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Dian Puspita Sari
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Journal : Paediatrica Indonesiana

 1 
Hepcidin levels, markers of iron overload, and liver damage in children with beta-thalassemia major Sari, Indah; Sari, Dian Puspita; Damayanti, Moretta; Salwan, Hasri
Paediatrica Indonesiana Vol. 64 No. 5 (2024): September 2024
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi64.5.2024.412-8

Abstract

Background Thalassemia is a hemoglobin synthesis disorder that causes patients to need lifelong blood transfusions, leading to iron overload and alter organ function, including the liver. Hepcidin, produced by the liver, plays a role in iron homeostasis and should be increased in excess iron stores. However, the level decreases in thalassemia due to some factors, such as ineffective erythropoiesis and liver damage. Recent publications revealed that hepcidin could be associated with iron overload and also a marker of liver diseases. Objective To analyse the correlation between hepcidin level, markers of iron overload, and liver damage in beta-thalassemia major. Methods This cross-sectional study included all ?-thalassemia major age 2-18 years admitted to Dr. Mohammad Hoesin Hospital, Palembang, South Sumatera, who underwent blood transfusions from March to August 2022. We measured the level of iron overload markers, hepcidin, liver function test (LFT), and performed liver ultrasonography (USG). Results Of 97 subjects, median hepcidin level was 10.01 ng/mL and 68% of the subjects showed a decrease. The iron overload parameters were evaluated from serum iron levels (P=0.13), ferritin levels (P=0.90), and transferrin saturation (P=0.29) and 24.7% had abnormal liver USG findings. Spearman’s correlation revealed that only direct bilirubin (DB) (r=0.35; P=0.001) and liver USG (r=0.20; P=0.05) had positive correlations with decreased levels of hepcidin. Also, it had 91.7% sensitivity in predicting liver damage from ultrasound. Conclusion The hepcidin level was not significantly associated with iron overload markers.
Kidney dysfunction in children with thalassemia Lestari, Hertanti Indah; Rahmawati, Eka; Ayu, Dewi Rosariah; Fitriana, Eka Intan; Sari, Dian Puspita
Paediatrica Indonesiana Vol. 65 No. 4 (2025): July 2025
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi65.4.2025.337-45

