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"Latar belakang: Diketahui sekitar 10-30% anak sindrom nefrotik resisten steroid (SNRS) mengalami varian patogenik (SNRS monogenik) dan kejadian ini lebih tinggi pada SNRS primer dibandingkan SNRS sekunder. Adanya varian patogenik yang terkonfirmasi dapat membantu memprediksi gejala klinis, berpengaruh terhadap terapi yang diberikan, memberikan informasi untuk konseling genetik, serta berpotensi untuk diagnosis antenatal atau pra-gejala. Di Indonesia, penelitian terkait pola mutasi genetik pada anak dengan SNRS primer masih sangat terbatas.Tujuan: Mengetahui pola mutasi genetik pada anak dengan SNRS primer di RSCM.Metode: Penelitian ini menggunakan metode studi prevalens dan potong lintang untuk mendeteksi pola varian genetik subjek dengan SNRS primer dan mengetahui hubungannya dengan profil klinis subjek. Pemeriksaan genetik yang dilakukan adalah whole exome sequencing (WES).Hasil: Dari 60 subjek, diperoleh 16 subjek yang merupakan SNRS dengan varian (26,7%) dan semuanya berusia <12 tahun, terbanyak di bawah 3 tahun (9 dari 16 subjek). Probable disease-causing variant terkait sindrom nefrotik yang ditemukan dalam penelitian ini adalah pada gen LAMA5, COL4A4, COL4A3, TBC1D8B, dan TRPC6 dengan masing-masing 2 subjek, serta pada gen ANLN, FN1, NUP93, AVIL, INF2, CUBN, dan COQ8B/ADCK4 dengan masing-masing 1 subjek. Tidak didapatkan hubungan secara signifikan antara temuan varian dengan faktor demografi (usia, jenis kelamin, riwayat keluarga, dan konsanguinitas), manifestasi klinis (respons terhadap siklosporin dan laju filtrasi glomerulus), dan hasil biopsi ginjal.Kesimpulan: SNRS dengan varian ditemukan sebanyak 26,7% dari seluruh subjek dengan SNRS primer. Pola varian bersifat acak dan terbanyak ditemukan pada gen terkait sindrom Alport yaitu pada 4 dari 16 subjek. Pasien SNRS primer dengan usia <3 tahun terindikasi untuk dilakukan pemeriksaan genetik.

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Background: Approximately 10-30% of children with steroid-resistant nephrotic syndrome (SRNS) have a pathogenic variant (monogenic SRNS) and this rate is higher in primary SRNS compared to secondary SRNS. The presence of confirmed pathogenic variants can help to predict clinical symptoms, affect the treatment, provide information for genetic counseling, and have the potential for antenatal or pre-symptomatic diagnosis. In Indonesia, research related to genetic mutation patterns in children with primary SRNS is still very limited.

Objective: To determine the genetic mutation patterns in pediatric subjects with primary SRNS.

Methods: This study used prevalence and cross-sectional study methods to detect the variant in primary SRNS subjects and determine its relationship with the clinical profile of the subjects. The genetic test performed was whole exome sequencing (WES).

Results: Out of 60 subjects, we found 16 subjects (26,7%) were SRNS with variants and all below 12 years-old, most were below 3 years-old (9 out of 16 subjects). Detected probable disease-causing variants related to nephrotic syndrome in this study were LAMA5, COL4A4, COL4A3, TBC1D8B, and TRPC6 genes each in 2 patients, and ANLN, FN1, NUP93, AVIL, INF2, CUBN, and COQ8B/ADCK4 genes each in 1 patient. No significant relationship was determined between variant finding and demographic factors (age, sex, family history, and consanguinity), clinical manifestations (response to cyclosporine and glomerular filtration rate), or kidney biopsy results.

Conclusion: We found 26,7% SRNS with variants in primary SRNS subjects. Variant patterns are scattered with most genes found were related to Alport syndrome in 4 out of 16 subjects. Primary SRNS patients below 3 years-old are indicated for genetic testing."

