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May-Hegglin Anomaly Diagnosed by Genetic Study in a Newborn Infant.

Na Hee LEE ; Eun Sun KIM ; Se In SUNG ; So Yoon AHN ; Myung Sook LEE ; Young Mi HAN ; Hee Jin KIM ; Won Soon PARK ; Yun Sil CHANG

Korean Journal of Perinatology.2012;23(2):108-112.

May-Hegglin anomaly is an autosomal dominant platelet disorder characterized by giant platelets, thrombocytopenia, and Dohle-like cytoplasmic inclusion bodies in granulocyte. Usually, diagnosis was delayed because they do not have life-threatening bleeding. We experienced a case of May-Hegglin anomaly, which was diagnosed with genetic study at neonate. A 3 days old female has bilateral cephalhematoma at birth after a Caesarean section delivery. Thrombocytopenia with inclusion bodies in granulocyte was observed at peripheral blood cell morphology. Her mother had thrombocytopenia at pregnancy and was diagnosed May-Hegglin anomaly through MYH9 mutation gene study. Accordingly, infant had genetic study and found same gene mutation with mother. Based on the family history, we can diagnose May-Hegglin anomaly in a newborn infant who has cephalhematoma and thrombocytopenia by genetic study.
Blood Cells ; Blood Platelets ; Cesarean Section ; Female ; Granulocytes ; Hemorrhage ; Humans ; Inclusion Bodies ; Infant ; Infant, Newborn ; Mothers ; Parturition ; Pregnancy ; Thrombocytopenia

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Giant Congenital Melanocytic Nevi with Hemangioma : A Case Report in a Newborn.

Hyun Seok CHO ; Young Kwang CHOO ; Se Ra MIN ; Sang Yeol LEE ; Seung Gu LEE ; Hyeon Soo LEE

Korean Journal of Perinatology.2012;23(2):101-107.

Giant congenital melanocytic nevi are very rare skin lesions with an estimated prevalence of 1 in 20,000 live births, and have high risk of malignant melanoma development and leptomeningeal melanocytosis. Hence, its early and exact diagnosis in the neonatal period is important and essential. Only seven cases of giant congenital melanocytic nevi have been reported in Korea, of which none of the cases were associated with hemangioma. Herein, the authors describe a case of giant congenital melanocytic nevi with huge hemangioma with the pathologic findings that exhibited benign and provide a literature review.
Hemangioma ; Humans ; Infant, Newborn ; Korea ; Live Birth ; Melanoma ; Nevus, Pigmented ; Prevalence ; Skin

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Neonatal Presentation of Prader-Willi Syndrome.

Sang Hun BAEK ; So Yoon AHN ; Se In SUNG ; Jae Won SHIM ; Jong Hee HWANG ; Dong Kyu JIN ; Won Soon PARK ; Yun Sil CHANG

Korean Journal of Perinatology.2012;23(2):95-100.

PURPOSE: In Korea, neonatal clinical features and feeding pattern are not reported systematically. So we report the neonatal presentation of Prader-Willi syndrome. METHODS: This is a retrospective study of 14 neonate with Prader-Willi syndrome whose diagnosed in neonatal intensive care unit, Samsung Medical Center between January 1st 1997 and December 30th 2011. RESULTS: The birth characteristics of patients, maternal age was 31.5+/-4.5 years and 5 (35.7%) were primiparous. Oligohydramnios were reported in 2 cases (14.2%), polyhydramnios were reported in 4 cases (28.5%). 5 pregnancies (35.7%) had complained that decreased fetal movements. Mean gestational age were 38.1+/-2.2 weeks and 12 patients (85%) were term infants, one patient (7.1%) was post term infant, the other patient was preterm infants (7.1%). The average weight at birth were 2509.0+/-442.5 grams. All 14 patients (100%) had hypotonia and a weak cry at birth. 9 patients (64.5%) had the characteristic facial dysplasia. In all, nine male patients (100%) had cryptorchidism. All patients had weak sucking power, so they started to eat via nasogastric tube at birth. The mean duration of use nasogastric tube were 3.2+/-2.0 months. The median age of diagnosis were 1.5 months. 7 cases (50%) were diagnosed within one month of life. Median time of starting GH treatment was 2.0 years. The mean interval of from diagnosis to treatment of growth hormone were 2.2+/-2.0 years. CONCLUSION: Early diagnosis of Prader-Willi syndrome is a very important to improve long-term prognosis.
Cryptorchidism ; Early Diagnosis ; Feeding Behavior ; Female ; Fetal Movement ; Gestational Age ; Growth Hormone ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Korea ; Male ; Maternal Age ; Muscle Hypotonia ; Oligohydramnios ; Parturition ; Phenotype ; Polyhydramnios ; Prader-Willi Syndrome ; Pregnancy ; Prognosis ; Retrospective Studies

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The Analysis of High Risk Infant Patients Being Transferred to Neonatal Intensive Care Units in Korea.

Jae Won SHIM

Korean Journal of Perinatology.2012;23(2):87-94.

