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Journal : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

Human immunodeficiency virus infection in children, special aspects Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 30, No 03 (1998)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

Human immunodeficiency virus infection in children and infants has some different characteristics compared to that in adults, including the transmission, the clinical manifestations and the clinical course. Many factors are associated with the vertical transmission, among others are maternal antigen p24 level, viral load, CD4+ -cell number, zidovudine therapy, and the presence of anti-Gp 120. Growth and development failure, interstitial pneumonia, and hepatomegaly are found in almost all pediatric AIDS. Lymphoid interstitial pneumonia (LIP) has a high frequency in vertical AIDS. Pneumocystic carinii pneumonia (PCP) as early complication occurs in about 10% of children with AIDS and has high mortality. Gastrohepatobillary dysfunction caused by various opportunistic microorganisms are common; disaccharidase intolerance also frequently occurs. The longer the children alive the higher the cardiac abnormalities. Neurologic abnormalities are found in 90% of vertical AIDS. The clinical course and the prognosis of vertical infection is associated with the maternal virus load, symptomatic HIV and p24 antigenemia, the lower the CD4+ cells in the mother, and the characteristic of the virus. The increased p24 antigenemia in the first 6 months of life, the opportunistic infections, and the progressive neurologic disease are related also to bad prognosis. Laboratory based diagnosis of HIV infection in infants is difficult especially due to the presence of maternal antibody. A number of tests is now under studies to find reliable and practical diagnostics, including polymerase chain reaction (PCR) of liquid or dried blood samples. IgA and other practical kit tests. HIV infected children show lower response against vaccinal antigens than normal infants. Anyhow, vaccination with killed vaccines is recommended for all HIV infected infants, including diphtheria, tetanus, killed polio. HIV infected children show response against H. influenzae vaccine. The advantages of BCG vaccination are weighted against the disadvantages. Varicella and hepatitis-B vaccine effectivity need further studies.Key words: pediatric HIV - vertical transmission - clinical manifestation - prognostic factor - laboratory diagnostics - recommended vaccination
A critical evaluation of peripheral blood smear in the diagnosis of thalassemia syndrome Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 30, No 01 (1998)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

The severe affected thalassemla syndrome Is common In Southeast Asian countries. Considering that the feature of its peripheral blood smear Is typical, this study was aimed at investigating the diagnostic value of the blood smear in thalassemia syndrome. Sixty five patients with severe anemia and splenomegaly who were admitted or came for follow up to Sardjito General Hospital in the period of 1996 and 1997 had been enrolled In this study. The peripheral blood smear was assessed, whether thalassemla or nonthalassemia, by two laboratory technicians, each of them read blindly each smear at two occasions with at least two weeks interval. The gold standard for diagnosis of thalassemla major was the evidence of increased HbA2 (>3,5% of total Hb) In both parents and for thalassemla-hemoglobin E disease was the increased HbA2 in one of the parents and the presence of HbE in the other. The measurements of HbA2 and HbE were carried out by quantification of HbA2 fraction following hemoglobin electrophoresis on cellulose acetate membrane (CAM) electrophoresis. The results of blood smear reading showed good intrarater agreement with kappa = 0.691 by the first rater and 0.634 by the second rater). The interrater agreement was high moderate to good (kappa = 0.567 - 0.728). The first reading by the first rater, the second reading by the first rater, the first reading by the second rater, and the second reading by the second rater showed sensitivities of 0.780, 0.780, 0.878, and 0.805 respectively; specificities of 0.708, 0.958, 0.542 and 0.792 respectively; positive predictive values of 0.820, 0.969, 0.766, and 0.868 respectively; and negative predictive values of 0.650, 0.719, 0.722, and 0.704 respectively. The peripheral blood smear has high sensitivity and specificity for diagnostic test of thalassemia syndrome.Key words: thalassemia syndrome - blood smear - hemoglobin electrophoresis - sensitivity and specificity -predictive value
Confidence interval in the analysis of clinical signicance Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 29, No 03 (1997)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

