No abstract available.
Endothelium* ; Hemostasis* ; Hydroxymethylglutaryl-CoA Reductase Inhibitors* ; Inflammation*

No abstract available.
Coronary Disease* ; Secondary Prevention*

Amphotericin B is widely used in the treatment of neonatal invasive fungal disease. Liposomal amphotericin B (LAmB) is now preferred over conventional amphotericin B deoxycholate due to its lower kidney toxicity. However, it is essential to monitor the possible side effects of LAmB when treating extremely premature infants. Herein, we present a case of an extremely premature infant who exhibited persistent metabolic acidosis, hypoglycemia, and dyselectrolytemia after administration of LAmB for a suspected fungal infection.

Left atrial myxoma is well-known mimicker of systemic vasculitis. In general, however, these patients showed some symptoms and signs of cardiovascular disease. We experienced a patient with left atrial myxoma who first presented as vasculitis without any symptoms and signs suggesting cardiovascular disorders. A 45 year-old lady showed tender skin nodules, livedo reticuiaris-like skin lesions, severe constitutional symptoms, multiple cerebral infarctions, and elevated ESR and globulin. Skin biopsy findings were thought to be consistent with polyarteritis nodosa. Echocardiography was performed as a routine test for evaluating suspected vasculitis patients and it revealed left atrial myxoma. However, we were not able to detect any auscultation abnormality, even after echocardiography, After echocardiography, we found the tumor emboli in skin biopy specimen with deeper section. This case demonstrated the importance of suspecting the possibility of left atrial myxoma when performing diagnostic work-up for vasculitis.
Auscultation ; Biopsy ; Cardiovascular Diseases ; Cerebral Infarction ; Echocardiography ; Humans ; Middle Aged ; Myxoma ; Polyarteritis Nodosa ; Skin ; Systemic Vasculitis ; Vasculitis

PURPOSE: To investigate the effectiveness of transient intubation for surfactant administration and extubated to nasal continuous positive pressure (INSURE) for treatment of respiratory distress syndrome (RDS) and to identify the factors associated with INSURE failure in extremely premature infants. METHODS: Eighty-four infants with gestational age less than 28 weeks treated with surfactant administration for RDS for 8 years were included. Perinatal and neonatal characteristics were retrospectively reviewed, and major pulmonary outcomes such as duration of mechanical ventilation (MV) and bronchopulmonary dysplasia (BPD) plus death at 36-week postmenstrual age (PMA) were compared between INSURE (n=48) and prolonged MV groups (n=36). The factors associated with INSURE failure were determined. RESULTS: Duration of MV and the occurrence of BPD at 36-week PMA were significantly lower in INSURE group than in prolonged MV group (P < 0.05), but BPD plus death at 36-week PMA was not significantly different between the 2 groups. In a multivariate analysis, a reduced duration of MV was only significantly associated with INSURE (P=0.001). During the study period, duration of MV significantly decreased over time with an increasing rate of INSURE application (P < 0.05), and BPD plus death at 36-week PMA also tended to decrease over time. A low arterial-alveolar oxygen tension ratio (a/APO2 ratio) was a significant predictor for INSURE failure (P=0.001). CONCLUSION: INSURE was the noninvasive ventilation strategy in the treatment of RDS to reduce MV duration in extremely premature infants with gestational age less than 28 weeks.
Bronchopulmonary Dysplasia ; Gestational Age ; Humans ; Infant ; Infant, Extremely Premature* ; Infant, Newborn ; Intubation* ; Intubation, Intratracheal ; Multivariate Analysis ; Noninvasive Ventilation ; Oxygen ; Pulmonary Surfactants ; Respiration, Artificial ; Retrospective Studies

Neonatal hyperinsulinism, whether permanent or transient, results in prolonged hypoglycemia, which increases the risk of hypoglycemic brain injury. Therefore, prompt diagnosis and management of hyperinsulinemic hypoglycemia is important. Drawing a “critical sample” at the time of hypoglycemia is useful for diagnosis. Genetic testing for defective insulin-regulating genes in pancreatic beta-cells might also be helpful in cases of prolonged hypoglycemia. High-calorie feeding or glucose infusion is necessary to maintain normoglycemia. Diazoxide is the treatment of choice for hyperinsulinism and should be continued until the hypoglycemia resolves. We describe a case of transient neonatal hyperinsulinemia hypoglycemia in a small-for-gestational-age preterm infant who underwent diazoxide treatment and achieved a favorable outcome.

