The Diagnosis And Therapy Of Primary Intestinal Lymphangiectasia Of Children,Report Of 21 Cases

Objective:To evaluate the clinical information,diagnosis and prognosis of primary intestinal lymphangiectasis(PIL)in children,which to improve the clinician's understanding of the disease.Methods:The diagnosis of twenty-one cases with PIL was established according to intestinal mucosal biopsy hospitallized in Children's Hospital of Chongqing Medical University from June 2001 to December 2016 were enrolled in this study.History traits,clinical symptoms,laboratory examination,imaging tests,endoscopy and the results of intestinal tissue biopsy were retrospectively analyzed.Result:(1)clinical manifestations: there were 21/21 cases(100%)with diarrhea,17/21 cases(81.0%),with edema 19/21 cases(90.5%)with respiratory co-infection caused by respiratory common pathogens,Which were: klebsiella pneumoniae,haemophilus parainfluenzae,escherichia coli,respiratory syncytial virus,parainfluenza virus,Coxsackie B virus,mycoplasma pneumoniae,other symptoms including malnutrition in 9/21 cases,anorexia in 5/21 cases,low calcium convulsions in 4/21 cases,vomiting in 3/21 cases,abdominal distension in 3/21 cases,and abdominal pain,joint pain,abdominal bulge and recurrent oral ulcer in 1/21 cases,respectively.(2)Laboratory examinations were found that 21/21 cases with lymphocytopenia and hypoalbuminemia,18/21 cases with serum IgG levels decreased significantly,19/21 cases with low CD4 cell counts,both which the most obvious decline in the number and degree,and in part of cases with hypocalcemia,hyponatremia,or hypolipidemia.(3)Chest and abdomen CT showed varying degrees of peritoneal effusion(13/21 cases),pleural effusion and pericardial effusion(1/21 cases,respectively),that no specific pathogens infection performance;Lymphoscintigraphy in 1 case revealed lymphatic leakage.(4)The endoscopy revealed that 21/21 cases of intestinal mucosal Snowflakes swollen uplift;Pathological examination showed 20/21 cases of mucosal and submucosal lymphangiectasis,with 4/21 cases of immunohistochemistry D2-40(+).The common lesion site is duodenal descending part.(5)The prime ways of treatment for PIL were introduction of a low-fat and MCT diet,albumin supplement,correct electrolyte imbalance,control infection and use somatostatin and so on.Diarrhea,edema and other symptoms were relief,infection was under control,which in most patient after medical therapy.Simultaneously,lymphocytes and albumin can be increased,but only 11/16 cases,6/17 cases beging to normalise,respectively.Conclusion:Edema and diarrhea are extremely common symptoms finding in children with PIL,and those patients are usually complicated with respiratory tract infection Currently,gastrointestinal endoscopy and pathological examination is suggested to be used as the first choice in diagnosing PIL in children,whose are based on medical treatment;surgical therapy can be used if lesion limitations and conservative treatment of internal medicine is ineffeciency.