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BACKGROUND: Angioedema (AE) manifests with intermittent, localized, self-limiting swelling of the subcutaneous and/or submucosal tissue. AE is heterogeneous, can be hereditary or acquired, may occur only once or be recurrent, may exhibit wheals or not, and may be due to mast cell mediators, bradykinin, or other mechanisms. Several different taxonomic systems are currently used, making it difficult to compare the results of studies, develop multicenter collaboration, and harmonize AE treatment. OBJECTIVE: We developed a consensus on the definition, acronyms, nomenclature, and classification of AE (DANCE). METHODS: The initiative involved 91 experts from 35 countries and was endorsed by 53 scientific and medical societies, and patient organizations. A consensus was reached by online discussion and voting using the Delphi process over a period of 16 months (June 2021 to November 2022). RESULTS: The DANCE initiative resulted in an international consensus on the definition, classification, and terminology of AE. The new consensus classification features 5 types and endotypes of AE and a harmonized vocabulary of abbreviations/acronyms. CONCLUSION: The DANCE classification complements current clinical guidelines and expert consensus recommendations on the diagnostic assessment and treatment of AE. DANCE does not replace current clinical guidelines, and expert consensus algorithms and should not be misconstrued in a way that affects reimbursement of medicines prescribed by physicians using sound clinical judgment. We anticipate that this new AE taxonomy and nomenclature will harmonize and facilitate AE research and clinical studies, thereby improving patient care.
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Angioedema , Consenso , Terminologia como Assunto , Humanos , Angioedema/classificação , Angioedema/diagnóstico , Abreviaturas como Assunto , Técnica DelphiRESUMO
Membranous nephropathy (MN) is a leading cause of kidney failure worldwide and frequently recurs after transplant. Available data originated from small retrospective cohort studies or registry analyses; therefore, uncertainties remain on risk factors for MN recurrence and response to therapy. Within the Post-Transplant Glomerular Disease Consortium, we conducted a retrospective multicenter cohort study examining the MN recurrence rate, risk factors, and response to treatment. This study screened 22,921 patients across 3 continents and included 194 patients who underwent a kidney transplant due to biopsy-proven MN. The cumulative incidence of MN recurrence was 31% at 10 years posttransplant. Patients with a faster progression toward end-stage kidney disease were at higher risk of developing recurrent MN (hazard ratio [HR], 0.55 per decade; 95% confidence interval [CI], 0.35-0.88). Moreover, elevated pretransplant levels of anti-phospholipase A2 receptor (PLA2R) antibodies were strongly associated with recurrence (HR, 18.58; 95% CI, 5.37-64.27). Patients receiving rituximab for MN recurrence had a higher likelihood of achieving remission than patients receiving renin-angiotensin-aldosterone system inhibition alone. In sum, MN recurs in one-third of patients posttransplant, and measurement of serum anti-PLA2R antibody levels shortly before transplant could aid in risk-stratifying patients for MN recurrence. Moreover, patients receiving rituximab had a higher rate of treatment response.
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Glomerulonefrite Membranosa , Transplante de Rim , Recidiva , Humanos , Glomerulonefrite Membranosa/etiologia , Glomerulonefrite Membranosa/patologia , Glomerulonefrite Membranosa/tratamento farmacológico , Transplante de Rim/efeitos adversos , Masculino , Estudos Retrospectivos , Feminino , Pessoa de Meia-Idade , Fatores de Risco , Seguimentos , Prognóstico , Adulto , Taxa de Filtração Glomerular , Falência Renal Crônica/cirurgia , Complicações Pós-Operatórias , Sobrevivência de Enxerto , Testes de Função Renal , Incidência , Rejeição de Enxerto/etiologia , Rejeição de Enxerto/patologia , Taxa de SobrevidaRESUMO
OBJECTIVE: To determine if children who present with an elbow flexion contracture (EFC) from brachial plexus birth injury (BPBI) are more likely to develop shoulder contracture and undergo surgical treatment. STUDY DESIGN: Retrospective review of children <2 years of age with BPBI who presented to a single children's hospital from 1993 to 2020. Age, elbow and shoulder range of motion (ROM), imaging measurements, and surgical treatment and outcome were analyzed. Patients with an EFC of ≥10° were included in the study sample. Data from 2445 clinical evaluations (1190 patients) were assessed. The final study cohort included 72 EFC cases matched with 230 non-EFC controls. Three patients lacked sufficient follow-up data. RESULTS: There were 299 included patients who showed no differences between study and control groups with respect to age, sex, race, ethnicity, or functional score. Patients with EFC had 12° less shoulder range of motion (95% CI, 5°-20°; P < .001) and had 2.5 times the odds of shoulder contracture (OR, 2.5; 95% CI, 1.3-4.7; P = .006). For each additional 5° of EFC, the odds of shoulder contracture increased by 50% (OR, 1.5; 95% CI, 1.2-1.8; P < .001) and odds of shoulder procedure increased by 62% (OR, 1.62; 95% CI, 1.04-2.53; P = .03). Sensitivity of EFC for predicting shoulder contracture was 49% and specificity was 82%. CONCLUSIONS: In patients with BPBI <2 years of age, presence of EFC can be used as a screening tool in identifying shoulder contractures that may otherwise be difficult to assess. Prompt referral should be arranged for evaluation at a BPBI specialty clinic, because delayed presentation risks worsening shoulder contracture and potentially more complicated surgery.
