• Journal of Chest Surgery
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• v.28 no.6
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• pp.565-570
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• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

• Clinical and Experimental Pediatrics
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• v.65 no.4
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• pp.153-166
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• 2022

During the coronavirus disease 2019 (COVID-19) pandemic, a novel multisystem inflammatory syndrome in children (MIS-C) has been reported worldwide since the first cases were reported in Europe in April 2020. MIS-C is temporally associated with severe acute respiratory syndrome coronavirus 2 infection and shows Kawasaki disease (KD)-like features. The epidemiological and clinical characteristics in COVID-19, KD, and MIS-C differ, but severe cases of each disease share similar clinical and laboratory findings such as a protracted clinical course, multiorgan involvement, and similar activated biomarkers. These findings suggest that a common control system of the host may act against severe disease insult. To solve the enigmas, we proposed the protein-homeostasis-system hypothesis in that every disease involves etiological substances and the host's immune system controls them by their size and biochemical properties. Also, it is proposed that the etiological agents of KD and MIS-C might be certain strains in the microbiota of human species and etiological substances in severe COVID-19, KD, and MIS-C originate from pathogen-infected cells. Since disease severity depends on the amounts of inflammation-inducing substances and corresponding immune activation in the early stage of the disease, an early proper dose of corticosteroids and/or intravenous immunoglobulin (IVIG) may help reduce morbidity and possibly mortality among patients with these diseases. Corticosteroids are low cost and an analogue of host-origin cortisol among immune modulators. This study's findings will help clinicians treating severe COVID-19, KD, and MIS-C, especially in developing countries, where IVIG and biologics supplies are insufficient.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.305-308
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• 2007

Vasoplegic syndrome occurs in $8{\sim}10%$ of patients following cardiac surgery, and this happens in part because of inducing the inflammatory response. Nitric oxide and guanylate cyclase play an important role in this response, and this is associated with increased morbidity and mortality. For our case, we administered methylene blue (MB), an inhibitor of guanylate cyclase, early after performing cardiopulmonary bypass in a patient with vasoplegic syndrome. The patient recovered immediately after MB administration and maintained an optimal blood pressure without the aid help of any vasopressors.

• Journal of Trauma and Injury
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• v.36 no.3
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• pp.249-252
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• 2023

Neuroleptic malignant syndrome (NMS) is a rare but fatal condition, with a high mortality rate. NMS is characterized by altered mental status, fever, myoclonus, autonomic dysfunctions, and elevated creatinine phosphokinase. The clinical manifestations may be confused with alcohol-related symptoms, trauma, sepsis, postoperative agitation, or malignant hyperthermia. A 69-year-old male patient with alcohol withdrawal was admitted to the operating theatre to rule out septic shock due to mesenteric injury after multiple trauma. He was suspected NMS with abrupt increase body temperature to 41.7℃ after haloperidol administration. Active cooling and rapid fluid infusion was done during anesthesia. Delayed diagnosis and treatment of NMS lead to catastrophic result. Therefore, if the patient's past medical history is unknown or clinical symptoms develop that are suggestive of NMS, early treatment must be considered.

• Clinical and Experimental Pediatrics
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• v.63 no.7
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• pp.239-250
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• 2020

The novel coronavirus disease 2019 (COVID-19) is spreading globally. Although its etiologic agent is discovered as severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), there are many unsolved issues in COVID-19 and other infectious diseases. The causes of different clinical phenotypes and incubation periods among individuals, species specificity, and cytokine storm with lymphopenia as well as the mechanism of damage to organ cells are unknown. It has been suggested that in viral pneumonia, virus itself is not a direct cause of acute lung injury; rather, aberrant immune reactions of the host to the insults from viral infection are responsible. According to its epidemiological and clinical characteristics, SARS-CoV-2 may be a virus with low virulence in nature that has adapted to the human species. Current immunological concepts have limited ability to explain such unsolved issues, and a presumed immunopathogenesis of COVID-19 is presented under the protein-homeostasis-system hypothesis. Every disease, including COVID-19, has etiological substances controlled by the host immune system according to size and biochemical properties. Patients with severe pneumonia caused by SARS-CoV-2 show more severe hypercytokinemia with corresponding lymphocytopenia than patients with mild pneumonia; thus, early immunomodulator treatment, including corticosteroids, has been considered. However, current guidelines recommend their use only for patients with advanced pneumonia or acute respiratory distress syndrome. Since the immunopathogenesis of pneumonia may be the same for all patients regardless of age or severity and the critical immune-mediated lung injury may begin in the early stage of the disease, early immunomodulator treatment, including corticosteroids and intravenous immunoglobulin, can help reduce morbidity and possibly mortality rates of older patients with underlying conditions.

