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PubMed1977

Immune complexes in cystic fibrosis.

Eleven patients with cystic fibrosis (CF) chronically infected with mucoid P. aeruginosa and ten patients without P. aeruginosa infection were examined for occurrence of circulating immune complexes, for immune complex deposits in the dermo-epidermal junction of the skin and for precipitins against P. aeruginosa, S. aureus, H. influenzae and D. pneumoniae antigens. The serum concentrations of haptoglobin, orosomucoid, immunoglobulins, C1q, C3, C4 and total haemolytic complement, antinuclear and rheumatoid factor activities as well as white blood cell counts and erythrocyte sedimentation rates were determined also. The results indicated that 6 patients from the chronically P. aeruginosa infected group, exhibiting a spectrum of serum precipitins against P. aeruginosa antigens, also had immune complexes in the serum, while only one patient (suffering from selective IgA deficiency) in the group without P. aeruginosa infection was positive for soluble immune complexes. Granular deposits of IgM was found in the skin of 10 of the chronically P. aeruginosa infected patients and in 7 of the patients without P. aeruginosa infection. A few pactients in both groups had dermo-epidermal deposits of C1q, C3 or fibrinogen as well. Eight of the patients in the chronically infected group and five in the group without P. aeruginosa infection had organ non-specific antinuclear factors. The haptoglobin levels appeared to be elevated in the chronically infected patients (p less than 0.05). None of the other parameters showed any significant difference between the two groups.

Adolescent↗

PubMed1999

Surface hydrophobicity is increased in the ileum and proximal colon of cystic fibrosis mice.

Patients with cystic fibrosis (CF) have abnormal concentrations and composition of electrolytes and macromolecules in gastrointestinal secretions. Such alterations could change intestinal surface properties, such as surface hydrophobicity, and may influence the adhesion of macromolecules, bacteria, or microbial toxins to the intestinal surface. The objective of this study was to compare the surface hydrophobicity of the gastrointestinal tract in wild type and CF mice. We used axisymmetric drop shape analysis-contact diameter to determine surface hydrophobicity by measuring contact angles of sessile water droplets placed onto epithelial surfaces. In wild type mice, there were no differences in contact angles between the duodenum, upper jejunum, lower jejunum, and ileum. The contact angle of the gastric mucosa was lower than the rest of the gastrointestinal tract. Contact angles of the proximal colon and distal colon were both higher than that of the gastric mucosa and those of the small intestinal sections. In CF mice, contact angles along the gastrointestinal tract followed the same pattern as in wild type mice. However, contact angles in the ileum and proximal colon of CF mice were greater than those from wild type mice. This study of the murine intestine showed regional differences in surface hydrophobicity comparable to those observed in other mammalian species. In addition, we showed that the ileum and proximal colon of CF mice were more hydrophobic than the corresponding segments in wild type mice. These observations are of potential clinical relevance because patients with CF exhibit clinical manifestations of gastrointestinal disease primarily in the ileum and proximal colon.

Animals↗

PubMed1990

A new polymorphic locus, D7S411, isolated by cloning from preparative pulse-field gels is close to the mutation causing cystic fibrosis.

The mutation causing cystic fibrosis (CF) has been localized to the DNA sequence of 700 kb bounded by the loci identified by the markers pMP6d-9 (D7S399) and pJ3.11 (D7S8). A 560-kb fragment obtained after SacII digestion of DNA from a cell line containing this region of human chromosome 7 in a mouse background was separated using pulse-field gel electrophoresis and isolated from the gel. The DNA was digested with BamHI prior to cloning into lambda EMBL3. Approximately 0.1% of the resulting clones contained human repetitive sequences, and 24 such recombinants were studied. Of these, 23 are on chromosome 7; 8 clones were duplicated, and of the 15 different recombinants, 7 are between MET and INT1L1, and a further 7 are between INT1L1 and pMP6d-9, leaving a single marker, pG2, which is between pMP6d-9 and pJ3.11. pG2 recognizes an RFLP with XbaI. A cosmid walk from pG2 has generated a further marker, H80, which recognizes an RFLP with PstI. This new locus (D7S411) divides the remaining region between the CF flanking markers, thereby making it more accessible to fine pulse-field mapping and allowing the precise localization of further clones to this region. Although it is not possible to position the CF locus unequivocally with respect to D7S411, both polymorphic markers at this locus exhibit low but significant linkage disequilibrium with CF, placing the emphasis for the search for the gene on the D7S399 to D7S411 interval of 250 kb.

