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| Primary intestinal lymphangiectasia (Waldmann's disease).Primary intestinal lymphangiectasia (Waldmann's disease).
Feb 2008 Orphanet J Rare Dis.
Vignes S, Bellanger J.
ABSTRACT:
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha-1 antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
Orphanet | | |
| Lymphamgiogenesis, Lymphangiectasia and Crohn's DiseaseLymphangiogenesis in Crohn’s disease: an immunohistochemical study using monoclonal antibody D2-40
F. Pedica1 , C. Ligorio1, P. Tonelli2, S. Bartolini3 and P. Baccarini1
(1)
Section of Pathology, Bellaria Hospital, University of Bologna, Via Altura 3, 40139 Bologna, Italy
(2)
General Surgery 1, Department of Medicine and General Surgery, Azienda Ospedaliero-Universitaria Careggi, Firenze, Italy
(3)
Unity of Oncology, Bellaria Hospital, Bologna, Italy
Received: 17 September 2007 Revised: 24 October 2007 Accepted: 26 October 2007 Published online: 27 November 2007
Abstract
Crohn’s disease (CD) is a chronic inflammatory bowel disorder of unknown etiology. An involvement of the intestinal lymphatic system has been suggested. Recently, monoclonal antibodies have become available to distinguish lymphatic vessels from blood vessels. The aim of the study was to examine the distribution of lymphatic vessels in ileal and colic walls of patients affected by CD and compare it with healthy controls and other inflammatory bowel diseases. Twenty-eight cases of CD, 13 cases of other inflammatory bowel diseases, and 10 normal ileal and colic walls were studied. Immunohistochemical staining was performed using the monoclonal antibody D2-40. Quantification of lymphatic vessels was performed by identifying four fields with high density of lymphatics and then counting the number of lymphatic vessels at high resolution. Lymphatic diameter was also evaluated by using an ocular micrometer. Lymphatic vessels showed the highest density in CD specimens. The median number of lymphatics was significantly higher both in ileal and colic samples of CD than the other inflammatory diseases as well as normal controls. Moreover, in patients with CD, diffuse lymphangiectasia was also observed. The present data suggest that lymphangiogenesis and lymphangiectasia probably play a role in the pathogenesis of CD.
Springer Link | | |
| Renal insufficiency in a boy with cystic renal lymphangiectasiaChronic renal insufficiency in a boy with cystic renal lymphangiectasia: morphological findings and long-term follow-up.
Clin Nephrol. 2007 Dec
Ueda S, Yanagida H, Sugimoto K, Fujita S, Yagi K, Okada M, Takemura T.
Dpartment of Pediatrics, Kinki University School of Medicine, Osaka-Sayama, Japan.
Cystic renal lymphangiectasia (CRL) is a rare malformation of lymphatics that can present in childhood and adulthood. Symptoms and radiologic features are relatively well defined, but clinical evolution and prognosis remain unclear. We treated a boy with CRL who developed chronic renal insufficiency. The first manifestation was abdominal swelling associated with an umbilical hernia noted incidentally at 1.6 years. Computed tomography with intravenous contrast administration demonstrated perirenal cysts with fluid collection, suggesting CRL.
Intractable ascites resisted pharmacologic treatments such as diuretics. After approximately 7 years, the ascites resolved spontaneously, but the perirenal cysts persisted. At 11 years, proteinuria was noted.
A renal biopsy specimen showed interstitial abnormalities consistent with CRL, glomeruli showed a focal segmental mesangial increase. Proteinuria persisted despite administration of an angiotensin-converting enzyme inhibitor, increasing as obesity and hypertension worsened. Renal function gradually declined in the ensuing years. Polycythemia coexisted with a normal serum erythropoietin concentration. A follow-up renal biopsy specimen disclosed glomerular enlargement together with focal segmental mesangial expansion, suggesting obesity-related glomerulopathy.
Our observation suggest that under some specific circumstances like our patient CRL may exacerbate. Management of complicating obesity and hypertension are likely to be important for maintaining normal renal function, especially in the diffuse bilateral type of CRL present in our patient.
PubMed | | |
| Immunohistochemical studies in a hydroptic fetus with pulmonary lymphangiectasia and trisomy 21.Immunohistochemical studies in a hydroptic fetus with pulmonary lymphangiectasia and trisomy 21. Lymphology. 2007
Rutigliani M, Boccardo F, Campisi C, Bonioli E, Fulcheri E, Bellini C.
Department of Pathology (DICMI), University of Genoa, Gaslini Institute, Genova, Italy.
This case report presents a hydroptic trisomy 21 fetus affected by lymphatic dysplasia with no other malformations. Our studies using CD31, CD34, smooth muscle actin, desmin, and D2-40 antibodies immunohistochemistry confirm the diagnosis of severe pulmonary lymphangiectasia associated with lymphangiectasia ih the mediastinum and small bowel.
PMID: 18062612 [PubMed - in process] | | |
| Sucessful dietetic-therapy in primary intestinal lymphangiectasia and recurrent chylosa ascites: a cSucessful dietetic-therapy in primary intestinal lymphangiectasia and recurrent chylosa ascites: a case report Nutr Hosp. 2007Martín CC, García AF, Restrepo JM, Pérez AS.Unidad de Nutrición Clínica y Dietética, Servicio de Endocrinología y Nutrición, Hospital Universitario Virgen Macarena, Sevilla, España. smaradigna@hotmail.com OBJECTIVES: Primary intestinal lymphangiectasia is a lymphatic system's disorder, where lymphatic drainage is blockaged. Clinically it produces malabsorption, protein-losing enteropathy, hypogammaglobulin in blood, and several degrees of malnutrition. Its treatment is not easy and includes dietetic-therapy and drugs. MATERIAL AND METHOD: A 35-year-old-woman case report is exposed. She has recurrent chylosa ascites, requiring several admissions and evacuatory paracentesis. After food-fat was replaced by medium-chain triacyl-glicerol-enriched diet, a clinical, analytical and anthropometric improvement was demonstrated. CONCLUSSIONS: The major way of treatment in intestinal lymphangiectasia in this case is the employement of specific-diet and adaptaded-basic-food. It's difficult and high collaboration of the patient is required, being necessary medical revisions during the whole life, due to the not well known evolution of this long-standing disease. PMID: 18051999 [PubMed - in process] | | |
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