Search results

1

Caregiver burden and the role of social support in the care of children with cystic fibrosis

100%

Dębska G., Milaniak I., Domańska D., Tomaszek L.

Family Medicine & Primary Care Review

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2019

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issue 2

98-103

XX

Background. The effort involved in caring for a patient suffering from cystic fibrosis lies with its parents/caregiver, becoming the cause of excessive burden. In such a situation, social support is an important strategy for coping with chronic illnesses. Objectives. The aim of the study was to assess the level of burden and social support for parents of children with CF and to establish a relationship between them. Material and methods. The study involved 88 parents of patients with cystic fibrosis. The study utilized the standardized Caregiver Burden Scale (CB ) and the Berlin Social Support Scale (BSSS ). Results. The study group experienced an average burden level, which is dependent on the level of education. The highest level of burden was found in two subscales: disappointment and general effort, and the lowest was in the emotional involvement subscale. The level of support in the studied group was high. The largest was observed in the subscale perceived support and received support, and the lowest in the subscale seeking support. Analysis of the regression of the dependent variable of the caregiver’s level of burden showed that the level of burden determines the need for support. It has been observed that as the level of the caregiver’s burden increases, the need for support also increases. In turn, the smaller the caregiver’s burden, the lower the need for support currently received. Conclusions. The caregiver burden on the parent of a children suffering from CF and the received social support are important factors influencing each other in the care of a chronically ill child. This means that the more support received, the lower the sense of burden the caregiver experiences

2

Adults with cystic fibrosis – medical challenges and a psychosocial adaptation in the context of risk of a premature death

100%

Zubrzycka R.

Człowiek - Niepełnosprawność - Społeczeństwo

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2020

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vol. 48(2)

83-96

EN

Cystic Fibrosis (CF) is the most common life-limiting genetic disorder of Caucasians. It impairs the functions of many organs with its greatest effects on the lungs and digestion. Treatment of the disease involves a multi- component regimen, including airway clearance techniques, nebulized medications, antibiotics, pancreatic enzymes and increased caloric intake. Cystic fibrosis is a burden for patients and their families in the context of their daily life tasks. The article presents generic medical, psychosocial problems of adult patients with CF, which are still little known in Poland. The detailed issues are: medical characteristics of cystic fibrosis as a rare disease, organizational conditions of rehabilitation, specific psychosocial problems including: a depression, an anxiety and a reproductive health in cystic fibrosis in the context of a premature death.

3

Serum and urinary carnitine in children with cystic fibrosis

100%

Kępka A., Minarowska A., Minarowski Ł., Ł, Waszkiewicz N., Chojnowska S., Trochimowicz L., Zwierz K., Chyczewska E., Szajda, Sł. D

Progress in Health Sciences

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2013

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vol. 3

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issue 1

13-18

EN

Purpose: Cystic fibrosis (CF) is inherited, congenital disease of multi-organ expression. Carnitine play a role as a lipid acid transporter to mitochondrium for beta-oxydation. Acylation of carnitine is inevitable for detoxication processes in cells. Low lean body mass In CF patients can lead to decreased levels. The aim of the study was the evaluation of free carnitine, acylcarnitine and acylcarnitine/free carnitine ratio in serum and urine of children with cystic fibrosis. Material and methods: The study was conducted in a group of 15 CF children (4 F, 11 M), aged 12.6 ±5.4 years. The serum for a control group was collected from 32 healthy children. Urine samples for control group was collected from 62 health children. Free carnitine and total carnitine was assessed using spectrophotomeric method in which acyl group is transferred from acetyl-CoA to carnitine by carnitine acetyltransferase (CAT). Acyl carnitine concentration and acylcarnitine/free carnitine ratio was counted using Schmidt- Sommerfeld and Seccombe equation. Results: In 12 CF patients (80%) free carnitine and total carnitine was below lower limit of normal (p<0.001). In 9 patients (60%) free carnitine level was ≤ 20 µmol/L, which can be clinically diagnosed. Acylcarnitine levels were also statistically lower in CF group (p<0.01). Acylcarinitie/free carnitine ratio did not differ between the groups (p=0.05). Urine excretion of free carnitine, total carnitine and acylcarnitine was lower in CF group (p<0.001). Conclusions: In CF pediatric patients statistically significant lower levels of free carnitine, total carnitine and acylcarnitine were observed in comparison to controls. Low urine excretion of free carnitine, total carnitine and acylcarnitine was lower in CF group. No correlation between serum and urine levels of free carnitine, total carnitine and acylcarnitine were observed.

