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Incidence of Peripheral Neuropathy in Major Beta-Thalassemia Patients at Hasan Sadikin General Hospital, Bandung, Indonesia Putri, Fanny Adhy; Gamayani, Uni; Lailiyya, Nushrotul; Panigoro, Ramdan
Cermin Dunia Kedokteran Vol 46, No 9 (2019): Neuropati
Publisher : PT. Kalbe Farma Tbk.

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (610.493 KB) | DOI: 10.55175/cdk.v46i9.416

Abstract

Bakground. Beta major thalassemia is the most common inherited blood disorder worldwide. It can lead to various neurological complications such as peripheral neuropathy. Toronto Clinical Neuropathy Score (TCNS) is helpful for peripheral neuropathy screening especially in diabetes mellitus. Objective. To investigate the prevalence of peripheral neuropathy in beta major thalassemia patient using Toronto Clinical Neuropathy Score (TCNS). Method. A descriptive study on beta major thalassemia patients aged more than 14 years who regularly underwent blood transfusions in Hasan Sadikin General Hospital Bandung, from July to August 2017. Normal TCNS values was < 4, mild neuropathy 5-7, moderate neuropathy 8-10 and severe neuropathy > 10. Results. Sixty subjects met the inclusion criteria, 48,3% were male with the mean (SD) age of 20,7 ± 7,6 years. Mean hemoglobin value was 6,7 ± 0,9 g/dL and median (IQR) blood ferritin serum was 2873 (1900-3859) μg/L. Thirty-two subjects had neuropathy; 19 (31,7%) with mild neuropathy and 13 (21,6%) with moderate neuropathy.Conclusion. The incidence of peripheral neuropathy in patients with thalassemia according to TCNS score is fairly high.Latar Belakang. Talasemia beta mayor adalah kelainan darah bawaan paling umum di dunia dan dapat menyebabkan berbagai komplikasi, salah satunya neuropati perifer. Toronto Clinical Neuropathy Score (TCNS) dapat digunakan untuk penilaian neuropati perifer yang disebabkan oleh berbagai penyakit sistemik. Objektif. Menyelidiki prevalensi neuropati perifer pada pasien talasemia beta mayor menggunakan Toronto Clinical Neuropathy Score (TCNS). Metode. Penelitian ini studi deskriptif skrining menggunakan TCNS pada pasien thalassaemia beta mayor berusia lebih dari 14 tahun yang secara teratur menjalani transfusi darah di Rumah Sakit Umum Hasan Sadikin Bandung, dari Juli hingga Agustus 2017. Nilai TCNS normal adalah <4, neuropati ringan 5-7, neuropati sedang 8-10 dan neuropati berat> 10. Hasil. Sebanyak 60 subjek memenuhi kriteria inklusi, 48,3% laki-laki dengan usia rata-rata (SD) 20,7 ± 7,6 tahun. Nilai hemoglobin rata-rata 6,7 ± 0,9 g / dL dan serum ferritin darah median (IQR) adalah 2873 (1900-3859) μg / L. Tiga puluh dua subjek memiliki neuropati; 19 (31,7%) neuropati ringan dan 13 (21,6%) neuropati sedang. Simpulan. Insiden neuropati perifer pada pasien dengan talasemia menurut skor TCNS cukup tinggi.
HUBUNGAN KADAR HEMOGLOBIN DAN FERITIN DENGAN GAMBARAN KONDUKSI SARAF PADA ANAK TALASEMIA BETA MAYOR Dewi, Windy Krisanti Kusuma; Gamayani, Uni; Lailiyya, Nushrotul; Reniarti, Lelani; Sekarwana, Nanan
Majalah Kedokteran Neurosains Perhimpunan Dokter Spesialis Saraf Indonesia Vol 35 No 1 (2018)
Publisher : PERDOSNI

