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1.
J Cancer Res Ther ; 16(3): 630-633, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32719279

RESUMO

Malignant peripheral nerve sheath tumors (MPNSTs) of parapharyngeal space are rare and if present are most often in association with neurofibromatosis type 1 (NF-1). Only a few cases of MPNST have been reported in the literature without coexisting NF. We report one such case of an MPNST of parapharyngeal space tumor in a 35-year-old female with no associated features of NF-1. She presented with right-sided neck swelling and ptosis. Magnetic resonance imaging showed a 7 cm × 8 cm × 11 cm irregular swelling in the right parapharyngeal space with invasion of surrounding muscles. The mass was excised using a transcervical approach. Postoperative histopathological examination of the specimen revealed MPNST possibly arising from the cervical sympathetic chain.


Assuntos
Neurofibrossarcoma/patologia , Neoplasias Faríngeas/patologia , Sistema Nervoso Simpático/patologia , Adulto , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Pescoço/diagnóstico por imagem , Pescoço/patologia , Neurofibrossarcoma/diagnóstico por imagem , Neurofibrossarcoma/cirurgia , Espaço Parafaríngeo/diagnóstico por imagem , Espaço Parafaríngeo/patologia , Neoplasias Faríngeas/diagnóstico por imagem , Neoplasias Faríngeas/cirurgia , Doenças Raras/diagnóstico por imagem , Doenças Raras/patologia , Doenças Raras/cirurgia , Sistema Nervoso Simpático/diagnóstico por imagem , Sistema Nervoso Simpático/cirurgia
2.
Ann Hematol ; 99(9): 1967-1977, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32621178

RESUMO

Thalassemia is characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin. This defect results in imbalance in the α/ß-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and iron overload. With advances in diagnosis, treatment, and transfusion support, the prognosis of patients with thalassemia has improved over the past few decades. An increasing number of patients with thalassemia is living with long-term complications, including cardiomyopathy, chronic liver disease, endocrinopathy, and infections. In this paper, we review common complications that bring the patient with thalassemia to urgent or emergent medical attention. We also discuss the aspects of emergency care that are most relevant while caring for the patient with thalassemia in the emergency department.


Assuntos
Serviços Médicos de Emergência/tendências , Serviço Hospitalar de Emergência/tendências , Doenças Raras/diagnóstico por imagem , Doenças Raras/terapia , Talassemia/diagnóstico por imagem , Talassemia/terapia , Betacoronavirus , Transfusão de Sangue/métodos , Transfusão de Sangue/tendências , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/epidemiologia , Cardiomiopatias/terapia , Infecções por Coronavirus/diagnóstico por imagem , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/terapia , Diagnóstico Diferencial , Serviços Médicos de Emergência/métodos , Humanos , Hepatopatias/diagnóstico por imagem , Hepatopatias/epidemiologia , Hepatopatias/terapia , Pandemias , Pneumonia Viral/diagnóstico por imagem , Pneumonia Viral/epidemiologia , Pneumonia Viral/terapia , Doenças Raras/epidemiologia , Talassemia/epidemiologia
3.
Rev. esp. patol. torac ; 32(2): 154-158, mayo 2020. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-193910

RESUMO

La proteinosis alveolar pulmonar es una enfermedad rara que se produce por un acúmulo anormal de lipoproteínas a nivel alveolar, siendo la broncoscopia una herramienta importante para su diagnóstico y tratamiento. Se presenta el caso de una mujer de 44 años, fumadora, con tos escasamente productiva blanquecina y disnea de tres meses de evolución, tratada de forma previa con antibioterapia por sospecha de neumonía adquirida en la comunidad, con escasa mejoría. La tomografía torácica mostró un patrón intersticial bilateral, compuesto por zonas en vidrio deslustrado, áreas geográficas y crazy paving. A nivel analítico, se observaron niveles aumentados de LDH e IgE junto a hipoxemia moderada, siendo la serología de VIH negativa. Las muestras de la broncoscopia fueron positivas para tinciones con ácido peryódico de Schiff, hallazgo compatible con el diagnóstico de proteinosis alveolar pulmonar. La paciente fue tratada con lavado broncoalveolar total, con buena respuesta y sin presentar recaídas hasta la fecha


