Person: UYSAL, HARUN
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Publication Metadata only Utility of the gastro-laryngeal tube during transesophageal echocardiography: A prospective randomized clinical trial.(2022-12-16) Calim M.; Uysal H.; Kahraman Ay N.; Karaaslan K.; Daskaya H.; ÇALIM, MUHITTIN; UYSAL, HARUN; DAŞKAYA, HAYRETTİNPublication Metadata only Determining the Length of Stay in the Intensive Care Unit of Patients With Sepsis Who Underwent Hemoadsorption Using the Artificial Neural Networks Model(2022-03-01) Koç S.; Uysal H.; UYSAL, HARUNObjectives: The artificial neural network (ANN) analysis was used to predict the exact staying period of patients with sepsis have undergone hemoadsorption hospitalized in the General Intensive Care Unit (ICU) of X University Hospital. Methods: ANN model was successfully developed having 21 neurons in total (11 neurons at the first level, 9 neurons at the second level, and 1 neuron at the third level) using a computer program. The created network was trained using our real data and the fermi function of the system was determined by the program and then the program was able to predict possible staying time prior to real-time. Main variables of interest: Staying periods (days), ages, number of comorbidities, hemoperfusion periods (days), blood pH, C-Reactive Protein, procalcitonin, and blood lactate levels were inputs. Results: Predicted values were plotted to real data and the determination coefficient was found to be r2= 0.802. This relation was found to be good to predict the possible staying times of patients with sepsis treated using hemoadsorption in the ICU for a better organization and reducing the total cost. Conclusion: The artificial neural network modeling and this prediction were found to be useful for predicting patients staying time at ICUPublication Metadata only General Anesthesia versus Sedation in Multi ParametricMagnetic Resonance Imaging (mpMRI) TransrectalUltrasound Guided (TRUS) Fusion Targeted Prostate Biopsy:A Prospective, Randomized Study(2022-03-01) Uysal H.; Koç S.; UYSAL, HARUNObjectives: Different anesthetic methods have been used in multi-parametric magnetic resonance imaging-guided (mpMRI) transrectal ultrasound guidance (TRUS) fusion-targeted prostate biopsy, but the consensus on the optimal anesthetic approach is not clear. In this study, the anesthesia management, procedural conditions, intraoperative adverse events, complications, discharge criteria, and cancer detection rates of general anesthesia and sedation were compared. Methods: Participants were randomly divided into general anesthesia (GA) and sedation (S) groups. The primary endpoint of the study was the surgical satisfaction score. The incidence of hypoxia, patient satisfaction, cancer detection rate, anesthetic agent consumption, recovery and hospitalization times, and complication rates were all compared as secondary outcomes. Results: There was no significant difference in the incidence of hypoxemia in both groups (Group G:0, Group S:2 patients, p=0.494). While there was no significant difference in surgical satisfaction scores (Group GA: 9.48 vs Group S: 9.23, p=0.353). PC detection rates (p=0.809) and complication rates were similar. Conclusion: With similar surgical conditions, complication incidence, and cancer detection rates, neither anesthesia approach did not provide surgical superiority over the other. The sedation approach, combined with careful monitoring of anesthesia depth, prevented hypoxemia, reduced anesthetic agent consumption, and allowed for faster recovery and discharge, allowing for ambulatory anesthesia.Publication Metadata only Analysis of the effects of ha-330 hemo adsorption column application on mortality andmorbidity of adult patients with sepsis in general intensive care unit(2022-03-01) Koç S.; Dokur M.; Uysal H.; UYSAL, HARUNSepsis is a leading cause of mortality and morbidity in critical care units. The objective of this study is to