Long-term therapy of a patient with summerskill-walshe-tygstrup syndrome by applying prometheus® liver dialysis: a case report.

[benign recurrent intrahepatic cholestasis]

Summerskill-Walshe-Tygstrup syndrome is a rare benign chronic liver disease characterized by recurring cholestasis with jaundice and severe pruritus . Due to insufficient conservative treatment , liver dialysis by Prometheus (®) was applied to a 45 -year -old female patient with resistant pruritus . Initially , other possible liver diseases were excluded and the patient was treated symptomatically since the diagnosis of Summerskill-Walshe-Tygstrup was stated in 1998 . As conservative and endoscopic methods progressively failed to relieve the patient 's suffering , Prometheus (®) liver dialysis was performed regularly since 2006 at 3 -month intervals and successfully led to a decrease in the patient 's symptoms . Cholestatic liver enzymes and also serum bile acids could be lowered significantly from an average of 22 .5 ± 2 .7 to 7 .3 ± 1 .7 µmol /l . Consequently , Prometheus (®) liver dialysis may be a beneficial option for patients with benign recurrent intrahepatic cholestasis suffering from therapy-resistant symptoms and may be used as well as other extracorporeal liver support devices which have already been reported to improve cholestatic pruritus .

Diseases
Validation

Diseases presenting "year-old female patient" symptom

adrenal incidentaloma

aniridia

benign recurrent intrahepatic cholestasis

cowden syndrome

cushing syndrome

epidermolysis bullosa simplex

esophageal squamous cell carcinoma

holt-oram syndrome

krabbe disease

lamellar ichthyosis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

monosomy 21

neuralgic amyotrophy

oculocutaneous albinism

oligodontia

oral submucous fibrosis

proteus syndrome

pyomyositis

sneddon syndrome

thoracic outlet syndrome

triple a syndrome

von hippel-lindau disease

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