Primary 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,lympho...Primary 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 conf irmed 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.展开更多
The relationship between primary intestinal lymphangiectasia(PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder.A recent paper reported that a six-month lowfat die...The relationship between primary intestinal lymphangiectasia(PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder.A recent paper reported that a six-month lowfat diet improved liver fibrosis.We report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year,despite dietetic support.This and the previous case report describe extraordinary events,which do not allow clear-cut clinical aspects to be established.Nevertheless,both cases suggest that in patients with PIL,it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography,but also elastography.展开更多
Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease...Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.展开更多
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropath...Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.展开更多
文摘Primary 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 conf irmed 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.
文摘The relationship between primary intestinal lymphangiectasia(PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder.A recent paper reported that a six-month lowfat diet improved liver fibrosis.We report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year,despite dietetic support.This and the previous case report describe extraordinary events,which do not allow clear-cut clinical aspects to be established.Nevertheless,both cases suggest that in patients with PIL,it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography,but also elastography.
文摘Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.
文摘Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
