Median Survival Age Research Articles

Survival analysis for cystic fibrosis in Minas Gerais State, Brazil.

A survival analysis was carried out on cystic fibrosis (CF) patients diagnosed and followed at the General Hospital, Federal University of Minas Gerais, Brazil. The study cohort consisted of 111 children, admitted to the Pediatric Pulmonology Section from 1 June 1970 to 31 December 1994. The survival curve and table, constructed by the Kaplan-Meier (product limit) method, show a mean survival age of 12.6 years. This life expectancy resembles the median survival age observed in developed countries by 1970. This study shows that problems relating to health system organization and unfavourable social and economic conditions in developing countries may directly affect the ultimate survival of CF patients.

Age-associated changes in the monoamine synthesis system in the brain of senescence-accelerated mouse-prone(SAM-P/8)-(II)

Senescence accelerated prone mouse strains (SAM-P) and resistant strains (SAM-R) have proven useful in elucidating aspects of the aging process. The senescence accelerated mouse SAM-P/8 strain exhibits severe age-related learning and memory impairments well before the median age of survival. Disruption of the brain cholinergic system produces learning and memory impairments as severe as those seen in aging SAM-P/8 mice. Therefore, we compared the effects of aging on cholinergic parameters in the septal–hippocampal pathway, a region known to play a role in learning and memory, in SAM-P/8 mice and mice of the senescence resistant SAM-R/1 strain. Between 4 and 12 months of age we observed a 40–50% decrease in choline acetyltransferase (ChAT) activity in two of three subregions of the hippocampus in the SAM-P/8, but not the SAM-R/1 strain. Between 4 and 12 months, SAM-P/8 mice also showed a 40–50% decrease in ChAT activity in the septal region that was maximal by 8 months of age. By contrast, these age-related changes were not observed in the control SAM-R/1 mouse strain. The changes in ChAT in the SAMP/8 mouse strain were limited to the septal–hippocampal cholinergic pathway. There were no differences in ChAT activity in the nucleus basalis of Meynert, nor any of several neocortical areas to which it projects. To determine the neurochemical specificity of these alterations, the activity of glutamic acid decarboxylase (GAD), was also measured in the septum and hippocampus of SAM-P/8 mice. There were no age-related alterations in the hippocampus, but a significant 50% increase in GAD activity in the septal nucleus at 12 months of age. There were no age-related alterations in either nicotinic (3H-cytisine) or muscarinic (3H-QNB) cholinergic receptor binding in the cortex or hippocampus of SAM-P/8 mice. However, there were significant strain differences. At 2 months of age, 3H-QNB binding was higher in hippocampus of the SAM-R/1 than in SAM-P/8 mice. Similarly, 3H-cytisine binding in cortex of SAM-R/1 mice was higher at both 2 and 13 months than in SAM-P/8 mice. The results suggest that a compromised septal–hippocampal cholinergic pathway may contribute to the previously reported early onset of impaired learning and memory in the SAM-P/8 mouse strain.

Impact and future of neonatal screening for cystic fibrosis

Cystic fibrosis (CF) is the commonest life-threatening inherited condition in the UK, affecting more than 6000 people at the present time. Advances in all aspects of clinical management, in particular early treatment of pulmonary infections and attention to nutrition, have contributed to a significant improvement in prognosis, with a current median age of survival of 30 years. There is increasing evidence that early intervention after establishing the diagnosis in asymptomatic patients has numerous medical, psychological and financial benefits. Neonatal screening programmes have been available in some populations since 1980 and in view of the benefits discussed below should play an integral part in the management of CE

Relationship between dyspnoea, pulmonry function and exercise capacity in patients with cystic fibrosis

