Measles outbreaks in the Kyeongin area of the Republic of Korea, 2013-2014: A single-center experience in a country of measles elimination

OBJECTIVE@#To identify the source of infection and determine the clinical features and laboratory finding of measles infection.@*METHODS@#In 27 measles patients, except for 3 adult patients, the rest of 24 pediatric measles cases were analyzed with regard to age, sex, immunization status, transmission routes and molecular genotyping of measles virus. Eighteen measles patients who admitted in isolation ward were set apart to investigate clinical findings and its correlation with laboratory characteristics. Retrospective analysis of cases was conducted in this study.@*RESULTS@#Of the 24 pediatric patients, 23 (95.8%) had not received any measles-containing vaccine (MCV). Sixteen of the patients (66.7%) were aged <12 months. The suspicious index case of a girl aged 34 months was not vaccinated with MCV1 and got measles after a trip to Philippines, and molecular genotype was revealed as B3. Measles outbreaks in the community such as a restaurant were followed by this one imported case. According to analysis of 18 patients admitted in isolation ward, the median level of C-reactive protein (CRP) was 0.38 mg/dL and that of lactate dehydrogenase (LDH) was 1200 IU/L. All of the 18 patients had LDH levels above the normal range. Age correlated with CRP (ρ = 0.528, P = 0.024) and LDH (ρ = 0.501, P = 0.034). The duration of fever was correlated with the duration of fever before rash (ρ = 0.898, P < 0.01). The duration of hospitalization was correlated with CRP (ρ = 0.586, P = 0.011). The white blood cell counts were correlated with the levels of LDH (ρ = 0.505, P = 0.033), aspartate aminotransferase (ρ = 0.507, P = 0.032), and alanine aminotransferase (ρ = 0.481, P = 0.043).@*CONCLUSIONS@#Early weaning of maternally derived measles antibodies therefore vaccination of MCV1 at a young age from 9 months to 12 months should be considered in situations of early exposure. Furthermore, there is a call for consideration of scheduling an earlier age for the first dose of MMR vaccine in Europe. It is necessary for Korea to investigate the duration of the presence and quantitative analysis of maternal measles antibodies in infants and to reconsider the timing of MCV1.

Measles outbreaks in the Kyeongin area of the Republic of Korea, 2013–2014: A single-center experience in a country of measles elimination

Objective To identify the source of infection and determine the clinical features and laboratory finding of measles infection. Methods In 27 measles patients, except for 3 adult patients, the rest of 24 pediatric measles cases were analyzed with regard to age, sex, immunization status, transmission routes and molecular genotyping of measles virus. Eighteen measles patients who admitted in isolation ward were set apart to investigate clinical findings and its correlation with laboratory characteristics. Retrospective analysis of cases was conducted in this study. Results Of the 24 pediatric patients, 23 (95.8%) had not received any measles-containing vaccine (MCV). Sixteen of the patients (66.7%) were aged <12 months. The suspicious index case of a girl aged 34 months was not vaccinated with MCV1 and got measles after a trip to Philippines, and molecular genotype was revealed as B3. Measles outbreaks in the community such as a restaurant were followed by this one imported case. According to analysis of 18 patients admitted in isolation ward, the median level of C-reactive protein (CRP) was 0.38 mg/dL and that of lactate dehydrogenase (LDH) was 1200 IU/L. All of the 18 patients had LDH levels above the normal range. Age correlated with CRP (ρ = 0.528, P = 0.024) and LDH (ρ = 0.501, P = 0.034). The duration of fever was correlated with the duration of fever before rash (ρ = 0.898, P < 0.01). The duration of hospitalization was correlated with CRP (ρ = 0.586, P = 0.011). The white blood cell counts were correlated with the levels of LDH (ρ = 0.505, P = 0.033), aspartate aminotransferase (ρ = 0.507, P = 0.032), and alanine aminotransferase (ρ = 0.481, P = 0.043). Conclusions Early weaning of maternally derived measles antibodies therefore vaccination of MCV1 at a young age from 9 months to 12 months should be considered in situations of early exposure. Furthermore, there is a call for consideration of scheduling an earlier age for the first dose of MMR vaccine in Europe. It is necessary for Korea to investigate the duration of the presence and quantitative analysis of maternal measles antibodies in infants and to reconsider the timing of MCV1.

