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Valentino Syndrome: Case Report
No full textAbdominal pain is a common complaint in the pediatric emergency department. Perforation of a peptic ulcer is a rare occurrence in children and is a commonly overlooked etiology. This study presents a case of a previously healthy 16-yearold male presenting with right lower quadrant abdominal pain. Physical examination, laboratory test, and ultrasound results were suggestive of acute appendicitis. The laparoscopy results, which showed a normal appendix, and the following clinical findings, led to the diagnosis of a perforated pre-pyloric ulcer. Valentino syndrome occurs when a patient with a perforated ulcer presents with pain in the right lower quadrant, which mimics appendicitis, a far more common condition.info:eu-repo/semantics/publishedVersio
Doença Renal na Espondilite Anquilosante: uma Série de Casos e Revisão da Literatura
No full textBackground: Kidney disease is a rare manifestation of ankylosing spondylitis (AS) and its pathological alterations remain poorly described. The aim of this study was to investigate the clinical presentation and pathological alterations on kidney biopsy of AS patients and review and discuss the current literature on the issue.
Methods: We retrospectively studied the clinical presentation and kidney pathological alterations of 15 Caucasian AS patients submitted to kidney biopsy between October 1985 and March 2021.
Results: Patients were predominantly male (66.7%) with median age at the time of kideney biopsy of 47 years [IQR 34 - 62]. Median serum creatinine at presentation was 1.3 mg/dL [IQR 0.9 - 3] and most patients also had either proteinuria (85.7%) and/or hematuria (42.8%). The most common indication for kidney biopsy was nephrotic syndrome (33.3%), followed by acute or rapidly progressive kidney injury (20%) and chronic kidney disease of unknown etiology (20%). Chronic interstitial nephritis (CIN) (n=3) and AA amyloidosis (n=3) were the most common diagnosis. Others included IgA nephropathy (IgAN) (n=2), focal segmental glomerulosclerosis (n=2), membranous nephropathy (n=1), and immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN)(n=1).
Conclusions: We present one of the largest series of biopsy-proven kidney disease in Caucasian AS patients. We found a lower prevalence of IgAN than previously reported in Asian cohorts. We found a higher prevalence of CIN and a lower prevalence of AA amyloidosis than that described in previous series of Caucasian patients. We also present the first case of AS-associated IC-MPGN.Antecedentes: A doença renal é uma
manifestação rara de espondilite anquilosante
(EA) e as suas alterações patológicas
permanecem pouco descritas. O objetivo
deste estudo foi investigar a apresentação
clínica e alterações patológicas na biópsia
renal de doentes com EA bem como rever e
discutir a literatura atual sobre o assunto.
Métodos: Estudamos retrospectivamente a
apresentação clínica e alterações patológicas
renais de 15 doentes caucasianos com EA
submetidos a biópsia renal entre Outubro de
1985 e Março de 2021. Resultados: Os doentes
eram predominantemente homens (66,7%)
com idade mediana no momento da biópsia
de 47 anos [IIQ 34 - 62]. A creatinina sérica
mediana na apresentação foi de 1,3 mg/dL [IIQ
0,9 - 3] e a maioria dos pacientes apresentava
também proteinúria (85,7%) e/ou hematúria
(42,8%). A indicação mais comum para
biópsia renal foi a síndrome nefrótica (33,3%),
seguida de lesão renal aguda ou rapidamente
progressiva (20%) e doença renal crónica
de etiologia desconhecida (20%). A Nefrite
intersticial crónica (NIC) (n=3) e a amiloidose
AA (n=3) foram os diagnósticos mais comuns.
Outros incluíram nefropatia por IgA (NIgA)
(n=2), glomeruloesclerose segmentar focal
(n=2), nefropatia membranosa (n=1) e
glomerulonefrite membranoproliferativa
mediada por imunocomplexos (GNMP-IC)
(n=1). Conclusões: Apresentamos uma das
maiores séries de doenças renais comprovadas
por biópsia em doentes caucasianos com EA.
