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"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."

"[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. 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."

"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."

"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."