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Traumatismos do Nascimento , Neuropatias do Plexo Braquial , Plexo Braquial , Contratura , Criança , Lactente , Humanos , Cotovelo , Ombro , Neuropatias do Plexo Braquial/complicações , Neuropatias do Plexo Braquial/diagnóstico , Contratura/diagnóstico , Contratura/etiologia , Plexo Braquial/lesões , Plexo Braquial/cirurgia , Amplitude de Movimento Articular , Traumatismos do Nascimento/complicações , Traumatismos do Nascimento/diagnóstico , Resultado do TratamentoRESUMO
BACKGROUND: Cholinergic urticaria (CholU) is a common subtype of chronic inducible urticaria, where signs and symptoms (e.g. pruritic wheals and angioedema) are triggered by sweating due to physical exercise, passive warming and by other sweat-inducing situations. While guidelines recommend treatment with second-generation H1 antihistamines, approximately 90% of patients report uncontrolled disease. Targeting the histamine 4 receptor (H4R) has shown promise in preclinical/clinical studies of allergic/inflammatory diseases. Izuforant (LEO 152020) is a selective oral H4R antagonist with expected dual antipruritic and anti-inflammatory effects. OBJECTIVES: To assess the effects of izuforant in adults with CholU, a common type of chronic urticaria driven by histamine and characterized by high skin levels of H4R expression. METHODS: This was a phase IIa randomized double-blind placebo-controlled multicentre crossover trial where patients with CholU with an inadequate response to ≥ 1 standard dose of H1 antihistamine received izuforant 100â mg twice daily or placebo (EUCTR2020-004961-38-DE; NCT04853992). The primary endpoint was change from baseline in Urticaria Activity Score. Exploratory endpoints included CholU activity score over 7â days, urticaria control test, Physician Global Assessment, patient global assessment of severity (PGA-S), provocation tests, Dermatology Life Quality Index and CholU quality of life (CholU-QoL). Pharmacokinetic and pharmacodynamic parameters, and serum biomarkers were assessed, as well as safety and tolerability. RESULTS: Nineteen patients were randomized and included in the full analysis set; 18 completed treatment [mean (SD) age 29.5 (9.8) years; mean (SD) CholU duration 8.0 (6.3) years]. The primary and most of prespecified exploratory endpoints were not met; there were significant improvements in PGA-S for izuforant vs. placebo (P = 0.02), and nonsignificant improvements for other endpoints in quality of life and histamine skin prick test. All adverse events (AEs) experienced with izuforant were considered mild. The most frequently reported (> 1 patient) were nausea (three patients) and upper abdominal pain (two patients), occurring more frequently with izuforant vs. placebo (one patient each). There were no treatment-related serious AEs and no patient receiving izuforant discontinued the study. Treatment with izuforant did not cause downregulation of H4R. CONCLUSIONS: This is the first study to explore the role of H4R as a therapeutic target in urticaria. Targeting H4R with izuforant was well tolerated but did not demonstrate significant improvements vs. placebo in the primary endpoint and all but one prespecified exploratory endpoint in CholU.
Cholinergic urticaria (CholU) is a common subtype of an inflammatory skin condition called chronic inducible urticaria, where signs and symptoms (e.g. hives and swelling in the skin) are triggered by sweating caused by physical exercise, passive warming and other sweat-inducing situations. While guidelines recommend treatment with second-generation H1 antihistamines (a type of medication), approximately 90% of people with the condition report that these medications do not control the disease. Targeting the histamine 4 receptor (H4R) has shown promise in studies of allergic/inflammatory diseases. CholU is driven by histamine (a chemical released in the body) and characterized by high skin levels of H4R. Izuforant is a medication that may reduce itch and inflammation. In our study, which was carried out across multiple sites in Germany, we assessed the effects of izuforant 100â mg in 18 patients with CholU using a range of measures covering symptom control, disease severity, provocation response and quality of life. The primary endpoint (the main result measured at the end of the study to see if the treatment worked) was change from baseline in the post-provocation Urticaria Activity Score, where areas of skin were provoked and the time until common symptoms of CholU appeared (sweating and whealing (hives)) was measured. Overall, the primary endpoint and most of the exploratory endpoints were not met. There were significant improvements in patients' global assessment for izuforant versus placebo. This was the first study to explore the role of H4R as a therapeutic target in urticaria. Our findings suggest that targeting H4R with izuforant was well tolerated but did not demonstrate significant improvements versus placebo in the primary endpoint, and all but one prespecified exploratory endpoint in CholU.