Risk Factors of Recurrent Kawasaki Disease

OBJECTIVE: This study looks into the risk factors that affect the recurrence of Kawasaki disease (KD). METHODS: Among 618 child patients that have been hospitalized for the KD from July 2002 to May 2015 at the Soonchunhyang University Bucheon Hospital, 13 patients who have been hospitalized more than twice for the KD were selected as the relapse group. A comparative analysis was retrospectively conducted between the relapse group and the control group RESULTS: Among 618 child patients with KD, 13 cases (2.1%) showed recurrence in the disease. At initial episode of the relapse group, the mean age was 29±14.2 months. One year or less and 1-2 years old took up 76.8%, and 11 patients (84.6%) suffered a recurrence within 2 years after the initial episode. The relapse group, when compared to the control group, showed lower neutrophils count, and red cell distribution width (RDW) and C-reactive protein (CRP) levels were higher at initial episode. These differences were statistically significant (P<0.001, P<0.001, and P=0.015, respectively). There were no differences in other findings between the two groups. By utilizing the Youden's index, the optimal cutoff value was found, and RDW was 12% and CRP was 5.955 mg/dL. CONCLUSION: The research suggests that it is possible to predict recurrence probabilities of KD by checking RDW and CRP values during blood tests at initial episode. Children less than 1 year old and 1-2 years old with Kawasaki disease should be carefully observed for recurrence during the 2 years after the initial episode.

Postnatal weight gain in the first two weeks as a predicting factor of severe retinopathy of prematurity requiring treatment / 소아과

PURPOSE: This study aimed to investigate the relative weight gain at 2-week intervals up to 6 weeks after birth to predict retinopathy of prematurity (ROP) requiring treatment among very low birth weight infants. METHODS: A total of 211 preterm infants with birth weights <1,500 g and gestational age <32 weeks were retrospectively reviewed. The main outcome was the development of ROP requiring treatment. Body weight measurements were recorded daily. Relative weight gains (g/kg/day) were calculated at the second, fourth, and sixth week after birth. RESULTS: Of the 211 infants, 89 developed ROP, of which 41 spontaneously regressed and 48 with early treatment of ROP type I required laser treatment. The relative weight gain at 2, 4, and 6 weeks postnatal age was significantly lower in infants with ROP requiring treatment than in infants without ROP or those with spontaneous regression (P<0.001, P=0.005, and P=0.004, respectively). On logistic regression, poor relative weight gain in the first 2 weeks was found to be related to ROP requiring treatment (adjusted odds ratio, 0.809; 95% confidence interval, 0.695-0.941; P=0.006). Relative weight gain at 2 weeks postnatal age was significantly lower in infants with ROP requiring treatment compared to that in ROP requiring no treatment (P=0.012). CONCLUSION: Poor postnatal weight gain in the first 2 weeks of life is an important and independent risk factor for ROP requiring treatment. Postnatal weight gain can predict the development of severe ROP requiring treatment.

Food Protein-Induced Enterocolitis Syndrome in a Neonate with Peripheral Epimerase Deficiency Galactosemia

Food protein-induced enterocolitis syndrome (FPIES) is a severe infantile form of non-immunoglobulin E-mediated gastrointestinal food hypersensitivity that manifests as profuse, repetitive vomiting, often with diarrhea, which leads to acute dehydration and lethargy and failure to thrive if chronic. Symptoms such as dehydration and lethargy are also observed in sepsis, viral infection, and food poisoning. It is difficult to differentiate FPIES from sepsis-like illness. The diagnosis is based on clinical criteria and/or an oral food challenge. FPIES developed in the patient with peripheral epimerase deficiency galactosemia after the use of soy formula. The change in feeding to soy formula is not required of a patient with peripheral epimerase deficiency galactosemia. Early intake of soy formula in our patient was harmful. Therefore, we think the changing the formula should be taken carefully. Another important point is the diagnosis. Late diagnosis and misdiagnosis are common, and inappropriate treatment or invasive treatment can occur.