Abstract

Background Children with thalassemia are at risk for kidney dysfunction due to chronic anemia, frequent blood transfusions, iron retention, and use of iron chelating agents. Cystatin C, an endogenous marker for assessing estimated glomerular filtration rate (eGFR), a novel biomarker for the early detection of kidney failure, has been reported to be potentially superior to the commonly used serum creatinine. Objective To evaluate various creatinine- and cystatin C-based formulas for eGFR to detect kidney dysfunction in children with thalassemia. Methods This was a cross-sectional study on children (age <18 years) with thalassemia. Kidney dysfunction was defined as eGFR <90 mL/minute/1.73 m2. Hyperfiltration was defined as eGFR >150 ml/minute/1.73 m2. This study compared the proportion of kidney dysfunction as determined using various creatinine- and cystatin C-based eGFR formulas, comprising the creatinine-based Schwartz formula, the cystatin C-based Filler formula, the creatinine-cystatin C-based New CKID formula, and the creatinine-cystatin C-based Schwartz formula. Results The median age of the 152 study subjects was 11.0 (range 2.0-18.0) years. When using the creatinine-based Schwartz formula, none of the subjects had kidney dysfunction. Kidney dysfunction was found in 21.7% of subjects when using the cystatin C-based Filler formula, in 26.3% of subjects when using the creatinine-cystatin C-based (New CKID) formula, and 59.9% of subjects using the creatinine-cystatin C-based Schwartz formula. When using the creatinine-based Schwartz formula, 38.2% subjects experienced hyperfiltration no hyperfiltration was found by when using other eGFR formulas. There was low correlation between creatinine and cystatin C (r=0.195; P=0.016). There was only mild agreement in eGFR between the creatinine-based Schwartz formula and the cystatin C-based Filler formula (k=0.195; P<0.001). Conclusion The proportion of kidney dysfunction in children with thalassemia based on eGFR calculation using cystatin C- and creatinine-cystatin C-based formulas ranged from 21.7% to  59.9%. No kidney dysfunction was found using a creatinine-only-based eGFR formula, whereas hyperfiltration was a common finding. Hence, more than one parameter should be considered for early detection of kidney dysfunction in thalassemia.
Co-Authors A.A. Ketut Agung Cahyawan W Abdiman, I Made Tobias Achirul Bakri, Achirul Afrizal, Sandra Agustini, Nur Indah Al Farisi Sutrisno, Muhammad Ali Murtopo Anak Agung Ayu Niti Wedayani Anjani, Mutiara Anugrah Ricky Wijaya Aprilia, Kartika Ardinia, Gusti Ayu Eka Radha Asmaul, Rina Ayu, Dewi Rosalia Baiti, Isma Nur Bangun, Primta Basuki Rahmat Masdi Siduppa Budi Muhammad Taftazani, Budi Muhammad Candra Eka Puspitasari Christian, Samuel Eza Dadang Sunendar Damayanti, Moretta Dedianto Dedianto, Dedianto Dedianto Hidajat Dewanti, Lintang Purwara Dewi Rosariah Ayu Dewi Suryani Dewi Yuliani, Dewi Dian Permata Sari Didik Wahyudi Dilaga, Marisa Syavitri Eka Intan Fitriana eka rahmawati Erial Bahar, Erial Estiyawati, Estiyawati Fadli, Muhammad Fajarianti, Yuniar Fitriyah, Sabilatul Fuad Fitriawan, Fuad Hafani, Moch Hasan Hamidah . Harahap, Alvin Abrar Hasri Salwan, Hasri Hertanti Indah Lestari, Hertanti Indah Hidayaturrahman, Taufik Hindra Hastuti, Nurnawati Husada, Abdillah Hy, Mc. Fulvia Ida Ayu Eka Widiastuti Iis Sopiawati Indrayana, Yanna Indrayana, Yanna Irma Santika Putri Jayakusuma, Muhamad Zaidan Jayawarsa, A.A. Ketut Josafat, Anom Kaimuddin, La Ode kinasih, Hanna ajeng Kusumaningrum, Irma Kartika Laras Sitoayu Larassati, Kartika Putri Lutfi Fanani M. Furqon Wahyudi Maghfiroh, Luluk Maharani, Chariztya Anggita Mahardika, Agustine Malihah, iis Ismiati Manafe, Winda Maringka, Kezia Michella Yusak Maserona, Lukas Masruroh MASRUROH Medha, Ied Andro Meiliana, Yutta Lilin Sabad Meilisa, Riska Metta Octora Mira Wahyuni, Mira Misbahuddin, Agus Ridwan Moraida Hasanah nabilla, Umniya Nadjadji Anwar Novi Darmayanti Noviyani, Ni Made Reditya Noviyanti, Thjiong Angelina Nurliana Cipta Apsari Patandianan, Firdaus Kamma Peby Maulina Lestari, Peby Maulina Peginusa, Stefani Switly Pintaningrum, Yusra Pramesti, Renita Priyono Priyono Putra, Chani Sinaro Putri, Baiq Ayu Wulan Maharani Rahman, Mohammad Haris Taufiqur Ramadhan, Muhammad Rizzqi Ramadhani, Dina Ramzi Amin, Ramzi Retno Anggraini Rika Mutiara, Rika Rina Lestari Rio Eka Nugraha, Rio Eka Romi Ermawan, Romi Rosati, Intania RR. Ella Evrita Hestiandari Rugayyah Salsabila, Arini Saputra Siregar, Damara Saputra, Dedech Muhammad Sari, Putu Suwita Sari Selfiana, Evi Setia, Devit Sidharti, Theresia Sri Stevanny, Bella Suharni, Sitti Sulistiawati Sulistiawati Sumitra, Putri Azahra Supit, Teve W. M. Surjani Wonorahardjo Suryanti, Dewi Suyanto Suyanto Syarifuddin, Muhammad Afif Syifah, Fuatis Tajudin, Ahmad Theodorus Umi Mardiyati Wibowo, Yusep Windhu Ari Wijaya, Christabella Natalia Windriani, Dian Yoga Pamungkas Susani Z, Fynnisa Zahar, Intan Ziske Maritska Zulfaizah, Ovi Riani