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"ABSTRAKLatar belakang. Proses timbulnya inhibitor bersifat multifaktorial baik genetik maupun lingkungan. Beberapa studi telah dilakukan untuk mengetahui faktor risiko terbentuknya inhibitor namun masih terdapat kontroversial pendapat. Tidak seperti di negara maju, di Indonesia skrining inhibitor tidak rutin dilakukan karena keterbatasan biaya dan alat sehingga diperlukan suatu penelitian yang dapat dijadikan acuan pemeriksaan inhibitor selektif.Tujuan. Mengetahui prevalens, karakteristik klinis dan faktor risiko timbulnya inhibitor pada anak dengan hemofilia A di departemen IKA- RSCM.Metode. Uji potong lintang dilakukan pada anak usia ≤18 tahun dengan perdarahan akut di pusat hemofilia terpadu IKA-RSCM. Pada subjek dilakukan pengambilan darah vena dan dilakukan pemeriksaan inhibitor menggunakan metode Bethesda assay. Orangtua diminta mengisi kuesioner mengenai usia saat pertama kali didiagnosis hemofilia, mendapat terapi faktor VIII, jenis terapi pengganti, derajat hemofilia, jenis perdarahan, dan suku bangsa ibu penderita. Analisis bivariat dilakukan dengan uji Fisher. Analisis multivariat tidak dilakukan karena tidak memenuhi syarat.Hasil penelitian. Dari 40 subjek penelitian, didapatkan prevalens inhibitor sebanyak 37,5% (15/40) dengan inhibitor high responder sebanyak 3/15 dan low responder 12/15. Median (rentang) usia subjek penelitian adalah 10 (1,5-18) tahun. Median usia saat diagnosis hemofilia pertama kali ditegakkan dan saat pertama kali mendapat terapi faktor VIII pada inhibitor positif adalah 8 dan 9 bulan. Hampir seluruh subjek (39/40) mendapat terapi konsentrat plasma, 11/15 subjek dengan inhibitor positif mendapat terapi pertama kali sebelum berusia 1 tahun, 14/15 subjek merupakan hemofilia berat dan sebagian besar (12/15) mendapat manifestasi perdarahan sendi. Suku bangsa ibu Jawa lebih sering ditemukan pada inhibitor positif (8/15). Tidak ditemukan hasil yang bermakna secara statistik antara faktor risiko dengan timbulnya inhibitor.Simpulan. Prevalens inhibitor pada penelitian ini sebesar 37,5%. Inhibitor positif lebih sering ditemukan pada penderita hemofilia berat yang mendapat terapi pertama kali sebelum berusia 1 tahun. Penelitian ini tidak berhasil membuktikan faktor risiko bermakna untuk timbulnya inhibitor pada anak dengan hemofilia A.

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ABSTRACTBackground. Several factors may influence inhibitor incidence including genetics and environment. Several studies have been conducted to determine the risk factors for inhibitor formation but there is still a controversial opinion. Unlike in developed countries, in Indonesia inhibitor screening is not routinely performed due to limited funds thus required a research that can be used as reference checks selective inhibitors.Objective. To find out the prevalence, clinical characteristics and risk factors of factor VIII inhibitor in children with hemophilia A in Child Health Department- Cipto Mangunkusumo HospitalMethods. A cross sectional descriptive study conducted in children aged ≤ 18 years old with acute bleeding at the National Hemophilia Care Center, Cipto Mangukusumo Hospital. All the subjects performed venous blood sampling and the examination of inhibitor using the Bethesda assay. Parents were asked to fill out questionnaires on age at first diagnosis of hemophilia, treated with factor VIII replacement therapy type, degree of hemophilia, types of bleeding, and the patient's mother tribes. Bivariate analysis performed by Fisher's test. Multivariate analysis was not performed because it does not qualify.Results. Out of 40 children study, showed prevalence inhibitor 37.5% (15/40) with a high responder inhibitor 3/15 and low responders 12/15. Median (range) age of subjects was 10 (1.5 to 18) years. The median age at diagnosis of hemophilia was first established and the first time the subjects get a factor VIII inhibitor therapy positive was 8 and 9 months. Almost all subjects (39/40) treated with plasma concentrates, 11/15 subjects with a positive inhibitor therapy gets first time before age 1 year, 14/15 subjects is severe hemophilia and most (12/15) of them had joint bleeding manifestations. Java native tribes more often found in the positive inhibitor (8/15). No results found a statistically significant association between the risk factors with the onset of inhibitor.Conclusion. The prevalence of inhibitors in this study was 37.5%. Positive inhibitors was more frequent in patients with severe hemophilia who received therapy for the first time before the age of 1 year old. This study failed to prove significant risk factor for the onset of inhibitors in children with hemophilia A."

