[Hormonal effects of adrenal glands on hyperplasia of lymphatic fauces].
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Adrenal Cortex↗
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Fetuses with cystic hygroma. A retrospective study.
BACKGROUND: The aim of this study is to analyse several theories of pathogenesis of cystic hygroma, its correlation with chromosomal abnormalities and the indicators of poor or good prognosis. METHODS EXPERIMENTAL DESIGN: This is a retrospective study that evaluates all cases of cystic hygroma seen during the four-year period from January 1994 to December 1997. SETTING: This study was performed in the center of prenatal diagnosis of institutional hospital in Caserta. PATIENTS: All cases of fetuses with cystic hygroma were examined in 2100 pregnant female who visited the ambulatory. INTERVENTIONS: The modality of diagnosis of this pathology and the presence of abnormal maternal serum levels of alpha-fetoprotein, human chorionic gonadotropin and unconjugated estriol were evaluated. Moreover, the presence of karyotype abnormalities or other non chromosomal abnormalities were also evaluated. Prognostic indicators such as the presence of septae seen by sonography were examined. RESULTS: Nine fetuses with cystic hygroma were diagnosed sonographically. Septae were identified in six cases. Chromosomal abnormalities were found in five cases. Two cases presented Turner's syndrome and one case Downs' syndrome. There were two cases with associated anomalies. The amniotic fluid alpha-fetoprotein (AFP) levels were high in all cases. CONCLUSIONS: Cystic hygroma is a malformation of the lymphatic system that is diagnosed by ultrasound very well from the first quarter of pregnancy. It is frequently associated with chromosomal and non chromosomal abnormalities. The presence of septae in it and amniotic fluid AFP levels are prognostic indicators.
Chromosome Aberrations↗
Lateral cysts and fistulas of the neck.
The embryonic origin of lateral cysts and fistulas remains controversial. They are, however, located in the general anatomic pattern of the embryonic thymic duct, which starts in the lateral pharyngeal wall and passes under the sternomastoid muscle to enter the mediastinum. The cyst is a unilocular, semiattached, non-translucent mass which has a tendency to become infected in 30 per cent of cases. The preauricular sinus occurs in front of the ear and is due either to a branchial cleft or an inclusion of one of the six primitive buds which form the external ear.Cystic hygroma is due to sequestrated lymphatic channels. These are always multilocular and rather invasive and may extend into mediastinum and arms. Diagnosis and treatment are considered in this presentation.
Branchial Region↗
Dentofacial changes and oral health status in mentally challenged children.
The study was carried out on 69 mentally challenged individuals. They were subjected to detailed clinical evaluation for dentofacial abnormalities and oral health status. Of the 69 mentally handicapped individuals 27 had Downs syndrome and 42 had cerebral palsy. Characteristic facial abnormalities were seen in children with Downs syndrome. In cerebral palsy, fracture maxillary anteriors were more evident. All the Downs syndrome cases had abnormal TMJ movements but in cerebral palsy only 35.7% of individuals had abnormal TMJ movements. In both the groups, submandibular lymph adenopathy was reported. Present study revealed dental caries in 56.0% of the individuals. Fair clinical level of oral hygiene in 60% of the individuals was seen.
Adolescent↗
Lymphatic tumor emboli of perirenal fat in patient with nephrotic syndrome receiving renal biopsy, ultimately revealed gastric adenocarcinoma with membranous glomerulonephritis.
The association of malignancy with nephrotic syndrome and renal histopathologic abnormalities is well documented. Paraneoplastic proteinuria caused by membranous glomerulonephritis usually is made simultaneously with the diagnosis of a malignant tumor, or the two conditions are diagnosed within a year of each other. We reported a patient who presented with nephrotic syndrome initially. Incidentally, in kidney specimens, pathologic findings showed perirenal fatty tissue with malignancy tumor emboli in lymphatics. Thereafter, gastric adenocarcinoma was diagnosed by gastrointestinal panendoscopy with gastric biopsy under impression of malignancy associated with glomerulonephritis. Patient died of complications of malignancy-related disseminated intravascular coagulation without chemotherapy after confirming diagnosis was made three months later.
Adenocarcinoma↗
Cytogenetically unrelated clones in hematological neoplasms.
