Transient elastography of the liver in children with Wilson's disease: Preliminary results

Stefanescu, Ana Cristina; Pop, Tudor Lucian; Ştefănescu, Horia; Miu, Nicolae

doi:10.1002/jcu.22281

Cited by 23 publications

(18 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…4 A few studies have demonstrated the potential of ultrasound (US) elastography and serologic markers as noninvasive monitoring modalities for WD. [5][6][7][8] Ultrasound elastographic techniques have been validated as accurate diagnostic tools for liver fibrosis. 9,10 However, because of the rarity of the disease, these techniques have not been widely researched in the context of WD, with only a few available studies [6][7][8] on transient elastography (TE) and acoustic radiation force impulse imaging.…”

mentioning

confidence: 99%

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Hwang

Yoon

Jung

et al. 2020

J of Ultrasound Medicine

View full text Add to dashboard Cite

Objectives-To investigate the diagnostic value of transient elastography (TE), 2-dimensional (2D) shear wave elastography (SWE), and the serologic fibrosis indices aspartate transaminase-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4) score for Wilson disease (WD). Methods-We retrospectively identified patients with a diagnosis of WD who underwent TE and 2D SWE on the same day. Their APRI and FIB-4 scores were calculated. Hepatic involvement was classified into 5 clinical categories (I-V) based on the laboratory findings, hepatic morphologic characteristics on ultrasound (US) imaging, and clinical symptoms of cirrhosis: I, normal (n = 17); II, only biochemical abnormality (n = 15); III, altered hepatic morphologic characteristics (n = 10); IV, compensated liver cirrhosis (n = 3); and V, decompensated liver cirrhosis (n = 0). We compared the area under the receiver operating characteristic curve (AUROC) data for TE, 2D SWE, the APRI, and the FIB-4 score. A combined assessment of the serologic markers and US elastography was performed, and the AUROCs of the combinations were compared. Results-Forty-five patients were included in the study (median age, 16.0 years; range, 3-35 years). Transient elastography, 2D SWE, and APRI were comparable in distinguishing the clinical categories (AUROC, 0.799-0.928). The FIB-4 score showed lower diagnostic value in distinguishing clinical category I from the other categories (AUROC, 0.647). Combining the serologic markers and US elastography significantly increased the AUROC value of the FIB-4 score (with TE and 2D SWE, P = .01 and .02). Conclusions-Transient elastography and 2D SWE showed excellent diagnostic accuracy for differentiating the clinical categories of hepatic involvement. The APRI showed better diagnostic performance than the FIB-4 score. The assessment of hepatic manifestations in WD can be improved by combining US elastography with serologic indices. Key Words-elasticity imaging techniques; liver fibrosis; ultrasound; Wilson disease W ilson disease (WD) is a congenital copper metabolism disorder that results in liver cirrhosis and neuropsychological deterioration due to copper accumulation in the liver and central nervous system. 1 Wilson disease is the manifestation of an abnormal ATP7B (ATPase copper-transporting beta) gene, which

show abstract

mentioning

confidence: 99%

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Hwang

Yoon

Jung

et al. 2020

J of Ultrasound Medicine

View full text Add to dashboard Cite

show abstract

“…Trabut et al (15) showed that the Fibroscan value of 137 patients who were hospitalized for the purpose of giving up alcohol decreased from 7,2 kPa to 6,1 kPa only in 7 days on which the subjects did not drink alcohol. Stefanescu et al (16) showed that the Fibroscan values decreased with the copper chelation treatment in patients with Wilson's disease with no fibrosis. According to these studies, it is understood that the accumulation diseases in except to fibrosis also increases stiffness of the liver, and may be detected with Fibroscan.…”

Section: Discussionmentioning

confidence: 99%

Evaluation the Iron Load in Chronic Hemodialysis Patients with Fibroscan: A Preliminary Study

