[LYMPHOGRAPHY. ROUTINE TECHNIC].
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Athymic mice (C3H/HeN) parasitized by Brugia malayi develop gross lymphatic dilations at the chronic stage of infection. The hyperplastic endothelial cells and low fluid pressure of the lymphatics, characteristic of these infections, suggest that abnormal changes in these cells may play an important role in the dilation. We studied the lymphatic and vascular endothelium of parasitized mice for morphological changes by scanning and/or transmission electron microscopy. The lymphatic endothelium of dilated lymphatics was perturbed, scalloped, bulbous and highly indented. Numerous mononuclear and giant cells were closely apposed to the endothelial wall. Endothelial cells of both the lymphatics and the adjacent venules revealed no focal cytoplasmic lesions. Growth factor-dependent cell proliferation was significantly suppressed in vitro in endothelial cell cultures containing adult female worms, male worms or microfilariae. The actin cytoskeletal network appeared intact in these cells, and no gross changes in distribution were evident. Although the lymphatic walls were highly tortuous, our examination revealed no significant alterations in their morphology. Perivascular infiltration of activated mast cells, lymphocytes and monocytes/macrophages indicated polarization of inflammatory cells into the lymphatic tissue. It is possible that these inflammatory cells might induce temporal functional changes in the lymphatics of infected athymic mice.
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Vascular lesions are common in infants and children and can be grouped into two main classifications: vascular tumors and vascular malformations. Hemangiomas are proliferative lesions characterized by increased endothelial cell turnover. Vascular malformations are congenital abnormalities involving capillaries, veins and/or lymphatics. A multidisciplinary approach regarding diagnosis and treatment of vascular anomalies in children is crucial. Several options are available for the treatment of hemangiomas. Since January 2003, 14 cases of hemangioma located in the cervicofacial region were treated at "La Sapienza" University. Seven patients underwent surgical resection, using Varioscope, and seven laser therapy. The Varioscope is an operating microscope, which is mounted on the head. Specific advantages convinced the authors to use the Varioscope initially. Autofocus, a magnification range between 3.6-7.2X and automatic parallax control allow unlimited freedom of movement which alleviates the discomfort of keeping the same distance for a long time. Laser therapy can selectively destroy specific targets within the skin by using an appropriate wavelength and pulse duration. Laser treatment parameters are based on several factors, including lesion location, lesion morphology, and patient skin type. The purpose of our study was aesthetic and functional improvement in patients comparing surgical and laser therapy. We obtained an aesthetic improvement in 11 patients and local pain control in all patients. Analysis of the cosmetic results was subjective but all parents indicated satisfaction with their decision regarding their child's treatment.
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The lymphatic vasculature is essential for the maintenance of normal fluid balance and for the immune responses, but it is also involved in a variety of diseases. Hypoplasia or dysfunction of the lymphatic vessels can lead to lymphedema, whereas hyperplasia or abnormal growth of these vessels are associated with lymphangiomas and lymphangiosarcomas. Lymphatic vessels are also involved in lymph node and systemic metastasis of cancer cells. Recent novel findings on the molecular mechanisms involved in lymphatic vessel development and regulation allow the modulation of the lymphangiogenic process and specific targeting of the lymphatic endothelium. Recent results show that the homeodomain transcription factor Prox-1 is an important lymphatic endothelial cell (LEC) fate-determining factor which can induce LEC-specific gene transcription even in blood vascular endothelial cells (BECs). This suggests that the distinct phenotypes of cells in the adult vascular endothelium are plastic and sensitive to transcriptional reprogramming, which might be useful for future therapeutic applications involving endothelial cells. Vascular endothelial growth factor-C (VEGF-C) and VEGF-D are peptide growth factors capable of inducing the growth of new lymphatic vessels in vivo in a process called lymphangiogenesis. They belong to the larger family which also includes VEGF, placenta growth factor (PlGF) and VEGF-B, VEGF-C and VEGF-D are ligands for the endothelial cell specific tyrosine kinase receptors VEGFR-2 and VEGFR-3. In adult human as well as mouse tissues VEGFR-3 is expressed predominantly in lymphatic endothelial cells which line the inner surface of lymphatic vessels. While VEGFR-2 is thought to be the main mediator of angiogenesis, VEGFR-3 signaling is crucial for the development of the lymphatic vessels. Heterozygous inactivation of the VEGFR-3 tyrosine kinase leads to primary lymphedema due to defective lymphatic drainage in the limbs. Other factors that seem to be involved in lymphangiogenesis include the Tie/angiopoietin system, neuropilin-2 and integrin alpha 9. VEGF-C induces lymphatic vessel growth, but high levels of VEGF-C also resulted in blood vessel leakiness and growth. The VEGFR-3-specific mutant form of VEGF-C called VEGF-C156S lacks blood vascular side effects but is sufficient for therapeutic lymphangiogenesis in a mouse model of lymphedema. As VEGF-C156S is a specific lymphatic endothelial growth factor in the skin, it provides an attractive molecule for pro-lymphangiogenic therapy.
Of 111 patients presenting with acute forms of leukemia, 44% had a chromosomally abnormal cell line in the bone marrow at diagnosis. In each of the acute leukemia forms (myeloid, lymphatic, stem-cell), patients with karyotypic abnormalities showed mean and median survival times like those with normal chromosomes. Both groups showed a wide range of survival times. The mean and median survival times of patients with mixed populations of chromosomally normal and abnormal cells did not differ from those of patients with exclusively abnormal cells in the bone marrow. The karyotypic abnormalities associated with acute leukemia, as well as having no etiologic significance, probably do not determine the subsequent course of the leukemia.
