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Biomedical subjects

A Munck

Publications and source records attributed to A Munck.

At least 19 recordsLinked to original sources
PubMed2004

Follow-up of 452 totally implantable vascular devices in cystic fibrosis patients.

The use and complications of totally implantable vascular access devices (TIVADs) were examined during multiple courses of antibiotics in cystic fibrosis (CF) patients. This retrospective study involved 36 CF centres. Risk factors for removal and septicaemia were sought by survival analysis of censored data. Multivariate Cox models were constructed with removal or septicaemia as the event and the characteristics of TIVADs as explanatory variables. TIVADs (n = 452) were implanted in 315 patients. The mean functional time per device was 32 +/- 25 months. Long-term complications occurred with 188 devices (42%); they consisted mainly of occlusion (21%, requiring removal in 77%), infection (9.3%, requiring removal in 851%; septicaemia in 7.3%; rate 0.3 per 1,000 days, Candida in 66%), and vascular thrombosis (4.7%, removal in 58%). Multivariate survival analysis showed that removal, whatever the reason, was associated with polyurethane (versus silicone) and routine use of the device for blood sampling (versus never). No risk factors, including heparin lock, were identified for septicaemia or for removal for obstruction. Totally implantable venous access devices appear to be safe and reliable for long-term intermittent venous access. Although retrospective, this study suggests that the characteristics of the material and blood sampling are risk factors for removal.

Anti-Bacterial Agents↗
PubMed2003

[Digestive symptoms in cystic fibrosis].

The prognosis of cystic fibrosis (CF) is strongly linked to the pulmonary status, nevertheless digestive disorders should be taken into account in order to prevent malnutrition which may increase pulmonary surinfections and speed up the deterioration of the respiratory function. Digestion, gut motility and absorption are the three major functions of the gastrointestinal tract. Functional disorders may involve the whole gut; in addition to pancreatic insufficiency we describe the failure of chloride secretion with dehydration of bowel content. Insufficient knowledge of the pathophysiology stresses the need for further studies to allow development of more rational and effective therapy.

Cystic Fibrosis↗
PubMed2003

Patient delay from onset of chest pain suggesting acute coronary syndrome to hospital admission.

OBJECTIVE: The aim of the study was to examine patient delay (time from onset of chest pain to patient seeking medical care) among patients who were admitted to hospital with suspected acute coronary syndrome (ACS). DESIGN AND RESULTS: For 337 patients acutely admitted to the Cardiology Department, Odense University Hospital, during a 3-month period in 1998 with suspected ACS, patient delay and the total pre-hospital delay were registered. In addition, information on patient characteristics, patient behaviour and symptom perception was obtained. The median patient delay was 2.85 h (range 0.2-91 h), of this the "silent" patient delay represented 1 h (range 1 min-11.3 h). The total pre-hospital delay was median 3.88 h. Thirty-one per cent of the patients had confirmed acute myocardial infarction (AMI), and this patient group had a significantly shorter patient delay compared with the group without AMI, 2.05 h vs 3.12 h, p = 0.01. Patient delay of more than 2 h was associated with the factors "self-medication" and "wanted to wait and see if the symptoms went away". A smaller than average risk of patient delay was found in the case of "suspicion of heart attack" and "suspicion of a serious condition". CONCLUSION: Patient delay is considered to be a serious impediment to markedly improving the prognosis in the case of ACS.

Acute Disease↗
PubMed2003

The development of quality circles/peer review groups as a method of quality improvement in Europe. Results of a survey in 26 European countries.