We have reviewed literature data on 6,306 cases of hematological neoplasia--acute and chronic lymphatic and myeloid leukemias (CML excepted), myelodysplastic and chronic lymphoproliferative and myeloproliferative disorders, and malignant lymphomas--with the goal of quantitatively ascertaining how often cytogenetically unrelated clones occur in these diseases. Unexpectedly wide variations were found: in ANLL, unrelated clones were present in 1.1% of the 2,506 known cases with chromosome abnormalities characterized with banding technique; in the various myelodysplastic (MDS) and chronic myeloproliferative (CMD) disorders (total number of cases 1,299) the frequency was 4.3% and in lymphatic malignancies 1.3% (total case number 2,501). In the latter group the proportions varied between 0.4% and 0.6% in ALL and malignant lymphoma (ML) to as much as 6.2% in CLD and 7.3% in CLL. Some karyotypic abnormalities were encountered more often than would be expected from their general frequency in the various diseases. This discrepancy was particularly evident in MDS and CMD, where 5q- was found in slightly less and +8 in somewhat more than half of the 56 cases. Furthermore, these two aberrations were found as the only changes in the two coexisting clones in one-fourth of the material. Although if viewed in isolation these data would undoubtedly be best explained by assuming a multicellular origin of the neoplasm, it is entirely possible that what are cytogenetically perceived as unrelated clones could be subclones with some invisible aberration in common. If so, this interpretation indicates that changes like +8 and 5q-, both of which are common rearrangements in bone marrow neoplasms, are actually secondary changes that develop during tumor progression.
Chromosome Aberrations↗
Review of head and neck lesions in infancy and childhood.
Common congenital and infectious lesions of the head and neck in infancy and childhood have been discussed. Those that are present at birth, asymptomatic, and frequently cystic are clearly benign lesions that require operative management for the potential complications of enlargement and infection. Solid lesions, particularly those of the lymph nodes, must be differentiated from neoplasms, particularly Hodgkin's disease and non-Hodgkin's lymphoma. Suppurative lymphadenitis is obvious from its local inflammatory signs, but the subacute and chronic adenopathies may be difficult to distinguish from neoplasia. An attitude that unless the benign nature of a lesion is clearly demonstrated, early excisional biopsy for histopathologic evaluation must be the rule will avoid delays in diagnosing those neoplastic lesions that can be treated successfully if managed early and aggressively.
Branchioma↗
The retrotracheal space: normal anatomic and pathologic appearances.
A variety of diseases can arise from the normal contents of the retrotracheal space or from adjacent structures. Mediastinal diseases in the retrotracheal space typically manifest radiographically as a contour abnormality or an area of increased opacity, although computed tomography (CT) or magnetic resonance (MR) imaging is usually required for diagnosis. The most common aortic arch anomaly, a right subclavian artery that originates from an otherwise normal left-sided aortic arch, appears at posteroanterior chest radiography as an obliquely oriented soft-tissue area of increased opacity that extends superiorly to the right from the superior margin of the aortic arch. CT and MR imaging can reveal associated vascular or mediastinal abnormalities. Aortic aneurysms and pseudoaneurysms can manifest radiographically as fusiform or saccular masslike lesions that protrude into the retrotracheal space. Thoracic MR imaging and spiral CT angiography are the diagnostic procedures of choice for evaluating diverse pathologic conditions of the thoracic aorta. Esophageal diseases can manifest as an abnormality in the retrotracheal space, which may be the initial clue to the diagnosis. At CT, lymphatic malformations in the mediastinum manifest as lobular, multicystic tumors that surround and infiltrate adjacent mediastinal structures. Familiarity with the normal radiologic appearance of the retrotracheal space and with the clinical manifestations of diseases that affect the retrotracheal space and adjacent structures can facilitate detection, diagnosis, and management.
Aortic Arch Syndromes↗
[Contribution of lymphoscintigraphy in the exploration of lymphedema in children].
OBJECTIVES: The objectives of this work were a) to assess the feasibility of lymphoscintigraphy in very young children, and b) to assess its usefulness in understanding the underlying pathophysiology. PATIENTS AND METHODS: Forty-one children (age range 3 months-16 years) with primary lymphedema of the lower and/or upper limbs underwent lymphosinctigraphy using 99mTC-labeled colloid (Nanocis or Nanocol) injected subcutaneous at the foot and/or hand level. Recording time, which depended on the child's age, included at least static images during the thirty minutes following injection and four hours later. RESULTS: Abnormal activity of the lymph collectors, nodes, and interstitial space were analyzed according to age (> or < 2 years). Abnormal findings were classed as simple hypoplasia (n=18) or other type of lymph disease: diffuse lymphatic dysplasia (n=6), mixed vascular dysmorphism (n=5), amniotic bride disease (n=1), and hypoplasia revealed by occurrence of a minor factor (n=11). These observations demonstrate the role of lymphoscintigraphy in the diagnosis and prognostic evaluation of lymphedema in children. CONCLUSION: Lymphoscintigraphy is a useful technique for very young children with lymphedema providing further information contributing to overall exploration.
