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

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Peripheral blood and hemoglobin electrophoresis pattern in beta thalassemia major patients receiving repeated blood transfusion

Medical Journal of Indonesia Vol 13, No 1 (2004): January-March
Publisher : Faculty of Medicine Universitas Indonesia

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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 – 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) Keywords: β thalassemia major, hematology parameter, hemoglobin electrophoresis

Hospital based cancer registry in Cipto Mangunkusumo hospital Jakarta

Medical Journal of Indonesia Vol 9, No 3 (2000): July-September
Publisher : Faculty of Medicine Universitas Indonesia

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[no abstract available]

Renal impairment in β thalassemia major patients receiving repeated blood transfusion

Medical Journal of Indonesia Vol 12, No 4 (2003): October-December
Publisher : Faculty of Medicine Universitas Indonesia

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Abstract

β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited in an autosomal recessive manner from both parents and which is marked by little or no β-globin chain synthesis. Treatment for β-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 β-thalassemia major patients. The subjects of this study were β-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 β2- microglobulin (β2-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: β-thalasemia major, repeated blood transfusion, renal impairment

Coagulation abnormality as a complication of L-asparaginase therapy in childhood lymphoblastic leukemia

Paediatrica Indonesiana Vol 46 No 1 (2006): January 2006
Publisher : Indonesian Pediatric Society

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Abstract

Background Bleeding, one of the most common symptoms ofacute leukemia in untreated patients, is mostly due to thrombocy-topenia as a result of myeloinvasion by leukemic cells. Neverthe-less, a further contributory factor for the additional hemorrhagiccomplication during intensive chemotherapy is the myelosuppressiveeffect of most active drugs. L-asparaginase, one of the cytostaticsused during remission induction therapy for childhood of acutelymphoblastic leukemia (ALL), is widely reported to impair the he-mostatic system.Objective To determine the influence of shorter courses of L-as-paraginase (L-Ase) on some of the hemostatic parameters in thetreatment of childhood ALL.Methods A prospective analytical study was carried out in theDepartment of Child Health, Cipto Mangunkusumo Hospital Jakartafrom July 1, 1999 to June 30, 2001 on newly diagnosed ALL pa-tients with normal liver function tests treated according to our na-tional ALL protocol which one of its composition contained 6 in-stead of 9 injections of L-asparaginase.Results All of 30 children with ALL included in the study, experi-enced prolongation of prothrombin time (PT), activated partialthromboplastin time (aPTT), and decreased fibrinogen concentra-tion, markedly during the administration of L-asparaginase. How-ever, none of the patients had additional hemorrhage or evidenceof disseminated intravascular coagulation (DIC).Conclusion The use of shorter courses of L-asparaginase, 6 in-jections, in the remission induction chemotherapy of childhood ALLin our department may reduce the blood clotting factors withoutfurther hemorrhage complication or evidence of DIC

Rabdomiosarkoma Pada Anak: Gambaran Klinis di 2 Institusi

Indonesian Journal of Cancer Vol 2, No 2 (2008): Apr - Jun 2008
Publisher : "Dharmais" Cancer Center Hospital

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Abstract

Rabdomiosarkoma (RMS) merupakan keganasan jaringan lunak yang banyak terjadi pada anak umur 1 sampai 5 tahun dan remaja. Sekitar 15 % anak dengan RMS datang dalam keadaan metastasis dan prognosisnya tidak ada perbaikan dalam 15 tahun terakhir.Penelitian ini untuk mengetahui keluaran terapi serta pengembangan selanjutnya Penelitian dilakukan secara retrospektif dari data pada catatan medis 30 pasien yang diterapi di RS Cipto Mangunkusumo (RSCM), Jakarta bulan Juni 2000 sampai Juli 2006 dan 11 pasien di RS Kanker Dharmais (RSKD), Jakarta bulan Januari 2003 sampai Desember 2007. Data dikumpulkan untuk melihat gambaran klinis dan keluaran terapi.Terdapat 30 pasien RMS di RSCM tahun 2000-2006 dan 11 di RSKD tahun 2003-2007. Perbandingan antara laki-laki dan perempuan adalah 2:1 (RSCM) dan 1:1,7 (RSKD).Kebanyakan menyerang anak umur antara 1 bulan sampai 5 tahun (43,3 % & 43,5 %) dengan median antara 6-7 tahun. Gambaran patologi terbanyak yaitu embrional (70% & 54,5%). Lokasi primer terbanyak pada bagian kepala dan leher (43,3% & 27,3%). Didapatkan stadium lanjut sebesar 80% & 45,5%. Sebagian besar metastasis ditemukan pada sumsum tulang (85,7% & 33,3%). Keluaran terapi didapatkan yang masih dalam terapi 66,7% & 27,3%, meninggal 30% & 18,2% dan lost to folloiv up 3,3% & 54,5%.RMS kebanyakan didapatkan pada anak umur 1 bulan sampai 5 tahun. Gambaran histologi tipe embrional dan lokasi primer pada kepala dan leher merupakan yang tersering. Pada umumnya, pasien datang dalam stadium lanjut tetapi jika datang pada stadium awal akan memberikan hasil yang lebih baik.Kata kunci: Rabdomiosarkoma, epidemiologi klinik, keluaran terapi.

