[Chyluria in lymphatic vessel anomalies].
Explore the source record for details and available documents.
Chyle↗
SEARCH · Search PubMed
Results for “Lymphatic Abnormalities”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
[Basic study and clinical evaluation of interpolated sagittal and coronal reconstruction of CT images in the screening of neck abnormalities].
Explore the source record for details and available documents.
Evaluation Studies as Topic↗
[Pigmentation disorders in systemic sclerodermia].
Skin pigmentation changes in systemic sclerosis have been known since the end of the last century. Many different aspects have been described. We report the case of a 48-year-old man with severe progressive systemic sclerosis who presented many different types of pigmentation changes of skin, but particularly a severe hyperpigmentation. This clinical observation suggests that local factors, including repetitive friction, trauma or variations in skin temperature, may influence the development, distribution and severity of pigmentation abnormalities in patients with systemic sclerosis. Physical treatments (connective tissue massage and lymphatic drainage) were very beneficial for our patient (healing of skin ulcerations and decrease of pigmentation.
Drainage↗
Prognostic factors in the treatment of lymphatic malformations.
OBJECTIVE: To find factors that may influence the treatment outcomes of lymphatic malformations of the head and neck in children. DESIGN: Charts of patients treated surgically for lymphatic malformations of the head and neck between 1988 and 1996 at our tertiary care children's hospital were reviewed retrospectively. Outcomes were correlated with age at presentation, associated symptoms, anatomical site (s) of involvement, extent of disease, length of time between first symptoms and surgery, completeness of removal, and histologic pattern. PATIENTS: Of 85 children treated, 74 underwent primary surgical excision at our hospital. Follow-up ranged from 6 months to 8 years, with a mean of 3 years. RESULTS: The overall recurrence rate, judged by functional or cosmetic deformity, was 22%. Two neonates died of the disease. Factors associated with a better prognosis were a single anatomical site of involvement; location in the neck, even if involving 2 sites; and the impression of completeness of resection at the time of surgery. Findings associated with a higher recurrence rate included younger age (especially neonates) and the presence of associated symptoms (ie, infection, dyspnea, dysphagia, and hemorrhage). The histologic pattern and the length of time from diagnosis to treatment were not significantly associated with the prognosis. CONCLUSIONS: We recommend aggressive, timely surgical excision for lymphatic malformations of the head and neck. The timing of surgery should be based on the child's functional and cosmetic deformity at the time of presentation and on the likelihood of complete excision, weighed against the morbidity associated with surgical excision.
Adolescent↗
Mesenteric lymphatic malformation associated with small-bowel volvulus - two cases and a review of the literature.
Cystic mesenteric lymphatic malformations are uncommon abdominal masses, which can cause abdominal signs and symptoms for a wide variety of reasons. Symptoms owing to volvulus are uncommon and have not been illustrated previously in the radiology literature. We present two cases to illustrate volvulus as a complication of mesenteric lymphatic malformations and how imaging can aid in the diagnosis of this complication. While uncommon, volvulus should be considered in any child with sonographic findings of an abdominal lymphatic malformation and acute abdominal pain.
Abnormalities, Multiple↗
Infantile hemangioma is a proliferation of LYVE-1-negative blood endothelial cells without lymphatic competence.
Infantile hemangiomas are common benign vascular tumors that exhibit a characteristic history of rapid proliferation in the first year of life and slow spontaneous involution during early childhood. The causative pathogenic event responsible for the abnormal endothelial proliferation remains elusive. The recent discovery of an immature phenotype of proliferating hemangioma endothelial cells due to the exclusive expression of the lymphatic endothelial hyaluronan receptor LYVE-1 led to the proposal that infantile hemangiomas are the result of a primary defect in endothelial cell maturation. To test this hypothesis, we looked for the expression of the lymphatic endothelial cell-specific markers LYVE-1, Prox-1, podoplanin and D2-40 in beta4 integrin-negative proliferating and beta4 integrin-positive involuting infantile hemangiomas. As beta4 integrin proved to be a suitable marker for staging infantile hemangiomas, we used it in combination with clinical and histological criteria to objectively determine the proliferative and involutional phases. In immunohistochemical and immunofluorescent stains, hemangioma vessels were negative for all lymphatic endothelial cell-specific markers tested during both proliferation and involution. LYVE-1 immunoreactivity, however, was found in the dense network of perivascular HLA-DR-positive cells with dendritic cell morphology that are supposed to play a role in hemangiogenesis by releasing pro- and antiangiogenic factors. Notably, this LYVE-1 staining failed to correlate with the growth status of infantile hemangiomas. Our results do not support the notion that LYVE-1 expression was restricted to the proliferative phase and downregulated during involution. Thus, LYVE-1 does not seem to be a reliable marker for proliferating infantile hemangiomas. We conclude that the suggested intrinsic defect in endothelial cell maturation is unlikely the cause for the post-natal rapid growth in infantile hemangiomas. In addition, the lack of lymphatic endothelial cell-specific markers implies that infantile hemangiomas are tumors of blood vessels without lymphatic competence.
