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PubMed1993

Demographic and social characteristics of adults with cystic fibrosis in the United Kingdom.

OBJECTIVE: To obtain information about social and demographic characteristics and lifestyle of adult patients with cystic fibrosis, including those who do not attend major specialist clinics. DESIGN: Confidential self completion postal questionnaire to adult patients with cystic fibrosis, asking about social and demographic characteristics, social class and occupation, employment, education, insurance and social security benefits, symptom severity, and medical care. SETTING: National association for adults with cystic fibrosis. SUBJECTS: 1052 adult members of the Association of Cystic Fibrosis Adults UK, accounting for 68% of those with cystic fibrosis in the United Kingdom population over 16 years of age and over 80% of those over 25 in June 1990. RESULTS: The response rate was 82% (397 women, 423 men). Most adults with cystic fibrosis were found to be living fulfilling lives into adulthood. Significantly fewer men were married or cohabiting than women (110 (26%) men, 175 (44%) women). 420 (55%) responders were working, and of these 235 (56%) had less than two weeks' sick leave a year. Half of those not employed gave ill health as the reason. Revealing that they had cystic fibrosis at job interviews reduced likelihood of being employed for those with mild to moderate disease. People with cystic fibrosis had been less successful than the general population in achieving O level or equivalent qualifications, but more successful in achieving A level or higher qualifications. Achievement of any qualifications enhanced employment prospects irrespective of disease severity. CONCLUSION: Contrary to an image of chronic ill health and disability, a high proportion of adults with cystic fibrosis are living full and productive lives.

Adolescent↗

PubMed2004

Cystic fibrosis and lung transplantation--determination of the survival benefit.

INTRODUCTION: Cystic fibrosis is a well acknowledged indication for lung transplantation; however, the optimal timing for transplantation remains debatable. Liou et al. described a score for calculating 5-year probability of survival for patients with cystic fibrosis and concluded that only patients with a probability of survival < 30% gained a survival benefit from transplantation; those between 30% and 50% had equivocal survival effects from transplantation and those > 50% suffered negative effects. The aim of the present study was to determine the validity and applicability of this model. METHODS: Data from patients with cystic fibrosis transplanted between January 1995 and July 2001 were retrospectively reviewed. Survival score according to Liou was calculated from data collected before transplantation. Patients were classified according to 5-year probability of survival (group 1: < 30%, group 2: 30%-50%, groups 3-5: > 50%). Actuarial survival rates were calculated separately for each group and compared with the predicted probability of survival. RESULTS: During the observation period 27 patients were transplanted for cystic fibrosis. Three patients had to be excluded from further analysis because of incomplete pretransplant data. Fifteen patients were classified as group 1 and nine patients as group 2. No patients were eligible for groups 3 to 5. There were nine female patients and six males in group 1, mean age 22.1 +/- 4.9 years. Mean survival time was 918 +/- 787 days; 1-, 3- and 5-year survival rates were 66.6%. Three male patients and six females were classified as group 2, mean age 26.2 +/- 12.2 years. Mean survival time for this group was 701 +/- 617 days, and 1-, 3- and 5-year survival rates were 66.6%. CONCLUSION: We found that only patients with a 5-year probability of survival < 50% had been transplanted. For patients in groups 1 and 2 we report identical 5-year survival rates of 66.6%. According to our experience, cystic fibrosis patients with a 5-year probability of survival < 30% and also those between 30% and 50% gain a clear survival benefit from bilateral lung transplantation.

Actuarial Analysis↗

PubMed2002

Resting energy expenditure in young patients with cystic fibrosis receiving antibiotic therapy for acute respiratory exacerbations.

BACKGROUND: patients with cystic fibrosis commonly have severe malnutrition and growth retardation. Among possible causes of these manifestations are low caloric intake, loss of nutrients and increased resting energy expenditure. This study was designed to assess the influence of antibiotic therapy for infectious exacerbations on resting energy expenditure in young patients with cystic fibrosis. METHODS: We studied 17 patients with cystic fibrosis (mean age, 13.6 years). All were hospitalized to receive intravenous antibiotic therapy (mean duration, 2 weeks) for acute respiratory exacerbations. At the beginning of therapy and after it ended, all patients underwent blood chemical tests, anthropometrical measures, determination of body composition by bioelectrical impedance, spirometry, and indirect calorimetry. RESULTS: Antibiotic therapy led to a significant improvement in biochemical, spirometric variables and in estimated calorimetry measurements expressed in relation to fat-free mass. These findings suggest that infective exacerbations are among the causes of increased resting energy expenditure in young patients with cystic fibrosis. CONCLUSIONS: Indirect calorimetry may prove useful in the diagnosis of infective exacerbations and in monitoring the effect of antibiotic therapy in patients with cystic fibrosis.

