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"ABSTRAKHemartrosis berulang dan artropati merupakan morbiditas utama pada hemofilia A berat. Bagi negara berkembang seperti Indonesia, terapi profilaksis dosis standar tidak terjangkau karena memerlukan biaya yang sangat mahal. Penelitian ini bertujuan mengetahui efektivitas terapi profilaksis sekunder dosis rendah dibandingkan terapi on-demand pada anak hemofilia A berat.Uji klinis acak terbuka selama 24 minggu telah dilakukan pada anak hemofilia A berat berusia 4?18 tahun dengan riwayat perdarahan sendi berulang, di Poliklinik Hematologi Departemen Ilmu Kesehatan Anak FKUI/RSCM. Subjek dialokasikan secara acak menjadi dua kelompok yaitu kelompok profilaksis dan on-demand. Kelompok profilaksis mendapat terapi faktor VIII 10 IU/kgBB 2 kali seminggu, sedangkan kelompok on-demand mendapat terapi sesuai protokol standar. Luaran primer adalah kekerapan perdarahan sendi dan luaran sekunder adalah skor HJHS) dan skor ultrasonografi (HEAD-US). Penelitian ini juga membandingkan kadar CTX-II urin dan inhibitor faktor VIII (Bethesda Assay) pada kedua kelompok.Sejak bulan Juni 2015?Februari 2016 didapatkan 50 subjek yang memenuhi kriteria inklusi. Kekerapan perdarahan sendi pada kelompok profilaksis (5 ± 4,3) lebih baik dari pada kelompok on-demand (8 (3?30)), IK95% 0.9?6.99; p = 0,009. Perubahan skor HJHS pada kedua kelompok menunjukkan perbaikan klinis pada kelompok profilaksis dan perburukan pada kelompok on-demand, walaupun tidak bermakna secara statistik (IK95% -0.99?3; p = 0,320). Skor HEAD-US kelompok profilaksis lebih baik dibandingkan kelompok on-demand (IK95% 2? 8,81; p = 0,003). Perubahan kadar CTX-II urin pada kedua kelompok berbeda bermakna (IK95% 2.777?16.742; p < 0,001). Tidak didapatkan subjek yang terbentuk inhibitor faktor VIII pada kedua kelompok selama penelitian.Dari hasil penelitian ini disimpulkan bahwa terapi profilaksis sekunder dosis rendah efektif mengurangi kekerapan perdarahan sendi, memperbaiki skor HEAD-US dan kadar CTX-II urin, dibandingkan terapi on-demand.

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ABSTRACTRepeated joint bleeds leading to irreversible progressive joint damage (hemophilic arthropathy) is the main problem in children with hemophilia. Current standard prophylacytic treatment in developed countries is beyond our capability as Indonesia has constraint resources. This study aimed to investigate the efficacy and safety of low dose secondary prophylaxis compare to on-demand treatment in children with severe hemophilia A.An open, randomized controlled trial was conducted on severe hemophilia A children aged 4?18 years in Pediatric Hematology-Oncology Division Dr. Cipto Mangunkusumo Hospital for 24 weeks. Eligible subjects were randomized into 2 groups: prophylaxis and on-demand group. All subjects were evaluated at week-0 and week-24 for inhibitor factor VIII (Bethesda Assay), ultrasonography (HEADUS scores) of six index joints (bilateral knees, ankles and elbows), HJHS (version 2.1, 2011) and urinary CTX-II (EIA). Subjects in prophylaxis group received factor VIII 10 IU/kgBW 2 times per week for 24 weeks. Any bleeding episodes in both groups were treated according to standard treatment (on-demand).During June 2015?February 2016 there were 50 subjects enrolled in the study. Mean age in prophylaxis group was 12 ± 3.5 years and median age in on-demand group was 11.9 (6.518.2) years. Mean frequency of joint bleeds in prophylaxis group was 5 ± 4.3 compare to 8 (3?30) in on-demand group (95%CI 0.9?6.99; p = 0.009). Mean difference of HJHS between two groups was not significant (95% CI -0.99?3; p = 0.320). HEAD-US scores and urinary CTX-II in prophylaxis group was significantly better compare to on-demand group (95%CI 2?8.81; p = 0.003 and 95%CI 2,777?16,742; p < 0.001 respectively). No subjects showed showed inhibitor factor VIII in both groups.We conclude that secondary low dose prophylaxis was effective to decrease joint bleeding episodes and improved HJHS scores, HEAD-US scores and urinary CTX-II, compared to on-demand treatment."

