Clinical course of cardiovascular involvement in the mucocutaneous lymph node syndrome: Relation between clinical signs of carditis and development of coronary arterial aneurysm

Seventy-nine patients with mucocutaneous lymph node syndrome were evaluated prospectively by clinical examination, electrocardiography, chest radiography, M mode and two dimensional echocardiography and thallium-201 myocardial scanning. Serial changes were categorized according to the duration of illness: stage I (1 to 10 days), stage II (11 to 20 days), stage III (21 to 30 days), stage IV (31 to 60 days) and stage V (61 days to 40 months). The presence of myocarditis in stages I and II was suggested in 40 of 79 patients (50.6 percent) by electrocardiographic, echocardiographic, radiographic and clinical abnormalities. Myocarditis was accompanied by pericarditis in six patients and by both endocarditis and pericarditis in one patient. These signs of inflammation were resolved by stage III in all but three patients with electrocardiographic abnormalities. In the active stage, large coronary arterial lesions were suspected only because of an abnormal spherical echo-free space in the region of the coronary arteries on two dimensional echocardiograph as well as electrocardiographic evidence of deep Q waves in leads II, III and aVF. One or more coronary aneurysms developed in 11 patients, primarily in stage II; regression of the aneurysm was noted in 5 of these patients during stages III, IV and V. Aneurysm regression demonstrated by angiography did not correlate with echocardiographic changes in aneurysm size in one patient. Moreover, the occurrence of coronary aneurysm did not correlate with the presence of signs of carditis, because the frequency of carditis was the same in patients with and without aneurysm.     

A giant rheumatoid nodule

Subcutaneous rheumatoid nodules occur commonly in advanced cases of rheumatoid arthritis and are the most common extra-articular lesion of this disease. We present a case of a very unusual giant rheumatoid nodule that developed on the lateral side of a knee. The case was devoid of systemic symptoms of arthritis and the lesion was limited to a rheumatoid nodule. The nodule was successfully treated by surgical excision. However, other new nodules developed in her hand. Her clinical course has not been satisfactory.     

Noninvasive visualization of coronary arterial aneurysm in infants and young children with mucocutaneous lymph node syndrome with two dimensional echocardiography

Real time two dimensional echocardiographic studies of the main coronary arteries were performed in 30 normal subjects and 58 patients with the mucocutaneous lymph node syndrome. Four echocardiographic coronary arterial patterns were observed: linear, dilated, fusiform or spherical. In normal subjects the left main coronary artery generally had a linear pattern but had a dilated pattern in two patients. The right coronary artery could not be visualized clearly. In 7 of the 58 patients with the mucocutaneous lymph node syndrome, a portion of the left main coronary artery had a fusiform or spherical pattern, or both, and the stem of the right coronary artery also had a spherical pattern in 2 patients. In all five patients who had coronary angiography, the procedure confirmed an aneurysm in the left main coronary artery or its branching point and the stem of the left anterior descending coronary artery or the stem of the right coronary artery, or both. In three of the five patients, coronary arterial aneurysms were predicted before coronary angiography was performed. Thus, two dimensional echocardiography is a reliable noninvasive method for evaluating coronary arterial aneurysms in infants and young children.     

Endoscopic papillary balloon dilation for treatment of common bile duct stones

BACKGROUND/AIMS: Use of endoscopic papillary balloon dilation (EPBD) for the treatment of common bile duct stones has increased in recent years, owing to its simplicity and its advantage of preserving sphincter function. It has been reported that EPBD is associated with a lower risk of bleeding, but a higher risk of pancreatitis than endoscopic sphincterotomy. However, there have been few reports on studies of post-EPBD pancreatitis. This report concerns the use of EPBD at our department for the treatment of common bile duct stones and early postoperative complications, with a focus on pancreatitis. METHODOLOGY: The study was conducted in 63 patients with choledocholithiasis, including 4 patients with cirrhosis and 21 patients with periampullary diverticula. The stones were extracted after EPBD conducted with an 8-mm dilatation balloon. RESULTS: Complete removal of stones was achieved in 53 out of 63 patients (84.1%). Pancreatitis meeting the criteria of Cotton et al. occurred in 7 of the 63 patients (11.1%), while 12 patients (19.5%) were affected when milder cases of pancreatitis were included. Severe pancreatitis occurred in 1 patient only. Cholangitis occurred in 3 patients (4.8%) and basket impaction occurred in 1 patient (1.6%), but no serious complications such as bleeding or perforation were encountered. CONCLUSIONS: These results suggest that EPBD is an effective procedure for the treatment of common bile duct stones, with a low risk of serious complications.     

