Leukotrienes and leukotriene modifiers in pediatric allergic diseases

Leukotrienes are potent pro-inflammatory lipid mediators derived from arachidonic acid through several enzymatic pathways. They have an essential role in allergic inflammation, where they induce bronchoconstriction, airway edema, and chemotaxis of the inflammatory cells in the airways, nasal and conjunctival tissues. Leukotriene modifiers include leukotriene receptor antagonists (montelukast, zafirlukast and pranlukast) and leukotriene synthesis inhibitors (zileuton). These medications have been extensively used in childhood allergic diseases. This review will highlight the leukotriene pathway and its role in allergy as well as the effects of leukotriene modifiers in different allergic disorders

Sesame seed sensitization in a group of atopic Egyptian children

Background: There are no published data on the prevalence of sesame allergy/sensitization in Egypt. Objective: In this pilot study, we thought to estimate the frequency of sesame seed sensitization in a group of atopic Egyptian infants and children. Methods: We consecutively enrolled 90 patients with physician diagnosed allergic disease. The study measurements included clinical evaluation for the site and duration of allergy, history suggestive of sesame seed allergy, and family history of allergy, as well as skin prick testing (SPT) using a commercial sesame extract, and serum sesame specific IgE (SpIgE) estimation. Results: None of the studied patients reported symptoms suggestive of sesame seed allergy. Nevertheless, two children (2.2%) showed positive SPT response to sesame (wheal diameter ≥ 3 mm above the negative control). Only one of them had a wheal diameter which exceeded that of the histamine control. The serum sesame SpIgE exceeded 0.35 IU/ml in all subjects [range = 0.35 - 3.0 IU/ml; median (IQR) = 0.9 (0.6) IU/ml]. Serum sesame SpIgE was significantly increased in patients with history of recurrent urticaria (p=0.03). Conclusion: Sesame seed sensitization is not uncommon in atopic Egyptian children. It can be associated with any clinical form of allergy and the causal relationship needs meticulous evaluation. Wider scale population-based studies are needed to assess the prevalence of sesame allergy and its clinical correlates in our country.Keywords: Food allergy, sesame, atopic children

The effect of serum angiotensin II and angiotensin II type 1 receptor gene polymorphism on pediatric lupus nephritis

Background: Angiotensin II (Ang II) is found to perpetuate inflammation and visceral damage in systemic lupus erythematosus (SLE). It mediates most of its actions through Ang II receptor type I (AT1) whose gene polymorphism A1166C (CC genotype) seems to have pathogenic effects. Objective: To measure serum Ang II and the frequency of AT1 receptor CC genotype among a group of Egyptian patients with pediatric onset lupus nephritis (pLN). Methods: This is a case-control cross sectional study which included 24 patients with pLN and 24 age and sex-matched healthy subjects as controls. Clinical evaluation and routine laboratory markers for SLE patients were done. SLE disease activity index (SLEDAI) and the British Isles Lupus Assessment Group (BILAG)-2004 renal score were measured. Serum Ang II was measured by enzyme linked immunosorbent assay and detection of ATI receptor CC genotype by polymerase chain reaction were done for both patients and controls. Results: Patients had significantly higher serum Ang II than the controls (p=0.0001). The frequency of AT1 receptor CC genotype was significantly higher among patients as compared to the control group (p=0.008). Both serum Ang II and AT1 receptor CC genotype were comparable between patients with proliferative LN class III and IV and those with LN class II (p>0.05). Serum Ang II did not correlate significantly with SLEDAI or BILAG-renal score (p>0.05). Conclusion: Serum Ang II and AT1 receptor CC genotype seem to have pathogenic role in pLN but with no deleterious effects on the phenotype of LN for further assessment.Keywords: Lupus nephritis; Angiotensin II; Angiotensin II type 1 receptor; Polymorphism; Pediatrics