"Latar belakang: Evaluasi sendi pada penyandang hemofilia memerlukan metode yang objektif dan terukur. USG sebagai metode yang relatif baru untuk artropati hemofilik AH belum memiliki konsensus sistem skor, sementara MRI telah memiliki sistem skor International Prophylaxis Study Group dari World Federation of Hemophilia IPSG-WFH . Penelitian ini mengembangkan sistem skor USG baru untuk artropati hemofilik lutut tahap dini, dan menilai keselarasannya dengan skor MRI IPSG-WFH dan kadar CTX-II urin.Metode: Penelitian ini menggunakan desain potong lintang. Formulasi skor USG berdasarkan pada studi pustaka terhadap 25 publikasi terkait sejak tahun 1999 - 2015 dan peer review. Terdapat 27 anak penyandang hemofilia A berat yang dipilih secara konsekutif. AH lutut tahap dini ditetapkan berdasarkan klasifikasi radiografi Arnold-Hilgartner derajat 0 - II. USG dan MRI lutut dilakukan dengan penilaian skor MRI IPSG-WFH dan skor USG yang baru. Kadar CTX-II urin ditetapkan dengan pemeriksaan ELISA. Data dianalisis dengan uji Spearman.Hasil: Sistem skor USG baru meliputi komponen efusi sendi, hipertrofi sinovium, hipervaskularisasi sinovium dengan Power Doppler, deposisi hemosiderin, dan kerusakan kartilago pada troklea femoris. Terdapat korelasi sedang antara skor USG dengan skor MRI. Tidak ada korelasi skor USG dengan CTX-II urin.Kesimpulan: Skor US baru ini dapat digunakan sebagai alternatif MRI pada AH lutut tahap dini.

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Introduction Assessment of knee haemophilic arthropathy HA required an objective measures. There was no consensus on preferrable US scoring system, while MRI already had a scoring system developed by the International Prophylaxis Study Group of the World Federation of Hemophilia IPSG WFH. This study developed a new US scoring system for early knee HA and its association with MRI scoring system and urinary CTX II level.Method The study was cross sectional. US scoring system was developed based on literature studies of 25 publications between 1999 - 2015 and peer review. Twenty seven children with severe haemophilia A was recruited consecutively. Early HA was confirmed by radiography as Arnold Hilgartner stage 0 - II. Knee MRI and US were scored using MRI IPSG WFH scoring system and the new US scoring system, while urinary CTX II level was measured using ELISA. Correlation was analyzed using Spearman test.Results US scoring system included joint effusion, synovial hypertrophy, synovial hypervascularization using Power Doppler, hemosiderin deposition, and cartilage damage. Moderate correlation was found between US score and MRI score. There was no correlation between US score and urinary CTX II level.Conclusion The new US score can be used as an alternative for MRI in the assessment of early knee HA."

"ABSTRAKLatar belakang dan tujuan : Artropati hemofilia merupakan salah satu komplikasi yang terjadi pada penyandang hemofilia dengan predileksi pada sendi siku, lutut dan pergelangan kaki. Deteksi dini artropati hemofilia dapat memperbaiki evaluasi dan tatalaksana. Ultrasonografi metode HEAD-US merupakan metode yang dapat digunakan untuk mendeteksi dini kelainan sendi penyandang hemofilia menggunakan sistem skoring yang cepat dan relatif mudah. Evaluasi sendi secara klinis pada artropati hemofilia dilakukan dengan pemeriksaan skor HJHS. Ultrasonografi metode HEAD-US dapat menjadi penunjang evaluasi artropati hemofilia .Metode : Uji korelatif dengan pendekatan potong lintang pada skor ultrasonografi metode HEAD-US dengan skor HJHS. Kedua pemeriksaan dilakukan pada 120 sendi penyandang hemofilia yaitu siku, lutut dan pergelangan kaki. Pemeriksaan HEAD-US dan HJHS dilakukan pada hari yang sama. Hasil : Rerata umur subyek penelitian adalah 9,3 tahun sebaran 5-14 tahun . Pemeriksaan HEAD-US memiliki median 8 dengan sebaran 1-28 dan mayoritas kelainan pada pergelangan kaki. Pemeriksaan HJHS memiliki sebaran skor 0-35 dengan median 3, mayoritas kelainan pada lutut.Kesimpulan : Terdapat korelasi positif kuat antara pemeriksaan ultrasonografi metode HEAD-US dengan pemeriksaan HJHS. p = 0,002, r= 0,65 . Didapatkan formula regresi linear yaitu : skor HJHS = = ndash; 3,74 0,86 x skor HEAD-US. Untuk memperkirakan skor HJHS setelah diketahui skor HEAD-US.