• Tuberculosis and Respiratory Diseases
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• v.79 no.4
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• pp.214-233
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• 2016

There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS). We generate strong (1) and weak (2) grade of recommendations based on high (A), moderate (B) and low (C) grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A) and prone position if it is not contraindicated (1B) to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B) and inhaled nitric oxide (1A) as a standard treatment. We also suggest high positive end-expiratory pressure (2B), extracorporeal membrane oxygenation as a rescue therapy (2C), and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B). The application of recruitment maneuver may reduce mortality (2B), however, the use of systemic steroids cannot reduce mortality (2B). In mechanically ventilated patients, we recommend light sedation (1B) and low tidal volume even without ARDS (1B) and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B). Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A). In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.404-408
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• 2003

Shaken baby syndrome is a serious form of child abuse, mostly involving children younger than 2 years. It results from extreme rotational cranial acceleration induced by violent shaking. The characteristic injuries include subdural hemorrhage, retinal hemorrhage, and fracture of ribs or long bones. If physicians have no recognition of, or suspicion about, shaken baby syndrome, this syndome is difficult to diagnosis because of a lack of obvious external signs and failure of the abuser to admit his or her actions. In addition to the high mortality, 60% of survivors have significant long term neurologic and developmental abnormality. The authors experienced five cases of shaken baby syndrome presented with seizures or vomiting, without external signs of trauma. All of these cases had subdural hemorrhages, and four cases had retinal hemorrahges. In our follow up, two children were found to have sequelae such as quadriplegia, monoplegia, and developmental delay. We emphasize that early recognition and prompt treament are key to overall success of case management. The incidence of shaken baby syndrome can be reduced through public awareness and education of parents not to shake a babies.

• Tuberculosis and Respiratory Diseases
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• v.84 no.2
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• pp.125-133
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• 2021

Background: The coronavirus disease (COVID-19) can manifest in a range of symptoms, including both asymptomatic systems which appear nearly non-existent to the patient, all the way to the development of acute respiratory distress syndrome (ARDS). Specifically, COVID-19-associated pneumonia develops into ARDS due to the rapid progression of hypoxia, and although arterial blood gas analysis can assist in halting this deterioration, the current environment provided by the COVID-19 pandemic, which has led to an overall lack of medical resources or equipment, has made it difficult to administer such tests in a widespread manner. As a result, this study was conducted in order to determine whether the levels of oxygen saturation (SpO₂) and the fraction of inhaled oxygen (FiO₂) (SF ratio) can also serve as predictors of ARDS and the patient's risk of mortality. Methods: This was a retrospective cohort study conducted from February 2020 to Mary 2020, with the study's subjects consisting of COVID-19 pneumonia patients who had reached a state of deterioration that required the use of oxygen therapy. Of the 100 COVID-19 pneumonia cases, we compared 59 pneumonia patients who required oxygen therapy, divided into ARDS and non-ARDS pneumonia patients who required oxygen, and then investigated the different factors which affected their mortality. Results: At the time of admission, the ratios of SpO₂, FiO₂, and SF for the ARDS group differed significantly from those of the non-ARDS pneumonia support group who required oxygen (p<0.001). With respect to the predicting of the occurrence of ARDS, the SF ratio on admission and the SF ratio at exacerbation had an area under the curve which measured to be around 85.7% and 88.8% (p<0.001). Multivariate Cox regression analysis identified that the SF ratio at exacerbation (hazard ratio [HR], 0.916; 95% confidence interval [CI], 0.846-0.991; p=0.029) and National Early Warning Score (NEWS) (HR, 1.277; 95% CI, 1.010-1.615; p=0.041) were significant predictors of mortality. Conclusion: The SF ratio on admission and the SF ratio at exacerbation were strong predictors of the occurrence of ARDS, and the SF ratio at exacerbation and NEWS held a significant effect on mortality.

• 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.