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All Journal The Indonesian Biomedical Journal Sari Pediatri Paediatrica Indonesiana Archives of Pediatric Gastroenterology, Hepatology, and Nutrition
Eka Laksmi Hidayati, Eka Laksmi
Departemen Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Indonesia/RS Cipto Mangunkusumo, Jakarta
Biochemistry, Genetics & Molecular Biology Health Professions Medicine & Pharmacology Public Health

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Incidence and risk factors of nephritis in childhood Henoch-Schonlein purpura Yaulia Yanrismet; Eka Laksmi Hidayati; Zakiudin Munasir; Klara Yuliarti; Afifa Fahriyani
Paediatrica Indonesiana Vol 63 No 4 (2023): July 2023
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi63.4.2023.304-14

Abstract

Background Henoch-Schönlein Purpura (HSP) is the most common systemic vasculitis disease in children. It is characterized by involvement of the skin, joints, gastrointestinal tract, and kidney. Kidney manifestations may progress to severe nephritis, even lead to end-stage kidney disease. Objective To identify the incidence and risk factors of nephritis in childhood HSP. Methods A retrospective cohort study was performed to evaluate clinical, demographic, laboratory, and therapeutic parameters of HSP patients aged 0-18 years between 2011-2019 at Dr. Cipto Mangunkusumo Hospital, Jakarta. Diagnoses of HSP were made according to the 2008 EULAR/PRES/PRINTO criteria. Wefollowed subjects’ medical records for at least 3 months after disease onset to observe incidence and risk factors of Henoch-Schönlein nephritis (HSN).Results There were 112 HSP patients (aged 2-17 years) included in this study. HSN was found in 40 out of 112 patients (35.7%). Nephritis developed within the first 4 weeks for a majority of cases. Multivariate analysis showed that persistent purpura (OR 3.306; 95%CI 1.315 to 8.315; P=0.011) and acute phase leukocytosis(OR 2.585; 95%CI 1.047 to 6.385; P=0.039) were significantly associated risk factors for HSN. We found that corticosteroid use did not reduce the risk of HSN. The accumulation of several risk factors was associated with the likelihood of developing HSN. Conclusion Persistent purpura and acute phase leukocytosis are independent risk factors for HSN. Therefore, blood tests are needed to estimate the risk of HSN. Early corticosteroid therapy do not reduce the risk of kidney impairment.
Elevated Levels of Urinary Podocyte-Derived Microparticles in Nephrotic Syndrome Eka Laksmi Hidayati; Bambang Supriyatno; Sudung Oloan Pardede; Partini Pudjiastuti Trihono; Dewi Sukmawati; Dewi Wulandari; Oke Rina Ramayani
The Indonesian Biomedical Journal Vol 15, No 5 (2023)
Publisher : The Prodia Education and Research Institute (PERI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.18585/inabj.v15i5.2553

Abstract

BACKGROUND: Nephrotic syndrome (NS) is the most common glomerular disease in childhood. The proposed hypothesis for the pathogenesis of this disease has changed over time, from immune dysregulation theory and systemic circulating factors theory, to the growing recognition of podocytopathies’ role. The existance of podocytopathies is usually examined by using podocyte-derived microparticles (MPs), such as nephrin, podocin, and podocalyxin (PCX). Therefore in this study, the difference between nephrin, podocin, and PCX expressions in NS children and healthy children was investigated.METHODS: An observational cross-sectional study was conducted, involving 33 children with NS and 22 age-matched healthy children as controls. Urine samples were collected from each subject in the early morning, before being processed and incubated with antibodies to detect nephrin, podocin, and PCX. The processed samples were then analyzed with flow cytometer methods.RESULTS: NS subjects had significantly higher expression of all three urinary podocyte-derived MPs compared to the control subjetcs. Nephrin, podocin, and PCX showed good discrimination in NS subjects with the area under curve (AUC) of 0.895, 0.849, and 0.728, respectively.CONCLUSION: This study revealed the differential expression of podocyte proteins in NS subjects compared to healthy controls. This supports the role of podocytopathies in the pathogenesis of NS. Therefore, nephrin, podocin, and PCX might have potentials to be future non-invasive diagnostic tools in glomerular disease.KEYWORDS: nephrin, nephrotic syndrome, podocalyxin, podocin, podocyte, urinary microparticle
Telaah Perbandingan Panduan Klinis Sindrom Nefrotik Idiopatik Resisten Steroid pada Anak Sudung Oloan Pardede; Reza Fahlevi; Edwin Kinesya; Eka Laksmi Hidayati; Henny Adriani Puspitasari; Partini Pudjiastuti Trihono
Sari Pediatri Vol 25, No 3 (2023)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/sp25.3.2023.137-46

