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"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.

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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."

"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."

"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."

"Latar belakang. Profil hormon tiroid belum banyak dipelajari pada anak dengan sindrom nefrotik idiopatik (SNI). Prevalens disfungsi tiroid pada anak dengan SNI di Indonesia belum jelas. Beberapa studi mempunyai hipotesis bahwa hipotiroidisme pada SNI dapat terjadi akibat peningkatan ekskresi protein pengikat hormon tiroid dan hormon tiroid. Terapi steroid merupakan salah satu faktor yang memengaruhi terjadinya hipotiroidisme.Tujuan. Mengetahui angka kejadian hipotiroidisme pada anak dengan SNI aktif dan remisi.Metode. Penelitian potong lintang yang dilakukan pada 103 pasien sindrom nefrotik idiopatik berusia 1-18 tahun di RSCM. Prevalens abnormalitas hormon tiroid adalah sebanyak 15,5% mengalami hipotiroidisme overt, 1,9% mengalami hipotiroidisme sekunder, 1,9% mengalami hipotiroidisme subklinis, 47,6% mengalami low-T3 syndrome, 10,7% mengalami low-T3 dan low-T4 syndrome dan sebanyak 22,3% subjek dengan status eutiroid. Sebanyak 16/103 subjek pada penelitian ini mengalami hipotiroidisme overt. Pada penelitian ini, seluruh subjek yang mengalami hipotiroidisme overt tersebut berasal dari kelompok SNI aktif. Secara statistik terdapat hubungan bermakna antara status SNI aktif dengan kejadian hipotiroidisme overt dengan nilai p <0,001. Pada penelitian ini, 13/16 subjek yang mengalami hipotiroidisme overt tersebut mengalami hipoalbuminemia Secara statistik terdapat hubungan bermakna antara hipoalbuminemia pada SNI dengan kejadian hipotiroidisme overt dengan nilai p <0,001. Rasio protein/kreatinin urin sewaktu berkorelasi negatif dengan kadar T3, T4, dan T4 bebas serum (r=-0,563, p=<0,001; r=-0,586, p=<0,001; r=-0,405, p=<0,001), secara berturut-turut. Rasio protein/kreatinin urin sewaktu berkorelasi positif dengan kadar TSH serum (r=0,618, p=<0,001).Kesimpulan. Prevalens abnormalitas hormon tiroid pada anak dengan SNI adalah sebanyak 15,5% mengalami hipotiroidisme overt. Proteinuria masif dan hipoalbuminemia merupakan salah satu faktor risiko terjadinya hipotiroidisme pada pasien anak dengan SNI. Pemeriksaan penapisan hipotiroidisme overt (TSH dan T4 bebas) dapat dilakukan pada kelompok SNI fase aktif dan/atau kelompok SNI yang mengalami hipoalbuminemia.

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Background. Thyroid hormone profiles in Indonesian pediatric idiopathic nephrotic syndrome (INS) patient has not been fully studied. The prevalence of hypothyroidism in INS has not been established. Nephrotic syndrome is a common kidney disease among children which is characterized by proteinuria, hypercholesterolemia, hypoproteinemia, and edema. The urinary losses of proteins including albumin, thyroid hormone and thyroid-binding globulin might affect the thyroid hormone levels in those children. Glucocorticoid might also affect the occurrence of hypothyroidism in INS patients.

Objectives. To evaluate the prevalence of hypothyroidism in active and remission pediatric INS patients.

Methods. In this cross-sectional study included 103 pediatric INS patients. The thyroid hormone profiles included serum levels of triiodothyronine (T3), thyroxine (T4), thyroid-stimulating hormone (TSH), and free T4.

Results. In this study we recruited 103 children aged 1-18 years with active and remission phase INS. Of the 103 patients, 15.5% had overt hypothyroidism, 1.9% had subclinical hypothyroidism, and had 47.6% low-T3 syndrome and 10.7% had low-T3 and low-T4 syndrome. Of the 16/103 patients, 16 had overt hypothyroidism. All subjects with overt hypothyroidism are active INS patients. There was significant relationship between active INS and overt hypothyroidism. There was also significant relationship between hypoalbuminemia and overt hypothyroidism. The urinary protein/ creatinine ratio was significantly negatively correlated with serum T3, T4, and free T4 levels (r=-0.563, P=<0.001; r=-0.586, P=<0.001; r=-0.405, P=<0.001, respectively) as well as it positively correlated with TSH levels (r=0.618, P=<0.001).

