Acute Disseminated Panniculitis Associated with Alpha-1 Antitrypsin Deficiency.

Alpha-1 antitrypsin deficiency, although one of the most common genetic diseases, is a very rare and often undiagnosed cause of panniculitis. The authors present a case characterized by an acute involvement of several areas in the thorax, abdomen, and limbs, occurring after repetitive trauma of the perineal area caused by a long period of cycling. After performing the differential diagnosis and establishing etiology, the patient was started on augmentation therapy with plasma-derived synthetic human alpha-1 proteinase inhibitor and the disease has been under control since then. We recommend lifelong treatment with this medication. At the end of a 10-year follow-up, there has been no evidence of pulmonary emphysema or liver disease. The authors perform a concise review of the genetic and pathogenic mechanisms behind this disease, with a special focus on panniculitis and its treatment.

Acute bivalvular left-sided methicillin-resistant Staphylococcus aureus endocarditis with cardiac, cerebral, renal and septic complications.

Infective endocarditis (IE) is now rare in developed countries, but its prevalence is higher in elderly patients with prosthetic valves, diabetes, renal impairment, or heart failure. An increase in health-care associated IE (HCAIE) has been observed due to invasive maneuvers (30% of cases). Methicillin-resistant Staphylococcus aureus (MRSA) and Enterococcus are the most common agents in HCAIE, causing high mortality and morbidity. We review complications of IE and its therapy, based on a patient with acute bivalvular left-sided MRSA IE and a prosthetic aortic valve, aggravated by congestive heart failure, stroke, acute immune complex glomerulonephritis, Candida parapsilosis fungémia and death probably due to Serratia marcescens sepsis. The HCAIE was assumed to be related to three temporally associated in-hospital interventions considered as possible initial etiological mechanisms: overcrowding in the hospital environment, iv quinolone therapy and red blood cell transfusion. Later in the clinical course, C. parapsilosis and S. marcescens septicemia were considered to be possible secondary etiological mechanisms of HCAIE.

Hepatic Abscess due to Streptococcus anginosus and Eikenella corrodens, Secondary to Gastric Perforation by a Fish Bone.

INTRODUCTION: Foreign-body ingestion is a common event, but in only less than 1% of the cases complications occur. Hepatic abscesses induced by foreign-body penetration are rare. To date, there are only 62 reported cases of hepatic abscess secondary to fish bone perforation of the gastrointestinal tract. CASE PRESENTATION: A 78-year-old male patient was admitted due to high fever and vomiting for 2 days, along with frequent eructations for the past 3 months. Abdominal ultrasound showed a liver abscess in the left lobe, and computed tomography revealed a hyperdense linear image that crossed the superior wall of the gastric antrum, contacting the liver lesion, suggestive of a foreign body, probably a fish bone. Blood cultures were positive with isolation of Streptococcus anginosus and Eikenella corrodens. Ultrasound-guided percutaneous drainage of the abscess was done, and S. anginosus was isolated in the pus. Surgical debridement and fish bone removal were performed; the patient completed 21 days of antibiotic therapy, with a favorable evolution. CONCLUSION: To the best of our knowledge, this is the first reported case of liver abscess caused by fish bone penetration with isolation of S. anginosus and E. corrodens. Bacterial coaggregation is one of the mechanisms that can explain their ability for causing invasive infections away from the oral cavity, by increasing their resistance to the innate immune system and survival of both species.

Malignant hypertension, severe hypertensive retinopathy and malignant nephroangiosclerosis.

Hypertensive crises are frequent in the emergency room. The term covers a group of syndromes that have in common high blood pressure. When accompanied by acute organ damage, it is known as a hypertensive emergency, as opposed to a hypertensive urgency, in which there is no such damage. One specific type of emergency is malignant hypertension, in which there are exudates and/or retinal hemorrhages or papilledema. Besides effective and prompt treatment, it is mandatory to screen for secondary causes of the hypertension. We present the case of a 40-year-old patient, with no previous history of hypertension, who was admitted for malignant hypertension. In this article we review the pathophysiology, clinical aspects and prognosis of this condition.