"[ABSTRAKLesi tubular lebih sering ditemukan pada sindrom nefrotik resisten steroid (SNRS)dengan proteinuria masif, yang menyebabkan disfungsi tubulus proksimal. Cederatubular dapat pula didiagnosis dengan uji fungsi tubulus, diantaranya adalah fraksiekskresi magnesium (FE Mg) dan β2-mikroglobulin (β2M) urin. Tujuanpenelitian ini membandingkan FE Mg dan β2M urin pada SNRS dan SN sensitifsteroid (SNSS) remisi. Penelitian potong lintang dilakukan di Departemen IlmuKesehatan Anak RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUD UlinBanjarmasin, RSUP Fatmawati dan RSAB Harapan Kita Jakarta pada Juli sampaiDesember 2015 pada penderita SNRS dan SNSS remisi berusia 2 ? 15 tahun. Padasubyek diperiksakan kadar β2M urin dan FE Mg. Didapatkan 62 subyek yangterdiri dari 31 subyek SNRS dan 31 subyek SNSS remisi. Rerata FE Mg padaSNRS lebih tinggi secara bermakna dibandingkan SNSS remisi (p=0,0065).Median kadar β2M urin pada SNRS lebih tinggi dibandingkan SNSS remisi (p <0,001). Peningkatan kadar β2M urin lebih banyak secara bermakna pada SNRSdibandingkan SNSS (p=0,007). Dengan titik potong 1,64%, peningkatan FE Mgpada SNRS lebih banyak dibandingkan SNSS remisi (p=0,022). Simpulan: Fraksiekskresi Mg dan β2M urin pada SNRS lebih tinggi dibandingkan SNSS remisi.Terdapat perbedaan proporsi peningkatan FE Mg antara SNRS dan SNSS remisi.Proporsi peningkatan β2M urin pada SNRS lebih besar dibandingkan SNSSremisi.

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ABSTRACTTubular lesions more often found in steroid-resistant nephrotic syndrome (SRNS)with massive proteinuria, leading to proximal tubular dysfunction. Tubular injurycan also be diagnosed by tubular function test, such as fractional excretion ofmagnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this studyis to compare the FE Mg and urinary β2M on SRNS and steroid-sensitivenephrotic syndrome (SSNS) in remission. A cross-sectional study was conductedin the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUDUlin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July toDecember 2015. Children aged 2-15 years who either had SRNS or SSNS inremission were recruited. Fractional excretion of magnesium and urinary β2Mlevels were examined. There were 62 subjects consisted of 31 subjects SRNS and31 subjects SSNS in remission. The mean FE Mg on SRNS was significantlyhigher than SSNS in remission (p=0.0065). Median levels of urinary β2M onSRNS was higher than SNSS remission (p<0.001). Increased levels of urinaryβ2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoffpoint of 1.64%, an increased of FE Mg proportion on SRNS was more thanSSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg andurinary β2M on SRNS were higher than SSNS in remission. There is a differencebetween the increased of FE Mg on SRNS and SSNS in remission. The increasedof urinary β2M on SRNS was higher than SSNS in remission.;Tubular lesions more often found in steroid-resistant nephrotic syndrome (SRNS)with massive proteinuria, leading to proximal tubular dysfunction. Tubular injurycan also be diagnosed by tubular function test, such as fractional excretion ofmagnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this studyis to compare the FE Mg and urinary β2M on SRNS and steroid-sensitivenephrotic syndrome (SSNS) in remission. A cross-sectional study was conductedin the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUDUlin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July toDecember 2015. Children aged 2-15 years who either had SRNS or SSNS inremission were recruited. Fractional excretion of magnesium and urinary β2Mlevels were examined. There were 62 subjects consisted of 31 subjects SRNS and31 subjects SSNS in remission. The mean FE Mg on SRNS was significantlyhigher than SSNS in remission (p=0.0065). Median levels of urinary β2M onSRNS was higher than SNSS remission (p<0.001). Increased levels of urinaryβ2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoffpoint of 1.64%, an increased of FE Mg proportion on SRNS was more thanSSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg andurinary β2M on SRNS were higher than SSNS in remission. There is a differencebetween the increased of FE Mg on SRNS and SSNS in remission. The increasedof urinary β2M on SRNS was higher than SSNS in remission., Tubular lesions more often found in steroid-resistant nephrotic syndrome (SRNS)with massive proteinuria, leading to proximal tubular dysfunction. Tubular injurycan also be diagnosed by tubular function test, such as fractional excretion ofmagnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this studyis to compare the FE Mg and urinary β2M on SRNS and steroid-sensitivenephrotic syndrome (SSNS) in remission. A cross-sectional study was conductedin the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUDUlin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July toDecember 2015. Children aged 2-15 years who either had SRNS or SSNS inremission were recruited. Fractional excretion of magnesium and urinary β2Mlevels were examined. There were 62 subjects consisted of 31 subjects SRNS and31 subjects SSNS in remission. The mean FE Mg on SRNS was significantlyhigher than SSNS in remission (p=0.0065). Median levels of urinary β2M onSRNS was higher than SNSS remission (p<0.001). Increased levels of urinaryβ2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoffpoint of 1.64%, an increased of FE Mg proportion on SRNS was more thanSSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg andurinary β2M on SRNS were higher than SSNS in remission. There is a differencebetween the increased of FE Mg on SRNS and SSNS in remission. The increasedof urinary β2M on SRNS was higher than SSNS in remission.]"

