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KORELASI NILAI T2*, T2 RELAKSOMETRI DAN SIR T2* HIPOFISIS DENGAN KADAR FSH DAN LH PADA PASIEN THALASSEMIA MAYOR Wita Septiyanti; Damayanti Sekarsari; Pustika Amalia W; Joedo Prihartono
Majalah Kedokteran Indonesia Vol 70 No 2 (2020): Journal of the Indonesian Medical Association Majalah Kedokteran Indonesia Volum
Publisher : PENGURUS BESAR IKATAN DOKTER INDONESIA (PB IDI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.1234/jinma.v70i2.174

Abstract

Pendahuluan: Thalassemia adalah penyakit anemia hemolitik yang diturunkan. Transfusi berkala pada pasien thalassemia menyebabkan deposit besi di hipofisis yang mengakibatkan hipogonadotropik hipogonadisme. Pemeriksaan MRI mulai digunakan unutuk mengukur kadar besi pada hipofisis. Metode: Uji korelasi dengan pendekatan potong lintang untuk mengetahui nilai korelasi nilai MRI T2 dan T2* relaksometri serta SIR T2* hipofisis dengan kadar FSH dan LH pada pasien thalassemia mayor. Pemeriksaan dilakukan 28 subjek penelitian dalam kurun waktu Desember 2016 hingga Maret 2017. Hasil: Terdapat korelasi antara nilai relaksometri T2 hipofisis potongan koronal dengan kadar FSH dan LH, serta terdapat pula korelasi antara nilai SIR T2* hipofisis dengan kadar LH. Kesimpulan: Nilai relaksometri T2 hipofisis potongan koronal dan SIR T2* hipofisis dapat digunakan sebagai acuan deposit besi pada hipofisis serta dapat memonitor terapi kelasi pada pasien thalassemia â mayor.
Prediction of Liver Volume from Liver Transplant Donor using Biometric Formula compared with Computed Topography Volumetry Putranto, Agi S; Syafina, Adinda B; Sekarsari, Damayanti; Mazni, Yarman; Moenadjat, Yefta
The New Ropanasuri Journal of Surgery
Publisher : UI Scholars Hub

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Abstract

Introduction. Liver volume calculation is critical in assessing the compatibility and resectability of the graft in living donor liver transplants (LDLT). An accurate estimation of liver volume is a predictor for successful LDLT. The gold standard of liver volume estimation is CT Volumetry. Despite several limitations in the availability of software, facility, and time consumed, there is still disagreement of biometric formula to predict liver volume in Indonesia. Methods: A cross-sectional design study was carried out in Dr. Cipto Mangunkusumo General Hospital, enrolling those who underwent liver transplantation from 1st January 2010 – 3rd October 2019. Bodyweight, body height, body mass index, body surface area, and CT volumetry were the variables of interest in the study and were subjected to analysis. Result. Body weight, body height, and body surface area are found from multivariate analysis in this research. Multivariate logistic regression of body weight with caudal liver volume giving out liver volume estimated equation of estimate liver volume of 479.23 + 13.95 (bodyweight). The equation in this study proposes a biometric formula to estimate liver volume using bodyweight based on Indonesian anthropometry. Conclusion: Bodyweight is proposed for equation formation based on a characteristic patient feature in Indonesia. Accuracy testing of the liver estimation equation discovered in this study proposed an entirely satisfactory result in the Indonesian population
Associations among the Degree of Hip Adductor Spasticity, the Level of Gross Motor Function Classification System (GMFCS) and the Migration Percentage in Children with Cerebral Palsy Lindrawati Tjuatja; Luh Karunia Wahyuni; Aryadi Kurniawan; Damayanti Sekarsari; Hamzah Shatri
Indonesian Journal of Physical Medicine & Rehabilitation Vol 5 No 01 (2016): Indonesian Journal Of Physical Medicine and Rehabilitation
Publisher : Indonesian Journal of Physical Medicine & Rehabilitation

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (1387.708 KB) | DOI: 10.36803/ijpmr.v5i01.195

