Clinical manifestation of pediatric granulomatosis with polyangiitis - the experience of two regions in Poland.

BACKGROUND: The purpose of this study was to describe clinical manifestations, laboratory findings and outcome of granulomatosis with polyangiitis (GPA) in pediatric patients living in two regions (Southern and Central) of Poland. METHODS: Retrospective analysis of patient hospital records from four large hospitals during a period from 1995 to 2013. Patients with confirmed diagnosis of GPA according to American College of Rheumatology (ACR) and EULAR/PRINTO/PRES criteria for GPA were analyzed. All patients were subjected to clinical, laboratory, radiological and immunological assessment. RESULTS: During this 18-year period only 9 children with confirmed diagnosis of GPA (6 girls, 3 boys) were identified. The average age of the disease onset was 12 years (range: 8-16 years). Average delay between first symptoms and diagnosis was approx. 20 months (range: 0-84 months). Organ system involvement at presentation included: kidneys 88.8% (8/9), lungs 77.7% (7/9), ear/nose/ throat 55.5% (5/9), gastrointestinal tract 55.5% (5/9), skin 44.4% (4/9), joints 22.2% (2/9), eyes 11.1% (1/9) and nervous system 11.1% (1/9). In 5 children disease course was progressive (constant progression of sinusitis in one case, end-stage renal disease in two, chronic kidney disease stage IV in one and one child died due to alveolar hemorrhage). CONCLUSION: The majority of our patients were females. Clinical features of pediatric GPA were similar to those described in adults. None of our patients developed subglottic stenosis and in only 2 children saddle-nose deformity was observed. Although GPA was treated according to contemporary standards care, disease progression was observed in more than a half of children.

[Primary ciliary dyskinesia in highlights of consensus statement. Presentation of pediatric cases]. / Pierwotna dyskinezja rzesek w swietle rekomendacji. Opis przypadków klinicznych u dzieci.

UNLABELLED: Cilia are evolutionarily conserved structures that play a role in diverse cell types. Motile cilia are involved in the most prominent ciliopathy called primary ciliary dyskinesia (PCD), which combines oto-sino-pulmonary symptoms (impaired mucociliary clearance that is important innate defense mechanism), male infertility and in nearly 50% cases situs inversus. Disease is usually inherited as autosomal recessive disorder, concerning mainly outer and/or inner dynein arms of cilia. Diagnosis of PCD requires the presence of characteristic clinical phenotype and confirmation the diagnosis by either identification of specific defect in electron microscopy or other evidence of abnormal ciliary function. The diagnosis of PCD may be delayed, missed or made incorrectly. The first ERS consensus statement which formulates recommendations regarding diagnostic as well as therapeutic approaches to children with PCD, is a very helpful tool in the management of this patients. We present our own experience with three children with PCD diagnosed in our Department. CONCLUSION: in children with clinical symptoms suggesting PCD, even with negative screening tests, the estimation of specific cilia defect in electron microscopy is indicated.

[Increasing prevalence of asthma in school children--ISAAC study (International Study of Asthma and Allergies in Children)]. / Wzrost wystepowania astmy oskrzelowej u dzieci szkolnych w Krakowie i w Poznaniu--badanie ISAAC.

UNLABELLED: The aim of the study was to assess changes in asthma prevalence between 1994/1995 (study I) and 2001/2002 (study II). Standardized ISAAC questionnaire was used. Randomly assigned population of school children 6-7 and 13-14 yrs of age was surveyed in Kraków and Poznan. RESULTS: The prevalence of wheezing ever increased significantly in group 6-7 yrs of age from 25.5% to 28.6% in Krakow and from 17.3% to 27.6% in Poznan, respectively in study I and study II. The similar rise was found in group 13-14 yrs of age in Krakow from 14.6% to 21.6%, in Poznan from 17.1% to 22.2%. In both age groups and in both centers the number of children with established asthma diagnosis increased: in group 6-7 yrs of age in Krakow from 4.0% to 5.8%, in Poznan from 1.3% to 5.9%; in group 13-14 yrs of age in Krakow from 2.3% to 6.8%, in Poznan from 2.0% to 5.2%. Analysis of current symptoms (in the last year) confirmed increasing prevalence of wheezing, wheezing after exercise and night cough in Poznan in both age group, in Krakow only in older children. In younger children in Kraków significant difference concerned only wheezing after exercise. The most frequent symptom reported by children was nocturnal cough in the last year. CONCLUSIONS: The prevalence of the asthma symptoms and established asthma diagnosis increased during last 7 years. However significant number of children presenting asthma symptoms remains still underdiagnosed.

[Relationships between atopy and bronchial hyper-reactivity in Polish school age children]. / Wzajemne powiazanie atopii z nadreaktywnoscia oskrzeli u dzieci szkolnych.

OBJECTIVE: To determine the prevalence of atopy and bronchial hyper-reactivity (BHR) and their relationship with wheezing and diagnosed asthma reported in schoolchildren. MATERIAL AND METHODS: In Cracow, 1347 schoolchildren (9.7 +/- 0.7 yrs of age) were surveyed with ISAAC questionnaire. Skin prick tests with 7 aeroallergens and the exercise challenge test for BHR evaluation were carried out. Atopy was established when a positive skin reaction with at least one allergen occurred. The BHR was confirmed in presence of at least 15% decrease of the relative post-exercise peak expiratory flow (D% PEF) or wheezing during or immediately after exercise. RESULTS: Atopy was found in 26.2% of schoolchildren, with a two-fold higher risk in boys/odds ratio (OR) = 2.14; 95% confidence interval (CI) (1.59-2.86)/. In the group of atopic children, wheeze in the last year and diagnosis of asthma occurred significantly more often. The risk of wheeze in last year related to the type of sensitizing allergen and was highest for Der. f./OR = 6.1; 95% CI (3.78-10.16)/, medium for allergens of trees, dog's fur, Der. p., cat's fur and grasses/OR = 5.17; 95% CI (2.27-11.76), OR = 4.36; 95% CI (2.35-8.13), OR = 4.00; 95% CI (3.12-8.01), OR = 3.86; 95% CI (2.18-6.82), OR = 2.58; 95% CI (1.50-4.45), respectively/. In case of Alternaria allergens no correlation with wheeze was observed/OR = 2.71; 95% CI (0.88-8.31)/. BHR was identified in 12.4% of schoolchildren and demonstrated a significant correlation with that reported in the questionnaire: wheeze in the last year/OR = 2.28; 95% CI (1.25-4.14)/, diagnosis of asthma/OR = 2.96; 95% CI (1.38-6.34)/and post-exercise wheeze/OR = 3.17; 95% CI (1.60-6.27)/. Moreover, a significant correlation was demonstrated between atopy and BHR/OR = 1.85; 95% CI (1.22-2.79)/. In the group of children without wheeze in the past and without diagnosis of asthma, BHR was significantly higher in atopic children (positive skin test to in-door allergens) comparing to non-atopic ones, mean D% PEF values were 3.95%, -0.39%, respectively (p. = 0.0014). CONCLUSIONS: In children, there is a significant correlation between atopy and BHR, modified by individual history of wheeze or asthma, sex and allergen type.