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All Journal Medical Journal of Indonesia Indonesian Journal of Cancer Sari Pediatri Paediatrica Indonesiana

Djajadiman Gatot

Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta

Author-ID : 268834

Health Professions Medicine & Pharmacology

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Peripheral blood and hemoglobin electrophoresis pattern in beta thalassemia major patients receiving repeated blood transfusion Wirawan, Riadi; Setiawan, Santy; Gatot, Djajadiman
Medical Journal of Indonesia Vol 13, No 1 (2004): January-March
Publisher : Faculty of Medicine Universitas Indonesia

One hundred and fifteen beta thalassemia major outpatients attending the Thalassemia Center Department of Child Health, Medical School University of Indonesia Dr. Cipto Mangunkusumo General Hospital for routine blood transfusion and hematology examination, participated in this study. There was a negative correlation between the size of the spleen and the peripheral blood parameters. All peripheral blood parameters tend to decrease with the enlargement of the spleen, and the condition is reversed after splenectomy. We observed that hypersplenism starts when the spleen is as big as S (V â VI). The hemoglobin electrophoresis pattern from beta thalassemia major patients receiving repeated blood transfusion did not show a dense HbF fraction, 90 patients showed a normal hemoglobin electrophoresis pattern. A hemoglobin analysis of both parents could be useful to confirm the diagnosis of beta thalassemia major for patients receiving repeated blood transfusion. In order to get a definite diagnosis, a genetic analysis by bio molecular technique is needed. (Med J Indones 2004; 13: 8-16)Â Keywords:Â Î² thalassemia major, hematology parameter, hemoglobin electrophoresis

Hospital based cancer registry in Cipto Mangunkusumo hospital Jakarta Sibuea, Wilfried H.; Mangunkusumo, R. R.; Akbar, Nurul; Widjanarko, Abidin; Gatot, Djajadiman; Windiastuti, Endang; Hamzah, Mochtar; Panigoro, Sonar S.; Prihartono, Joedo; Krisnuhono, Ening; Lisnawati, Lisnawati; Utami, Sri M.S.; Ramli, Irwan; Roezin, Averdi; Pribadi, Sigit; Wilarso, Iik; Nasar, I Made; Cornain, Santoso
Medical Journal of Indonesia Vol 9, No 3 (2000): July-September
Publisher : Faculty of Medicine Universitas Indonesia

[no abstract available]

Renal impairment in Î² thalassemia major patients receiving repeated blood transfusion Wirawan, Riadi; Kusnandar, Simon; Suherli, Abas; Gatot, Djajadiman
Medical Journal of Indonesia Vol 12, No 4 (2003): October-December
Publisher : Faculty of Medicine Universitas Indonesia

Î²-thalassemia major is a disease caused by Î² polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or noÂ Î²-globin chain synthesis. Treatment for Î²-thalassemia major patients is by giving repeated blood transfusions, which causes iron accumulation, leading to hemochromatosis. Iron accumulation can occur in various body organ, including the kidneys. The aim of this study was to investigate the existence of renal impairment in Î²-thalassemia major patients. The subjects of this study were Î²-thalassemia major patients aged 15 - 28 years old who had received 6 units of packed red cells or more within 6 months. In this study, urine and serum samples of the subjects were taken and examined. Assay of serum iron was performed with Hitachi 737. Results were that 94.7% patients showed an increase in transferrin saturation and 40% of them had hemochromatosis; 73.4% had microalbuminuria; 1.3% had albuminuria and 21.3% had increased urinary Î²2- microglobulin (Î²2-m). A total of 78.6% of patients showed renal impairment. Conclusion of this study suggested that glomerular dysfunction happens in an earlier stage of the disease process. The high incidence of microalbuminuria is also attributed to defective ability of the proximal tubular cells to reabsorb protein besides dysfunction of the glomeruli. (Med J Indones 2003; 12: 215-223)Keywords: Î²-thalasemia major, repeated blood transfusion, renal impairment

Steroid response as prognostic factor and its correlation with molecular assessment of childhood acute lymphoblastic leukemia Andriastuti, Murti; Gatot, Djajadiman; Wirawan, Riadi; Setiabudy, Rianto; Mansyur, Muchtaruddin; Ugrasena, I Dewa G.
Medical Journal of Indonesia Vol 24, No 4 (2015): December
Publisher : Faculty of Medicine Universitas Indonesia

