Pub Date : 2024-08-01Epub Date: 2024-01-04DOI: 10.5546/aap.2023-10220.eng
Ana Braslavsky, María E López
Here we describe the case of a 2-month-old infant who consulted several times due to excessive crying, initially interpreted as having a gastrointestinal cause. Since the symptom persisted, a fracture was suspected due to its association with mobilization of the limbs and palpation of a mass on the anterior aspect of the right tibia. X-rays showed diaphyseal polyostotic involvement and lesions compatible with cortical involvement of long bones. Caffey-De Toni-Silverman syndrome was diagnosed and treatment with nonsteroidal anti-inflammatory drugs was initiated, resulting in symptom remission. Subsequently, the diagnosis was confirmed by the identification of the pathogenic heterozygous variant COL1A1. This is a rare condition with an estimated incidence of 48/100 000 individuals, and less than 150 cases have been described to date.
{"title":"Congenital cortical hyperostosis: a rare cause of inconsolable crying in a baby. Clinical case report.","authors":"Ana Braslavsky, María E López","doi":"10.5546/aap.2023-10220.eng","DOIUrl":"10.5546/aap.2023-10220.eng","url":null,"abstract":"<p><p>Here we describe the case of a 2-month-old infant who consulted several times due to excessive crying, initially interpreted as having a gastrointestinal cause. Since the symptom persisted, a fracture was suspected due to its association with mobilization of the limbs and palpation of a mass on the anterior aspect of the right tibia. X-rays showed diaphyseal polyostotic involvement and lesions compatible with cortical involvement of long bones. Caffey-De Toni-Silverman syndrome was diagnosed and treatment with nonsteroidal anti-inflammatory drugs was initiated, resulting in symptom remission. Subsequently, the diagnosis was confirmed by the identification of the pathogenic heterozygous variant COL1A1. This is a rare condition with an estimated incidence of 48/100 000 individuals, and less than 150 cases have been described to date.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139058111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-01-18DOI: 10.5546/aap.2023-10138.eng
Victoria Fontana, Gabriela S Aboud, Liliana Sabbaj
Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.
{"title":"Herlyn-Werner-Wünderlich syndrome: Two case report.","authors":"Victoria Fontana, Gabriela S Aboud, Liliana Sabbaj","doi":"10.5546/aap.2023-10138.eng","DOIUrl":"10.5546/aap.2023-10138.eng","url":null,"abstract":"<p><p>Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139401619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-03-14DOI: 10.5546/aap.2023-10148.eng
Juan I Degiuseppe, María T Soto, Christian Barrios Mathieur, Karina A Gomes, Juan A Stupka
Introduction. Data on the frequency of enteric adenoviruses, sapoviruses, and astroviruses in cases of sporadic acute gastroenteritis in Argentina are scarce. Methods. Descriptive design of a selection of fecal samples of children with diarrhea younger than 5 years referred between 2010 and 2021, with a previous negative result for rotavirus and norovirus. The presence of enteric adenovirus, sapovirus, and astrovirus was tested by molecular methods, with subsequent genotyping of positive samples. Results. At least 1 of the tested viruses was detected in 226 (39.4%) of the 574 selected samples. Specifically, adenovirus, sapovirus, and astrovirus were detected in 30.7%, 5.6%, and 3.1% of the samples, respectively. The most frequent viruses detected were adenovirus 41, sapoviruses GI.1 and GI.2, and astrovirus 1. Non-classic astroviruses were detected in 2 samples. Conclusions. Despite being less frequent, these enteropathogens are responsible for a large number of sporadic diarrhea events. Therefore, their study and surveillance contribute significantly to reduce the gap of undiagnosed cases.
