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A Case of Giant Congenital Melanocytic Nevi in a Neonate.

Seo Eun CHO ; Lim CHOI ; Kyu Hee PARK ; Jang Hoon LEE ; Byung Min CHOI ; Young Sook HONG ; Kee Hwan YOO ; Joo Won LEE

Journal of the Korean Society of Neonatology.2010;17(1):147-151.

Giant congenital melanocytic nevi are very rare, with an estimated incidence of 1 in 20,000 live births. They have a high risk of malignant melanoma transformation and neurological deficits such as neurocutaneous melanocytosis and epilepsy. Early evaluation, surgical intervention and careful long term follow up are recommended to monitor for malignant transformation. We report one case of giant congenital melanocytic nevi diagnosed at birth with the related literatures.
Epilepsy ; Humans ; Incidence ; Infant, Newborn ; Live Birth ; Melanoma ; Nevus ; Nevus, Pigmented ; Organothiophosphorus Compounds ; Parturition

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A Newborn with Lethal Metatropic Dysplasia.

Hye Jung CHO ; Seon Tae HWANG ; Sang Seon LEE ; Jee Eun KIM ; Ae Ran JO ; So Yeon SHIM ; In Sang JEON ; Dong Woo SON

Journal of the Korean Society of Neonatology.2010;17(1):141-146.

Metatropic dysplasia is a rare spondylo epi metaphyseal dysplasia characterized by progressive kyphoscoliosis, short limbs with relatively large hands and feet and limited of motion and enlargement of the large joints. It is diagnosed based on the characteristic clinical and radiological features. Even though benign cases of this disease are frequently reported, metatropic dysplasia can often have a fatal outcome. We describe a sporadic case of the well-delineated lethal metatropic dysplasia.
Dwarfism ; Extremities ; Fatal Outcome ; Foot ; Hand ; Humans ; Infant, Newborn ; Joints ; Osteochondrodysplasias

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A Case of Infantile Hepatic Hemangioendothelioma Incidentally Detected during the Evaluation of Galactosemia.

Ryoung Kyoung LIM ; Shin Yun BYUN ; Seong Shik PARK ; Young Don KIM

Journal of the Korean Society of Neonatology.2010;17(1):136-140.

Galactosemia is a group of inherited enzyme deficiencies characterized by increase in the blood galactose levels. This condition may be associated with deficiencies of galactose-1-phosphate uridyl transferase, galactokinase, or uridine diphosphate galactose-4-epimerase. However, the elevated galactose identified by neonatal screening tests has several other possible etiologies, including hepatic hemangioendothelioma, hepatic hemangioma, and patent ductus venosus with hypoplasia of the portal vein. We report a 13-day-old Korean male with hepatic hemangioendothelioma, which was incidentally detected during the evaluation for suspected galactosemia. Laboratory studies revealed that mildly elevated levels of galactose, galactose-1-phosphate and alpha-fetoprotein, at the time of admission, were gradually decreased to the normal range over the 6 months of observation. Ultrasonography showed a well-defined heterogeneous hypoechoic mass in the liver, and magnetic resonance imaging study showed multiple enhanced mass lesions, which was compatible with the diagnosis of a hepatic hemangioendothelioma. Thus, hepatic imaging, especially ultrasonography, should be performed if neonatal screening suggests galactosemia.
alpha-Fetoproteins ; Galactokinase ; Galactose ; Galactosemias ; Galactosephosphates ; Hemangioendothelioma ; Hemangioma ; Humans ; Infant ; Infant, Newborn ; Liver ; Magnetic Resonance Imaging ; Male ; Neonatal Screening ; Portal Vein ; Reference Values ; UDPglucose-Hexose-1-Phosphate Uridylyltransferase ; Uridine Diphosphate ; Vascular Malformations

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A Case of Hemophilia A Diagnosed in a Premature Infant.

Ki Won OH ; Kyung Yeon LEE ; Ja Hyeong KIM ; Kang Won RHEE ; Jin Young JEONG ; Sang Kyu PARK

Journal of the Korean Society of Neonatology.2010;17(1):132-135.