In the clinical study researchers want to answer the most important question whether a new therapy is better than the old one. Many researchers continue to frame the question in terms of null hypothesis and answer the question in terms of P value. The null hypothesis itself is typically not plausible, and even in a study where the null hypothesis was plausible, our concern is typically not only limited to the issue of whether or not the treatment has any effect but we also want to know how much the impact. In the study which the researcher pressed the P value into service as an indicator of effect size, it, lends itself to misinterpretation because it combines information about the magnitude of an effect with information about the precision with which that effect is estimated. By contrast, confidence intervals (Cis) focus one's attention on an estimate of a more meaningful parameter (e.g. the rate difference) and, as a separate matter, on the precision of the estimate. The CI is a range of values that is likely to cover the true but unknown value (the extremely low up to the extremely high value of e.g. rate difference, mean difference, and Odds Ratio) if we measure the value many times on samples using the same method. Cis in a clinical trial where the result is statistically significant we might find that it is of no clinically Importance (in a very large sample). On the other hand, the effect of a treatment might be statistically not significant but In fact it is of clinical importance (in a small sample). Cl Is affected by sample size: the larger the sample size the narrower the interval of Cl. The interval Is also affected by the standard error and hence by standard deviation and the confidence level we claim. The higher the confidence level (90%, 95% or 99%, arbitrarily 95% Cl is commonly used) the wider the confidence interval. In case that the 95% Cl does not include zero value (in mean or proportion difference) or one (in Odds ratio or relative risk) it also reflects statistical significance (p<0.05) with a = 5%. A sample size that is enough in terms of power might not be enough in terms of precision due to the confidence Interval level we choose.Key Words : P value - confidence interval - clinical importance - sample size - statistical significant
Diagnostik molekular thalassemia Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 28, No 01 (1996)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

A diagnosis of thalassemias has advanced from clinical to molecular in concordance with the advances in molecular biology. Since the introduction of polymerase chain reaction procedure - a practical in vitro procedure of deoxyribonucleic acid amplification - various diagnostic methods have been developed, to detects either gene deletions or point mutations. In a population where the spectrum of mutations is not too heterogenous, direct methods such as dot blot or reverse dot blot hybridization, ligase chain reaction and amplification refractory mutation system may be applied with high effectivity dan efficiency. But, in a population where the spectrum of mutations is very heterogenous other methods such as mismatch analysis, denaturing gradient gel electrophoresis and single strand conformation analysis as the screening step followed by deoxyribonucleic acid sequencing are chosen. Each of the above methods has advantages and shortcomings, in relation to various problems among others the sensitivity, the specificity, the ease, the reproducibility and the cost.In this paper the molecular diagnostics, concerning the principle, the advantages and the shortcomings, espebially that have been used in the field are discussed.Key words: gene disorders - ligase chain reaction - dot blot and reverse dot blot hybridization - amplification refractory mutation system - denaturing gradient gel electrophoresis - single strand conformation analysis - chemical cleavage of mismatch
Analysis of haplotype associated with mutation in a-thalassemia Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 27, No 03 (1995)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

B-thalassemia is mainly caused by point mutation of B-gene. There are more than 130 mutations, while only a little more than 20 haplotypes in B-gene. Many types of mutation are assosiated with one haplotype. On the other hand, one type of mutation may be associated with more than one chromosome haplotypes. The association of mutation and haplotype raises many questions. An analysis of haplotype has proved to be very useful to discover many aspects of thalassemia. The occurrence of certain mutation in a population might be predicted from the common haplotype of the population. Haplotype has been used to answer the question whether a certain mutation has multiple or single origin; for example the multiple origin of BE-gene in Cambodian population and the single origin of codon 39(CAG -+TAG) are associated with nine different haplotypes in Sardinia. It is suggested that haplotype probably influences the manifestation of thalassemia resulting in thalassemia intermedia.Key words : thalassemia - haplotype - point mutation - recombination - phenotype
Hemophilia with intracranial bleeding - A case report Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 27, No 01 (1995)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

A hemophiliac boy was hospitalized due to head injury in the previous sixteen days. On admission the' consciousness decreased and visus of both eyes was 1/on. CT scan showed blood clot in the area of dorsomedial of medial cerebral arteria and sign of hydrocephalus. A surgical procedure could not be taken due to lack of facilities (antihemophilic factor supply and assay of factor VIII activity). The patient was given 6 cryoprecipitate everyday and supportive treatment as indicated. Fortunately the patient recovered, no neurologic complication occurred except the visus of the right eye that was still 1/oo and the hydrocephalus decreased.Key words : hemophilia - laboratory diagnostics - head injury - hydrocephalus and decreased visus - cryoprecipitate
Talasemia di Rumah Sakit Umum Pusat Dr. Sardjito Tahun 1986 - 1992 Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 26, No 03 (1994)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

