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1.
Autops Case Rep ; 7(4): 42-50, 2017.
Article in English | MEDLINE | ID: mdl-29259931

ABSTRACT

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.

2.
Autops. Case Rep ; 7(4): 42-50, Oct.-Dec. 2017. ilus
Article in English | LILACS | ID: biblio-905406

ABSTRACT

Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.


Subject(s)
Humans , Male , Adult , Bone Marrow/pathology , Embolism, Fat/pathology , Hemoglobin SC Disease/complications , Autopsy , Fatal Outcome , Hemoglobin SC Disease/diagnosis , Hemoglobin SC Disease/pathology
3.
Autops Case Rep ; 7(2): 15-26, 2017.
Article in English | MEDLINE | ID: mdl-28740835

ABSTRACT

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.

4.
Autops. Case Rep ; 7(2): 15-26, Apr.-June 2017. ilus
Article in English | LILACS | ID: biblio-905200

ABSTRACT

In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents. This entity characteristically presents reduced DLCO with preserved lung volumes and severe pulmonary hypertension, which is not observed in emphysema and fibrosis alone. We present the case of a 63-year-old woman with a history of heavy tobacco smoking abuse, who developed progressive dyspnea, severe pulmonary hypertension, and cor pulmonale over a 2-year period. She attended the emergency facility several times complaining of worsening dyspnea that was treated as decompensate chronic obstructive pulmonary disease (COPD). The imaging examination showed paraseptal emphysema in the upper pulmonary lobes and fibrosis in the middle and lower lobes. The echo Doppler cardiogram revealed the dilation of the right cardiac chambers and pulmonary hypertension, which was confirmed by pulmonary trunk artery pressure measurement by catheterization. During this period, she was progressively restricted to the minimal activities of daily life and dependent on caregivers. She was brought to the hospital neurologically obtunded, presenting anasarca, and respiratory failure, which led her to death. The autopsy showed signs of pulmonary hypertension and findings of fibrosis and emphysema in the histological examination of the lungs. The authors highlight the importance of the recognition of this entity in case of COPD associated with severe pulmonary hypertension of unknown cause.


Subject(s)
Humans , Female , Middle Aged , Hypertension, Pulmonary/pathology , Pulmonary Disease, Chronic Obstructive/pathology , Pulmonary Emphysema/pathology , Pulmonary Fibrosis/pathology , Autopsy , Dyspnea/diagnosis , Edema/diagnosis , Fatal Outcome , Lung/pathology , Pulmonary Heart Disease/diagnosis , Tobacco Smoking/adverse effects
5.
Autops Case Rep ; 6(3): 7-15, 2016.
Article in English | MEDLINE | ID: mdl-27818953

ABSTRACT

Spontaneous hemoperitoneum due to vascular injury is a life-threatening condition mostly associated with aortic or splanchnic arterial disease, which stems from atherosclerotic, inflammatory, or infectious origin. However, in 1976, Slavin and Gonzales described a nonatherosclerotic arterial disease that may render aneurysmal formation predominantly in the splanchnic arterial bed. The clinical presentation is diverse, but abdominal pain and shock prevail. We report the case of a middle-aged man who presented a hemoperitoneum due to a middle colic artery aneurysm rupture and died after undergoing a surgical treatment attempt. The preoperative imaging study revealed the presence of a huge hematoma in the epiplon retrocavity, and abdominal free liquid as well as extensive arterial disease with multiple aneurysms. The autopsy findings included hemoperitoneum, hematoma in the upper left abdominal quadrant, the surgical ligature of the middle colic artery, and histologic features consistent with segmental arterial mediolysis. The authors call attention to this rare entity and highlight the autopsy as a fundamental examination to accurately reach this diagnosis.

