Hasil Pencarian  ::  Simpan CSV :: Kembali

"Lupus Eritematosus Sistemik LES merupakan penyakit autoimun kronik yang ditandai dengan adanya autoantibodi yang menyerang antigen sendiri. Kasus LES setiap tahunnya mengalami peningkatan. LES pada anak berkontribusi sebesar 15-20 dari keseluruhan penderita. Penyakit ini dapat menyerang berbagai sistem organ. Komplikasi endokrinopati berupa gangguan pubertas yang cukup sering terjadi pada anak. Hingga saat ini belum ada penelitian mengenai gangguan pubertas pada anak LES di Indonesia. Penelitian ini bertujuan untuk mengetahui prevalensi dan faktor-faktor yang berhubungan dengan terjadinya amenore pada anak LES. Penelitian dilakukan menggunakan desain potong lintang pada pasien anak perempuan usia 11-18 tahun di Poliklinik Alergi dan Imunologi dan Poliklinik Endokrinologi Rumah Sakit Cipto Mangunkusumo Kiara. Terdapat 25 subjek yang diikutsertakan. Hasil penelitian dilakukan analisis statistik menggunakan uji Chi-square dan uji Fisher. Prevalensi pubertas terlambat terjadi pada satu subjek 4 ; IK95 = -0,04-0,12 dan amenore terjadi pada dua belas subjek 48 ; IK95 = 0,27-0,69. Tidak terdapat hubungan yang bermakna antara amenore dengan durasi penyakit p=1,000; PR=1,05, aktivitas penyakit p=1,000; PR=0,86, durasi kortikosteroid p=0,673; PR=1,23, dan jenis terapi p=0,198; PR=0,55. Hasil penelitian tidak bermakna karena besar sampel tidak memenuhi jumlah minimal. Dari hasil tersebut, belum dapat disimpulkan bahwa tidak terdapat hubungan antara amenore terhadap durasi penyakit, aktivitas penyakit, durasi kortikosteroid, dan jenis terapi.

---

Systemic Lupus Erythematosus SLE is a chronic autoimmune disease characterized by autoantibodies that attack self antigen. The case of SLE each year has increased. SLE in children contributes 15 20 of all patients. This disease can attack various organ systems. Complications of endocrinopathy in the form of pubertal disorders are quite common in children. Until now, there has been no research on pubertal disorders in children with SLE in Indonesia.

This study aims to determine the prevalence and associated factors of amenorrhea in children with SLE. This study was a cross sectional study in girls aged 11 18 years at Allergy and Immunology Polyclinic and Endocrinology Polyclinic of Cipto Mangunkusumo Kiara Hospital. There are 25 subjects included.

The result of the research was statistical analysis using Chi square test and Fisher test. The prevalence of delayed puberty occurs in one subject 4 IK95 0,04 0,12 and amenorrhea occurs in twelve subjects 48 IK95 0,27 0,69. There was no significant association between amenorrhea with disease duration p 1,000 PR 1,05, disease activity p 1,000 PR 0,86, corticosteroid duration p 0,673 PR 1,23, and type of therapy p 0,198 PR 0,55.

The results were not significant because the sample size did not meet the minimum amount. From these results, it can not be concluded yet that there is no association between delayed puberty and amenorrhea with disease duration, disease activity, corticosteroid duration, and type of therapy."

"ABSTRAKLatar Belakang: Diabetes Melitus Tipe 1 DM tipe 1 merupakan salah satu jenis diabetes yang menyerang usia anak. Diabetes tipe ini dicirikan dengan riwayat ketoasidosis diabetikum KAD dan penggunaan insulin seumur hidup. Penderita DM tipe 1 rentan terhadap komplikasi mikrovaskular dan makrovaskular. Atherosklerosis merupakan komplikasi makrovaskular yang dapat dideteksi secara dini dengan melihat penebalan tunika intima dan media dari pembuluh darah. Sampai saat ini, belum ada data mengenai prevalensi penebalan pembuluh darah pada pasien diabetes melitus tipe 1 dan apakah penebalan tersebut berhubungan dengan riwayat kejadian KAD di Indonesia. Metode: Terdapat 30 pasien kontrol diabetes melitus tipe 1 yang mengikuti penelitian dengan desain potong lintang ini. Pemeriksaan penebalan Arteri Karotis Komunis AKK yang dilakukan menggunakan alat ultrasonografi resolusi tinggi dengan 2 operator. Dilakukan pula pemeriksaan tekanan darah dan pengukuran antropometri. Riwayat KAD, kadar HbA1c terakhir, dan durasi sakit ditentukan melalui rekam medis. Hasil penelitian dilakukan analisis statistik menggunakan uji Pearson, uji Spearman, dan Uji Chi-Square untuk mendapat nilai kemaknaan. Hasil: Prevalensi Penebalan AKK pada pasien anak dengan DM Tipe 1 sebesar 43,3 IK95 0,343-0,523. Tidak terdapat hubungan antara penebalan AKK dengan riwayat KAD p=0,64 dan frekuensi KAD p= 0,499, r=0,128. Dilakukan pengujian 4 variabel independen yakni durasi sakit p=0,249, nilai IMT p=0,944, nilai Sistolik p=0,077, dan kadar HbA1c terakhir p=0,249 dengan hasil tidak ada hubungan bermakna secara statistik. Kesimpulan: Prevalens penebalan AKK pada pasien anak dengan DM Tipe 1 sebesar 43,3. Durasi sakit,Riwayat KAD, Frekuensi KAD, kadar HbA1c terakhir, nilai sistolik, dan nilai IMT belum dapat disimpulkan karena keterbatasan sampel. K

