22q11.2 deletion syndrome (22qDS) resulting from a microdeletion of 22q11.2, is usually diagnosed in the postnatal period, and generally manifests as various combinations of cardiac defects, hypoparathyroidism, facial dysmorphism, palate deformity and cellular immunodeficiency. We report a case of a 32-year-old woman presenting with seizures and hypocalcemia, who was diagnosed with 22qDS, along with a literature review of adult cases. Physicians should recognize the 22qDS in adults presenting with any combinations of hypocalcemia, hypothyroidism, cardiac defects and psychiatric disorders. Pathognomonic facial dysmorphism or short stature can be the key to diagnosis.

To investigate the effect of teeth clenching on isokinetic knee extension at various velocities, isokinetic muscle strength during knee extension was measured in association with teeth clenching at 30, 60, 150, 300 and 450 degrees per second (deg/s) using the Cybex 6000 isokinetic dynamometer. The volunteer subjects were 9 healthy males (26.2±0.97 years) . The peak torque per body weight and average power per body weight were statistically analyzed. Our results demonstrated that the peak torque per body weight with teeth clenching at 30, 60 and 150 deg/s significantly increased by 7.0%, 7.4% and 4.9%, respectively (p<0.05), but no significant differences were found at 300 and 450 deg/s. While the average power per body weight with teeth clenching at 30, 60 and 150 deg/s significantly increased by 6.5%, 6.1% and 6.9%, respectively (p<0.05), no sig-nificant differences were found at 300 and 450 deg/s. A significant negative correlation was shown between the isokinetic angular velocity and the difference in peak torque per body weight derived from with and without teeth clenching (r=-0.699; p<0.05) . These findings suggested that the effect of teeth clenching on isokinetic muscle strength of knee extension was dependent on the angular velocity, and at lower angular velocities teeth clenching had the effect of increasing the isokinetic muscle strength during knee extension.

The purpose of this study was to investigate the effect of teeth clenching on isokinetic knee extension force during repeated voluntary contractions. We assessed isokinetic muscle strength in association with teeth clenching during 100 consecutive knee extensions at 60 degrees per second (deg/s) using a Cybex 6000 isokinetic dynamometer. In this study, 8 healthy male volunteers (28.4 ± 3.89 years) were asked to perform isokinetic contractions in an extended cycle of five contractions without teeth clenching followed by five contractions with the teeth clenching. The peak torque per body weight was statistically analyzed. In our results, the peak torque per body weight with teeth clenching were significantly greater than those without teeth clenching in the first 70 cycles; however, no significant differences were shown at 71-100 cycles. There was a significant negative correlation between the number of knee extensions and the difference in peak torque per body weight derived from with and without teeth clenching (r=-0.475, p<0.0001) . Our findings reveal that the effect of teeth clenching on the isokinetic muscle strength of knee extension is dependent on muscular fatigue, and that the improving effect of teeth clenching on isokinetic muscle strength declines with increasing muscular fatigue.

Previous research has demonstrated that the concentric muscle strength of knee extension exerted at slow to medium angular velocities is increased by teeth clenching. This study evaluated the isokinetic eccentric strength of knee extensors at 4 angular velocities (60, 120, 180 and 240 degrees per second) with and without teeth clenching on the Cybex 6000 Extremity Testing and Rehabilitation System. Twelve healthy adult male (26.8±1.8 years) volunteered for the study. Peak torque per body weight and average power per body weight were analyzed. The results demonstrated that peak torque per body weight accompanied by teeth clenching was significantly increased at 60, 120, 180 and 240 deg/s by 9.5%, 8.2%, 8.2% and 9.8%, respectively (p<0.01) . Similarly average power per body weight during teeth clenching was significantly higher at 60, 120, 180 and 240 deg/s by 9.1%, 9.5%, 9.4% and 7.7%, respectively (p<0.05) . These findings suggest that teeth clenching is a factor that leads to augmented eccentric strength of knee extensors. Its effect appears to be independent of angular velocity.

We report a rare case of primary cardiac lymphoma in the right atrium. An 85-year-old woman with severe heart failure was referred to our hospital. The echocardiography revealed a huge tumor occupying the right atrial cavity. We conducted an emergency operation to resect the tumor. However, as the tumor strongly adhered to the wall of the right atrium and tricuspid valve, we performed partial resection of the tumor to improve hemodynamics. The pathological examination of the tumor was consistent with malignant lymphoma of B-cell origin. Although the postoperative chemotherapy was effective to reduce a volume of the tumor, the patient died because of the adverse reaction to medication.

