Influence of lung transplantation on the essential fatty acid profile in cystic fibrosis.

Lung transplantation is assumed to normalize essential fatty acid (EFA) profile in the plasma, described as abnormal in patients with cystic fibrosis (CF). This study sought to evaluate the EFA profile in both the plasma and erythrocyte membrane according to lung status by comparing CF patients with or without a lung transplant. A total of 50 homozygous F508del patients (33 CF patients [CF group] and 17 CF patients with a lung transplant [TX CF group]) were included. In comparison with the CF group, in the plasma, the levels of total n-3, α-linolenic, eicosapentaenoic, and docosahexaenoic acids were higher and the n-6/n-3 ratio was lower in the TX CF group. Yet, these differences were not observed in the erythrocyte membrane. This study supports that lung transplantation improves the EFA profile in the plasma but not in the erythrocyte membrane by means of the different mechanisms suggested in this article.

[An atypical case of systemic juvenile idiopathic arthritis]. / Un cas atypique d'arthrite juvénile idiopathique systémique.

The systemic juvenile idiopathic arthritis (SJIA) is a rare, auto-inflammatory and chronic childhood disease. Arthritis of at least one joint is associated with a daily fever lasting more than two weeks and with one of the following signs: lymphadenopathy, hepatomegaly, serositis, or skin rash. Systemic symptoms are often initially preponderant, in the absence of arthritis than can occur weeks or months later. The typical rash is maculopapular discrete, fleeting and dew. We present an unusual SJIA case, where the rash, like urticaria, presents itself as persistent, itchy hives. An arthritis of the elbow appeared only one month after the onset of the disease. This case illustrates the difficulty of diagnosis, which can only be made after the exclusion of more common (infectious) and severe (hematooncology) diseases.

L'arthrite juvénile idiopathique systémique (AJIS) est une maladie rare, auto-inflammatoire et chronique de l'enfant. Le tableau associe la présence d'une arthrite touchant au moins une articulation, à une fièvre quotidienne d'une durée minimum de 2 semaines et à l'un des signes suivants : adénopathies, hépatomégalie, sérosite ou éruption cutanée. Les symptômes systémiques sont souvent prépondérants initialement, en l'absence d'arthrite avérée qui peut n'apparaître qu'après plusieurs semaines ou mois. L'éruption typique est maculo-papuleuse rosée discrète et fugace. Nous présentons un cas atypique d'AJIS, dont l'éruption se présente comme une urticaire persistante et prurigineuse. Une arthrite du coude n'est apparue qu'un mois après le début de l'affection. Ce cas illustre la difficulté du diagnostic qui ne peut être posé qu'après l'exclusion de pathologies plus courantes (infectieuses) et plus graves (hémato-oncologiques).

Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance.

BACKGROUND: Body composition (BC) analysis based on bioelectrical impedance analysis (BIA) provides conflicting results. The purpose of the study was to validate an equation specific for young patients with cystic fibrosis (CF), describe their BC and investigate its association with lung function. METHODS: Fifty-four young CF patients were evaluated by BIA and dual X-ray absorptiometry (DXA). An empirically derived CF-specific equation for fat-free mass (FFM) estimation by BIA was elaborated after stepwise multivariate regression and the agreement between BIA and DXA was assessed by Bland-Altman plots. The association between BC and lung function was investigated by regression analysis. RESULTS: The mean difference between the BIA and DXA assessment was close to zero. A total of 22.5% of patients (n=9) presented a FFM z-score≤-2. They had a worse pulmonary function and diaphragmatic impairment. Among these 9 patients, 7 had a normal BMI z-score>-1. CONCLUSIONS: BIA, based on a CF-specific equation, is a reliable method for BC assessment and allows the identification of patients at risk of nutritional degradation and bad respiratory prognosis.

The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients - A pilot study.