---

ABSTRACTIntroduction Type 1 Diabetes Mellitus T1DM is one of the kind of diabetes whom common in childhood. The characteristics of this disease are the event of Diabetics Ketoacidosis DKA and permanently dependent on insulin therapy. There are several microvascluar and macrovascular complications associated with diabetes mellitus. Atherosclerosis is the macrovascular complications that the progression of plaque from Atherosclerosis can be detected early by knowing the thickness of intima and media layer of vascular. Until now, there is no data about the prevalence of Carotid Intima media thickness CIMT and its relationship with DKA history in Indonesia. Methods thirty consecutive patients with T1DM were registered into this cross sectional study. High resolution ultrasonography scanner performed by 2 operator was used to examine CIMT. There were examination on blood pressure and measurement on BMI. The history of DKA, the last HbA1c, and duration of DM were determined by medical record. Pearson, Spearman, and Chi Square test were used for the statistical analysis. Results the prevalence of the CIMT in patients with T1DM was 4.,3 IK95 0.343 0.523. There was no correlation between CIMT and the history of DKA p 0.64 as well as the frequency of DKA p=0.499, r 0.128. After analyzed the duration of diabetes p 0.249, BMI p 0.944, HbA1c p 0.249, Systolic value p 0.077, the results was not significantly correlated. Conclusion The Prevalence of CIMT in patients with T1DM is 43.3. The history of DKA, duration of diabetes, BMI, Systolic value, HbA1c are not yet concluded due to samples limitiation."

"ABSTRAKIndonesia tergolong dalam negara sabuk thalassemia yang memiliki prevalensi thalassemia yang tinggi. Thalassemia β mayor dan β-HbE merupakan dua jenis thalassemia yang paling banyak ditemukan di Indonesia. Studi-studi sebelumnya menemukan bahwa thalassemia menyebabkan gangguan pertumbuhan, di antaranya berupa perawakan pendek dan abnormalitas proporsi tubuh. Belum ada penelitian di Indonesia yang mencari perbedaan kejadian perawakan pendek dan proporsi tubuh antara anak thalassemia β mayor, β-HbE, dan normal usia prepubertal. Penelitian ini dilakukan dengan desain potong-lintang dengan menyertakan sampel berusia 5-12 tahun sebanyak 130 orang. Prevalensi perawakan pendek pada anak thalassemia β mayor ditemukan sebesar 34,0, pada anak thalassemia β-HbE sebesar 24,3, dan pada anak normal sebesar 9,4. Tidak terdapat perbedaan rasio segmen atas terhadap segmen bawah pada anak thalassemia β mayor, β-HbE, dan kontrol. Rerata rasio rentang lengan terhadap tinggi badan lebih pendek pada anak thalassemia β mayor dan β-HbE dibanding pada anak normal. Jenis kelasi, usia diagnosis, durasi sakit, frekuensi transfusi, rerata Hb pretransfusi dalam 6 bulan terakhir, dan rerata ferritin dalam 6 bulan terakhir tidak berbeda antara anak thalassemia yang pendek dan yang tidak pendek.

