We report the dynamic magnetic resonance imaging findings (MRI) of a metaplastic carcinoma of the breast. A 44-year-old woman presented with a 2-month history of a mass in the right breast. The tumor showed a central irregular necrosis that was depicted upon low signal intensity on a T1-weighted image and high signal intensity on a T2-weighted image. The periphery of the tumor showed strong enhancement on a contrast enhanced T1-weighted image. The time-signal intensity curve revealed an early strong contrast enhancement and a delayed washout pattern.
Adult ; Breast* ; Female ; Humans ; Magnetic Resonance Imaging ; Necrosis

Kabuki make-up syndrome (KMS) was firstly reported in 1981 by Niikawa, et al. and Kuroki et al. in a total of ten unrelated Japanese children with characteristic array of multiple congenital anomalies and mental retardation. The name reflects the resemblance between the facial features of patients and the actors of Kabuki, one of the most famous traditional performing arts in Japan. The syndrome is characterized by mental and developmental retardations and peculiar facial features including long palpebral fissures with eversion of the lateral portion of lower eyelid and arching of eyebrows. In addition, dermatoglyphic and skeletal abnormalities are commonly associated. In Japan, the syndrome appears to have an incidence of about 1 : 32,000 newborns. Outside of Japan, a growing number of patients have been recognized. However, this syndrome has been reported only a few cases in Korea. We report a boy diagnosed by clinical features with a brief review of the literature.
Asian Continental Ancestry Group ; Child ; Dermatoglyphics ; Eyebrows ; Eyelids ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Japan ; Korea ; Male

PURPOSE: This study was performed to compare the growth and neurodevelopmental outcome at 15 months of corrected age in very-low-birth weight infants between chronic lung disease(CLD) group and the control group. METHODS: Very-low-birth-weight infants who were admitted and survived in the NICU(neonatal intensive care units) of Chonnam Univeristy Hospital from Jan. 1997 to Jan. 2000 were divided into two groups, CLD group(n=55) and the control group(n=130). Physical assessment for body weight, length and head circumference, and neurologic examination were done at postconceptional 40 weeks, and then one to two months of interval until the baby reached 15 months of corrected age. Frequency of readmission and presence or absence of cerebral palsy were also examined. Statistical analysis was done between the two groups by SPSS program. RESULTS: Infants in the CLD group were lighter birth weight with shorter gestational age, and had more neonatal respiratory morbidity and higher readmission rate than that of the control group. Incidence of head growth below third percentile was significantly higher in CLD group, however when the comparison was done below the tenth percentile, there showed no difference between the two groups. Incidences of motor and language delay, and cerebral palsy were not different between the two groups, but the personal-social delay was higher in the CLD group. CONCLUSION: Incidences of head circumference below third percentile and of personal-social delay at 15 months of corrected age were significantly higher in CLD group than in the control group. But the incidence of cerebral palsy was not different.
Birth Weight ; Body Weight ; Cerebral Palsy ; Gestational Age ; Head ; Humans ; Incidence ; Infant* ; Infant, Very Low Birth Weight* ; Critical Care ; Jeollanam-do ; Language Development Disorders ; Lung Diseases* ; Lung* ; Neurologic Examination

Vascular leiomyoma (Angiomyoma) is a benign tumor composed of smooth muscle cell and vascular endothelium. Most of them occur at the skin of the lower leg. Cases of vascular leiomyomas developing in the nasal cavity and paranasal sinuses are extremely rare. Most common symptoms are nasal obstruction, epistaxis and nasal pain but none are specific for diagnosis. Complete surgical excision is the treatment of choice and was used in all cases reported. Local recurrence rate is very low. We experienced a case of vascular leiomyoma in the inferior turbinate of a 70-year-old woman who complained of intermittent epistaxis. The tumor was successfully removed via an endoscopic approach. It displayed the characteristic histological findings of abundant thick-walled blood vessels blending with proliferative smooth muscle tissue.
Aged ; Angiomyoma ; Blood Vessels ; Endothelium, Vascular ; Epistaxis ; Female ; Humans ; Leg ; Muscle, Smooth ; Myocytes, Smooth Muscle ; Nasal Cavity ; Nasal Obstruction ; Paranasal Sinuses ; Recurrence ; Skin ; Turbinates

Trisomy 9 mosaic syndrome is a rarely reported chromosomal abnormality with high incidence of intrauterine growth retardation and perinatal death. Even a baby lives, he has severe mental retardation and significant malformations. The incidence and severity of malformations and mental retardation correlate with the percentage of trisomic cells in the different tissues. The characteristic craniofacial abnormalitis are narrow bifrontal diameter, up-slanted and short palpebral fissures, a prominent nasal bridge with a short root, a prominent lip covering a receding lower lip, low-set, posteriorly rotated, and misshapen ears. Ventricular septal defect is a main cardiac abnormality. Bony hypoplasia and dislocated hips have been frequently reported. Central nervous system, hepatobiliary, gastrointestinal and genitourinary abnormalities also had been reported. The authors report a baby who had characteristic abnormalities of trisomy 9 mosaicism with narrow temples, up-slanted palpebral fissures, a bulbous nose, thin and protruding upper lip, low set and malformed ears, hyperextended wrist and overlapping fingers. Cytogenetic analysis performed to confirm the chromosomal abnormality revealed trisomy 9, low level mosaic type.
Central Nervous System ; Chromosome Aberrations ; Cytogenetic Analysis ; Ear ; Fetal Growth Retardation ; Fingers ; Heart Septal Defects, Ventricular ; Hip ; Incidence ; Intellectual Disability ; Lip ; Mosaicism* ; Nose ; Trisomy* ; Urogenital Abnormalities ; Wrist

PURPOSE: We investigated the factors influencing the time to reach full enteral feeding in very low birth weight infants. We compared the days of life to start tube or oral feeding, to regain birth weight, and to establish full enteral feeding in very low birth weight infants and determined the risk factors delaying full enteral feeding. METHODS: We reviewed the medical records of 214 newborn infants less than 1,500 g at birth, who were delivered at Chonnam National University Hospital from January 1, 1997 to May 31, 2000. These infants were divided into three groups by birth weight (less than 1,000 g, 1,000-1,249 g, and 1,250-1,499 g). Gestational age, birth weight, the days of life to start first tube or oral feeding, to regain bith weight and to reach full enteral feeding, and the possible risk factors delaying the progress of enteral feeding were analyzed. RESULTS: The gestational age was 29.69+/-2.33 weeks and the birth weight was 1,212+/-194.3 g. The time to start tube or oral feeding, to regain birth weight, and to establish full enteral feeding were significantly later and longer in the lower birth weight group. The time to reach full enteral feeding was related to the duration of mechanical ventilation and oxygen therapy, and was significantly longer in the infants with severe respiratory distress syndrome (RDS), chronic lung disease (CLD), apnea, intraventricular hemorrhage (IVH), cystic periventricular leukomalacia, sepsis/disseminated intravascular coagulopathy, ricketts, and total parenteral nutrition. CONCLUSION: The time to reach full enteral feeding was inversely related to the birth weight, and the risk factors for delayed full enteral feeding were the presence and/or the severity of diseases related to the prematurity such as the duration of mechanical ventilation and oxygen therapy, RDS, CLD, apnea, IVH, and sepsis.
Apnea ; Birth Weight ; Enteral Nutrition* ; Gestational Age ; Hemorrhage ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Jeollanam-do ; Leukomalacia, Periventricular ; Lung Diseases ; Medical Records ; Oxygen ; Parenteral Nutrition, Total ; Parturition ; Respiration, Artificial ; Risk Factors ; Sepsis

PURPOSE: The present study was aimed to investigate the clinical effects of percutaneous central venous catheterization (PCVC) such as frequency of venipuncture, duration of fluid therapy and changes of weight gain in very low birth weight infants. METHODS: The subjects were 70 very low birth weight infants, weighing from 1,000 to 1,500 g, hospitalized in the neonatal intensive care unit at Chonnam University Hospital during the period from January 1998 to December 1999. The control group of 35 infants was subjected to a conventional peripheral venipuncture during the first one year, and the study group of 35 infants used PCVC during the later one year. Data were analyzed using repeated measures of GLM and Student' t-test in SAS program. RESULTS: The frequency of venipuncture was significantly lower in PCVC group than in the control (P=0.0001). Duration of total parenteral nutrition was significantly longer in PCVC group than in the control (P=0.03). The weight gain after the initial weight loss was significantly greater in PCVC group than in the control (P=0.009). CONCLUSION: It is suggested that PCVC may reduce the stress of repeated venipucture. In addition, PCVC may keep the parenteral nutrition longer and may enhance the weight gain in very low birth weight infants.
Catheterization, Central Venous* ; Central Venous Catheters* ; Fluid Therapy ; Humans ; Infant* ; Infant, Newborn ; Infant, Very Low Birth Weight* ; Intensive Care, Neonatal ; Jeollanam-do ; Parenteral Nutrition ; Parenteral Nutrition, Total ; Phlebotomy ; Weight Gain ; Weight Loss

