Gall bladder contractility in children with beta-thalassaemia

Nasr, Mona Ramadan; Shaker, Mohamed; Mahdy, Hamdy; Hafez, Asmaa Abdel

doi:10.26719/2009.15.2.315

Cited by 4 publications

(1 citation statement)

References 18 publications

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“…Biliary sludge (pelumpuran pada kandung empedu) merupakan keadaan untuk terjadinya kolelitiasis. [4][5][6][7][8][9] Sejak tahun 1990-an sejumlah penelitian telah dilakukan di Mesir, Iran, Thailand, dan Turki untuk mempelajari kejadian kolelitiasis dan biliary sludge pada kasus talassemia anak, terutama talassemia mayor dan intermedia. Insidens yang ditemukan bervariasi antara 8% sampai 18%, yang semakin meningkat dengan bertambahnya usia.…”

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Hubungan Kekerapan Transfusi Darah dengan Kejadian Kolelitiasis dan Biliary Sludge pada Pasien Talassemia Mayor Anak

Jaya

Reniarti

Chairulfatah

2016

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Latar belakang. Kolelitiasis (batu kandung empedu) merupakan penyakit kandung empedu yang dapatterjadi pada talassemia. Biliary sludge (pelumpuran kandung empedu) merupakan keadaan yang dapatmenjadi kolelitiasis. Perubahan fungsi kandung empedu akibat penumpukan besi dan peningkatan kadarbilirubin serum akan mempermudah terjadinya kolelitiasis dan biliary sludge.Tujuan.Mengetahui hubungan kekerapan transfusi darah dengan kejadian kolelitiasis dan biliary sludgepada talassemia mayor anak.Metode. Penelitian menggunakan rancangan analitik cross-sectional. Subjek penelitian adalah pasienthalassemia mayor anak di Poliklinik Talassemia RS Hasan Sadikin Bandung berusia 5−14 tahun pada bulanJuni−Agustus 2010. Pada setiap subjek dilakukan anamnesis, pemeriksaan fisis, pemeriksaan kadar feritindan bilirubin indirek serum serta pemeriksaan ultrasonografi hepatobilier. Analisis statistik menggunakanuji komparasi eksak Fisher, uji t tidak berpasangan dan uji Mann-Whitney.Hasil. Sejumlah 60 subjek memenuhi kriteria penelitian. Kadar rata-rata feritin serum dan bilirubin indirekserum pada subjek yang mengalami kolelitiasis dan biliary sludge dibandingkan dengan yang tidak mengalami,tidak ditemukan perbedaan yang bermakna secara uji statistik (5682 vs 5113,9; 1,19 vs 1,12; p>0,05). Didapatkan6/60 subjek yang mengalami kolelitiasis dan biliary sludge, yaitu 3/60 subjek mengalami kolelitiasis,2/60 subjek mengalami biliary sludge dan 1/60 subjek mengalami kolelitiasis dan biliary sludge Ditemukanperbedaan yang bermakna pada kejadian kolelitiasis dan biliary sludge antara pasien yang mendapat intervaltransfusi darah 􀁤3 minggu dibandingkan dengan >3 minggu (p<0,05).Kesimpulan. Terdapat hubungan kekerapan transfusi darah dengan kejadian kolelitiasis dan biliary sludgepadatalassemia mayor anak.

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Hubungan Kekerapan Transfusi Darah dengan Kejadian Kolelitiasis dan Biliary Sludge pada Pasien Talassemia Mayor Anak

Jaya

Reniarti

Chairulfatah

2016

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Clinicopathological and Radiological Study of Egyptian β-Thalassemia Intermedia and β-Thalassemia Major Patients: Relation to Complications and Response to Therapy

et al. 2011

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The clinico epidemiological characteristics, frequency of complications, and response to various therapeutic modalities in 80 Egyptian β-thalassemia intermedia (β-TI) patients were compared with 70 β-thalassemia major (β-TM) patients. β-Thalassemia intermedia patients had a higher incidence of left atrium dilatation, right ventricular dilatation and pulmonary hypertension, whereas, β-TM patients showed a higher incidence of left ventricular (LV) dilatation, restrictive LV filling and impaired LV contractility, with an overall higher incidence of heart disease (p <0.001). Short stature, delayed puberty, osteoporosis, bone fractures, diabetes mellitus and viral hepatitis was frequently observed in β-TM patients compared with β-TI patients (p <0.05). Administration of hydroxyurea (HU) alone was associated with significant improvement in hematological parameters and quality of life for β-TI patients. In conclusion, the risk of complications still burdens the life of Egyptian thalassemia patients and their frequency varies between β-TI and β-TM. We provide evidence that calls for the use of HU in β-TI patients.

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Descriptive clinical study of children with beta-thalassemia at Damanhour Medical National Institute

Monem

Shehata

El-Sayed

et al. 2021

Alexandria Journal of Pediatrics

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Background Thalassemia is a genetic disorder of hemoglobin synthesis. Beta-thalassemia is particularly prevalent among Mediterranean people. Long-term transfusion therapy and iron chelating agents are the mainstay treatments of thalassemia patients. Aim The aim of this study was to identify the demographic, clinical, laboratory profile, and therapeutic features of B-thalassemia patients attending the Pediatric Hematology Clinic at Damanhour Medical Institute. Patients and methods This study included 74 thalassemia patients in the age range of 2–16 years, mostly from rural areas (el Behera Governorate). The total number of thalassemia patients followed up in the Pediatric Hematology Unit at Damanhour Medical Institute is up to 400 cases; the patients of this study were selected randomly from among them. Results Dysmorphic features were demonstrated in 44% of cases; 74% of cases were receiving packed red blood cells every month and 26% were receiving packed red blood cells more than once a month. Oral iron chelator (Deferasirox) was the main chelator used. Most of the patients had serum ferritin greater than 1000 µg/ml; thyroid dysfunction was evident in 21% of cases, with no definite clinical manifestations.

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Gall bladder contractility in children with beta-thalassaemia

Cited by 4 publications

References 18 publications

Hubungan Kekerapan Transfusi Darah dengan Kejadian Kolelitiasis dan Biliary Sludge pada Pasien Talassemia Mayor Anak

Hubungan Kekerapan Transfusi Darah dengan Kejadian Kolelitiasis dan Biliary Sludge pada Pasien Talassemia Mayor Anak

Clinicopathological and Radiological Study of Egyptian β-Thalassemia Intermedia and β-Thalassemia Major Patients: Relation to Complications and Response to Therapy

Descriptive clinical study of children with beta-thalassemia at Damanhour Medical National Institute

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