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Opoka-Winiarska V.,Medical University of Lublin | Cofta S.,Poznan University of Medical Sciences | Mazurek H.,National Institute for Tuberculosis and Lung Diseases | Kozielski J.,Medical University of Silesia, Katowice
Pneumonologia i Alergologia Polska | Year: 2015

The proper care of cystic fibrosis patients extends over their lifetime. More than half of the children with the disease die before adulthood. An important element in the patient’s care is a time of transition from a paediatric to the care of an internist and the patient’s acceptance of this necessity. Transition from paediatric care to an internist should be adequately prepared. It is not only a question of transfer of medical records, but also careful preparation of patients for such transition. The patients expect not only continuity of care but also the introduction to the management with the disease. The creation of a base for specialist hospital treatment for exacerbation of the disease at the adulthood is an important element in the care of these patients. The problem has been solved in the children group, but is still waiting for solution in adults with cystic fibrosis. It has been proven that care in the centres carried out by a specialized team ensures longer life and better quality of life of these patients. The paper is an overview of these two important elements of care of adults with cystic fibrosis. © 2015 PTChP.


Lisowska A.,Poznan University of Medical Sciences | Pogorzelski A.,National Institute for Tuberculosis and Lung Diseases | Oracz G.,Child Memorial Health Institute | Skorupa W.,National Tuberculosis and Lung Diseases Research Institute | And 4 more authors.
Acta Scientiarum Polonorum, Technologia Alimentaria | Year: 2010

Background. Available data suggests that small intestine bacterial overgrowth (SIBO) may frequently occur in cystic fibrosis (CF) subjects. SIBO may result in synthesis of enterotoxic and unabsorbable metabolites which may cause mucosal damage and - additionally - interfere with digestion and absorption. Such a relationship was documented in CF mouse model. Therefore, in the present study we aimed to assess the influence of bacterial overgrowth in small intestine in CF patients on lipid digestion and absorption. Material and methods. The study comprised 60 pancreatic insufficient CF patients, 30 children and 30 adults. All enrolled CF subjects were tested for the presence of SIBO using hydrogen/methane breath test with glucose loading. According to the obtained results CF patients were divided into SIBO positive and negative subgroups. Subsequently, 13C-labelled mixed triglyceride breath test was performed to assess lipid digestion and absorption. Cumulative percentage dose recovery (cPDR) was considered to reflect digestion and absorption of lipids. Results. SIBO was detected in 12 (40.0%) children and 11 (36.7%) adults with CF. The cPDR did not differ between SIBO positive and negative subgroups, neither when assessed separately for children (mean ±SEM: 5.5 ±0.8 vs. 7.4 ±1.0%) and adults (4.9 ±0.8 vs. 7.1 ±0.7%) nor for the entire studied population. Conclusions. Small intestine bacterial overgrowth does not seem to play a key role in lipid digestion and absorption in cystic fibrosis patients. © Copyright by Wydawnictwo Uniwersytetu Przyrodniczego w Poznaniu.


Lisowska A.,Poznan University of Medical Sciences | Mdry E.,Poznan University of Medical Sciences | Pogorzelski A.,National Institute for Tuberculosis and Lung Diseases | Szydlowski J.,Poznan University of Medical Sciences | And 3 more authors.
Scandinavian Journal of Clinical and Laboratory Investigation | Year: 2010

Introduction. Small intestine bacterial overgrowth (SIBO) has been reported in cystic fibrosis (CF) patients. However, the potential link to intestinal inflammation has not been studied so far. Therefore, we aimed to assess whether SIBO correlates with intestinal inflammation in CF patients. Material and methods. As a preliminary study, we assessed whether calprotectin is detectable in sputum expectorated by 10 CF patients. Since significant immunoreactivity was documented, in the major study we have included exclusively CF subjects not expectorating sputum for at least two weeks. Fecal calprotectin was measured in 25 CF patients and 30 healthy subjects (HS). All CF subjects were tested for the presence of SIBO using the hydrogen-methane breath test (BT). According to obtained results CF patients were divided into SIBO positive and negative subgroups. Subsequently, the intensity of intestinal inflammation in both subgroups was compared. Results. Fecal calprotectin concentrations in CF patients (range: 1.8-302.5; median 80.0 mg/L) were significantly higher (p < 0.00001) than in HS (not detectable - 15.5; 2.5 mg/L). Calprotectin levels were abnormal in 21 (84%) studied CF subjects and none of HS. Abnormal BT results were found in 10 (40.0%) of CF patients. Calprotectin concentrations in SIBO positive and negative patients did not differ. Conclusions. Gastrointestinal inflammation is a frequent finding in cystic fibrosis patients. However, small intestine bacterial overgrowth does not seem to be the major or at least not the only determinant of intestinal inflammation. Indirect measures of intestinal inflammation in CF patients may give false positive results. © 2010 Informa Healthcare.