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Urticária Crônica , Estudos Cross-Over , Receptores Histamínicos H4 , Humanos , Método Duplo-Cego , Adulto , Masculino , Feminino , Pessoa de Meia-Idade , Receptores Histamínicos H4/antagonistas & inibidores , Resultado do Tratamento , Urticária Crônica/tratamento farmacológico , Adulto Jovem , Antagonistas dos Receptores Histamínicos/administração & dosagem , Antagonistas dos Receptores Histamínicos/uso terapêutico , Antagonistas dos Receptores Histamínicos/efeitos adversos , Urticária/tratamento farmacológico , Qualidade de VidaRESUMO
BACKGROUND: Concerns regarding contact allergies and intolerance reactions to dental materials are widespread among patients. Development of novel dental materials and less frequent amalgam use may alter sensitization profiles in patients with possible contact allergy. OBJECTIVES: To analyse current sensitization patterns to dental materials in patients with suspected contact allergy. METHODS: This retrospective, multicentre analysis from the Information Network of Departments of Dermatology (IVDK) selected participants from 169 834 people tested in 2005-2019 and registered with (i) an affected area of 'mouth' (and 'lips'/'perioral'), (ii) with the dental material in question belonging to one of three groups (dental filling materials, oral implants or dentures or equivalents) and (iii) with patch-testing done in parallel with the German baseline series, (dental) metal series and dental technician series. RESULTS: A total of 2730 of 169 834 tested patients met the inclusion criteria. The patients were predominantly women (81.2%) aged ≥ 40â years (92.8%). The sensitization rates with confirmed allergic contact stomatitis in women (n = 444) were highest for metals (nickel 28.6%, palladium 21.4%, amalgam 10.9%), (meth)acrylates [2-hydroxyethyl methacrylate (HEMA) 4.8%] and the substances propolis (6.8%) and 'balsam of Peru' (11.4%). The most relevant acrylates were HEMA, 2-hydroxypropyl methacrylate, methyl methacrylate, ethylene glycol dimethacrylate and pentaerythritol triacrylate. Few men were diagnosed with allergic contact stomatitis (n = 68); sensitization rates in men were highest for propolis (14.9%) and amalgam (13.6%). CONCLUSIONS: Allergic contact stomatitis to dental materials is rare. Patch testing should not only focus on metals such as nickel, palladium, amalgam and gold, but also (meth)acrylates and the natural substances propolis and 'balsam of Peru'.
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Amálgama Dentário , Materiais Dentários , Dermatite Alérgica de Contato , Testes do Emplastro , Humanos , Feminino , Masculino , Estudos Retrospectivos , Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/etiologia , Dermatite Alérgica de Contato/epidemiologia , Dermatite Alérgica de Contato/imunologia , Adulto , Pessoa de Meia-Idade , Materiais Dentários/efeitos adversos , Amálgama Dentário/efeitos adversos , Idoso , Adolescente , Adulto Jovem , Criança , Metacrilatos/efeitos adversos , Bálsamos/efeitos adversos , Implantes Dentários/efeitos adversos , Estomatite/epidemiologia , Estomatite/induzido quimicamente , Estomatite/imunologia , Estomatite/diagnóstico , Estomatite/etiologia , Própole/efeitos adversos , Dentaduras/efeitos adversos , Alemanha/epidemiologia , Alérgenos/efeitos adversos , Alérgenos/imunologia , Pré-EscolarRESUMO
BACKGROUND: Chagas disease (ChD) is endemic in many parts of the world and can be transmitted through organ transplantation or reactivated by immunosuppression. Organs from infected donors are occasionally used for transplantation, and the best way of managing the recipients remains a subject of debate. METHODS: We present a single-center cohort study describing a 10-year experience of kidney transplantation in patients at risk of donor-derived ChD and or reactivation. Patients received prophylactic treatment with Benznidazole and were monitored for transmission or reactivation. Monitoring included assessing direct parasitemia, serology, and polymerase chain reaction (PCR). RESULTS: Fifty-seven kidney transplant recipients (KTRs) were enrolled in the study. Forty-four patients (77.2%) were at risk of primary ChD infection, nine patients (15.8%) were at risk of disease reactivation, and four patients (7.0%) were at risk of both. All patients received Benznidazole prophylaxis, starting on the first day after transplantation. Parasitemia was assessed in 51 patients (89.5%), serology also in 51 patients (89.5%), and PCR in 40 patients (70.2%). None of the patients exhibited clinically or laboratory-detectable signs of disease. A single patient experienced a significant side effect, a cutaneous rash with intense pruritus. At 1-year post-transplantation, the patient and graft survival rates were 96.5% and 93%, respectively. CONCLUSION: In this study, no donor-derived or reactivation of Trypanosoma cruzi infection occurred in KTRs receiving Benznidazole prophylaxis.