PURPOSE: We present the clinical and radiological results of open reduction and internal fixation for scaphoid fracture with retrograde headless screw fixation via dorsal approach. METHODS: This study carried out a survey targeting 15 patients who have a retrograde headless screw fixation on nonunion of scaphoid fracture without previous operation, 2 patients who have a retrograde headless screw fixation on nonunion of scaphoid fracture with previous operation and 8 patients who have a trans-scaphoid perilunate dislocation. We figured out a mechanism of injury, and clinical symptom, radiologic findings. The surgery was done with open dorsal approach which is retrograde headless screw fixation internally, with or without bone graft. We analyzed the result by Maudsley method, in terms of bone union, duration for union, radiologic finding, clinical outcomes. RESULTS: After surgery, 22 of 25 patients had union result on fracture and other 3 patients had nonunion result. It took 12 weeks to achieve bone union on average. Based on radiograhs, we had one case of partial avascular necrosis of proximal fragment without clinical symptoms. We had one case of each scaphoid nonunion without previous operation, with operation and trans-scaphoid perilunate dislocation had arthritic change and non-symptomatic nonunion result. In terms of clinical outcome, 22 patients showed satisfactory results and 3 patients had slight limitation of range of motion. CONCLUSION: Retrograde headless screw fixation with or without bone graft for the treatment of scaphoid fracture is recommendable.
Dislocations ; Humans ; Necrosis ; Transplants

PURPOSE: Many studies have reported associations of early postnatal growth failure in preterm infants with several morbidities. However, the risk factors for postnatal weight loss (PWL) in late preterm infants have not been identified. We investigated the independentrisk factors for PWL in late preterm infants.METHODS: This was a retrospective cohort study. We enrolled 369 late preterm infants born at 34⁺⁰ to 36⁺⁶ weeks gestational age who were admitted to the Soonchunhyang University Cheonan Hospital between 2015 and 2017. PWL% was calculated as (birth weight–lowest weight)/birth weight×100. The infants were classified into lower (< 5%) and higher (≥10%) PWL% groups by propensity score matching for gestational age, sex, and birth weight. Perinatal risk factors were analyzed using multivariable logistic regression.RESULTS: The lower and higher PWL% groups included 62 and 31 infants, respectively. Antenatal steroids administered within 1 week before birth (odds ratio [OR], 3.26; 95% confidence interval [CI], 1.015 to 10.465; P=0.047), lower total calorie intake during days 1 to 7 (OR, 0.98; 95% CI, 0.977 to 0.999; P=0.027), and phototherapy (OR, 5.28; 95% CI, 1.327 to 21.024; P=0.018) were independent risk factors for the higher PWL%.CONCLUSION: Further studies are needed to identify the risk factors that cause high PWL% according to gestational age and short- and long-term morbidities based on the degree of PWL.
Betamethasone ; Birth Weight ; Chungcheongnam-do ; Cohort Studies ; Comorbidity ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Logistic Models ; Parturition ; Phototherapy ; Propensity Score ; Retrospective Studies ; Risk Factors ; Steroids ; Weight Loss

Neonatal diabetes mellitus (NDM) is defined as hyperglycemia that persists for more than 2 weeks and requires insulin therapy. NDM principally occurs before 6 months of age. Transient NDM (TNDM) is a clinical form of NDM that persists for a median of 12 weeks and resolves completely by 18 months. However, it may relapse as type 2 DM during early adulthood. The major causes of TNDM are mutations in chromosome 6q24 or the KCNJ11 or ABCC8 genes; the latter encode the two subunits of the pancreatic adenosine triphosphate (ATP)-sensitive potassium channel (KATP-channel). This condition responds well to oral sulfonylurea therapy. Herein, we report a neonate who was small for gestational age and exhibited TNDM symptoms. Genetic analysis revealed a nonspecific mutation in ABCC8; he was successfully treated with oral sulfonylurea.

Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (P<0.05). Comorbid conditions such as obesity, mental retardation, and central precocious puberty were also prominent in this group. In conclusion, this study showed the importance of understanding the clinical symptoms as well as genetic analysis for early diagnosis and management of congenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.
17-alpha-Hydroxyprogesterone/blood ; 46, XY Disorders of Sex Development/drug therapy/*genetics ; Adolescent ; Adrenal Hyperplasia, Congenital/drug therapy/*genetics ; Adrenal Insufficiency/*congenital/diagnosis/drug therapy/genetics ; Adrenocorticotropic Hormone/*metabolism ; Bone Development/genetics ; Child ; Child, Preschool ; DAX-1 Orphan Nuclear Receptor/genetics ; Female ; Genetic Diseases, X-Linked/drug therapy/*genetics ; Genotype ; Glucocorticoids/therapeutic use ; Humans ; Intellectual Disability/complications ; Male ; Mineralocorticoids/therapeutic use ; Obesity/complications ; Phosphoproteins/genetics ; Puberty, Precocious/complications ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics