Faktor  Risiko  Timbulnya  Inhibitor  Faktor  VIII  pada Anak dengan Hemofilia A

Simatupang, Grace N.A.; Windiastuti, Endang; Oswari, Hanifah

doi:10.14238/sp14.5.2013.320-5

Cited by 2 publications

(6 citation statements)

References 18 publications

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“…The incidence of inhibitor formation was higher in those who received replacement therapy before age of 6 months. There was 41%, 29%, and 12% increase of inhibitors if replacement therapy was given at age of <6 months, between 6-12 2,8,22 months, and> 12 months.…”

Section: Resultsmentioning

confidence: 96%

“…The process of inhibitors formation is multifactorial, consisting of genetic and environmental factors. Several studies have been performed to determine the factors that play a role in the emergence of inhibitors, but there 8,18,19 are still differences of opinion.…”

Section: Resultsmentioning

confidence: 99%

“…This study showed the 17 same results with a cohort study by Gouw et al The difference of the results in some of these studies were possibly due to the type of replacement therapy or the mutation of certain genes. The most common type of gene mutation in patients treated at an early age is 22 inversions, therefore, it was suspected that these gene mutations mostly 6,8,22 affected the emergence of inhibitors.…”

Section: Resultsmentioning

confidence: 99%

“…Prevalence of factor VIII inhibitors in hemophilia patients varies greatly, but overall ranges are from 3.6% to 52%. The prevalences of inhibitors in Taiwan, Jordan, and Saudi Arabia are 10.4%, 9%, 9.7%, respectively, whereas the prevalences in 1,[6][7][8] Indonesia vary from 35% to 37.5%. Inhibitors are problem in management of hemophilia because 2 treatment becomes difficult and expensive.…”

Section: Introductionmentioning

confidence: 99%

“…The factor VIII Inhibitors are immunoglobulin G which can neutralize factor VIII activity in hemophilia 3,4,8,9 patients.…”

Section: Introductionmentioning

confidence: 99%

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Non-Genetic Risk Factors for The Formation of Factor VIII Inhibitors in Hemophilia A Patients in RSUD Dr. Soetomo

Fauzi¹,

Larasati²,

Ugrasena³

2019

Indonesian J. Clin. Pathol. Med. Lab.

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Background: Neutralizing alloantibodies (inhibitors) is one of the complications that affect the morbidity and quality of life of hemophilia patient and can be caused by multifactorials. Genetic and inhibitors examination are not routine and expensive. Therefore, identification of non-genetic factors is important to predict the formation of inhibitors. Objective: To analyze non-genetic risk factors for the formation of factor VIII inhibitors in children with Hemophilia A. Methods: A cross-sectional study of hemophilia children aged 1-18 years at the pediatric hematology oncology outpatient clinic of Dr. Soetomo hospital in March-April 2018. Factors analyzed included the severity of hemophilia, early age of diagnosis, initial age of therapy, type of replacement therapy, frequency of factor VIII administration, and severity of bleeding by bleeding score. Statistical analysis using Chi square, Fisher, ANOVA and logistic regression analysis. Results: A total of 29 children were evaluated, 7/29 mild, 15/29 moderate, and 7/29 severe hemophilia. Inhibitors were found in 11/29 subjects, 7/11 low (1-5 BU) and 4/11 high titer (> 5 BU). The initial age of diagnosis ≤ 1 year is associated with the formation of factor VIII inhibitors (OR 8.75; 95% CI = 1.5-50.2; p = 0.015). Severity of hemophilia, early age of therapy, type of replacement therapy, frequency of therapy, and bleeding score was not significantly associated with the formation of factor VIII inhibitors. Conclusion: The initial age of diagnosis less than 1 year is a significant risk factor for the formation of factor VIII inhibitors.

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Section: Resultsmentioning

confidence: 96%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…The factor VIII Inhibitors are immunoglobulin G which can neutralize factor VIII activity in hemophilia 3,4,8,9 patients.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Non-Genetic Risk Factors for The Formation of Factor VIII Inhibitors in Hemophilia A Patients in RSUD Dr. Soetomo

Fauzi¹,

Larasati²,

Ugrasena³

2019

Indonesian J. Clin. Pathol. Med. Lab.

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Serum TNF-α Level as a Possible Predictor of Inhibitor Levels in Severe Hemophilia A

Susanah

Raspati

Sari

et al. 2021

BioMed Research International

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Background. The development of factor VIII (FVIII) inhibitor in patients with hemophilia A (PWHA) is a great challenge for hemophilia care. Both genetic and environmental factors led to complications in PWHA. The development of inhibitory antibodies is usually induced by the immune response. Tumor necrosis factor α (TNF-α), one of the cytokines, might contribute to its polymorphism. In this study, we investigated the clinical factors, level of serum TNF-α, and polymorphism of c . − 308 G > A TNF − α gene in inhibitor development in severe PWHA. Methods. A cross-sectional study was conducted among all PWHA in West Java province. The clinical parameters, FVIII, FVIII inhibitor, and serum TNF-α level were assessed. The genotyping of − 380 G > A TNF-α gene polymorphism was performed using polymerase chain reaction and Sanger sequencing. Results. Among the 258 PWHA, 216 (83.7%) were identified as severe PWHA. The FVIII inhibitor was identified in 90/216 (41.6%) of severe PWHA, consisting of 45 high-titer inhibitors (HTI) and 45 low-titer inhibitors (LTI). There was a significant correlation between serum TNF-α level and the development of HTI ( p = 0.043 ). The cutoff point of serum TNF-α level, which can be used to differentiate between HTI and LTI, was 11.45 pg/mL. The frequency of FVIII replacement therapy was significant only in HTI of severe PWHA regarding serum TNF-α level ( p = 0.028 ). There is no correlation between polymorphisms of − 380 G > A TNF-α gene and inhibitor development ( p = 0.645 ). Conclusions. The prevalence of FVIII inhibitor in severe PWHA in West Java, Indonesia, was 41.6%. The frequency of replacement therapy is a risk factor for inhibitor development. Serum TNF-α level might be used to differentiate between high and low inhibitor levels in severe hemophilia A, and this might support decision making regarding treatment options for inhibitor in severe hemophilia A.

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Faktor Risiko Timbulnya Inhibitor Faktor VIII pada Anak dengan Hemofilia A

Cited by 2 publications

References 18 publications

Non-Genetic Risk Factors for The Formation of Factor VIII Inhibitors in Hemophilia A Patients in RSUD Dr. Soetomo

Non-Genetic Risk Factors for The Formation of Factor VIII Inhibitors in Hemophilia A Patients in RSUD Dr. Soetomo

Serum TNF-α Level as a Possible Predictor of Inhibitor Levels in Severe Hemophilia A

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