Abstract

Latar belakang. Sebagai salah satu penyakit ginjal anak tersering di dunia, sindrom nefrotik dapat dibedakan menjadi sensitif dan resisten steroid. Penelitian dan tata laksana sindrom nefrotik resisten steroid pada anak terus berkembang. Panduan klinis yang digunakan seringkali berbeda dan bervariasi antar fasilitas kesehatan ataupun negara di dunia.Tujuan. Membandingkan panduan klinis sindrom nefrotik idiopatik resisten steroid pada anak. Metode. Kami menentukan topik dan lingkup bahasan yang akan dibahas. Sesudah itu, dilakukan telaah dan perbandingan terhadap empat panduan klinis dari Ikatan Dokter Anak Indonesia tahun 2012, Kidney Disease: Improving Global Outcomes tahun 2021, Indian Society of Pediatric Nephrology tahun 2021, dan International Pediatric Nephrology Association tahun 2020. Empat lingkup bahasan kajian antara lain diagnosis, pemeriksaan penunjang, batasan kriteria, dan terapi.Hasil. Didapatkan beberapa perbedaan mendasar yang ditemukan, antara lain, adanya periode konfirmasi, beberapa istilah baru, anjuran pemeriksaan genetik, serta pilihan utama terapi imunosupresan. Kesimpulan. Sesudah menelaah panduan klinis sindrom nefrotik idiopatik resisten steroid dari Ikatan Dokter Anak Indonesia tahun 2012 dan panduan klinis baru lainnya, ditemukan beberapa pebedaan dasar. Oleh karena itu, diperlukan pembaharuan konsensus sindrom nefrotik resisten steroid yang disesuaikan dengan bukti ilmiah terbaru serta ketersediaan fasilitas kesehatan dan obat-obatan di Indonesia.
Perdarahan Saluran Cerna pada Anak dengan Penyakit Ginjal Tahap Akhir Beatrix Siregar; Eka Laksmi Hidayati; Sudung Oloan Pardede
Sari Pediatri Vol 25, No 2 (2023)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/sp25.2.2023.130-6

Abstract

Penyakit ginjal tahap akhir (PGTA) adalah penyakit ginjal kronik (PGK) stadium 5 yang ditandai dengan laju filtrasi glomerulus (LFG) < 15 mL/menit/1,73m2 selama tiga bulan atau lebih, atau suatu kondisi dengan pasien memerlukan terapi pengganti ginjal seperti hemodialisis, dialisis peritoneal, atau transplantasi ginjal. Pasien dengan PGTA sering mengalami gangguan saluran cerna, termasuk perdarahan saluran cerna, yang dapat terjadi dari atas maupun bawah. Perdarahan saluran cerna atas lebih umum ditemukan dan berkaitan dengan erosi saluran cerna. Pada pasien anak dengan PGTA, erosi saluran cerna umum ditemukan namun angka kejadian perdarahan saluran cerna berkaitan dengan erosi pada anak dengan PGTA belum diketahui. Patofisiologi perdarahan saluran cerna pada pasien PGTA belum sepenuhnya diketahui, namun diduga berkaitan dengan disfungsi trombosit akibat uremia, anemia, dan penggunaan obat-obatan. Mekanisme perdarahan saluran cerna tergantung etiologinya, terutama erosi dan angioektasia. Belum ada pendekatan dan tata laksana perdarahan saluran cerna khusus pada anak dengan PGTA yang terbukti secara uji klinis, namun optimalisasi dialisis dan tata laksana anemia dapat dilakukan sebagai tindakan pencegahan.
Sindrom Nefrotik Monogenik: Pendekatan Klinis dan Diagnosis Fahlevi, Reza; Trihono, Partini Pudjiastuti; Muktiarti, Dina; Wahidiyat, Pustika Amalia; Hidayati, Eka Laksmi; Hafifah, Cut Nurul
Sari Pediatri Vol 26, No 3 (2024)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/sp26.3.2024.189-96