4

Electrophoretical mobility of nuclei in buccal cells of cystic fibrosis patients

100%

Minarowska A., Litwiejko-Pietryńczyk E., Minarowski Ł., Ł, Trochimowicz L., Sierżantowicz R., Dzięcioł J., Chyczewska E.

Progress in Health Sciences

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2013

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vol. 3

|

issue 1

61-64

EN

Introduction: Cystic fibrosis (CF) is inherited, metabolic, multisystem disease with various clinical symptoms. In the airways neutrophilic inflammation and increased levels of neutrophil elastase (NE) are observed even in young children. The method of electrophoretic mobility of cell nuclei (EMN) is a way of assessment cell maturity. Observed distorted nuclei mobility is mostly dependent on chromatin distribution and remodeling. Purpose: To evaluate the EMN index in buccal cells collected from CF patients. Materials and methods: The study was conducted in the group of 15 CF patients aged 5-26 years. Results: In the healthy subjects we have observed that the lowest EMN index values were reach around 2 year of life (7.6 ± 3.1) and in the late senescence (8.5 ± 2.5) with the peak values around 16-21 years (61.4 ± 2.5). The results differed significantly from the healthy controls. Conclusion: EMN index in CF buccal cells may be a simple method to quickly assess the chromatin remodeling.

5

Ocena realizacji zaleceń dietetycznych wśród dzieci chorych na mukowiscydozę

100%

Komorowska-Szczepańska W., Sobiecka M.

Family Medicine & Primary Care Review

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2014

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issue 3

245-247

6

Hand radiograms as an alternative for bone mass screening in cystic fibrosis

88%

Minarowska A., Minarowski Ł., Ł, Tomalak W., Zając J., Pogorzelski A., Radliński J., Milewska, A.J, Kirpsza B., Szajda, Sł. D, Chyczewska E.

Progress in Health Sciences

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2013

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vol. 3

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issue 1

7-12

EN

Purpose: Cystic fibrosis (CF) is a genetic, metabolic disease. Long-term therapy often leads to inappropriate calcification of bones. Dual X-ray absorptiometry (DXA) is considered a „gold-standard” for bone mineral density (BMD) assessment, but high usage costs can limit its availability. This paper compares two methods for BMD assessment in CF patients: hand radiograms method and densitometry using DXA method. Materials and methods: The study was performed in a group of 26 CF patients (10F, 16M), aged 7-30 years. In all cases, DXA measurements were performed, along with bone mass assessment using DENSY2004 system for digital assessment of hand radiograms. Stepwise binary logistic regression was used to examine the contribution of bone age, BMI, Cole's index and hand radiograms parameters to low BMD expressed as Z-score ≤-1SD. Results: Statistical analysis of the gathered data revealed that hand radiograms method allow for estimation of Z-score below -1SD with accuracy of 84.62% comparing to DXA. Sensitivity and specificity of this estimation in the studied group of CF patients was 86.67% and 81.82%. Conclusions: Hand radiograms method has good accuracy, sensitivity and specificity; therefore, it can be an alternative for DXA in BMD assessment. It can be implemented in chronic diseases affecting BMD like cystic fibrosis.

7

Mukowiscydoza

88%

Kloch M., Stanisławska I.

