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Profil Lipodistrofi dan Dislipidemia pada Pasien Prepubertas dengan HIV yang Mendapat Terapi ARV di Rumah Sakit Cipto Mangunkusumo Yessi Yuniarti; Aryono Hendarto; Nia Kurniati; Djajadiman Gatot; Pramita Gayatri; Mulya Rahma Karyanti
Sari Pediatri Vol 18, No 1 (2016)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (568.896 KB) | DOI: 10.14238/sp18.1.2016.55-62

Abstract

Latar belakang. Terapi antiretroviral (ARV) kombinasi telah berhasil menurunkan angka morbiditas dan mortalitas pasien HIV, tetapi menimbulkan efek samping jangka panjang berupa sindrom lipodistrofi.Tujuan. Mengidentifikasi adanya lipodistrofi dan dislipidemia pada pasien prepubertas dengan HIV yang mendapatkan terapi ARV jangka panjang.Metode. Penelitian potong lintang dilakukan pada 76 pasien HIV usia prepubertas di Poli Alergi Imunologi RSCM. Pemeriksaan klinis lipodistrofi dilakukan oleh tenaga klinis, tebal lipatan kulit (TLK) triceps dan subscapular, lingkar pinggang serta rasio lingkar pinggang-panggul. Data kadar CD4 awal, status gizi awal terdiagnosis, jenis terapi ARV, dan lama terapi ARV didapatkan dari rekam medis. Subyek juga dilakukan analisis diet, pemeriksaan profil lipid, dan gula darah puasa.Hasil. Subyek prepubertas dengan HIV yang mendapatkan terapi ARV yang mengalami lipodistrofi dan dislipidemia berturut-turut 47% dan 46%. Subyek lipodistrofi berupa lipohipertrofi 35%, lipoatrofi 5%, dan tipe campuran 7%. Mayoritas subyek lipodistrofi memiliki massa lemak tubuh, serta TLK triceps dan subscapular normal. Subyek lipohipertrofi dan tipe campuran seluruhnya memiliki rasio lingkar pinggang-panggul meningkat. Terdapat hubungan yang bermakna antara penggunaan regimen ARV 2NRTI + PI meningkatkan risiko 6,9 kali untuk terjadinya dislipidemia (p=0,001 IK95%: 2,03-23,7) dibandingkan regimen 2NRTI+ NNRTI.Kesimpulan. Prevalensi lipodistrofi dan dislipidemia cukup tinggi pada pasien prepubertas dengan HIV yang mendapatkan terapi ARV. Mayoritas subyek yang mengalami lipodistrofi memiliki massa lemak tubuh, TLK triceps dan subscapular yang normal.
Clinical and laboratory profiles of hepatitis C in hemophiliac children Hanifah Oswari; Ferry Damardjati S.P.; Djajadiman Gatot; Zakiudin Munasir; Julfina Bisanto
Paediatrica Indonesiana Vol 47 No 5 (2007): September 2007
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (220.944 KB) | DOI: 10.14238/pi47.5.2007.229-33

Abstract

Background Hepatitis C virus (HCV) infection is common in hemophiliac receiving multiple coagulation factor transfusions before the introduction of donor screening and viral inactivation techniques. Information on the clinical profiles of HCV infection in children is still limited.Objective To describe clinical and laboratory profiles of HCV infection in hemophiliac children. Methods Patients registered at the Hemophilia Society of Department of Child Health, Cipto Mangunkusumo Hospital, who had positive anti-HCV were enrolled. None of them received antiviral treatment. All subjects infected by HCV before the age of 18 years and at least had positive anti-HCV test result for 6 months underwent clinical examination, alanine aminotransferase (ALT), platelets, and HCV RNA tests.Results Thirty nine subjects were available for review, with the median age of 15 years, and the mean age of the first time getting transfusion was 15 months. Twenty two (56%) of 39 subjects showed clinical manifestations. Liver and spleen enlargement were not found in any of the subjects. Ten (26%) subjects showed elevated ALT. Platelet count was within normal limit in all subjects. Twenty four (61%) patients had chronic hepatitis, whereas the remaining 15 (39%) subjects spontaneously cleared from HCV.Conclusions Pediatric HCV infection showed mild clinical manifestations. Sixty one percent subjects developed chronic hepatitis. The abnormality of laboratory finding may be associated with the future chronic liver disease.
Risk factors and outcomes, a case series of ischemic stroke in children Shirley L. Anggriawan; lrawan Mangunatmadja; Djajadiman Gatot; Sofyan lsmael; Najib Advani
Paediatrica Indonesiana Vol 49 No 1 (2009): January 2009
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (155.353 KB) | DOI: 10.14238/pi49.1.2009.59-64