A Study of Thyroid Function in Partial Thyroxine-Binding Globulin Deficiency

OBJECTIVE: It is generally thought that thyroxine-binding globulin (TBG)-deficient individuals are euthyroid and do not require treatment. However, there have been case reports of TBG deficiency combined with hypothyroidism. The purpose of this study was to investigate the relationship between TBG deficiency and thyroid function. METHODS: We reviewed the medical records of 32 patients diagnosed with TBG deficiency between 1997 and 2008 in Soonchunhyang University Seoul Hospital. All were partial TBG deficiency. Eighteen patients had combined hypothyroidism, and 14 patients had normal thyroid function. We compared the TBG, thyroid-stimulating hormone, free thyroxine, and total triiodothyronine levels between these 2 groups. Eighteen patients with TBG deficiency with hypothyroidism started thyroxine medication and continued for 2-3 years. After, they were followed up with thyroid function tests after discontinuing medication for 4 weeks at 2-3 years of age. RESULTS: The TBG level in TBG deficiency with hypothyroidism patients was significantly lower than that in TBG deficiency with normal thyroid function (4.43+/-2.22 mg/L vs. 6.23+/-1.81 mg/L; P=0.02). The percent TBG compared with normal mean TBG level according to age in the hypothyroidism patients was also significantly lower than that of patients with normal thyroid function (13.42%+/-6.92% vs. 19.08%+/-4.87%; P=0.014). Sixteen of 18 patients diagnosed with TBG deficiency with hypothyroidism showed persistent hypothyroidism at 2-3 years of age. CONCLUSION: We conclude that TBG-deficient patients should be observed closely and undergo thyroid function testing in order not to miss hypothyroidism. More investigations of TBG deficiency and thyroid function are needed in the future.

Current Situation of Postmenopausal Grandmothers Raising Their Grandchildren

As the average life expectancy of women increases, the family and social roles of postmenopausal women have become more important. With the growing number of dual-income households, postmenopausal grandmothers occupy a large role in child-rearing. Postmenopausal women mainly experience social changes as a family member along with personal changes. Postmenopausal women face changes in physical and mental aspects due to drastic hormonal changes. Grandmothers sharing the burden of raising children are actually encountering a number of challenges while dealing with hardships to adapt to physical and mental changes at the same time. It is thought to be important to understand the impact of raising grandchildren on physical and mental conditions among grandmothers experiencing hardships between social reality and personal changes from medical perspective based on sociological studies. Focusing mainly on studies on related fields, this study aims to investigate personal and social supports from medical perspective and to device practical measures.

Metameric Arteriovenous Malformation in the Spinal Cord of a Neonate

We present a case of metameric arteriovenous malformation (AVM) in a neonate. The neonate appeared normal, except for a dimple and blue discoloration in the lower thoracic spine. Spinal ultrasonography revealed cutaneomeningiospinalangiomatosis in the thoracic spine. Spinal magnetic resonance imaging revealed a definite metameric AVM at the T10-T11 level and a cutaneous hemangioma at T11. The neonate was conclusively diagnosed with spinal metameric AVM at the T10-T11 level. The neonate was asymptomatic with no, abnormal physical or neurological complications, including urinary incontinence, fecal incontinence, or muscle weakness.

Comparison of Respiratory Morbidity in Late Preterm and Term Infants at a Single Institution

OBJECTIVE: This study was carried out to evaluate short-term respiratory morbidity in late preterm births compared with term births at a single center in Korea. METHODS: In retrospective study, we reviewed medical records of neonates that were born at Soonchunhyang University Bucheon Hospital from January 2002 to January 2011 and their mothers. The neonates were divided into two groups by their gestational age (GA): 535 late preterm births (GA, 34+0 to 36+6 weeks) and 3,037 term births (GA, 37+0 to 41+6 weeks). We compared late preterm births and term births in regard to maternal characteristics, neonatal characteristics, respiratory morbidities, and respiratory supports. RESULTS: Of 535 late preterm births, 117 (21.9%) had respiratory tract disease. Of 3,037 term births, 105 (3.5%) had respiratory tract disease. Compared term births, late preterm births had significantly more respiratory diseases such as apnea (odds ratio [OR], 37.3), respiratory distress syndrome (OR, 34.0), transient tachypnea of newborn (OR, 5.9), and pneumonia (OR, 2.2). Duration of oxygen supply was longer in term than in preterm births. Oxygen supply through isolette and use of surfactant were more frequent in late preterm than term births. It was not differ from two groups to use continuous positive airway pressure and mechanical ventilation. CONCLUSION: This study showed that late preterm births had significantly higher respiratory morbidity than term births. Therefore, we should have more attention to evaluate and manage when late preterm birth occur than before. Also it is necessary to study long-term respiratory outcomes of late preterm births and discuss about perinatal care system.

