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Selli Muljanto
"[ABSTRAK
Lesi tubular lebih sering ditemukan pada sindrom nefrotik resisten steroid (SNRS)
dengan proteinuria masif, yang menyebabkan disfungsi tubulus proksimal. Cedera
tubular dapat pula didiagnosis dengan uji fungsi tubulus, diantaranya adalah fraksi
ekskresi magnesium (FE Mg) dan β2-mikroglobulin (β2M) urin. Tujuan
penelitian ini membandingkan FE Mg dan β2M urin pada SNRS dan SN sensitif
steroid (SNSS) remisi. Penelitian potong lintang dilakukan di Departemen Ilmu
Kesehatan Anak RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUD Ulin
Banjarmasin, RSUP Fatmawati dan RSAB Harapan Kita Jakarta pada Juli sampai
Desember 2015 pada penderita SNRS dan SNSS remisi berusia 2 ? 15 tahun. Pada
subyek diperiksakan kadar β2M urin dan FE Mg. Didapatkan 62 subyek yang
terdiri dari 31 subyek SNRS dan 31 subyek SNSS remisi. Rerata FE Mg pada
SNRS 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 SNRS
dibandingkan SNSS (p=0,007). Dengan titik potong 1,64%, peningkatan FE Mg
pada SNRS lebih banyak dibandingkan SNSS remisi (p=0,022). Simpulan: Fraksi
ekskresi 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 SNSS
remisi.

ABSTRACT
Tubular lesions more often found in steroid-resistant nephrotic syndrome (SRNS)
with massive proteinuria, leading to proximal tubular dysfunction. Tubular injury
can also be diagnosed by tubular function test, such as fractional excretion of
magnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this study
is to compare the FE Mg and urinary β2M on SRNS and steroid-sensitive
nephrotic syndrome (SSNS) in remission. A cross-sectional study was conducted
in the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUD
Ulin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July to
December 2015. Children aged 2-15 years who either had SRNS or SSNS in
remission were recruited. Fractional excretion of magnesium and urinary β2M
levels were examined. There were 62 subjects consisted of 31 subjects SRNS and
31 subjects SSNS in remission. The mean FE Mg on SRNS was significantly
higher than SSNS in remission (p=0.0065). Median levels of urinary β2M on
SRNS was higher than SNSS remission (p<0.001). Increased levels of urinary
β2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoff
point of 1.64%, an increased of FE Mg proportion on SRNS was more than
SSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg and
urinary β2M on SRNS were higher than SSNS in remission. There is a difference
between the increased of FE Mg on SRNS and SSNS in remission. The increased
of 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 injury
can also be diagnosed by tubular function test, such as fractional excretion of
magnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this study
is to compare the FE Mg and urinary β2M on SRNS and steroid-sensitive
nephrotic syndrome (SSNS) in remission. A cross-sectional study was conducted
in the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUD
Ulin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July to
December 2015. Children aged 2-15 years who either had SRNS or SSNS in
remission were recruited. Fractional excretion of magnesium and urinary β2M
levels were examined. There were 62 subjects consisted of 31 subjects SRNS and
31 subjects SSNS in remission. The mean FE Mg on SRNS was significantly
higher than SSNS in remission (p=0.0065). Median levels of urinary β2M on
SRNS was higher than SNSS remission (p<0.001). Increased levels of urinary
β2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoff
point of 1.64%, an increased of FE Mg proportion on SRNS was more than
SSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg and
urinary β2M on SRNS were higher than SSNS in remission. There is a difference
between the increased of FE Mg on SRNS and SSNS in remission. The increased
of 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 injury
can also be diagnosed by tubular function test, such as fractional excretion of
magnesium (Mg FE) and urinary β2-microglobulin (β2M). The aim of this study
is to compare the FE Mg and urinary β2M on SRNS and steroid-sensitive
nephrotic syndrome (SSNS) in remission. A cross-sectional study was conducted
in the Department of Pediatrics RSUPN Dr. Cipto Mangunkusumo Jakarta, RSUD
Ulin Banjarmasin, RSUP Fatmawati and RSAB Harapan Kita Jakarta from July to
December 2015. Children aged 2-15 years who either had SRNS or SSNS in
remission were recruited. Fractional excretion of magnesium and urinary β2M
levels were examined. There were 62 subjects consisted of 31 subjects SRNS and
31 subjects SSNS in remission. The mean FE Mg on SRNS was significantly
higher than SSNS in remission (p=0.0065). Median levels of urinary β2M on
SRNS was higher than SNSS remission (p<0.001). Increased levels of urinary
β2M was more significantly in SRNS compared to SSNS (p=0.007). With a cutoff
point of 1.64%, an increased of FE Mg proportion on SRNS was more than
SSNS in remission (p = 0.022). Conclusion: Fractional excretion of Mg and
urinary β2M on SRNS were higher than SSNS in remission. There is a difference
between the increased of FE Mg on SRNS and SSNS in remission. The increased
of urinary β2M on SRNS was higher than SSNS in remission.]"
2016
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UI - Tesis Membership  Universitas Indonesia Library
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Nila Akbariyyah
"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 memengaruhi
Metode: 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.