evaluate if using the HA-330 Sepsis Adsorption Column in the intensive care unit reduces mortality and morbidity in patients with sepsis. This study was designed as a retrospective study evaluating demographic and laboratory parameters in the General Intensive Care Unit. The study involved 200 sepsis patients who were followed in the critical care unit between June 2019- December 2020 and were treated with the HA-330 Sepsis Adsorption Column. The length of hemoperfusion in patients who died was statistically significantly longer than in patients who recovered. The mean lactate and the hemoperfusion time/day value had a weak but significant negative correlation. In the recovery and death observations, the improvements in repeated C-reactive protein and Procalcitonin measurements were statistically significantly different. The differences in repeated pH and lactate measurements in the recovery and mortality observations were statistically identical, according to the observations. Sepsis-related deaths are a severe issue in critical care units. HA-330 Sepsis Adsorption Column, it can be thought that it can be an inexpensive, useful, and effective method that can be used in the prognosis of the patients. In antibiotic-resistant sepsis, hemoperfusion may be recommended.Publication Metadata only MUKOPOLİSAKKARİDOZ TİP 3 TANISI OLAN HASTADA ANESTEZİ DENEYİMİMİZ: OLGU SUNUMU(2022-02-20) Sümer İ.; Esen A.; Uysal H.; SÜMER, İSMAİL; ESEN, ASIM; UYSAL, HARUNMUKOPOLİSAKKARİDOZ TİP 3 TANISI OLAN HASTADA ANESTEZİ DENEYİMİMİZ: OLGU SUNUMUDr. Öğr. Üyesi. İsmail SÜMERBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0002-0133-0218drismailsumer@gmail.com, 0533 2201576Dr. Öğr. Üyesi. Asım ESENBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0001-7222-7499esen1122@hotmail.com, 0533 5219455Dr. Öğr. Üyesi. Harun UYSALBezmialem Vakıf Üniversitesi, Tıp FakültesiORCID ID: https://orcid.org/0000-0003-0426-8525drharunuysal@hotmail.com, 0506 3433494ÖzetMukopolisakkaridozlar (MPS), lizozomal enzim eksikliğine bağlı olarak glikozaminoglikan vucutta birikimi ile giden, multisistem tutulum gösteren, kalıtsal, kronik ve ilerleyici bir metabolik depo hastalığıdır. Enzim eksikliğine bağlı olarak biriken maddeler hücrelerin normal işlevlerini bozarlar ADDIN EN.CITE <EndNote><Cite><Author>Danos</Author><Year>1995</Year><RecNum>7</RecNum><DisplayText>(1)</DisplayText><record><rec-number>7</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717026">7</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Danos, O.</author><author>Heard, J. M.</author></authors></contributors><auth-address>Laboratoire Rétrovirus et Transfert Génétique, Institut Pasteur, Paris, France.</auth-address><titles><title>Mucopolysaccharidosis</title><secondary-title>Mol Cell Biol Hum Dis Ser</secondary-title></titles><periodical><full-title>Mol Cell Biol Hum Dis Ser</full-title></periodical><pages>350-67</pages><volume>5</volume><edition>1995/01/01</edition><keywords><keyword>Animals</keyword><keyword>Bone Marrow Transplantation</keyword><keyword>Central Nervous System/metabolism</keyword><keyword>Clinical Trials as Topic</keyword><keyword>Disease Models, Animal</keyword><keyword>Genetic Therapy</keyword><keyword>Humans</keyword><keyword>Mucopolysaccharidoses/genetics/physiopathology/*therapy</keyword></keywords><dates><year>1995</year></dates><isbn>1470-0573 (Print)
1470-0573</isbn><accession-num>9532574</accession-num><urls></urls><electronic-resource-num>10.1007/978-94-011-0547-7_17</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(1). Mukopolisakkaritler bağ doku komponenti oldukları için biriktikleri dokularda farklı etkilere sebep olabilirler. Deri, solunum yolları, santral sinir sistemi kalp, karaciğer, dalak, kemik ve ligamanlarda depolanır ve bu dokular etkilenebilir. Tipik klinik belirtiler; kaba yüz görünümü, kulak burun boğaz problemleri, iskelet displazileri, büyüme gelişme geriliği, servikal instabilite, servikal kord kompresyonu, organomegali, görme ve işitme bozuklukları, eklem kontraktürleri, herniler ve kardiyopulmoner problemler olarak sıralanabilir ADDIN EN.CITE <EndNote><Cite><Author>Kliegman</Author><Year>2011</Year><RecNum>8</RecNum><DisplayText>(2)</DisplayText><record><rec-number>8</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717817">8</key></foreign-keys><ref-type name="Book">6</ref-type><contributors><authors><author>Kliegman, R., </author><author>Stanton, B., </author><author>St. Geme, J. W.,</author><author>Schor, N. F.,</author><author>Behrman, R. E. </author><author> </author></authors></contributors><titles><title><style face="normal" font="default" charset="238" size="100%">Nelson Textbook of Pediatrics</style></title></titles><pages><style face="normal" font="default" charset="238" size="100%">480-510</style></pages><volume><style face="normal" font="default" charset="238" size="100%">19th edition</style></volume><dates><year><style face="normal" font="default" charset="238" size="100%">2011</style></year></dates><pub-location>Phialdelphia</pub-location><publisher>Elsevier</publisher><urls></urls></record></Cite></EndNote>(2).MPS tip III olan hastalarda, heparan sülfat yıkımında yetersizlik mevcuttur. Ortak özellikleri hafif somatik bulgularla birlikte yavaş ilerleyen ağır SSS tutulumudur. Bu hastalar genelde MSS tutulumuna bağlı ilerleyici psikomotor gerilik gösterir. Erken dönemde yüzde kabalaşma görülür, nörolojik belirtiler genellikle 2-4 yaş arasında belirlenir ADDIN EN.CITE <EndNote><Cite><Author>Kliegman</Author><Year>2011</Year><RecNum>8</RecNum><DisplayText>(2)</DisplayText><record><rec-number>8</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643717817">8</key></foreign-keys><ref-type name="Book">6</ref-type><contributors><authors><author>Kliegman, R., </author><author>Stanton, B., </author><author>St. Geme, J. W.,</author><author>Schor, N. F.,</author><author>Behrman, R. E. </author><author> </author></authors></contributors><titles><title><style face="normal" font="default" charset="238" size="100%">Nelson Textbook of Pediatrics</style></title></titles><pages><style face="normal" font="default" charset="238" size="100%">480-510</style></pages><volume><style face="normal" font="default" charset="238" size="100%">19th edition</style></volume><dates><year><style face="normal" font="default" charset="238" size="100%">2011</style></year></dates><pub-location>Phialdelphia</pub-location><publisher>Elsevier</publisher><urls></urls></record></Cite></EndNote>(2).Organ patolojileri nedeniyle sık operasyon gerektiren bu hastaların anestezisi sistemik sorunlar nedeniyle özellik gösterir. Özellikle yüz deformitesi, nazofaringeal mukopolisakkarid birikimi ve eklem-kemik deformitelerine bağlı hava yolu sağlanmasındaki güçlük bu hastalarda sık karşılaşılan bir sorundur ADDIN EN.CITE <EndNote><Cite><Author>Kamin</Author><Year>2008</Year><RecNum>9</RecNum><DisplayText>(3)</DisplayText><record><rec-number>9</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718047">9</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Kamin, W.</author></authors></contributors><auth-address>Pediatric Pneumology, Allergy, Endoscopy and Cystic Fibrosis Center, Children's Hospital, University of Mainz, Mainz, Germany. kamin@kinder.klinik.uni-mainz.de</auth-address><titles><title>Diagnosis and management of respiratory involvement in Hunter syndrome</title><secondary-title>Acta Paediatr</secondary-title></titles><periodical><full-title>Acta Paediatr</full-title></periodical><pages>57-60</pages><volume>97</volume><number>457</number><edition>2008/05/28</edition><keywords><keyword>Airway Obstruction/etiology</keyword><keyword>Anesthesia, General</keyword><keyword>Continuous Positive Airway Pressure</keyword><keyword>Forced Expiratory Volume</keyword><keyword>Humans</keyword><keyword>Magnetic Resonance Imaging</keyword><keyword>Mucopolysaccharidosis II/*complications/diagnosis/physiopathology/therapy</keyword><keyword>Respiration