Effectiveness of omega-3 supplementation in cystic fibrosis (CF) remains controversial. This study sought to evaluate clinical status, exercise tolerance, inflammatory parameters, and erythrocyte fatty acid profile after 1 year of oral omega-3 supplementation in CF patients. Fifteen ΔF508-homozygous patients undergoing chronic azithromycin were randomized to receive omega-3 fish oil supplementation at a dose of 60mg/Kg/day or placebo. In comparison with the previous year, in the supplemented group, the number of pulmonary exacerbations decreased at 12 months (1.7 vs. 3.0, p<0.01), as did the duration of antibiotic therapy (26.5 days vs. 60.0 days, p<0.025). Supplementation significantly increased the levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) as early as <3 months of administration, with concomitant decreases in arachidonic acid (AA) levels. This pilot study suggests that long-term omega-3 supplementation offers several clinical benefits as to the number of exacerbations and duration of antibiotic therapy in CF patients.

[Treatment of main chronic diseases in childhood from birth]. / Prise en charge des principales pathologies chroniques de l'enfant dès la naissance.

Children suffering from chronic diseases are very quickly diagnosed by neonatal screening and follow-up of the mother during the pregnancy. Early screening and diagnosis are essential to obtain continuous improvement of the prognosis in term of treatment and psychosocial outcome. Multidisciplinary teams are now well organized to treat all the complications of the disease. Registers at national and international levels allow professionals to compare themselves and to evaluate the improvement of clinical status and mid-life expectancy.

[Foreign body aspiration in Kigali University Teaching Hospital, Rwanda]. / Inhalation d'un corps étranger bronchique au Centre Hospitalier Universitaire de Kigali, Rwanda.

We present the case of a 12-year-old girl referred to Kigali University Teaching Hospital (KUTH) for persistent cough, fever and haemoptysis. Respiratory symptoms started acutely with a stridor at age 4. Thereafter she developed a chronic cough with intermittent fever. She was treated ambulatory in the health care centre with oral antibiotics and finally referred to the district hospital at age 7. The chest X-ray then suggested tuberculosis for which a 6 month treatment was given with no improvement. The cough persisted and haemoptysis appeared so the patient was referred to the reference hospital (KUTH). Chest X-ray showed diffuse lesions of the left lung with bronchiectasis. Bronchoscopy revealed the presence of a foreign body in the left intermediary bronchus and a piece of plastic was extracted. Symptoms rapidly disappeared with antibiotic treatment. This case illustrates how important it is to include foreign body inhalation in the differential diagnosis of respiratory disease in children. Bronchoscopy plays a key role in diagnosis and treatment. The authors point out the advantages of the joint efforts of the Belgian Development Aid Agency (BTC) and the Université libre de Bruxelles (ULB) in the development of this activity in the Rwandese context.

[Food allergies in children: which diet?]. / Les allergies alimentaires de l'enfant: quel régime?

Food allergies are very frequent in children (between 4 and 8% of population). There are many clinical manifestations, that can be lifethreatening. In children, compared to adults, a limited number of food allergens are responsible for the disease: egg, cow milk, peanuts, nuts (hazelnut, nut, ...), fish, cereals, exotic fuits, and soya. Eviction of the offending food is the first treatment of allergy. This eviction diet is sometimes difficult to organize and can alter the quality of life (child and family). Diagnosis must be well established; sensitivity to an allergen must be differenciated from real allergy. This can lead to perform a provocation test (oral challenge) in the hospital. It is now proposed that the eviction diet will be less strict than before, adapted according to the allergen, symptoms in each case, age of patient and ideally to the reacted dose of the offending allergen. A collaboration with a dietist is necessary to optimalize the nutritionnal schedule. Induction of oral tolerance seems to be an interesting optional treatment for patients presenting persistant food allergy.

Clinical aspects of chronic ENT inflammation in children.

In children, all ENT cavities are particularly prone to the development of chronic inflammation. This is due to many predisposing factors, of which the most common are unfavourable anatomy, absence of nasal blowing, day care attendance, allergy, immature immunity, gastro-oesophageal reflux and tobacco smoke exposure. The aim of this paper is to outline the most specific paediatric clinical aspects of chronic pharyngo-tonsillitis, rhinosinusitis, otitis media, adenoiditis and laryngotracheitis and the important influence that some of these pathologies exert on the others.

Perceived insomnia, anxiety, and depression among older Russian immigrants.

There are few data on the relationships of anxiety and depression to insomnia among immigrants in the United States. Observations of high rates of symptoms of these associated conditions among older Russians indicate the need to focus clinical attention on this population. Relationships of self-reported depression and anxiety to insomnia complaints were investigated in a community-based sample of older Russian immigrants. Volunteers (N=307) were urban community-residing Russians (ages 50 to 95 years; 54% women). Surveys were conducted in a semistructured environment by bilingual educators in various community centers. 93% reported a major health problem, 83% experienced pain, and 62% had problems engaging in daily activities. Of the sample, 61% reported an insomnia complaint, and 43% considered depression and/or anxiety to be a major impairment. Logistic regression analysis showed that insomnia was the most important predictor of perceived anxiety and depression; the corresponding multivariate-adjusted odds ratio was 4.37. Insomnia complaints and perceptions of depression and anxiety among older Russians may have a synergistic effect. Both patients and primary-care physicians should be better educated regarding the recognition of barriers limiting access to adequate health care among older Russians.

[Acute respiratory dyspnea in children]. / La dyspnée aiguë chez l'enfant.

Acute respiratory dyspnea is very frequent in children and must be quickly treated to obtain the best prognosis. The diagnosis depends from the natural history of the disease and from the quality of clinical assessment. The use of an algorithm according to the presence of stridor or bronchospasm is very contributive to the diagnosis. The paper reviews the pathophysiology of dyspnea in children and the more common diseases that are causing respiratory distress. Finally, treatment of respiratory failure and management of specific diseases are defined.

Basophil activation tests for the diagnosis of food allergy in children.

BACKGROUND: Positive skin prick tests (SPT) for food allergens and specific IgE (sIgE) in serum indicate sensitization but do not enable distinction between sensitized but tolerant and clinically allergic patients. OBJECTIVE: Herein, we evaluate the clinical relevance of basophil activation tests (BATs) for peanut or egg allergy diagnosis. METHODS: Thirty-two peanut-allergic, 14 peanut-sensitized (sIgE(+) and/or SPT(+) to peanuts) but tolerant children and 29 controls with no history of an adverse reaction to peanuts were included. Similarly, 31 egg-allergic, 14 egg-sensitized children (sIgE(+) and/or SPT(+) to egg white) and 22 controls were studied. Flow cytometric analysis of CD63 expression or CD203c upregulation on basophils and the production of leukotrienes (LT) were performed in response to an in vitro crude peanut extract or ovalbumin (OVA) challenge. RESULTS: After in vitro peanut challenge, the basophils from peanut-allergic children showed significantly higher levels of activation than those from controls (P<0.001). After OVA challenge, a similar distinction (P<0.001) was observed between egg-allergics and controls. Interestingly, the majority of egg- or peanut-sensitized children failed to activate basophils, respectively, in response to OVA and peanut challenge. The sensitivity of the CD63, CD203c and LT assay was 86.7%, 89.5% and 76.0% with a specificity of 94.1%, 97.1% and 94.6% for peanut allergy diagnosis. The corresponding performances of BATs applied to egg allergy diagnosis were 88.9%, 62.5% and 77.8% for the sensitivity and 100%, 96.4% and 96.4% for the specificity. CONCLUSION: Neither conventional tests nor BATs are sensitive and specific enough to predict food allergy accurately. However, BATs may helpfully complete conventional tests, especially SPT, allowing improved discrimination between allergic and non-allergic individuals.

Early referral to cystic fibrosis specialist centre impacts on respiratory outcome.

BACKGROUND: Published studies concerning the impact of specialist care on lung disease in cystic fibrosis remain limited and most are either biased due to comparison with historical controls and/or underpowered. METHODS: In this retrospective multicentric study, data from all CF children fulfilling the following criteria were collected: 1) Age 6-<18 at the end of 2003; 2) diagnosis before 8 y; 3) follow-up in an accredited CF Belgian centre; 4) at least 1 spirometry and respiratory culture available for 2003. Group A included children referred > or =2 years after the diagnosis. Patients from Group A were then matched with a single early referred patient on the basis of 2 criteria: same centre, as closest age as possible (Group B). RESULTS: Data from 217 children were collected (Group A: 67/217). Late referred patients had a lower FEV(1) (77.2%+/-22.4 vs 86.7% pred.+/-19.4, p=0.01) and a higher prevalence of Pseudomonas aeruginosa (38.6 vs 17.5%, p<0.05). CONCLUSION: In this population of CF children, a delay of 6.1 y (vs 0.1 y) between diagnosis and referral to a specialist clinic resulted in poorer respiratory outcome at age 13.