"Latar belakang: Pengukuran suhu tubuh sebenarnya (core body temperature) tidak lazim dilakukan pada anak karena invasif dan sulit, sehingga pengukuran suhu aksila dan membran timpani lebih disukai. Namun sampai saat ini ketepatan hasil pengukuran suhu membran timpani dan aksila masih diperdebatkan.Tujuan: Membandingkan ketepatan hasil pengukuran suhu membran timpani dan aksila dengan rektal, mengetahui metode terbaik pengukuran suhu tubuh, dan cut off demam pada anak berdasarkan masing-masing metode pengukuran suhu.Metode: Penelitian diagnostik potong lintang pada anak demam berusia 6 bulan ? 5 tahun yang dipilih secara konsekutif di Poliklinik Anak, IGD Anak, dan Ruang Perawatan Anak Gedung A RSCM antara bulan Desember 2014 ? Januari 2015.Hasil: Dengan cut off demam suhu aksila 37,4oC dan membran timpani 37,6oC didapatkan sensitivitas 96% (IK 95% 0,88-0,98) dan 93% (IK 95% 0,84-0,97), spesifisitas 50% (IK 95% 0,47-0,84) dan 50% (IK 95% 0,31-0,69), NDP 90% (IK 95% 0,81-0,95) dan 85% (IK 95% 0,75-0,91), NDN 83% (IK 95% 0,61-0,94) dan 69% (IK 95% 0,44-0,86). Cut off optimal demam suhu membran timpani dan aksila pada penelitian ini adalah 37,8oC (AUC 0,903 dan 0,903).Simpulan: Hasil pengukuran suhu aksila sama baik dengan membran timpani dalam mendeteksi demam dan dapat digunakan dalam praktik klinis sehari-hari maupun di rumah. Dengan cut off demam 37,8oC didapatkan sensitivitas 81% dan 88%, spesifisitas 86% dan 73%, NDP 95% dan 91%.

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Background: Core body temperature measurement is not common in pediatric population because it is invasive and difficult. Therefore tympanic and axillary temperature measurement are prefereble but their accuracy still debated.Objective: To compare the accuracy of axillary and tympanic temperature to rectal temperature in children with fever and measure the cut off point for fever based on each temperature measurement method.Methods: A cross-sectional diagnostic study was conducted among children age 6 months ? 5 years which was selected consecutively at Pediatric Out-patient clinic, Pediatric emergency unit, and the in-patient wards in building A RSCM from December 2014 to January 2015.Results: With the cut off for fever on axilla 37,4oC and tympanic membrane 37,6oC the sensitivity was 96% (95% CI 0,88-0,98) and 93% (95% CI 0,84-0,97), specificity 50% (95% CI 0,47-0,84) and 50% (95% CI 0,31-0,69), NDP 90% (95% CI 0,81-0,95) and 85% (95% CI 0,75-0,91), NDN 83% (95% CI 0,61-0,94) and 69% (95% CI 0,44-0,86). Optimal cut off of tympanic membrane and axilla temperature was 37,8oC (AUC 0,903 dan 0,903).Conclusions: Axillary temperature measurement is as good as the tympanic membrane temperature measurement and can be used at the daily clinical practice or at home. By increasing the optimum fever cut off point for axilla temperature and tympanic membrane to 37,8oC, we found sensitivity 81% and 88%, specificity 86% and 73%, NDP 95% and 91%."

