Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea cycle. Hyperammonemic coma may develop within 2-3 days of life, although its etiology is unknown. Laboratory studies reveal marked hyperammonemia (>$4,000{\mu}mol/L$). The degree of neurologic impairment and developmental delay in this disorder depends on the duration of hyperammonemic coma. Moreover, the infant may succumb to the disease if treatment is not started immediately and continued vigorously. Hyperammonemic coma as a medical emergency requires dialysis therapy. Here, we report a case of severe transient hyperammonemia in a preterm infant (35 week of gestation) presented with respiratory distress, seizure, and deep coma within 48 hours and required ventilatory assistance and marked elevated plasma ammonia levels. He survived with aggressive therapy including peritoneal dialysis, and was followed 2 years later without sequelae.
Perez-Aguirre, Brenda;Soto-Barreras, Uriel;Loyola-Rodriguez, Juan Pablo;Reyes-Macias, Juan Francisco;Santos-Diaz, Miguel Angel;Loyola-Leyva, Alejandra;Garcia-Cortes, Obed
Clinical and Experimental Pediatrics
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v.61
no.9
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pp.279-284
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2018
Purpose: This study aimed to determine the frequency of abnormalities in the newborn oral cavity and to evaluate the association with prenatal and perinatal factors. Methods: This cross-sectional study evaluated 2,216 newborns. Oral findings were assessed in the first 24 hours of life using visual examination. Sex, weight, length, gestational age, and medical disorders at birth were recorded. Maternal demographic and medical information was also obtained. Results: The most common oral findings were Bohn's nodules, Epstein's pearls, and dental lamina cysts. Other intraoral findings included odontogenic cysts, ankyloglossia, and natal teeth, among others. In logistic regression analyses, folic acid consumption during pregnancy was significantly associated with Bohn's nodules (odds ratio [OR], 1.79; 95% confidence interval [CI], 1.23-2.55; P=0.002), Epstein's pearls (OR, 1.63; 95% CI, 1.14-2.33; P=0.007), and dental lamina cysts (OR, 1.45; 95% CI, 1.02-2.05; P=0.038). Moreover, preterm births were negatively associated with prevalence of Bohn's nodules (OR, 0.63; 95% CI, 0.50-0.80; $P{\leq}0.0001$). Comparison between newborns with and without oral inclusion cysts showed that maternal folic acid and iron intake were significantly different (P<0.05). Conclusion: Maternal folic acid and iron intake were associated with the prevalence of oral inclusion cysts.
Lee, Eui Young;Kim, Sung Shin;Park, Ga Young;Lee, Sun Hyang
Clinical and Experimental Pediatrics
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v.63
no.2
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pp.56-62
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2020
Background: Red blood cell (RBC) transfusion improves cardiorespiratory status of preterm infants by increasing circulating hemoglobin, improving tissue oxygenation, and reducing cardiac output. However, RBC transfusion itself has also been suggested to negatively affect short-term outcomes such as intraventricular hemorrhage (IVH), bronchopulmonary dysplasia (BPD), retinopathy of prematurity (ROP), and necrotizing enterocolitis (NEC) in premature infants. Purpose: This study aimed to analyze the relationship between RBC transfusion and short-term outcomes in very low birth weight (VLBW) infants (birth weight, <1,500 g). Methods: We retrospectively reviewed the medical records of VLBW infants admitted to the Soonchunhyang University Bucheon Hospital between October 2010 and December 2017. Infants who died during hospitalization were excluded. The infants were divided into 2 groups according to RBC transfusion status. We investigated the relationship between RBC transfusion and short-term outcomes including BPD, ROP, NEC, and IVH. Results: Of the 250 enrolled VLBW infants, 109 (43.6%) underwent transfusion. Univariate analysis revealed that all short-term outcomes except early-onset sepsis and patent ductus arteriosus were associated with RBC transfusion. In multivariate analysis adjusted for gestational age, birth weight and Apgar score at 1 minute, RBC transfusion was significantly correlated with BPD (odds ratio [OR], 5.42; P<0.001) and NEC (OR, 3.40; P= 0.009). Conclusion: RBC transfusion is significantly associated with adverse clinical outcomes such as NEC and BPD in VLBW infants. Careful consideration of the patient's clinical condition and appropriate guidelines is required before administration of RBC transfusions.