Adolescent↗

PubMed2000

Identification of transport abnormalities in duodenal mucosa and duodenal enterocytes from patients with cystic fibrosis.

BACKGROUND & AIMS: The duodenum is a cystic fibrosis transmembrane conductance regulator (CFTR)-expressing epithelium with high bicarbonate secretory capacity. We aimed to define the role of CFTR in human duodenal epithelial bicarbonate secretion in normal (NL) subjects and patients with cystic fibrosis (CF). METHODS: Endoscopic biopsy specimens of the duodenal bulb were obtained from 9 CF patients and 16 volunteers. Tissues were mounted in modified Ussing chambers. Bicarbonate secretion and short-circuit current (Isc) were quantitated under basal conditions and in response to dibutyryl adenosine 3',5'-cyclic monophosphate (db-cAMP), carbachol, and the heat-stable toxin of Escherichia coli (STa). Duodenocytes were also isolated and loaded with the pH-sensitive fluoroprobe BCECF/AM, and intracellular pH (pH(i)) was measured at rest and after intracellular acidification and alkalinization. RESULTS: Basal HCO(3)(-) secretion and Isc were significantly lower in the CF vs. NL duodenal mucosa. In contrast to NL, db-cAMP failed to alter either HCO(3)(-) or Isc in CF tissues. However, in CF, carbachol resulted in an electroneutral HCO(3)(-) secretion, whereas STa induced electrogenic HCO(3)(-) secretion that was similar to NL. In CF and NL duodenocytes, basal pH(i) and recovery from an acid load were comparable, but pH(i) recovery after an alkaline load in CF duodenocytes was Cl(-) dependent, whereas in NL duodenocytes it was Cl(-) independent. CONCLUSIONS: These findings implicate CFTR in NL duodenal alkaline transport and its absence in CF. Although duodenal bicarbonate secretion is impaired in CF tissues, alternate pathway(s) likely exist that can be activated by carbachol and STa.

Acid-Base Equilibrium↗

PubMed1989

Differences in adhesion of Pseudomonas aeruginosa to mucin glycopeptides from sputa of patients with cystic fibrosis and chronic bronchitis.

Pseudomonas aeruginosa is the most prominent colonizer of the respiratory tract of patients with cystic fibrosis, but it is not known why this occurs. P. aeruginosa adheres to mucins from normal individuals, but mucins from cystic fibrosis patients have not been studied. To compare adhesion to mucins from cystic fibrosis with other mucins, we prepared highly glycosylated mucin glycopeptides from cystic fibrosis and chronic bronchitis patients by ion-exchange and gel-filtration chromatography and measured the adhesion of P. aeruginosa 1244 to these glycopeptides. We found (i) that the most mucinlike glycopeptides from P. aeruginosa-infected cystic fibrosis sputa showed less bacterial adhesion than did the corresponding bronchitis samples, (ii) that the most adhesive activity in cystic fibrosis samples came from a fraction that contains O and N glycopeptides and may be in part a degradation product of P. aeruginosa infection, and (iii) that highly glycosylated glycopeptides of the most acidic species (sialylated and sulfated) showed no adhesion at all. A single cystic fibrosis sample not infected by P. aeruginosa showed better binding in the adhesion-positive fractions than did the infected sputa. These studies suggest that cystic fibrosis mucins may be altered after infection is established, resulting in less binding to some fragments. However, since the clinical picture shows heavy mucus colonization, other receptors, such as cellular glycolipids which have been shed into mucus, may be contributing to this colonization.

Bacterial Adhesion↗

PubMed1981

Reappraisal of the chloride plate test as screening test for cystic fibrosis.

