Lymphangiectasies intestinales primitives (maladie de Waldmann)

Vignes, S.; Bellanger, J

doi:10.1016/j.revmed.2017.07.009

Cited by 11 publications

(9 citation statements)

References 67 publications

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“…The gastrointestinal abnormalities that may cause PLE are the following: (1) abnormalities of the lymphatic system, resulting in leakage of protein-rich lymph as in primary intestinal lymphangiectasia or secondary to obstruction (neoplasm, large bezoars) or elevated lymph pressure (congestive heart failure or after Fontan operation); (2) mucosal injury, resulting in increased mucosal permeability due to mucosal erosion (IBD like Crohn's disease and ulcerative colitis, certain enteric The diagnosis of PLE is suspected from clinical manifestations and physical examination and it can be confirmed by the detection of increased level of alpha-1-antitrypsin in a stool sample as observed in our patient (4). The elevated alpha-1antitrypsin clearance is due to its passage from the blood to the intestinal lumen from where it is not reabsorbed.…”

Section: Discussionsupporting

confidence: 63%

“…The most affected protein is albumin, but also other proteins with a slow turn-over rate such as immunoglobulins and ceruloplasmin can be loss. Edema, ascites, pleural, and cardial effusions might complicate hypoproteinemia (3,4).…”

Section: Discussionmentioning

confidence: 99%

“…The elevated alpha-1antitrypsin clearance is due to its passage from the blood to the intestinal lumen from where it is not reabsorbed. However, its detection in stool sample does not localize the site of mucosal injury and protein loss (3,4).…”

Section: Discussionmentioning

confidence: 99%

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An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

et al. 2020

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Protein-losing enteropathy (PLE) is a rare condition characterized by protein loss through the gastrointestinal tract, leading to hypo-proteinemia. Patients may be asymptomatic or present with variety of complications of hypoproteinemia (e.g., oedema, ascites, pleural, and cardial effusions). We describe a case report of a young girl suffering from behavioral disorder since childhood who presented with generalized oedema, hypoproteinaemia, and microcytic hypochromic anemia. In addition, the girl had an intervention for jejunal atresia and intestinal malrotation in her past medical history. Upper gastrointestinal endoscopy revealed a trichobezoar extending from stomach into the small bowel, thus classified as Rapunzel Syndrome (RS), causing mechanical obstruction of intestinal lumen and intestinal lymphatic drainage resulting in a protein-losing enteropathy (PLE). Trichobezoar was successfully removed by a surgical laparotomy resulting in resolution of symptoms and normalization of biochemical parameters. Possibly, previous surgery might have had an influence on intestinal dysmotility and trichobezoar formation. PLE is a very rare presenting symptom of RS, developing as result of intestinal obstruction caused by large trichobezoars. RS has to be considered in patients, especially adolescents, suffering from behavior disorder as trichotillomania and trichophagia. Surgical removal and nutritional supplementation are the gold treatment of large trichobezoar.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 99%

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An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

et al. 2020

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“…It is a rare entity in pediatrics of unclear etiology to date. It is an exudative enteropathy due to a congenital or obstructive malformation of the intestinal lymphatic drainage system, and secondary the dilation of mucosal and submucosal lymphatic vessels [ 2 ]. This causes the leakage of the lymph and its components through the intestinal lumen responsible for clinical and biological symptoms [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

“…The presence of paucisymptomatic forms makes its prevalence difficult to define. It is a disease that most often begins at an early age before 3 years old, but several cases have been diagnosed in adolescence or even in adulthood [ 2 ]. Edema of the lower limbs and chronic diarrheas are the two main clinical signs, while hypoalbuminemia, hypogammaglobulinemia, and lymphopenia are among the first-line biological signs [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting

Haddar

Sbia

Rkain

et al. 2021

Annals of Medicine &Amp; Surgery

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Introduction and importance The primary intestinal lymphangiectasia is a rare exudative enteropathy of unknown etiology that affects the lymphatic system. It causes lymphedema and malabsorption syndrome by the escape of the lymph and its elements into the intestinal lumen. Case presentation A female patient, diagnosed at the age of 11 with Waldmann's disease, has initially manifested chronic diarrhea with a stature-ponderal delay at the age of 6 months old; she was treated for a long time as celiac disease patient. Edematous syndrome, chronic diarrhea, staturo-ponderal delay and asymmetric lymphedema of the upper limb are the main clinical symptoms in this case. In addition, the exclusion of secondary intestinal lymphangiectasia was important for the diagnosis. Before and during her follow-up, the patient presented two complications of the disease: warts and osteomalacia. The patient did not respond to treatment with the low-fat diet; therefore, the need to add treatment with octreotide was necessary, which has given quite pleasant results. Octreotide was the therapeutic choice to treat the patient as she was resistant to the appropriate regimen with clinical improvement; nevertheless, certain biological elements of lymphatic leakage persisted. Discussion Waldmann's disease is rare. It can be responsible, besides the typical signs, for complications including warts and osteomalacia. The histopathological study of intestinal biopsies may be normal if lymphangiectasias are localized. The treatment is based on a nutritional diet associated with octreotide. During the patient's follow-up, the evolution after almost two years of the introduction of octreotide compared to the diet alone showed improved outcomes. Conclusion The treatment of Waldmann's disease is based on an adapted diet and octreotide. This case highlighted the importance of the long term follow-up in this disease.

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