Antigens, CD34↗
MR imaging of the spleen: spectrum of abnormalities.
The spleen has the same relationship to the circulatory system that the lymph nodes have to the lymphatic system. A wide range of diseases can affect the spleen. Pathologic conditions of the spleen can be classified into the following categories: congenital diseases (accessory spleen, polysplenia, and asplenia); trauma; inflammation (abscess, candidiasis, histoplasmosis, and sarcoidosis); vascular disorders (infarction, diseases affecting the splenic vasculature, and arteriovenous malformation); hematologic disorders (sickle cell disease and extramedullary hematopoiesis); benign tumors (cysts, hemangioma, diffuse hemangiomatosis of the spleen, and hamartoma); malignant tumors (sarcoma, lymphoma, and metastases); and other disease processes that affect the spleen diffusely (portal hypertension, Gaucher disease, and sickle cell disease) or focally (Gamna-Gandy nodules). New magnetic resonance (MR) imaging techniques have increased the role of MR imaging in detection and characterization of splenic diseases. MR imaging is an excellent tool for diagnosis and evaluation of focal lesions and pathologic conditions of the spleen.
Humans↗
Experimental model for MDS-like myelodysplasia in transgenic mice harboring the SV40 large-T antigen under an immunoglobulin enhancer.
The SV40 large T gene under the control of immunoglobulin enhancer induced hyperproliferation of multi-lineage hematopoiesis in transgenic mice. Hence the disease has been considered to be an appropriate experimental model for MDS-like myelodysplasia, sequential pathological changes in the development of the disease are introduced in the report. Huge splenomegaly was the major gross abnormality, which developed with 100% frequency; neither hepato-renal, nor other thymico-lymphatic involvement was common. During the progressive increase in splenic weight, extensive proliferation of multi-lineage hemopoiesis was prominent, although no differences were apparent in the cellular proportions of each hematopoietic element compared with normal spleens, either in flow-cytometric analysis using markers for each subset of hematopoietic elements, or in the histological findings. In the later phases of the disease, the proliferating cell type tended to shift to a variety of single to oligo-lineage hemopoiesis, but the majority of mice still showed the presence of multi-lineage hemopoiesis; histologically, such hemopoiesis was somewhat dysplastic, but had no apparent nature of leukemic infiltration. Several transplantation-assays essentially supported the low neoplastic potential of proliferating cells even in later phase. A long-term observation was made aiming to induce more frequent transition of this abnormal hemopoiesis into a single-lineage neoplasm by transplantation of pre-onset spleen cells, as well as bone-marrow cells from transgenic mice at an early phase of the disease, into lethally irradiated C57BL/6 mice. This trial resulted in a variety of neoplastic growths in the recipients; not only was myelodysplastic hypercellularity seen, but also, single-lineage hemopoietic malignancies, such as B-cell lymphomas/leukemias, histiocytic malignancies, and even myeloid leukemias. The transition from multi-lineage myelodysplasia into single lineage hemopoiesis at some frequency is reminiscent of myelodysplastic syndromes (MDS) in humans. Higher frequency of transition into lymphoid malignancies may be due partly to the immunoglobulin enhancer used as a promoter unit. The results that the SV40 large T antigen was expressed in every proliferating cells, there was no apparent increase in multi-CSFs activity; together with the results of the transplantation assays suggest that the hyperproliferation of the cells is directly induced by the expression of SV40 large T antigen in the hemopoietic cells themselves.
Animals↗
Foxc2 is expressed in developing lymphatic vessels and other tissues associated with lymphedema-distichiasis syndrome.
