Evaluation of the clinical value of capsule endoscopy in patients with unexplained abdominal pain / 西安交通大学学报(医学版)

【Objective】 To evaluate the clinical value of capsule endoscope in the diagnosis of unexplained abdominal pain. 【Methods】 We made a retrospective analysis of 191 patients with unexplained abdominal pain who sought medical help in our hospital and 25 normal controls. Capsule endoscopy was performed in both groups, small bowel lesions were detected, and clinical data were collected for further analysis. 【Results】 The total small bowel lesion detection rate was 52.87% (101/191) in abdominal pain (AP) patients and 20% (5/25) in the control group, respectively. The detection rate of significant findings (ulcers, erosions, polyps, diverticula, parasites, and neoplastic organisms) was only 16.23% (31/191) in AP patients. In the non-significant findings, no statistical difference in the detection rates for vascular malformation, capillary dilation, and lymphoid follicular hyperplasia were found between the two groups, while the detection rate of intestinal lymphangiectasia was significantly higher in the AP patients (23.56% vs. 4%, P<0.05, OR=7.089). 【Conclusion】 Capsule endoscopy can be an optional choice for diagnosis of unexplained abdominal pain, while the relationship between positive findings and abdominal pain should be further investigated.

Linfangiectasia intestinal primaria: Ascitis quilosa como forma de presentación / Primary intestinal lymphangiectasia: Chylous ascites as a presentation form

Presentamos el caso de lactante de 6 meses de edad referido por aumento progresivo de perímetro abdominal. A la valoración con datos de ascitis. La paracentesis diagnóstica muestra líquido ascítico quiloso. El diagnóstico de linfangiectasia se realizó a través de endoscopia digestiva alta, con biopsia intestinal

We present the case of 6-month-old infants referred for ascites. The paracentesis shows uid of chilosas characteristics. The diagnosis of lymphangiectasia was made through upper digestive endoscopy, with intestinal biopsy

Linfangiectasia intestinal primaria o enfermedad de Waldmann / Primary Intestinal Lymphangiectasia or Waldmann Disease

La linfangiectasia intestinal primaria en una patología infrecuente causada por la malformación de los conductos linfáticos intestinales. Normalmente se diagnostica antes de los 3 años de edad, pero puede aparecer en población adulta. Los síntomas más frecuentemente encontrados son la presencia de anasarca y dolor abdominal acompañado de malnutrición. El tratamiento es sintomático y se basa en la sustitución dietética de los triglicéridos de cadena larga por triglicéridos de cadena mediana con aumento del aporte proteico. En este trabajo se reporta el caso de una paciente femenina de 22 años de edad la cual presenta manifestación clínicas, imaginológicas y anatomopatológicas que permiten realizar el diagnóstico de una linfangiectasia intestinal primaria, caso extremadamente infrecuente y sobre todo a esta edad(AU)

Primary intestinal lymphangiectasia in an uncommon pathology caused by malformation of the intestinal lymphatic ducts. It is usually diagnosed before 3 years of age, but may appear in the adult population. The most frequent symptoms are the presence of anasarca and abdominal pain accompanied by malnutrition. The treatment is symptomatic and is based on the dietary substitution of long chain triglycerides by medium chain triglycerides with increased protein intake. This paper reports the case of a female patient of 22 years of age who presents clinical, imaging and anatomopathological manifestations that allow the diagnosis of primary intestinal lymphangiectasia, an extremely rare case, especially at this age(AU)

Linfangiectasia intestinal: Reporte de un caso / A case report of Intestinal Lymphangiectasia

A propósito del caso de un paciente de 7 meses de vida remitido desde Yopal a la ciudad de Bogotá, se revisa el tema de la linfagiectasia intestinal. Esta es una rara enfermedad que involucra los vasos linfáticos intestinales, y origina hipoproteinemia, edemas, ascitis y enteropatía perdedora de proteínas.

This is the case report of a 7 month old child from Yopal with intestinal lymphangiectasia who was sent to Bogota. We also review the issue of intestinal lymphangiectasia, a rare disease involving intestinal lymphatic vessels which caused hypoproteinemia, edema, ascites and protein-losing enteropathy.