Conclusion. Overt hypothyroidisms was observed in 15.5% pediatric patients with active INS. Massive proteinuria and hypoalbuminemia are risk factors of overt hypothyroidism in INS patients. Thyroid profile should be evaluated routinely in active and/or hypoalbuminemia subset of patients."

"[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.]"

"Latar belakang: Sindrom nefrotik (SN) idiopatik merupakan penyakit glomerulus dengan proteinuria akibat peningkatan permeabilitas glomerulus. Transferin merupakan salah satu protein yang keluar di urin dan dapat mengganggu homeostasis besi. Keadaan ini dapat menyebabkan defisiensi besi dan anemia defisiensi besi (ADB). Tujuan: Mengetahui perbedaan status besi, transferin urin, proporsi defisiensi besi dan ADB pada pasien SN idiopatik aktif dan remisi. Metode: Penelitian potong lintang pada pasien SN idiopatik aktif dan remisi usia 1-18 tahun di RSCM. Pengukuran status besi menggunakan Hb,MCV, MCH, Ret-He, SI, TIBC, ferritin, dan saturasi transferin. Pengukuran transferin urin menggunakan metode enzyme-linked immunosorbent assay (ELISA). Hasil: Terdapat 65 subyek, dengan 32 pasien SN idiopatik aktif dan 33 pasien remisi. Kadar SI antara kelompok aktif dan remisi adalah 60,7±33,5 µg/dL dan 84,6±35,3 µg/dL (p<0,05). Kadar TIBC antara kelompok aktif dan remisi adalah 220±90,7 µg/dL dan 309,4(±47,7) µg/dL (p<0,05). Kadar transferin urin antara kelompok aktif dan remisi adalah 435,3(7,7-478,4) ng/mL dan 23,4 (0-358) ng/mL (p<0,05). Proporsi defisiensi besi dan ADB pada kelompok aktif adalah 7(21,9%) dan 5 (15,6%) subyek, sedangkan pada kelompok remisi adalah 4(12,6%) dan 1(3%) subyek. Perbedaan proporsi tersebut tidak bermakna (p=0,04; RR 2,47; IK95% 0,98-6,23). Kesimpulan: Kelompok SN idiopatik aktif memiliki nilai SI dan TIBC yang rendah serta transferin urin yang tinggi. Proporsi defisiensi besi dan ADB pada kelompok SN idiopatik aktif lebih tinggi walaupun tidak bermakna secara statistik.

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Background: Idiopathic nephrotic syndrome (NS) is a common glomerular disease in children, which cause increased glomerular permeability resulting in proteinuria. Transferrin is one of the protein that is excreted in the urin, thus disturbing iron homeostasis and may lead to iron deficiency (ID) or iron deficiency anemia (IDA).

Objective: To know the differences in iron status, urinary transferrin, and the proportion of ID and IDA in children with active and remission idiopathic NS.

Methods: A cross-sectional design study was conducted on patients with active and remission idiopathic NS aged 1-18 years at RSCM. Measurement of iron status using Hb, MCV, MCH, Ret-He, SI, TIBC, ferritin, and transferrin saturation. Measurement of urinary transferrin using enzyme-linked immunosorbent assay (ELISA).

Result: There were 65 study subjects, with 32 patients with active idiopathic NS and 33 subjects were in remission.The SI levels between the active and remission groups were 60.7±33.5 g/dL and 84.6±35.3 g/dL (p<0.05). The TIBC levels between the active and remission groups were 220±90.7 g/dL and 309.4(±47.7) g/dL (p<0.05). The median of urinary transferrin levels between the active and remission groups were 435.3(7.7-478.4) ng/mL and 23.4 (0-358) ng/mL (p<0.05). The proportions of ID and IDA in the active group were 7(21.9%) and 5(15.6%) subjects, while in the remission group were 4(12.6%) and 1(3%) subjects. Nonetheless the difference were not statistically significant (p=0.04; RR 2.47; CI95% 0.98-6.23).

Conclusion. Active idiopathic NS had significant lower values of SI and TIBC, and higher urinary transferrin levels. The proportion of ID and IDA in the active group was higher, although not significant."

"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

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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."

"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."

