Partial splenectomy for portal hypertension in cystic fibrosis related liver disease.

Aims: To review the middle- and long-term effects of partial splenectomy (PS) on portal hypertension (PHT) and its complications in patients with cystic fibrosis (CF) related liver disease risky PHT.

Method: Over a 20 years period, 19 patients aged 7-23 years underwent partial PS for massive splenomegaly, hypersplenism, and/ or severe PHT.

Results: In all but three cases, PHT and hypersplenism have improved for long periods.

[Bilateral vas deferens agenesis and inguinal hernia in a child. A rare, early presentation of cystic fibrosis].

Unlabelled: Epididymal and ductal anomalies can be discovered incidentally during inguinal herniorraphy in children. The congenital bilateral absence of vas deferens is frequently associated with cystic fibrosis.

Case Report: This agenesia of vas deferens was detected in a 5-month-old boy who underwent an inguinal herniorraphy.

Clinical significance of Scedosporium apiospermum in patients with cystic fibrosis.

The incidence of airway colonization by Scedosporium apiospermum and of related sensitization was investigated prospectively in 128 patients with cystic fibrosis over a 5-year period, and results were compared with clinical data. Scedosporium apio-spermum, recovered from sputum samples in 11 of 128 (8.6%) patients, was the most frequent filamentous fungus after Aspergillus fumigatus.

Imaging changes in the pancreas in cystic fibrosis: a retrospective evaluation of 55 cases seen over a period of 9 years.

Background: Pathologic changes of the pancreas have been observed as early as the recognition of the disease termed initially "cystic fibrosis of the pancreas". Atrophy of the gland and its fatty infiltration were considered as usual features. The aim of this study was to follow-up the evolution of cystic fibrosis pancreas and to define its successive stages in correlation with the clinical, biochemical, and imaging findings.

Complex allele [-102T>A+S549R(T>G)] is associated with milder forms of cystic fibrosis than allele S549R(T>G) alone.

We recently reported a novel complex allele in the cystic fibrosis transmembrane regulator (CFTR) gene, combining a sequence change in the minimal CFTR promoter (-102T>A) and a missense mutation in exon 11 [S549R(T>G)]. Here we compare the main clinical features of six patients with cystic fibrosis (CF) carrying the complex allele [-102T>A+S549R(T>G)] with those of 16 CF patients homozygous for mutation S549R(T>G) alone. Age at diagnosis was higher, and current age was significantly higher (P=0.

Chronic airway colonization by Penicillium emersonii in a patient with cystic fibrosis.

Penicillium emersonii Stolk, the conidial state of Talaromyces emersoniii Stolk, is a heat-resistant fungus usually isolated from soil. In this paper the authors report, to our knowledge, the first human case in which P. emersonii chronically colonized the respiratory tract and induced an immune response in a patient with cystic fibrosis.

Improvement of cystic fibrosis airway mucus transportability by recombinant human DNase is related to changes in phospholipid profile.

The aim of this study was to test whether changes in mucus surface properties by rhDNase treatment could be related to an increased recovery of phospholipids. Purulent sputa from 18 patients with cystic fibrosis (CF) were incubated with either rhDNase (4 microg/ml) or control excipient. The incubation of mucus samples with rhDNase induced a significant increase (p < 0.

Production of elastase, exotoxin A, and alkaline protease in sputa during pulmonary exacerbation of cystic fibrosis in patients chronically infected by Pseudomonas aeruginosa.

Secretion of Pseudomonas aeruginosa elastase, exotoxin A, and alkaline protease in sputum during bronchopulmonary exacerbations was examined in 18 cystic fibrosis patients chronically infected with this microorganism. The patients were studied during one or several exacerbation periods necessitating hospitalizations of 12 to 20 days. In all cases, P.

Mutation analysis in 600 French cystic fibrosis patients.

The cystic fibrosis transmembrane conductance regulator (CFTR) gene of 600 unrelated cystic fibrosis (CF) patients living in France (excluding Brittany) was screened for 105 different mutations. This analysis resulted in the identification of 86% of the CF alleles and complete genotyping of 76% of the patients. The most frequent mutations in this population after delta F508 (69% of the CF chromosomes) are G542X (3.

