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  • 文章类型: Case Reports
    背景:副孢子菌病是一种被忽视的热带病,由副孢子菌属真菌引起。广泛的症状与疾病有关;然而,肺和皮肤是主要受影响的部位。这种疾病主要见于生活在拉丁美洲农村地区的人们。
    方法:我们介绍了一例对抗真菌治疗反应缓慢的严重播散性副病菌的儿科病例。三个月内,症状演变成肝脾肿大,坏死的颈部和腹部淋巴结,和脾脓肿。两性霉素B脱氧胆酸盐和伊曲康唑的临床反应缓慢,导致胸膜和腹膜腔积液,心力衰竭和休克。两性霉素B脱氧胆酸被脂质体制剂取代,没有回应。随后,治疗中加入了泼尼松,这导致了临床反应的改善。血清学副球菌抗体滴度不典型,在关键阶段滴度非常低,在恢复期显着增加。最终用两性霉素B脱氧胆酸盐清除了感染,脂质体两性霉素B和皮质类固醇的使用。副孢子菌病血清学在出院后两年无反应性。
    结论:由于副球菌细胞引发的强烈炎症反应,短时间给予小剂量泼尼松可调节炎症反应并支持抗真菌治疗.
    BACKGROUND: Paracoccidioidomycosis is a neglected tropical disease caused by fungi of the genus Paracoccidioides. A wide range of symptoms is related to the disease; however, lungs and skin are the sites predominantly affected. The disease is mostly seen in people living in rural areas in Latin America.
    METHODS: We present a pediatric case of severe disseminated paracoccidioidomycosis that slowly responded to the antifungal treatment. Within three months, symptoms evolved into hepatosplenomegaly, necrotic cervical and abdominal lymph nodes, and splenic abscess. Clinical response to amphotericin B deoxycholate and itraconazole was slow, resulting in pleural and peritoneal cavity effusions, heart failure and shock. Amphotericin B deoxycholate was replaced by the liposomal formulation, with no response. Subsequently, prednisone was added to the treatment, which led to improvement in the clinical response. Serological Paracoccidioides antibody titers were atypical, with very low titers in the critical phase and significant increase during the convalescence phase. The infection was finally cleared up with amphotericin B deoxycholate, liposomal amphotericin B and the use of corticosteroids. Paracoccidioidomycosis serology was non-reactive two years post-discharge.
    CONCLUSIONS: Due to the intense inflammatory response triggered by Paracoccidioides cells, giving low-dose prednisone for a short period of time modulated the inflammatory response and supported antifungal treatment.
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  • 文章类型: Case Reports
    Postinfectious bronchiolitis obliterans is a rare lung disease; there are limited reports in South America.
    We report 10 patients with this disease diagnosed at the Instituto Nacional de Salud del Niño-Breña (Lima-Peru). The median age at diagnosis was 19 months and all patients had a history of severe acute respiratory infection. The most frequent symptoms were cough, respiratory distress, wheezing, and hypoxemia. The mosaic attenuation pattern was the most frequent on the tomography. All the patients had positive serology for adenovirus. The treatment received was methylprednisolone pulses, azithromycin, hydroxychloroquine, and inhaled corticosteroids. No patient died during the follow-up.
    In previously healthy children with a history of severe acute respiratory infection and persistent bronchial obstructive symptoms, the diagnosis of postinfectious bronchiolitis obliterans should be considered. This is the first report in Peru with a therapeutic regimen adapted to our institution.
    La bronquiolitis obliterante postinfecciosa es una enfermedad pulmonar poco frecuente; existen limitados reportes en Sudamérica.
    En esta serie se reportan 10 pacientes con esta enfermedad diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú). La mediana de edad al diagnóstico fue de 19 meses. Todos los pacientes presentaron el antecedente de infección respiratoria aguda grave. Los síntomas más frecuentes fueron tos, dificultad respiratoria, sibilancias e hipoxemia; el patrón de atenuación en mosaico fue la característica más frecuente en la tomografía. Todos tenían serología positiva para adenovirus. Se administró tratamiento con pulsos de metilprednisolona, azitromicina, hidroxicloroquina y corticoides inhalados. Ningún paciente falleció durante el seguimiento.
