RESUMEN
Clinical descriptions about influenza-like illness in children seem non-specific during the co-circulation of SARS-CoV-2 and influenza. This paper aimed to summarize recent studies comparing clinical features and outcome, laboratory and radiological findings of COVID-19 patients with influenza patients in the paediatric population.
RESUMEN
An association of autoimmune hemolytic anemia with disseminated tuberculosis is an exceedingly rare entity. We describe herein a case of cold hemolytic autoimmune anemia associated with miliary tuberculosis resolved with blood transfusions, therapeutic plasma exchange, and antituberculous agents. We discuss the advantages of therapeutic plasma exchange at an early stage in the management of this condition.
Asunto(s)
Anemia Hemolítica Autoinmune , Tuberculosis Miliar , Anemia Hemolítica Autoinmune/complicaciones , Anemia Hemolítica Autoinmune/terapia , Humanos , Intercambio Plasmático , Tuberculosis Miliar/complicaciones , Tuberculosis Miliar/terapiaRESUMEN
Eosinophilic fasciitis is a rare connective tissue disease with a clinical presentation of scleroderma-like disease. We report a case of a 36-year-old female patient with a 6-month history of progressive stiffness involving her forearms and legs with joint pain. Laboratory examinations showed hypereosinophilia and elevated C-reactive protein. 18F FDG PET/CT showed diffuse and symmetrical increased uptake in the fasciae of the upper and lower limbs, sparing both muscles and fat tissues. Guided biopsy and histologic examination confirmed the diagnosis of eosinophilic fasciitis. 18F FDG PET/CT is of great help in the diagnosis of eosinophilic fasciitis, as it can guide the biopsy where FDG uptake is strongest and also help rule out possible associated neoplasms.
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Eosinofilia , Fascitis , Adulto , Eosinofilia/diagnóstico por imagen , Fascitis/diagnóstico por imagen , Femenino , Fluorodesoxiglucosa F18 , Humanos , Tomografía Computarizada por Tomografía de Emisión de PositronesRESUMEN
Hyperferritinemia can be the result of inflammation, infection, iron overload, or other uncommon pathologies including hemophagocytic lymphohistiocytosis (HLH). The significance of its elevation and its association with poor prognosis and critical clinical situations is unclear. To study the spectrum of diagnosis associated with elevated serum ferritin, we made a retrospective review of patients admitted to our center from 2015 and 2017 with serum ferritin levels above 2000 µg L-1. The H score was retrospectively assessed in all cases to evaluate the possible presence of HLH. The degree of ferritinemia found was compared with the evaluation of the undelying diagnosis and the results of laboratory examinations. A total of 77 patients were identified with a serum ferritin level >2000 µg L-1. Hematological malignancy was the most prevalent diagnosis with n=20; severe infection was next with n=14. Eleven patients were diagnosed with HLH. The hemophagocytosis pictures on bone marrow smear and mortality rate were significantly correlated with ferritin level above 6000 µg L-1 (p<0.01). The comparison of the HLH subgroup with the rest of the cohort showed that fever, cytopenia (anemia, leucopenia, neutropenia and thrombocytopenia), hemophagocytosis pictures, and major liver disturbances were significantly increased in the HLH subgroup. The H score was significantly elevated in the subgroup of patients with ferritin above 6000 µg L-1, with a significatively higher probability of HLH (p<0.01). The mortality rate at 3 months was significantly increased in the HLH subgroup. Extreme hyperferritin cannot be considered as a specific marker for the diagnosis. The cut off of 6000 µg L-1 is significantly associated with HLH diagnosis. The H score is an interesting screening tool that physicians should use to rule out the probability of HLH when facing critical clinical situations.
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Hiperferritinemia/etiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia/etiología , Niño , Preescolar , Citofagocitosis , Femenino , Ferritinas/sangre , Fiebre/etiología , Neoplasias Hematológicas/sangre , Neoplasias Hematológicas/complicaciones , Humanos , Hiperferritinemia/mortalidad , Infecciones/sangre , Infecciones/complicaciones , Sobrecarga de Hierro/sangre , Sobrecarga de Hierro/complicaciones , Linfohistiocitosis Hemofagocítica/diagnóstico , Masculino , Persona de Mediana Edad , Pancitopenia/etiología , Pronóstico , Valores de Referencia , Insuficiencia Renal Crónica/sangre , Insuficiencia Renal Crónica/complicaciones , Estudios Retrospectivos , Adulto JovenRESUMEN
Wells' syndrome, also called eosinophilic cellulitis, is a rare eosinophilic dermatosis characterized by an unspecific inflammatory erythematous eruption often associated with systemic symptoms. Here we report the case of a 57-year-old female with bilateral painful pitting and pruritic feet progressive for two weeks despite one week of oral antibiotics. Skin biopsy was performed showing dermal eosinophilic infiltration. The patient showed a spontaneous progressive improvement of the condition. The presented case demonstrates both clinical and histologic presence of lesions of Wells' syndrome in the course of the disease. A careful diagnostic approach is needed because of the lack of specific signs. The global outcome is favorable and spontaneous resolution is possible.
