Neonatal Hemolytic Disease Caused by Anti-e and Anti-C Antibodies Treated with Intensive Phototherapy: A Case Report

ABO and Rh incompatibility are the leading causes of hemolytic disease of the fetus and newborn (HFDN). Due to the high antigenicity of the D antigen, hemolysis and symptoms progress rapidly. However, minor blood group incompatibility manifests varying clinical symptoms, from asymptomatic cases to neonatal jaundice and fetal hydrops. This study presents a case of HFDN in which anti-C and anti-e antibodies were identified and treated with intensive phototherapy. A full-term infant weighing 3,100 g at birth, with no complications during delivery, presented with jaundice and was admitted for intensive phototherapy. Antibody testing detected anti-C and anti-e antibodies in the neonate and the mother. The patient responded well to phototherapy, and intravenous immunoglobulin was administered. The total bilirubin levels decreased, and the infant was discharged after 5 days. At the age of 12 months, the infant exhibited normal neurodevelopment. In conclusion, neonates with HFDN, due to rare minor blood incompatibility, specifically anti-C and anti-e antibodies, can mitigate hyperbilirubinemia using phototherapy. Future research should also consider the severity of the minor blood group incompatibility.

Proper Depth of Percutaneous Central Venous Catheter via the Great Saphenous Vein for Very Low Birth Weight Infants: A Single-Center, Prospective Cohort Study

Background@#A proper depth of percutaneous central venous catheter (PCVC) is very important to reduce procedural time and prevent various complications in very low birth weight (VLBW) infants who require minimal handling or have a sensitive skin. The objective of this study was to suggest a formula for faster and proper insertion of PCVC in VLBWIs to prevent unintended consequences of patients’ conditions. @*Methods@#Prospective data of VLBW infants admitted from June 2015 to January 2018 who had PCVC inserted via the great saphenous vein within seven days after birth were analyzed.Correlations of length of inserted PCVC with body weight, body length, and postmenstrual age at the date of PCVC insertion were determined with a linear regression analysis. Using results of this analysis, a formula to determine the optimal insertion length of PCVC was derived. Coefficient of determination was used to assess how well outcomes were replicated by the formula. @*Results@#The formula to predict the proper insertion length of PCVC via the great saphenous vein at popliteal crease level was obtained as follows: Optimal Length (cm) = 3.8 × Body Weight (kg) + 11.1. With everyday movements such as flexion and extension of the lower extremities, the mean difference in catheter tip position was 7.0 ± 3.9 mm, which was not significant enough to escalate the risk of catheter tip displacement. The rate of catheterrelated complications was as low as 4.9% in this study. @*Conclusions@#The formula derived from this study to predict the optimal PCVC insertion length could benefit VLBW infants by reducing procedural time and lowering the risk of complications.

Plan for Establishing a Safe Perinatal Transport System

The collapse of perinatal healthcare is inevitable considering demographic factors and factors associated with medical resources due to the rapid decrease in the fertility rate in South Korea. In order to maintain the perinatal healthcare system, an appropriate healthcare delivery system must be established, but even the regional hospitals are facing a situation in which medical care is disabled due to a shortage of workforce. Government support is required to prevent the collapse of perinatal healthcare as public healthcare, emergency healthcare, and national essential healthcare system. In reality, it is necessary to strengthen perinatal healthcare through regionalization and to establish a maternal and child transport system. How­ever, there are no guidelines for the perinatal transport system in South Korea, which also lacks professionalism, with a focus on the simple transportation of patients. Therefore, by integrating the transport systems of advanced countries and domestic efforts to improve transport systems, a realistic perinatal transport system establishment plan is proposed as follows. In order to strengthen the perinatal healthcare center under legal and institutional support, a professional workforce equivalent to that of the emergency healthcare center is ensured to organize a specialized perinatal transport team, and a perinatal emergency information center is established to form a safe transport linkage system. The perinatal emergency information center, which provides information on perinatal healthcare, linking and evaluating transportation between hospitals, should be incorporated into the emergency healthcare system, playing a pivotal role in the perinatal emergency healthcare management system. In addition, a dedicated transport team for perinatal healthcare should be formed for each region, transport guidelines for transport vehicles and equipment should be established, and legal and financial support should be provided to ensure safe perinatal healthcare transport. The prehospitalization level perinatal transport is configured as an integrated model of the 119 paramedics, aiming at quick transport. A team of perinatal special paramedics should be organized for each base to transport patients by special vehicles, and information from the perinatal emergency information center should be used to select a transfer hospital and to receive medical guidance from a perinatal care physician during transport between hospitals. Transport between hospitals aims at safe transport with triangular transport. A perinatal transport team is in charge of transport between hospitals, and a specialist is on board in a special ambulance to provide medical care during transport between hospitals. Support from the government and local governments is absolutely necessary to strengthen the perinatal healthcare center and establish a perinatal transfer system, which must be realized through a pilot project with fee support.

