Lee, Sang Hyun;Shin, Jeong Hee;Choi, Byung Min;Kim, Yun-Kyung
Pediatric Infection and Vaccine
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v.23
no.1
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pp.72-76
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2016
Infantile osteopetrosis is a rare congenital disorder caused by abnormal bone resorption. Patients with osteopetrosis can have severe anemia, thrombocytopenia, hepatosplenomegaly, rickets, visual impairment, and deafness. Cytomegalovirus also can cause a congenital infection with anemia, thrombocytopenia, hepatosplenomegaly, and calcifications in the brain. We report a 38-day-old infant with severe hepatosplenomegaly, thrombocytopenia, hypocalcemia, and growth failure. Real time polymerase chain reaction detected cytomegalovirus in the plasma. Skeletal radiography revealed generalized bone sclerosis. He was diagnosed with osteopetrosis along with cytomegalovirus infection. Only the test for mutation of the CLCN7 gene, representing the most common and heterogeneous form of osteopetrosis, was available, and the result was negative. With supportive care and antiviral treatment, severe thrombocytopenia due to the cytomegalovirus infection almost normalized despite the possible immunosuppression caused by osteopetrosis. We present the first report of an infant who suffered from osteopetrosis and CMV infection which was successfully treated by long term antiviral agent therapy.
In human, sudden infant death syndrome(SIDS) is synonyms for the sudden, unexpected and unexplained death of an infant. The incidence of SIDS has been estimated to be from 1 to 3%. Cloning has a relatively high rate of late abortion and early postnatal death, particularly when somatic cells are used as donors of nuclei and rates as high as 40 to 70% have been reported. However, the mechanisms for SIDS in cloned animals are not known yet. To date, few reports provide detailed information regarding phenotypic abnormality of cloned pigs. In this study, most of the cloned piglets were alive at term and readily recovered respiration. However, approximately 82% of male cloned piglets (81/22) died within a week after birth. Significant findings from histological examinations showed that 42% of somatic cloned male piglets died earlier than somatic cloned female piglets, most probably due to severe congestion of lung and liver or neutrophilic inflammation in brain, which indicates that unexpected phenotypes can appear as a result of somatic cell cloning. No anatomical defects in cloned female piglets were detected, but three of the piglets had died by diarrhea due to bacterial infection within 15 days after birth. Although most of male cloned piglets can be born normal in terms of gross anatomy, they develop phenotypic anomalies that include leydig cell hypoplasia and growth retardation post-delivery under adverse fetal environment and depigmentation of hair- and skin-color form puberty onset. This may provide a mechanism for development of multiple organ system failure in some cloned piglets. Th birth weights of male cloned pig in comparison with those of female cloned piglets are significantly reduced(0.8 vs 1.4kg) and showed longer gestational day(120 vs 114). In conclusion, brain meningitis and hepatopneumonic congestion are a major risk factor for SIDS and such pregnancy in cloned animals requires close and intensive antenatal monitoring.
Anthropometric and body compositional changes and the outcomes of the pregnancies of 90 healthy Korean women were investigated in a longitudinal study. Their weight increased from 51.3$\pm$5.9kg to 65.1$\pm$7.8kg during their pregnancies. The total weight gain was 13.8$\pm$4.5kg, and therefore, weekly weight gain was 340$\pm$110g during the entire period of the pregnancy. The weight gain was composed of approximately 50-60% fat mass and 40-50% fat-free mass. Skinfold thicknesses, both of triceps and subscapular, increased during the pregnancies. The fat mass calculated from skinfold thickness and that measured with bioelectrical impedance analysis went on increasing during the pregnancies. Although there was a considerable difference with respect to the fat mass observed using the three methods, fat mass gain was 5.0-6.1kg and fat-free mass gain was 4.0-5.3kg from the first trimester to the third trimester of pregnancies. There were significant correlations between maternal anthropometric parameters and indices of pregnancy outcomes. Especially, the infant's birth weight was associated with maternal pre-pregnancy weight and weight gains during the pregnancies. The infant's birth length was related to the maternal weight observed at term(p<0.05) and weight gain during the entire pregnancies (p<0.05) . Neither increase of fat mass nor fat-free mass affected the outcomes of pregnancy. These results show that maternal weight gain during pregnancy is led by increments of approximately above 50% fat mass. The fat mass increase seems to be larger in central areas than in subcutaneous areas. Maternal weight gain during pregnancy, especially during late pregnancy, is a factor affecting the birth weight and length of infant. On the basis of the body compositional changes, it can be predicted that the additional energy requirement for pregnancy in Korean women is more than 200-230 MJ (64,500-76,250kca1). (Korean J Nutrition 31(6) : 1057-1065, 1998)