The median age of survival in patients with cystic fibrosis (CF) has improved considerably. Despite this improvement, deterioration of pulmonary function and decrease in exercise capacity are still the main problems for many patients. Although dyspnoea is a common complaint in CF patients, relatively little regard has been paid to this symptom. This study examined the relationship between dyspnoea, bicycle exercise capacity and pulmonary function in patients with CF. In 14 patients in a stable clinical condition, pulmonary function [forced expiratory volume in 1 s (FEV 1), inspiratory vital capacity (IVC)], bicycle exercise capacity [maximum exercise capacity ( Wmax)], subjective degree of dyspnoea during daily living [Medical Research Council (MRC) dyspnoea scale], and during exercise (Borg scale) were assessed. The mean ( sd) age of the patients was 25 (6·8) years, FEV 1 was 41 (19)% predicted, IVC was 63 (17)% predicted and FEV 1 IVC ratio was 47 (10)%; median (range) Wmax was 55 (0–79)% predicted. Bicycle exercise test performance appeared to be mainly determined by pulmonary function and MRC dyspnoea grade; multiple regression equation containing FEV 1 and dyspnoea accounted for 76% of the variance in Wmax (% predicted) ( Wmax= −7·9 dysp+1·1FEV 1, +24). Exercise dyspnoea, assessed by the Borg scale, showed a significant linear correlation with minute ventilation. (V̇ e), maximal voluntary ventilation (MVV) (%) ( r=0·76; P<0·001). Medical Research Council dyspnoea score correlated relatively poorly with FEV 1 (% predicted) ( r= −0·17; n.s.) and IVC (% predicted) ( r= −0·48; n.s.). Borg score at maximal exercise did not correlate with MRC dyspnoea score ( r= −0·07). Borg 50% score correlated significantly with MRC dyspnoea score ( r= 0·61; P<0·05). These results show that dyspnoea has an influence on exercise capacity. Dyspnoea score showed a large inter-individual variation, not strongly related to pulmonary function. It is concluded that dyspnoea deserves more attention in CF patients and needs to be assessed in rehabilitation programmes and other intervention studies in these patients.

Neuropeptides and neurotrophin receptor mRNAs in primary sensory neurons of aged rats.

Neuropeptides and neurotrophin receptors are regulated in primary sensory neurons in response to axonal injury, and axonal lesions are characteristic stigmata of aging primary sensory neurons. We have therefore examined the expression of neuropeptides and neurotrophin receptor mRNAs in 30-month-old (median survival age) Sprague-Dawley rats to see if similar adaptive mechanisms operate in senescence. The content of neuropeptides was examined with immunohistochemistry (IHC) and in situ hybridization (ISH), and the cellular mRNA expression of neurotrophin receptors was studied with ISH. All of the aged rats had symptoms of hind limb incapacity (posterior paralysis), but fore limbs did not seem affected. The size-distribution of neuronal profiles in cervical and lumbar dorsal root ganglia (DRGs) was similar in aged and young adult (2-3 months old) rats. In aged rats, the DRG neurons showed an increase in both immunolabelling and mRNA content of neuropeptide tyrosine (NPY), as well as an increased cellular expression of galanin mRNA. In the same animals, there were decreased cellular levels of calcitonin gene-related peptide (CGRP; IHC and ISH) and substance P (SP; IHC and ISH), while the difference in neuronal somatostatin (IHC and ISH) was small. The distribution of neuropeptide immunoreactivities in the dorsal horn of the corresponding spinal cord segments revealed a decreased labelling for CGRP-, SP-, and somatostatin-like immunoreactivities (LI) in the aged rats at both cervical and lumbar levels. NPY- and galanin-LI had a similar distribution in aged and young adult rats. NPY-immunoreactive fibers were also encountered in the dorsal column of aged but not young adult rats. ISH revealed that most of the primary sensory neurons express mRNA for the p75 low-affinity neurotrophin receptor (p75-LANR) and that there was no discernible difference between young adult and aged rats. The labelling intensity for mRNA encoding high-affinity tyrosine kinase receptors (TrkA, TrkB, and TrkC) was decreased in aged rat DRG neurons, while the percentage of neuronal profiles expressing mRNA for TrkA/B/C was similar in young adult and aged rats. The changed pattern of neuropeptide expression in primary sensory neurons of aged rats resembled that seen in young adult rats subjected to axonal injury of peripheral sensory nerves and may, thus, indicate aging-related lesions of sensory fibers. Since NPY is primarily present in large and galanin in small DRG neurons, the stronger effect on NPY as compared to galanin expression may indicate that aging preferentially affects neurons associated with mechanoreception (A alpha and A beta fibers) as compared to nociceptive units (A delta and C fibers). Furthermore, the observed changes in neuropeptide expression were most pronounced in lumbar DRGs, that harbors the sensory neurons supplying the affected hindlimbs of the rats.