---

ABSTRACTIndonesia belongs to thalassemia belt countries which has high prevalence of thalassemia. major and HbE thalassemia are the two most common types of thalassemia found in Indonesia. Previous studies have found that thalassemia causes growth disorders, including short stature and abnormalities of body proportions. There have been no studies in Indonesia that looked for differences in the incidence of short stature and body proportion between β major and β-HbE thalassemia and normal children in prepubertal age. This research was conducted as cross sectional study towards 130 children with age of 5 12. The prevalence of short stature was found to be 34.0 in major thalassemia, 24.3 in HbE thalassemia, and 9.4 in normal children. There was no difference in upper segment to lower segment ratio between major and HbE thalassemia and control. Arm range to stature ratio is shorter in β major and β-HbE thalassemia than control. Types of chelating agent, age of diagnosis, duration of sickness, frequency of transfusion, mean pretransfusion Hb in the last 6 months, and mean ferritin in the last 6 months did not differ between short and not short thalassemia children. "

"Latar belakang. Penatalaksanaan komprehensif remaja diabetes melitus tipe-1 (DM tipe-1) meliputi terapi insulin, pengaturan nutrisi, aktivitas fisis, pemantauan gula darah, dan edukasi. Aktivitas fisis memengaruhi tingkat kebugaran fisis, melalui optimalisasi fungsi sistem kardiorespirasi, peningkatan kekuatan dan daya tahan otot, peningkatan sensitivitas insulin, perbaikan kadar hemoglobin A1c (HbA1c), serta peningkatan kualitas hidup remaja DM tipe-1. Hingga saat ini belum tersedia data mengenai hubungan aktivitas dan kebugaran fisis dengan HbA1c pada remaja DM tipe-1 di Indonesia.Tujuan. Mengetahui hubungan kebugaran fisis dengan hemoglobin A1c pada remaja DM tipe-1.Metode Studi potong lintang dilakukan pada 68 remaja DM tipe-1 di Rumah Sakit dr. Cipto Mangunkusumo (RSCM) dan pengisian kuesioner aktivitas fisis dan uji kebugaran EUROFIT fitness test battery yang dilakukan bersama divisi Kedokteran Olahraga.Hasil. Sebanyak 55,9% remaja memiliki aktivitas fisis sedang. Persentase kadar lemak optimal didapatkan 73,5% dan massa otot yang baik pada 75% anak. Jenis tes hasil buruk yaitu pada sit and reach test (92,6%), hand dynamometer test (82,3%), standing broad jump (54,4%), bent arm hang test (73,5%), sit-up test (91,2%), dan shuttle run (100%). Jenis tes dengan hasil mayoritas baik yaitu plate tapping test (50%), flamingo balance test (89,6%) dan 6-minute run test (50%). Kadar HbA1c >7% memiliki hubungan dengan standing broad jump buruk (p=0,017). Tingkat aktivitas fisis yang kurang pada remaja DM tipe-1 berhubungan dengan performa uji kebugaran fisis yang kurang, yaitu persentase lemak (p=0,002), massa otot (p=0,002), standing broad jump test (p=0,009), bent arm hang test (p=<0,001), dan 6-minute run test (p=<0,001).Kesimpulan. Mayoritas remaja DM tipe-1 memiliki aktivitas fisis sedang. Tidak ada hubungan kebugaran fisis dengan HbA1c pada remaja DM tipe-1. Tingkat aktivitas fisis yang kurang pada remaja DM tipe-1 berhubungan dengan performa uji kebugaran fisis yang kurang.

---

Background. The comprehensive management of adolescents with type-1 diabetes mellitus (type-1 DM) consisted of insulin therapy, nutritional management, physical activity, blood sugar monitoring, and education. Physical activity affects the level of physical fitness, which can optimize the function of the cardiorespiratory function, increase muscle strength and endurance, and insulin sensitivity, reduce hemoglobin A1c (HbA1c), and improve the quality of life. Until recently, data of the activity level and physical fitness profile in adolescents with type-1 DM has not yet available in Indonesia.

Objectives. To evaluate relationship of activity level and physical fitness with hemoglobin A1c in type-1 DM in adolescents.

Methods. This is a cross-sectional study of 68 adolescents with type-1 DM at RSCM. Study subjects were asked to fill the physical activity questionnaire and the EUROFIT fitness test battery. This study is a collaboration with the Sports Medicine Division of University of Indonesia.

Results. Physical activity with moderate results based on the questionnaire is 55.9%. The average HbA1c was 9% (7,5-11.15). The percentage of good fat results obtained was 73,5% and good results of muscle mass were as much as 75%. The majority of results were poor on the sit and reach test (92.6%), hand dynamometer (82.3%), standing broad jump (54.4%), bent arm hangs test (73.5%), sit-ups test (91.2%), and shuttle run (100%). Good results on the flamingo balance test (89.6%) and 6-minute run test (50%), and plate tapping test (50%). HbA1c levels >7% are associated with poor level of standing broad jump (p=0.017). The physical activity were poor in adolescents is associated with poor physical fitness test performance, the percentage of fat (p=0.002), muscle mass (p=0.002), standing broad jump test (p=0.009), bent arm hang test (p=<0.001), and 6-minutes run test (p=<0.001).

