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At least 793 records · Page 44Linked to original sources
PubMed1976

[A contribution to the pseudo-LE-syndrome. Investigations of the cardiovascular system, abdominal organs, lymphatic system and drug histories (author's transl)].

13 of 30 patients suffering from pseudo-LE-syndrome showed a usually reversible enlargement of the heart during the acute stage of the disease. In two patients carditis occurring in pseudo-LE-syndrome lead to congestive heart failure and an additional patient died in the acute stage of carditis. As opposed to Systemic Lupus Erythematosus hypertension did not occur in a higher frequency than normal. In five cases cardiac catheter examinations showed slight elevation of the end-diastolic pressure in the right ventricle, in two cases an abnormal high mean pressure in the right atrium and a systolic gradient at the pulmonic valve was found. -Scintigrams showed definite enlargement of the spleen and to a lesser degree enlargement of the liver was seen. Laparascopy showed multiple concretions after peritonitis. Lymphographic changes in the retroperitoneal lymph nodes and lymphatic ducts were not observed in contrast to rheumatic diseases. Drug histories in most cases discovered intake of Venopyronum dragees prior to onset of the disease. But recurrent attacks of the disease also occurred without further intake of the drug.

Adult↗
PubMed2000

Therapeutic tactics and late results in predominant truncal congenital malformation.

BACKGROUND: Congenital vascular malformations are rare vascular lesions of unknown etiology, non-degenerative or of inflammatory nature, which begin during embryological development; they are characterized by anomalies of the vascular system, apparently due to hemodynamic and metabolic disturbances. METHODS: Our diagnostic and therapeutic management in addition to the late results in 60, mainly truncal cases, out of 265 congenital vascular malformations, are analyzed in the present study. In a 20-year period 25,000 vascular examinations were carried out, among which 265 (1.06%) congenital vascular malformations (CVMs) were discovered, that is 77% (205/265) extra-truncal venous angiomata and 22.7% (60/265) truncal diffuse or localized types. The distribution of the above types was: 22 (36.6%) arteriovenous, 30 (50%) venous and 8 (13.4%) lymphatic. RESULTS: Surgery was carried out in 48.3% (29/60) of the truncal types of which 37.9% of the cases, on average, recurred 8 years later. Of the 22 arteriovenous malformations 20 patients were operated on (90%), of whom 35% (7/20) had a recurrence; of the 30 venous defects 30% (9/30) were operated on and 44.5% (4/9) of these had a recurrence. The recurrence rate rose to 50% (5/10) in cases of operative therapy of arteriovenous defects and to 20% (2/10) with combined surgical and non surgical methods. The recurrence incidence of venous defects with surgical treatment and sclerotherapy was 54.1% (6/11). CONCLUSIONS: Timely diagnosis, microsurgical techniques and highly specialized surgical and interventional experience are expected to improve these results significantly.

Abnormalities, Multiple↗
PubMed1982

Perfusion lung scan: an aid in detection of lymphangitic carcinomatosis.

Lymphangitic carcinomatosis is usually a late manifestation of metastatic disease. The patient usually presents with cough or dyspnea, and the chest radiograph is often nondiagnostic. Two patients are presented who developed symptoms while on adjuvant chemotherapy. Both had abnormal perfusion lung scans. One had matching ventilation defects; the other a normal ventilation study. Biopsy revealed metastatic carcinoma; in one case tumor was seen in both the pulmonary lymphatics and arterioles; in technique which can speed diagnosis and institution of therapy in lymphangitic carcinomatosis.

Adult↗
PubMed1986

Bullous morphea profunda.

A deep sclerotic process developed on the shins of a 58-year-old man, and eosinophilic fasciitis or morphea profunda was suspected clinically. Bullae later arose on the plaques, and histologic examination of a skin biopsy specimen revealed sclerosis and inflammation of the deep dermis, panniculus, and fascia, with subepidermal edema causing formation of bullae. No lymphatic obstruction or vasculitis was seen. Two plaques of typical morphea on the penis were noticed 10 months later. The patient had no peripheral or tissue eosinophilia, hypergammaglobulinemia, hematologic abnormality, or history of exertion before the onset of the disease. The sclerotic process involved more than the fascia. In describing this deeper variant of morphea, the term "morphea profunda" appears to be more appropriate than "eosinophilic fasciitis."