Pulmonary alveolar proteinosis is a rare disease caused by an abnormal accumulation of lipoproteins at the alveolar level in which bronchoscopy is an important tool for diagnosis and treatment. We present the case of a 44-year-old woman, smoker, with a slightly productive cough producing milky sputum and dyspnea progressing over three months, previously treated with antibiotics for suspected community-acquired pneumonia with little improve-ment. The thoracic CT showed a bilateral interstitial pattern composed of areas of ground glass opacity, geographic areas and crazy paving. At the analytical level, increased levels of LDH and IgE along with moderate hypoxemia were observed, with negative HIV serology. Bronchoscopy samples were positive for Periodic Acid-Schiff staining, a finding compatible with the diagnosis of pulmonary alveolar proteinosis. The patient was treated with a full bronchoal-veolar lavage with a good response and she has not suffered a relapse to date


Assuntos
Humanos , Feminino , Adulto , Proteinose Alveolar Pulmonar/diagnóstico , Broncoscopia/métodos , Doenças Raras/diagnóstico por imagem , Proteinose Alveolar Pulmonar/patologia , Doenças Raras/patologia , Lavagem Broncoalveolar , Radiografia Torácica , Ceftriaxona/administração & dosagem , Levofloxacino/administração & dosagem , Prednisona/administração & dosagem , Tomografia Computadorizada por Raios X , Biópsia
4.
Indian J Pathol Microbiol ; 63(Supplement): S47-S49, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32108627

RESUMO

Collagenofibrotic glomerulopathy (CFG) is a rare idiopathic kidney disease characterized by abnormal deposition of atypical Type III collagen fibers in the glomerulus causing subendothelial and mesangial expansion, manifesting as progressive renal dysfunction accompanied by proteinuria. The majority of CFG cases reported in literature are from Japan where this disease entity was initially recognized. There is an increased awareness and diagnosis of this rare renal disease in India with the recent increase in utilization of electron microscopy (EM) in clinical diagnostic settings. We describe a 28-year-old Bangladeshi woman who presented with hypertension and nephrotic range proteinuria not amenable to treatment with steroids and cyclophosphamide, whose renal biopsy demonstrated diagnostic ultrastructural features of CFG. This illustrative case is presented to highlight the role of EM analysis for diagnostic accuracy in renal biopsy evaluation in addition to demonstrating the unusual renal biopsy findings of this rare entity.


Assuntos
Colágeno Tipo III/análise , Glomerulonefrite/diagnóstico por imagem , Nefropatias/diagnóstico por imagem , Glomérulos Renais/patologia , Doenças Raras/diagnóstico por imagem , Adulto , Biópsia , Feminino , Fibrose , Humanos , Índia , Rim/patologia , Microscopia Eletrônica de Transmissão , Proteinúria/etiologia , Doenças Raras/patologia
6.
Acta Med Port ; 33(1): 61-64, 2020 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-31928605

RESUMO

Granular cell tumors are uncommon, and are usually benign neoplasms that can mimic malignancy on breast imaging tests. These tumors can originate anywhere in the body and the breast accounts for only a few cases of all granular cell tumors. We report a case of a 54-year-old woman with a granular cell tumor of the breast presenting clinically on breast imaging (ultrasound, mammography and magnetic resonance) as a suspicious lesion. Core needle biopsy was performed for tissue diagnosis and was consistent with granular cell tumor that was confirmed in the histopathological report of the surgical specimen following a breast lumpectomy. Given the rarity of this tumor, we present this case to highlight this diagnostic hypothesis, that can be challenging and frequently confused with breast carcinoma.


Assuntos
Neoplasias Encefálicas , Tumor de Células Granulares , Doenças Raras , Biópsia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Mama/diagnóstico por imagem , Mama/patologia , Feminino , Tumor de Células Granulares/diagnóstico por imagem , Tumor de Células Granulares/patologia , Humanos , Imagem por Ressonância Magnética , Mamografia , Pessoa de Meia-Idade , Doenças Raras/diagnóstico por imagem , Doenças Raras/patologia
7.
Clin. biomed. res ; 40(1): 58-60, 2020.
Artigo em Inglês | LILACS | ID: biblio-1117425

RESUMO

Report of two cases of gastric diverticulum (GD) documented by upper gastrointestinal contrast radiographic studies and computed tomography (CT).


