Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange

Hurşitoğlu, Mehmet; Kara, Osman; Çıkrıkçıoğlu, Mehmet Ali; Celepkulu, Tahsin; Aydin, Sengul; Tükek, Tufan

doi:10.1002/jca.20186

Cited by 16 publications

(10 citation statements)

References 11 publications

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“…It may sometimes mimic nonalcoholic steatohepatitis (NASH) [1,4]. It may occur as fulminant hepatic disorder or cirrhosis [1,4,5]. Extensive hepatocelullar apoptosis liberates free copper into the circulation.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…It may be confused with acute viral hepatitis or drug-induced hepatitis [4]. Correct diagnosis and urgent treatment is important and life saving [1,4,5].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…It is present in 60% of adults with WD and is seen less frequently in children. It is almost always seen in neurological cases of Wilson's disease [1,4,5,11,12]. In a study from Turkey, KFR was positive in 81,8% of the symptomatic patients, while only in 33.3% of the asymptomatic patients [15].…”

Section: Kayser-fleischer Ringmentioning

confidence: 99%

“…In long standing WD, screening for abdominal malignancies by ultrasonographic examination is indicated [22]. D-penicillamine has been proven to be effective in WD [5,[23][24][25]. Worsening of neurological symptoms is reported in 10-50% of the patients treated with penicillamine during the early phase of treatment.…”

Section: Managementmentioning

confidence: 99%

“…This is an automosal recessive disorder of hepatic copper sequence with a prevalance of 1/30000, arising from a mutation in the ATP7B gene located on chromosome 13 [2][3][4][5]. This gene was identified in 1993.…”

Section: Introductionmentioning

confidence: 99%

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Wilson’s disease

et al. 2010

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AbstractWilson’s disease is an autosomal-recessive disorder caused by mutation in the ATP7B gene. Absent or reduced function of ATP7B protein leads to decreased hepatocellular excretion of copper into bile. Subsequent copper accumulation, first in the liver but ultimately in the brain and other tissues, produces different clinical manifestations such as hepatic, neurological, hematological, ophthalmological, and psychiatric problems. Diagnosis is based on clinical suspicion, parameters of copper metabolism, ophthalmic examination (Kayser-Fleischer rings) and a liver biopsy. Genetic studies are of limited use. Early diagnosis and initiation of therapy with chelators and therapeutic plasma exchange therapy are essential for prognosis. Liver transplantation corrects the underlying pathophysiology and can be lifesaving in fulminant hepatic failure. Screening of siblings and 1st degree relatives of the patients is also important.

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Section: Clinical Featuresmentioning

confidence: 99%

“…It may be confused with acute viral hepatitis or drug-induced hepatitis [4]. Correct diagnosis and urgent treatment is important and life saving [1,4,5].…”

Section: Clinical Featuresmentioning

confidence: 99%

Section: Kayser-fleischer Ringmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Wilson’s disease

et al. 2010

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Guidelines on the use of therapeutic apheresis in clinical practice—Evidence‐based approach from the apheresis applications committee of the American Society for Apheresis

Szczepiórkowski

Winters

Bandarenko

et al. 2010

J of Clinical Apheresis

499

285

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The American Society for Apheresis (ASFA) Apheresis Applications Committee is charged with a review and categorization of indications for therapeutic apheresis. Beginning with the 2007 ASFA Special Issue (fourth edition), the subcommittee has incorporated systematic review and evidence-based approach in the grading and categorization of indications. This Fifth ASFA Special Issue has further improved the process of using evidence-based medicine in the recommendations by refining the category definitions and by adding a grade of recommendation based on widely accepted GRADE system. The concept of a fact sheet was introduced in the Fourth edition and is only slightly modified in this current edition. The fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis. The article consists of 59 fact sheets devoted to each disease entity currently categorized by the ASFA as category I through III. Category IV indications are also listed.

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Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease

Morgan

Zantek

2012

J of Clinical Apheresis

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Wilson's disease (WD) is an autosomal‐recessive disorder of impaired copper metabolism resulting in accumulation of copper primarily in the liver but ultimately in many organs and tissues. A small number of patients with WD initially present with fulminant hepatic failure (FHF), hypercupremia, and intravascular hemolysis. The therapeutic goals for these patients include quickly removing the copper and preparing the patient for liver transplantation. Here, we report on a 6‐year‐old male with WD in FHF with anemia, renal insufficiency, and coagulopathy. The patient received a series of therapeutic plasma exchanges (TPE) as adjunctive therapy to remove copper and stabilize his coagulopathy and anemia until a transplant was possible. A total of five single plasma volume (1500 mL) TPE were performed over the course of 11 days with plasma as the replacement fluid. Laboratory results demonstrated temporary improvement after each procedure. Liver transplantation was performed 12 days after beginning TPE and 35 days after admission to the hospital. TPE was a successful adjunctive therapy to bridge this patient with WD to transplantation. J. Clin. Apheresis 27:282–286, 2012. © 2012 Wiley Periodicals, Inc.

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Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange

Cited by 16 publications

References 11 publications

Wilson’s disease

Wilson’s disease

Guidelines on the use of therapeutic apheresis in clinical practice—Evidence‐based approach from the apheresis applications committee of the American Society for Apheresis

Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease

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