No abstract available.
Heart Diseases* ; Heart*

No abstract available.
Arteries* ; Cardiomyopathy, Hypertrophic* ; Humans ; Infant* ; Mothers*

No abstract available.
Echocardiography* ; Echocardiography, Doppler, Pulsed ; Heart Diseases* ; Heart*

Incontinentia pigmenti (IP) is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eye, CNS, teeth, hair, and nail. It is regarded as an X-linked dominant genetic disorder. We recently experienced a case with IP, who presented with irregular, reticular, and slate-gray to brown colored pigmentation on the whole body at birth. Skin lesions were much improved by 6 month of age. The mother of this infant had the history of same cutaneous lesions in her neonatal period, suggesting that these lesions had familial tendency.
Ectoderm ; Hair ; Humans ; Incontinentia Pigmenti* ; Infant ; Mothers* ; Nuclear Family* ; Parturition ; Pigmentation ; Skin ; Tooth

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

PURPOSE: The human fetus is primarily dependent on the placenta for its nutrition. However, as the fetus matures, it swallows increasing amounts of amniotic fluid, which contributes to the growth of fetus. Accordingly fetuses with congenital obstruction of the gut at high level have a reduced capacity for intestinal absorption of amniotic fluid. We undertook a study to investigate the effect of congenital gut obstruction on fetal growth. METHOD: A retrospective review of the records of all patients presenting congenital gut obstruction over 6-year period (from 1992 to 1997) in Chung-ang Gil hospital was performed. Patients with a complete proximal obstruction were included in group A; patients with incomplete or lower obstruction were included in group B. RESULT: 1) The ratio of male to female was 1.5:1. The mean birth weight and gestational age were 2.89+/-0.60kg and 38.7+/-0.20weeks. The mean birth weight and gestational age in group A were 2.68+/-0.69kg and 37.8+/-0.25weeks. The mean birth weight and gesnal age in group B were 2.980.54kg and 39.1+0.17weeks. There was significant difference between group A and B (P<0.01). 2) Significant differences were found between group A and B in prematurity and growth retardation rate (P<0.01). No significant difference was found between group A and B in associated anomaly rate (P>0.05). 3) In group A, 3 (42.8%) of 7 patients with associated anomalies had IUGR, whereas 8 (40.0%) of 20 patients without associated anomalies had IUGR (P>0.05). The corresponding figures for group B were 23.0% and 14.8%, respectively (P>0.05). CONCLUSION: Congenital gut obstruction causes IUGR by reducing intestinal absorption of amniotic fluid and the effect of IUGR is more pronounced as the obstruction is proximal to jejunum rather than distal to it.
Amniotic Fluid ; Birth Weight ; Female ; Fetal Development* ; Fetal Growth Retardation ; Fetus ; Gestational Age ; Humans ; Intestinal Absorption ; Jejunum ; Male ; Placenta ; Retrospective Studies ; Swallows

Chronic recurrent multifocal osteomyelitis(CRMO) is an unusual inflammatory process involving multiple osseous sites. No causative agent can be consistently isolated from these lesions despite multiple biopsies and the affected child purses a clinical course of chronic remissions and exacerbations independent of antibiotic therapy. Biopsy of the lesions did not reveal any pathogens. Immunologic investigation revealed no abnormality common to the patient and there was no indication of a genetic etiology. The natural history of chronic recurrent multifocal osteomyelitis appears to be slow, spontaneous resolution of the osseous lesions without specific treatment. We experienced a case of CRMO in an 11-year-old girl who had complained of fever, pain on knee and wrist joints for several weeks. We studied the clinical, radiographic, histological findings in this patient and we report a case of CRMO with brief review of related literature.
Biopsy ; Child ; Female ; Fever ; Humans ; Knee ; Natural History ; Osteomyelitis* ; Wrist Joint