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BACKGROUND: Chronic kidney disease (CKD) is a global health problem with rising prevalence, morbidity, mortality, and associated costs. Early identification and risk stratification are key to preventing progression to kidney failure. However, there is a paucity of data on practice patterns of kidney function assessment to guide the development of improvement strategies, particularly in lower-income countries. METHODS: A retrospective observational analysis was conducted in a nationwide laboratory database in Brazil. We included all adult patients with at least one serum creatinine assessment between June 2018 and May 2021. Our primary objective was to determine the proportion of patients with estimated glomerular filtration rate (eGFR) evaluations accompanied by predicted levels of urinary albumin-to-creatinine ratio (pACR) assessments within 12 months. RESULTS: Out of 4,5323,332 serum creatinine measurements, 42% lacked pACR measurements within 12 months. Approximately 10.8% of tests suggested CKD, mostly at stage 3a. The proportion of serum creatinine exams paired with pACR assessment varied according to the CKD stage. Internal Medicine, Cardiology, and Obstetrics/Gynecology were the specialties requesting most of the creatinine tests. Nephrology contributed with only 1.1% of serum creatinine requests for testing. CONCLUSION: Our findings reveal that a significant proportion of individuals with a creatinine test lack an accompanying urinary albuminuria measurement in Brazil, contrary to the recommendations of the international guidelines. Non-Nephrologists perform most kidney function evaluations, even among patients with presumable advanced CKD. This highlights the urge to incorporate in clinical practice the early detection of CKD and to encourage more collaborative multidisciplinary care to improve CKD management.
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Albuminúria , Creatinina , Taxa de Filtração Glomerular , Insuficiência Renal Crônica , Humanos , Brasil/epidemiologia , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/fisiopatologia , Creatinina/sangue , Estudos Retrospectivos , Feminino , Masculino , Medição de Risco/métodos , Pessoa de Meia-Idade , Bases de Dados Factuais , Adulto , Testes de Função Renal/métodos , IdosoRESUMO
BACKGROUND: Cutaneous manifestations of drug-induced type IV reactions vary widely, with symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) being a less common presentation. Corticosteroids (CS), primarily known for their anti-inflammatory effects, rarely induce hypersensitivity reactions. OBJECTIVE: The aim of this case series is to report four cases of SDRIFE following systemic prednisolone therapy and to review existing CS classification proposals to better understand cross-reactivity of CS. PATIENTS/METHODS: Patients recruited at a German dermatology centre underwent allergologic evaluation including prick and patch testing with various CS. Positive cases underwent oral challenge testing with alternative agents. The classification systems of Coopman et al. and Baeck et al. were taken into account. DISCUSSION: Despite a paucity of literature, CS-induced type IV reactions do occur, including SDRIFE. Classification systems based on chemical structure provide insight into cross-reactivity patterns. Provocation tests with alternative CS highlight the complexity of managing CS hypersensitivity. CONCLUSION: SDRIFE may develop following systemic prednisolone therapy. Classification systems are helpful in understanding cross-reactivity and help in the selection of alternative preparations but are not always reliable. Individualised assessment is crucial for managing CS hypersensitivity, with consideration of alternative agents and emergency use of CS when necessary.
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Toxidermias , Exantema , Prednisolona , Humanos , Feminino , Toxidermias/etiologia , Toxidermias/diagnóstico , Exantema/induzido quimicamente , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Prednisolona/administração & dosagem , Masculino , Adulto , Reações Cruzadas , Idoso , Testes do Emplastro , Glucocorticoides/uso terapêutico , Glucocorticoides/administração & dosagemRESUMO
BACKGROUND: Mainly women work as foot care specialists (FCS). They are at risk to develop occupational dermatitis (OD). OBJECTIVES: The objective of this study is to describe the contact sensitisation pattern of female FCS with OD. METHODS: In a retrospective study, patch test and clinical data collected by the Network of Departments of Dermatology (IVDK) from 2008 to 2022 were analysed. Data of 116 female FCS with OD were compared with data of 13 930 female patients with OD working in other professions and 78 612 female patients without OD. RESULTS: Hand dermatitis (93.1%) was significantly more common and face dermatitis (0.9%) significantly less common in female FCS with OD compared to other female patients with or without OD. Frequent suspected allergen sources were disinfectants, gloves, leave-on and nail cosmetics. Occlusion and wetness were important co-factors. The most common diagnoses were irritant contact dermatitis (26.7%) and allergic contact dermatitis (21.6%). No sensitisation to any of the baseline series allergens was significantly more frequent in female FCS with OD than in the two control groups. However, sensitisations to allergens which FCS are abundantly exposed to, including fragrances, preservatives, rubber ingredients and disinfectants, were most common. CONCLUSIONS: FCS should be aware of the OD risk and prevention should be promoted.
