Cystic fibrosis and renal disease: a case report

Al-Shawwa, Baha; Rao, A. Ravishankar

doi:10.1186/1752-1947-1-24

Cited by 7 publications

(3 citation statements)

References 16 publications

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“…Al- Shawwa and Rao, 2007 early life, which may induce wheeze that may develop into asthma and is also a major precipitant of asthma exacerbations. Epidemiology -RSV is the most important respiratory pathogen of children under the age of 2 years and primary infections are a common cause of LRT disease (Hall, 1999;Henrickson et al, 2004).…”

Section: Rsv and Asthmamentioning

confidence: 99%

Understanding the mechanisms of viral induced asthma: New therapeutic directions

Hansbro

Horvat

Wark

2008

Pharmacology & Therapeutics

115

113

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Asthma is a common and debilitating disease that has substantially increased in prevalence in Western Societies in the last 2 decades. Respiratory tract infections by respiratory syncytial virus (RSV) and rhinovirus (RV) are widely implicated as common causes of the induction and exacerbation of asthma. These infections in early life are associated with the induction of wheeze that may progress to the development of asthma. Infections may also promote airway inflammation and enhance T helper type 2 lymphocyte (Th2 cell) responses that result in exacerbations of established asthma. The mechanisms of how RSV and RV induce and exacerbate asthma are currently being elucidated by clinical studies, in vitro work with human cells and animal models of disease. This research has led to many potential therapeutic strategies and, although none are yet part of clinical practise, they show much promise for the prevention and treatment of viral disease and subsequent asthma.

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Section: Rsv and Asthmamentioning

confidence: 99%

Understanding the mechanisms of viral induced asthma: New therapeutic directions

Hansbro

Horvat

Wark

2008

Pharmacology & Therapeutics

115

113

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“…(11) However, some of the case studies in this field have reported relevant issues, including the fact that CF patients with renal disease can present significant proteinuria, although kidney function (blood urea nitrogen and creatinine) can be normal. (12) Proteinuria progressing to nephrotic syndrome has an extremely poor prognosis. (13,14) In healthy persons, mean urinary protein excretion is 60-80 mg, with an upper limit of 150 mg/day.…”

Section: Methodsmentioning

confidence: 99%

Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype

Cemlyn-Jones

Gamboa

2009

J. bras. pneumol.

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Objective: To assess proteinuria in patients with cystic fibrosis (CF), and to correlate proteinuria with genotype, CF-related diabetes and disease severity. Methods: A prospective study was carried out over a six-month period and involving 22 CF patients. After the collection and analysis of 24-h urine samples, the patients were divided into two subgroups: protein excretion < 150 mg/day (low-proteinuria); and protein excretion ≥ 150 mg/day (highproteinuria). Patient charts were reviewed to obtain data on genotype and CF-related diabetes. Disease severity was assessed based on acute exacerbations in the last six months and FEV 1 measured during the study period. To assess the correlation between genotype and proteinuria, the two main mutations (ΔF508 and R334W) were evaluated. Due to the existence of genotype ΔF508/R334W, two categories were created to enable statistical analysis, ΔF508 being evaluated in category 1 and R334W being evaluated in category 2. Results: The ΔF508 mutation tended to be associated with normal protein excretion: 100% of the low-proteinuria subgroup patients were considered ΔF508 in category 1, compared with 86.7% in category 2. Protein excretion tended to be higher in patients with the R334W mutation: 60.0% of the high-proteinuria subgroup patients were considered R334W in category 1, compared with 80.0% in category 2 (p = 0.009 and p = 0.014, respectively). No significant association was found for any of the other variables. Conclusions: The results suggest that genotype is associated with renal phenotype, depending on the mechanism by which the genotype alters the function of the cystic fibrosis transmembrane conductance regulator gene.Keywords: Proteinuria; Cystic fibrosis; Genotype. ResumoObjetivo: Avaliar a proteinúria em pacientes com fibrose cística (FC) e correlacioná-la com o genótipo, com a diabetes relacionada à FC e com a gravidade da doença. Métodos: Estudo prospectivo realizado num período de seis meses com 22 pacientes com FC. Efetuada proteinúria de 24 h com a divisão dos pacientes em dois subgrupos: proteinúria < 150 mg/dia (proteinúria-baixa); e proteinúria ≥ 150 mg/dia (proteinúria-alta). Revisamos os prontuá-rios clínicos para a coleta de informações sobre o genótipo e a presença de diabetes relacionada à FC. A gravidade da doença foi avaliada pelas exacerbações agudas no último semestre e pelo VEF 1 durante o período de estudo. Para avaliar a correlação entre genótipo e proteinúria, consideraram-se as duas principais mutações, ΔF508 e R334W. Dada a existência do genótipo ΔF508/R334W, foram criadas duas categorias para se proceder à avaliação estatística, sendo esse genótipo considerado ΔF508 na categoria 1 e R334W na categoria 2. Resultados: A mutação ΔF508 se associou com valores normais de proteinúria: 100% dos pacientes do subgrupo proteinúria-baixa foram considerados ΔF508 na categoria 1, comparados a 86,7% na categoria 2. Em pacientes com a mutação R334W, os valores de proteinuria foram mais elevados: 60,0% dos pacientes do subgrupo proteinúria-alta for...

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“…Additional renal manifestations occasionally reported in children and teenagers include amiloid protein A (AA) type amyloidosis, membranoproliferative glomerulonephritis, and postinfectious glomerulonephritis 9 - 11) . The number of CF-NS associated cases is limited in the literature 10 , 12) . In this report, we present two rare cases of both NS and CF in children.…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

et al. 2013

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Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

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Cystic fibrosis and renal disease: a case report

Cited by 7 publications

References 16 publications

Understanding the mechanisms of viral induced asthma: New therapeutic directions

Understanding the mechanisms of viral induced asthma: New therapeutic directions

Proteinuria in cystic fibrosis: a possible correlation between genotype and renal phenotype

Cystic fibrosis of pancreas and nephrotic syndrome: a rare association

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