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Marijke Proesmans

Publications and source records attributed to Marijke Proesmans.

10 recordsLinked to original sources
PubMed2006

Coexistence of (partial) immune defects and risk of recurrent respiratory infections.

BACKGROUND: Respiratory infections are major causes of morbidity and mortality, but determinants of susceptibility are poorly defined. We studied whether and to what extent immunologic and genetic factors are associated with increased susceptibility to respiratory infections. METHODS: We evaluated the prevalence of IgA, IgM, IgG, and IgG subclass deficiencies, impairment in the antibody response against pneumococcal polysaccharides, G2m(n) allotypes, Fc gamma RIIa polymorphisms, partial C2 and partial C4 deficiency, promoter polymorphisms in MBL2, and lymphocyte subset deficiencies in a control population and in consecutive children with recurrent respiratory infections. RESULTS: IgA and/or IgG subclass deficiency was found in 27 of 55 patients (49%) and 6 of 43 controls (14%) (P = 0.0006). An impaired antibody response to polysaccharides was found in 7 patients (19%) and in 0 of 37 controls (P = 0.002). The Gm(n)marker was absent in 25 of 55 patients (45%) and 6 of 42 controls (14%) (P = 0.009). The MBL2 variants O/O, A/O, and A/A occurred in 9, 14, and 32 of the 55 patients, respectively, and in 1, 19, and 23 of the 43 controls, respectively (P = 0.05). There was no increase in the prevalence of partial C4 deficiency, C2 deficiency, lymphocyte subset deficiency, or Fc gamma RIIa polymorphism in the patients compared to the controls. A combination of at least 2 immune defects was found in 31 of 55 patients (56%) and in 4 of 42 controls (11.6%) (P <0.0001). CONCLUSION: Specific antipolysaccharide antibody deficiency, IgA and/or IgG subclass deficiency, Gm(n) allotype, and MBL2 genotype are susceptibility factors for recurrent respiratory infections, and coexistence of several immune defects is the strongest risk factor in this study.

Adolescent↗
PubMed2006

Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients?

Despite absence of clear proof of efficacy, the use of inhaled corticosteroids (ICS) is widespread in cystic fibrosis (CF) patients. Therefore, the effect of ICS on lung function and other clinical variables was studied in 27 prepubertal CF children with mild to moderate lung disease. In a prospective double-blind case-controlled study, fluticasone propionate 500 microg or placebo were administered twice daily during 12 months. The mean (standard error of the mean, SEM) patient age was 8.2 (0.6) years in the placebo group and 9.0 (0.5) years in the fluticasone group. The mean (SEM) forced expiratory volume in 1 s (FEV(1)) was 91% (4%) in the placebo group and 86% (4%) in the fluticasone group. There was no statistically significant difference in the evolution of lung function and the number of respiratory exacerbations between groups. However, longitudinal growth in fluticasone patients was significantly slower than in placebo patients: 3.96 (0.29) cm versus 5.49 (0.38) cm [p<0.005, analysis of variance (ANOVA)] over the 12-month study duration. This resulted in a significant change in height standard deviation score (SDS) of -0.38 (0.09) in the fluticasone group versus -0.01 (0.07) in the placebo group (p<0.003, ANOVA). No catch-up growth was noted 1-2 years after discontinuation of inhaled steroids. The use of high-dose ICS in CF patients with mild lung disease may lead to persistent growth impairment.

Administration, Inhalation↗
PubMed2005

Massive lung collapse with partial resolution after several years: a case report.

BACKGROUND: Bronchitis obliterans is a severe and extremely rare complication of respiratory tract infections in children and is characterized by massive atelectasis and collapse of the affected lung. Of the rare cases reported in the literature all surviving children underwent surgical resection of the collapsed lung. CASE PRESENTATION: We report an infant with bronchitis obliterans that was treated conservatively. 5 years after the initial event, partial lung re-expansion was documented. CONCLUSION: This case therefore supports a conservative treatment whenever possible with pneumonectomy only as a last treatment option.

Bronchiolitis Obliterans↗
PubMed2005

Clericuzio type poikiloderma with neutropenia is distinct from Rothmund-Thomson syndrome.

Two siblings from a consanguineous family presented with a poikiloderma of limbs and face, plantar keratoderma, and toenail pachyonychia. Neutropenia and neutrophil dysfunction with impairment of the respiratory burst and bacterial killing resulted in frequent respiratory tract infections. A bronchocentric granulomatous pneumonia was a fatal complication. The clinical presentation is consistent with Clericuzio type poikiloderma with neutropenia. Literature review identified several additional probable patients. Genetic linkage analysis excluded the locus of the RECQL4 gene, mutations in which have been described in some patients with the Rothmund-Thomson poikiloderma syndrome. This report confirms the clinical and genetic identity of the Clericuzio type of poikiloderma with neutropenia syndrome.

Abnormalities, Multiple↗
PubMed2003

Omeprazole, a proton pump inhibitor, improves residual steatorrhoea in cystic fibrosis patients treated with high dose pancreatic enzymes.