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Dermatite Alérgica de Contato , Dermatite Irritante , Dermatite Ocupacional , Dermatoses da Mão , Testes do Emplastro , Humanos , Feminino , Dermatite Ocupacional/epidemiologia , Dermatite Ocupacional/etiologia , Dermatite Ocupacional/diagnóstico , Dermatite Alérgica de Contato/etiologia , Dermatite Alérgica de Contato/epidemiologia , Dermatite Alérgica de Contato/diagnóstico , Estudos Retrospectivos , Adulto , Pessoa de Meia-Idade , Estudos de Casos e Controles , Dermatoses da Mão/epidemiologia , Dermatoses da Mão/etiologia , Dermatite Irritante/epidemiologia , Dermatite Irritante/etiologia , Alérgenos/efeitos adversos , Dermatoses Faciais/epidemiologia , Dermatoses Faciais/etiologia , Cosméticos/efeitos adversos , Desinfetantes/efeitos adversos , Luvas Protetoras/efeitos adversos , Dermatoses do Pé/epidemiologiaRESUMO
BACKGROUND: Earlier studies suggested a potential association between tobacco smoking and nickel sensitization, but little is known about other contact allergens. OBJECTIVES: To investigate the association of smoking status and contact sensitizations as well as subtypes of dermatitis, and to analyse the sensitization profiles of tobacco smokers. PATIENTS AND METHODS: Within the Information Network of Departments of Dermatology (IVDK), we performed a cross-sectional multicentre pilot study comprising 1091 patch-tested patients from 9 departments, comparing 541 patients with a history of cigarette smoking (281 current and 260 former smokers) with 550 never-smokers. RESULTS: We could not confirm the previously reported association between nickel sensitization and tobacco smoking. Moreover, sensitizations to other allergens, including colophony, fragrance mix I, Myroxylon pereirae and formaldehyde, were not increased in cigarette smokers compared with never smokers. Hand dermatitis (50.6% vs. 33.6%) and occupational cause (36.2% vs. 22.5%) were significantly more frequent among cigarette smokers compared with never-smokers as shown by non-overlapping 95% confidence intervals. CONCLUSIONS: Although our study does not allow a firm conclusion on whether smoking status contributes to certain contact sensitizations, it confirms an association of smoking with hand dermatitis and occupational cause.
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Dermatite Alérgica de Contato , Dermatite Ocupacional , Testes do Emplastro , Humanos , Estudos Transversais , Projetos Piloto , Dermatite Alérgica de Contato/etiologia , Dermatite Alérgica de Contato/epidemiologia , Dermatite Alérgica de Contato/diagnóstico , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Dermatite Ocupacional/epidemiologia , Dermatite Ocupacional/etiologia , Dermatoses da Mão/epidemiologia , Dermatoses da Mão/etiologia , Níquel/efeitos adversos , Fumar Tabaco/efeitos adversos , Fumar Tabaco/epidemiologia , Alérgenos/efeitos adversos , IdosoRESUMO
Patch testing is the only clinically applicable diagnostic method for Type IV allergy. The availability of Type IV patch test (PT) allergens in Europe, however, is currently scarce. This severely compromises adequate diagnostics of contact allergy, leading to serious consequences for the affected patients. Against this background, the European Society of Contact Dermatitis (ESCD) has created a task force (TF) (i) to explore the current availability of PT substances in different member states, (ii) to highlight some of the unique characteristics of Type IV vs. other allergens and (iii) to suggest ways forward to promote and ensure availability of high-quality patch testing substances for the diagnosis of Type IV allergies throughout Europe. The suggestions of the TF on how to improve the availability of PT allergens are supported by the ESCD, the European Academy of Allergy and Clinical Immunology, and the European Academy of Dermatology and Venereology and intend to provide potential means to resolve the present medical crisis.