A rapid and simple screening test for detecting cystic fibrosis, described in 1956, has been used routinely for 21 years; the results during a 15-month period are compared with those using the quantitative pilocarpine iontophoresis sweat test. In the chloride agar plate test the concentration of chloride on the finger tips is evaluated accordingly to the intensity of the imprint. Readings of 2+ or less excluded cystic fibrosis in 1589 cases with only two doubtful instances, whereas 4+ readings were recorded in 198 cases of cystic fibrosis and 3+ readings in 15 cases of cystic fibrosis. In doubtful cases 4 individuals had 4+ readings and 11 had 3+ readings. This screening test does not replace the quantitative pilocarpine iontophoresis test but it does identify an individual who is likely to have the disease and who requires further tests. It is not reliable for infants aged under 2 months.

Agar↗

PubMed2001

Mutations of the cystic fibrosis gene in patients with chronic pancreatitis.

OBJECTIVE: Several studies have reported an increased frequency of cystic fibrosis gene mutations in idiopathic but not in alcoholic chronic pancreatitis. The impact of cystic fibrosis gene mutations on the long-term course of chronic pancreatitis has not been analyzed. The aim of our study was to determine the frequency of cystic fibrosis gene mutations in patients with chronic pancreatitis with long-term follow-up and to see whether patients with mutations have a clinically different natural course compared to those without mutations. METHODS: Eighty two patients with chronic pancreatitis and 11 patients with recurrent acute pancreatitis of our well defined pancreatitis cohort were screened for the 31 most common cystic fibrosis gene mutations. The impact of cystic fibrosis gene mutations on the long-term course of chronic pancreatitis was assessed. RESULTS: A cystic fibrosis gene mutation was detected in five of 49 patients with alcoholic chronic pancreatitis (10.2%; 2.3 times the expected frequency) and in three of 14 patients with idiopathic-juvenile chronic pancreatitis (21.4%; 4.8 times the expected frequency). No mutations were found in the remaining patients with chronic pancreatitis of rare causes, hereditary pancreatitis, and recurrent acute pancreatitis. The frequency of pancreatic calcifications was significantly higher in patients with alcoholic chronic pancreatitis without mutations. This result was not confirmed in patients with idiopathic-juvenile chronic pancreatitis. The duration of pain and the frequency of exocrine and endocrine insufficiency was comparable in both subgroups irrespective of the mutation status. CONCLUSION: Our data indicate a significantly increased frequency of cystic fibrosis gene mutations both in patients with alcoholic and idiopathic-juvenile chronic pancreatitis. The natural course was similar in patients with mutations compared to those without mutations.

Adult↗

PubMedDate not supplied

Screening for cystic fibrosis: the importance of using the correct tools.

BACKGROUND: Cystic Fibrosis (CF) is a potentially lethal genetic disorder. The most frequent mutation worldwide in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene is designated as the Delta F508 mutation. This mutation was found in only 33% of Pakistani patients studied. Since the common Pakistani mutations remain to be identified, appropriate screening tools are required to identify disease. Sweat chloride determinations remain the gold standard for diagnosing CF. This study was done to emphasize the importance of using the correct tests. METHODS: The study was conducted at the Aga Khan University Hospital. The CFTR delta F508 mutation was tested on blood samples from patients suspected with CF. Sweat chloride analysis using pilocarpine iontopharesis was done with a positive value of greater than 60 meq/L. RESULTS: 57 pediatric samples were screened for the delta F508 mutation and were positive in only 10.6% of all patients tested. 12/57 (21%) had a preliminary sweat test. 6/12 (50%) of these patients had an abnormal sweat test and 3/6 patients with an abnormal sweat chloride (50%) had deltaF508 mutations-- 2/6 (33%) were homozygotes and 1 was a compound heterozygote. Since 79% did not have a sweat test, it was difficult to assess whether this subset of patients had cystic fibrosis with a CFTR mutation other than the delta F508 tested or no CF. CONCLUSION: Sweat chloride analysis is critical to distinguish CF from other causes of severe pulmonary and pancreatic insufficiencies and to define patients requiring further analysis.

Chlorides↗

PubMed1992

The spectrum of cystic fibrosis mutations.

