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PubMed1999

Blood immunoreactive trypsinogen concentrations are genetically determined in healthy and cystic fibrosis newborns.

Newborns with cystic fibrosis (CF) have increased blood immunoreactive trypsinogen concentrations. When screening for CF in the newborn by immunoreactive trypsinogen measurement, an abnormally high proportion of healthy deltaF508 carriers is found among false-positive neonates, suggesting that a relationship could exist between immunoreactive trypsinogen concentration at birth and the genetic status. Therefore, this study analysed the possible relationships between neonatal blood immunoreactive trypsinogen concentrations and genotype in 1842 healthy newborns and 111 CF patients detected by a neonatal screening programme. A close correlation was found between immunoreactive trypsinogen and deltaF508: the probability of a healthy newborn being a carrier of this mutation increased regularly with the neonatal immunoreactive trypsinogen concentration. In CF patients, there was a significant difference between deltaF508 homozygotes and deltaF508/X (X = other mutation) compound heterozygotes with respect to the mean neonatal blood immunoreactive trypsinogen concentration. CF neonates with two mutations affecting the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator protein had significantly higher mean immunoreactive trypsinogen concentrations than patients with one mutation affecting a membrane-spanning domain. The data strongly suggest that the neonatal immunoreactive trypsinogen concentration is, in part, genetically determined, with a wide range of variations, similar to the features which have been shown for the relations between the genotype and clinical phenotypes of CF patients.

Case-Control Studies↗

PubMed2005

Behavioral aspects of nutrition in children with cystic fibrosis.

PURPOSE OF REVIEW: Nutritional status in cystic fibrosis is important to the health and long-term survival of patients; however, children with cystic fibrosis are not achieving the dietary recommendations or achieving normal growth. This paper reviews current knowledge about behavioral and environmental barriers to dietary adherence in this population. RECENT FINDINGS: Children with cystic fibrosis do not have the same appreciation of the need to gain weight as their parents. Parents of children with cystic fibrosis describe meal times as 'battling' with the child. Direct observation studies of meal times demonstrate that school-aged children with cystic fibrosis do not differ from children without cystic fibrosis on the frequency of behaviors incompatible with eating, as was seen in younger age groups, but parents of school-aged children with cystic fibrosis continue to engage in ineffective management styles at a greater frequency than parents of children without cystic fibrosis. Assessment of family functioning during mealtime demonstrates that these interaction patterns have a negative effect on family functioning in families of children with cystic fibrosis compared with families of same-age peers in all age groups: infancy/toddler, preschool, and school age. SUMMARY: Achieving the cystic fibrosis dietary recommendations is challenging, and typical parenting strategies to encourage eating not only are ineffective but may have a negative impact on family functioning. To improve dietary adherence and decrease conflict at meals, children with cystic fibrosis and their parents need to be taught more effective management strategies as part of dietary counseling.

Child↗

PubMed1994

Differentiation of immortalized epithelial cells derived from cystic fibrosis airway submucosal glands.

Cystic fibrosis (CF) involves abnormalities in mucus production and secretion of the airway. Studies of the regulation of airway mucin production and secretion has been difficult due to the lack of in vitro models of the airway epithelial cells which express functional differentiation. Because the majority of the mucin in the airway is apparently produced by the submucosal glands, we have focused our attention on the development of cell culture models of human airway submucosal glands. This report describes the propagation of CF airway submucosal gland epithelial cells which continue to express mucin production. The CF bronchus was obtained from a 31-yr-old patient who received a double lung transplant. The glands were dissected out and primary cultures prepared by the explant/outgrowth procedure. The cells were immortalized by infection with Ad12-SV40 hybrid virus. The cultures are maintained in serum-free keratinocyte basal medium supplemented with insulin (5 micrograms/ml), hydrocortisone (0.5 microgram/ml), epidermal growth factor (10 ng/ml), bovine pituitary extract (25 micrograms/ml), and antibiotics. Cultures were passaged using 0.125% trypsin in Ca+2 and Mg(+2)-free Hanks', balanced salt solution. Polymerase chain reaction (PCR) analysis demonstrated that the cells were homozygous for the delta F508 mutation. Morphologic observations showed that the cells were epithelial and were interconnected by sparsely distributed desmosomes. Their cytoplasm contained secretory-type structures including abundant Golgi, rough endoplasmic reticulum, and secretory vesicles. Immunofluorescent studies determined that all cells were positive for cytokeratins, mucin glycoconjugates, and cystic fibrosis transmembrane conductance regulator. The cultures secreted substantial amounts of mucin glycoproteins and expressed the MUC-2 mucin gene.(ABSTRACT TRUNCATED AT 250 WORDS)

Base Sequence↗

PubMed1992

Effect of taurine supplementation on fat and energy absorption in cystic fibrosis.

