The number of laboratory-confirmed CRS cases among Indonesian infants is high. Furthermore, hearing impairment is the most common clinical feature of CRS in infants. Our findings indicate the importance of implementation of rubella vaccine in Indonesia. Conducting hospital-based surveillance of CRS in other hospitals in Indonesia may be appropriate. What is Known: •Congenital rubella syndrome (CRS) has serious consequences in infants resulting from rubella virus infection during pregnancy. •The incidence of CRS in most developed countries has greatly decreased since implementation of rubella vaccination. •Rubella vaccine has not yet been implemented in many developing countries. What is New: •The number of laboratory-confirmed CRS cases among Indonesian infants was high. •Implementation of rubella vaccine into immunization programs in Indonesia is important because of the high number of CRS cases. •Our study highlights the need for ongoing prospective surveillance of CRS in Indonesia.

ObjectiveDuchenne/Becker muscular dystrophy (DMD/BMD) is the most common genetic neuromuscular disease in children, resulting from a defect in the DMD gene located on Xp21.2. The new emerging treatment using exon skipping strategy is tailored to specific mutations, thus molecular diagnostics are particularly important. This study aimed to detect the DMD gene deletion in Indonesian DMD/BMD patients and analyze the potential amenability by exon skipping therapy.ResultsThirty-four male patients were enrolled in this study, 23 of them (67.6%) underwent muscle biopsy and showed the absence or partially expressed dystrophin protein in immunohistochemistry staining. All patients had very high serum CK levels (10.529 ± 9.97 IU/L). Multiplex PCR revealed the DMD gene deletions in 15 (44.1%) cases. Seventy-eight percent of deletions were clustered in the hot-spot region of exon 43 to 52. Furthermore, seven (20.5%) patients were potentially amenable to exon skipping treatment. Therefore, multiplex PCR is one feasible method to detect DMD gene deletion in Indonesian DMD/BMD patients that can further determine the potential amenability of exon skipping therapy. In addition, this study is the first report of DMD gene deletion analysis in Indonesia.

The incidence of CRS in infants in Yogyakarta Indonesia is considered high, with most clinical manifestations being CHD, hearing impairment and congenital cataract. This emphasizes the necessity for epidemiological study of CRS in other hospitals and the importance of establishing a national rubella vaccination program in Indonesia.

Background Brain abscess is a severe infection of brain parenchyma, which occurs in 25-46% of cases of uncorrected cyanotic congenital heart disease. Low arterial oxygen saturation is the main risk factor for brain abscess in children with cyanotic congenital heart disease, however, the arterial oxygen saturation test is invasive and not routinely done in our setting. Objective To evaluate low peripheral oxygen saturation as a risk factor for brain abscess in children with cyanotic congenital heart disease. Methods We conducted a matched, case-control study at Sardjito Hospital, Yogyakarta for children aged less than 18 years with cyanotic congenital heart disease, from 2010-2016. Case subjects were children with brain abscess complications. The control group had only cyanotic congenital heart disease, and were matched for age and sex to the case group. During hospitalization due to the brain abscess complication in the case group, data regarding peripheral oxygen saturation, polycythemia, pneumonia, sepsis, dental caries and restricted pulmonary blood flow were collected and compared between both groups. Results During the study period, 18 children with cyanotic congenital heart disease had brain abscesses. This group was compared to the control group of 36 children. Bivariate analysis revealed that the lowest level of peripheral oxygen saturation (OR 0.92; 95%CI 0.85 to 0.98; P=0.02) and dental caries (OR 3.3; 95%CI 1.01 to 11.18; P=0.04) were significant risk factors for brain abscess. However, in the multivariate analysis, the only statistically significant risk factor associated with brain abscess was the lowest level of peripheral oxygen saturation (OR 0.92; 95%CI 0.86 to 0.99; P=0.04). Conclusion Low peripheral oxygen saturation is a significant risk factor for brain abscess development in children with cyanotic congenital heart disease.  A decrease of 1% peripheral oxygen saturation may increase the risk of brain abscess by 8%.

Latar belakang. Meningitis bakterialis merupakan salah satu penyebab morbiditas dan mortalitas yang penting pada anak. Anak dengan meningitis biasa datang ke rumah sakit dan mendapat antibiotik intrakranial tanpa menunggu hasil kultur. Hal ini dilakukan karena membedakan meningitis bakterialis dan meningitis bukan bakteri pada awal perjalanan penyakit terkadang sulit. Beberapa indikator dapat digunakan untuk membedakan hal itu. Salah satunya adalah bacterial meningeal score (BMS), terdiri dari pengecatan gram cairan serebrospinal positif, protein cairan serebrospinal ≥80 mg/dL, neutrofil darah tepi ≥10.000 sel/mm3, riwayat kejang, neutrofil absolut cairan serebrospinal ≥1000 sel/ mm3.Tujuan. Mengetahui apakah bacterial meningeal score merupakan indikator yang baik untuk menegakkan diagnosis meningitis bakterialis akut pada bayi dan anakMetode. Uji diagnostik pada anak usia >1 bulan-18 tahun, yang dicurigai sebagai meningitis berdasarkan kriteria WHO, mulai Februari 2011 sampai dengan April 2011. Diagnosis meningitis bakterialis ditegakkan apabila hasil kultur ditemukan bakteri.Hasil. Di antara 31 anak subjek penelitian, 16 laki-laki. Semua datang dengan demam, kejang (29/31), penurunan kesadaran (15/31), dan tanda meningeal (17/31). Pengecatan gram positif pada 9/31 sampel dan kultur positif 12/31 sampel. Hasil analisis statistik didapatkan sensitivitas BMS 83,3%, spesifisitas 89,5%, nilai praduga negatif 83,3%, nilai praduga positif 89,5%, likelihood ratio positif 7,92, dan likelihood ratio negatif 0,186. Bakteri yang tumbuh dari hasil kultur adalah P. aerogenosa, S.epidermidis and Paracoccus sp, Bacillus. Sp dan Enterococcus sp.Kesimpulan. Bacterial meningeal score merupakan indikator yang baik untuk menilai meningitis bakteri pada bayi dan anak karena memiliki sensitivitas, spesifisitas, nilai praduga negatif, nilai praduga positif, likelihood ratio positif dan likelihood ratio negatif yang tinggi.