Neuropeptides and neurotrophin receptor mRNAs primary sensory neurons of aged rats

Neuropeptides and neurotrophin receptors are regulated in primary sensory neurons in response to axonal injury, and axonal lesions are characteristic stigmata of aging primary sensory neurons. We have therefore examined the expression of neuropeptides and neurotrophin receptor mRNAs in 30-month-old (median survival age) Sprague-Dawley rats to see if similar adaptive mechanisms operate in senescence. The content of neuropeptides was examined with immunohistochemistry (IHC) and in situ hybridization (ISH), and the cellular mRNA expression of neurotrophin receptors was studied with ISH. All of the aged rats had symptoms of hind limb incapacity (posterior paralysis), but fore limbs did not seem affected. The size-distribution of neuronal profiles in cervical and lumbar dorsal root ganglia (DRGs) was similar in aged and young adult (2–3 months old) rats. In aged rats, the DRG neurons showed an increase in both immunolabelling and mRNA content of neuropeptide tyrosine (NPY), as well as an increased cellular expression of galanin mRNA. In the same animals, there were decreased cellular levels of calcitonin gene-related peptide (CGRP; IHC and ISH) and substance P (SP; IHC and ISH), while the difference in neuronal somatostatin (IHC and ISH) was small. The distribution of neuropeptide immunoreactivities in the dorsal horn of the corresponding spinal cord segments revealed a decreased labelling for CGRP-, SP-, and somatostatin-like immunoreactivities (LI) in the aged rats at both cervical and lumbar levels. NPY- and galanin-LI had a similar distribution in aged and young adult rats. NPY-immunoreactive fibers were also encountered in the dorsal column of aged but not young adult rats. ISH revealed that most of the primary sensory neurons express mRNA for the p75 low-affinity neurotrophin receptor (p75-LANR) and that there was no discernible difference between young adult and aged rats. The labelling intensity for mRNA encoding high-affinity tyrosine kinase receptors (TrkA, TrkB, and TrkC) was decreased in aged rat DRG neurons, while the percentage of neuronal profiles expressing mRNA for TrkA/B/C was similar in young adult and aged rats. The changed pattern of neuropeptide expression in primary sensory neurons of aged rats resembled that seen in young adult rats subjected to axonal injury of peripheral sensory nerves and may, thus, indicate aging-related lesions of sensory fibers. Since NPY is primarily present in large and galanin in small DRG neurons, the stronger effect on NPY as compared to galanin expression may indicate that aging preferentially affects neurons associated with mechanoreception (Aα and Aβ fibers) as compared to nociceptive units (A and C fibers). Furthermore, the observed changes in neuropeptide expression were most pronounced in lumbar DRGs, that harbors the sensory neurons supplying the affected hindlimbs of the rats. © 1996 Wiley-Liss, Inc.

Long-term survival and nutritional data in patients with cystic fibrosis treated in a Danish centre.

