juvenile

青少年
  • 文章类型: Case Reports
    儿童中最常见的良性喉肿瘤是乳头状瘤。喉乳头状瘤病是一种慢性疾病,在儿童中很少见。我们报告了一个四岁的马来女孩,她患有慢性喉乳头状瘤病,最有可能在分娩过程中垂直获得,被检测到。她三年来一直声音嘶哑,柔性喉镜检查显示声门区乳头状瘤的特征。患者接受了直接喉镜检查,然后使用冷仪器切除肿块。手术干预是喉乳头状瘤病的主要治疗方式,以维持气道通畅和语音质量。
    The most common benign laryngeal neoplasm in children is a papilloma. Laryngeal papillomatosis is a chronic disease and is rare in children. We report the case of a four-year-old Malay girl in whom chronic laryngeal papillomatosis, most likely acquired vertically during labor, was detected. She presented with hoarseness of voice for three years, and a flexible laryngoscopy examination revealed features of papilloma in the glottis area. The patient underwent direct laryngoscopy followed by excision of mass using the cold instrument. Surgical intervention is the primary treatment modality for laryngeal papillomatosis to maintain airway patency and voice quality.
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  • 文章类型: Case Reports
    毛霉和根霉被认为是毛霉菌病的主要罪魁祸首,以其机会性而闻名的严重真菌感染。这种感染主要针对免疫系统受损的个体,包括糖尿病患者和接受糖皮质激素治疗的患者,免疫反应减弱的地方。本文旨在强调及时诊断和强化治疗在控制毛霉菌病中的关键作用。特别是在儿科患者中,因为它可以避免死亡和减轻严重的发病率。该病例报告强调了早期识别糖尿病患者的真菌感染并随后积极治疗以防止不良结局的紧迫性。当及时诊断毛霉菌病并通过强化治疗进行管理时,它强调了出色治疗结果的潜力。通过这样做,可以有效预防与这种情况相关的显著发病率和死亡率,强调对有真菌感染易感因素的患者保持警惕和积极管理的重要性。
    Mucor and Rhizopus species are recognized as the primary culprits responsible for mucormycosis, a severe fungal infection known for its opportunistic nature. This infection primarily targets individuals with compromised immune systems, including those with diabetes mellitus and patients undergoing glucocorticoid therapy, where the immune response is weakened. This article aims to underscore the pivotal role of prompt diagnosis and intensive treatment in managing mucormycosis, particularly in pediatric patients, as it can avert death and mitigate serious morbidity. This case report emphasizes the urgency of identifying fungal infections in patients with diabetes early on and subsequently treating them aggressively to prevent adverse outcomes. It highlights the potential for excellent treatment outcomes when mucormycosis is promptly diagnosed and managed with intensive therapy. By doing so, significant morbidity and mortality associated with this condition can be effectively prevented, underscoring the importance of vigilance and proactive management in patients with predisposing factors for fungal infections.
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  • 文章类型: Journal Article
    这项回顾性队列研究评估了Janus激酶(JAK)抑制剂的疗效和安全性。托法替尼和巴利替尼,在14例难治性皮肌炎(DM)患者中,治疗选择有限的多系统自身免疫性疾病。结果表明,皮肤皮肌炎疾病面积和严重程度指数(CDASI)评分中位数显着下降21分,下降76%,以及64%的患者肌肉症状的完全缓解。JAK抑制剂可有效治疗各种亚型的难治性DM,并伴有轻度和可控制的不良事件。
    This retrospective cohort study assessed the efficacy and safety of Janus kinase (JAK) inhibitors, tofacitinib and baricitinib, in 14 patients with refractory dermatomyositis (DM), a multisystemic autoimmune disorder with limited therapeutic options. Results demonstrated a significant median decrease of 21 points and a 76% reduction in the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) scores, along with a complete resolution of muscular symptoms in 64% of the patients. JAK inhibitors were effective in managing refractory DM across various subtypes with mild and manageable adverse events.
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  • 文章类型: Letter
    背景:儿童幼年特发性复发性腮腺炎(JIRP)是一种以特发性腮腺炎症反复发作为特征的疾病。由于没有明确的诊断和治疗指南,我们提供了连续病例系列,随访超过10年的患者,以及他们对泼尼松龙短期治疗的显著反应.