The molecular events involved in lymphatic development are poorly understood. Hence, the genes responsible for hereditary lymphedema are of great interest due to the potential for providing insights into the mechanisms of lymphatic development, the diagnosis, prevention and treatment of lymphedema, and lymphangiogenesis during tumor growth. Mutations in the FOXC2 transcription factor cause a major form of hereditary lymphedema, the lymphedema-distichiasis syndrome. We have conducted a study of Foxc2 expression during mouse development using immunohistochemistry, and examined its expression in lymphatics compared to its paralog Foxc1 and to Vegfr-3, Prox1 and other lymphatic and blood vascular proteins. We have found that Foxc2 is expressed in lymphatic primordia, jugular lymph sacs, lymphatic collectors and capillaries, as well as in podocytes, developing eyelids and other tissues associated with abnormalities in lymphedema-distichiasis syndrome.
Animals↗
Metastatic adenocarcinoma to the uterine cervix from gastric cancer. A clinicopathologic analysis of 16 cases.
BACKGROUND: Metastatic adenocarcinoma to the uterine cervix from gastric cancer is rare, and the clinicopathologic features of this metastasis are unclear. METHODS: A clinicopathologic review of 16 patients with metastatic adenocarcinoma to the uterine cervix from gastric cancer was performed. RESULTS: The ages of the patients ranged from 29 to 57 years, and 81.3% of the patients were premenopausal. Nine of the patients had undergone gastrectomy previously. In 11 patients the histologic type of the gastric cancer was poorly differentiated adenocarcinoma and, in 5 patients, signet ring cell carcinoma. The cervical metastasis was diagnosed 11-121 months (mean, 57.5 months) after the diagnosis of the gastric cancer in 10 of the patients. In six patients, the cervical metastasis was discovered synchronously or before the diagnosis of the gastric cancer. The colposcopic findings were normal in 57.1%, but 56.3% had abnormal cervical smears. In all patients, tumor cells were present in the dilated lymphatics of the cervix. Metastases to the uterine body and bilateral ovaries were common, and half of the patients had metastases to the paraaortic lymph nodes. Extirpation of the cervix was performed in six patients. The prognosis was poor, regardless of the treatment method. CONCLUSIONS: The route of metastasis to the cervix is surmised to be retrograde lymphatic, and this extension is often slow. Periodic gynecologic examinations should be performed indefinitely for premenopausal female patients with advanced gastric cancer.
Adenocarcinoma↗
From the archives of the AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation.
Lymphangioleiomyomatosis (LAM) is an uncommon interstitial lung disease that exclusively affects women, usually during their reproductive years. LAM is characterized pathologically by abnormal proliferation of LAM cells in the lungs and in thoracic and retroperitoneal lymphatics. Thirty-three cases of LAM were reviewed retrospectively for clinical and radiologic findings. Twenty-eight (85%) of 33 women (aged 21-62 years; mean, 37.5 years) were symptomatic. Radiographs (n = 32) demonstrated reticular opacities in 21 (66%) patients, large lung volumes in 17 (53%), pleural effusion in 14 (44%), and pneumothorax in 13 (41%). High-resolution CT (n = 15) and conventional CT (n = 3) showed 2-5-mm bilateral thin-walled cysts in all patients and cysts that were 6-12 mm or larger in patients with severe lung involvement. CT depicted diffuse lung involvement by cysts in nine (50%) patients, relative sparing of lung apices in seven (39%), and relative sparing of lung bases in two (11%). Pleural effusion and pneumothorax were seen at CT in four (22%) and three (17%) patients, respectively. Four cases of tuberous sclerosis complex-associated LAM (TSC-LAM) (women aged 27-50 years; mean, 35.7 years) were similarly reviewed. Three (75%) were symptomatic. Radiographs (n = 4) demonstrated reticular opacities in three (75%) and large lung volumes in two (50%). All high-resolution CT (n = 3) and conventional CT (n = 1) studies showed 2-5-mm bilateral thin-walled cysts and cysts that were 6-12 mm or larger in two patients with severe lung involvement. Pleural effusion and pneumothorax were demonstrated at CT in three (75%) and two (50%) patients, respectively. LAM and TSC-LAM affect symptomatic women who often exhibit reticular opacities and large lung volumes at radiography and bilateral uniform small thin-walled cysts at CT. Large (>12 mm) cysts occur in patients with severe cystic lung involvement. Pneumothorax and pleural effusion are common associated findings.