Abstract

Objectives : This study was aimed at detecting the occurrence of hip dislocation in children with cerebral palsy (CP) by seeking the association among the degree of hip adductor spasticity, level of Gross MotorFunction Classification System (GMFCS) and Migration Percentage (MP).Methods : It was a cross sectional study with inclusion criterias were children with cerebral palsy, both male and female aged 2-10 years old, and parent’s approval by signing informed consent. Exclusion criterias werecomorbidities of other neuromotor impairments, such as spina bifida; other neuromuscular or musculoskeletal diseases, etc; uncooperative; and presenting severe diseases at the day of assessment. Measurement methodsincluded the Modified Tardieu Scale (MTS) R2, R1 and R2-R1 components to measure hip adductor spasticity; Gross Motor Function Classification System (GMFCS) protocol to assess gross motor ability; andAnterior-Posterior (AP) pelvic plain radiograph to calculate the Migration Percentage (MP) value.Results : At the initial phase, 31 children were included, however only 57 legs were analyzed for hip adductor spasticity and MP. There were no correlation between the degree of hip adductor spasticity and MPboth the R2 with MP (r = -0.060; p = 0.658), and R1 with MP (r = -0.136; p = 0.314). Moreover, there was insignificant difference between level of GMFCS and MP (p = 0.831).Conclusion : This study indicates nill correlation between the degree of hip adductor spasticity and the MP, and insignificant difference between the level of GMFCS and MP to detect the occurence of hip dislocationin children with cerebral palsy.Keywords : Modified Tardieu Scale, R2, R1, level of GMFCS, AP pelvic plain radiograph, MP value.
Hemostatic abnormalities in children with thalassemia major and liver iron overload Pustika Amalia Wahidiyat; Stephen Diah Iskandar; Novie Amelia Chozie; Damayanti Sekarsari
Paediatrica Indonesiana Vol 58 No 4 (2018): July 2018
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (217.634 KB) | DOI: 10.14238/pi58.4.2018.175-9

Abstract

Background Thalassemia major (TM) patients are susceptible to liver dysfunction due to iron deposition. Pediatric TM patients often present with bleeding. Blood loss necessitates transfusions, leading to increased iron absorption from the gut. Objective To study hemostatic abnormalities in children with TM and iron deposition in the liver. Methods This cross-sectional study involved 190 non-splenectomized children with TM. Liver iron deposition was evaluated using T2* MRI. Prothrombin time (PT), activated partial thromboplastin time (aPTT), and platelet counts were assessed from blood specimens. Results Most subjects were diagnosed with β-thalassemia and β-thalassemia/HbE. The majority of subjects were on deferiprone (DFP) treatment. Approximately 89.5% of subjects had liver iron overload. Prolongation of PT and aPTT, as well as thrombocytopenia were observed in 60%, 27.9%, and 19.5% of subjects, respectively. Prolonged aPTT and thrombocytopenia were observed three times more frequently in subjects with moderate - severe liver iron overload than in subjects with normal - mild liver iron overload (P=0.04 and 0.001, respectively). Conclusion Most TM subjects have liver iron overload ranging from mild to severe. Prothrombin time and prolongation, as well as aPTT prolongation, and thrombocytopenia are easily found in TM children. There were significantly more moderate - severe liver iron deposition patients with aPTT prolongation and thrombocytopenia than normal – mild patients with these conditions. Hence, we suggest that pediatric TM patients undergo liver iron deposition evaluations and use iron chelators in an optimal manner, in order to limit the risk of bleeding.
Liver iron overload and hepatic function in children with thalassemia major Pustika Amalia Wahidiyat; Stephen Diah Iskandar; Ludi Dhyani Rahmartani; Damayanti Sekarsari
Paediatrica Indonesiana Vol 58 No 5 (2018): September 2018
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (273.956 KB) | DOI: 10.14238/pi58.5.2018.233-7