Blotting, Southern↗

PubMed1991

Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium.

Cystic fibrosis (CF) airway epithelia exhibit raised transepithelial Na+ transport rates, as determined by open-circuit isotope fluxes and estimates of the amiloride-sensitive equivalent short-circuit current (Ieq). To study the contribution of apical and basolateral membrane paths to raised Na+ transport in CF, CF nasal epithelial cultures were studied with double-barreled Na(+)-selective microelectrodes and the Ussing chamber technique. Intracellular Na+ activity (acNa) was 24.1 +/- 1.5 mM (n = 36), a value similar to acNa of normal nasal epithelial cells. Reduction of luminal [Na+] to 3 mM abolished Ieq and reduced acNa. Amiloride (10(-4) M) abolished Ieq but increased acNa from 20 +/- 2 to 36 +/- 7 mM (n = 10). Amiloride-induced increase in acNa was not affected by serosal [Na+] reduction but was blocked by preexposure to reduced luminal [Na+]. Amphotericin B increased Ieq during amiloride exposure, indicating that amiloride did not inhibit NA(+)-K(+)-ATPase. Ouabain abolished Ieq and slowly raised acNa. Reduction of serosal [Na+] led to a decrease in acNa that was blocked by bumetanide. It is concluded that 1) CF airway epithelia exhibit an increased apical membrane Na+ permeability, 2) acNa is regulated to a normal level in CF cells despite increased transcellular Na+ fluxes, 3) the abnormal increase in acNa in response to amiloride is dependent on luminal Na+, 4) Na+ is transported across the basolateral membrane by a bumetanide-sensitive cotransport mechanism, and 5) ouabain inhibits the basolateral Na(+)-K(+)-ATPase, causing slow dissipation of the chemical and electrical gradients across the cell membranes.

Adolescent↗

PubMed1987

Cystic fibrosis--an otolaryngologic perspective.

Cystic fibrosis (CF) is the most common lethal genetic disorder in white patients. The protean manifestations of the disease result from exocrine gland dysfunction and include chronically debilitating pulmonary and pancreatic compromise and clinically inconsequential (although diagnostically extremely important) sweat electrolyte abnormalities. The subject of this article is the otolaryngologic manifestations of the disease, based on a retrospective analysis of 450 cases. Nasal polyposis and sinusitis occurred in 10% and 11% of patients, respectively, and polypectomy was, after laparotomy, the most common surgical procedure these children underwent. The extent of intranasal surgery for polyposis was found to be inversely proportional to the recurrence rate. A simple polypectomy was relatively ineffective treatment; when performed in conjunction with a Caldwell-Luc and either an intranasal or extranasal ethmoidectomy, the recurrence rate was less than 13%. Otologic problems, found in 8% of patients, included chronic otitis media (2.5%) and acute otitis media (5.5%). Only five patients required pressure-equalizing tubes. Recent genetic advances of immense importance are also described. Although the basic gene defect has yet to be elucidated, by use of a technique known as restriction-fragment-linked polymorphism, the gene associated with CF has been found in the middle of the long arm of chromosome 7. By following gene markers closely associated with this gene, it is possible to do carrier tests within affected families and, if certain criteria are met, perform prenatal diagnosis. Eventual isolation and characterization of the gene will follow, hopefully making prevention possible and treatment more effective.

Adolescent↗

PubMed1994

Effect of salbutamol on gas compression in cystic fibrosis and asthma.