Encontramos uma prevalência de NIgA menor
do que a relatada anteriormente em coortes
asiáticas. Encontramos uma maior prevalência
de NIC e uma prevalência menor de amiloidose
AA do que a descrita em séries anteriores de
pacientes caucasianos. Também apresentamos
o primeiro caso de GNMP-IC associada à EA.info:eu-repo/semantics/publishedVersio
Quadripolar Left Ventricle Only Single Lead Pacing in a Patient With a Tricuspid Mechanical Valve: a Less Invasive Approach
No full textIn the presence of prosthetic tricuspid valve, the inaccessibility to the right ventricle makes permanent pacing challenging. The placement of a left ventricle (LV) single lead in the coronary sinus (CS) is a well-accepted alternative, with some limitations regarding sensing and threshold. We describe a clinical case of a patient who had a previous LV only lead in the CS due to the presence of a prosthetic tricuspid valve and, after a surgical valvular intervention, presented with recurrent syncope episodes due to lead malfunction with lack of pacing capture and significant ventricular pauses. A quadripolar lead was chosen to be placed in the CS connected to a cardiac resynchronization therapy pacemaker device, programmed at biventricular VVI and using a specific manufacturer T-wave protection algorithm to prevent pacemaker-induced arrhythmias and to use the patient's own rhythm. This approach prevented a fourth surgical intervention to place an epicardial lead and resulted in reasonable LV sensing and pacing threshold.
Learning objectives: This paper reports an alternative and atypical approach that could solve some of the limitations associated with ventricular pacing in patients with tricuspid prosthetic valves and multiple previous surgeries.info:eu-repo/semantics/publishedVersio
Epidemiological, Social and Economic Burden of Severe Hypoglycaemia in Patients with Diabetes Mellitus in Portugal: A Structured Literature Review
No full textIntroduction: The aim of this review was to identify and review studies reporting on the epidemiological, social and economic impact associated with severe hypoglycaemia (SH) in people with diabetes mellitus (DM) in Portugal.
Methods: A structured literature search was carried out in PubMed and Embase using a predefined selection criterion. Studies published in either Portuguese or English, between January 2010 and February 2021 were deemed eligible for inclusion.
Results: Twelve studies including adults (aged ≥ 18 years) with type 1 and/or type 2 diabetes mellitus (T1DM/T2DM) were eligible for inclusion. Epidemiological estimates varied according to the setting and type of data source used. The proportion of patients who experienced ≥ 1 SH episode (SHE) in the previous 6-12 months varied from 3.1% in adults with T2DM to 36.8% in adults with T1DM. In adults with T2DM the prevalence in a community-based study was highest in the insulin and secretagogue combination treated group (9.1%), while in an emergency department setting prevalence was highest in the insulin-based therapy group and the oral hypoglycaemic agent without secretagogues group (32.0% and 20.0%, respectively). The prevalence of SH in other studies in patients with DM ranged from 0.1% (emergency department) to 18.1% (hospital ward). Patients treated with secretagogues had the highest rates of hospitalisations. In patients with T1DM, the annual rate of SHE was higher in those with impaired hypoglycaemia awareness than in those with intact awareness. Mean total cost (direct and indirect) per SHE ranged from €1493.00 in patients with T2DM treated in an emergency setting to €2608.51 in patients with T1DM who were hospitalised.
Conclusion: Hypoglycaemic events, especially SHE, have a significant effect on the life of persons living with DM and their caregivers. Studies show that the prevalence of this acute complication of diabetes is not negligible. In addition to the negative impact on the quality of life, the burden of SHE in Portugal translates into a significant impact on the global health expenditure.info:eu-repo/semantics/publishedVersio
Case Report: Varicella Associated Neuropsychiatric Syndrome (VANS) in Two Pediatric Cases
No full textBackground: Viral or bacterial infections can trigger auto-immune inflammatory reactions and conditions in children. Self-reactivity arises due to similarities in molecular structures between pathogenic microorganisms and regular body structures with consequent immune-cross reactions. Reactivation of latent Varicella Zoster Virus (VZV) infections can cause neurological sequalae, including cerebellitis, post-herpetic neuralgias, meningo/encephalitis, vasculopathy and myelopathy. We propose a syndrome caused by auto-immune reactivity triggered by molecular mimicry between VZV and the brain, culminating in a post-infectious psychiatric syndrome with childhood VZV infections.