Abstract

Ischemic strokes in children, although generallyrare events, are more prevalent  than  commonlybelieved. Ischemic stroke in children have  beenassociated with coagulation disorders, cardiacanomalies, infections  and  trauma.
Serological profile and hemolytic disease in term neonates with ABO incompatibility Desiana Dharmayani; Djajadiman Gatot; Rinawati Rohsiswatmo; Bambang Tridjaja
Paediatrica Indonesiana Vol 49 No 4 (2009): July 2009
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (110.423 KB) | DOI: 10.14238/pi49.4.2009.219-23

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.
Comparison of pulmonary functions of thalassemic and of healthy children Mardjanis Said; Sudigdo Sastroasmoro; Djajadiman Gatot; Bambang Supriyatno; Yovita Ananta
Paediatrica Indonesiana Vol 45 No 1 (2005): January 2005
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (411.462 KB) | DOI: 10.14238/pi45.1.2005.1-6

Abstract

Objectives The aim of this study was to compare some pulmo-nary functions of thalassemic patients and those of normal chil-dren. Factors correlated with lung dysfunction were assessed.Methods This cross-sectional study compared some pulmonaryfunctions of thalassemic patients with those of healthy children.The study was performed in the Department of Child Health, CiptoMangunkusumo Hospital, Jakarta, Indonesia. Pre- and post-trans-fusion hemoglobin levels of the thalassemic subjects were deter-mined. Other data such as chelation therapy and serum ferritinlevels were also obtained. Both thalassemic and control subjectsunderwent routine physical examinations and lung function testsusing an electronic spirometer. Spirometry was repeated threetimes for each subject, and only the best result was recorded.Results Sixty-three thalassemic patients were enrolled, consist-ing of 32 male and 31 female subjects. Healthy subjects consistedof 31 males and 31 females. Most thalassemic patients (46/63)were found to have lung function abnormalities. This was signifi-cantly different from control subjects, of whom most (39/62) hadnormal lung function. Restrictive lung function abnormality was themost common (42/63) observation documented. Serum ferritin lev-els were obtained from 28 male and 29 female thalassemic sub-jects. There was no correlation between percentage from predictedforced vital capacity and serum ferritin levels, whether in male(r=0.191; P=0.967) or female (r =-0.076, P=0.695) thalassemicsubjects.Conclusion Thalassemic patients have significantly lower lungfunction than healthy children. More thalassemic patients had lungfunction abnormalities compared to healthy children. Restrictivedysfunction was the most common finding in the thalassemic group.No correlation was found between lung function and serum ferritinlevels
The prevalence of factor VIII inhibitor in patients with severe hemophilia-A and its clinical characteristics Harijadi Harijadi; Djajadiman Gatot; Arwin AP Akib
Paediatrica Indonesiana Vol 45 No 4 (2005): July 2005
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi45.4.2005.177-81

