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PubMed1989

Cystic fibrosis in Saudi Arabia.

Cystic fibrosis (CF) is generally believed to be rare or nonexistent in Saudi Arabia. The aim of this report is to document the occurrence of CF in Saudi Arabia. Thirteen Saudi children were diagnosed as having CF, evidenced by typical clinical features and elevated sweat chloride concentrations (greater than 60 mmol/l). Duration of symptoms prior to diagnosis varied from 1 month-5 years (mean 23 months). The main clinical manifestations of the children were abdominal distention, failure to thrive, steatorrhoea, hepatomegaly, rectal prolapse and recurrent respiratory infections, often with Pseudomonas aeruginosa. In addition, eight patients with symptoms and a family history highly suggestive of CF, but without confirmatory sweat test results are presented. We hope that this report will increase the awareness of CF and ensure an earlier diagnosis of the disease in Saudi Arabia.

Child, Preschool↗

PubMed2007

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy.

Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the initiating event in CF airways disease pathogenesis is reduced airway surface liquid (ASL) volume, i.e., dehydration. CF ASL volume regulation depends on a single extracellular signaling system, ATP, which renders CF airways more vulnerable to disease-causing insults (e.g., viruses) than are normal airways, which regulate ASL volume by dual ATP and adenosine signaling pathways. Clinical studies have explored the hypothesis that treating the dehydration of CF airways will be therapeutically beneficial. Inhaled hypertonic saline osmotically draws water onto airway surfaces, improves mucus clearance and pulmonary function, and reduces acute exacerbations in CF patients. Thus, rehydration therapies may slow the progression of CF lung disease in patients with established bacterial infection and may prevent the onset of CF lung disease if initiated early in life.

Cystic Fibrosis↗

PubMed1983

Piperacillin and tobramycin in the treatment of Pseudomonas lung infections in cystic fibrosis.

Fourteen children with cystic fibrosis and pulmonary lung infection due to Pseudomonas aeruginosa were treated with piperacillin (300 mg/kg/day) alone or piperacillin and tobramycin (7 mg/kg/day) iv. The outcome with respect to clinical state, chest X-ray and lung function tests was better with combination therapy than with piperacillin alone. Bacteriological response was the same with both regimens: leucocyte content of the sputum decreased, non-mucoid Ps. aeruginosa strains were eliminated, but mucoid strains were only suppressed (11 children). Peak serum levels of piperacillin averaged 102 mg/l, the overall serum elimination was 0.75 h and the mean sputum concentrations ranged from 1.07 to 2.2 mg/l. Peak serum levels of tobramycin averaged 5.15 mg/l, the half life was 1.25 h and the mean sputum concentrations ranged from 0.57 to 0.68 mg/l. The clearance of piperacillin and tobramycin was increased significantly. Drug-resistance did not develop during therapy.

Adolescent↗

PubMed2005

Oral bisphosphonates improve Bone Mineral Density in adults with cystic fibrosis.

In adults with Cystic Fibrosis (CF) we sought to establish the effect of oral bisphosphonate therapy. Bone densitometry measured by dual energy X-ray absorptiometry (DXA), and clinical patient data, were reviewed retrospectively. Eighty-one patients (median age 27 years) had baseline and follow-up DXA, with an interval of 19.2 +/- 7.1 months. Thirty-six patients were treated with bisphosphonates (alendronate=23 and risedronate=13). Median follow-up Bone Mineral Density in the bisphosphonate group was 3.7% greater at the lumbar spine (95%CI 1.9 to 5.7%, P<0.0005) and 2.4% greater at the femur (95%CI 0.8 to 3.9%, P<0.005) than the group not treated with bisphosphonates. Oral bisphosphonate therapy had a beneficial effect on BMD in adults with CF.

Administration, Oral↗

PubMed1986

Pulmonary function and oxygen uptake during exercise in 11-year-old patients with cystic fibrosis.

Ten patients with cystic fibrosis (CF), 5 girls and 5 boys, 11 years of age, underwent pulmonary function and bicycle exercise testing. The material includes all but 2 CF patients in this age group in southern and eastern Norway. The CF children had a moderately reduced pulmonary function, the girls more pronounced than the boys. The mean maximal oxygen uptake (max VO2) in the CF boys was 55 ml/kg/min (94% of age predicted value; range 79-105%), and the CF girls 42 ml/kg/min (79% of age predicted value; range 67-89%). The mean maximal peak heart rate was 195 beats/min. Only one CF girl showed apparent pulmonary limitation during exercise. Increased ventilatory equivalent for oxygen was found during both rest and exercise for the CF girls, but only during rest for the CF boys. Max VO2 was fairly well related to the habitudinal physical activity of the children (r = 0.79).

