Partial recessive IFN-γR1 deficiency: genetic, immunological and clinical features of 14 patients from 11 kindreds.

We report a series of 14 patients from 11 kindreds with recessive partial (RP)-interferon (IFN)-γR1 deficiency. The I87T mutation was found in nine homozygous patients from Chile, Portugal and Poland, and the V63G mutation was found in five homozygous patients from the Canary Islands. Founder effects accounted for the recurrence of both mutations.

[Invasive aspergillosis (IA) in patients treated in the Institute of Tuberculosis and Chest Diseases in the years 1993-2005--diagnostic problems].

The aim of this study was to present the diagnostic problems in pts treated for invasive aspergillosis (IA) in the IIIrd Clinic of the Institute of Tuberculosis in the years 1993-2005. The material consisted of clinical documentation of 18 pts. 15 out of 18 pts (83,4%) died.

[A case of a patient with idiopathic pulmonary arterial hypertension treated with lung transplantation: a "bumpy road" to success].

We describe a case of 29 year old man, a first Polish patient with idiopathic arterial hypertension (IPAH) listed from Poland and successfully treated with lung transplantation in Vienna. Time from diagnosis to lung transplant was merely 11 months. Rapid clinical deterioration required treatment with most of currently approved or emerging methods, including oral and parenteral prostacyclin analogues administration by inhalation and chronic subcutaneous infusion.

[The significance of the serum tumour markers as a prognostic and predictor factors in nonsmall cell lung cancer patients].

The aim of the study was to assess the role of serum tumour markers (NSE, Cyfra 21-1, CEA, LDH, ferritin) as a prognostic and predictive factors in 79 patients with advanced NSCLC treated with chemotherapy. Objective response to treatment was significantly more frequent in the patient with serum NSE > 12.5 ng/ml.

Disseminated Mycobacterium avium infection in a 20-year-old female with partial recessive IFNgammaR1 deficiency.

We report the case of a 20-year-old female with disseminated Mycobacterium avium disease involving bones, lungs and brain. She was completely healthy up until the present illness and had been vaccinated with BCG in infancy without complications. Mycobacteriosis progressed in spite of treatment with antituberculous drugs and was controlled only after addition of interferon-gamma subcutaneously.

[Organizing pneumonia--analysis of 18 own cases].

Organizing pneumonia (OP) is a rarely diagnosed disease, however the incidence ratio was estimated as 6-7/100000. Disease can occur in cryptogenic form or as a secondary reaction to various noxious agents, drugs, and ionising radiation, as a concomitant disease to infections, lympho- and myeloproliferative disorders, and connective tissue diseases. Symptoms of OP are non-specific therefore lung biopsy and histological examination are necessary for diagnosis.

[Prognosis in limited disease (LD) small cell lung cancer (SCLC) patients according to status performance, local extension of lesions, type of treatment and the completeness of staging].

The aim of this study was to assess the prognosis in LD SCLC pts according to their performance status, local extension of lesions, type of treatment and the completeness of staging. In the period 01.01.

[Pulmonary mycobacterioses--frequency of occurrence, clinical spectrum and predisposing factors].

Various types of non-tuberculous mycobacteria can be the aetiologic factors of chronic lung infections especially in patients with underlying chronic lung diseases. The aim of this study is to present the cases of pulmonary mycobacterioses observed in Institute of Tuberculosis and Lung Diseases in the years 1995-2001. There were 23 patients, 12 men and 11 women in the age between 35-77 years, mean 56 years.

[Evaluation of mediastinal lymph nodes in the course of lung diseases in dynamic contrast-enhanced magnetic resonance imaging].

The purpose of this study was to evaluate the ability of dynamic contrast-enhanced magnetic resonance to provide differentiation between malignant and benign mediastinal or hilar lymph nodes. The group of 109 patients were examined, with lung carcinoma (63 patients) and sarcoidosis (46 patients). Relative signal intensity increase after administration of Gd-DTPA in standard dose (0.