"Peningkatan atensi terhadap penggunaan Screen Time orang tua maupun anak sudah menjadi bagian integral dalam kehidupan. Sayangnya, anak usia sekolah saat ini lebih sering beraktivitas dengan hanya menatap layar selama waktu yang lama. Hal itu, membuat anak terpapar layar dengan durasi yang melebihi rekomendasi sehingga menimbulkan efek negatif terhadap tumbuh kembang anak. Penelitian ini bertujuan untuk melihat gambaran Screen Time dan mengidentifikasi hubungan lama Screen Time dengan perkembangan sosial. Penelitian menggunakan pendekatan cross-sectional pada 285 responden orang tua yang sesuai dengan kriteria inklusi melalui metode stratified sampling. Instrumen SCREENS-Q untuk mengukur Screen Time dan Strength and difficulties Questionnaire (SDQ) mengukur perkembangan sosial. Hasil penelitian menunjukkan 74,4% anak mengalami Screen Time berlebihan dan terdapat hubungan antara lama Screen Time dengan setiap sub-skala perkembangan sosial (p value <0,05). Peneliti merekomendasikan adanya sosialisasi dan kerjasama pihak tenaga kesehatan dengan orang tua untuk mencari solusi bersama mengatasi permasalahan ini.

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Increasing attention to the use of Screen Time for parents and children has become an integral part of life. Unfortunately, today's school-age children are more active by just staring at the screen for a long time. This causes children to be exposed to screens for a duration that exceeds the recommendations, which has a negative effect on children's development. This study aims to look at the description of Screen Time and identify the relationship between long Screen Time and social development. The study used a cross-sectional approach to 285 parents who fit the inclusion criteria through a stratified sampling method. The SCREENS-Q instrument to measure Screen Time and the Strength and Difficulty Questionnaire (SDQ) to measure social development. The results showed that 74.4% of children experienced excessive Screen Time and there was a relationship between the length of Screen Time and each social development sub-scale (p value <0.05). Researchers recommend socialization and collaboration between health workers and parents to find solutions together to overcome this problem."

"Latar belakang: Sindrom nefrotik merupakan manifestasi glomerulopati yang tersering ditemukan pada anak. SNRS sering mengalami penurunan fungsi ginjal dan dalam perjalanan penyakitnya dapat mengalami gagal ginjal tahap terminal. Data mengenai kesintasan dan faktor-faktor yang memengaruhi penurunan fungsi ginjal pada SNRS anak di Indonesia masih terbatas.Tujuan: Penelitian ini bertujuan untuk mengetahui kesintasan fungsi ginjal dalam lima tahun pertama pengobatan serta faktor-faktor yang memengaruhiMetode: Penelitian ini merupakan studi prognostik dengan rancangan penelitian kohort retrospektif di Rumah Sakit Cipto Mangunkusumo menggunakan data rekam medis pasien yang terdiagnosis dengan SNRS pada bulan Januari 2012 hingga Desember 2022. Subjek yang diteliti adalah anak berusia 1 - 18 tahun saat terdiagnosis dengan SNRS. Faktor yang diteliti untuk kesintasan dan faktor penurunan fungsi ginjal adalah usia awitan, hematuria saat awitan, hipertensi saat awitan, respon terhadap terapi imunosupresi, jenis histopatologi, dan fungsi ginjal saat awitan.Hasil: Sebanyak 212 anak terdiagnosis sindrom nefrotik resisten steroid dengan median usia 7 tahun (IQR 3-12 tahun), dan 65,1% berjenis kelamin laki-laki. Jenis histopatologi yang ditemukan terbanyak yaitu GSFS sebesar 57%. Sebanyak 51,9% mengalami hipertensi saat awitan nefrotik, dan pada 32,7% pasien ditemukan hematuria saat awitan nefrotik. Proporsi fungsi ginjal saat awitan yaitu masing-masing 68.9%, 12.7%, 5.7%, 4.7%, 4.2%, dan 3.8% pada kategori fungsi ginjal G1, G2, G3a, G3b, G4, dan G5. Secara umum pasien mengalami tren penurunan fungsi ginjal selama periode pemantauan, dengan kesintasan ginjal sebanyak 53,3% pada tahun pertama pemantauan, 47,2% di tahun kedua, 43,9% di tahun ketiga, 41,5% di tahun keempat, dan 40,6% di tahun kelima. Uji regresi Cox menemukan bahwa usia awitan di atas 6 tahun (HR 1,638; IK95% 1,132 – 2,370; p=0,009), hematuria saat awitan (HR 1,650; IK95% 1,135 – 2,400; p<0,009), dan respon buruk terhadap terapi imunosupresi (HR 1,463; IK95% 1,009 – 2,120; p=0,045) merupakan prediktor penurunan fungsi ginjal.Kesimpulan: Usia awitan di atas 6 tahun, hematuria awitan, dan respon buruk terhadap terapi imunosupresi merupakan prediktor penurunan fungsi ginjal pada anak dengan SNRS.