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ABSTRACTBackground and objective Hemophilic artropathy is one of complication of hemophilia. The elbows, knees and ankles are the most affected joints. Detection of early blood induced joint changes may improve monitoring of treatment. HEAD US scanning protocol is a quick and simple method that can be used to detect hemophilic arthropathy. Clinically, hemophilic arthropathy was assessed using HJHS. The aim of this study was to explore the value HEAD US scanning protocol on evaluating hemophilic arthropathy. Methods Cross sectional correlation study between HEAD US and HJHS. Both were done on 120 hemophilic elbow, knee and ankle joints . HEAD US and HJHS were done on the same day. Results Mean age was 9,3 years range 5 14 years . Median score of HEAD US was 8 range 1 28 , mostly on ankles. Median score of HJHS was 3 range 0 35 , mostly on knees. . Conclusion There is strong correlation between HEAD US with HJHS. p 0,002, r 0,65 . Linear regression formula was HJHS score ndash 3,74 0,86 x HEAD US score. This formula can be used to predict HJHS score. "

"ABSTRAKLatar Belakang: Penyakit Graves merupakan penyebab terbanyak hipertiroidisme. Remodeling pada hipertiroidisme dilaporkan meningkat terutama resorpsi tulang. Peningkatan turnover tulang terus menerus bertanggung jawab terhadap percepatan keropos tulang. Tujuan penelitian ini adalah untuk melihat korelasi antara status tiroid dengan kadar ALP, OC sebagai penanda formasi tulang dan CTx sebagai penanda resorpsi tulang.Metode: Metode yang digunakan adalah potong lintang dengan consecutive sampling pada wanita penyakit Graves usia reproduktif di Poliklinik Metabolik Endokrin RSCM pada periode Juli–September 2014. Analisis statistik dilakukan dengan Mann Whitney, korelasi Spearman dan analisis ROC.Hasil: Pada 68 subyek penelitian, didapatkan 28 (41.2%) eutiroid, 23 (33.8%) hipertiroid subklinis dan 17 (25%) hipertiroid. Terdapat perbedaan median kadar penanda remodeling tulang antara kelompok eutiroid dan kelompok belum eutiroid (hipertiroid subklinis/hipertiroid) yaitu ALP (71 U/L [40-165] vs 91.5 U/L [39-256]), OC (19.48 ng/mL [10.95-92.70] vs 32.46 ng/mL [13.31-137.0]), dan CTx (0.36 ng/mL [0.11-1.24] vs 0.613 [0.11-1.93]).Pada uji Spearman didapatkan tidak ada korelasi yang bermakna antara FT4 dengan ALP (r=0.106 p=0.389); terdapat korelasi positif yang bermakna FT4 dengan OC dan CTx (r=0.289 p=0.017 dan r=0.265 p=0.029); terdapat korelasi negatif yang bermakna antara TSH dengan ketiga penanda tulang yaitu ALP (r=- 0.240 p=0.049), OC (r=-0.450 p=<0.001) dan CTx (r=-0.420 p<0.001). Sensitivitas dan spesifisitas diskriminasi TSH dengan kadar serum CTx adalah baik dengan nilai 70.72% dan 70.96% dan titik potong TSH yang didapatkan adalah 0.015 μIU/mL.Simpulan: Median ALP, OC dan CTx pada kelompok belum eutiroid lebih tinggi daripada kelompok eutiroid. Terdapat korelasi positif yang bermakna antara FT4 dengan OC dan CTx. Terdapat korelasi negatif yang bermakna antara TSH dengan ALP, OC dan CTx. Titik potong TSH 0.015μIU/mL merupakan penanda yang sensitif dan spesifik untuk kadar serum CTx.