Thalassemia is a great health problem in the affected countries, especially in the developing countries including Indonesia. There is no curative therapy up to now, while the supportive therapy should be done life4ong and such therapy brings many problems to the patients and an enormous burden to the community who should afford the health services.Ninety thalassemia syndrome, at Dr. Sardjito General Hospital during the period of 1986-1992 were retrospectively studied. The diagnosis was based on clinical manifestations, routine blood examination, HbF investigation either with alkali denaturation test or electrophoresis and on the evidence of trait in the parents. Thirty six patients had undergone molecular studies. Aspects of therapy were also studied.The study found that 57 and 33 patients were major thalassemia and thalassemia-hemoglobin E disease, respectively. There was no difference between major thalassemia and thalassemia-hemogolobin E disease in term of male and female proportion (p > .05), and on the level (p > .05). Foutty eight patients came from Yogyakarta Province, the rest came from various parts of Java, especially nearby Yogyakarta Province. Compared with the estimation, we conclude that many thalassemic patients had not yet been covered. The expenses for supportive therapy were so great, while most of the patients belonged to the middle to low sosioeconomic level, so that only 30-40% had come regularly for routine transfusion. Among the 36 patients who underwent molecular studies, 31 have been genotypically identified. Molecular diagnostic was aimed at the first step of prenatal diagnosis.Key words: thalassemia syndrome-thalassemia trait-blood transfusion-molecular study-prenatal diagnosis
Molecular pathogenesis of thalassemia Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 25, No 02 (1993)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

Since the first description in 1925, thalassemia has been studied intensively and extensively. Thalassemia is inherited by Mendelian recessive genes, in which there is a mutation or deletion of the DNA nucleotide or results in defective production of one or more globin chains of the hemoglobin. There are two important forms of tha/assemias, a- and ft-thalassemia that are health problems in the tropical belt, extending from Africa and Meditenanian countries up to West Melanesia. The molecular studies of thalassemia and other hemoglobinopathies had discovered such a lot of abnormal hemoglobin structures and pathogenesis of the disease, so that the hemoglobin is now indeed a paradigm for our understanding of gene action at the molecular level. The thalassemia occurs essentially due to deletion, whereas 13thalassemia is mostly due to mutation of the gene(s). The understanding of the molecular pathogenesis of thalassemia is very essential for the diagnosis of the trait and especially for prenatal diagnosis, and in the future might be for genetic therapy.Key Words: thalassemia gene deletion gene mutation polymerise chain reaction haemoglobin
Aspek-AspekPenyakit Perdarahan Dalam Praktek Pada Anak Sunarto Sunarto
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) Vol 13, No 02 (1981)
Publisher : Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

