Objectives The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis and to describe the sociodemographic, clinical, and laboratory profile of these patients. Methods This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered. Results Hepatobiliary disease was diagnosed in 16.4% of the patients and occurred as an initial manifestation of cystic fibrosis in 55.6% of these cases. The diagnosis of hepatopathy occurred before or concomitantly with the diagnosis of cystic fibrosis in 88.9% of the children. All patients with hepatobiliary disease were considered non-white, with a predominance of females (77.8%) and median (IQR) of 54 (27–91) months. Compared with the group without hepatobiliary disease, children with liver disease had a higher frequency of severe mutations identified in the CFTR gene (77.8% vs . 39.6%, p  = 0.033) and severe pancreatic insufficiency (88.9% vs . 31.6%, p  = 0.007). Conclusion The frequency of hepatobiliary disease was high, with a very early diagnosis of the disease and its complications in the studied series. A statistical association was observed between the occurrence of hepatobiliary disease and the presence of pancreatic insufficiency and severe mutations in the CFTR gene. It is emphasized that cystic fibrosis is an important differential diagnosis of liver diseases in childhood.

http://www.scielo.br/pdf/jped/v94n5/0021-7557-jped-94-05-0504.pdf

Hepatobiliary disease in children and adolescents with cystic fibrosis.

Attitudes of pain and opioids prescription practices in U.S. cystic fibrosis centers.

Resumen Objetivo Precisar la prevalencia de enfermos con fibrosis quistica (FQ) diagnosticados en edades superiores a los 18 anos y analizar sus caracteristicas clinicas, geneticas y microbiologicas. Pacientes y metodos Estudio observacional, transversal y descriptivo de todos los pacientes diagnosticados de FQ a edad igual o superior a los 18 anos. Las variables analizadas fueron: edad actual, edad al diagnostico, sexo, nacionalidad, parametros de funcion pulmonar, patologias presentes al momento del diagnostico, caracteristicas microbiologicas y hallazgos geneticos. Resultados Se incluyeron 89 pacientes (14,8%, del total de 600 pacientes en seguimiento en las unidades participantes), 45 mujeres (50,6%) y 44 varones (49,4%), con una edad media al diagnostico de 36,4 anos. Ochenta y un pacientes (91%) eran de nacionalidad espanola. La prueba del sudor fue diagnostica en 77 de los 89 estudiados (86,5%). Las mutaciones detectadas con mayor frecuencia fueron la F508del/otra y la G542X/otra y los hallazgos clinicos mas frecuentes en el momento del diagnostico fueron las bronquiectasias en 33 pacientes (37,1%) y la esterilidad en 12 (13,5%). Los microorganismos colonizadores mas frecuentes fueron Staphylococcus aureus (S.aureus) sensible a meticilina (23,6%) y Pseudomonas aeruginosa (P. aeruginosa) (13,5%). La mayoria de los pacientes presentaban una alteracion ventilatoria obstructiva leve y no tenia afectacion pancreatica. La prueba del sudor con frecuencia ofrecio resultados no concluyentes. Conclusiones La FQ es tambien una enfermedad de diagnostico en la edad adulta. Los pacientes diagnosticados en edad adulta presentan una funcion pulmonar levemente alterada y una baja incidencia de afectacion pancreatica, por lo que su pronostico tiende a ser favorable.

Fibrosis quística diagnosticada en edad adulta

Aim To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Patients and methods Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Results Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508 del /other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus ( S. aureus ) (23.6%) and Pseudomonas aeruginosa ( P . aeruginosa ) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. Conclusions CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable.

Cystic fibrosis in adult age

This review evaluates the effects of specialized food diets for children with cystic fibrosis (CF). Children with CF have special dietary needs that can be met by adding specialty foods. Some dietary needs that children with cystic fibrosis encounter include the need for more calories to balance the extra energy it takes to breathe. Another dietary need is extra vitamins and antioxidants to help fight off lung infections. The effects on the dietary needs of children with CF are also described in detail. Issues associated with diet leading to growth and daily function, like CF-related diabetes, are important factors affecting novel food diets. The benefits and risks of specialty foods and specific diets are reviewed. For example, the benefits of blueberries include their antioxidant components and low sugar content. However, the risk of eating too many blueberries is that the low sugar content can be dangerous for children with diabetes. Also, the results of clinical studies are discussed in terms of specific foods that pertain to the diets of children with CF. A table documents the specialty foods and diets, as well as a brief outline of the benefits and risks of each food and diet. For example, carrot juice is included in the table, noting the high amount of bioaccessible provitamin A as a benefit, and the high sugar content as a risk. Another example included in the table is avocado, which has the benefits of monounsaturated, polyunsaturated fats, fiber, vitamins, and potassium. The risks involved with eating avocado is that it is high in protein, so it may require an extra dose of pancreatic enzyme to assist in digestion.

Specialty Foods for Children with Cystic Fibrosis

The international literature shows that the demography of cystic fibrosis (CF) is changing, with patients increasingly surviving into adulthood. As they age, patients with CF become more susceptible to specific non-pulmonary chronic diseases. In this study, adult data from the CF Registry of Ireland (CFRI) was used to determine the prevalence and associated features of these diseases. 104 (25.7%) adults had diabetes versus 13 (2.9%) children (p < 0.001). Liver disease was present in 47 (11.6%) adults and 26 (5.7%) children (p = 0.002). 173 (42.7%) adults had bone disease versus 25 (5.5%) children (p < 0.001). Adults with one non-pulmonary chronic disease, for example liver disease, were more likely to have another (p = 0.002), those with diabetes and bone disease had a higher number of hospital admissions in the last 12 months (p < 0.001 for both) and higher rates of depression (p = 0.046 and p = 0.049, respectively). These results highlight a number of challenges for the Irish healthcare system.

Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the Cystic Fibrosis Registry.

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Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the Cystic Fibrosis Registry.