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ABSTRACTBackground: Grave's disease (GD) is one of the main causes of hyperthyroidism. Bone remodelling has been reported to increase in hyperthyroidisme, especially bone resorption. Continuous increase in bone remodelling has been held responsible for accelerated bone loss. The aim of this study is to find correlation between thyroid status and serum ALP and OC levels as bone formation marker as well as serum CTx as bone resorption marker.Methods: This is a cross-sectional study involving reproductive-age women with GD who attended endocrine metabolic outpatient clinic Cipto Mangunkusumo General Hospital from July to September 2014. Sampling was conducted by mean of consecutive sampling. Statistical analysis was performed using Mann-Whitney, Spearman correlation and ROC analysis.Results: From 68 subjects, 28 (41.2%) were euthyroidism, 23 (33.8%) were subclinical hyperthyroidism and 17 (25%) were hyperthyroidism. We found the difference in median concentration of bone markers between euthyroidism group and non euthyroidism group (subclinical hyperthyroidism/hyperthyroidism) i.e. ALP (71 U/L [40-165] vs 91.5 U/L [39-256]), OC (19.48 ng/mL [10.95-92.70] vs 32.46 ng/mL [13.31-137.0]), and CTx (0.36 ng/mL [0.11-1.24] vs 0.613 [0.11- 1.93]). Spearman test used to find correlation between FT4 and bone markers showed no significant correlation between FT4 and ALP (r=0.106, p=0.389). Nevertheless, FT4 was significantly correlated with OC and CTx in a positive manner (r=0.017 and r=0.265, p=0.029). Correlation between TSH and bone markers was found to be significantly negative (ALP [r=-0.240, p=0.049], OC [r=-0.450, p=<0.001] and CTx [r=-0.420, p=<0.001]). Sensitivity and specificity of TSH discrimination with serum concentration of CTx was 70.72% and 70.96% respectively with obtained cut off for TSH was 0.015 μIU/mL.Conclusion: Median value of the three bone markers are higher in non euthyroidism group compared to that of euthyroid group. The correlation between FT4 and OC or CTx is positive and significant. Cut-off point of 0.015 μIU/mL for TSH is a sensitive and specific marker for serum concentration of CTx."

"Latar belakang: Perdarahan sendi berulang merupakan morbiditas utama pada pasien hemofilia karena dapat menimbulkan artropati hemofilik yang menyebabkan keterbatasan gerak dan disabilitas sehingga menurunkan kualitas hidup. Penelitian bertujuan mengetahui korelasi pemeriksaan klinis sendi, penilaian aktivitas fungsional dan kualitas hidup pada anak hemofilia.Metode: Penelitian dengan desain potong lintang di RSCM pada Agustus−November 2022 pada anak 4−16 tahun, hemofilia A atau B derajat sedang atau berat yang mengalami perdarahan sendi berulang. Penelitian dilakukan dengan menilai HJHS, PedHALshort serta Haemo-QoL dan mencari korelasi skor HJHS dengan skor PedHALshort dan Haemo-QoL.Hasil: Sebanyak 95 subyek hemofilia, dengan hemofilia A (77,3%) dan 70,1% hemofilia berat. Skor HJHS median 4 (1−9), skor PedHALshort median 74,5 (62,73-89,09), skor Haemo-QoL mean (SD) 74,51 (15,58). Skor HJHS berkorelasi negatif sedang dengan PedHALshort (r= -0,462, p< 0,0001), skor HJHS berkorelasi sedang dengan Haemo-QoL (r= 0,469, p< 0,001).Simpulan: Semakin tinggi skor HJHS menunjukkan adanya kerusakan pada sendi maka semakin rendah skor PedHALshort dan semakin tinggi skor Haemo-QoL yang menunjukkan semakin terganggu aktivitas fungsional serta kualitas hidupnya.

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Background: Recurrent joint bleeding is the major morbidity in patient with hemophilia that can cause hemophilic arthropathy causes limitation of daily activities, disability, and reducing quality of life. Research objective are to determine the relationship between the clinical evaluation of joints, the assessment of functional activity determined and assessment of the quality of life with HJHS, so we can diagnose arthropathy, prevent disability and better management.

Methods: Study with cross-sectional design at RSCM on August-November 2022, children aged 4-16, with moderate or severe hemophilia A and B with recurrent joint bleeding. The study was conducted by assessing HJHS, PedHALshort and Haemo-QoL, determine the relationship between HJHS with PedHALshort and Haemo-QoL score.