"Latar belakang: Thalassemia merupakan kelainan hemoglobinopati yang cukup banyak di Indonesia. Terapi utama thalassemia mayor adalah transfusi seumur hidup. Transfusi berulang memiliki efek samping. Salah satunya adalah terbentuknya aloantibodi sel darah merah. Prevalens dan faktor-faktor yang memengaruhi aloantibodi pada pasien thalassemia masih belum ada di Indonesia. Uji Coombs sebagai standar diagnosis merupakan pemeriksaan yang mahal dan hanya tersedia di pusat tertentu. Metode lain yang lebih mudah diperlukan untuk memprediksi terbentuknya aloantibodi tersebut. Tujuan: Untuk mengetahui prevalens aloantibodi sel darah merah di populasi Indonesia dan mendapatkan faktor-faktor yang memengaruhinya. Membuat sistem skoring untuk memprediksikan probabilitas terbentuknya aloantibodi sel darah merah berdasarkan faktor-faktor tersebut. Metode: Analisis terhadap 162 rekam medis subjek yang telah dilakukan uji Coombs di Pusat Thalassemia Jakarta pada tahan 2005-2013. Hasil: Dari 162 subjek didapatkan 31 (19%) subjek memiliki aloantibodi dan 4 (2,4%) subjek menderita AIHA. Jenis aloantibodi terbanyak yang terdeteksi adalah anti-M (29%). Faktor-faktor yang memengaruhi terbentuknya aloantibodi adalah tingginya volume transfusi, jarak antar transfusi, lama transfusi, kadar leukosit dan pajanan PRC biasa. Berdasarkan faktor-faktor risiko tersebut, sistem skoring didisain untuk memprediksi kemungkinan terbentuknya aloantibodi. Kesimpulan: Prevalens aloantibodi pada pasien thalassemia di Indonesia cukup tinggi. Pemberian PRC leukodeplesi pelu direkomendasikan pada pasien dengan transfusi berulang. Prediksi terbentuknya aloantibodi dapat dilakukan melalui sistem skoring terutama di tempat yang tidak tersedia uji Coombs.

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Background: Thalassemia major is a common genetic disease in Indonesia. The principal treatment of thalassemia major is lifelong blood transfusion, which is frequently complicated by alloantibody. Limited data are available on the frequency of RBC alloantibody and factors influencing in major β-thalassemia patients. Coombs test, as a standard tool to diagnose alloantibody, is only available on particular Red Cross Centre. Therefore, it is necessary to find another tool to predict the probability of alloantibody formation.

Aim: To investigate the prevalence of RBC alloantibody among thalassemia major patients in Thalassemia Centre Jakarta. To describe factors influencing RBC alloantibody production and develop scoring system to predict its probability.

Methods: We analyzed the clinical and transfusion records of 162 thalassemia major patients who have been examined for Coombs test. All of the patients were registered in Thalassemia Center, Cipto Mangunkusumo hospital from 2005 until 2013.

Results: Of the 162 subjects, 31 (19%) developed RBC alloantibody and four patients (2,4%) developed autoimmune hemolytic anemia. The most common alloantibody was anti-M (29%).Several factors were found to contribute to high alloantibody rate in this study, including high volume of transfusion, duration of transfusion, white blood count level, transfusion interval, and PRC exposure. From those factors, scoring system has been developed to predict alloantibody formation in thalassemia patients.

Conclusion: We concluded that there is a high rate of RBC alloantibody in major thalassemia patients in our center. We also suggest that leukocyte-poor PRC should be given to all patients with multiple transfusions. In remote area where Coombs test is not available, scoring system can be used to predict the probability of alloantibody formation."