Adequate nutrition and optimal treatment of bronchopulmonary infections are both of critical importance in maintaining the health of patients with cystic fibrosis. The cystic fibrosis centre in Copenhagen has followed a regimen of very early and aggressive antimicrobial treatment, especially against Pseudomonas aeruginosa infection. An unrestricted diet of low fat and high protein without hyperalimentation was recommended before 1985 which was then changed to a high fat, high calorie intake. The overall impact of the treatment regimen was evaluated by a cross sectional analysis of all 223 patients who attended the centre in 1989. Growth and nutritional parameters were combined with lung function parameters and with a retrospective analysis of chronic P aeruginosa infection and its duration. Survival curves for all 313 patients treated at the centre since 1949 were calculated. All the patients with cystic fibrosis had normal height, although the final height was achieved a little later than in healthy controls. Body weight was lower than normal in males above 15 and in females above 10 years of age. The body mass index (BMI), which was approximately 98% of normal in the younger patients, declined to 90% in adult men and to 83% in adult women with cystic fibrosis, and was strongly correlated with lung function parameters. In 1989 the median age of survival of all patients treated in the centre since 1949 was 30 years (32 years in males and 29 years in females). The overall treatment regimen in the cystic fibrosis centre in Copenhagen is associated with growth and survival rates that are at least equal to those in other cystic fibrosis centres in other countries.

Transferring to adult health care: Experiences of adolescents with cystic fibrosis

YSTIC FIBROSIS (CF) is a genetic disorder resulting in the dysfunction of exocrine glands; it is mainly restricted to, and is the most common autosomal genetic disorder in the Caucasian race (Harris & Super, 1991; Jackson, 1989). The incidence of CF is 1:2,500 (Harris & Super, 1991) to 1:3,500 (Fitzsimmons, 1993) live births in 1990. The severity of symptoms results in a range of differing problems for the child and family. Almost all children with CF have respiratory problems, often culminating in secondary pulmonary infection that requires hospitalization for treatment of the infection. Gastrointestinal problems are also experienced by many of these children. The focus of management is strategies to improve and maintain pulmonary function and to prevent secondary infection. Data from the United States cite the median survival age as 10 years in the 1960s, and 28 years in 1990 (Fitzsimmons, 1993). The increase in life expectancy for individuals with CF is a result of advances in treatment and management of the disease (Fitzsimmons, 1993). This study investigated the experience of transferring to adult health care from the perspective of adolescents with CF and their parents. The adult hospital is seen as the most appropriate place to provide care for adolescents or young adults with CF because adolescent independence and self-sufficiency can be

Determinants of mortality from cystic fibrosis in Canada, 1970-1989.

The frequency, prevalence, and mortality patterns of cystic fibrosis were analyzed in 3,795 patients documented in the Canadian Patient Data Registry in 1970-1989. Cystic fibrosis frequency in the 1970-1979 birth cohort was virtually identical to the commonly quoted 1 in 2,500. In 1985-1989, median survival age was 36.7 years for males and 27.8 years for females, compared with 26.6 and 19.7, respectively, in 1970-1974. However, there were significant regional differences when Canada was divided into the four regions, East, Quebec, Ontario, and West. In Quebec, patients were younger at diagnosis and until recently had greater mortality than patients in other regions, which suggests more severe disease; dramatically improved survival in the 1980s coincided with a change from a restricted fat diet to a high fat diet. Improved survival in Ontario in the 1970s accompanied this change in dietary therapy, which may also account for good survival throughout the study period in the East. The West showed gradually improving survival, similar to that reported in other parts of the world. Proportional hazards analysis showed pulmonary function to be the best predictor of survival. Poorer survival in females was associated with poorer weight, but the interrelation of declining pulmonary function, weight maintenance, sex, and mortality requires further study. The effect of pulmonary colonization with Pseudomonas aeruginosa was confounded with degree of pulmonary dysfunction, but colonization with Burkholderia cepacia (previously Pseudomonas cepacia) was associated with increased mortality at all levels of pulmonary function.

Prognosis in cystic fibrosis.

Prognosis for patients with cystic fibrosis has improved dramatically over the past three decades. In the United States, median survival age is now 28.9 years. Although genotype predicts exocrine pancreatic function, it does not correlate with pulmonary status or overall clinical outcome. However, there are a number of parameters, such as exocrine pancreatic sufficiency, male gender, absence of colonization with mucoid Pseudomonas aeruginosa, presentation with predominantly gastrointestinal symptoms, balanced family functioning and coping, and compliance with treatment regimens, that predict a more favorable outcome. The impact of early diagnosis and treatment is still controversial. Although nonblinded studies indicate decreased morbidity in the first 2 to 4 years of life among patients diagnosed by newborn screening, no data support long-term benefit in terms of pulmonary function or survival. With increased longevity, there is now evidence of a small but significantly increased risk of gastrointestinal tract cancer among patients with cystic fibrosis.

Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection

The median survival age for females with cystic fibrosis (CF) is approximately 3 years younger than for males. We tested whether earlier acquisition of Pseudomonas aeruginosa (PA) by female CF patients or the greater impact of this organism on their lung disease, or both, contribute to their poorer survival. PA infection status, survival, pulmonary function tests, and chest X-ray scores from patients who were followed at our center for at least 2 years with a minimum of three respiratory cultures per year were analyzed ( n = 848). The median age of chronic infection with mucoid PA was 1.7 years earlier in females than in males. Patients infected with mucoid PA had poorer survival, chest X-ray scores, and pulmonary function tests than patients who had either no Pseudomonas species or only the nonmucoid phenotype. Acquisition of mucoid PA was associated with an accelerated rate of decline in pulmonary function. However, the rate of change of pulmonary function after mucoid PA infection was similar for males and females. Moreover, even among patients who had only the mucoid form or only the nonmucoid form, males had better percent predicted forced expiratory volume in 1 sec and better survival. Therefore, factors in addition to earlier acquisition of mucoid PA may contribute to the poorer survival of female CF patients.

Introduction

Cystic fibrosis (CF) is a recessive genetic disease affecting 1 in 2,500 live white births. The major morbidity and mortality in patients with CF results from chronic bacterial infection of the airways with Staphylococcus aureus and Pseudomonas aeruginosa, usually of the mucoid variety. Currently, the median age of survival is 30 years, but there have been tremendous recent advances in our understanding of the pathogenesis of cystic fibrosis lung disease at both an organ level and a cellular/molecular level. We are witnessing renewed efforts to develop and implement novel therapies derived from new insights and advances in technology with the goal being to improve the quality of life and longevity in this challenging disease. The purpose of this symposium is to address some of those recent advances.

The changing epidemiology of cystic fibrosis

Data from 17,857 patients with cystic fibrosis submitted in 1990 to the registry maintained by the Cystic Fibrosis Foundation were used to describe their demographic characteristics, survival rates, pulmonary function, anthropometry, microbiologic data, complication rates, and health care utilization. Comparisons with similar data collected in 1969, 1972, and 1978 demonstrated a significant shift in the age distribution of patients with cystic fibrosis. The proportion of adult patients increased fourfold between 1969 (8%) and 1990 (33%). In 1990 the median age of all patients in the cystic fibrosis registry was 12.5 years; the median age at diagnosis was 7 months; cystic fibrosis was diagnosed in 90% of all patients by age 12 years. Meconium ileus at birth was reported for 16% of all patients with a new diagnosis in 1990. Median survival age doubled between 1969 and 1990, from 14 to 28 years. Female patients consistently had a lower median survival age than male patients (25 vs 30 years in 1990). The most frequently reported respiratory pathogen was Pseudomonas aeruginosa , cultured in specimens from 61% of all patients, ranging from 21% of those less than 1 year of age to more than 80% of those aged 26 years or older. Overall, patients with cystic fibrosis are living much longer than in the past but still have chronic pulmonary infections and other medical complications related to their disease, including diabetes, intestinal obstruction, cirrhosis, hemoptysis, and pneumothorax.