Case presentation: Two individuals, a 6-year-old male and 10-year-old female developed a neuro-psychiatric syndrome 3-6 weeks following a confirmed VZV infection with intrathecal oligoclonal bands. The 6-year-old male presented with a myasthenic syndrome, behavior deterioration and regression in school, he was poorly responsive to IVIG and risperidone, however had a pronounced response to steroid treatment. The 10-year-old female presented with marked insomnia, agitation, and behavioral regression as well as mild bradykinesia. A trial of neuroleptics and sedatives resulted in a mild unsustained reduction in psychomotor agitation and IVIG was also unsuccessful, however the patient was very responsive to steroid therapy.
Conclusion: Psychiatric syndromes with evidence of intrathecal inflammation temporally related to VZV infections that are responsive to immune modulation have not been described before. Here we report two cases demonstrating neuro-psychiatric symptoms following VZV infection, with evidence of persistent CNS inflammation following the resolution of infection, and response to immune modulation.info:eu-repo/semantics/publishedVersio
Consenso Português para a Identificação Precoce de Esclerose Múltipla Secundária Progressiva: Painel Delphi
No full textIntroduction: Multiple sclerosis is a disease with a heterogeneous evolution. The early identification of secondary progressive multiple sclerosis is a clinical challenge, which would benefit from the definition of biomarkers and diagnostic tools applicable in the transition phase from relapsing-remitting multiple sclerosis to secondary progressive multiple sclerosis. We aimed to reach a Portuguese national consensus on the monitoring of patients with multiple sclerosis and on the more relevant clinical variables for the early identification of its progression.
Material and methods: A Delphi panel which included eleven Portuguese Neurologists participated in two rounds of questions between July and August of 2021. In the first round, 39 questions which belonged to the functional, cognitive, imaging, biomarkers and additional evaluations were included. Questions for which no consensus was obtained in the first round (less than 80% of agreement), were appraised by the panel during the second round.
Results: The response rate was 100% in both rounds and consensus was reached for a total of 33 questions (84.6%). Consensus was reached for monitoring time, evaluation scales and clinical variables such as the degree of brain atrophy and mobility reduction, changes suggestive of secondary progressive multiple sclerosis. Additionally, digital devices were considered tools with potential to identify disease progression. Most questions for which no consensus was obtained referred to the cognitive assessment and the remaining referred to both functional and imaging domains.
Conclusion: Consensus was obtained for the determination of the monitorization interval and for most of the clinical variables. Most questions that did not reach consensus were related with the confirmation of progression taking into account only one test/domain, reinforcing the multifactorial nature of multiple sclerosis.Introdução: A esclerose múltipla é uma doença de evolução heterogénea. A identificação precoce da forma secundária progressiva é um desafio clínico, carecendo da definição de biomarcadores e ferramentas de diagnóstico aplicáveis na fase de transição da forma surto-remissão para a forma secundária progressiva. Este trabalho teve como objetivo estabelecer um consenso nacional português sobre a monitorização dos doentes e das variáveis clínicas mais relevantes para a identificação precoce da progressão da esclerose múltipla.
Material e Métodos: Um painel Delphi constituído por 11 neurologistas portugueses respondeu a duas rondas de perguntas entre julho e agosto de 2021. Na primeira ronda foram incluídas 39 questões relacionadas com a avaliação funcional, cognitiva, imagiológica, de biomarcadores e outras, e na segunda, as questões para as quais não foi atingido consenso (menos de 80% de concordância) na primeira ronda voltaram a ser submetidas a avaliação pelo painel.