Cutaneous Neonatal Lupus Erythematosus Associated with Brain Infarction

Neonatal lupus erythematosus (NLE) is an acquired autoimmune disorder caused by the transplacental passage of maternal autoantibodies, usually anti-Ro/SSA or anti-La/SSB antibodies, and less commonly U1-ribonucleoprotein. NLE usually involves a single organ, but multiple organ involvement has also been reported. Manifestations of NLE may include cutaneous lesions, congenital heart block, hematological diseases (anemia, thrombocytopenia, neutropenia), hepatic diseases (hepatitis, hepatosplenomegaly, cholestasis), and neurological diseases. Neurological involvement is very rare in infants with NLE. Here, we report a 2-day-old female neonate, born to a clinically asymptomatic mother, presenting with cutaneous lupus lesions and brain infarction as a neurological disease.

Characteristics of Pneumothorax in a Neonatal Intensive Care Unit

PURPOSE: The development of postnatal pneumothorax and its common causes and clinical aspects were studied to promote early diagnosis and proper management. METHODS: A retrospective study of neonates who were hospitalized in the neonatal intensive care unit at Soonchunhyang University Bucheon Hospital from 2001 to 2010 was performed. Term neonates were divided into a spontaneous pneumothorax group and a secondary pneumothorax group. The secondary group was divided into term and preterm groups. RESULTS: Of 4,414 inpatients, 57 (1.3%) were diagnosed with pneumothorax. Of term newborn patients, 28 (80%) had a secondary pneumothorax, and seven (20%) had a spontaneous pneumothorax. No differences were observed for gender, birth weight, resuscitation, or duration of admission between the spontaneous and control groups. The duration of treatment with a thoracostomy (20 patients, 57%) was longer in the spontaneous group (5.4+/-2.9 days vs. 2.7+/-2.0 days) than that in the control group. Patients with respiratory distress syndrome (RDS) developed a pneumothorax 22.8 hours after surfactant treatment, whereas patients with transient tachypnea of the newborn (TTN), pneumonia, and meconium aspiration syndrome (MAS) developed pneumothorax after 16.6 hours. Of 50 patients with a secondary pneumothorax, 19 (38%) had RDS, 11 (22%) had MAS, 7 (14%) had TTN, and six (12%) had pneumonia. Among term newborns, 42.9% were treated only with 100% oxygen. Among preterm newborns, 72.6% and 27.3% needed a thoracostomy or ventilator care, respectively. CONCLUSION: A pneumothorax is likely to develop when pulmonary disease occurs in neonates. Therefore, it is important to carefully identify pneumothorax and provide appropriate treatment.

Clinical characteristics of 2009 pandemic influenza A (H1N1) infection in children and the performance of rapid antigen test / 소아과

PURPOSE: In autumn 2009, the swine-origin influenza A (H1N1) virus spread throughout South Korea. The aims of this study were to determine the clinical characteristics of children infected by the 2009 H1N1 influenza A virus, and to compare the rapid antigen and real-time polymerase chain reaction (PCR) tests. METHODS: We conducted a retrospective review of patients > or =18 years of age who presented to Soonchunhyang University Hospital in Seoul with respiratory symptoms, including fever, between September 2009 and January 2010. A real-time PCR test was used to definitively diagnose 2009 H1N1 influenza A infection. Medical records of confirmed cases were reviewed for sex, age, and the time of infection. The decision to perform rapid antigen testing was not influenced by clinical conditions, but by individual factors such as economic conditions. Its sensitivity and specificity were evaluated compared to real-time PCR test results. RESULTS: In total, 934 patients tested positive for H1N1 by real-time PCR. The highest number of patients (48.9%) was diagnosed in November. Most patients (48.2%) were aged between 6 and 10 years. Compared with the H1N1 real-time PCR test results, the rapid antigen test showed 22% sensitivity and 83% specificity. Seventy-eight patients were hospitalized for H1N1 influenza A virus infection, and fever was the most common symptom (97.4%). CONCLUSION: For diagnosis of 2009 H1N1 influenza A virus infection, the rapid antigen test was inferior to the real-time PCR test in both sensitivity and specificity. This outcome suggests that the rapid antigen test is inappropriate for screening.