Background: Survival rate of children with acute lymphoblastic leukemia (ALL) in Indonesia remains low. Risk stratification accuracy is important to improve survival. In developed countries, risk stratification is determined based on gene fusion that is known related to steroid resistency. Steroid response at day-8 correlates with prognosis. The assessment can be applied in centers that cannot perform molecular assessment. This study aims to evaluate whether steroid response correlated to molecular assessment. Methods: A cross-sectional study was performed at Child Health Department, Cipto Mangunkusumo Hospital (January 2013-March 2014), a total of 73 patients were enrolled. Steroid was given for 7 days. Peripheral blast count at day 8 was evaluated, good response if blast count <1000 /µL and poor if ≥1000 /µL. Fusion gene detection was also performed. The data was analysed using Statistical Package for Social Sciences (SPSS) version 20.0.Results: Fusion gene was detected in 45 patients. In 1–10 years age group, 26/32 (81%) subjects had good response, while 75% in <1 year age group and 7/9 (78%) in ≥10 years age group had poor response. 5/7 (71%) subjetcs had leukocyte count >100,000 /µL and 7/8 (88%) with T-cell showed poor response. Age, leukocyte count, and T-cell were statistically correlated with steroid response (p<0.05). E2A-PBX1 fusion gene was the most common 19/45 (42%), followed by TEL-AML1 17/45 (38%), BCR-ABL 5/45 (17%), and MLL-AF4 1/45 (3%). Four of five subjects (80%) with BCR-ABL and one subject with MLL-AF4 had poor steroid response. On the other hand, 12/19 (63%) with E2A-PBX1 and 13/17 (77%) with TEL-AML1 had good response. There was no correlation between steroid response and molecular assessment.Conclusion: Steroid response correlates with age, leukocyte count, and T-cell but not with molecular assessment.

Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center Sari, Teny T.; Chozie, Novie A.; Gatot, Djajadiman; Tulaar, Angela B.M.; Dharma, Rahayuningsih; Sukrisman, Lugyanti; Bardosono, Saptawati; Achmad, Harry R.; Prasetyo, Marcel
Medical Journal of Indonesia Vol 26, No 1 (2017): March
Publisher : Faculty of Medicine Universitas Indonesia

Background: Recurrent joint bleeds leading to arthropathy is the main problem in severe hemophilia children. This study aimed to investigate joint status in severe hemophilia A children receiving episodic treatment in Cipto Mangunkusumo Hospital, Jakarta.Methods: A cross-sectional study was conducted in Cipto Mangunkusumo Hospital as Indonesian National Hemophilia Treatment Center on children (4–18 years) with severe hemophilia A, who previously received episodic treatment, with no history of inhibitor factor VIII. Hemophilia Joint Health Score was evaluated according to HJHS version 2.1 2011. Joint ultrasonography was done for six index joints (bilateral elbows, knees and ankles) using Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) methods. Data of age of first joint bleed, number of target joints and inhibitor factor VIII were obtained from the Pediatric Hemophilia Registry and medical records.Results: There were 59 subjects aged 4 to 18 years. Twenty-nine out of 59 (49.2%) subjects experienced first joint bleed before of 2 years of age. The most common of joint bleeds was a right ankle. Mean total HJHS was 8.71±8.73. Subjects aged 4–10 years showed lower HJHS (4.6±3.7) as compared to subjects aged >10–18 years (12.3±10.3), p<0.001; 95% CI=4.9–13. Mean HEAD-US scores in subjects aged 4–10 years (18.7±5.6) was lower than in subjects aged >10–18 years (28±7.9), p<0.001, 95% CI= -12.9–-5.6.Conclusion: HJHS and HEAD-US scores of severe hemophilia A children receiving episodic treatment aged 4–10 years are lower compared to subjects aged >10–18 years, indicating more severe joint destruction in older children and progressivity of joint damage over time. It is important to start prophylactic treatment to prevent progressivity of joint damage.

Co-Authors Abas Suherli Abidin Widjanarko Abigail, Dina Clarisa Rumora Achmad, Harry R. Alida R Harahap, Alida R Anak Agung Istri Sri Wiadnyani Andri Maruli T Lubis, Andri Maruli T Angela BM Tulaar anky tri rini, anky tri Arwin A.P. Akib, Arwin A.P. Arwin AP Akib, Arwin AP Atmakusuma, Tubagus Djumhana Averdi Roezin Chozie, Novie A. Dharma, Rahayuningsih Dwirestuti, Ratna Endang Windiastuti Ening Krisnuhono Fitri Primacakti, Fitri Gutama, Gutama Harahap, Alida Roswita I Dewa Gede Ugrasena I Made Nasar Iik Wilarso Irwan Ramli Joedo Prihartono Kurniadi Husodo, Kurniadi Kusnandi Rusmil Kusumaning Edhy, Kusumaning Lugyanti Sukrisman Marcel Prasetyo Mochtar Hamzah Muchtaruddin Mansyur Murti Andriastuti, Murti Novie Amelia Chozie Nurul Akbar Padmonodewo, Suminarti Ponpon S Idjradinata R. R. Mangunkusumo Riadi Wirawan Rianto Setiabudy Santoso Cornain Santy Setiawan Saptawati Bardosono Sari, Teny T. Sarita, Raisa Cecilia Sarwono Waspadji Sigit Pribadi Simon Kusnandar Sitorus, Rita Soedjatmiko Soedjatmiko Sonar S. Panigoro Sri M.S. Utami Sri R.S. Hadinegoro Sri Rezeki S Hadinegoro, Sri Rezeki S Teny T Sari1, Teny T Teny Tjitra Sari, Teny Tjitra Wilfried H. Sibuea Yanto Ciputra, Yanto

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