Abstract

Sindrom nefrotik merupakan penyakit ginjal yang sering ditemukan pada anak-anak, dengan insiden 1-3 per 100.000 anak di bawah usia 16 tahun. Sekitar 10-20% anak dengan sindrom nefrotik mengalami sindrom nefrotik resisten steroid (SNRS), dan 10-30% dari kasus ini disebabkan oleh kelainan genetik. Pada SNRS monogenik, terdapat dua jenis yaitu sindromik (dengan gejala ekstra-renal) dan non-sindromik (tanpa gejala ekstra-renal). Penanganan SNRS memerlukan pendekatan klinis yang berbeda tergantung pada etiologi genetiknya. Pemeriksaan genetik, termasuk gen tunggal, panel multigen, dan genomik komprehensif, dapat mengidentifikasi varian patogenik, menetapkan diagnosis yang akurat, menyesuaikan terapi (termasuk penghentian terapi imunosupresan dan pemberian terapi yang lebih spesifik) konseling genetik, serta penanganan komprehensif terhadap manifestasi ekstra-renal terkait. Oleh karena itu, pendekatan klinis yang efektif harus didasarkan pada hasil pemeriksaan genetik untuk pengelolaan yang optimal dan konseling yang lebih tepat.
Bone Mineral Disorders in Children with Predialysis Chronic Kidney Disease Correlates with Short Stature Rania, Ghina; Puspitasari, Henny Adriani; Hidayati, Eka Laksmi; Hafifah, Cut Nurul
Archives of Pediatric Gastroenterology, Hepatology, and Nutrition Vol. 3 No. 1 (2024): APGHN Vol. 3 No. 1 February 2024
Publisher : The Indonesian Society of Pediatric Gastroenterology, Hepatology, and Nutrition

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.58427/apghn.3.1.2024.12-23

Abstract

Background: The increasing prevalence of malnutrition and growth impairment among children with CKD could impact the prognosis and the preferred intervention. Therefore, this study aimed to identify the nutritional status of children with CKD and its relating factors. Method: A cross-sectional study was conducted in a tertiary, national-referral teaching hospital in Jakarta. Sociodemographic and laboratory data were obtained from medical records. Body weight and height of participants were measured using digital scale and stadiometer, respectively. Growth and nutritional status indicators such as BMI-for-age, weight-for-height, height for age, and weight for age were quantified and plotted using WHO Anthro and Anthroplus application. Result: A total of 18 participants aged 3-17 years old with CKD stage 3 – 5 were included in this study. BMI measurements showed a z-score average of -1.02, while the mean z-score for height-for-age was of -2.71. Our study demonstrated a significant association between the height-for-age and mineral bone in children with CKD (p = 0.005). However, we found no association between mineral bone disorder with other indicators of nutritional status. Furthermore, our study also found no significant relation between nutritional status and other influencing factors including the stage of CKD, duration of CKD, age, gender, primary etiological factor, hypertension, anemia, age, familial economic status, disease duration, and parental education level demonstrates no significant correlation (p > 0.05). Conclusion: Children with stage 3 – 5 CKD in the pre-dialysis phase are shown to be underweight and short statured but with normal nutritional status. Mineral bone disorder was revealed to be significantly associated with height-for-age in children with CKD.
Sindrom Nefrotik Idiopatik Sensitif Steroid pada Anak: Telaah Perbandingan Panduan Klinis Trihono, Partini Pudjiastuti; Fahlevi, Reza; Kinesya, Edwin; Hidayati, Eka Laksmi; Puspitasari, Henny Adriani; Pardede, Sudung Oloan
Sari Pediatri Vol 25, No 4 (2023)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/sp25.4.2023.231-42

