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  • 文章类型: Review
    担子菌病是由担子菌属引起的罕见真菌感染。在有免疫能力的儿童中,它通常会引起皮肤感染,很少影响胃肠道,这种疾病的传播极为罕见。本研究报告了第一例由OmanensisBasidiobolusomanensis引起的播散性担子菌病,该儿童患有急性淋巴细胞白血病,尽管接受了L-AMB和伏立康唑的手术和抗真菌治疗,但由于不受控制的感染和多器官衰竭而死亡。文献回顾产生了76例,包括目前的病例,其中大部分报告为侵袭性胃肠道感染。平均年龄为4岁(61名男性和15名女性),这些儿童中的大多数来自中东(80%),特别是沙特阿拉伯(45%)。大多数患者接受全身抗真菌药物(主要是伊曲康唑和两性霉素B)治疗。这些患者中有25%进行了手术干预,死亡率为12%。
    Basidiobolomycosis is an uncommon fungal infection caused by the genus Basidiobolus. In immunocompetent children, it usually causes cutaneous infection and rarely affects the gastrointestinal tract, and it is extremely rare for the disease to spread. The present study reports the first case of disseminated basidiobolomycosis caused by Basidiobolus omanensis in a child with acute lymphoblastic leukemia who died as a result of uncontrolled infection and multi-organ failure despite surgical and antifungal therapy with L-AMB and voriconazole. A review of the literature yielded 76 cases, including the current case with the majority of which were reported as invasive gastrointestinal infection. The median age was 4 years (61 male and 15 female) and the majority of these children were from the Middle East (80%), specifically Saudi Arabia (45%). Most patients were treated with systemic antifungal agents (mostly itraconazole and amphotericin B). Surgical intervention was done in 25% of these patients and the death rate was 12%.
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  • 文章类型: Case Reports
    化脓性肉芽肿(PG)是一种常见的获得性血管肿瘤,主要表现为面部孤立性丘疹结节病变,树干,和四肢。虽然PG的病因尚不清楚,创伤,感染,荷尔蒙可能会发挥作用。多个播散的PG是一种非常罕见的形式,大多数是在烧伤等创伤后看到的。我们为一名患者提供了继发于燃油燃烧的多个PG。以前没有因油烫伤引起PG的报道。我们还回顾了英文文献,发现了其他24例播散的PG,其中大多数是煮后牛奶。
    Pyogenic granuloma (PG) is a common acquired vascular tumor and may appear mostly as a solitary papulonodular lesion on the face, trunk, and extremities. Although the etiology of PG is unclear, trauma, infections, and hormones may play a role. Multiple disseminated PGs are a very rare form and mostly are seen after traumas such as burn. We presented a patient with multiple PGs secondary to oil burning. There has been no report of PG caused by scald burn due to oil before. We also reviewed the literature in English and found 24 other cases of disseminated PG that most of them developed post-boiling milk.
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  • 文章类型: Journal Article
    结核病相关的噬血细胞性淋巴组织细胞增生症(HLH)在儿科中很少见,如果不及时识别和治疗,可能会致命。描述了患有结核病和HLH的3个月大婴儿。他成功地接受了抗结核治疗(ATT),包括异烟肼,利福平,吡嗪酰胺,乙胺丁醇,链霉素和地塞米松(10mg/m2/天)。在治疗的第28天,他对ATT产生了矛盾的升级反应,为此他再次接受了(口服)皮质类固醇治疗4周.他成功康复,现在完全康复,无症状。据我们所知,这是首例儿童在接受TB-HLH治疗后出现矛盾升级反应的病例.缩写ATT:抗结核治疗;CB-NAAT:基于药筒的核酸扩增试验;CECT:对比增强计算机断层扫描;HLH:噬血细胞淋巴组织细胞增多症;NK:自然杀伤,PUR:矛盾的升级反应;sHLH:次级HLH。
    Tuberculosis-associated haemophagocytic lymphohistiocytosis (HLH) is rare in paediatrics and can be fatal if not recognised and treated on time. A 3-month-old infant with tuberculosis and HLH is described. He was successfully treated with anti-tuberculous therapy (ATT) which comprised isoniazid, rifampicin, pyrazinamide, ethambutol, streptomycin and dexamethasone (10 mg/m2/day). On Day 28 of therapy, he developed a paradoxical upgrading reaction to ATT for which he was again treated with (oral) corticosteroids for 4 weeks. He recovered successfully and is now completely well and asymptomatic. To the best of our knowledge, this is the first case of a child having a paradoxical upgrading reaction following treatment for TB-HLH.Abbreviations ATT: anti-tuberculous therapy; CB-NAAT: cartridge-based nucleic acid amplification test; CECT: contrast-enhanced computed tomography; HLH: haemophagocytic lymphohistiocytosis; NK: natural killer, PUR: paradoxical upgrading reaction; sHLH: secondary HLH.