Foot Dermatoses↗
PubMed2004

Lymphangiogenesis in lymphangioleiomyomatosis: its implication in the progression of lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, and/or other organs. We examined lymphangiogenesis using immunohistochemistry for Flt-4 (VEGFR-3), a new specific marker for lymphatic endothelial cells, as well as the expression of vascular endothelial growth factor (VEGF)-C in LAM. Specimens were obtained from 6 autopsy cases, a single lung transplant case, and 8 surgical cases for analyses. We demonstrated that lymphatics were extremely abundant in both pulmonary and extrapulmonary LAM and that lymphatic endothelial cells not only proliferated encompassing LAM foci but also infiltrated the intra-LAM foci, and that in advanced LAM, lymphangiogenesis involved vascular walls and interstitium surrounding the area where LAM cells proliferate. In contrast, angiogenesis, confirmed with CD31 immunostaining, was observed less in the LAM foci. LAM cells demonstrated positive reactivity against anti-VEGF-C antibody at varying intensities. Significant correlation (P < 0.001) was noted between the degree of lymphangiogenesis in LAM or VEGF-C expression on LAM cells and lymphagioleiomyomatosis histologic score (LHS), which represents the histologic severity of pulmonary LAM and has been reported to have prognostic significance. Our study is likely to provide a novel point of view on the pathophysiologic significance of lymphangiogenesis in LAM.

Adult↗
PubMed2001

Blood borne transit of CJD from brain to gut at early stages of infection.

BACKGROUND: In Creutzfeldt-Jakob disease (CJD) and other related transmissible spongiform encephalopathies it is critical to understand the various pathways by which the infectious agent spreads to different organs. METHODS: We injected a CJD agent into mice, either intracerebrally (ic) or intraperitoneally (ip) and monitored the progressive appearance of abnormal PrP in peripheral tissues over time. RESULTS: Abnormal PrP was detected in lymphoreticular tissues of the gastrointestinal tract as early as 28 to 32 days after infection by both routes. This change persisted until the terminal stages of disease. In contrast, abnormal PrP was not detected in brain or spinal cord until 80 to 120 days after ic inoculation, or until 170 days after ip inoculation. CONCLUSIONS: Brain lacks significant lymphatic drainage, and has little infectivity before 40 days, even after ic inoculation. Thus the infectious inoculum must spread to the gut by a vascular route, a direction opposite to that generally assumed. This interpretation is consistent with previous studies demonstrating white blood cell infectivity as well as perivascular PrP accumulations in CJD. Notably, enteric infection at early as well as later stages of disease, and regardless of the route of agent entry, implicates potential environmental spread by fecal matter.

Animals↗
PubMed1975

Acute iron intoxication with intestinal infarction managed in part by small bowel resection.

This report deals with a case of acute iron intoxication in a child in which acute and chronic gastrointestinal abnormalities developed as a consequence of severe intestinal infarction and corrosion. Microscopic examination of resected small bowel revealed prominent iron deposits in areas of necrosis, in basement membranes of lymphatics, capillaries, and venules, and within platelet-fibrin thrombi. The clinical history and microscopic findings are consistent with the hypothesis of a direct action of absorbed iron on vascular walls as the pathophysiologic mechanism for the toxic effects of iron on the gastrointestinal tract. Acute and long-term management of iron intoxication is discussed in the context of the observations in this case.

Deferoxamine↗
PubMed1987

Rheumatoid factor and immune networks.

Rheumatoid factors represent a normal component of the immune network. The autoantibodies promote complement fixation and clearance of immune complexes. They amplify the avidity of polyclonally induced IgG. Genes related to the primary structure of rheumatoid-factor light chains are widely distributed in the human population and have been conserved during the evolution and dispersion of the species. Products of these genes may be detected with anti-idiotypic antibodies against synthetic peptides corresponding to individual hypervariable regions on rheumatoid-factor light chains. Such anti-peptide antibodies provide unique reagents for analyzing the genetics of immunoglobulins in outbred populations. Precursors of rheumatoid factor are abundant among immature B lymphocytes. Some of these cells may tend to localize to mucosal surfaces, where they are stimulated directly by pathogenic microorganisms with polyclonal B cell-activating properties. Synthesis of rheumatoid factor regularly accompanies all secondary immune responses but is usually transient. Production of the autoantibody is T-cell dependent. The T cells may recognize antigen in an IgG-antigen immune complex that is processed and presented by B-cell precursors of rheumatoid factor. Rheumatoid factor-associated light-chain idiotypes are rare in serum IgG and on IgG myeloma proteins. They are common among monoclonal IgM proteins and on the surface of the malignant B cells from patients with chronic lymphatic leukemia. The rheumatoid factors that are produced by patients with mixed cryoglobulinemia, or primary Sjogren's syndrome can share idiotypic antigens with monoclonal rheumatoid factors. Rheumatoid factor synthesis in the diseases may reflect an abnormal proliferation of B-cells that is not antigen-driven and that can degenerate into malignancy. The rheumatoid factors in patients with rheumatoid arthritis are diverse and almost certainly represent the outcome of antigen-induced, T cell-dependent mechanisms. The antigens that drive the T cells have not been identified but could represent exogenous microorganisms, self components, or idiotypic antigens that fortuitously interact with rheumatoid factors.