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Celulitis (Flemón)/complicaciones , Eosinofilia/complicaciones , Dermatosis del Pie/etiología , Biopsia , Celulitis (Flemón)/patología , Eosinofilia/patología , Femenino , Dermatosis del Pie/patología , Humanos , Persona de Mediana Edad , Fotograbar , Piel/patologíaRESUMEN
INTRODUCTION: To investigate the thrombotic tendency in patients with systemic lupus erythematosus (SLE) by evaluating congenital and acquired abnormalities with an increased risk of thrombosis. PATIENTS AND METHODS: A total of 53 patients with SLE were included in the study. Fifty-three healthy controls paired by age and sex were assessed. Anticardiolipin antibodies (aCL), anti ß2 glycoprotein (aß2GP), lupus anticoagulant (LAC), protein C (PC), protein S (PS), antithrombin (AT), acquired activated protein C, and homocysteinemia were evaluated. Comparisons for categorical variables were analyzed by Chi2 and student tests. RESULTS: The patients were all female and had a mean age of 30.6 years (16/58). The healthy controls were all female and their mean age was 30.8 years (17/56). Five patients (9.4%) developed venous thrombosis during the 24 months of follow-up. The antiphospholipid antibodies were positive in 17 patients (32.1%) and negative in all healthy controls (P=0.01). PS deficiency was noted in 17 patients (32.1%) and in 5 controls (P=0.004). Hyperhomocysteinemia was noted in 16 patients (30.2%) versus 3 controls (5.6%) (P=0.002). Test for PC deficiency and acquired activated protein C showed no significant difference between the two groups. No AT deficiency was found in the patients. The study of clinical and biological correlations based on the presence and absence of thrombophilic parameters concluded to a significant association between Protein C deficit and thrombosis (P=0.02) and acquired activated protein C resistance and thrombosis (P=0.04). There was no significant association between the APL and thrombosis. CONCLUSION: Thrombophilic abnormalities were significantly more frequent in lupus patients than in healthy controls. Thrombotic events were significantly associated with PC deficit and acquired protein C resistance. There was no correlation between antiphospholipid antibodies and thrombosis.
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Resistencia a la Proteína C Activada/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Deficiencia de Proteína C/complicaciones , Trombosis/etiología , Resistencia a la Proteína C Activada/sangre , Resistencia a la Proteína C Activada/diagnóstico , Adulto , Anticuerpos Anticardiolipina/sangre , Biomarcadores/sangre , Factores de Coagulación Sanguínea/análisis , Estudios de Casos y Controles , Femenino , Homocisteína/sangre , Humanos , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/diagnóstico , Deficiencia de Proteína C/sangre , Deficiencia de Proteína C/diagnóstico , Trombosis/sangre , Trombosis/diagnóstico , Microglobulina beta-2/inmunologíaRESUMEN
A 50-year-old man had a sudden severe abdominal pain. An abdominal enhanced computed tomographic scan revealed irregular stenosis of the celiac artery, dissection of the hepatic artery and of the left common iliac artery, and mid stenosis of the right renal artery with extended renal parenchymal hypodensity. After careful evaluation, the patient was diagnosed with fibromuscular dysplasia. Systematic head and neck CT angiography revealed right vertebral arterial stenosis and saccular aneurysm of the left vertebral artery. We have chosen conservative medical treatment for the multifocal fibromuscular dysplasia.
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Dolor Abdominal/etiología , Displasia Fibromuscular/complicaciones , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Behçet's disease is a systemic inflammatory disorder with a wide variety of cardiovascular complications. Intracranial aneurysm is extremely rare. Herein, we are presenting a 41-year-old male with Behçet's disease and a headache. A cranial computed tomography scan angiography showed unruptured right supra cavernus internal carotid aneurysm and right sylvian artery aneurysm. Treatment consisted of prednisone and azathioprine. Control imaging showed no aneurysm.