Sporadic Cleidocranial Dysplasia in a Newborn: A 4-Year Follow-up Study

Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplastic disease associated with mutations in the gene encoding the Runt-related transcription factor 2 (RUNX2). CCD is characterized by hypoplastic clavicles and patent cranial sutures. Management is essential to prevent complications during growth of the patient. Herein, we report a sporadic case of an infant with hypoplastic skull and clavicles at birth, which correlated with clinical findings of CCD. A heterozygous mutation was identified in the RUNX2 gene, which confirmed the diagnosis of CCD.

Association between Serum Hyponatremia and Severity of Respiratory Symptoms in Infants with Respiratory Syncytial Virus Infection

Purpose@#Association between hyponatremia and the severity of respiratory symptoms in infants with respiratory syncytial virus (RSV) infection has not yet been studied. This study aimed to compare respiratory symptoms, assessed using the Pediatric Respiratory Score (PRS), in infants with RSV infection, with or without hyponatremia. @*Methods@#RSV­positive patients aged <12 months who were admitted with respiratory symptoms within 7 days of onset at Jeonbuk National University Children’s Hospital from January 2016 to December 2019 were retrospectively analyzed. Each patient was categorized into those with or without hyponatremia (serum sodium concentration of <136 mmol/L). Clinical findings included PRS on the day of admission. @*Results@#The mean±standard deviation age of the 125 patients included in the study was 2.7±3.3 months, and, 20 patients (16.0%) showed hyponatremia. Infants with RSV infection and hyponatremia had lower birth weights, longer hospital stays, and higher blood urea nitrogen level. The C­reactive protein level was significantly higher in the hyponatremic infants, who had higher PRSs. The non­hyponatremia group had more normal PRSs than the hyponatremia group, which had more severe PRSs. After adjustment for age at admission, blood urea nitrogen level (OR, 1.218; 95% CI, 1.023 to 1.451; P<0.05), and PRS grade (OR, 2.885; 95% CI, 1.158 to 7.187; P< 0.05) were identified as independent risk factors. @*Conclusion@#Hyponatremia was strongly associated with respiratory severity in infants with RSV. Therefore, infants admitted with RSV infection who show higher PRS grade need to be evaluated and treated for hyponatremia.

A Case of Neonatal Pneumomediastinum with Subcutaneous Emphysema Suspected to Be Caused by Pharyngoesophageal Injury

Iatrogenic esophageal perforation is a rare condition in children, but occasionally occurs in premature infants due to repeated intubation or nasogastric tube insertion. Esophageal perforation is common in pneumothorax and interstitial emphysema, but rarely occurs in the absence of pneumothorax. Although complications, such as mediastinitis, after esophageal perforation are rare, they can be fatal. Therefore, rapid and accurate diagnosis and appropriate treatment are important. The authors report a case of huge retrocardiac pneumomediastinum and subcutaneous emphysema suspected to be caused by esophageal perforation after repeated intubation.