A Case of Partial Trisomy 9 due to Maternal Balanced Translocation t(9;21)(q21.2;q11.2) / 대한주산의학회잡지

Trisomy 9 syndrome was first reported by Retheore in 1970 and has been rarely reported. This syndrome consists of partial and complete trisomy 9. It is characterized by growth and mental retardation, craniofacial abnormalities including microcephaly, hypertelorism, prominent nose, deep-set ears and down-slanting palpebral fissures. Congenital heart diseases and congenital dislocations of knee are also common in trisomy 9 syndrome. Here, we report a very rare case of partial trisomy 9 due to maternal balanced translocation t(9;21). He showed craniofacial abnormalities, brain malformation, cardiac defect, hydronephrosis and congenital dislocations of hip and knee joints.

A Case of Partial Trisomy 9 due to Maternal Balanced Translocation t(9;21)(q21.2;q11.2) / 대한주산의학회잡지

Trisomy 9 syndrome was first reported by Retheore in 1970 and has been rarely reported. This syndrome consists of partial and complete trisomy 9. It is characterized by growth and mental retardation, craniofacial abnormalities including microcephaly, hypertelorism, prominent nose, deep-set ears and down-slanting palpebral fissures. Congenital heart diseases and congenital dislocations of knee are also common in trisomy 9 syndrome. Here, we report a very rare case of partial trisomy 9 due to maternal balanced translocation t(9;21). He showed craniofacial abnormalities, brain malformation, cardiac defect, hydronephrosis and congenital dislocations of hip and knee joints.

A Case of Gilbert's Syndrome with Severe Neonatal Hyperbilirubinemia

Gilbert's syndrome is caused by a reduction in the activity of uridine diphosphate glucuronosyltransferase (UGT) and induces chronic, non-hemolytic unconjugated hyperbilirubinemia. It has been suggested that 3-10% of the population has Gilbert's syndrome. Commonly, Gilbert's syndrome causes mild symptoms. However, a case of Gilbert's syndrome with severe neonatal hyperbilirubinemia is presented here. The patient developed jaundice three days after birth. Five days after birth, the patient's total serum bilirubin level was 34 mg/dL. The patient received intensive phototherapy and was given oral phenobarbital. Hemolytic hyperbilirubinemia was excluded on the basis of laboratory tests. Heterozygote polymorphisms of the promoter region (-3279T>G) and exon 1 (211G>A) were found in UGT1A1 gene. After discharge, the patient did not require any further treatment. This is the first case of proven Gilbert's syndrome with severe neonatal hyperbilirubinemia in Korea.

A Case of Gilbert's Syndrome with Severe Neonatal Hyperbilirubinemia

Gilbert's syndrome is caused by a reduction in the activity of uridine diphosphate glucuronosyltransferase (UGT) and induces chronic, non-hemolytic unconjugated hyperbilirubinemia. It has been suggested that 3-10% of the population has Gilbert's syndrome. Commonly, Gilbert's syndrome causes mild symptoms. However, a case of Gilbert's syndrome with severe neonatal hyperbilirubinemia is presented here. The patient developed jaundice three days after birth. Five days after birth, the patient's total serum bilirubin level was 34 mg/dL. The patient received intensive phototherapy and was given oral phenobarbital. Hemolytic hyperbilirubinemia was excluded on the basis of laboratory tests. Heterozygote polymorphisms of the promoter region (-3279T>G) and exon 1 (211G>A) were found in UGT1A1 gene. After discharge, the patient did not require any further treatment. This is the first case of proven Gilbert's syndrome with severe neonatal hyperbilirubinemia in Korea.

Symptomatic Hepatitis A Virus Infection in a Newborn

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.

A Case of Infantile Spasms in Tuberous Sclerosis with Fetal Cardiac Tumors / 대한소아신경학회지

Tuberous sclerosis(TS), a type of neurocutaneous syndrome, is inherited in an autosomal dominant manner. Approximately 60% of children with TS have rhabdomyomas of the heart, and 40% of fetuses in whom rhabdomyomas are detected by a prenatal ultrasonography eventually end up with TS. Therefore, when multiple cardiac rhabdomyomas are detected by a fetal ultrasonography, TS should be suspected and further examination should be considered after birth. Infantile spasms is a common type of seizure among young children with TS. We describe a patient with TS who showed cardiac tumors on a fetal ultrasound. Also, hypomelanotic macules, retinal tumors, brain cortical tubers, nodules in subependymal regions, and infantile spasms was detected after birth.