Yeni doğan Sarılığı PDF

Yenidoğan Sarılığı Prof. Dr. Esra ÖZER İzmir Tınaztepe Üniversitesi Tıp Fakültesi  Bilirubin An)oksidan Nörotoksik  Yenidoğan Sarılığı Bilirubin cilt ve sklerayı sarıya boyar – Erişkin > 2 mg/dl – Yenidoğan > 5 mg /dl Yaşamın ilk ha7ası...

Yenidoğan Sarılığı Prof. Dr. Esra ÖZER İzmir Tınaztepe Üniversitesi Tıp Fakültesi  Bilirubin An)oksidan Nörotoksik  Yenidoğan Sarılığı Bilirubin cilt ve sklerayı sarıya boyar – Erişkin > 2 mg/dl – Yenidoğan > 5 mg /dl Yaşamın ilk ha7ası içerisinde – term bebeklerin yaklaşık %60 ında, – preterm bebeklerin ise %80 inde Haya;n ilk 2 ha7asında hastaneye yeniden ya;şın en sık nedenidir  Original Contributions THE FACTORS AFFECTING NEONATAL PRESENTATIONS TO THE PEDIATRIC EMERGENCY DEPARTMENT Ezgi Deniz Batu, MD,* Serap Yeni, RN,† and Ozlem Teksam, MD† *Division of Rheumatology, Department of Pediatrics and †Division of Emergency Medicine, Department of Pediatrics, Hacettepe University Medical Faculty, Ankara, Turkey Reprint Address: Ezgi Deniz Batu, MD, Division of Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara 06100, Turkey , Abstract—Background: A pediatric emergency depart- newborn care may help to decrease nonacute PED visits ment (PED) may be utilized by neonates for nonurgent com- The Journal of Emergency Medicine, by neonates. ! Vol. 2015 48, No.Elsevier 5, pp. 542–547,Inc. 2015 plaints. Various factors, such as primiparity, maternal age, early postnatal discharge, race, income, and maternal and , Keywords—newborn; emergency Copyright ! 2015 Elsevier Inc. department; acute Printed inprematurity the USA. All rights reserved Acil servise yenidoğan döneminde en sık paternal educational levels, have been reported to affect visit; primiparity; the acuity of neonatal emergency department utilization. 0736-4679/$ - see front matter Objective: To determine the characteristics of PED visits INTRODUCTION by neonates (infants # 28 days of age) and to evaluate the başvuru nedeni (%23.4) http://dx.doi.org/10.1016/j.jemermed.2014.12.031 factors affecting the acuity of these visits. Methods: We pro- spectively collected the data of neonates who were admitted to the PED of a tertiary university hospital within a 6-month A pediatric emergency department (PED) may be utilized by neonates for nonurgent complaints. However, the arrival of a newborn to a busy emergency department period. Presenting problems were classified as acute if diag- Acil servisten ya;rılan yenidoğan hastaların nostic tests were requested or the patient was hospitalized, (ED) is a concern for PED physicians. This concern unless the final diagnosis was ‘‘normal newborn.’’ Results: grows out of several factors, such as crowded waiting Over this period, 28,389 children (0–18 years of age) visited and evaluation areas, which are inappropriate for Original the PED, of which 531 were newborns (1.9%). The mean age (%23.2) arasında en sık hastaneye ya;ş newborns; high risk of acute deterioration; and similar was 14.1 ± 8.3 days, with a slight predominance of males response of newborns to variable types of stress, which (57.3%). The chief complaints were jaundice (23.4%), irrita- makes it difficult to decide about the acuity of the bility (9.5%), and vomiting (7.1%), and the most common condition (1). In addition, the differential diagnosis nedeniContributions (%17.9) diagnoses were normal newborn (33.9%), indirect hyperbi- for each nonspecific symptom in