Abstract

Background Hemophilia is a hereditary blood-clotting disorderdue to factor VIII deficiency. Up to this date, the administration offactor VIII in preventing and managing bleeding has been the maintreatment. One of the complications, which may occur due to re-peated administrations of factor VIII, is the formation of factor VIIIantibody (factor VIII inhibitor).Objective To find out the prevalence of severe hemophilia-A withfactor VIII inhibitor and its clinical characteristics.Methods A cross-sectional descriptive study was performed onchildren with severe hemophilia-A at the National Hemophilia CareCentre, Cipto Mangunkusumo Hospital, Jakarta, Indonesia, in June-August 2004.Results Out of 45 children studied, 16 had factor VIII inhibitor withaverage inhibitor titre of 1.15 Bethesda units (BU) (range 0.15-15BU). Most of them (12 patients) had inhibitor titre <5 BU. Chronicarthropathy was found in 17 out of 45 (37%) children with severehemophilia-A, consisting of nine patients from positive inhibitorgroup and 8 patients from negative inhibitor group. Thirty-ninepatients (86%) used an on-demand treatment pattern, among whom15 had positive inhibitor. Among patients receiving prophylactictreatment pattern, only one had positive inhibitor. There were 39patients (86%) treated using cryoprecipitate, among whom factorVIII inhibitor was found in 12, while among those treated with fac-tor VIII concentrate, the inhibitor was positive in 4/6. The averageamount of factor VIII transfused in positive and negative factor VIIIinhibitor groups was similar.Conclusion The prevalence of factor VIII inhibitor in severe he-mophilia-A patients was 35%. Chronic arthropathy occurred moreoften in patients with positive factor VIII inhibitor. Factor VIII inhibi-tor was found more frequently in patients with an on-demand treat-ment pattern and in those using factor VIII concentrate
Clinical features of patients with hemolytic anemia due to red blood cells membrane defect Pustika Amalia W; Djajadiman Gatot; Teny Tjitrasari; Iswari Setianingsih; Nanis Sacharis Marzuki
Paediatrica Indonesiana Vol 46 No 1 (2006): January 2006
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi46.1.2006.41-5

Abstract

Background Hemolytic anemia may result from corpuscular orextracorpuscular abnormalities. One of the types of corpuscularabnormalities is membrane defect. The diagnosis is sometimesdifficult and it may need special hematologic investigations. Thereare no data yet on the clinical features of red blood cell membranedefect in Cipto Mangunkusumo Hospital.Objective To evaluate the clinical features and laboratory find-ings of patients with hemolytic anemia due to red blood cells mem-brane defect in Cipto Mangunkusumo Hospital.Methods This was a descriptive study on patients with red bloodcells membrane defect who came to the Thalassemia Center atCipto Mangunkusumo Hospital during 2002-2004.Results In 2002-2004, there were 241 new cases of hemolyticanemia consisted of 116 patients with beta-thalassemia, 109 withHbE-beta thalassemia, 3 with alpha-thalassemia, and 13 with redblood cells membrane defect. The red cells membrane defect pa-tients consisted of 4 males and 9 females, ranging in age from 1months to 14 years. All subjects came to the hospital due to pale-ness as a chief compaint. Hepato-splenomegaly was found in 5 of13 cases. Laboratory findings revealed hemoglobin level 6.4-13.1g/dl (mean 9.4+2.1 g/dl), MCV 58.4-94.5 fl (mean 81.2+10.2 fl),MCHC 31.7-35.8 g/dl (mean 33.9+1.1g/dl), RDW 15.8-28.4%(mean 20.1+3.6%) and normal hemoglobin electrophoresis. Pe-ripheral blood smear showed anisocytosis, poikilocytosis,spherocytes, ovalocytes, stomatocytes, target cells, and fragmentedcells. The most common diagnosis in this group was SoutheastAsian Ovalocytosis (5/13).Conclusions In facing hemolytic anemia with normal Hb electro-phoresis or normal RBC enzyme level, the possibility of red cellsmembrane defect should be taken into consideration as a causeof this disorder. The clinical features and laboratory findings of redblood cells membrane defect patients are highly variable. Occa-sionally, hematologic investigations are necessary
Coagulation abnormality as a complication of L-asparaginase therapy in childhood lymphoblastic leukemia Djajadiman Gatot; Keumala Pringgardini; Rulina Suradi
Paediatrica Indonesiana Vol 46 No 1 (2006): January 2006
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi46.1.2006.46-50

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
Phenotypic diversity in beta-HbE thalassemia patients Pustika Amalia Wahidiyat; Djajadiman Gatot; Tenny Tjitrasari; Harapan Parlindungan Ringoringo; N S Marzuki; R A Taufani; I Setianingsih; A Harahap
Paediatrica Indonesiana Vol 46 No 2 (2006): March 2006
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi46.2.2006.82-6

