Course of the disease

  • 文章类型: Journal Article
    这份共识文件分析了Gender(广泛性脓疱型银屑病)患者的管理和情绪历程,以及疾病的理想病程,同时检测关键点并将其转化为需求和建议。该项目分三个阶段进行,咨询委员会(n=8)参与,一个专家小组(n=15)和一些患者(n=6)。由于疾病变化,患者的疾病进展是异质的,实施不同的医疗保健模式和可用资源,以及缺乏诊断和治疗指南。总共提出了45种不同的建议,以优化管理并解决这些患者的情绪问题。其中五个因其影响力和生存能力而脱颖而出。因此,已普遍制定了优先事项路线图,以改善对3GPP患者的管理.
    This consensus document analyzed the management and emotional journey of patients with GPP (generalized pustular psoriasis), and the desirable course of the disease while detecting critical points and translating them into needs and recommendations. This project was conducted in 3 phases with participation from an advisory committee (n=8), an expert panel (n=15) and patients with GPP (n=6). The patients\' disease progression was heterogeneous due to disease variations, different health care models implemented and available resources, and the lack of diagnostic and treatment guidelines. A total of 45 different recommendations have been made to optimize management and address the emotional component of these patients. Five of them stand out for their impact and viability. Therefore, a roadmap of priorities has been made generally available to improve the management of patients with GPP.
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  • 文章类型: Journal Article
    这份共识文件分析了Gender(广泛性脓疱型银屑病)患者的管理和情绪历程,以及疾病的理想病程,同时检测关键点并将其转化为需求和建议。该项目分三个阶段进行,咨询委员会(n=8)参与,一个专家小组(n=15)和一些患者(n=6)。由于疾病变化,患者的疾病进展是异质的,实施不同的医疗保健模式和可用资源,以及缺乏诊断和治疗指南。总共提出了45种不同的建议,以优化管理并解决这些患者的情绪问题。其中五个因其影响力和生存能力而脱颖而出。因此,已普遍制定了优先事项路线图,以改善对3GPP患者的管理.
    This consensus document analyzed the management and emotional journey of patients with GPP (generalized pustular psoriasis), and the desirable course of the disease while detecting critical points and translating them into needs and recommendations. This project was conducted in 3 phases with participation from an advisory committee (n=8), an expert panel (n=15) and patients with GPP (n=6). The patients\' disease progression was heterogeneous due to disease variations, different health care models implemented and available resources, and the lack of diagnostic and treatment guidelines. A total of 45 different recommendations have been made to optimize management and address the emotional component of these patients. Five of them stand out for their impact and viability. Therefore, a roadmap of priorities has been made generally available to improve the management of patients with GPP.
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  • 文章类型: Journal Article
    背景:反复注射A型或单乳毒素(aboBoNT/A,onaBoNT/A)可能导致中和抗体(NAB)的诱导和/或继发性治疗失败(STF)。NAB与STF之间的关系尚不清楚。
    目的:证明STF患者在接受或接受前BoNT/A治疗后,当转换为A型incobotulinumtoxin(incoBoNT/A)时,可以观察到显着的改善,而在NAB阳性患者中,没有STF的前或前BoNT/A治疗可以继续进行,而不会显着恶化。
    方法:在60例宫颈肌张力障碍(CD)和STF患者中,分析了小鼠隔膜测定(MHDA)的麻痹时间(PT)和临床结果(TSUI评分)。治疗(STF组),这些患者改用了A型incoBoNT毒素(incoBoNT/A)。将这些数据与仅接受incoBoNT/A(INCO组)治疗的34例患者进行比较。此外,对9例NAB但没有STF的患者进行了为期7年的PT和TSUI评分的随访,这些患者被转换为inco-BoNT/A(SWI组),其他9例NAB患者仍在进行先前的BoNT/A准备(NO-SWI组)。
    结果:在STF组中,切换到incoBoNT/A后,可以检测到TSUI评分的显着改善。这种改善不如INCO组明显。SWI和NO-SWI组之间的长期结果没有显着差异。
    结论:最佳策略是避免诱导NAB。转换为incoBoNT/A可能导致STF患者的改善。然而,在一些没有STF的NAB患者中,BoNT/A-治疗可以继续而没有显著恶化。
    BACKGROUND: Repeated injections with abo- or onabotulinumtoxin type A (aboBoNT/A, onaBoNT/A) may lead to induction of neutralizing antibodies (NABs) and/or a secondary treatment failure (STF). The relation between NABs and STF is still unclear.