Linfangiectasia intestinal asociada a hemihipertrofia: Reporte de caso / Intestinal Lymphangiectasia associated with Hemihypertrophy: A case report

La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.

Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.

Primary intestinal lymphangiectasia in 8 infants / 中华实用儿科临床杂志

Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia (PIL) in infants.Methods Clinical,laboratory,gastroscopy imaging,lymph radionuclide imagining,and therapeutic intervention data in eight patients admitted with PIL in Beijing Children's Hospital from Jan.2007 to Feb.2012 were reviewed.Results Their ages ranged from 4 to 8 months old.The common complaints were edema(8/8 cases),diarrhea (8/8 cases),infection (8/8 cases),and ascites (7/8 cases).Other symptoms included vomiting (5/8 cases),low body weight (2/8 cases),and convulsions (2/8 cases).Infections involving respiratory tract,blood,and gastrointestinal system were prominent.The causal pathogens for the infections were bacteria,virus,fungi,and ectosarc.The laboratory abnormalities included lymphocytopenia (8/8 cases),hypoalbuminemia (8/8 cases),and hypogammaglobulinemia (8/8 cases).On immunologic evaluation,the CD4 cell counts and serum IgG levels were significantly decreased(7/7 cases) while B cell and NK cell counts were normal.The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots(8/8 cases).Further pathological examinations indicated dilated lymphatic channel in mucosal and submucosal(5/8 cases).Lymph radionuclide imaging discovered abnormalities consistent with PIL in 6 out of 8 patients,including 3 cases that were negative for pathological examination.A multidisciplinary approach was taken to treat each patient.The low-fat and medium chain triglycerides-rich diet was introduced with supplements of albumin and globulin.Infection control and edema alleviation were well managed through medication.However,diarrhea remained a problem.Only 2 out of 8 patients had recovered from hypoalbuminemia and lymphocytopenia.One case had a complete remission after surgical therapy withno relapse.Conclusions PIL in infant has common clinical presentations with an exception of more severe infection.Lymph radionuclide imaging is a sensitive diagnostic method.Medication treatment for infant PIL is not satisfactory.Surgical management is recommended if the locus of pathology is confined.

A Case of Primary Intestinal Lymphangiectasia Diagnosed by Double Balloon Enteroscopy

Primary intestinal lymphangiectasia is a congenital lymphatic disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein losing enteropathy. As a result, generalized edema, hypoalbuminemia, and lymphocytopenia are clinically manifested. We could not find the reason by several examinations. Therefore, we performed double balloon enteroscopy (DBE), and intestnal lymphangiectasia was diagnosed histologically by a biopsy. DBE is a safe and effective method to diagnose small bowel lymphangiectasia. We report a case of primary intestinal lymphangiectasia, which occurred in a 54-year-old male patient with generalized edema and ascites.

Primary intestinal lymphangiectasia / 国际儿科学杂志

Primary intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction of lymphatic return, dilated lymphatics and distorted structure of villi. Lymph fluid leaks into the small bowel lumen as a result of the obstruction of lymphatics and elevated pressure of lymphatics, and leads to malabsorption and loss of proteins. The causes and pathogenesis of PIL are not clear, hut the abnormal immunologic mechanism has got people's attention. Recently, the endoscopic technique has been improved, and the diagnosis rate of PIL has increased obviously. Antisecosis is the basic and effective treatment, especially for children.

White spots on the mucosal surface of the duodenum in dogs with lymphocytic plasmacytic enteritis

Distended lacteals, described as expanded white villi in duodenum, are strongly indicative of primary intestinal lymphangiectasia. In the present study, we evaluated the significance of white spots present in the duodenal mucosa of dogs with lymphocytic plasmacytic enteritis (LPE). Fifty dogs with LPE were included in this study, and white spots were detected in the duodenal mucosa in 22 dogs during endoscopy. Hypoproteinemia was more frequent in dogs with white spots than in dogs without spots (p = 0.02). Serum protein and albumin concentration were significantly lower in LPE dogs with white spots (p = 0.038) compared to LPE dogs without white spots (p = 0.039). There was a significant correlation between white spots density and lymphatic dilatation histological scores (p = 0.023; rho = 0.481). These results suggest that the presence of white spots in the duodenal mucosa of dogs is not a finding exclusive for intestinal lymphangiectasia. Low serum protein and albumin concentrations together with lymphatic dilatation seem to be related to the presence of white spots in the duodenal mucosa of LPE dogs.