[Production of elastase, exotoxin A and alkaline protease during bronchopulmonary exacerbations in patients with mucoviscidosis chronically infected by Pseudomonas aeruginosa].

The authors have studied the production of exoproteins by Pseudomonas aeruginosa in the sputa of 18 patients suffering from cystic fibrosis, during 29 bronchopulmonary exacerbations and also after the recovery of a stable state. Significant levels of exoproteins were detected but with a large heterogenity of intra and inter individual variations. A significant decrease in the production of the three exoproteins was found after twelve days of antibiotherapy, without any correlation between exoprotein levels and colony forming units in the sputa.

Cystic fibrosis and portal hypertension interest of partial splenectomy.

Extra pulmonary complications can be major in patients suffering of cystic fibrosis who survive long enough without pulmonary problems. Hepatic cirrhosis is often present and is responsible for portal hypertension with splenomegaly, hypersplenism and esophageal varices. In six patients we have performed a partial splenectomy with conservation of the upper pole of the spleen vascularized by gastrosplenic omentum and its vessels.

Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis.

Ursodeoxycholic acid, 10 to 20 mg/kg per day, was administered for 1 year to 22 patients with cystic fibrosis and chronic cholestasis, resulting in significantly improved liver enzyme values. However, evidence of cholestasis continued, as shown by the pattern of alkaline phosphatase isoenzymes.

[Lung transplantation and cystic fibrosis].

It is only in 1983-84 that lung transplantation has been contemplated in cystic fibrosis patients. The French programme, reported here, began in 1987. The criteria of inclusion are detailed, including antibiotic dependence, oxygen dependence, poor spirometric profile, destabilized nutritional status and very low Shwachman's score.

Behaviour of serum immunoreactive trypsin after serum activation by enterokinase in normal and cystic fibrosis patients. Evidence of a trypsin-alpha 1-proteinase inhibitor complex in some cystic fibrosis patients.

Serum immunoreactive trypsin (IRT) concentrations are elevated in newborn children with cystic fibrosis (CF) and subsequently fall, in most cases, to values below normal. To evaluate the molecular form(s) of IRT present in serum, we have performed serum activation by enterokinase and have measured serum IRT before and after activation. This approach is based on the postulate that enterokinase converts trypsinogen into trypsin, and this trypsin would then be mainly trapped by alpha 2-macroglobulin, thus escaping the assay.

[Double lung transplantation. Report of the 1st French case and comments on the 5 subsequent cases].

Using a modified version of the technique described by the Toronto group, the Marseille group has performed 6 double lung transplantation procedures. In 6 cases the underlying disease was cystic fibrosis. Four patients are currently alive.

[Contribution of molecular biology to the prevention of cystic fibrosis. Experience in Lyon].

Enzymatic prenatal diagnosis of cystic fibrosis was performed in 113 amniotic fluids and DNA polymorphism was studied in 104 families, including 28 cases with prenatal material analysis. According to the results, the enzymatic diagnosis should be cautiously interpreted when the risk is less than 1/4. In these situations DNA analysis in the parents is very helpful to assess the reliability of enzymatic diagnosis.

Efficacy of pancrease: crossover comparative study versus eurobiol in cystic fibrosis.

An open crossover study in patients with documented cystic fibrosis (CF) compared the efficacy and acceptability of Pancrease (Cilag, PC), consisting of pH-sensitive, enteric-coated microspheres, with those of Eurobiol, consisting of lyophilized total pancreas. Eleven patients, age 6-17 years, were hospitalized for study. A hypercaloric, normal fat diet as well as previous antibiotic, vitamin, and other CF therapy were maintained during the 2-week study period.

[Proteins of bronchial secretions in mucoviscidosis. Role of infection].

The protein composition of sputum collected by a protected method in 17 children with cystic fibrosis (CF) was analysed. The severity of the disease was assessed using the Shwachman score and the degree of bronchial superinfection was determined by a quantitative cyto-bacteriological study. The main proteins with antibacterial activity (IgA, IgG, IgM, lysozyme and lactoferrin) or reflecting the degree of bronchial inflammation (serumalbumin and alpha 1-antitrypsin) were determined by immunological methods.