    En los niños previamente sanos con antecedente de infección respiratoria aguda grave y sintomatología obstructivo bronquial persistente se debe considerar el diagnóstico de bronquiolitis obliterante postinfecciosa. Este es el primer reporte en Perú con un régimen terapéutico adaptado a nuestra institución.
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  • 文章类型: Case Reports
    甲状腺内胸腺组织(ITTT)是一种罕见的,良性疾病;由于对该实体不熟悉,其诊断可能具有挑战性。然而,它具有超声和细胞学特征,可以提示其存在,因此在甲状腺结节的鉴别诊断中应考虑。目前,免疫组织化学可用于细针穿刺(FNA)细胞学,从而减少不必要手术的需要。我们讨论了免疫组织化学在甲状腺细胞学中的有用性,关于一个10岁病人的病例,因可疑甲状腺结节接受部分甲状腺切除术,最终通过手术标本的组织病理学诊断为ITTT。
    Intrathyroidal thymus tissue (ITTT) is a rare, benign condition; its diagnosis can prove challenging due to unfamiliarity with this entity. However, it has ultrasonographical and cytological characteristics which can suggest its presence and thus should be considered in the differential diagnosis of thyroid nodules. Presently, immunohistochemistry can be used with fine needle aspiration (FNA) cytology, thus decreasing the need for unnecessary surgery. We discuss the usefulness of immunohistochemistry in thyroid cytology, with reference to a case of a 10-year-old patient, who underwent partial thyroidectomy for a suspicious thyroid nodule which was eventually diagnosed as ITTT by the histopathology of the surgical specimen.
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  • 文章类型: Case Reports
    尽管嗜人性皮肤癣菌仍然是西班牙头癣的主要原因,据报道,由于嗜人物种的增加。我们报告了一项回顾性观察研究,包括24名儿童,在2004年至2019年期间,他们被诊断为由于嗜人物种而患有头癣。75%的患者是男性,平均年龄为4,88岁。我们观察到83,3%的病例来自非洲,4,2%来自南美,12,5%来自西班牙。临床上,70,8%的患者出现鳞状斑块和非瘢痕性脱发。soudanense毛癣菌是该系列的主要皮肤癣菌(45,8%),其次是奥杜尼微孢子菌(20%),扁桃体毛癣菌(12,5%)和紫毛癣菌(12,5%)。尽管这种感染模式似乎与非洲移民有关,我们看到了三个本地案例。嗜人性而不是嗜人性皮肤癣菌更容易传播,可以预测头癣的发病率和公共卫生问题的增加。
    Although zoophilic dermatophytes remain the predominant cause of tinea capitis in Spain, an increase due to anthropophilic species has been reported. We report a retrospective observational study that included 24 children, who were diagnosed with tinea capitis due to anthropophilic species between 2004 and 2019. 75% of the patients were males with a mean age of 4,88 years. We observed 83,3% of cases from Africa, 4,2% from South America and 12,5% from Spain. Clinically, 70,8% of the patients presented scaly patches and non-scaring alopecia. Trichophyton soudanense was the main dermatophyte of the series (45,8%), followed by Microsporum audouinii (20,8%), Trichophyton tonsurans (12,5%) and Trichophyton violaceum (12,5%). Although this pattern of infection appears to be linked to immigration from Africa, we saw three native cases. The easier transmission of anthropophilic rather than zoophilic dermatophytes could predict a rise in the incidence of tinea capitis and a public health problem.