Disorders/diagnosis/etiology/*therapy</keyword><keyword>Sleep Apnea, Obstructive/etiology/therapy</keyword><keyword>Spirometry</keyword><keyword>Vital Capacity</keyword></keywords><dates><year>2008</year><pub-dates><date>Apr</date></pub-dates></dates><isbn>0803-5253</isbn><accession-num>18339190</accession-num><urls></urls><electronic-resource-num>10.1111/j.1651-2227.2008.00650.x</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(3). Bu hastaların tipik anestezik problemleri; indüksiyon veya ekstübasyon sonrası görülen havayolu obstrüksiyonları, entübasyon zorlukları ve/ veya başarısızlıkları, acil trakeostomi ihtiyaçları, kardiyovasküler ve servikal vertebra sorunlarını içerir ADDIN EN.CITE ADDIN EN.CITE.DATA 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(4) 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Bu olgu sunumunda, MPS tip 3 tanılı diş cerrahisi geçirecek olan hastanın anestezi yönetiminden bahsedilmiştir.Olgu:Olgumuzda diş tedavisi amacıyla anestezi uygulanan MPS Tip 3 hastası 10 yaşında, 35 kg ağırlığında erkek çocuğu sunulmaktadır. Hastanın fizik muayenesinde büyük kafa ve dil, boyunda hareket kısıtlılığı ve mental retardasyon mevcuttu.(Resim 1) Labaratuvar tetkiklerinde bir sorun yoktu. Anestezi öncesinde çocuk nöroloji, çocuk metabolizma hastalıkları ve çocuk kardiyoloji konsültasyonları istendi. Konvulziyon riski nedeniyle antiepileptiklere devam etmesi, anlantoaksiyal subluksasyon riski nedeniyle entübasyonda dikkatli olunması gerektiği belirtildi. Ayrıca çocuk kardiyoloji tarafından yapılan ekokardiyografide MPS’ye bağlı hafif-orta mitral yetmezlik ve hafif aort yetmezliği tespit edildi. İşlem öncesinde endokardit proflaksisi önerildi.Hastaya işlemden 30 dk önce ampisilin 50 mg/kg uygulandı, başka herhangi bir premedikasyon uygulanmadı, operasyona alınan hasta için zor entübasyon şartları hazırlandı. Rutin monitorizasyonu takiben sol el dorsalinden 24G intraket ile damar yolu açıldı. İndüksiyon 1 mg midozolam, 3 mg/kg propofol ve 1mcg/kg fentanil ile yapıldı. Preoperatif ventilasyon sırasında airway yerleştirildi ve ventilasyonda zorluk yaşanmadı, rocuronyum 0,6 mg/kg uygulandı. Sol burun deliğinden yerleştirilen 5 numara spiralli tüp, video laringoskop ve Magill pensi yardımıyla ilerletilerek hasta nazal olarak entübe edildi.(Resim 2) Genel anestezi idamesi %50-%50 oksijen-medikal hava ve %2 sevofluran ve 0.15-0.3 mg/kg/dk remifentanil infuzyonu ile sağlandı. Postoperatif analjezi için 500 mg IV parasetamol uygulandı. Operasyon sonunda 100 mg sugammadex uygulandı ve ventilasyon problemi olmayan hasta ekstübe edildi. Gözlem amaçlı postoperatif bakım ünitesine alındı. Operasyondan sonra 1 gün yoğun bakımda takip edilen hasta problemsiz bir şekilde taburcu edildi. Tartışma:MPS’li hastaların anestezi açısından preoperatif, perioperatif ve postoperatif dönemde zorlukları ve riskleri vardır. MPS hastalarında preoperatif dönemde iyi bir değerlendirme yapılması gereklidir. Anestezi öncesi değerlendirmede zor entübasyon, zor ventilasyon, kardiyak ve servikal spinal gibi sorunlar değerlendirilir ve gerekli hazırlıkları yapılırsa anestezik riskler oldukça düşer. Hastanın servikal ve trakeolaringeal anatomisi ile altta yatan respiratuar ve kardiyak patofizyolojisi hakkında dikkatli olunmalıdır. Preoperatif olarak başta çocuk nörolojisi ve çocuk kardiyolojisi olmak üzere gerekli bölümlerce konsülte edilmelidir ADDIN EN.CITE ADDIN EN.CITE.DATA 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(4) 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Biz hastamızı preoperatif gerekli bölülerle konsulte ederek gerekli hazırlıkları yaptık ve konsültasyon sonucunda verilen tavsiyelere uyduk. Ayrıca göğüs hastalıkları konsültasyonu istenebilir. MPS hastalarında sıklıkla OSAS görülmektedir ve şiddetli OSAS hikayesi olan hastaların anestezi sırasında havayolu acili yaşama ihtimali yüksektir ADDIN EN.CITE <EndNote><Cite><Author>John</Author><Year>2011</Year><RecNum>11</RecNum><DisplayText>(5)</DisplayText><record><rec-number>11</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718173">11</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>John, A.