Although the major mutation causing cystic fibrosis accounts for almost 70% of mutant chromosomes screened, almost 300 sequence alterations have been identified in the gene during the past two and a half years. At least 230 of these mutations are probably associated with disease. This rapid accumulation of data is in part due to the highly coordinated effort by members of the Cystic Fibrosis Genetic Analysis Consortium. The information is not only essential to genetic diagnosis, but also will aid in understanding the structure and function of the protein, and possibly in correlating genotype with phenotype.

Cystic Fibrosis↗

PubMed2005

Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners.

The cystic fibrosis transmembrane conductance regulator (CFTR) is the product of the gene mutated in patients with cystic fibrosis (CF). CFTR is a cAMP-regulated chloride channel localized primarily at the apical or luminal surfaces of epithelial cells lining the airway, gut, exocrine glands, etc., where it is responsible for transepithelial salt and water transport. CFTR chloride channel belongs to the superfamily of the ATP-binding cassette (ABC) transporters, which bind ATP and use the energy to drive the transport of a wide variety of substrates across extra- and intracellular membranes. A growing number of proteins have been reported to interact directly or indirectly with CFTR chloride channel, suggesting that CFTR might regulate the activities of other ion channels, receptors, or transporters, in addition to its role as a chloride conductor. The molecular assembly of CFTR with these interacting proteins is of great interest and importance because several human diseases are attributed to altered regulation of CFTR, among which cystic fibrosis is the most serious one. Most interactions primarily occur between the opposing terminal tails (N- or C-) of CFTR and its binding partners, either directly or mediated through various PDZ domain-containing proteins. These dynamic interactions impact the channel function as well as the localization and processing of CFTR protein within cells. This review focuses on the recent developments in defining the assembly of CFTR-containing complexes in the plasma membrane and its interacting proteins.

Animals↗

PubMed2002

Molecular diagnosis of cystic fibrosis.

A review of the current molecular diagnosis of cystic fibrosis including an introduction to cystic fibrosis, the gene function, the phenotypic variation, who should be screened for which mutation, newborn and couple screening, quality assurance, phenotype-genotype correlation, methods and method limitations, options, statements, recommendations, useful Websites and treatments.

Base Sequence↗

PubMed1993

Proinsulin, proinsulin intermediate and insulin in cystic fibrosis.

OBJECTIVE: We determined the changes in insulin, intact proinsulin and 32-33 split proinsulin as markers of beta cell damage in assessing the state of carbohydrate intolerance in patients with cystic fibrosis. DESIGN: Measurements of insulin, proinsulin and 32-33 proinsulin concentrations in cystic fibrosis following oral glucose tolerance test (OGTT). SUBJECTS: Sixteen cystic fibrosis patients attending the chest outpatient clinic for follow-up, age range 14-42 years, and 14 healthy controls matched for age and body mass index. MEASUREMENTS: Insulin, intact proinsulin and 32-33 split proinsulin were measured immunoradiometrically at 0, 30, 60, 90 and 120 minutes after an OGTT. Glucose and HbA1 were also measured. RESULTS: Cystic fibrosis patients were divided into two groups according to their OGTT results: those with diabetes mellitus or impaired glucose tolerance and those with normal glucose tolerance. Insulin concentrations and insulin/glucose ratios at 30 minutes were lower in both cystic fibrosis groups in comparison with the control. There was also a significant increase in the time to reach peak insulin levels in both cystic fibrosis groups. Fasting intact proinsulin concentrations and the proportion of proinsulin-like molecules were significantly higher in cystic fibrosis with diabetes mellitus or impaired glucose tolerance than in the control group, but not in the normal glucose tolerance cystic fibrosis group. There was no significant difference in the plasma concentrations of 32-33 split proinsulin amongst the three groups. CONCLUSIONS: Abnormal beta cell function in cystic fibrosis patients was reflected initially in a diminished 30-minute insulin response to oral glucose. A significant rise in fasting intact proinsulin and the proportion of proinsulin-like molecules was seen only in cystic fibrosis patients who had progressed to impaired glucose tolerance or diabetes mellitus. Cystic fibrosis patients with normal glucose tolerance showed changes intermediate between the control and the other group.

Adolescent↗

PubMed1995

Pancreatic insufficiency and pulmonary disease in German and Slavic cystic fibrosis patients with the R347P mutation.