Edukacja Biologiczna i Środowiskowa

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2014

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issue 2

11-17

PL

Białko CFTR (ang. cystic fibrosis transmembrane conductance regulator, błonowy regulator przewodnictwa) jest transporterem ABC (ang. ATP-binding cassette) aktywowanym przez cAMP, który przenosi jony chlorkowe przez błonę komórkową. Transportery ABC stanowią dużą grupę białek błonowych, które wykorzystują energię z hydrolizy ATP do transportu rozmaitych cząsteczek przez błonę biologiczną wbrew gradientowi stężenia przenoszonej substancji. Białko CFTR różni się tym od pozostałych białek należących do rodziny transporterów ABC, że działa jako nabłonkowy kanał jonowy aktywowany przez cAMP, umożliwiający bierny transport jonów chlorkowych zgodnie z ich gradientem. Ponadto, białko CFTR może regulować inne nabłonkowe kanały jonowe lub transportery, wpływając w ten sposób na ruch Cl-, HCO3-, Na+, K+ i Ca2+, a także na ogólną równowagę jonową w nabłonku. Dysfunkcja białka CFTR jest przyczyną chorób, m.in. mukowiscydozy i przewlekłego zapalenie trzustki. W obu przypadkach nieprawidłowy przepływ jonów w obrębie tkanki nabłonkowej różnych narządów jest pierwotną przyczyną choroby.

EN

The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that functions as a cAMP-activated chloride channel. The ATP-binding cassette (ABC) transporters are a large group of transmembrane proteins that utilize the energy derived from ATP hydrolysis to transport various substrates across the membrane against the concentration gradient. The cystic fibrosis transmembrane conductance regulator (CFTR) differs from other members of this superfamily in that it functions as an epithelial cAMP-activated anion channel that allows for passive transport of chloride ions down their electrochemical gradient. Additionally, CFTR may regulate other epithelial ion channels or transporters, mediating the transmembrane Cl-, HCO3-, Na+, K+ and Ca2+ transport, thereby significantly contributing to the maintaining of the overall ion balance in the epithelia. Defective function of CFTR is responsible for several human diseases, including cystic fibrosis (CF), chronic pancreatitis, all associated with abnormal ion fluxes in epithelial tissues of different organs.

8

Identification of factors determining anxiety, depression and aggression, with particular emphasis on pain in Polish adolescents and young adults with cystic fibrosis

75%

Cepuch G., Tomaszek L., Pawlik L.

Family Medicine & Primary Care Review

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2018

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issue 2

112-116

9

Employment after lung transplantation in Poland – a single center study

75%

Ochman M., Latos M., Orzeł G., Pałka P., Urlik M., Nęcki M., Stącel T., Zembala M.

International Journal of Occupational Medicine and Environmental Health

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2019

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vol. 32

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issue 3

379-386

EN

Objectives Lung transplantation not only saves a patient’s life but also creates the opportunity for becoming more self-reliant and getting back to work. The aim of this single center study was to assess the prospects of employment, as well as its influence on the quality of life and physical activity, of the lung transplant recipients of the Silesian Center for Heart Diseases in Zabrze, Poland. Material and Methods A retrospective study covered 67 lung transplant recipients of the Silesian Center of Heart Diseases. Only patients with ≥ 6-month follow-up were included. All of the patients gave their written consent to be included in the study before filling out the questionnaire containing questions about employment, income, education and how work affected their quality of life before and after lung transplantation. A physical capability assessment was performed by climbing flights of stairs and by means of a 6-min walk test, and spirometry parameters were also measured. Results Twenty of the patients included in the study (31.7%) were employed after lung transplantation, 63.2% of whom worked full-time. Profession was changed by 2 patients (14.3%). The patients diagnosed with cystic fibrosis were found to have the highest chance of finding employment after lung transplantation. The statistical analysis revealed that the employed patients were able to cover longer distances during the 6-min walk test (556 m, on average) than the unemployed ones (494 m, on average). Conclusions One in 3 patients finds employment after lung transplantation. Work improves the quality of life of the majority of lung transplant recipients. The patients who are employed are also in a better physical condition, and they are more self-reliant in comparison to those who remain unemployed. Lung transplant recipients with cystic fibrosis are most likely to find employment, and so are patients with higher education. Int J Occup Med Environ Health. 2019;32(3):379–86

10

The disease and an immediate cause of death of Frederic Chopin

75%

Witt M.