Autoantibodies↗
PubMed1985

Lymphographic abnormalities in patients with Brugia malayi filariasis and "idiopathic tropical eosinophilia".

Peripheral lymphography was carried out in 17 patients with elephantiasis and microfilaremia (Brugia malayi) and the findings compared to ten patients with "idiopathic tropical eosinophilia." There were extensive changes in peripheral lymphatic and regional nodal architecture in each group suggesting that "occult filariasis" is the cause of idiopathic tropical eosinophilia.

Adult↗
PubMed1989

Kawasaki disease and cervical adenopathy.

Kawasaki disease (KD) is an acute illness of unknown cause that affects infants and children. The diagnosis is confirmed in patients with prolonged fever and four of the following clinical features: (1) nonexudative conjunctivitis; (2) oral cavity changes; (3) rash; (4) extremity changes; and (5) cervical adenopathy. Complications of KD include coronary artery aneurysms, which may lead to myocardial infarction, chronic coronary insufficiency, or death. We describe a series of 83 patients with KD in whom 43 (52%) of 83 developed cervical adenopathy during their acute illness. Eighteen (42%) of these 43 patients were initially misdiagnosed as having cervical adenitis and were treated with antibiotics. The otolaryngologist may see these patients in referral and should consider the diagnosis of KD in patients with cervical adenopathy, prolonged fever, signs of mucosal inflammation, or rash. Early diagnosis and intravenous treatment with high-dose gamma-globulin is effective in reducing the prevalence of coronary artery abnormalities.

Child, Preschool↗
PubMed1980

Pulmonary function studies in adenoid hypertrophy.

Tests of pulmonary function were performed on children with severe adenoid hypertrophy, before and one month after adenoidectomy. Five types of subjects were selected: (1) normal; (2) cases with isolated increase of residual volume (RV); (3) supernormal type with increased RV; (4) obstructive type of ventilatory defect, and (5) restrictive type of ventilatory defect. Following adenoidectomy there is an objective evidence of improved pulmonary function. The data suggest that 65.7% of clinically normal children with adenoid hypertrophy show pulmonary function abnormalities.

Adenoidectomy↗
PubMed1983

ECG abnormalities in patients with sarcoidosis.

The frequency and type of some abnormal ECG findings in 86 consecutive patients with intrathoracic sarcoidosis were studied and compared with 86 age- and sex-matched healthy controls. All ECGs from the patients and controls were mixed and evaluated blindly by one of the investigators. Ectopic beats were seen in 6 patients in the sarcoidosis group and in 3 individuals in the control group. Patients with stage III sarcoidosis had more conduction defects compared with stage I and II patients and with the controls. Repolarization disturbances were found in 17 patients and 6 controls. These disturbances were more common in patients with stage I and II than in those with stage III sarcoidosis. The total number of patients with some ECG abnormality was 27 compared to 12 controls.

Adult↗
PubMed1994

Nuclear accumulation of p53 protein correlates with mutations in the p53 gene on archival paraffin-embedded tissues of human breast cancer.