Rabdomiosarkoma pada Anak: Luaran Klinis pada Pasien yang Mendapat Terapi

Indonesian Journal of Cancer Vol 5, No 2 (2011): Apr - Jun 2011
Publisher : "Dharmais" Cancer Center Hospital

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Abstract

Latar belakang. Rabdomiosarkoma (RMS) merupakan keganasan jaringan lunak yang banyak terjadi pada anak umur 1 sampai 5 tahun dan remaja. Sekitar 15 % anak dengan RMS datang dalam keadaan metastasis dan prognosisnya tidak ada perbaikan dalam 15 tahun terakhir.Tujuan. Penelitian ini untuk mengetahui gambaran epidemiologi RMS, hasil penanganannya dan luaran klinis untuk pengembangan selanjutnya.Metode. Penelitian dilakukan secara retrospektif dari data pada catatan medis 44 pasien yang diterapi di RS Cipto Mangunkusumo (RSCM), Jakarta bulan Juni 2000 sampai Juli 2008. Data dikumpulkan untuk melihat gambaran epidemiologi klinik dan luaran klinis.Hasil. Terdapat 44 pasien RMS di RSCM selama tahun 2000-2008.. Perbandingan antara laki-laki dan perempuan adalah 2:1 .Kebanyakan menyerang anak umur antara 3 bulan sampai 5 tahun (47,7% ) dengan median antara 6 -7 tahun. Gambaran patologi terbanyak yaitu embrional (65,9% ). Lokasi primer terbanyak pada bagian kepala dan leher (47,7% ). Berdasarkan sistem TNM , didapatkan stadium lanjut sebesar 61,4% . Sebagian besar metastasis ditemukan pada sumsum tulang (74%). Hasil luaran klinis didapatkan yang masih dalam terapi 52,3% , meninggal 36,4% dan lost to follow up 11,3% .Kesimpulan. RMS kebanyakan didapatkan pada anak umur 3 bulan sampai 5 tahun. Gambaran histologi tipe embrional dan lokasi primer pada kepala dan leher merupakan yang tersering. Pada umumnya, pasien datang dalam stadium lanjut tetapi jika datang pada stadium awal akan memberikan hasil yang lebih baik.Kata kunci Rabdomiosarkoma, epidemiologi klinik, luaran klinis

Serological profile and hemolytic disease in term neonates with ABO incompatibility

Paediatrica Indonesiana Vol 49 No 4 (2009): July 2009
Publisher : Indonesian Pediatric Society

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Abstract

Background Hemolytic disease of the newborn (HDN) due toABO blood type incompatibility is one of the most commoncause of neonatal hyperbilirubunemia that potentially leads tobilirubin encephalopathy. Data on ABO-hemolytic disease of thenewborn (ABO-HDN), especially regarding umbilical cord bloodserological profile, are limited.Objective To identify the serological profile and hemolytic disease in term neonates with ABO incompatibility.Methods This was a cross-sectional descriptive study, conductedat RSIA Budi Kemuliaan Jakarta.Results We found 68 healthy term neonates with ABOincompatibility, nine of them had positive direct antiglobulintest (OAT) result, and 38 subjects had a positive result onOAT with elution method. The highest titer of IgG was 1:8.Hyperbilirubinemia was found in 30 ( 44%) subjects, andABO-HDN was diagnosed in 28 (41 %) subjects. Within thepositive OAT group, eight out of nine subjects had sufferedfrom hyperbilirubinemia and ABO-HDN. Meanwhile, withinthe positive OAT with elution method group; 24 subjects hadsuffered from hyperbilirubinemia with 23 of them having ABOHDN. Based on the chi-square analysis; those with positive OAT with elution method had 3.2 times higher risk of suffering from ABO-HDN. Furthermore, there was a 3.6 times higher risk ofsufferring from hyperbilirubinemia.Conclusion In healthy term neonates with ABO incompatibility,the incidence of ABO-HDN is 41%. OAT serological examinationwith elution method is better than OAT in assessing riskfor hyperbilirubinemia and ABO-HDN.