Screening for cystic fibrosis: medical and ethical issues

Cystic fibrosis (CF) is one of the most common severe, recessively inherited diseases in the Caucasian population. With the identification of the gene and the most common mutations responsible for the condition”‘, carrier screening has become a real option to be considered by the families of sufferers, the public at large, health planners and policy makers. In the UK, approximately 1 in 25 individuals are carriers and therefore, there is a 1 in 625 chance of two carriers marrying. If both parents are carriers, there is a 1 in 4 risk of each child inheriting both CF alleles and being affected. The birth incidence of CF is around 1 in 25003. Affected individuals have a variety of clinical problems which are dominated by recurrent respiratory tract infections (often with Pseudomonas species) associated with viscid, inspissated mucus, failure to thrive secondary to a lack of pancreatic secretions reaching the gut (85%) and, occasionally, meconium ileus presenting in the newborn period (5510%). Infertility is found in most adult males with CF and is frequent in females. The prognosis for CF has improved in recent years with a current median age of survival of 25 years. It has been estimated that the median survival of CF children born in 1990 may even be as high as 40 years if current trends continue4. The management of CF includes physiotherapy (chest percussion) to aid clearance of infected secretions, antibiotic therapy to treat infection, pancreatic enzyme replacement with meals and snacks and nutritional supplementation. The cloning and identification of the gene and protein product defective in CF has allowed studies of new approaches to therapy, including the possibility of transferring a normal gene into airway epithelial

The changing epidemiology of cystic fibrosis

Data from 17,857 patients with cystic fibrosis submitted in 1990 to the registry maintained by the Cystic Fibrosis Foundation were used to described their demographic characteristics, survival rates, pulmonary function, anthropometry, microbiologic data, complication rates, and health care utilization. Comparisons with similar data collected in 1969, 1972, and 1978 demonstrated a significant shift in the age distribution of patients with cystic fibrosis. The proportion of adult patients increased fourfold between 1969 (8%) and 1990 (33%). In 1990 the median age of all patients in the cystic fibrosis registry was 12.5 years; the median age at diagnosis was 7 months; cystic fibrosis was diagnosed in 90% of all patients by age 12 years. Meconium ileus at birth was reported for 16% of all patients with a new diagnosis in 1990. Median survival age doubled between 1969 and 1990, from 14 to 28 years. Female patients consistently had a lower median survival age than male patients (25 vs 30 years in 1990). The most frequently reported respiratory pathogen was Pseudomonas aeruginosa, cultured in specimens from 61% of all patients, ranging from 21% of those less than 1 year of age to more than 80% of those aged 26 years or older. Overall, patients with cystic fibrosis are living much longer than in the past but still have chronic pulmonary infections and other medical complications related to their disease, including diabetes, intestinal obstruction, cirrhosis, hemoptysis, and pneumothorax.

Cystic Fibrosis from Bench to Bedside

The highest goal of biomedical bench research is to translate test-tube discoveries into real-world benefits for patients. The extraordinary advances in research on cystic fibrosis in the past 10 years have provided a wealth of opportunities for therapeutic intervention and will surely add new strategies to the current armamentarium. Conventional treatment for cystic fibrosis, devised 30 years ago by intrepid clinicians without knowledge of the basic defect, has been remarkably successful. Therapeutic programs aimed at clearing viscid secretions from the lung, correcting nutritional deficits, and controlling pulmonary infections have increased the median survival age for patients with cystic fibrosis to . . .

Prolonged survival in patients with beta-thalassemia major treated with deferoxamine

To determine whether survival of patients with beta-thalassemia major has been prolonged by management that utilizes hypertransfusion and chelation with deferoxamine, we analyzed longevity by the Kaplan-Meier product-limit method. Group 1 patients (n = 71) followed between 1960 and 1976 with a low-transfusion regimen (pretransfusion hemoglobin level 7 to 8 gm/dl) and no chelation had an estimated median age of survival of 17.4 years, whereas it was 31.0 years for group 2 subjects (n = 80), who began hypertransfusion between 1976 and 1978 (pretransfusion hemoglobin level 10.5 to 11.5 gm/dl) and chelation with deferoxamine (20 to 60 mg/kg per day) (p less than 0.0001). For 70 patients who were treated with hypertransfusion and deferoxamine, we had data to calculate the ratio of total milligrams of transfusional iron to cumulative grams of deferoxamine. The 24 patients who died had a total iron burden of greater than 1.05 gm/kg; the ratio for them exceeded 31. These patients were characterized by poor compliance with chelation or by late start of therapy, with inability to receive enough deferoxamine before death. Death was preceded by arrhythmia requiring therapy in all but one, and by cardiac failure in all. Of 41 similarly iron-loaded survivors, 33 had a ratio of less than 31; only three had an arrhythmia, and five had cardiac failure. We conclude that treatment with deferoxamine, when used in amounts proportional to iron burden, delayed cardiac complications and improved longevity.

