Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

ANALYSIS OF BURIED BUMPER SYNDROME AFTER PERCUTANEOUS ENDOSCOPIC GASTROSTOMY DUE TO USE OF A BUTTON‐TYPE KIT

Background: Buried bumper syndrome (BBS) is a rare complication of percutaneous endoscopic gastrostomy (PEG). Along with the widespread use of the button‐type kit, BBS is encountered frequently. Methods: In the present study, we examined causes and treatments for BBS among 1400 patients who had undergone PEG. Results: The causes of BBS after PEG were classified into two categories: early causes consisted of wound infection, inappropriate size of kit and severe lordosis, while late causes were inappropriate exchange of kit, rough management or weight gain. The treatments for BBS could be determined by the degree of wound infection, fistula and burial of the bumper. Conclusion: We prepared a flowchart for replacement, by which BBS can be managed safely and quickly without surgical or endoscopic intervention.     

Anterior spinal artery syndrome after minimally invasive direct coronary artery bypass grafting under general combined epidural anesthesia

A 65-year-old patient with ischemic heart disease and severe diabetes mellitus underwent minimally invasive direct coronary artery bypass grafting under general combined with epidural anesthesia. Paraplegia developed after surgery and the diagnosis of anterior spinal artery syndrome was made based on the patient s neurological condition and magnetic resonance imaging findings. Paraplegia following epidural anesthesia is a rare but recognized complication and this complication should be taken into account, especially in patients at risk, when considering epidural analgesia techniques in the minimally invasive cardiac surgery.     

Association of the GNAS1 gene variant with hypertension is dependent on alcohol consumption.

The beta-adrenoceptor (beta-AR)-stimulatory guanine nucleotide-binding (Gs) protein system has been shown to play important roles in the cardiovascular system. The gene encoding the alpha-subunit of Gs proteins (GNAS1) is a candidate genetic determinant for hypertension. Because alcohol consumption is known to affect blood pressure partly through the beta-AR-Gs protein system, we examined the possible interaction between GNAS1 T393C polymorphism and drinking status in the association with hypertension in the present study. As a result, a non-significant but reasonable trend supporting the presence of an interaction was shown (p = 0.076). In line with this trend, the T393C polymorphism significantly interacted with drinking status in the association with systolic blood pressure (p = 0.028). Moreover, supporting the presence of an interaction, T allele carriers consistently had a higher probability of hypertension, higher systolic blood pressure, and higher diastolic blood pressure than CC homozygotes in non-drinkers and light drinkers. In contrast, CC homozygotes consistently had a higher probability of hypertension, higher systolic blood pressure, and higher diastolic blood pressure than T allele carriers in moderate to heavy drinkers. The present study also showed a significant interaction between the T393C polymorphism and drinking status in the association with pulse pressure (p = 0.026), reflected by a significant association between the T393C polymorphism and pulse pressure in moderate to heavy drinkers (p = 0.026). These findings may be helpful in conducting further molecular and biological studies on the relationship among the effects of alcohol, the beta-AR-Gs protein system, and hypertension.     

Surgical treatment of aortic coarctation associated with multi-vessel brachiocephalic involvement in takayasu's arteritis.

In Takayasu's arteritis (TA), both atypical coarctation (CO) and brachiocephalic involvement are common features that occasionally require operative correction. A combination of these abnormalities could duplicate underlying illness in patients, posing an increased risk of operative morbidity. We present, herein, two TA patients in which hypertensive heart disease secondary to CO was surgically corrected. Both patients had multi-vessel brachiocephalic disease. One patient who showed occlusion of all brachiocephalic arteries underwent aorto-aortic bypass, while another with two-vessel lesion underwent axillo-bifemoral bypass grafting. Subclavian reconstruction was supplemental to each procedure, resulting in relief of neurologic stigmata. Strategies to avoid intraoperative cerebral ischemia played an important role in the surgical repair of such TA-related extensive vascular lesions.     

Combined aortic valve replacement and coronary artery bypass grafting with in situ arterial grafts for porcelain aorta.

Patients with porcelain aorta carry a high risk of cerebral as well as systemic embolism during cardiac surgery. Here we describe a case of severe aortic stenosis and coronary artery disease combined with the circumferentially calcified aorta. The patient was a 77-year-old man who successfully received four coronary artery bypass grafts with in situ arterial grafts without clamping the aorta and aortic valve replacement. Aortic valve replacement and two distal coronary artery anastomoses to the left circumflex artery and obtuse marginal branch were performed under cardiac arrest during hypothermic perfusion with endoaortic balloon occlusion, followed by partial endarterectomy and closure of the aorta buttressed with bovine pericardium under deep hypothermic circulatory arrest. While rewarming, the other two distal coronary anastomoses to the left anterior descending artery and diagonal branch were done on the beating heart in order to minimize cardiac arrest time. On-pump beating heart coronary artery bypass grafting (CABG) can be useful especially for combined complex cardiac surgery.