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PubMedDate not supplied

[Hygroma colli in the ultrasound image as an early indication of fetal Ullrich-Turner syndrome].

Fetal chromosomal anomalies are frequently associated with malformations of the lymphatic system, which occur with a wide variability as local edema or generalized hydrops of the fetus. From January 1, 1988 to December 31, 1989 chromosomal analysis were performed in two patients, because ultrasound examination revealed large cystic septated sacs in the nuchal area and severe fetal edema. In both cases the diagnosis was: Turner's syndrome XO. As in more than a half of all cases with nuchal hygromes in ultrasound Turner's syndrome and furthermore in 25% other chromosomal anomalies are found, the value of early and subtle ultrasound examination is to be emphasized. In every case with a sonographic suspicion of an abnormal nuchal area of the fetus, specific prenatal diagnostic investigations should be performed, because of the rare, but possible event of involution of fetal cystic hygroma in ultrasound.

Adult↗
PubMed1993

Prenatal diagnosis of a multifocal lymphangioma.

Lymphangiomas or cystic hygromas are malformations of the lymphatic vessels and are characterized by single or multiple cysts which have developed within the soft tissues. They occur most commonly in the neck (75 per cent of cases) and are often associated with hydrops or chromosomal abnormalities (Romero et al., 1988). Mediastinal or abdominal locations are rare and represent less than 5 per cent of cases in the literature (Singh et al., 1971). This paper represents the first reported case of prenatal diagnosis of a lymphangioma in the neck, mediastinum, and abdomen. Diagnosis was made before fetal viability, and allowed us to recommend termination of the pregnancy because of the poor prognosis.

Abdominal Neoplasms↗
PubMed2006

Campest-5-en-3-one, an oxidized derivative of campesterol, activates PPARalpha, promotes energy consumption and reduces visceral fat deposition in rats.

Dietary campest-5-en-3-one (campestenone), an oxidized derivative of campesterol, significantly reduced visceral fat weight and the concentration of triacylglycerol in serum and liver of rats. Dietary campestenone dramatically increased the activities and the mRNA expressions of mitochondrial and peroxisomal enzymes involved in beta-oxidation in the liver. Campestenone activated human peroxisome proliferator-activated receptor (PPAR) alpha as determined using the novel GAL4 ligand-binding domain chimera assay system with coactivator coexpression. In contrast, dietary campestenone reduced the activities and the mRNA expressions of enzymes involved in fatty acid synthesis, except for the malic enzyme. Dietary campestenone decreased the sterol regulatory element binding protein-1 (SREBP-1) mRNA level. Energy expenditure was significantly higher in the feeding of campestenone in rats. Dietary campestenone reduced hepatic cholesterol concentration and increased fecal excretion of neutral steroids originated from cholesterol. Lymphatic absorption of cholesterol was reduced by the coadministration of campestenone in rats cannulated in the thoracic duct. These observations suggest a possibility that campestenone has an ability to prevent coronary heart disease by improving obesity and abnormality of lipid metabolism.

Animals↗
PubMed1977

The lymphogram in abdominal tuberculosis.

Lymphograms of 11 patients with histologically proven abdominal tuberculosis have been assessed in an attempt to define a typical pattern of appearance in retroperitoneal lymph glands. Glandular enlargement, poor glandular filling and lymphovascular obstruction were present. A gradient of abnormality with more marked adenopathy in the upper para-aortic chain extending to a lesser involvement below in the iliac chain was demonstrated in keeping with a retrograde spread of the tuberculosis process from the abdominal lymphatics cauded along the retroperitoneal chain. Lymphography may be valuable in cases where data from clinical observations and routine radiological studies is inconclusive in cases of vague abdominal disease.

Adolescent↗
PubMed1994

[The relation between p53 expression and lymph node metastasis of breast cancer].

