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Objective: To explore the diagnostic value of high-frequency ultrasound in children with primary intestinal lymphangiectasia (PIL). Methods: Totally 20 children with clinically diagnosed PIL were retrospectively analyzed. All 20 children underwent abdominal high-frequency ultrasound,13 of them underwent pathologic examinations. The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of ultrasound in diagnosis of PIL were calculated. Results: The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of high-frequency ultrasound in diagnosis of PIL was 80.00% (16/20), 92.31% (12/13), 57.14% (4/7), 80.00% (12/15) and 80.00% (4/5), respectively. Conclusion: High frequency ultrasound can accurately detect PIL from children with thickened intestinal wall.
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Objective@#To analyze the clinical manifestations, diagnosis, treatment and prognosis of intestinal lymphangiectasia (IL) in children in order to improve the skills of diagnosis and treatment of IL.@*Method@#Clinical manifestations, laboratory findings, gastroscopic findings, histopathological examinations and lymphatic radionuclide imaging assessments were analyzed retrospectively among 47 IL patients who were hospitalized in the Gastroenterology Department of Beijing Children's Hospital Affiliated to Capital Medical University from June 2007 to December 2015. All patients were followed up by telephone. According to the various causes, the patients were divided into the primary intestinal lymphangiectasia (PIL) group and secondary IL group, and their clinical manifestations were compared by t test, Rank sum test or Chi-square test.@*Result@#In 47 IL patients, there were 38 children (81%) younger than 3 years old. There were 43 PIL patients (91%) and 4 secondary IL patients (9%). Between PIL and secondary IL, there were statistical differences in serum albumin (t=-3.950, P<0.005) , globulin(t=-2.850, P=0.007), age of onset(U=27.000, P=0.024), age at diagnosis(U=29.000, P=0.030) and course of disease(U=26.500, P=0.023), whereas there were no statistical differences in lymphocyte count, IgG, lymphatic radionuclide imaging, histopathology and gender(all P>0.05). Edema (44 cases, 94%), diarrhea (42 cases, 89%), accompanied with infection (35 cases, 74%) and ascites (30 cases, 64%) were the main clinical manifestations. In 47 IL patients, 45 patients were done gastroscopy and histopathological examinations, and there were 31 patients' histopathological examinations(69%) were positive. Forty patients were done lymphatic radionuclide imaging, and there was evidence of protein losing from gut via lymphatic radionuclide imaging in 39 patients(98%). Among 47 patients, 35 patients (74%) were followed up, 32 patients had good prognosis, 2 patient failed to show evidence of improvement, 1 patient died and no patient experienced a relapse till the end of the follow-up. In 35 patients, 28 patients were treated with medium chain triglycerides (MCT) dietary therapy, 26 patients showed improvement in symptoms, and 2 patients had no improvement. Among 35 patients with follow-up, there were 6 patients received surgical treatment, and their symptoms were improved.@*Conclusion@#PIL are the majority of IL in children younger than 3 years old. The main clinical manifestations are edema, diarrhea, accompanied with infection and ascites. For the patients without the evidence of lymphangiectasia from duodenum histopathological examination, further consideration of lymphatic radionuclide imaging, clinical manifestations, and laboratory studies are needed to make a final diagnosis. MCT dietary therapy is the cornerstone of IL medical management.
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Objective To investigate the incidence,diagnosis,treatment and prognosis of abnormal lymphatic vessels in intestinal lymphangiectasia (IL) patients complicated by portal vein hypertension.Methods General information,clinical manifestations,laboratory tests,endoscopy and pathological examination,the results of direct and nuclide lymphangiography,treatment and prognosis of seven IL patients complicated by portal vein hypertension were retrospective analyzed.Results Among seven IL patients complicated by portal vein hypertension,there were five cases with abnormal lymphatic vessels and two cases were merely secondary to portal vein hypertension.The main symptoms were edema,abdominal distension and diarrhea.The results of laboratory tests mainly were lymphocytopenia (0.3× 109 to 1.7 × 109/L),hypoalbuminemia (16.0 to 27.8g/L) and hypoglobulinemia (13.2 to 17.7 g/L).Under endoscope,mucosal edema,chronic inflammatory reaction,intestinal villi spot and patched white nodular changes and polyp-like changes appeared.For treatment,the low-fat with medium-chain triglyceride diet and symptomatic support was the basic treatment.The condition could be relieved by surgical remission of abnormal lymphatic vessels or partial small-bowel resection.If the etiological factors could not be removed,the therapeutic effect was poor in the patients merely secondary to portal vein hypertension.Conclusion The suspicious IL patients complicated by portal vein hypertension need further examination to explore whether abnormal lymphatic vessels existed,and to provide evidence for surgical intervention.