No abstract available.
Lymphoma*

Infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring abdominal surgery in early infancy, and is caused by hypertrophied pyloric muscle. The development of successful surgical treatment in the early 1900s by Fredet and Ramstedt made it possible for infants worldwide to survive. Modern pediatric anesthetic techniques have virtually eliminated mortality from surgical management. Atropine sulfate is a cholinergic blocking agent with potent antimuscarinic activity that decreases peristaltic contractions by relaxing smooth muscles. We treated two cases of IHPS with incomplete pyloromyotomy in 3-month-old and 5-month-old male infants by administering atropine sulfate intravenously. They were free from vomiting after 5 days of intravenous atropine sulfate treatment. In these rare cases of persistent vomiting or refractory emesis following incomplete pyloromyotomy, there may be a role for atropine sulfate.
Atropine* ; Constriction, Pathologic* ; Humans ; Infant ; Male ; Mortality ; Muscle, Smooth ; Pyloric Stenosis, Hypertrophic ; Vomiting*

BACKGROUND: The ubiquitin-proteasome system (UPS) is an important pathway of proteolysis in pathologic hypertrophic cardiomyocytes. We hypothesize that MG132, a proteasome inhibitor, might prevent hypertrophic cardiomyopathy (CMP) by blocking the UPS. Nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) and androgen receptor (AR) have been reported to be mediators of CMP and heart failure. This study drew upon pathophysiologic studies and the analysis of NF-κB and AR to assess the cardioprotective effects of MG132 in a left ventricular hypertrophy (LVH) rat model. METHODS: We constructed a transverse aortic constriction (TAC)-induced LVH rat model with 3 groups: sham (TAC-sham, n=10), control (TAC-cont, n=10), and MG132 administration (TAC-MG132, n=10). MG-132 (0.1 mg/kg) was injected for 4 weeks in the TAC-MG132 group. Pathophysiologic evaluations were performed and the expression of AR and NF-κB was measured in the left ventricle. RESULTS: Fibrosis was prevalent in the pathologic examination of the TAC-cont model, and it was reduced in the TAC-MG132 group, although not significantly. Less expression of AR, but not NF-κB, was found in the TAC-MG132 group than in the TAC-cont group (p<0.05). CONCLUSION: MG-132 was found to suppress AR in the TAC-CMP model by blocking the UPS, which reduced fibrosis. However, NF-κB expression levels were not related to UPS function.
Animals ; Animals* ; B-Lymphocytes ; Cardiomyopathy, Hypertrophic ; Constriction ; Fibrosis ; Heart Failure ; Heart Ventricles ; Hypertrophy* ; Hypertrophy, Left Ventricular ; Models, Animal* ; Myocytes, Cardiac* ; NF-kappa B ; Proteasome Endopeptidase Complex* ; Proteasome Inhibitors* ; Proteolysis ; Rats* ; Receptors, Androgen ; Ubiquitins

BACKGROUND: The prostaglandin E1(PGE1) is a well known protent dilator of arteriosus. Maintaining of the patency of ductus arteriosus is crucial for the survival of patients suffering from ductus-dependent cyanotic congenital heart disease. We aimed to analyse the efficacy and the influencing factors upon PGE1 in patients suffering from this disease. METHODS: Between May 1991 and April 1993, 26 neonates and infants with ductus- dependent cyanotic congenital heart disease received on intravenous infusion of PGE1 in the Division of Pediatric Cardiology. Yonsei Cardiovascular Center. The result was a dramatic improvement in systemic arterial oxygen tension and oxygen saturation during infusion of PGE1with a dependency on the infusion of PGE1. We evaluated the arterial blood gas analysis both at the immediate pre-infusion stage and 2 hours after infusion. We aimed to analyse the factors which may influence the intravenous of PGE1to infant suffers of ducts-dependent cyanotic congenital heart disease, such as pulmonary atresia(n=14), severe pulmonary stenosis(n=7) or complete transposition of the great arteries(n=5). RESULTS: 1) There was a significant increase in PaO2 and Oxygen saturation 2 hours after the infusion of PGE1. This appeared to be unrelated to the different forms of the disease when compared with the pre-infusion values. 2) The infants' responsiveness of the ductus arteriosus appeared to be age related with significant differences emerging between the 2 group(p<.05). In infants younger than 9 hours old, the differences in PaO2 changes between pre-infusion and post-infusion of PGE1 were 16.3+/-3.7mmHg compared to just 10.4+/-0.4mmHg in infants older than 96 hours. 3) No significant difference emerged between an increase in PaO2or oxygen saturation relating to the shape of ductus arteriosus ; or the level of PaO2prior to the infusion. 4) The side effects of PGE1were as follows ; fever(84.6%),loose stool(61.5%), apnea(30.8%) and hypotension(15.4%), etc.. CONCLUSION: PGE1provides excellent medical palliation for infants suffering from ductus-dependent cyanotic congenital heart disease until the pulmonary arteries are large enough for a modified Blalock-Taussig shunt ; or until corrective surgery is possible.
Alprostadil ; Blalock-Taussig Procedure ; Blood Gas Analysis ; Cardiology ; Ductus Arteriosus ; Heart Defects, Congenital* ; Humans ; Infant* ; Infant, Newborn ; Infusions, Intravenous ; Oxygen ; Pulmonary Artery

No abstract available.