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Background: Nephrotic syndrome is the most common manifestation of glomerulopathy in children. SNRS often has decreased kidney function and during the course of the disease may develop end stage renal disease. However, data on survival kidney function and prognostic factors are still lacking.

Objective: This study aimed to evaluate the first five year survival rate and prognostic factors of outcome.

Method: We conducted a retrospective cohort study in Cipto Mangunkusumo Hospital which included patients aged 1 to 18 years at diagnosis from Januari 2012 to December 2022. Subjects were followed for 1 to 5 years up to December 2023. Factors analyzed for renal function decline were age at onset, hematuria and hypertension at onset, response to immunosuppression therapy, type of histopathology and renal function at onset. Results: A total of 212 patients with SNRS were included with median age of 7 (IQR 3- 12 years) and 65.1% were male patients. The majority of histopathology type was GSFS (57%). 51,9% had hypertension at SNRS onset, and 32,7% hematuria was found at the onset of SNRS. The proportion of kidney function at onset was 68.9%, 12.7%, 5.7%, 4.7%, 4.2%, and 3.8% in the G1, G2, G3a, G3b, G4, and G5 kidney function categories, respectively. In general, patients experienced a trend of decreasing kidney function during the monitoring period, with renal survival 53,3% in the first year monitoring, 47,2% in the second year, 43,9% in the third year, 41,5% in the fourth year, and 40,6% in the fifth year. Cox regression analysis found that age of onset over 6 years (HR 1.638; 95%CI 1.132 – 2.370; p=0.009), hematuria at onset (HR 1,650; IK95% 1,135 – 2,400; p<0,009), and bad response to immunosuppressive therapy (HR 1,463; IK95% 1,009 – 2,120; p=0,045) were predictors of decreased kidney function.

Conclusion: Age of 6 years or older at onset, onset hematuria, and bad response to immunosuppressive therapy were independent predictors of worsening kidney function in children with SRNS."

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Latar Belakang: Talasemia I² mayor merupakan penyakit dengan gen carrier yang cukup banyak ditemukan di Indonesia sehingga dibutuhkan penelitian lebih lanjut tentang pola talasemia β mayor terlebih lagi penderitanya mengalami inefektif hematopoesis sehingga pasien talasemia I² mayor sangat bergantung dengan terapi transfusi dan kelasi untuk bertahan hidup sehingga penelitian ini bertujuan untuk mengetahui efek yang ditimbulkan dari kepatuhan terapi kelasi pada populasi Indonesia terhadap kadar alanin aminotransferase, aspartat aminotransferase, dan AST to patelet ratio index (APRI) score.

Metode: Penelitian ini menggunakan metode observatif cross sectional dan seluruh partisipan penelitian adalah pasien RSCM Kiara. Data kepatuhan pasien didapat dari kuisioner morisky medication adherence scale -8 serta pertanyaan singkat alasan ketidakpatuhan dalam terapi yang akan dicocokan dengan data laboratorium pasien pada rekam medik elektronik dan selanjutnya data dianalisis menggunakan uji bivariat nonparametrik Kruskal-Wallis dan uji Post-Hoc Mann-Whitney.