In 10 children with cystic fibrosis and persisting steatorrhoea, supplementation with taurine (30-40 mg/kg/day) was given for two months as an adjunct to the usual pancreatic enzyme treatment. A three day fat and energy balance was performed in patients with cystic fibrosis, before and after the supplementation, and in seven healthy controls who did not receive taurine. Faecal fat was measured by a gravimetric method and stool energy was determined using a bomb calorimeter. Patients with cystic fibrosis, before and after taurine, and healthy controls received the same fat and energy intake (calculated by a dietitian). In patients with cystic fibrosis taurine did not produce any improvement of steatorrhoea (mean (SD) faecal fat 8.7 (3.3) v 11.2 (7.0) g/day, respectively before and after the supplementation), of faecal energy loss (0.978 (0.468) v 1.133 (0.539) MJ/day), of faecal fat expressed as percent of fat intake (13.4 (5.6) v 15.1 (9.8)%), and of faecal energy expressed as percent of energy intake (9.9 (3.6) v 11.2 (5.7)%). Healthy controls had significant lower fat (3.5 (2.3) g/day) and energy 0.576 (0.355) MJ/day faecal losses. In conclusion, taurine failed to decrease significantly fat and energy losses. Our study does not support the use of taurine supplementation in the nutritional management of cystic fibrosis.

Adolescent↗

PubMed2005

Nebulised hypertonic saline for cystic fibrosis.

BACKGROUND: Lung disease in cystic fibrosis is characterised by impaired mucociliary clearance. Hypertonic saline has been shown to enhance mucociliary clearance in vitro and this may act to lessen the destructive inflammatory process in the airways. OBJECTIVES: To investigate the effects of treatment with nebulised hypertonic saline on people with cystic fibrosis compared to placebo and or other treatments that enhance mucociliary clearance. SEARCH STRATEGY: 'We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. Most recent search of the Trials Register: September 2004. SELECTION CRITERIA: All controlled trials assessing the effect of hypertonic saline compared to placebo or other mucolytic therapy, for any duration or dose regimen in people with cystic fibrosis of any age or severity. DATA COLLECTION AND ANALYSIS: Two authors independently reviewed all identified trials and all data collected. Trial quality was assessed along with allocation concealment. MAIN RESULTS: Fourteen controlled trials were identified. Nine trials met the inclusion criteria involving 235 participants with an age range of 6 years to 46 years. Two short-term trials of immediate effect on mucociliary clearance demonstrated that hypertonic saline increased isotope clearance compared to control. Lung function, measured by improvement in forced expiratory volume at one second (FEV1 litre per minute), was observed in four trials. When 3% to 7% saline was used in a volume of 10 ml twice-a-day, in comparison to placebo, hypertonic saline led to a significant increase in FEV1, weighted mean difference 12.20 (95%CI 4.28 to 20.10). Two further trials compared a similar concentration and volume of hypertonic saline to recombinant deoxyribonuclease. Over a three-week period one trial showed a non-significant difference, mean difference 1.60 (95% CI -7.96 to 11.16). However, in a further trial, after 12 weeks treatment in participants with moderate to severe lung disease, recombinant deoxyribonuclease led to a greater increase in FEV1 than hypertonic saline (5 ml twice-daily), mean difference 8.00 (95%CI 2.00 to 14.00). No serious adverse events were noted. AUTHORS' CONCLUSIONS: Nebulised hypertonic saline improves mucociliary clearance in short-term clinical trials and appears to increase lung function compared to control. In comparison to recombinant deoxyribonuclease it may be less effective at improving lung function after three months. Currently there is insufficient evidence to support the use of hypertonic saline as routine treatment for people with cystic fibrosis.

Administration, Inhalation↗

PubMed1992

A study of aqueous humor formation in patients with cystic fibrosis.

