SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Intestinal lymphangiectasia is one of the protein-losing enteropathies. Herein, a case with intestinal lymphangiectasia responded well to diet and octreotide is presented.
The yellow nail syndrome, a combination of yellow discoloured nails, lymphedema and pleural effusions, is a rare clinical condition. We report a case of the yellow nail syndrome associated with intestinal lymphangiectasia revealed by chylous ascites and protein-losing gastroenteropathy. This association reported in only three cases in the literature leads us to discuss the relations between yellow nail syndrome, primitive intestinal lymphangiectasia and primary lymphatic disorders.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
UNLABELLED: Hennekam syndrome is a disorder comprising intestinal lymphangiectasia, facial anomalies and moderate mental retardation. Eight cases have been previously reported. CASE REPORT: A 17-month-old girl was admitted to hospital for peripheral edema. On physical examination, she presented with a normal mental development. Facial anomalies were noted including a flat face, depressed and broad nasal bridge, puffy eye lids, mild down-slanting palpebral fissures, hypertelorism, epicanthal folds, bulbous nasal tip, small mouth, and low set ears. A simian line and haemangiomas on the arms, trunk and left limb were also noted. There was no organomegaly. Laboratory investigations showed iron deficiency anemia, hypoproteinemia, hypogammaglobulinemia and an elevated level of alpha-1 antitrypsin excreted in the feces. Endoscopic investigation and the small bowel biopsy showed findings consistent with lymphangiectasia. The patient did well on 24 hour enteral nutrition including medium-chain triglyceride rich diet and infusion of human albumin. CONCLUSION: We have aimed to remind that Hennekam syndrome should be included in differential diagnosis when intestinal lymphangiectasia are associated with facial anomalies.
A unique case of enamel hypoplasia is presented. This appears to be the first case to be published in connection with the rare protein-losing enteropathy, intestinal lymphangiectasia. It seems likely that if the treatment of systemic disease is delayed, disruption of normal tooth formation is inevitable.
Steatorrhea is seen in 18-24% of patients with autoimmune polyglandular disease (APD) type 1. The etiology and pathophysiology of the steatorrhea in this disease are unknown. We present a patient with APD type 1 and steatorrhea in whom biopsies revealed intestinal lymphangiectasia. This association has not been previously described. Intestinal lymphangiectasia may explain the steatorrhea in some patients with ADP type 1. As blind intestinal biopsies may miss areas of intestinal lymphangiectasia, endoscopically directed intestinal biopsies should be included in the evaluation of steatorrhea in APD type 1.
Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport, and concomitant problems of hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, and peripheral edema. Patients develop diarrhea, steatorrhea, and hypocalcemia secondary to fat-soluble vitamin malabsorption. Treatment is a restrictive diet of low fat, medium chain triglycerides. Oral manifestations are gingivitis due to poor PMN function and enamel defects due to poor calcium absorption. A case of a 14-year-old boy with both gingival and enamel problems secondary to intestinal lymphangiectasia is reported.
A 37-year-old woman presented with an 18 month history of recurrent severe abdominal pain, recurrent diarrhea since her second year of life, and mild peripheral edema. CT and more clearly MRI revealed signs of small bowel and mesenterial edema with partial tubular appearance, which correlated well with the histopathologic findings shown in duodenal biopsy. This is the first report of MR findings in congenital intestinal lymphangiectasia with correlation with other imaging modalities.
A 13-year-old girl presented with malabsorption which was ascribed to intestinal lymphangiectasia. Three years later a generalised seizure resulted from hypocalcaemia that was shown to be due to hypoparathyroidism during investigation of which toxic copper accumulation was recognised. The chance occurrence of three rare conditions is extremely remote making intestinal lymphangiectasia likely as the primary pathology. It is suggested that chronic intestinal loss of the copper-carrying caeruloplasmin resulted in toxic parathyroid deposition of copper leading to hypoparathyroidism with consequent hypocalcaemic seizure.