A Case of Small Bowel Lymphangiectasia that Presented with Gastrointestinal Bleeding and was Treated by Small Bowel Resection / 대한내과학회지

Intestinal lymphangiectasia (IL) is a rare disorder, characterized by dilatation of intestinal lymphatics and leakage from ruptured lacteals to the intestinal lumen. Primary IL may be due to a congenital malformation of the lymphatic system, whereas secondary IL is caused by decreased lymph flow from thoracic ductsdue to elevated left subclavian vein pressure as a result of a preceding inflammatory or neoplastic disease. IL can present as protein-losing enteropathy with clinical manifestations of hypoproteinemia, hypoalbuminemia, edema, ascites, or pleural effusions. In very rare cases, it can present as severe intestinal bleeding. We experienced a 48-year-old woman presenting with recurring hematochezia and melena. She was diagnosed bydouble balloon enteroscopy, and surgical resection was needed to stop bleeding. In conclusion, IL can present clinically as painless chronic blood loss. If IL is locally distributed, surgical resection may be needed to control bleeding and to exclude other underlying causes in some patients.

Primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy . We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.

A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia / 대한소화기내시경학회지

Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of alpha(1)-antitrypsin and (99m)Tc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient's clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements.

A Case of Intestinal Lymphangiectasia Improved with Antiplasmin and Octreotide Treatment / 대한소화기내시경학회지

Intestinal lymphangiectasia is characterized by protein- losing enteropathy, and is diagnosed by a small bowel biopsy demonstrating dilated lymphatics in the mucosa, submucosa and serosa in the absence of coexistent inflammation. We report a case of primary intestinal lymphangiectasia that occurred in a 2-year-6-month-old girl who was treated successfully with antiplasmin and octreotide. Initially, the patient was treated with a lipid restriction diet with medium chain triglyceride oil, but her symptoms were not relieved. This case shows that antiplasmin and octreotide therapy might be useful for treating refractory primary intestinal lymphangiectasia.

A Case of Primary Intestinal Lymphangiectasia / 대한소아소화기영양학회지

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

A Case of Intestinal Lymphangiectasia

Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as diffuse nodular thickening of the small bowel and as linear hypodense streaking densities in the small bowel caused by dilated lymphatic channels. Demonstration of increased enteric protein loss using 51Cr-, 131I- or 99mTc-labeled albumin, timed measurement of fecal excretion of radioactivity or by measuring fecal clearance of alpha 1-antitrypsin can also help the diagnosis. We experienced a rare case of intestinal lymphangiectasia in an eight year old boy who presented with facial edema, abdominal distension and intermittent diarrhea. We report a patient with intestinal lymphangiectasia, in whom abdominal CT, 99mTc-labeled albumin scintitigraphy, and stool alpha 1-antitrypsin measurement played key roles in determining the diagnosis. A brief review of literature was made.

Follow up of a Case of Primary Intestinal Lymphangiectasia

Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or the peritoneal cavity. The mechanism of this lymphatic loss is believed to be due to a rupture of lymphatics across the mucosa with subsequent leakage of the lymph into the bowel lumen. Presentation of primary intestinal lymphangiectasia may occur any time throughout infancy and childhood. Prominent clinical features include protein-losing enteropathy, lymphocytopenia, hypogammaglobulinemia, edema, growth failure, and chronic diarrhea. The mainstay of treatment for intestinal lymphangiectasia is the use of a low-fat, high- protein, medium-chain triglycerides diet. The natural history of this rare disorder is not well known as only a few cases have been reported in the literature. We present herein the follow up of a case with primary intestinal lymphangiectasia who responded to octreotide therapy but did not respond to dietary or antiplasmin therapy.

