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"Tata laksana nutrisi pada sindroma nefrotik idiopatik anak meliputi penilaian status gizi, kebutuhan nutrisi baik makronutrien, mikronutrien, maupun managemen cairan. Penyakit sindroma nefrotik anak dapat menyebabkan berbagai komplikasi yang mengganggu pertumbuhan, memperberat kerja ginjal hingga berakhir pada keadaan gagal ginjal. Untuk itu peran nutrisi menjadi sangat penting dalam menekan progresifitas penyakit dan memperbaiki kualitas hidup pasien. Keempat pasien serial kasus ini memiliki karakteristik penyakit sindroma nefrotik idiopatik, dengan rentang usia 1–8 tahun, semua kasus merupakan serangan pertama dan sedang menjalani perawatan di sebuah rumah sakit. Penghitungan kebutuhan energi menggunakan rumus Schoefield (W–H) dikalikan faktor stres, kebutuhan protein sesuai RDA dikalikan faktor stres, dan lemak tidak lebih dari 28% total kalori, dengan komposisi SAFA 8 %, PUFA 8% dan MUFA 12 %. Berdasarkan hasil analisis keempat kasus tersebut, pencapaian asupan sesuai kebutuhan energi total sudah mencapai 100 % pada kisaran hari perawatan ke–3 sampai ke–6, dengan rata–rata kepulangan pasien setelah perawatan hari ke–7. Terjadinya peningkatan tekanan darah di atas persentil rata–rata mengalami perbaikan seiring perbaikan klinis yang terjadi. Pemberian nutrisi pada pasien sindroma nefrotik anak dilakukan secara individual, menyangkut status gizi, analisis asupan, serta berbagai komplikasi yang terjadi. Monitoring dan evaluasi meliputi keadaan klinis, tanda vital, analisis asupan dan toleransi, keseimbangan cairan dan elektrolit, keadaan hipoalbuminemia, proteinuria, hematuria dan gambaran darah lengkap. Tata laksana nutrisi yang optimal harus disertai konseling dan motivasi kepada orang tua pasien ataupun pengasuh, dengan harapan dapat menekan progresifitas penyakit, meminimalisir kekambuhan, menekan komplikasi lebih lanjut, tercukupinya kebutuhan nutrisi, perbaikan status nutrisi, dan tercapainya tumbuh kembang yang optimal

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Nutritional management therapy for idiopathic nephrotic syndrome in children includes nutritional status assessment, nutritional requirement including macronutrient, micronutrient, and fluid management. Nephrotic syndrome in children could cause several complications which disrupt growth and worsening kidney function which ends to kidney failure. According to that condition, nutritional therapy has become more important to alleviate disease progression and increase quality of life of the patient. On this case series, four patients had the characteristics of idiopathic nephrotic syndrome. All of them was on the age group of 1–8 years, on the first attack, and admitted in certain hospital. Energy requirement calculation was done using Schoefield (W-H) formula multiplied by stress factor, protein requirement based on RDA multiplied by stress factor, and fat requirement was no more than 28% of total calories, with the composition of SAFA 8%, PUFA 8%, and MUFA 12%. Based on the analysis of those patients, energy intake of the patients which met 100% of total energy requirement had accomplished on day 3 to day 6 of hospitalization, and they were discharged from hospital after 7 days hospitalization. An increase in blood pressure above the median percentile improved as clinical improvement occurs. Nutritional management therapy for nephrotic syndrom in children was done individually, includes nutritional status, dietary assesment, and the possible complications. Monitoring and evaluations included clinical condition, vital signs, dietary assesment and tolerance, fluid and electrolyte balance, hypoalbuminemia condition, proteinuria, hematuria, and full blood count. Optimal nutritional management therapy should be completed with counseling and encouragment to parents or caregiver to alleviate the disease progression, prevent relaps, and avoid further complications, nutritional requirement completion, nutritional status improvement, and optimal growth and development."

"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."

"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."

"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: 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."

"[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.]"