Chronic hepatitis B viral (HBV) infection remains a major health threat, especially in high-prevalence areas. Most infants infected by mother-to-infant HBV transmission become chronic carriers. In Taiwan, many important preventive interventions have been implemented to block the perinatal transmission of HBV in the past 35 years. The first nationwide universal HBV vaccination program was launched in Taiwan in July 1984. The three-dose HBV vaccine completion rate reached 98.1% in 2018. The prevalence of Hepatitis B surface antigen (HBsAg) decreased from 9.8% in pre-vaccinated period in 1984 to 0.5% in the vaccinated cohort in 2014. The incidence of hepatocellular carcinoma in children aged 6-9 years significantly declined from 0.52 to 0.13 per 100,000 children born before and after 1984, respectively. Furthermore, we have performed a maternal HBV screening program during pregnancy since 1984, with the screening rate peaked at 93% in 2012. The HBsAg- and HBeAg-seropositive rate in pregnant women declined from 13.4% and 6.4% in 1984-1985 to 5.9% and 1.0% in 2016, respectively. To closely control perinatal HBV infection, we have administered hepatitis B immunoglobulin immediately after birth and checked the serum level of HBsAg and anti-HBs in high-risk babies born to HBsAg-seropositive mothers, irrespective of their HBeAg status, since July 2019. We have also adopted short-term antiviral treatments such as tenofovir 300 mg daily in the third trimester for highly viremic mothers and reduced the perinatal infection rates from 10.7 to 1.5%. Through all these efforts, we expect to meet the global goal of eliminating HBV infection by 2030.
Failure to thrive (FTT) is a term generally used to describe an infant or child whose current weight or rate of weight gains is significantly below that expected of similar children of the same age, sex and ethnicity. It usually describes infants in whom linear growth and head circumference are either not affected, or are affected to a lesser degree than weight. FTT is a common problem, usually recognized within the first 1-2 years of life, but may present at any time in childhood. Most cases of failure to thrive involve inadequate caloric intake caused by behavioral or psychosocial issues. The most important part of the outpatient evaluation is obtaining an accurate account of a child's eating habits and caloric intake. Routine laboratory testing rarely identifies a cause and is not generally recommended. FTT, its evaluation, and its therapeutic interventions are best approached by a multi-disciplinary team includes a nutritionist, a physical therapist, a psychologist and a gastroenterologist. Long term sequelae involving all areas of growth, behavior and development may be seen in children suffering from FTT. Early detection and early intervention by a multidisciplinary team will minimize its long term disadvantage. Appropriate nutritional counseling and anticipatory guidance at each well child visit may help prevent some cause of FTT.
Purpose: An exploratory study was done to examine the validity of the new Ballard scale with extended scoring system(eNBS) in estimating gestational age(AG) in full-term newborns. Method: The eNBS scoring system was extended to include all numbers of total score of NBS and GA to allow a 3-days variation in GA estimatio compared to the original scale which has a 2-week variation due to the application of a 5-score interval for the total NBS score and only even numbers for GA. GA by eNBS(GA-eNBS) was compared with GA by LMP(GA-LMP) and GA by standard NBS(GA-sNBS) in 133 full-term newborns. Difference between GA-LMP and GA-eNBS was analyzed for each GA. Results: Positive correlations were observed in GA-sNBS and GA-eNBS with GA-LMP. There was no difference between GA-LMP and GA-eNBS at 39GA and 40GA. At 37GA and 38GA, GA-eNBS overestimated GA-LMP up to 1 week, while underestimating up to 1 week at 41GA. Conclusions: The accuracy of eNBS was validated within 3 days of variation in GA estimation at 39-40GA. Overestimation by eNBS suggests the possible acceleration of fetal maturity in premature newborns, while underestimation, of the deceleration of fetal maturity in postterm newborns.
Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.