A Case of Primary Intestinal Lymphangiectasia / 대한소화기내시경학회지

We described a 22-month-old girl, who suffered from a gereralized edema and intermittent diarrhea for 1 month. Intestinal lymphangiectasia was confirmed by endoscopic biopsy. Endoscopic examination revealed showed the characteristic appearance of tiny white flakes scattered in the duodenum, which a histological examination confirmed to be dilated lymph vessels. The other findings from laboratory examinations included lymphopenia, hypoproteinemia, hypogammaglobulinemia and increased fecal a1-antitrypsin clearance. Treatment consisting of a low-fat diet and MCT supplementation was successful. The therapy has resulted in the disappearance of duodenal lesions previously observed endoscopically.

Endoscopic Diagnosis of Primary Intestinal Lymphangiectasia Using a High-fat Meal in a 20-Month-Old Boy / 대한소아소화기영양학회지

We experienced a case of primary intestinal lymphangiectasia in a 20-month-old boy, which was confirmed gastrofiberscopically and histologically only after a high-fat meal before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenal mucosa, which were proved dilated lymph vessels in the lamina propria on histological examination. Treatment with low-fat diet and medium chain triglycerides supplementation was satisfactory.

Significance of Endoscopic Small Bowel Biopsy in the Diagnosis of Intestinal Lymphangiectasia in Children / 대한소화기내시경학회지

BACKGROUND/AIMS: Intestinal lymphangiectasia is a disease characterized by dilated lymphatics of the intestinal mucosa and excessive enteric loss of plasma proteins. Instead of multiple blind peroral jejunal biopsy, duodenal endoscopy and endoscopic small bowel biopsy were performed. We evaluated the significance of endoscopic small bowel biopsy and the usefulness of the other diagnostic methods in the diagnosis of intestinal lymphangiectasia in children. METHODS: Fourteen children seen between August 1989 and August 1997 with clinically suggestive intestinal lymphangiectasia were analysed. The median age at onset of symptoms was 4 years. Primary intestinal lymphangiectasia occurred in 10 children and secondary intestinal lymphangiectasia occurred in 4 children, of whom two had Fontan operation, one had constrictive pericarditis, and one had Crohn's disease. Low fat, high protein diet with medium chain triglycerides was the mainstay of treatment. RESULTS: 1) Diarrhea was present in 14 patients, and edema in 11 patients. Hypocalcemic tetany occurred in 6 children and vomiting in 5 children. Dight children had ascites and three of these had chylous ascites. Growth retardation was present in four patients, chylothorax in one, and lymphedema in one. The initial serum albumin concentration was 1.8 g/dl, the serum calcium level 6.7 mg/dl, and the total lymphocyte count 623 /mm(3). 2) Dilated lymphatics in the small bowel mucosa was confirmed by endoscopic biopsy in 14 children(100%). The sensitivity of alpha1-antitrypsin clearance was 100%. Duodenal endoscopy showed scattered white spots covering mucosa in 11 children(79%). Small bowel series revealed thickened mucosal folds in 10 children(77%). Four(31%) had positive finding of 99mTc-antimony lymphoscintigraphy. 3) Responses to treatment in children with primary intestinal lymphangiectasia were graded as good if the symptoms resolved, and poor if there was no lasting resolution of symptoms and repeated albumin administration. Response to therapy was good in four and poor in six patients. The mean age at onset of symptoms was 8 years in good resonse group, and 2 years in poor response group (p<0.05). CONCLUSION: The diagnosis of intestinal lymphangiectasia is confirmed by duodenoscopy and endoscopic small bowel biopsy in a child with diarrhea, edema, hypoalbuminemia, and lymphocytopenia. As compared with other diagnostic methods such as small bowel series and lymphoscintigraphy, duodenoscopy and endoscopic small bowel biopsy are very sensitive and should be performed early.

Idiopathic Intestinal Lymphangiectasia

This is a case report of idiopathic intestinal lymphangiectasia occurring in a 3-year-old boy. Idiopathic intestinal lymphangiectasia is believed to be a part of the generalized congenital disorder of lymphatic system. The present case revealed markedly ectatic lymphatics in the lamina propria of duodenal villi with lymphorrhagia. Clinical features were typical of the protein-losing enteropathy; hypoalbuminemia, hypocalcemia, chylous ascites, edema of the lower extremities and mild lymphocytopenia. This case indicates that serious protein loss may ensue, even in cases of intestinal lymphangiectasia without abnormalities of extraintestinal lymphatics.