In cystic fibrosis (CF), it has been suggested that increases in FEV1 postbronchodilator (BD) can be accompanied by paradoxical decreases in isovolume maximal flow at 25% of vital capacity (V25iso) measured from maximum expiratory flow-volume curves (MEFVC), raising concerns about determining the benefits of BD in CF. MEFVC measured using expired volume has been shown to be subject to errors due to gas compression. In the present study, BD response was assessed in 91 patients with asthma and 78 with CF using the percentage of change in FEV1 and V25iso determined using MEFVC from both mouth (m) and plethysmograph (p) volumes. From the two curves, volume of compression (Vcomp) was measured. Baseline measurements were similar for both groups, except that the residual volume to total lung capacity ratio (RV/TLC) was higher and Vmax25 and FVC were lower in CF. Both groups showed significant (p < 0.05) increases in FVC, FEV1, and V25iso after BD. The percentage of change in FEV1 correlated with the percentage of change in V25iso (r = 0.53 for CF and 0.66 for asthma, p < 0.001). Baseline Vcomp25 was higher in asthma than in CF. The percentage of change in V25iso was not related to the change in Vcomp25iso for either group. Only four patients with CF showed a paradoxical decrease in V25iso, and the differences in flow were small. Two had an increase in Vcomp and two had a decrease. We conclude that paradoxical decreases in V25iso are rare, are associated with small changes in flow, and are not related to changes in Vcomp.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed2005

Effect of nebulized albuterol on blood glucose in patients with diabetes mellitus with and without cystic fibrosis.

Over 90% of cystic fibrosis (CF) patients are treated with bronchodilators, and 6% have diabetes. Some with asthma also have diabetes, and most are treated with bronchodilators. Systemic administration of adrenergic agents can cause increases in blood glucose, but the effect of inhaled agents is unclear. A double-blind study was performed on 10 patients with type 1 diabetes mellitus (DM) without CF (3 male, 7 female, mean age 25.5 years) and 9 patients with insulin-dependent CF-related diabetes (CFRD) (8 male, 1 female, mean age 21.9 years). On 2 separate days before 9 AM fasting and the morning dose of insulin, 2.5 mg of albuterol or nebulized placebo were given. Blood glucose was measured by finger stick with a glucose reflectance meter before and 15, 30, 45, and 60 min after treatment. No significant changes from baseline or differences between placebo and albuterol occurred in either group. The mean maximum increase from baseline in DM was 20 mg/dl on placebo, and 38 mg/dl on albuterol; in the CFRD, the respective changes were 7 and 7 mg/dl. Two DM patients had a > 50 mg/dl increase on albuterol vs. placebo; no CFRD patients had differences of such magnitude. DM patients had greater increases from baseline than CFRD patients on placebo and albuterol. Differences reached statistical significance at 30 and 45 min on placebo, and 45 min on albuterol. Albuterol 2.5 mg by nebulizer causes no clinically significant increases in blood glucose in DM or CFRD patients. Diabetes patients without CF have a significantly greater increase of glucose with time (placebo or albuterol) than CFRD patients.

Administration, Inhalation↗

PubMed2006

Factors increasing the risk for stone formation in adult patients with cystic fibrosis.