Sporadic Cleidocranial Dysplasia in a Newborn: A 4-Year Follow-up Study

Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplastic disease associated with mutations in the gene encoding the Runt-related transcription factor 2 (RUNX2). CCD is characterized by hypoplastic clavicles and patent cranial sutures. Management is essential to prevent complications during growth of the patient. Herein, we report a sporadic case of an infant with hypoplastic skull and clavicles at birth, which correlated with clinical findings of CCD. A heterozygous mutation was identified in the RUNX2 gene, which confirmed the diagnosis of CCD.

Association of Fasting Glucose Level with Neutrophil-Lymphocyte Ratio Compared to Leukocyte Count and Serum C-Reactive Protein / 가정의학회지

BACKGROUND: The neutrophil-lymphocyte ratio (NLR) is often used as an inflammatory marker in chronic diseases such as cancer or cardiovascular diseases. However, there are few studies about the association between the NLR and diabetes mellitus (DM) or impaired fasting glucose (IFG) patients in Korea. This study investigated the association between the fasting plasma glucose (FPG) level and NLR in Koreans. METHODS: This cross-sectional retrospective study included 3,219 healthy subjects who visited Konyang University Hospital in South Korea for regular health examinations. Participants with a history of insulin administration, anti-diabetic drugs, anti-inflammatory drugs, or underlying diseases related to inflammation were excluded. RESULTS: All statistical evaluation was performed by dividing participants into males and females. Based on FPG levels, the subjects were classified into three groups, with normal fasting glucose (n=1,969), IFG (n=1,138), and DM (n=122). The NLR had no significant mean differences among these groups for both sexes. Multiple linear regression analysis between FPG level and NLR showed an independent and significantly negative association (β±standard error, −0.67±0.24; P=0.006) in normal subjects after adjustment. Log(serum C-reactive protein [S-CRP]) showed an independently and significantly positive association with FPG in male IFG/DM patients. Total leukocyte (white blood cell [WBC]) showed an independently and significantly positive association with FPG in female IFG/DM patients. CONCLUSION: In normal subjects, NLR shows an independently and significantly negative association with FPG. In IFG/DM patients, NLR was not significantly related to FPG. WBC count in female patients and S-CRP level in male patients were significantly positively associated with FPG only in IFG/DM.

The clinical characteristics and prognosis of subgaleal hemorrhage in newborn / 소아과

PURPOSE: Subgaleal hemorrhage (SGH) is a rare but potentially fatal condition in newborns; however, few studies have reported on this condition. We aimed to identify the clinical characteristics and prognostic factors of SGH. METHODS: We retrospectively reviewed the medical records of 20 neonates diagnosed with SGH between January 2000 and June 2017. Enrolled neonates were clinically diagnosed when they had tender fluctuant scalp swelling that crossed the suture lines. RESULTS: Among 20 neonates with SGH, 12 were boys and 7 were girls; median hospitalization duration was 9.7±6.9 days. Fourteen neonates (70%) were born via vacuum-assisted vaginal delivery, and 4 via vacuum-assisted cesarean section. Of the neonates enrolled, half of them initially showed unstable vital signs, including apnea, desaturation, and cyanosis. Ten neonates had acidosis and 3 had asphyxia (pH < 7.0). Intracranial lesions associated with SGH were observed in 15 neonates (75%), including subdural hemorrhage (50%), subarachnoid hemorrhage (15%), intraventricular hemorrhage (5%), cerebral infarct (15%), skull fracture (30%), and cephalohematoma (20%). Twelve neonates (60%) required transfusion, 5 (25%) had seizures, and 3 (15%) died. Eight neonates (40%) had hyperbilirubinemia (mean total bilirubin, 13.1±7.4). The mean follow-up period was 8.4±7.5 months. At follow-up, 10 neonates (58.8%) were healthy with normal development, whereas 7 (41.2%) had neurological deficits. CONCLUSION: The morbidity rate was 41.2% due to severe metabolic acidosis. Anemia, hyperbilirubinemia, low Apgar scores, and subdural hemorrhage did not affect the prognosis. The long-term outcomes of neonates with SGH are generally good. Only arterial blood pH was significantly associated with death.