Steroid response as prognostic factor and its correlation with molecular assessment of childhood acute lymphoblastic leukemia

Medical Journal of Indonesia Vol 24, No 4 (2015): December
Publisher : Faculty of Medicine Universitas Indonesia

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Abstract

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.

Ewing's Sarcoma Family Tumors pada ANak [Keganasan Kelompok Sarkoma Ewing] di RS Cipto Mangunkusumo

Sari Pediatri Vol 13, No 2 (2011)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

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Abstract

Latar belakang.Terapi multimodalitas pada Ewing’s sarcoma family tumors(keganasan kelompok sarkoma Ewing) telah banyak meningkatkan keberhasilan terapi. Guna menilai keberhasilan terapi diperlukan data mengenai luaran pasien keganasan kelompok sarkoma Ewing.Tujuan.Mengetahui gambaran klinis dan luaran pasien sarkoma Ewing yang dirawat di Divisi Hematologi Onkologi Departemen Ilmu Kesehatan Anak RS Cipto Mangunkusumo Metode.Studi deskriptif dilakukan pada pasien yang didiagnosis keganasan kelompok sarkoma Ewing di Divisi Hematologi Onkologi Departemen Ilmu Kesehatan Anak RS Cipto Mangunkusumo, pada tahun 2000–2010. Terapi yang diberikan adalah kemoterapi, pembedahan, dan radioterapi. Hasil. Selama periode pengamatan sepuluh tahun dijumpai 26 pasien keganasan kelompok sarkoma Ewing dari seluruh 2112 pasien keganasan anak. Usia berkisar 6 bulan – 13 tahun 1 bulan (median 8 tahun 3 bulan). Lokasi tersering adalah ekstremitas, tulang belakang, dan pelvis. Sebagian besar pasien (16 dari 26 pasien) datang dengan stadium lanjut. Kemoterapi terutama diberikan pada lokasi tumor aksial (12 dari 26 pasien), sedangkan pembedahan yang dilanjutkan kemoterapi dilakukan bila lokasi tumor berada di ekstremitas (4 dari 26 pasien). Pasien meninggal lebih banyak dengan lokasi tumor di aksial (9 pasien) dibanding ekstremitas (3 pasien). Jumlah pasien hidup lebih banyak yang berusia < 10 tahun dibanding umur yang lebih tua (6 berbanding 1). Residif terjadi pada dua pasien dengan jangka waktu 11 bulan.Kesimpulan. Luaran pasien keganasan kelompok sarkoma Ewing masih jauh dari memuaskan. Pasien meninggal lebih banyak daripada pasien hidup, terutama letak tumor di aksial. Sebagian besar pasien datang pada stadium lanjut dan telah mengalami metastasis. Modalitas yang lebih intensif perlu diberikan untuk meningkatkan luaran pasien keganasan kelompok sarkoma Ewing.

Perdarahan Intrakranial pada Hemofilia: Karakteristik, Tata Laksana, dan Luaran

Sari Pediatri Vol 13, No 4 (2011)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

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Abstract

Latar belakang. Perdarahan intrakranial merupakan salah satu penyebab mortalitas tertinggi pada hemofilia dan morbiditas berupa gangguan neurologis. Tujuan.Mengetahui gambaran klinis, tata laksana dan luaran pasien hemofilia anak yang mengalami perdarahan intrakranial di Departemen Ilmu Kesehatan Anak RS Dr. Cipto Mangunkusumo, Jakarta selama periode 2007-2010.Metode. Studi retrospektif pasien hemofilia berusia >1 bulan hingga 18 tahun yang dirawat dengan diagnosis perdarahan intrakranial di Departemen Ilmu Kesehatan Anak RSCM dalam kurun waktu 1 Januari 2007 – 31 Desember 2010. Data dikumpulkan dari Registrasi Hemofilia Divisi Hematologi-Onkologi dan rekam medik Departemen Ilmu Kesehatan Anak RSCM, Jakarta.Hasil. Selama kurun waktu penelitian, dari 154 pasien hemofilia anak (usia <18 tahun) yang terdaftar di Registrasi Hemofilia Divisi Hematologi-Onkologi Departemen Ilmu Kesehatan Anak RSCM, terdapat 13 episode perdarahan intrakranial yang dialami oleh 11 pasien (7,1%). Pasien adalah hemofilia A dengan median usia 6 tahun (3 - 15 tahun). Delapan dari 13 episode perdarahan intrakranial didahului oleh trauma kepala. Manifestasi klinis tersering adalah nyeri kepala (7), muntah (6), kejang (4), penurunan kesadaran (3), iritabilitas (2), paresis motorik (2), paresis saraf kranial (2) dan vulnus laceratumdi kepala (1). Perdarahan terbanyak adalah pada lokasi subdural (7). Median durasi pemberian faktor VIII adalah 10,5 hari (7-16 hari). Sembilan pasien membaik tanpa komplikasi, satu pasien mengalami epilepsi dan satu pasien meninggal dunia.Kesimpulan. Angka kejadian perdarahan intrakranial pada hemofilia di RSCM 7,1%. Ketersediaan faktor pembekuan untuk replacement therapydan kerjasama tim multidisiplin sangat penting untuk memperbaiki luaran pasien hemofilia yang mengalami perdarahan intrakranial.