PURPOSE: Hypoxic-ischemic encephalopathy is a major neurologic problem and one of the most important perinatal causes of neurological morbidity. Evaluation of the presence, extent, and subsequent evolution of hypoxic-ischemic lesions may be very important. We studied the significance of imaging modality through the analysis of brain MRI findings of hypoxic-ischemic encephalopathy during neonatal period and comparison with findings of brain ultrasonography. METHODS: We analysed the forty-seven infants retrospectively who were diagnosed as hypoxic-ischemic encephalopathy and underwent brain MRI and ultrasonography from Jan. 1992 to May 1996. RESULTS: 1) The mean gestational age and birth weight of the twenty-seven infants who were premature were 32.8+/-2.08weeks and 1.97+/-0.44kg respectively. The mean gestational age and birth weight of twenty infants who were fullterm were 39.3+/-1.04weeks and 2.98+/- 0.93kg respectively. The primary hypoxic-ischemic insults occurred during antenatal, intrapartum and postnatal period. 2) The findings of brain MRI were classified into periventricular leukomalacia, encephalomalacia, basal ganglia lesion, focal parenchymal hemorrhage, ventriculomegaly without other lesion and normal finding. 3) Three infants among twenty-five infants with periventricular leukomalacia, four infants among seven infants with basal ganglia lesion and six infants among seven infants with focal parenchymal hemorrhage were not diagnosed by brain ultrasonography. 4) All of ten infants with encephalomalacia and four infants with ventriculomegaly without other lesion were diagnosed by brain ultrasonography. CONCLUSIONS: MRI can diagnose the hypoxic-ischemic lesions which would not be possible by brain ultrasonography. Therefore MRI is the imaging modality of choice for diagnosis in infants with hypoxic-ischemic encephalopathy. We believe that the benefits of MRI outweigh its somewhat higher cost, lack of portability and monitoring difficulties.
Basal Ganglia ; Birth Weight ; Brain ; Diagnosis ; Encephalomalacia ; Gestational Age ; Hemorrhage ; Humans ; Hypoxia-Ischemia, Brain* ; Infant ; Infant, Newborn ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging* ; Retrospective Studies ; Ultrasonography

PURPOSE: Hypoxic-ischemic encephalopathy is a major neurologic problem and one of the most important perinatal causes of neurological morbidity. Evaluation of the presence, extent, and subsequent evolution of hypoxic-ischemic lesions may be very important. We studied the significance of imaging modality through the analysis of brain MRI findings of hypoxic-ischemic encephalopathy during neonatal period and comparison with findings of brain ultrasonography. METHODS: We analysed the forty-seven infants retrospectively who were diagnosed as hypoxic-ischemic encephalopathy and underwent brain MRI and ultrasonography from Jan. 1992 to May 1996. RESULTS: 1) The mean gestational age and birth weight of the twenty-seven infants who were premature were 32.8+/-2.08weeks and 1.97+/-0.44kg respectively. The mean gestational age and birth weight of twenty infants who were fullterm were 39.3+/-1.04weeks and 2.98+/- 0.93kg respectively. The primary hypoxic-ischemic insults occurred during antenatal, intrapartum and postnatal period. 2) The findings of brain MRI were classified into periventricular leukomalacia, encephalomalacia, basal ganglia lesion, focal parenchymal hemorrhage, ventriculomegaly without other lesion and normal finding. 3) Three infants among twenty-five infants with periventricular leukomalacia, four infants among seven infants with basal ganglia lesion and six infants among seven infants with focal parenchymal hemorrhage were not diagnosed by brain ultrasonography. 4) All of ten infants with encephalomalacia and four infants with ventriculomegaly without other lesion were diagnosed by brain ultrasonography. CONCLUSIONS: MRI can diagnose the hypoxic-ischemic lesions which would not be possible by brain ultrasonography. Therefore MRI is the imaging modality of choice for diagnosis in infants with hypoxic-ischemic encephalopathy. We believe that the benefits of MRI outweigh its somewhat higher cost, lack of portability and monitoring difficulties.
Basal Ganglia ; Birth Weight ; Brain ; Diagnosis ; Encephalomalacia ; Gestational Age ; Hemorrhage ; Humans ; Hypoxia-Ischemia, Brain* ; Infant ; Infant, Newborn ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging* ; Retrospective Studies ; Ultrasonography

PURPOSE: Brainstem auditory evoked response(BAER) is used as screening test for hearing disorders, damages of the central nervous system and congenital anomalies. We studied the difference values according to gender and stimulation sites in normal full-term infants. METHODS: We performed BAER in 38 male and 28 female normal full-term infants, delivered in the Gil Medical Center, Gachen Medical School, from March to July 1996, aged one to seven days. Amplitude I, V, V/I and latency I, III, V and interpeak latency(IPL) I-III, III-V, I-V were measured at 90, 60, 45, 30 dB. Data were analyzed between both sex and between both ears with Student t-test. RESULTS: There were no significant difference in male and female group with the same side's stimulation. At 90 dB, amplitude I of left ear stimulation was significantly higher than right in male and female. Amplitude V/I of right ear stimulation was significantly higher than left ear stimulation in total only. At 90 dB, latency I of right was significantly longer than left in male and female. Latency III of right was longer significantly in total only. IPL I-III, I-V was significantly longer in left than right in male and total. At 60 dB intensity, all data except latency I in total, showed no significant difference. CONCLUSIONS: Interpreting BAER, stimulation site and intensity should be considered. and further studies will be needed for the evaluation of the difference between left and right ear.
Brain Stem* ; Central Nervous System ; Ear* ; Evoked Potentials, Auditory* ; Female* ; Hearing Disorders ; Humans ; Infant* ; Male* ; Mass Screening ; Schools, Medical