In complete heart block(CHB), there is a complete failure of the atrial impulse leading into a ventricular response, the atria and ventricles beat independently, with the latter having a slower rate. Approximately one-third of infants with congenital CHB have associated structural heart disease such as corrected transposition of the great arteries, single ventricle, and the heterotaxy syndrome. For those patients without associated anomalies in whom bradycardia causes heart failure unresponsive to drugs, a pacemaker is necessary. We report a case of congenital complete heart block showing a ventricular beat of 57 /min despite atrial beat of 125/min. The mother of the baby was asymptomatic but her serologic tests were positive for connective tissue disease. The patient had positive serologic tests for anti-Ro antibody and anti-La antibody but associated structural heart anomalies were not found. He remained well without signs and symptoms of heart failure and no treatment was required except 02 supply.
Arteries ; Bradycardia ; Connective Tissue Diseases ; Heart Block* ; Heart Diseases ; Heart Failure ; Heart* ; Heterotaxy Syndrome ; Humans ; Infant ; Mothers ; Serologic Tests

PROPOSE: This study was designed to compare the efficacy of a synthetic surfactant (Exosurf) and a modified bovine surfactant (SurfactenR) in the treatment of neonatal respiratory distress syndrome. METHODS: A total of 90 infants with respiratory distress syndrome who were admitted to neonatal intensive care unit at Samsung Medical Center between October 1994 to September 1996 were includeeach surfactant. RESULTS: There was no significant difference in between two groups regarding birth weight, gestational age, and initiation of treatment after birth. ExosurfR group received less supplemental oxygen therapy and ventilator care. Survival rate were 81.3R in Exosurf group and 79.2% in SurfactenR group. The incidences of patent ctus arteriosus in the ExosurfR and SurfactenR groups were 75% and 62.5%, grade 3-4 intcular hemorrhage were 18.8% and 10.4%, respectively,' retinopathy of prematurity were 9.4% and 18.8%, respectively. There was significant improvernent of a/APO2 and VI at 30 minutes and 2 hours after the treatment in SurfactenR group', 2 hours and 6 hours after the treatment in ExosurfR group, however, dynamic compliance and respiratory resistance did not improve during 24 hours. CONCLUSION: Although SurfactenR treatment appears to induce faster improvement in oxygenation and pulmonary function than ExosurfR treatment, this study does not reveal any difference in clinical outcomes among those who received two different surfactant preparations.
Birth Weight ; Compliance ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Oxygen ; Parturition ; Respiratory Distress Syndrome, Newborn* ; Retinopathy of Prematurity ; Survival Rate ; Ventilators, Mechanical

PURPOSE: High flow rate (FR) and pressure limit (PL) strategy with time-cycled pressure-limited (TCPL) ventilator is employed routinely in the neonates. Theoretical basis of this strategy is the two-compartment theory that the lung with acute respiratory failure consists of units with different compliance and resistance. But such constant pressure strategy has the risk of ventilator induced lung injury. We compared the ventilatory indices and clinical outcomes of two different strategies, high FR-constant pressure and low FR-constant FR in the ventilator care of the neonates with acute respiratory failure. METHODS: For the neonates born in our hospital and treated with mechanical ventilation from March to August in 1997, two different ventilator strategies were employed randomly with flow control ventilator. In the high-FR group, the FR was fixed at 10 L/ min and the PL was adjusted according to the arterial blood gas analysis (ABGA) results. In the low-FR group, the FR was adjusted to 10 mL/kg of tidal volume. Sixty neonates were enrolled, 32 in high-FR and 28 in low-FR group. Ventilatory indices and clinical outcomes were statistically cornpared in the two groups. RESULTS: Perinatal factors were not different in the two groups. Initial ventilator settings, ABGA results and ventilatory indices were not different. The tidal volume, fraction of inspired oxygen, peak inspired pressure and oxygenation index were higher and dynamic compliance was lower in the high-FR group compared to the low-FR group after 3 to 72 hours of ventilator care. In clinical outcomes, incidences of pulmonary interstitial emphysema, pneumothorax and chronic lung disease were significantly lower in the low-FR group. CONCLUSION: Low-FR with constant FR strategy resulted in better clinical outcomes in the ventilator care of neonates. We conclude that constant FR strategy prevents damage of the better compliant lung units and decreases the incidence of acute and chronic complications of ventilator care.
Blood Gas Analysis ; Compliance ; Emphysema ; Humans ; Incidence ; Infant, Newborn* ; Lung ; Lung Diseases ; Oxygen ; Pneumothorax ; Respiration, Artificial ; Respiratory Insufficiency* ; Tidal Volume ; Ventilator-Induced Lung Injury ; Ventilators, Mechanical