Altunören¹,

Gişi²,

Yılmaz³

et al. 2017

Turk Neph Dial Transpl

View full text Add to dashboard Cite

OBJECTIVE: Iron preperations are frequently used in hemodalysis (HD) patients and iron may accumulate liver and other tissues. Noninvasively methods are needed to detect liver iron accumulation in HD patients. Hepatic iron accumulation with Fibroscan was shown in non-renal population. In this study, we aim to investigate whether or not the hepatic iron load can be determined with Fibroscan in hemodialysis patients. MATERIAL and METHODS:42 chronic HD patients included this study. Serum iron, iron binding capasity, ferritin and transferrin saturation were used to determine iron status. Liver stiffness was measured by an experienced gastroenterolog with Fibroscan device. RESULTS:While mean Fibroscan value was 9,15 kPa, ferritin was 565,99 mg/dl. Fibroscan value were positive correlated with transferrin saturation and ferritin but negative correlated with albumin. In multiple regression analysis, it was observed that only ferritin and albumin levels related with Fibroscan value. CONCLUSION:This study showed that the stiffness of the liver increasing while the ferritin value increasing. This result might be due to iron accumulation in the liver.KEy wORDS: Anemia, Fibroscan, Hemodialysis, Hepatic iron Öz AMAÇ: Hemodiyaliz (HD) hastalarında demir preparatları sıklıkla kullanılmaktadır ve belkide bu demir karaciğer ve diğer organlarda birikmektedir. HD hastalarında karaciğerde demir birikimini noninvaziv olarak değerlendirecek bir metoda ihtiyaç vardır. Non renal hasta popülasyonunda Fibroscan ile karaciğer demir yükünü değerlendiren çalışmalar mevcuttur. Çalışmada Fibroscan ile HD hastalarında karaciğer demir yükünün ölçülüp ölçülemeyeceğini araştırdık. GEREÇ ve yÖNTEMLER:Çalışmaya 42 kronik HD hastası dahil edildi. Demir içeriği, serum demir, demir bağlama kapasitesi, transferin satürasyonu ve ferritin değeri ölçülerek değerlendirildi. Karaciğer sertliği tecrübeli bir gastroenterolog tarafından Fibroscan cihazı ile ölçüldü. BULGULAR:Ortalama Fibroscan değeri 9,15 kPa, ferritin ise 565,99 mg/dl idi. Fibroscan değeri transferin satürasyonu ve ferritin ile pozitif, albümin ile negatif korele idi. Çoklu regresyon analizinde sadece ferritin ve albümin Fibroscan değeri için belirleyici olarak bulundu. SONUÇ:Çalışma serum ferritin değeri arttıkça karaciğer sertliğinin arttığını gösterdi. Bu, karaciğerde demir birikimin bir sonucu olabilir.

show abstract

“…However, both TE and APRI/FIB‐4 have been mostly validated for fibrosis staging in viral liver disease and in non‐alcoholic fatty liver disease . Data on non‐invasive methods to stage fibrosis in WD are scarce . After long discussions with Food and Drug Administration (FDA) and European Medicines Agency (EMA) it became clear that our armamentarium to predict the outcome of liver disease (ie surrogate endpoints) in WD is very limited.…”

Section: Introductionmentioning

confidence: 99%

“…10,13,14 Data on non-invasive methods to stage fibrosis in WD are scarce. 15 After long discussions with Food and Drug Administration (FDA) and…”

Section: Introductionmentioning

confidence: 99%

Non‐invasive diagnosis of cirrhosis and long‐term disease monitoring by transient elastography in patients with Wilson disease

Paternostro

Pfeiffenberger

Ferenci

et al. 2020

Liver International

View full text Add to dashboard Cite

Background & Aims The value of liver stiffness measurement (LSM) by transient elastography (TE) for non‐invasive fibrosis staging and disease monitoring has not been established in patients with Wilson disease (WD). Methods Liver stiffness measurement by TE and non‐invasive fibrosis scores (APRI, FIB‐4) were analysed from 188 WD patients with liver biopsy (LBX). Longitudinal LSM was performed in 128 (68.1%) patients. Results One hundred and eighty‐eight patients (mean age: 35 ± 14 years, 54.8% women; 27.1% with histological cirrhosis) were studied. Forty‐four[23.4%] patients were recently diagnosed with WD, while 144[76.6%] were previously diagnosed (>1 year between LBX and LSM). Overall, LSM (11.3 vs 6.1 kPa, P < .001), APRI (0.72 vs 0.38, P < .001) and FIB‐4 (1.54 vs 0.89, P < .001) were higher in cirrhotic than in non‐cirrhotic patients. This was even more pronounced in recently diagnosed patients (35.2 kPa vs 6.4 kPa, P < .001). Accuracy for diagnosing cirrhosis at an LSM cut‐off ≥9.9 kPa was better in recently diagnosed (PPV: 74%, NPV: 100%) vs previously diagnosed (PPV: 53%, NPV: 82%) patients. Recently diagnosed patients had higher Area Under the Curve (AUC) for APRI (0.79 vs 0.61) and FIB‐4 (0.84 vs 0.65) than previously diagnosed patients. At APRI <1.5 and FIB‐4 <3.25 cirrhosis was ruled out with a specificity of 93% and 95% respectively. During a median follow‐up of 46 (24‐66) months, only 5.9% (5/85) of non‐cirrhotic WD patients showed progression to cirrhotic LSM values, while 30.8% (4/13) of cirrhotic WD patients showed LSM suggestive of cirrhosis regression. Conclusion TE‐based LSM ≥9.9 kPa accurately identifies cirrhosis in WD patients. Next to TE‐LSM <9.9 kPa, APRI <1.5 and FIB‐4 <3.25 values assist to non‐invasively rule out cirrhosis. LSM remains stable in most non‐cirrhotic patients on WD therapy, while one‐third of cirrhotic patients present clinically relevant decreases in LSM.

show abstract

Transient elastography of the liver in children with Wilson's disease: Preliminary results

Abstract: In pediatric patients with WD, LS is high at the time of diagnosis and decreases during specific treatment, in parallel with an increase in the urinary copper concentration.

Cited by 23 publications

References 28 publications

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Diagnostic Performance of Ultrasound Elastography and Serologic Fibrosis Indices for Evaluation of Hepatic Involvement in Wilson Disease

Evaluation the Iron Load in Chronic Hemodialysis Patients with Fibroscan: A Preliminary Study

Non‐invasive diagnosis of cirrhosis and long‐term disease monitoring by transient elastography in patients with Wilson disease

Contact Info

Product

Resources

About