Cystic fibrosis (CF) is caused by mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) that codes for a cAMP-regulated chloride channel. The R347P is a missense mutation located within the first membrane spanning domain (MSD1) of the CFTR protein. This mutation occurs with an overall worldwide frequency of about 0.2%. The patients, originally described with this mutation were compound heterozygotes with the delta F508 mutation and had a very mild course of CF, suggesting that R347P, similar to other missense mutations affecting the MSD1 domain, causes a mild phenotype. We report here a group of 19 CF patients with the R347P mutation of German, Bulgarian, Czech, and Slovak origin, including two homozygotes. Most patients presented with early disease onset, pancreas insufficiency (PI), and early pulmonary involvement, suggesting that this mutation can lead to a severe course of CF. Most R347P alleles in the group studied share a common polymorphic haplotype. In addition, these analyses gave evidence for recurrence of the mutation in two CF patients of German and Czech origin.

Cystic Fibrosis↗

PubMed1998

Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator.

Hepatic dysfunction in cystic fibrosis (CF) has been attributed to accumulation of viscous mucoid secretions in intrahepatic bile ducts. The purpose of our study was to compare glycoconjugate secretion by intrahepatic biliary epithelial (IBE) cells derived from normal livers and livers of CF patients with the delta F508 mutation of the cystic fibrosis transmembrane conductance regulator (CFTR). Confluent cells were incubated with 3H-glucosamine (GlcN) for 16 hours, and radiolabeled macromolecules were analyzed for the amount and type of glycoconjugates. Incorporation of 3H-GlcN into macromolecular glycoconjugates was two- to threefold higher in CF cells versus normals, as was uptake of 3H-Glcn into the cytoplasm of CF cells. Gel exclusion chromatography on Sepharose Cl 4B revealed that the secreted glycoconjugates from CF cells eluted entirely in the excluded fraction (molecular weight > 2 x 10(6)), while, in the normal cells, 60% of the glycoconjugates eluted as lower-molecular-weight species. The high-molecular-weight glycoconjugates in both CF and normal cells were identified as chondroitin sulfates, as evidenced by susceptibility to beta elimination, chondroitinase digestion, and amino acid composition. Western blotting of IBE cell secretions with a polyclonal antibody to chondroitin sulfate revealed proteoglycan bands at 100 and 210 kd. Our results indicate that secretion of chondroitin sulfate is markedly increased in CF biliary epithelium in vitro compared with non-CF cells. Increased uptake of precursor 3H-GlcN may contribute to enhanced glycosylation of chondroitin sulfate in CF cells.

Amino Acids↗

PubMed1988

Longitudinal study of antibody response to lipopolysaccharides during chronic Pseudomonas aeruginosa lung infection in cystic fibrosis.

Antibodies to Pseudomonas aeruginosa from 10 cystic fibrosis patients with chronic P. aeruginosa lung infections were quantitatively and qualitatively analyzed. The development of specific antibodies in patient serum was evaluated in a longitudinal study (1972 to 1987). The concentrations and specificities of immunoglobulin G (IgG) and IgM antibodies to purified lipopolysaccharides (LPS) from clinical isolates of P. aeruginosa and to a variety of other gram-negative bacteria were studied by immunoblotting and enzyme-linked immunosorbent assay techniques. Results were compared with the number of immunoprecipitates to P. aeruginosa whole-cell extracts detected by crossed immunoelectrophoresis. IgG, but not IgM, anti-Pseudomonas LPS concentrations increased significantly at the onset of chronic infection and continued to increase during the course of the infection. There was a good positive correlation between the concentration of IgG anti-Pseudomonas LPS antibodies and the number of crossed-immunoelectrophoresis precipitins. The increases in IgG anti-LPS antibody concentrations were much higher to Pseudomonas LPS than to other LPSs. Binding studies demonstrated an increase in binding of IgG anti-Pseudomonas LPS during infection, whereas the binding of other anti-LPS antibodies decreased. Immunoblotting studies confirmed that antibodies reacted strongly with Pseudomonas LPS and weakly with Escherichia coli core-lipid A. The specificity of the reaction with Pseudomonas LPS increased with the duration of infection. It is concluded that anti-LPS response in cystic fibrosis patients during chronic P. aeruginosa infection demonstrates a marked increase in IgG anti-Pseudomonas LPS antibody concentration, specificity, and affinity. The anti-LPS enzyme-linked immunosorbent assay is proposed as a routine test to diagnose and to follow the course of chronic P. aeruginosa lung infection in patients with cystic fibrosis.