The monoclonal antibody to p53 and a rapid immunohistochemical method were used in this study to detect p53 expression in 50 paraffin-embedded breast cancers and 7 benign hyperplastic diseases of breast. The results of immunostaining showed that 12 (24%) cases of breast cancers were positive and all benign lesions were negative. No significant difference was found between primary and metastatic sites of 11 paired samples of breast cancers in p53 expression, suggesting that the abnormal expression of had p53 occurred before lymph node metastasis of breast cancer. Statistical analysis revealed that the abnormal expression of p53 was significantly correlated with lymph node metastasis of breast cancer (P < 0.005) and also correlated with medullary breast cancer in which two of four cases strongly positive to p53 had lymph node metastasis. Our results indicate that the abnormal expression of p53 is not only involved in the development, but also involved in the metastasis of breast cancer. Therefore, detection of p53 expression may have significant clinical importance in the evaluation of the tendency of lymphatic metastasis of breast cancer.

Antibodies, Monoclonal↗
PubMed2001

Sclerosing cholangitis and systemic lymphadenopathy.

A 48-year-old man was admitted to our hospital with jaundice and systemic lymphadenopathy. Cholangiographic findings and liver histology disclosed the presence of sclerosing cholangitis. The patient also had a marked polyclonal increase in IgG levels. The cholangiographic findings, the systemic lymphadenopathy, and the increase in IgG levels resolved completely after treatment with prednisolone. This case suggests that there is an association between sclerosing cholangitis and immunologic abnormalities, and that corticosteroid treatment is useful for this disorder.

Cholangitis, Sclerosing↗
PubMed2005

Achlorhydria, parietal cell hyperplasia, and multiple gastric carcinoids: a new disorder.

We describe a 54-year-old woman who had multiple gastric carcinoid tumors arising in the setting of marked hypergastrinemia associated with a lack of acid production by hypertrophic parietal cells. The serum gastrin level was 1,400 pg/mL, and investigation revealed no evidence for either of the recognized causes for hypergastrinemia-associated carcinoids, autoimmune gastritis, and Zollinger-Ellision syndrome. Partial gastrectomy was performed. Pathologic examination showed multiple intramucosal and invasive carcinoid tumors of the body and fundus in a background of marked ECL cell hyperplasia. There were no gastric or duodenal ulcerations. One perigastric lymph node was metastatically involved. The oxyntic mucosa showed marked hyperplasia and hypertrophy of the parietal cells. Some of these cells were vacuolated, and many displayed protrusions of apical cytoplasm into dilated oxyntic glands filled with inspissated eosinophilic material. Similar findings have occurred in 1 other patient, strongly indicating that the clinicopathologic alterations in the 2 cases are not random but, on the contrary, represent a very rare disorder of gastric carcinoids associated with an intrinsic acid secretion abnormality of the parietal cells.

Achlorhydria↗
PubMed2006

Ultrasound-guided chest biopsies.

Pulmonary nodules that are surrounded by aerated lung cannot be visualized with sonography. Therefore, percutaneous biopsy must be guided with computed tomography or fluoroscopy. Although this restriction only applies to central lung nodules, it has permeated referral patterns for other thoracic lesions and has retarded the growth of ultrasound-guided interventions. Nevertheless, sonography is an extremely flexible modality that can expeditiously guide many biopsy procedures in the thorax. Peripheral pulmonary nodules can be successfully biopsied with success rates exceeding 90% and complications rates of less than 5%. Orienting the probe parallel to the intercostal space facilitates biopsies of peripheral pulmonary nodules. Anterior mediastinal masses that extend to the parasternal region are often easily approachable provided the internal mammary vessels, costal cartilage, and deep great vessels are identified and avoided. Superior mediastinal masses can be sampled from a suprasternal or supraclavicular approach. Phased array probes or tightly curved arrays may provide improved access for biopsies in this location. Posterior mediastinal masses are more difficult to biopsy with ultrasound guidance because of the overlying paraspinal muscles. However, when posterior mediastinal masses extend into the posterior medial pleural region, they can be biopsied with ultrasound guidance. Because many lung cancers metastasize to the supraclavicular nodes, it is important to evaluate the supraclavicular region when determining the best approach to obtain a tissue diagnosis. When abnormal supraclavicular nodes are present, they often are the easiest and safest lesions to biopsy.

Biopsy↗
PubMed1999

Follicular dendritic cell dysfunction and disorganization of lymphoid structures in MRL/lpr mice.