BACKGROUND: Peer review groups (PRGs) and quality circles (QCs) commenced in The Netherlands and have grown to become an important method of quality improvement in primary care in several other European countries. OBJECTIVE: Our aim was to provide an overview of QC/PRG activities and exemplary programmes in European countries. METHODS: A survey was performed in three consecutive steps by EQuiP (European Working Party on Quality in Family Practice), which is a representative association of experts from 26 European countries. The national representatives initially completed a structured questionnaire documenting the number and objectives of QCs/PRGs, sources of support and special programmes in their countries (step 1). In step 2, these sources were used to extend and validate the expert statements. Step 3 studied paradigmatic initiatives in depth. RESULTS: Step 1 took place in 2000; the response rate was 100% (26 countries). QCs/PRGs were very active in 10 countries; 16 countries showed little or no activity. Participation ranged from <2 to 86% of all GPs. Step 2 concentrated upon the countries with a high level of activity. Development appeared to be associated with establishment in private practice and the portion of GPs with vocational training. Eight programmes from six countries describing the establishment and the targeting of QC/PRG work are presented as case reports (step 3). CONCLUSION: In the last 10 years, substantial development of QCs/PRGs has taken place in The Netherlands, the UK, Denmark, Belgium, Ireland, Sweden, Norway, Germany, Switzerland and Austria. Further evaluation is needed to clarify the impact on quality of care.

Europe↗
PubMed2003

Population pharmacokinetics of ciprofloxacin in pediatric and adolescent patients with acute infections.

The aim of the present study was to characterize the population pharmacokinetics of ciprofloxacin in patients with and without cystic fibrosis ranging in age from 1 day to 24 years and to propose a limited sampling strategy to estimate individual pharmacokinetic parameters. Patients were divided into four groups according to the treatment schedule. They received ciprofloxacin by intravenous infusion (30 min) or by the oral route. The number of samples collected from each patient ranged from 1 to 12. The population parameters were computed for an initial group of 37 patients. The data were analyzed by nonlinear mixed-effect modeling by use of a two-compartment structural model. The interindividual variability in clearance (CL) was partially explained by a dependence on age and the patient's clinical status. In addition, a significant relationship was found between weight and the initial volume of distribution. Eighteen additional patients were used for model validation and evaluation of limited sampling strategies. When ciprofloxacin was administered intravenously, sampling at a single point (12 h after the start of infusion) allowed the precise and accurate estimation of CL and the elimination half-life, as well as the ciprofloxacin concentration at the end of the infusion. It should be noted that to take into account the presence of a lag time after oral administration, a schedule based on two sampling times of 1 and 12 h is needed. The results of this study combine relationships between ciprofloxacin pharmacokinetic parameters and patient covariates that may be useful for dose adjustment and a convenient sampling procedure that can be used for further studies.

Acute Disease↗
PubMed2002

[Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports].

UNLABELLED: Primary intestinal lymphangiectasia induce symptoms of protein-losing gastroenteropathy. Only very few studies evaluate the long term follow up of such patients. We reviewed six children diagnosed at 17 +/- 12 months and followed for 11 +/- 4.9 years. CASE REPORTS: As soon as the diagnosis was made the patients were submitted to a strict low fat diet with added medium chain triglycerides and intermittent liposoluble vitamins perfusions. The diet allowed the disappearance of symptoms for all the patients but laboratory findings indicated continuing chyle leak for most of the children. Only one child who had normal biological parameters tolerates a normal diet since four years. Relaxation of the diet by two patients who had moderate hypoalbuminemia and lymphopenia led to severe clinical relapses 14 and 17 years after the diagnosis period with therapeutic difficulties. Three patients with long term strict low fat diet remain asymptomatic. CONCLUSION: In most asymptomatic patients, the underlying lymphatic defect remains with permanent biological abnormalities. Clinical relapses may be severe and difficult to treat; thus the need for dietary treatment appears to be permanent.

Adolescent↗
PubMed2002

[Pancreatic involvement in inflammatory bowel diseases in children].