Fifty invasive ductal carcinomas of the breast were analyzed by immunohistochemistry, polymerase chain reaction-single strand conformational polymorphism (PCR-SSCP) and direct sequencing after microdissection of conventional formalin-fixed, paraffin-embedded tissues. A highly significant association between the presence of p53 gene mutation and nuclear accumulation of p53 protein was found (P < 0.0001). Of 13 tumors that demonstrated p53 gene mutations, 11 (84.6%) showed nuclear accumulation of p53 protein. However, of 37 tumors in which gene mutations were not detected, only 5 (13.5%) showed nuclear accumulation of p53 protein. There was a statistically significant association between the nuclear accumulation of p53 protein and a higher histological grade (P < 0.001) or mitotic index (P < 0.01). In addition, gene mutations had a statistically significant association with a higher histological grade (P < 0.05) or mitotic index (P < 0.0001). Therefore, p53 abnormalities might be associated with an aggressive phenotype in breast cancer. We conclude that the immunohistochemical detection of nuclear p53 protein accumulation is highly associated with p53 gene mutations in archival paraffin-embedded tissues, and that this method is useful for rapid screening of p53 abnormalities.

Base Sequence↗
PubMedDate not supplied

Morphological observations and morphometric analysis in three human fetuses with bilateral cervical cystic hygroma.

Three human fetuses (crown-rump length, CRL, ranging from 71 to 77 mm), presenting bilateral cervical cystic hygroma were examined. The specimens were cleared and double-stained with alcian blue and alizarin red S for detecting the ossification growth patterns in the vertebral column, ribs, ischium, limbs, and face. Longitudinal measurements of some long bones in the upper (humerus, ulna, radius) and lower (femur, tibia, fibula) limb were taken. The values of both the total length (TL) and the ossified part (OL) of each long bone, as well as the OL/TL per cent ratio were considered. Reference points were located on the mandible, i.e. condylar process (Pcl), coronoid process (Pco), gnathion (GN), gonion (GO), inferior interdental point (IDI) for measuring linear dimensions. All values obtained were related with those relative to a group of fetuses, without any detectable malformation and chromosomal abnormalities, with CRL mean value 75 mm, in order to assess the presence of further anomalies, besides the cystic hygroma, in the three fetuses considered.

Anthropometry↗
PubMed1991

[Retrocervical cystic hygroma: diagnosis, prognosis and management. A series of 13 cases].

Fetal cystic hygroma (CH) are congenital malformation of the lymphatic system which are seen in antenatally ultrasonography from the end of the first trimester of pregnancy. From a 13 CH retrospective study, the authors discuss the ultrasound diagnosis, the prognosis and the management of CH. In our study, CH is associated with lymphedema (69%), hydrops (46%), diminution of amniotic fluid (69%) and other abnormality (31%). In all cases a diminution or an absence of fetal movement are found. In seven cases a cytogenetic analysis are performed on amniocentesis or fetal tissues: there are five 45XO, one 47XX + 13 and one 46XX. In six cases the chromosome culture failed: two male phenotypes and two histologic appearances of chromosomic anomaly. The prognosis depends on chromosome analysis, other ultrasound abnormality and the size of CH. It is usually fatal in utero. If chromosome anomaly are found, a genetic counsel is proposed.

Adult↗
PubMedDate not supplied

Study of capillary filtration by double labelling I131-albumin and Tc99m red cells. Application to the pharmacodynamic activity of Daflon 500 mg.

The Tc99m albumin test can be used to confirm idiopathic cyclic oedema (ICO) syndrome by a pathological retention of albumin of more than 8% of the maximum level, following release of the tourniquet (results obtained in 420 patients compared with 100 healthy women). This test was completed by a study involving double labelling using I131-albumin and Tc99m red cells in ten healthy subjects and ten female patients with untreated ICO syndrome. The tests were repeated at a six week interval. Evaluation of retention levels after tourniquet release showed nil retention of red cells in both populations and nil albumin retention in the healthy subjects whilst it was pathological in the patients with ICO. The curve of decreasing radioactivity was often marked by irregular oscillations after tourniquet removal. Study of these oscillations by the Fast Fourier Transform revealed reproducible abnormalities in the low frequency zone only, between 37 and 28 mHz in the ICO patients. These abnormalities took the form of more frequent peaks with higher amplitudes than in the healthy subjects and were linked--in the case of excessive leak of albumin from capillaries--to lymphatic resorption. In the high frequency zone between 630 and 39 mHz, results corresponding to variations related to arteriovenous compliance were similar in both populations and by both tests. A double blind placebo-controlled trial in 30 patients with ICO was carried out to study the pharmacodynamic activity of a flavonoid, Daflon 500 mg (2 tabs daily for 6 weeks), which revealed a decrease in the degree of retention--initially high--of labelled albumin (p = 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