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.
"
Jakarta: Fakultas Kedokteran Universitas Indonesia, 2024
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UI - Tugas Akhir  Universitas Indonesia Library
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Andini Striratnaputri
"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.

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."
Jakarta: Fakultas Kedokteran Universitas Indonesia, 2020
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UI - Tugas Akhir  Universitas Indonesia Library
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Reza Fahlevi
"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.

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.
"
Jakarta: Fakultas Kedokteran Universitas Indonesia, 2024
SP-pdf
UI - Tesis Membership  Universitas Indonesia Library
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Stephanie Adelia
"

Kortikosteroid merupakan terapi utama pada sindrom nefrotik, tetapi memiliki efek samping penurunan kepadatan massa tulang. Pemeriksaan bone mineral density (BMD) merupakan cara terbaik untuk mengetahui kesehatan tulang, tetapi belum rutin dilakukan dan banyak diteliti di Indonesia, khususnya pada pasien sindrom nefrotik resisten steroid (SNRS). Penelitian ini bertujuan untuk mengetahui pengaruh durasi penggunaan, dosis kumulatif kortikosteroid, kadar vitamin 25(OH)D, dan kalsium terhadap penurunan nilai BMD pasien SNRS. Sebuah penelitian potong lintang terhadap 63 subjek SNRS di RSUPN Dr. Cipto Mangunkusumo yang berusia 5 – 18 tahun, mendapatkan kortikosteroid minimal 4 minggu dengan laju filtrasi glomerulus >60 ml/menit/1,73 m2. Dilakukan pemeriksaan laboratorium darah, BMD total body less head (TBLH) dan vertebrae lumbar. Subjek mendapatkan kortikosteroid dengan median durasi 37,4 (16,27 – 67,30) bulan, dosis harian 0,4 (0,30 – 0,67) mg/kgbb/hari dan dosis kumulatif 488,89 (309,62-746,05) mg/kgbb. Terdapat 66,7% subjek dengan defisiensi 25(OH)D. Hasil pemeriksaan BMD rendah lebih banyak didapatkan pada pemeriksaan BMD L1-L4 dibandingkan TBLH (25,4% vs 7,9%). Analisis bivariat dan multivariat menunjukkan bahwa durasi dan dosis kumulatif kortikosteroid memengaruhi penurunan nilai BMD L1-L4 (p<0,0001, p=0,0001, berturut – turut), tetapi kadar vitamin 25(OH)D dan kalsium darah tidak memengaruhi penurunan nilai BMD. Sebagai kesimpulan, durasi penggunaan dan dosis kumulatif kortikosteroid memengaruhi penurunan nilai BMD L1-L4 pasien SNRS.