Adolescent↗

PubMed1997

Polypoidal rhinosinusitis in cystic fibrosis: a clinical and histopathological study.

The eosinophil may play a key role in the pathogenesis of nasal polyposis. Polyps in cystic fibrosis, however, have been described as neutrophilic. We compared the cell counts in polyps from 44 patients with cystic fibrosis to polyps from 50 patients without cystic fibrosis. The clinical profile, CT-scan and time to polyp recurrence were also compared with the cell counts in the patients with cystic fibrosis. No significant difference was detected in the number of patients with eosinophils (P > 0.25). Significantly more patients in the group with cystic fibrosis had polyp neutrophils (P < 0.01). Polyps from patients without cystic fibrosis contained more eosinophils (P < 0.001) whilst polyps from patients with cystic fibrosis contained more neutrophils (P = 0.001) and plasma cells (P = 0.038). Significant correlation was found between the neutrophil count and the CT score (P = 0.025) and between the recurrence time of polyps and the macrophage count (P = 0.01). Eosinophils are present in varying degrees in polyps from patients with and without cystic fibrosis and to classify polyps as eosinophilic or neutrophilic may be a false distinction.

Adolescent↗

PubMed1986

Sweat bromide excretion in cystic fibrosis.

Sweat reabsorption of bromide and chloride was studied in controls, obligate carriers of the cystic fibrosis gene, and individuals with cystic fibrosis. Sweat and serum ultrafiltrate concentrations of bromide and chloride were determined by an anion-exchange chromatographic method. The reabsorption of each ion was determined by the ratio of the sweat concentration to the serum ultrafiltrate concentration. Sweat bromide reabsorption paralleled chloride reabsorption in each of the groups. The mean bromide reabsorption ratio for 12 controls was 0.10 +/- 0.10 (range 0 to 0.26), for 11 obligate carriers 0.09 +/- 0.09 (range 0 to 0.24), and for 13 individuals with cystic fibrosis 0.86 +/- 0.26 (range 0.47 to 1.38). The mean chloride reabsorption ratio for the controls was 0.24 +/- 0.11 (range 0.06 to 0.45), for the obligate carriers 0.22 +/- 0.10 (range 0.10 to 0.45), and for individuals with cystic fibrosis 0.95 +/- 0.09 (range 0.76 to 1.1). Thus, there was a significant decrease in bromide reabsorption in individuals with cystic fibrosis compared with controls (P less than 0.001) and heterozygotes (P less than 0.001), which was similar for chloride. As with chloride reabsorption, no difference was found in bromide reabsorption between obligate carriers and controls, indicating that this measurement could not be used for heterozygote detection. In each of the three groups bromide reabsorption was greater than chloride reabsorption. This finding is consistent with a size-dependent anion channel in the sweat epithelial cell because bromide has a smaller hydrated radius than chloride.

Absorption↗

PubMed2000

[Genetic counseling in cystic fibrosis].

Genetic counseling is an important part of health care in patients with cystic fibrosis or respiratory diseases associated with the CFTR (cystic fibrosis transmembrane conductance regulator) gene, including certain types of allergic bronchopulmonary aspergilloses or bronchial diseases (diffuse bronchiectasia). The basic goal is to provide patients with information on the transmission of cystic fibrosis and to asses the risk of recurrence. This risk is determined from molecular biology analyses examining the CFTR gene. Genotyping is the only means of screening for the heterozygous state, frequent in the French population (about 1/30). Because of the large number of mutated alleles not covered entirely by the genetic tests, there remains a question of probability expressed as a residual risk of a heterozygous state. A prenatal genotype diagnosis should be proposed to heterozygous couples who have a 25% risk of having a diseased child. Technically, this is almost always possible and the results are highly reliable. Nevertheless, there remains the risks related to sample taking and the ethical issue about which the patients must be informed. Management of these at risk couples who desire a child must be based on a multidisciplinary approach, particularly important when one of the parents has overt cystic fibrosis.

Cystic Fibrosis↗