ABSTRACT
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.
ABSTRACT
<p><b>OBJECTIVE</b>To analyze the clinical characteristics and treatment of eosinophilic gastroenteritis (EG) in children.</p><p><b>METHOD</b>A retrospective analysis of the clinical manifestation, laboratory test, endoscopy, biopsy, bone marrow examination and treatment of 6 children diagnosed with EG in Beijing Children's Hospital seen from Jun. 2005 to May 2009 was performed.</p><p><b>RESULT</b>Five of the cases were boys and 1 was girl, the range of age was from 2 to 13 years; food allergy test was positive in 4 cases. Clinical manifestations included:abdominal pain, diarrhea, vomiting, digestive tract hemorrhage, and ascites was found in 4 children. The eosinophilic cell count in blood increased in 5 cases [(160 - 13,560) × 10(6)/L]. The percentage of eosinophilic cell in bone marrow increased in 6 cases (0.18 - 0.41). Beside stomach and duodenum, anatomical foci were found in mesentery (5 cases), small intestine (4 cases), colon (3 cases), and esophagus (2 cases). Endoscopy and imaging showed edema, congestion, erosion, ulcer, bile reflux and gastric juice retention. Stomach and duodenum pathological changes included epithelial erosion, large quantities of eosinophilic cell infiltration in lamina propria and scattered lymphoid cells, which were more obvious in duodenum. In the group received glucocorticoid therapy, clinical symptoms disappeared 3 days later, the eosinophilic cell count in blood returned to normal one-week later;in the group of non-glucocorticoid therapy, most of the clinical symptoms disappeared after two weeks. The eosinophilic cell count in blood was still high 5 weeks later.</p><p><b>CONCLUSION</b>Compared to the common gastroenteritis, gastrointestinal symptoms in EG cases were nonspecific, the increase of blood eosinophilic cells can suggest the possibility of EG, but biopsy is the key to the diagnosis of EG. The therapeutic effect of glucocorticoid seemed to be better than non-glucocorticoid therapies in the improvement of clinical symptoms and the decrease of blood eosinophilic cell.</p>