BACKGROUND: Patients with cystic fibrosis (CF) are at high risk of nephrolithiasis (NL), but controversy still exists in terms of causes, including low urine output, hypercalciuria, hyperoxaluria, hyperuricosuria and hypocitraturia. Moreover, heterozygotes (H-CF), which may exhibit altered renal concentrating and diluting ability, have never studied so far. We, therefore, evaluated the metabolic and physicochemical data of adult CF and H-CF patients, comparing them to controls (C). METHODS: Twenty-nine CF patients (16 females, aged 28.4 +/- 7.1 years), 20 H-CF (12 females, aged 58.6 +/- 6.3 years) and 30 C (19 females, aged 39.1 +/- 11.5 years) underwent kidney ultrasound and metabolic evaluation to assess stone risk profile. RESULTS: There was a 21% prevalence of NL in CF vs 15% in H-CF. The CF group had elevated uric acid, but no other serological differences compared with the H-CF and C group. Conversely, the citrate and oxalate content in the urine differed significantly, being lower and higher, respectively. These changes held after correction for urine creatinine. Consequently, urine specimens were more supersaturated with calcium oxalate, despite exhibiting no differences for other relevant constituents. Uric acid increased only after normalization for the body weight and urine creatinine. Lower urine volume and more acidic pH produced mild supersaturation with uric acid in samples from CF, while urine from both H-CF and C remained undersaturated. H-CF had only minor increases in both urine oxalate and calcium oxalate supersaturation. CONCLUSIONS: This study confirms a high prevalence of kidney stones among CF patients associated with supersaturated urine. Their longer survival justifies diets and/or medications aimed at reducing the risk of forming stones.

Adult↗

PubMed2006

Relationship between insulin-like growth factor I, dehydroepiandrosterone sulfate and proresorptive cytokines and bone density in cystic fibrosis.

INTRODUCTION: Patients with cystic fibrosis (CF) are known to be at risk for early osteoporosis, and the mechanisms that mediate bone loss are still being delineated. The aim of the present investigation was to investigate if a correlation exists in these patients between skeletal measurements by dual-energy x-ray absorptiometry (DXA) and two anabolic factors, dehydroepiandrosterone (DHEA) and insulin-like growth factor I (IGF-I), and proresorptive factors such as the cytokines interleukin-1beta, tumor necrosis factor alpha, and interleukin-6. METHODS: We studied 32 outpatients (18 females; mean age: 26.2+/-7.9 years) at a tertiary care medical center. The subjects had venous samples obtained, underwent anthropometric and bone mineral density (BMD) measurements, and completed a health survey. Serum IGF-I concentrations were below the age-adjusted mean in 78% of the participants, and DHEA sulfate (DHEAS) concentrations were low in 72%. Serum concentrations of all cytokines were on the low side of normal; nonetheless, there was a modest inverse correlation between IL-1beta and BMD at all sites. RESULTS: In univariate analyses, IGF-I and DHEAS were significant correlates of BMD or bone mineral content. In final multivariate models controlling for anthropometric and other variables of relevance to bone density, only IGF-I was identified as a significant independent skeletal predictor. While alterations in DHEAS, IGF-I, and specific cytokines may contribute to skeletal deficits in patients with CF, of these factors a low IGF-I concentration appears to be most strongly correlated with BMD. CONCLUSIONS: These findings may have therapeutic implications for enhancing bone density in these patients.

Adolescent↗

PubMed2005

The effect of infective exacerbations on sleep and neurobehavioral function in cystic fibrosis.

RATIONALE: Adults with cystic fibrosis (CF) are susceptible to hypoxemia, hypercapnia, arousal from sleep, and neurobehavioral impairment. OBJECTIVES: We hypothesized that pulmonary exacerbations would adversely affect sleep and neurobehavioral performance. METHODS: Patients with exacerbations (cases) had sleep studies and neurobehavioral testing before and after inpatient intravenous therapy. Adults with stable CF underwent the same testing procedures (control subjects). MEASUREMENTS AND MAIN RESULTS: When clinically stable, cases and control subjects had similar lung function, intelligence, and body mass index. Among cases, treatment of an exacerbation improved lung function, quality of life, mood, sleepiness, and activation. Cases spent more time awake after sleep onset (p = 0.02), less time in REM sleep (p = 0.03), and were more hypoxemic than control subjects when unwell. The severity of hypoxemia correlated with lung function. On admission, cases had slower throughput than control subjects in the serial addition and subtraction task (cases, 16 +/- 4, vs. control subjects, 17 +/- 3; F[1, 36] = 5.15, p = 0.03) and a slower response time on the digit symbol substitution task (F[1, 36] = 11.91, p = 0.001), which persisted after treatment (F[1, 36] = 8.48, p = 0.006). Cases experienced significant improvements in sleep efficiency, amount of REM sleep, and hypoxia with treatment. Their performance in the serial addition and subtraction task, psychomotor vigilance task, and simulated driving task also improved with treatment. Sex modified the effect of an exacerbation on some aspects of performance. CONCLUSIONS: Exacerbations of lung disease in adults with CF adversely affect sleep and tests of neurobehavioral performance regardless of underlying disease severity. The implications for performance in daily life need further evaluation because patients often delay admission to hospital to fulfill study or work commitments.