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  • 文章类型: Review
    背景:脉络丛肿瘤是由脉络丛引起的罕见神经外胚层肿瘤。脉络丛乳头状瘤(CPP)代表这些类型肿瘤的最低等级,并具有WHOI级名称。尽管他们典型的低等级,一些CPPs可以表现出攻击行为,包括实质浸润和在整个神经轴传播。由于它们与脉络丛有关联,CPP患者通常表现为脑积水和颅内压升高的体征和症状.
    方法:一名2岁男性,表现为急性脑积水和癫痫发作。发现他的左心室内肿块很大,在整个神经轴上都有无数的实质内和轴外囊肿。文献综述显示,有5例类似的播散性CPP病例,有无数病变。这是报道的最年轻的播散性CPP患者,也是第一位患有多个压迫性病变的患者。在颅骨切除和胸减压后,患者的病变保持稳定(随访2年)。截至2021年3月,使用搜索词[\“播散性脉络丛乳头状瘤\”或\“脉络丛乳头状瘤\”或\“转移性脉络丛乳头状瘤\”]对PubMed/Medline数据库进行了文献检索。然后对文章进行了类似的患者影像学表现和组织学诊断筛选。为了减轻出版偏见,参考文章用于确定其他病例报告和病例系列。
    结论:我们描述了一例罕见的侧脑室CPP,广泛的软脑膜播散导致急性梗阻性脑积水和压迫性脊髓病,需要脑脊液改道和颅内切除,然后进行胸椎减压。此病例报告有助于扩大对播散性CPP的了解,并鼓励对脉络丛肿瘤进行完整的神经轴成像。此外,我们提出了一种包括射线照相特征的命名范式改进。
    BACKGROUND: Choroid plexus tumors are rare neuroectodermal tumors that arise from the choroid plexus. Choroid plexus papillomas (CPPs) represent the lowest grade of these types of tumors and have a WHO grade I designation. Despite their typical low grade, some CPPs can exhibit aggressive behaviors including parenchymal invasion and dissemination throughout the neuro-axis. Due to their association with the choroid plexus, patients with CPP commonly present with signs and symptoms of hydrocephalus and increased intracranial pressure.
    METHODS: A 2-year-old male presented in extremis with acute hydrocephalus and seizure. He was found to have a large left intraventricular mass with innumerable intraparenchymal and extra-axial cysts throughout his neuro-axis. A literature review revealed five similar disseminated CPP cases with innumerable lesions. This is the youngest reported patient with disseminated CPP and the first with multiple compressive lesions. Following cranial resection and thoracic decompression, the patient\'s lesions have remained stable (2 years of follow-up). A literature search of the PubMed/Medline databases was performed using the search terms [\"disseminated choroid plexus papilloma\" OR \"choroid plexus papilloma\" OR \"metastatic choroid plexus papilloma\"] up to March 2021. Articles were then screened for similar patient radiographic presentation and histological diagnosis. To mitigate publication bias, referenced articles were utilized to identify other case reports and case series.
    CONCLUSIONS: We describe a rare case of a lateral ventricle CPP with widespread leptomeningeal dissemination causing acute obstructive hydrocephalus and compressive myelopathy requiring cerebrospinal fluid diversion and intracranial resection followed by thoracic spine decompression. This case report serves to broaden knowledge of disseminated CPP and to encourage complete neuro-axis imaging for choroid plexus tumors. Additionally, we propose a naming paradigm refinement that includes radiographic characteristics.
