Centro Hospitalar do Porto
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Artrite Séptica de Articulação Nativa em Adultos: Estudo Retrospetivo de Nove Anos num Hospital Universitário Português
Get PDFntroduction: Septic arthritis of a native joint represents a medical emergency. Drainage and effective antibiotic treatment are critical to avoid joint destruction and long-term impairment. The aim of this study was to evaluate epidemiological and clinical characteristics of patients with the diagnosis of septic arthritis to help establish local guidelines for empirical antibiotic treatment.
Material and methods: Retrospective analysis of adult patients admitted at Centro Hospitalar Universitário do Porto from 2009 to 2017 with suspected native joint septic arthritis. Relevant demographics, microbiology findings and respective antibiotic susceptibilities were analysed.
Results: Ninety-seven patients, predominantly males (59.8%) with a median age of 61 years old were included. The most commonly reported comorbidity associated with septic arthritis was diabetes mellitus (20.6%). The knee was the most commonly affected joint (71.1%). Arthrocentesis was performed in all patients, but only 50.5% had positive microbial growth in the synovial fluid. Staphylococcus aureus was the most frequently identified microorganism, 86% of which were methicillin susceptible. Gram-negative bacteria were the causative agent in 15% of cases. A wide range of empirical antibiotic regimens were prescribed with a combination of vancomycin/carbapenem being the most common (30.9%). Analysis of antibiotic susceptibility profiles revealed that amoxicillin/clavulanate would have been appropriate as the initial regimen in 89% of cases.
Discussion: The main causative pathogen was Staphylococcus aureus, with methicillin resistant Staphylococcus aureus remaining rare. The proportion of Gram-negative bacteria implies that these agents should be covered by empirical treatment, although no case of Pseudomonas infection has been identified. Therefore, antipseudomonal coverage is not necessary in empirical regimens.
Conclusion: Routine coverage of methicillin-resistant Staphylococcus aureus and Pseudomonas aeruginosa is not warranted but must be considered when specific risk factors are found. Amoxicillin/clavulanate can provide adequate antibiotic coverage as an empirical treatment for adult native joint septic arthritis. Its use may allow a reduction in use of broader spectrum antibiotics.info:eu-repo/semantics/publishedVersio
Síndrome de Dravet − experiência de uma Unidade de Neuropediatria
Get PDFIntroduction: Dravet syndrome (DS) is a rare and complex genetic epilepsy syndrome. The first seizures are generally induced by fever in the first year of life of a previously healthy child, and the condition is typically associated with impaired psychomotor development.
The authors present a clinical review of DS patients followed at a Neuropediatric Unit of a level III Pediatric Hospital.
Material and methods: Retrospective study of pediatric patients with DS followed at a Neuropediatric Unit between 2001 and 2019.
Results: Twenty-two patients were diagnosed and followed in this institution. The median (interquartile range [IQR]) age at first seizure was 4.5 (4-5.75) months, which was described as generalized tonic-clonic, focal seizure, or focal to bilateral tonic-clonic seizure, and 95% of patients had fever during this first episode. Neuroimaging and first electroencephalogram (EEG) were normal in all patients. SCN1A gene mutations were detected in 21 (95%) patients. All patients underwent multiple antiepileptic drug (AED) regimens. Psychomotor development was delayed in 20 (91%) patients, and 13 (59%) presented ataxia. At the end of follow-up, the median (IQR) age was 19 (8-23) years, with no reported deaths.
Discussion: The characteristics of the first DS seizures are crucial for diagnosis, which can be supported by genetic sequencing, with most patients presenting an SCN1A gene mutation. Neuroimaging and EEG are typically normal at disease onset, but most patients present EEG abnormalities over time. Seizure management can be challenging, requiring a combination of multiple AEDs.
Conclusion: DS is a progressive disease associated with poor cognitive and motor skill outcomes, resulting in great morbidity. Early diagnosis can help avoid unnecessary studies, optimize the therapeutic strategy, allow genetic counseling, and improve long-term outcomes.info:eu-repo/semantics/publishedVersio
Parotidite recorrente em crianças − casuística e revisão da literatura
Get PDFIntroduction: Recurrent parotitis is defined as the occurrence of two or more episodes of the parotid gland. Several etiologies should be addressed in the approach to these patients. The aim of this study was to investigate the clinical, laboratory, and imaging profile of children with recurrent parotitis. Material and Methods: Retrospective review of the medical records of patients referred to a Pediatric Outpatient Clinic between January 2013 and June 2018. Results: The medical records of 24 patients with recurrent parotitis (66.7% male) and a mean age of seven years and five months were reviewed. The median age of onset of episodes was five years and three months. Unilateral and non-febrile episodes prevailed. Non-steroidal anti-inflammatory drugs were universally used to treat symptoms. Non-acute parotid and neck ultrasound predominantly showed the presence of a heterogeneous gland (57.1%). Sialography performed in five patients suggested chronic parotitis in two and Sjögren syndrome/ sarcoidosis in one. No significant immunologic defects were found beside a mild C3 reduction in one patient and C4 reduction in another patient, apparently without clinical relevance. A single patient tested positive for antinuclear antibodies. Immunoglobulin A deficit was found in one case. The most common final diagnosis was juvenile recurrent parotitis (37.5%). Conclusions: Most cases of recurrent parotitis in pediatric age have benign etiology. A more judicious request of complementary exams in the acute and non-acute phases could be time- and cost-effective.info:eu-repo/semantics/publishedVersio
Obstrução nasal num recém-nascido
Get PDFDacryocystocele is an uncommon congenital disease affecting less than 1% of newborns.