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Alérgenos , Dermatite Alérgica de Contato , Dermatite Ocupacional , Testes do Emplastro , Humanos , Testes do Emplastro/métodos , Europa (Continente) , Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/etiologia , Alérgenos/efeitos adversos , Dermatite Ocupacional/diagnóstico , Dermatite Ocupacional/etiologia , Sociedades Médicas , Comitês ConsultivosRESUMO
PURPOSE: To investigate the impact on caregivers of caring for a child with congenital upper extremity differences. METHODS: In this cross-sectional study, caregivers of patients enrolled in the multi-institutional Congenital Upper Limb Difference (CoULD) registry were contacted. Demographic information and the Impact on Family Scale (IOFS), a validated measure of perceived caregiver strain, were collected. Patient-reported outcome measures from the CoULD registry, the Pediatric Outcomes Data Collection Instrument (PODCI), and Patient-Reported Outcomes Measurement Information System (PROMIS) were also analyzed for correlation with IOFS. RESULTS: Two hundred ninety-nine caregivers participated. Factors with significantly stronger impact on family included public insurance; bilateral upper extremity involvement; household income of $20,000-40,000; additional musculoskeletal diagnosis; and a single adult caregiver household. There was a significantly increased subcategory of IOFS-Finance score for distant travel to see the surgeon. Additionally, all categories of the PODCI (upper extremity, mobility, sports, pain, happiness, and global) demonstrated a negative correlation with IOFS. PROMIS upper extremity and peer relations also demonstrated an inverse relationship with IOFS, whereas PROMIS pain interference had a positive correlation with IOFS. The overall IOFS for children with CoULDs was greater than previously reported for children with brachial plexus birth injury, and less than cerebral palsy and congenital heart disease. CONCLUSIONS: Caregivers of children with congenital upper extremity differences report a significant impact on family life. Socioeconomic factors, such as economically disadvantaged or single-caregiver households, and clinical factors, such as bilateral upper extremity involvement, correlate with greater family impact. These findings represent opportunities to identify at-risk families and underscore the importance of caring for the whole family through a multidisciplinary approach. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic II.
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Cuidadores , Deformidades Congênitas das Extremidades Superiores , Humanos , Estudos Transversais , Feminino , Masculino , Criança , Adulto , Cuidadores/psicologia , Pré-Escolar , Sistema de Registros , Adolescente , Medidas de Resultados Relatados pelo Paciente , Lactente , Pessoa de Meia-Idade , Extremidade Superior , Sobrecarga do Cuidador/psicologiaRESUMO
PURPOSE: The purpose of this study was to investigate whether radiographs can be used to aid in the determination of Blauth IIIA and IIIB thumbs. METHODS: Six pediatric hand surgeons were asked to evaluate the radiographs of 77 thumbs and classify the thumb as IIIA or IIIB and indicate which morphologic features influenced their decision. Quantitative measurements and ratios of radiographs were obtained and compared between IIIA and IIIB thumbs. RESULTS: The radiographic features selected for type IIIA thumbs include near-normal length and near-normal width and for type IIIB thumbs, abnormally short, tapered proximal end, and round proximal end. The six surveyed surgeons reached consensus in 82% (63/77) of thumbs, and this matched the enrolling surgeon's classification in 77% (59/77) cases. The ratio of the length of the thumb metacarpal compared with the length of the index metacarpal was different between IIIA and IIIB thumbs (66% ± 0.08% and 46% ± 0.18%, respectively). The ratio of the width of the thumb metacarpal shaft at its narrowest aspect to the width of the thumb metacarpal base was notably different between IIIA and IIIB (68% ± 0.13% and 95% ± 0.28%, respectively). CONCLUSIONS: Near-normal length and near-normal width of the metacarpal were used to predict IIIA and abnormally short, abnormally narrow, and a round or tapered base of the metacarpal were used to predict IIIB classification. The length of the thumb metacarpal relative to the index metacarpal is on average 66% of the length of the index metacarpal in IIIA thumbs compared with 46% in IIIB thumbs. The width of the shaft of the thumb metacarpal at its narrowest is 68% of the width of the thumb metacarpal base in IIIA thumbs, indicating a flared base. In IIIB thumbs, the shaft width was on average 95% of the base width, indicating a tapered base. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic level III.
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BACKGROUND: Concern about disease exacerbations and fear of reactions after coronavirus disease 2019 (COVID-19) vaccinations are common in chronic urticaria (CU) patients and may lead to vaccine hesitancy. OBJECTIVE: We assessed the frequency and risk factors of CU exacerbation and adverse reactions in CU patients after COVID-19 vaccination. METHODS: COVAC-CU is an international multicenter study of Urticaria Centers of Reference and Excellence (UCAREs) that retrospectively evaluated the effects of COVID-19 vaccination in CU patients aged ≥18 years and vaccinated with ≥1 dose of any COVID-19 vaccine. We evaluated CU exacerbations and severe allergic reactions as well as other adverse events associated with COVID-19 vaccinations and their association with various CU parameters. RESULTS: Across 2769 COVID-19-vaccinated CU patients, most (90%) received at least 2 COVID-19 vaccine doses, and most patients received CU treatment and had well-controlled disease. The rate of COVID-19 vaccination-induced CU exacerbation was 9%. Of 223 patients with CU exacerbation after the first dose, 53.4% experienced recurrence of CU exacerbation after the second dose. CU exacerbation most often started <48 hours after vaccination (59.2%), lasted for a few weeks or less (70%), and was treated mainly with antihistamines (70.3%). Factors that increased the risk for COVID-19 vaccination-induced CU exacerbation included female sex, disease duration shorter than 24 months, having chronic spontaneous versus inducible urticaria, receipt of adenovirus viral vector vaccine, having nonsteroidal anti-inflammatory drug/aspirin intolerance, and having concerns about getting vaccinated; receiving omalizumab treatment and Latino/Hispanic ethnicity lowered the risk. First-dose vaccine-related adverse effects, most commonly local reactions, fever, fatigue, and muscle pain, were reported by 43.5% of CU patients. Seven patients reported severe allergic reactions. CONCLUSIONS: COVID-19 vaccination leads to disease exacerbation in only a small number of CU patients and is generally well tolerated.