The follicular dendritic cell (FDC) in secondary lymphoid organs plays a key role in the function of the germinal center (GC) of the lymphoid follicle (LF) for regulation of the humoral immune response. MRL MpJ-lpr/lpr (MRL/lpr) mice, which have an abnormal humoral immune response, are a model for autoimmune disease. The present study was undertaken to investigate FDC dysfunction and LF disorganization in the spleen and lymph nodes of MRL/lpr mice. In 12-week-old MRL/lpr mice, antigen (Ag) trapping of FDC and half-life of trapped Ag on FDC was reduced. Although immunohistochemistry revealed well-developed FDC networks positive for complement receptors and having in vitro immune complex trapping in cryostat sections, Ag-trapping was not detected in 16-week-old MRL/lpr mice. Moreover a marked decrease in the number of GC and that of GC cell of residual GC in the MRL/lpr mice was observed by 12 weeks of age. Also reduced expression of intercellular adhesion molecule-1, FcgammaRII/III, and FDC-specific Ag (FDC-M1) on FDC was detected in 16-week-old MRL/lpr mice. The accumulation of double negative (CD4-CD8-) T cells, which is a characteristic feature of MRL/lpr mice, was observed around LF; however they did not infiltrate the LF and FDC network. In conclusion the loss of Ag-trapping on FDC and simultaneous disorganization of the lymphoid structure may be related closely to the immunologic abnormality observed in MRL/lpr mice.

Animals↗
PubMed1990

[Omenn syndrome].

Omenn's syndrome is a rare autosomal recessive disease characterized by the onset, from the first weeks of life, of an exsudative skin rash, alopecia, hepatosplenomegaly, diffuse lymph-nodes, diarrhea and increased susceptibility to infections with hypereosinophilia. The associated severe combined immunodeficiency (SCID) differs from the other SCID by the existence of lymphocytosis. The number of T lymphocytes is normal or elevated; they are sometimes immature; the distribution of their subsets (CD4/CD8) is variable. The B lymphocytes are quantitatively and functionally deficient. Pathological investigations reveal major lymphoid depletion with severe thymic hypoplasia associated with a proliferation of cells which have the whole immunohistochemical characteristics of Langerhans' cells but do not contain their specific granulations (Birbeck's granulations) on the ultrastructural examination. These cells are also found in the skin, lungs and liver. The outcome is usually fatal before 1 year of age. The pathogenesis of this disease is still discussed: it might be the result, in an immunodeficient child, either of a graft-versus-host reaction after materno-fetal transfusion of immunocompetent cells, or of an abnormal immunologic reaction after antigenic stimulation, or a deficit of molecules in lymphocyte ecotaxy.

Eosinophilia↗
PubMed1990

Conventional radiography of the hilum and mediastinum in bronchogenic carcinoma.

Knowledge of normal and abnormal hilar and mediastinal anatomy as depicted by chest radiography is important for the practicing radiologist. This article is an illustrated review of some of the alterations of normal anatomy that can indicate signs of metastatic spread of bronchogenic carcinoma. Thorough analysis of conventional radiographs with recognition of normal variants and detection of abnormal alterations will expedite or obviate certain therapeutic options in patients with bronchogenic carcinoma.

Carcinoma, Bronchogenic↗
PubMed1990

The relevance of lymphoid cell migration to immunodeficiency syndromes.

It has been known for some time that antigen stimulation can alter lymphocyte traffic patterns and that viruses are particularly potent in this respect; such alterations may be a consequence of host-derived factors. The retention of lymphocytes in lymph nodes can be sustained for several hours with locally administered interferon (IFN)alpha. The extravasation of lymphocytes from blood into non-lymphoid tissues can be induced in the skin with IFN gamma and particularly tumor necrosis factor (TNF)alpha. Recent evidence supports the concept that the migratory capacity of CD4+ cells differs from the capacity of CD8+ cells. Agents (cytokines?) which differentially affect the traffic of these two sub-sets have not yet been described but such a possibility has not been adequately tested. Several new molecules have been defined which alter the interactions between lymphocytes and blood vascular endothelial cells, and these may be important in the critical adhesive event in lymphocyte traffic. In both rat and sheep, it has been possible to cultivate post-capillary endothelial cells from lymphoid tissue, and this may be a helpful approach to studying the mechanisms and molecules involved in adhesion. New cell tracking dyes recently available (Zynaxis Cell Science) permit more significant, long-term studies on the life span of lymphocyte sub-sets and their migratory status. In our experiments, labeled lymphocytes can be followed in vivo for over 30 days. Traffic alterations may explain some of the abnormalities in immunodeficiency states.

