Implementation of a Quality Improvement Program to Improve Sweat Test Performance in a Pediatric Hospital

2014 ◽  
Vol 138 (7) ◽  
pp. 920-922 ◽  
Author(s):  
Barina Aqil ◽  
Aaron West ◽  
Michael Dowlin ◽  
Estella Tam ◽  
Cristy Nordstrom ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Quality Improvement ◽  
Test Performance ◽  
Sweat Test ◽  
Pediatric Hospital ◽  
Improvement Program ◽  
Sweat Chloride ◽  
Sweat Production ◽  
Sweat Testing ◽  
Set Up

Context.—All positive screening of newborns for cystic fibrosis using the dried blood spot 2-tiered immunoreactive trypsinogen/DNA method requires subsequent sweat chloride testing for confirmation. Obtaining an adequate volume of sweat to measure chloride is a challenge for many cystic fibrosis centers across the nation. The standard for patients older than 3 months is less than 5% quantity not sufficient (QNS) and for patients 3 months or younger is less than 10% QNS. Objective.—To set up a quality improvement (QI) program for sweat testing to improve QNS rates using the Wescor Macroduct (Wescor, Inc, Logan, Utah) method at Texas Children's Hospital's laboratory, Houston, Texas. Design.—Single-center study. Results.—Quantity not sufficient rates were evaluated for 4 months before and 8 months after implementation of the QI program for patients aged 3 months or younger and those older than 3 months. The QI program included changes in technician training, service, site of collection, mode of collection, weekly review, and forms to screen patients for medications that may alter sweat production. A marked improvement was observed in the rates of QNS, which declined considerably from 16.7% to 8.5% (≤3 months old) and from 9.3% to 2.2% (>3 months old) after implementation of the QI initiative in both age categories. Conclusion.—This report demonstrates the effectiveness of the QI program in significantly improving QNS rates in sweat chloride testing in a pediatric hospital.

Chronic Sinusitis and a Negative Sweat Test in a Patient with Cystic Fibrosis

1995 ◽  
Vol 9 (4) ◽  
pp. 225-228 ◽  
Author(s):  
Todd T Kingdom ◽  
Kelvin C. Lee ◽  
Gerd J. Cropp
Keyword(s):  
Cystic Fibrosis ◽  
Unusual Case ◽  
Dna Analysis ◽  
Chronic Sinusitis ◽  
Carrier State ◽  
Sweat Test ◽  
Genetic Mutations ◽  
Dna Mutation ◽  
Sweat Chloride ◽  
Sweat Testing

The diagnosis of cystic fibrosis (CF) is based on sweat chloride and DNA mutation testing. A subset of CF patients may have normal sweat chloride levels, thus requiring DNA analysis for confirmation of the diagnosis. These patients may escape diagnosis if sweat testing is the only modality used for screening. Recently, the putative structural gene for CF was localized to chromosome 7. The delta-F508 mutation accounts for approximately 70% of the CF chromosomes identified in North American Caucasians. Over 400 identified mutations constitute the remainder. It is now possible to screen patients for the presence of many of these genetic mutations, thus establishing the diagnosis of CF or defining a carrier state. We report an unusual case of a 17-year-old male with chronic sinusitis, mild pulmonary disease, and pancreatic sufficiency with nondiagnostic sweat chloride levels diagnosed to have CF after DNA analysis. This technique may thus serve as an important tool that pediatricians and otolaryngologists can use to diagnose children suspected of having CF.

scholarly journals Results of a Quality Improvement Program for Sweat Testing to Diagnose Cystic Fibrosis

2012 ◽  
Vol 43 (4) ◽  
pp. 12-14 ◽  
Author(s):  
Steven R. Boas ◽  
Joseph Hageman ◽  
Jason Washburn ◽  
Susan Piasecki ◽  
Marissa Liveris
Keyword(s):  
Cystic Fibrosis ◽  
Quality Improvement ◽  
Quality Improvement Program ◽  
Improvement Program ◽  
Sweat Testing

Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

2014 ◽  
Author(s):  
Michael J Stephen
Keyword(s):  
Cystic Fibrosis ◽  
Differential Diagnosis ◽  
Forced Expiratory Volume ◽  
Sweat Test ◽  
Inherited Disease ◽  
Injury Rates ◽  
Computed Tomographic ◽  
Sweat Chloride ◽  
Average Survival ◽  
Pancreatic Exocrine Deficiency

