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At least 505 records · Page 28Linked to original sources
PubMed1996

Thrombomodulin as a marker of vascular and lymphatic tumours.

The aim of this study was to evaluate the utility of a new commercially available antibody to thrombomodulin as an endothelial marker in formalin-fixed paraffin-embedded tissue. The expression of thrombomodulin in a variety of 50 vascular and lymphatic neoplasms and malformations was compared to the expression of von Willebrand factor, QBend 10 (CD34) and JC70 (CD31). We showed that thrombomodulin was the best marker of lymphatic endothelium and also stained a higher percentage of malignant vasoformative tumours when compared to the other markers. We recommend the assessment of thrombomodulin expression in the differential diagnosis of malignant vasoformative neoplasms and in the detection of lymphatic endothelium for evidence of tumour permeation.

Biomarkers, Tumor↗
PubMed2000

Computed tomography of diffuse interstitial lung disease in children.

Unlike diseases of the airways, interstitial lung diseases in childhood are exceedingly rare and are usually associated with significant morbidity and mortality. Interstitial lung disease in the paediatric age group is a particular diagnostic challenge because the clinical presentation and radiographic features are so non-specific. High resolution computed tomography (HRCT) has proved its worth in adults with interstitial lung disease and has a role, albeit more limited, in the non-invasive evaluation of paediatric interstitial lung disease.

Adolescent↗
PubMed2000

Pulmonary lymphangiectasia revisited.

PURPOSE: Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. METHODS: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. RESULTS: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. CONCLUSIONS: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected.

Abortion, Spontaneous↗
PubMed1978

Functional lymphangiectasia of the duodenal mucosa.

Tiny millet-like prominent villi with dilated lymphatics may be part of a food-induced functional lymphangiectasia. Peroral olive oil load causes diffuse transient lymphangiectasia, the gross aspect, histological findings and electron-microscopy of which is described in detail.

Dietary Fats↗
PubMed2003

[4199 biopsies from the endoscopic normal lower duodenum].

Lower duodenal biopsies (LDB) are not taken at every oesophago-gastro-duodenoscopy (EGD). In the present study, biopsies from the endoscopic normal lower duodenum were checked as a measure of quality assurance. From 1996 to 2000, 9,955 EGD were performed and 4,199 LDB were taken (42.2 %). Of these, 667 showed pathological histology (15.9 %). A non-specific inflammation was seen in 537 cases and lymphangiectasia in 30 cases. Signs of indigenous sprue were described histologically in 6 LDB. In 4 of the 6 first diagnoses, the LDB was taken owing to clinical suspicion of malabsorption syndrome. Giardia lamblia could be detected in 22 patients. Only 6 of the 22 patients had diarrhoea. A total of 18 clinically relevant first diagnoses were made by LDB in asymptomatic patients with normal endoscopic findings in the duodenum. In order to make a relevant first diagnosis, 233 LDB had to be taken. LDB can be dispensed within EGD when there is neither diarrhoea nor loss of weight, and no anemia, iron deficiency, vitamin deficiency, macrocytosis, hypoproteinaemia, meteorism, joint symptoms or fever.

Adult↗
PubMed2006

[Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants].

BACKGROUND: Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns. PATIENT, METHODS AND RESULTS: This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy. CONCLUSION: In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.

Biopsy↗
PubMed1986

Endoscopic diagnosis of primary intestinal lymphangiectasia using a high-fat meal.

The case of a 17-year-old woman is described, in whom a suspected diagnosis of primary intestinal lymphangiectasia was confirmed endoscopically and histologically only after a high-fat meal had been given the night before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenum and jejunum, which on histological examination proved to be dilated lymph vessels. Treatment with low-fat diet and MCT-supplementation was successful.

Adolescent↗
PubMed1981

Synopsis of endoscopic and other morphological findings in intestinal lymphangiectasia.

Intestinal lymphangiectasia may present as a primary congenital disorder as well as a secondary form due to numerous underlying diseases. Endoscopically, whitish swollen tips of the villi are fairly characteristic; histologically, dilatation of the lymphatics can be demonstrated. By means of electron microscopy dilated intercellular spaces with aggregation of protein precipitations as well as ectatic lymph vessels with abnormal structure are found. Possible relations to the pathomechanism are discussed.

Duodenum↗
PubMed1981

Intestinal telangiectasis in Turner's syndrome.

A 37-year old female with Turner's syndrome, iron deficiency anemia and intermittent gastrointestinal hemorrhage is described. Gastrointestinal endoscopy with biopsies, revealed telangiectasia in the duodenal bulb, the cecum and the ascending colon. Endoscopy should be performed in patients with Turner's syndrome and anemia even if there are not signs of active gastrointestinal bleeding.

Adult↗
PubMed1997

[Idiopathic conjunctival lymphangiectasia].

PATIENT: A 41-year-old male was evaluated for multiple yellowish cysts on the temporal bulbar conjunctiva of his right eye. These cysts have spontaneously developed over a period of one week, have been punctuated, and recurred one week later in an even greater number. The patient's ophthalmologic and medical history were normal. Complete excision of the largest cysts and marsupialization of the smaller cysts were performed. Histologic features of the excised cysts were consistent with lymphangiectasia and displayed dilated lymphatic channels. CONCLUSION: Conjunctival lymphangiectasia is a benign condition of unknown etiology. It has been observed with many underlying diseases as well as spontaneously and the dilated lymphatic channels can secondarily become hemorrhagic (Hemorrhagic lymphangiectasia of the conjunctiva). Simple excision and/or marsupialization are therapeutic options.

Adult↗