Result: A total of 95 hemophilia subjects, with hemophilia A (77.3%) and 70.1% severe hemophilia. HJHS median score 4 (1-9), PedHALshort median score 74.5 (62.73-89.09), Haemo-QoL mean (SD) 74.51 (15.58). The HJHS score had a moderate negative correlation with PedHALshort (r= -0.462, p<0.0001), the HJHS score had a moderate correlation with Haemo-QoL (r= 0.469, p<0.001).

Conclusion: The higher of HJHS score indicates a joint disorder, the lower of PedHALshort and the higher of Haemo-QoL indicates the more impaired functional activity and poorer quality of life."

"Latar belakang: Koagulasi intravaskular diseminata (KID) merupakan komplikasi dari sepsis yang ditandai oleh perdarahan dan trombosis mikrovaskular dan berkaitan erat dengan terjadinya disfungsi organ multipel. KID terjadi akibat ketidakseimbangan antara sistem koagulasi dengan sistem fibrinolisis. Plasminogen activator inhibitor type 1 (PAI-1) merupakan protein fase akut yang berperan penting penekanan sistem fibrinolisis. Peningkatan PAI-1 pada sepsis diketahui memiliki korelasi dengan luaran yang buruk.Tujuan: Untuk mengetahui hubungan kadar PAI-1 dengan kejadian KID dan kematian pada pasien sepsis anak.Metode: Penelitian analitik prospektif dilakukan pada 35 subjek sepsis yang dirawat di PICU, Instalasi Gawat Darurat serta Ruang perawatan anak RS Cipto Mangunkusumo antara bulan Januari-April 2015. Pengukuran kadar PAI-1 dilakukan pada hari pertama dan keempat sejak sepsis ditegakkan. Pemeriksaan profil koagulasi sistemik dilakukan pada hari keempat sepsis. Diagnosis KID overt menggunakan skor KID berdasarkan International Society of Thrombosis and Haemostasis. Subjek diikuti sampai hari ke 28 perawatan untuk menilai luaran kematian.Hasil: Kadar PAI-1 lebih tinggi secara bermakna pada sepsis berat. Terdapat perbedaan yang bermakna antara kadar PAI-1 hari keempat dengan hari pertama pada KID non overt (95,25 (SB 46,57) ng/mL vs 60,36 (SB 37,31) ng/mL, p=<0,001) dan subjek hidup (82,47 (SB 44,43) ng/mL vs 58,39 (SB 32,98) ng/mL, p=0,021). Terdapat perbedaan kadar PAI-1 hari keempat dengan hari pertama pada subjek KID overt (111,25 (SB 32,93) ng/mL vs 96,26 (SB 52,84) ng/mL) dan subjek meninggal (99,33 (SB 47,53) ng/mL vs 128,58 (SB 37,12) ng/mL), namun tidak bermakna secara statistik. Korelasi kadar PAI-1 dengan skor KID adalah r = 0,606 (p = <0,001).Simpulan: Kadar PAI-1 mengalami penurunan yang bermakna pada hari keempat sepsis dibanding hari pertama pada subjek yang mengalami KID non-overt dan subjek yang bertahan hidup. Sedangkan pada subjek yang mengalami KID overt dan subjek yang meninggal, kadar PAI-1 hari keempat sepsis tetap tinggi. Terdapat korelasi kuat berbanding lurus antara kadar PAI-1 dengan skor KID.