Corticosteroids are the main therapy in nephrotic syndrome with the side effect of reducing bone density. Bone mineral density (BMD) examination is the best way to determine bone health, but it is not routinely carried out and studied in Indonesia, especially ​​in patients with steroid-resistant nephrotic syndrome (SRNS). This study aims to determine the effect of duration and cumulative dose of corticosteroids, vitamin 25(OH)D and calcium levels on decreasing BMD values ​​in SNRS patients. A cross-sectional study was conducted on 63 SRNS subjects at RSUPN Dr. Cipto Mangunkusumo, aged 5 – 18 years, received corticosteroids for at least 4 weeks with a glomerular filtration rate >60 ml/minute/1.73 m2. Laboratory blood test, total body less head (TBLH) and lumbar vertebrae BMD were carried out. Subjects received corticosteroids with a median duration of 37.4 (16.27 – 67.30) months, daily dose of 0.4 (0.30 – 0.67) mg/kgbb/day and cumulative dose of 488.89 (309, 62-746.05) mg/kgbb. There were 66.7% of subjects with 25(OH)D deficiency. Low BMD examination results were more frequently in L1-L4 BMD examinations compared to TBLH (25.4% vs 7.9%). Statistic analysis showed that the duration and cumulative dose of corticosteroids influenced the decrease in L1-L4 BMD values ​​(p<0.0001, p=0.0001, respectively), but the levels of vitamin 25(OH)D and calcium does not affect it. In conclusion, the duration of use and cumulative dose of corticosteroids influence the reduction in L1-L4 BMD values ​​in SNRS patients."
Jakarta: Fakultas Kedokteran Universitas Indonesia, 2024
T-pdf
UI - Tesis Membership  Universitas Indonesia Library
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Nurussafitri Laila Anawati
"[ABSTRAK
Sindrom nefrotik merupakan masalah ginjal yang sering terjadi pada anak.
Sindrom nefrotik ditandai dengan manifestasi klinis yang khas seperti proteinuria,
hipoalbuminemia, edema, dan hiperlipidemia. Edema sebagai salah satu
menifestasi klinis dari sindrom nefrotik menandakan bahwa anak mengalami
masalah keperawatan berupa kelebihan volume cairan. Karya ilmiah ini bertujuan
untuk memberikan gambaran dan analisis asuhan keperawatan pada An. A yang
mengalami masalah kelebihan volume cairan. Hasil dari intervensi keperawatan
yang telah dilakukan menunjukkan bahwa pemantauan cairan secara tepat mampu
mengatasi masalah kelebihan volume cairan, yang ditandai dengan berkurangnya
edema, penurunan berat badan dan lingkar abdomen. Pemantauan cairan yang
akurat pada klien dengan masalah kelebihan volume cairan sebaiknya dilakukan
untuk mencegah kelebihan atau defisit volume cairan berlebih dalam tubuh.ABSTRACT Nephrotic syndrome is the most common kidney problem in children. Clinical
manifestation of nephrotic syndrome is proteinuria, hypoalbuminemia, edema,
and hyperlipidemia. Edema is a sign that the child has fluid volume excess. This
case study aimed to describe and analyze nursing intervention in Child A with
fluid volume excess. The result showed that an accurate fluid monitoring was
effective to solve fluid volume excess problem and the child showed less edema,
decreased body weight and abdominal girth. An accurate fluid monitoring for the
client with fluid volume excess problem should be given to prevent volume excess
or deficit of body fluid, Nephrotic syndrome is the most common kidney problem in children. Clinical
manifestation of nephrotic syndrome is proteinuria, hypoalbuminemia, edema,
and hyperlipidemia. Edema is a sign that the child has fluid volume excess. This
case study aimed to describe and analyze nursing intervention in Child A with
fluid volume excess. The result showed that an accurate fluid monitoring was
effective to solve fluid volume excess problem and the child showed less edema,
decreased body weight and abdominal girth. An accurate fluid monitoring for the
client with fluid volume excess problem should be given to prevent volume excess
or deficit of body fluid]"
Fakultas Ilmu Keperawatan Universitas Indonesia, 2015
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UI - Tugas Akhir  Universitas Indonesia Library
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Monalisa Heryani
"Pendahuluan: Gejala sindroma nefrotik (SN) adalah proteinuria massif. Penambahan Angiotensin Converting Enzyme Inhibitor (ACEi) atau Angiotensin Receptor Blocker (ARB) membantu menurunkan proteinuria pasien SN yang telah mendapat steroid. Belum ada penelitian mengenai efektivitas dan keamanan kombinasi ACEi+ARB dalam penatalaksanaan pasien SN sensitif steroid (SNSS) relaps sering atau SN dependen steroid (SNDS). Metode: Penelitian kohort retrospektif yang menggunakan data rekam medis anak RSUPN dr. Cipto Mangunkusumo 2014-2018. Hasil: Dari 63 pasien yang dievaluasi, 33 pasien menggunakan ACEi+ARB dan 30 pasien menggunakan ACEi. Tidak terdapat perbedaan bermakna onset tercapainya proteinuria negatif (ACEi+ARB minggu ke-3 vs ACEi minggu ke-4, p=0.125). Tidak terdapat perbedaan bermakna proporsi pasien yang mencapai proteinuria negatif dalam 4 minggu terapi (ACEi+ARB 72.7% vs ACEi 63.3%, RR=1.148; IK95%: 0.815-1.619, p=0.424). Tidak terdapat perbedaan efek samping yang bermakna dalam hal hipotensi, peningkatan ureum dan kreatinin, hiperkalemia dan penurunan laju filtrasi glomerulus. Kesimpulan: Tidak terdapat perbedaan bermakna efektivitas dan keamanan kombinasi ACEi+ARB dibandingkan ACEi sebagai antiproteinuria pada pasien anak SNSS relaps sering atau SNDS.