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COVID-19 , Urticária Crônica , Urticária , Humanos , Feminino , Adolescente , Adulto , Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Estudos Retrospectivos , Urticária/tratamento farmacológico , Vacinação/efeitos adversosRESUMO
BACKGROUND AND OBJECTIVES: Outdoor workers are at increased risk of developing non-melanoma skin cancer. We aimed to address the lack of validated German-language measurement instruments for outdoor workers' sun safety behavior and knowledge by compiling and validating two questionnaires. PARTICIPANTS AND METHODS: By expert consensus, items for the assessment of protective behavior (OccuSun) were compiled based on existing instruments. For knowledge, a translation of the Skin Cancer and Sun Knowledge (SCSK) scale was selected. After a pre-test, a validation study including 68 outdoor workers (62% female) was conducted in 2020. RESULTS: The retest reliability was r = 0.93 (95% confidence interval: 0.86-0.96) for the protection score and rs = 0.78 (0.67-0.86) for the knowledge score. Protective behaviors were correlated with respective diary data (0.38 ≤ rs ≤ 0.74, p < 0.001) and skin pigmentation changes (-0.23 ≥ rs ≥ -0.42, 0.007 ≤ p ≤ 0.165) but not with self-reported sunburn frequency (0.21 ≥ rs ≥ -0.04). CONCLUSIONS: Among German outdoor workers, two questionnaires for the assessment of sun protection behavior (OccuSun) and knowledge (SCSK) demonstrated good reliability. The OccuSun had generally good validity. Both instruments are fit for subsequent validation to determine their sensitivity to change.
Assuntos
Conhecimentos, Atitudes e Prática em Saúde , Neoplasias Cutâneas , Queimadura Solar , Protetores Solares , Humanos , Feminino , Inquéritos e Questionários , Masculino , Reprodutibilidade dos Testes , Neoplasias Cutâneas/prevenção & controle , Adulto , Queimadura Solar/prevenção & controle , Protetores Solares/uso terapêutico , Comportamentos Relacionados com a Saúde , Alemanha , Pessoa de Meia-Idade , Luz Solar/efeitos adversos , Exposição Ocupacional/prevenção & controleRESUMO
BACKGROUND AND OBJECTIVES: Outdoor workers are at increased risk of developing non-melanoma skin cancer. We aimed to address the lack of validated German-language measurement instruments for outdoor workers' sun safety behavior and knowledge by compiling and validating two questionnaires. PARTICIPANTS AND METHODS: By expert consensus, items for the assessment of protective behavior (OccuSun) were compiled based on existing instruments. For knowledge, a translation of the Skin Cancer and Sun Knowledge (SCSK) scale was selected. After a pre-test, a validation study including 68 outdoor workers (62% female) was conducted in 2020. RESULTS: The retest reliability was r = 0.93 (95% confidence interval: 0.86-0.96) for the protection score and rs = 0.78 (0.67-0.86) for the knowledge score. Protective behaviors were correlated with respective diary data (0.38 ≤ rs ≤ 0.74, p < 0.001) and skin pigmentation changes (-0.23 ≥ rs ≥ -0.42, 0.007 ≤ p ≤ 0.165) but not with self-reported sunburn frequency (0.21 ≥ rs ≥ -0.04). CONCLUSIONS: Among German outdoor workers, two questionnaires for the assessment of sun protection behavior (OccuSun) and knowledge (SCSK) demonstrated good reliability. The OccuSun had generally good validity. Both instruments are fit for subsequent validation to determine their sensitivity to change.