Acquired Immunodeficiency Syndrome↗
PubMed2005

Fetal surgery in otolaryngology: a new era in the diagnosis and management of fetal airway obstruction because of advances in prenatal imaging.

OBJECTIVE: To evaluate the efficacy, safety, and outcome of prenatal imaging and fetal surgery in the diagnosis and management of fetal airway obstruction caused by cervical teratoma or lymphatic malformation. SETTING: Tertiary care medical center. Patients A retrospective study of all consecutive fetal patients with cervical teratoma or lymphatic malformation between January 2001 and December 2003. RESULTS: The indication was potential airway obstruction due to a fetal neck mass in 8 patients. Prenatal images were obtained by ultrasonography and magnetic resonance imaging, and were consistent with teratoma in 4 patients. The mean cervical mass was 8.3 x 7.3 x 6.7 cm, with airway obstruction suspected in all 4 patients. All 4 patients were successfully delivered by ex utero intrapartum treatment, during which 3 newborns required tracheotomy and 1 was successfully intubated. Prenatal images were consistent with lymphatic malformation in the remaining 4 patients. The mean cervical mass was 4.6 x 4.4 x 3.4 cm. There was no indication of airway obstruction based on prenatal images. All 4 patients had an uncomplicated vaginal delivery. CONCLUSIONS: Technological advances in prenatal ultrasonography and magnetic resonance imaging have improved the ability to diagnose congenital abnormalities in utero. This allows for proper assessment of the airway to prevent any unexpected problems at delivery. We believe that many airway emergencies can be avoided by prenatal imaging and initiation of airway management in the prenatal period.

Adult↗
PubMed2000

[The interphase fluorescence in situ hybridization (I-FISH) technique in patients with chronic lymphatic leukemia (CLL)].

BACKGROUND: Trisomy 12 was found to be the most frequent chromosomal aberration identified by conventional cytogenetic studies of bone marrow cells and peripheral lymphocytes of patients with CLL. Molecular-cytogenetic techniques which enable examination of dividing and/or non-diving interphase nuclei (I-FISH), proved existence of other chromosomal abnormalities, mainly deletions, which could have in CLL patients relation to the origin, course and prognosis of the disease. METHODS AND RESULTS: During the last two years bone marrow chromosomes of all patients with CLL were examined by G-banding and by I-FISH. The numerical changes of chromosome 12 were followed by centromeric DNA probe in dividing and non-dividing cells. The small deletions were ascertained by locus specific probes for 13q14 (Rb gene), 17p13 (p53 protein) and 11q23 (MLL gene). These genes are responsible for cell division and their function is probably in connection with neoplastic process. It is of interest whether numerical and structural chromosomal rearrangements are primary or secondary changes and what is their impact on etiology of CLL. 93 patients were examined by DNA prove CEP12 and trisomy 12 was found in 24 of them (25.8%), the range of the clone was 2.5-75.5% of the screened cells. Deletion del(13)(q14) was examined by probe D13S319 in 73 patients and proved in 24 of them (32.8%), pathological clone ranged 2.5-80.0% of the cells. Deletion del(17)(p13) was found in 14 patients out of 61 examined by probe LSI p53 (22.9%). The extent of the clone was 2.5-34.0% of examined cells. Deletion 11q23 was not ascertained in any of 11 patients by means of probe LSI 11q23 (MLL). All probes used for FISH were manufactured by VYSIS. CONCLUSIONS: FISH is very sensitive method, suitable for molecular-cytogenetic examination of leukemic patients. With I-FISH the deletion of 13q14 was ascertained as the most frequent chromosomal aberration in series of 73 patients with CLL. We continue to increase the number of patients screened by I-FISH with all eligible DNA probes and start the prospective study on patients with chromosomal pathology. We will correlate the immunophenotype, morphology, clinical course and prognosis with karyotypic findings.

