RESUMO
Morbihan syndrome (MS) is characterized by solid facial edema, usually related to rosacea or acne vulgaris. The facial edema deforms the patient's features, can impair peripheral vision, and affects quality of life. Its pathophysiology remains unclear. The disease usually has a slow and chronic course. MS most commonly affects middle-aged Caucasian men with rosacea and is rare in people below 20 years of age. MS is a diagnosis of exclusion. There is no standard treatment for MS, though systemic isotretinoin and antihistamines are mainly used. We present the case of an adolescent girl with MS nonresponding to 19 months of isotretinoin treatment with add-on antihistamines. Therapy with monthly administration of omalizumab (anti-IgE) for 6 months was an effective therapeutic option, improving the quality of life. Our case is the second description of omalizumab use in Morbihan syndrome, the first in an adolescent.
Assuntos
Angioedema , Rosácea , Masculino , Pessoa de Meia-Idade , Feminino , Humanos , Adolescente , Isotretinoína/uso terapêutico , Omalizumab/uso terapêutico , Qualidade de Vida , Rosácea/diagnóstico , Rosácea/tratamento farmacológico , Síndrome , Edema/diagnóstico , Edema/tratamento farmacológico , Antagonistas dos Receptores Histamínicos/uso terapêuticoRESUMO
BACKGROUND: Patients with a history of anaphylaxis are at risk of future anaphylactic reactions. Thus, secondary prevention measures are recommended for these patients to prevent or attenuate the next reaction. METHODS: Data from the Anaphylaxis Registry were analyzed to identify secondary prevention measures offered to patients who experienced anaphylaxis. Our analysis included 7788 cases from 10 European countries and Brazil. RESULTS: The secondary prevention measures offered varied across the elicitors. A remarkable discrepancy was observed between prevention measures offered in specialized allergy centers (84% of patients were prescribed adrenaline autoinjectors following EAACI guidelines) and outside the centers: Here, EAACI guideline adherence was only 37%. In the multivariate analysis, the elicitor of the reaction, age of the patient, mastocytosis as comorbidity, severity of the reaction, and reimbursement/availability of the autoinjector influence physician's decision to prescribe one. CONCLUSIONS: Based on the low implementation of guidelines concerning secondary prevention measures outside of specialized allergy centers, our findings highlight the importance of these specialized centers and the requirement of better education for primary healthcare and emergency physicians.
Assuntos
Anafilaxia , Prevenção Secundária , Anafilaxia/epidemiologia , Anafilaxia/etiologia , Anafilaxia/prevenção & controle , Brasil , Epinefrina , Europa (Continente)/epidemiologia , Humanos , Sistema de RegistrosRESUMO
The aim of this study was to: (1) find out whether laryngomalacia (LM) types are related to clinical course; (2) which patients with LM are at higher risk of other airway malacia [tracheomalacia (TM) and/or bronchomalacia (BM)]; and (3) evaluate the prevalence of LM in our region. Patients with established LM diagnosis and complete clinical and endoscopy records were enrolled. They were classified into different LM types according to classification based on the side of supraglottic obstruction. One hundred ten children were included. The most common LM appearance was type I-58 children, followed by combine types (I + II and I + III)-38. The other airway malacia were found in 47 patients: TM in 31, BM in 10, and TM with BM in 6. Other comorbidities (cardiac, neurological, and genetic disorders) were identified in 30 children. Patients with combine types of LM differ from those with single type of LM in terms of prematurity (13 vs 31 %, p = 0.04) and higher weight on the examination day (p = 0.006). Patients with other airway malacia differ from children with isolated LM in terms of prematurity (40 vs 13 %, p = 0.008), comorbidities (38 vs 19 %, p = 0.024), and lower weight on the examination day (p = 0.014). The prevalence of clinically relevant LM was one in 2600-3100 newborns. Clinical course of LM cannot be anticipated on the basis of solely endoscopic evaluation of the larynx. Comorbidities and prematurity increase the risk of other airway malacia. The prevalence of LM is relatively high in the middle-south part of Poland.
Assuntos
Obstrução das Vias Respiratórias , Broncomalácia/epidemiologia , Laringomalácia , Traqueomalácia/epidemiologia , Obstrução das Vias Respiratórias/patologia , Obstrução das Vias Respiratórias/fisiopatologia , Broncoscopia/métodos , Comorbidade , Feminino , Humanos , Lactente , Recém-Nascido , Laringomalácia/classificação , Laringomalácia/epidemiologia , Laringomalácia/fisiopatologia , Masculino , Polônia/epidemiologia , Prevalência , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Supraglotite/patologiaRESUMO
During their lifetime, 94.5% of people are stung by wasps, honeybees, hornets, or bumblebees (order Hymenoptera). After a sting, most people show typical local symptoms, 5% to 15% develop local allergic reactions, and 3% to 8.9%--systemic allergic reactions (SARs), which may be potentially life-threatening in about 10% of them. In mild forms of Hymenoptera-venom allergy (HVA), the leading symptoms are urticaria and edema (grades I and II, respectively, according to the Mueller classification). Severe SARs are classified as grade III (respiratory symptoms) and IV (cardiovascular symptoms). Rare manifestations of HVA are Kounis syndrome and takotsubo cardiomyopathy. All patients after an SAR require standard (skin test, IgE, tryptase) or comprehensive (component diagnosis, basophil activation test) allergy testing. All patients with severe systemic symptoms (hypertension, disturbances in consciousness) should be tested for mastocytosis. Additionally, a relationship was found between the severity of HVA symptoms and intake of angiotensin-converting enzyme inhibitors (ACEIs). There is a similar concern, although less well-documented, about the use of ß-blockers. Patients with HVA who have experienced a SAR are potential candidates for venom immunotherapy (VIT), which is effective in 80% to 100% of individuals treated for 3 to 5 years. An increased risk of a VIT failure has been reported in patients with systemic mastocytosis and those treated with ACEIs. In certain groups (beekeepers, patients who develop a SAR to stings during a VIT with a standard dose, as well as those with a SAR to maintenance doses of VIT), a twice higher maintenance dose is recommended. Indications, contraindications, treatment protocols, and vaccine doses are regulated by the international guidelines of allergy societies.
Assuntos
Himenópteros , Hipersensibilidade/diagnóstico , Mordeduras e Picadas de Insetos/imunologia , Adulto , Animais , Venenos de Artrópodes/imunologia , Humanos , Hipersensibilidade/terapiaRESUMO
Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left bronchus by mucoid casts. Pharmacotherapy consisted of glucocorticosteroids, azithromycin, and enalapril maleate. The child also received nebulized 3% NaCl solution, which proved to be beneficial. In children submitted to Fontan-type procedures, physicians must be alert for PB, which can be triggered by respiratory tract infection.
Assuntos
Bronquite/tratamento farmacológico , Bronquite/etiologia , Técnica de Fontan/efeitos adversos , Solução Salina Hipertônica/administração & dosagem , Azitromicina/administração & dosagem , Quimioterapia Combinada , Enalapril/administração & dosagem , Feminino , Glucocorticoides/administração & dosagem , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Nebulizadores e VaporizadoresRESUMO
Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left bronchus by mucoid casts. Pharmacotherapy consisted of glucocorticosteroids, azithromycin, and enalapril maleate. The child also received nebulized 3% NaCl solution, which proved to be beneficial. In children submitted to Fontan-type procedures, physicians must be alert for PB, which can be triggered by respiratory tract infection.
A bronquite plástica (BP), embora uma causa rara de obstrução de vias aéreas, apresenta taxas de mortalidade de até 50% em crianças submetidas a cirurgia cardíaca do tipo Fontan. Apresentamos o caso de uma menina de 18 meses de idade com BP secundária a pneumonia. Aos 6 meses de idade, a paciente havia sido submetida à operação de Glenn devido a coração funcionalmente univentricular. A fibrobroncoscopia revelou obstrução completa do bronco esquerdo por moldes mucoides. A farmacoterapia consistiu em glicocorticosteroides, azitromicina e maleate de enalapril. Adicionalmente, a criança recebeu nebulização de solução de NaCl a 3%, que provou ser benéfica. Em crianças submetidas a operações do tipo Fontan, devemos nos manter alerta quanto à BP, que pode ser desencadeada por infecção do trato respiratório.
Assuntos
Feminino , Humanos , Lactente , Bronquite/tratamento farmacológico , Bronquite/etiologia , Técnica de Fontan/efeitos adversos , Solução Salina Hipertônica/administração & dosagem , Azitromicina/administração & dosagem , Quimioterapia Combinada , Enalapril/administração & dosagem , Glucocorticoides/administração & dosagem , Cardiopatias Congênitas/cirurgia , Nebulizadores e VaporizadoresRESUMO
AIM: To evaluate markers of mast cell and basophil activation in children undergoing the initial phase of honeybee venom immunotherapy (VIT). PATIENTS & METHODS: Five children (four boys and one girl) aged 9.5-18 years with severe systemic bee sting reactions and confirmed IgE-mediated allergy were enrolled. Plasma and urine concentrations of 9α,11ß-PGF2 and serum tryptase levels were measured at four time points and peripheral blood basophil count and CD63 expression were measured at three time points in the course of VIT, including 5-day rush initial immunotherapy (cumulative dose of 223 µg of bee venom allergen) and two subsequent maintenance doses of 100 µg. RESULTS: In the first 40 days of VIT, there was a decrease in mean plasma levels of 9α,11ß-PGF2 (from 41.5 to 27.9 pg/ml; p < 0.05), accompanied by an increase in baseline basophil activation (from 2 to 15%; p < 0.05). The median serum tryptase levels increased from 3.45 to 4.40 ng/ml during rush phase and subsequently returned to initial values (statistically not significant). In four patients, the basophil activation test in response to bee venom allergens remained positive throughout the study. The fifth patient was basophil activation test-negative at all three measurements, and a post hoc analysis revealed clinical peculiarities that are discussed in the paper. CONCLUSIONS: Our preliminary results indicate that plasma levels of 9α,11ß-PGF2 decrease while numbers of activated basophils increase during the initial phase of bee venom rush immunotherapy in children.
Assuntos
Basófilos/metabolismo , Dessensibilização Imunológica , Hipersensibilidade/diagnóstico , Hipersensibilidade/imunologia , Adolescente , Basófilos/efeitos dos fármacos , Basófilos/imunologia , Basófilos/patologia , Venenos de Abelha/efeitos adversos , Biomarcadores/sangue , Biomarcadores/urina , Contagem de Células , Degranulação Celular/efeitos dos fármacos , Criança , Dinoprosta/análogos & derivados , Dinoprosta/sangue , Dinoprosta/urina , Feminino , Humanos , Hipersensibilidade/tratamento farmacológico , Masculino , Mastócitos/patologia , Monitorização Fisiológica/métodos , Tetraspanina 30/biossíntese , Triptases/sangueRESUMO
BACKGROUND AND AIM: Bronchopulmonary dysplasia (BPD) is one of the most common late complications of prematurity. The study aimed to evaluate: 1) somatic development, 2) psychomotor development, 3) parental opinion of health status of the child and 4) prevalence of bronchial hyperreactivity among 5-7 years old children with history of BPD. METHODS: Case-control study included 56 newborns born < or =32 weeks of gestation with mean birthweight (SD) 1057+/-218g. BPD was defined as at least 28 days of oxygen therapy and oxygen or positive pressure support at 36 weeks postmenstrual age. The control group consisted of newborns matched by sex, birthweight (+/-100g), gestational age (+/-1 weeks) and year of hospitalization. Somatic and psychomotor developments were prospectively evaluated up to age of 5-7 years. Parents were asked to complete two questionnaires: one assessing normal daily activity of the child and the standardized questionnaire used by International Study of Asthma and Allergies In Childhood (ISAAC). Peak expiratory flow rates (PEFR) before and after the inhalation of betamimetic were evaluated. RESULTS: No significant difference in somatic development was found between the study group and the control group. Normal psychomotor development was diagnosed in 11 (39%) children with BPD and in 13(46%) children without BPD. Mild or moderate impairment was diagnosed in 12 (43%) cases and 13 (36%) controls, and severe impairment in 5 (18%) and 2 (7%) children. At the age of 5-7 years children with BPD presented lower IQ as compared to children without BPD (94+/-25 vs. 103+/-16, p= 0.2). Parents of children with BPD more often declared that their child's development is impaired (39% vs. 11%, OR: 4.96; 95%CI: 1.2-20). Wheezing in past history was noted in 18 cases (64%) and only in 10 (35%) children without BPD (OR: 3.24; 95% CI: 1.09-9.67). Mean PEFR did not differ significantly between the studied groups (80.5+/-16.3% vs 84.5+/-15.4%; p=0.4). Moreover, changes of PEFR after a dose of betamimetic were similar in both groups (21% vs. 19%, p=0.7). CONCLUSION: The intellectual development of children with BPD at the age of 5-7 is worse than in the control group, especially as declared by their parents. Episodes of wheezing in infancy occurred more frequently in the group of children with BPD. However, bronchial hyperreactivity at the age of 5-7 is not significantly more frequent.
Assuntos
Atividades Cotidianas , Displasia Broncopulmonar/epidemiologia , Displasia Broncopulmonar/fisiopatologia , Nível de Saúde , Doenças do Prematuro/epidemiologia , Doenças do Prematuro/fisiopatologia , Recém-Nascido Prematuro/crescimento & desenvolvimento , Hiper-Reatividade Brônquica/epidemiologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Comorbidade , Progressão da Doença , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Desempenho Psicomotor , Sons Respiratórios , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Prevalence of wheezing during infancy and pre-school age among very low birth weight (VLBW) infants is high. There is too little data to determine exactly the cause of wheezing in this group of children--it may result from early neonatal lung injury or increased susceptibility for allergic reactions. AIM: Analysis of wheezing episodes up to 5-7 years of age in a group of VLBW infants and recognition of risk factors. METHODS: A sample of 104 children aged 5-7 years with birth weight < or = 1500g was prospectively evaluated. The standardized ISAAC (International Study of Asthma and Allergies in Childhood) protocol was used to evaluate the number and quality of wheezing episodes. Total IgE level, specific IgE, lymphocyte Th1/Th2 ratio and skin prick tests (SPT) were performed. Children were divided into 3 groups: without wheezing, with episodes of wheezing in the history (more than 12 months previously), and with persistent episodes of wheezing. RESULTS: Episodes of wheezing were diagnosed in 52 (50%) cases: 27% of children showed signs of wheezing during 12 previous months and 23% of children reported wheezing only in their past history. Children with persistent wheezing presented more frequently than other groups: positive family history for atopy, allergic rhinitis, and positive allergic laboratory markers. Children with wheezing only during early infancy had bronchopulmonary dysplasia and more severe respiratory distress syndrome after birth. CONCLUSION: Prevalence of wheezing during infancy and preschool age among VLBW infants is high. Chronic and recurrent episodes of wheezing are more directly related to atopy than derive from neonatal problems.