Conclusion. Most adolescents with type-1 DM have moderate physical. There is no relationship between physical fitness and HbA1c in type-1 diabetes in adolescents. The physical activity were poor in adolescents is associated with poor physical fitness test performance."

"Latar belakang. Penyakit jantung bawaan (PJB) merupakan penyebab kelainan kongenital dengan 9 per 1.000 kelahiran hidup bayi dengan PJB per tahun. Angka kematian karena PJB sendiri mencapai 250.000 kematian per tahun di seluruh dunia. Pasien PJB memiliki risiko lebih tinggi terkena infeksi, terutama infeksi respirasi. Penyakit infeksi pada pasien PJB berisiko untuk berprogres menjadi komplikasi yang serius. Penyakit menular seperti pneumonia merupakan sumber utama morbiditas dan angka kematian pada anak-anak penderita PJB di bawah usia lima tahun, oleh karena itu, menghindari infeksi sangat penting bagi populasi PJB. Imunisasi merupakan salah satu strategi yang penting untuk mencegah timbulnya morbiditas dan mortalitas pada anak dengan PJB. Masih ditemukan banyak anak dengan PJB yang belum mendapatkan imunisasi secara lengkap. Studi di berbagai negara menunjukkan bahwa cakupan imunisasi pada anak PJB hanya mencapai 34%. Tujuan. Penelitian ini bertujuan untuk menilai kelengkapan imunisasi pada pasien PJB dan faktor yang berhubungan. Metode. Penelitian merupakan studi potong lintang yang melibatkan anak PJB berusia 6 bulan hingga 5 tahun yang berobat ke RSCM. Pengambilan data dilakukan dengan kuesioner dan wawancara, serta rekam medis. Analisis dengan regresi logistik dilakukan untuk mendapatkan akurasi pengaruh gabungan dari parameter yang diuji. Hasil. Penelitian melibatkan 127 pasien dengan PJB. Imunisasi yang lengkap didapatkan pada 26,77% subyek. Dari subyek yang memiliki imunisasi lengkap, 35,29% diantaranya diberikan imunisasi tepat waktu. Imunisasi dengan cakupan yang paling tinggi adalah Hepatitis B yang diberikan segera setelah lahir. Pengetahuan, sikap, dan perilaku orang tua terhadap imunisasi secara umum baik. Lama rawat, perilaku orang tua, dan penundaan imunisasi oleh tenaga kesehatan berhubungan dengan kelengkapan imunisasi. Simpulan. Kelengkapan dan ketepatan waktu imunisasi pada pasien PJB di Indonesia masih rendah. Rawat inap, perilaku orang tua terhadap imunisasi, dan penundaan oleh tenaga kesehatan merupakan faktor yang berhubungan dengan kelengkapan imunisasi.

---

Background. Congenital heart disease (CHD) is a most common cause of congenital abnormalities, accounting for 9 per 1,000 live births each year. CHD causes 250,000 mortalities per year worldwide. CHD patients have an increased risk of infections, particularly respiratory infections. Infectious illnesses in CHD patients can lead to significant consequences. Infectious infections such as pneumonia are the leading cause of morbidity and mortality in children with PJB under the age of five; hence, avoiding infection is critical for the CHD patients. Immunization is an important strategy for reducing morbidity and mortality in children with CHD. There are still many children with CHD who have not been fully immunized. Studies in various countries show that the scope of immunization in children only reaches 34%. Objectives. This study aims to assess the completeness of immunization in PJB CHD and related factors. Method. This research was a cross sectional study of pediatric patients aged 6 months to 5 years who came to Cipto Mangunkusumo Hospital. Data were collected via questionnaires, interviews, and medical records. Analysis with logistic regression was used to determine of the combined effect of the parameters investigated. Result. The study involved 127 patients with CHD. Complete immunization was obtained in 26.77% of subjects. Of the subjects who had complete immunization, 35.29% were given timely immunization. The immunization with the highest coverage was Hepatitis B which was given immediately after birth. Parental knowledge, attitudes, and behavior towards immunization were generally good. Length of stay, parental practice towards immunization, and delays in immunization by health workers were associated with completeness of immunization. Conclusion. The completeness and timeliness of immunization in CHD patients in Indonesia are still low. Hospitalization length of stay, parents' attitude towards immunization, and delay by health workers are factors related to immunization completeness."