the neonatal period is lirubinemia (13.2%), and colic (5.8%). Acute visits extensive (2). were 55.7% of the total visits. Premature infants, infants of multiparous mothers, infants of older mothers There has also been an increase in early discharges af- ($25 years), and physician-referred infants were more ter birth in Turkey over the last few decades, as a result of likely to present with acute problems (p values were 0.001, changing psychosocial factors, health considerations, and 0.013, 0.006, and 12 mg/dL, preterm bebekte >15 mg/dL 4. Direkt bilirubin düzeyinin 1. TB ≤5 mg/dl olanlarda >1 mg/dl veya 2. TB >5 mg/dl olanlarda TB’nin > %20’si olması 5. Klinik olarak sarılığın term bebekte 2 haNa, preterm bebekte ise 3 haNadan fazla uzaması  Tanı CilQeki sarılık gün ışığında veya iyi aydınla;lmış bir odada Sararma öncelikle yüzde görülür Sefalopedal dağılım gösterir  Yenidoğanda Hiperbilirübinemi Nedenleri ARTMIŞ BİLİRÜBİN YÜKÜ HEMOLİTİK NEDENLER HEMOLİTİK OLMAYAN NEDENLER COOMBS POZİTİF COOMBS NEGATİF -Rh uygunsuzluğu -Eritrosit membran defektleri (Sferositoz,eliptositoz) -ABO uygunsuzluğu -Eritrosit enzim defektleri (G6PD eksikliği,PK eksikliği) -Minör grup uygunsuzluğu -İlaçlar -HemoglobinopaUler -Sepsis  ABO uyuşmazlığı Anne 0, bebek A veya B kan grubu Kan grubu uyuşmazlıkları içinde en sık İlk gebelikte de ortaya çıkabilir Sarılık ilk 24 saaQe başlar Anemi genellikle hafif D. Coombs tes[ nega[f veya zayıf pozi[f Rh uyuşmazlığına göre daha selim  Rh İzoimmünizasyonu İzoimmünizasyon, Rh (D)-negaUf annenin Rh (D)- poziUf eritrositlerle karşılaşmasıyla olur. Anne kanının fetal eritrositlerle karşılaşması; doğum olayı, düşükler yada fetomaternal transfüzyonla olabilmektedir. 0.1 ml fetal eritrosit yeterli  Rh (D)-pozi[f eritrositlerle annenin ilk karşılaşmasında Ig M yapısındaki an[korlar oluşur. İkinci karşılaşmada Ig G yapısındaki an[korlar ortaya çıkarlar ve plasentayı geçerek hemolizi başla;rlar.(ikinci bebekte risk artar)  Hafif olgularda; Coombs pozi*fliği dışında, hiperbilirubinemi ve anemi belirgin değildir. Orta ağırlıktaki olgularda; Belirgin hemoliz ve hiperbilirubinemi vardır. İleri derecede ağır olgularda; Ağır anemi, hepatoselüler hasar, hipoproteinemi ve hidrops gelişir.  Rhogam AnU D immunglobulin Gebeliğin 7. ayında ve doğumdan sonra ilk 72 saa\e Annede İDC(-) /bebekte DC(-) ise  ARTMIŞ BİLİRÜBİN YÜKÜ HEMOLİTİK NEDENLER HEMOLİTİK OLMAYAN NEDENLER İndirekt bilirübin ReUkülosit normal ARTMIŞ ENTEROHEPATİK EKSTRAVASKÜLER KAYNAKLAR POLİSİTEMİ SİRKÜLASYON Sefal hematom Fetal-maternal transfüzyon KisUk fibrozis Ekimoz İkizden ikize transfüzyon Pilor stenozu SSS kanamaları Göbek kordonunun geç İlial atrezi Yutulmuş kan klemplenmesi Hirschprung hastalığı Anne sütü sarılığı  Bilirubinin hepatosite uptake ve transport kusuru Konjugasyonun yetersiz olması  Azalmış Bilirübin Konjugasyonu ve Karaciğer Uptake Kusuru İndirekt bilirübin Normal reAkülosit oranı Crigler –Najjar sendromu Ap I –II Gilbert Sendromu Anne sütü Sarılığı HipoAroidi Ligandin (Y proteini ) azlığı Y ve Z proteinlerinin başka anyonlar taraMndan bağlanması Fizyolojik sarılık Yetersiz Bilirübin AOlımı  Yetersiz Bilirübin AOlımı Direkt ve İndirekt Bilirübin NegaUf Coombs tesU Direkt Bilirübin 2 mg/dl Direkt Bilirübin T. Bil. %20 sinden fazla olması BİLİYER OBSTRÜKSİYON KROMOZOMAL ANOMALİLER İLAÇLAR ENFEKSİYONLAR METABOLİK BOZUKLUKLAR Biliyer atrezi Turner Sendromu Asetominofen Sepsis Koledeok kisI Trizomi 