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  • 文章类型: Journal Article
    多器官/播散性包虫病在儿童时期很少见。我们旨在评估患有多器官/播散性包虫病的儿科患者的临床和实验室特征以及预后。
    我们回顾性评估了累及三个或更多器官的包虫病患儿。
    13名患者被纳入研究。中位年龄为120(范围71-189)个月。三个(23%)是偶然诊断的。5例(38.4%)患者出现腹痛,4人呕吐(30.7%),头痛3例(23%),咳嗽2例(15.3%),1例腹股沟疼痛(7.6%),1例(7.6%)有黄疸,1例(7.6%)有发热。投诉的中位持续时间为48(0-140)天。最常见的三部分器官为38.4%(5/13)肝脏,肺和脾。在两名患者中检测到孤立的腹部播散。两名患者多器官受累,多个囊肿伴播散。在3例患者中观察到囊肿破裂;复发性尿路感染,输尿管肾积水,继发性腹膜炎伴腹内脓肿,胆道瘘各观察1例。3例(23%)患者出现复发。
    棘球蚴病是一种生长缓慢且复杂的寄生虫病,影响许多器官和组织。在我们的研究中,嗜酸性粒细胞增多,复发,在多器官受累的儿科患者中,并发症的发生率更高,他们需要重复手术和长期医疗。然而,关于风险因素的数据很少,最佳治疗和预后。
    Echinococcosis with multi-organ/disseminated involvement is rare in childhood. We aimed to evaluate the clinical and laboratory characteristics and prognosis in paediatric patients with echinococcosis having multiorgan/disseminated involvement.
    We evaluated retrospectively children with echinococcosis with involvement of three or more organs.
    Thirteen patients were included in the study. The median age was 120 (range 71-189) months. Three (23%) were diagnosed incidentally. Abdominal pain was seen in 5 (38.4%) patients, vomiting in 4 (30.7%), headache in 3 (23%), cough in 2 (15.3%), groin pain in 1 (7.6%), 1 (7.6%) had jaundice and 1 (7.6%) had fever. The median duration of complaints was 48 (0-140) days. The most common tripartite organ was 38.4% (5/13) liver, lung and spleen. Isolated abdominal dissemination was detected in two patients. Two patients had multi-organ involvement and multiple cysts with dissemination. Cyst rupture was observed in three of the patients; recurrent urinary tract infection, hydroureteronephrosis, secondary peritonitis with intra-abdominal abscess, and biliary tract fistula were each observed in one patient. Relapse developed in 3 (23%) patients.
    Echinococcosis is a very slow growing and complex parasitic disease that affects many organs and tissues. In our study, eosinophilia, recurrence, and complications were seen at a higher rate in paediatric patients with multiorgan involvement, who required repetitive surgeries and long-term medical treatment. However, there are scanty data on risk factors, optimum treatment and prognosis.
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  • 文章类型: Case Reports
    隐球菌病是一种机会性感染,如果不及时诊断和治疗,死亡率很高。这项研究的目的是回顾最终尸检诊断为播散性隐球菌感染的死者的临床病理信息。这项研究收集了2015年1月1日至2018年12月31日期间到学术医院/实验室的4名死者的数据。他们的临床,我们回顾了包括治疗在内的放射学和病理学结果.两名患者出现呼吸道症状,而其他两名患者出现脑膜症状。其中3人被确认为HIV阳性。一位decendent在ART上,一个人没有接受治疗,一个人是幼稚的ART。两名死者被诊断出患有隐球菌性脑膜炎,一个患有细菌性肺炎,一个患有肺结核。三名死者在接受抗真菌治疗时在急诊室死亡,一名在病房死亡。尸检结果证实了所有病例中播散的隐球菌感染。在正确的临床背景下,应保持高度怀疑。所有患者都应怀疑有多器官受累,并积极寻找。
    Cryptococcosis is an opportunistic infection with high mortality if not diagnosed and treated in time. The objective of this study was to review the clinicopathological information of decendents with final autopsy diagnosis of disseminated cryptococcal infection. This study collected data from 4 decendents who presented to an academic hospital/laboratory between 1 January 2015 to 31 December 2018. Their clinical, radiological and pathological findings including treatment were reviewed. Two decendents presented with respiratory symptoms whilst the other 2 presented with meningeal symptoms. Three were confirmed HIV positive. One decendent was on ART, one had defaulted treatment and one was ART naïve. Two decendents were diagnosed with cryptococcal meningitis, one with bacterial pneumonia and one with pulmonary tuberculosis. Three decendents died in emergency unit and one in the ward whilst on antifungal therapy. The autopsy findings confirmed disseminated cryptococcal infection in all cases. A high index of suspicion should be maintained in the right clinical context. Multi-organ involvement should be suspected in all patients and be actively sought out.