Cystic fibrosis (CF) is an autosomal recessive disease characterized by an elevated sweat chloride level, diffuse bronchiectasis, and pancreatic exocrine deficiency. It is the most common lethal inherited disease in whites. Most patients present at birth or early childhood, although later diagnoses are not infrequent. Once CF was uniformly fatal at an early age, but advances in nutrition, airway clearance, and infection management have led to an average survival of 37 years. The newest aspect of care is the advent of protein modulators, which may increase life expectancy even further. This chapter discusses the epidemiology, genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of CF. The definition, epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis of non-CF bronchiectasis are also covered. Figures illustrate normal and abnormal CF transmembrane conductance regulators, the vicious cycle hypothesis of lung injury, rates of respiratory germs by age, the diagnosis of CF, the therapeutics pipeline for CF, forced expiratory volume in 1 second lung function percent predicted versus body mass index, and the median predicted survival age of patients with CF. A chest x-ray and chest computed tomographic scan of CF are also provided. Tables outline the most common CF mutations in 2011, class mutations of CF, a mnemonic for acute exacerbations of CF, the diagnosis of CF-related diabetes in a stable patient, sweat test values, and the differential diagnosis of bronchiectasis.This chapter contains 9 highly rendered figures, 6 tables, 143 references, 1 teaching slide set, and 5 MCQs.

Test for the Concentration of Electrolytes in Cystic Fibrosis of the Pancreas Utilizing Pilocarpine by Iontophoresis, by Lewis E. Gibson and Robert E. Cooke,Pediatrics; 1959;24:545–549

PEDIATRICS ◽  
1998 ◽  
Vol 102 (Supplement_1) ◽  
pp. 230-231
Author(s):  
Victor Chernick
Keyword(s):  
Cystic Fibrosis ◽  
Filter Paper ◽  
Direct Injection ◽  
Heat Stroke ◽  
Chloride Concentration ◽  
Hot Water ◽  
Sweat Test ◽  
High Concentration ◽  
Sweat Chloride ◽  
Plastic Bag

Aim. To develop a method for stimulating sweating that is rapid, painless, and avoids the risk of heat stress. Background. Since the discovery that there is a high concentration of sodium and chloride in the sweat of patients with cystic fibrosis of the pancreas in 1953, the sweat test has been performed by placing the patient's body in a plastic bag with or without hot water bottles to stimulate sweating. This method is unsatisfactory because of complications such as hyperpyrexia and heat stroke. Direct injection of a cholinergic agent intradermally is painful and therefore not practical. Methods. A rheostat with a milliampere meter was constructed at a cost of ∼$7 that allowed the iontophoresis of pilocarpine into the skin using negative and positive (2-cm diameter) electrocardiography electrodes. The positive electrode was placed on the flexor surface of the arm over a filter paper soaked in 0.2 mL of 0.2% pilocarpine nitrate. Current (0.2 mA) was applied for 5 minutes and then sweat was collected onto a preweighed filter paper for 30 minutes. Sweat chloride was determined by a polarographic method. Sweat tests were performed on 25 patients with cystic fibrosis (CF), 17 asymptomatic relatives and 27 control patients. Patients with CF had sweat chloride concentration >80 mEq/L; relatives, 32.5 mEq/L (highest 57 mEq/L); and control subjects, 21.1 mEq/L (highest 60 mEq/L). Conclusions. The iontophoresis of pilocarpine into the skin is a rapid, painless, safe, and reliable method for stimulating sweating and facilitating the determination of sweat chloride concentration.

Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

2014 ◽  
Author(s):  
Michael J Stephen
Keyword(s):  
Cystic Fibrosis ◽  
Differential Diagnosis ◽  
Forced Expiratory Volume ◽  
Sweat Test ◽  
Inherited Disease ◽  
Injury Rates ◽  
Computed Tomographic ◽  
Sweat Chloride ◽  
Average Survival ◽  
Pancreatic Exocrine Deficiency

Cystic fibrosis (CF) is an autosomal recessive disease characterized by an elevated sweat chloride level, diffuse bronchiectasis, and pancreatic exocrine deficiency. It is the most common lethal inherited disease in whites. Most patients present at birth or early childhood, although later diagnoses are not infrequent. Once CF was uniformly fatal at an early age, but advances in nutrition, airway clearance, and infection management have led to an average survival of 37 years. The newest aspect of care is the advent of protein modulators, which may increase life expectancy even further. This chapter discusses the epidemiology, genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of CF. The definition, epidemiology, etiology, pathogenesis, diagnosis, management, and prognosis of non-CF bronchiectasis are also covered. Figures illustrate normal and abnormal CF transmembrane conductance regulators, the vicious cycle hypothesis of lung injury, rates of respiratory germs by age, the diagnosis of CF, the therapeutics pipeline for CF, forced expiratory volume in 1 second lung function percent predicted versus body mass index, and the median predicted survival age of patients with CF. A chest x-ray and chest computed tomographic scan of CF are also provided. Tables outline the most common CF mutations in 2011, class mutations of CF, a mnemonic for acute exacerbations of CF, the diagnosis of CF-related diabetes in a stable patient, sweat test values, and the differential diagnosis of bronchiectasis.This chapter contains 9 highly rendered figures, 6 tables, 143 references, 1 teaching slide set, and 5 MCQs.