It has been estimated that chromosomal abnormality occurs in 0.4% of live births. A balanced translocation between chromosome 2 and 18, t (2;18) (p23;q23), is extremely rare. We report a neonate whose karyotype was 46, XY t (2;18) (p23;q23). He had multiple anomalies such as micrognathia, low-set ears, short neck, undescended testes, atrial septal defect, and decreased physical activity. Chromosomal analysis with G banding in high resolution showed a balanced translocation t (2;18) (p23;q23). The same chromosomal abnormality was found on the family for 3 generations.
Chromosome Aberrations ; Chromosomes, Human, Pair 2 ; Cryptorchidism ; Ear ; Family Characteristics ; Heart Septal Defects, Atrial ; Humans ; Infant, Newborn ; Karyotype ; Live Birth ; Male ; Motor Activity ; Neck

Miller-Dieker Syndrome consists of severe type I lissencephaly and a characteristic abnormal facial appearance at birth and may progress to severe neurologic defects such as intractable seizure and growth failure. This syndrome is associated with microdeletion of p13.3 in the distal portion of chromosome 17. Lissencephaly is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle, and microscopic evidence of incomplete neuronal migration. We diagnosed Miller-Dieker syndrome in a case in which there are charcteristic craniofacial appearance and neurologic symptoms and type I lissencephaly on the MRI. : We confirmed this syndrome with the a microdeletion of p13.3 portion in the short arm of chromosome 17 by the FISH method. We have experienced a baby with this syndrome, who showed characterisic craniofacial abnormalities and a microdeletion of p13.3 portion in the short arm of chromosome 17. Then we report this rare case with brief review of literature.
Arm ; Brain ; Chromosomes, Human, Pair 17 ; Classical Lissencephalies and Subcortical Band Heterotopias* ; Craniofacial Abnormalities ; Lissencephaly ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Neurons ; Parturition ; Seizures

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

Fusion of the eyelid margins is normal in human fetal development, but it is abnormal at birth. The approximated margins of developing eyelids fuse during the 9 th week of gestation and remain fused until the end of the 5 th month of gestation. Subsequently, they became separated although the process may not be completed until the 7 th month of gestation. On occasion, the entire or a portion of eyelid margins remain fused postnatally, resulting in a spectrum of eyelid abnormalities that are usually classified according to the morphology. Ankyloblepharon filiforme adnatum comprises single or multiple fine bands of extensile tissue connecting the lid margins at the grey line and it reduces the palpebral fissure by interfering with the movements of lids. This abnormality has been reported as an isolated finding, in association with other anomalies, or as a part of a well-defined syndrome. We have experienced a case with this syndrome. Then we report this rare case with brief review of literature.
Eyelids ; Fetal Development ; Humans ; Parturition ; Pregnancy

We hereby report on a newborn infant with a lipomeningomyelocele present with hydrosyringomyelia. MR scans disclosed a low-lying spinal cord to be continuous dorsally with the neural placode, the nerve roots crossed the subarachnoid space and the lipoma lied outside the dura and contiguous with subcutaneous fat. And hydrosyringomyelia was noted on lower spinal cord. She had no neurologic signs at birth and she got operation at 4 months of age before any neurologic signs accompanied and she is doing well not presenting any neorologic or orthopedic complications until 8 months of age.
Humans ; Infant, Newborn ; Lipoma ; Neurologic Manifestations ; Orthopedics ; Parturition ; Spinal Cord ; Subarachnoid Space ; Subcutaneous Fat ; Syringomyelia*

The large size and vascularity of the neonatal adrenal glands are vulnerable to traumatic and asphyxial injuries. This condition varies in presentation, which the most common is an abdominal mass alone or mass with jaundice and anemia. Some infants show signs of adrenal insufficiency. Abdomial ultrasonogram is the most valuable diagnostic tool. To result in adrenal insuffiency, hemorrhage must involve both adrenals and at least 90% of the adrenocortical tissue must be destroyed. To affect infant may show signs of hypovolemic shock, electrolyte imbalance and metabolic acidosis. Treatment for adrenal insufficiency must be immediate and vigorous, and consists of intravenous glucose, fluid, and electolyte replacement. And conservative treatment failure is candidate for steroid replacement. We experienced a case of bilateral hemorrhage with adrenal insufficiency, who improved with hydrocortisone.
Acidosis ; Adrenal Glands ; Adrenal Insufficiency* ; Anemia ; Glucose ; Hemorrhage* ; Humans ; Hydrocortisone ; Infant ; Jaundice ; Shock ; Treatment Failure ; Ultrasonography

The finding of extraluminal gas on plain abdominal radiogram is usually associated with a perforated viscus, and an emergency laparatomy is indicated. But we report herein an immature neonate with spontaneous pneumoperitoneum that was treated successfully with conservative management, although this infant she showed positive result in stool occult blood test. As this case shows, the finding of pneumoperitoneum is not of a surgical emergency, because there are many benign explanations for pneumoperitoneum. A through history taking and physical examination are most important in differentiating between surgical and nonsurgical pneumoperitonium.
Emergencies ; Humans ; Infant ; Infant, Newborn* ; Occult Blood ; Peritonitis ; Physical Examination ; Pneumoperitoneum*