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Journal : Paediatrica Indonesiana

Variable Severity of B-Thalassemia/Hemoglobin E Disease - the Genetic Factors Sunarto, Sunarto
Paediatrica Indonesiana Vol 37, No 1-2 (1997): January-February 1997
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi37.1-2.1997.6-12

Abstract

Hemoglobin E (Hb E) is prevalent in Southeast Asia. Heterozygotes give no manifestation, even homozygotes show no manifestation or only slight anemia. However, compound heterozygote with 6-thalassemia gives anemia with variable severity. The severely affected individuals show anemia similar to homozygous 6-thalassemia. Many factors play a role in determining the severity. SThnl/6E patients have increased superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) activities. The increased GSH-Px is thought to be needed for the elimination of hydrogen peroxide produced by SOD decomposition of peroxide. The content of antioxidants vitamine E and C is reduced, whereas MDA, the final product of lipid peroxidation increases significantly. The genotype of the Xmn 1 polymorphism, -158 bases upstream from the transcription site of the n-globin and the level of Hb F are associated with clinical severity, but the extent of the S -globin mRNA cryptic splicing is more associated with the severity of tire manifestation than does the pattern of the Xmn I polymorphism.
Pericarditis and Pleuritis Caused by Extramedullary Plasmacytoma Wahab, A. Samik; Sunarto, Sunarto; Utomo, Utomo
Paediatrica Indonesiana Vol 30 No 11-12 (1990): November 1990
Publisher : Indonesian Pediatric Society

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Abstract

The following illustrates a case study of a 9 years-old girl with combined pericarditis and pleuritis caused by solitary extramedullary plasmacytoma. Pericardiocentesis and permanent thoracocentesis were performed, both yielded serohemorrhagic and serous fluid in succession. In the beginning etiological diagnosis was made on the basis of the clinical pattern for tuberculosis infection and growth of three species of bacteria for bacterial infections and candida species for candidiasis. The initial treatment was in accordance with the etiological diagnosis mentioned above. The final diagnosis was establish in the fourth month after the discovery of plasmacytoma in the pleural fluid and CT scan examination disclosing masses in the right lung. Accordingly, cytostatic therapy was started. The result of therapy was very good, exudation into the pleural and pericardia/ sac regressed gradually and eventually ceased completely. According to the literature the prognosis of these neop/asma is good.  
Takayasu s Disease Wahab, A. Samik; Sunarto, Sunarto; Soebardi, Aris; Harlistyanti, Ryna
Paediatrica Indonesiana Vol 30 No 11-12 (1990): November 1990
Publisher : Indonesian Pediatric Society

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Abstract

A fourteen years old girl developed Takayasu s arteritis (pulseless disease) since six months prior to investigation. This unusual form of arteritis is common in Japan and Korea but has rarely been reported in individuals born in the United States. In Indonesian literatu;e it has never been documented so far. The etiology is unclear. The literature currently but forward the hypothesis of an autoimmune basis and treatment with steroid. Although a tuberculin sensitization pathogenesis has been suggested, a close temporal relationship with the onset of a tuberculous process has not previously been documented. The likelihood of uncovering tuberculin sensitivity or active tuberculosis in patients with Takayasu, arteritis is substantially higher than in the general population in all countries analyzed. The natural history of his arteritis is highly variable. The adolescent described in this paper has demonstrated no response either  to antituberculosis therapy, or to heparin and corticosteroid as suggested by Ishikawa, 1987. The patient died on the 59th day of hospitalization after getting syncopal attacks followed by shock.
Translocation ETS leukemia-acute myeloid leukemia 1 (TEL-AML1) gene fusion in childhood acute lymphoblastic leukemia Mulatsih, Sri; Liang, Yeow; Yeoh, Allen; Sutaryo, Sutaryo; Sunarto, Sunarto
Paediatrica Indonesiana Vol 49 No 5 (2009): September 2009
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (133.364 KB) | DOI: 10.14238/pi49.5.2009.270-5

Abstract

Background Acute lymphoblastic leukemia (ALL) in childrenis a heterogeneous disease with different subtypes based on their cellular and molecular characteristics. This condition wouldinfluence the treatment outcome and subsequent risk for relapse. Accurate assignment of individual patients to risk groups is a critical issue for better outcome. TEL-AML1 gene fusion is themost frequent in childhood ALL.Objective The aim of this study was to investigate the incidenceofTEL-AML1 children with ALL in Sardjito Hospital.Methods This was a cross sectional study. In this preliminarystudy, we used nested reverse-transcriptase polymerase chainreaction (RT-PCR) to analyze the present of TEL-AML1 genefusion in bone marrow sample of childhood ALL patients.Results We analyzed 41 samples. Out of these, 30 (73%) wereamplified. Twenry three out of 30 ALL patients with good medicalrecord were analyzed for this gene fusion. Out of 30 patients, there were five patients (17%) with TEL-AML1-positive gene fusion and 25 (83%) were TEL-AML1-negative. Among five patients with TEL-AML1-positive gene fusion, four patients (80%) were one year to less than 10 year old. All of the patients (100%) were with leukocyte < 50x109/L.Conclusions TEL-AML1 gene fusion was found in 17 % ofsamples. This gene fusion was more frequent in standard risk group (based on age and leukocyte). These data must be clarified with more samples. RT-PCR must be apply in all center as one part of improving diagnostic quality, especially in managing leukemia patients.
Prenatal Diagnosis of Thalassemia Sunarto, Sunarto
Paediatrica Indonesiana Vol 33 No 7-8 (1993): July 1993
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi33.7-8.1993.191-9