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.52386/neurona.v35i1.42

Abstract

    CORRELATION BETWEEN HAEMOGLOBINE AND FERRITIN LEVEL WITH NERVE CONDUCTION STUDY IN CHILDREN WITH BETA THALASSEMIA MAJORABSTRACTIntroduction: Beta thalassemia major is inherited hemoglobin synthesis disorder found in thalassemia belt, including Indonesia. Peripheral neuropathy is one of its underdiagnosed neurological complications results in poor management of the patients.Aims: To identify correlation between hemoglobin and ferritin serum with Nerve conduction study (NCS) in Beta thalassemia major patients.Methods: This was a cross-sectional study involving children with beta thalassemia major aged 8-14 years, who regularly underwent blood transfusions in Hasan Sadikin Hospital, Bandung. Nerve conduction study of motor and sensory nerves of all limbs were conducted to the patients. Data was analyzed using Spearman’s correlation analysis.Results: Fifty subjects were included in this study with the mean hemoglobin level (SD) of 6.99 (0.87)g/dL and the mean blood ferritin level (SD) of 3.925 (1.993)μg/L. Based on NCS, most of the subjects had polyneuropathy and 46.94% had axonal demyelinating lesions in sensory and motor nerves. Among patients with neuropathy, there was a statistically significant negative correlation  between mean hemoglobin level and the numbers of the abnormal  nerves and also a statistically significant positive correlation between mean blood ferritin level and the numbers of the abnormal nerves.Discussion: The lower ferritin serum level, the less nerve involved based on NCS.Keyword: Beta thalassemia, ferritin, hemoglobin, nerve conduction study, neuropathyABSTRAKPendahuluan: Talasemia beta mayor merupakan suatu kelainan sintesis hemoglobin yang herediter yang banyak ditemukan di wilayah tropis, termasuk Indonesia. Salah satu komplikasinya adalah neuropati perifer yang sering tidak terdiagnosis, sehingga penyandangnya tidak mendapatkan tata laksana yang adekuat.Tujuan: Mengetahui hubungan antara kadar hemoglobin dan feritin serum dengan gambaran konduksi saraf (nerve conduction study/NCS) pada penyandang talasemia beta mayor.Metode: Studi potong lintang terhadap penyandang talasemia berusia 8-14 tahun yang rutin menjalani transfusi darah di RSUP Dr. Hasan Sadikin, Bandung. Dilakukan pemeriksaan NCS motorik dan sensorik pada keempat ekstremitas. Data dianalisis menggunakan uji korelasi Spearman.Hasil: Diperoleh 50 subjek dengan rerata kadar hemoglobin 6,99+0,87g/dL dan kadar feritin serum 3.925+1.993μg/L. Berdasarkan pemeriksaan NCS, sebagian besar subjek mengalami polineuropati yang 46,94% di antaranya berupa gambaran demielinasi aksonal sensorik motorik. Pada subjek dengan neuropati perifer didapatkan korelasi negatif yang bermakna antara kadar hemoglobin dengan jumlah keterlibatan saraf pada NCS dan korelasi positif yang bermakna antara kadar feritin serum dengan jumlah keterlibatan saraf pada NCS.Diskusi: Semakin rendah kadar feritin serum, maka semakin sedikit jumlah saraf yang terlibat pada NCS, dan sebaliknya.Kata kunci: Feritin, hemoglobin, nerve conduction study, neuropati, talasemia beta mayor
Perbedaan Nilai The Clinic GBS Severity Evaluation Scale (CGSES) dan Skala Disabilitas Sindroma Guillain-Barre (SDSGB) pada Pasien Sindroma Guillian Barre dengan dan tanpa Imunoterapi Sidabutar, Berliana; Ganiem, Ahmad Rizal; Lailiyya, Nushrotul; Kurniani, Nani; Amalia, Lisda; Sobaryati, Sobaryati
Jurnal Neuroanestesi Indonesia Vol 10, No 2 (2021)
Publisher : https://snacc.org/wp-content/uploads/2019/fall/Intl-news3.html