The circadian pattern of aqueous formation and the effect of timolol on aqueous flow was studied in 12 patients with cystic fibrosis. Cystic fibrosis is a disease characterized by a defect in a chloride channel-associated regulatory protein found in epithelial cells. Improper regulation of these chloride channels, causes abnormal composition of exocrine secretions, including respiratory tract, gastrointestinal tract, exocrine pancreas, and sweat glands. Ocular findings previously reported include abnormal endothelial cell permeability, decreased tear secretion, and abnormal tear composition. In this study, aqueous humor flow was measured by fluorophotometry. No statistically significant difference was found when flow rates measured during the morning, during the afternoon, at night, and after topical timolol treatment were compared to normal values. The conclusion is that the beta adrenergically regulated chloride selective channels defective in patients in cystic fibrosis do not play a major role in the formation of aqueous humor or they are not regulated by the cystic fibrosis transmembrane conductance regulator (CFTR).

Administration, Topical↗

PubMed1996

In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis.

BACKGROUND: Viscoelastic secretions in cystic fibrosis cause impaired mucus clearance and persistence of bacteria within the lung. The abnormal rheology is partly due to the presence of high molecular weight deoxyribonucleic acid (DNA). Recombinant human DNase I (rhDNase) has been shown to depolymerise DNA and thereby reduce the in vitro viscoelasticity of sputum in patients with cystic fibrosis. A phase II double blind placebo controlled study showed that rhDNase improved pulmonary function in patients with cystic fibrosis. The object of the present study was to evaluate the in vivo effects of rhDNase on sputum rheology and to determine whether these were correlated with changes in pulmonary function. METHODS: Patients were randomised to receive either placebo or rhDNase 2.5 mg twice daily for 10 days. Sputum samples were collected in sterile containers during screening and during treatment with the study drug. Pulmonary function and rheological analysis were the primary outcomes evaluated. Other parameters assessed were quantitative sputum bacteriology, sputum DNA concentration, and change in molecular mass of DNA polymers. RESULTS: The viscoelasticity of the sputum in untreated patients with cystic fibrosis was high and treatment with rhDNase reduced all the rheological parameters measured: dynamic storage modulus (a measure of elasticity), dynamic loss modulus (a measure of viscosity), and log complex modulus (a measure of mucus rigidity). The calculated cough clearance index was also improved following treatment with rhDNase. These rheological parameters showed a correlation with forced expiratory volume in one second (FEV1) which was improved by a mean (SE) of 13.3 (5.6)% on day 10 of treatment with rhDNase compared with a change of 0.2 (3.1)% in the placebo group. There was no change in bacterial colony counts or sputum DNA concentrations following treatment with rhDNase, but a small decrease in high molecular weight DNA was observed. CONCLUSIONS: Patients with cystic fibrosis treated with rhDNase show an improvement in rheological properties and pulmonary function, one of the mechanisms being a reduction in the proportion of high molecular weight DNA.

Adolescent↗

PubMed1987

Serum concentrations of a granulocyte-derived calcium-binding protein in cystic fibrosis patients and heterozygotes.

Serum samples from cystic fibrosis homozygotes and heterozygotes contain elevated levels of a protein, known as cystic fibrosis antigen, which is synthesized primarily in granulocytes. We have produced monoclonal antibodies against cystic fibrosis antigen and developed a sensitive two-site immunoassay. Antigen levels were evaluated in serum samples from 50 cystic fibrosis homozygotes, 34 heterozygotes, 60 healthy controls and 25 disease controls. Simultaneous measurement was made of another granulocyte-derived serum protein, lactoferrin, and also of the acute-phase reactant, C-Reactive Protein. Attempts to use the concentrations of cystic fibrosis antigen in serum to distinguish cystic fibrosis patients from heterozygotes were unsuccessful, even when these concentrations were expressed as a ratio with lactoferrin or with C-Reactive Protein. However, examination of the ratio of cystic fibrosis antigen to lactoferrin in serum samples from cystic fibrosis heterozygotes suggests that there is some specific association between this antigen and the cystic fibrosis gene.

Antibodies, Monoclonal↗

PubMed1995

Effect of oral digoxin, topical ouabain and salbutamol on transepithelial nasal potential difference in patients with cystic fibrosis.