Gastrointestinal dysfunction and its effects on nutrition in CF.

Prevention of malnutrition, a consequence of elevated energy requirements, increased losses and low caloric intake, is one of the main goals in the treatment of cystic fibrosis. Caloric stool losses, catch-up growth and an elevated energy expenditure, even in the absence of overt lung disease and malabsorption, have led to recommendations for a caloric intake of 120-150% of the recommended daily allowances. A high energy intake with a fat content of at least 40 calorie % and adequate pancreatic supplementation has shown to improve growth and median age of survival. As a rational treatment of the fundamental disturbance in cystic fibrosis, a decrease in chloride permeability across epithelia, is not yet available, treatment should be concentrated on adequate nutritional support in combination with optimal correction of those gastrointestinal abnormalities including faecal bile acid loss, small intestinal abnormalities, pancreatic insufficiency, hormonal abnormalities and disturbances in gastrointestinal motility, which may aggravate maldigestion and malabsorption.

A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto

Two cystic fibrosis (CF) clinic populations of similar size and age distribution were compared with respect to growth, pulmonary function, and survival. Height and weight measurements were made on 499 patients in Boston (mean ± SD age 15.9 ± 9.6, range 1 month to 45 years) and on 534 patients in Toronto (mean ± SD age 15.2 ± 8.3, range 1 month to 43 years). Males constituted 57% in the Boston group, 58% in Toronto. Pulmonary function test results were recorded for 64% of the Boston patients and 77% of the Toronto patients. Survival curves for the period 1972–1981 generated by the CF Patient Registry were compared. Patients in Boston tended to be shorter than patients in Toronto. This pattern was seen in both sexes in the 10–20 year age groups. Toronto males also weighed more than Boston males. Mean forced expiratory volume in one second (FEV,) was not different in Boston and Toronto CF patients whether expressed as a percent of predicted or in litres by age groups. Median age of survival in Boston was 21 years, in Toronto 30, the two curves showing a marked separation from age 10. Although progressive pulmonary disease is the major cause of mortality in cystic fibrosis, the differences in growth and survival in these two patient groups, with very similar age-specific pulmonary function, suggest further examination of nutritional guidance and intervention in CF, especially regarding the traditional restriction of dietary fat.

Sociomedical conditions of swedish patients with cystic fibrosis: a review of the past twelve years.

Cystic fibrosis--a hereditary disease with chronic pulmonary infections and malnutrition--demands on intensive, troublesome, costly and time-consuming therapy. Only a few years ago nearly all CF-patients died in childhood, but today most of them are expected to reach adulthood. All Swedish CF-patients are registered centrally. This registry has been used for demographic studies, and all the CF-families in the registry have got enquiries about sociomedical conditions. In the years 1968-1977 the number of CF-patients has increased from 116 to 200 and median survival age from 7.5 to 16.0 years. The incidence is estimated to 1:2,200 - 1:4,500. The medical care has been intensified and follows the guidelines of the "Swedish CF-board". The clinical condition of the patients has improved in the period. The most striking social aspects are the long time spent on treatment (mean: 18, maximum: greater than 50 hours/week) and the little help that is given by physiotherapists or community paid helps. The parents have to do all the work themselves! Even if the families receive several specific benefits the economical burden is heavy and strikes very unevenly. School results of CF-children are good. The main wishes from CF-parents are: intensive research to find the basic defect, uniform support to CF-families throughout the country, much information to be given especially to physicians.