"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.

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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."

"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."

"Latar belakang. Sindrom nefrotik (SN) merupakan penyakit ginjal yang sering ditemukan pada anak. Komplikasi SN terkait perjalanan penyakit, terapi, fisik dan psikososial yang memengaruhi kualitas hidup. Tujuan. Mendapatkan gambaran tentang kualitas hidup anak SN yang berobat di Departemen Ilmu Kesehatan Anak (IKA) Rumah Sakit Dr. Cipto Mangunkusumo (RSCM) dan faktor yang berhubungan. Metode. Studi deskriptif analitik dengan desain potong lintang di poliklinik nefrologi dan rawat inap Departemen IKA RSCM periode April 2012 - Desember 2013. Subjek berusia 2-18 tahun. Penilaian kualitas hidup menggunakan PedsQLTM 4.0 modul generik dan pola asuh menggunakan Kuesioner Pola Asuh Anak (KPAA). Hasil. Seratus pasien SN mengikuti penelitian dan didapatkan gangguan kualitas hidup berdasarkan laporan orangtua dan laporan anak 19%. Usia 5-7 tahun, usia 13-18 tahun, status sosioekonomi rendah, dan kondisi relaps jarang pada SN sensitif steroid (SNSS) merupakan faktor risiko gangguan kualitas hidup pada laporan orangtua (p<0,05) dengan rasio prevalens secara berurutan 5,22, 7,5, 3,48, 10,33. Penggunaan steroid saat penelitian memiliki hubungan dengan kualitas hidup berdasarkan laporan orangtua (p<0,05). Tingkat pendidikan ayah yang semakin rendah merupakan faktor risiko gangguan kualitas hidup pada laporan anak (p<0,05) dengan rasio prevalens 5,22. Lama diagnosis memiliki hubungan dengan kualitas hidup pada laporan orangtua dan anak (p<0,05). Status sosioekonomi dan pola asuh merupakan faktor risiko gangguan kualitas hidup. Simpulan. Usia prasekolah, remaja, kondisi relaps jarang, penggunaan steroid, lama diagnosis, tingkat pendidikan ayah semakin rendah, status sosioekonomi dan pola asuh akan memengaruhi kualitas hidup anak SN. Gangguan kualitas hidup pada pasien SN merupakan masalah yang perlu diperhatikan dalam tata laksana.

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Background. Nephrotic syndrome (NS) is the most common kidney disease in children. Complication of NS is associated with course of disease, therapy, and psychosocial condition affecting the quality of life (QoL).

Aim. Describing the QoL in children with NS and its associated factors in pediatric outpatient clinic and ward in Dr. Cipto Mangunkusumo Hospital.

Method. Cross sectional study was performed in pediatric nephrology clinic and ward in Dr. Cipto Mangunkusumo Hospital, from April 2012 to December 2013. Subjects were 2 to 18 years old. Quality of life was assessed using PedsQLTM 4.0 generic module and parenting style was assessed with Kuesioner Pola Asuh Anak.

Results. One hundred subjects participated in this study and QoL impairment was reported in 19% subjects based on report from parents and children. Factors associated with impairment QoL from parent's report were age group 5 - 7 years old, age group 13 - 18 years old, low socioeconomic status, and infrequent relapse condition in steroid sensitive NS (P<0.05), with prevalence ratio 5.22, 7.5, 3.48, 10.33, respectively. Steroid use was also associated with QoL according to parent’s report (P<0,05). Lower father's education was risk factor for QoL impairment according to patient's report (P<0,05) with prevalence ratio 5.22. Duration of diagnosis was associated with QoL according to parent’s and patient’s report (P<0,05). Socioeconomic status and parenting style were risk factors of QoL impairment in children with NS.

Conclusion. Preschool age, teen age, infrequent relapse, steroid use, duration of diagnosis, low father’s education, socioeconomic status, and parenting style are associated with QoL in children with NS. Quality of life impairment is important in management of children with NS. "

"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."