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Results for “Lymphangiectasis”

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PubMed2005

MRI findings of renal lymphangiectasia.

We present the case of a 20-year-old male with renal lymphangiectasia who presented with hypertension and hematuria. We discuss the role of magnetic resonance imaging (MRI) with gadolinium-enhanced MR urography in confirming the diagnosis, extensions of this rare benign entity, and associated conditions.

Adult↗
PubMed2006

Validation of 16S rDNA sequencing in microdissected bowel biopsies from Crohn's disease patients to assess bacterial flora diversity.

The bowel flora is implicated in Crohn's disease (CD) pathogenesis but its precise role is still unclear. Several non-mutually exclusive hypotheses have been proposed: an unidentified persistent pathogen; excessive bacterial translocation; an immune system abnormality in response to normal bacteria; or a breakdown in the balance between protective and harmful bacteria. These hypotheses can be tested by identifying bacteria in specific microscopic bowel structures or lesions. The present paper describes a novel technique to assess bacterial flora diversity in bowel biopsies, by combining laser capture microdissection with broad-range 16S rDNA sequencing. Fifty-four samples comprising histologically normal and pathological mucosa, MALT, ulcers, submucosal lymphangiectasias, epithelioid granulomas, and lymph nodes were microdissected out of 30 bowel biopsies from five CD patients. Bacterial 16S rDNA was successfully amplified by PCR in all samples, and PCR products from 15 samples were selected for cloning and sequence analysis. A total of 729 bacterial DNA sequences were analysed, which could be attributed to six different phyla (Proteobacteria, Firmicutes, Bacteroidetes, Actinobacteria, Fusobacteria, and Planctomycetes). DNA from typical bowel bacteria (Enterobacteriaceae, Clostridiales, Bacteroidetes, Fusobacteria) was detected in all microdissected areas. It was thus convincingly demonstrated that 16S rDNA sequencing can be combined with microdissection to study the bowel flora. However, no specific persistent pathogen causal for CD was identified. The results suggest that Enterobacteriaceae may initiate or colonize ulcers in CD. Translocation of bacteria through established mucosal lesions or as a result of increased permeability may be involved in the evolution towards chronic inflammation and in the establishment of persistent lesions. Further study is needed to confirm these preliminary findings.

Adolescent↗
PubMedDate not supplied

Prenatal pleural effusion associated with congenital pulmonary lymphangiectasia.

A pleural effusion associated with congenital pulmonary lymphangiectasia was detected in a fetus in utero but was absent at the time of delivery. The pleural effusion was unilateral although the disease involved both lungs. In this case there was an association between polyhydramnios and congenital pulmonary lymphangiectasia.

Adult↗
PubMed2001

Congenital pulmonary lymphangiectasia: a case report of thoracic duct agenesis.

We present a 17-year-old Caucasian male with congenital pulmonary lymphangiectasia and an absent thoracic duct. This patient is unique as he did not present with the disorder until age 9.5 years. Since his initial presentation he has had recurrent chylothoraces and has been treated symptomatically. We discuss the possible implications of his disorder as well as some of the limited treatment that is available.

Adolescent↗
PubMed1992

Generalized lymphangiomatosis and chylothorax in the pediatric age group.

Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.

Bone Diseases↗
PubMed2004

Pulmonary lymphangiectasia: diagnosis and clinical course.

Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the normal lung lymphatics. The disease was previously associated with a poor prognosis. However, several recent case reports and case studies indicate that survival is possible in the severe neonatal form of PL, and the prognosis is good for infants who present after the neonatal period. In this report, we describe the etiology, clinical presentation, diagnosis, management, and outcome of PL. A revised classification of PL is proposed, based on the current state of knowledge of its etiology and presentation.

Age Factors↗
PubMed2005

Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal.

Pulmonary lymphangiectasia is a rare cause of respiratory distress in the newborn associated with a very poor outcome. We describe three premature newborns presenting at birth with nonimmune hydrops, bilateral chylothorax, and severe respiratory distress in the immediate newborn period secondary to pulmonary lymphangiectasia. We review the similarities of these cases and discuss their antenatal and neonatal course. One patient survived and is thriving at 9 months of age. With continuing advances in antenatal and neonatal care, an improved outcome may be possible in what was previously described as a uniformly fatal condition.

Fatal Outcome↗
PubMed1993

Acute jejunal ileus in intestinal lymphangiectasia.

A 26-year-old patient presented with epigastric pain of sudden onset and severe puffy swelling of both legs and forearms. An irregularly shaped nodular filling defect on selective jejunal films, severe hypoproteinemia, low IgG concentration, and lymphopenia were suggestive of primary intestinal lymphangiectasia with protein-losing enteropathy, and the patient was placed on a low-fat diet with medium-chain triglycerides. This initially improved his condition, but some weeks later he developed obstructive ileus of the small intestine. On laparotomy yellowish to whitish deposits were found to be present in some segments of the small intestine and a fist-sized mass 100 cm distal to the duodenojejunal flexure was resected without complications. Histologically, the submucosal lymphatics were dilated, and the jejunal wall showed extensive pseudocystic, intramural submucosal lymph edema with secondary bleeding and tight stenosis of the jejunal lumen. During the 14-month follow-up time after discharge the patient has been asymptomatic and working, on no treatment other than a low-fat diet with medium-chain triglycerides.

Acute Disease↗
PubMed1978

A simple screening method for detecting gastrointestinal protein loss in intestinal lymphangiectasia.

A ten month old female Turkish child with chylous ascites, diarrhea, steatorrhea, peripheral edema and hypoproteinemia was investigated for protein losing enteropathy which probably dated from the first weeks of life. Gastrointestinal protein loss appeared to be due to abnormalities of the intestinal lymphatics. In order to detect a localized lymphoenteric fistula, lymphangiography was tried but failed due to hypoplasia of peripheral lymphatics. However, three hours after intradermal injection of Patent Blue, the dye appeared in the stools of the patient, suggesting intestinal protein loss via a lympho-enteric fistula. This observation may provide the basis for a diagnostic test for gastro-intestinal protein loss in patients with intestinal lymphangiectasia.

Coloring Agents↗