[Immunoallergology in children: diagnosis and treatment]. / Immuno-allergologie chez l'enfant: diagnostic et traitement.

Atopy is the most frequent allergic disease in western countries: about 30% of children are suffering from various forms of its manifestations. During the 20th century, its frequency gradually increased. This is not only true for sensitization (presence of specific immunoglobulin E (IgE) in the skin or in the serum), but also for clinical symptoms associated with sensitization. It is usually a disease of skin and mucosae, but atopy can also become systemic (anaphylaxis). Hygienist hypothesis (reduction of infectious diseases and Th1 pressure) gives a possible explanation to the impressive increase of allergic diseases during the last decades. In the child, atopic dermatitis and food allergies are first observed (allergic march): 5 allergens explain more than 85% of cases (white egg, milk, peanuts, fish and nuts). Skin prick tests are more sensible than specific IgE measurements in the serum; they are usually done before. Provocation tests (labial and oral) can confirm the diagnosis when necessary. Four families of aeroallergens are described (pollens, house dust mites, pets and moistures). Eviction is the first line of treatment, than drugs are prescribed to reduce symptoms and inflammation (corticosteroids, antihistamine). Desensitization and immunomodulators that can induce tolerance are also proposed in defined situations.

National survey of molecular epidemiology of Staphylococcus aureus colonization in Belgian cystic fibrosis patients.

OBJECTIVES: Epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) is poorly defined in cystic fibrosis (CF) patients, and S. aureus detection may be hampered by the presence of small colony variants (SCVs). We conducted a multicentre survey to determine the prevalence of S. aureus and MRSA colonization in Belgian CF patients and characterize the phenotype and clonal distribution of their staphylococcal strains. METHODS: S. aureus isolated from CF patients attending nine CF centres were collected. Oxacillin resistance was detected by oxacillin agar screen and mecA PCR. Antibiotic susceptibility was tested by microdilution. MRSA strains were genotyped by PFGE and SCCmec typing and compared with hospital-associated MRSA strains. RESULTS: Laboratories used a diversity of sputum culture procedures, many of which appeared substandard. S. aureus was isolated from 275/627 (44%) CF patients (20% to 72% by centre). The prevalence of SCV colonization was 4%, but SCVs were almost exclusively recovered from patients in two centres performing an SCV search. Phenotypically, 14% of S. aureus isolates were oxacillin-resistant: 79% carried mecA and 19% were SCVs lacking mecA. The mean prevalence of 'true' MRSA colonization was 5% (0% to 17% by centre). By PFGE typing, 67% of CF-associated MRSA were related to five epidemic clones widespread in Belgian hospitals. CONCLUSIONS: This first survey of S. aureus colonization in the Belgian CF population indicated a diversity in local prevalence rates and in proportion of oxacillin-resistant and SCV phenotypes, probably related to variation in bacteriological methods. These findings underscore the need for standard S. aureus detection methods and MRSA control policies in Belgian CF centres.

Molecular mechanisms of cell death in periventricular leukomalacia.

OBJECTIVE: To investigate the cytokine-related molecular cascade leading to neural cell death in periventricular leukomalacia (PVL). METHODS: The authors explored potential tumor necrosis factor alpha (TNFalpha) signaling pathways in human brains with PVL and conducted in situ immunohistochemical investigations to search for possible expression of cytokine receptors in these brains. They also investigated likely links to molecules potentially involved in neurocytotoxicity, particularly pathways involving nitrosative-induced apoptosis. RESULTS: TNFalpha overexpression was associated with immune reactivity for p75TNFalphaR2 and p55TNFalphaR1 receptors in affected PVL areas. p75TNFalphaR2 labeling was intense on cerebrovascular endothelial cells in PVL areas, whereas no vascular p55TNFalphaR1 immunoreactivity was detected therein. Immune labeling for both receptors was detected on many white matter parenchymal cells. In contrast, there was no immune reactivity for either receptor in tissues taken from non-PVL areas. Additionally, in situ overexpression of inducible nitric oxide synthase was found in PVL brain regions where apoptotic cell death was detected. CONCLUSIONS: Both p75TNFalphaR2 and p55TNFalphaR1 receptors and nitric oxide may be implicated in the pathogenesis of periventricular leukomalacia.