18-21 Alkol Galaktozemi Primer sklerozan kolanjit İdrar yolu enfeksiyonu KisUk Fibrozis Dubin Johnson Sendromu Rifampisin Sifiliz Alfa 1 anUtripsin eksikliği Rotor Sendromu Eritromisin Toksoplazmozis Glikojen depo hastalıkları Neoplazmlar KorUkosteroid Tüberküloz Gaucher Hastalığı Safra taşı Tetrasiklin HepaUt HipoUroidi Rubella Wilson Hastalığı Herpes Nieman Pick Hastalığı  Bilirubin EnsefalopaDsi Bilirubin Ensefalopa0si: Akut klinik bulgular Kernikterus : Kronik ve kalıcı sekel Mortalite % 10, uzun dönem morbidite %70  Akut bilirubin ensefalopaAsinde klinik bulgular EVRE 1 (ilk birkaç gün) : Uyuklama, hipotoni, emmede azalma EVRE 2 (3-7 gün): Ekstansör kaslarda hipertoni, +/- opistotonus, stupor, iritabilite, retrokollis Hastaların bir kısmında acil kan değişimi ile SSS bulguları geri dönebilir EVRE 3 (1 haVadan büyük bebek) : İrreversibl SSS hasarı, opistotonus-retrokollis, 0z sesle ağlama, beslenememe, apne, ateş, derin stupor-koma, nöbet ve ölüm  Bilirubin ensefalopa1sinde klinik sınıflandırma 1. Klasik kernikterus 2. İşitsel kernikterus 3. Motor kernikterus 4. Hafif “subtle” kernikterus (BIND) Erken tanı ve tedavi ile kern ikterus önlenebilir !  Amaç Ciddi/ağır hiperbilirubinemi gelişimini önlemek Bilirubin ensefalopaAsi ve kernikterus gelişimini önlemek Anksiyeteyi azaltmak Anne sütünün azalmasını önlemek, Gereksiz tedavi ve maliyeA azaltmak Pediatrics 2004 Ağır hiperbilirubinemi ve nörotoksisite risk faktörleri  conducted for previously unaddressed topics (photother adverse effects and effectiveness of intravenous immune to prevent exchange transfusion). CLINICAL PRACTICE GUIDELINE New evidence indicates that neurotoxicity does n RESULTS: Guidance for the Clinician in Rendering Pediatric Care bilirubin concentrations are well above the 2004 exchan thresholds. Systematic review of phototherapy-associate found limited and/or inconsistent evidence of late adver including cancer and epilepsy. IVIG has unclear benefit fo exchange transfusion in infants with isoimmune hemolyt Clinical Practice Guideline a possible Revision: risk of harm due to necrotizing enterocolitis. Management ofLIMITATIONS: Hyperbilirubinemia in The search was limited to 1 database and English the Newborn Infant 35 CONCLUSIONS: or More Accumulated evidence justified narrowly rais Weeks of Gestation treatment thresholds in the updated clinical practice gui evidence for effectiveness with some evidence of risk of Alex R. Kemper, MD, MPH, MS, FAAP,a Thomas B. Newman, MD, MPH, FAAP,b Jonathan L. Slaughter, MD, MPH, FAAP,c M. Jeffrey Maisels, MB BCh, DSc, FAAP,d Jon F. Watchko, MD, FAAP,e Stephen M. Downs, MD, MS,f Randall W. Grout, MD, MS, FAAP,g David G. Bundy, MD, MPH, FAAP,h Ann R. Stark, MD, FAAP,i Debra L. Bogen, MD, FAAP,j the revised recommendations to limit IVIG use. Alison Volpe Holmes, MD, MPH, FAAP,k Lori B. Feldman-Winter, MD, MPH, FAAP,l Vinod K. Bhutani, MD,m Steven R. Brown, MD, FAAFP,n Gabriela M. Maradiaga Panayotti, MD, FAAP,o Kymika Okechukwu, MPA,p Peter D. Rappo, MD, FAAP,q Terri L. Russell, DNP, APN, NNP-BCr More than 80% of newborn infants will have some degree of a Division of Primary Care Pediatrics, Nationwide Children’s Hospital, aundice.1,2 Careful monitoring of all newborn infants and the PEDIATRICS VolumeColumbus,150, numberof3, Ohio; bDepartments September Epidemiology 2022:e2022058865 & Biostatistics and application of appropriate treatments are essential, because