Managementul Sas In Pw

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  • MANAGEMENTUL SAS IN PRADER - WILLIStefan MihaicutaUMF V. BabesClinica de Pneumologie

  • Caz

    S. M., n. 20.09.1988, Carei, SMIn noiembrie 2008, 150 cm, 105 kg, Circumferinta gat 39 cm, circumferinta abdominala 121, Circumferinta bazin 141 cm HTA 120/70 mm HgTrimisa de prof. dr. Puiu Maria

  • Apnei raportate, fara sforait. Fara somnolenta diurna excesiva, Epworth 3 Rar cefalee matinala, Somn agitat, nicturie 1x/noapte, cu treziri din somn. Fara modificari ORL (deviatie sept, polipoza nazala, uvula hipertrofica), Malampati III. Tratament cu Levotiroxin (hipotiroidism)

  • Epworth Sleepiness ScoreIn poziia ezndVizionare TV

    Aezat, inactiv, ntr-un loc public (cinema, sedine, sal de ateptare, etc.)Pasager ntr-o main, o or fr oprire

    Aezat pe spate pentru odihn diurn

    Aezat i discutnd cu cineva

    Aezat linitit dup un prnz fr alcool

    n main, la un stop

  • PSG traseu normal

  • PSG cu apnee centrala

  • PolisomnografieDurations

    Patient name: SERESANAcq: 707First name: MARIAType: AdultSex: FStarted: 11/4/2008 at 10:18:13 PMBirth date: 9/20/1988Stopped: 11/5/2008 at 7:01:13 AMPatient age: 20 yearsDuration: 8:43:00 (523.0 min)

    Recording duration:523.0 minRecording start -> endTIB:523.0 minLight off -> Light onSPT:488.5 minSleep Onset -> Last Sleep PageTST:469.0 minREM + NREM + MVT (during SPT)WK before sleep:32.0 minWK from Light off to Sleep onsetWK during sleep:19.5 minSPT - TSTWK after sleep:0.0 minWK from Last sleep page to Light onTWK duration (tot):51.5 minAll WK summedREM duration:55.0 minREM (during TIB)NREM duration:416.0 minS1 + S2 + S3 + S4 (during TIB)SWS duration:78.5 minS3 + S4 (during TIB)Movement:0.5 minMVT (during TIB)

  • Caracteristicile somnuluiLatencies

    From Light off (min)From Sleep onset (min)Sleep onset34.5-S132.012.5S234.50.0S3144.5110.0S4 REM253.5219.0

    Sleep efficiency 1: 89.7%100 x TST/TIBSleep efficiency 2: 96.0%100 x TST/SPTSleep efficiency 3 : 28.5%100 x (N3+REM)/TSTInter-sleep WK: 4.0%WK in sleep / SPT

  • Evenimente respiratorii

    CAOAMASum ApHYPEventsSettings (sec)10.010.010.0-10.0-Number447152376428Max (sec)15.522.512.022.553.053.0Mean (sec)13.412.012.012.116.015.5Tot duration (min)0.99.40.210.5100.0110.5TST (469.0 min)% of TST0.22.00.02.221.323.6Index [#/h TST]0.56.00.16.748.154.8

  • PSG hipopnee cu desaturari

  • PSG

  • REM #/h (REM)NREM #/h(NREM)TST #/h (sleep)RDI31.657.854.8

    Central apnea total duration: 1.68 minutes(0.3% of sleep)Total number of central apnea : 12(1.5 CA/h (sleep))Sigh rate: 8%Periodic breathing total duration : 1.57 minutes(0.3% of sleep)

  • PSG apnei centrale, desaturari, flux respirator scazut

  • Cardio-vascularStatistics

    WKREMNREMS1S2S3MVTDuration (min)51.5 55.0 416.086.0 251.5 78.5 0.5 Mean HR (BPM)90.588.888.694.287.386.4113.0 Stand. dev.8.3566.8918.7778.9088.2327.7182.000 Coef. var.0.0920.0780.0990.0950.0940.0890.018Median (BPM)89.00087.00087.00094.50086.00085.000112.000 IQ8.0004.00010.00012.0008.0005.0002.000# of LHR001601150LHR min (BPM) 57 5758 # of HHR073491870HHR max (BPM) 124186135186130 Mean RR (BPM)92.489.789.795.488.487.3112.9 Stand. dev.20.21512.92812.54813.06712.5219.6772.641 Coef. var.0.2190.1440.1400.1370.1420.1110.023Mean RR (msec)663.0676.0677.6636.9687.4694.6531.7 Stand. dev.130.18156.64372.33065.54070.50667.92112.488 Coef. var.0.1960.0840.1070.1030.1030.0980.023ECG fail (min)0.00.00.00.00.00.00.0

  • Saturatia in O2

    WKREMNREMTOTAL Fail duration (min) 0.20.00.00.2 Average (%) 98979696Desat Index (#/hour) 8.729.727.2Duration desat/hour 1.86.35.8Desat max (%)051919Desat max dur (sec)0.020.045.045.0

  • Desaturari

    Longest continuous duration spent below 0%:0.0 minute(s)Lowest SpO2 (>= 2 seconds) [%]:79# Episodes (>= 5.0 minutes) SpO2 < 88 %:0Longest duration SpO2 < 88 %0.0 minute(s)

    Mean of the resp. event SpO2 min levels [%] :93Mean of the resp. event SpO2 min levels with desat [%] : 92Minimum of the resp. event SpO2 min levels [%] 79

  • Total number of WK or MVT episodes : 3Arousal index : 5.8/h(sleep)% of pages with arousal during sleep: 4.8Number of arousals associated with leg movements: 0Number of arousals NOT associated with leg movements: 45

  • Hipnograma completa

  • DiagnosticSindrom de apnee in somn forma obstructiva (predominant hipopnei) cu indice de apnee- hipopnee 54,8/h, indice de desaturare 27/h, saturatia medie 96%. Tahicardie sinusala nocturna (88/min). Hipnograma cu alterarea structurii somnului, reducerea somnului REM.Scaderea duratei si eficientei somnului profund la 28%. Se recomanda titrare CPAP in timpul somnului.