Herein is presented the case of a female newborn with respiratory distress with onset in the first hours of life and worsening during breastfeeding.
Resistance to endoscope progression due to narrowing of both patent nasal cavities was noticed during flexible nasal endoscopy, and computed tomography revealed an extremely rare case of bilateral dacryocystocele.
A conservative approach was chosen, with surveillance in the Intensive Care Unit.
Although most dacryocystocele cases resolve spontaneously in the first year of life, recognition of this rare condition (particularly in Otolaryngology and Ophthalmology clinical practice) is crucial to prevent complications.info:eu-repo/semantics/publishedVersio
Terapêutica com surfactante em recém-nascidos pré-termo: tendência para uma abordagem menos invasiva
Get PDFIntroduction: Minimally invasive surfactant therapy (MIST) is a surfactant administration procedure that intends to reduce intubations and associated risks. The aim of this study was to compare MIST with INtubation-SURfactant-Extubation (INSURE) technique.
Material and methods: Retrospective analysis (from January 2015 to June 2019) of preterm infants on nasal continuous positive airway pressure (nCPAP) treated with surfactant.
Results: Fifty-four preterm infants were included and divided in two groups: MIST (n=34) and INSURE (n=20). No significant differences were found between groups regarding gestational age (p=0.480), birth weight (p=0.299), fraction of inspired oxygen (FiO2) prior to surfactant (p=0.220), oxygen therapy duration (p=0.306), progression to intubation (p=0.712), or length of Neonatal Intensive Care Unit stay (p=0.778). FiO2 variation before and after surfactant administration was higher in MIST group (14% vs 9%, p=0.078). No significant complications were reported with either technique.
Conclusions: MIST is a safe technique in preterm infants on nCPAP. This study shows similar outcomes with MIST and INSURE procedures, with a greater reduction in FiO2 requirements with MIST. Overall, MIST is less invasive and as effective as INSURE in preterm infants.info:eu-repo/semantics/publishedVersio
Caso clínico dermatológico
Get PDFIncontinentia pigmenti is an X-linked neuroectodermal dysplasia. It is a rare genetic disease with multiorgan involvement, and hence a multidisciplinary approach is of paramount importance. Although diagnosis is based on clinical findings, genetic molecular testing can be performed to confirm diagnosis and allow future genetic counselling.
The authors describe the case of a 4-month-old girl accidentally diagnosed with incontinentia pigmenti following routine physical examination in the Emergency Department. Timely diagnosis enabled appropriate multidisciplinary approach and follow-up.info:eu-repo/semantics/publishedVersio
Doença de Kwasaki num lactente de cinco meses
Get PDFIntroduction: Kawasaki disease, an acute self-limited vasculitis of small and medium arteries of unknown etiology, is the leading cause of heart disease in children in most developed countries. If untreated, it can lead to coronary artery dilatation and aneurysm, placing patients at risk for coronary thrombosis, myocardial infarction, or sudden death.
Case Report: A previously healthy five-month-old boy presented with a history of respiratory symptoms and fever, being admitted for respiratory syncytial virus bronchiolitis. He maintained fever for five days associated with progressive clinical worsening and sequential development of conjunctival injection, cracked lips, swelling of hands and feet, and polymorphous rash of the limbs. Diagnostic procedures showed increased levels of inflammatory markers, hyponatremia, hypoalbuminemia, sterile pyuria, and aseptic meningitis. Despite an initial normal echocardiogram, Kawasaki disease was suspected and the boy started intravenous immunoglobulin on the fifth day. On the seventh day, echocardiogram showed signs of pancarditis and mild coronary artery ectasia and acetylsalicylic acid was started. The boy was discharged, but eight days later echocardiogram revealed major and diffuse coronary artery ectasia in a follow-up consultation and he was readmitted to start corticosteroid therapy. After five months, the boy displayed normalization of cardiac changes, without further complications.
Comments: Kawasaki disease below six months of age is rare and associated with a high risk of coronary artery aneurysm. It is important to recognize the clinical and laboratory criteria associated with this entity and start early treatment, avoiding cardiac complications.info:eu-repo/semantics/publishedVersio
Rare occurrence of severe blindness and deafness in Friedreich ataxia: a case report
Get PDFBackground: Friedreich ataxia is the most frequent hereditary ataxia worldwide. Subclinical visual and auditory involvement has been recognized in these patients, with co-occurrence of severe blindness and deafness being rare.
