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"ABSTRAKLatar belakang: Sindrom nefrotik idiopatik (SNI) relaps anak terjadi karena ketidakseimbangan sel T-helper dan sel T-regulator. Perubahan komposisi bakteri usus besar dapat menyebabkan gangguan integritas usus, responsi imun, mungkin berperan terhadap relaps pada SNI.Tujuan: Untuk mengetahui jenis dan komposisi bakteri usus besar pada SNI remisi dan relaps, hubungan jenis dan komposisi bakteri usus besar dengan IL-8 serum SNI relaps, gangguan integritas usus besar pada SNI relaps.Metode: Penelitian prospektif di Departemen Ilmu Kesehatan Anak, FKUI- RSCM. Penelitian dua tahap yaitu SNI remisi yang diikuti sampai relaps. Diperiksa komposisi bakteri Enterococcus, Bacteroides, Escherichia, Clostridium, Lactobacillus, dan Bifidobacterium usus besar, alpha-1 antitrypsin dan calprotectin feses, IL-8 serum.Hasil: Terdapat 49 subjek yang relaps berumur 2?12 tahun. Proporsi Enterococcus, Bacteroides, Escherichia, Clostridium lebih tinggi pada SNI relaps daripada SNI remisi. Proporsi Bifidobacterium lebih tinggi pada SNI remisi daripada SNI relaps. Terdapat peningkatan alpha-1 antitrypsin pada 51% SNI remisi dan 48% SNI relaps, serta peningkatan calprotectin pada 91.8% SNI remisi dan 95.9% SNI relaps. Median IL-8 serum lebih tinggi pada SNI relaps (13.2 pg/mL) dibandingkan SNI remisi (11.8 pg/mL).Simpulan: Proporsi bakteri menguntungkan Bifidobacterium lebih tinggi pada SNI remisi dibandingkan SNI relaps. Proporsi bakteri patogen lebih tinggi pada SNI relaps dibandingkan dengan SNI remisi. Tidak terdapat hubungan antara jenis dan komposisi bakteri usus besar dengan peningkatan kadar IL-8 serum pada SNI relaps. Pada SNI relaps terdapat gangguan integritas usus besar.

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ABSTRACT

Backgound: Relapses in idiopathic nephrotic syndrome (INS) may occur due to imbalance of T-helper and regulator T-cells. Alteration of colonic bacteria composition may cause a defect in colonic mucosal integrity and activate the immune system, leading to INS relapse. The aim of this study are to determine the composition of gut bacteria in INS remission and relapse, serum IL-8 in INS relapse, and defective bowel integrity INS relapse.

Methods: This prospective study on children with INS was conducted in two phases, starting in remission and followed up to relapse. Both during remission and during relapse, we collected stool samples from all subjects to examine intestinal bacteria composition comprising Enterococci, Bacteroides, Escherichiae, Clostridia, Lactobacilli, and Bifidobacteria, fecal alpha-1 antitrypsin, and fecal calprotectin. We also collected peripheral blood to measure serum IL-8 levels during remission and relapse.

Results: The proportions of pathogenic bacteria Enteroccocus, Bacteroides, Escherichia, and Clostridium were higher in INS relapse compared to remission. The proportion of the beneficial Bifidobacteria was statistically higher in INS remission compared to relapse. There was an increase of alpha-1 antitrypsin in 51% of INS in remission and 48% in relapse. Fecal calprotectin was increased in 91.8% of INS in remission and 95.9% in relapse. Median serum IL-8 in INS relapse (13.2 pg/mL) was higher than in remission (11.8 pg/mL).

Conclusions: The proportion of Bifidobacteria is higher in INS remission than in relapse, while the proportion of pathogenic bacteria is higher in relapse than in remission. There is no association between the composition of gut bacteria with serum IL-8 increase in relapsing INS. There is a defect in mucosal integrity in relapsing INS as demonstrated by elevated fecal alpha-1-antitrypsin and calprotectin."

"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."

"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."