . . . . . . . . . . . . . . . . 293 MichaelR. Knowles,HildaMetjian,MargaretW. Leigh, andMaimoonaA. Zariwala 15 PulmonaryAlveolarMicrolithiasis. . . . . . . . . . . . . . . . . . . . . 325 KoichiHagiwara,TakeshiJohkoh,andTeruoTachibana 16 CysticFibrosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 AndréM. Cantin 17 PulmonaryLangerhans’CellHistiocytosis–Advances intheUnderstandingofaTrueDendriticCellLungDisease. . . . . . . 369 RobertVassallo 18 Sarcoidosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 389 RalphJ. PanosandAndrewP. Fontenot 19 SclerodermaLungDisease. . . . . . . . . . . . . . . . . . . . . . . . . 409 BrentW. Kinder SubjectIndex. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 421 Contributors JamesP. Bridges,PhD, DepartmentofNeonatologyinPulmonaryBiology,Children’s HospitalMedicalCenter,Cincinnati,OH AndréM. Cantin,MD, Department of Medicine, University of Sherbrooke, Sherbrooke,QC,Canada EdwardD. Chan,MD, DepartmentofInternalMedicine,NationalJewishMedicaland ResearchCenter,Denver,CO KathrynChmura,BA, Department of Medicine, University of Colorado School of Medicine,Denver,C

Su questo libro

Recensione:

From the reviews:

“This unique book attempts to briefly review the clinical manifestations and evolution of a few uncommon, mostly very rare pulmonary disorders, some of which even subspecialist pulmonologists rarely or never encounter during their careers. ... The editors derive insight and input from contributors of eclectic knowledge and experience. ... Certainly, the audience would be limited to basic scientists and a modicum of clinicians with unique interests. ... The book achieves its likely intended goals well. ... a potentially useful addition to my medical library.” (Joel C. Seidman, Doody’s Review Service, July, 2010)

Contenuti:

Chapter 1. A Clinical Approach to Rare Lung Diseases Ralph Panos, M.D. Chapter 2. Clinical Trials for Rare Lung Diseases Jeffrey Krischer, Ph.D. Chapter 3. Idiopathic and Familial Pulmonary Arterial Hypertension Jean M. Elwing, M.D., Gail Deutsch, M.D., William C. Nichols, Ph.D., and Timothy LeCras, Ph.D., Chapter 4. Lymphangioleiomyomatosis Francis X. McCormack, M.D, and Elizabeth P. Henske, M.D., Ph.D. Chapter 5. Autoimmune Pulmonary Alveolar Proteinosis Bruce Trapnell, M.D., Koh Nakata, M.D., Ph.D., and Yoshikazu Inoue, M.D., Ph.D. Chapter 6. Mutations in Surfactant Protein C and Interstitial Lung Disease James P. Bridges, Ph. D. and Ralph Panos, M.D. Chapter 7. Hereditary Hemorrhagic Telangiectasia Claire Shovlin, M.D and S. Paul Oh, Ph.D. Chapter 8. Hermansky Pudlak Syndrome Lisa Young, M.D. and Bill Gahl, M.D., Ph.D. Chapter 9. Alpha One Antitrypsin Deficiency Charlie Strange, M.D. and Sabrina Janciauskiene, Ph.D. Chapter 10. The Marfan Syndrome Amaresh Nath, M.D and Enid Neptune, M.D. Chapter 11. Surfactant Deficiency Disorders SP-B and ABCA3 Larry Nogee, M.D. Chapter 12. Pulmonary Capillary Hemangiomatosis Edward D. Chan, M.D., Kathryn Chmura, B.A, and Andrew Sullivan, M.D. Chapter 13. Goodpasture's Syndrome Gangadar Taduri, M.D., D.M., Raghu Kalluri, Ph.D., and Ralph P. Panos, M.D. Chapter 14. Primary Ciliary Diskinesia Michael R. Knowles, M.D., Hilda Morillas, M.D., Margaret W. Leigh, M.D., Maimoona Zariwala, Ph.D. Chapter 15. Pulmonary Alveolar Microlithiasis Koichi Hagiwara, MD, Takeshi Jokoh, M.D., Teruo Tachibana, MD Chapter 16. Cystic Fibrosis Andre Cantin, M.D. Chapter 17. Pulmonary Langerhan’s CellHistiocytosis Robert Vassallo, M.D. Chapter 18. Sarcoidosis Ralph Panos, M.D. and Andrew Fontenot, M.D. Chapter 19. Scleroderma Lung Disease Brent Kinder, M.D.

Su questa edizione

• Editore: Humana Pr Inc
• Data di pubblicazione: 2010
• ISBN 10: 1588299635
• ISBN 13: 9781588299635
• Rilegatura: Copertina rigida
• Numero di pagine: 438
• Redattore: McCormack Frank, Panos Ralph J., Trapnell Bruce C.