Diagnosis and treatment of blue rubber bleb nevus syndrome in children.

BACKGROUND: Blue rubber bleb nevus syndrome (BRBNS) is characterized by distinctive vascular malformations of skin and the gastrointestinal tract, often leading to chronic anemia and intestinal bleeding. It usually presents right after birth or during early infancy. Though the disease is inherent, its occurrence is sporadic. Thus it is usually not timely diagnosed. We analyzed the clinical characteristics and treatment of this disorder in order to improve the diagnosis and treatment. METHODS: Three patients with BRBNS treated at our hospital during 2002-2003 and 39 patients from the literature reported during 1965-2003 were reviewed in terms of the diagnosis and treatment. BRBNS may be diagnosed as cutaneous cavernous hemangioma associated with the same lesion of the gastrointestinal tract and other organs. RESULTS: Our 3 patients suffered from cutaneous angioma and gastrointestinal hemangioma. In 39 patients reported in the literature, cutaneous angioma was observed in all of them, and gastrointestinal hemangioma in 31. Additionally, the lesions were also found in other organs such as the brain (7 patients), joint (2), liver (2), eye (1), kidney (1) and spleen (1). Cutaneous angioma was located on the surface of the skin, including body (93%), limbs (86%), hip (36%) and face (26%). Gastrointestinal hemangioma was more common in the small intestine (100%) than in the colon (74%) and stomach (26%). When the joint was involved by hemangioma, pathologic fracture or overgrowth of bone needed traction and amputation (1 patient respectively). For significant gastrointestinal bleeding, endoscopic techniques (8 patients), surgical excision (5), or both (1) were performed. Recurrent bleeding was successfully treated by endoscopic laser combined with steroid or interferon in one patient. CONCLUSIONS: BRBNS in children presents atypical symptom and systemic complications. It should be dealt with seriously if gastrointestinal bleeding or orthopedic complication occurs. Treatment includes conservative, endoscopic and surgical options. Its recurrence with new angioma in the gastrointestinal tract needs laser-steroid therapy.

[Correlation of intestinal mucosal injury with serum diamine oxidase].

OBJECTIVE: To study the relationship between the changes of intestinal mucosal tumor necrosis factor alpha (TNF-alpha), plasma diamine oxidase (DAO) values and the degree of mucosal injuries in young rat model of colitis and thereby to explore if plasma DAO could be used as a potential index for monitoring intestinal mucosal injury. METHODS: One hundred and four healthy young male Sprague-Dawley (SD) rats aged 5-6 weeks were randomly divided into three groups: zero time group (n = 8), model group (n = 48) and control group (n = 48). The model and control groups were further divided into 24 h, 72 h, 1 week, 2 weeks, 3 weeks and 4 weeks subgroups, respectively, with 8 rats in each. The rats in model group were given 2, 4, 6-trinitrobenzene sulfonic acid (TNBSA) via enema to induce colitis, while the rats in the control group were given normal saline (NS) solution in the same way and those in zero time group were not treated. TNF-alpha and DAO were measured by immunohistochemical technique and spectrophotometry. RESULTS: The most serious enteric mucosal injury was seen 24 hours after giving TNBSA. Plasma DAO and TNF-alpha decreased as the intestinal mucosal injury was alleviated. CONCLUSIONS: Plasma DAO values may be used as a marker for intestinal mucosal injury. TNF-alpha is a factor for causing mucosal injury. Young rat colitis model can be used to study intestinal mucosal injury.

[Long-term outcome after injection sclerotherapy for esophageal variceal bleeding in children with portal hypertension].

OBJECTIVE: Endoscopic sclerotherapy has emerged as an effective treatment for bleeding esophageal varices in adults and children but the long-term outcome is poorly defined in children. The present study aimed to study the long-term effect of endoscopic sclerotherapy in children with portal hypertension. METHODS: Fifteen patients (age 3 to 14 years) with esophageal variceal bleeding underwent endoscopic injection treatments with 1% Aethoxy-sclerol since 1996. All subjects continued to receive the therapy by repeated intra and extravariceal endoscopic sclerotherapy at intervals of 3 - 4 weeks until the varices disappeared, and received regular endoscopic follow-up. RESULTS: Fifteen patients had totally 43 injections, and were followed up from 40 to 86 months (mean 66 months) by endoscopy. Two patients received 2 injections and 5 received 3 before eradication of varices. The mean time needed for varices eradication was 3 to 6 months. Recurrence of varices and bleeding was seen in 3 patients who had duodenal ulcer. CONCLUSION: Endoscopic sclerotherapy is a safe and effective treatment for pediatric esophageal varices.