From 1980-1986 intestinal mucosal lymphangiectasia was diagnosed histologically in eight patients (6 weeks to 16 years; four males/four females; seven white). The presenting features were diarrhea (six/eight), vomiting (four/eight), and growth deficit (seven/eight). Additional conditions in these patients included asthma, urinary tract infection, esophageal atresia, hydrops fetalis, inflammatory bowel disease, malabsorption syndrome, and thymic hypoplasia. Hypoalbuminemia and edema (four/eight) were more prominent in those patients under 5 years of age. Two had systemic lymphangiectasia and lymphopenia. The patients responded variably to hyperalimentation and dietary supplements, depending on the extent of their lymphangiectasia and the age at onset of symptoms. Dilated lymphatics were seen in the small intestinal mucosa under the surface epithelium. Lesions were often focal, requiring several biopsies or serial sections for detection. Other common findings were mild to moderate lymphoplasmacytic inflammation and mild to moderate villous injury with blunting and edema. Mild inflammation without lymphangiectasia was also present in esophageal, gastric, or colonic biopsies. Diagnosis should be made on the basis of endoscopic findings or in small-intestinal inflammatory conditions even in the absence of a classic clinical picture. Histologic confirmation may require more than one serially sectioned biopsy. This study confirms the diversity of disorders that may be associated with intestinal lymphangiectasia and shows that the disease in infants is more severe and generalized.
A 7-year-old white girl presented with recurrent abdominal pain because of gastroesophageal reflux. At endoscopy, we found intestinal lymphangiectasia in the duodenal biopsy. There were no physical signs, nor any laboratory evidence of enteric protein loss.
Impaired neutrophil chemotaxis and phagocytosis were shown in three patients with intestinal lymphangiectasia. Abnormalities in cell associated and serum derived activity occurred, and possible mechanisms are suggested.
The results from gross and microscopic examination of the stomachs of Lundehunds and examination of stomachs of control dogs from other breeds were compared. Over a 13-year period, 12 of 14 autopsied Lundehunds have been diagnosed as having intestinal lymphangiectasia. In the present study, histological examination revealed gastritis as manifested by an increase in the number of mononuclear cells infiltrating the lamina propria in all the Lundehunds. The inflammation was chronic and restricted to the fundic and body regions, except in one Lundehund where antral gastritis was also present. Computer-assisted morphometric analysis was used to quantify the increased number of mononuclear cells. Atrophy of mucosal fundic glands was prominent in most Lundehunds and mucous metaplasia was often present. Conventional morphometry revealed a significant decrease in the height of the gastric mucosa. A relative expansion in area of the basal part of the lamina propria in Lundehunds with chronic atrophic gastritis corresponded to the observed increase in mononuclear cells and stromal elements. Primary gastric carcinoma with neoplastic cells infiltrating layers of the stomach wall was found in four Lundehunds. The high incidence of gastric carcinoma and the consistent presence of gastritis in Lundehunds suffering from intestinal lymphangiectasia suggest that these changes represent features of a single pathogenetic process.
In this report we describe a rare association of pachydermoperiostosis with protein-losing enteropathy (PLE) in a family of three brothers. The first brother had the complete form of pachydermoperiostosis along with PLE. The second brother had the 'forme fruste' of pachydermoperiostosis, with minimal skin changes, bony abnormalities and PLE, which was due to intestinal lymphangiectasia. The third brother had an incomplete form of pachydermoperiostosis without evidence of PLE. To our knowledge, the association of pachydermoperiostosis with PLE due to intestinal lymphangiectasia has not been reported previously.
Clinical symptoms and diagnostic procedures in a 12 1/2 years old girl with protein-losing enteropathy due to intestinal lymphangiectasia are reported. The child had suffered from intestinal protein loss, hypoproteinemia, hypocalcemia and lymphocytopenia. Dietary fat reduction and medium chain triglyceride formula diet made all the symptoms disappear.
We report a case of intestinal lymphangiectasia secondary to radiotherapy and chemotherapy. The patient also had small bowel bacterial overgrowth and pancreatic insufficiency. Lymphatic ectasia as a histological feature has been described previously in association with postradiotherapy malabsorption, but radiation-induced lymphangiectasia producing clinical manifestations has hitherto not been reported. Replacement of dietary long-chain fats with medium-chain triglycerides, pancreatic enzyme supplements, and a short course of oxytetracycline, resulted in dramatic clinical improvement. The possibility of intestinal lymphangiectasia should be borne in mind in patients with postradiotherapy malabsorption. A low serum albumin and lymphocyte count should draw attention to this possibility.
We describe a patient with intestinal lymphangiectasia who developed hyposplenism and speculate that it resulted from chronic loss of lymphocytes into the gut.