The supraclavicular artery island flap: a salvage option for head and neck reconstruction / 대한악안면성형재건외과학회지

BACKGROUND: Some of head and neck cancer patients are in compromised general condition after ablation surgery and chemoradiation therapy, which makes secondary free tissue transfer quite challenging. Elderly cancer patients also have some risk for microvascular surgery with lengthened general anesthesia. In those cases, the pedicled flap vascularized by supraclavicular artery could be considered as an alternative to free flap. Despite several authors have demonstrated the clinical reliability of supraclavicular artery island flap (SCAIF), to date, SCAIF has not been widely used among reconstructive surgeon. In this article, we clarified vascular flow pattern and introduce simple surgical technique of SCAIF with a literature review. CASE PRESENTATION: Three patients who had underwent previous neck surgery and adjuvant therapy received maxillofacial reconstruction using SCAIF. It required only a few landmarks, flap harvesting was carried out, and the elapsed time gradually decreased to 15 min with experiences. There were no remarkable morbidities in both donor and recipient sites. CONCLUSION: SCAIF exhibited minimal anatomic variations and short learning curve of surgical techniques, which might be valuable reconstruction modality for beginning surgeon. And it can be beneficial option for the patients with vessel-depleted neck, medically compromised status for lengthened general anesthesia and failed free tissue transfer.

Does Obesity Affect Clinical and Radiological Outcomes in Minimally Invasive Total Knee Arthroplasty? Minimum 5-Year Follow-up of Minimally Invasive TKA in Obese Patients

BACKGROUND: The aim of this study was to evaluate the clinical and radiological outcomes of minimally invasive total knee arthroplasty (MIS-TKA) in obese patients. METHODS: We examined the records of 371 cases of MIS-TKA performed using the mini-midvastus approach from January 2006 to December 2006. According to body mass index (BMI), the cases were classified into group A (BMI 0.05). There was no difference in terms of the accuracy of the tibial implant alignment, with 97.6%, 95.2%, and 93.4% of each group showing 90°± 3° varus angulation (p > 0.05). With respect to the accuracy of the femorotibial angle, 93.9%, 94.6%, and 90.2% of each group had 6°± 3° valgus angulation, with group C demonstrating the lowest level of accuracy (p 0.05). CONCLUSIONS: MIS-TKA in obese patients showed satisfactory clinical and radiological results without significant difference in surgical results compared to nonobese patients.

Relationship between coronary atherosclerosis in coronary computed tomography angiography and serum vitamin D level

OBJECTIVES: Vitamin D deficiency has been shown to influence the development of some cardiovascular disease. In this study, the association between the existence of coronary artery plaque and vitamin D was examined among participants who were not previously diagnosed with coronary artery disease. METHODS: A total of 339 participants (246 men and 93 women) who visited a health examination center for check-up including blood test for serum vitamin D level and coronary computed tomography angiography (CCTA) were selected for this study. RESULTS: Among the total 339 participants, 106 displayed coronary artery plaques. The serum 25-hydroxy vitamin D (25(OH)D) level of the group with plaque was lower than that of the group without (17.7 ± 7.72 ng/mL vs. 19.6 ± 7.12 ng/mL, P = 0.0316). The group with plaque had higher incidence rates of diabetes mellitus, hypertension, and dyslipidemia than that without (P = 0.0078, P = 0.0065, and P = 0.0174, respectively). The former displayed higher serum glucose and glycated hemoglobin levels than the latter (P = 0.0055 and P = 0.0137, respectively). The group with plaque showed higher systolic and diastolic blood pressure than that without (P < 0.0001 and P = 0.0012, respectively). Stepwise multivariate logistic regression analysis revealed that 25(OH)D (coefficient, −0.06; odd ratio, 0.9433; 95% confidence interval, 0.8967–0.9924), age, and sex were independently related with presence of coronary artery plaque. CONCLUSIONS: Relatively low vitamin D level was observed among participants with plaque, which was determined through CCTA during a health examination. Plaque formation and serum 25(OH)D level showed inverse relationship.

