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Continuous Renal Replacement Therapy in the Neonatal Intensive Care Unit: A Single Center Study
오문연,이병섭,오성희,장희진,도현정,김애란,김기수,이주훈,박영서,이범희,유한욱 대한신생아학회 2014 Neonatal medicine Vol.21 No.4
Purpose: Continuous renal replacement therapy (CRRT) has become an essentialmodality for the care of critically ill pediatric patients who require renal support. However, experience with CRRT in the neonatal population is not common in Korea. In this study, we aimed to investigate the clinical features, outcomes, and complicationsof CRRT in neonates in a single neonatal intensive care unit (NICU). Methods: We reviewed the medical records of 17 neonates who underwent CRRT at aNICU of a tertiary hospital. The data included demographic characteristics, diagnosis,complications, and laboratory and CRRT parameters. Results: The median age at initiation of CRRT was 6 days after birth. All patients weretreated with CRRT in continuous venovenous hemodiafiltration mode, with a mediantreatment duration of 57 hours. The main indication for CRRT was an inborn error ofmetabolism (IEM), followed by congenital renal disease and multiorgan failure. Inpatients with an IEM, the median plasma ammonia level at the CRRT initiation was1,232 μmol/L, and the mean duration until the ammonia level decreased to half of thepeak ammonia level was 7.3±2.5 hours. The overall hospital mortality rate was 41.2%. The outcomes of the 10 survivors after discharge included death (n=2), loss to followup(n=3), and survival with developmental delay (n=4). Conclusion: Although CRRT was effective in lowering the plasma ammonia level ofneonates with IEM, the associated mortality and morbidity were high. Hence, furtherstudies are needed to optimize the CRRT protocol and to establish an effective patientreferral system in Korea.
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오문연,유한욱,김준석,김자혜,조자항,이범희,김구환,최진호 대한의학유전학회 2014 대한의학유전학회지 Vol.11 No.2
Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductasedeficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiplemalformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient withSLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second andthird toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a reviewof the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This reportdescribes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient withSLOS.
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Surgical Interventions and Clinical Outcomes of Patients with Trisomy 18: a Single Center Experience
오문연,이병섭,Taehyen Cha,Jiyoon Jeong,Euiseok Jung,김애란,김기수,이범희,Han-Wook Yoo 대한주산의학회 2020 Perinatology Vol.31 No.3
Objective: The purpose of this study was to investigate the impact of surgical interventions on the survival rate of the trisomy 18 and to provide the clinical information of the developmental status of the neonatal intensive care unit (NICU) survivors. Methods: We retrospectively reviewed the medical records of the patients who were confirmed genetically as trisomy 18 in a tertiary hospital. Patients were divided into those who were treated with active surgical interventions and those who were not (the control group). Data on mortality and long- term developmental outcomes were obtained and compared between the study groups. Results: A total of 24 patients were included. All of them had major congenital anomalies with a 100% prevalence of congenital heart diseases. The overall survival rate was 75%, and 35% at one month, and one year of age, and the median survival time was 170 days. The one-year survival rate of the group of active surgical intervention (n=14) was 56% with a median survival of 398 days. Meanwhile, none in the control group (n=7) survived for more than three months. The NICU survivors were unable to stand up even after two years of age. Their cognitive and social developmental stages generally consistent with those under six months of age. Conclusions: Despite the potential survival benefits of active surgical intervention, NICU survivors with trisomy 18 had severe developmental delays. To guide early decision-making for newborns with trisomy 18, further research is needed regarding the quality of life of trisomy 18 survivors and their families.
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Long-term follow-up on Cushing disease patient after transsphenoidal surgery
정인숙,오문연,김자혜,조자향,최진호,유한욱 대한소아내분비학회 2014 Annals of Pediatirc Endocrinology & Metabolism Vol.19 No.3
Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH) production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed- up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low- dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.
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A case of Mowat–Wilson syndrome with developmental delays and Hirschsprung’s disease
유한욱,이다래,김자혜,조자항,오문연,이범희,김구환,최진호 대한의학유전학회 2014 대한의학유전학회지 Vol.11 No.2
Mowat-Wilson syndrome is an extremely rare genetic disease that is characterized by intellectual disability, facialdysmorphism, Hirschsprung’s disease, and other congenital anomalies. This disorder is caused by heterozygous mutationsor deletions in the zinc finger E-box-binding homeobox-2 gene (ZEB2). Thus far, approximately 200 cases of Mowat-Wilsonsyndrome have been reported worldwide. In Korea, only one case with a 2q22 deletion, which also affects ZEB2, has beenpreviously reported. Here, we describe a patient with Mowat-Wilson syndrome who presented with developmental delays,typical facial dysmorphism, and Hirschsprung’s disease. Molecular analysis of ZEB2 identified a novel heterozygous mutationat c.190dup (p.S64Kfs*6). To our knowledge, this is the second report of a Korean patient with Mowat-Wilson syndrome thathas been confirmed genetically.
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