high Pediatrics, School of Medicine, University of California, San Francisco, San Downloaded bilirubin concentrations can cause acute bilirubin from http://publications.aap.org/pediatrics/article-pdf/150/3/e2022058865/1358160/peds_2022 encephalopathy and Francisco, California; cCenter for Perinatal Research, Nationwide Children’s by guest kernicterus.3 Kernicterus is a permanent disabling neurologic condition Hospital, Columbus, Ohio; dDepartment of Pediatrics, Oakland University on 08 September 2022 William Beaumont School of Medicine, Rochester, Michigan; eDepartment characterized by some or all of the following: choreoathetoid cerebral of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania; fDepartment of Pediatrics, Wake Forest University, Winston- palsy, upward gaze paresis, enamel dysplasia of deciduous teeth, Salem, North Carolina; gChildren’s Health Services Research, Indiana sensorineural hearing loss or auditory neuropathy or dyssynchrony University School of Medicine, Indianapolis, Indiana; hMedical University of 4 South Carolina, Charleston, South Carolina; iDepartment of Neonatology, spectrum disorder, and characteristic findings on brain MRI. A Beth Israel Deaconess Medical Center, Boston, Massachusetts; jAllegheny description of kernicterus nomenclature is provided in Appendix A. County Health Department, Pittsburgh, Pennsylvania; kGeisel School of  TÜRK NEONATOLOJİ DERNEĞİ YENİDOĞAN SARILIKLARINDA YAKLAŞIM, İZLEM VE TEDAVİ REHBERİ 2022 GÜNCELLEMESİ Prof. Dr. Asuman ÇOBAN Prof. Dr. Münevver KAYNAK TÜRKMEN Prof. Dr. Tuğba GÜRSOY  Tanımlamalar Anlamlı hiperbilirubinemi Postnatal yaş ve sarılık nedenine göre değişen, fototerapi gerek8ren bilirubin düzeyi (8pik olarak TSB ≥ 12 mg/dL). Ciddi hiperbilirubinemi Postnatal yaş ve sarılık nedenine göre değişen, kan de- ğişimi sınırına yakın veya sınırında bilirubin düzeyi (TSB ≥ 20 mg/dL) veya hafif derecede akut bilirubin ensefalopa8si erken bulgularıyla seyreden herhangi bir yüksek TSB düzeyi. Aşırı hiperbilirubinemi Kan değişimi sınırında bilirubin düzeyi (TSB ≥ 25 mg/dL), veya hafif-orta derecede akut bilirubin ensefalopa8si bulgularıyla seyreden herhangi bir yüksek TSB düzeyi. Zararlı veya kriUk hiperbilirubinemi Kan değişimi sınırında bilirubin düzeyi (TSB ≥ 30 mg/dL), veya orta-ağır derecede akut bilirubin ensefalopa8si bulgularıyla seyreden herhangi bir yüksek TSB düzeyi  Topics addressed in the previous clinical DATA EXTRACTION: (2004) and follow-up commentary (2009) were update evidence published through March 2022. Evidence revie conducted for previously unaddressed topics (photothe Ciddi hiperbilirubinemi için risk faktörleri (AAP) adverse effects and effectiveness of intravenous immun to prevent exchange transfusion). New evidence indicates that neurotoxicity does RESULTS: 1. bilirubinyüksek-risk Taburculuk öncesi TSB veya TcB’nin concentrations areorta-yüksek veya well above the 2004 exchan risk bölgesinde olması thresholds. Systematic review of phototherapy-associate found limited and/or inconsistent evidence of late adve 2. Erken doğum (< 40 haQa) including cancer and epilepsy. IVIG has unclear benefit f 3. Tümüyle anne sütüyle beslenme (emzirme exchange iyi değil transfusion ve/veya in infants withaşırı tarW hemoly isoimmune kaybı %8-10) a possible risk of harm due to necrotizing enterocolitis. 