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  • 文章类型: Case Reports
    尽管嗜人性皮肤癣菌仍然是西班牙头癣的主要原因,据报道,由于嗜人物种的增加。我们报告了一项回顾性观察研究,包括24名儿童,在2004年至2019年期间,他们被诊断为由于嗜人物种而患有头癣。75%的患者是男性,平均年龄为4,88岁。我们观察到83,3%的病例来自非洲,4,2%来自南美,12,5%来自西班牙。临床上,70,8%的患者出现鳞状斑块和非瘢痕性脱发。soudanense毛癣菌是该系列的主要皮肤癣菌(45,8%),其次是奥杜尼微孢子菌(20%),扁桃体毛癣菌(12,5%)和紫毛癣菌(12,5%)。尽管这种感染模式似乎与非洲移民有关,我们看到了三个本地案例。嗜人性而不是嗜人性皮肤癣菌更容易传播,可以预测头癣的发病率和公共卫生问题的增加。
    Although zoophilic dermatophytes remain the predominant cause of tinea capitis in Spain, an increase due to anthropophilic species has been reported. We report a retrospective observational study that included twenty-four children, who were diagnosed with tinea capitis due to anthropophilic species between 2004 and 2019. 75% of the patients were males with a mean age of 4,88 years. We observed 83,3% of cases from Africa, 4,2% from South America and 12,5% from Spain. Clinically, 70,8% of the patients presented scaly patches and non-scaring alopecia. Trichophyton soudanense was the main dermatophyte of the series (45,8%), followed by Microsporum audouinii (20,8%), Trichophyton tonsurans (12,5%) and Trichophyton violaceum (12,5%). Although this pattern of infection appears to be linked to immigration from Africa, we saw three native cases. The easier transmission of anthropophilic rather than zoophilic dermatophytes could predict a rise in the incidence of tinea capitis and a public health problem.
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  • 文章类型: Case Reports
    背景:抗精神病药恶性综合征(NMS)是一种罕见且可能致命的药物不良反应。在儿童青少年人群中,对这一实体的研究仍然很少。
    目的:描述临床,儿童和青少年NMS患者的实验室和治疗特征。分析同一人群中NMS中存在的症状分组。
    方法:对2000年1月至2018年11月报告的所有NMS病例进行了MEDLINE/PubMed搜索,临床,确定了实验室和治疗变量.对症状进行阶乘分析。
    结果:包括57例患者(男42例,女15例),(平均年龄13.65±3.89岁)。典型抗精神病药的NMS发作时间为11.25±20.27天,非典型抗精神病药的NMS发作时间为13.69±22.43天。最常见的症状是肌肉僵硬(84.2%),自主神经不稳定(84.2%)和发热(78.9)。最常见的实验室发现是CPK升高和白细胞增多(42.1%)。最常用的治疗方法是苯二氮卓类药物(28.1%)。在对症状的探索性因子分析中,我们发现了3个因素:1)“Catatonic”伴mutism(0.912),消极主义(0.825)和蜡质柔韧性(0.522);2)步态改变的“锥体外系”(0.860),非自愿性异常运动(0.605),肌肉僵硬度(0.534)和唾液带(0.430);3)“自主神经不稳定”伴发热(0.798),意识受损(0.795)和自主神经不稳定(0.387)。
    结论:儿童和青少年的NMS可能有3种类型:锥体外系和自主神经不稳定。
    BACKGROUND: Neuroleptic malignant syndrome (NMS) is a rare and potentially fatal drug adverse reaction. There are still few studies of this entity in the child-adolescent population.
    OBJECTIVE: Describe the clinical, laboratory and therapeutic characteristics of children and adolescent patients with NMS. Analyse the grouping of symptoms present in NMS in the same population.
    METHODS: A MEDLINE/PubMed search of all reported cases of NMS from January 2000 to November 2018 was performed and demographic, clinical, laboratory and therapeutic variables were identified. A factorial analysis of the symptoms was performed.