    方法:我们通过回顾性回顾诊断为JIRP的儿童的医学图表进行了这项研究,从2002年1月1日至2023年2月29日。我们进行了常规的血清学测试,以排除一些可能的背景。我们在发作的第一天给予短程泼尼松龙,分剂量(0.5mg/kg)。
    结果:在本病例系列中,有10名患者(70%)为男性,发病年龄中位数为5岁,发作持续时间5天,平均病程为3.8年。患者的平均随访时间接近10年。与他们的自然病程相比,所有患者在使用泼尼松龙的第一天对治疗表现出戏剧性的反应(P值0.005)。在十年的随访中,没有任何其他伴随的自身免疫性疾病。
    结论:每次发作第一天的短期强的松龙及其在我们患者中的戏剧性和有意义的反应,介绍一个新的,有效,快,JIRP患者的廉价治疗方案。
    BACKGROUND: Juvenile idiopathic recurrent parotitis (JIRP) in children is a condition characterized with recurrent episodes of idiopathic parotid gland inflammation. Since there are no definitive guidelines for diagnosis and management of this condition, we present a consecutive case series of patients with more than one decade follow up and their dramatic response to short course treatment by prednisolone.
    METHODS: We conducted this study by retrospectively reviewed medical charts of children who were diagnosed with JIRP, from 1 January 2002 to 29 February 2023. We performed usual serological tests to exclude some possible background. We administered short course prednisolone on first day of episode as divided dosage (0.5 mg /kg).
    RESULTS: In this case series of 10 patients (70%) were male, median age of onset was 5 years, duration of episodes 5 days, and the mean course of disease were 3.8 years. The average follows up of patients was near 10 years. In comparison with their natural course of disease all patients showed a dramatic response to treatment on the first day of administration of prednisolone (P Value 0.005). For ten years follow up there was not any additional accompanying autoimmune disorder.
    CONCLUSIONS: Short course prednisolone on first day of each episode and its dramatic and meaningful response in our patients, introduce a new, effective, fast, and inexpensive regimen of therapy in patients with JIRP.
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  • 文章类型: Journal Article
    记录一例幼犬下颌骨次全切除术后下颌体自发再生的病例。
    一只3个月大的雄性完整法国斗牛犬在左下颌骨磨牙区的背侧出现乳头状口腔鳞状细胞癌。肿块的初始活检由初级保健兽医进行。完整的临床分期显示没有转移迹象。头部的计算机断层扫描图像显示肿块的对比度增强最小,没有骨膜或骨骼受累的迹象。进行下颌骨次全切除术。组织病理学提示肿瘤完全切除。8周后患者返回进行随访和腭裂手术修复,此时,在触诊时在下颌骨切除术区域发现了骨骼。头部的重复计算机断层扫描显示,左下颌体从支的水平到下颌联合的完全再生。由于功能性下颌骨的重建,不需要治疗咬合不正。
    本病例表明,未成熟犬下颌骨次全切除术后,下颌体的自发再生是可能的。下颌骨次全切除术是一种根治性手术,可导致包括下颌骨漂移在内的长期并发症。错牙合,口腔疼痛。此病例报告提供的证据表明,在接受此手术的年轻患者中,这些后遗症可能会减轻或消除。
    UNASSIGNED: To document a case of spontaneous regeneration of the mandibular body following subtotal mandibulectomy in a juvenile dog.
    UNASSIGNED: A 3-month-old male intact French bulldog was presented with papillary oral squamous cell carcinoma located at the dorsal aspect of the molar region of the left mandible. Initial biopsy of the mass was performed by the primary care veterinarian. Complete clinical staging revealed no signs of metastasis. Computed tomographic images of the head showed minimal contrast enhancement of the mass with no signs of periosteal or bone involvement. Subtotal mandibulectomy was performed. Histopathology indicated complete excision of the tumor. The patient returned 8-weeks later for follow up and cleft palate surgical repair, at which time bone was noted in the mandibulectomy area on palpation. Repeat computed tomography of the head revealed complete regeneration of the left mandibular body from the level of the ramus to the mandibular symphysis. No treatment for malocclusion was necessary due to the reformation of a functional mandible.
    UNASSIGNED: The present case demonstrates that spontaneous regeneration of the mandibular body is possible following subtotal mandibulectomy in immature dogs. Subtotal mandibulectomy is a radical procedure that can lead to long term complications including mandibular drift, malocclusion, and oral pain. This case report provides evidence that these sequelae may be mitigated or eliminated in young patients undergoing this procedure.
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  • 文章类型: Case Reports
    背景:星状非遗传性特发性视网膜裂孔(SNIFR)是一种罕见类型的视网膜裂孔,在外丛层发生辐条状分裂。我们介绍了一例青少年的星状非遗传性特发性黄斑视网膜裂孔,其中两只眼睛表现出不同的发展趋势,黄斑视网膜裂孔可能与Henle纤维层中的机械力有关。去除机械力可以部分恢复视力。
    方法:诊断为双侧SNIFR的14岁女孩,并进行谱域光学相干断层扫描(SD-OCT)随访。在两次后续访问中,左眼玻璃体粘连释放,和视力改善。右眼神经上皮脱离加重,视力下降。因此,右眼行玻璃体切除术.手术后,病人的视网膜重新连接,她的视力得到了部分恢复.