Adolescent↗

PubMed2003

Effect of chondroitinase ABC on purulent sputum from cystic fibrosis and other patients.

Cystic fibrosis (CF) patients develop chronic lung infections associated with airway obstruction by viscous and insoluble mucus secretions. Although mucus glycoproteins (mucins) are thought to be responsible for mucus plugs, other glycoconjugate components of airway secretions have not been systematically evaluated. The aim of the present study was to determine whether chondroitin sulfate proteoglycans (CSPG) contribute to the insolubility of CF sputum. Sputa obtained from 18 CF patients were incubated with chondroitinase ABC (ChABC) or buffer (control) for 18 h at 37 degrees C, and after centrifugation at 12,000 g, the volume of the insoluble pellet and turbidity of the supernatant were determined as measures of solubility. ChABC caused a 70-90% reduction in supernatant turbidity and a 60-70% decrease in pellet volume of the 13 purulent CF sputa, but had much less effect on the five nonpurulent CF sputa tested. Similar results were obtained with two non-CF purulent and two non-CF, nonpurulent sputa. Gel electrophoresis, Western blot, and slot blot immunoassays with antichondroitin sulfate and antimucin antibodies revealed that purulent sputa (CF and non-CF) contained more CSPG and less mucin than nonpurulent sputa. In vitro mixing experiments showed that mucin in nonpurulent sputa was reduced upon incubation with purulent sputa, presumably because of degradation or a loss of immunoreactive mucin epitopes from leukocyte and/or bacterial enzymes present in purulent sputa. Our results suggest that CSPG contribute more significantly than mucins to the insolubility of purulent tracheobronchial secretions from CF patients. Because purulent sputa from non-CF patients showed a similar pattern, our observations with CF sputa may have wider applicability.

Adult↗

PubMed1999

Prevalence of vitamin K deficiency in cystic fibrosis.

BACKGROUND: Patients with cystic fibrosis (CF) are at risk of developing vitamin K deficiency because of pancreatic insufficiency, hepatobiliary disease, or both. OBJECTIVE: Our objective was to determine the prevalence of vitamin K deficiency in unsupplemented patients with CF and to identify risk factors that might be associated with the deficiency. DESIGN: Ninety-eight patients with CF-83 who were pancreatic insufficient (age: 15.2 +/- 10.7 y; range: 0.6-45.8 y), 15 who were pancreatic sufficient (age: 26.2 +/- 11.6 y; range: 6.5-45.3 y), and 62 healthy individuals (age: 16.2 +/- 12. 8 y; range: 1-45 y)-were studied prospectively. None had taken vitamin K supplements. Eight pancreatic-insufficient patients had advanced CF-associated liver disease. Plasma prothrombin in vitamin K absence (PIVKA-II) was measured by immunoassay. All control subjects had PIVKA-II concentrations <3 microg/L. RESULTS: Seventy-eight percent of pancreatic-insufficient patients had PIVKA-II concentrations >/=3 microg/L (22.8 +/- 35.7 microg/L). All patients with CF-associated liver disease had abnormal PIVKA-II concentrations. The mean PIVKA-II concentration of pancreatic-insufficient patients with liver disease was greater than that of those without liver disease (46.6 +/- 65.3 compared with 15. 3 +/- 26.1 microg/L; P < 0.05). Five pancreatic-sufficient patients had mildly elevated PIVKA-II concentrations. Six (7%) pancreatic insufficient patients (3 with CF-associated liver disease) had mildly prolonged prothrombin time but no clinical bleeding. There was no correlation between PIVKA-II concentrations and severity of fat malabsorption or antibiotic use. CONCLUSIONS: Vitamin K deficiency is common in unsupplemented patients with CF and pancreatic insufficiency and routine supplementation should be considered in all of these patients.

