Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet

Alfano, Vincenzo; Tritto, Giovanni; Alfonsi, L.; Cella, A; Pasanisi, Fabrizio; Contaldo, Franco

doi:10.1016/s0899-9007(00)00223-9

Cited by 38 publications

(20 citation statements)

References 5 publications

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“…A low-fat diet with supplemental MCT forms the cornerstone of management in PIL [23] . MCT are directly absorbed into the portal venous system, which prevents lacteal engorgement [24] . Dietary intervention is more effective in children than in adults, therefore early diagnosis of intestinal lymphangiectasia and consistent attention to diet are very important for early nutrition and growth [25] .…”

Section: Discussionmentioning

confidence: 99%

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Oh¹

2011

WJGE

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Author contributions: Oh TG designed the case report as a main author; Chung JW and Kim HM contributed to provide background knowledge; Han SJ provided many informations about the patient; Lee JS provided genetic information and accessment; Park JY participated in endoscopic evaluation and accessment; Song SY supervised and checked up the manuscript finally as a corresponding author; all authors reviewed and approved the final manuscript. AbstractPrimary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.

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Section: Discussionmentioning

confidence: 99%

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Oh¹

2011

WJGE

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“…MCT are directly absorbed into the portal venous circulation and thus provide nutrient fat but avoid lacteal engorgement. After a few weeks, this treatment may lead to reversal of clinical and biochemical signs (albuminemia, Ig levels and lymphocyte counts) [24]. In our patient, clinical signs reversed within a week on diet; however, moderate hypogammaglobulinemia and hypoalbuminemia as well as intermediate platelet excess and digital clubbing remained unchanged after one year.…”

Section: Discussionmentioning

confidence: 53%

Digital clubbing in primary intestinal lymphangiectasia: a case report

Wiedermann

Kob

Benvenuti

et al. 2010

Wien Med Wochenschr

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Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, 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. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.

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“…In practice, reduction of the intestinal lymph flow through fat restriction has been reported (1,2,14). Likewise, a diet containing MCTs as a substitute for long-chain triglycerides is of benefit, because MCTs are absorbed directly into the portal vein rather than the lymphatics, thereby reducing the pressure of the lymph channels (3,4).…”

Section: Discussionmentioning

confidence: 99%

Enteral Nutrition as a Primary Therapy for Intestinal Lymphangiectasia: Value of Elemental Diet and Polymeric Diet Compared with Total Parenteral Nutrition

et al. 2005

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Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.

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Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet

Cited by 38 publications

References 5 publications

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Digital clubbing in primary intestinal lymphangiectasia: a case report

Enteral Nutrition as a Primary Therapy for Intestinal Lymphangiectasia: Value of Elemental Diet and Polymeric Diet Compared with Total Parenteral Nutrition

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