1. Airway epithelium in cystic fibrosis is characterized by a defect in chloride secretion across the apical membrane and an increase in sodium absorption. The increased rate of sodium absorption can be inhibited in vitro by ouabain, a Na(+)-K(+)-ATPase inhibitor, and in cystic fibrosis patients the number and activity of nasal epithelial Na(+)-K(+)-ATPase pumps is increased. 2. We have performed a series of studies to determine whether drugs which modify airway epithelial Na(+)-K(+)-ATPase activity in vitro can modify nasal potential in cystic fibrosis patients in vivo. As transepithelial nasal potential difference measurements were used to study the effect of drug modulation of airway epithelial ion transport in vivo, the repeatability of the technique was first evaluated. In order to assess the effectiveness of the technique used for measuring nasal potential difference, a pilot study was carried out using topical amiloride, a drug which has previously been shown to inhibit airway epithelium sodium transport in vivo. We then studied the effects of ouabain and digoxin, two inhibitors of Na(+)-K(+)-ATPase, on nasal potential difference. 3. In study 1, nasal potential difference measurements were repeated on non-consecutive days in 20 patients with cystic fibrosis and 20 healthy individuals. Healthy subjects had a mean (SEM) potential difference value of -19.5 (0.9) mV with a 95% range for a single estimate of 75-133%. In patients with cystic fibrosis, the mean (SEM) potential difference was -40.4, (2.1) mV, with a 95% range for a single estimate of 74-136%.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1994

Patient and parental attitudes toward genetic screening and its implications at an adult cystic fibrosis centre.

General population screening for cystic fibrosis carrier status in the United Kingdom would detect 72% of at-risk couples. Proper counselling would allow these couples to make informed reproductive choices, including the possibility of prenatal diagnosis and the termination of an affected pregnancy. However, children with cystic fibrosis born in this decade, given optimum treatment, now have an average life expectancy of 40 years, and there is no unanimity of opinion on how, where, when, or even if, screening should be offered. The purpose of this questionnaire-based study was to examine the attitudes of an adult clinic population who have grown up with cystic fibrosis, and of their parents, towards genetic screening programmes and the controversies and ethical dilemmas surrounding such programmes in cystic fibrosis. Both patients and parents supported prenatal screening (88% and 90%) and the option of terminating an affected pregnancy (68% and 84%). Only 22% of patients and 10% of parents felt that screening should be limited to families with a history of cystic fibrosis, and 19% and 6%, respectively, that prenatal diagnosis should be restricted to those with a previous child with cystic fibrosis. Despite the negative aspects of any screening programme and the acknowledged ethical problems peculiar to cystic fibrosis, the conclusion of our patients and parents who have lived intimately with the illness is that there should be the option of utilising information available from genetic screening for cystic fibrosis to guide reproductive choices. Pilot programmes to define the optimum management of such screening should continue.

Abortion, Therapeutic↗

PubMed1978

Automated immunoprecipitation of meconium albumin for cystic fibrosis screening in the newborn.

Cystic fibrosis is the most frequent substantially lethal inherited disease in the United States. Newborn screening for cystic fibrosis has been suggested because early diagnosis permits genetic counselling of parents and improved treatment and prognosis for cystic fibrosis patients. The fact that cystic fibrosis newborns have abnormally high meconium albumin seems to offer the best screening approach. Because we have had equivocal and false positive results with meconium albumin test strips, we have developed a nephelometric automated immunoprecipitation method for the quantitative and specific analysis of meconium albumin. On a total of 3895 meconiums so far examined, eleven albumin elevations of undetermined origin have been detected. Nine of these were associated with prematurity. There have been, so far, no detected true positives and no known false negatives. The normal range for meconium albumin is in agreement with previously published ranges determined using manual immunochemical methods. This method is rapid and economic. The question of sensitivity, specificity and predictive value of this and other detection methods is discussed.

Albumins↗

PubMed1991

Absence of an association between HLA typing in cystic fibrosis arthritis and hypertrophic osteoarthropathy.

Fourteen patients with cystic fibrosis arthritis and eight patients with cystic fibrosis and hypertrophic osteoarthropathy were typed for HLA-A, B, C, DR, and DQ antigens and were compared with age and sex matched controls with cystic fibrosis. The diagnosis of cystic fibrosis arthritis and hypertrophic osteoarthropathy was confirmed by radiography and bone scanning. The prevalence of HLA-A, B, C, D, antigens in the cystic fibrosis group (44 patients) did not differ from that in the control group. A comparison between patients with cystic fibrosis arthritis or hypertrophic osteoarthropathy and their respective controls did not show any significant differences in HLA prevalence. It is concluded that HLA antigens may not be a factor in the susceptibility of patients with cystic fibrosis to cystic fibrosis arthritis or hypertrophic osteoarthropathy.

Adult↗

PubMed1994

Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium.