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Divertículo Gástrico/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem
9.
BMC Infect Dis ; 19(1): 952, 2019 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-31703612

RESUMO

BACKGROUND: Osteomyelitis of the pubic symphysis is a rare cause of pelvic pain after delivery, mainly caused by Staphylococcus aureus and Pseudomonas aeruginosa. The clinical context is the same as the more common diastasis of the pubic bone, but the presence of intense local pain in association with fever should prompt further clinical work-up based on blood chemistry, microbiology and diagnostic imaging. We report the first case of methicillin-resistant Staphylococcus aureus osteomyelitis of the pubic symphysis occuring after the delivery. CASE PRESENTATION: A 39-year-old woman developed pain over the pubic bone 12 h after the delivery. After 72 h fever rose and laboratory examination showed elevation of C-reactive protein and procalcitonin levels. Pelvic x-rays and magnetic resonance showed pubic diastasis, joint effusion, tiny irregularities of articular surfaces and, severe bone edema. The patient was started on broad spectrum intravenous (IV) antibiotics (piperacillin-tazobactam) and then replaced to IV vancomycin and oral levofloxacin based on antibiogram result. She was then discharged with oral antibiotic therapy and fully recovered. CONCLUSIONS: Due to the rarity of this disease, we compared our experience with the other cases of osteomyelitis of pubic symphysis occurring in peri-postpartum reported in the literature. The course of osteomyelitis was favourable in all patients, and only in one case an additional orthopedic procedure for symphysis fixation was necessary. Knowledge of this rare condition is important to enable prompt diagnosis and treatment.


Assuntos
Parto Obstétrico , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Osteomielite/microbiologia , Sínfise Pubiana/patologia , Infecções Estafilocócicas/microbiologia , Adulto , Antibacterianos/uso terapêutico , Feminino , Febre/tratamento farmacológico , Humanos , Levofloxacino/uso terapêutico , Imagem por Ressonância Magnética , Testes de Sensibilidade Microbiana , Osteomielite/diagnóstico por imagem , Osteomielite/tratamento farmacológico , Período Pós-Parto , Gravidez , Pseudomonas aeruginosa/isolamento & purificação , Sínfise Pubiana/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Doenças Raras/tratamento farmacológico , Doenças Raras/microbiologia , Infecções Estafilocócicas/tratamento farmacológico , Resultado do Tratamento , Vancomicina/uso terapêutico
10.
Rev Esp Enferm Dig ; 111(12): 965-967, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31663363
12.
Indian J Pathol Microbiol ; 62(4): 605-607, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31611451

RESUMO

Splenic cysts are rare with an overall incidence of 0.07% in a large autopsy series and 0.5% among all the splenectomies done. The parasitic splenic cysts, usually caused by Echinococcus granulosus, account for 60% of all primary splenic cysts. The primary epithelial cysts account for 10% of all splenic cysts. We report a case of 30-year-old female presenting with left upper abdominal pain and heaviness. Computed tomography revealed a multiloculated cyst in spleen. Hydatid serology was negative. Total splenectomy was done. Histopathological evaluation was done and a diagnosis of primary epithelial splenic cyst was given.


Assuntos
Cisto Epidérmico/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Baço/patologia , Esplenopatias/diagnóstico por imagem , Dor Abdominal/etiologia , Adulto , Feminino , Humanos , Baço/citologia , Esplenectomia , Tomografia Computadorizada por Raios X
13.
BMC Cancer ; 19(1): 888, 2019 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-31488091

RESUMO

BACKGROUND: Glomus tumors in the digital nerve are extremely rare. Multiple intraneural glomus tumors in different digital nerve fascicles have not been previously reported. CASE PRESENTATION: We report the case of a 54-year-old male with a 1-year history of progressive numbness of the middle finger with point tenderness at the level of the middle phalanx. Surgical incision revealed the presence of two glomus tumors within different fascicles of the ulnar digital nerve of the middle finger. One tumor was excised along with surrounding fascicle, the other was removed leaving the fascicle intact. Subsequently, the patient regained function of the finger and no tumors have recurred. CONCLUSIONS: Patients and physicians should be aware of the properties of intraneural glomus tumors so that early diagnosis and treatment can be sought.