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  • 文章类型: Case Reports
    Scedosporium和Lomentospora的感染,特别是Lomentospora(以前的Scedosporium)prolificans,在移植人群中几乎普遍致命且进展迅速。我们报告了一例接受骨髓抑制化疗的弥漫性大B细胞淋巴瘤患者,该患者发生了播散性长乳杆菌感染,随后持续存在于他的膝关节中。感染采用针对协同作用研究的早期经验性三联抗真菌疗法进行治疗。生长因子快速解决中性粒细胞减少症,和侵袭性清创(在可能的情况下)感染部位,包括截肢.他获得了11个月的缓解,直到接受深度骨髓抑制的自体造血干细胞移植,其中发生了复发的长株乳杆菌感染,导致死亡。我们强调早期治疗的重要性,协同作用研究,特别是在治疗这种破坏性疾病时,中性粒细胞减少症的恢复。
    Infections with Scedosporium and Lomentospora species, in particular Lomentospora (previously Scedosporium) prolificans, are nearly universally fatal and rapidly-progressive in the transplant population. We report a case of a patient with diffuse large B-cell lymphoma undergoing myelosuppressive chemotherapy who developed disseminated L. prolificans infection which afterward persisted in his knee joint. The infection was treated with early empiric triple antifungal therapy tailored to synergy studies, growth factors to quickly resolve neutropenia, and aggressive debridement (where possible) of infection sites, including amputation. He achieved an 11-month remission until undergoing autologous hematopoietic stem cell transplantation with deep myelosuppression, wherein recrudescent L. prolificans infection occurred, causing death. We highlight the importance of early treatment, synergy studies, and especially recovery of neutropenia in treating this devastating condition.
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  • 文章类型: Case Reports
    播散性化脓性肉芽肿是一种罕见的实体。患者需要对这种良性疾病感到放心,并建议进行全面手术切除后复发的风险和瘢痕形成的风险。应该排除红旗诊断。
    Disseminated pyogenic granuloma is a rare entity. Patients need reassurance for this benign condition and are advised about the risk of recurrence and the risk of scarring with a total surgical excision. Red-flag diagnoses should be ruled out.
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  • 文章类型: Journal Article
    BACKGROUND: Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs).
    METHODS: A 15-year retrospective review was conducted in KK Women\'s and Children\'s Hospital in Singapore, from January 2003 to October 2017.
    RESULTS: Ten patients were identified, the majority male (60.0%). The median age at presentation of symptoms of BCG infections was 3.8 (0.8 - 7.4) months. All the patients had likely underlying PIDS - four with Severe Combined Immunodeficiency (SCID), three with Mendelian Susceptibility to Mycobacterial Diseases (MSMD), one with Anhidrotic Ectodermal Dysplasia with Primary Immunodeficiency (EDA-ID), one with combined immunodeficiency (CID), and one with STAT-1 gain-of-function mutation. Definitive BCGosis was confirmed in all patients by the identification of Mycobacterium bovis subsp BCG from microbiological cultures. The susceptibility profiles of Mycobacterium bovis subsp BCG are as follows: Rifampicin (88.9%), Isoniazid (44.47%), Ethambutol (100.0%), Streptomycin (100.0%), Kanamycin (100.0%), Ethionamide (25.0%), and Ofloxacin (100.0%). Four patients (40.0%) received a three-drug regimen. Five patients (50.0%) underwent hematopoietic stem cell transplant (HSCT), of which three (60%) have recovered. Overall mortality was 50.0%.
    CONCLUSIONS: Disseminated BCG disease (BCGosis) should prompt immunology evaluation to determine the diagnosis of the immune defect. A three-drug regimen is adequate for treatment if the patient undergoes early HSCT.
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  • 文章类型: Case Reports
    Herpes zoster (HZ) is related with the reactivation of latent varicella-zoster virus (VZV) infection. This infection is associated with HZ-oticus, HZ-ophthalmicus, and disseminated-cutaneous HZ. Here, we report a case of an adolescent male who presented with vesicular-eruptions in the left-forehead. The physical examination showed vesicles on the left V1-dermatome and external auditory canal, associated with ipsilateral periorbital oedema, peripheral VII nerve paralysis, hyperacusis, and tinnitus. Acyclovir, eye lubrication, and ophthalmic prednisolone were started. On second admission day, he developed vesicular lesions throughout the body. Polymerase chain reaction (PCR) of the draining vesicles was VZV-positive.
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