6.
Sao Paulo Med J ; 133(2): 115-24, 2015.
Article in English | MEDLINE | ID: mdl-26018881

ABSTRACT

CONTEXT AND OBJECTIVE: Noninvasive strategies for evaluating non-alcoholic fatty liver disease (NAFLD) have been investigated over the last few decades. Our aim was to evaluate the diagnostic accuracy of a new hepatic ultrasound score for NAFLD in the ELSA-Brasil study. DESIGN AND SETTINGS: Diagnostic accuracy study conducted in the ELSA center, in the hospital of a public university. METHODS: Among the 15,105 participants of the ELSA study who were evaluated for NAFLD, 195 individuals were included in this sub-study. Hepatic ultrasound was performed (deep beam attenuation, hepatorenal index and anteroposterior diameter of the right hepatic lobe) and compared with the hepatic steatosis findings from 64-channel high-resolution computed tomography (CT). We also evaluated two clinical indices relating to NAFLD: the fatty liver index (FLI) and the hepatic steatosis index (HSI). RESULTS: Among the 195 participants, the NAFLD frequency was 34.4%. High body mass index, high waist circumference, diabetes and hypertriglyceridemia were associated with high hepatic attenuation and large anteroposterior diameter of the right hepatic lobe, but not with the hepatorenal index. The hepatic ultrasound score, based on hepatic attenuation and the anteroposterior diameter of the right hepatic lobe, presented the best performance for NAFLD screening at the cutoff point ≥ 1 point; sensitivity: 85.1%; specificity: 73.4%; accuracy: 79.3%; and area under the curve (AUC 0.85; 95% confidence interval, CI: 0.78-0.91)]. FLI and HSI presented lower performance (AUC 0.76; 95% CI: 0.69-0.83) than CT. CONCLUSION: The hepatic ultrasound score based on hepatic attenuation and the anteroposterior diameter of the right hepatic lobe has good reproducibility and accuracy for NAFLD screening.


Subject(s)
Fatty Liver/diagnostic imaging , Multidetector Computed Tomography/methods , Non-alcoholic Fatty Liver Disease/diagnostic imaging , Adult , Aged , Body Mass Index , Brazil/epidemiology , Cholesterol/blood , Diabetes Complications , Fatty Liver/epidemiology , Female , Humans , Hypertriglyceridemia/complications , Longitudinal Studies , Male , Middle Aged , Non-alcoholic Fatty Liver Disease/epidemiology , Reproducibility of Results , Sensitivity and Specificity , Severity of Illness Index , Ultrasonography , Waist Circumference/physiology
7.
São Paulo med. j ; 133(2): 115-124, Mar-Apr/2015. tab, graf
Article in English | LILACS | ID: lil-746649

ABSTRACT

CONTEXT AND OBJECTIVE: Noninvasive strategies for evaluating non-alcoholic fatty liver disease (NAFLD) have been investigated over the last few decades. Our aim was to evaluate the diagnostic accuracy of a new hepatic ultrasound score for NAFLD in the ELSA-Brasil study. DESIGN AND SETTINGS: Diagnostic accuracy study conducted in the ELSA center, in the hospital of a public university. METHODS: Among the 15,105 participants of the ELSA study who were evaluated for NAFLD, 195 individuals were included in this sub-study. Hepatic ultrasound was performed (deep beam attenuation, hepatorenal index and anteroposterior diameter of the right hepatic lobe) and compared with the hepatic steatosis findings from 64-channel high-resolution computed tomography (CT). We also evaluated two clinical indices relating to NAFLD: the fatty liver index (FLI) and the hepatic steatosis index (HSI). RESULTS: Among the 195 participants, the NAFLD frequency was 34.4%. High body mass index, high waist circumference, diabetes and hypertriglyceridemia were associated with high hepatic attenuation and large anteroposterior diameter of the right hepatic lobe, but not with the hepatorenal index. The hepatic ultrasound score, based on hepatic attenuation and the anteroposterior diameter of the right hepatic lobe, presented the best performance for NAFLD screening at the cutoff point ≥ 1 point; sensitivity: 85.1%; specificity: 73.4%; accuracy: 79.3%; and area under the curve (AUC 0.85; 95% confidence interval, CI: 0.78-0.91)]. FLI and HSI presented lower performance (AUC 0.76; 95% CI: 0.69-0.83) than CT. CONCLUSION: The hepatic ultrasound score based on hepatic attenuation and the anteroposterior diameter of the right hepatic lobe has good reproducibility and accuracy for NAFLD screening. .


