Clinical scoring systems in cystic fibrosis

Abstract

The first cystic fibrosis (CF) scoring system was published in 1958. Since then, many other scoring systems were developed. Clinical parameters, details about statistical evaluations, and recent strategic uses of scores were identified. Several similar scores aiming to assess chronic illness severity (Shwachman-Kulczycki score and a modification, Cooperman, Berneze-score and the NIH score) have not been evaluated and are out of date, given the changing natural history of CF. Of the current scoring systems, the modified Shwachman score by Doershuk is perhaps most reliable for describing follow-up studies. Scores designed for acute changes and short-term evaluation were also developed. The modified Huang score may be useful in the prognostic evaluation of patients with end-stage disease. It could also be used for discrimination of adult patients with differing disease severity and for longitudinal evaluation. Scores assessing pulmonary exacerbations could help provide consensus among clinicians regarding the need for intervention. Most of these scores require further evaluation. Although scores could provide an objective measure of disease severity, progression, need for and response to interventions, including value in selecting patients for lung transplantation and as an outcome measure for research studies, no scoring system can fulfill all these objectives. Nevertheless, there is a need for the development of a modern day longitudinal score that is sensitive, valid and reproducible, to reflect the milder disease status of patients.

Direct monitoring of pulmonary disease treatment biomarkers using plasmonic gold nanorods with diffusion-sensitive OCT

“…Therefore, accurate temporal/spatial measurements of wt% are necessary to measure treatment efficacy. Current outcome measures rely on days-long observation of patients after treatment 40 , with only recent developments in more rapid measures, on the order of hours 39 . Here, we presented a new quantitative method for sensing mucus wt% that provides real-time measures of respiratory treatment efficacy.…”

Evaluation of micronutrient profile of North Indian children with cystic fibrosis: a case–control study

“…These included both stable CF cases (n = 21) and those with pulmonary exacerbation (n = 6) with Brasfield score ≥7 or Shwachman-Kulczycki score ≤70 (31,32). Age-and sex-matched healthy children attending Pediatrics outpatient department for minor ailments and vaccination were enrolled as controls (n = 27).…”

Lungentransplantation bei Mukoviszidose – ein Positionspapier

“…Die in der Literatur verbreiteten klinischen CF-Scores sind für die Entscheidung über den richtigen Transplantationszeitpunkt ungeeignet [17]. In früheren Jahren wurden vor allem CF-Patienten mit einer FEV1 unter 30 % des Sollwertes für eine Lungentransplantation evaluiert [18].…”