[The University Hospital of Children Queen Fabiola is twenty years old!]. / L'Hôpital Universitaire des Enfants Reine Fabiola (HUDERF) a vingt ans!

HUDERF is the only pediatric hospital autonomous in Belgium because the other academic pediatric departments are included in general hospitals. However, the model of the children hospital is the most common, both in the industrial nations and in the undeveloped countries and evolves in hospital "mother-child" for the optimaisation of cares in infants that often begin from before the birth, for example in the case of predictable genetic diseases. The children's first hospitals date of more than 150 years ago as that of London for example. Huderf considerably developed in 20 years: it is the topic of this article. Its future is shared with the academic Hospital today and St-Pierre Hospital through the Iris-ULB convention.

[Child food allergy: results of a Belgian cohort]. / L'allergie alimentaire chez l'enfant: étude d'une cohorte belge.

INTRODUCTION: Food Allergy is a public health problem because of its increasingly prevalence, its severity and the difficulty of diagnosis. OBJECTIVE: to describe the responsible food allergens and the clinical features of food allergy in a large group of Belgian children. METHOD: 156 cases of food allergy observed between May 2002 and May 2005 were retrospectively reviewed. All patients have specific IgE. Unequivocal history, response to elimination diet, labial or oral food challenge confirmed the diagnostic. RESULTS: Median age is 26 months (range 1 months-14 years) and male to female ratio is 1.6. Fifty-nine percent of the cases are younger than 3 years. Family history of atopic disease is found in 80.3%. Foods most commonly involved are: eggs (31%), tree nuts (18.1% including hazelnuts 12.3%), cow's milk (16.1%), peanuts (13.2%), fish (4.5%), latex fruits (4.5%).The clinical manifestations are: atopic dermatitis (44.2%), urticaria (40.4%), angioedema (36.5%), gastrointestinal symptoms (21.8%), laryngeal edema (11.5%), asthma (9%), oral allergy syndrome (7.7%), systemic reactions (7.7%) and anaphylactic shock (3.8%). Multiple food allergies are rare: 78.9% of our population have one or two food allergies. CONCLUSION: This study confirms the usual distribution of food allergens in children. It shows that only four allergens (egg, tree nut, cow's milk, peanut) are responsible for 78.4% of the reactions and highlights the emergence of tree nuts allergy, particularly hazelnuts, in Belgium.

Sleep-disordered breathing and hypertension among African Americans.

This study investigated differences in sleep-disordered breathing (SDB) between hypertensives without a family history of hypertension and hypertensives with a family history. Furthermore, it examined whether these two groups differed in the severity of SDB. Patients were African Americans (n=162, mean age=51.19+/-13.77 years; mean body mass index (BMI)=37.85+/-9.51 kg/m2, male=57%), who were referred to the clinic because of a sleep complaint. Sleep was recorded in the laboratory using standard physiological parameters; all parameters were analysed by a trained scorer. Altogether, 91% of the patients received an SDB diagnosis. Of these patients, 25% were hypertensives without a family history, 20% were hypertensives with a family history, and 55% were normotensives. We found a significant difference between these patient groups regarding the severity of SDB (F14,158=1.823, P<0.05), but no significant group difference was observed in the rate of SDB. Increasing weight was accompanied by increasing severity of SDB. The finding that hypertensive patients with or without a positive family history showed worse oxygenation and respiratory characteristics than did normotensives is consistent with previous research. Of note, hypertensives reporting a family history were characterized by a greater number of oxygen desaturations and apnoea hypopnoea index than those typified only by a current diagnosis of hypertension. Hypertensives with a family history are likely to show a profile of greater blood pressure, higher BMI, and more severe SDB, which by all accounts are more common among African Americans.