Present study aimed to investigate morphological change and gene expression in the gill of Cyprinus carpio after exposure against muddy water caused by riverbed disturbance with various rearing condition. The gill of C. carpio showed abnormal shapes in its secondary lamellae and a rough surface with impure debris after exposed to muddy water for 80 days. In addition, the gills showed the edema, the exfoliation of epithelial cell, and the fusion of the secondary lamellae. Using 20 ACPs, 24 differentially expressed genes (DEGs) exposed to muddy water for 80 days were identified. 17 genes among them were up-regulated, while 7 genes were down-regulated in preterm deliveries. A BLAST searches revealed that 3 genes were revealed known genes as calcium transporter 1 (TRPV6) mRNA, macha mRNA for putative puroindoline b protein, and Efnb3 protein-like. Therefore, it is considered that Efnb3 gene from gill would be a useful indicator for neurobehavioral changes in fish influenced by muddy water.
This study was conducted to examine the trend of national health insurance service use with relation to pregnancy, childbirth, and the puerperium among pregnant and postpartum women older than 35 over the last decade. A descriptive analysis was conducted, using the data which were drawn from the "nationwide claim database of Korean National Health Insurance Corporation(NHIC)". Data were composed of the total cases related to pregnancy, childbirth, and the puerperium (International Classification of Disease, $10^{th}$revision [ICD-10] codes O00-O99) from 2001 to 2008. During 2001-2008, the number of pregnant and postpartum women older than 35 had continuously increased and the percentage of them also had increased in both hospital and ambulatory care. There are similar trends in their total use of national health insurance service and total expenditure. According to demographic characteristics, there was the biggest increase of the percentage in residents in large cities, self-employed workers, ones in the highest income level. According to ICD-10 codes, there was the biggest increase of the percentage in O10-O16 (oedema, proteinuria and hypertensive disorders in pregnancy, childbirth and the puerperium). According to the major prevalent disease, there was the biggest increase of the percentage in O60 (preterm labor and delivery). Throughout the past decade, the necessity has been emphasized of supporting pregnant and postpartum women older than 35. But in maternal and child health care, they are in an early stage of development. The findings of this study would be helpful in developing the support programs for the aged pregnant and postpartum women.
In order to study twin pregnancies, a retrospective survey was carried out in Yonsei University, Severance Hospital. Twin deliveries during 1967-1976 numbered altogether 264, and their relative frequency was 1.30%. Clinical palpation in addition to auscultation and roentgenologic technique had been used in the twin diagnosis. The diagnosis was made prior to delivery in 93.18% of the cases. The deliveries took place in the 37.26th (S.D. 3.95) gestational weeks on an average. The mean weights of the infants were-A (first baby) 2416.03g. (S.D. 802.61), and B (second baby) 2299.81g. (S.D. 190.31). The most common manner of twin delivery was spontaneous vaginal delivery. Cesarean section was done in 14.39%, of which the most common indication was hypotonic uterine dysfunction (34.21 %). Low one minute Apgar scores occured more often in B twins than among A twins. Breech delivery gave low one minute Apgar scores more often than did spontaneous vertex delivery in both twins. Full term twins and infants weighing more than 2500g. had fewer low one minute Apgar scores than the preterm infants and those with low birth weight. Perinatal mortality (PNM) in the total series was 14.77% (A 12.50% and B17.05%). The most common cause of perinatal mortality was prematurity in 44.87%. The worst outcome was recorded for the age groups 15-19 and ${\geqq}$40, in which perinatal mortality were 50.00%, respectively. The perinatal mortality of both A and B infants was lowest in the group diagnosed early during antenatal care before delivery. On the basis of our findings, we wish to emphasize particularly the importance of the early diagnosis of twins.
Park, Yang Hee;Lee, Gyung Min;Yoon, Jung Min;Cheon, Enn Jung;Ko, Kyung Ok;Lee, Yung Hyuk;Lim, Jae Woo
Clinical and Experimental Pediatrics
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v.55
no.12
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pp.462-469
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2012
Purpose: In this study, we aimed to investigate the perinatal clinical conditions of very low birth weight (VLBW) infants born to mothers with pregnancy-induced hypertension (PIH) focusing on the effects of early postnatal neutropenia. Methods: We reviewed the medical records of 191 VLBW infants who were born at Konyang University Hospital, between March 2003 and May 2011. We retrospectively analyzed the clinical characteristics of the infants and their mothers and compared the incidence of perinatal diseases and mortality of the infants according to the presence or absence of maternal PIH and neutropenia on the first postnatal day. Results: Infants born to mothers with PIH showed an increased incidence of neutropenia on the first postnatal day (47.4%), cesarean delivery, and intrauterine growth restriction. When the infants born to mothers with PIH showed neutropenia on the first postnatal day, their incidence of respiratory distress syndrome (RDS) was increased (P=0.031); however, the difference was not found to be significant through logistic regression analysis. In all the VLBW infants, neutropenia on the first postnatal day was correlated with the development of RDS. The incidence of the other perinatal diseases involving sepsis and mortality did not significantly differ according to the presence or absence of neutropenia in infants born to mothers with PIH. Conclusion: In VLBW infants born to mothers with PIH, the incidence of neutropenia on the first postnatal day was increased and it was not significantly correlated with the development of perinatal diseases involving RDS, sepsis, and mortality.
