Reported Exercise-Related Respiratory Symptoms and Exercise-Induced Bronchoconstriction in Asthmatic Children

BACKGROUND: Unlimited physical activity is one of the key issues of asthma control and management. We investigated how reliable reported exercise-related respiratory symptoms (ERRS) are in predicting exercise-induced bronchoconstriction (EIB) in asthmatic children. METHODS: In this prospective study, 179 asthmatic children aged 7 - 15 years were asked for specific questions on respiratory symptoms related to exercise and allocated into two groups according to whether they complained about symptoms. Group I (n = 134) consisted of children answering "yes" to one or more of the questions and group II (n = 45) consisted of children answering "no" to all of the questions. RESULTS: Sixty-four of 179 children showed a positive exercise challenge test (ECT). There was no difference in the frequency of a positive test between children in group I (n = 48) and group II (n = 12) (P = 0.47). The sensitivity of a positive report for ERRS to predict a positive ECT was only 37%, with a specificity of 0.72. CONCLUSION: According to current guidelines, the report or lack of ERRS has direct consequences on treatment decisions. However, the history of ERRS did not predict EIB and one-third of asthmatic children without complaints of ERRS developed EIB during the ECT. This raises the question of the need for objective measures of bronchial hyperresponsiveness (BHR) in pediatric asthma management

Coexistence of 2 rare autosomal recessively inherited disorders manifesting with immune deficiency; IL-12 receptor beta 1 and biotinidase deficiencies

WOS: 000463332100019PubMed ID: 30968642In this report, we described an infant with both partial biotinidase and IL-12R beta 1 deficiencies as these two entities are rare and unrelated inherited disorders. One-month-old girl was diagnosed as partial biotinidase deficiency with newborn screening programme. Mutation analysis revealed a compound heterozygous mutation BTD: c.1330G>C (p.Val444Leu) / c.196_197dupCATC (p.Leu69HisfsTer24). At the age of 6 months, a nodule on her left axilla with purulent discharge was noticed which was related to BCG vaccination. A mutational analysis revealed a homozygous c.783+1G>A mutation on IL-12R beta 1 gene. Interferon-gamma and anti-tuberculosis treatment were initiated together and the nodule with purulent discharge regressed dramatically. Here, we want to emphasize consideration of coexistence of two rare autosomal recessively inherited diseases in a patient due to the high rate of consanguinity in our country

Exhaled breath condensate MMP-9 level and its relationship with asthma severity and interleukin-4/10 levels in children

WOS: 000303441900004PubMed ID: 22541398Background: Matrix metalloproteases (MMPs) are key mediators in airway remodeling, and MMP-9 is the main type investigated to discover its implication for the pathogenesis and severity of asthma. Objective: To evaluate MMP-9 and its natural tissue inhibitors of metalloproteinases (TIMP-1) levels of exhaled breath condensate (EBC) in children with asthma. We also analyzed any potential relationship between these enzymes and EBC interleukin (IL)-4/10 levels as well as asthma severity. Methods: Three study groups were formed: group 1, children with persistent asthma (n = 20); group 2, children with intermittent asthma (n = 10), and group 3, healthy controls (n = 12). Pulmonary functions were measured as forced expiratory volume in 1 second (FEV1), peak expiratory flow (PEF), and forced expiratory flow from 25% to 75% of vital capacity values by spirometry, and MMP-9, TIMP-1 and IL-4/10 levels in EBC were analyzed by enzyme-linked immunosorbent assay (ELISA). Results: The MMP-9 levels of EBC were found to be 57.7 +/- 17.5, 35.4 +/- 11.7, and 30.6 +/- 3.7 ng/mL in children belonging to group 1, group 2 and group 3, respectively. Children belonging to group 1 and group 2 showed significantly higher MMP-9 levels of EBC in comparison with the controls (P < .001 and P < .047, respectively). No statistically significant difference was found between groups regarding TIMP-1 levels of EBC. EBC MMP-9 levels were inversely correlated with both FEV1 and PEF values (r = -0.472, P = .011, and r = -0.571, P = .002, respectively) in children with asthma. Positive correlations were also seen between MMP-9 levels and IL-4/10 levels of EBC (r = 0.419, P = .027 and r = 0.405, P = .032, respectively) in children with asthma. Conclusion: We showed that MMP-9 levels of EBC are elevated in children with asthma and correlated with lung functions and other inflammatory markers such as IL-4/IL10 in EBC. (C) 2012 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved

Decreased serum vitamin B-12 and vitamin D levels affect sleep quality in children with familial Mediterranean fever

WOS: 000419778900010PubMed ID: 29143127Familial Mediterranean fever is an autoinflammatory disorder characterized by recurrent fever and serositis. We aimed to describe serum vitamin B-12 and vitamin D levels and their correlation with self-reported sleep quality of pediatric FMF patients. Sixty-three children with FMF were enrolled to the study. Information on sleep quality was obtained using self-administration of Pittsburg Sleep Quality Index (PSQI). The patients were divided into subgroups depending on vitamin D serum concentrations: >= 20 and = 200, < 200 pg/ml. B-12 levels were not correlated with PSQI scores, whereas significant correlation was found between vitamin D and total PSQI scores and daytime sleepiness. Total PSQI score, sleep disorders and daytime sleepiness sub-scores were statistically high in patients with serum D vitamin levels below 20 ng/ml. Sleep efficiency was found lower in patients with serum B-12 levels below 200 pg/ml. B-12 may have a positive role on effective sleep. More importantly, we suggest that vitamin D is protective against sleep disorders and poor sleep, it may also improve daytime activities

MEW gene variants in children with Henoch-Schonlein purpura and association with clinical manifestations: a singie-center Mediterranean experience

WOS: 000457336800001PubMed ID: 30513227Objectives: Henoch-Schonlein purpura (HSP) is characterized by non-thrombocytopenic palpable purpura, abdominal pain, and arthralgia/arthritis. We aimed to describe the clinical presentations of children with HSP in a single center and compare the prevalence of each manifestations between patients with MEFV variants, particularly in exon 10 and those without. Methods: This cohort retrospectively included 144 HSP (59 females, 85 males) patients without Familial Mediterranean Fever (FMF) symptoms and followed for at least 6 months. We utilized the MEFV gene sequencing by using next-generation sequencing platform (MiSeq System, Illumina). Results: At least one MEFV variant was detected in 73 (50.7%) of 144 HSP patients and 5 (3.5%) patients were homozygote for M694V mutation. Although severe gastrointestinal involvement and nephritis rates were similar, we found that serum IgA, leukocyte, and platelet count at diagnosis were higher and hemoglobin was lower in HSP patients with MEFV gene variants in exon 10 than those without. Additionally, HSP patients with MEFV variants in exon 10 more often present with abdominal pain and intussusception. Conclusion: MEFV variants in exon 10 may affect clinical presentation of HSP in populations where FMF is common. While HSP may be an initial symptom of FMF, we speculate that physicians should be aware of FMF possibility in children with intussusception and lower hemoglobin, higher serum IgA, leukocyte, and platelet count.Cukurova University Scientific Research UnitCukurova University [TAY-2016-4020]This study was supported by the Cukurova University Scientific Research Unit under TAY-2016-4020

Distribution of Allergen Sensitization in Childhood with the Skin Test

WOS: 000404965700007Objective: Skin prick testing (SPT) is widely used for assessing the clinical reactivity to aeroallergens, foods, and drugs. The aim of our study was to investigate the prevalence of allergic sensitivity in the patients presenting to our clinic. Materials and Methods: The retrospective study included 2,512 patients who presented to our clinic and underwent SPT due to suspected atopy between January and December 2014. SPT was based on sensitivity against house dust mites, molds, pollens, and foods including milk, egg, wheat, chestnut, and latex. Based on their ages, the patients were divided into four groups: Group I; 2 years or younger, Group II; 3-5 years, Group III; 6-12 years, and Group IV; over 12 years. The SPT scores were evaluated based on the age groups, gender, and the month. Results: The 2,512 patients included 1,444 (57.5%) boys and 1,068 (42.5%) girls. At least one SPT was positive in 1,058 (42.3%) patients. Skin test was positive in 44.8% of boys as compared to 38.4% of girls. Multiple allergen sensitivity was present in 93% of the patients. Most of the patients were allergic to house dust mites [Dermatophagoides pteronyssinus (% 73.8) and Dermatophagoides farinae (% 71.6)], followed by mold (23.4%) and grasses-cereals mix (23.8%). Among the foods, eggs (3.8%) and milk (3.1%) had the highest rate of allergic reaction. No patient had allergic reaction to latex. Among the age groups, Group III had the highest rate (50.4%) of positivity. An evaluation based on the months revealed that the patients were sensitive against aeroallergens, particularly pollens, in May and June, against molds in May and September, and against house dust mites all year round. Conclusion: The skin prick test is a practical method for the assessment of allergic sensitivity

