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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2804_Библиотеки_им_академика_М_И_Перельмана

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expiratorywheezes. A thorough examination should always be performed, observing any manifestations suggesting underlyingcardiopulmonary,infectious,immunologic,ormalignantdisease.
DifferentialDiagnosis
Onemustdistinguishbetweentruehemoptysisandpseudohemoptysis,whichcomesfromtheupper airway(abovetheglottis),oraspiratedGItractbleedingthatislaterexpectorated.
DiagnosticTesting
Athoroughhistoryandphysicalexaminationisimportanttoprovidecluesandguidanceinadditional testing. Additional testing is aimed at determining and localizing the bleeding source and identifying the underlyingetiology.
LABORATORIESANDELECTROCARDIOGRAPHY
BasiclabworkincludingCBC,comprehensivemetabolicpanel,andcoagulationstudiesareindicated. Typeandcross-matchingofbloodareindicated,especiallyincasesofmassivehemoptysis. AnABGanalysismaybeindicatedincasesofshortnessofbreathorrespiratorycompromise. Sputumstudiesmaybehelpfulincases ofinfectionandcanbeanalyzedwithroutineGram stainand culture,fungalculture,andacid-fastbacillismear/cultureasindicated. Serologicstudies andurinalysiswithmicroscopymaybeclinicallyindicatedbased onthesuspicion for rheumatologic disease or vasculitis. These mayinclude ANA screen, antineutrophil cytoplasmic antibody(ANCA)screen,includingreflextestingtomyeloperoxidaseandproteinase3,antiglomerular basement membraneantibodies, complementlevels, cryoglobulins, double-strandedDNAantibodies, andothers. Brain natriuretic peptide or N-terminal pro b-type natriuretic peptide levels may be helpful when cardiacfailureissuspected. ECGcanhelpassessforunderlyingcardiacdisease.
IMAGING
PosteroanteriorandlateralCXRareperformedinallcasesofhemoptysis.
Unfortunately,thesemaybenormalornonlocalizinginupto50%ofallcases.
123,124
Furthermore, CXR may be normal in up to 10% of hemoptysis cases caused by bronchogenic carcinoma.
123
ChestCT:CTshouldbeperformed ifthe diagnosisremainsindoubtafterinitialclinical andCXR evaluation (see Figure 10-5). CT chest can be performed with or without contrast; however, CT angiographyisincreasinglyusefulinlocalizingthesourceofhemoptysis.
Advantages:
Canvisualizeparenchyma,vasculature,andairwaystovaryingextent.  Especially useful for hemoptysis resulting from bronchiectasis, cavitary lung disease, masses,andvascularmalformations.
125
Candetectupto96%ofCXR-occultmalignancies.
126
CTvisualizestumorswithefficacycomparablewithbronchoscopy.
127
CT angiography may be better than bronchoscopy in determining the etiology and source of hemoptysis.
128
https://t.me/med1917
CTangiographyisusefulforplanningembolizationprocedures.
129
Disadvantages:
CTislessefficaciousthanbronchoscopyinrecognizingsubtlebronchialandmucosallesions.
125
Itisnonspecificincasesofparenchymal/alveolarhemorrhage. Delayintreatmentplacesunstablepatientsathighrisk.
Echocardiographymaybeperformedifstructuralorvalvularcardiacdiseaseissuspected.
DIAGNOSTICPROCEDURES
Fiber-optic(flexible) bronchoscopy:Generallylocalizes/lateralizes bleedingsource inover two- thirdsofcases,dependingonthesetting.
130
Indications:
Ifthesourceisunclearafterinitialevaluationandimaging Persistentorrecurrenthemoptysis Toruleoutinfection Iftheclinicalpresentationsuggestsanairwayabnormality Toobtainbiopsyspecimens,ifimagingsuggestsmalignancyorisnonlocalizinginthepresenceof
atleasttworiskfactorsforbronchogeniccarcinoma: □ Malesex □ Age>40years □ >40pack-yearsmoking □ Durationofhemoptysis>1week □ Volumeexpectorated>30mL6,
131,132
Toidentifypotentialanatomicareaforarterialembolization Toprovideendobronchialtreatments
Toruleoutalveolarhemorrhage The timing of bronchoscopy is controversial, althoughyields increase when performed during or within48hoursofbleeding.