Adolescent↗

PubMed2000

Comparison of total body chlorine, potassium, and water measurements in children with cystic fibrosis.

BACKGROUND: Symptoms of cystic fibrosis (CF) may limit the utility of total body chlorine (TBCl) and total body potassium (TBK) measurements for assessing body fluid compartments of children. OBJECTIVE: This study assessed relations among independent measurements of TBCl, TBK, and total body water (TBW) in children with CF. DESIGN: We compared cross-sectional measurements of TBCl by in vivo neutron activation analysis, TBK by whole-body counting of (40)K, TBW by D(2)O dilution [TBW(D(2)O)], and TBW from TBCl and TBK [TBW(Cl + K)] in 19 prepubertal children (13 boys) aged 7.6-12.5 y who had mild symptoms of CF. Body-composition measurements were compared with data from previous studies of healthy children. RESULTS: Subjects with CF had deficits in TBCl, TBK, TBW, and body weight compared with control reference data (P < 0.05). The ratios (TBCl + TBK)/TBW and TBCl/TBK were not significantly different from control reference values, and plasma chlorine and potassium concentrations were within control reference ranges. The sum of TBCl and TBK correlated with TBW(D(2)O) (r(2) = 0.79, P < 0.001), and TBW(Cl + K) correlated with TBW(D(2)O) (r(2) = 0.78, P < 0.001). TBW(Cl + K) was similar to TBW(D(2)O) (mean +/- SEM: 19.0 +/- 0.5 compared with 19.4 +/- 0.5 L; NS). CONCLUSIONS: Prepubertal children with mild symptoms of CF can develop deficits in TBCl, TBK, and TBW that reflect chronic energy malnutrition. Mild symptoms of CF do not appear to affect normal relations among TBCl, TBK, and TBW. Measurements of TBCl and TBK may be used to assess body fluid compartments in these patients.

Analysis of Variance↗

PubMed2001

Effect of intravenous antibiotics on exercise tolerance (3-min step test)) in cystic fibrosis.

Most children with cystic fibrosis (CF) feel better and display more energy after a course of intravenous antibiotics (IVABs), but this is not always reflected by a satisfactory improvement in lung function. We assessed the change in exercise tolerance after treatment with IVABs using the 3-min step test, and compared it with changes in spirometric lung function and arterial oxygen saturation (SaO(2)). Thirty-six children (mean age, 13.8 years) were enrolled from two tertiary CF centers during an inpatient stay for IVABs. After 10-14 days of treatment, there was a significant improvement in median FEV(1) from 43% to 57% of predicted values (P < 0.0001), and median FVC from 66% to 73% of predicted values (P < 0.0001), while median SaO(2) significantly increased from 95% to 96.5% (P < 0.05). This was accompanied by a reduction in resting heart rate (median 118 bpm to 109 bpm, P < 0.005) and subjective breathlessness at rest (median visual analogue score 2.2 to 0.8, P < 0.005). All outcomes of exercise tolerance were improved after IVABs. There was a reduction in maximum heart rate (median 156 bpm to 150 bpm, P < 0.05) and an increase in minimum SaO(2) (median 93.5% to 94.5%, P = 0.08) measured during the step test. There was also a reduction in subjective breathlessness (median visual analogue score of 5.5 to 4.2, P < 0.005) and objective breathlessness (median 15-count score of 3 to 2, P < 0.0001) measured immediately after the step test. Exercise testing was a useful outcome measure for monitoring effectiveness of inpatient therapy, and complemented spirometry and SaO(2) monitoring. The simple ward-based 3-min step test was found to be a particularly suitable method for measuring changes in exercise tolerance in children with CF.

