Results Since 2006, 281 patients (1-73 year old) were included in the Wilson France database (sex ratio: 1). Mean age at diagnosis was 19 years. First symptoms were neurological for 36% of the patients, hepatic for 38%, renal, psychiatric or hematologic for 11%. Fifteen percent were diagnosed after familial screening. At time of diagnosis, Kayser-Fleischer ring was observed in 95% of patients w...

Purpose — to discover peculiarities of Wilson disease course in children dependently on the variant liver affection. Materials and methods. Anamnesis clinical paraclinical with consideration affection severity have been studied 50 aged 5–17 years. Results. It was estimated that 52% had a form chronic hepatitis minimal symptoms, 44% patients cirrhosis predominant signs edematous ascitic syndrome...

Abstract A rare genetical autosomal recessive disorder called Wilson disease (WD) is characterized by excess copper being deposited in numerous major organs, mainly the liver and brain causing hepatic, neurological mental symptoms. WD caused mutations gene ATP7B. Although not always present, Kayser-Fleischer (KF) ring pathognomonic indication of deposition corneas eyes. The condition worsens wi...

BACKGROUND Fleischer's ring is considered to be a characteristic of keratoconus, but we have seen a ring similar to Fleischer's ring in patients with secondary keratoconus, in which the cornea becomes thinner secondarily for undetermined reasons. CASES We report 6 cases of secondary keratoconus with a corneal epithelial ring similar to the Fleischer's ring pattern. OBSERVATIONS In these 6 c...

The hyperspectral satellite EnMAP (Environmental Mapping and Analysis Program) is scheduled to launch in 2014. It is a joint project by the GFZ (Deutsches GeoForschungsZentrum), the DLR (German Aerospace Center), Kayser-Threde and OHB-System. Apart from the project management, the DLR is responsible for the development of the ground segment for EnMAP. One of the key steps of the developed proce...

Abstract Patients suffering from Wilson’s disease accumulate copper in their body. Copper load may become visible as Kayser-Fleischer (KF) rings - colorful copper-sulfur granules the base of cornea. Reoccurrence KF patients under anti-copper medication be a sign for ineffective treatment. This work proposes hand-held sensing system based on infrared reflectivity measurement cornea potential met...

Emily S. Birkholz, MD, Thomas A. Oetting, MS, MD July 28, 2009 Chief Complaint: Referral for evaluation of Kayser-Fleischer Rings History of Present Illness: The patient was a 25 year old male who had been experiencing a right-sided subcostal tenderness and increasing fatigue for the last several months. He also had noticed dark urine, jaundice and a 60 pound weight loss. The patient was admitt...

INTRODUCTION Wilson's disease is typically manifested in two clinical forms, neurological and hepatic and in rare cases it starts with psychiatric symptoms exclusively. We presented a rare atypical case of Wilson's disease with psy- chotic onset. CASE REPORT A 22-year-old male patient was initially presented with predominant signs and symptoms of psychiatric disorder and then later with the d...

We report a rare case of Wilson Disease with neurologic features in 31-years-old man. This disease consists disturbance copper metabolism secondary to mutation the gene responsible for encoding tissue transporter and enzyme that incorporates excess element into bile, generating toxic accumulation liver, cornea, central nervous system. According his wife, patient had been undergoing treatment an...