PURPOSE: The purpose of this study is to identify the national status of high risk infants who are being transferred at the neonatal intensive care units (NICU), and to analyze the differences in hospital facility resources and regions. METHODS: Survey was conducted in 81 of 117 hospital providing NICU in July of 2010. Data included information on number of NICU bed, resources in the NICU and birth place. RESULTS: Among the patients, 39% were transferred patients and 7.5% were transferred from other regions. In the comparison among the 6 regions, transfer rate was higher in Gyeongin, Chungcheong-Gangwon region and low in Jeolla and Jeju region. According to the multinominal logistic regression analysis, there were more transferred patients when the birth weight was heavier and hospital level and volume was higher. The relative risk ratio of transfer compared to Seoul area was 1.64 in Gyeongin region, 1.68 in Chungcheong-Gangwon region, and 0.63 in Jeolla region. When the each factor was adjusted, the higher number of transfers when there was heavier birth weight and higher level of hospital level and volume remained the same, but the differences among the regions, other than Gyeongin region, had disappeared. CONCLUSION: The transfer of high risk infants after their birth was usually being made for infants with heavier birth weight to hospitals with higher NICU level and volume, and differences among regions also existed. This kind of differences among regions is primarily due to the differences in the local NICU facilities.
Birth Weight ; Humans ; Infant ; Infant, Newborn ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Logistic Models ; Odds Ratio ; Parturition

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Statistic Observation of Marriages, Births, and Children in Multi-cultural Families and Policy Perspectives in Korea.

Ji Hyun LEE ; Myung Hee HAHN ; Sung Hoon CHUNG ; Yong Sung CHOI ; Ji Young CHANG ; Chong Woo BAE ; Yu Kyung KIM ; Hye Ryun KIM

Korean Journal of Perinatology.2012;23(2):76-86.

PURPOSE: Recently, the trend of multi-cultural families is rising in number due to increasing marriage-based immigrants in Korea. We evaluated statistic changes of characteristics in international marriage, births, and off-springs for the past 20 years in Korea. METHODS: The annual report of national population from Statistics Korea, the survey results about the present status of resident foreigners from Korea Ministry of Health and Welfare, Korea Ministry of Public Administration and Security, and editorials from Health and Welfare Forum and other related reports of Korea Institute for Health and Social Affairs and Korea Ministry of Health and Welfare were used. RESULTS: There were 35,098 (10.8%) international marriages among total 326,104 marriages in 2010 in Korea. The regional distribution of the international marriage was the highest in Gyeonggi (18.5%), and the lowest in Jeju (1.1%). The metropolitan area Seoul (15.4%), Incheon (4.5%) and Gyeonggi (18.3%) comprised one third of the entire international marriage. The number of childbirths was 13,443 (2.9%) in 2008, 19,024 (4.3%) in 2009, and 20,312 (4.3%) in 2010 with increasing trend. The number of off-springs was 44,258 in 2007, 58,007 in 2008, 99,684 in 2009 and 105,502 in 2010. Children less than 18 years old was 93.1%. We expected 1.5 times of increase over the next coming 10 years in the number of multi-cultural international marriage and off-springs compared with 2011. CONCLUSION: When we evaluated the characteristics of multi-cultural marriages, childbirths, and off-springs, systemic nationwide care seemed to be necessary in the aspect of health problems such as perinatology, obstetrics, neonatology, and pediatrics. We expect our results to be utilized for the basic health data.
Birth Rate ; Child ; Emigrants and Immigrants ; Epidemiologic Methods ; Humans ; Korea ; Marriage ; Neonatology ; Obstetrics ; Parturition ; Pediatrics ; Perinatology

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Effect of Initial Empirical Antibiotics Duration on Neonatal Outcomes in Very Low Birth Weight Infants.

Nury BAG ; Myo Jing KIM

Korean Journal of Perinatology.2012;23(2):69-75.

PURPOSE: Little is known about the duration of antibiotics for suspected early-onset sepsis (EOS) with negative blood cultures. The purpose of this study is to identify associations between the duration of empirical antibiotics and neonatal outcomes. METHODS: We retrospectively reviewed medical records of very low birth weight infants (VLBW) who admitted to NICU in 2007-2010. We defined empirical antibiotic therapy group as those who started antibiotic therapy in first 3 postnatal days. We compared the neonatal outcomes between short empirical antibiotic therapy (<5 days) and long empirical antibiotic therapy (> or =5 days). RESULTS: Of 122 VLBW, 72 infants were long empirical antibiotic therapy group. In the long empirical antibiotic therapy group, there were lower birth weight, higher rate of out-born, higher rate of vaginal delivery, and had lower Apgar scores. Prolonged antibiotic therapy was associated with delayed start of enteral feeding and incidence of ESBL. CONCLUSION: Prolonged antibiotic therapy may be associated with some adverse neonatal outcomes. Therefore, wide spread agreement regarding the short empirical antibiotic therapy was needed.
Anti-Bacterial Agents ; Birth Weight ; Enteral Nutrition ; Humans ; Incidence ; Infant ; Infant, Very Low Birth Weight ; Medical Records ; Retrospective Studies ; Sepsis

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Comparison of Outcomes according to the Time of Initial Surfactant Treatment for Very Low Birth Weight Infants: A Multicenter Study.