Child↗

PubMedDate not supplied

Prospects for the prevention and control of pseudomonal infection in children with cystic fibrosis.

Most patients with cystic fibrosis (CF) experience recurrent and chronic endobronchial Pseudomonas aeruginosa infections. It is possible to prevent or delay the onset of these chronic infections in most patients with CF by eliminating cross-infection and by early aggressive antibiotic treatment of the first positive sputum culture and of subsequent intermittent colonisation. Lung tissue damage is caused by activation of the immunologically specific inflammatory defence mechanisms of the lungs, which are initiated by the antibody response and dominated by polymorphonuclear neutrophil leucocytes and their proteolytic and oxidative products. This inflammation induces a phenotypic shift from nonmucoid to mucoid, alginate-producing phenotypes of P. aeruginosa which then grow, endobronchially, as a biofilm. Such biofilms are impossible to eradicate with antibiotics. By using chronic suppressive antibiotic maintenance therapy and anti-inflammatory drugs it is however, possible to maintain the lung function of these patients for a number of years.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1987

[What genetic risk should suggest prenatal diagnosis for cystic fibrosis?].

Prenatal diagnosis of cystic fibrosis is today possible by chemical study of amniotic fluid during the 18th week of pregnancy. We have, among 90 families seen for genetic counseling between 1972 and 1985, estimated the risk of recurrence; it was of 0.25 in 55 cases; between 0.05 and 0.16 in 30 cases, greater than 0.01 in 16 cases. Before suggesting a prenatal diagnosis, it is necessary to take as basis the risk of abortion and the reliability of the method. The first risk is well known, less 0.005, in these young women; reliability can be estimated: almost 0.01 of false wrong negative, and this number is probably overvalued if all technical conditions are perfect. In front of these risks, it seems possible to propose a prenatal diagnosis from a risk of 0.01, the families being informed of all risks. We think so to hearten some families and to make possible for them to live quietly these pregnancies, probably non undertaken without our help.

Abortion, Spontaneous↗

PubMed1983

[A critical study of technetium 99m and xenon 133 lung scintigraphy in patients with cystic fibrosis].

During investigations for cystic fibrosis, 41 children, most of whom were aged 0 to 7 years, were submitted to lung scintigraphy with technetium 99m first and xenon 133 subsequently. This procedure ensured accurate location of pulmonary lesions. The reliability of the radio-isotopic study was assessed through comparison to conventional clinical and radiological criteria, arterial blood gas determinations and pulmonary function tests. Perfusion scintigraphy with technetium 99m labelled macroaggregated albumin (n = 90) was compared to functional parametrical xenon, 133 study (n = 25) (perfusion-ventilation). The degree of lung disease was evaluated during serial examinations in order to improve prognostic prediction. Technetium 99m scintigraphy proves a reliable and sensitive procedure which accurately reflects the patients' clinical status when it is performed in the absence of patent pulmonary superinfection. Xenon 133 scintigraphy, which is more difficult to perform and interpret, has the advantage of providing a topographic and quantitative evaluation of pulmonary blood flow and ventilatory efficiency.

Adolescent↗

PubMed1982

Scotopic thresholds and plasma retinol in cystic fibrosis.

Patients with cystic-fibrosis (CF) often have low plasma concentrations of vitamin A. We have measured dark-adapted scotopic thresholds of 56 patients with CF, ages 4 to 34 years, either with a two-alternative forced-choice procedure or as the final threshold after a full dark-adaptation curve. Fasting plasma vitamin A alcohol (retinol) was measured in 34 of the 56 patients. The average thresholds were higher and retinol values lower in patients than in controls. In two patients with very low (less than 7 micrograms/dl) initial retinol levels and elevated thresholds, decreased rhodopsin densities were observed; rhodopsin density and thresholds returned to normal after treatment with oral vitamin A. Rhodopsin density and log sensitivity were linearly related. Only marked decreased in plasma retinol were associated with elevations of dark-adapted threshold and decreases in rhodopsin density, suggesting that the tissues of patients with CF sequester vitamin A to maintain retinal function.

Adolescent↗

PubMed2006

Cystic fibrosis: a balancing act?