Abstract

Thalassemia is an individual as well as a community health problem in some countries. It causes a lifelong suffering for the affected individuals. There is no treatment other than supportive, i.e. regular transfusions and removal of iron overload from the body. Only by such continuous and expensive treatment thalassemic patients can-generally achieve nearly normal health, but the health burden of such therapy for a large number of thalassemic patients is unaffordable by the affected communities. Prevention of the births of thalassemic babies is the choice for controlling the thalassemia and has been successful in many countries. For this purpose reliable and time accurate prenatal diagnosis is a conditio sine qua non. Blood fetal sampling is safe and can be done after 16 weeks gestation, amniocentesis after 14 weeks, and even chorionic villi sampling as early as 8 weeks gestation. In vitro globin synthesis analysis applied to the fetal blood sample is very reliable to measure the rate of synthesis of the globin chains that make up the hemoglobin. The-DNA analysis of the fibroblasts obtained by amniocentesis or of the chorionic villus sample is very sensitive and specific for the diagnosis of the genetic disorder in thalassemias. By involving the prenatal diagnosis, the birth of B-homozygous thalassemia has decreased by up to 90%.
Thrombocytopenia in Dengue Hemorrhagic Fever Sunarto, Sunarto; Sutaryo, Sutaryo
Paediatrica Indonesiana Vol 32 No 3-4 (1992): March 1992
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi32.3-4.1992.75-83

Abstract

According to WHO thrombocytopenia is one of the diagnostic criteria of DHF. On the other hand many studies have reported DHF cases without evidence of thrombocytopenia. One hundred fifty nine DHF out of485 suspected cases were assessed for their platelet counts. Diagnosis of DHF was established based on the WHO criteria, and confirmed by the hemagglutination inhibition test. The platelet counts were done serially and intensively by phase contrast microscope from the first day of hospitalization until the patient's discharge. In 77 patients thrombocytopenia appeared for the first time during hospitalization. Mild thrombocytopenia appeared in almost all of these cases before thrombocyte count of 100,000 reached. Seventy two patients had shown thrombocytopenia on the day of admission. No thrombocytopenia was found on the second day of illness, the earliest time of the detection of thrombocytopenia was on the Jrri and the latest was on the 8th day of illness. Thrombocytopenia lasted 1-5 days. It is concluded that many DHF diagnosis would have been missed if the thrombocyte investigation had not been done serially and intensively. Mild thrombocytopenia in DHF suspected patients should call attention to do platelet investigation intensively in those cases.
Aids in Infants and Children Sunarto, Sunarto
Paediatrica Indonesiana Vol 32 No 11-12 (1992): November 1992
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi32.11-12.1992.332-42