  • Hipnograma

  • ManagementFORMA CLINIC TRATAMENT RECOMANDATIAH 30/H, CU SIMPTOME SEVERE NCPAP, DIET, IGIENA SOMNULUI

    IAH 30, CU SIMPTOME UOAREDIET I IGIENA SOMNULUI

    IAH 30, CU SIMPTOME SEVEREDIET, IGIENA SOMNULUI, CPAP PROVIZORIU

    IAH 30/H, RISC CVIGIENA SOMNULUI, DIET, CPAPHIPERTROFIE ESUT MOALE FARINGIAN SAU ANOMALII SCHELETALE INTERVENII CHIRURGICALE ORL

  • Managementul opiunilor n SASOform sever

    1. CPAP- opiune de elecie.

    2. Altele nonresponderi la CPAP, cnd nu este tolerat: scdere ponderal, poziie non-supin.

    3. Msurile de a avansa mandibula:(i) dispozitive orale(ii) Osteotomia maxilomandibular f rar...Doar la pacienii tineri, slabi i foarte motivai.

    4. Intervenia chirurgical asupra cilor aeriene superioare (UPPP) i ablaia prin radiofrecven - nu n cazurile severe.

  • PSG

  • Dispozitive

  • CPAP 80% din pacieni accept CPAP ca terapie de lung durat la domiciliu

    W. T. McNicholas, M. R. Bonsignore, Eur Respir J, January 2007; 29:156-178

  • CPAP: impactul asupra activitii din timpul zilei

    Engleman HM et al, Lancet 1994;343:572575Engleman HM et al, Thorax 1998;53:341345Redline S et al, AJRCCM 1998;57:858865Jenkinson C et al, Lancet 1999;353:21002105Ballester E et al, AJRCCM 1999;159:495501Montserrat et al, AJRCCM, 2001, 164:608-13

    nCPAP aduce mbuntiri semnificative pe somnolen i calitatea vieii la pacieniicu simptome de SAS moderat sau severR Davies and J Stradling, AJRCCM, 2000, 161:1775-8

  • Morbiditate cardiovascular

  • Cauze de HTA secundar

    Sindromul de apnee n somn HTA indus de medicamente sau droguri Nefropatiile cronice Hiperaldosteronismul primar Boala renovascular Corticoterapia cronic i sindromul Cushing Feocromocitomul Coarctaia de aort Afeciunile tiroidiene sau paratiroidiene

    (The Seventh Report of the Joint National Comittee on Prevention, Detection, Evaluation and Treatment of High Blood Pressure. The JNC Report). JAMA 2003; 289: 2560-2572.

  • Somers et al, NEJM 2005Moarte subit

  • Mortalitatea crescut la pacienii care nu primesc CPAPDoherty LS Chest. 2005;127:2076-2084Long-term Effects of Nasal Continuous Positive Airway Pressure Therapy on Cardiovascular Outcomes in Sleep Apnea Syndrome* Chest. 2005;128:624-633.Mortality in Obstructive Sleep Apnea-Hypopnea Patients Treated With Positive Airway Pressure* Francisco Campos-Rodriguez, Marti, S, Sampol, G, Muoz, X, et al Mortality in severe sleep apnoea/hypopnoea syndrome patients: impact of treatment. Eur Respir J 2002;20,1511-1518

  • Medical care

    Initial management of hypotonia or poor feeding

    Evaluation for hypogonadism or hypopituitarism

    Management of obesity Monitoring for scoliosis

    Therapy for behavioral issues

  • Surgical Care

    Complications of obesity: gastric bypass CryptorchidismScoliosis intervention. Urgent surgical attention for abdominal issues: cholecystitis, appendicitis, or acute gastric dilation with risk for progression to necrosis.

    Tonsillectomy, adenoidectomy, or tracheostomy placement may be required in patients with obstructive sleep apnea.

  • Consultations

    Geneticist for initial diagnosis and counseling Developmental pediatrician for stimulation programs

    Endocrinologist for management of hypogonadism

    Nutritionist for dietary counseling

    Ophthalmologist for management of strabismus

    Pulmonologist for management of sleep apnea

    Psychiatrist, psychologist, or both for management of behavioral issues

  • Circumferina abdominal medie 120,06 cm Obezitatea abdominal (plus 2 elemente) - element obligatoriu n noua definiie a sindromului metabolic: peste 80 cm la femei i peste 94 la brbai la europeni

    Lancet 2007; 369:2059-2061

  • Diet +Activity

    Limitation of access to foodsSupplemental occupational and physical therapy to promote acquisition of gross and fine motor skills and to strengthen spinal musculature in order to minimize scoliosis. Encouragement of physical activity at home, at school (eg, increased physical education periods), and in the community (eg, Special Olympics)

  • Follow-up

    Inpatient evaluation and treatment for hypotonia and poor feeding during infancy.

    Individuals with scoliosis and complications of obesity or pickwickian syndrome, may require inpatient therapy.

    Patients with severe behavioral problems may merit admission to a facility staffed with individuals with long-term experience with PWS.

  • Follow-up

    Routinely monitor for symptoms of sleep apnea.

    Obtaining a sleep study within the few months after initiation of growth hormone therapy at the first sign of symptoms.

    **