Cystic fibrosis has evolved from an illness where children died in their teens to one with a longer life expectancy into adult life. Patients now usually keep themselves in a good physical condition, which gives them an opportunity to live a relatively 'normal' life. There is a catch: a patient can only achieve this by adhering to a strict, complex and time-consuming therapeutic schedule, thereby introducing an abnormal element into a 'normal' life. In this paper we discuss the issue of balancing 'being ill' with 'living like healthy children', and argue that 'normality' is as crucial in any developing CF child as the 'permission' to be ill. An important question arises as a consequence of the major improvements in CF care, namely--'Should all care be done at home or are there advantages in doing part of it in hospital or clinic?' We contend that this is where the balance lies as there are advantages for some care to continue to be done in hospital.

Anti-Bacterial Agents↗

PubMed2006

Growth hormone normalizes pubertal onset in children with cystic fibrosis.

UNLABELLED: Children with cystic fibrosis (CF) have a high incidence of delayed puberty and poor growth. We retrospectively reviewed pubertal maturation data from 105 children with CF who had participated in studies on growth hormone (GH). As part of the GH study, participants were randomized into two cohorts, one of which was treated with GH for 1 year, and then followed off GH, and the other group was first followed off GH, and then treated with GH for 1 year. Pubertal staging was obtained throughout these studies and we have retrospectively analyzed the data. RESULTS: In prepubertal females, GH treatment resulted in a normalized onset of breast development as compared to delayed onset in non-treated females. Females treated during puberty had a normal tempo of breast development. In prepubertal males, GH treatment resulted in a normalized onset of testicular volume compared to non-treated males. Testicular size progression was not accelerated in pubertal boys treated with GH. CONCLUSION: GH treatment normalizes pubertal onset in prepubertal children with CF.

Adolescent↗

PubMed1987

The clinical effect of correction of vitamin E depletion in cystic fibrosis.

Thirty patients with cystic fibrosis, 24 of whom had longstanding low serum vitamin E, were treated with 50 mg alpha-tocopherol acetate per day for periods of 18-24 months. None of the patients received haematinics during the study period. Throughout the period of study the overall clinical state of the patients did not alter significantly. The serum vitamin E level increased in all patients, but to a variable degree which was not related to the severity of steatorrhoea. A range of nutritional parameters, including anthropometry, vitamin levels, essential elements, haemoglobin and albumin, as well as dietary intake and faecal fat excretion, were assessed at the beginning and end of the study. Haemoglobin was the only parameter to change significantly from 13.14 to 13.47 g/100 ml. Twenty one of the 30 patients showed some increase in haemoglobin values and this increase could not be related to clinical state, fat absorption or dietary intake but was related to the improved vitamin E status.

Adolescent↗

PubMed1995

[Perspective of use of rhDNase and new therapeutics for cystic fibrosis].

The prognosis of cystic fibrosis has dramatically improved during the last three decades. This improvement was due to optimization of symptomatic treatment: antibiotics to combat infection, nutritional support, emphasis on bronchial drainage by physiotherapy and aerosols. The discovery of the gene responsible for the disease and the encoded protein opens the way to a specific and curative approach based upon the knowledge of the intrinsic properties of the protein, and its role in ionic flux. Recently, owing to the development of bioengineering, a recombinant human DNase has become available for clinical use. When aerosolized in the patients, it leads to deep changes in rheological parameters of the secretions that facilitate bronchial drainage and clearing of the airways. Gene therapy, the ultimate expression of these advances, will soon enter the era of clinical applications. Unsolved methodological and ethical issues still preclude immediate use in the patients. The results of transplantation surgery have also dramatically improved since the beginning. Indications are more precisely defined and restricted to the more advanced forms of the disease, according to severe criteria of inclusion.

Administration, Inhalation↗

PubMed2006

Chloroquine normalizes aberrant transforming growth factor beta activity in cystic fibrosis bronchial epithelial cells.

Cystic fibrosis (CF) remains a fatal progressive disease in spite of the discovery and characterization of the CFTR gene. Transforming growth factor beta (TGF-beta) has been implicated in pathophysiology of CF. Previous reports have shown the trans-Golgi network (TGN) is hyperacdified in CF epithelial cells in culture and that this hyperacidification can be corrected with the membrane permeant weak base, chloroquine. In this study bioactive TGF-beta produced by CF and normal cells was measured using a reporter cell line with a TGF-beta responsive promoter linked to luciferase. Increased levels of TGF-beta were detected in the conditioned media from CF epithelial cells compared to their matched controls-(IB3-1 vs. S9; pCEP-R vs. pCEP, CuFi-4 vs. NuLi-1). Levels of TGF-beta were normalized with chloroquine indicating that the hyperacidification of the TGN of CF cells is responsible for the altered TGF-beta levels.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1986

Chronic jejunostomy feeding with a non-elemental formula in undernourished patients with cystic fibrosis.