Hasil: Tidak ditemukan adanya hubungan yang bermakna antara kepatuhan terapi kelasi terhadap kadar alanin amintotransferase, aspartat aminotransferase, dan APRI score namun, ditemukan hubungan yang bermakna pada umur, lama transfusi, dan jenis kelator terhadap nilai APRI score.

Kesimpulan: Tidak ditemukan adanya hubungan bermakna pada kepatuhan terapi kelasi terhadap kadar alanin aminotransferase, aspartat aminotransferase, dan APRI score namun dibutuhkan penelitian lebih lanjut untuk mengkonfirmasi hasil tersebut dikarenakan terdapat keterbatasan dalam penelitian.

 

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Background: Thalassemia I² major is a disease with carrier gene common enough to be found in Indonesia therefore further research was needed to know the exact pattern and characteristics of thalassemia I² major because the patients has ineffective hematopoiesis depend their life with transfusion and chelation therapy to survive therefore it need further research to know the effect of chelation therapy for population in Indonesia with alanin aminotransferase, aspartat aminotransferase, and AST to platelet ratio index (APRI) score level.

Methods: This study used observative cross sectional method and all of the participants are patients at RSCM Kiara. Participants compliance were measured by morisky medication adherence scale-8 with some adjustment to know the reason why participants isnt complying with therapy and will be compared with laboratory result through electronic medical record then both results were then analyzed non-parametrically using Kruskal-Wallis followed by Mann-Whitney for Post-Hoc.

Results: There arent any correlation between chelation therapy compliance with aspartat aminotransferase, alanine aminotransferase, and AST to platelet ratio index score level but it has been found that age, transfusion duration, and type of chelator have some degree of correlation.

Conclusion: There arent any correlation between chelation therapy compliance with aspartat aminotransferase, alanine aminotransferase, and AST to platelet ratio index score level but the result need further research to confirm the result because this research has its own degree of limitation

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"Patogenesis sindrom nefrotik resisten steroid (SNRS) dan sindrom nefrotik sensitif steroid (SNSS) belum diketahui secara menyeluruh. Antioksidan seperti enzim glutation peroksidase (GPx) dan kofaktornya yaitu selenium diperkirakan berpengaruh dalam menghambat progresivitas penyakit sindrom nefrotik (SN). Namun sampai saat ini belum ada studi yang menilai peran selenium dalam patogenesis terjadinya SNRS dan SNSS. Penelitian ini bertujuan untuk membandingkan kadar selenium pada pasien SNSS dan SNRS menggunakan studi potong lintang. Penelitian dilakukan pada 81 pasien SNRS dan SNSS berusia 2-18 tahun yang datang ke poliklinik rawat jalan nefrologianak RSUPNCM pada bulan November-Desember 2019 dengan metode consecutive sampling. Hasil penelitan menunjukkan tidak ada perbedaan signifikan antara kadar selenium pada kedua kelompok. Peran selenium sebagai antioksidan terhadap patogenesis SNRS dan SNSS sulit dibuktikan karena patogenesis penyakit ini bersifat multifaktorial. Penelitian lanjutan dengan desain penelitian kasus kontrol dan pengukuran selenium serial diperlukan untuk memastikan hal ini.

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The pathogenesis of steroid resistant nephrotic syndrome (SRNS) and steroid sensitive nephrotic syndrome (SSNS) has not yet been fully known. Antioxidants such as glutathione peroxidase enzyme (GPx) and its cofactor, selenium, are thought to have an effect of slowing down the progress of nephrotic syndrome (NS). However, until now, there are no studies that evaluate the role of selenium in SNRS and SNSS’s pathogenesis. The purpose of this research is to compare the selenium levels of SNRS and SNSS patients using a cross-sectional study. This research was conducted on 81 SNRS and SNSS patients ages 2 to 18, who visited RSUPNCM’s pediatric nephrology outpatient clinic in November 2019 to December 2019, using consecutive sampling method. The result shows that there’s no significant difference in the selenium levels of both groups. Selenium’s role as an antioxidant for the pathogenesis of SNRS and SNSS is hard to prove because it is multifactorial. Advance research using a case-control study and a serial of selenium examination is needed to confirm this."