Assuntos
Dedos/patologia , Tumor Glômico/cirurgia , Paraganglioma Extrassuprarrenal/cirurgia , Neoplasias do Sistema Nervoso Periférico/cirurgia , Doenças Raras/cirurgia , Neuropatias Ulnares/cirurgia , Competência Clínica , Tumor Glômico/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Paraganglioma Extrassuprarrenal/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Resultado do Tratamento , Nervo Ulnar/patologia , Neuropatias Ulnares/diagnóstico por imagem
14.
G Chir ; 40(3): 188-192, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31484007

RESUMO

INTRODUCTION: Although inguinal hernia repair is a routine procedure and frequently performed as one-day surgery, we should be particularly aware of the possible complications, which could be life-threatening if not recognized in due course. CASE REPORT: We report a case of life-threatening extra-peritoneal bleeding after open inguinal hernia repair requiring damage control surgery. DISCUSSION: Several vessels can be responsible for massive extraperitoneal bleeding - external iliac vessels, lower epigastric artery, crema steric vessels and corona mortis. Although damage control surgery was developed to treat the severe trauma, it can also be a life-saving maneuver in cases as the presented one. Hemodynamic instability with distended abdomen is a primary indication for laparotomy, but in some cases the contrast CT provides valuable information about the location and the size of hematoma and can guide the operative approach - midline laparotomy or revision of the wound as in our case. To the best of our knowledge, this is the first reported case of life-threatening retroperitoneal bleeding after open inguinal hernia repair. The present case is a good example for the application of damage control in pathology considered as one-day surgery. CONCLUSIONS: Although casuistic, the life-threatening bleeding after open hernia repair should be suspected. The prompt surgical response with damage control can be life-saving maneuver even in the routine hernia surgery. Detailed knowledge of the anatomy and carefull dissection are required to avoid this kind of complications.


Assuntos
Artérias Epigástricas/lesões , Hematoma/cirurgia , Hérnia Inguinal/cirurgia , Herniorrafia/efeitos adversos , Hemorragia Pós-Operatória/cirurgia , Doenças Raras/cirurgia , Terapia de Salvação/métodos , Idoso de 80 Anos ou mais , Feminino , Hematoma/diagnóstico por imagem , Humanos , Intestino Delgado , Hemorragia Pós-Operatória/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Reoperação , Espaço Retroperitoneal , Tomografia Computadorizada por Raios X
15.
G Chir ; 40(3): 217-224, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31484012

RESUMO

Clear cell sarcoma of the kidney is an uncommon renal neopla sm of childhood. It represents about 4% of childhood malignant neoplasms and is generally more common in children under 5 years of age. In the present article, we describe the case of a 12-year-old male patient who came to our observation with left renal mass and with a clinical-laboratory picture indicative of inflammatory pathology.


Assuntos
Neoplasias Renais/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Sarcoma de Células Claras/diagnóstico por imagem , Biópsia , Criança , Humanos , Rim/diagnóstico por imagem , Rim/patologia , Neoplasias Renais/patologia , Imagem por Ressonância Magnética , Masculino , Pielonefrite/diagnóstico , Doenças Raras/patologia , Sarcoma de Células Claras/patologia , Tomografia Computadorizada por Raios X , Ultrassonografia
16.
G Chir ; 40(3): 225-229, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31484013

RESUMO

INTRODUCTION: Jejunal adenocarcinoma is a very rare disease but the frequency of this rare carcinoma is higher in celiac patients. We report the first case report of a second jejunal loop adenocarcinoma associated with celiac disease. PRESENTATION OF CASE: A 47-year-old woman, with a history of celiac disease. Computerized tomographic scans of the abdomen and pelvis demonstrated a severe retroperitoneal lymphoadenopathy, para-aortic, inter-aorto-caval, porto-caval, posterior pancreaticoduodenal space, celiac trunk, lesser gastric curvature, lymph node grouping. The patient underwent digiunal resection and regional lymphadenectomy. Diagnosis was poorly differentiated jejunal adenocarcinoma, infiltrating subserosal adipose tissue, metastasing in five out of eight regional lymph nodes. U.I.C.C. 2017 grading = pT3 pN2 G3 R0; Stage IIIB. DISCUSSION: The jejunum accounts for 11-25% of small bowel adenocarcinoma, that accounts for less than 5% of gastrointestinal cancer, notwithstanding that 90% of the mucosa surface area of the digestive tract is made by small intestine. To the best of our knowledge, this is the first report on a second loop jejunal adenocarcinoma complicating celiac disease. In our study, the diagnosis of cancer was made by computed tomography (CT) of abdomen and the patient was operated. For the diagnosis of small bowel tumour, CT enteroclysis has a sensitivity of 85-95% and a specificity of 90-96%. Complete resection (RO) of the jejunal adenocarcinoma, with regional lymph nodes resection and jejuno-jejunal anastomosis should be performed. CONCLUSION: After curative surgical resections of small bowel adenocarcinoma, adjuvant chemo-therapy has not shown a clear benefit in retrospective studies. Preoperative Chemo-Radio-therapy and careful Imaging Staging are the first steps to planning surgery.