Cardiac angiosarcoma--a review.

Based on a case of a patient with angiosarcoma (AS) of the right atrium with superior vena cava syndrome associated with urticaria and polyarthralgias, who died soon after surgery, the authors present a brief review of the subject of cardiac AS, an extremely rare pathology, usually diagnosed late due to its non-specific symptomatology. Several topics are discussed, including mechanisms of clinical manifestations caused by blood flow obstruction and valve dysfunction, local invasion with arrhythmias and pericardial effusion, embolic phenomena and constitutional symptoms. Imaging and histopathologic methods of diagnosis are considered, as well as references to cytogenetic analysis. Surgery is the first treatment choice, but heart AS are frequently not completely resectable and concomitant metastases at the time of surgery are common, both usually leading to a dismal prognosis. Chemotherapy, radiotherapy and even heart transplantation do not substantially improve the survival of these patients. Urticaria is not generally assumed by most authors to be associated with malignancy, but there are rare reports of its association with some malignant tumors.

[Pulmonary embolism associated to HIV infection]. / Tromboembolismo pulmonar associado a infecção VIH.

Antiphospholipid antibodies are frequently found in patients with HIV infection mainly in advanced forms of disease. Despite its elevated prevalence the association with thrombotic events is rare. The author present a clinical case of a HIV patient in which the first manifestation of the disease was a pulmonary embolism.

Hipercalcemia paraneoplásica de carcinoma anaplásico da tireoide / Paraneoplastic hypercalcemia of anaplastic thyroid carcinoma: case report

A hipercalcemia deve ser considerada no diagnóstico diferencial de alterações neuropsiquiátricas agudas. Em 90% dos casos, a etiologia corresponde a hiperparatireoidismo primário ou neoplasias. Valores séricos superiores a 14mg/dL e sintomáticos são frequentemente tradutores de causa maligna. O carcinoma anaplásico da tireoide consiste em um tumor indiferenciado, com progressão rápida e prognóstico reservado, que evolui, em alguns casos, a partir de lesões tireóideas preexistentes, benignas ou malignas (desdiferenciação). Embora a apresentação clínica mais frequente destes tumores consista no desenvolvimento de massa cervical, eles podem ser diagnosticados no esclarecimento etiológico de metástases ou síndromes paraneoplásicos. A hipercalcemia, associada à neoplasia, pode ocorrer em contexto de metástases ósseas, com libertação de citocinas, ou por mecanismo humoral, mediada pela proteína relacionada ao hormônio hormônio paratireóideo (PTHrP). Os autores descrevem o caso de uma mulher de 85 anos, com antecedentes de bócio multinodular benigno, internada para esclarecimento etiológico de hipercalcemia grave, com manifestações neuropsiquiátricas, diagnosticando-se, após avaliação, carcinoma anaplásico da tireoide. O caso foi abordado em reunião multidisciplinar, optando-se por limitação terapêutica a cuidados paliativos. A doente faleceu 3 meses após o diagnóstico.(AU)

Hypercalcaemia should be considered in the differential diagnosis of acute neuropsychiatric disorders. In 90% of the cases, the etiology corresponds to primary hyperparathyroidism or neoplasms. Serum values above 14mg/dL and symptomatic are often indicative of a malignant cause. The anaplastic thyroid carcinoma consists of an undifferentiated tumor, with rapid progression and poor prognosis, which in some cases progresses from pre-existing benign or malignant thyroid diseases (dedifferentiation). Although the most frequent clinical presentation of these tumors consists of the development of a cervical mass, they can be diagnosed in the etiological clarification of metastases or paraneoplastic syndromes. Neoplasm-associated hypercalcaemia may occur in the context of bone metastasis, with release of cytokines, or through a humoral mechanism, mediated by the parathyroid hormone (PTHrP)-related protein. The authors describe the case of an 85-year-old woman with a history of multinodular benign goiter, hospitalized for etiological elucidation of severe hypercalcaemia with neuropsychiatric manifestations, with a final diagnosis of anaplastic thyroid carcinoma, after the diagnostic evaluation. The case was approached in a multidisciplinary meeting, and the therapeutic limitation to palliative care was chosen. The patient died 3 months after the diagnosis.(AU)