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Síndrome de Behçet/complicaciones , Aneurisma Intracraneal/etiología , Adulto , Humanos , Aneurisma Intracraneal/patología , MasculinoRESUMEN
Granulomatosis with polyangiitis (GPA) is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56%) were male, and 13 (44%) were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%), neurological (50%), ocular (33%), vascular (20%), ureteral (16%), and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA) was detected in 27 (90%) patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90%) and oral methotrexate in 3 patients (10%). Trimethoprime-sulfamethoxazole was used in 26 patients (86%). Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.
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Anticuerpos Anticitoplasma de Neutrófilos/sangre , Azatioprina/uso terapéutico , Ciclofosfamida/uso terapéutico , Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Metotrexato/uso terapéutico , Adulto , Antibacterianos/uso terapéutico , Biomarcadores/sangre , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Granulomatosis con Poliangitis/epidemiología , Hospitales Universitarios , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Recurrencia , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Túnez/epidemiologíaRESUMEN
Behçet's disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet's disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet's disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.
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Síndrome de Behçet/complicaciones , Cardiopatías/diagnóstico por imagen , Cardiopatías/etiología , Imagen por Resonancia Cinemagnética , Trombosis/diagnóstico por imagen , Trombosis/etiología , Tomografía Computarizada por Rayos X , Adulto , Anticoagulantes/uso terapéutico , Quimioterapia Combinada , Glucocorticoides/uso terapéutico , Cardiopatías/tratamiento farmacológico , Cardiopatías/epidemiología , Humanos , Inmunosupresores/uso terapéutico , Masculino , Prevalencia , Factores de Riesgo , Trombosis/tratamiento farmacológico , Trombosis/epidemiología , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento , Túnez/epidemiologíaRESUMEN
Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome.
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Antirreumáticos/uso terapéutico , Rituximab/uso terapéutico , Enfermedad de Still del Adulto/tratamiento farmacológico , Adulto , Femenino , Humanos , Enfermedad de Still del Adulto/diagnóstico , Resultado del TratamientoRESUMEN
Superior ophthalmic vein thrombosis is a rare entity, which may lead to serious vision complications. We report the unusual observation of a 78-year-old patient who developed exophtalmos, chemosis, and blurred vision due to isolated superior ophthalmic vein thrombosis. This rare complication is likely due to tamoxifen therapy. In contrast with published data, in the present case, cessation of tamoxifen therapy did not improve ocular symptoms.
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Antineoplásicos Hormonales/efectos adversos , Neoplasias de la Mama/tratamiento farmacológico , Carcinoma Ductal de Mama/tratamiento farmacológico , Exoftalmia/inducido químicamente , Oclusión de la Vena Retiniana/inducido químicamente , Vena Retiniana/patología , Tamoxifeno/efectos adversos , Anciano , Antineoplásicos Hormonales/administración & dosificación , Exoftalmia/diagnóstico , Femenino , Humanos , Imagen por Resonancia Magnética , Oclusión de la Vena Retiniana/diagnóstico , Tamoxifeno/administración & dosificaciónRESUMEN
Inflammatory optic neuropathy (ON) is a rare event in Behçet's disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.
Asunto(s)
Síndrome de Behçet/complicaciones , Enfermedades del Nervio Óptico/diagnóstico , Enfermedades del Nervio Óptico/etiología , Corticoesteroides/uso terapéutico , Adulto , Potenciales Evocados Visuales , Femenino , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Enfermedades del Nervio Óptico/tratamiento farmacológico , Enfermedades del Nervio Óptico/epidemiología , Prevalencia , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Túnez/epidemiología , Campos VisualesRESUMEN
Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion. The two main features of this syndrome are livedo racemosa and ischemic stroke. Here, we describe the case of a 37-year-old woman with a past medical history of Moskowitz's syndrome and migraine, and a 2-year history of livedo racemosa and Raynaud's phenomenon. She presented with acute aphasia related to cortical ischemic stroke. Extensive diagnostic workup allowed to diagnose a Sneddon's syndrome. Her symptoms improved on a platelet aggregation inhibitor (aspirin), and speech therapy. This condition can be associated with other disorders affecting small vessels such as illustrated in this case with Moskowitz's syndrome.