Maria Abdulsalam, Maria
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The prevalence of insulin resistance in patients with β -thalassemia major at Cipto Mangunkusumo Hospital

Paediatrica Indonesiana Vol 43 No 4 (2003): July 2003
Publisher : Indonesian Pediatric Society

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Abstract

Background Diabetes mellitus is a common complication in pa-tients with thalassemia major. Iron overload plays an important roleby damaging the pancreatic β-cell and the liver cell, with the con-sequences of insulin deficiency and insulin resistance. Family his-tory of diabetes mellitus is one of the critical factors for the devel-opment of glucose metabolism derangement. However, the patho-genesis of glucose metabolism derangement remains unclear.Objective To evaluate the prevalence of impaired glucose toler-ance, diabetes mellitus, and insulin resistance in patients with β-thalassemia major treated in the Thalassemia Outpatient Clinic,Department of Child Health, Cipto Mangunkusumo Hospital,Jakarta.Methods This was a descriptive cross sectional study conductedin May 2002. Forty-eight subjects aged 10 to 18 years, grouped bytotal volume of transfusions and family history of diabetes mellitus,underwent an oral glucose tolerance test (OGTT), serum transfer-rin saturation, and insulin level examinations. Insulin resistancewas calculated from fasting plasma glucose and insulin concen-trations using the homeostasis model assessment (HOMA).Results One of 48 patients (2%) had impaired glucose toleranceat the age of 17 years. Diabetes mellitus occurred in three of 48patients (6%) at the age of 15.5 years in one patient and 18 yearsin two patients. Family history of diabetes mellitus was found in 2patients with diabetes mellitus and in the only one with impairedglucose tolerance. Insulin resistance was not detected in this study.Conclusion The prevalence of glucose metabolism derangementin patients with thalassemia major was low. No insulin resistancewas found in this study

Osteopetrosis

Sari Pediatri Vol 6, No 2 (2004)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

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Abstract

Dilaporkan kasus osteopetrosis maligna pada seorang anak perempuan berusia 3 tahun.Kelainan yang diturunkan secara otosomal resesif ini disebabkan defek pada osteoklas.Pasien datang dengan keluhan pucat dan abses submandibula dengan perdarahan, disertaigigi yang mudah patah dan kebutaan sejak lahir, terdapat frontal bossing, adenoidappearance disertai nistagmus, serta hepatosplenomegali. Pemeriksaan darah tepimenunjukkan pansitopenia dengan gambaran leukoeritroblastosis. Aspirasi sumsumtulang sulit dilakukan karena kerasnya jaringan tulang, memperlihatkan gambaranhipoplasia sistem eritropoietik dan trombopoietik. Diagnosis ditegakkan berdasarkanbone survey yang memperlihatkan keseluruhan tulang yang sklerotik dengan peningkatandensitas tulang, disertai tanda khas berupa mask sign pada tulang tengkorak dan sandwichsign pada tulang vertebra. Dilakukan terapi suportif berupa transfusi sel darah merahdan trombosit, pengobatan medikamentosa berupa prednison dan kalsitriol. Prognosispenyakit ini buruk, dengan angka kematian yang tinggi pada dekade pertama kehidupansebagai akibat perdarahan dan infeksi berulang.

Diagnosis, Pengobatan dan Pencegahan Anemia Defisiensi Besi

Sari Pediatri Vol 4, No 2 (2002)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

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Abstract

Anemia defisiensi besi (ADB) masih merupakan suatu masalah kesehatan di Indonesia.Hasil survai rumah tangga tahun 1995 ditemukan 40,5% anak balita dan 47,2% anakusia sekolah menderita ADB. Anemia defisiensi besi dapat menyebabkan terjadinyaberbagai komplikasi antara lain berupa gangguan fungsi kognitif, penurunan daya tahantubuh, tumbuh kembang yang terlambat, penurunan aktivitas, dan perubahan tingkahlaku. Oleh karena itu masalah ini memerlukan cara penanganan dan pencegahan yangtepat. Diagnosis ditegakkan berdasarkan adanya gejala pucat menahun tanpa disertaiperdarahan maupun organomali. Pemeriksaan darah tepi menunjukkan anemia mikrositerhipokrom, sedangkan jumlah leukosit, trombosit dan hitung jenis normal. Diagnosisdipastikan dengan pemeriksaan kadar besi dalam serum. Pemberian preparat besi secaraselama 3-5 bulan ditujukan untuk mengembalikan kadar hemoglobin dan persediaanbesi di dalam tubuh ke keadaan normal. Mencari dan mengatasi penyebab merupakanhal yang penting untuk mencegah kekambuhan. Antisipasi harus di lakukan sejak pasiendalam stadium I (stadium deplesi besi) dan stadium II (stadium kekurangan besi).Dianjurkan pula untuk memberikan preparat besi pada individu dengan risiko tinggiuntuk terjadinya ADB antara lain untuk individu dari keluarga dengan sosial ekonomirendah.