Abstract

 Children are infected by HIV, 80% vertically, from HIV infected mothetS mostly near or at delivery. Because heterosexual transmission of HIV among adults is more and more important it is estimated that at the end of this century there will be totally more than 10 million HIV infected children. Three quarters of HIV-infected babies show non specific symptoms at the early phase, including failure to thrive, chronic diarrhea, recurrent bacterial injections, mucocutanous infection. Cytotropism of HIV to neroe cells resul.ts tn inflammation, neroe cell damage and neuronal loss. Progressive neurologic abnormalities and developmental milestone regression or developmental retardation will be the results. Pneumocystics carinii infection has worse prognosis than lymphocytic interstitial pneumonia which more commonly occurred in HIV injected children. Diarrhea is a troublesome problem in children with AIDS. Kaposi's sarcoma and secondary cancer are rare in pediatric AIDS.  Anemia and thrombocytopenia is common among AIDS children. In developing countries children with AIDS die within the year following the appearance of the symptoms, whereas asymptomatic HIV-injected children will live longer with high risk of recurrent and opportunistic injections. The hallmark of AIDS in children is the same as in adults,iI.e. the decrease of the number and function of CD4 lymphocytes. This in turn influences the junctions of other immunocompetent cells and loss of immunity is the result. Many things are still unexplainable in children AIDS.
Co-Authors 08.05.52.0064 Novi Perwitasari 09.05.52.0075 Lia Yuliana 10.05.52.0010 Dwi Sulistyowati 10.05.52.0030 Christina Dewi 12.05.52.0210 Resty Andini Putri, 12.05.52.0210 12.52.02.0204 Kinayah, 12.52.02.0204 12.5202.0172 Sukowati 1352020069 Prijatno Sri Eko Putranto, 1352020069 1352020070 Teguh Purnomo, 1352020070 1352020104 Pujadi, 1352020104 1352020105 SRI HERANINGSIH, 1352020105 A, Reo A. Samik Wahab, A. Samik Abas, Abas Abdurrachman, Mirzam Adi Nugroho Adi Ratriyanto Adnan Sofyan Afiana Praditasari, Afiana Agres Vivi Susanti Agung Budi Raharjo Agung Budiharjo Agus Candra Agus Naryoso Agus Prasetyo Budi Agustian Ipa Aji Setiawan Alim, Muhamad Miftahul Alimuna, Wa Allen Yeoh, Allen Amalia Yunika Putri, 13.05.52.0204 Ana, Testian Yushli Anada Leo Virganta, Anada Leo Andi Suhardiyanto Andriani, Beti Angga Dwi Pratama, 14.05.52.0003 Angga Widhi Saputro Anggun Parameswari, Anggun Anindya Ratna Pratiwi Annisa Arum Putri Anton Styo Wibowo, Anton Styo Ardang, Rifvan Yuniar Ari Setyowati Arief, Ahmad Fikri ARIF INDIARTO, ARIF Arifin Arifin Arintina Rahayuni Ariyanti, Wahyu Arizona, Meivy Armeina Nur Rachmawati ARTINI PANGASTUTI Arum, Rizki Sekar Asri Nugraheningtyas Astuti, Satria Dwi Aulia, Vina Auliya Hakim, Muhammad Andi Avi Budi Setiawan Ayi Yustiati Azhari Azhari Azizzah, Farah B. Setiawan Baedhowi Baedhowi Bagus Indrawan, Bagus Bambang Sigit Bambang Wasito Adi Bardi Murachman Baskoro Adi Prayitno Bayu Setya Hertanto Bungaran, Andreas Cahyo, Reza Dwi cahyono, hadi Carlina Soetjiono Chairunnisa Chairunnisa Chandra Wijaya Condro Hadi Mulyono, Condro Hadi David David Dawa, Willy DEWI IRAWATI Dewi Kusuma Wardani Dhimas Arvico, 13.05.52.0138 Djarot Sadharto, Djarot Djati Mardiatno Dwi Pratama, 14.05.52.0003 Angga Dwi Ratnawati, Dwi Dyah Nur Subandriani, Dyah Nur EDWI MAHAJOENO Eka Kusumawati, Eka Eka Wahyudi Eko Haryono Eko Mujiyanto, 13.05.52.0092 Ekowati, Yunik Endah Tri Wahyuni Endang Widjayanti LFX Endarti, Tutik Dwi Enik Sulistyowati Erland, Radja Ernawan Setyono Eryani, Riris Euis Soliha F.X. Hartono Fareza, Muhamad Salman Fasani, Rizkan Faif Fattah, Fuad Abdul Febrianto, Aji Sofian Feri Setyowibowo Freza, Freza Gema Paku Bumi Gumilang, Anggita Moro Hani Faurizka Happy Ade Permanasari Hardyanto S HARI SUTRISNO Harini Harini Harlistyanti, Ryna Hartono Hartono Hartono, Rudy Harwanto, Dody Candra Haryadi haryadi Hazhari, Alvan Herbowo Hardianto Hermin Poedjiastoeti Hersugondo Hersugondo Heru Santoso Wahito Nugroho, Heru Santoso Wahito Hosiana MD Labania Idham Halid Ika Merdekawati, Ika Ikhsan Jaslin, Ikhsan Ilham Futaki Indra Surjati Indrawati, Like Indriyani Rebet Insani, Kaefiyah Nurul Intan Permata Sari, 12.05.52.0029 Isna Putri Indayani, Isna Putri Isni Nurruhwati Iva Yuniasih, 14.05.52.0001 JaisSetiawan, 13.05.52.0232 Jamaludin MALIK Jaslin Ikhsan Jayanti, Noviana Luthfi Jazilah, S. Jennifer Jennifer Joko Riyanto Joko Sutrisno Jonet Ariyanto Nugroho, Jonet Ariyanto Junun Sartohadi Karyadi, Agus Ketut Prasetyo Kristanto, Alfa Kristiani Kristiani Kristiara, Sandy Kristiawan, Dona Kuat Rahardjo TS Kun Aristiati Susiloretni, Kun Aristiati Kusuma Putri, Devi Ayu Kusumah, Fitrah Satrya Fajar Kusumo, Pandansari Langgeng Wahyu Santosa Larasati, Meirina Dwi Latif Sahubawa Leidena Sekar Negari Leny Noviani Lestari, Wijayanti Puji Lia Faiqoh Lies Rahayu Wijayanti Faida Luasunaung, Alfred Lukita, Andre Luqmana Chakim, 15.05.52.0010 Luzi, Ahmad Surya M Masykuri Malarsih Malarsih, Malarsih Malawani, M. Ngainul Manik, Astri Marita Martien Herna Susanti Martinus, Ade Megauleng, Khaeriya Mintasih Indriayu, Mintasih Mohammad Jaelani, Mohammad Muchamad Yulianto Muhammad Cahyadi, Muhammad Muhammad Sabandi, Muhammad Muhammad, Arfaningsi Mujadid, Ahmad Zahry Muslimawati, Claudia Musripah, 14.05.52.0098 Mustamin Mustamin N Wahyuningsih, N N Widyas, N Nachrowi D. 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