RESUMO
This S3 guideline was created based on the European S3 guideline, with special consideration of the medical conditions in the German-speaking region and incorporating additions from the previous German-language version. The interdisciplinary guideline commission consisted of representatives from the German Dermatological Society, the Professional Association of German Dermatologists, the Austrian Society of Dermatology and Venereology, the Swiss Society of Dermatology and Venereology, the German Society for Allergology and Clinical Immunology, the German Society for Pediatric and Adolescent Medicine, the Professional Association of Pediatricians and Adolescent Medicine, the Society for Pediatric Allergology and Environmental Medicine, the German Society for Pediatric Rehabilitation and Prevention, the German Society for Psychosomatic Medicine and Medical Psychotherapy, the German Network for Health Services Research, the German Eczema Association and the German Allergy and Asthma Association. This first part of the guideline focuses on the definition and diagnostic aspects of atopic dermatitis (AD), addressing topical therapy as well as non-pharmacological treatment approaches such as UV therapy, psychoeducational therapy, dietary interventions for AD, allergen immunotherapy for AD, and complementary medicine. This part of the guideline also covers specific aspects of AD in children and adolescents, during pregnancy and lactation, and in the context of family planning. Additionally, it addresses occupational aspects of AD and highlights the perspective of the patients. The second part of the guideline, published separately, addresses the systemic therapy of AD.
Assuntos
Asma , Dermatite Atópica , Adolescente , Feminino , Gravidez , Humanos , Criança , Dermatite Atópica/terapia , Dermatite Atópica/tratamento farmacológicoRESUMO
The present S3 guideline was created based on the European English-language S3 guideline, with special consideration given to the medical conditions in the German-speaking region, and with additions from the previous German-language version, in accordance with the criteria of the AWMF. This second part of the guideline addresses the systemic therapy of atopic dermatitis (AD). It covers topics such as the indication for systemic therapy in children, adolescents, and adult patients with AD. Furthermore, it addresses all medications approved for AD, such as the biologics dupilumab and tralokinumab, the Janus kinase inhibitors abrocitinib, baricitinib, and upadacitinib, as well as conventional immunosuppressive therapies with systemic glucocorticosteroids and ciclosporin. Additionally, it discusses systemic off-label therapies. The first part of the guideline, published separately, includes the definition and diagnostic aspects of AD, describes topical therapy, non-drug therapy approaches, and addresses aspects related to special patient groups.
Assuntos
Produtos Biológicos , Dermatite Atópica , Adolescente , Adulto , Criança , Humanos , Dermatite Atópica/diagnóstico , Dermatite Atópica/tratamento farmacológico , Administração Cutânea , Ciclosporina , Terapia de Imunossupressão , Resultado do TratamentoRESUMO
OBJECTIVE: Pancreatic ductal adenocarcinoma (PDAC) is a lethal malignancy. Differentiation from chronic pancreatitis (CP) is currently inaccurate in about one-third of cases. Misdiagnoses in both directions, however, have severe consequences for patients. We set out to identify molecular markers for a clear distinction between PDAC and CP. DESIGN: Genome-wide variations of DNA-methylation, messenger RNA and microRNA level as well as combinations thereof were analysed in 345 tissue samples for marker identification. To improve diagnostic performance, we established a random-forest machine-learning approach. Results were validated on another 48 samples and further corroborated in 16 liquid biopsy samples. RESULTS: Machine-learning succeeded in defining markers to differentiate between patients with PDAC and CP, while low-dimensional embedding and cluster analysis failed to do so. DNA-methylation yielded the best diagnostic accuracy by far, dwarfing the importance of transcript levels. Identified changes were confirmed with data taken from public repositories and validated in independent sample sets. A signature of six DNA-methylation sites in a CpG-island of the protein kinase C beta type gene achieved a validated diagnostic accuracy of 100% in tissue and in circulating free DNA isolated from patient plasma. CONCLUSION: The success of machine-learning to identify an effective marker signature documents the power of this approach. The high diagnostic accuracy of discriminating PDAC from CP could have tremendous consequences for treatment success, once the result from still a limited number of liquid biopsy samples would be confirmed in a larger cohort of patients with suspected pancreatic cancer.
Assuntos
Carcinoma Ductal Pancreático , Neoplasias Pancreáticas , Pancreatite Crônica , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia , Pancreatite Crônica/diagnóstico , Pancreatite Crônica/genética , Carcinoma Ductal Pancreático/diagnóstico , Carcinoma Ductal Pancreático/genética , Metilação de DNA , DNA , Biomarcadores Tumorais/genética , Neoplasias PancreáticasRESUMO
Long-acting injectable (LAI) formulations can provide several advantages over the more traditional oral formulation as drug product opportunities. LAI formulations can achieve sustained drug release for extended periods of time, which results in less frequent dosing requirements leading to higher patient adherence and more optimal therapeutic outcomes. This review article will provide an industry perspective on the development and associated challenges of long-acting injectable formulations. The LAIs described herein include polymer-based formulations, oil-based formulations, and crystalline drug suspensions. The review discusses manufacturing processes, including quality controls, considerations of the Active Pharmaceutical Ingredient (API), biopharmaceutical properties and clinical requirements pertaining to LAI technology selection, and characterization of LAIs through in vitro, in vivo and in silico approaches. Lastly, the article includes a discussion around the current lack of suitable compendial and biorelevant in vitro models for the evaluation of LAIs and its subsequent impact on LAI product development and approval.