Assuntos
Adenocarcinoma/complicações , Doença Celíaca/complicações , Neoplasias do Jejuno/complicações , Doenças Raras/complicações , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/cirurgia , Feminino , Humanos , Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Jejuno/diagnóstico por imagem , Jejuno/cirurgia , Excisão de Linfonodo , Pessoa de Meia-Idade , Doenças Raras/diagnóstico por imagem , Doenças Raras/cirurgia , Tomografia Computadorizada por Raios X
18.
Heart Surg Forum ; 22(4): E287-E288, 2019 07 03.
Artigo em Inglês | MEDLINE | ID: mdl-31398093

RESUMO

The rupture of sinus of Valsalva aneurysm (SVA) into the pericardial cavity is extremely rare and fatal. A 52-year-old man presented with an abrupt onset of chest pain and dizziness. An echocardiography and a computed tomographic angiography revealed a giant aneurysm of the noncoronary sinus (NCS) (maximum, 70 mm) and pericardial effusion in favor of tamponade with a moderate degree of aortic insufficiency. On the way to the operating room, he suffered cardiac arrest, and extracorporeal cardiopulmonary resuscitation (ECPR) was initiated immediately. While he was prepared for surgery, the patient was supported by extracorporeal life support (ECLS) until cardiopulmonary bypass (CPB) was initiated. The sinus defect was reconstructed using a Dacron patch, followed by resection of the aneurysm. ECLS and CPB were discontinued successfully in the operating room. This study reports a case of an extracardiac rupture of SVA and the successful completion of surgery following ECPR.


Assuntos
Aneurisma Aórtico/cirurgia , Ruptura Aórtica/cirurgia , Reanimação Cardiopulmonar/métodos , Oxigenação por Membrana Extracorpórea/métodos , Doenças Raras/cirurgia , Seio Aórtico/cirurgia , Aneurisma Aórtico/diagnóstico por imagem , Ruptura Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Angiografia por Tomografia Computadorizada , Ecocardiografia Transesofagiana , Parada Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Derrame Pericárdico/diagnóstico por imagem , Doenças Raras/diagnóstico por imagem , Seio Aórtico/diagnóstico por imagem
19.
Rev Esp Enferm Dig ; 111(9): 712-713, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31368344

RESUMO

To the best of our knowledge, this is the first report of ectopic gastric mucosa appearing between muscularis mucosae and submucosa, at esophagogastric junction. Currently there are two theories explaining the mechanism. The most widely accepted one is that Ectopic gastric mucosa is an embryological remnant, and an alternative theory is abnormal proliferation under inflammation1.Although we don't have many evidences to clarify this case into specific mechanism mentioned above, Nomura et reported two cases of Adenocarcinoma of the cervical esophagus arising from ectopic gastric mucosa, Completely resected by ESD with no signs of recurrence2. Therefore, In this present case, the lesion was successfully managed with ESD. The symptoms were released after the operation and the patient remained well during a year follow-up. Long-term follow-up is recommended in case of recurrence or gastric cancer.


Assuntos
Coristoma/diagnóstico por imagem , Doenças do Esôfago/diagnóstico por imagem , Junção Esofagogástrica , Mucosa Gástrica , Adulto , Cárdia/diagnóstico por imagem , Coristoma/patologia , Doenças do Esôfago/patologia , Junção Esofagogástrica/patologia , Fundo Gástrico/diagnóstico por imagem , Humanos , Masculino , Doenças Raras/diagnóstico por imagem
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