Adolescent↗
Navigation-assisted sclerotherapy of orbital venolymphatic malformation: a new guidance technique for percutaneous treatment of low-flow vascular malformations.
Percutaneous sclerotherapy of orbital low-flow vascular malformations requires precise procedural guidance. For the treatment of a patient with an orbital venolymphatic malformation, we sought to optimize guidance by combining navigation assistance for needle placement with intralesional contrast medium injection for assessment of venous drainage. By using a surgical navigation system (Vector Vision, BrainLAB, Munich, Germany), multiplanar target lesion visualization was performed after fusion of CT and MR imaging data, which allowed precise puncture planning.
Adult↗
[The problem of tissue developmental defects in childhood].
Chronic diseases of the thymus, lungs and kidneys observed in children are viewed basing on analysis of the malformations detected in the children at tissue and cellular structural level, using autopsy and biopsy evidence. Multiple modality investigation was focused on dysplasia, hypoplasia and dyschronism. Dysplasia was characterized by congenital and acquired forms similar in the development and presentation while dyschronism by enhancement or inhibition of structural development seen in immaturity or premature postnatal involution. Immaturity shows the possibility of further development and structural and functional patency. The occurrence of tissue immaturity is not rare, being found in one or many organs of the child. Tissue malformations are chronic inflammation risk factors.
Cell Differentiation↗
[Value of hilus tomography in tuberculin positive, non BCG vaccinated children].
X-ray films of the thorax in two levels were prepared of 15 children of 3-14 years of age who had not been BCG-inoculated, after the tuberculin test had shown a positive reaction. Besides bacteriology and bronchoscopy, tomography of the hilus and of the anterior mediastinum was performed to exclude hilar lymph node enlargements. In this manner, it became possible to disclose an enlargement of lymphatic nodes in four children in whom plain radiography of the thorax had not yielded any abnormal findings.
Adolescent↗
[Association of 2 congenital diseases--cervical rib and aberrant pancreas simulating cancer of the stomach with Virchow's metastasis].
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Abnormalities, Multiple↗
Leukoencephalopathy following high-dose iv methotrexate chemotherapy with leucovorin rescue.
Seven patients with bone or soft tissue sarcomas but without metastatic CNS disease developed a chronic leukoencephalopathy after high-dose (8000-15,000 mg/m2) iv methotrexate (MTX) chemotherapy with leucovorin (LV) rescue. Approximately 12 MTX-LV treatments were administered over a 3-7 month period. None of the patients had cranial irradiation. The syndrome usually began several months after the initiation of chemotherapy with subtle personality changes followed by a progressive dementia, focal seizures, pseudobulbar palsy, spastic quadriparesis, and stupor. Computerized tomographic scans revealed diffuse white matter hypodensity in five patients and atropic changes in five patients. Serum MTX concentrations were elevated in four of six patients prior to several MTX-LV treatments, suggesting that MTX persisted in tissues for a long time. Abnormally high levels of MTX were detected in the cerebrospinal fluid of all four patients several days after an MTX-LV treatment, at a time when their encephalopathy was most severe. Pathologic brain material was obtained from three patients and revealed a spectrum of abnormalities. The syndrome observed in our patients clinically resembles the one described in children with acute lymphatic leukemia who received cranial irradiation and large cumulative amounts of low-dose (12-20 mg/m2) systemic MTX without LV.
Adolescent↗
[On the chloranilic acid reaction (RAC) proposed by Closs (author's transl)].
On the serum of 3790 patients (2131 women and 1659 male), aged from 3 days to 84 years, the RAC has been performed at the same time with the Mc Lagan's Kunkel's and Takata's reactions. The test of sulfobromophthalein's excretion has been performed in 246 of these patients. Between RAC and T.D., disagreement has been observed in 111 cases (- 2,9%). RAC positive and T.D. negative occurred in 68 cases; T.D. positive and RAC negative in the other 43. Repetition of the RAC on 100 sera (after complement inactivation at 56 degrees C for 30 min; after permanence at 4 degrees C for 7 days; after congealment at -18 degrees C) has always given the same results obtained on fresh sera. The complement, therefore, has no way influence on the RAC. The study of disagreeing cases has shown the RAC become positive for: 1) an apperance in serum of abnormal lipoproteins (chloestasis, cholangitis, some dislipemias), 2) a rise in gamma-M (biliary cirrhosis, macroglobulinaemia, lymphatic chronic leukema); 3) 1 and 2. It is always necessary to perform T.D. and RAC on the same serum, because these two test express different modification of the serum-proteins picture. Their agreement, or their disagreement, is very important in every single case, particularly in the diagnostic of jaundice and of bilary ducts pathology.