Masalah pada Tata Laksana Anemia Aplastik Didapat

Sari Pediatri Vol 7, No 1 (2005)
Publisher : Badan Penerbit Ikatan Dokter Anak Indonesia (BP-IDAI)

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Abstract

Anemia aplastik adalah kegagalan sumsum tulang baik secara fisiologis maupun anatomis.Penyakit ini ditandai oleh penurunan atau tidak ada faktor pembentuk sel darah dalamsumsum tulang, pansitopenia darah perifer, tanpa disertai hepatosplenomegali ataulimfadenopati. Penanganan anemia aplastik masih merupakan masalah yang penting karenapatofisiologi penyakit ini masih belum pasti. Tata laksana anemia aplastik terdiri dari tatalaksana suportif terhadap keadaan yang disebabkan oleh pansitopenia seperti anemia, infeksidan perdarahan, serta tata laksana serta pengobatan yang bertujuan untuk mengganti selinduk yang gagal dalam memproduksi sel-sel darah dan menekan proses imunologis yangterjadi. Tata laksana kuratif terdiri dan transplantasi sumsum tulang dan penggunaanobat-obat imunosupresan. Namun demikian tata laksana anemia aplastik baik yang bersifatsuportif maupun kuratif, dapat menimbulkan masalah-masalah yang mempengaruhiprognosis pasien. Prognosis pasien anemia aplastik umumnya buruk, sekitar dua pertigapasien meninggal setelah 6 bulan diagnosis ditegakkan sebagai anemia aplastik.

Immunosuppressive agents in the treatment of chronic idiopathic thrombocytopenic purpura

Paediatrica Indonesiana Vol 13, No 3 (1973): March 1973
Publisher : Indonesian Pediatric Society

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Abstract

Acute idiopathic thrombocytopenic purpura (I. T.P.) in childhood is acondition with a self-limiting course that will usually improve spontaneously. In severe cases, however, blood transfusion and corticosteroids are indicated. Lusher and Zuelzer (1966) who analysed 152 cases of acute I.T.P. in children concluded that 92.6% of the untreated as well as 97.5% of the corticosteroid-treated gave uneventful recovery without recurrence of thrombocytopenia. On the other hand, in chronic I.T.P., where the immune mechanisms playa more important role than in acute I.T.P., administration of corticosteroids seems of little value. Harrington et al. (1951) found the presence of platelet antibodies in 8 out of 10 cases of chronic I.T.P. The importance of platelet antibodies which either destroy or inhibit platelet formation from megakaryocytes in the bone marrow; has been demonstrated by other investigators (Kuramoto, 1966; Rolovic et aI., 1970). 

Nutritional status changes in children with malignant solid tumor before and after chemotherapy

Paediatrica Indonesiana Vol 45 No 4 (2005): July 2005
Publisher : Indonesian Pediatric Society

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Abstract

Background Although aggressive multimodal treatment programsin childhood cancer have significantly increased survival rates, themorbidity caused by protein energy malnutrition related to therapyis still high.Objective To describe nutritional status changes in children withmalignant solid tumors after 21 days of chemotherapy.Methods A descriptive prospective study with pre- and post-testdesign in children with malignant solid tumors was conducted inthe Department of Child Health, Medical School University of In-donesia/Cipto Mangunkusumo Hospital, Jakarta between Janu-ary and July 2004. Anthropometrics (body weight, BW and mid-upper-arm circumference, MUAC) and serum albumin measure-ments were performed before and after 21 days of chemotherapy.Results Twenty-two children were enrolled in this study. After 21days of chemotherapy, 8 children had decreased BW and 6 chil-dren had decreased MUAC, but 3 children gained weight and hadincreased MUAC. Based on MUAC-for-age, 7 children had de-creased nutritional status. Fifteen children had reduced serum al-bumin levels based on a 10% cut-off point. The number of childrenwho had reduced serum albumin was larger than those who hadreduced BW and MUAC. In the evaluation of average oral foodconsumption during 21 days, 7 out of 16 children could acceptmore than 2/3 portion of served food. All of the children who re-ceived enteral feeding could accept more than 2/3 portion of servedfood.Conclusion There was a decrease of nutritional status, BW,MUAC, and serum albumin in most of the subjects after chemo-therapy. Serum albumin level measurement was the more sensi-tive parameter in determining nutritional status changes. Enteralfeeding seems more appropriate to fulfill nutritional needs than oralfeeding

Osteopetrosis: A Report of Four Cases

Paediatrica Indonesiana Vol 24 No 9-10 (1984): September 1984
Publisher : Indonesian Pediatric Society

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Abstract

Four cases of osteopetrosis in infancy had been reported. All cases showed signs of anemia, thrombocytopenia and hepatosplenomegaly which at the beginning suggested the possibility of the diseases more commonly found in our country such as malaria, Cooley anemia, autoimmunne hemolytic anemia and malignancy. The authors then concluded that next to those diseases mentioned above the symptoms and signs of anemia, thrombocytopenia and hepatosplenomegaly especially in infancy should also lead to the possibility of the diagnosis of osteopetrosis. This latter is more obvious when it is accompanied by the difficulty in obtaining the bone marrow specimen.