"ABSTRAKLatar Belakang. Sindrom nefrotik resisten steroid (SNRS) jarang ditemukan pada anak. Kesintasan kehidupan anak SNRS pada umumnya baik. Akan tetapi, anak SNRS sering mengalami penurunan fungsi ginjal dan pada perjalanan penyakitnya dapat mengalami end stage renal disease (ESRD). Tujuan. Mengetahui kesintasan kehidupan dan fungsi ginjal anak SNRS pada tahun ke-1, 2, 3, 4, dan 5. Mengetahui pengaruh usia, fungsi ginjal, dan hipertensi saat awitan serta tipe resistensi terhadap kesintasan kehidupan dan fungsi ginjal anak SNRS.Metode. Penelitian kohort retrospektif dengan menggunakan data sekunder berupa rekam medis anak SNRS yang datang berobat ke Poliklinik Nefrologi Departemen Ilmu Kesehatan Anak dan praktik swasta konsultan Divisi Nefrologi dalam periode Januari 2000-Januari 2011. Kesintasan fungsi ginjal yang dinilai pada penelitian ini adalah kenaikan kreatinin ≥2 kali dan ESRD.Hasil. Sebanyak 45 anak SNRS diikutsertakan dalam penelitian. Lama sakit adalah 24 (rentang 3-95) bulan. Sebanyak 20% anak meninggal dunia, 31,1% anak mengalami kenaikan kreatinin ≥2 kali, dan 13,4% anak menjadi ESRD pada akhir penelitian. Kesintasan kehidupan anak SNRS pada tahun ke-1, 2, 3, 4, dan 5 berturut-turut adalah 93, 84, 80, 72, dan 61%. Kesintasan anak SNRS terhadap terjadinya kenaikan kreatinin ≥2 kali pada tahun ke-1, 2, 3, 4, dan 5 berturut-turut adalah 92, 72, 56, 42, dan 34%. Kesintasan anak SNRS terhadap terjadinya ESRD pada tahun ke-1, 2, 3, 4, dan 5 berturut-turut adalah 97, 88, 81, 70, dan 58%. Usia, fungsi ginjal, hipertensi saat awitan dan tipe resistensi tidak berpengaruh terhadap kesintasan kehidupan, kenaikan kreatinin ≥2 kali, maupun terjadinya ESRD (semua nilai p>0,05).Simpulan. Penelitian ini mendapatkan hasil bahwa anak SNRS rentan untuk mengalami kenaikan kreatinin ≥2 kali dan ESRD. Faktor-faktor prognostik yang dipikirkan mempengaruhi kesintasan kehidupan dan fungsi ginjal seperti usia, fungsi ginjal dan hipertensi saat awitan serta tipe resistensi tidak terbukti berperan dalam kesintasan.

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ABSTRACTBackground: Steroid resistant nephrotic syndrome (SRNS) is seldom found in children. Children with SRNS generally have good survival although during the course of the disease may develop decreased kidney function, leading to end stage renal disease (ESRD). Data on survival of children with SRNS is still scarce. Objective: To determine survival in children with SRNS on the first, second, third, fourth and fifth year; to study the effect of age at onset, initial kidney function, hypertension and type of resistance towards the survival of children with SRNS.Method: A retrospective cohort is performed using secondary data obtained from medical record of outpatient and inpatient clinic from Division of Nephrology, Department of Child Health, Cipto Mangunkusumo Hospital as well as private clinic of the Pediatric Nephrology consultant from January 2000-January 2011. Kidney survival was determined as doubling of base creatinine levels and ESRD.Results: This study includes 45 children with SRNS. Median time of illness was 24 (range 3-95) months. Twenty percent died due to various reasons; 31.1% had a doubling of base creatinine levels and 13.4% develop ESRD. Survival on the first, second, third, fourth and fifth year are 93, 84, 80, 72 and 61% respectively. Kidney survival on the first, second, third, fourth and fifth year towards doubling of base creatinine levels are 92, 72, 56, 42 and 34%, whereas towards ESRD are 97, 88, 81, 70 and 58% respectively. Age at onset, initial kidney function, hypertension and type of resistance does not affect the survival of children with SRNS (all P>0.05).Conclusion: Children with SRNS is prone to develop a doubling of base creatinine levels and ESRD. Factors such as age at onset, initial kidney function, hypertension and type of resistance does not affect the survival of children with SRNS."