131
Bronchial and pulmonary arteriography is performed in the setting of persistent, recurrent, or
massivehemoptysis.
Advantages:
Can be both diagnostic andtherapeutic via simultaneous embolization of the visualizedculprit
vessel.
Useful in hemoptysis of varying degrees in the setting of different etiologies including
bronchiectasis,malignancy,aspergilloma,andothers.
133
CanbeprecededbyCTangiographyforprocedureplanning.
129
Disadvantages:
Variableandinexactlocalizationofthebleedingsourcedependingonclinicalsetting.
130
Anatomicvariability.
Bleedingincaseswhereitisinsufficientforcontrastextravasation.
TREATMENT
Theapproachtohemoptysisisprimarilyaimedatdistinguishingmassive/life-threateninghemoptysis fromnonmassivehemoptysis.Thethreemaingoalsare:
https://t.me/med1917
Nonmassivehemoptysisisusuallytreatedconservatively.Treatmentmayincludethefollowing:
Reversalofcoagulopathy Antitussives Bronchoscopyifrecurrent Steroidsand/orimmunosuppressionforrheumatologicconditions Antibioticsforinfection(fungal,TB,mycobacteria) Diureticsand/orinotropesforheartdisease(LVfailure,mitralstenosis)
Massive hemoptysis: management requires urgent action, intensive care monitoring, and an early multidisciplinary approach including an interventional pulmonologist, a thoracic surgeon, and an interventionalradiologist(seeFigure10-5).
Initialstabilization:
Airwaymanagementmayrequireintubation,withalarge(>8mm)endotrachealtube.
134
□ Single-lumenmainstemintubationforselectiveventilationofunaffectedlung.
□ Double-lumenendotrachealintubationfor selective ventilationofunaffectedlung.Shouldbe
performedandmanagedonlyunderappropriatelyskilledsupervision. Lateraldecubituspositioning(affectedlungdown)tominimizeaspirationintounaffectedlung. Inhaledtranexamicacidmayreducevolumeofexpectoratedblood,reducehospitallengthofstay, leadtomorefrequentresolutionofhemoptysis,reducerecurrencerates,andreducetheneedfor invasive procedures, withoutincreased adverse effects noted.More investigation is needed on thisintervention.
135
Bronchoscopywithdirectedairwaytherapy:Rigidbronchoscopyisfavoredifavailablebecauseit provides optimal airway access and ventilatory control and easier suctioning and allows for manipulationofinstruments.
Directtamponadewiththedistalendofthebronchoscope.Balloon tamponade: Left in place for 1–2 days. Monitor for ischemic mucosal injury or postobstructive pneumonitis.
136
Fogarty balloons, bronchial blockers, and pulmonary artery
catheterballoonshaveallbeendescribedfortamponadingbleeding. Endobronchialelectrocautery.
137
Argonplasmacoagulation.
137,138
Endobronchialstentplacement.
139
Topical hemostatic agents: Cold saline, epinephrine, vasopressin, thrombin, and oxidized regeneratedcellulosehavebeenusedtocontrolbleeding.
140–142
Arteriographyandembolizationshouldbeperformedearlyinmassiveorrecurrenthemoptysis.
Successfulembolizationin>85%ofcasescanbeachievedwithcarefullocalization.
133,143
Embolizationisparticularlyusefulincysticfibrosis(CF)patients.
144,145
Treatmentfailureisusuallybecauseofinadequateorincompletesourcevesselidentification. Postembolization arteriography may identify additional systemic culprit vessels, most commonly fromtheintercostalandphrenicarteries.
146
Rebleeding is common in embolized patients, occurring in up to 20% of cases over 1 year. Rebleedingappearstobemorecommoninpatientswithsarcoidosis,malignancy,andaspergilloma.