Purpose: This study was to investigate the effects of non-nutritive sucking on physiological and behavioral state of pre-term infants during tube feeding. Method: This nonequivalent, non-synchronized experimental study included 50 pre-term infants. An attempt was made to match gestational age and birth weight of infants in each group. Infants in the experimental group were given a pacifier 2 minutes before, during, and for 2 minutes after tube feeding. Infants in the control group did not get a pacifier. Both groups were tested at three stages for changes in the physiologic state and behavioral state - 2 minutes before, during, and 2 minutes after feeding. Date was analyzed with SPSS WIN 10.0 using an $X^2$-test, t-test, and repeated measures ANOVA. Result: Heart rates and oxygen saturation levels of the two groups were significantly different(P=.001, P=.000). The behavioral states of the two groups were significantly different during and post feeding(P=.000, P=.000). Conclusion: This result suggests non-nutritive sucking by using a pacifier is an effective intervention for pre-term infants during tube feeding.
Kim, Shin-Hye;Hur, Hae Young;Lee, Kyu Hyoung;Moon, Ja Young;Chae, Kyu Young
Clinical and Experimental Pediatrics
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v.50
no.8
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pp.746-752
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2007
Purpose : Total hydroperoxide (TH), free radical-mediated oxidation product can be used as a measure of free radical injury. The aim of the present study was to see if preterm newborns are at increased risk for oxidative stress compared with term newborns, and to determine whether oxidative stress during postnatal first 1 week is associated with clinical outcomes in preterm infants. Methods : Serum TH levels of preterm infants (n=39) were compared with those of term infants (n=24) on the postnatal day 1. Among the preterm infants, serum TH levels of uncomplicated group (n=23) were also compared with those of complicated group (n=16) who developed oxygen radical related diseases on the postnatal day 1 and 7. Retrospective analysis was performed to find out risk factors for oxygen radical injuries based on birth history, laboratory data, neuroimaging findings and clinical progress in two preterm groups. Results : Serum TH levels on postnatal day 1 were higher in the preterm infant group than the term infant group. Serum TH levels on postnatal day 1 in the complicated preterm infant group were significantly higher compared with uncomplicated group, but there was no significant difference in serum TH levels on postnatal day 7. Also, there was no significant difference in serum TH levels between uncomplicated preterm infants and term infants. Serum TH level on postnatal day 1 was independently associated with higher morbidity after adjusting for gestational age, Apgar score (5 min), arterial blood gas analysis. Conclusion : Complicated preterm newborns are at increased risk for oxidative stress compared with uncomplicated newborns and term newborns. Oxidative injury during the prenatal or postnatal day 1 is associated with adverse outcomes in preterm infants. Elevated TH levels on postnatal day 1 may have a value to predict clinical outcomes in preterm infants.
The study aims to investigate the clinical utility of Bayley-III using US norm in Korea. A total of 98 preterm infants and 93 term infants were assessed with the K-Bayley-III. The performance pattern of preterm infants was analyzed with mixed design ANOVA which examined the differences of scaled scores and composite scores of Bayley-III between full term- and preterm- infant group and within preterm infants group. Then, We have investigated agreement between classifications of delay made using the BSID-II and Bayley-III. In addition, ROC plots were constructed to identify a Bayley-III cut-off score with optimum diagnostic utility in this sample. The results were as follows. (1) Preterm infants have significantly lower function levels in areas of 5 scaled scores and 3 developmental indexes compared with infants born at term. Significant differences among scores within preterm infant group were also found. (2) Bayley-III had the higher scores of the Mental Development Index and Psychomotor Developmental Index comparing to the scores of K-BSID-II, and had the lower rates of developmental delay. (3) All scales of Bayley-III, Cognitive, Language and Motor scale had the appropriate level of discrimination, but the cut-off composite scores of Bayley-III were adjusted 13~28 points higher than 69 for prediction of delay, as defined by the K-BSID-II. It explains the lower rates of developmental delay using the standard of two standard deviation. This study has provided empirical data to inform that we must careful when interpreting the score for clinical applications, identified the discriminating power, and proposed more appropriate cut-off scores. In addition, discussion about the sampling for making the Korean norm of Bayley-III was provided. It is preferable that infants in Korea should use our own validated norms. The standardization process to get Korean normative data must be performed carefully.
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