Adult↗
PubMed1987

B-cell chronic lymphatic leukemia in Hodgkin's disease. A report of two patients with unusual chromosome features.

The development of B-cell chronic lymphatic leukemia in two patients who had previously been treated for Hodgkin's disease is described. In both cases aneuploidy and multiple chromosome aberrations of hemopoietic cells were evident. In one patient these changes included a clonal 14q + abnormality in association with other complex rearrangements, interpreted as translocation abnormalities involving t(6;14), 5(1;15), and t(17;19). Although the chromosome abnormalities in the other patient were nonclonal, a 14q abnormality also was detected, namely t(14q+;18q-). Other chromosome abnormalities (all nonclonal) in the two patients included translocations involving chromosome 5 and deletion of 7q in one patient and trisomy of chromosome 8 in the other. Although these abnormalities have been associated with the presence or development of non-Hodgkin's lymphoma, acute nonlymphoblastic anemia, or myelodysplastic disorders, the findings in these patients suggest that the detection of clones and potential clones with these abnormalities may be only one stage in the development of secondary malignancy.

Adult↗
PubMed1985

The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management.

Forty-nine patients with the Klippel-Trenaunay syndrome have been studied. Sixty-eight per cent have a superficial, embryological venous channel on the lateral aspect of the limb, 25 per cent have had one or more severe spontaneous haemorrhages from dilated varices and 22 per cent have suffered a venous thrombo-embolism. Twenty-nine per cent have had episodes of rectal bleeding or haematuria associated with pelvic angiomas. Foot volumetry (n = 31) showed that calf emptying was decreased in only five patients and only one of these had phlebographic evidence of deep venous obstruction, which conflicts with previous findings. The rate of calf refilling was increased in 81 per cent and phlebography demonstrated incompetent communicating veins in 45 per cent of patients. No patient had clinical evidence of an arteriovenous fistula, and arteriography (n = 22) and calf blood flow (n = 33) were normal, but lymphangiography (n = 14) showed lymphatic hypoplasia in 55 per cent of limbs. Eighty-eight operations have been performed on 38 patients but symptoms persist in 90 per cent. We suggest that surgery should be limited to the excision of localized symptomatic abnormalities and that the best form of control is provided by graduated compression stockings.

Adolescent↗
PubMed1998

[Predictive significance of reflex otalgia in local radical radiotherapy of oropharyngeal carcinomas].

BACKGROUND: Patients with carcinomas of the upper aero-digestive tract often suffer from ear pain as a tumor associated syndrome. This prospective study examines the predictive and prognostic value of this symptom. PATIENTS AND METHODS: Ninety-six consecutive patients who completed a locally radiotherapy of a carcinoma of the oropharynx were prospectively evaluated and followed. Forty-nine out of 96 patients stated the symptom, either spontaneously or after questioning. The 2 groups showed no difference regarding TNM-classification, histology and total dose. Overall survival, local control and disease specific survival were calculated according to Kaplan-Meier and compared by the log-rank test. RESULTS: A clinically complete remission was obtained in significantly fewer patients with reflex-otalgia as compared to patients without reflex-otalgia, 61.2% versus 89.3%, p < 0.002. Local control of patients with reflex-otalgia was significantly less with 49%, mean follow-up 564 days, in comparison to local control in patients without reflex-otalgia, mean follow-up 613 days, p = 0.01. Disease specific survival was significantly worse for patients with reflex-otalgia, p < 0.012. The probability of local control of T1/T2 tumors with reflex-otalgia was similar to T3/T4 tumors without reflex-otalgia. Local control for all tumor categories combined is 74% for patients without reflex-otalgia versus 49% for patients with reflex-otalgia. CONCLUSION: In our patients, reflex-otalgia is a new and statistically significant parameter for the probability of local control and disease specific survival.

Adult↗