Cat-scratch disease: a rare cause of osteomyelitis.

Cat-scratch disease is a zoonosis caused by Bartonella henselae, characterised by regional lymphadenopathy. Rarer presentations, such as osteomyelitis, can occur.We present an adolescent girl with severe right lumbar pain and fever, without animal contacts or recent travels. On examination, pain on flexion of torso, movement limitation and marked lordosis were noted, but there were no inflammatory signs, palpable masses or lymph nodes. Serological investigations revealed elevated inflammatory markers. Imaging revealed a paravertebral abscess with bone erosion. Several microbiological agents were ruled out. After a second CT-guided biopsy, PCR for Bartonella spp was positive. At this point, the family recalled having a young cat some time before. Cat-scratch disease was diagnosed, and complete recovery achieved after treatment with doxycycline and rifampicin.Cat-scratch disease is a challenging diagnosis in the absence of typical features. However, B. henselae must be investigated if common pathogens are ruled out and response to therapy is poor.

Neuroimaging of central nervous system tuberculosis.

A 20-month-old female, not immunized with Bacillus Calmette-Guérin (BCG) vaccine, was admitted due to a four-day history of fever and cough. In the past three months, she presented respiratory infections, weight loss and enlarged cervical lymph nodes. On day two of admission, she displayed drowsiness and positive Romberg's sign; cerebrospinal fluid (CSF) workout revealed 107/ul cells, low glucose and high protein levels. Ceftriaxone and acyclovir were initiated, and she was transferred to our tertiary hospital. Brain magnetic resonance imaging showed punctiform focal areas of restricted diffusion in left capsular lenticular region suggestive of vasculitis secondary to infection. Tuberculin skin test and interferon-gamma release assay were positive. She started tuberculostatic therapy, but two days later she presented tonic-clonic seizures and impaired consciousness. Cerebral computed tomography (CT) revealed tetrahydrocephalus (Figure 1), needing external ventricular derivation. She had a slow clinical improvement, requiring several neurosurgical interventions and developing a syndrome of inappropriate antidiuretic secretion alternating with cerebral salt wasting. Positive results for Mycobacterium tuberculosis were obtained by CSF culture and by polymerase chain reaction in CSF, bronchoalveolar lavage and gastric aspirate specimens. Repeated brain CT showed a large-vessel vasculitis with basal meningeal enhancement, typical of central nervous system (CNS) tuberculosis (Figure 2). She completed one month of corticosteroids and maintained antituberculosis treatment. At two years of age, she has spastic paraparesis and no language skills. Portugal had 1836 cases of tuberculosis (17.8 per 100000) in 2016 and was considered a low-incidence country; consequently, BCG vaccination is not universal (1). We present a severe case of CNS tuberculosis with intracranial hypertension, vasculitis and hyponatremia, associated with poorer outcomes (2). A high index of suspicion allowed prompt start of antituberculosis treatment. Diagnosis was corroborated by microbiological positivity and a typical triad in neuroimaging (hydrocephalus, vasculitis and basal meningeal enhancement) (3), which we wish to emphasize.

Actinomyces and Shewanella algae complicated paediatric mastoiditis: a case report of a multidisciplinary approach.

Cerebral venous sinus thrombosis in children is a rare complication of acute mastoiditis that can potentially be fatal. Clinical expertise is essential for early diagnosis and management due to its subtle course. We present the first known case of paediatric acute mastoiditis with venous sinus thrombosis caused by Shewanella algae and Actinomyces europaeus . A 17-year-old male presented clinical signs of right acute otitis media and mastoiditis. Brain computed tomography showed mastoid opacification, cerebral sinus thrombosis and an extradural collection. Microbiology revealed the presence of S. algae and A. europaeus . A multidisciplinary approach combining medical and surgical treatment allowed the patient to make a full recovery.

Varicella Admissions in Children and Adolescents in Portugal: 2000-2015.