</author><author>Fagondes, S.</author><author>Schwartz, I.</author><author>Azevedo, A. C.</author><author>Barrios, P.</author><author>Dalcin, P.</author><author>Menna-Barreto, S.</author><author>Giugliani, R.</author></authors></contributors><auth-address>Sleep Disorders Center, Pulmonary Service, Hospital de Clínicas de Porto Alegre, Brazil. angelajohn@terra.com.br</auth-address><titles><title>Sleep abnormalities in untreated patients with mucopolysaccharidosis type VI</title><secondary-title>Am J Med Genet A</secondary-title></titles><periodical><full-title>Am J Med Genet A</full-title></periodical><pages>1546-51</pages><volume>155a</volume><number>7</number><edition>2011/06/04</edition><keywords><keyword>Adolescent</keyword><keyword>Child</keyword><keyword>Child, Preschool</keyword><keyword>Cross-Sectional Studies</keyword><keyword>Echocardiography, Doppler</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Hypertension, Pulmonary/etiology</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidosis VI/*complications</keyword><keyword>Polysomnography</keyword><keyword>Prevalence</keyword><keyword>Respiratory Tract Diseases/pathology</keyword><keyword>Sleep Apnea, Obstructive/diagnosis/epidemiology/*etiology</keyword></keywords><dates><year>2011</year><pub-dates><date>Jul</date></pub-dates></dates><isbn>1552-4825</isbn><accession-num>21638759</accession-num><urls></urls><electronic-resource-num>10.1002/ajmg.a.33902</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(5).MPS’de görülen spinal ve kraniofasiyal anomaliler nedeniyle zor maske ventilasyonu ve entübasyon beklenebilir. Bizim hastamızda bulunan büyük kafa ve dil, boyunda hareket kısıtlılığı nedeniyle işlem öncesi zor entübasyon şartları hazırlandı. Entübasyon sırasında video laringoskop kullanıldı. Ayrıca her türlü ihtimale karşılık laringeal maske airway ve fleksible laringoskop kullanılmaya hazır bir şekilde bekletildi.Hastalar cerrahi bitiminden sonraki en erken dönemde ekstübe olmalıdır. Bu durum entübasyondan kaynaklanan havayolunda oluşabilecek ödemi azaltmaya ve erken nörolojik değerlendirmeye izin verir ADDIN EN.CITE ADDIN EN.CITE.DATA 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.Ekstübasyon tam olarak uyanmadan, yeterli solumadan, güçlü bir şekilde öksürmeden ve bilinçli hareket etmeden gerçekleştirilmemelidir ADDIN EN.CITE <EndNote><Cite><Author>Walker</Author><Year>2003</Year><RecNum>12</RecNum><DisplayText>(6)</DisplayText><record><rec-number>12</rec-number><foreign-keys><key app="EN" db-id="aawxpsrpzwsf9aewp9gv0xw2frwwvr9afwrx" timestamp="1643718223">12</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Walker, R. W.</author><author>Colovic, V.</author><author>Robinson, D. N.</author><author>Dearlove, O. R.</author></authors></contributors><auth-address>Department of Anaesthetisia, Royal Manchester Children's Hospital, Pendlebury, Manchester, UK.</auth-address><titles><title>Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses</title><secondary-title>Paediatr Anaesth</secondary-title></titles><periodical><full-title>Paediatr Anaesth</full-title></periodical><pages>441-7</pages><volume>13</volume><number>5</number><edition>2003/06/07</edition><keywords><keyword>Adolescent</keyword><keyword>Airway Obstruction/*complications/diagnostic imaging</keyword><keyword>Anesthesia/*adverse effects</keyword><keyword>Bronchoscopy</keyword><keyword>Child</keyword><keyword>Decompression, Surgical</keyword><keyword>Humans</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidoses/*complications/diagnostic imaging</keyword><keyword>Mucopolysaccharidosis I/complications</keyword><keyword>Mucopolysaccharidosis II/complications</keyword><keyword>Mucopolysaccharidosis VI/complications</keyword><keyword>Pulmonary Edema/diagnostic imaging/*etiology</keyword><keyword>Radiography</keyword><keyword>Spinal Fusion</keyword></keywords><dates><year>2003</year><pub-dates><date>Jun</date></pub-dates></dates><isbn>1155-5645 (Print)