Although the majority of abnormal bleeding during the neonatal period results from acquired coagulation disorders, inherited coagulation disorders can also manifest at this time. Hemophilia is the most common of inherited coagulation disorder. Although 40-70% of cases with hemophilia are diagnosed in the neonatal period, few cases have been reported in premature infants. We report a case of a premature infant born at 31 weeks of gestation, diagnosed with hemophilia A by blood coagulation test, coagulation factor assay and study of the F8 gene. The baby was treated with recombinant factor VIII (Recombinate(R), USA) because of repeated seizures and intramuscular hematoma.
Blood Coagulation Disorders, Inherited ; Blood Coagulation Factors ; Blood Coagulation Tests ; Factor VIII ; Hematoma ; Hemophilia A ; Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Pregnancy ; Seizures

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A Case of Supernumerary Derivative (22) Syndrome Resulting from a Paternal Balanced Translocation.

Youn Soo JUN ; Cheol Hwan SO ; Seung Taek YU ; Do Sim PARK ; Eun Hae CHO ; Yeon Kyun OH

Journal of the Korean Society of Neonatology.2010;17(1):127-131.

Supernumerary derivative (22) syndrome is a rare genomic syndrome. It is characterized by severe mental retardation, microcephaly, failure to thrive, preauricular tag or sinus, ear abnormalities, cleft and/or high-arched palate, micrognathia, kidney abnormalities, congenital heart defects, and genital abnormalities in males. In 99% of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. To date, there have been about 100 case reports of supernumerary derivative (22) syndrome. In most of the cases, supernumerary derivative (22) syndrome was the result of 3:1 meiotic segregation in the maternal 11;22 translocation carrier. We now report a case of 47,XX, + der(22)t(11;22)(q23;q11.2) resulting from 3:1 meiotic segregation of the paternal translocation carrier.
Chromosomes, Human, Pair 11 ; Chromosomes, Human, Pair 22 ; Congenital Abnormalities ; Ear ; Failure to Thrive ; Heart ; Humans ; Intellectual Disability ; Kidney ; Male ; Microcephaly ; Palate ; Parents ; Trisomy

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Symptomatic Hepatitis A Virus Infection in a Newborn.

Jang Yong JIN ; Ye Seul HONG ; Woo Ryoung LEE

Journal of the Korean Society of Neonatology.2010;17(1):123-126.

Hepatitis A virus (HAV) infection is a common cause of acute hepatitis, but is rarely responsible for neonatal hepatitis. HAV infection is usually transmitted by the fecal-oral route, but during the neonatal period can be transmitted by the intrauterine vertical route or postnatal horizontal route. HAV infection is usually self-limited, but it can be potentially life-threatening in adults. The clinical course and symptoms are mild and the infection can be asymptomatic in newborns. Recently, we experienced a case of symptomatic neonatal hepatitis A. To our knowledge, this is the first case of neonatal hepatitis with HAV infection in Korea.
Adult ; Hepatitis ; Hepatitis A ; Hepatitis A virus ; Humans ; Infant, Newborn ; Korea

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A Case of Intestinal Hemangioma Complicated with Thrombocytopenia (Kasabach-Merritt syndrome) in Premature Infant.

Young Jin LEE ; Sul Hee BAE ; Eun Song SONG ; Soo Jin CHOI ; Yoon Ha KIM ; Young Youn CHOI

Journal of the Korean Society of Neonatology.2010;17(1):116-122.

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.
Ascites ; Hemangioma ; Humans ; Infant, Newborn ; Infant, Premature ; Kasabach-Merritt Syndrome ; Laparotomy ; Thrombocytopenia ; Tomography, Emission-Computed, Single-Photon

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Two Cases of Neonatal Persistent Pulmonary Hypertension Treated by Veno-venous Extracorporeal Membrane Oxygenation (V-V ECMO).

Hee Joon YU ; Se In SUNG ; Jin Kyu KIM ; Hyun Joo SEO ; Seo Heui CHOI ; Hye Soo YOO ; So Yoon AHN ; Eun Sun KIM ; Ji Hyuk YANG ; June HUH ; Yun Sil CHANG ; I Seok KANG ; Tae Kook JUN ; Won Soon PARK

Journal of the Korean Society of Neonatology.2010;17(1):109-115.