Adolescent↗

PubMed2006

Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis.

BACKGROUND: Persons with cystic fibrosis (CF) and pancreatic insufficiency (PI) are at risk of vitamin A deficiency because of steatorrhea, despite pancreatic enzyme replacement. Long-standing vitamin A supplementation may increase the risk of vitamin A toxicity. OBJECTIVE: The aim was to describe the vitamin A intake and serum retinol concentrations of preadolescent children with CF, PI, and mild-to-moderate pulmonary disease, who were cared for under current practice recommendations. DESIGN: This cross-sectional study evaluated children aged 8.0-11.9 y with CF and PI from 13 US CF centers. Dietary and supplemental vitamin A intakes were compared with the Dietary Reference Intakes (DRIs) for healthy children, CF recommendations, and data from the National Health and Nutrition Examination Survey (NHANES), 1999-2000. Serum retinol concentrations were compared with NHANES data. RESULTS: The 73 subjects with CF had a dietary vitamin A intake of 816 +/- 336 microg retinol activity equivalents (165 +/- 69% of the recommended dietary allowance), which was similar to the NHANES value. The supplement intake provided 2234 +/- 1574 microg retinol activity equivalents/d and exceeded recommendations in 21% of the subjects with CF. Total preformed retinol intake exceeded the DRI tolerable upper intake level in 78% of the subjects with CF. The serum retinol concentration was 52 +/- 13 microg/dL (range: 26-98 microg/dL), which was significantly higher than the NHANES value (37 +/- 10 microg/dL; range: 17-63 microg/dL; P < 0.001). CONCLUSION: Although supplementation helps to prevent vitamin A deficiency in children with CF and PI, their high vitamin A intakes and serum retinol concentrations suggest that usual care may result in excessive vitamin A intake and possible toxicity that would increase the risk of CF-associated liver and bone complications.

Child↗

PubMed2006

Moxifloxacin but not ciprofloxacin or azithromycin selectively inhibits IL-8, IL-6, ERK1/2, JNK, and NF-kappaB activation in a cystic fibrosis epithelial cell line.

Cystic fibrosis (CF) is associated with severe neutrophilic airway inflammation. We showed that moxifloxacin (MXF) inhibits IL-8 and MAPK activation in monocytic and respiratory epithelial cells. Azithromycin (AZM) and ciprofloxacin (CIP) are used clinically in CF. Thus we now examined effects of MXF, CIP, and AZM directly on CF cells. IB3, a CF bronchial cell line, and corrected C38 cells were treated with TNF-alpha, IL-1beta, or LPS with or without 5-50 microg/ml MXF, CIP, or AZM. IL-6 and IL-8 secretion (ELISA), MAPKs ERK1/2, JNK, p38, and p65 NF-kappaB (Western blot) activation were measured. Baseline IL-6 was sixfold higher in IB3 than C38 cells but IL-8 was similar. TNF-alpha and IL-1beta increased IL-6 and IL-8 12- to 67-fold with higher levels in IB3 than C38 cells post-TNF-alpha (P < 0.05). Levels were unchanged following LPS. Baseline phosphorylated form of ERK1/2 (p-ERK1/2), JNK, and NF-kappaB p65 were higher in IB3 than C38 cells (5-, 1.4-, and 1.4-fold), and following TNF-alpha increased, as did the p-p38, by 1.6- to 2-fold. MXF (5-50 microg/ml) and CIP (50 microg/ml), but not AZM, suppressed IL-6 and IL-8 secretion by up to 69%. MXF inhibited TNF-alpha-stimulated MAPKs ERK1/2, 46-kDa JNK, and NF-kappaB up to 60%, 40%, and 40%, respectively. In contrast, MXF did not inhibit p38 activation, implying a highly selective pretranslational effect. In conclusion, TNF-alpha and IL-1beta induce an exaggerated inflammatory response in CF airway cells, inhibited by MXF more than by CIP or AZM. Clinical trials are recommended to assess efficacy in CF and other chronic lung diseases.

