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All Journal Paediatrica Indonesiana Journal of the Indonesian Medical Association : Majalah Kedokteran Indonesia The New Ropanasuri Journal of Surgery Indonesia Journal of Physical Medicine and Rehabilitation

Damayanti Sekarsari

Departemen Radiologi, Fakultas Kedokteran Universitas Indonesia, RSUPN Cipto Mangunkusumo, Jakarta

Author-ID : 3472766

Biochemistry, Genetics & Molecular Biology Decision Sciences, Operations Research & Management Education Health Professions Immunology & microbiology Medicine & Pharmacology Neuroscience Physics Public Health

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Hemostatic abnormalities in children with thalassemia major and liver iron overload Pustika Amalia Wahidiyat; Stephen Diah Iskandar; Novie Amelia Chozie; Damayanti Sekarsari
Paediatrica Indonesiana Vol 58 No 4 (2018): July 2018
Publisher : Indonesian Pediatric Society

Background Thalassemia major (TM) patients are susceptible to liver dysfunction due to iron deposition. Pediatric TM patients often present with bleeding. Blood loss necessitates transfusions, leading to increased iron absorption from the gut. Objective To study hemostatic abnormalities in children with TM and iron deposition in the liver. Methods This cross-sectional study involved 190 non-splenectomized children with TM. Liver iron deposition was evaluated using T2* MRI. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet counts were assessed from blood specimens. Results Most subjects were diagnosed with Î²-thalassemia and Î²-thalassemia/HbE. The majority of subjects were on deferiprone (DFP) treatment. Approximately 89.5% of subjects had liver iron overload. Prolongation of PT and aPTT, as well as thrombocytopenia were observed in 60%, 27.9%, and 19.5% of subjects, respectively. Prolonged aPTT and thrombocytopenia were observed three times more frequently in subjects with moderate - severe liver iron overload than in subjects with normal - mild liver iron overload (P=0.04 and 0.001, respectively). Conclusion Most TM subjects have liver iron overload ranging from mild to severe. Prothrombin time and prolongation, as well as aPTT prolongation, and thrombocytopenia are easily found in TM children. There were significantly more moderate - severe liver iron deposition patients with aPTT prolongation and thrombocytopenia than normal â€“ mild patients with these conditions. Hence, we suggest that pediatric TM patients undergo liver iron deposition evaluations and use iron chelators in an optimal manner, in order to limit the risk of bleeding.

Liver iron overload and hepatic function in children with thalassemia major Pustika Amalia Wahidiyat; Stephen Diah Iskandar; Ludi Dhyani Rahmartani; Damayanti Sekarsari
Paediatrica Indonesiana Vol 58 No 5 (2018): September 2018
Publisher : Indonesian Pediatric Society

Background Routine blood transfusions and increased intestinal iron absorption lead to iron accumulation in various organs, especially the liver. To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs. Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values. Methods This cross-sectional study was conducted in Cipto Mangunkusumo Hospital. The degree of liver iron overload was evaluated by T2*MRI. Assessments of serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and bilirubin levels were done to evaluate liver function. Results A total of 291 TM children were included in this study. The mean age of subjects was 12 years. Most of the subjects were diagnosed as Î²-thalassemia homozygote (54.6%) and Î²-thalassemia/HbE (41.2%). Deferiprone (DFP) was the most commonly used iron chelator. Less than 10% of the subjects had normal liver iron deposition. The AST and ALT values increased proportionally with the severity of liver iron overload, with significant, moderately negative correlation coefficients (r=-0.388 and -0.434, respectively). However, albumin level decreased proportionally with the severity of liver iron overload, with a significant, moderately positive correlation coefficient (r=0.323). Liver T2* MRI had no significant correlations with direct, indirect, and ratio of direct/total bilirubin levels. Conclusion Most of the children with TM have mild to severe liver iron overload. Liver T2* MRI had significant, moderate correlations with AST, ALT, and albumin values. Bilirubin level has no correlation with T2* MRI. Our findings suggest that monitoring of AST, ALT, and albumin levels is important because they may reflect the severity of liver iron overload. However, they should not be used as the only predictors of iron overload.

Short-term intermittent prophylaxis post-intracranial hemorrhage in children with hemophilia Novie Amelia Chozie; Fitri Primacakti; Made Citra Saraswati; Damayanti Sekarsari
Paediatrica Indonesiana Vol 62 No 3 (2022): May 2022
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi62.3.2022.174-9

Background Intracranial hemorrhage (ICH) is one of the major bleeding events causing mortality and long-term morbidity in children with hemophilia, especially those who receive on-demand therapy. Objective To evaluate the outcome of children with hemophilia after ICH receiving short-term intermittent prophylactic treatment. Methods This retrospective study was conducted in the Department of Child Health, Dr. Cipto Mangunkusumo Hospital, Jakarta. Children £18 years of age with hemophilia presenting with ICH between 2015-2020 were included. We recorded patients’ demographics, type and severity of hemophilia, the presence of factor VIII (FVIII) inhibitor, brain CT scan, treatment, and outcomes of these patients. Patients who received short-term intermittent prophylaxis using clotting factor concentrate (CFC) post-ICH episodes were observed for ICH recurrence. Results There were 19 episodes of ICH experienced by 18 patients, consisting of 16 patients with hemophilia A and 2 with hemophilia B. Patients’ median age was 4 years (range 0-16 years). Hemophilia was classified as severe in 13 patients, moderate in 4 patients, and mild in 1 patient. Thirteen episodes were preceded by head trauma. The most common clinical manifestation was seizures (13.2%). The most common type of ICH was subdural hematoma. Two patients died and 2 patients had neurological sequelae during hospitalization. The median dose of short-term intermittent prophylaxis using CFC (n=16) was 20 IU/kg of FVIII twice a week and 30 IU/kg of FIX twice a week, for a median duration of 8 weeks (range 5-12 weeks). One patient who did not adhere to the prophylaxis regimen had recurrent ICH at a similar location 6 months after the first episode. Conclusion Our findings suggest that short-term intermittent prophylaxis is important to prevent the recurrence of ICH in children with hemophilia.

Co-Authors Amalia W, Pustika Aryadi Kurniawan Aryadi Kurniawan E. Mudjaddid A. Siswanto Deddy N.W.Achadiono Hamzah Shatri Fitri Primacakti Iskandar Budianto Iskandar, Stephen Diah Joedo Prihartono Joedo Prihartono Joedo Prihartono Lindrawati Tjuatja Ludi Dhyani Rahmartani Made Citra Saraswati Mazni, Yarman Novie Amelia Chozie Pustika Amalia W Pustika Amalia Wahidiyat Pustika Amalia Wahidiyat, Pustika Amalia Putranto, Agi S Rumuat S.W Manangka S.Pd. M Kes I Ketut Sudiana . Septiyanti, Wita Stephen Diah Iskandar Stephen Diah Iskandar Syafina, Adinda B Wita Septiyanti Yefta Moenadjat

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