Hen’s egg white hypersensitivity among a group of Egyptian atopic children

Background: Egg allergy is potentially life-threatening. The prevalenceof egg allergy in Egypt is still unclear. This study is to evaluate thefrequency of egg hypersensitivity in a group of Egyptian atopic children.Methods: Eighty allergic children were enrolled, each is subjected toclinical evaluation, skin prick testing (SPT) using a commercial eggwhite extract, and serum egg white specific IgE (SpIgE) estimation. Sixpatients with suspected egg allergy consent to perform open oral eggchallenge. Results: Twenty-eight patients had history of exacerbation oftheir allergic diseases upon exposure to egg white, of these patients, 8had negative SPT and serum egg white SpIgE. SPT was positive in 25(31.2%) patients, of these patients, 3 (4%) were +3, 22 (28%) were +2,of whom 5 patients tolerate eggs without adverse effects. Serum eggwhite SpIgE was positive in 19 (24%) patients with a mean of 0.81 IU/ml(range: 0.35-4.52 IU/ML). Egg white allergy based on positive history,positive SPT and/or egg white SpIgE was detected in 23 (28.8%)patients. Open oral egg challenge was positive in one patient withpositive history but negative tests giving an overall frequency of eggallergy of 30 % (n=24).While egg white SpIgE did not correlate with theages, positive SPT was significantly more frequent among youngerpatients (t= 1.7, p=0.02). Egg sensitization and allergy did not affect theseverity of asthma (p > 0.05). Conclusion: Although positive SPT/ serumspecific IgE to eggs are good tools for diagnosis, oral food challengeremains the gold standard in suspected cases. Further wide-scale studiesare needed to outline the real prevalence of egg allergy in Egypt.Keywords: Egg allergy; children; skin prick test

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: An international collaboration

Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) to Simple Measure of Impact of Illness in Youngsters (SMILY-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Conclusion: SMILY-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY-Illness with its available translations may be used as useful adjuncts to clinical practice and research

Defining criteria for disease activity states in systemic juvenile idiopathic arthritis based on the systemic Juvenile Arthritis Disease Activity Score

Objective To develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MiDA), moderate disease activity (MoDA), and high disease activity (HDA) in children with systemic juvenile idiopathic arthritis (sJIA), based on subjective disease state assessment by the treating pediatric rheumatologist. Methods The cutoffs definition cohort was composed of 400 patients enrolled at 30 pediatric rheumatology centers in 11 countries. Using the subjective physician rating as an external criterion, 6 methods were applied to identify the cutoffs: mapping, calculation of percentiles of cumulative score distribution, Youden index, 90% specificity, maximum agreement, and ROC curve analysis. Sixty percent of the patients were assigned to the definition cohort and 40% to the validation cohort. Cutoff validation was conducted by assessing discriminative ability. Results The sJADAS10 cutoffs that separated ID from MiDA, MiDA from MoDA, and MoDA from HDA were ≤ 2.9, ≤ 10, and > 20.6. The cutoffs discriminated strongly among different levels of pain, between patients with or without morning stiffness, and between patients whose parents judged their disease status as remission or persistent activity/flare or were satisfied or not satisfied with current illness outcome. Conclusion The sJADAS cutoffs revealed good metrologic properties in both definition and validation cohorts, and are therefore suitable for use in clinical trials and routine practice