4. İlk 24 saaZe gözle görülen sarılık LIMITATIONS: The search was limited to 1 database and Englis 5. İzoimmun ya da diğer hemoli\k hastalıklar (Örn G6PD eks) CONCLUSIONS: Accumulated evidence justified narrowly rai 6. Önceki kardeşte/ebeveyndetreatment FT/kan değişimi gerek\ren sarılık thresholds in the updated clinical practice gu 7. Sefal hematom veya geniş ekimozlar evidence for effectiveness with some evidence of risk of 8. Down sendromu the revised recommendations to limit IVIG use. 9. Taburculuk öncesi FT 10. Diyabe\k annenin makrozomik bebeği PEDIATRICS Volume 150, number 3, September 2022:e2022058865 Downloaded from http://publications.aap.org/pediatrics/article-pdf/150/3/e2022058865/1358160/peds_202 by guest  değildir. hazları geliştirme, akıllı telefonlarla yenidoğan sarılığının 10.Tedavi gerektiren tüm sarılıklı bebeklerde total bilirubin Ciddi hiperbilirubinemi için risk faktörleri ölçülmesi çalışmaları mevcuttur (79-81). Bu çalışmalarda ile birlikte direkt bilirubin ölçülmedir. skleral konjunktival bilirubin ile TSB arasında iyi korelas- (TND) 11.Direkt hiperbilirubinemi her zaman patolojiktir. Tablo 4. Ağır hiperbilirubinemi için önemli risk faktörleri Taburcu olmadan önceki TSB veya TcB düzeyi yüksek veya yüksek –orta risk bölgesinde olması Düşük gebelik haftası ( 15 mg/dL ise TSB ölçülmeli Bilirubin araş hızı ilk 24 saa\e > 0.3 mg/dL/saat veya 24 saa\en sonra > 0.2 mg/dL/saat ise hemolizi düşün (DAT, ETCO…)  TCB : Öneriler (TND) Aşağıdaki durumlarda TSB bakılması önerilir 1. TcB > 13 mg/dL olan bebekler 2. İlk 24 saaXe sarılığın görüldüğü bebekler 3. Tedavi kararı verilen bebekler 4. Fototerapi tedavisi alan bebekler 5. Bhutani eğrisine göre 75. persenBlin üzerinde bilirubin değeri olanlar 6. TcB düzeyi, önerilen FT değerinin %70’i kadar olan bebekler  Fototerapi kararı Gebelik yaşı Nörotoksisite risk faktörleri risk factors, and age of the infant concentration should not be that there is a high likelihood of in hours. (Aggregate Evidence subtracted from the total serum exceeding the threshold after Quality Grade X, bilirubin concentration when using discharge.22 Those making the Recommendation) Figs 2 or 3. If the direct-reacting or decision to begin phototherapy below 24 22 20 Total Serum Bilirubin (mg/dL) 18 Phototherapy Thresholds: No Hyperbilirubinemia Neurotoxicity Risk Factors 16 14 Gestational Age ≥ 40 Weeks 12 39 Weeks 38 Weeks 37 Weeks 10 36 Weeks 35 Weeks 8 6 0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180 192 204 216 228 240 252 264 276 288 300 312 324 336 (1d) (2d) (3d) (4d) (5d) (6d) (7d) (8d) (9d) (10d) (11d) (12d) (13d) (14d) Age – hours (days) FIGURE 2 Phototherapy thresholds by gestational age and age in hours for infants with no recognized hyperbilirubinemia neurotoxicity risk factors other than gesta tional age. These thresholds are based on expert opinion rather than strong evidence on when the potential benefits of phototherapy exceed its potential harms. Use total serum bilirubin concentrations; do not subtract direct-reacting or conjugated bilirubin from the total serum bilirubin. In rare cases of  20 18 16 Total Serum Bilirubin (mg/dL) Phototherapy Thresholds: One or More Hyperbilirubinemia Neurotoxicity Risk Factors 14 12 Gestational Age 10 ≥ 38 Weeks 37 Weeks 8 36 Weeks 35 Weeks 6 4 0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180 192 204 216 228 240 252 264 276 288 300 312 324 336 (1d) (2d) (3d) (4d) (5d) (6d) (7d) (8d) (9d) (10d) (11d) (12d) (13d) (14d) Age – hours (days) GURE 3 ototherapy thresholds by