Ten patients with cystic fibrosis, moderate to severe lung disease (forced expiratory flow % predicted 26.0, 15.7 [SD]), and undernutrition resistant to oral supplementation have been treated for 10 to 36 months with night-time feeds of a non-elemental formula given via a jejunostomy. All patients gained some weight and showed a significant increase in weight for height (change % wt/ht 8.1, 6.0 [SD] and mid-arm muscle circumference (change MAMC % standard 6.2, 5.7 [SD]). Although the forced expiratory flow indicated progression of the small airways disease during the period of feeding, the forced vital capacity did not significantly decline. In nine patients improvement in % wt/ht was maintained. One patient tolerated the feeding poorly and died of severe pulmonary disease after 10 months. The non-elemental formula used cost less than half that of an elemental formula. The jejunostomy tubes were unobtrusive and secured with only a small transparent dressing, which facilitated replacement. No serious complications resulted from jejunostomy feeding.

Adolescent↗

PubMed2002

Primary inflammation in human cystic fibrosis small airways.

Most cystic fibrosis (CF) patients die of lung failure, due to the combined effects of bacterial infection, neutrophil-mediated inflammation, and airway obstruction by hyperviscous mucus. To this day, it remains unclear where and how this pathological vicious circle is initiated in vivo. In particular, it has proven difficult to investigate whether inflammatory pathways are dysregulated in CF airways independently of infection. Also, the relative involvement of large (tracheobronchial) vs. small (bronchiolar) airways in CF pathophysiology is still unclear. To help address these issues, we used an in vivo model based on the maturation of human fetal CF and non-CF small airways in severe combined immunodeficiency mice. We show that uninfected mature CF small airway grafts, but not matched non-CF controls, undergo time-dependent neutrophil-mediated inflammation, leading to progressive lung tissue destruction. This model of mature human small airways provides the first clear-cut evidence that, in CF, inflammation may arise at least partly from a primary defect in the regulation of neutrophil recruitment, independently of infection.

Animals↗

PubMed2004

Cytokines and inflammatory mediators in cystic fibrosis.

Airway disease in cystic fibrosis (CF) is characterised by a continuous cycle of chronic infection and inflammation dominated by a neutrophilic infiltrate. This inflammation is characterised by an increased production of pro-inflammatory cytokines in the lung. The relationship between the abnormal CFTR gene product and the development of inflammation and progression of lung disease in CF is not fully understood. This review article studied the mechanisms of pulmonary inflammation in CF, the profiles of cytokines and inflammatory mediators in the lung in CF, the mechanisms that predispose to chronic Pseudomonas aeruginosa infection, cytokine involvement in diseases other than CF and reviewed current therapeutic strategies for CF. Imbalances of cytokine secretion are now better understood due to recent advances in understanding CF at a molecular level and it is increasingly thought that the normal inflammatory process is deranged in CF early in the course of the disease and may occur in the absence of detectable infection. However, the relationship between this unbalanced cytokine production, the mutations in CFTR and its actual consequence for pathogenesis need further investigation.

Anti-Inflammatory Agents↗

PubMed2006

Betterland: an interactive cd-rom guide for children with cystic fibrosis.

Effective management of cystic fibrosis (CF) depends on adherence to a daily multi-component regimen. Segregation policies have been reported to lead to a dramatic fall in the incidence of cross infection rates in specialist CF centres. Previously, peers provided a valuable source of information and support; segregation means that there is virtually no contact with other people with CF. In this context the Betterland CD-ROM was developed with the aim of improving knowledge and understanding and helping children and young people cope with time-consuming CF treatments (inhalation therapies and physiotherapy) and painful procedures by using filmed-model approaches. Betterland is an interactive computer game, designed in the style of a theme park. Patient and carer feedback has been positive and the CD-ROM has been incorporated into a nurse-led Moving to High School (MotHS) programme, as well as the patient education work of the CF multidisciplinary team, particularly clinical nurse specialists.

Adaptation, Psychological↗