Abstract

Latar belakang. Sindrom nefrotik idiopatik merupakan penyakit ginjal tersering pada anak di dunia. Penelitian terkait sindrom nefrotik idiopatik pada anak terus berkembang. Namun, pada praktiknya masih terdapat variasi yang lebar terkait evaluasi dan tata laksana sindrom nefrotik idiopatik pada anak di dunia. Tujuan. Membandingkan panduan klinis sindrom nefrotik idiopatik sensitif steroid pada anak. Metode. Membandingkan empat panduan klinis sindrom nefrotik idiopatik sensitif steroid pada anak, yaitu panduan klinis sindrom nefrotik idiopatik Ikatan Dokter Anak Indonesia tahun 2012, Kidney Disease Improving Global Outcome tahun 2021, International Pediatric Nephrology Association tahun 2022, dan Indian Society of Pediatric Nephrology tahun 2021. Dikembangkan 7 lingkup bahasan kajian, meliputi diagnosis, pemeriksaan penunjang awal, batasan kriteria, dan terapi sindrom nefrotik inisial, sindrom nefrotik relaps jarang, sindrom nefrotik relaps sering dan sindrom nefrotik dependen steroid.Hasil. Didapatkan beberapa perbedaan mendasar yang ditemukan, antara lain, terkait batasan proteinuria dan hipoalbuminemia yang digunakan, dosis maksimal steroid, definisi relaps sering, pilihan terapi imunosupresan pada SN relaps sering, dependen steroid, dan pemeriksaan genetik yang dirasonalisasikan berdasarkan bukti-bukti penelitian terbaru.Kesimpulan. Terdapat beberapa perbedaan mendasar antara panduan klinis sindrom nefrotik idiopatik Ikatan Dokter Anak Indonesia tahun 2012 dengan panduan klinis terbaru lainnya. Perlu dipertimbangkan pembaharuan konsensus sindrom nefrotik di Indonesia dengan menelaah bukti ilmiah terbaru dan disesuaikan dengan ketersediaan obat serta fasilitas pemeriksaan di Indonesia. 
Co-Authors Afifa Fahriyani Agus Firmansyah Alan R. Tumbelaka Anisa Rahmadhany Aulia Fitri Swity Bambang Supriyatno Beatrix Siregar Cahyani Gita Ambarsari Dewi Sukmawati Dewi Wulandari Dian Puspita Sari Dina Muktiarti, Dina Dion Darius Samsudin Edwin Kinesya Evita Kariani B. Ifran Hafifah, Cut Nurul Henny Adriani Puspitasari Henny Adriani Puspitasari Henny Adriani Puspitasari Henny Adriani Puspitasari, Henny Adriani Hikari Ambara Sjakti, Hikari Ambara Irene Yuniar, Irene Kinesya, Edwin Mulya Rahma Karyanti, Mulya Rahma Namira Metasyah Nathanne Septhiandi Novie Amelia Oke Rina Ramayani Pardede, Sudung Oloan Partini P. Trihono Partini P. Trihono Partini Pudjiastuti Trihono Partini Pudjiastuti Trihono Partini Pudjiastuti Trihono, Partini Pudjiastuti Partini Pudjiati Trihono Piprim B Yanuarso Pustika Amalia Wahidiyat, Pustika Amalia Rania, Ghina Reza Fahlevi Reza Fahlevi REZA FAHLEVI Rismala Dewi Rosalina Dewi Roeslani Sudung O Pardede, Sudung O Sudung O. Pardede Sudung Oloan Pardede Sudung Oloan Pardede Sudung Oloan Pardede Taralan Tambunan Teny Tjitra Sari Yaulia Yanrismet Yuliarti, Klara Zakiudin Munasir