"Latar belakang: Epilepsi Rolandik adalah salah satu sindrom epilepsi terbanyak pada anak dan umumnya dianggap benign dan self-limited. Tetapi komorbiditas kognitif dan perilaku dapat ditemukan pada anak-anak dengan epilepsi yang bernama lainnya benign Rolandic epilepsy with centrotemporal spikes (BECTS) ini. Pasien epilepsi Rolandik harus dievaluasi adanya komorbiditas kognitif dan perilaku. Tujuan: Mengetahui hubungan antara usia awitan kejang dan jumlah hemisfer yang menjadi fokus kejang dengan kemampuan kognitif dan gangguan perilaku pada anak-anak dengan epilepsi Rolandik. Metode: Penelitian potong-lintang di sembilan RS/klinik dengan konsultan neurologi anak di Jakarta dan Banten, terhadap anak-anak berusia 6 – 12 tahun dengan epilepsi Rolandik. Penelitian meliputi anamnesis semiologi kejang, interpretasi hasil elektroensefalografi (EEG) oleh dua orang konsultan neurologi anak, dan pemeriksaan intelligence quotient (IQ) oleh psikolog.Hasil: Nilai verbal IQ sebagai parameter kognitif berbicara/berbahasa lebih banyak bernilai  average pada subjek dengan usia awitan kejang yang lebih muda (< 6 tahun). Nilai verbal IQ < average lebih banyak didapatkan pada subjek dengan fokus gelombang kejang pada salah satu sisi hemisfer. Jumlah subjek yang mengalami gangguan perilaku berupa gangguan pemusatan perhatian dan hiperaktivitas (GPPH) dan gangguan spektrum autism (GSA) lebih banyak pada kelompok usia awitan kejang < 6 tahun dan fokus kejang hemisfer bilateral. Tidak ada hubungan yang bermakna secara statistik antara usia awitan kejang dan jumlah hemisfer yang menjadi fokus kejang dengan komorbiditas kognitif dan perilaku. Simpulan: Anak-anak dengan epilepsi Rolandik dapat memiliki komorbiditas kognitif berupa gangguan berbicara/berbahasa dan komorbiditas perilaku berupa GPPH/GSA, dan dapat dipengaruhi oleh usia awitan kejang dan jumlah hemisfer yang menjadi fokus kejang, meskipun hubungannya tidak bermakna secara statistik.

---

Background: Rolandic epilepsy is one of the most common epilepsy syndromes in children and is generally considered benign and self-limited. But cognitive and behavioral comorbidities can be found in children with this epilepsy, which has another name: benign Rolandic epilepsy with centrotemporal spikes (BECTS). Rolandic epileptic patients should be evaluated for cognitive and behavioral comorbidities.

Objective: To determine the relationship between age at onset of seizures and the number of hemispheres that are the focus of seizures with cognitive abilities and behavioral disorders in children with Rolandic epilepsy.

Methods: Cross-sectional study in nine hospitals/clinics with pediatric neurology consultants in Jakarta and Banten, on children aged 6-12 years with Rolandic epilepsy. The research includes interviews on seizure semiology history, interpretation of electroencephalographic (EEG) results by two child neurology consultants, and intelligence quotient (IQ) tests performed by psychologists.

Results: The verbal IQ score as a cognitive speaking/language parameter  average was found more in subjects with younger seizure onset (<6 years). Verbal IQ < average scores were greater in subjects with seizure focus on one side of the hemisphere. The number of subjects who experienced behavioral disorders in the form of attention deficit hyperactivity disorder (ADHD) and autism spectrum disorders (ASD) was more in the age group of seizure onset < 6 years and bilateral hemispheric seizure focus. There was no statistically significant relationship between age at onset of seizures and the number of hemispheres that were the focus of seizures with cognitive and behavioral comorbidities.

Conclusion: Children with Rolandic epilepsy may have cognitive comorbidities in the form of speech/language disorders and behavioral comorbidities in the form of ADHD/ASD, and may be affected by the age of seizure onset and the number of hemispheres that are the focus of seizures, although the relationship is not statistically significant."