Madry E.,Poznan University of Medical Sciences | Fidler E.,Poznan University of Medical Sciences | Sobczyska-Tomaszewska A.,Genomed Inc. | Lisowska A.,Poznan University of Medical Sciences | And 12 more authors.
European Journal of Human Genetics | Year: 2011

Taking into account the reported incidence of hypolactasia in cystic fibrosis (CF) and the possible impact of milk products on nutritional status we aimed to assess the genetic predisposition to adult-type hypolactasia (ATH) and its incidence in CF. Single nucleotide polymorphism upstream of the lactase gene (LCT) was assessed in 289 CF patients. In subject with 13910C/C genotype (C/C) predisposing to ATH, hydrogen-methane breath test (BT) with lactose loading was conducted and clinical symptoms typical for lactose malabsorption were assessed. The percentage of CF patients with C/C was similar to that observed in healthy subjects (HS) (31.5 vs 32.5%). Eleven out of 52 (24.5%) CF C/C patients had abnormal BT results. The recalculated frequency of lactose malabsorption was similar for the entire CF and HS populations (6.9 vs 7.2%). Similarly as in the control group, few CF patients have identified and linked to lactose consumption clinical symptoms. The frequency of LCT polymorphic variants in CF patients having and not having severe mutations of CFTR gene showed significant differences. The C allele was more frequent in homozygotes of the severe mutations than in patients carrying at least one mild/unknown mutation (P0.0028) and in patients with at least one mild mutation (P0.0377). In conclusion, CF patients carrying mild CFTR mutations seem to have lower genetic predisposition to ATH. Lactose malabsorption due to ATH in CF is not more frequent than in the general population. Symptomatic assessment of lactose malabsorption in CF is not reliable. © 2011 Macmillan Publishers Limited All rights reserved.


Walkowiak J.,University of Life Sciences in Poznan | Blask-Osipa A.,Poznan University of Medical Sciences | Lisowska A.,Poznan University of Medical Sciences | Oralewska B.,Child Memorial Health Institute | And 6 more authors.
Acta Biochimica Polonica | Year: 2010

Background: The coexistence of cystic fibrosis (CF) and celiac disease (CD) has been reported. To our knowledge there is no study directly comparing the incidence of CD in CF patients to that in the general population at the same time. There is no published data on genetic predisposition to CD in CF patients either. Therefore, in the present study we aimed to assess the genetic predisposition to CD and its incidence in CF patients comparing it to data from the general population. Patients and methods: Two hundred eighty-two CF patients were enrolled in the study. In 230 CF patients the genetic predisposition to CD (the presence of HLA-DQ2/DQ8) was assessed. In all CF patients, serological screening for CD was conducted. In patients with positive antiendomysial antibodies (EMA) gastroduenoscopy was offered. Intestinal histology was classified according to modified Marsh criteria. The results of serological CD screening in 3235 Polish schoolchildren and HLA-DQ typing in 200 healthy subjects (HS) were used for comparison. Results: Positive EMA was found in 2.84 % of the studied CF patients. The incidence of proven CD was 2.13 %. The incidence of CD as well as positive serological screening were significantly more frequent in the CF group than in the general population. The frequency of CD-related HLA-DQ alleles in CF and HS did not differ. Conclusions: Genetic predisposition to celiac disease in cystic fibrosis patients is similar to that of the general population. However, our results suggest that cystic fibrosis is a risk factor for celiac disease development.

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