gestational age and age in hours for infants with any recognized hyperbilirubinemia neurotoxicity risk factors other than gesta nal age. These thresholds are based on expert opinion rather than strong evidence on when the potential benefits of phototherapy exceed its potential ms. Use total serum bilirubin concentrations; do not subtract the direct-reacting or conjugated bilirubin from the total serum bilirubin. In rare cases of exceeds the escalation-of-care hydration should be initiated and threshold, management should Start (TSB exceeds escalation of care level) STAT labs: total and direct serum bilirubin, CBC, albumin, serum chemistries, type and At appropriate cross match Acute bilirubin encephalopathy Yes Yes location for Notify blood bank OR Urgent exchange exchange Measure TSB at least every 2 hours Latest TSB at or above the transfusion transfusion? Intensive phototherapy and PO+IV hydration exchange transfusion threshold? See IVIG therapy and B/A measurement No options No Consult neonatologist for urgent Yes Return to regular transfer to NICU, directly if TSB below the escalation-of- phototherapy possible care level? guidelines Intensive phototherapy and PO+IV hydration during transfer, if possible No Continue intensive phototherapy and PO+IV hydration and measuring TSB at least every 2 hours FIGURE 4 Approach to escalation of care. The escalation-of-care threshold is 2 mg/dL below the exchange transfusion threshold. IVIG, intravenous immune globulin; B/A, bilirubin to albumin ratio.  28 26 Total Serum Bilirubin (mg/dL) 24 Exchange Transfusion Thresholds: No Hyperbilirubinemia Neurotoxicity Risk Factors 22 20 Gestational Age ≥ 38 Weeks 18 37 Weeks 36 Weeks 35 Weeks 16 14 0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180 192 204 216 228 240 252 264 276 288 300 312 324 336 (1d) (2d) (3d) (4d) (5d) (6d) (7d) (8d) (9d) (10d) (11d) (12d) (13d) (14d) Age − hours (days) FIGURE 5 Exchange transfusion thresholds by gestational age for infants with no recognized hyperbilirubinemia neurotoxicity risk factors other than gestational age. See Fig 4, which describes escalation of care. These thresholds are based on expert opinion rather than strong evidence on when the potential benefits of escalation of care exceed its potential harms. The stippled lines for the first 24 hours indicate uncertainty because of the wide range of clinical circumstan-  24 22 Total Serum Bilirubin (mg/dL) 20 Exchange Transfusion Thresholds: 1 or More Hyperbilirubinemia Neurotoxicity Risk Factors 18 Gestational Age 16 ≥ 38 Weeks 37 Weeks 36 Weeks 35 Weeks 14 12 0 12 24 36 48 60 72 84 96 108 120 132 144 156 168 180 192 204 216 228 240 252 264 276 288 300 312 324 336 (1d) (2d) (3d) (4d) (5d) (6d) (7d) (8d) (9d) (10d) (11d) (12d) (13d) (14d) Age – hours (days) IGURE 6 xchange transfusion thresholds by gestational age for infants with any recognized hyperbilirubinemia neurotoxicity risk factors other than gestational  Start (TcB or TSB measure in infant ≥12 hours old) Determine hour-specific phototherapy threshold based on gestational age and presence of a known hyperbilirubinemia neurotoxicity risk factor (Table TSB at or above the Yes 2) from Figure 2 or Figure 3 phototherapy Begin phototherapy Measure TSB if TcB exceeds 3.0 mg/dL below the phototherapy treatment threshold? threshold or if the TcB is ≥15 mg/dL. No Phototherapy threshold minus TcB or TSB Discharge Recommendations 0.1-1.9 mg/dL Age