PURPOSE: Minocycline appears to reduce the mortality rate of patients with myocardial infarction. Therefore, the cardioprotective effects of minocycline through reduction of apoptosis were studied in a rat myocardiac cell culture induced by hypoxia. METHODS: Cultured cells from the hearts of Sprague-Dawley rats were divided into two sets of groups: normoxia groups treated with 5% O2 and hypoxia groups treated with 1% O2. After several days of incubation, the control groups were not treated with minocycline, while the sample groups were treated with 1 mug/mL of minocycline. The damaged cells were observed under a microscope, while apoptosis was detected using a Tdt-mediated dUTP nick and labeling(TUNEL) assay control-stained with 4'-6'-diamidino-2-phenylindole-2HCl(DAPI). RESULTS: In normoxia, the control group showed a statistically significant difference from the sample group treated with 1 mug/mL of minocycline. In addition, in hypoxia, there was also a statistically significant difference between the two groups. When comparing the normoxia and hypoxia groups to each other, there was a statistically significant difference between the control groups and sample groups. CONCLUSION: Minocycline was found to be protective for heart in normoxia and in hypoxia in the model of rat myocardiac cell culture.
Animals ; Anoxia* ; Apoptosis ; Cell Culture Techniques* ; Cells, Cultured ; Heart ; Humans ; Minocycline* ; Mortality ; Myocardial Infarction ; Rats* ; Rats, Sprague-Dawley

Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial calcifications in the particularly periventricular area. It undergoes an autosomal recessive inheritance. Twelve cases of Raine syndrome have been reported in the literature. However, a case of Raine syndrome in Korea has not been reported yet. Therefore, we describe a female newborn with Raine syndrome with a brief review of the literatures.
Female ; Infant, Newborn ; Humans

PURPOSE: Pulmonary vascular hypertension is a common problem in congenital heart disease, the most common cardiac condition in childhood. However, the mechanisms responsible for this pathologic change, treatment, and prevention are poorly understood. Therefore, we studied the gene expression of vascular endothelial growth factor(VEGF) by using a hypoxic model of the pulmonary artery smooth muscle cells. METHODS: The main pulmonary artery and its proximal branches of a 6 wk old Fischer rat were excised. They were cut into multiple small pieces and suspended in DMEM medium supplemented with 20% fetal bovine serum and incubated in 5% CO2-95% air atmosphere. The smooth muscle cells were confirmed by immunostaining with smooth muscle myosin and alpha-smooth muscle actin antibodies. The VEGF gene expression in the hypoxic group was compared with the one in control the group as well as the one in the starved group by RT-PCR and Northern blot hybridization. RESULTS: There was no statistically significant difference among the control, hypoxic and starved groups. CONCLUSION: There are few studies of pulmonary vascular hypertension at the molecular level in Korea. Therefore, we studied the expression of VEGF gene in hypoxic pulmonary vascular smooth muscle cells. Further studies will be needed to find the difference between newly born and adult rats, or human and rat pulmonary vascular smooth muscle cells in gene expression. We hope that the study will lead to a better understanding of pulmonary vascular hypertension.
Actins ; Adult ; Animals ; Anoxia* ; Antibodies ; Atmosphere ; Blotting, Northern ; Gene Expression ; Heart Defects, Congenital ; Hope ; Humans ; Hypertension ; Korea ; Muscle, Smooth* ; Muscle, Smooth, Vascular ; Myocytes, Smooth Muscle* ; Myosins ; Pulmonary Artery* ; Rats* ; Vascular Endothelial Growth Factor A*

Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal cell carcinoma, intravesical ureterocele, and osteoporosis. The incidence is 1 in 400-1,000 of the population and urological abnormalities are not common. However a case of Klinefelter syndrome associated with multicystic dysplastic kidney has not been not reported up to date. Therefore, we describe a 1- day-year old baby boy who presented with Klinefelter syndrome with unilateral multicystic kidney dysplastic disease, plus with a brief review of the literature.
Autoimmune Diseases ; Carcinoma, Renal Cell ; Chromosome Aberrations ; Diabetes Mellitus ; Gonadotropins ; Gynecomastia ; Hair ; Humans ; Hypogonadism ; Incidence ; Infant, Newborn* ; Infertility ; Karyotype ; Klinefelter Syndrome* ; Male ; Multicystic Dysplastic Kidney* ; Osteoporosis ; Testosterone ; Ureterocele

Scoliosis describes a lateral curvature of the spine and is often associated with cosmetic and functional impairments due to severe deformity of the spine. The incidence of adolescent scoliosis is 2-4% of children between 10 and 16 years of age. Eighty five percent of them are idiopathic, in which the most common type of scoliosis is right side-bending. In addition, it is classified into congenital, and secondary scoliosis such as neuromuscular disease and neurofibromatosis. Congenital scoliosis is associated with abnormalities of urinary system(20%), congenital heart diseases(15 %), and other abnormalities(10%) such as syringomyelia. We experienced a case of scoliosis by a foreign body(a pencil) in the colon which has never been reported up to date. A brief review of the literature was made.
Adolescent ; Child ; Colon* ; Congenital Abnormalities ; Foreign Bodies* ; Heart ; Humans ; Incidence ; Neurofibromatoses ; Neuromuscular Diseases ; Scoliosis* ; Spine ; Syringomyelia

PURPOSE: Food protein-induced enterocolitis syndrome (FPIES) is a symptom complex of vomiting and diarrhea caused by non-IgE mediated allergy to cow's milk and/or soy in young infants. Transforming growth factor (TGF)-beta has been reported to protect the epithelial barrier of the gut from foreign antigens. We studied the expression of type 1 and 2 TGF-beta receptors in the mucosa of small intestine to investigate their roles in the pathogenesis of FPIES. METHODS: Twenty-eight patients, aged 7 to 120 days (mean 49 days) who were diagnosed with FPIES by clinical criteria and challenge tests were included. Immunohistochemical stainings for type 1 and 2 TGF-beta receptors were performed on endoscopic duodenal biopsy specimens. RESULTS: Type 1 and 2 TGF-beta receptors were expressed in the villous and crypt epithelial cells but nearly absent in the lamina propria in both patients and controls. Type 1 TGF-beta receptor expression was significantly lower in the patients who had villous atrophy than in the patients who had not and in controls. The expression of type 1 TGF-beta receptor was negatively correlated with the severity of villous atrophy. Type 2 TGF-beta receptor expression showed no significant difference between the patients and controls. CONCLUSION: Our results suggests that the decreased activity of type 1 TGF-beta receptor is implicated in the pathogenesis of FPIES in young infants.
Atrophy ; Biopsy ; Diarrhea ; Enterocolitis* ; Epithelial Cells ; Humans ; Hypersensitivity ; Infant ; Intestine, Small ; Milk ; Mucous Membrane ; Receptors, Transforming Growth Factor beta ; Transforming Growth Factors ; Vomiting

Chronic granulomatous disease(CGD) is a genetically inherited disorder caused by the failure of phagocytic cells to produce superoxide. It is characterized by frequent and uncontrollable infection which often lead to death in early childhood. The first clinical signs may be confined to skin and manifest themselves as abscesses, pyoderma, eczema or draining sinuses. The disease was first reported in 1957 and thereafter the biomolecular mechanism has been found. The first report in Korea was an autopsy case in 1979 and since then there have been a few case reports. This disease is diagnosed by symptoms, such as high fever, cervical lymphadenopathy, skin nodule, lung field infiltration, periumbilical abscess, liver abscess, pyoderma and pericordal abscess. The pathogen here were catalase producing bacteria and fungi. However, we have experienced a case of CGD, in which symptoms were perianal, scrotal abscesses and lung field nodular infiltration. In this case the pathogen was found as a catalase negative Enterococcus. (J Korean Pediatr Soc 2000;43-846-850)
Abscess ; Autopsy ; Bacteria ; Catalase ; Eczema ; Enterococcus ; Fever ; Fungi ; Granulomatous Disease, Chronic* ; Korea ; Liver Abscess ; Lung ; Lymphatic Diseases ; Phagocytes ; Pyoderma ; Skin ; Superoxides