143,147,148
Risksincludebronchialorpartialpulmonaryinfarctionand,rarely,ischemicmyelopathybecauseof inadvertentembolizationofaspinalartery.
Medications
https://t.me/med1917
Systemicprocoagulants:Theseareusedonlyinunstablemassivehemoptysisasatemporizingmeasure. Alternatively,theymayberequiredwhenconventionalbronchoscopic,interventional,orsurgical therapiesarecontraindicatedand/orunavailable.ExamplesareadministrationoffactorVII,vasopressin, andaminocaproicacid.
SurgicalManagement
Emergentsurgeryhashighmorbidityandmortalitycomparedwithelectivesurgeryfollowingpatient stabilization.
119
Lobectomyorpneumonectomyoffersdefinitivecure.
Indications: Persistent focal/unilateral massive hemoptysis despite other therapy. It is particularly useful for stable patients with hemoptysis due to cavitary lung disease, localized bronchogeniccarcinoma,AVM,ortraumaticinjuries.
149
Contraindications:Poorpulmonaryreserve,advancedmalignancy,activeTB,diffuselungdisease, ordiffusealveolarhemorrhage.
Referral/Consultation
Pulmonary(interventionalformassivehemoptysis,ifavailable) Thoracicsurgery Interventionalradiology
Outcome/Prognosis
Mortalitydependsonetiologyandvolumeofhemoptysis.
119,150,151
Mortalitymaybeashighas80%incasesofmassivehemoptysisbecauseofmalignancy. Mortalitytendstobe<10%withnonmassivehemoptysis. Mortalityinpatientswithbronchiectasisandlunginfectionsis<1%.
CysticFibrosis
GENERALPRINCIPLES
Definition
CFisanautosomalrecessivedisordercausedbymutationsoftheCFtransmembraneconductance regulatorgene(CFTR),whichresultsinmultisystemexocrineorgandysfunction.
Epidemiology
IntheUS,>30,000peopleareaffectedbyCF,andabout1000newcasesarediagnosedeveryyear.
152,
153
CFisthemostcommonlife-shorteninggeneticdiseaseinCaucasians;however,thediagnosisshouldbe consideredinpatientsofdiverseethnicbackgroundsaswell. Theprognosis ofCFhas continuouslyimproved,andtoday>50% ofpatientswith CF intheUS are aged≥18years.
Etiology
https://t.me/med1917
CFiscausedbymutationsintheCFTRgene,acyclicadenosinemonophosphate–regulatedchloride channel,whichnormallymaintainshydrationofexocrineorgansecretions. AbnormalCFTRfunctioncausesdecreasedchloridesecretionandincreasedsodiumabsorptiononthe surfaceofepithelialcells,whichcanresultinthickenedsecretionsintheairways,sinuses,pancreatic ducts,biliarytree,intestines,andreproductivetract. CFTRmutationsarecategorizedintofive classes:(1)defective synthesis,(2)defectiveprocessing
and maturation, (3) defective regulation, (4) defective conductance, and (5) reduced function/synthesis.
ThemostcommonmutationisF508del,aclassIImutationresultingfromthedeletionofDNAcoding forphenylalanine(F) amino acid atposition508. Themajority oftheresultant misfolded proteinis destroyedintracellularlyanddoesnotreachthecellsurface.Morethan85%ofpatientsintheUSwith CFhaveatleastonecopyofthismutation.
153
Todate,>2000otherpotentiallycausativemutationsintheCFTRgenehavebeenidentified.
153
Pathophysiology
TheprimarypulmonarymanifestationsofCFarerelatedtothemalfunctionofchloridetransportacross the airway epithelium, resulting in diminished airway surface liquid and impaired mucociliary clearance. Poor mucociliary clearance, infection, inflammation, and chronic airway obstruction often result in bronchiectasis,chronicinfection,respiratoryfailure,andprematuredeath. Similarly,thickenedsecretionsinthepancreaticandbiliaryductsleadtomaldigestion,malabsorption, and,occasionally,liverdiseaseanddiabetes.