CONTEXTO E OBJETIVO: Estratégias não invasivas para avaliar doença hepática gordurosa não alcoólica (DHGNA) têm sido investigadas nas últimas décadas. Nosso objetivo foi avaliar a acurácia diagnóstica de um novo escore de ultrassonografia hepática para DHGNA no estudo ELSA-Brasil. TIPO DE ESTUDO E LOCAL: Estudo de acurácia diagnóstica realizado no centro ELSA, no hospital de uma universidade pública. MÉTODOS: Dos 15.105 participantes do estudo ELSA avaliados para DHGNA, 195 indivíduos foram incluídos neste estudo. Foi realizada ultrassonografia hepática (atenuação do feixe de profundidade, índice hepatorrenal e diâmetro anteroposterior do lobo direito do fígado) que foi comparada aos achados de esteatose hepática da tomografia computadorizada (TC) de alta resolução de 64 canais. Avaliamos também dois índices clínicos relacionados à DHGNA: o índice de gordura hepática (FLI) e o índice de esteatose hepática (HSI). RESULTADOS: Entre os 195 participantes, a frequência de DHGNA foi de 34,4%. Altos índices de massa corpórea, circunferência de cintura, diabetes e hipertrigliceridemia foram associados a aumento da atenuação hepática e do diâmetro anteroposterior do lobo hepático direito, mas não ao índice hepato-renal. A pontuação da ultrassonografia hepática, que incluiu atenuação hepática e diâmetro anteroposterior do lobo direito do fígado, apresentou o melhor desempenho para o rastreio DHGNA sob o ponto de corte ≥ 1 ponto (sensibilidade: 85.1%; especificidade: 73.4%; acurácia: 79.3%), e área sob a curva (AUC, 0,85, IC 95%: 0,78-0,91). FLI e HSI apresentaram menor desempenho (AUC 0,76; IC 95%: 0,69-0,83) comparados à TC. CONCLUSÃO: O escore de ultrassonografia hepática, que incluiu atenuação hepática e o diâmetro anteroposterior do lobo direito do fígado, possui boa reprodutibilidade e acurácia para o rastreio de DHGNA. .


Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Fatty Liver , Multidetector Computed Tomography/methods , Non-alcoholic Fatty Liver Disease , Body Mass Index , Brazil/epidemiology , Cholesterol/blood , Diabetes Complications , Fatty Liver/epidemiology , Hypertriglyceridemia/complications , Longitudinal Studies , Non-alcoholic Fatty Liver Disease/epidemiology , Reproducibility of Results , Sensitivity and Specificity , Severity of Illness Index , Waist Circumference/physiology
8.
Autops Case Rep ; 5(4): 65-70, 2015.
Article in English | MEDLINE | ID: mdl-26894047

ABSTRACT

Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman's colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients.

9.
Autops Case Rep ; 4(3): 31-37, 2014.
Article in English | MEDLINE | ID: mdl-28573116

ABSTRACT

Although the Pseudomonas aeruginosa infection is well known and frequently found in hospitals and nursing care facilities, many cases are also reported outside these boundaries. In general, this pathogen infects debilitated patients either by comorbidities or by any form of immunodeficiency. In cases of respiratory infection, tobacco abuse seems to play an important role as a risk factor. In previously healthy patients, community-acquired pneumonia (CAP) with P. aeruginosa as the etiological agent is extremely rare, and unlike the cases involving immunocompromised or hospitalized patients, the outcome is severe, and is fatal in up to 61.1% of cases. Aerosolized contaminated water or solutions are closely linked to the development of respiratory tract infection. In this setting, metalworking fluids used in factories may be implicated in CAP involving previously healthy people. The authors report the case of a middle-aged man who worked in a metalworking factory and presented a right upper lobar pneumonia with a rapid fatal outcome. P. aeruginosa was cultured from blood and tracheal aspirates. The autopsy findings confirmed a hemorrhagic necrotizing pneumonia with bacteria-invading vasculitis and thrombosis. A culture of the metalworking fluid of the factory was also positive for P. aeruginosa. The pulsed-field gel electrophoresis showed that both strains (blood culture and metalworking fluid) were genetically indistinguishable. The authors highlight the occupational risk for the development of this P.aeruginosa-infection in healthy people.

