Profile of cystic fibrosis in two reference centers in southern Brazil

Rizzo, Laís Cristina; Fischer, Gilberto Bueno; Maróstica, Paulo José Cauduro; Mocelin, Helena Teresinha

doi:10.1590/1806-9282.61.02.150

Cited by 4 publications

(7 citation statements)

References 20 publications

(26 reference statements)

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“…Most of the study subjects were female, Caucasian, from the countryside of the State of Rio Grande do Sul, with a F508del mutation and the presence of MSSA in the sputum. These data were similar to those observed in studies conducted in this ( 10 , 11 ) and other states of Brazil. ( 12 , 13 )…”

Section: Discussionsupporting

confidence: 92%

Medications used in pediatric cystic fibrosis population

Alves¹,

Frank²,

Bueno³

2018

Einstein (São Paulo)

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Objective To describe the drug utilization profile used by pediatric cystic fibrosis patients.Methods A transversal study comprising the analysis of records and interviews with caregivers of pediatric patient in a reference center of Southern Brazil. We collected information about patients’ clinical condition, medication used and household therapy.Results Out of 78 patients participating in the study, prevailing characteristics were: female, self-declared white color, mutation F508del and countryside resident. Forty-three patients had health monitoring exclusively in the hospital’s outpatient division. We analyzed 509 prescribed medication (6.5 medication/patient). The caregiver acknowledged the correct indication in 83% of cases. Patients with pulmonary complications and diseases and/or comorbities related to the cystic fibrosis had an increased quantity of prescribed medication. Vitamins, pancreatic enzymes, hypertonic saline solution, dornase alpha, acid ursodesoxicolic and inhalation antibiotics were most commonly prescribed. Out of the sum of medication, 265 (52.1%) were registered in the Relação Nacional de Medicamentos Essenciais , 26.7% were registered in the basic component and 25.4% were registered in the specialized component of pharmaceutical assistance. Seventy-four interviewees informed difficulty in the acquisition of at least one prescribed medication. Most of the reports acknowledge the State Health Department as the place to find and receive medication for cystic fibrosis.Conclusion This study allowed reaching a deeper understanding about therapy, caring needed with patients with cystic fibrosis, highlighting to implement strategies that might contribute to enhance life quality and to execute the patients’ therapy plan.

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Section: Discussionsupporting

confidence: 92%

Medications used in pediatric cystic fibrosis population

Alves¹,

Frank²,

Bueno³

2018

Einstein (São Paulo)

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“…The pulmonary phenotype was the most frequent (92.2%), similar to that described by Rizzo et al (92.0%) [ 17 ]. Half of the patients capable of performing spirometric maneuvers showed normal spirometry, higher than the reported data in the REBRAFC, where 40% of the patients underwent normal spirometry [ 14 ].…”

Section: Discussionsupporting

confidence: 83%

“…Gastrointestinal involvement was present in almost half of the cases, reflecting the trend reported in the Brazilian literature [ 17 ]. Constipation was the most frequent gastrointestinal manifestation (33.8%), similar to that described by Gelfond and Borowitz (32.0%) [ 2 ].…”

Section: Discussionsupporting

confidence: 67%

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents

Santos

Nogueira

et al. 2018

Pediatr Gastroenterol Hepatol Nutr

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PurposeThe objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis.MethodsA cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations.ResultsOf the 77 patients, 51.9% were male, with a median age of 147 months (7.0–297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02).ConclusionThe pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

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“…Si bien, P. aeruginosa y S. aureus son reportadas como las principales cepas en pacientes con FQ, generalmente, las infecciones por S. aureus y H. influenzae son las que predominan en pacientes pediátricos de países del Norte (14,15) o en poblaciones más cercanas como Argentina (20) o Brasil (21) . Sin embargo, en los pacientes con FQ de Perú, P. aeruginosa sería el patógeno predominante, tanto en pacientes pediátricos como en adultos.…”

Section: Discussionunclassified