154
DIAGNOSIS
Today,childrenwithCFaretypicallydiagnosedvianewbornscreeningorduringchildhood,butthere isincreasingrecognitionofmildervariantsthatmaynotpresentuntillaterinlife. In2018,62%ofnewdiagnosesweretheresultofnewbornscreening.
153
Newbornscreeningis now
routinelyperformedthroughouttheUS. AdiagnosisofCFismadewhenthereare:
155
CompatibleclinicalfeaturesofCFor ApositivefamilyhistoryofCFor Apositivenewbornscreeningtestand Elevated(>60mmol/L)sweatchlorideor Intermediate(30–59mmol/L)sweatchlorideand
Presenceoftwodisease-causingmutationsinCFTRor
AbnormalCFTRfunctionalassay AdiagnosisofCF-relatedmetabolicsyndromeis madewhenthereisapositivenewbornscreening test, and an intermediate sweat chloride level without two causative mutations, or a negative (<30 mmol/L) sweat chloride level with two CFTR mutations, at least one with unclear phenotypic consequences. Althoughgenotypingmayassistinthediagnosis,italonecannotestablishorruleoutthediagnosisof CF,andtheinitialtestofchoiceremainsthesweatchloridetest.
ClinicalPresentation
Pulmonarymanifestations:
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Cough with purulent sputum production, wheezing, hemoptysis, dyspnea, progressive airflow obstruction,bronchiectasis,andpneumothorax.
Extrapulmonarymanifestations:
Chronic sinusitis, nasal polyposis, pancreatic insufficiency (vitamins A, D, E, and K deficiency), steatorrhea, malnutrition, failure tothrive, meconium ileus, distal intestinal obstruction syndrome, volvulus, intussusception, rectal prolapse, diabetes mellitus, liver cirrhosis, portal hypertension, cholelithiasis,cholecystitis,nephrolithiasis,maleinfertility(bilateralabsenceofthevasdeferens),
epididymitis,growthretardation,hypertrophicpulmonaryosteoarthropathy,andosteopenia. OlderpatientswithundiagnosedCFoftenstrugglewithrecurrentsinopulmonaryinfections,refractory uncontrolled asthma, multiple episodes of pneumonia, unexplained bronchiectasis, recurrent pancreatitis, or male infertility. Diagnosis at an older age is generally associated with milder phenotypesandrarermutations.
DifferentialDiagnosis
Primaryciliarydyskinesia:Bronchiectasis,sinusitis,andinfertility.LimitedGIsymptomsandnormal sweat chloride levels. Occasionally seen with dextrocardia or situs inversus totalis (Kartagener syndrome).  Immunodeficiency (e.g., severe combined immunodeficiency, common variable immunodeficiency): Recurrent sinus and pulmonary infections but typically no GI symptoms and normalsweatchloridelevels.  Alpha-1 antitrypsin deficiency: Early-onset emphysema, airflow obstruction, chronic sputum production,panniculitis,chronichepatitis,cirrhosis,andhepatocellularcarcinoma.Bronchiectasisisa commonradiographicfeature
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withnormalsweatchloridelevels.
Shwachman–Diamondsyndrome:Pancreaticinsufficiency,cyclicneutropenia,andshortstature.May leadtolungdisease,butnormalsweatchloridelevels.
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Youngsyndrome:Bronchiectasis,sinusitis,andazoospermia.Mildrespiratorysymptoms,lackofGI symptoms,andnormalsweatchloridelevels. Idiopathicbronchiectasis
DiagnosticTesting
Skin sweat testing with a standardized quantitative pilocarpine iontophoresis method remains the goldstandardforthediagnosisofCF.
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A sweat chloride concentration of ≥60 mmol/L on two separate occasions is consistent with the diagnosisofCF. Intermediatesweattestresults(30–59mmol/Lsweatchloride)ornondiagnosticresultsshouldlead torepeatsweattesting,genetictesting,ornasalpotentialdifferencetesting. AnormalsweatchlorideconcentrationisgenerallysufficienttoruleoutCF,butshouldberepeated in cases with a high clinical suspicion as normal values can be observed with very uncommon mutations. SweattestingshouldbeperformedataCFcarecentertoensureaccuracyofresults. Abnormalsweatchlorideconcentrationsarerarelydetectedinnon-CFpatients.
Genetictestshavedetected>2000putativeCFmutations.
TworecessivemutationsonseparateallelesmustbepresenttocauseCF. CommerciallyavailableCFscreensidentify>90% oftheabnormal genesinaCaucasianNorthern European population, although they test for only a minority of the known CF genes. Full gene sequencingiscommerciallyavailable,butinterpretationmaybecomplex.Informationaboutspecific
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mutationsandreportedclinicalphenotypemaybefoundathttp://www.cftr2.org/.
LABORATORIESANDTESTING
CXRs often demonstrate hyperinflation with upper lobe–predominant cystic lung disease, bronchiectasis,andmucusplugging. High-resolutionCTscansmaybehelpfulinevaluatingpatientswithearlyormilddiseasebydetecting earlyairwaychanges. PFTsoftendemonstrateexpiratoryairflow obstruction with increased residual volume (air trapping) andtotal lungcapacity (hyperinflation).Impairment ofalveolar gasexchangemaybepresent laterin thecourseofdisease. Sputumcultures typicallyidentify multiple organisms including Staphylococcus aureus, nontypeable
Haemophilus influenzae, Pseudomonas aeruginosa, Stenotrophomonas maltophilia, and Burkholderia cepacia complex. Isolation of mucoid variants of P. aeruginosa from the respiratory
tractoccursfrequently.Useofspecialculturemediaforfastidiousorganismsisrecommended. Nontuberculousmycobacteria(NTM)arealsofrequentlyisolatedfromtheairwaysofpersonswithCF andmaybepathogenic.
TREATMENT
CFtherapyaimstoimprovequalityoflife,decreasethenumberofexacerbationsandhospitalizations, reducetherateofdeclineinlungfunction,anddecreasemortality. AcomprehensiveprogramprovidedatanaccreditedCFcarecenterisrecommended. Treatmentburdencanbe a significantbarriertoproperadherence.Themediannumberofminutesto completedailytherapiesisover90min/d.
ChronicTherapies
PULMONARYTHERAPY
Primarilyfocusedonclearingpulmonarysecretionsandcontrollinginfection.  Inhaled bronchodilators: β-Adrenergic agonists (such as albuterol, salmeterol, or formoterol). Recommendedinall CFpatients. Used totreat the reversible component ofairflow obstruction and facilitatemucusclearance. RecombinanthumanDNase(dornasealpha,Pulmozyme):DigestsextracellularDNA,decreasingthe viscoelasticityofthesputum.Improvespulmonaryfunctionanddecreasestheincidenceofrespiratory tractinfectionsthatrequireparenteralantibiotics.
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Hypertonicsaline(4 mL of inhaled 7% saline): Improves clearance ofsecretions, resultsinfewer exacerbations, and improves lung function.
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Albuterol should be administered prior to reduce
bronchospasm.  Mechanical airway clearance devices (oral oscillating device, high-frequency chest oscillation vests): Used in combination with medical therapy to promote airway clearance. Other alternatives include postural drainage with chest percussion andvibration. Exercise is also anexcellentformof airwayclearance. Immunizations: PneumovaxandPrevnar13are recommendedfor all patientswithCF,asis yearly influenzavaccination.
ANTIBIOTICS
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P. aeruginosa is the most prevalent organism in CF patients and is associated with significant morbidity and mortality. Over time, the percentage of patients colonized with P. aeruginosa (and multidrug-resistantPseudomonas)increases. InpatientschronicallyinfectedwithP.aeruginosa,theinhaledaerosolizedantibioticstobramycin(300 mg nebulized twice daily) and aztreonam lysinate (75 mg nebulized 3× daily) can be used by alternating28daysonwith28daysoff.Theseimprovepulmonaryfunction,decreasethedensityofP. aeruginosa,anddecreasetheriskofhospitalization.Continuousalternatinginhaledantibiotictherapy hasbecomethestandardofcareformanypatientswithchronicPseudomonasinfectionandpulmonary impairment.
