A therapeutic regimen for patients with cystic fibrosis

Matthews, Larry S.; Doershuk, Carl F.; Wise, Melvin; Eddy, George; Nudelman, Hermann; Spector, Samuel

doi:10.1016/s0022-3476(64)80290-0

Cited by 97 publications

(42 citation statements)

References 45 publications

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“…The initial TGV and conductance were within normal limits and remained so at follow-up 3 years later while continuing on a comprehensive preventive treatment program [15].…”

Section: Discussionmentioning

confidence: 84%

A Method for Ventilatory Measurements in Subjects 1 Month-5 Years of Age: Normal Results and Observations in Disease

et al. 1970

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ExtractUsing the total body plethysmograph, a method for the measurement of lung volume and airway resistance was developed for infants and young children of 1 month-5 years of age. The subjects were studied in a supine position using a reproducible method of sedation. The procedure providing the most consistent results involved use of a Rendell-Baker mask directly controlled by the operator while the subjects were breathing through the nose. Availability of an oscilloscope permitted immediate detection of pressure leaks, artifacts, and abnormal patterns during each determination and minimized potential errors during the study of each subject. Care was required to avoid pressure on the tip of the nose because an abnormally high airway resistance resulted.The logarithmic relation of thoracic gas volume (TGV) at functional residual capacity (FRC) to body length for 52 normal subjects compared well with results obtained on newborn infants and with those obtained on older subjects. The equation was: TGV at FRC, liters = 1.57 x 10-5 x length, cm 2.238 (correlation coefficient = 0.948). The logarithmic relation of airway conductance to TGV was: conductance, liters/sec/cm H,O = 0.1431 TGV, liters 0 . 6441 (correlation coefficient = 0.835). Comparison with older subjects was limited by the difference in airflow rates at which the airway conductance was determined (0.06-0.28 literslsec versus about 0.5 literslsec for adults) and by the use of sedation and nasal breathing in this study; adults are studied awake and during mouth breathing. Comparison of our conductance values with those obtained on unsedated newborns suggests that either sedation alters the airway resistance or that some change occurs in the airway size-lung volume relation during the first weeks of life.Examples of the application of this method to patients with asthmatic bronchitis and cystic fibrosis of the pancreas are provided. SpeculationIt is possible that an airflow-lung volume-airway resistance relation exists throughout life; however, the results presented in this study, combined with those previously presented on newborns for airway resistance, suggest that a change may occur in the relation of the size of the airways to lung volume during early infancy, or that sedation alters airway resistance. Development of this and other methodology should permit more adequate studies of the early changes in pulmonary physiology associated with growth and the acute and chronic pulmonary conditions seen during the first 5 years of life. DOERSHUK, DOWNS, MATTHEWS and LOUGH

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“…The initial TGV and conductance were within normal limits and remained so at follow-up 3 years later while continuing on a comprehensive preventive treatment program [15].…”

Section: Discussionmentioning

confidence: 84%

A Method for Ventilatory Measurements in Subjects 1 Month-5 Years of Age: Normal Results and Observations in Disease

et al. 1970

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“…The pillars of care still include optimizing nutritional status, airway clearance, and aggressive management of airway infections provided by a multidisciplinary team (26). Close monitoring of nutritional, pulmonary, and general health status linked with longitudinal data registries (27) captures key outcome measures such as survival.…”

Section: Clinical Descriptionmentioning

confidence: 99%

“…Advances in step 6 were catalyzed by the development of transgenic animal models (mouse, rat, ferret, pig, rabbit, and zebrafish [49][50][51][52][53][54]), allowing investigators to interrogate the earliest manifestations of the disease. Finally, treatment to improve the lives of patients with cystic fibrosis (step 7), began in the 1950s with availability of a diagnostic sweat test (3) and establishment of care centers (26). Until 2010, all therapies treated the consequences of organ dysfunction and failure.…”

Section: Clinical Descriptionmentioning

confidence: 99%

AJRCCM: 100-Year Anniversary.Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis

Ramsey

Welsh²

2017

Am J Respir Crit Care Med

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“…Patients referred after 1964 who survived at least 1 but less than 5 years were also included. With the exception of newer anti-pseudomonas antibiotics, there is no difference in the treatment of cystic fibrosis today from that employed in the 1958-1964 period (9,14). Informed consent was obtained.…”

Section: Study Samplementioning

confidence: 99%

Cystic Fibrosis: The Prognosis for Five-year Survival

et al. 1978

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SummaryStatistical discriminant analysis is applied to 41 concomitant variables obtained during the first year of study of 226 patients with cystic fibrosis. A discriminant function based on six variables is developed which can be used as a predictive index. This index estimates the probability of 5-year survival for an individual patient and can also be used to classify patients into one of two groups: (1) will live for 5 years or (2) will die within 5 years. SpeculationA classification rule for cystic fibrosis is presented which correctly assigns 90% of the 5-year survivors and 84% of those who expire. Such a classification scheme is useful for clinical and research purposes.

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A therapeutic regimen for patients with cystic fibrosis

Cited by 97 publications

References 45 publications

A Method for Ventilatory Measurements in Subjects 1 Month-5 Years of Age: Normal Results and Observations in Disease

A Method for Ventilatory Measurements in Subjects 1 Month-5 Years of Age: Normal Results and Observations in Disease

AJRCCM: 100-Year Anniversary.Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis

Cystic Fibrosis: The Prognosis for Five-year Survival

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