{"title":"Enteric viruses other than rotavirus and norovirus in children under 5 years of age with gastroenteritis in Argentina, 2010-2021. A descriptive study.","authors":"Juan I Degiuseppe, María T Soto, Christian Barrios Mathieur, Karina A Gomes, Juan A Stupka","doi":"10.5546/aap.2023-10148.eng","DOIUrl":"10.5546/aap.2023-10148.eng","url":null,"abstract":"<p><p>Introduction. Data on the frequency of enteric adenoviruses, sapoviruses, and astroviruses in cases of sporadic acute gastroenteritis in Argentina are scarce. Methods. Descriptive design of a selection of fecal samples of children with diarrhea younger than 5 years referred between 2010 and 2021, with a previous negative result for rotavirus and norovirus. The presence of enteric adenovirus, sapovirus, and astrovirus was tested by molecular methods, with subsequent genotyping of positive samples. Results. At least 1 of the tested viruses was detected in 226 (39.4%) of the 574 selected samples. Specifically, adenovirus, sapovirus, and astrovirus were detected in 30.7%, 5.6%, and 3.1% of the samples, respectively. The most frequent viruses detected were adenovirus 41, sapoviruses GI.1 and GI.2, and astrovirus 1. Non-classic astroviruses were detected in 2 samples. Conclusions. Despite being less frequent, these enteropathogens are responsible for a large number of sporadic diarrhea events. Therefore, their study and surveillance contribute significantly to reduce the gap of undiagnosed cases.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140064733","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-03-21DOI: 10.5546/aap.2023-10187.eng
María J Aráoz, María M Buján, María Del V Centeno, Andrea B Cervini
Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.
{"title":"Eosinophilic cellulitis (Wells' syndrome) in a female pediatric patient.","authors":"María J Aráoz, María M Buján, María Del V Centeno, Andrea B Cervini","doi":"10.5546/aap.2023-10187.eng","DOIUrl":"10.5546/aap.2023-10187.eng","url":null,"abstract":"<p><p>Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140130623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-03-21DOI: 10.5546/aap.2023-10219.eng
Ramiro J Del Río, Santiago E Cicutti, Javier D González Ramos
The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published. The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.
{"title":"Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift.","authors":"Ramiro J Del Río, Santiago E Cicutti, Javier D González Ramos","doi":"10.5546/aap.2023-10219.eng","DOIUrl":"10.5546/aap.2023-10219.eng","url":null,"abstract":"<p><p>The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published. The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140130626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-05-23DOI: 10.5546/aap.2024-10400.eng
María S Ciruzzi
{"title":"To flip the coin: around children's and adolescents' capacity to participate in the decision-making process.","authors":"María S Ciruzzi","doi":"10.5546/aap.2024-10400.eng","DOIUrl":"10.5546/aap.2024-10400.eng","url":null,"abstract":"","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141070473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-02-01DOI: 10.5546/aap.2023-10173.eng
Natalia A Petriz, Carolina Antonietti, Cecilia Parente, Claudio A S Parisi
Introduction. Allergic rhinitis (AR) is one of the most frequent chronic diseases in the pediatric population; it affects the quality of life of children and their families, has economic impact, and is frequently underdiagnosed and undertreated. Given the scarcity of local data, here we describe the prevalence of AR and the clinical characteristics of the study population. Population and methods. Observational, cross-sectional study in patients younger than 19 years. Results. A total of 250 patients were randomly included; their mean age was 9 years (SD: 5). AR was diagnosed in 14 of them. The prevalence of AR was 6%. Conclusions. The prevalence of AR in our setting was 6%. AR should be given the relevance it deserves so as to provide an adequate diagnosis and treatment.