10.
Autops Case Rep ; 3(1): 5-14, 2013.
Article in English | MEDLINE | ID: mdl-31528592

ABSTRACT

Lung adenocarcinomas with a mixture of tubular or papillary pattern, sheet-like or trabecular architecture, eosinophilic cytoplasm with centrally located nuclei and alpha-fetoprotein-producing cells have been described as hepatoid adenocarcinomas. Hepatoid adenocarcinomas are mainly found in the stomach but rare cases in other organs have been described. Immunostaining for alpha-fetoprotein (AFP), hepatocyte paraffin 1 (HepPar-1) and thyroid transcription factor-1 (TTF-1) helps in the diagnostic workup. Tumor behavior is still not entirely known but it seems to be associated with early metastases. We report on a 66-year-old, heavy-smoker male patient who had a 10-month history of respiratory complaints and weight loss. At the time he was hospitalized, respiratory failure was already established. The computed tomography corresponded to a collapsed right lung due to a poorly defined expanding mass. The bronchoscopy revealed narrowing of the inferior and medium lobar bronchi. The patient developed irreversible shock and died. At the right lung inferior lobe was extensively replaced by a grayish diffuse neoplasia in a "pneumonia-like" gross pattern. Metastatic disease was found in the right adrenal gland and thoracic and abdominal lymph nodes. Microscopic dissemination through lymphatics, pleura, and airways was detected. Histological examination revealed a poorly differentiated adenocarcinoma with hepatoid features. Immunohistochemmistry stains were positive for keratin 7, polyclonal carcinoembryonic antigen (CEA) in a diffuse pattern, AFP and HepPar-1 antibody. TTF-1 showed a diffuse granular cytoplasmic staining of the neoplastic cells, and only focal nuclear positivity. Multiple bilateral emboli originated from deep venous thrombosis were present in large and medium branches of the pulmonary artery and contributed to the cause of death.

11.
Autops Case Rep ; 3(1): 45-51, 2013.
Article in English | MEDLINE | ID: mdl-31528597

ABSTRACT

Since the 1950s, aortic graft infections (AGIs) constitute one of the most feared complications after reconstructive vascular surgery. This complication is not frequent, ranging from 1% to 2% in the recently reported series; however, the high rate of death and morbidity after therapeutic attempts justifies its dreadful fame. The majority of cases occur during the first month after surgery. Staphylococcus aureus is the cause of 70% of the early infection cases. Late infections, on the other hand, are even rarer, showing a strong relationship with low virulence microorganisms, where Staphylococcus epidermidis is the main cause. Gram-negative bacteria are also observed in late infections, mainly when an aortic or graft enteric fistula is present. Treatment modalities are plenty, but still debatable. The authors report a case of a woman who was operated on 6 years ago for a reconstructive aortic aneurysm with the implantation of an infrarenal Dacron graft in the aorto bifemoral position. She looked for medical assistance with a 2-month history of weight loss, abdominal/back pain, and fever. Her clinical status rapidly deteriorated. A computed tomography of the abdomen disclosed the diagnosis of an AGI. The patient was promptly treated with antibiotics. Surgery was undertaken to explant the infected graft and another graft was placed into the axillobifemoral position. Culture from the infections site was negative. After surgery the patient quickly developed refractory septic shock and died immediately post-operatively.

12.
Autops Case Rep ; 3(2): 21-29, 2013.
Article in English | MEDLINE | ID: mdl-31528604

ABSTRACT

First described by Berti in 1866, gastric volvulus (GV) is an uncommon and potentially lethal entity. GV occurs when the stomach twists by more than 180º resulting in obstruction of the alimentary tract, visceral ischemia, necrosis, and perforation. It is classified according to the rotation axis in organoaxial, mesenteroaxial or a combination of both. The clinical presentation can be acute, and is usually severe or chronic, which sometimes may be asymptomatic. It predominantly occurs in the fifth decade of life, but children, mainly those under the age of 1 year, may be affected. No ethnicity or gender was observed to show predominance. This entity is related to gastric, diaphragmatic disorders as well as laxity of gastric ligaments. Acute GV may complicate with incarceration and strangulation of the stomach when gastric necrosis ensues. These cases show a mortality rate of 60%. The authors report the fatal case of a surgically treated GV in a 43-year-old female patient who looked for medical care only after 1 month of initial symptoms. Diagnosis was confirmed with a thoracic and abdominal axial computed tomography. Besides the entire stomach being herniated and twisted into the thoracic cavity, the pancreas was pulled up through the hiatal orifice, provoking acute pancreatitis. Because of gastric necrosis and perforation, gastroenteric fluid drained into the mediastinum and left pleural space. The postoperative outcome was unfavorable resulting in the patient's death. The authors call attention to the severeness of the disease, and therefore the need of precocity of diagnosis and surgical treatment.