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration

Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases.Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY (c)) to Simple Measure of Impact of Illness in Youngsters (SMILY (c)-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY (c)-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n= 15) and 17 parents participated. the mean age was 12 +/- 4 years, with median disease duration of 21 months (1-172 months). We translated SMILY (c)-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa.Conclusion: SMILY (c)-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY (c)-Illness with its available translations may be used as useful adjuncts to clinical practice and research.Rutgers State Univ, Robert Wood Johnson Med Sch, New Brunswick, NJ 08903 USARutgers State Univ, Child Hlth Inst New Jersey, New Brunswick, NJ 08901 USAHosp Special Surg, New York, NY 10021 USAUniv Michigan, Ann Arbor, MI 48109 USARed Cross War Mem Childrens Hosp, Cape Town, South AfricaAin Shams Univ, Pediat Allergy Immunol & Rheumatol Unit, Cairo, EgyptAin Shams Univ, Pediat Rheumatol Pediat Allergy Immunol & Rheum, Cairo, EgyptKing Faisal Specialist Hosp & Res Ctr, Riyadh 11211, Saudi ArabiaCharles Univ Prague, Prague, Czech RepublicGen Univ Hosp, Prague, Czech RepublicUniv Hosp Motol, Dept Pediat, Prague, Czech RepublicAarhus Univ, Hosp Skejby, Aarhus, DenmarkRigshosp, Juliane Marie Ctr, DK-2100 Copenhagen, DenmarkUniv Med Ctr, Dept Pediat Immunol, Utrecht, NetherlandsWilhelmina Childrens Hosp, Utrecht, NetherlandsGreat Ormond St Hosp Sick Children, Children NHS Fdn Trust, Renal Unit, London, EnglandLyon Univ, Hosp Civils Lyon, Rheumatol & Dermatol Dept, Lyon, FranceMed Univ Innsbruck, A-6020 Innsbruck, AustriaPrim Univ Doz, Bregenz, AustriaHamburg Ctr Pediat & Adolescence Rheumatol, Hamburg, GermanyAsklepios Clin Sankt, Augustin, GermanyUniv Zurich, Childrens Hosp, Zurich, SwitzerlandAristotle Univ Thessaloniki, Pediat Immunol & Rheumatol Referral Ctr, GR-54006 Thessaloniki, GreeceIsrael Meir Hosp, Kefar Sava, IsraelSanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, IndiaSemmelweis Univ, H-1085 Budapest, HungaryAnna Meyer Hosp, Florence, ItalyUniv Siena, Res Ctr System Autoimmune & Autoinflammatory Dis, I-53100 Siena, ItalyUniv Florence, Florence, ItalyOsped Pediat Bambino Gesu, IRCCS, Pediat Rheumatol Unit, Rome, ItalyUniv Genoa Pediat II Reumatol, Ist G Gaslini EULAR, Ctr Excellence Rheumatol, Genoa, ItalyUniv Cattolica Sacro Cuore, Inst Pediat, Rome, ItalyUniv Padua, Dept Pediat, Pediat Rheumatol Unit, Padua, ItalyYokohama City Univ, Sch Med, Yokohama, Kanagawa 232, JapanUniv Estadual Paulista, UNESP, Botucatu, SP, BrazilUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilUniv Estadual Campinas, Dept Med, Campinas, SP, BrazilUniv Fed Rio de Janeiro, Dept Pediat, Rio de Janeiro, BrazilUniv Estado do, Adolescent Hlth Care Unit, Div Pediat Rheumatol, Rio de Janeiro, BrazilUniv São Paulo, Fac Med, Childrens Inst, Dept Pediat,Pediat Rheumatol Unit, São Paulo, BrazilChildrens Inst, Pediat Rheumatol Unit, São Paulo, BrazilClin Pediat I, Cluj Napoca, RomaniaInst Rheumatol, Belgrade, SerbiaUniv Childrens Hosp, Univ Med Ctr Ljubljana, Ljubljana, SloveniaHead Rheumatol Hosp Pedro Elizalde, Buenos Aires, DF, ArgentinaHosp Gen Mexico City, Mexico City, DF, MexicoHosp Infantil Mexico Fed Gomez, Mexico City, DF, MexicoHosp San Juan Dios, Barcelona, SpainHosp Univ Valle Hebron, Barcelona, SpainMt Sinai Med Ctr, New York, NY 10029 USAMt Sinai Med Ctr, Miami Beach, FL 33140 USAComplejo Hosp Univ Ruiz & Paez, Bolivar, VenezuelaHacettepe Univ, Dept Pediat, Ankara, TurkeyIstanbul Univ, Cerrahpasa Med Sch, Istanbul, TurkeyFMF Arthrit Vasculitis & Orphan Dis Res Ctr, Inst Hlth Sci, Ankara, TurkeyUniv Calgary, Dept Pediat, Alberta Childrens Hosp, Res Inst, Calgary, AB T2N 1N4, CanadaUniversidade Federal de São Paulo, Dept Pediat, São Paulo, BrazilWeb of Scienc

Surgical treatment and outcome of conventional pelvic chondrosarcoma

We reviewed 124 patients with a conventional pelvic chondrosarcoma who had been treated over a period of 20 years. We recorded the type of tumour (central or peripheral), type of operation (limb salvage surgery or hemipelvectomy), the grade of tumour, local recurrence and/or metastases, in order to identify the factors which might influence survival. More satisfactory surgical margins were achieved for central tumours or in those patients treated by hemipelvectomy. However, grade 1 tumours, whatever the course, did not develope metastases or cause death, while grade 3 tumours had the worst outcome and prognosis. Central, high-grade tumours require aggressive surgical treatment in order to achieve adequate surgical margins, particularly in those lesions located close to the sacroiliac joint. By contrast, grade 1 peripheral chondrosarcomas may be treated with contaminated margins in order to reduce operative morbidity, but without reducing surviva