Recurrent Henoch Schonlein purpura without renal involvement successfully treated with methotrexate

WOS: 000471179900013PubMed ID: 30453828Introduction Henoch Schonlein purpura is characterised by palpable purpura, abdominal pain, arthritis/arthralgia, often with a self-limiting course. Herein, we report a patient with recurrent Henoch Schonlein purpura and severe gastrointestinal involvement, successfully treated with methotrexate. Case presentation A 12-year-old boy was admitted to our department with palpable purpura, abdominal pain and arthralgia. Since gastrointestinal complaints were severe, systemic steroids were administered, with tapering of dosage. Henoch Schonlein purpura recurred several times with severe abdominal pain, maelena and purpura during next two months. Colchicine and hydroxychloroquine were initiated. After four months, we also introduced methotrexate, which enabled discontinuation of previous medications including corticosteroids. Methotrexate was ceased four months later, and remission was sustained without any medications for 24 months. Conclusion Besides the conflicting data regarding the use of methotrexate in recurrent Henoch Schonlein purpura, our case introduces successful methotrexate experience in a child with Henoch Schonlein purpura and recurrent severe gastrointestinal involvement

Clinical manifestations and outcomes of 420 children with Henoch Schonlein Purpura from a single referral center from Turkey: A three-year experience

WOS: 000496376300001PubMed ID: 31662011Objectives: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 +/- 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease

Do practical laboratory indices predict the outcomes of children with Henoch-Schonlein purpura?

WOS: 000476946700007PubMed ID: 31021301Objectives: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis of childhood and often has a self-limiting course. We aimed to study whether practical laboratory parameters at the diagnosis predict disease course including recurrence and nephritis in addition to severe gastrointestinal involvement in children with HSP.Methods: This retrospective cohort study included 214 HSP patients, 43.5% (n = 93) female and 56.5% (n =121) male, who were diagnosed in our department. Laboratory parameters before treatment, including neutrophil, lymphocyte and platelet counts, mean platelet volume (MPV), neutrophil-to-lymphocyte (NLR), and platelet-to-lymphocyte ratios (PLR) were obtained retrospectively. Age at diagnosis, duration of follow-up, gender, preceding infections, medications, arthritis and arthralgia, abdominal pain, severe GI involvement, invagination, renal involvement and presence of nephritis, outcomes, and presence of recurrences were retrospectively recorded from medical files. Severe GI involvement was determined as severe colicky abdominal pain, bowel edema in ultrasonography or overt GI bleeding. A relapse was defined as a new flare of cutaneous lesions or other manifestations in a patient at least four asymptomatic weeks after the initial HSP episode.Results: Mean age at diagnosis was 7.6 3.1 years. Biopsy-proven nephritis was found in 16 (7.5%) patients. Severe GI involvement was present in 77 (36%) patients, whereas only 12 (5.6%) patients were diagnosed with intussusception and in 29 (13.5%) patients, HSP recurred. Neutrophil count and NLR were found higher in HSP patients with severe gastrointestinal involvement and biopsy-proven nephritis. Additionally, only platelet count was lower and MPV was higher in patients with recurrent HSP.Conclusion: Elevated neutrophil count and NLR may be relevant markers for severe GI involvement and nephritis, whereas platelet count and MPV were the only laboratory parameters associated with disease recurrence