    OBJECTIVE: To demonstrate that a significant improvement can be observed in patients with STF after abo- or onaBoNT/A-treatment when switched to incobotulinumtoxin type A (incoBoNT/A) and that in NAB-positive patients without STF abo- or onaBoNT/A-treatment can be continued without significant worsening.
    METHODS: Paralysis times (PT) of the mouse hemidiaphragm assay (MHDA) and clinical outcome (TSUI-score) was analyzed in 60 patients with cervical dystonia (CD) and STF after abo- or onaBoNT/A-treatment (STF-group) who were switched to incobotulinumtoxin type A (incoBoNT/A). These data were compared to those of 34 patients who were exclusively treated with incoBoNT/A (INCO-group). Furthermore, PTs and TSUI-scores were followed up over 7 years in 9 patients with NABs but without STF who were switched to inco-BoNT/A (SWI-group) and 9 other patients with NABs who remained on their previous BoNT/A preparation (NO-SWI-group).
    RESULTS: In the STF-group, a significant improvement of TSUI-scores could be detected after switch to incoBoNT/A. This improvement was less pronounced than in the INCO-group. There was no significant difference in long-term outcome between the SWI- and NO-SWI-group.
    CONCLUSIONS: The best strategy is to avoid the induction of NABs. A switch to incoBoNT/A may lead to improvement in patients with STF. However, in some patients with NABs without STF, BoNT/A-treatment can be continued without significant worsening.
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  • 文章类型: Case Reports
    Introduction: In 2010, dampness damage in a single-family house caused a massive mold infestation. In the further course, the 5 family members developed severe health problems. This report investigates the extent and cause of the water damage. In addition, the various visible fungal infestations were analyzed in a specialized laboratory. Results: Due to building construction errors, starting from the basement, an increased moisture penetration of the residential building was detected. Within 2 years, massive mold infestation occurred. In 2016, the following species were detected: Cladosporium sphaerospermum, Chaetomium globosum, Penicillium chrysogenum, Scopularis brevicaulis, Acremonium furculum, A. charticola and A. sclerotigenum, Trichomonascus apis Aspergillus versicolor and Debaryomyces hansenii. Additionally, different black molds were macroscopically detected. The severity of the disease process varied, probably due to the different daily exposure of the family members, and possibly influenced by age. The children presented acute episodes with nocturnal cough, associated with sleep disturbances and respiratory infections with severe rhinitis. In addition, general fatigue was noticeable. The course of the disease was complicated by recurrent nightly nosebleeds. The mother developed a much more severe course as chronic fatigue syndrome. Additionally, the following continuous complaints occurred: sore throat and headache, nocturnal irritable cough, chronic rhinitis, difficulty concentrating, increasing forgetfulness and word-finding disorders, cognitive impairment with reduced short-term memory, extremely dry eyes with red sclerae, morning stiffness, dyspnea, disturbed temperature regulation (chills), increased feeling of thirst, and menstrual disorders. The father\'s building-related illness (BRI) was comparatively mild due to much lower exposure, with nocturnal irritable cough, rhinitis, and marked fatigue. In 2018, after moving out of the house, the father was symptom-free after 2 weeks, the three children after 6 months, but the mother only after 18 months. Discussion: The symptoms are consistent with reports from the literature, according to which fatigue, sleep disturbances, lack of concentration and headache as well as recurrent infections of the upper respiratory tract are caused by microbial volatile organic compounds (MVOCs) released by molds. The association with recurrent nosebleeds in childhood has not been described in this form before. Conclusion: Since in all family members complete remission of symptoms occurred after cessation of the 6-year exposure, there is no doubt that the BRI was caused by the massive mold infestation.