    结论:我们报道了一个双侧SNIFR的少年。她的每一个眼睛都表现出不同的发展趋势,所以我们对每只眼睛采用不同的治疗方法。对患者进行玻璃体切除术以解决进行性视力丧失,这改善了病人的视力。进一步证实,SNIFR患者的Henle层易发生玻璃体后膜粘连。
    BACKGROUND: Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is a rare type of retinoschisis with a spoke-like splitting that occurs in the outer plexus layer. We present a case of stellate nonhereditary idiopathic foveomacular retinoschisis in a juvenile, in which two eyes show different development trends and macular retinoschisis could be associated with mechanical force in the Henle fibre layer. The removal of mechanical force can partially restore vision.
    METHODS: A 14-year-old girl with bilateral SNIFR was diagnosed and followed up with spectral-domain optical coherence tomography (SD-OCT). During the two follow-up visits, vitreous adhesion was released in the left eye, and visual acuity improved. Neuroepithelial detachment was aggravated in the right eye, and visual acuity decreased. Therefore, vitrectomy was performed on the right eye. After surgery, the patient\'s retina was reattached, and her vision was partially restored.
    CONCLUSIONS: We reported a juvenile with bilateral SNIFR. Each of her eyes showed different development trends, so we adopted different treatment methods for each eye. Vitrectomy was performed on the patient to address progressive vision loss, which improved the patient\'s vision. It was further confirmed that the Henle layer of SNIFR patients was susceptible to posterior vitreous membrane adhesion.
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  • 文章类型: Journal Article
    反应性关节炎是一种急性炎症性无菌性关节炎,在遗传易感个体中发生感染过程。它与胃肠道或泌尿生殖道感染有关。反应性关节炎在儿童中很少见。在这次审查中,我们介绍了两个需要生物治疗的指标病例,然后对儿童和青少年的反应性关节炎进行了全面审查,并提出了治疗方法.
    Reactive arthritis is an acute inflammatory aseptic arthritis that is preceded by an infectious process in genetically predisposed individuals. It has been associated with gastrointestinal or genitourinary infection. Reactive arthritis is rare in children. In this review, we present two index cases that need biologic treatment followed by a thorough review of reactive arthritis in children and adolescents with proposed treatment algorithm.
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  • 文章类型: Case Reports
    青少年皮肌炎(JDM)是一种自身免疫性结缔组织疾病,其特征是18岁以下儿童的上肢和下肢近端肌肉发炎。这种情况主要涉及近端肌肉和皮肤,但肌肉外受累,如胃肠道,肺,心也是常见的。
    我们介绍了一个12岁的南亚男性病例,他在3岁时在所有4个四肢都出现了无力和肌肉疼痛。最近病情逐渐恶化,患者出现了细嫩的溃疡皮肤结节。所有四肢的力量都下降了,患者无法进行常规工作,例如梳理头发,关上衬衫纽扣,和走路。实验室检查显示,白细胞总数(TLC)和红细胞沉降率(ESR)升高,近端肌肉和皮肤病变的活检显示,局灶性轻度坏死浸润,分别涉及非坏死肌纤维和钙化性皮肤。诊断为JDM,患者开始接受免疫抑制治疗(类固醇)和地尔硫卓。
    JDM与其他自身免疫,遗传,和炎症条件。正确的历史,彻底的临床检查,需要实验室检查以排除其他伪装条件。该病例报告还强调了地尔硫在治疗钙质角膜炎中的重要性,这在皮肌炎患者中更为常见。
    UNASSIGNED: Juvenile dermatomyositis (JDM) is an autoimmune connective tissue disorder characterized by an inflammation of proximal muscles of both upper and lower limbs in children below the age of 18 years. The condition mainly involves the proximal muscles and skin but extra-muscular involvement such as the gastrointestinal tract, lungs, and heart are also common.
    UNASSIGNED: We present a case of a 12-year-old south Asian male who developed weakness and muscular pain in all 4 extremities at 3 years of age. The condition gradually worsened recently, and the patient developed tender ulcerated skin nodules. Power in all 4 limbs was decreased and the patient was not able to perform his routine work such as combing of hair, closing a shirt button, and walking. Laboratory investigations revealed raised total leukocyte count (TLC) and erythrocyte sedimentation rate (ESR) and biopsy of the proximal muscles and skin lesions showed focal mild necrotic infiltrate involving nonnecrotic muscle fibers and calcinosis cutis respectively. A diagnosis of JDM was made and the patient was started on immunosuppressive therapy (steroids) and diltiazem.
