A Case of Partial Congenital Pericardial Defect Presenting as Acute Coronary Syndrome

Congenital pericardial defects are rare and asymptomatic for both partial and complete defects. However, some patients can experience syncope, arrhythmia, and chest pain. When a patient experiences a symptom, it may be caused by herniation and dynamic compression or torsion of a heart structure including the coronary arteries. Diagnosis of a congenital pericardial defect may be difficult, especially in old patients with concomitant coronary artery disease. The clinical importance of congenital pericardial defect has not been stressed and congenital pericardial defects are regarded as benign, but in this case, pericardial defect was responsible for myocardial ischemia. The authors report a case of partial congenital pericardial defect causing herniation and dynamic compression of the coronary arteries, presenting as an acute coronary syndrome in an old man, with an emphasis on the unique features of the coronary angiogram that support the diagnosis of partial pericardial defects.

Suppression of ROS generation by 4,4'-diaminodiphenylsulfone in non-phagocytic human diploid fibroblasts

The action mode of 4,4'-diaminodiphenylsulfone (DDS) is still under debate, although it has long been used in treatment of several dermatologic diseases including Hansen's disease. In this study, we tested the effect of DDS as an antioxidant on paraquat-induced oxidative stress in non-phagocytic human diploid fibroblasts (HDFs). Overall, preincubation of HDFs with DDS prevented the oxidative stress and the resulting cytotoxic damages caused by paraquat in these cells. The specific effects of DDS in paraquat-treated HDFs are summarized as follows: a) reducing the expression of NADPH oxidase 4 (NOX4) by inhibiting paraquat-induced activation of PKC; b) inhibiting paraquat-induced decreases in mitochondrial complex protein levels as well as in membrane potentials; c) consequently, inhibiting the generation of cytosolic and mitochondrial superoxide anions. Taken together, these findings suggest that DDS would suppress the radical generation in non-phagocytic HDFs during oxidative stress, and that DDS might have the extended potential to be used further in prevention of other oxidative stress-related pathologies.

No Associations between Schizophrenia and D22S280 Marker on Synapsin III Gene in Korean Males

OBJECTIVES: Synapsin III near VCFS region on chromosome 22q affects. It could be an interesting candidate gene for schizophrenia. D22S280 is a highly polymorphic genetic marker residing in synapsin III. We examined association of D22S280 marker on synapsin III with Korean patients with schizophrenia. METHODS: The subjects were 46 male Korean patients with schizophrenia and 60 male normal controls. Using polymerase chain reaction, gel electrophoresis, ABI 310 genetic analyzer, and GeneScan Collection 3.1 software, we confirmed genotypes of D22S280 marker. We examined Hardy-Weinberg equilibrium and case-control association using SAS/Genetic 9.1.3. RESULTS: Genotypes of both schizophrenia and control groups were in Hardy-Weinberg equilibrium. We could not find any significant statistical differences in allele-wise(chi-square=10.4, df=6, p=0.098) and genotype-wise (chi-square=22.1 df=19, p=0.258) analyses of D22S280 marker between schizophrenia and normal controls. Individual allele analyses with df=1 showed significant differences in A1(p=0.025) and A7(p=0.034) allele, which were not significant following Bonferroni corrections(A1 : p=0.177, A7 : p=0.235). CONCLUSION: We couldn't find any association between schizophrenia and the synapsin III gene. Given the small number of subjects studied, further investigations are needed.

Uncommon Complications Related to Pulmonary Artery Catheterization (CCOmbo Swan-Ganz catheter): Two case report / 대한마취과학회지

Case 1: A 30-year-old woman was scheduled for mitral valvuloplasty. A pulmonary artery (PA) catheter (Swan-Ganz CCOmbo model: 744HF75, Edwards(r), USA) was inserted via an introducer (8.5 Fr) placed in the right external jugular vein. The tip of the catheter could not be passed from the external jugular vein to the subclavian vein. When we attempted to advance the PA catheter again 30 minutes later, it could not be advanced or withdrawn. The PA catheter was removed with an introducer. The tip of the introducer had curled up into the intralummial space and the PA catheter had impacted in the narrowed lumen. Case 2: A 48-year-old woman was scheduled for tricuspid annuloplasty. A PA catheter was inserted via an introducer placed in the right internal jugular vein without problem. After opening the right atrium and withdrawing the PA catheter, a vinyl strip was found to have torn away from the distal part of the PA catheter.

A Cilinical Study of Diarrhea-Associated Benign Infantile Convulsion / 대한소아신경학회지

PURPOSE: The aim of this study is to reveal the detailed clinical features of diarrhea- associated benign infantile convulsion. METHODS: We studied 34 patients with diarrhea-associated benign infantile convulsion between March 2000 and February 2004. RESULTS: There were 34 patients with diarrhea-associated benign infantile convulsion : 19 boys and 15 girls. The age of the disease onset ranged from 3 to 34(mean; 18.7+/-6.6) months. The incidence was high from November to March. The types of the seizures were generalized tonic-clonic or generalized tonic in 32(94%) of 34 episodes, while the seizure types changed during episodes for 2 patients. The durations of seizures were from 3 sec to 10 min. 2 or more seizures occurred in 22(64.7%) episodes. A family history of febrile or afebrile convulsions was noted in 3 patients. There were no abnormalities in serum biochemistry tests. 12 out of 22 patients showed positive rotavirus antigen tests. Interictal EEG's were normal in 26 out of 30 episodes. CT or MRI demonstrated no neuroradiological abnormalities in 13 out of 14 patients. 4 patients experienced recurrence of diarrhea-associated benign infantile convulsion, but none had more than 2 episodes. Epilepsy developed in none of the patients during the follow-up period. CONCLUSION: Diarrhea-associated benign infantile convulsion is characterized by a cluster of seizures. A continous or intermittent antiepileptic treatment is not required because recurrence or later development of epilepsy is rare. Appropriate treatment for a cluster of seizures will be the subject of future studies.

A Case of Wandering Spleen / 대한소아소화기영양학회지

Wandering spleen is a rare condition characterized by migration of the spleen from its normal position due to laxity or absence of the supporting splenic ligaments. Ultrasonography shows the absence of the spleen in left upper quadrant (LUQ) and the presentation in the lower abdomen of a homognous mass whose sonographic appearance is consistent with that of a spleen. Contrast-enhanced CT reveals the absence of the spleen in LUQ, a soft tissue mass resembling spleen in the lower abdomen, and the splenic pedicle of whirled appearance. We present a case of wandering spleen, which could be diagnosed with US and CT by the ectopic location of spleen and the whirling pattern of splenic pedicle.

A Case of Membranoproliferative Glomerulonephritis Type II (Dense-Deposit Disease)

Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).

A Case of Membranoproliferative Glomerulonephritis Type II (Dense-Deposit Disease)

Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).

A Case of Secondary Renal Amyloidosis in Children with Juvenile Rheumatoid Arthritis

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain and characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.