cystic fibrosis/мачнина

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The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report.

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Cystic fibrosis (CF) is a genetic disorder of the epithelial CFTR apical chloride channel resulting in multi-organ manifestations, including pancreatic exocrine secretion. In the pancreas, CFTR abnormality results in abnormally viscous secretions that obstruct proximal ducts leading to

Distal intestinal obstruction syndrome in cystic fibrosis: presentation, outcome and management in a tertiary hospital (2007-2012).

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BACKGROUND Cystic fibrosis (CF) can result in distal intestinal obstruction syndrome (DIOS) due to inspissated mucus. This paper describes the clinicopathological characteristics of adult CF patients with DIOS and assesses risk factors for surgery. METHODS A retrospective audit of CF patients at the

Effects of cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome.

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In a double-blind, placebo-controlled, crossover trial, we investigated the effects of the prokinetic drug cisapride in patients with cystic fibrosis and chronic recurrent distal intestinal obstruction syndrome (DIOS). After a baseline period, 17 patients (12.9 to 34.9 years; 12 boys) received, in

[Fatty replacement of the pancreas in a patient with asymptomatic pulmonary cystic fibrosis].

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The authors report the case of a 26-year-old woman with hepatomegaly, who had recurrent and progressive nausea and abdominal pain. Computed tomography and magnetic resonance imaging demonstrated fatty replacement of the entire pancreas resulting from cystic fibrosis. The pulmonary disease was

Clinical evaluation of meropenem versus ceftazidime for the treatment of Pseudomonas spp. infections in cystic fibrosis patients.

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Cystic fibrosis patients (children and young adults) with Pseudomonas spp. chest infections were treated with meropenem or ceftazidime. This study was the first to investigate the use of meropenem in cystic fibrosis. Meropenem was well tolerated with only transient elevations of serum transaminases.

Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis.

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The efficacy, adverse reactions, and long-term effects of intestinal lavage treatment with a balanced electrolyte solution (Golytely) was evaluated in patients with cystic fibrosis and distal intestinal obstruction syndrome. Twenty-two patients with cystic fibrosis (mean age 21.8 years, range 14 to

How good is end of life care for patients dying with cystic fibrosis?

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Background: Cystic Fibrosis (CF) is a life-limiting illness. Audit of the care of patients dying of CF has not been published to date. Methods: Newcastle and Oxford teams

The use of macrolide antibiotics in patients with cystic fibrosis.

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OBJECTIVE There has been much recent interest in the use of macrolide antibiotics as chronic suppressive therapy in patients with cystic fibrosis. Three recent randomized, placebo-controlled trials have been conducted. RESULTS All three trials used similar regimens of azithromycin, and lung function

Experience with ceftazidime in cystic fibrosis.

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Twelve adults and children with cystic fibrosis received a total of 17 courses of ceftazidime for the treatment of an acute exacerbation of respiratory tract infection. In 6 cases ceftazidime was given as the sole antibiotic. All patients who were clinically assessable were considered to be cured of

Macrolide antibiotics for cystic fibrosis.

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BACKGROUND Chronic severe infection with Pseudomonas aeruginosa, affects many people with cystic fibrosis (CF). There is evidence from the laboratory and from other disease processes that macrolide antibiotics, whilst not directly active against Pseudomonas aeruginosa, may have indirect actions

Successful outcome following pneumonectomy in a teenage boy with cystic fibrosis: a case report.

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Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as

Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis.

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Cystic fibrosis (CF) is an inherited disease of the secretory glands caused by mutations of the cystic fibrosis transmembrane regulator (CFTR) gene. The clinical manifestations of CF are repetitive lung infections, biliary cirrhosis, pancreatic abnormalities, and gastrointestinal disorders. We

The prevalence of nausea and vomiting in pediatric patients receiving home parenteral nutrition.

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We have observed that many home parenteral nutrition (HPN) recipients experience nausea, vomiting, or both during cyclic parenteral nutrition infusions. The current investigation was performed to determine the prevalence and course of these symptoms and effectiveness of therapeutic maneuvers.

Eradication of chronic methicillin-resistant Staphylococcus aureus infection in cystic fibrosis patients. An observational prospective cohort study of 11 patients.

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BACKGROUND Chronic airway infection with methicillin-resistant Staphylococcus aureus (MRSA) in patients with cystic fibrosis (CF) is an increasing clinical problem, and therapeutic options are limited. Because chronic infection with MRSA can be associated with accelerated decline in lung function,

Recreational use of psychoactive drugs by patients with cystic fibrosis.

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We assessed unprescribed psychoactive drug use in 173 adults with cystic fibrosis. Twenty (11%) regularly smoked tobacco. Cigarette smoking ranged from 1 to 30 years (2 to 60 pack-years). Alcohol was used by 60%, and marijuana by 20% of the patients. Pulmonary symptoms were often increased the day

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