简介过敏性鼻炎(AR)是儿科人群中最常见的慢性疾病之一;它影响儿童及其家庭的生活质量,对经济造成影响,而且常常诊断不足、治疗不及时。鉴于本地数据稀缺,我们在此介绍 AR 的发病率和研究人群的临床特征。研究对象和方法。针对 19 岁以下患者的横断面观察研究。研究结果。共随机纳入 250 名患者,他们的平均年龄为 9 岁(标准差:5 岁)。其中 14 人确诊为 AR。AR 患病率为 6%。结论。在我们的环境中,AR 的发病率为 6%。应给予 AR 应有的重视,以便提供适当的诊断和治疗。
{"title":"Epidemiological study of allergic rhinitis in a population of children seen at the Department of Pediatrics of a general hospital. Cross-sectional study.","authors":"Natalia A Petriz, Carolina Antonietti, Cecilia Parente, Claudio A S Parisi","doi":"10.5546/aap.2023-10173.eng","DOIUrl":"10.5546/aap.2023-10173.eng","url":null,"abstract":"<p><p>Introduction. Allergic rhinitis (AR) is one of the most frequent chronic diseases in the pediatric population; it affects the quality of life of children and their families, has economic impact, and is frequently underdiagnosed and undertreated. Given the scarcity of local data, here we describe the prevalence of AR and the clinical characteristics of the study population. Population and methods. Observational, cross-sectional study in patients younger than 19 years. Results. A total of 250 patients were randomly included; their mean age was 9 years (SD: 5). AR was diagnosed in 14 of them. The prevalence of AR was 6%. Conclusions. The prevalence of AR in our setting was 6%. AR should be given the relevance it deserves so as to provide an adequate diagnosis and treatment.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139545590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction. Levetiracetam (LEV) is an antiepileptic drug approved by the Chilean Institute of Public Health as concomitant therapy for epileptic seizures in children older than 4 years of age. However, it is widely prescribed from the neonatal period, which makes it necessary to evaluate its off-label use. Objective. To determine the prescription-indication profile of LEV in the treatment of epileptic seizures in children younger than 4 years in a tertiary care hospital in southern Chile. Population and method. Observational, descriptive, and retrospective study. The medical records of patients who started treatment with LEV between 2014 and 2019 were reviewed, and data on sociodemographic, pharmacological, and clinical variables were collected. The analysis was based on the description of the profile of patients, prescriptions, follow-up, and safety. Results. A total of 68 patients were included: 40 (58.8%) were males, 49 (72.1%) were born at a gestational age ≥ 37 weeks. The main etiology of epilepsy was structural (35.3%); LEV was mostly used in children diagnosed with central nervous system malformation (17.6%), and monotherapy was the prevailing dosage (55.9%). LEV was used for focal seizures in 50% of cases. Five children (7.3%) had psychiatric disorders, classified as probable adverse drug reactions. Conclusion. LEV was used in children with various diagnoses, with a low rate of adverse events. The profile of drug use varied in the different age groups. Future studies are needed to identify effectiveness, especially in newborn infants and patients with refractory epilepsy.
简介左乙拉西坦(LEV)是智利公共卫生研究所批准用于治疗 4 岁以上儿童癫痫发作的一种抗癫痫药物。然而,该药在新生儿期就被广泛使用,因此有必要对其标签外使用情况进行评估。目的确定智利南部一家三级医院在治疗 4 岁以下儿童癫痫发作时 LEV 的处方-适应症概况。人群和方法。观察性、描述性和回顾性研究。研究人员查阅了2014年至2019年期间开始使用LEV治疗的患者病历,并收集了社会人口学、药物学和临床变量数据。分析基于对患者概况、处方、随访和安全性的描述。结果共纳入 68 名患者:40例(58.8%)为男性,49例(72.1%)胎龄≥37周。癫痫的主要病因是结构性癫痫(35.3%);LEV主要用于确诊为中枢神经系统畸形的儿童(17.6%),单药治疗是主要用药方式(55.9%)。50%的病例使用 LEV 治疗局灶性癫痫发作。5名儿童(7.3%)患有精神疾病,被归类为可能的药物不良反应。结论LEV用于各种诊断的儿童,不良反应发生率较低。不同年龄组的用药情况各不相同。今后需要进行研究,以确定其有效性,尤其是对新生儿和难治性癫痫患者的有效性。
{"title":"Levetiracetam prescription profile in children younger than 4 years treated at a tertiary care hospital in Chile.","authors":"Mónica Kyonen, Lily Acuña, Bárbara Ramos, Jocelyn Gutiérrez, R Mauricio Barría","doi":"10.5546/aap.2023-10233.eng","DOIUrl":"10.5546/aap.2023-10233.eng","url":null,"abstract":"<p><p>Introduction. Levetiracetam (LEV) is an antiepileptic drug approved by the Chilean Institute of Public Health as concomitant therapy for epileptic seizures in children older than 4 years of age. However, it is widely prescribed from the neonatal period, which makes it necessary to evaluate its off-label use. Objective. To determine the prescription-indication profile of LEV in the treatment of epileptic seizures in children younger than 4 years in a tertiary care hospital in southern Chile. Population and method. Observational, descriptive, and retrospective study. The medical records of patients who started treatment with LEV between 2014 and 2019 were reviewed, and data on sociodemographic, pharmacological, and clinical variables were collected. The analysis was based on the description of the profile of patients, prescriptions, follow-up, and safety. Results. A total of 68 patients were included: 40 (58.8%) were males, 49 (72.1%) were born at a gestational age ≥ 37 weeks. The main etiology of epilepsy was structural (35.3%); LEV was mostly used in children diagnosed with central nervous system malformation (17.6%), and monotherapy was the prevailing dosage (55.9%). LEV was used for focal seizures in 50% of cases. Five children (7.3%) had psychiatric disorders, classified as probable adverse drug reactions. Conclusion. LEV was used in children with various diagnoses, with a low rate of adverse events. The profile of drug use varied in the different age groups. Future studies are needed to identify effectiveness, especially in newborn infants and patients with refractory epilepsy.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140064734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-01-25DOI: 10.5546/aap.2023-10137.eng
Romina F Pombar, Romina L Tellería, Belén Bianco, María Del V Centeno, Andrea B Cervini
Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.
获得性感染后紫癜是一种罕见的急性重症疾病,其特点是在没有活动性感染或既往凝血功能障碍的情况下,皮肤坏死并伴有弥散性血管内凝血(DIC)。它主要影响儿童群体,90%的病例在发病前有感染过程。其病理生理机制是一过性自身抗体介导的蛋白 S 缺乏,这种缺乏会导致高凝状态。在这里,我们描述了一个病例:一名原本健康的 8 岁男孩出现了典型的紫癜性皮肤损害,并伴有 DIC,但没有败血症。他被确诊为一过性血浆蛋白 S 缺乏症。他需要使用新鲜冰冻血浆和抗凝剂进行替代治疗,但疗效良好。蛋白 S 活性在两个月内持续下降。
{"title":"Postinfectious purpura fulminans: A case report.","authors":"Romina F Pombar, Romina L Tellería, Belén Bianco, María Del V Centeno, Andrea B Cervini","doi":"10.5546/aap.2023-10137.eng","DOIUrl":"10.5546/aap.2023-10137.eng","url":null,"abstract":"<p><p>Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139485011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-08-01Epub Date: 2024-02-01DOI: 10.5546/aap.2023-10151.eng
Ignacia Viana, María L Nucifora, Luciana Vera, Agostina B Vietri, Alejandra Ariovich
A rare complication of frontal sinusitis includes Pott's puffy tumor. It manifests as a swelling of the forehead due to the presence of a subperiosteal abscess secondary to osteomyelitis of the frontal bone. A timely diagnosis allows for an early, intensive medical and surgical treatment, which is critical to prevent serious intracranial complications. Here we describe the case of a 12-year-old boy with Pott's puffy tumor as a complication of pansinusitis. This case was a diagnostic challenge; however, a timely treatment allowed for a favorable clinical course.
{"title":"Pott's puffy tumor: A rare complication of sinusitis. A case report.","authors":"Ignacia Viana, María L Nucifora, Luciana Vera, Agostina B Vietri, Alejandra Ariovich","doi":"10.5546/aap.2023-10151.eng","DOIUrl":"10.5546/aap.2023-10151.eng","url":null,"abstract":"<p><p>A rare complication of frontal sinusitis includes Pott's puffy tumor. It manifests as a swelling of the forehead due to the presence of a subperiosteal abscess secondary to osteomyelitis of the frontal bone. A timely diagnosis allows for an early, intensive medical and surgical treatment, which is critical to prevent serious intracranial complications. Here we describe the case of a 12-year-old boy with Pott's puffy tumor as a complication of pansinusitis. This case was a diagnostic challenge; however, a timely treatment allowed for a favorable clinical course.</p>","PeriodicalId":8338,"journal":{"name":"Archivos argentinos de pediatria","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139545591","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}