Adult↗
Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study.
A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common iliac lymph nodes. Abdominal CT scan revealed a possible periaortic mass. Small intestinal biopsies demonstrated normal villous architecture with dilated lacteals, and electron microscopy revealed enterocytes with normal as well as blunted microville, enlarged, dilated lacteals and intercellular vacuoles. An exploratory laparatomy was performed and revealed no masses but dilated serosal lymphatic vessels. Medical treatment, including marked restriction of long chain triglyceride intake, resulted in loss of peripheral edema, weight stabilization, and normal activity.
Adolescent↗
Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features.
A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O(2)).
Antibodies, Neoplasm↗
[Anesthetic approach in 2 patients with pulmonary lymphangiomyomatosis].
Pulmonary lymphangiomyomatosis (PLM) is an idiopathic disease of females in fertile age. It results in respiratory failure characterized by obliteration of the small airways, emphysema, formation of bullae, hemoptysis, pneumothorax, pulmonary fibrosis, severe hypoxemia and reduced carbon monoxide diffusion capacity. The major lymphatic ducts are also involved, resulting in chylous pleural effusion and ascites. Pulmonary abnormalities improve objectively and subjectively after surgical therapy, which consists in bilateral oophorectomy. Its evolution results in death in no more than 10 years. We report the anesthetic approach to 2 patients with PLM. Patient 1 was a female who had already been diagnosed of PLM and had received medical and surgical therapy, requiring reoperation for the resection of cystic intestinal masses and abdominal eventration. Patient 2 had been scheduled for bilateral oophorectomy after a diagnosis of PLM. We discuss the clinical condition of both patients, the course of the disease, the previous treatments and the anesthetic technique in each case.
Adult↗
Recombinant interferon alpha in the treatment of acquired immune deficiency syndrome-related Kaposi's sarcoma.
Acquired immune deficiency syndrome (AIDS)-related Kaposi's sarcoma (KS) is more virulent than previously reported cases of KS and affects a much younger population. The cell of origin is lymphatic endothelium, with the histopathology characterized by a proliferation of small vessels with abnormal endothelial cells, extravasated erythrocytes, and spindle-shaped-cell infiltration. Clinical manifestations are pigmented, nodular lesions affecting the skin, mucous membranes, lymph nodes, and/or visceral organs. The head and oral cavity, uncommon sites in other KS populations, are frequently involved. Pain is not an early part of the clinical picture. AIDS-related KS is often widespread and rapidly progressive. However, the cause of death in most cases is attributed to opportunistic infection, which is believed to increase as a result of conventional cytotoxic chemotherapy. Recombinant interferon alpha has been the most thoroughly tested of immune-stimulating or nonimmunosuppressive drugs for the treatment of AIDS-related KS. An objective antineoplastic response rate (25% to 60%) comparable to single-agent chemotherapy with vinblastine or VP-16 has been demonstrated in several trials. Response rates to interferon alpha may be enhanced by such factors as absence of lymphoma-like B symptoms, low levels of circulating acid-labile interferon alpha, and absent history of recent serious infection. Varied drug dose, schedule, and route of administration have been employed; doses equal to or greater than 30 million U/day administered intravenously or intramuscularly appear to give the best results. Daily dosing regimens may induce tolerance for subjective toxicities (eg, fever, asthenia, and anorexia), which are often dose limiting. Direct immune stimulation following treatment with interferon alpha has not been conclusively established, but evidence suggests that respondents have low rates of opportunistic infection. Other recent studies demonstrate interferon alpha inhibiting activity against the AIDS-associated retrovirus in vitro and trials in vivo are in progress to define the clinical relevance of this observation.
Acquired Immunodeficiency Syndrome↗
Angiographic assessment of lymphatic metastasis from gastric cancer.
Lymph node metastasis was evaluated angiographically in 151 patients with gastric cancer. In 20% of the patients with nodal metastases, abnormally staining masses were demonstrated by angiography. Superselective angiography is useful in confirming the presence of lymph node involvement.
Aged↗
Plasma protein abnormalities in cancer.
Explore the source record for details and available documents.
Blood Proteins↗