Abstract

Background Routine blood transfusions and increased intestinal iron absorption lead to iron accumulation in various organs, especially the liver. To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs. Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values. Methods This cross-sectional study was conducted in Cipto Mangunkusumo Hospital. The degree of liver iron overload was evaluated by T2*MRI. Assessments of serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and bilirubin levels were done to evaluate liver function. Results A total of 291 TM children were included in this study. The mean age of subjects was 12 years. Most of the subjects were diagnosed as β-thalassemia homozygote (54.6%) and β-thalassemia/HbE (41.2%). Deferiprone (DFP) was the most commonly used iron chelator. Less than 10% of the subjects had normal liver iron deposition. The AST and ALT values increased proportionally with the severity of liver iron overload, with significant, moderately negative correlation coefficients (r=-0.388 and -0.434, respectively). However, albumin level decreased proportionally with the severity of liver iron overload, with a significant, moderately positive correlation coefficient (r=0.323). Liver T2* MRI had no significant correlations with direct, indirect, and ratio of direct/total bilirubin levels. Conclusion Most of the children with TM have mild to severe liver iron overload. Liver T2* MRI had significant, moderate correlations with AST, ALT, and albumin values. Bilirubin level has no correlation with T2* MRI. Our findings suggest that monitoring of AST, ALT, and albumin levels is important because they may reflect the severity of liver iron overload. However, they should not be used as the only predictors of iron overload.
Short-term intermittent prophylaxis post-intracranial hemorrhage in children with hemophilia Novie Amelia Chozie; Fitri Primacakti; Made Citra Saraswati; Damayanti Sekarsari
Paediatrica Indonesiana Vol 62 No 3 (2022): May 2022
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi62.3.2022.174-9

Abstract

Background Intracranial hemorrhage (ICH) is one of the major bleeding events causing mortality and long-term morbidity in children with hemophilia, especially those who receive on-demand therapy. Objective To evaluate the outcome of children with hemophilia after ICH receiving short-term intermittent prophylactic treatment. Methods This retrospective study was conducted in the Department of Child Health, Dr. Cipto Mangunkusumo Hospital, Jakarta. Children £18 years of age with hemophilia presenting with ICH between 2015-2020 were included. We recorded patients’ demographics, type and severity of hemophilia, the presence of factor VIII (FVIII) inhibitor, brain CT scan, treatment, and outcomes of these patients. Patients who received short-term intermittent prophylaxis using clotting factor concentrate (CFC) post-ICH episodes were observed for ICH recurrence. Results There were 19 episodes of ICH experienced by 18 patients, consisting of 16 patients with hemophilia A and 2 with hemophilia B. Patients’ median age was 4 years (range 0-16 years). Hemophilia was classified as severe in 13 patients, moderate in 4 patients, and mild in 1 patient. Thirteen episodes were preceded by head trauma. The most common clinical manifestation was seizures (13.2%). The most common type of ICH was subdural hematoma. Two patients died and 2 patients had neurological sequelae during hospitalization. The median dose of short-term intermittent prophylaxis using CFC (n=16) was 20 IU/kg of FVIII twice a week and 30 IU/kg of FIX twice a week, for a median duration of 8 weeks (range 5-12 weeks). One patient who did not adhere to the prophylaxis regimen had recurrent ICH at a similar location 6 months after the first episode. Conclusion Our findings suggest that short-term intermittent prophylaxis is important to prevent the recurrence of ICH in children with hemophilia.
Korelasi Nilai T2*, T2 Relaksometri dan SIR T2* Hipofisis dengan Kadar FSH dan LH pada Pasien Thalassemia Mayor Septiyanti, Wita; Sekarsari, Damayanti; Amalia W, Pustika; Prihartono, Joedo
Majalah Kedokteran Indonesia Vol 70 No 2 (2020): Journal of The Indonesian Medical Association - Majalah Kedokteran Indonesia, Vo
Publisher : PENGURUS BESAR IKATAN DOKTER INDONESIA (PB IDI)

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.47830/jinma-vol.70.2-2020-174

Abstract

Background: Thalassemia is a hereditary hemolytic anemia disorder. Periodic transfusion for thalassemia patients may lead iron deposit in pituitary gland and induce hypogonadotropic hypogonadism. MRI examination has been started to be used for measurement of iron level in pituitary gland. Method: This study uses cross sectional method. MRI T2 and T2* relaxometry value and SIRT2* of pituitary gland was correlated with FSH and LH level in patients with major thalassemia. This study involves 28 subjects and conducted from December 2016 to March 2017. Result: There is correlation between relaxometry values of coronal slice T2 pituitary with FSH and LH level. There is also a correlation between pituitary SIRT2* value with LH level. Conclusion: Relaxometry value of coronal slice T2 pituitary and pituitary SIRT2* value may be use as reference for iron deposit on pituitary gland as well as to monitor chelating therapy in major thalassemia â patients.