Co-Authors A Harahap, A Abas Suherli Abidin Widjanarko Achmad, Harry R. Alida R Harahap, Alida R Aman Bakti Pulungan, Aman Bakti Andri Maruli T Lubis, Andri Maruli T Angela B.M. Tulaar anky tri rini, anky tri Arwin A.P. Akib, Arwin A.P. Arwin AP Akib, Arwin AP Aryono Hendarto, Aryono Averdi Roezin Badriul Hegar Bambang Madiyono Bambang Supriyatno Bambang Tridjaja, Bambang Chozie, Novie A. Darlan Darwis, Darlan Desiana Dharmayani, Desiana Dharma, Rahayuningsih Elizabeth Yohmi, Elizabeth Ellya Marliah, Ellya Endang Windiastuti Ening Krisnuhono Gita Widyapuri, Gita Hanifah Oswari Harapan Parlindungan Ringoringo, Harapan Parlindungan Harijadi Harijadi Heru Noviat Herdata, Heru Noviat Hikari Ambara Sjakti, Hikari Ambara I Dewa G. Ugrasena, I Dewa G. I Made Nasar I Setianingsih, I Iik Wilarso Irwan Ramli Iswari Setianingsih Joedo Prihartono Julfina Bisanto Ketut Ariawati Kirana Kamima, Kirana Kurniadi Husodo, Kurniadi Kusumaning Edhy, Kusumaning Lisnawati Lisnawati lsmael, Sofyan Lugyanti Sukrisman Luszy Arijanty, Luszy Mangunatmadja, lrawan Marcel Prasetyo Mardjanis Said, Mardjanis Marzuki, Nanis Sacharis Melissa Gandi, Melissa Mochtar Hamzah Muchtaruddin Mansyur MUHAMMAD ALI Mulya Rahma Karyanti, Mulya Rahma Mulyadi M Djer, Mulyadi M Murti Andriastuti, Murti N S Marzuki, N S Najib Advani Nia Kurniati Nila Kusumasari Novie Amelia C, Novie Amelia Novie Amelia Chozie, Novie Amelia Nurul Akbar Partini Pudjiastuti T, Partini Pudjiastuti Ponpon S Idjradinata Pramita Gayatri, Pramita Pringgardini, Keumala Pustika Amalia Wahidiyat, Pustika Amalia R A Taufani, R A R. Dina Garniasih, R. Dina R. R. Mangunkusumo Renno Hidayat, Renno Riadi Wirawan Rianto Setiabudy Rinawati Rohsiswatmo Ringoringo HP, Ringoringo Rulina Suradi, Rulina S.P., Ferry Damardjati Santoso Cornain Santy Setiawan Saptawati Bardosono Sari, Teny T. Selvi Nafianti, Selvi Setyo Handryastuti, Setyo Shirley L. Anggriawan Sigit Pribadi Simon Kusnandar Sonar S. Panigoro Sri M.S. Utami Sri R.S. Hadinegoro Sri Rezeki S. Hadinegoro, Sri Rezeki S. Sri Sudaryati Nasar, Sri Sudaryati Sudigdo Sastroasmoro Sukman Tulus Putra, Sukman Tulus Tenny Tjitrasari, Tenny Teny T Sari1, Teny T Teny Tjitra Sari, Teny Tjitra Teny Tjitrasari, Teny Tumpal Y Sihombing, Tumpal Y W, Pustika Amalia Wilfried H. Sibuea Yanto Ciputra, Yanto Yessi Yuniarti, Yessi Yovita Ananta, Yovita Zakiudin Munasir