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Abstract

Hemorrhagic diathesis in pediatrics, covering the mechanism of hemostasis and the diagnostic and therapeutic aspects, is discussed.Hemorrhagic cases due to various causes are not uncommon in children. It is essential to understand aspects of hemorrhagic diathesis to be able to manage such cases efficiently. The diagnostic approach should be in an ordered fashion. By such a way and by understanding the principles of management, even with limited facilities, we can diagnose and treat many cases and at least recognize difficult cases and act appropriately before referring.Key Words: hemorrhagic diathesis - mechanism of hemostasis - hemophilia - purpura - hypoprothrombinemia
Co-Authors Abdul Rachman Achmad Asrori Addi Mardi Harnanto Adelina Adelina Adi Martono Adriyani Adam Afrilia Miratul Ulfa Aga Ardhianto Feryyanshah Aga Dwi Sintha Agung Budi Raharjo Agus Naryoso S.Sos, M.Si, Agus Naryoso Agus Rahmad Hidayat Aldhila Gusta H. Yoga Allen Yeoh Andre Lukita Angga Dwi Pratama Angga Widhi Saputro Anindya Ratna Pratiwi Annisa Arum Putri Ari Sugeng Armeina Nur Rachmawati Asikin, Hijrah Asri Nugraheningtyas Aulia Ramandani Ayi Yustiati Bambang Subali Bambang Sudarmanto Bambang Wasito Adi Baskoro Adi Prayitno Chairunnisa Chairunnisa Chandra Wijaya Charles Parningotan Haratua Simanjuntak Dandi Wahyu Agung Darusman Darusman David David Devi Ayu Kusuma Putri Diah Ariani Dona Kristiawan Dowes Ardi Nugroho Dwi Safrudin Dwi Septiani Dwiky Erlangga Edhi Martono Eka Prasetyanta Endang D Endang Djuana Ervin Devi Pratiwi Esti Dyah Utami Evi Gravitiani Fadillah Januar Fahad Nuraini Farah Azizzah Faridah Laeli Syarifah Fatma Lestari Ferdiansyah Ferdiansyah Feri Setyowibowo Ferrianto G Ferrianto Gozali Fige Arief Kusumo Fitri Sagantha Florenchia Yohana Tellu Fredi Ganda Putra Fuad Abdul Fattah Furi styo Siskawati Hanif Nasiatul Baroroh Harini Harini Harliyandri Iqbal Nugroho Harry Supriyono Hermin Poedjiastoeti Heti Herawati I Made Sukanadi Igviro Yurki Lailina Ilham Futaki Inas Khairani Indra Suharman Intan Sonia Apriani Irfanda Rizki Harmono Sejati Iskandar Iskandar Isna Putri Indayani Iva Yuniasih Jennifer Jennifer K.I. Ismara Kandar Kandar Katisya Abrina Prastyanti Khaeriya Megauleng Kristiani Kristiani Kuat Rahardjo Kuat Rahardjo T.S Kuat Rahardjo TS Kurnia Roossenda Langgeng Wahyu Santosa Leidena Sekar Negari Leny Noviani Lia Faiqoh M. Mudrik H. Rohman M. Ngainul Malawani M. Rudyansyah Ismail Mariska Diah Kusumasari Mega Laksmini Syamsudin Mintasih Indriayu Mochamad Candra Wirawan Arief Muchamad Yulianto Muhammad Andi Auliya Hakim Muhammad Rizki Romadhon Muhammad Sabandi Mustamin Nanang Supriadi Naris Dyah Prasetyawati Neazar Astina Prabawani Niken Ambar Pratiwi Niko Sangaji Noor Sudiyati Nugroho Wahyu Sumartono Nur Amalia Choirani Nur Amalia Choironi Nursetya Danusaputra Nursyifa Asilah Sasongko Nuryanti Nuryanti Pandansari Kusumo Ponendi Hidayat Putri Oktaviana Rahmi Lubis Rani Rakhmaputri Wiranto Regina Tutik Padmaningrum Retno Dewi Kurniasari Ridhah Taqwa Rihadini Rihadini Rizkan Faif Fasani Robert Adriaan Philipus Rosalia H S Rosalia H Subrata S. Jazilah Sahora Dina Pangesty Salman Alfarisy Totalia Sandy Kristiara Sari Rosiati Nur Khasanah Sigid Sudaryanto Sischa Wahyu Dwiningsih Siti Alliyah Siti Kartika Sari Siti Marwati Slamet Haryono Slamet Mardiyanto Rahayu Slamet Suprayogi Sri Budi Lestari Sri Widowati Herieningsih Sri Woro Asih Sudarmadji Sudarmadji Sudarno Sudarno Sudrajat sudrajat Sumardino Sumardino Suparmi Suparmi Supriyanto Supriyanto Suriani Rauf Sutaryo Sutaryo Syahrun Syahrun Syahrun, Syahrun Syahrun Syaiko Rosyidi Syscha Pyae Tandiyo Pradekso Testian Yushli Ana Tiara Handayani Titi Yuli Astuti Titik Kuntari Titin Nurfiatin toto tohir Tri Melisa Selviani Trisnawiyana Trisnawiyana Triyono Lukmantoro Tuti Sri Suhesti Tutik Fitri Wijayanti Ubad Badrudin Utami Utami Vania Reani Valmai Wahyu Lestari Wahyu Yudha Pranoto Wahyu Yun Santoso Widha Sunarno Wijayanti Puji Lestari Winda Saputri Wiryanto Wiryanto Wiwid Noor Rakhmad Yasarah Diswari Ditiya Yeow Liang Yoseph Santosa Yudi Prana Hikmat Yuli Andriani Yuliyati Yuliyati Yun Ismi Wulandari Yuyuk Liana Zahidah Hasan Zulfikar Zulfikar Zulfikri Suleman