2014年 · 第52卷第05期

Digestive endoscopy has played a revolutionary role in the diagnosis and treatment of digestive system diseases. Since the digestive endoscopy began to be used in pediatrics in the late 1980s, it has been widely used in pediatrics as an advanced medical diagnosis method. Many units have successively carried out pediatric gastroscopy, colonoscopy and various endoscopic treatment items. In recent years, with the popularization of double balloon enteroscopy, endoscopic ultrasound, capsule endoscopy and endoscopic retrograde cholangiopancreatography (ERCP) in pediatrics, digestive endoscopy is no longer a simple technology, but has become a new discipline with theory, technology, diagnosis and treatment, namely pediatric digestive endoscopy.

Abnormal intestinal wall permeability plays a very important role in many human diseases. In the field of pediatrics, it has been found that increased intestinal wall permeability is associated with some diseases^[1,2,3], such as sepsis, systemic multiple organ system failure, food allergies, allergic diseases, some studies in recent years have found that asthma, type I diabetes, inflammatory bowel disease, autism, etc. are accompanied by increased permeability of the intestinal wall, and this phenomenon occurs before the disease and is thought to be related to the pathogenesis of the disease. What directly affects the permeability of the intestinal wall is the mechanical barrier of the intestinal mucosa, that is, the intestinal mucosal epithelial structure, including intact epithelial cells, the tight lipid bilayer at the brush edge of epithelial cells, and the cellular junctions at the lateral edges of cells. The junction between intestinal epithelial cells, from apical to basement membrane, is tight junction, adhesive junction, desmosome and gap junction. Tight junction is the most important structure constituting the epithelial barrier function, and it has the greatest relationship with the permeability of intestinal wall. Damage to tight junction directly leads to the increase of intestinal wall permeability, which is the so-called "leaky gut". The correlation between the structure and regulation of intestinal epithelial tight junction and disease is a hot research topic in recent years, which needs to attract the attention of pediatric clinicians and basic workers. Studying the function of intestinal epithelial barrier, the structure of tight junction protein and the corresponding regulatory mechanism can improve our understanding of the etiology and pathophysiology of disease, and it is of great significance for early detection of disease and secondary prevention of disease.

Crohn's disease (CD) is a chronic non-specific inflammation of unknown cause, with clinical manifestations of recurrent abdominal pain, diarrhea, abdominal mass, intestinal fistula, anal lesions, weight loss and growth retardation, and extraintestinal lesions such as arthritis and iridocyclitis. At present, the treatment methods include drug therapy, nutrition therapy, psychotherapy and surgery, but it is still unable to fundamentally cure the disease. As a special group, children are in the process of continuous growth and development, and their physiology and psychology are facing tremendous changes in adolescence. Therefore, the treatment goals of CD in children are not only limited to inducing and maintaining remission, preventing recurrence, avoiding long-term complications, improving the quality of life, but more importantly, promoting children's growth and development and maintaining a healthy physiological and psychological state.

A male, 7 months and 18 days old, was admitted to the hospital on December 5, 2012 due to "difficulty eating for more than 6 months, cough with fever for 2 days". After suffering from "pneumonia" more than 6 months ago, the child had repeated choking and difficulty eating, manifested as vomiting during feeding or immediately after feeding, choking from the mouth and nose, with a large amount of liquid milk, no curd, no hematemesis, no coffee dregs, no yellow-green bile, no cyanosis, no abdominal distension and diarrhea, no fever, no crying and moving less, and no shortness of breath. The local hospital considered "gastroesophageal reflux and esophageal stenosis" and was treated with nasal feeding so far. There was no vomiting, abdominal distension and diarrhea after nasal feeding, but he suffered from pneumonia repeatedly for 5 to 6 times, all of which improved after anti-infective symptomatic treatment in the local hospital. Two days ago, the child had a cough after catching a cold, which was a paroxysmal monophonic cough, with phlegm in the throat, no cyanosis, no wheezing and shortness of breath, and fever, with a fever peak of 38.9℃, no chills and convulsions, so he came to the outpatient clinic of our hospital and was hospitalized with "gastroesophageal reflux, hiatal hernia, and esophageal stenosis". Since the onset of the disease, the child has been clear and energetic. In the past 2 months, 80 ml of milk was fed into the nasogastric tube every time, 20 ml was fed orally, once every 2 hours or so, and the stool was once a day, yellow paste, the urine output was acceptable, and the weight gain was slow, with a total increase of about 2.0 kg in half a year. Admission physical examination: body temperature 38.9 ℃, heart rate 140 beats/min, breathing 32 beats/min, blood pressure 93/57 mmHg (1 mmHg =0.133 kPa), body length 63.5 cm, weight 5.6 kg, thin appearance, lethargy, pale complexion, flat and soft fontanel, red throat, superficial lymph nodes not swollen, coarse breathing sounds in both lungs, more dry and wet rales can be heard, heart rhythm is uniform, heart sounds are in the middle, no pathological murmur, abdomen is flat and soft, mass is not reached, liver and spleen are not reached under the costs, and the neurological examination is negative. Auxiliary examination (local hospital, September 2012): upper gastrointestinal angiography: "C5~6 level esophagus is thin, arcuate right process, contrast medium passes acceptable, horizontal transverse stomach, pneumonia"; Esophageal pH measurement: "pathological acid reflux visible"; Electronic gastroscopy: "middle and upper esophageal lumen stenosis, gastric antrum edema, duodenal mucosal edema"; Fiberoptic bronchoscopy: "tracheobronchial endometrial inflammation, no airway malformation"; Laryngoscopy: "Laryngeal chondromalacia"; Skull MRI showed "T2WI bilateral globus pallidus symmetry slightly high signal; paranasal sinus and bilateral mastoid long T2 signal".

The proband, a 25-day-old male, was recalled due to the abnormal acyl carnitine index found by tandem mass spectrometry screening of neonatal dried blood filter paper, and was first diagnosed in the outpatient department of genetics and metabolism, Children's Hospital affiliated to Zhejiang University School of Medicine on January 23, 2013. The child was the fourth child of the mother, delivered naturally at full term, with a birth weight of 3 600 g, no history of asphyxia rescue, breastfed after birth, fed milk, did not spit milk, and had normal urine and stool.

The child was a 12-year-old male. He was admitted to hospital due to intermittent fever for 10 days and erosion of lips and external genitals for 5 days. The child developed fever 10 days before admission, with a maximum body temperature of 39 ℃. A cough with yellow sticky sputum appeared 8 days before admission. Oral mucosa and glans erosion, difficulty in eating and pain in urination occurred 5 days before admission; At the same time, it was accompanied by conjunctival congestion and increased secretion in both eyes, and blurred vision. The child had no history of taking medication before the illness, and denied the history of previous drug allergy. Before admission, the local blood test routine was roughly normal, and no other tests were performed. Ribavirin, dexamethasone and ambroxol were given. The specific diagnosis, drug dose and course of treatment were unknown.