"Latar belakang. Anak dan remaja dengan leukemia limfoblastik akut (LLA) berisiko mengalami osteoporosis sekunder, salah satunya karena pemberian obat kemoterapi metotreksat dan steroid. Saat ini belum terdapat data prevalens osteoporosis sekunder pada anak dengan LLA di Indonesia dan bukti keterkaitan dosis kumulatif metotreksat dan steroid terhadap kejadian osteoporosis sekunder pada anak dengan LLA.Tujuan. Mengetahui ada tidaknya kaitan antara dosis kumulatif metotreksat dan/atau steroid terhadap kejadian osteoporosis sekunder pada anak dan remaja dengan LLA.Metode. Penelitian ini merupakan studi potong lintang terhadap 52 anak dan remaja dengan LLA yang sedang menjalani kemoterapi di Rumah Sakit dr. Cipto Mangunkusumo (RSCM). Pengambilan darah dan foto polos tulang belakang dilakukan untuk menilai parameter kesehatan tulang, serta pemeriksaan dual energy X-ray absorptiometry (DEXA) untuk menilai densitas mineral tulang. Analisis regresi logistik digunakan untuk menganalisis keterkaitan dosis kumulatif metotreksat dan steroid terhadap kejadian osteoporosis sekunder.Hasil. Median usia subyek adalah 10 (7-14) tahun dengan lelaki 54% (n=52). Didapatkan kejadian osteoporosis sekunder 6/52 (11,5%) dan densitas mineral tulang rendah 11/52 (21,2%). Tidak didapatkan kaitan antara dosis kumulatif steroid (adjusted RP 0,474 [0,057-3,935], p = 0,489) dan dosis kumulatif metotreksat (adjusted RP 0,083 [0,006-1,126], p = 0,061)  dengan kejadian osteoporosis sekunder. Pasien berusia di bawah 10 tahun, memiliki kadar vitamin D rendah, dan status prepubertas memiliki kecenderungan mengalami osteoporosis sekunder.Kesimpulan. Tidak didapatkan hubungan yang bermakna secara statistik antara dosis kumulatif steroid dan/atau metotreksat terhadap osteoporosis sekunder pada anak dan remaja dengan LLA.

---

Background. Children and adolescents with acute lymphoblastic leukemia (ALL) are at risk of secondary risk, one of which is the administration of chemotherapy drugs (methotrexate and steroids). Currently, there are no data on the prevalence of secondary osteoporosis in children with ALL in Indonesia and evidence about association between methotrexate and steroids with the incidence of secondary osteoporosis with ALL.

Objective. To determine whether there is an association between the cumulative dose of methotrexate and/or steroids on the incidence of secondary osteoporosis in children and adolescents with ALL.

Methods. This study was a cross-sectional study of 52 children and adolescents with ALL who were undergoing chemotherapy at the Cipto Mangunkusumo Hospital (CMH). Blood sampling and plain radiographs of the spine were performed to assess bone health parameters, as well as dual energy X-ray absorptiometry (DEXA) examination to assess bone mineral density. Logistic regression analysis was used to analyze the association between the cumulative dose of methotrexate and steroids on the incidence of secondary osteoporosis.

Result. The median age of the subjects was 10 (7-14) years with 54% men (n=52). The incidence of secondary osteoporosis was 6/52 (11.5%) and low bone mineral density 11/52 (21.2%). There was no association between the cumulative dose of steroids (adjusted PR 1.501 [0.124-18.124], p=0.75) and the cumulative dose of methotrexate (adjusted PR 0.071 [0.005-0.951], p=0.05) and the incidence of secondary osteoporosis. None of the confounding factors (pubertal status, vitamin D levels, income level, age, and sex) were associated with secondary osteoporosis. Patient aged below 10 years old, have prepubertal status, and with low vitamin D serum tends to have osteoporosis more likely.

Conclusion. There was no statistically significant relationship between the cumulative dose of steroids and/or methotrexate on secondary osteoporosis in children and adolescents with ALL."

"Latar belakang dan Tujuan: Perawakan pendek berhubungan erat dengan gangguan kualitas hidup seorang anak serta orang tua ataupun pengasuhnya. Quality of Life in Short Stature Youth (QoLISSY) merupakan alat bantu penilaian kualitas hidup pada anak dengan perawakan pendek. Penelitian ini bertujuan untuk mengadaptasi instrumen QoLISSY ke dalam Bahasa Indonesia dan membuktikan validitas serta reliabilitas instrumen tersebut. Metode: Penelitian ini menggunakan desain studi potong lintang yang merekrut subyek anak yang bersekolah di SMAN 68 dengan perawakan pendek. Penerjemahan instrumen dilakukan oleh dua pasang penerjemah bersertifikat. Validitas konkuren dinilai menggunakan uji korelasi Spearman dengan membandingkan masing-masing pertanyaan instrumen QoLISSY versi Bahasa Indonesia, sedangkan penilaian reliabilitas dilakukan dengan melihat nilai Cronbach’s alpha.Hasil: Sebanyak 448 subyek anak berhasil diukur tinggi badannya, 72 (16,1%) di antaranya berperawakan pendek. Dari 72 subyek berperawakan pendek, 33 subyek bersedia mengisi kuesioner QoLISSY. Hanya 2 subyek yang melakukan pengobatan hormon pertumbuhan, oleh karena itu uji validitas dan realibilitas dilakukan pada 36 dari 50 butir pernyataan. Uji validitas menunjukkan 6 dari 36 butir pernyataan memiliki korelasi lemah, namun korelasi antarpertanyaannya baik. Nilai Cronbach’s alpha instrument ini adalah 0,930, yang menunjukkan konsistensi internal yang sangat baik.Kesimpulan: Instrumen QoLISSY versi Bahasa Indonesia memiliki validitas dan reliabilitas yang baik sebagai alat penilaian kualitas hidup anak dengan perawakan pendek.