Dermatology offers many clinical examples of abnormalities in the production, absorption and transport of lymph. Lymphatic thromboses also occur. Many diseases are not named in accordance with their underlying pathogenesis. Malignant lymphedema must be recognized immediately. The differential diagnosis of regional lymphedema is also part of dermatology. Stemmer sign is a clue to chronic lymphedema. Lymphoedema is first divided into primary and secondary forms, depending on the cause, and then subdivided into stages depending on severity. Complications of chronic lymphedema include recurrent erysipelas, superficial lymphangiectases, verrucous lymphedema (papillomatosis lymphostatica) and angiosarcoma, which is often multifocal. Established diagnostic approaches are available to correctly diagnose lymphatic disease. The treatment of chronic lymphedema centers around reducing the edema which then influences all of the other problems. The physical therapeutic approach to reducing blockage is the best way to achieve these goals.
A small group of acute leukemias can be classified by morphological, cytochemical, or immunological marker analysis neither as acute lymphatic leukemia nor as acute myeloid leukemia. These leukemias are referred to as acute undifferentiated leukemia (AUL) and make up 2%-7% of the acute nonmyeloid leukemias. These leukemias are poorly defined in the literature and are also sometimes referred to as AML-MO. Most of the definitions include in the morphological and cytochemical criteria the expression of myeloid antigens. Here the value of these markers and of other techniques used in the diagnosis of undifferentiated or minimally differentiated leukemia is discussed. More than half of the leukemias that are undifferentiated by morphology and cytochemistry on the light-microscopic level show a positive reaction for myeloperoxidase by electron microscopy, which points to an early myeloid differentiation of those leukemias. Immunological marker analysis in most cases is inconclusive. Most show a positive reaction for CD 13, CD 33 or other myeloid-associated markers. However, in about half of these leukemias co-expression of lymphatic markers is seen. In a small minority, only lymphatic markers are expressed. Cytogenetic abnormalities which are found in these leukemias vary in type, and antigen receptor rearrangements are not lineage specific. Receptor studies, gene expression, and in vitro culture studies may, in the near future, contribute substantially to our knowledge about the commitment of these undifferentiated or minimally differentiated blasts. Recent definitions for AUL and AML-MO based on these different techniques are discussed.
AIMS: To study the significance of lymphatic drainage disruption due to a surgical scar in sentinel node mapping (SNM) in breast cancer patients. METHODS: We reviewed patients with stage I breast cancer who had undergone SNM and had an old surgical scar in the ipsilateral breast. RESULTS: Of 534 breast cancer patients who had undergone SNM, five patients had an old scar in the ipsilateral breast. Inter-pectoral nodes, internal nodes, intramammary nodes, and contralateral axillary nodes were identified as sentinel nodes in three cases. In the remaining two cases, no sentinel lymph nodes were identified. CONCLUSIONS: An old surgical scar in the breast may cause lymphatic drainage disruption, resulting in abnormal radioactive colloid uptake during SNM.
Nuclear imaging in the definition of the components of the congenitally enlarged extremity is important in the design of a successful therapeutic regimen. In our series of 32 patients evaluated for primary lymphedema, 85% were determined to have abnormalities that were not purely lymphatic in origin. The multiscintigraphic approach defines the components of the lymphatic and vascular systems with the use of technetium-99m [99mTc] antimony sulfide colloid (99mTc Sb2S3) for lymphatic, 99mTc diethylenetriamine pentacetic acid (DTPA) for capillary-interstitial, and 99mTc-tagged red blood cells for venous systems.
We report a case of scrotal dermal chylorrhea associated with chyluria. This is an uncommon complication of chyluria and the only case recorded in our series of 206 patients. Lymphograms showed the presence of abnormal reflux from the inguinal lymphatics into the scrotum.
A markedly obese, 41-year-old Japanese man who had suffered from psoriasis vulgaris for several years visited us with elephantiasis-like swelling of his lower legs of three months' duration. His right lower leg showed marked papillomatosis with thick scales, and the left lower leg was eroded and papillomatous. Although direct lymphography of his lower extremities showed no abnormality, indirect lymphography revealed local lymphatic damage in the involved skin. Histological examination showed hyperkeratosis, marked papillomatosis, proliferation of capillaries in the upper dermis, and lymphectasia in the lower dermis. The lesions were much improved by washing and topical use of corticosteroids for two months. It was suspected that obesity and the preceding psoriatic lesions caused local lymphatic disturbances, followed by the development of stasis papillomatosis.
Abnormal growth and development of lymphatic pulmonary structures leads to severe hypoxia in congenital pulmonary lymphangiectasis (CPL). This case study aims to determine the cellular source and topographical distribution of the nitric oxide synthases in CPL. It studies the post mortem tissue of a term newborn with the clinical course and histological findings of CPL and three controls without pulmonary pathology. It was found that endothelial cells of pulmonary arteries and lymphatic structures stained significantly more for endothelial nitric oxide synthase protein in the CPL patient compared to the controls. The authors conclude that synthesis of endothelial nitric oxide synthase is upregulated in vascular and lymphatic endothelial cells in congenital pulmonary lymphangiectasis.
Congenital pulmonary lymphangiectasia is a rare abnormality with dilatation of pulmonary lymphatics and the radiological pattern of a pulmonary interstitial syndrome. It is usually symptomatic at birth and is almost always lethal. We report the case of an infant with congenital pulmonary lymphangiectasis and congenital heart disease who is still alive at 11 years. This case is interesting from the pathogenic, nosologic and prognostic point of view.
Congenital pulmonary lymphangiectasia is a rare abnormality with dilatation of pulmonary lymphatics and the radiological pattern of a pulmonary interstitial syndrome. It is usually symptomatic at birth and is almost always lethal. The authors report the case of an infant with congenital pulmonary lymphangiectasis and congenital heart disease who is still alive. This case report is interesting from the pathogenic, nosologic and prognostic point of view.