"Laporan ini memuat efek samping klinis dan biokimia pemberian zoledronic acid dosis awal pada tiga anak bersaudara dengan osteogenesis imperfecta berat. Zoledronic acid diberikan dalam 50 ml larutan garam fisiologis selama 30 menit. Semua pasien mengalami demam dalam kurun waktu 6-48 jam setelah pemberian infus pertama. Tidak ada efek samping terhadap fungsi ginjal, kecuali hipokalsemia dan hipofosfatemia, yang terjadi dalam 48 jam dan 72 jam setelah infus zoledronic acid. Efek samping minimal dapat diatasi dengan mudah.

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This report documented the clinical and biochemical side effects on the first dose of intravenous zoledronic acid therapy in three siblings with severe osteogenesis imperfecta. Zoledronic acid was administered in 50 ml 0.9% saline solution over a period of 30 minutes. All patients had fever during the first 6 to 48 hours after the first infusion. There were no renal side effects, apart from asymptomatic hypocalcemia and hypophosphatemia at 48 and 72 hours after zoledronic acid infusion. The minimal clinical side effects were easily manageable."

"Latar belakang: Prevalens late steroid resistance (LSR) makin meningkat pada anak dengan sindrom nefrotik idiopatik (SNI). Fungsi ginjal yang menurun dapat memperburuk prognosis LSR. Penelitian terkait mengenai faktor risiko LSR pada anak (SNI) masih terbatas, padahal pengenalan terhadap faktor risiko ini diperlukan untuk deteksi dini dan mengotimalkan terapi. Tujuan: Mengidentifikasi karakteristik anak yang didiagnosis SNI awitan inisial seperti jenis kelamin, usia awitan SNI, hipertensi, kadar hemoglobin, albumin, ureum, laju filtrasi glomerulus, hematuria mikroskopik dan jangka waktu sejak dinyatakan remisi dan telah menyelesaikan pengobatan inisial terhadap terjadi relaps pertama kali dapat menjadi faktor risiko LSR pada anak dengan SNI. Metode penelitian: Penelitian kasus-kontrol dengan penelusuran retrospektif yang dilakukan di Departemen Ilmu Kesehatan Anak di FKUI-RSCM, RSUP. Fatmawati dan RSUP. Dr. Mohammad Hoesin periode Maret-Mei 2018 yang terbagi menjadi kelompok LSR dan SNSS. Pengambilan rekam medis anak dengan diagnosis SNI yang melakukan kunjungan pengobatan di poli nefrologi dalam kurun waktu lima tahun terakhir. Faktor risiko dianalisis secara bivariat dan multivariat.Hasil penelitian: Dilakukan analisis pada 100 anak dengan LSR dan 100 anak dengan SNSS. Anak laki-laki didapatkan lebih banyak daripada anak perempuan pada dua kelompok dengan median usia 4,12 (1,0-17,40) tahun. Faktor yang secara bermakna berpengaruh terhadap kejadian LSR pada anak dengan SNI pada analisis bivariat adalah: kadar ureum ≥ 40mg/dL (OR 1,68; IK 95% 1,45-4,53) dan adanya hematuria mikroskopik (OR 2,45; IK 95% 1,35-4,47). Simpulan: Faktor risiko yang berperan terhadap kejadian LSR pada anak dengan SNI adalah kadar ureum ≥ 40 mg/dL dan terdapat hematuria mikroskopik.

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Background: Prevalence of late steroid resistance (LSR) tends to be increased in children with idiopathic nephrotic syndrome (INS). Renal function deterioration may worsen the prognosis. Previous studies about the risk factors for LSR in children with INS were still limited, while early detection is the most important thing to do proper treatment. Objectives: to determine whether age of onset, sex, hypertension, hemoglobin level, albumin, ureum, filtration glomerular rate, microscopic hematuria, and first relaps may influence the occurrence of LSR in children with INS. Methods. Case control study with restrospective medical record investigation was performed in INS children who visited to dr. Cipto Mangunkusumo, dr. Fatmawati and dr. Mohammad Hoesin General Hospital, during March-May 2018. Case and control group was children with LSR and sensitive steroid. Bivariate and multivariate analysis to identify significant risk factors.Results: There were each 100 children with LSR and steroid sensitive. No different of sex ratio in each group with median of age 4,12 (1,0-17,40) years old. Factors which associated significantly with LSR on bivariate analysis were ureum level ≥ 40mg/dL (OR 1,68; IK 95% 1,45-4,53), microscopic hematuria (OR 2,45; IK 95% 1,35-4,47), and glomerular filtration rate (OR 1,43 IK 95% 0,79-2,57). Factors which associated significantly with LSR on multivariate analysis include ureum level ≥ 40mg/dL (OR 2,199; IK 95% 1,19-4,04), microscopic hematuria (OR 2,05; IK 95% 1,08-3,88). Simpulan: Risk factors associated with LSR in INS are ureum level ≥ 40 mg/dL and microscopic hematuria."

"Katarak subkapsular posterior (SKP) dan peningkatan tekanan intraokular (TIO) adalah komplikasi okular tersering akibat penggunaan kortikosteroid oral. Hal ini dapat terjadi pada pemberian dosis tinggi dan jangka panjang. Di Indonesia, tidak data mengenai hubungan antara dosis dan lama terapi terhadap kedua komplikasi tersebut pada anak sindrom nefrotik idiopatik (SNI). Tujuan penelitian ini adalah untuk mengetahui hubungan antara dosis kumulatif, lama terapi dengan kejadian katarak SKP maupun peningkatan TIO dan faktor yang memengaruhinya pada anak SNI di rumah sakit Cipto Mangunkusumo (RSCM). Studi ini merupakan studi potong lintang pada anak SNI usia 4-18 tahun yang mendapat terapi kortikosteroid oral minimal enam bulan secara terus menerus. Pemeriksaan mata lengkap dilakukan untuk mengevaluasi katarak SKP, tajam penglihatan dan peningkatan TIO. Dari 92 anak yang dianalisis, terdapat 19,6% anak yang menderita katarak SKP, 12% anak dengan peningkatan TIO dan satu anak dengan best corrected visual acuity (BCVA) <6/20. Median dosis kumulatif kortikosteroid oral adalah 12.161 mg (rentang 1.795-81.398) dan median lama terapi adalah 23 bulan (rentang 6-84). Terdapat hubungan antara dosis kumulatif (P=0,007) dan lama terapi (P=0,006) terhadap kejadian katarak SKP dengan titik potong optimal 11.475 mg dan 24 bulan. Jenis kelamin perempuan akan meningkatkan kejadian katarak SKP sebesar empat kali dibandingkan lelaki (PR=4; IK 95%=1,57-13,38; P=0.001). Penelitian ini menunjukkan makin tinggi dosis kumulatif dan/atau makin lama terapi kortikosteroid oral, maka makin besar angka kejadian katarak SKP (nilai batasan ≥ 11.475 mg dan  ≥ 24 bulan). Dosis kumulatif dan lama terapi tidak berhubungan dengan kejadian peningkatan TIO.

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Posterior subcapsular cataract (PSC) and raised intraocular pressure (IOP) are the most common ocular complications due to administration oral corticosteroid. These can occur in high dose and long term use. In Indonesia, no data regarding correlation between dose, therapeutic duration and both complications in children with idiopathic nephrotic syndrome (INS). The aim of this study was to evaluate the correlation between cumulative dose, therapeutic duration with the occurrence of PSC and raised IOP and factors associated with these complications in children with INS at Cipto Mangunkusumo Hospital (CMH).

This is a cross-sectional study of children with INS aged 4-18 years who received oral corticosteroid therapy for at least six months continuously. A complete eye examination was performed to evaluate PSC, raised IOP and visual acuity. Of the 92 children analyzed, 19.6% had PSC, 12% had raised IOP and one child with best corrected visual acuity (BCVA) <6/20. The median cumulative dose of oral corticosteroids was 12,161 mg (range 1,795-81,398) and the median duration of therapy was 23 months (range 6-84). There were associaton between cumulative dose (P=0.007) and duration of therapy (P=0.006) to the occurrence of PSC with cut off point 11,475 mg and 24 months. Female sex will increase the occurence of PSC four times compared to male

(PR=4; 95% CI=1.57-13.38; P=0.001). This study revealed that the higher cumulative dose and/or

the longer of oral corticosteroid therapy, the higher occurence of PSC (cut off point ≥ 11.475 mg and ≥ 24 months). Cumulative dose and therapeutic duration were not associated with the occurence of raised IOP."