Novel extracorporeal granulocyte apheresis column inhibits myocardial reperfusion injury: future clinical applications

OBJECTIVES: Leukocyte infiltration is very important in myocardial ischemia/reperfusion injury. A new extracorporeal circulation therapy using a granulocyte-apheresis column (G-1) has recently been proved to be effective and safe in patients with rheumatoid arthritis and with ulcerative colitis. This study investigated whether this therapy would reduce myocardial ischemia/reperfusion injury. METHODS: Rabbits were subjected to 30 min ischemia followed by 180 min reperfusion with no treatment (control group: n = 12); or treated with extracorporeal blood circulation (2 ml/min) passing through the G-1 (G-1 group: n = 12), or sham column (sham group: n = 12). Infarct size was determined by tetrazolium staining and expressed as a percentage of the area at risk. RESULTS: Infarct size in the G-1 group (15.9 +/- 3.4%) was significantly (p < 0.01) smaller than in the control (34.7 +/- 3.9%) and sham (35.2 +/- 4.1%) groups. Granulocyte emigration into the ischemic myocardium assessed by histopathological score was significantly (p < 0.01) less in the G-1 group (0.58 +/- 0.17) than in the sham group (1.42 +/- 0.26). There was a significant (r = 0.78, p < 0.0001) positive correlation between this score and infarct size. Flow cytometry showed alterations in the adhesion molecule expression on granulocytes during the passage through the G-1, but not sham, columns as a low L-selectin and high Mac-1 form, which is associated with the inability to home to the inflamed site. CONCLUSIONS: Extracorporeal circulation with the G-1 column, a newly-developed clinically applicable therapy, appears to reduce infarct size at least in part by limiting the emigration capacity of granulocytes into the ischemic myocardium.     

Responsiveness of the ductus arteriosus to prostaglandin E1 assessed by combined cross sectional and pulsed Doppler echocardiography.

Cross sectional echocardiography combined with Doppler echocardiography was used to record either ductal morphology or the flow profile within the ductus arteriosus before and after infusion of prostaglandin E1 in 25 newborn infants with cyanotic and acyanotic congenital heart disease with ductus dependent blood flow. The ultrasound results were compared with changes in arterial oxygen tension and the overall clinical response to prostaglandin E1 seen during the same period in 24 of the 25 patients in whom the degree of ductal narrowing could be determined with the ultrasound method. At the time of the study, the ductus was widely patent or slightly narrowed in 12 patients and was closed in two patients. These patients did not respond to prostaglandin E1. There was prominent localised narrowing of the ductus in seven patients and generalised narrowing in three. After the infusion of prostaglandin E1 there was no ductal narrowing in these patients, except for one patient who had slight residual localised narrowing. There was also a considerable change in the ductal flow profiles in each patient. In these 10 patients infusion of prostaglandin E1 resulted in an increase in arterial oxygen tension, clinical improvement, or both. The present study indicates that prostaglandin E1 is effective in patients with prominent narrowing of the ductus but is not in patients in whom the ductus is widely patent or closed. Cross sectional echocardiography combined with Doppler echocardiography was useful for predicting the responsiveness of the ductus arteriosus to the infusion.     

Use of an ultrasonic surgical aspirator to expose buried coronary arteries

In coronary artery bypass grafting, the target arteries for grafting can be buried in thick adipose tissue or myocardium, and exposure may be difficult. We used an ultrasonic surgical aspirator to expose buried coronary arteries. Fat tissue or myocardium covering coronary arteries was removed, and target arteries were exposed with little bleeding. No arrhythmia or coronary spastic event occurred during its application, and bypass grafting could be completed. In postoperative angiography, no deformity or shrinkage of coronary arteries was observed at the sites at which the ultrasonic aspirator was applied.     

Patient with adult‐onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation

We report a 51‐year‐old female patient with adult‐onset type II citrullinemia (CTLN2) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 years, paying special attention to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of CTLN2 was made by DNA analysis of the SLC25A13 gene and treatment with conservative therapies was begun, including a low‐carbohydrate diet and supplementation with arginine and sodium pyruvate. However, despite these treatments, frequent attacks of encephalopathy occurred with markedly elevated plasma ammonia levels. While we were apprehensive regarding the risk of recurrence of somatostatinoma due to immunosuppressive therapy after liver transplantation, the patient was in a critical condition with CTLN2 and it was decided to perform living‐donor liver transplantation using a graft obtained from her son. Her postoperative clinical course was uneventful and she has had an active life without recurrence of somatostatinoma for 2 years. This is the first case of CTLN2 with somatostatinoma. As the condition of CTLN2 patients with rapidly progressive courses is often intractable by conservative therapies alone, liver transplantation should be considered even after surgery for malignant tumors in cases with neither metastasis nor recurrence.     

Responsiveness of the ductus arteriosus to prostaglandin E1 assessed by combined cross sectional and pulsed Doppler echocardiography

Cross sectional echocardiography combined with Doppler echocardiography was used to record either ductal morphology or the flow profile within the ductus arteriosus before and after infusion of prostaglandin E1 in 25 newborn infants with cyanotic and acyanotic congenital heart disease with ductus dependent blood flow. The ultrasound results were compared with changes in arterial oxygen tension and the overall clinical response to prostaglandin E1 seen during the same period in 24 of the 25 patients in whom the degree of ductal narrowing could be determined with the ultrasound method. At the time of the study, the ductus was widely patent or slightly narrowed in 12 patients and was closed in two patients. These patients did not respond to prostaglandin E1. There was prominent localised narrowing of the ductus in seven patients and generalised narrowing in three. After the infusion of prostaglandin E1 there was no ductal narrowing in these patients, except for one patient who had slight residual localised narrowing. There was also a considerable change in the ductal flow profiles in each patient. In these 10 patients infusion of prostaglandin E1 resulted in an increase in arterial oxygen tension, clinical improvement, or both. The present study indicates that prostaglandin E1 is effective in patients with prominent narrowing of the ductus but is not in patients in whom the ductus is widely patent or closed. Cross sectional echocardiography combined with Doppler echocardiography was useful for predicting the responsiveness of the ductus arteriosus to the infusion.