Adult↗
Novel sites of cytosolic glutathione peroxidase expression in colon.
Glutathione peroxidases (Gpx) are important moderators of oxidative stress that is implicated in the pathogenesis of numerous diseases including colon cancer. Previous studies report limited examinations of cytosolic glutathione peroxidase location of expression in colon tissue. This study reports evidence of both common sites of Gpx1 and Gpx2 expression in rat colon and sites that are exclusive to each isoform. Semi-quantitative PCR performed previously demonstrated RNA expression of Gpx1 and Gpx2 in proximal, transverse and distal colon. Mapping the distribution throughout the entire colon has revealed specific, novel sites of glutathione peroxidase expression in colon lymphatic tissue. In situ hybridisation and immunohistochemistry confirmed micro-anatomical location of Gpx1 within lymphatic tissue and the lamina propria, sub-mucosa, muscularis and serosa, but not the lumenal epithelium. In situ hybridisation and immunohistochemistry were consistent with reports of microanatomical location of Gpx2 in the lumenal epithelium. Novel sites of Gpx2 expression were also observed in lymphatic tissue. Immunolocalisation in the vicinity of aberrant crypt foci was also examined to further investigate the link between glutathione peroxidases and colon cancer. This did not reveal significant abnormalities, nor did measurement of cytosolic glutathione peroxidase activity or gene expression in colon tissue from rats treated with the colontropic chemical, 1,2-dimethylhydrazine. These results support the potential for Gpx1 and Gpx2 redundancy in lymphatic tissue, but not in epithelial cells of the colon crypt or in the lamina propria, sub-mucosa, muscularis or serosa.
Animals↗
Assessment of leg oedema by dynamic lymphoscintigraphy with intradermal injection of technetium-99m human serum albumin and load produced by standing.
This study was a preliminary evaluation of the utility of dynamic lymphoscintigraphy with technetium-99m human serum albumin (HSA) and a load produced by standing in the assessment of lymphatic dysfunction in patients with leg oedema. The 71 subjects investigated included 53 patients with lymphoedema, six with venous occlusion alone and five with lymphovenous occlusion, as well as seven normal subjects. After intradermal injection of 99mTc-HSA into an interdigital space in each foot, dynamic scintigrams were recorded with the patient supine for 15 min. The subjects then stood in place and images were recorded for an additional 15 min. Relative changes in lymphatic tracer transport before and after standing were analysed on time-activity curves (TACs). This test was compared with a conventional test in a supine position in six patients with lymphoedema, and was repeated in five other patients with lymphoedema. It was found that in the normal limbs, a standing load activated tracer transport to the draining lymphatic vessels, resulting in a rapid stepwise increase in tracer activity, large spiking waves and a decreasing phase following a peak in tracer activity on TACs. In 59 lymphoedematous limbs, including some with a mild form of oedema without morphological abnormalities on scintigrams, this load failed to induce a sufficient activation of tracer transport, and the frequencies of each of the three normally appearing changes described above significantly decreased compared with those in the 14 normal limbs (P < 0.0001, P < 0.01 and P < 0.0001, respectively). In addition, there were significant reductions in the relative increases in maximum activity and clearance times after standing (both P < 0.0001). These abnormalities significantly correlated with the grade of severity of oedema. Six limbs with lymphovenous occlusion showed significant reductions in tracer transport compared to six limbs with venous occlusion. Lymphatic dysfunction was accentuated more by this test than by the conventional test, and repeated tests showed consistent results in the same individuals. It is concluded that under a standardized load, this quick test seems of value in providing a sensitive and objective assessment of lymphatic dysfunction in the lower limbs, and is also advantageous for image interpretation since accelerated tracer transport clearly visualizes compromised lymphatics. This test may also be helpful in distinguishing purely venous oedema from mixed lymphovenous disease.
Adult↗
Diffuse histiocytosis of the spleen and idiopathic thrombocytopenic purpura (ITP): histochemical and ultrastructural studies.
A case of ITP where the spleen was infiltrated diffusely and extensively by foamy macrophages is described. Among these macrophages were also clusters of cells with the morphologic and tinctorial characteristics of ceroid or "sea-blue" histiocytes. The ultrastructural characteristics of the foamy and ceroid histiocytes are described. Transition of ceroid cells into foamy macrophages is demonstrated, and intermediate forms were observed. It is postulated that the ceroid or sea-blue histiocytes are macrophages that contain platelets at an early stage of digestion, and when the phagocytosis of these platelets is completed, the macrophages become foamy. Therefore, it is suggested that the foamy cells and ceroid or sea-blue histiocytes seen in the spleen in cases of ITP are macrophages that have ingested and metabolized the abnormal platelets.
Aged↗