UNLABELLED: Pancreatitis in inflammatory bowel disease (IBD) in children is anecdotal. In adults, symptomatic pancreatitis occurs in 2% and asymptomatic in 8 to 21%. PATIENTS AND METHODS: The aim of our study was to review retrospectively the frequency of pancreatitis in 124 pediatric patients (54.8% boys, 45.2% girls; 97 with Crohn disease, 16 with ulcerative colitis and 11 with undetermined colitis). Diagnostic criterion of pancreatitis was an increase of amylasemia > or = +2 SD of the normal with or without evocative clinical symptoms. RESULTS: Symptomatic or asymptomatic pancreatitis was found in 27% (respectively 14.5 and 12.5%). Pancreatitis was significantly more frequent in girls as compared to boys (P = 0.04). Symptomatic pancreatitis was moderate and non complicated, often recurrent. It occurred mainly during active and severe diseases (P = 0.006). The localizations of IBD were not discriminant. Strong relation with drug was found in 25% of pancreatitis mainly due to azathioprine or 5-aminosalicylic acid, and salazopyrin. Duodenal localisation of Crohn disease or hepatobiliary complications were found associated with pancreatitis in 18% and 15% respectively. CONCLUSION: These data suggest the high incidence of symptomatic and asymptomatic pancreatitis in children with IBD, the importance of its regular monitoring but also its multifactorial causes. Precise diagnosis of pancreatitis in pediatric IBD has to be done in order to avoid inappropriate drug pancreatitis diagnosis.

Adolescent↗
PubMed2001

Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization.

Chronic infection by Pseudomonas aeruginosa (PA) in patients with cystic fibrosis (CF) is preceded by a period of colonization and acute infection. Early aggressive antibiotic treatment of initial colonisation may prevent or at least delay chronic pulmonary infection. We initiated treatment with a combination of IV beta-lactam tobramycin, followed by nebulized colistin when PA was first isolated from patients with CF. Subsequent serial PA isolates obtained from these colonized CF patients were characterized by means of molecular methods to determine whether they were genetically related to the initial strain. Initial colonization was eradicated in all 19 patients. All patients reacquired PA within 3-25 months during the 3 years of follow-up. Fourteen patients acquired a new PA strain with a distinct genotypic profile, suggesting a new source of contamination. Five patients had two PA isolates with identical genotypes, suggesting either previous undetected respiratory tract colonization or a persistent environmental source of contamination.

Administration, Inhalation↗
PubMed2001

Apolipoprotein B48 glycosylation in abetalipoproteinemia and Anderson's disease.

BACKGROUND & AIMS: Abetalipoproteinemia and Anderson's disease are hereditary lipid malabsorption syndromes. In abetalipoproteinemia, lipoprotein assembly is defective because of mutations in the microsomal triglyceride transfer protein. Here, we evaluated the intracellular transport of apolipoprotein B48 to localize the defect in Anderson's disease. METHODS: Asparagine-linked oligosaccharide processing of apolipoprotein B48 in normal and affected individuals was determined by the endoglycosidase H and F sensitivities of the protein after metabolic labeling of intestinal explants in organ culture. Cell ultrastructure was evaluated with electron microscopy. RESULTS: In Anderson's disease as in normal individuals, there was a time-dependent transformation of high mannose endoglycosidase H-sensitive oligosaccharides, of endoplasmic reticulum origin, to complex endoglycosidase H-resistant oligosaccharides, added in the Golgi network. In contrast, despite the translocation of apolipoprotein B48 into the endoplasmic reticulum in patients with abetalipoproteinemia and in biopsies treated with Brefeldin A, which blocks anterograde transport between the endoplasmic reticulum and the Golgi network, there was no transformation of endoglycosidase H-sensitive oligosaccharides. CONCLUSIONS: In abetalipoproteinemia and Anderson's disease, apolipoprotein B48 is completely translocated into the endoplasmic reticulum, but only in Anderson's disease is the protein transported to the Golgi apparatus. This suggests that Anderson's disease is caused by a post-Golgi cargo-specific secretion defect.

Abetalipoproteinemia↗
PubMed2001

Cystic fibrosis-related diabetes mellitus: clinical impact of prediabetes and effects of insulin therapy.