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (2999.551 KB) | DOI: 10.24244/jni.v10i2.328

Abstract

Latar Belakang dan Tujuan: The Clinical GBS Severity Evaluation Scale (CGSES) dikembangkan untuk penentuan imunoterapi pasien Sindroma Guillian Barre (SGB) secara lebih obyektif. Skala Disabilitas SGB (SDSGB) menggambarkan tingkat keparahan SGB dan dapat digunakan menilai efektifitas pemberian imunoterapi. Tujuan penelitian untuk mengetahui perbedaan penilaian CGSES dan SDSGB pasien SGB dengan dan tanpa imunoterapi dan membandingkan kesesuaian keputusan subyektif dengan CGSES. Subjek dan Metode: Penelitian observasional analitik potong lintang komparatif secara retrospektif pada pasien rawat SGB periode Januari 2015 Maret 2020 di RSUP Dr Hasan Sadikin Bandung. Hasil: Terdapat 92 subjek (35 dengan dan 57 tanpa imunoterapi). Rerata usia 41,5 tahun, dengan pria:wanita (57,6%:42,4%). Tidak didapatkan perbedaan demografi dan pemeriksaan fisik kedua kelompok, kecuali paresis saraf kranial (62,9% vs. 33,3%; p=0,006). Terdapat perbedaan rerata lama perawatan dengan dan tanpa imunoterapi (29,534,4 vs. 11,44,1 hari, p=0,0001). Hasil penilaian CGSES pasien SGB dengan dan tanpa imunoterapi memiliki perbedaan bermakna (p=0,035). Terdapat perbedaan signifikan SDSGB saat masuk dan pulang pasien dengan imunoterapi (p=0,007) dan tanpa imunoterapi (p=0,025). Terdapat ketidaksesuaian keputusan subyektif dengan nilai CGSES (nilai Kappa 0,117; CI95% 0,021-0,213)Simpulan: Terdapat perbedaan skor CGSES dan SDSGB pada kelompok pasien SGB dengan dan tanpa imunoterapi. Terdapat ketidaksesuaian penilaian subyektif keputusan pemberian imunoterapi dengan skoring CGSESDifferences in Value of The Clinic GBS Severity Evaluation Scale (CGSES) and Guillain-Barre Syndrome Disability Scale (GBSDS) in Guillian Barre Syndrome (GBS) Patients with and without ImmunotherapyAbstractBackground and objective: The Clinical GBS Severity Evaluation Scale (CGSES) was developed to determine immunotherapy of GBS patients more objectively. GBS Disability Scale (SDSGB) describes severity of GBS and assesses effectiveness of immunotherapy. Purpose of this study was to measure difference of CGSES and GBSDS in GBS patients with and without immunotherapy and to compare the suitability of subjective decisions with CGSES. Subject and Methods: This is a comparative cross-sectional analytic observational study retrospectively in GBS patients from January 2015-March 2020 hospitalized at Dr Hasan Sadikin Hospital, Bandung. Results: There were 92 subjects (35 with and 57 without immunotherapy). Mean age was 41.5 years, and male:female ratio was 57.6%:42.4%. There were no differences in demographics and physical examination between two groups, except for cranial nerve paresis (62.9% vs. 33.3%; p=0.006). There was a difference in mean length of stay with and without immunotherapy (29.5 34.4 vs. 11.4 4.1 days, p=0.0001). Results of the CGSES assessment with and without immunotherapy had a significant difference (p=0.035). There were significant differences in GBSDS at admission and discharge with (p=0.007) and without immunotherapy (p=0.025). There was a discrepancy between subjective decisions and CGSES value (Kappa value 0.117; 95% CI 0.021-0.213).Conclusion: There were differences in CGSES and GBSDS in group of GBS patients with and without immunotherapy. There was a discrepancy between subjective assessment of decision to give immunotherapy with CGSES scoring.