New treatments for cystic fibrosis (CF), including gene therapy, are currently being assessed. These aim to correct the basic defects of increased sodium absorption and decreased chloride secretion in airway epithelia. Assessment of these bioelectric parameters, particularly in the nasal epithelium, is likely to be used as a measure of treatment efficacy. However, the optimal in vivo protocol to discriminate cystic fibrosis from non-cystic fibrosis subjects is unclear. We have, therefore, compared three protocols for measurement of the cystic fibrosis ion transport defects in vivo in the nasal epithelium. Sodium absorption was measured using both the baseline potential difference and the response to the sodium channel blocker, amiloride. Chloride secretion was assessed in the presence of amiloride, using perfusion with isoprenaline, or terbutaline, or a low chloride solution followed by isoprenaline. Baseline potential difference (PD) and the absolute response to amiloride clearly differentiated the increased sodium absorption in the cystic fibrosis subjects. The responses both to terbutaline (delta PD: non-CF: -0.8 (SEM 0.7) mV; CF: -3.6 (0.5) mV) and isoprenaline (non-CF: 1.5 (0.6) mV; CF: -2.9 (0.6) mV) differentiated the two groups of subjects, but there was considerable overlap of values. Perfusion with a low chloride solution (non-CF: 12.6 (1.2) mV; CF: 0.6 (0.4) mV), as well as subsequent perfusion with isoprenaline (non-CF: 10.0 (1.1) mV; CF: -1.4 (0.4) mV) allowed clear separation of the two groups, with no overlap of values. Some CF subjects showed a transient hyperpolarization to these stimuli, which could clearly be differentiated from the sustained responses seen in non-cystic fibrosis subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2001

Recent advances in cystic fibrosis.

The median life expectancy for cystic fibrosis is now over 30 years, and it is projected that in newborn infants it will become more than 40 years. The identification of the cystic fibrosis gene and its product, cystic fibrosis transmembrane conductance regulator (CFTR), has widened the spectrum of the disease from the classical case of the infant with cystic fibrosis to the elderly childless man with unexplained bronchiectasis. There is increasing evidence of the advantages of newborn screening for cystic fibrosis and subsequent specialist care. Management concentrates on optimising nutritional status and preventing lung infection and inflammation.

Adolescent↗

PubMed1985

Cystic fibrosis. A WHO/ICF(M)A meeting.

Cystic fibrosis represents a major health problem in developed countries. Although its distribution is worldwide, in most developing countries there is little awareness of its prevalence and little opportunity to provide treatment. The improvements in survival of cystic fibrosis patients in developed countries over the past two decades have brought mean life expectancy to 20 years or more, and many adult patients with cystic fibrosis at present are fully integrated in society and have an acceptable lifestyle. Improvements in standard of living, general health, diet, surgical and medical care, including nutritional support, pancreatic enzyme supplementation and antibiotic therapy have all contributed to extended survival of patients and improved quality of life. The role of the community is important in providing support for cystic fibrosis patients and their families. Cystic fibrosis organizations, and mutual-help parent groups play a vital part in educating the public about the disease, disseminating information, and supporting research.The lack of knowledge regarding the fundamental defect of the disease and the lack of appropriate methods for fetal diagnosis and heterozygote detection, limit the possibility of controlling the disease. International collaboration is required to provide internationally useful guidelines for treatment, to promote initiatives in public health, and to assist in the dissemination of educational material. In practice this will be most important in developing countries, where underdiagnosis of cystic fibrosis is widespread. Definition of the gene for cystic fibrosis and its resultant metabolic defect will require intensive specialized and systematic fundamental research, which could also be accelerated by international collaboration.

Adult↗

PubMed1998

Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis.

BACKGROUND: Osteoporosis occurs in patients with cystic fibrosis as they age, but its clinical implications are not well defined. OBJECTIVE: To determine the clinical effect of decreased bone mineral density in adults with cystic fibrosis and to assess possible clinical predictors of osteoporosis. DESIGN: Retrospective cohort study. SETTING: Academic cystic fibrosis center. PATIENTS: 70 adults with late-stage cystic fibrosis who were referred for lung transplantation. MEASUREMENTS: Bone mineral density was measured with dual-energy x-ray absorptiometry, patient-reported fracture events were confirmed by radiography, and kyphosis angles were measured by using the Cobb method. RESULTS: Mean bone mineral densities for the spine, femur, and total body were severely depressed in patients with cystic fibrosis, averaging 2 SDs below those of age-matched normal controls (P < 0.001). Patient interviews showed that 54 fractures had occurred over 1410 patient-years, and chest radiographs showed evidence of 14 additional rib and 62 additional vertebral compression fractures. The database (which covered 1410 patient-years) showed that fracture rates were approximately twofold greater in women with cystic fibrosis aged 16 to 34 years (P = 0.015) and men with cystic fibrosis aged 25 to 45 years (P = 0.04) than in the general population. Vertebral compression and rib fractures were 100- and 10-fold more common than expected, respectively (P < 0.001 for both comparisons). The mean kyphosis angle (+/- SD) for this group was markedly abnormal (44 +/- 14 degrees; 62% > or = 40 degrees) and probably contributed to diminished stature (mean height loss, 5.8 cm in men with cystic fibrosis and 5.9 cm in women with cystic fibrosis). Cumulative prednisone dose, body mass index, and age at puberty were the strongest predictors of bone mineral density. CONCLUSIONS: Osteoporosis is universal in adults with late-stage cystic fibrosis, and its complications include increased fracture rates and severe kyphosis.