"Sindrom nefrotik merupakan penyakit ginjal yang sering terjadi pada anak ditandai dengan proteinuria, hipoalbuminemia, dan edema. Pasien anak sindrom nefrotik dapat mengalami relaps yang dipicu oleh infeksi sebelumnya. Infeksi yang sering dilaporkan pada sindrom nefrotik adalah infeksi saluran pernapasan akut (ISPA). Penelitian dilakukan untuk mengetahui hubungan ISPA dengan kejadian relaps pada anak dengan sindrom nefrotik. Desain penelitian ini adalah kasus-kontrol berpasangan mengamati apakah terdapat ISPA sebelum relaps pada sindrom nefrotik. Penelitian dilakukan di Departemen Ilmu Kesehatan Anak RSCM pada bulan Mei-Desember 2015. Uji untuk mengetahui perbedaan proporsi pajanan ISPA antara sindrom nefrotik relaps dan remisi adalah z-test. Metode uji hipotesis digunakan McNemar dan rasio odds (RO) menggunakan program SPSS for Windows versi 20.0. Dengan menggunakan z-test 2 proporsi, terdapat perbedaan bermakna antara proporsi ISPA pada relaps vs kontrol (42,1 % vs 18,4 %; p=0,02). Uji hipotesis McNemar menunjukkan pajanan ISPA dan relaps pada sindrom nefrotik memiliki hubungan yang signifikan (p=0,049) dengan RO 3,25(IK 95% 1,06-9,97). Disimpulkan bahwa ISPA merupakan faktor risiko relaps pada anak dengan sindrom nefrotik.

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Nephrotic syndrome is a kidney disease commonly found in children. This disease is characterized by proteinuria. In its natural course, some patients may experience relapse. Relapse in nephrotic syndrome can be triggered by previous infections. Respiratory tract infection (RTI) is frequently reported to occur in patients with relapse of nephrotic syndrome. This research aimed to investigate the association between RTI and relapse in nephrotic syndrome. This research was a matched case-control study that observed whether there was RTI before relapse of nephrotic syndrome. The research was done at Department of Child Health RSCM from May-December 2015. Z-test was used to investigate the difference of RTI exposure in relapse and remission. McNemar test was used to test the hypothesis and Odds Ratio (OR) was calculated with the program SPSS for Windows version 20.0. Using 2 proportions z-test, there was a significant difference between RTI in relapse patients vs control (42.1 % vs 18.4 %; p=0.02). McNemar hypothesis test for RTI exposure to relapse had a significant association (p=0.049) with OR 3.25(CI 95% 1.06-9.97). Therefore, RTI was a risk factor of relapse for pediatric patients with nephrotic syndrome."