    Einleitung: Im Jahr 2010 kam es auf Grund eines Feuchteschadens in einem Wohnhaus zu massivem Schimmelpilzbefall und im weiteren Verlauf bei der 5-köpfigen Familie zu zunehmenden gesundheitlichen Beschwerden. Aus diesem Anlass sollte eine Ursachenklärung mit Ermittlung des Ausmaßes des Wasserschadens und Differenzierung des optisch sichtbaren Pilzbefalls durchgeführt werden. Ergebnisse: Es konnte ausgehend vom Kellergeschoss eine baulich bedingte zunehmende Durchfeuchtung des Wohnhauses nachgewiesen werden. Innerhalb von 2 Jahren kam es zu massivem Schimmelpilzbefall. Im Jahr 2016 wurden folgende Species nachgewiesen: Cladosporium sphaerospermum, Chaetomium globosum, Penicillium chrysogenum, Scopularis brevicaulis, Acremonium furculum, A. charticola, d A. sclerotigenum, Trichomonascus apis Aspergillus versicolor und Debaryomyces hansenii. Zusätzlich waren makroskopisch verschiedene Schwarzschimmelspecies vertreten. Die Erkrankung war unterschiedlich schwer ausgeprägt, vermutlich bedingt durch die unterschiedliche tägliche Exposition der Familienmitglieder und möglicherweise altersabhängig beeinflusst. Bei den Kindern traten die Beschwerden in Form akuter Schübe mit nächtlichem Husten, verbunden mit Schlafstörungen und respiratorischen Infektionen mit starker Rhinitis auf. Zusätzlich war eine generelle Ermüdung auffällig. Der Krankheitsverlauf wurde durch nächtlich auftretendes rezidivierendes Nasenbluten kompliziert. Bei der Mutter entwickelte sich eine deutlich schwerere Verlaufsform in Form eines Chronic Fatigue Syndroms. Zusätzlich traten folgende Dauerbeschwerden auf: Hals- und Kopfschmerzen, nächtlicher Reizhusten, chronische Rhinitis, Konzentrationsschwierigkeiten, zunehmende Vergesslichkeit und Wortfindungsstörungen, kognitive Einschränkungen mit reduziertem Kurzzeitgedächtnis, extrem trockene Augen mit roten Skleren, morgendliche Steifigkeit, Dyspnoe, gestörte Temperaturregulierung (Frösteln), vermehrtes Durstgefühl und Störungen der Menstruation. Beim Vater war das BRI wegen deutlich geringerer Exposition vergleichsweise gering ausgeprägt mit nächtlichem Reizhusten, Rhinitis und ausgeprägter Müdigkeit. Nach dem Wechsel der Wohnung waren der Vater nach 2 Wochen, die drei Kinder nach 6 Monaten und die Mutter erst nach 18 Monaten beschwerdefrei. Diskussion: Das Beschwerdebild deckt sich mit Berichten aus der Literatur, wonach durch von Schimmelpilzen freigesetzte MVOCs Müdigkeit, Schlafstörungen, Konzentrationsschwäche und Kopfschmerz sowie rezidivierende Infektionen der oberen Atemwege verursacht werden. Die Vergesellschaftung mit rezidivierendem Nasenbluten im Kindesalter ist in dieser Form bisher nicht beschrieben. Schlussfolgerung: Da es nach der Unterbrechung der insgesamt über 6 Jahre währenden Exposition zur kompletten Remission der Beschwerden kam, gibt es keinen Zweifel daran, dass die BRI durch den massiven Schimmelpilzbefall ausgelöst worden ist.
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  • 文章类型: Journal Article
    OBJECTIVE: To evaluate the outcome of chronic hepatitis B (CHB) in children with or without malignancies.
    METHODS: Twenty four children (15 boys and 9 girls) with malignancies, followed up by the pediatric gastroenterology outpatient clinic for CHB between January 2000 and December 2013, were enrolled in the study (Group 1). Group 2 was formed with twenty five children (11 girls and 14 boys) diagnosed with CHB without malignancies. The data from the patients\' records were compared between the two groups.
    RESULTS: Hepatitis B e antigen (HBeAg)/antiHBe seroconversion was observed in 3 patients (12.5%) in group 1 and 15 patients (60%) in group 2, with annual seroconversion rates of 1.61% and 16.6%, respectively, and the difference was significant (P<0.01). One patient (6.6%) in Group 1 and 9 patients (53%) in Group 2 showed HBeAg/antiHBe seroconversion after treatment and the difference between the two groups was significant (P<0.06) Loss of hepatitis B surface antigen was observed in one patient in each of group 1 and 2. No clinical, laboratory and imaging findings of liver disease were observed in any of the patients at the end of the study.
    CONCLUSIONS: HBeAg/antiHBe seroconversion rate was lower in patients who had recovered from cancer.
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