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Background: Sepsis-induced disseminated intravascular coagulation (DIC) is characterized by massive bleeding and microvascular thrombosis and it is closely related to the development of multiple organ dysfunctions. The imbalance between activation of coagulation system and inhibition of the fibrinolysis system in sepsis leads to the development of DIC. The acute-phase protein, plasminogen activator inhibitor type 1 (PAI-1) is a key element in the inhibition of fibrinolysis. Elevated levels of PAI-1 have been related to worse outcome in sepsis.Objective: To investigate the relationship between plasma PAI-1 level and clinical outcome in children with sepsis.Methods: A total of 35 children with sepsis admitted to Cipto Mangunkusumo hospital between January and April 2015 were enrolled to this analitic prospective study. Plasma PAI-1 was measured on day 1 and 4 since sepsis was diagnosed. Systemic coagulation profile was measured on day 4. The Diagnosis of overt DIC was made using the International Society of Thrombosis and Haemostasis scoring system. Subjects were followed up until death or 28 days of care.Results: PAI-1 levels were significantly higher in severe sepsis. There were significant difference between PAI-1 levels on day 4 compared to day 1 in non- overt DIC subjects (95.25 (SB 46.57) ng/mL vs 60.36 (SB 37.31) ng/mL, p=<0.001) and survivors (82.47 (SB 44.43) ng/mL vs 58.39 (SB 32.98) ng/mL, p=0.021). There were no significant difference between PAI-1 levels on day 4 compared to day 1 in overt DIC subjects (111.25 (SB 32.93) ng/mL vs 96.26 (SB 52.84) ng/mL) and nonsurvivors (99.33 (SB 47.53) ng/mL vs 128.58 (SB 37.12) ng/mL). The correlation observed between PAI-1 and DIC score was r=0.606 (p= < 0.001).Conclusions: There were significant decrease of PAI-1 levels on day 4 compared to day 1 in non-overt DIC subjects and survivors. Meanwhile, in overt DIC subjects and nonsurvivors there were no differences. PAI-1 levels were positively correlated with DIC score."

"Latar Belakang: N-asetilsistein (NAS) memiliki banyak manfaat, salah satunya sebagai antikoksidan dan antiinflamasi. Belum banyak penelitian pemberian NAS pada pasien COVID-19. Pemberian NAS pada pasien COVID-19 derajat berat memiliki hasil luaran yang bervariasi, salah satunya diduga disebabkan lama terapi yang hanya beberapa jamhari. Tujuan: Rancangan penelitian ini adalah kohort retrospektif di ICU RS PELNI, Jakarta. Penelitian ini dimulai setelah mendapat sertifikat etik dan ijin lokasi yang dimulai pada bulan Februari-April 2023. Pengambilan sampel secara consecutive sampling. Kriteria penerimaan meliputi pasien COVID-19 derajat berat dengan usia ≥18 tahun. Kriteria penolakan meliputi pasien sedang hamil/menyusui. Kriteria pengeluaran meliputi pasien meninggal sebelum pemberian NAS mencapai 14 hari. Luaran yang diamati adalah kejadian intubasi, mortalitas, nilai rasio netrofil limfosit, kadar D-dimer, dan CRP. Data penelitian merupakan data sekunder dari rekam medis. Data dianalisis dengan uji statistik yang sesuai menggunakan program SPSS versi 27. Hasil: Didapatkan total 112 pasien dengan 55 pasien tidak mendapatkan terapi NAS dan 57 pasien mendapatkan terapi NAS. Dari hasil analisis bivariat didapatkan pasien dengan terapi NAS memiliki kemungkinan untuk diintubasi sebesar 2,7 kali dan tidak berhubugan dengan mortalitas. Dari hasil analisis multivariat, didapatkan hanya variabel kejadian intubasi yang bermakna terhadap mortalitas. Simpulan: Terapi ajuvan NAS tidak menurunkan kejadian intubasi dan mortalitas.

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Background: N-acetylcysteine (NAS) has many benefits, one of which is as an antioxidant and anti-inflammatory. There have not been many studies of giving NAS to COVID-19 patients. Giving NAS to patients with severe degrees of COVID-19 has varied outcomes, one of which is thought to be caused by the duration of therapy which is only a few hours-days.

Purpose: This retrospective cohort study was conducted in the ICU of PELNI Hospital, Jakarta. This research was started after obtaining an ethical certificate and location permit which began in February-April 2023. The samples were taken using consecutive sampling. Inclusion criteria was patients with severe degree of COVID-19 aged ≥18 years. Exclusion criteria was patients who are pregnant/breastfeeding. Drop out criteria was patients who died before 14 days of NAS administration. The observed outcomes were intubation events, mortality, neutrophil lymphocyte ratio D-dimer and CRP levels. The research data is secondary data from medical records. Data were analyzed with appropriate statistical tests using the SPSS version 27 program.

Results: There were a total of 112 patients with 55 patients not receiving NAS therapy and 57 patients receiving NAS therapy. From the results of bivariate analysis, it was found that patients with NAS therapy had a 2.7 times the likelihood of being intubated and had no association with mortality. From the results of the multivariate analysis, it was found that only the intubation event variable had a significant effect on mortality.

Conclusion: Adjuvant therapy for NAS does not reduce the incidence of intubation and mortality.

