α干扰素诱导治疗1例存在大片骨溶解和淋巴管血管瘤播散的患者获得完全缓解:Gorham-Stout综合征重症病例1例

The treatment of massive osteolysis with lymphangioma and/or hemangioma(Gorha m- Stout syndrome) has been controversial. The authors report on a patient with multiple massive osteolyses and extensive lymph- hemangiomatosis- whose lesions were reduced by interferon alfa therapy. A 2- year- old girl had complained of left chylothorax. Thoracoscopy showed an increase in small lym phatic vessels in the chest wall. The chylothorax was improved by coagulation of the lymphatic vessels. Later, multiple massive osteolyses appeared in the left 11th and 12th ribs, the TH10- L3 vertebrae, and the right femur. There were als o hemangiomas in the liver and spleen, a tumor lesion in the left lower chest wa ll, and hemangiomatous change on the skin surface of the left back. The left lun g had only a minimal air content. After OK- 432 was injected into the femur and chest wall lesions, the femur lesion disappeared. Then, as right chylothorax ap peared, OK- 432 was injected into the right pulmonary cavity. The chylothorax d isappeared, but pericardial effusion appeared. After steroid pulse therapy, peri cardial effusion disappeared. During these treatments, the 7th to 10th ribs disa ppeared from the x- ray and scoliosis developed. One month later, a cloudy flui d collection in the right lung was found on computed tomography. Interferon alfa and steroid pulse therapy were started. Interferon alfa (1,500,000 units) was s ubcutaneously administered daily for 2 months and was gradually reduced and main tained at 1,500,000 unit/wk. Steroids were also reduced and maintained at 5 mg/d of predonine. Later, the progress of osteolysis and the extension of lymph- he mangiomatosis stopped. Ten months later, hemangioma in the back disappeared, and the 7th to 10th ribs, which had disappeared, reappeared. The interferon alfa th erapy was stopped 14 months after it was administered. The patient’s condition has been stable for 10 months since then. At this time, computed tomography sho ws regression of the hemangiomatous lesion in the back. The authors clinically d iagnosed the patient as having Gorham- Stout syndrome with extension of lymph- hemangiomatosis. Interferon alfa with or without steroid therapy should be a choice for patients with extension lesions.

起源于肺动脉的室性心动过速的心电图及电生理特征

OBJECTIVES: We investigated the electrocardiographic(ECG) and electrophysiologic characteristics of ventricular tachycardia(VT) originating within the pulmonary artery(PA). BACKGROUND: Radiofrequency catheter ablation(RFCA) is routinely applied to the endocardial surface of the right ventricular outflow tract(RVOT) in patients with idiopathic VT of left bundle branch block morphology. It was recently reported that this arrhythmiamay originate within the PA.METHODS:Activation mapping and ECG analysis were performed in 24 patients whose VTs or ventricular premature contractions(VPCs) were successfully ablated within the PA(PA group) and in 48 patients whose VTs or VPCs were successfully ablated from the endocardial surface of the RVOT(RV-end-OT group). RESULTS: R-wave amplitudes on inferior ECG leads, aVL/aVR ratio of Q-wave amplitude, and R/S ratio on lead V2 were significantly larger in the PA group than in the RV-end-OT group. On intracardiac electrograms, atrial potentials were more frequently recorded in the PA group than in the RV-end-OT group(58%vs. 12%; p < 0.01). The amplitude of local ventricular potentials recorded during sinus rhythm within the PA was significantly lower than that recorded from the RV-end-OT(0.62±0.56 mV vs. 1.55±0.88 mV; p < 0.01). CONCLUSIONS: Ventricular tachycardia originating within the PA has different electrocardiographic and electrophysiologic characteristics from that originating from the RV-end-OT.When mapping the RVOT area, the catheter may be located within the PA if a low-voltage atrial or local ventricular potential of < 1-mV amplitude is recorded. Heightened attention must be paid if RFCA is required within the PA.

远端胃切除患者食管中胰岛素活性和胆汁酸含量

The pathogenesis of reflux esophagitis is not well understood and remains controversial.Distal gastrectomy serves as a model to assess the role of duodenal reflux with low gastric acidity in the development of reflux esophagitis.We investigated the relationship between the severity of esophagitis and gastroduodenal juice reflux,with particular focus on trypsin and bile acids after distal gastrectomy reconstructed with Billroth I anastomosis.Twenty-eight patients with gastroesophageal reflux disease after distal gastrectomy were enrolled.Esophageal and duodenal contents were aspirated under endoscopical examination,and their trypsin activity and bile acid concentrations were measured.The grade of reflux esophagitis was assessed by endoscopy and the symptoms were scored.Moreover,the grade of infiltration of inflammatory cells and the expression of COX-2 mRNA in the esophageal epithelium were evaluated.Patients with severe esophagitis had a higher amount of trypsin activity and bile acid concentrations in the esophagus,but not in the duodenum,compared to patients with mild esophagitis(P < 0.05) .There was a strong positive correlation between the trypsin activity and the bile acid concentrations in the esophagus(r = 0.743,P = 0.0001) .Moreover,the COX-2 mRNA expression and the grade of infiltrating inflammatory cells in the esophageal mucosa significantly correlated with the trypsin activity and bile acid concentrations in the esophagus.Thus,duodenogastroesophageal reflux with low gastric acidity is one of the pathogeneses in the development of reflux esophagitis from the present clinical study with patients after distal gastrectomy reconstructed with Billroth I anastomosis.