Abstract

Background Thalassemia is a monogenic disease, yet the clini-cal manifestations (phenotype) are variable although they havethe same genotype. The clear-cut correlation between genotypeand phenotype in β-thalassaemia/HbE patients remains unex-plained. There are several factors that play a role in the severity ofthe clinical manifestations, i.e. two alpha-gene deletion, homozy-gote Xmn1 polymorphism +/+, -+-++, ++-++ haplotype, and hemo-globin Constant Spring.Objective To understand the clinical diversity of patients with HbE/α thalassemia and to determine whether it is possible to predictphenotypic severity from genetic factors.Methods A descriptive study on clinical presentations and hema-tological data of beta-HbE thalassemia patients. DNA analysis wasperformed to detect β-thalassemia mutations and the amelioratingfactors (alpha-globin genes deletions and Xmn1 restriction site poly-morphism at position –158 upstream of the G γ-globin gene) whichwere already known.Results Thirty patients with HbE/β thalassemia (4 to 29 years old)were recruited. IVS1-nt5 (G>C) severe β + mutation was detectedin 20 patients. Eighteen of 20 patients with positive IVS1-nt5 mu-tation group were heterozygous for Xmn1 restriction site polymor-phism and none of the patients was co-inherited with two á-globingene deletion. Almost all patients (19/20) with positive IVS1-nt5mutation group required regular transfusions, yet the mean age atfirst blood transfusion was older in negative IVS1-nt5 mutation groupthan that of positive IVS1-nt5 mutation group (5.7 vs 4 years). Meanhemoglobin before initial transfusion was higher in negative IVS1-nt5 mutation group than that of positive IVS1-nt5 mutation group(5.88 vs 5.39 g/dl). The mean total transfusion per year was lowerin the negative IVS1-nt5 mutation group than that of positive IVS1-nt5 mutation group (190.6 vs 215.1 ml/year).Conclusions Beta-HbE thalassemia patients with identical betathalassemia mutation (IVS1-nt5) show remarkable clinical diver-sity. Neither two alpha-gene deletion, nor the Xmn1- G γ polymor-phism can explain the phenotypic variation. Other amelioratingdeterminants or genetic modifications responsible for the variableclinical severity remain to be explored.
Treatment of childhood acute lymphoblastic leukemia in Jakarta: Result of modified Indonesian National Protocol 94 Djajadiman Gatot; Endang Windiastuti
Paediatrica Indonesiana Vol 46 No 4 (2006): July 2006
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi46.4.2006.179-84

Abstract

Background Before 1990, the survival rates of childhood acutelymphoblastic leukemia (ALL) patients remained low. In 1994, theHematology Oncology Working Group of the Indonesian PediatricAssociation constructed a national protocol based on standard in-ternational protocol. As the outcome was still not promising, in 1998the protocol was modified by introducing low dose MTX infusion forCNS prophylaxis.Objective To analyze the survival of pediatric ALL patient treated withthe modified protocol in Cipto Mangunkusumo Hospital, Jakarta.Methods A prospective study was carried out to all newly diag-nosed and relapsed children with ALL from January 1998 throughDecember 2004. Patients were stratified into standard risk group(SRG) and high risk group (HRG). HRG met with one of thesecriteria: WBC >50 000/ìl, the presence of CNS involvement, medi-astinal mass, relapse, or L 3 morphology. After completing induc-tion therapy, all patients received low-dose MTX (LDMTX) infusion(500 mg/m 2 ), especially for those aged less than 3 years. If thepatient could not afford LDMTX, cranial irradiation (CRT) was given.Results There were 309 patients, consisted of 190 SRG and 119 HRGpatients. Male to female ratio was 1.8:1. Complete remission wasachieved in 86.3% SRG patients compared with 63.8% in HRG pa-tients (P<0.05). Event-free survival (EFS) rate in SRG and HRG were65.9% (95%CI 59.8; 71.9%) and 40.4% (95%CI 32.5; 48.4%), respec-tively. The overall survival (OS) rates in SRG was 81.2% (95%CI 76.3;86.2%) and in HRG was 56.0% (95%CI 47.8; 64.2%). The overall OSand EFS for both groups were 71.6% (95%CI 67.0; 76.2%) and 59.6%(95%CI 54.5; 64.7%), respectively. Failure of therapy was mostly dueto severe aplasia resulted in bleeding and severe infection. CNS re-lapse was rare in both groups, i.e. 3.1% in SRG and 0.8% in HRG.Conclusion Treatment of ALL using modified national protocol forSRG shows promising results. However, the outcome of HRG pa-tients is still inferior to those reported elsewhere. The use of low-dose MTX infusion can replace the role of cranial irradiation asCNS prophylaxis measure.