1155-5645</isbn><accession-num>12791120</accession-num><urls></urls><electronic-resource-num>10.1046/j.1460-9592.2003.00969.x</electronic-resource-num><remote-database-provider>NLM</remote-database-provider><language>eng</language></record></Cite></EndNote>(6) Hastamızı mümkün olduğu kadar erken ekstübe etmek amacıyla kas gevşeticinin geri çevirilmesi için sugammadex kullanıldı. Hastamız sorunsuz şekilde ekstübe edildi.Postoperatif apne riski nedeniyle postoperatif bakım ünitesinde dikkatli takip gerekmektedir. Postoperatif dönemde, hastada oluşabilecek komplikasyonlar ve solunum problemleri olabileceği için hastanın yoğun bakıma transferi düşünülmelidir. MPSli hastalarda multisistem tutulumu olması nedeniyle farklı sistemler etkilenebilir. Hem havayolu problemleri hem de sistemik diğer problemler nedeniyle tüm riskler göz önünde bulundurulmalı ve yaşanabilecek tüm bu zorluklar için dikkatli hazırlık yapılmalıdır.Anahtar Kelimeler: Mukopolisakkaridoz, MPS, MPS tip 3 anestezi yönetimi, pediatrik anestezi ADDIN EN.REFLIST 1. Danos O, Heard JM. Mucopolysaccharidosis. Mol Cell Biol Hum Dis Ser. 1995;5:350-67.2. Kliegman R, Stanton B, St. Geme JW, Schor NF, Behrman RE, . Nelson Textbook of Pediatrics. Phialdelphia: Elsevier; 2011. 480-510 p.3. Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr. 2008;97(457):57-60.4. Walker R, Belani KG, Braunlin EA, Bruce IA, Hack H, Harmatz PR, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36(2):211-9.5. John A, Fagondes S, Schwartz I, Azevedo AC, Barrios P, Dalcin P, et al. Sleep abnormalities in untreated patients with mucopolysaccharidosis type VI. Am J Med Genet A. 2011;155a(7):1546-51.6. Walker RW, Colovic V, Robinson DN, Dearlove OR. Postobstructive pulmonary oedema during anaesthesia in children with mucopolysaccharidoses. Paediatr Anaesth. 2003;13(5):441-7.Publication Metadata only Genel anestezi altında diş tedavisi planlanan akçaağaç şurubu idrar hastalığı olan hastada anestezik yaklaşım(2022-02-13) Esen A.; Uysal H.; Sümer İ.; ESEN, ASIM; UYSAL, HARUN; SÜMER, İSMAİLGenel anestezi altında diş tedavisi planlanan akçaağaç şurubu idrar hastalığı olan hastada anestezik yaklaşımDr. Öğr. Üyesi Asım ESENBezmialem Vakıf Üniversitesi Tıp Fakültesi Anesteziyoloji ve Reanimasyon AD, esen1122@hotmail.com, ORCİD No: 0000-0001-7222-7499, GSM: +90 533 521 94 55Dr. Öğr. Üyesi Harun UYSALBezmialem Vakıf Üniversitesi Tıp Fakültesi Anesteziyoloji ve Reanimasyon AD, drharunuysal@hotmail.com, ORCİD No: 0000-0003-0426-8525, GSM: +90 506 343 34 94Dr. Öğr. Üyesi İsmail SümerBezmialem Vakıf Üniversitesi Tıp Fakültesi Anesteziyoloji ve Reanimasyon AD, isosumer@gmail.com, ORCİD No: 0000-0002-0133-0218 GSM: +90 533 220 15 76GirişAkçaağaç şurubu idrar hastalığı (MSUD) otozomal resesif, doğumsal ve sıklığı yaklaşık 1/185000 olan bir metabolik hastalıktır. Hastalığın sebebi dokularda yaygın olarak bulunan ve dallı zincirli aminoasitlerin oksidatif dekarboksilasyonundan sorumlu bir enzimin eksikliğidir. Bu eksiklik, dallı zincirli aminoasitler olan valin, lösin ve izolösinin kanda ve idrarda birikmesine neden olur. Hastalığın beş ayrı klinik fenotipi vardır. Klinik gidiş, fenotipe ve tedaviye başlanma zamanına bağlı olarak değişir.OlguHastamız 5 yaşında, 22 kg, 96 cm ve anne-babası akraba olan erkek çocuktu. Genel anestezi altında diş tedavisi planlanan hastamızda hafif bir motor-mental ve büyüme-gelişme geriliği mevcuttu. Tedavisine erken dönemde (postpartum onbeşinci gün) başlanan hasta valin-lösin replasman tedavisi, profilaktik antiepileptik ve multivitamin ilaçları almaktaydı. Preoperatif testlerinde önemli bir anormallik yoktu. Çocuk metabolizması ünitesine danışılan hastanın preoperatif açlık döneminde alması için total parenteral nutrisyon (TPN) hazırlandı. 