Na Mi LEE ; Jung Ju LEE ; Chun Soo KIM ; Eun Ryoun KIM ; Jae Won SHIM ; Jae Woo LIM ; Min Hee KIM ; Sang Kee PARK ; Young Ah LEE

Korean Journal of Perinatology.2012;23(2):61-68.

PURPOSE: The prophylactic surfactant treatment has been found to improve patient outcomes, compared to the rescue treatment. We performed a multicenter study to determine the relationship between the timing of the initial surfactant treatment and patient outcomes. METHODS: One hundred and seventy one neonates, born at eight different centers, from January 1, 2004 to December 31, 2005, were enrolled. The included subjects were gestational age less than 34 weeks, birth weights less than 1500 g and had respiratory distress syndrome (RDS) that received surfactant. First, a group that received surfactant within two hours after birth was compared to a group that received surfactant after two hours. Next, a group that received surfactant within 30 minutes after birth was compared to a group that received surfactant after 30 minutes. RESULTS: The mean time after birth at which the initial surfactant was administered to neonates was 140.0 +/- 114.3 minutes. The incidence of patent ductus arteriosus (PDA), duration of ventilatory support and hospital days were significantly reduced in the group that received surfactant within two hours after birth. The incidence of PDA and duration of ventilatory support were significantly reduced in the group that received surfactant within 30 minutes after birth. CONCLUSION: Surfactant treatment should be provided to premature infants, as soon as possible.
Birth Weight ; Ductus Arteriosus, Patent ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Parturition ; Pulmonary Surfactants

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The Cause, Diagnosis, and Management of Polyhydramnios.

Seong Yeon HONG

Korean Journal of Perinatology.2012;23(2):50-60.

Amniotic fluid plays an important role in fetal development and growth. Amniotic fluid volume should be maintained adequately throughout gestational periods. Polyhydramnios is a relatively common obstetrical problem, but is associated with poor perinatal outcome both in mother and fetus. For the proper management of polyhydramnios, it is important to find out accurate causes, but it is not easy even if obstetricians perform thorough prenatal assessments. It is also important to understand the regulation of amniotic fluid volume. Based on these backgrounds, this article will briefly review mechanisms of the regulation of amniotic fluid, and will also review diagnosis, etiologies, managements and prognosis of polyhydramnios.
Amniotic Fluid ; Female ; Fetal Development ; Fetus ; Humans ; Mothers ; Polyhydramnios ; Prognosis

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Steroid use in Perinatal Medicine.

Jin A LEE

Korean Journal of Perinatology.2012;23(2):39-49.

Steroid use in perinatal period consists of the prenatal betamethasone or dexamethasone, the hydrocortisone treatment for transient adrenal insufficiency, and the dexamethasone treatment for bronchopulmonary dysplasia. Antenatal steroid treatment is a standard therapy for impending preterm delivery. Antenatal steroid reduced mortality and severe morbidities including intraventricular hemorrhage and periventricular leukomalacia. Usually betamethasone was superior to dexamethasone. Postnatal hydrocortisone can be administered if hypotension cannot be recovered by volume challenge and maximal inotropics support. High-dose dexamethasone cannot be recommended for the treatment of bronchopulmonary dysplasia because of increased cerebral palsy. Further randomized controlled trials should be performed for the low-dose dexamethasone therapy for bronchopulmonary dysplasia.
Adrenal Insufficiency ; Betamethasone ; Bronchopulmonary Dysplasia ; Cerebral Palsy ; Dexamethasone ; Hemorrhage ; Humans ; Hydrocortisone ; Hypotension ; Infant, Newborn ; Leukomalacia, Periventricular

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Subchorionic Placental Cyst with Placental Infarction on Maternal Floor: A Case Report.

Chang Ho CHO ; Mi Ok PARK ; Jae Bok PARK ; In Hwa BAEK

Korean Journal of Perinatology.1999;10(2):226-229.

We reported a case of subchorionic placental cyst with maternal floor infarction in 32-year-old multigravida. In this case the infant without growth retardation was delivered at fuU term by repeat cesarean section. A 5.8*5cm placental cyst detected prenatally by ultrasound was shown subsequentty to be a subchorionic cyst without thrombohematoma. The cyst was unilocular and attached near the cord insertion, and it contained straw-colored fluid. There was no other placental abnormalities except placental inFarction on matemal floor with fibrin deposition. It is necessary to differentiate placental cystic lesions by ultrasonographic and pathologic examination because placental abnormalities could be associated with perinatal complications.
Adult ; Cesarean Section, Repeat ; Female ; Fibrin ; Humans ; Infant ; Infarction* ; Placenta ; Ultrasonography

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