---

Background and Objectives: Short stature is closely related to impaired quality of life for a child and their parents or caregivers. The Quality of Life in Short Stature Youth (QoLISSY) is an assessment tool for evaluating the quality of life in children with short stature. This study aims to adapt the QoLISSY instrument into Indonesian and to prove its validity and reliability.

Methods: This study used a cross-sectional design and recruited children with short stature from SMAN 68. The instrument translation was performed by two pairs of certified translators. Concurrent validity was assessed using the Spearman correlation test by comparing each question of the Indonesian version of the QoLISSY instrument, while reliability was assessed by looking at Cronbach’s alpha values.

Results: A total of 448 children were measured for height, 72 (16.1%) of whom were short. Out of the 72 short-statured children, 33 subjects were willing to complete the QoLISSY questionnaire. Only 2 subjects were undergoing growth hormone therapy, thus validity and reliability tests were performed on 36 out of 50 questionnaire items. Validity tests showed that 6 out of 36 items had weak correlations, but the inter-item correlations were good. The Cronbach’s alpha value of this instrument was 0.930, indicating excellent internal consistency.

Conclusion: The Indonesian version of the QoLISSY instrument has good validity and reliability as a tool for assessing the quality of life of children with short stature."

"

Kortikosteroid merupakan terapi utama pada sindrom nefrotik, tetapi memiliki efek samping penurunan kepadatan massa tulang. Pemeriksaan bone mineral density (BMD) merupakan cara terbaik untuk mengetahui kesehatan tulang, tetapi belum rutin dilakukan dan banyak diteliti di Indonesia, khususnya pada pasien sindrom nefrotik resisten steroid (SNRS). Penelitian ini bertujuan untuk mengetahui pengaruh durasi penggunaan, dosis kumulatif kortikosteroid, kadar vitamin 25(OH)D, dan kalsium terhadap penurunan nilai BMD pasien SNRS. Sebuah penelitian potong lintang terhadap 63 subjek SNRS di RSUPN Dr. Cipto Mangunkusumo yang berusia 5 – 18 tahun, mendapatkan kortikosteroid minimal 4 minggu dengan laju filtrasi glomerulus >60 ml/menit/1,73 m². Dilakukan pemeriksaan laboratorium darah, BMD total body less head (TBLH) dan vertebrae lumbar. Subjek mendapatkan kortikosteroid dengan median durasi 37,4 (16,27 – 67,30) bulan, dosis harian 0,4 (0,30 – 0,67) mg/kgbb/hari dan dosis kumulatif 488,89 (309,62-746,05) mg/kgbb. Terdapat 66,7% subjek dengan defisiensi 25(OH)D. Hasil pemeriksaan BMD rendah lebih banyak didapatkan pada pemeriksaan BMD L1-L4 dibandingkan TBLH (25,4% vs 7,9%). Analisis bivariat dan multivariat menunjukkan bahwa durasi dan dosis kumulatif kortikosteroid memengaruhi penurunan nilai BMD L1-L4 (p<0,0001, p=0,0001, berturut – turut), tetapi kadar vitamin 25(OH)D dan kalsium darah tidak memengaruhi penurunan nilai BMD. Sebagai kesimpulan, durasi penggunaan dan dosis kumulatif kortikosteroid memengaruhi penurunan nilai BMD L1-L4 pasien SNRS.

---

Corticosteroids are the main therapy in nephrotic syndrome with the side effect of reducing bone density. Bone mineral density (BMD) examination is the best way to determine bone health, but it is not routinely carried out and studied in Indonesia, especially ​​in patients with steroid-resistant nephrotic syndrome (SRNS). This study aims to determine the effect of duration and cumulative dose of corticosteroids, vitamin 25(OH)D and calcium levels on decreasing BMD values ​​in SNRS patients. A cross-sectional study was conducted on 63 SRNS subjects at RSUPN Dr. Cipto Mangunkusumo, aged 5 – 18 years, received corticosteroids for at least 4 weeks with a glomerular filtration rate >60 ml/minute/1.73 m2. Laboratory blood test, total body less head (TBLH) and lumbar vertebrae BMD were carried out. Subjects received corticosteroids with a median duration of 37.4 (16.27 – 67.30) months, daily dose of 0.4 (0.30 – 0.67) mg/kgbb/day and cumulative dose of 488.89 (309, 62-746.05) mg/kgbb. There were 66.7% of subjects with 25(OH)D deficiency. Low BMD examination results were more frequently in L1-L4 BMD examinations compared to TBLH (25.4% vs 7.9%). Statistic analysis showed that the duration and cumulative dose of corticosteroids influenced the decrease in L1-L4 BMD values ​​(p<0.0001, p=0.0001, respectively), but the levels of vitamin 25(OH)D and calcium does not affect it. In conclusion, the duration of use and cumulative dose of corticosteroids influence the reduction in L1-L4 BMD values ​​in SNRS patients."