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"Latar belakang. Dermatitis atopik (DA) merupakan penyakit kulit kronik residif dengan manifestasi utama berupa gatal dan iritasi kulit yang berkepanjangan. Antihistamin oral telah digunakan secara luas untuk mengurangi gatal pada DA namun efektivitasnya masih kontroversial. Setirizin merupakan antihistamin-1 generasi kedua yang digunakan pada penyakit alergi, termasuk gatal yang berhubungan dengan DA. Tujuan. Untuk menilai efektivitas penggunaan setirizin dibandingkan dengan plasebo dalam terapi DA. Metode. Studi klinis acak terkontrol dilakukan selama Agustus 2014 sampai Mei 2015. Subjek yang memenuhi kriteria inklusi usia 6 bulan sampai 15 tahun dengan DA derajat sedang dibagi menjadi kelompok perlakuan dan kelompok kontrol. Kelompok perlakuan diterapi dengan setirizin (0,25mg/kgBB, dua kali sehari untuk pasien < 2 tahun dan sekali sehari untuk pasien > 2 tahun) sedangkan kelompok kontrol mendapat plasebo. Derajat keparahan DA pada kedua kelompok diukur dengan indeks SCORAD dan kekambuhan DA dievaluasi setiap bulan selama 6 bulan. Hasil penelitian. Total 38 subjek penelitian (18 plasebo, 20 setirizin) ikut serta dalam penelitian dan dianalisis dengan per protocol analysis. Karakteristik dasar meliputi usia, jenis kelamin dan riwayat atopi tidak berbeda di kedua kelompok. Derajat keparahan DA berdasarkan indeks SCORAD pada kedua kelompok adalah derajat sedang (kelompok kontrol 31,5 vs kelompok perlakuan 34,75). Selama pengobatan 6 bulan derajat keparahan DA menurun bertahap dengan tidak ada perbedaan bermakna antara kelompok kontrol dan perlakuan (31,5 menjadi 0 vs 34,75 menjadi 0, p=0,200). Kekambuhan DA pada kelompok setirizin tidak lebih rendah daripada kelompok kontrol dengan tidak terdapat perbedaan bermakna (2 dari 17 subjek vs 2 dari 14 subjek, p=1,000). Simpulan. Pengobatan setirizin selama 6 bulan pada anak dengan DA derajat sedang tidak dapat mengurangi kekambuhan maupun derajat keparahan penyakit.

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Background. Atopic dermatitis (AD) is chronic relapsing skin disease, characterized by intense itching and inflammation. Oral antihistamine has been widely used to reduce pruritus of AD but the effectiveness is still controversial. Cetirizine is a second generation H1 selective antagonist that has been used in allergic diseases, including AD-associated pruritus.

Objective. To assess the efficacy of cetirizine compared with placebo for the treatment of AD.

Method. A randomized clinical controlled trial was performed during August 2014 until May 2015. Eligible patients aged 6 months ? 15 years with moderate AD was divided into treatment group and control group. Treatment group were treated for 6 months with cetirizine (0.25 mg/kg twice daily for patients < 2 years old, once daily for patients > 2 years old), while the control group was given placebo. The severity of AD between both groups was measured by SCORAD index and recurrence was evaluated every month for 6 month-period.

Results. A total of 38 subjects (18 with placebo, 20 with cetirizine) participated in this study and a per protocol analysis was performed. The baseline characteristics, including age, gender and atopic history were similar in both groups. The severity of AD according to SCORAD index were moderate (control group 31,5 vs treatment group 34,75). During 6 month-study period, the severity of AD decreased steadily with no statistical differences between placebo and treatment group (31,5 to 0 vs 34,75 to 0, p=0,200). The recurrence of AD in cetirizine group were not lower than control group with no statistical differences (2 from 17 subject vs 2 from 14 subject, p=1,000).

Conclusion. Cetirizine treatment in children with atopic dermatitis for 6 month-period cannot reduce reccurence and disease severity of moderate AD."