UNLABELLED: Despite treatment with supra-physiological doses of pancreatic enzyme supplements, residual steatorrhoea is a common problem in patients with cystic fibrosis (CF) and pancreatic insufficiency. Strategies to enhance the activity of pancreatic enzymes include decreasing duodenal acidity. The aim of this study was to evaluate the effect of omeprazole (Losec), a proton-pump inhibitor, on fat absorption in CF patients with residual steatorrhoea despite high dose pancreatic enzyme supplements (> or =10,000 U lipase/kg per day). A random cross-over design was chosen. Fat digestion was evaluated with and without omeprazole by means of chemical fat measurements in 3-day stool collections together with 3-day weighed food records for calculation of fat absorption. The results of 15 patients (3 girls and 12 boys) with confirmed steatorrhoea during the control evaluation were analysed. Median age was 8.7 years (range 3.5-15.9 years). Median daily lipase intake was 13,500 U/kg per day (range 10,000-22,000 U/kg per day). During treatment with omeprazole, median faecal fat loss (g fat/day) decreased from 13 g (quartiles 11.5-16.5 g/day) to 5.5 g (quartiles 4.9-8.1 g/day) (P<0.01). The same improvement was noted when fat absorption was calculated: 87% (quartiles 81-89%) without versus 94% (quartiles 90-96%) with omeprazole (P<0.001). CONCLUSION: Omeprazole improves fat digestion and absorption in cystic fibrosis patients with residual faecal fat loss despite maximal pancreatic enzyme substitution.

Adolescent↗
PubMed2003

Tidal off-line exhaled nitric oxide measurements in a pre-school population.

UNLABELLED: Exhaled nitric oxide (ENO) is used as a non-invasive marker of airway inflammation. The aim of this study was to measure ENO in a pre-school population using a relatively novel method, the off-line tidal breathing method, and to investigate differences in ENO between subjects with different presentations of wheezing. ENO was measured in 129 children (median age 4.4 years, quartiles 4.0-4.8 years) through a mouth mask attached to a two-way valve with an expiratory resistance of 5 cm H(2)0. Mean tidal ENO concentration (tENO) was calculated from triplicate measurements. Mean +/- SEM tENO for 89 control subjects was 13+/-0.4 ppb (95%CI 11.8-13.7 ppb); this level was significantly different from tENO in 15 children with a history of recurrent wheezing (18.6+/-1.9 ppb; 95%CI 14.5-22.7 ppb; t-test P<0.0001). Mean tENO in 16 children with a single wheezing episode was 11.4+/-1.0 ppb (95%CI 9.2-13.6 ppb) and thus significantly different from the recurrent wheezing group (t-test P=0.0024). CONCLUSION: The off-line tidal breathing method is a feasible and appealing method for measuring exhaled nitric oxide in pre-school children. With this method, higher tidal exhaled nitric oxide levels were found in children with recurrent wheezing.

Breath Tests↗
PubMed2003

Exhaled nitric oxide corresponds with office evaluation of asthma control.

Exhaled NO (ENO) has been studied as a noninvasive marker of airway inflammation, and has been shown to be elevated in asthma patients. The aim of this study was to investigate whether ENO measurements differ significantly between groups of asthmatic children with different disease control and to compare ENO measurements with the clinical assessment of asthma control. Seventy-three children between 5-18 years old with a diagnosis of asthma were recruited. ENO was measured online during a slow vital capacity maneuver. The mean of three plateau NO levels was used for analysis. Baseline and postbronchodilator spirometry were performed. The assessment of disease control was based on the frequency of use of beta2-agonists, occurrence of day- and nighttime asthma symptoms, and spirometry results. Twenty-one children (group 1) had good asthma control. In 31 patients (group 2), asthma control was acceptable. In 21 patients (group 3), asthma was insufficiently controlled. ENO levels were (median (quartiles)): group 1, 11 ppb (9-21); group 2, 15 ppb (11-26); and group 3, 28 ppb (19-33). Measurements were significantly different between all three groups (P = 0.009, Kruskal-Wallis), between groups 1 and 3 (P = 0.01, Mann-Whitney U test), and between groups 2 and 3 (P = 0.01, Mann-Whitney-U test). The same was true for reversibility testing. We found significantly different ENO levels between a group of pediatric asthma patients with insufficient and good/sufficient control, as defined by clinical assessment. These results suggest that ENO measurements may be useful for monitoring asthma patients.

Albuterol↗
PubMed2003

Unusual and severe disease course in a child with ataxia-telangiectasia.

Ataxia-telangiectasia (AT) is an autosomal recessive syndrome of combined immunodeficiency. Hallmarks of the disease comprise progressive cerebellar ataxia, oculocutaneous telangiectasia, cancer susceptibility and variable humoral and cellular immunodeficiency. We describe a patient with AT presenting with autoimmune haemolytic anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy and hyper-IgM at the age of 6 months. At the age of 26 months she developed persistent fever, progressive lymphadenopathy and pulmonary nodular infiltrates, which were responsive to steroid therapy.

Abnormalities, Multiple↗