[Application of metal hemoclip for endoscopic polypectomy in children with thick-pedunculated intestinal polyps].

OBJECTIVE: With the development of endoscopic therapy in children, endoscopic electrocoagulation polypectomy had gradually replaced surgery and became an important method to resect gastrointestinal polyps in children. Simple electrocoagulation polypectomy could often bring some complications of gastrointestinal bleeding and perforation because of incomplete electrocoagulation or mechanical incision, especially in gastrointestinal thick-pedunculated polyps which always have thick nutrient blood vessel. Hemoclips can successfully interdict arteriovenous blood because it can clamp tissue firmly without causing necrosis around the target area. Based on its good mechanical hemostasis, hemoclips are not only widely used in treating bleeding like from ulcer, tumor and variceal ligation but also used in removal of thick-pedunculated gastrointestinal polyps in adults. This paper describes the application of endoscopic electrocoagulation with metal hemoclips to remove thick-pedunculated intestinal polpys in children for the first time, sums up the experience and evaluates its efficacy and safety. METHODS: Between October, 2001 and December, 2002, 5 cases with thick-pedunculated intestinal polpys were presented. The age of the patients ranged from 3 to 5 years. The clinical features were gastrointestinal bleeding or abdominal pain. The longest course of disease was 2 years. Enough preparations for alimentary tract were necessary for polypectomy. The procedures were performed under general anesthesia in order to avoid the risk of bleeding aspiration. Endoscopy was performed in the standard fashion. The apparatus included electronic colonic endoscope (XQ 200, Fuji Corp, Tokyo, Japan), snare (XQ200, Fuji Corp, Tokyo, Japan), impeller of the clip (HX-5QR-1) and hemoclip (MD850) which could be passed through the biopsy channel of endoscope. The clip was completely covered with a hood avoiding any injury to the mucous membrane. The pedicel with diameter of more than 1.0 cm underwent endoscopic electrocoagulation polypectomy with hemoclips. The clip contacted polyps in upright direction. One or more hemoclips were selected to clamp the proximal basement of the pedicel in terms of the pedicel diameter. Turning of the red colour of polyps to purple suggested that hemoclip interdicted arteriovenous blood effectively. The clip was then shut off and electrocoagulation polypectomy was followed. Six polyps were observed and removed. RESULTS: Six polyps including 2 transverse colon polyps and 4 descending colon polyps were resected. Pathological results showed that 3 were juvenile polyps and the other 3 adenomatous polyps. All the polyps were completely resected. The diameter of pedicel were 1.2 - 2.2 cm. The head and pedicel of the biggest polyp was about 5 cm x 5 cm and 2.2 cm, respectively, and five clips were used in order to remove it. No complications of bleeding and perforation were observed in these children. All hemoclips were expelled from intestines within one week. The symptoms of these patients disappeared. CONCLUSION: Mechanical hemostasis with hemoclips successfully interdicted arteriovenous blood of thick-pedunculated polyps. Hemoclips can successfully prevent the complications of bleeding and perforation. The clipping brings about a new method in endoscopic therapy. Endoscopic electrocoagulation polypectomy with hemoclips is a simple, safe and effective method to treat thick-pedunculated gastrointestinal polyps in children and it is a valuable tool in polypectomy for children.

Protection of gastric mucosa from ethanol induced injury by recombinant epidermal growth factor in rats.

AIM:To determine whether recombinant human epidermal growth factor (rhEGF) can protect gastric mucosa against ethanol induced injury in rats.METHOD: Fifty-four SD rats weighing 200g-500g each were divided into six groups after fasting for 24 hours.Three groups received different doses of oral rhEGF (30, 60 and 120&mgr;gcenter dotkg(-1)center dotd(-1)), one group was given cimetidine,one subcutaneous rhEGF (rhEGF IV) and one received saline as control.RESULTS:Acute gastric dilatation developed in the control and cimetidine groups and bloody gastric juice was found in the control group. The ulcer index was 58 in control group, 53 in rhEGF I, 46 in rhEGF II (P < 0.01), 11 in rhEGFIII (P < 0.01), 19 in rhEGF IV (P < 0.01), and 39 in cimetidine group (P < 0.05).CONCLUSION: rhEGF protected gastric mucosa against ethanol induced damage. The effect was dose-dependent with blood levels of epidermal growth factor (EGF) at a dosage range of 60&mgr;gcenter dotkg(-1)center dotd(-1)-120&mgr;gcenter dot kg(-1)center dotd(-1). It was more effective by injection than via oral route at the same dosage.