"Latar belakang: Penyakit Kawasaki adalah penyebab utama penyakit jantung didapat pada anak, yang merupakan suatu vaskulitis sistemik akut. Penyakit ini berhubungan dengan luaran aneurisme arteri koroner, yang dapat dicegah dengan pemberian imunoglobulin intravena (IGIV). Terapi baku emas pada penyakit Kawasaki adalah IGIV dosis tinggi (2 g/kgBB). Namun, IGIV dosis medium (1 g/kgBB) merupakan terapi berbiaya lebih rendah dan mungkin memiliki efikasi yang sama. Melalui penelitian ini, kami mengevaluasi keberhasilan terapi IGIV dosis 1 g/kgBB.Metode: Studi kohort retrospektif multisenter dari data rekam medis dengan total 507 pasien dengan penyakit Kawasaki komplit. Penelitian dilakukan di Rumah Sakit Umum Pusat Nasional Cipto Mangunkusumo dan Kawasaki Center, Indonesia dari Januari 2012 hingga Januari 2022. Pasien yang mendapatkan terapi IGIV dengan dosis 1 g/kgBB didefinisikan sebagai grup A, dan pasien yang mendapatkan terapi IGIV dengan dosis 2 g/kgBB didefinisikan sebagai grup B. Karakteristik dasar subjek dibandingkan antar-kelompok tersebut; demografi, hasil laboratorium, keterlibatan mukokutan, hari demam saat diberikan IGIV, durasi demam pasca-IGIV, lama rawat, dan temuan aneurisme arteri koroner dari ekokardiografi pada periode follow-up.Hasil: Sebanyak 24 pasien (grup A), mendapatkan IGIV dosis medium (1 g/kgBB). Sementara itu, sebanyak 483 pasien (grup B) mendapatkan IGIV dosis tinggi (2 g/kgBB). Distribusi usia dan jenis kelamin, nilai leukosit dan trombosit, hari demam saat diberikan IGIV, durasi demam pasca-IGIV, dan lama rawat tidak berbeda bermakna antar-kelompok (p >0,05). Semua pasien mengalami keterlibatan mukokutan. Berat badan menurut umur (WAZ) dan nilai CRP antar-kelompok bermakna secara statistik (p <0,05). Aneurisme arteri koroner tidak ditemukan pada pasien di grup A dan pada 9 pasien (1,9%) di grup B pada periode follow-up (p >0,05).Simpulan: Terapi dengan dosis inisial IGIV 1 g/kgBB untuk pasien dengan penyakit Kawasaki menunjukkan keberhasilan yang sama dengan IGIV dosis tinggi (2 g/kgBB). Hal ini dapat menjadi opsi terapi bagi negara berkembang.

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Background: Kawasaki disease (KD), the leading cause of acquired heart disease in children, is an acute childhood systemic vasculitis. It is associated with coronary artery aneurysms (CAA), that could be prevented by intravenous immunoglobulin (IVIG) administration. High-dose IVIG (2 g/kg) is usually given in the treatment of Kawasaki disease (KD). However, medium-dose IVIG (1 g/kg) is a low-cost treatment and may have the same efficacy. We aim to determine whether the treatment with IVIG at an initial dose of 1 g/kg is effective for preventing CAA.

Methods: A multicenter retrospective cohort study was conducted. A total of 507 patients with complete KD who were treated with high-dose and medium-dose immunoglobulin at Cipto Mangunkusumo Hospital and Kawasaki Center, Indonesia from January 2012 to January 2022 were enrolled. Patients treated with a single infusion of medium-dose IVIG (1 g/kg) were defined as group A, and patients treated with high-dose IVIG (2 g/kg) were defined as group B. Patient characteristics were compared between the two groups; demographic features, laboratory findings, mucocutaneous involvement, day of fever, duration of fever after treatment, length of stay, and rates of CAA from echocardiography during the follow-up period.

Results: Medium-dose IVIG was given in 24 patients (group A). High-dose IVIG was given in 483 patients (group B). Age and gender distributions, white blood cell and platelet counts, day of fever when IVIG was administered, duration of fever after IVIG treatment, and length of stay did not differ significantly between the two groups (p >0.05). All patients had mucocutaneous involvement. Median of WAZ was higher in group A (+0,35 vs -0,26; p <0.05). Median of concentrations of C-reactive protein was higher in group B (59,5 mg/L vs 81 mg/L; p <0.05). Coronary artery aneurysms were not found in group A and in 9 patients (1.9%) in group B during the follow-up period (p >0.05).

Conclusion: Treatment of KD with IVIG at an initial dose of 1 g/kg could show the same effectiveness as the high-dose IVIG (2 g/kg) and might be an option for low- and middle-income country.

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