Anti-Infective Agents↗

PubMedDate not supplied

Forced expirations against a variable resistance: a new chest physiotherapy method in cystic fibrosis.

Twenty patients with cystic fibrosis (CF) were trained to mobilize intrabronchial secretions by a new method of positive expiratory pressure mask (PEP mask) physiotherapy (PT). Patients repeatedly expired forcefully through the PEP mask; expiratory resistance was varied by eight different internal diameter resistors at the outlet. Forced vital capacity with the mask (FVCPEP) was determined for each resistor and was compared with the one measured without the instrument (FVC); simultaneously the sustained expiratory pressure (SEP) developed against the resistance was recorded. After training, each patient exceeded FVC with one or more resistors; "optimum" FVCPEP was 124 +/- 16% FVC at a SEP of 61 +/- 21 cm H2O. Using the PEP mask, patients cleared a higher percentage of their daily sputum volume than with conventional PT (78 +/- 22% versus 53 +/- 17%, p less than 0.01). In 11 patients (subgroup 1), multiple aspects of lung function were measured at the beginning of the study, after 10 months with PEP mask PT, after two further months without using the mask but with conventional PT, and after six more months with PEP mask PT. Results showed significantly increased expiratory flow rates, significantly decreased hyperinflation and airway instability with PEP mask PT, and a marked decline of lung function without it. The remaining nine patients (subgroup 2) entered into the same protocol; based on findings in subgroup 1 and on ethical considerations, however, the control period without PEP mask PT was then omitted, resulting in a steady and statistically significant improvement of lung function over the entire observation period. In the course of preliminary trials, two children transiently used a suboptimal resistance (FVCPEP less than FVC) and responded with a deterioration of lung function. By dilating airways and evacuating trapped gas, this method of PEP mask PT improves lung function and mucus clearance in CF. Thorough practice with the technique and frequent control of the optimal resistance are mandatory.

Adolescent↗

PubMed1990

[Quantitative two-dimensional echocardiography. Study results in patients with cystic fibrosis].

15 patients with cystic fibrosis aged 8-19 years were graduated into 3 stages of the cor pulmonale dependent on results of an examination with the microcatheter: stage 0:6, stage I: 4 and stage II: 5 patients. These patients were studied in the period from April 1987 to January 1988 echocardiographically. With this size and contractility of the right ventricle were especially considered. The end-diastolic and end-systolic area of the right ventricle could be measured in the apical 4-chamber-view in 14 of the altogether 15 patients and out of this the fractional area-shortening could be calculated. The end-diastolic area of the right ventricle in the patients with stage 0 was on an average 11.4 cm2/m2 body surface bs (8.7-13.5), in stage I 11.2 cm2/m2 bs (9.2-13.5), and in stage II 9.7 cm2/m2 bs (9.0-10.8). The end-systolic area of the right ventricle in stage 0: 7.2 cm2/m2 bs (6.8-8.4), stage I: 7.1 cm2/m2 bs (6.0-8.5), and stage II: 8.02 cm2/m2 bs (6.4-10.1). The results we found don't differ significantly in the 3 groups. Contrary to this fractional area-shortening of the right ventricle shows a falling tendency: stage 0.35.2% (22-49), stage I 32% (7-56), stage II 17.2% (4-38) with an increasing part of patients with a value under 28% (stage 0:1 patients, stage I 2 patients, stage II 3 patients). Contractility and pump-efficiency of the left ventricle were calculated in the apical 4-chamber-view according of the area-length-method. The findings don't show an increasing size of the right ventricle in the sense of a cor pulmonale but a decreasing contractility and pump-efficiency of the right ventricle as expressions of a cardial manifestation of the basic illness in the sense fo a symptomatic cardiomyopathy. A disturbance of the contractility of the left ventricle could be seen in 7 of the 15 patients (47%).

Adolescent↗