    RESULTS: 57 patients (42 males and 15 females) were included, (mean age 13.65 ± 3.89 years). The onset of NMS occurred at 11.25 ± 20.27 days with typical antipsychotics and at 13.69 ± 22.43 days with atypical antipsychotics. The most common symptoms were muscle stiffness (84.2%), autonomic instability (84.2%) and fever (78.9). The most common laboratory findings were CPK elevation and leucocytosis (42.1%). The most used treatment was benzodiazepines (28.1%). In the exploratory factorial analysis of the symptoms we found 3 factors: 1) \"Catatonic\" with mutism (0.912), negativism (0.825) and waxy flexibility (0.522); 2) \"Extrapyramidal\" with altered gait (0.860), involuntary abnormal movements (0.605), muscle stiffness (0.534) and sialorrhoea (0.430); and 3) \"Autonomic instability\" with fever (0.798), impaired consciousness (0.795) and autonomic instability (0.387).
    CONCLUSIONS: NMS in children and adolescents could be of 3 types: catatonic, extrapyramidal and autonomic unstable.
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  • 文章类型: Journal Article
    背景:抗精神病药恶性综合征(NMS)是一种罕见且可能致命的药物不良反应。在儿童青少年人群中,对这一实体的研究仍然很少。
    目的:描述临床,儿童和青少年NMS患者的实验室和治疗特征。分析同一人群中NMS中存在的症状分组。
    方法:对2000年1月至2018年11月报告的所有NMS病例进行了MEDLINE/PubMed搜索,临床,确定了实验室和治疗变量.对症状进行阶乘分析。
    结果:包括57例患者(男42例,女15例),(平均年龄13.65±3.89岁)。典型抗精神病药的NMS发作时间为11.25±20.27天,非典型抗精神病药的NMS发作时间为13.69±22.43天。最常见的症状是肌肉僵硬(84.2%),自主神经不稳定(84.2%)和发热(78.9)。最常见的实验室发现是CPK升高和白细胞增多(42.1%)。最常用的治疗方法是苯二氮卓类药物(28.1%)。在对症状的探索性因子分析中,我们发现了3个因素:1)“Catatonic”伴mutism(0.912),消极主义(0.825)和蜡质柔韧性(0.522);2)步态改变的“锥体外系”(0.860),非自愿性异常运动(0.605),肌肉僵硬度(0.534)和唾液带(0.430);3)“自主神经不稳定”伴发热(0.798),意识受损(0.795)和自主神经不稳定(0.387)。
    结论:儿童和青少年的NMS可能有3种类型:锥体外系和自主神经不稳定。
    BACKGROUND: Neuroleptic malignant syndrome (NMS) is a rare and potentially fatal drug adverse reaction. There are still few studies of this entity in the child-adolescent population.
    OBJECTIVE: Describe the clinical, laboratory and therapeutic characteristics of children and adolescent patients with NMS. Analyse the grouping of symptoms present in NMS in the same population.
    METHODS: A MEDLINE/PubMed search of all reported cases of NMS from January 2000 to November 2018 was performed and demographic, clinical, laboratory and therapeutic variables were identified. A factorial analysis of the symptoms was performed.
    RESULTS: 57 patients (42 males and 15 females) were included, (mean age 13.65±3.89 years). The onset of NMS occurred at 11.25±20.27 days with typical antipsychotics and at 13.69±22.43 days with atypical antipsychotics. The most common symptoms were muscle stiffness (84.2%), autonomic instability (84.2%) and fever (78.9). The most common laboratory findings were CPK elevation and leucocytosis (42.1%). The most used treatment was benzodiazepines (28.1%). In the exploratory factorial analysis of the symptoms we found 3factors: 1) \"Catatonic\" with mutism (0.912), negativism (0.825) and waxy flexibility (0.522); 2) \"Extrapyramidal\" with altered gait (0.860), involuntary abnormal movements (0.605), muscle stiffness (0.534) and sialorrhoea (0.430); and 3) \"Autonomic instability\" with fever (0.798), impaired consciousness (0.795) and autonomic instability (0.387).
    CONCLUSIONS: NMS in children and adolescents could be of 3types: catatonic, extrapyramidal and autonomic unstable.
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  • 文章类型: Journal Article
    BACKGROUND: Autoimmune hemolytic anemia (AIHA) is a rare and generally self-limiting disease in children.