"Latar belakang: Pengukuran proteinuria kuantitatif sewaktu (rasio protein/kreatinin urin sewaktu) merupakan metode terbaik untuk evaluasi proteinuria sebagai penanda remisi komplit dan nephrotic-range proteinuria pada pasien anak sindrom nefrotik (SN), karena dianggap lebih praktis dibandingkan baku emas (protein urin tampung 24 jam). Tujuan: Mencari cut-off optimal rasio protein/kreatinin urin sewaktu untuk evaluasi nephrotic-range proteinuria dan remisi komplit dalam penelitian kami serta membandingkan sensitivitas, spesifisitas, nilai duga positif, dan nilai duga negatif antara cut-off yang ditemukan dalam penelitian versus KDIGO (Kidney Disease: Improving Global Outcomes) untuk evaluasi nephrotic-range proteinuria dan remisi komplit. Metode: Penelitian ini merupakan studi potong lintang dengan uji diagnostik yang melibatkan 96 sampel urin 24 jam dan urin sewaktu yang diambil dari anak dengan sindrom nefrotik berusia 3−18 tahun. Subjek penelitian selain diambil sampel urin untuk pemeriksaan protein urin tampung 24 jam dan rasio protein/kreatinin urin sewaktu, juga dilakukan pemeriksaan antropometri untuk menentukan status nutrisi. Analisis menggunakan kurva ROC untuk menentukan cut-off optimal rasio protein/kreatinin urin sewaktu untuk evaluasi nephrotic-range proteinuria dan remisi komplit dalam penelitian kami, kemudian dihitung nilai sensitivitas, spesifisitas, nilai duga positif, dan nilai duga negatif serta dibandingkan nilainya dengan cut-off yang telah ditetapkan oleh KDIGO. Hasil: Cut-off optimal rasio protein/kreatinin urin sewaktu dalam peneltian kami untuk evalusi proteinuria yang menandai remisi komplit adalah <0,4 g/g dan yang menandai nephrotic-range proteinuria (tidak remisi/relaps) adalah >1,5 g/g. Perbandingan nilai sensitivitas, spesifisitas, PPV, dan NPV antara cut-off rasio protein/kreatinin urin sewaktu <0,4 g/g (temuan penelitian) berturut-turut 80,1%, 82,3%, 89,1%, dan 68,3% versus cut-off rasio protein/kreatinin urin sewaktu <0,2 g/g (KDIGO) berturut-turut 95,2%, 44, 1%, 75,6 %, dan 83,3%. Perbandingan nilai sensitivitas, spesifisitas, PPV, dan NPV antara cut-off rasio protein/kreatinin urin sewaktu >1,5 g/g (temuan penelitian) untuk evaluasi nephrotic-range proteinuria berturut-turut 88,5%, 84,3%, 67,7%, dan 95,2% versus cut-off rasio protein/kreatinin urin sewaktu >2 g/g (KDIGO) berturut-turut 84,6%, 91,4%, 78,6%, dan 94,1%. Kesimpulan: Cut-off rasio protein/kreatinin urin sewaktu untuk evaluasi proteinuria nephrotic-range proteinuria (tidak remisi/relaps) pada penelitian kami memperkuat cut-off yang telah dikeluarkan oleh KDIGO sebesar >2 g/g, sementara cut-off untuk evaluasi remisi komplit lebih tinggi nilainya dibandingkan KDIGO sebesar <0,4 g/g.

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Background: Quantitative measurement of proteinuria while (urinary protein/creatinine ratio) is the best method for evaluating proteinuria as a marker of complete remission and nephrotic-range proteinuria in nephrotic syndrome (NS) pediatric patients, because it is considered more practical than the gold standard (24 hours urine protein collection). Objective: Finding the optimal cut-off of urinary protein/creatinine ratio while evaluating nephrotic-range proteinuria and complete remission in our study and comparing sensitivity, specificity, positive predictive value, and negative predictive value between the cut-off found in the study versus KDIGO (Kidney Disease : Improving Global Outcomes) for evaluation of nephrotic-range proteinuria and complete remission.

Method: This study is a cross-sectional study with diagnostic tests involving 96 24-hour urine samples and urine samples taken from children with nephrotic syndrome aged 3−18 years. The subjects of the study were not only taking urine samples for 24-hour storage of urine protein and urine protein/creatinine ratio, as well as anthropometric examination to determine nutritional status. Analysis used the ROC curve to determine the optimal cut-off of urinary protein/creatinine ratio while evaluating nephrotic-range proteinuria and complete remission in our study, then calculated the values of sensitivity, specificity, positive predictive value, and negative predictive value and compared their values with the cut-off values set by KDIGO.

Result: The optimal cut-off of the urinary protein/creatinine ratio during our study for the evaluation of proteinuria that characterized complete remission was <0,4 g/g and that of nephrotic-range proteinuria (no remission/relapse) was >1,5 g/g. Comparison of the values of sensitivity, specificity, PPV, and NPV between the cut-off ratio of urine protein/creatinine when <0,4 g/g (study finding) were 80,1%, 82,3%, 89,1%, and 68,3% versus cut-off urinary protein/creatinine ratio at <0,2 g/g (KDIGO) 95,2%, 44,1%, 75,6%, and 83,3%. Comparison of the values of sensitivity, specificity, PPV, and NPV between the cut-off ratio of urine protein/creatinine when >1,5 g/g (study finding) for evaluation of nephrotic-range proteinuria 88,5%, 84,3%, 67,7%, and 95,2% versus cut-off urinary protein/creatinine ratio at >2 g/g (KDIGO) 84,6%, 91,4%, 78,6%, and 94,1%.

Conclusion: The cut-off of the urine protein/creatinine ratio during the evaluation of nephrotic-range proteinuria (non-remitting/relapsed) in our study reinforces the cut-off that has been issued by KDIGO of >2 g/g, while the cut-off for evaluation of complete remission is more higher value compared to KDIGO of <0,4 g/g."