Introduction: Epilepsy is a worldwide and common child health problem. One of its complications is cognitive impairment that will impact on children’s cognitive development and quality of life. The objective of this study is to find out whether epilepsy affects cognitive function in children. Material and Methods: This was a cross-sectional descriptive study using secondary data from medical records. We collected data from children with epilepsy from 2004 until 2014 that have been actively managed in the Growth and Development Clinic, Sardjito General Hospital, Yogyakarta, Indonesia. Cognitive function referred to low IQ score that was gained from patients’ data. We analyzed the data using Fischer’s exact and two-sample Kolmogorov-Smirnov test methods. Results: Among 40 data samples, there were 19 patients (42.5%) with cognitive impairment (IQ < 70) and 21 patients (57.5%) without cognitive impairment (IQ ≥ 70). There were twenty-five patients examined by the Stanford-Binet IQ test, which found 15 patients with cognitive impairment. In fifteen patients who were examined with the WISC test, we found more children with epilepsy without cognitive impairment (11 patients). Comorbidity with neuro-developmental disorder significantly affected cognitive impairment in children with epilepsy (p value = 0.034, 95% CI 1.08-21.76). Conclusions: More children with epilepsy without cognitive impairment were found. Further studies are needed with larger number of samples utilizing a prospective study design to better determine the association of epilepsy in children and cognitive impairment.

E pilepsi merupakan masalah pediatrik yang besar dan lebih sering terjadi pada usia dini dibandingkan usia dewasa. Di seluruh dunia saat ini, terdapat sekitar 40-50 juta pasien epilepsi, 85% berasal dari negara berkembang. 1Prognosis epilepsi dapat diklasifikasikan berdasar kelompok prognosis, yaitu sangat baik, baik, bergantung obat antiepilepsi (OAE), dan buruk. Kelompok prognosis sangat baik ditemukan pada 20%-30% dari semua orang yang mengalami bangkitan kejang tanpa provokasi dan kemungkinan besar remisi spontan. Kelompok prognosis baik ditemukan pada 30%-40% kasus, kejang biasanya terkontrol dengan baik dengan OAE dan ketika remisi tercapai sifatnya permanen dan OAE dapat dengan baik diturunkan atau dihentikan.

Latar belakang. Infeksi Cytomegalovirus (CMV) merupakan penyebab tersering infeksi kongenital anak di negara berkembang. Infeksi ini dapat menyebabkan tuli sensorineural (SNHL) dan gangguan perkembangan. Di RSUP dr Sardjito, pasien dengan infeksi CMV aktif bergejala akan menjalani terapi 6 minggu Ganciclovir atau 2 minggu terapi Ganciclovir dilanjutkan 4 minggu terapi Valganciclovir. Namun, luaran terapi tersebut belum diteliti lebih lanjut.Tujuan. Melihat luaran terapi ganciclovir dan atau valganciclovir pada pasien infeksi Cytomegalovirus di Instalasi Kesehatan Anak RSUP Dr Sardjito.Metode. Penelitian ini menggunakan metode kohort retrospektif dari data rekam medis pasien dengan diagnosis infeksi CMV aktif di Instalasi Kesehatan Anak RSUP dr Sardjito periode Januari 2014 sampai dengan April 2018. Variabel luaran (BERA dan Denver II) dibandingkan antara pre dan post terapi ganciclovir. Analisis statistik data dasar menggunakan analsisi deskriptif. Untuk variable luaran menggunakan T test.Hasil. Didapatkan hasil yang signifikan untuk perbaikan fungsi pendengaran pada telinga kanan (p<0,001) dan kiri (p<0,03) dibandingkan dengan yang mengalami perburukan. Sementara untuk perbandingan gangguan perkembangan sebelum dan sesudah terapi ganciclovir tidak berbeda bermakna (p>0,05).Kesimpulan. Pemberian terapi ganciclovir dan valganciclovir dapat memperbaiki fungsi pendengaran (tes BERA), tetapi perbaikan tidak didapatkan pada aspek neurodevelopmental (tes Denver II) dari pasien dengan infeksi CMV.