Annular pustular psoriasis is a rare variant of the generalized pustular psoriasis characterized by annular lesions with rapidly evolving pustules, associated mild toxicity, and rapid response to therapy. We present a case of annular pustular psoriasis in a 17-year-old male, in whom no previous history of psoriasis was found. The skin lesions were characterized clinically by annular to gyrate erythematous lesions with pinpoint pustules near the periphery on both shoulders, upper arms, and back areas. The histology showed large intraepidermal spongiform pustules. Most of the skin lesions disappeared within 1 week with topical corticosteroid cream.

Systemic lupus erythematous (SLE) is systemic autoimmune disease of unknown etiology. SLE involve all part of heart but clinically important myocarditis is an unusual feature and is potentially fatal complication of SLE. We describe the woman who had diagnosed as SLE, 5 years ago and in that time, she had sinus tachycardia, mild dyspnea, chest discomfort, and depressed left ventricular function. She was diagnosed to myocarditis clinically and was treated by high-dose corticosteroids of intravenous pulse methylprednisolone. After treatment, she improved and showed improvement of left ventricular function. However, a sudden pneumomediastinum was occurred without trauma, and she died. The cause of pneumomediastinum was unexplained. We report a sudden pneumomediastinum in the course of lupus myocarditis presenting as sinus tachycardia with review of literature.
Female ; Humans

We operated on a 41-year-old man using venous bypass shunt for superior vena cava (SVC) syndrome caused by mediastinal fibrosis. The patient had substantially high venous pressure and high risk of postoperative neurologic deficits. The collateral veins were deemed to be interrupted during the surgical reconstruction of SVC. Treatment included resection of the obstructed SVC and innominate vein and reconstruction with an autologous pericardial tube graft. During the operation, venous drainage from upper body was maintained with an extraluminal bypass shunt. The shunt was effective at prompt relief of venous hypertension, eliminating the time constraints, and preventing the postoperative complications.
Adult ; Brachiocephalic Veins ; Drainage ; Fibrosis ; Humans ; Hypertension ; Neurologic Manifestations ; Pericardium ; Postoperative Complications ; Superior Vena Cava Syndrome ; Transplants ; Veins ; Vena Cava, Superior* ; Venous Pressure

BACKGROUND: To date, many methods have been proposed to resolve the sunken eyelid. We treated our clinical cases of sunken upper eyelid based on whether there is a concurrent presence of the dermatochalasis or blepharoptosis and then performed the autologous fat grafting using either closed or open technique. METHODS: According to the classification of the patients, we used the following injection techniques: First, we solely performed the autologous fat grafting using the closed technique in the sunken upper eyelid only group, for which we did the injection using a micro-injector cannula on the suborbicularis plane. Second, we performed such procedures as skin excision, orbicularis oculi muscle strip excision and levator aponeurosis advancement to manage each symptom in the sunken upper eyelid with dermatochalasis or blepharoptosis group. Simultaneously, we also made a small window in the lateral portion of the orbital septum using the open technique with metzenbaum scissors. Thus, we performed the intraseptal injection of the autologous fat. RESULTS: During a period ranging from January of 2012 to April of 2014, we treated a total of 53 patients. The patients were followed up during a mean period of four months. Thus, we obtained satisfactory treatment outcomes without notable complications. CONCLUSIONS: In conclusion, our results indicate not only that surgeons should consider the fat grafting as one of eyelid-surgery procedures but also that they should perform it concurrently with blepharoplasty or blepharoptosis correction for the purposes of obtaining good treatment outcomes.
Blepharoplasty ; Blepharoptosis* ; Catheters ; Classification ; Eyelids* ; Humans ; Orbit ; Skin ; Transplants*

Acute pancreatitis is an uncommon complication of CAPD. The clinical diagnosis of acute pancreatitis is difficult, since the presenting features are similar to those of dialysis associated peritonitis, and a high index of suspicion is necessary. Serum amylase values three times greater than the upper limit of normal and effluent amylase greater than 100U/L are helpful in diagnosing acute pancreatitis in CAPD patients. We report a case of pancreatitis in a patient on CAPD in whom diagnosis was made by serum amylase, effluent amylase, abdominal sonogram.
Amylases ; Diagnosis ; Dialysis ; Humans ; Pancreatitis* ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis