MUKOWISCYDOZA PDF

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Request PDF on ResearchGate | On Jan 1, , Jarosław Walkowiak and others published Mukowiscydoza - nieuchronne niedożywienie?. Request PDF on ResearchGate | On Jan 1, , Patrycja Krzyżanowska and others published Mukowiscydoza i witaminy rozpuszczalne w tłuszczach. Zaakceptowano do Słowa kluczowe: mukowiscydoza, próba potowa, jonoforeza pilokarpniowa druku/Accepted: ABSTRACT 1 Klinika.


Mukowiscydoza Pdf

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Słowa kluczowe: mukowiscydoza, atypowa postać mukowiscydozy, diagnostyka, CFTR, genetyka .. tions/en/fepipvawoobig.cf 5. Wstęp: Mukowiscydoza (cystic fibrosis, CF) to choroba genetyczna, przewlekła, wpływająca na funkcjonowanie wielu układów i narządów w organizmie. Rola personelu pielęgniarskiego w terapii żywieniowej dziecka z mukowiscydoza. Iwona Czerwińska Pawluk. Radom College in Radom.

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Carbohydrates, the main dietary component, supply energy to the body, but at the same time are the major cariogenic agent. Etiology Although several hundreds of genetic mutations are known, the most common mutation in Western Europe is Delta- F The prevalence of the disease is one in every live new born children [4].

In essence; the disease affects all exocrine glands including the salivary glands and renders their respective secretions more viscous than under healthy conditions. CF patients consequently suffer from gastro-intestinal due to pancreatic insufficiency and respiratory problems.

Because of this, they are compelled to have supplemental pancreas enzymes with every meal, drink and snack, supplemental vitamins A, D, E and K if necessary, frequent antibiotics per os, via erosols and IV and daily expectorantia and mucolytica mostly via aerosols [].

Signs and Symptoms Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium in new born babies [11]. As the child grows, he or she will need to exercise to release mucus in the alveoli. The poor growth and poor weight gain despite a normal food intake in children.

There will be accumulation of thick, sticky mucus leads to frequent chest infections, coughing or short breath. Congenital absence of vas deferens in males leads to infertility [12]. CF causes salty tasting skin and sweat will be very salty, as a result body loses large amounts of salt when they sweat.

Hence it causes dehydration, increased heart rate, tiredness, weakness, decreased blood pressure, heart stroke and death which is very rare. CF can cause clubbing and low bone density lead to osteoporosis, and in rare cases it can present as a coagulation disorder. Young children are sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K passes through placenta, leaving the child with very low reserves.

Other signs and symptoms of CF develops later in life which may include infections that blocks the airways which causes frequent coughing, frequent botus of sinusitis, bronchitis, pneumonia, pneumothorax, Diarrhea, foul smelling, greasy stools, severe constipation pancreatitis, liver disease, diabetes, and Gall stones etc.

Other glands which may be affected are the salivary glands, the sub maxillary gland may be enlarged, the secretion of the sublingual gland is thick and tenacious, and there may be destruction as in the pancreas. The parotid gland, which is not a mucus-secreting gland, may have an increased output with high electrolyte content [14]. Most important effect of the abnormal mucus is in the lungs. Here again there is obstruction by the thick secretion, causing a generalized emphysema which is usually persistent and secondary infection follows, due to Staphylococus aureus.

This chronic pulmonary disease occurs in almost all patients with cystic fibrosis and is often severe and progressive. Osteoclast function, bone turnover and inflammatory cytokines during infective exacerbations of cystic fibrosis.

J Cyst Fibros ; 9: Haworth CS. Impact of cystic fibrosis on bone health. Curr Opin Pulm Med ; Cortet B. Bone loss in adults with cystic fibrosis: Prevalence, associated factors and usefulness of biological markers.

Joint Bone Spine ; Neri AS, Lori I, et al. Bone mineral density in cystic fibrosis patients under the age of 18 years. Minerva Pediatr ; Cystic fibrosis and bone disease: are we missing a genetic link? Eur Respir J ; Reduced bone density in cystic fibrosis: deltaF mutation is an independent risk factor.

LeHeron L, Guillaume C, et al.

Advances in Rehabilitation

J Cyst Fibros ; Matuszewska A. Markery obrotu kostnego. Przegl Reumat ; 3: 5- 7. Hand Radiograms as an alternative for bone mass screening in cystic fibrosis.

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Prog Health Sci ; 3: J Am Acad Nurse Pract ; Zienab S, Guilbault C, et al. J Cyst Fibros ; 7: Papaioannou A, Freitag A, et al.

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Chest ; Effect of oral bisphosphonates on bone mineral density and body composition in adult patients with cystic fibrosis: a pilot study. Bisphosphonates for osteoporosis in people with cystic fibrosis.

The Journal of Academy of Physical Education, Warsaw

Exercise and physical activity in children with cystic fibrosis. Bone mineral status in prepubertal children with cystic fibrosis.

J Pediatr ; Hardin DS, Arumugam R, et al. Normal bone mineral density in cystic fibrosis. Arch Dis Child ; Sood M, Hambleton G, et al. Bone status in cystic fibrosis.

However, the disease is most common among Caucasians of northern European descent and less common among African Americans, especially the pueblo of Zuni. About 12 million Americans are carriers of a faulty cystic fibrosis gene. It is caused by mutations of the gene situated on the long-arm of the seventh 7q31 chromosome coding. Accumulation of the secretion in the outlet ducts caused by a dysfunction or lack of CFTR proteins leads to abnormal activity of exocrine glands, especially in the respiratory and alimentary tracts.

Carbohydrates, the main dietary component, supply energy to the body, but at the same time are the major cariogenic agent.

Etiology Although several hundreds of genetic mutations are known, the most common mutation in Western Europe is Delta- F The prevalence of the disease is one in every live new born children [4]. In essence; the disease affects all exocrine glands including the salivary glands and renders their respective secretions more viscous than under healthy conditions. CF patients consequently suffer from gastro-intestinal due to pancreatic insufficiency and respiratory problems.

Because of this, they are compelled to have supplemental pancreas enzymes with every meal, drink and snack, supplemental vitamins A, D, E and K if necessary, frequent antibiotics per os, via erosols and IV and daily expectorantia and mucolytica mostly via aerosols [].

Signs and Symptoms Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium in new born babies [11]. As the child grows, he or she will need to exercise to release mucus in the alveoli. The poor growth and poor weight gain despite a normal food intake in children. There will be accumulation of thick, sticky mucus leads to frequent chest infections, coughing or short breath.

Congenital absence of vas deferens in males leads to infertility [12]. CF causes salty tasting skin and sweat will be very salty, as a result body loses large amounts of salt when they sweat.

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Hence it causes dehydration, increased heart rate, tiredness, weakness, decreased blood pressure, heart stroke and death which is very rare.

CF can cause clubbing and low bone density lead to osteoporosis, and in rare cases it can present as a coagulation disorder. Young children are sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K passes through placenta, leaving the child with very low reserves. Other signs and symptoms of CF develops later in life which may include infections that blocks the airways which causes frequent coughing, frequent botus of sinusitis, bronchitis, pneumonia, pneumothorax, Diarrhea, foul smelling, greasy stools, severe constipation pancreatitis, liver disease, diabetes, and Gall stones etc.

Other glands which may be affected are the salivary glands, the sub maxillary gland may be enlarged, the secretion of the sublingual gland is thick and tenacious, and there may be destruction as in the pancreas.If a patient is infirm, and his potential for walking and self-service is low, is preferred above the knee amputation, because care of stump is easier.

Effort tests should be performed during hospitalization or shortly after leaving the hospital in patients with myocardial infarction and non-invasive treatment in order to assess ischemia. Med Sci Sports Exerc ; Oppression of the bandage on the soft tissues should cause them to shrinkage. Exercise and physical activity in children with cystic fibrosis. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy.

Clinical aspects and pathophysiology of narcolepsy. The purposes of those procedures are: improvement of peripheral circulation, muscle tension strengthening and reducing contractures, relieving joint and local tissues pain. Along with the main disease, there are frequent additional diseases and complaints which require specially prepared diet.