BACKGROUND: Cyclosporine is an immunosuppressive agent used to treat and prevent graft versus host reaction (GVHR)-a complication associated with stem cell transplantation. This study aimed to develop a population pharmacokinetic model of cyclosporine and investigate factors affecting cyclosporine clearance in pediatric hematopoietic stem cell transplant patients. METHODS: A total of 650 cyclosporine concentrations recorded in 65 patients who underwent hematopoietic stem cell transplantation were used. Data including age, sex, weight, height, body surface area (BSA), type of disease, chemotherapy before stem cell transplantation, type of donor, serum creatinine levels, total bilirubin concentration, hematocrit value, and type of concomitant antifungal agents and methylprednisolone used were retrospectively collected. Data related to cyclosporine dosage, administration time, and blood concentration were also collected. All data were analyzed using the non-linear mixed effect model; a two-compartment model with first-order elimination was used. RESULTS: The population pharmacokinetic model of cyclosporine using the NONMEM program was as follows: CL (L/h) = 5.9 × (BSA / 1.2)0.9, V2 (L) = 54.5, Q (L/h) = 3.5, V3 (L) = 1080.0, ka (h-1) = 0.000377. BSA was selected as a covariate of cyclosporine clearance, which increased with an increase in BSA. CONCLUSION: A population pharmacokinetic model for Korean pediatric hematopoietic stem cell transplant patients was developed, and the important factor affecting cyclosporine clearance was found to be BSA. The model might contribute to the development of the most appropriate dosing regimen for cyclosporine. Further studies on population pharmacokinetics should be carried out, prospectively targeting pediatric patients.

PURPOSE: Neonates in neonatal intensive care units(NICU) have a high risk of acquiring nosocomial infection because of their impaired host defence mechanism and invasive procedures. Nosocomial infection result in considerable morbidity and mortality among neonates. This study was carried out to survey both the epidemiology of nosocomial infection in our NICU and the annual trends of pathogens. METHODS: We retrospectively reviewed culture proven nosocomial infection which occurred in our NICU from January 1995 to December 1999. The data included clinical characteristics, site of infection, pathogens, and mortality. RESULTS: Nosocomial infection rates was 9.0 per 100 NICU admissions during the five-year period. Major sites of infection were bloodstream(32.3%), skin(18.4%), endotracheal tube(17.2%), and catheter(10.6%). The most common pathogen was S. aureus(29.9%). and the others were coagulase-negative staphylococci(CONS)(14.8%), Enterobacter(12.4%), and Candida(9.0%). During the five-year period, nosocomial infection rates increased from 9.5 to 11.6 per 100 admissions with the increase of CONS, Candida, Klebsiella, and Acinetobacter baumannii. The infection rate of S. aureus decreased. Multiple episodes of nosocomial infection occurred in 26.1% of all nosocomial infections. Overall bloodstream infection rates were 3.6 per 100 NICU admissions during five years. CONS(29.1%) and S. aureus(27.1%) were the two most common pathogens. Increasing rates of bloodstream infection by CONS, Candida, Klebsiella, and Acinetobacter baumannii were observed. Bloodstream infection related mortality was 11.9%. CONCLUSION: The predominant pathogens of nosocomial infection in NICU were S. aureus and CONS. Bloodstream infection, the most frequent nosocomial infection, should be a major focus of surveillance and prevention efforts in NICU.
Acinetobacter baumannii ; Candida ; Cross Infection* ; Epidemiology ; Humans ; Infant, Newborn ; Intensive Care, Neonatal* ; Klebsiella ; Mortality ; Retrospective Studies

PURPOSE: Twins have a higher mortality and morbidity than singletons. Co-twin with one fetal death is particularly at risk. We investigated the neonatal outcome of live co-twins when one fetus had died after the 20th gestational week, and associated risk factors. METHODS: A retrospective study was performed in fifteen cases of twin pregnancy with single intrauterine fetal deaths after the 20th gestational week during the period from January 1996 to December 2000 at Chonnam University Hospital. RESULTS: Gestational age was 33.7+/-3.2 weeks, birth weight was 1,992+/-592 g. Interval between one fetal death being detected and the delivery of a live co-twin was 32.4+/-29.5 days. There were 11 cases(73.3%) of premature babies less than 37 gestational weeks. Main causes of preterm delivery were preterm labor and premature rupture of membranes. Hematologic findings suggesting disseminated intravascular coagulopathy(DIC) were not found in all mothers before delivery, and was not associated with DIC and encephalomalacia of the live co-twin. Perinatal outcome of fifteen live co-twins was as follows : six were normal(40%), three were DIC(20.0%), three were encephalomalacia(20.0%), one suffered intrauterine growth retardation, there was one case of twin to twin transfusion syndrome, and one of congenital heart disease(atrial septal defect with pulmonary stenosis). The occurrence of DIC and encephalomalacia in live co-twins was not related to placental chorionicity, birth weight, gestational week, and the interval between the detection one fetal death and the delivery of a live co-twin. CONCLUSIONS: We could not find any maternal hematologic problems in twin pregnancies complicated by one fetal death. Twenty percent of live co-twins showed DIC and encephalomalacia. However, its associated risk factors were not found. We need to investigate more closely the cases of live co-twins with one intrauterine fetal death.
Birth Weight ; Chorion ; Dacarbazine ; Encephalomalacia ; Female ; Fetal Death* ; Fetal Growth Retardation ; Fetofetal Transfusion ; Fetus ; Gestational Age ; Heart ; Humans ; Jeollanam-do ; Membranes ; Mortality ; Mothers* ; Obstetric Labor, Premature ; Pregnancy ; Pregnancy, Twin* ; Retrospective Studies ; Risk Factors ; Rupture