Hemophagocytic lymphohistiocytosis of indeterminate cause: a fatal adult case

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening disorder characterized by wide spread non-neoplastic proliferation and inappropriate activation of mature macrophages resulting in hypercytokinemia.This uncontrollable and ineffective systemic immune response causes fever, hepatosplenomegaly, cytopenias and subsequently multiorgan failure. The authors report a case of a 41-year-old male patient with a 30-day history of weight loss, fever, icterus, hepatomegaly, and cytopenias. The diagnosticworkup disclosed hypertriglyceridemia, hypofibrinogenemia, and elevated ferritin. Bone marrow examination and clinical course raised the suspicion of HLH and treatment was started with high-dose corticosteroids and immune globulin. The patient underwent multi-organ failure and expired after 58 days ofhospitalization. The autopsy finding included massive bone marrow infiltration by non-neoplastic histiocytes, many of them showing hemophagocytosis, which immunohistochemical study revealed diffuse CD68-positive histiocytes, which were negative for S100 protein. Hemophagocytosis was also observedin the lungs, lymph nodes and liver. The immediate cause of death was attributed to a massive intestinal bleeding due to extensive ischemic necrosis at the duodenum/jejunal transition area.nul

Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis

Angiomyolipomas (AMLs) are mesenchymal neoplasms, named so because\ud of the complex tissue composition represented by variable proportions of\ud mature adipose tissue, smooth muscle cells, and dysmorphic blood vessels.\ud Although AMLs may rise in different sites of the body, they are mostly observed\ud in the kidney and liver. In the case of renal AMLs, they are described in two\ud types: isolated AMLs and AMLs associated with tuberous sclerosis (TS). While\ud most cases of AMLs are found incidentally during imaging examinations and\ud are asymptomatic, others may reach huge proportions causing symptoms.\ud Pulmonary lymphangioleiomyomatosis (LAM) is a rare benign disease\ud characterized by cystic changes in the pulmonary parenchyma and smooth\ud muscle proliferation, leading to a mixed picture of interstitial and obstructive\ud disease. AML and LAM constitute major features of tuberous sclerosis\ud complex (TSC), a multisystem autosomal dominant tumor-suppressor gene\ud complex diagnosis. The authors report the case of a young female patient\ud who presented a huge abdominal tumor, which at computed tomography (CT)\ud show a fat predominance. The tumor displaced the right kidney and remaining\ud abdominal viscera to the left. Chest CT also disclosed pulmonary lesions\ud compatible with lymphangioleiomyomatosis. Because of sudden abdominal\ud pain accompanied by a fall in the hemoglobin level, the patient underwent an\ud urgent laparotomy. The excised tumor was shown to be a giant renal AML with\ud signs of bleeding in its interior. The authors call attention to the diagnosis of\ud AML and the huge proportions that the tumor can reach, as well as for ruling\ud out the TSC diagnosis, once it may impose genetic counseling implications

A list of land plants of Parque Nacional do Caparaó, Brazil, highlights the presence of sampling gaps within this protected area

Brazilian protected areas are essential for plant conservation in the Atlantic Forest domain, one of the 36 global biodiversity hotspots. A major challenge for improving conservation actions is to know the plant richness, protected by these areas. Online databases offer an accessible way to build plant species lists and to provide relevant information about biodiversity. A list of land plants of “Parque Nacional do Caparaó” (PNC) was previously built using online databases and published on the website "Catálogo de Plantas das Unidades de Conservação do Brasil." Here, we provide and discuss additional information about plant species richness, endemism and conservation in the PNC that could not be included in the List. We documented 1,791 species of land plants as occurring in PNC, of which 63 are cited as threatened (CR, EN or VU) by the Brazilian National Red List, seven as data deficient (DD) and five as priorities for conservation. Fifity-one species were possible new ocurrences for ES and MG states

Study of the Myocardial Contraction and Relaxation Velocities through Doppler Tissue Imaging Echocardiography: A New Alternative in the Assessment of the Segmental Ventricular Function

OBJECTIVE: Doppler tissue imaging (DTI) enables the study of the velocity of contraction and relaxation of myocardial segments. We established standards for the peak velocity of the different myocardial segments of the left ventricle in systole and diastole, and correlated them with the electrocardiogram. METHODS: We studied 35 healthy individuals (27 were male) with ages ranging from 12 to 59 years (32.9 ± 10.6). Systolic and diastolic peak velocities were assessed by Doppler tissue imaging in 12 segments of the left ventricle, establishing their mean values and the temporal correlation with the cardiac cycle. RESULTS: The means (and standard deviation) of the peak velocities in the basal, medial, and apical regions (of the septal, anterior, lateral, and posterior left ventricle walls) were respectively, in cm/s, 7.35(1.64), 5.26(1.88), and 3.33(1.58) in systole and 10.56(2.34), 7.92(2.37), and 3.98(1.64) in diastole. The mean time in which systolic peak velocity was recorded was 131.59ms (±19.12ms), and diastolic was 459.18ms (±18.13ms) based on the peak of the R wave of the electrocardiogram. CONCLUSION: In healthy individuals, maximum left ventricle segment velocities decreased from the bases to the ventricular apex, with certain proportionality between contraction and relaxation (P<0.05). The use of Doppler tissue imaging may be very helpful in detecting early alterations in ventricular contraction and relaxation