Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Saturday, April 28, 2007

Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia.

Remission of protein-losing enteropathy after nodal lymphoma treatment in a patient with primary intestinal lymphangiectasia.
Eur J Gastroenterol Hepatol. 2005 Dec

Laharie D,
Degenne V,
Laharie H,
Cazorla S,
Belleannee G,
Couzigou P,
Amouretti M.
Service d'Hepatogastroenterologie, Hopital Haut-Leveque, Pessac, France.

Primary intestinal lymphangiectasia (PIL), so-called Waldmann's disease, is an uncommon condition, characterized by dilated intestinal submucosal and subserosal lymphatics of the gastrointestinal tract. Protein-losing enteropathy is the most common manifestation of this supposed congenital disease. Since the initial description in 1961, 11 cases of lymphoma have been reported suggesting that PIL predisposes to lymphoma. Here, we report the first case of primary nodal location lymphoma during PIL with recovery of the protein-losing enteropathy after its treatment by radiochemotherapy.

Lippincott Williams & Wilkins

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Friday, April 20, 2007

A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.

A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.
Pediatrics. 2001 Feb

Salvia G,
Cascioli CF,
Ciccimarra F,
Terrin G,
Cucchiara S.
Via Sergio Pansini 5, I-80131, Naples, Italy.

Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. Endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. Histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables.

These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.


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Friday, April 13, 2007

Capsule Endoscopy in the Pediatric Patient

Capsule Endoscopy in the Pediatric Patient
Curr Treat Options Gastroenterol. 2006 Sep

Seidman EG,
Dirks MH.
Division of Gastroenterology, Montreal Children's Hospital, 2300 Tupper Street, Room D562, Montreal, Quebec H3H 1P3, Canada.

Wireless capsule endoscopy represents an extraordinary technical innovation in diagnostic gastrointestinal endoscopy. As in adult patients, it opens new horizons that permit an accurate and noninvasive approach to identifying occult lesions in the small bowel in children and adolescents. A limitation in the pediatric age group is the size of the capsule, precluding its use in infants and small toddlers.

In children unable to swallow the capsule, "front loading" the gastroscope to introduce it into the duodenum is a suitable alternative approach. Capsule endoscopy is highly useful to evaluate for inflammatory changes in patients suspected to have small bowel Crohn's disease in whom conventional imaging failed to confirm the diagnosis. It is now the method of first choice to assess for small bowel polyps or tumors, to find a source of blood loss in obscure intestinal bleeding, and for undiagnosed malabsorptive conditions such as intestinal lymphangiectasia.

Capsule retention is the one major potential adverse effect of capsule endoscopy. In patients suspected to have a small bowel stenosis, consideration should be given to using the patency capsule prior to using the real videocapsule so as to decrease the risk of capsule retention.


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Friday, April 06, 2007

Protein-losing enteropathy in Crohn's disease.

Protein-losing enteropathy in Crohn's disease.

Ferrante M, Penninckx F, De Hertogh G, Geboes K, D'Hoore A, Noman M, Vermeire S,
Rutgeerts P, Van Assche G.

Department of Gastroenterology, University Hospital Gasthuisberg, Leuven, Belgium.

Protein-losing enteropathy (PLE) is a rare but severe complication of Crohn's disease (CD) and hypoalbuminemia can be one of the presenting symptoms of this illness. The diagnosis of PLE can only be made after exclusion of malnutrition and liver or kidney failure. Significant intestinal leakage can be caused by mucosal injury, increased lymphatic pressure or dilated lymphatics and has been reported in a large number of diseases.

The protein-losing can be diagnosed by assessing the excretion of different radiolabeled macromolecules in the faeces or by the clearance of alpha-1-antitrypsine in stools. The primary approach should be the optimization of the nutritional status. Medical treatment of the underlying disease is primordial. In other cases surgical resection of the most affected areas is inevitable. We report a case of a 21-year-old male with a 4 year history of CD, who developed significant hypoproteinemia with pitting oedema, initially in the absence of any other sign of severe disease activity.

A "Cr-chloride albumin excretion confirmed our hypothesis of protein-losing enteropathy. Because of sub-obstruction signs some months later, a laparotomy was performed which revealed a severely affected loop with dilatation of the proximal jejunum. Interestingly, multiple large lymph nodes and dilated lymphatics were seen. A partial jejunal resection was performed for stricturing Crohn's disease. Histology showed severe mesenteric granulomatosis, dilated lymph vessels and granulomatous vasculitis. After the resection our patient improved without further albumin infusions and the oedema resolved.

PMID: 17343081 [PubMed - in process]

Like intestinal lymphangiectasia Crohn's disease involves distortions in the intestinal lymphatics. I thought it would be interesting to present this.

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