UNLABELLED: In patients with cystic fibrosis (CF), glucose intolerance preceding diabetes (prediabetes) may have adverse effects on nutritional status and respiratory function, which are reversible after the start of insulin therapy. Respiratory function (forced vital capacity and forced expiratory volume in one second) and body mass index (BMI) were compared retrospectively in a French cohort of 14 patients during the 5 y preceding insulin therapy for diabetes and in 14 age- and sex-matched controls with normal oral glucose tolerance tests. In the diabetic group, all three parameters deviated increasingly from the values in the controls; the differences became statistically different during the 6 mo before insulin therapy. The effect was more important in patients for whom diabetes mellitus was diagnosed on the basis of symptoms of hyperglycaemia than in patients for whom it was diagnosed by systematic screening, but still present in the latter. After insulin was started, respiratory function improved and the BMI returned to normal within 1 y. The annual insulin requirement increased from 0.62 during the first year to 1.25 during the fifth year. Glycosylated haemoglobin (HbAIc) values ranged from 6.6 to 7.8%. Only 2 episodes of severe hypoglycaemia were recorded over 42 patient-years of follow-up. The insulin regimen most often used was two daily injections of a mixture of short- and intermediate-acting insulin (n = 10) given with an insulin pen. CONCLUSION: The clinical status of CF patients who will need insulin therapy deteriorates before the start of insulin. In patients with CF-related diabetes, with or without fasting hyperglycaemia, insulin therapy improves anabolism and provides good glycaemic control with few severe hypoglycaemic episodes.

Adolescent↗
PubMed2000

[Nutritional management of cystic fibrosis in children].

A major goal in the management of cystic fibrosis patients is to maintain a good nutritional status as it improves long-term survival. A link is clearly established between the degree of malnutrition and the severity of the disease. Clinical and biological follow-up, better knowledge of energy requirements, dietary counseling and nutritional intervention help to optimize the growth of these patients through childhood and adolescence.

Adolescent↗
PubMed2000

[Digestive and nutritional management of adults with cystic fibrosis].

Digestive disturbances are frequent in adults with cystic fibrosis. They can lead to malnutrition which in turn is deleterious to the prognosis. We summarise the information on epidemiology, pathogenicity, signs, diagnostic criteria and treatments of exocrine pancreatic insufficiency, gastro-oesophageal reflux and denutrition (which are all frequent) but also of constipation, rectal prolapse, distal intestinal obstruction syndrome, and liver diseases. The main recommendations are the following: 1--know how to treat pancreatic insufficiency with enzyme replacement and vitamins; 2--know how to treat aggressively any gastro-esophageal reflux; 3--diagnose and treat denutrition as early as possible; 4--know the distal intestinal obstruction syndrome to avoid abusive and dangerous surgery; 5--know that the most severe hepatic diseases can be treated by liver transplantation.

Adult↗
PubMed1999

Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension.

BACKGROUND/AIM: Variceal bleeding is the most severe complication in patients with cystic fibrosis-associated liver cirrhosis, who often do not have severe respiratory failure. The advent of liver transplantation has broadened the treatment options. The purpose of this study was to report our experience with the management of portal hypertension. METHODS: Clinical and biochemical features, outcome of liver disease and management of portal hypertension were analyzed retrospectively in 44 children with cystic fibrosis-associated liver cirrhosis. RESULTS: The mean age at diagnosis of liver cirrhosis was 9 years. Eighty-six per cent of the children developed esophageal varices, 50% of whom bled early in their second decade. Injection sclerotherapy of esophageal varices did not prevent recurrence of bleeding in five of seven children. Elective surgical portosystemic shunting was successfully performed in nine of 11 patients considered being at high risk of bleeding or with recurrent bleeding episodes but without severe pulmonary failure and liver dysfunction, allowing prolonged post-operative survival up to 15 years. Two of three children who underwent isolated liver transplantation for severe portal hypertension died post-operatively. CONCLUSIONS: Management emphasis in cystic fibrosis patients with liver cirrhosis should be on control of bleeding and variceal decompression. These results suggest that surgical portosystemic shunting may be considered to relieve portal hypertension in patients without progressive liver failure and severe lung disease as an alternative to liver transplantation. With this policy, patients may be stabilized for many years until progression of liver or lung diseases indicates liver or lung-liver transplantation.

Adolescent↗