OBJECTIVES: Varicella is a common, usually benign, and autolimited disease in children but can lead to severe complications and hospitalization. With this study, we aim to analyze all varicella hospitalizations to provide epidemiological information to help outline preventive policies. METHODS: We assessed all varicella hospitalizations in children aged 0 to 17 years, from 2000 to 2015, in mainland, public Portuguese hospitals using a Portuguese administrative database. Seasonality, geographic distribution, severity, complications, risk factors, use of diagnostic and treatment procedures and hospitalization costs were analyzed. RESULTS: A total of 5120 hospitalizations were registered, with an annual rate of 17.3 hospitalizations per 100 000 inhabitants. A higher number of hospitalizations occurred during the summer period and in Southern regions. The median length of stay was of 4 days (interquartile range: 3.0-7.0). We found a high rate of severe complications, mostly dermatologic (19.6%), neurologic (6.0%), and respiratory (5.1%). Of the total number of patients, 0.8% were immunocompromised and 0.1% were pregnant. Total direct hospitalization costs during the 16-year period were estimated to be 7 110 719€ (8 603 970 USD), with a mean annual cost of 444 419.92€ (537 748.10 USD). CONCULSIONS: This is the first national study in which useful epidemiological data to evaluate the burden and impact of varicella in Portugal is provided.

Multisystem inflammatory syndrome in children (MISC): A systematic review.

OBJECTIVE: The aim of this systematic review was to analyse current literature and reported cases of multisystem inflammatory syndrome in children (MISC), concerning its clinical spectrum, complications associated, therapeutic strategies and distinguishing features of other clinical syndromes. METHODS: Extensive literature research was performed in MEDLINE (through PubMed), Scopus and Web of Science from December 2019 to December 2020. First analysis included all article titles and abstracts screening to identify relevant studies, and second analysis included a full-text screening of previously selected studies. Eligibility was assessed independently by two authors, and disagreements were resolved by discussion and consensus. Data were extracted on MISC definition, demographic data, clinical features, diagnostic tests, laboratory analysis and imaging, therapeutical approach and outcomes. RESULTS: Common symptoms included gastrointestinal (70%), rash (57%) and cardiovascular (52% with shock). Notable differences with Kawasaki disease were identified including age, clinical presentation and cardiac involvement. Thirty per cent presented positive severe acute respiratory syndrome coronavirus-2 reverse transcription polymerase chain reaction and 51% positive serologies. Sixty-two per cent received intravenous immunoglobulin and 42% glucocorticoids. Sixty-two per cent required intensive care and 21 children died (<2%). Severe presentations were associated with neurological symptoms, hepatitis and acute kidney injury. CONCLUSIONS: MISC raises concern on its severe cardiac involvement at presentation, with frequent intensive care and immunomodulatory therapy need. Short-term outcomes seem to be favourable, with cardiac dysfunction recovery and low mortality rates.

Perianal Disease and Granulomas: Think Out of the Box .

BACKGROUND: Chronic granulomatous disease (CGD) is a primary immunodeficiency due to a malfunction of NADPH oxidase. It is characterized by recurrent and severe infections caused by catalase-positive microorganisms and autoinflammatory manifestations. Recently, there has been described an NCF4 gene variant that causes a deficiency of p40phox, a subunit of NADPH oxidase. Patients with this deficiency appear to have a less severe clinical form as compared to classic CGD. CASE: A 15-year-old girl with vulvar lichen planus since she was 2 years old and suspected Crohn's disease (CD) was first seen at our hospital. At the age of 12 years, she had been submitted to sacrococcygeal cyst exeresis, without cicatrization of the surgical wound and extension of the lesion to the perianal area. The diagnosis of CD was questioned, and the patient underwent an endoscopic and radiologic assessment, which was normal. A skin biopsy from the perianal area revealed a granuloma; thus, CD with isolated perianal disease was assumed. After several different treatments including antibiotics, infliximab, and adalimumab, the perianal lesion persisted, with no associated gastrointestinal symptoms. Therefore, the hypothesis of an immunodeficiency was considered. An immunologic and genetic study revealed reduced oxidative burst in the phorbol myristate acetate test, with diminished reactive oxygen species production and a homozygous mutation in the NCF4 gene. The adolescent started prophylactic trimethoprim-sulfamethoxazole and became asymptomatic. CONCLUSIONS: The present case highlights that alternative diagnoses to CD must be considered in the presence of isolated perianal disease with granulomatous inflammation, especially when the disease is refractory to conventional CD therapy.