Introduction: Symptoms of nephrotic syndrome (NS) is a massive proteinuria. The addition of Angiotensin Converting Enzyme Inhibitors (ACEi) or Angiotensin Receptor Blocker (ARB) can help to reduce proteinuria in NS patients who received steroids. There has been no study on the effectiveness and safety of ACEi+ARB combinations in the management of patients with frequent relapse NS (FRNS) or steroid-dependent NS (SDNS). Method: A retrospective cohort study using data collected from medical record of pediatrics with FRNS or SDNS at Cipto Mangunkusumo Hospital between 2014-2018 was conducted. Results: Out of the 63 patients who were evaluated, 33 patients were in ACEI+ARB while 30 other patients were in ACEi. There was no significant difference in the onset of negative proteinuria (3 weeks in ACEi+ARB vs 4 weeks in ACEi, p=0.125. There was no significant difference in the proportion of patients who achieved negative proteinuria in 4 weeks therapy (ACEi+ARB 72.7% vs ACEi 63.3%, RR=1,148; 95% CI: 0.815-1,619, p=0.424). There was no significant difference between ACEi+ARB and ACEi groups in the occurrence of hypotension, hyperkalemia, increased of creatinine serum, and decreased of glomerular filtration rate. Conclusion: There was no significant difference in the effectiveness and safety of the use of ACEi+ARB compared to ACEi as antiproteinuric in patients with FRNS or SDNS."
Jakarta: Fakultas Kedokteran Universitas Indonesia, 2019
SP-pdf
UI - Tugas Akhir  Universitas Indonesia Library
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Toruan, Yulia Margareta L.
"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.

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.
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Jakarta: Fakultas Kedokteran Universitas Indonesia, 2019
T58737
UI - Tesis Membership  Universitas Indonesia Library
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Ina Zarlina
"Latar Belakang. Sebagian anak dengan sindrom nefrotik sensitif steroid (SNSS) akan menjadi sindrom nefrotik relaps sering (SNRS) dan sindrom nefrotik dependen steroid (SNDS). Mereka akan mengalami relaps saat dosis kortikosteroid diturunkan atau dihentikan. Infeksi merupakan salah satu pencetus relaps pada SN. Defisiensi seng plasma ditemukan pada SN fase relaps dan remisi. Akibat defisiensi seng plasma terdapat peningkatan risiko infeksi.
Tujuan. Mengetahui rerata kadar seng plasma pada SNRS dan SNDS.
Metode. Uji potong lintang dilakukan di Poliklinik Nefrologi Departemen Ilmu Kesehatan anak FKUI/RSCM dan Poliklinik Asoka RSAB Harapan Kita selama bulan Desember 2014 sampai Juni 2015. Subjek adalah penderita SN relaps sering dan dependen steroid usia 5-15 tahun dalam keadaan relaps atau remisi. Pada subjek dilakukan pemeriksaan kadar seng plasma dan albumin. Sebagai kontrol adalah anak sehat yang dipilih secara matching dalam usia.
Hasil penelitian. Dalam penelitian ini diikutsertakan 51 subjek yang terdiri dari 23 pasien SN relaps dan 28 SN remisi. Hasil penelitian menunjukkan bahwa pencetus relaps terbanyak adalah ISPA (84,3%). Kadar seng plasma pada SN fase remisi lebih tinggi secara bermakna dibandingkan dengan kadarnya pada SN fase relaps.[46,6 (18,1) vs 67,4 (14,8) ug/dL, P= 0,0001]. Proporsi defisiensi seng plasma pada SN relaps (17/23anak) lebih besar secara bermakna terhadap SN remisi (4/28 anak), P=0,0001. Defisiensi seng plasma merupakan faktor risiko untuk timbulnya relaps pada SNRS dan SNDS [RP 4,05 (IK95% 1,92-8,52),P=0,0001].
Simpulan. Proporsi defisiensi seng plasma pada SN fase relaps lebih besar secara bermakna dibandingkan fase remisi. Rerata kadar seng plasma pada penderita SN relaps lebih rendah secara bermakna dibandingkan SN remisi.