    METHODS: A descriptive cross-sectional study was performed in children under 18 years diagnosed with AIHA from January/1997 to July/2019. Clinical variables were collected and AIHA was classified according to the direct antiglobulin test (DAT) in warm AIHA (IgG+/-C3d) and cold AIHA (C3d). Response to treatment and evolution were analyzed.
    RESULTS: 25 patients were included and 72% were males. The median age at diagnosis was 2 years (range 0.4 to 9). Fever (72%), pallor (68%), jaundice (64%), hepatosplenomegaly and coluria (48%) were the most common presenting symptoms. The median hemoglobin at diagnosis was 5.4 g/dl. DAT was positive in 96%, with detection of IgG antibodies in 76%. A single case presented negative DAT. 20% of the patients associated another cytopenia, one of which was subsequently diagnosed with common variable immunodeficiency. Concomitant viral infection was suspected or documented in 32%. Most of the cases were self-limiting and responded to corticosteroid treatment (72%). Those with partial response (24%), mainly those associated with other cytopenias, required other lines of treatment (rituximab, mycophenolate, immunoglobulins). Complications (32%) and relapses (26%) were detected only in warm AIHA.
    CONCLUSIONS: Our case series confirms that AIHA is a very rare disease in childhood. Most cases evolve favorably, although up to a quarter of them require second lines of treatment and, in exceptional cases, they need very aggressive treatments. These latter cases generally correspond to patients who present more than one cytopenia in the course of the disease.
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  • 文章类型: Journal Article
    BACKGROUND: Histoplasmosis is a fungal disease, endemic in South America, and seldom reported in paediatrics.
    OBJECTIVE: To report the epidemiology, clinical features and outcome of children diagnosed with histoplasmosis in an Argentinian Children\'s Hospital.
    METHODS: A retrospective and descriptive study was performed from January 2008 to December 2016 in Hospital de Pediatría \'Prof. Dr. Juan Pedro Garrahan\'. Patients under 18 years with clinical features, serological tests, cultures and/or histological findings compatible with histoplasmosis were included. Thirteen patients were selected (seven male and six female; mean age was 9 years with interquartile range 3.4-13); three children (23%) were from Buenos Aires province, three (23%) from Santa Fe province, and seven (54%) from other provinces.
    RESULTS: In ten cases (77%) the clinical form was disseminated, and it was pulmonary in three (23%). Eight cases (62%) suffered other comorbidities. Serological tests were positive in seven patients (54%), with positive cultures obtained in nine patients (69%). Histological findings compatible with histoplasmosis were found in 10 cases (77%). All patients received treatment with amphotericin B (liposomal formulation in seven cases, deoxycholate in six), with 10 patients continuing with oral itraconazole. Three patients (23%) died from causes unrelated to histoplasmosis.
    CONCLUSIONS: The majority of children in the series had comorbidities and disseminated histoplasmosis. Mortality was not directly associated with histoplasmosis.
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  • 文章类型: Journal Article
    BACKGROUND: Although results show an association between the presence of generalised joint hypermobility (GJH) and functional gastrointestinal disorders (FGIDs) in children, they are limited and controversial.
    OBJECTIVE: To determine the association between GJH and FGIDs and the search for risk factors for GJH in girls from a Public Educational Institution of Tuluá, Colombia.
    METHODS: The students completed the Rome IV Questionnaire to identify FGIDs. Each girl with a diagnosis of some FGIDs was matched with a healthy control of the same age. Joint laxity was assessed according to the Beighton score and was considered as GJH when it was ≥ 4. The prevalence of GJH was compared in girls with and without FGIDs.
    RESULTS: Out of a total of 921 girls between 10 and 18 years of age that participated in the study, 219 (23.8%) of them had some FGIDs. The analysis was performed on a total of 169 girls with FGIDs and 169 healthy control girls. There were no significant differences in GJH between girls with and without a diagnosis of some FGIDs (OR=1.12: 95% CI; 0.71-1.77, P=.5838), nor were there any risk factors.
    CONCLUSIONS: In this study, no relationship or any risk factor was found between GJH and the presence of FGIDs.
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