Nauka

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2021

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issue 2

11

Przystosowanie społeczne dziecka z chorobą przewlekłą. Studium przypadku

63%

Kowalczyk K.

Roczniki Pedagogiczne

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2018

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vol. 10(46)

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issue 3

91-104

PL

Zdrowie to stan pełnego fizycznego, umysłowego i społecznego dobrego samopoczucia. To nie tylko brak choroby, ale pełna zdolność człowieka do życia w harmonii ze środowiskiem i prawidłowego reagowania na jego zmiany. Po przeciwnej stronie kontinuum, zgodnie z modelem salutogenezy A. Antonovsky’ego, znajduje się choroba. W życiu każdego człowieka, zwłaszcza dziecka, choroba jest sytuacją trudną, która często wymaga przeorganizowania, a niekiedy i całkowitej zmiany funkcjonowania. Specyficzne zmiany w tym funkcjonowaniu narzuca choroba przewlekła – o przedłużonym czasie trwania, nieuleczalna lub nawracająca. Jedną z ciężkich chorób przewlekłych jest mukowiscydoza. Oprócz czynników stresogennych związanych z czynnościami medycznymi, podejmowanymi w celu leczenia, istotną kwestią jest znaczenie tej choroby dla psychicznej i społecznej sfery rozwoju dziecka. Ocenę stopnia funkcjonowania tych sfer implikuje stopień przystosowania społecznego dziecka. Artykuł podejmuje próbę określenia percepcji przystosowania społecznego chorego dziecka przez jego matkę, nauczycielkę oraz obraz własnego funkcjonowania w szkole dziecka chorego na mukowiscydozę.

EN

Health is a state of complete physical, mental and social well-being. It is not only the absence of illness, but a full human capacity to live in harmony with the environment and to react appropriately to its changes. On the opposite side of the continuum, in line with A. Antonovsky’s salutogenic model, there is illness. In the life of every person, particularly a child, illness is a difficult situation which frequently requires to reorganise and at times also to change the functioning completely. Specific changes in this functioning are imposed by an illness that is chronic – of extended duration, incurable or recurrent. One of the serious chronic illnesses, being at the same time a genetic disease that most frequently occurs in people, is cystic fibrosis. Apart from stressors related to medical acts performed in order to treat the illness, significance of this illness for psychological and social sphere of a child development is also a relevant issue. An evaluation of the level of functioning of these spheres is implied by the level of social adaptation of a child. The article attempts to define the perception of social adaptation of an ill child by his mother and teacher, and a personal view of functioning at school of a child suffering from cystic fibrosis.

Refine search results

Caregiver burden and the role of social support in the care of children with cystic fibrosis

Adults with cystic fibrosis – medical challenges and a psychosocial adaptation in the context of risk of a premature death

Serum and urinary carnitine in children with cystic fibrosis

Electrophoretical mobility of nuclei in buccal cells of cystic fibrosis patients

Ocena realizacji zaleceń dietetycznych wśród dzieci chorych na mukowiscydozę

Hand radiograms as an alternative for bone mass screening in cystic fibrosis

Mukowiscydoza

Identification of factors determining anxiety, depression and aggression, with particular emphasis on pain in Polish adolescents and young adults with cystic fibrosis

Employment after lung transplantation in Poland – a single center study

The disease and an immediate cause of death of Frederic Chopin

Przystosowanie społeczne dziecka z chorobą przewlekłą. Studium przypadku