ANTI-INFLAMMATORYTHERAPY
Azithromycin (500 mgoral 3×/wk)usedchronicallyshows mildimprovementinlungfunction and reducesdaysinthehospitalfortreatmentofrespiratoryexacerbations.Patientsshouldbescreenedfor NTM beforeinitiationofmacrolide antibiotics becausechronicmonotherapycanlead tomacrolide­resistantNTM. Glucocorticoidsusedinshortcoursesmaybehelpfultosomepatientswithasthma-likesymptoms,but long-termtherapyshouldbeavoidedtominimizethesideeffects.
RESTORATIONOFCFTRFUNCTION
Recently, effectivemodulator therapy hasdramaticallychanged the treatment ofCFandchanged the diseasecourseformanypatients. CFTR modulators treat the underlying cause of the disease by correcting protein misfolding, transportingCFTRtothecellsurface,andrestoringchlorideconductance. CFTRcorrectorsincreasetheamountofmatureCFTRonthecellsurfacewhilepotentiatorsincrease thechannel-gatingactivityofCFTRprotein. Thecombinationofcorrectorsandapotentiatorismoreeffectivethaneitherapproachalone. SeeTable 10-11for details regarding the currently Food andDrug Administration–approved CFTR modulators. One should note that the indications for CFTR modulators are constantly changing especiallywithrespecttoagecutoffs.
TABLE10-11
CURRENTLYFDA-APPROVEDCFTRMODULATORS
CFTR Modulator
FDA-Approved Indications
Mechanism ofAction
ClinicalEffects Adverse
Effects
SingleTherapy
Ivacaftor (Kalydeco)
>4mowithresponsive partialfunctionCFTR mutations
PotentiatoraImprovesFEV1
Weightgain Reduces hospitalizationsand exacerbation frequency Decreases
Pseudomonas
Transaminitis
c
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airwayinfection Improvessinus disease
DualTherapy
Lumacaftor– ivacaftor (Orkambi)
>2ywithtwocopiesofthe F508delmutation
Potentiator plus corrector
b
b
Modestly improvesFEV1 Reduces hospitalizationsand exacerbation frequency
Transaminitis Lumacaftoris apotent inducerof CYP3A Chest discomfort anddyspnea
Tezacaftor– ivacaftor (Symdeko)
>6ywithtwocopiesofthe F508delmutationor>1 otherresponsiveCFTR mutations
Potentiator plus corrector
ImprovesFEV1 Reduces hospitalizationsand exacerbation frequency
Transaminitis Fewer adverse effectsthan lumacaftor– ivacaftor
TripleTherapy
Elexacaftor– tezacaftor– ivacaftor (Trikafta)
>12yandatleastonecopy ofF508delmutationora responsiveCFTRmutation
>90%ofpatientswithCF intheUSareeligiblefor thistherapy
Potentiator plustwo correctors
Significantly improvesFEV1 (increasedby13.8 pointsat4wk)
63%lowerannual rateof exacerbation comparedto placebo
Transaminitis GIupset Elevated bilirubin
Generalapproachtoinitiationoftherapy:Ifapatienthasagenotypethatiseligibleformorethanonetherapy,generally recommendedtostartmaximaltherapyavailableforthatagegroup(tripletherapy>doubletherapy>singletherapy).Advance therapywhenpatientmeetsagecriteriaforeachcombinationdrug.DoseadjustmentinChild–PughClassBcirrhosis,avoidin Child–PughclassCdisease.ReducedosewhencoadministeredwithCYP3Ainhibitors.Seepackageinsert.CF,cysticfibrosis; CFTR,CFtransmembraneconductanceregulatorgene;FDA,FoodandDrugAdministration;FEV1,forcedexpiratoryvolumein thefirstsecond.
a
Potentiator:IncreasesCFTRchannelopentimeatthecellsurface.
b
Corrector:IncreasescellsurfaceproteinexpressionbyimprovingtheprocessingandtraffickingofCFTR.
c
LFTsarerecommendedpriortoinitiationofCFTRmodulatortherapy,every3monthsforthefirstyear,andannuallythereafter.
