An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis.

Patient registries are organized systems of data collection for scientific, clinical or health strategy purposes. Aims of our review were to document scientific literature based on data and information from cystic fibrosis (CF) registries; to understand which clinical problems have been addressed and for which of these the studies concerned have correctly answered the questions raised (i.e. a methodological critique) and to identify clinical issues in need of further investigation. The review included primary studies starting from a formally constituted CF registry of at least national level, using data from the registry to evaluate research hypotheses. This article is an overview of the research undertaken, focusing in detail on the issues of mortality and survival. The studies considered here focused mainly or secondarily on survival in CF, the aim being to ascertain an improving trend, identify any prognostic factors and, in some cases, attempt to provide a predictive model of survival.

Bronchial to subclavian shunt in a CF patient. A potential pitfall for embolization.

Bronchial artery embolization is a well accepted and widely used technique in the management of massive haemoptysis in cystic fibrosis (CF). It can be a complex procedure requiring a deep knowledge of the bronchial artery anatomy including the possible bronchial anastomoses. We report a case of complex vascular anatomy of the left bronchial artery with multiple anastomoses with the ipsilateral subclavian artery as cause of non-attempted embolization.

Lateral thoracic artery embolization in cystic fibrosis.

We report the embolization of an aberrant origin of haemoptysis - from the internal branch of the lateral thoracic artery - in a patient affected by cystic fibrosis. The technical implications for embolotheraphy in case of an aberrant origin of a haemorrhage are emphasized. Many different systemic arteries may contribute to the blood supply of the lung and many connections may exist between the systemic, bronchial and pulmonary circulations. The presence of non-bronchial systemic arteries supply should be investigated when inconclusive findings are seen during bronchial artery embolization for haemoptysis.

[Anti-Pseudomonas aeruginosa antibodies and lung disease in cystic fibrosis] / Anticorpi anti Psudomonas aeruginosa e malattia polmonare in fibrosi cistica.

The aim of our study was to diagnose and to control three aspects of the evolution of lung disease in CF: the absence of infection, the intermittent colonization and chronic infection by Pseudomonas aeruginosa. Therefore a study of anti-pseudomonas antibodies (Ab) (anti-protease, anti-elastin and antihexo-toxin A) for diagnosis and follow-up of CF patients was considered. Moreover, we related the presence of Ab to the sputum culture, to FEV1, to patient age and to genotype. Tbe Ab were dosed in 121 patients by quantitative ELISA method. Values < 1: 500 were considered negative, values> 1: 500 and < 1:1250 borderline, and > 1:1250 positive. 16.5% of patients did not have Ab, 17% had borderline values and 69.5% had positive values. All the patients with negative Ab had negative sputum culture; 47% of patients with borderline values had at least one positive culture while 53% were negative. 87% of patients with positive values had chronic colonization, 13% intermittent colonization. The increase in the Ab rate is statistically related to a more severe lung disease (p < 0.013). The presence of a severe mutation (?F 508) is related to positive values of Ab. Evaluation of anti-Pseudomonas aeruginosa is an important tool for diagnosis and follow-up of CF lung disease

[Tuberculous meningo-encephalitis in pediatrics. Considerations on 71 cases (1968-1993)]. / Meningoencefalite tubercolare in età pediatrica. Considerazioni su 71 casi (1968-1993).

The tuberculous meningitis is an uncommon but severe complication of the tuberculosis mainly in childhood. Neurological sequelae are frequent and high mortality rate, till now, occurs. We reviewed the medical records of 71 cases and we evaluated the most frequent symptoms before the admission and signs and symptoms on the admission according to prognosis. An early diagnosis and specific anti-tuberculous treatment are essential in order to prevent permanent neurological sequelae and fatal outcome

Acute post-streptococcal glomerulonephritis in an 8-month-old girl.

We describe a middle class Sicilian girl who at 8 months of age suffered acute post-streptococcal glomerulonephritis documented by clinical hist a positive type 12 beta-haemolytic streptococcus throat culture, a raised anti-sterptolysin O titre and a low C3 rising to normal after 8 weeks