Cystic fibrosis newborn screening: outcome of infants with normal sweat tests

2017 ◽  
Vol 103 (8) ◽  
pp. 753-756 ◽  
Author(s):  
Claire Edmondson ◽  
Christopher Grime ◽  
Ammani Prasad ◽  
Jacqui Cowlard ◽  
Chinedu E C Nwokoro ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Newborn Screening ◽  
Genome Sequencing ◽  
Dna Analysis ◽  
Gene Mutations ◽  
High Serum ◽  
Sweat Test ◽  
Sweat Chloride ◽  
South East England

Newborn babies positively screened for cystic fibrosis (CF) (high serum immunoreactive trypsin (IRT) with DNA analysis) are referred for a diagnostic sweat test, which may be normal (sweat chloride <30 mmol/L). Unless two gene mutations are identified during Newborn screening (NBS), the babies are discharged from follow-up. We wished to check that none had subsequently developed symptoms suggestive of CF. We retrospectively reviewed patient notes and contacted general practitioners of all babies with a negative sweat test, conducted in one of the four paediatric specialist CF centres in London, over the first 6 years of screening in South East England.Of 511 babies referred, 95 (19%) had a normal sweat test. Five (5%) had CF diagnosed genetically, two of them on extended genome sequencing after clinical suspicion. Eleven (12%) were designated as CF screen positive inconclusive diagnosis (CFSPID); one of the five CF children was originally designated as CFSPID. Seventy-nine (83%) were assumed to be false-positive cases and discharged; follow-up data were available for 51/79 (65%); 32/51 (63%) had no health issues, 19/51 (37%) had other significant non-CF pathology.These results are reassuring in that within the limitations of those lost to follow-up, CF symptoms have not emerged in the discharged children. The high non-CF morbidity in these children may relate to known causes of high IRT at birth. Clinicians need to be aware that a child can have CF despite a normal sweat test following NBS, and if symptoms suggest the diagnosis, further testing, including extended genome sequencing, is required.

scholarly journals A quality improvement program for adolescents with cystic fibrosis: focus on psychosocial skills

2018 ◽  
Vol 13 (S1) ◽  
Author(s):  
Michele Gérardin ◽  
Anne Pesle ◽  
Dominique Pougheon-Bertrand ◽  
Pilar Léger ◽  
Céline Vallet ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Quality Improvement ◽  
Quality Improvement Program ◽  
Improvement Program ◽  
Psychosocial Skills

scholarly journals The Need for Quality Improvement in Sweat Testing Infants after Newborn Screening for Cystic Fibrosis

2010 ◽  
Vol 157 (6) ◽  
pp. 1035-1037 ◽  
Author(s):  
Vicky A. LeGrys ◽  
Susanna A. McColley ◽  
Zhanhai Li ◽  
Philip M. Farrell
Keyword(s):  
Cystic Fibrosis ◽  
Quality Improvement ◽  
Newborn Screening ◽  
Sweat Testing

scholarly journals Sweat test and cystic fibrosis: overview of test performance at public and private centers in the state of São Paulo, Brazil

2017 ◽  
Vol 43 (2) ◽  
pp. 121-128 ◽  
Author(s):  
Maria Fátima Servidoni ◽  
Carla Cristina Souza Gomez ◽  
Fernando Augusto Lima Marson ◽  
Adyléia Aparecida Dalbo Contrera Toro ◽  
Maria Ângela Gonçalves de Oliveira Ribeiro ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Test Performance ◽  
Sao Paulo ◽  
The State ◽  
Sweat Test ◽  
São Paulo ◽  
Cross Sectional ◽  
Public And Private ◽  
Qualified Personnel ◽  
Increasing Demand

ABSTRACT Objective: The sweat test (ST) measures chloride levels in sweat and is considered the gold standard for the diagnosis of cystic fibrosis (CF). However, the reliability of a ST depends on their being performed by experienced technicians and in accordance with strict guidelines. Our aim was to evaluate how sweat stimulation, sweat collection, and chloride measurement are performed at 14 centers (9 public centers and 5 private centers) that routinely perform STs in the state of São Paulo, which has the highest frequency of CF in Brazil. Methods: This was a cross-sectional cohort study, using a standardized questionnaire administered in loco to the staff responsible for conducting STs. Results: No uniformity regarding the procedures was found among the centers. Most centers were noncompliant with the international guidelines, especially regarding the collection of sweat (the samples were insufficient in 10-50% of the subjects tested); availability of stimulation equipment (which was limited at 2 centers); modernity and certification of stimulation equipment (most of the equipment having been used for 3-23 years); and written protocols (which were lacking at 12 centers). Knowledge of ST guidelines was evaluated at only 1 center. Conclusions: Our results show that STs largely deviate from internationally accepted guidelines at the participating centers. Therefore, there is an urgent need for standardization of STs, training of qualified personnel, and acquisition/certification of suitable equipment. These are essential conditions for a reliable diagnosis of CF, especially with the increasing demand due to newborn screening nationwide, and for the assessment of a possible clinical benefit from the use of modulator drugs.

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