"Hiperplasia adrenal kongenital (HAK) adalah gangguan yang ditandai dengan defek pada enzim jalur steroidogenesis adrenal. Lebih dari 90% disebabkan mutasi pada CYP21A2 yang mengkode enzim 21-hidroksilase (21-OH). Monitoring tata laksana pasien HAK cukup sulit dicapai dengan menjaga keseimbangan antara overtreatment dan undertreatment.Penelitian bersifat cross sectional dilakukan di RSCM berlangsung selama 7 bulan (Juni-Desember 2020). Pemilihan sampel dilakukan dengan consecutive sampling dengan total 142 sampel. Sampel pasien HAK sebanyak 71 pasien, serta pemilihan sampel kontrol dilakukan dengan matching jenis kelamin dan usia. Kelompok HAK dilakukan pemeriksaan kadar 17-hidroksiprogesteron (17-OHP) serta androsteron, etiokolanolon urin dan rasio androsteron/etiokolanolon urin (A/E). Uji korelasi dilakukan antara androsteron, etiokolanolon, rasio A/E dengan 17-OHP. Kelompok kontrol dilakukan pemeriksaan androsteron, etiokolanolon urin dan rasio A/E. Hasil kedua kelompok dilakukan komparasi.Dari 71 kelompok HAK dan 71 kelompok kontrol mempunyai karakteristik dasar yang sebanding. Kadar androsteron kelompok HAK dibandingkan dengan kelompok kontrol berbeda bermakna (683,89(29,42-61061,43) vs 123,97(30,16- 16463,05) ng/mL;p<0,001). Kadar etiokolanolon kelompok HAK berbeda bermakna dibandingkan dengan kelompok kontrol (235,88(12,77-78446,65) vs 70,96(12,61-17332,62)ng/mL;p<0,001). Rasio A/E kelompok HAK berbeda bermakna dibandingkan dengan kelompok kontrol (2,31(0,37-40,12) vs 1,99(0,52- 5,45); p0,003). Kadar androsteron, etiokolanolon, rasio A/E mempunyai korelasi positif dengan kadar 17-OHP (r=0,505; r=0,367; r=0,313).Kesimpulan: Androsteron, etiokolanolon dan rasio A/E mempunyai kadar yang lebih tinggi pada pasien HAK. Androsteron mempunyai korelasi sedang, etiokolanolon dan rasio A/E mempunyai korelasi lemah terhadap 17-OHP.

---

Congenital adrenal hyperplasia (CAH) is a disorder characterized by defects in one of the enzymes of the adrenal steroidogenesis pathway. More than 90% of cases are due to mutations in CYP21A2, the gene coding for 21-hydroxylase (21-OH) enzyme. Treatment monitoring in CAH patients is quite difficult to achieve due to fine balance of overtreatment and undertreatment.

Cross sectional study was conducted in RSCM for 7 months (June-December 2020). Consecutive sampling was used with total 142 samples. There were 71 patients CAH were included in this study. Control samples were selected by matching age and sex. In CAH group, 17-hydroxyprogesterone (17-OHP), urine androsterone, etiocholanolone, and ratio androsterone/etiocholanolone (A/E) were measured. Correlations were measured between androsterone, etiocholanolone, ration A/E with 17-OHP. In control sample urine androsterone, etiocholanolone, and ratio androsterone/etiocholanolone (A/E) were also measured. These results were compared between two groups.

In 71 CAH group and 71 control group had almost same characteristics. Androsterone level in CAH group had a significant different compared to the control group (683.89(29.42-61061.43) vs 123.97(30.16-16463.05) ng/mL;p<0.001). Etiocholanolone level in CAH group had a significant different compared to the control group (235.88(12.77-78446.65) vs 70.96(12.61- 17332.62)ng/mL; p<0.001). Ratio A/E n CAH group had a significant different compared to the control group (2.31(0.37-40.12) vs 1.99(0.52-5.45); p=0.003). Androsterone, etiocholanolone and ratio A/E had positive correlation with 17-OHP level (r=0.5050; r=0.367; r=0.313).

Conclusions: Androsterone, etiocholanolone, and ratio A/E had higher level in CAH subjects. Androsterone had intermediate correlation with 17-OHP, meanwhile etiocholanolone and ratio A/E had weaker correlation."