    UNASSIGNED: JDM shares clinical features with other autoimmune, genetic, and inflammatory conditions. Proper history, thorough clinical examination, and laboratory workup is needed to rule out other masquerading conditions. This case report also highlighted the importance of diltiazem in the treatment of calcinosis cutis which is more commonly seen in patients with dermatomyositis.
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  • 文章类型: Case Reports
    UNASSIGNED: Takayasu arteritis (TA) is a chronic granulomatous vasculitis with unknown pathophysiology. TA with severe aortic obstruction has a poor prognosis. However, the efficacy of biologics and appropriate timing of surgical intervention remain controversial. We report a case of tuberculosis (TB)-associated TA with aggressive acute heart failure (AHF), pulmonary hypertension (PH), thrombosis, and seizure, who failed to survive after surgery.
    UNASSIGNED: A 10-year-old boy who developed a cough with chest tightness, shortness of breath, hemoptysis with reduced left ventricular ejection fraction, PH, and increased C-reactive protein and erythrocyte sedimentation rate was hospitalized at the pediatric intensive care unit of our hospital. He had strongly positive purified protein derivative skin test and interferon-gamma release assay result. Computed tomography angiography (CTA) showed occlusion of proximal left subclavian artery and stenosis of descending aorta and upper abdominal aorta. His condition did not improve after administration of milrinone, diuretics, antihypertensive agents, and intravenous methylprednisolone pulse followed by oral prednisone. Intravenous tocilizumab was administered for five doses, followed by two doses of infliximab, but his HF worsened, and CTA on day 77 showed complete occlusion of the descending aorta with large thrombus. He had a seizure on day 99 with deterioration of renal function. Balloon angioplasty and catheter-directed thrombolysis were performed on day 127. Unfortunately, the child\'s heart function continued to deteriorate and died on day 133.
    UNASSIGNED: TB infection may be related to juvenile TA. The biologics, thrombolysis, and surgical intervention failed to achieve the anticipated effect in our case with aggressive AHF due to severe aortic stenosis and thrombosis. More studies are needed to determine the role of biologics and surgery in such dire cases.
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  • 文章类型: Meta-Analysis
    发育过程中的次优条件可以缩短端粒,染色体末端的保护性DNA帽。较短的早期端粒长度(TL)可以表明减少的躯体维持,导致较低的生存率和较短的寿命。然而,尽管有明确的证据,并不是所有的研究都显示了生命早期的TL和生存或寿命之间的关系,这可能是由于生物学或研究设计的差异(例如,测量的生存期)。在精湛的仙女(Maluruscyaneus),我们评估了早期生命TL是否可以预测不同生活史阶段的死亡率(初出茅庐,少年,adult).然而,与对同类物的类似研究相反,早期生命TL不能预测该物种任何生命阶段的死亡率。然后,我们进行了一项荟萃分析,包括来自23项研究(15只鸟类和3种哺乳动物)的32种效应大小,以量化早期TL对死亡率的影响,同时考虑了生物学和方法学变异的潜在来源。总的来说,生命早期TL对死亡率的影响是显著的,对应于死亡率风险降低15%,TL每增加一个标准差。然而,校正发表偏倚时,效果变得更弱.与我们的预测相反,没有证据表明早期生命TL对死亡率的影响随物种寿命或测量存活时间的不同而不同.然而,早年TL对死亡风险的负面影响在一生中普遍存在.这些结果表明,早期TL对死亡率的影响更可能是环境依赖性的,而不是年龄依赖性的。虽然大量的权力和出版偏见问题突出了更多研究的必要性。
    Suboptimal conditions during development can shorten telomeres, the protective DNA caps on the end of chromosomes. Shorter early-life telomere length (TL) can indicate reduced somatic maintenance, leading to lower survival and shorter lifespan. However, despite some clear evidence, not all studies show a relationship between early-life TL and survival or lifespan, which may be due to differences in biology or study design (e.g., survival period measured). In superb fairy-wrens (Malurus cyaneus), we assessed whether early-life TL predicts mortality across different life-history stages (fledgling, juvenile, adult). However, in contrast to a similar study on a congener, early-life TL did not predict mortality across any life stage in this species. We then performed a meta-analysis including 32 effect sizes from 23 studies (15 birds and three mammals) to quantify the effect of early-life TL on mortality whilst taking into consideration potential sources of biological and methodological variation. Overall, the effect of early-life TL on mortality was significant, corresponding to a 15% reduction in mortality risk with each standard deviation increase in TL. However, the effect became weaker when correcting for publication bias. Contrary to our predictions, there was no evidence that effects of early-life TL on mortality varied with species lifespan or the period over which survival was measured. However, negative effects of early-life TL on mortality risk were pervasive throughout life. These results imply that effects of early-life TL on mortality are more likely to be context-dependent than age-dependent, although substantial power and publication bias issues highlight the need for more research.
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