Thrombocytopenic patients without detectable bound antiplatelet antibody should be diagnosed with idiopathic thrombocytopenic purpura (ITP) if no other cause of their decreased platelet count could be found. More recently the term "autoimmune thrombocytopenic purpura (ATP) has supplanted ITP since the disease is related to the production of autoantibodies against one's own platelets. This entity should not be confused with isoimmune thrombocytopenic purpura (also called alloimmune thrombocytopenic purpura). In this cases maternal antiplatelet antibodies directed against the PLA 1 antigen on the fetal platelets causes severe fetal and neonatal thrombocytopenia in a situation analogous to Rheusus disease. Antibodies to the negatively charged phospholipids, lupus anticoagulant, and anticardiolipin have been linked to adverse pregnancy events. Pregnant women possessing these antibodies have an increased risk of spontaneous abortion, stillbirths, intrauterine fetal growth retardation, preterm birth, and arterial and venous thrombosis. Antiphospholipid antibodies decrease or may even disappear between pregnancies only to recur with increased activity in a subsequent pregnancy and lead to loss. We have experienced a case of antiphospholipid syndrome associated with autoimmune thrombocytopenic purpura in patient with recurrent spontaneous abortion. So we report this case with a brief review of literatures.
A 33-year-old woman visited the emergency department presenting with fever and dyspnea. She was pregnant with gestational age of 31 weeks and 6 days. She had dysuria for 7 days, and fever and dyspnea for 1 day. The vital signs were as follows: blood pressure 110/70 mmHg, heart rate 118 beats/minute, respiratory rate 28/minute, body temperature $38.7^{\circ}C$, and oxygen saturation by pulse oximetry 84% during inhalation of 5 liters of oxygen by nasal prongs. Crackles were heard over both lung fields. There were no signs of uterine contractions. Chest X-ray and chest computed tomography scan showed multiple consolidations and air bronchograms in both lungs. According to urinalysis, there was pyuria and microscopic hematuria. She was diagnosed with community-acquired pneumonia and urinary tract infection (UTI) that progressed to severe sepsis and acute respiratory failure. We found extended-spectrum beta-lactamase producing Escherichia coli in the blood culture and methicillin-resistant Staphylococcus aureus in the sputum culture. The patient was transferred to the intensive care unit with administration of antibiotics and supplementation of high-flow oxygen. On hospital day 2, hypoxemia was aggravated. She underwent endotracheal intubation and mechanical ventilation. After 3 hours, fetal distress was suspected. Under 100% fraction of inspired oxygen, her oxygen partial pressure was 87 mmHg in the arterial blood. She developed acute kidney injury and thrombocytopenia. We diagnosed her with multi-organ failure due to severe sepsis. After an emergent cesarean section, pneumonia, UTI, and other organ failures gradually recovered. The patient and baby were discharged soon thereafter.
Journal of the Korean Society of Manufacturing Process Engineers
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v.18
no.9
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pp.1-6
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2019
Preterm children require a controlled environment that is as close as possible to that inside the womb. Incubators are well equipped to fulfill this requirement; however, they are cumbersome and expensive, thereby restricting their portability and availability in less developed and rural areas. This research comprises the development and system validation of a portable incubator. The system consists of a collapsible baby enclosure that can be stowed inside the system base when not in use. The enclosure is made from acrylic such that it is easy to clean and allows unhindered visual observation of the occupant while being robust enough to withstand transit conditions. The system can be powered either by a mains supply or a 12-VDC automobile power supply. Additionally, it has an onboard battery to ensure a continuous supply during transit. A Peltier plate controlled using a microcontroller ensures the desired enclosure temperature irrespective of the ambient temperature. Built-in sensor probes can measure the skin temperature, pulse rate, blood oxygenation level, and ECG of the infant and display them on the system screen. The system function is validated by testing its peak power consumption and the heating and cooling performances of the environment control system.
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