13.
Autops Case Rep ; 3(4): 13-22, 2013.
Article in English | MEDLINE | ID: mdl-28584802

ABSTRACT

Although infective endocarditis (IE) has been described in reports dating from the Renaissance, the diagnosis still challenges and the outcome often surprises. In the course of time, diagnostic criteria have been updated and validated to reduce misdiagnosis. Some risk factors and epidemiology have shown dynamic changes since degenerative valvular disease became more predominant in developed countries, and the mean age of the affected population increased. Despite streptococci have been being well known as etiologic agents, some groups, although rare, have been increasingly reported (e.g., Streptococcus milleri.) Intracardiac complications of IE are common and have a worse prognosis, frequently requiring surgical treatment. We report a case of a middle-aged diabetic man who presented with prolonged fever, weight loss, and ultimately severe dyspnea. IE was diagnosed based on a new valvular regurgitation murmur, a positive blood culture for Streptococcus anginosus, an echocardiographic finding of an aortic valve vegetation, fever, and pulmonary thromboembolism. Despite an appropriate antibiotic regimen, the patient died. Autopsy findings showed vegetation attached to a bicuspid aortic valve with an associated septal abscess and left ventricle and aortic root fistula connecting with the pulmonary artery. A large thrombus was adherent to the pulmonary artery trunk and a pulmonary septic thromboemboli were also identified.

14.
Autops Case Rep ; 3(4): 29-36, 2013.
Article in English | MEDLINE | ID: mdl-28584804

ABSTRACT

Primary lung lymphoma is a rare entity accounting for approximately 0.3% of all primary neoplasia of the lung and includes diffuse large B-cell lymphoma (DLBL) and lymphomatoid granulomatosis (LYG). Considering that clinical features may be similar, whereas epidemiology, morphology, and radiological features are different, the authors report a case of a middle-aged man who presented multiple pulmonary nodules in the lower lobes and ground-glass opacities scattered bilaterally on computed tomography. Clinically, he presented a consumptive syndrome with respiratory failure and pleurisy, which progressed until death. The autopsy findings were consistent with lymphomatoid granulomatosis (LYG) grade 3/ diffuse large B-cell lymphoma (DLBL). The authors call attention to the difficulty of establishing an accurate diagnosis, mainly when the demonstration of EBV-infected atypical B-cells fails.

15.
Radiol. bras ; 45(4): 230-232, jul.-ago. 2012. ilus
Article in Portuguese | LILACS | ID: lil-647870

ABSTRACT

Tumores miofibroblásticos inflamatórios raramente acometem vias urinárias ou crianças, comumente mimetizando neoplasias malignas nos exames de imagem. Foram descritos apenas 35 casos desses tumores na bexiga de crianças, segundo a literatura recente. Os autores apresentam o caso de uma criança com um tumor miofibroblástico vesical que evoluiu favoravelmente após ressecção cirúrgica completa.


Inflammatory myofibroblastic tumors rarely affect the urinary tract or children, and frequently mimic malignancy on imaging studies. According to the recent literature, only 35 cases of such bladder tumors in children have been reported. The authors present the case of a child with a bladder myofibroblastic tumor with favorable progression following complete surgical resection.


Subject(s)
Humans , Female , Adolescent , Urinary Bladder Neoplasms/diagnosis , Pelvis , Abdominal Pain , Biopsy , Hematuria , Magnetic Resonance Spectroscopy , Tomography, X-Ray Computed , Ultrasonography, Doppler , Weight Loss
16.
Autops Case Rep ; 2(1): 7-17, 2012.
Article in English | MEDLINE | ID: mdl-31528556

ABSTRACT

Schistosomiasis is one of the most common parasitic diseases, still considered of public health significance. Acute schistosomiasis is of difficult diagnosis and therefore has been overlooked, misdiagnosed, underestimated and underreported in endemic areas. The delay between the exposure to contaminated water and the initial symptoms may explain this challenging diagnosis. Acute schistosomiasis is frequently reported in non-immune individuals while reinfection cases occurring in endemic areas is scarcely documented. The later usually shows a benign course but fatal cases do exist. The authors report a case of a young female patient, in the late puerperium, with a three-month history of weight loss, intermittent fever, cough, thoracic and abdominal pain and increased abdominal girth. Physical examination showed a tachycardia, tachypnea and hypotension. Laboratory tests showed a mild anemia, eosinophilia, and a slightly elevation of liver enzymes. Thorax and abdominal multidetector computed tomography evidenced a diffuse and bilateral pulmonary micronodules and peritoneal and intestinal wall thickening. The patient progressed rapidly to hepatic insufficiency, and death after respiratory insufficiency. An autopsy was performed and the findings were compatible with acute Schistosomiasis in a patient previously exposed to Schistosoma mansoni.

17.
Autops Case Rep ; 2(2): 25-29, 2012.
Article in English | MEDLINE | ID: mdl-31528568

ABSTRACT

The focal calcification or ossification of the ligamentum flavum is a rare cause of thoracic myelopathy and most often occurs among individuals of Japanese descent. It is rare in other ethnic groups and in individuals below the age of 50. It is most often described at the lower thoracic level, being uncommon in the lumbar region and rare in the cervical region. Here, we present the case of a 44-year-old White female patient who sought medical attention with an eight-month history of paraesthesia of the lower limbs and progressive difficulty in walking. The clinical profile, together with computed tomography and nuclear magnetic resonance imaging of the spine, led to a diagnosis of compressive thoracic myelopathy due to ossification of the ligamentum flavum in the thoracic and lumbar spine. The patient underwent laminectomy and dissection of some of the affected ligamentum flavum, without any intraoperative complications. After three months of clinical follow-up, the patient had progressed favorably, having no sensory complaints and again becoming ambulatory.

18.
Autops Case Rep ; 2(2): 43-47, 2012.
Article in English | MEDLINE | ID: mdl-31528571

ABSTRACT

Omental infarction is a rare cause of acute abdominal pain, and the cause of omental infarction itself remains unknown. The diagnosis of omental infarction is occasionally made intra-operatively, because it is poorly known and presents no specific clinical features. Therefore omental infarction should always be considered in the differential diagnosis of abdominal inflammatory processes, mainly those affecting the right lower quadrant. The increasing use of computed tomography in cases of acute abdomen has facilitated the diagnosis of omental infarction in the pre-operative period. Here, we present the case of a 36-year-old, overweight female patient with clinical symptoms of acute abdomen. Computed tomography of the abdomen revealed indications of omental infarction. The patient was treated conservatively with analgesics and a nonsteroidal anti-inflammatory. The clinical evolution was favorable.

19.
Autops Case Rep ; 2(3): 45-49, 2012.
Article in English | MEDLINE | ID: mdl-31528579

ABSTRACT

Since when the first transesophageal echocardiography (TEE) was undertaken in 1975, technological advances have made this diagnostic modality more reliable. TEE indications became widespread in cardiac and non-cardiac surgeries, intensive care units, and ambulatory clinics. The procedure is generally considered a safe diagnostic tool, but occasionally complications do occur. The insertion and manipulation of the ultrasound probe can cause oropharyngeal, esophageal, or gastric trauma. Although rare, these complications may present a mortality rate of up to 56% depending on the treatment approach and the elapsed time to the diagnosis. The authors report a case of a 65-year-old woman submitted to attempt a TEE in order to better study or diagnose an inter-atrial communication. After 3 days of the procedure, the patient was admitted to the hospital with edema, hyperemia of the anterior face of the neck, accompanied by systemic symptoms. The imaging diagnostic work-up evidenced signs of esophageal rupture and upper mediastinal involvement, the former confirmed by upper gastrointestinal endoscopy. The patient was treated with antibiotics and cervical and mediastinal drainage, with a favorable outcome.

20.
Autops Case Rep ; 2(3): 51-56, 2012.
Article in English | MEDLINE | ID: mdl-31528580

ABSTRACT

Sclerosing encapsulating peritonitis (SEP), also called encapsulating peritonitis, is a rare and benign cause of intestinal obstruction of unknown etiology. Its onset may be acute or subacute although there are some reports with a two-month history. More commonly, this entity is secondary to chronic peritoneal dialysis, ventriculoperitoneal and peritoneovenous shunting, the use of ß-blockers and systemic lupus erythematous. Recurrent episodes of bacterial peritonitis, intestinal tuberculosis, sarcoidosis, familial Mediterranean fever, gastrointestinal cancer, liver transplantation, intra-abdominal fibrogenic foreign bodies, and luteinized ovarian thecomas are also related to SEP. The idiopathic presentation is more rare. Abdominal pain, nausea, vomiting, weight loss, malnutrition, and clinically palpable mass characterize the clinical features. Diagnosis is frequently made with gross findings during surgery, imaging workup and histopathology. The authors report the case of a 36-year-old male patient with a 10-day history of abdominal pain that was operated on because of intestinal obstruction. Diagnosis was made preoperatively and confirmed by the intraoperative findings and histopathology.

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