Resultados: A taxa de resposta foi de 100% em ambas as rondas e 33 das 39 questões (84,6%) atingiram concordância. Foi atingido consenso relativamente ao tempo de monitorização dos doentes, às escalas de avaliação a empregar e a variáveis clínicas tais como o grau de atrofia cerebral ou redução da mobilidade, cuja alteração é sugestiva de esclerose múltipla secundária progressiva. Adicionalmente, os dispositivos digitais foram considerados ferramentas com potencial para identificar a progressão da doença. A maioria das questões para as quais não foi obtido consenso dizem respeito à avaliação cognitiva, estando as restantes inseridas nos domínios funcional e imagiológico.
Conclusão: Foi obtido consenso para a determinação do intervalo de monitorização e para a maioria das variáveis clínicas. A maioria das questões sem consenso estavam relacionadas com a confirmação do diagnóstico de progressão tendo em conta apenas um teste/domínio, realçando a natureza multifatorial da esclerose múltipla.info:eu-repo/semantics/publishedVersio
Nephrotic Syndrome and Waldenstrom’s Macroglobulinemia: A Case Report
No full textWaldenstrom’s macroglobulinemia (WM) is an IgM associated lymphoplasmacytic lymphoma and kidney disease is a rare complication.
We report a case of a 41-year-old male with a history of WM diagnosed 16 years ago, currently with no indication for treatment. The patient presented with nephrotic syndrome and acute kidney injury, hypertension, and multiple lymphadenopathies. A kidney biopsy was performed, showing minimal change disease with lymphomatous infiltration of B-lymphocytes CD20+. Therapy with bortezomib, dexametahasone and rituximab was started and after 6 months of follow-up he presented progressive recovery of renal function and remission of proteinuria.
This case illustrates the importance of screening renal disease in WM patients. A kidney biopsy should be performed in those presenting with otherwise unexplained renal failure and/or nephrotic syndrome
Predictors of Functional Outcome After Symptomatic Intracranial Hemorrhage Complicating Intravenous Thrombolysis: Results From the SITS-ISTR.
No full textBackground and purpose: Several risk factors of symptomatic intracerebral hemorrhage (SICH) following intravenous thrombolysis for acute ischaemic stroke have been established. However, potential predictors of good functional outcome post-SICH have been less studied.
Methods: Patient data registered in the Safe Implementation of Treatment in Stroke-International Stroke Thrombolysis Register (SITS-ISTR) from 2005 to 2021 were used. Acute ischaemic stroke patients who developed post intravenous thrombolysis SICH according to the SITS Monitoring Study definition were analyzed to identify predictors of functional outcomes.
Results: A total of 1679 patients with reported SICH were included, out of which only 2.8% achieved good functional outcome (modified Rankin Scale scores of 0-2), whilst 80.9% died at 3 months. Higher baseline National Institutes of Health Stroke Scale (NIHSS) score and 24-h ΔNIHSS score were independently associated with a lower likelihood of achieving both good and excellent functional outcomes at 3 months. Baseline NIHSS and hematoma location (presence of both SICHs, defined as remote and local SICH concurrently; n = 478) were predictors of early mortality within 24 h. Independent predictors of 3-month mortality were age, baseline NIHSS, 24-h ΔNIHSS, admission serum glucose values and hematoma location (both SICHs). Age, baseline NIHSS score, 24-h ΔNIHSS, hyperlipidemia, prior stroke/transient ischaemic attack, antiplatelet treatment, diastolic blood pressure at admission, glucose values on admission and SICH location (both SICHs) were associated with reduced disability at 3 months (≥1-point reduction across all modified Rankin Scale scores). Patients with remote SICH (n = 219) and local SICH (n = 964) had comparable clinical outcomes, both before and after propensity score matching.
Conclusions: Symptomatic intracerebral hemorrhage presents an alarmingly high prevalence of adverse clinical outcomes, with no difference in clinical outcomes between remote and local SICH