0,5 mg/kg midazolam ile premedike edilen hasta ameliyathaneye alındı ve intravenöz anestezi indüksiyonu sonrası nazotrakeal entübasyon uygulandı. İnhalasyon anestezisi ile idame sağlandı. Ameliyat ve anestezi süreci olaysızdı. Ameliyat esnasında ve derlenme ünitesi sürecinde aralıklı kan şekeri takibi yapıldı. Derlenme ünitesinde yaklaşık yarım saat takip edildi ve her hangi bir problem yaşanmadı. Postoperatif dönemde oral beslenme başlanana kadar TPN nin devam edilmesi önerisiyle hasta servise verildi.Tartışma ve SonuçMSUD hastalarının preoperatif dönemde detaylı olarak değerlendirilmesi gerekir. İyi kontrol edilmiş bir süreçle ameliyata hazırlanan hastaların peroperatif dönemi genellikle sıkıntısız seyretme eğilimindedir. Ancak kontrol altında bir hasta değilse, nörokognitif gelişme geriliği, kasılma epizodları, epileptik nöbetler, hipoglisemi atakları, asidoz, dehidratasyon, beyin ödemi ve kafa içi basınç artışı gibi morbidite ve mortalite ihtimali yüksek problemler görülebilir. Bu nedenle peroperatif dönem için özellikle ilaçların düzenli alınıyor olması, sıvı replasmanı, beslenmeye dikkat ve yakın kan şekeri takibi önemlidir.Anahtar kelimelerAkçaağaç şurubu idrar hastalığı, genel anestezi, havayolu yönetimiAnesthetic approach to the patient with maple syrup urine disease which is planned for dental treatment under general anesthesiaBackroundMaple syrup urine disease (MSUD) is an autosomal recessive congenital metabolic disease. The frequency of disease is approximately 1/185000. The cause of the disease is the deficiency of an enzyme located in the inner membrane of the mitochondria, which is responsible for the oxidative decarboxylation of branched-chain amino acids. This enzyme is found in many tissues such as skeletal muscles, liver, brain and kidney. Deficiency of this enzyme causes the branched-chain amino acids valine, leucine and isoleucine to accumulate in the blood and urine. The disease has five different clinical phenotypes. The clinical course varies depending on the phenotype and the time of initiation of treatment.CaseOur patient is a 5-year-old boy, 22 kg, 96 cm, and whose parents are related. Our patient, who was scheduled for dental treatment under general anesthesia, had mild motor-mental and growth retardation. The patient, whose treatment was started in the early period (postpartum fifteenth day), was taking valine-leucine replacement therapy, some prophylactic antiepileptic drugs and multivitamin drugs. There was no significant abnormality in the preoperative laboratory tests. Total parenteral nutrition (TPN) solution was prepared for the patient to take during the preoperative fasting period, who was consulted with the pediatric metabolism unit. The patient, who was premedicated with 0.5 mg/kg midazolam, was taken into the operating room. After induction of intravenous anesthesia, nasotracheal intubation was performed. Maintenance of anesthesia was provided with inhalation anesthesia. Surgery and anesthesia were uneventful. Intermittent blood glucose monitoring was performed during the surgery and during the recovery unit. He was followed in the recovery unit for about half an hour and no problems were encountered. In the postoperative period, the patient was transferred to the ward with the recommendation to continue TPN until oral feeding was started.Discussion and ConclusionMSUD patients should be evaluated in detail in the preoperative period. The perioperative period of patients who are prepared for surgery with a well-controlled process generally tends to be uneventful. However, if the patient is not under control, problems such as neurocognitive disorders, convulsive episodes, epileptic seizures, hypoglycemia attacks, acidosis, dehydration, brain edema and increased intracranial pressure may be seen with a high probability of morbidity and mortality. For this reason, it is important to take medications regularly, fluid replacement, attention to nutrition and close blood sugar monitoring for the peroperative period.Keywords Maple syrup urine disease, general anesthesia, airway management