No abstract available.
Aorta, Thoracic* ; Child* ; Echocardiography, Doppler* ; Humans

Williams syndrome is a progressive and multisystemic disorder. We experienced one case of Willams syndrome which was characterized by elfin facies, mental retardation, diffuse aortic hypoplasia, mitral regurgitation and chronic nonparoxysmal sinus tachycardia. The tachycardia has been managing with beta-blocker successfully. We presented a case of unusual form of Williams syndrome with a reivew of literatures.
Facies ; Intellectual Disability ; Mitral Valve Insufficiency ; Tachycardia ; Tachycardia, Sinus ; Williams Syndrome*

Two-dimensional Doppler echocardiographic velocity profiles of four cardiac valves in a group of 85 infants and children with left to right shunt lesions(VSD, ASD and PDA) are presented. Velocities were obtained before operation and 10 days afterward. The results were as follows ; 1) VSD(type 2) Peak velocities of mitral valve were significantly decreased when compared preop. with postop. echocardiogram(n=37, preop 132+/-24, postop 98+/-16 cm/sec, p<0.0005). Peak velocities of tricuspid valve were also significantly decreased(n=30, perop 60+/-21, postop 50+/-17cm/sec, p<0.01). Peak velocities of aortic valve were decreased(n=18, perop 120+/-20, postop 108+/-26cm/sec, 0.010.05). Peak velocities of pulmonic valve were significantly decreased(n=17, perop 168+/-46, postop 104+/-51cm/sec, p<0.0005). 3) PDA Peak velocities of mitral valve were significantly decreased when compared perop. with postop. echocardiogram(n=29, perop 138+/-28, postop 111+/-19cm/sec, p<0.0005). Tricuspid valve velocities showed no significant changes between preop. and postop. echocardiogram (n=15, perop 47+/-12, postop 44+/-10cm/sec, p>0.05). Aortic valve velocities showed no significant changes (n=10, perop 134+/-23, postop 121+/-25cm/sec, p>0.05). Peak velocities of pulmonic valve were significantly decreased(n=28, perop 138+/-37, postop 107+/-27cm/sec, p<0.005). This study demonstrates that Doppler echocardiographic assessment of velovity changes are correlated with known hemodynamic data and also will be a good index in the evaluation of operation.
Aortic Valve ; Child ; Echocardiography* ; Echocardiography, Doppler ; Heart Valves* ; Hemodynamics ; Humans ; Infant ; Mitral Valve ; Tricuspid Valve

No abstract available.
Child* ; Humans

No abstract available.
Heart*

No abstract available.

No abstract available.
Empyema, Subdural* ; Haemophilus influenzae* ; Haemophilus* ; Meningitis*

Thirty - five cases of coarctation of aorta, diagnosed by cardiac catheterization and angiography during the period of 1980-1984, at the department of Pediatrics of Seoul National University Hospital, were studied on the clinical features and management. The followoing results were obtained : 1) The incidence of coartation of aorta was 1.2% of congenital heart disease and sex ratio of male to female was 2.2 : 1. 2) The anatomical type of coarctation was preductal in 19 cases, juxtaductal in 15 cases and postductal in 1 case. Most of preductal coarctation (89.5%) were associated with aortic arch or isthmus hypoplasia. 3) All of these patients had various type of additional cardiac anomalies and among them, PDA was the most common associated lesion and followed by VSD. 4) Significant BP gradient between upper and lower extremies and weak femoral pulsation were noticed in 15 cases(42.8%). 5) Congestive heart failure was present in all but one patient less than 1 year old, while only 3 cases(15%) had congestive heat failure in patients older than one year. 6) Overall surgical mortality was 31.8%, but significant decrease in surgical mortality was found since 1983.
Angiography ; Aorta ; Aorta, Thoracic ; Aortic Coarctation* ; Cardiac Catheterization ; Cardiac Catheters ; Estrogens, Conjugated (USP) ; Female ; Heart Defects, Congenital ; Heart Failure ; Hot Temperature ; Humans ; Incidence ; Male ; Mortality ; Pediatrics ; Seoul ; Sex Ratio

OBJECTIVES: Mechanisms of clinical synergistic effects, induced by co-treatments of lithium and valproate, are unclear. Extracellular signal-regulated kinase (ERK) has been suggested to play important roles in mechanisms of the action of mood stabilizers. In this study, effects of co-treatments of lithium and valproate on the ERK1/2 signal pathway and its down-stream transcription factors, ELK1 and C-FOS, were investigated in vitro. METHODS: PC12 cells, human pheochromocytoma cells, were treated with lithium chloride (30 mM), valproate (1 mM) or lithium chloride + valproate. The phosphorylation of ERK1/2 was analyzed with immunoblot analysis. Transcriptional activities of ELK1 and C-FOS were analyzed with reporter gene assay. RESULTS: Single treatment of lithium and valproate increased the phosphorylation of ERK and transcriptional activities of ELK1 and C-FOS, respectively. Combined treatments of lithium and valproate induced more robust increase in the phosphorylation of ERK1/2 and transcriptional activities of ELK1 and C-FOS, compared to those in response to single treatment of lithium or valproate. CONCLUSIONS: Co-treatments of lithium and valproate induced synergistic increase in the phosphorylation of ERK1/2 and transcriptional activities of its down-stream transcription factors, ELK1 and C-FOS, compared to effects of single treatment. The findings might suggest potentiating effects of lithium and valproate augmentation treatment strategy.
Animals ; Genes, Reporter ; Humans ; Lithium Chloride ; Lithium* ; PC12 Cells* ; Pheochromocytoma ; Phosphorylation* ; Phosphotransferases ; Signal Transduction ; Transcription Factors ; Valproic Acid*

Histiocytic Medullary Reticulosis(H.M.R.) is a rapidly progressing fatal disease seen most often in adults, but it has been reported in children younger than 15 years of age. H.M.R. is clinically characterized by fever, wasting generalized lymphadenopathy and hepatosplenomegaly. In the terminal stage, jaundice, purpura, anemia and pancytopenia are all present with or without skin involvement. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the lymphoreticular tissues. Four cases of H.M.R. occurring in pediatric age group, i.e., 5 years, 6 years, 12 years and 14 years of age, respectively, are presented with discussion of the clinico-pathologic characteristics and management. Cases 1,2 and 3 died within 6 months after the onset of illness. Case 1 of our series was particularly younger than in previously reported cases. This was the case that the diagnosis was confirmed by postmortem examination. Analysis of previously reported 25 cases occurring in ages younger than 20 years of age was done.
Adult ; Anemia ; Autopsy ; Child* ; Diagnosis ; Fever ; Histiocytes ; Humans ; Jaundice ; Lymphatic Diseases ; Pancytopenia ; Purpura ; Skin