INTRODUÇÃO: A doença granulomatosa crônica (DGC) é uma imunodeficiência primária devido a uma disfunção da NADPH oxidase. É caracterizada por infeções recorrentes e graves causadas por microrganismos catalase positivos e manifestações auto-inflamatórias. Recentemente, foi identificada uma variante do gene NCF4 responsável por deficiência de p40 phox , uma proteína constituinte da NADPH oxidase e clinicamente esta doença manifesta-se como uma imunodeficiência menos grave quando comparada com a DGC clássica. CASO: Adolescente de 15 anos, com líquen planovulvardesdeos2 anos. Aos 12anos, submetida a exérese de quisto sacrococcígeo não tendo ocorrido cicatrização da ferida cirúrgica e com extensão da lesão para a região perianal. Perante a suspeita de doença Crohn (DC), realizada investigação endoscópica e radiológica que foi normal. A biópsia de pele da lesão perianal identificou granuloma, tendo sido admitido o diagnóstico de DC com apresentação perianal. Foi submetida a vários tratamentos sem resolução da lesão. Aos 15 anos, colocada a hipótese de imunodeficiência primária; o estudo imunológico mostrou diminuição da explosão oxidativa no teste de imunidade com acetato miristato de forbol, com produção reduzida de radicais livres de oxigénio (RLO). Geneticamente identificada mutação homozigótica no gene NCF4. Atualmente, sob antibiótico profilático e clinicamente assintomática. CONCLUSÃO: Este caso permite alertar para a investigação de diagnósticos alternativos à DC perante doença perianal isolada com inflamação granulomatosa, em particular quando é refratária à terapêutica dirigida.

COMPLICATED PNEUMONIA WITH EMPYEMA CAUSED BY STREPTOCOCCUS ANGINOSUS IN A CHILD.

OBJECTIVE: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. CASE: description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. COMMENTS: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.

Neuroimagen en la encefalitis por virus Epstein-Barr / Neuroimaging of Epstein-Barr virus-associated encephalitis

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Complicated pneumonia with empyema caused by streptococcus anginosus in a child / Complicated pneumonia with empyema caused by streptococcus anginosus in a child

ABSTRACT Objective: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. Case description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. Comments: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.

RESUMO Objetivo: Alertar para a patogenicidade do Streptococcus anginosus que, apesar de raro em pediatria, pode causar infeções graves que necessitam de tratamento invasivo e antibioterapia de longo curso para obter um melhor prognóstico. Descrição do caso: Criança de seis anos, com atraso do desenvolvimento psicomotor, avaliado no serviço de urgência por febre e dificuldade respiratória. O exame físico, juntamente com os exames complementares, revelou uma pneumonia complicada com empiema no hemitórax esquerdo, tendo iniciado antibioterapia e sido submetido à drenagem do líquido pleural. Foi identificado Streptococcus anginosus nesse líquido. No 11º dia de doença, a criança agravou o seu estado clínico, com recidiva da febre, hipoxemia e dificuldade respiratória. Considerando-se o microrganismo identificado, o paciente foi submetido à decorticação pulmonar por videotoracoscopia, com boa evolução clínica posterior. Comentários: Streptococcus anginosus é uma bactéria comensal da cavidade oral humana, que pode causar infecções sistêmicas graves. Apesar de serem raros os casos descritos em pediatria, têm sido cada vez mais descritas infecções torácicas complicadas em adultos. Esse microrganismo também tem a capacidade de formar abcessos e empiemas, que precisam de intervenções terapêuticas diferentes, quando comparados a pneumonias complicadas causadas por outros agentes.

Nocardia lung abscess in an immunocompetent adolescent.

The authors report a case of a lung abscess caused by Nocardia sp. in a previously healthy adolescent. A 17-year-old young man presented with tonsillitis that did not respond to ß-lactamic antibiotic, dyspnoea and thoracic pain. The X-ray revealed a cavitation in the right pulmonary upper lobe. He was admitted and completed a 14-day empirical antimicrobial therapy for a pulmonar abscess, although no clinical recovery was observed. He then underwent pulmonary biopsy of the lesion and PCR analysis of the collected pulmonary tissue, which revealed the presence of Nocardia This case emphasises the importance of considering nocardiosis in the differential diagnosis of a lung abscess, particularly if no response to empirical therapy is obtained.