Persistent pulmonary hypertension in newborns (PPHN) is a disorder of the vascular transition from fetal to neonatal circulation. It results in cyanosis due to right-to-left shunting of the blood through the ductus arteriosus and/or foramen ovale manifesting as hypoxemic respiratory failure. We managed two cases of PPHN after meconium aspiration with high frequency oscillating ventilators and inhaled nitric oxide. They did not respond to conventional management. Veno-venous extracorporeal membrane oxygenation (ECMO) was provided, and ECMO weaning was possible resulting survivals in two cases. We report two PPHN cases, which were treated successfully with veno-venous ECMO for the first time in Korea.
Cyanosis ; Ductus Arteriosus ; Extracorporeal Membrane Oxygenation ; Foramen Ovale ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Korea ; Meconium Aspiration Syndrome ; Nitric Oxide ; Respiratory Insufficiency ; Ventilators, Mechanical ; Weaning

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Nomogram of Transcutaneous Bilirubin Level after Birth Driven from a Single Center.

Young Ji HAN ; Eun Ryoung KIM ; Myung Sook LEE ; Won Uk LEE ; Su Hwa PARK ; Jung Ju LEE

Journal of the Korean Society of Neonatology.2010;17(1):102-108.

PURPOSE: The goal of this study was to measure bilirubin levels over 6 hours using a transcutaneous bilirubinometer. The change in the bilirubin levels were recorded in a nomogram. The natural progress of jaundice in neonates was monitored using the nomogram and cases were identified that needed further follow-up observation and treatment. METHODS: The subjects of this study were 986 healthy term or near-term infants at the age of 35 weeks or older who were born at Sung-Ae General Hospital during the period from October 1, 2007 to April 30, 2009 and whose parents were both Koreans. Transcutaneous bilirubin measurements were obtained using a transcutaneous bilirubinometer (Minolta, JM-103) from 6 hours of life to discharge at intervals of 6 hours. A nomogram was derived from the obtained data and compared to the delivery method, gestational age, and feeding method. RESULTS: Percentile graphs were drawn according to time. Based on the graphs, phototherapy was necessary in more than 90 percent of the infants between 35 and 37.6 weeks of age and in 95 percent of the infants 38 weeks and older. The mean bilirubin level at 24, 48, 72 and 96 hours after birth were compared according to the delivery method, gestational age, and feeding method. The bilirubin level in 48 hours was significantly higher in neonates born via cesarean section delivery compared to the neonates born via vaginal delivery, however the levels were not statistically different at the other hours. CONCLUSION: The results of this study show the nomogram derived from hour-specific transcutaneous bilirubin levels. This information can be used to predict the risk for subsequent significant hyperbilirubinemia.
Bilirubin ; Cesarean Section ; Feeding Methods ; Female ; Follow-Up Studies ; Gestational Age ; Hospitals, General ; Humans ; Hyperbilirubinemia ; Infant ; Infant, Newborn ; Jaundice ; Jaundice, Neonatal ; Nomograms ; Parents ; Parturition ; Phototherapy ; Pregnancy

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Liver Abscess Associated with Maternal Perinatal Infection in a Premature Infant.

Ju Hee LEE ; Bong Ok SEO ; Eun Sun SEO ; Sung Mi KIM ; June Hyoung KIM

Journal of the Korean Society of Neonatology.2008;15(1):105-110.

Neonatal liver abscesses are rare, carry a high mortality rate, and are difficult to diagnose. The diagnosis of liver abscesses in the neonate cannot be established from the clinical presentation alone. Risk factors for liver abscesses in neonates are maternal infection, sepsis, umbilical venous catheterization, omphalitis, and necrotizing enterocolitis. In this report, we describe a preterm infant (32 weeks, 1,580 g) who presented with abdominal distension, respiratory difficulties, and a persistent inflammatory response in spite of broad spectrum antibiotic treatment; a large (6x5 cm) solitary pyogenic liver abscess was identified at 9 days of age. It appeared that the liver abscess had originated in the uterus and umbilical venous catheterization facilitated its spread. Percutaneous drainage under abdominal ultrasound guidance was performed and prolonged antibiotics were treated for 5 weeks, effecting a cure.
Anti-Bacterial Agents ; Catheterization ; Catheters ; Drainage ; Enterocolitis, Necrotizing ; Humans ; Infant, Newborn ; Infant, Premature ; Liver ; Liver Abscess ; Liver Abscess, Pyogenic ; Risk Factors ; Sepsis ; Uterus

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