Case report: We describe a patient, homozygous for a 873 GAA expansion in the FXN gene, whose first symptoms appeared by the age of 8. At 22 years-old he developed sensorineural deafness, and at 26 visual impairment. Deafness had a progressive course over 11 years, until a stage of extreme severity which hindered communication. Visual acuity had a catastrophic deterioration, with blindness 3 years after visual impairment was first noticed. Audiograms documented progressive sensorineural deafness, most striking for low frequencies. Visual evoked potentials disclosed bilaterally increased P100 latency. He passed away at the age of 41 years old, at a stage of extreme disability, blind and deaf, in addition to the complete phenotype of a patient with Friedreich ataxia of more than 30 years duration.
Discussion: Severe vision loss and extreme deafness has been described in very few patients with Friedreich ataxia. Long duration, severe disease and large expanded alleles may account for such an extreme phenotype; nonetheless, the role of factors as modifying genes warrants further investigation in this subset of patients.info:eu-repo/semantics/publishedVersio
Graves’ disease with spontaneous resolution following ocrelizumab in primary progressive multiple sclerosis
Get PDFObjectives. Immune reconstitution therapies (IRT), which include antibody-based cell-depleting therapies targeting CD52+ (alemtuzumab) or CD20+ (rituximab, ocrelizumab) leukocytes, are approved for the treatment of multiple sclerosis. Thyroid autoimmunity is a common adverse effect of alemtuzumab treatment, Graves' disease (GD) being the most prevalent manifestation. To date, thyroid autoimmunity events have not been reported with CD20-targeting monoclonal antibodies. Case Report. A 59-year-old woman with primary progressive multiple sclerosis with no prior personal history of thyroid disease or autoimmunity, was diagnosed with GD 6 months following the first ocrelizumab infusion. She was asymptomatic and had no signs of ophthalmopathy. Due to the temporal association of GD diagnosis with ocrelizumab infusion, absence of symptoms and our experience with alemtuzumab-induced GD, we decided for an active surveillance strategy and antithyroid drugs were not started. She underwent spontaneous resolution of hyperthyroidism with thyroid-stimulating hormone (TSH) receptor antibodies (TRAb) negativity and a mild and transitory period of subclinical hypothyroidism, while she continued the biannually ocrelizumab administration schedule. To present date, she has maintained close clinical and biochemical surveillance with normal TSH, free thyroxine (fT4) and free triiodothyronine (fT3) levels and undetectable TRAb. Conclusions. This is the first case of GD reported after ocrelizumab administration. The timing, onset and course of this case is similar to alemtuzumab-induced GD, usually interpreted as an "immune reconstitution syndrome"; however, ocrelizumab cell count depletion is inferior in severity, cell population affected and duration of depletion. This case highlights the importance of pre-screening and follow-up with thyroid function tests in patients treated with ocrelizumab. As a novel therapeutic antibody, further investigation is required to unravel the causes of thyroid autoimmunity.info:eu-repo/semantics/publishedVersio
Choroidal Blood Flow After Intravitreal Ranibizumab in Vitrectomized and Non-Vitrectomized Eyes with Diabetic Macular Edema
Get PDFAim: Diabetic retinopathy staging system and progression predictors are soon to be considered insufficient for ophthalmologic practice. Given the growing evidence of the role of choroidal dysfunction, our purpose was to assess choroidal vascular changes with intravitreal ranibizumab (RBZ) treatment in diabetic macular edema (DME).
Methods: This was a prospective longitudinal cohort study. The study included DME eyes, grouped in vitrectomized (group 1) and non-vitrectomized (group 2) eyes, submitted to RBZ in a pro re nata regimen, with 24 weeks of follow-up. Main outcome measures such as central subfield foveal thickness (CFT), choroidal thickness (CT), and choroidal vascular index (CVI) were obtained from structural OCT, and choriocapillaris flow density (CCD) was obtained from OCT angiography and analyzed before and after treatment.
Results: Thirty-one patients were included, 10 eyes in group 1 and 24 eyes in group 2. The mean number of injections was 5.18 (range 2-6). Globally, there was an improvement in BCVA (+4.3 ETDRS letters, p=0.004) and CFT (-84.6 µm, p0.05). When considering only group 2, there was a significant decrease in CT (p=0.033) and a significant increase in CCD (p=0.010) 6 months after treatment, with no differences in CVI (p=0.111). Baseline CVI was correlated with visual acuity at week 24 both globally (r=0.406, p=0.029) and in group 2 (r=0.604, p=0.004).
Conclusion: In non-vitrectomized eyes, choriocapillaris blood flow improves with RBZ. Baseline CVI may correlate with visual function after RBZ. ClinicalTrials.gov NCT04387604.info:eu-repo/semantics/publishedVersio