Adult↗

PubMed2004

Systemic inflammatory mediators and cystic fibrosis genotype.

Morbidity and mortality in cystic fibrosis patients is mainly attributed to pulmonary infection and inflammation. Chemokines play a pivotal role in the inflammatory process. Although genotype-phenotype correlation in cystic fibrosis patients has been defined, a clear relationship between the defect in the cystic fibrosis transmembrane regulator (CFTR) gene and pulmonary inflammation has not been established. The aim of this study was to assess whether serum chemokines levels in cystic fibrosis patients correlate with genotype and pulmonary function tests, as well as with other clinical characteristics. Serum levels of interleukin-8, RANTES, and monocyte chemoattractant protein-1 were measured in 36 cystic fibrosis patients grouped according to their genotype. Group A included 25 patients who carried two mutations associated with a pathological sweat test and pancreatic insufficiency (deltaF508, W1282X, G542X, N1303K, S549R). Group B included 11 compound heterozygote patients who carried one mutation known to cause mild disease with borderline or normal sweat test and pancreatic sufficiency (3849+10kb C to T, 5T). Associations between chemokine levels, genotype, pulmonary function, Pseudomonas aeruginosa colonization, age, sweat chloride level, and pancreatic and nutritional status were examined. Mean interleukin-8 and monocyte chemoattractant protein-1 levels were significantly higher in group A than group B (11.4 +/- 2.1 pg/ml vs. 5 +/- 0.9 pg/ml and 157 +/- 16 pg/ml vs. 88.8 +/- 16.4 pg/ml, respectively) (P < 0.01). No difference in RANTES levels were found between groups. interleukin-8 levels were inversely related to forced expiratory volume in 1 s (r = -0.37, P < 0.02), while there was no association between the latter and RANTES and monocyte chemoattractant protein-1 levels. The Pseudomonas colonization rate was higher among group A patients than group B (88% vs. 40%, P < 0.01). No relationship was found between measured chemokines and age, sweat chloride levels, and pancreatic and nutritional status. Our study demonstrates an association between interleukin-8, forced expiratory volume, and cystic fibrosis genotype. Hence, elevated interleukin-8 serum levels could serve as an indicator of an early inflammatory process and encourage the initiation of anti-inflammatory treatment.

Adolescent↗

PubMed1986

Low levels of pyridoxal 5'-phosphate in patients with cystic fibrosis.

To determine the effect of cystic fibrosis on the regulation of plasma pyridoxal 5'-phosphate (PLP), the biologically active form of vitamin B6, we measured this compound in plasma from 56 patients with cystic fibrosis. The concentration of PLP in plasma was assayed by a radioenzymatic technique. The results of this study showed that PLP concentration was decreased significantly (6.44 +/- 5.20 ng/mL, mean +/- SD; median 4.45 ng/mL) in patients with cystic fibrosis as compared with a group of hospitalized children with neither cystic fibrosis nor hepatic disease serving as a control group (13.2 +/- 5.04 ng/mL, mean +/- SD; median 12.5 ng/mL). Additionally, 25% of the population with cystic fibrosis exhibited exceedingly low plasma PLP level (less than 2.75 ng/mL). In patients with cystic fibrosis, significant inverse linear associations were found between plasma PLP and serum levels of SGOT and SGPT (PLP v SGOT: r = -.60, P less than .03; PLP v SGPT: r = -.50, P less than .03). This study demonstrated that a deficiency of plasma PLP is a common abnormality in cystic fibrosis and that the low PLP level may be a reflection of impaired vitamin B6 metabolism associated with this disorder.

Adolescent↗