[Early enzyme replacement therapy - hope for patients with mucopolysaccharidosis Type II].

We present an unexpected outcome of 10 years of enzyme replacement therapy of a boy with mucopolysaccharidosis type II. Due to a positive familiar history (severe disease course in a sibling) the diagnosis was established in the first month of life. Treatment with Elaprase was introduced two months later.

Small-cell predominant extranodal NK/T cell lymphoma, nasal type: clinicopathological analysis of a series of cases diagnosed in a Western population.

Aims: Extranodal NK/T cell lymphoma, nasal type (ENKTCL) is usually composed of medium- to large-sized lymphoid cells showing prominent angiotrophism and tumour cell necrosis. We report 13 cases composed predominantly of small lymphocytes diagnosed in the United States and Western Europe.

Methods And Results: Patients included seven females and six males aged 17-75 years.

Monitoring of dipeptidyl peptidase-IV (DPP-IV) activity in patients with mucopolysaccharidoses types I and II on enzyme replacement therapy - Results of a pilot study.

Objectives: Mucopolysaccharidoses (MPSs) are a group of rare, inherited metabolic disorders which result from the lack of one of the lysosomal enzymes responsible for the degradation of glycosaminoglycans. Early recognition of MPS is important as it enables prompt implementation of enzyme replacement therapy (ERT). Dipeptidyl peptidase-IV (DPP-IV) is a ubiquitous ectopeptidase which activity has been associated with the cell surface protein CD26.

Coronary artery abnormalities in Kawasaki disease.

Introduction: Kawasaki disease is the number one cause of acquired heart disease among children in developed countries.

Aim: The aim of the study was a retrospective analysis of the factors that may influence the persistence of coronary artery abnormalities in patients with Kawasaki disease.

Materials And Methods: Analyzing the medical records of patients hospitalized in the University Children's Hospital of Krakow in the years 2005-2011 we collected the data of 28 patients diagnosed with Kawasaki disease.

Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease.

Background: Chronic sclerosing sialadenitis is a fibroinflammatory disease of the salivary glands, characteristically of the submandibular gland. One prior Asian study proposed that chronic sclerosing sialadenitis is a part of the spectrum of IgG4-associated disease. This association has not been confirmed in Western populations.