PULMONARYREHABILITATION
Pulmonaryrehabilitationmayimprovefunctionalstatusandpromoteclearanceofairwaysecretions.
OXYGENTHERAPYANDNONINVASIVEVENTILATION
OxygentherapymaybeindicatedbasedonstandardrecommendationsforthetreatmentofCOPD.Rest andexerciseoxygenassessmentsshouldbeperformedasindicated.
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OxygenmaybeindicatedforpatientswitharestingPaO2≤55mmHgorSpO2≤88%atrestorPaO2≤ 59 mm Hg or SpO2 ≤ 89% with certain comorbidities (right heart failure, cor pulmonale, or erythrocytosis).
NocturnalnoninvasiveventilationmaybeindicatedforpatientswithadaytimerestingPCO2>50mm Hgoranocturnaloxygensaturation<88%formorethan5minutesonusualdaytimeoxygen.
MANAGEMENTOFEXTRAPULMONARYMANIFESTATIONS
Pancreaticinsufficiency
Primary managementinvolves pancreaticenzyme supplementation.Enzymedose should be titrated toachieveonetotwosemisolidstoolsperdayandtomaintainadequategrowthandnutrition. Enzymesaretakenimmediatelybeforemealsandsnacks. Dosing of pancreatic enzymes shouldbe initiated at 500 units lipase/kg/meal and should notexceed 2500unitslipase/kg/meal.
Vitamindeficiency
VitaminsA,D,E,andKcanallbetakenorallywithmealsandenzymes. Irondeficiencyanemiarequiresironsupplementation.
CF-relateddiabetesmellitus
Related to pancreatic insufficiency and managed with insulin; oral hypoglycemics are not recommended. Typicaldiabeticdietaryrestrictionsareliberalized(high-caloriedietwithunrestrictedfat)tomeetthe increased energy requirements of patients with CF and to encourage appropriate growthandweight maintenance. ItisrecommendedthatpregnantwomenwithCFundergoscreeningforgestationaldiabeteswith oral glucosetolerancetestatboth12–16weeksandat24–48weeksgestation.
Bowelimpaction/distalintestinalobstructionsyndrome
Presents as colicky abdominal pain. Radiographic pattern consistent with a complete or partial obstruction. Laxatives such as senna, magnesium citrate, or polyethylene glycol canbe tried initially. Refractory casesmayrequireahypaqueenemawithvisualizationofclearanceoftheobstruction. Narcotic use and/or significant dehydration and/or nonadherence with pancreatic enzyme supplementationcanprecipitateseverebowelobstruction.
CF-relatedliverdisease
HepatobiliarymanifestationsrangefrommildabnormalitiesinLFTstohepaticsteatosisorcirrhosis. Mostcasesofcirrhosisarediagnosedduringchildhood(<18years).
Osteopenia
ScreeningshouldberoutinelyperformedonpatientswithCF. Managedwithcalcium,vitaminDsupplementation,andbisphosphonatetherapyasclinicallyindicated.
Chronicsinusitis
Manypatientswillbenefitfromchronicnasalsteroidadministrationandnasalsalinewashes. Patientswhosesymptomscannot becontrolledwithmedicalmanagementmaybenefitfromfunctional endoscopicsinussurgeryandnasalpolypectomy.
Mentalhealth
DepressionandanxietyarecommonamongpatientsandcaregiversofCFpatientsandbothpatientsand caregiversshouldbescreenedonaregularbasis. Psychologicalsymptomsareassociatedwithworseadherence,heath-relatedqualityoflife,decreased lungfunction,andlowerBMI.
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