We studied 73 patients, 70 years of age or older, who underwent aortic valve replacement for aortic stenosis between October, 1990 and October, 2004. There were 31 men and 42 women with a mean age of 75.7±3.6 years. Mechanical valves were implanted in 37 patients, and bioprostheses in 36 patients. Operative mortality was 1 of 73 (1.4%) and the New York Heart Association functional class improved to class I or class II in all of the hospital survivors. Follow-up (100%) extended from 0.3 to 11.6 years (mean 3.7 years). There were 16 late deaths (5.9% per patient-year), including valve-related deaths in 6 patients. The overall survival rates at 5 and 10 years was 74.2% and 44.3%, respectively. The freedom from valve-related events at 5 and 10 years was 78.8% and 78.8%, respectively. The 10-year survival rates and freedom from valve-related events were not different between the patients with mechanical valves and those with bioprostheses. The size of the implanted valve did not influence the late survival or freedom from valve-related events. The outcome after aortic valve replacement in the elderly (70 years and older) was excellent with low operative mortality, and acceptable late mortality and morbidity. Thus, aortic valve replacement for elderly patients should have the same indications as for younger patients. Bioprostheses showed good long-term results with no structural valve deterioration, thromboembolism, or bleeding events. Mechanical valves, which required the maintenance of an anticoagulant therapy, were also useful with acceptable late morbidity. The long-term results with small valves (≤19mm) were comparable to the results with large valves (>19mm) in the elderly. Thus, the use of these small valves in this particular age group seems to be acceptable.

BACKGROUND/AIMS: To determine the nutritional markers important for assessing the degree of pancreatic insufficiency due to chronic pancreatitis in routine clinical practice. METHODS: A total of 137 patients with chronic pancreatitis were followed up for more than 1 year. They were divided into two groups: a pancreatic diabetes mellitus (DM) group, consisting of 47 patients undergoing medical treatment for DM of pancreatic origin, and a nonpancreatic DM group, consisting of 90 other patients (including 86 patients without DM). Serum albumin, prealbumin, total cholesterol, cholinesterase, magnesium, and hemoglobin were compared between the two groups. RESULTS: The total cholesterol was significantly lower in the pancreatic than the nonpancreatic DM group (164 mg/dL vs 183 mg/dL, respectively; p=0.0028). Cholinesterase was significantly lower in the former group (263 U/L vs 291 U/L, respectively; p=0.016). Among the 37 patients with nonalcoholic pancreatitis, there was no difference in the cholinesterase levels between the pancreatic and nonpancreatic (296 U/L vs 304 U/L, respectively; p=0.752) DM groups, although cholesterol levels remained lower in the former (165 mg/dL vs 187 mg/dL, respectively; p=0.052). CONCLUSIONS: Cholinesterase levels are possibly affected by concomitant alcoholic liver injury. The total cholesterol level should be considered when assessing pancreatic insufficiency due to chronic pancreatitis.
Adult ; Aged ; Aged, 80 and over ; Cholesterol/*blood ; Cholinesterases/blood ; Diabetes Mellitus, Type 2/complications ; Exocrine Pancreatic Insufficiency/*blood/etiology ; Female ; Follow-Up Studies ; Humans ; Liver Cirrhosis, Alcoholic/blood ; Male ; Middle Aged ; Nutritional Status ; Pancreas/enzymology ; Pancreatitis, Alcoholic/blood/complications ; Pancreatitis, Chronic/blood/*complications ; Serum Albumin/analysis

Introduction : Prevention of embolic stroke is the key issue to perform aortic arch replacement in patients with a shaggy aorta. The aim of this study is to report the utility of the isolation technique for total arch replacement in patients with a shaggy aorta. Methods : Clinical results of seven patients (71.7 years old, all men) with a shaggy aorta who underwent total arch replacement between January 2017 and November 2018 were retrospectively reviewed. The operative indications were a distal arch or proximal descending aortic aneurysm in 6 patients and a thrombus inside brachiocephalic artery in one. A cerebral perfusion was established by inserting a cannula directly into all supra-aortic branches before starting systemic perfusion. Result : Utilizing the isolation technique with clamping of all branches in 4 patients and the functional isolation technique with clamping of two branches in 3, total arch replacement was performed in all patients (operation time : 513 min, selective cerebral perfusion time : 162 min). No operative death was observed and no newly developed stroke was encountered. Conclusion : The isolation technique is a useful method to prevent stroke during total arch replacement in patients with a shaggy aorta.

We present a 62-year-old woman who was diagnosed with primary pulmonary arterial sarcoma with pulmonary hypertension. CT showed a large defect inside both main pulmonary arteries in accordance with the accumulation of FDG-PET. To relieve the symptom and to prevent sudden death, removal of a massive pulmonary tumor and postoperative chemotherapy were planned. Utilizing the intermittent systemic circulatory arrest under deep hypothermia (18°C), the pulmonary artery trunk and both main pulmonary arteries were opened. The tumor stacking inside the pulmonary artery was removed and its origin at the commissure of the pulmonary artery valve was resected. The defect was repaired with a pulmonary valve replacement. Histopathological examination revealed high grade sarcoma. Her postoperative course was uneventful ; however, she died of cerebral hemorrhage during chemotherapy six months after surgery.