Aplasia medular transitória associada a infecção por parvovírus B19 / Transient aplastic crisis caused by Parvovirus B19 infection

O parvovírus B19 é um eritrovírus humano com tropismo para as células progenitoras da medula óssea, sendo responsável por um grande espectro de manifestações clínicas, desde infecções assintomáticas até crises aplásicas graves. Os autores apresentam o caso de uma mulher de 40 anos, com história de anemia ferropênica por menorragias, que desenvolveu quadro clínico com febre, cefaleias, petéquias e, osteriormente, exantema nas pernas, associado à hipoplasia medular com redução transitória da contagem de todas as linhagens celulares hematológicas. A suspeita de infecção aguda por parvovírus B19 foi confirmada pela detecção de anticorpos IgM antiparvovírus B19 no sangue periférico, por meio de teste imunoenzimático (ELISA). Os achados do mielograma no 5o dia após a admissão, apesar de haver ainda tão só recuperação parcial das linhagens celulares hematológicas no sangue periférico, revelaram linhagens celulares medulares normais. A paciente teve recuperação espontânea, apenas com terapêutica de suporte.

Parvovirus B19 is a human erythrovirus with tropism for erythroid progenitor cells. It is responsible for a wide range of clinical manifestations, from asymptomatic infections to severe aplastic crises. The authors present the case of a 40 year-old female patient with history of iron-deficiency anemia from menorrhagia who presented with fever, headache, petechiae,and later,rash on lower limbs, associated with medullary hypoplasia and transient decrease of all hematologic cell lines.The suspicion of acute Parvovirus B19 infection was confirmed by the detection of anti-Parvovirus B19 IgM antibodies through Enzyme-Linked Immunosorbent Assay (ELISA). Although there was only partial recovery of the hematologic cell lineage in peripheral blood, findings on myelogram 5 days after admission showed normal hematopoietic cell lines. The patient had spontaneous recovery only with supportive treatment.

Thoracic empyema - a review based on three cases reports.

Complicated parapneumonic effusion is one in which an invasive procedure is necessary for its resolution and empyema means pus in the pleural space. An early diagnosis and therapy of these conditions results in less morbidity and mortality. CT of the chest is important to study complex pleural effusions. Loculated effusions, those occupying more than 50% of the thorax, or which show positive Gram stain or bacterial culture, or a purulent effusion with a pH below 7.20, with a glucose level below 60 mg/dl or a LDH level more than three times the upper limit of normal for serum, are indications for an invasive procedure. These characteristics result from the evolution of a not well treated parapneumonic effusion, through the three stages: (1) exsudative; (2) fibrinopurulent; (3) fibrotic. Depending on the stage therapeutic methods vary from therapeutic thoracentesis, insertion of a chest tube with or without instillation of fibrinolytics, video-assisted thoracoscopic surgery, and lung decortication. A review of all these aspects are done based on a series of three cases reports with very different clinical presentation: one patient with empyema by Streptococcus pyogenes and that died rapidly due to massive hemoptysis; a patient with empyema due to acute pneumonia developing during an airflight; a patient with empyema and bacteraemia by Streptococcus pneumonia leading to the diagnosis of an unknown HIV infection.

Blood loss anemia due to adenocarcinoma of the jejunum: case report and review of the literature.

BACKGROUND: Small bowel tumors are rare, accounting for only 3-6% of gastrointestinal neoplasms, 1-2% of these being malignant. They must be considered whenever a patient presents with gastrointestinal bleeding, with normal upper gastrointestinal endoscopy and colonoscopy. CASE PRESENTATION: We report a case of jejunal adenocarcinoma presenting as a blood loss anemia in a 65 year-old male, doing a brief review on the subject. CONCLUSION: Our case intends to highlight the fact that small bowel tumours are rare and frequently present to the Internist as non-specific clinical symptoms.