Since Alfi et al. first described the 9p deletion syndrome in 1973, approximately 40 cases with deletion of the chromosome 9p have been reported. These patients have multiple anomalies in craniofacies, limbs, and cardiovascular system, and mental retardation. In most cases, the breakpoint is located at the band 9p22 and the deletion is de novo. We report a neonate with 9p deletion syndrome diagnosed by clinical features and chromosomal analysis. He had multiple anomalies such as up slanting of palpebral fissures, epicanthal folds, arched eyebrows, anteverted nares, cleft palate, micrognathia, pectus excavatum, widely spaced nipples, cryptorchidism, atrial septal defect, tricuspid regurgitation (grade : III) and persistent muscle hypotonia.
Cardiovascular System ; Cleft Palate ; Cryptorchidism ; Extremities ; Eyebrows ; Funnel Chest ; Heart Septal Defects, Atrial ; Humans ; Infant, Newborn ; Intellectual Disability ; Male ; Muscle Hypotonia ; Nipples ; Tricuspid Valve Insufficiency

PURPOSE: To assess wall motion of the left ventricle in the various forms of congenital heart disease and to select appropriate methods by which to gauge left ventricular wall motion. METHODS: We evaluated left ventricular function, including volume, global ejection fraction, and regional wall motion(Centerline method, Local Ejection Fraction, Curved Perimeter method and Radial method). The evaluation was done on review mode of 30-degree elongated right anterior oblique projection of left ventriculogram by using cardiac analysis program of the DXC Hiline Digital System(GER, USA) in 53 pediatric patients undergoing diagnostic(or therapeutic) cardiac catheterization from June 1995 to July 1996 at Kyung-pook National University Hospital. RESULTS: Cardiac indices calculated by Dodge's and Simpson's methods were 5.76+/-2.13 L/min/ m2, 5.86+/-2.31 L/min/m2 respectively with significant correlation to each other(r=0.99, P<0.01). Global ejection fractions using both methods were 69.81+/-8.33%, and 69.69+/-8.20%, respectively (r=0.99, P<0.01). After dividing the left ventricular outline contour into five regions such as posterobasal, diaphragmatic, apical, anterolateral and anterobasal portions, all four models could ascertain regional wall motion abnormalities well(P<0.01). And among them, the Centerline method and the Curved Perimeter method correlated well in assessing wall motion(for each portion r=0.67, 0.84, 0.94, 0.91 and 0.61 respectively, P<0.01). CONCLUSION: In conclusion, we confirmed regional wall motion abnormalities in the various forms of congenital heart disease in spite of normal global ejection fractions, showing that the Centerline and Curved Perimeter methods are preferable methods in the assessment of wall motion in patiens with congenital heart disease.
Cardiac Catheterization ; Cardiac Catheters ; Child* ; Heart Defects, Congenital* ; Heart Ventricles* ; Humans ; Ventricular Function, Left

It is well known that both acute glomerulonephritis and rheumatic fever are a common sequelae of group A streptococcal infection. However, their simultaneous occurrence is uncommon. Only a few cases have been reported because they are different in epidemiologic, clinical, serologic and immunopathogenic characteristics. We experienced a 10-year-old boy who presented the manifestation of acute rheumatic fever and acute poststreptococcal glomerulonephritis. We report this case with brief review of literature.
Child ; Glomerulonephritis* ; Humans ; Male ; Rheumatic Fever* ; Streptococcal Infections