Background. Fifty percents of children with steroid-sensitive nephrotic syndrome (SSNS) develop frequent relapsers and steroid-dependent nephrotic syndromes. Relapses can occur after corticosteroid therapy was stopped or rapid tappering off the prednisolone dose. Infections are the common causes of relapses in nephrotic syndrome. Low zinc level was found in nephrotic syndrome either in relapse or remission and this might lead to increased risk of infection.
Objectives. To analyze the mean of plasma zinc level in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.
Methods. This cross sectional study was conducted from December 2014 to June 2015 in Nephrology clinic, Child Health Departement, FKUI/RSCM dan Asoka clinic, RSAB Harapan Kita. Fifty-one children aged 5-15 years who either had frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome during remission or relapses were recruited. Twenty-eight healthy children who were matched for age were included as control. Plasma zinc levels and albumin were measured.
Results. Among 51 children with nephrotic syndrome, 28 were in remission while 23 were in relapses. Acute respiratory tract infection were the commonest (83,4%) cause triggering relapses. Plasma zinc levels in remission phase of nephrotic syndrome was significantly higher than relapse phase.[46,6 (18,1) vs 67,4 (14,8) ug/dL, P= 0,0001]. Zinc deficiency proportion in nephrotic syndromes during relapses (17/23 children) was significantly higher than remission (4/28 children), P=0,0001. Plasma zinc deficiency was the risk factor of relapses in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.[PR 4,05 (CI95% 1,92-8,52),P=0,0001].
Conclusions. Plasma zinc deficiency was significantly higher in nephrotic syndrome during relapses compared to remission. The mean plasma zinc levels in nephrotic syndrome during relapses was significantly lower compared to remission."
2015
SP-Pdf
UI - Tugas Akhir  Universitas Indonesia Library
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Yoshua Billy Lukito
"Relaps pada sindrom nefrotik dapat memengaruhi tumbuh kembang anak. Relaps dapat dipicu oleh beberapa faktor, salah satunya adalah infeksi. Diare adalah salah satu infeksi yang perlu diwaspadai pada anak, karena prevalensi diare di Indonesia cukup tinggi. Studi ini dilakukan untuk meneliti diare sebagai faktor risiko sindrom nefrotik idiopatik relaps pada anak di poliklinik anak RSCM. Studi ini dilakukan dengan kasus-kontrol berpasangan pada 38 pasang episode relaps dan remisi dari delapan belas pasien yang dilaksanakan Mei-Oktober 2015. Dalam studi ini dilakukan peninjauan adanya diare atau tidak dalam 2 minggu sebelumnya untuk setiap pasangan. Dengan uji hipotesis McNemar menggunakan program SPSS 20.0 for Windows didapatkan bahwa diare bukan merupakan faktor risiko relaps pada sindrom nefrotik (p = 0,18) dengan nilai RO = 3,5 (95%CI = 0,73-16,84). Uji perbandingan 2 proporsi menggunakan z-test menunjukkan perbedaan proporsi diare pada kelompok relaps dengan kelompok remisi tidak bermakna secara statistik (z = 1,34; p = 0,07) sehingga tidak dapat disimpulkan bahwa diare merupakan faktor risiko dari sindrom nefrotik relaps pada anak di RSCM. Terdapat kemungkinan bahwa diare bukan merupakan faktor risiko relaps dan dibutuhkan penelitian lain dengan bentuk studi kohort untuk membuktikannya

Relapse on Nephrotic Syndrome can cause abnormalities in children’s growth and development. Relapse can be caused by several factors, such as infection. Diarrhea is one of the infection which requires special attention in children due to prevalence of diarrhea in Indonesia which is quite high. This study was conducted to see the diarrhea as a risk factor of idiopathic nephrotic syndrome relapse in Pediatrics Health Center RSCM. Study was conducted with matched case control on 38 pairs of relapse-remission episodes from 18 patients and was conducted on May 2015 until October 2015. In this study, the occurence of diarrhea within 2 weeks prior of each control was valued. With hypothesis McNemar test by SPSS 20.0 for Windows result was obtained that diarrhea is not a risk factor of relapse in nephrotic syndrome (p = 0.18) with OR = 3.5 (95%CI = 0,73-16,84). Proportion of diarrhea between relapse group and remission group was analyzed through Z test and the difference between two groups is not statistically significant (Z = 1.34; p = 0.07) which is not conclusive enough to determine diarrhea as a risk factor of idiopathic nephrotic syndrome relapse in children in RSCM. There is a possibility that diarrhea is not a risk factor of nephrotic syndrome relapse. Another study with a cohort design is needed to prove the possibility.
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Jakarta: Fakultas Kedokteran Universitas Indonesia, 2015
S-Pdf
UI - Skripsi Membership  Universitas Indonesia Library
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