Response of Vancomycin according to Steroid Dosage in Pediatric Patients with Culture-Proven Bacterial Meningitis

BACKGROUND: This study aimed to evaluate the efficacy of combined vancomycin and steroid therapy for the treatment of culture-proven bacterial meningitis in pediatric patients. MATERIALS AND METHODS: We identified a total of 86 pediatric patients with culture-positive cerebrospinal fluid who were treated at our facility between 2005 and 2015. Ten of these patients (5 boys and 5 girls) received first-line treatment with vancomycin as the initial form of therapy. All cultured bacteria were sensitive to vancomycin. We retrospectively analyzed these cases to examine the relationship between concomitant steroid dosage and antibiotic treatment effectiveness. RESULTS: Nine of the 10 patients included in our analysis received steroid treatment. Of these, 3 received high-dose steroid therapy and 6 received low-dose steroid therapy. Five patients did not respond to vancomycin, including all 3 patients in the high-dose steroid group and 2 patients in the low-dose steroid group. Our analysis confirmed that the response rate to vancomycin treatment was significantly reduced in accordance with steroid dosage (P = 0.035). Patients who did not to respond to vancomycin with concomitant high-dose steroid administration improved clinically after the substitution of vancomycin with teicoplanin. CONCLUSION: The use of steroids, especially in high doses, may impair the effectiveness of vancomycin for treating bacterial meningitis in pediatric patients. Physicians should be cautious when administering concomitant steroid therapy and should carefully monitor the steroid dosage.

Reply on “Vitamin D and coronary atherosclerosis”

No abstract available.

Identification of a Novel Nonsense Mutation in the ARSE Gene of a Patient with X-Linked Recessive Chondrodysplasia Punctata

X-linked recessive chondrodysplasia punctata (CDPX1) is caused by a hemizygous mutation in the arylsulfatase E (ARSE) gene located on chromosome Xp22.3. It is a rare congenital disorder of punctate calcifications in cartilages, leading to short stature and facial and limb anomalies. These clinical features are frequently observed in all types of chondrodysplasia punctata and have also been seen in other cartilage developmental disorders. Because of the phenotypical similarities, specific testing for only one gene is inefficient and time consuming. The advent of next-generation sequencing has provided an opportunity to improve diagnostic accuracy as well as save on time and cost. Here, we report on a patient diagnosed with CDPX1, who was identified via diagnostic exome sequencing to have a novel nonsense mutation in the ARSE gene, that was inherited from the mother.

Trends in Survival and Incidence of Bronchopulmonary Dysplasia in Extremely Preterm Infants at 23-26 Weeks Gestation

The aim of this study was to investigate the relationship between survival and incidence of bronchopulmonary dysplasia (BPD) in extremely premature infants, and identify clinical factors responsible for this association. Medical records of 350 infants at 23-26 weeks gestation from 2000 to 2005 (period I, n = 137) and 2006 to 2010 (period II, n = 213) were retrospectively reviewed. The infants were stratified into 23-24 and 25-26 weeks gestation, and the survival, BPD incidence, and clinical characteristics were analyzed. BPD was defined as oxygen dependency at 36 weeks postmenstrual age. The overall survival rate was significantly improved in period II compared to period I (80.3% vs. 70.0%, respectively; P = 0.028), especially in infants at 23-24 weeks gestation (73.9% vs. 47.4%, respectively; P = 0.001). The BPD incidence in survivors during period II (55.0%) was significantly decreased compared to period I (67.7%; P = 0.042), especially at 25-26 weeks gestation (41.7% vs. 62.3%, respectively; P = 0.008). Significantly improved survival at 23-24 weeks gestation was associated with a higher antenatal steroid use and an improved 5-minute Apgar score. A significant decrease in BPD incidence at 25-26 weeks gestation was associated with early extubation, prolonged use of less invasive continuous positive airway pressure, and reduced supplemental oxygen. Improved perinatal and neonatal care can simultaneously lead to improved survival and decreased BPD incidence in extremely premature infants.

Mitten-Line Hyperpigmentation of an Infant

No abstract available.

Mitten-Line Hyperpigmentation of an Infant

No abstract available.

A novel PRF1 gene mutation in a fatal neonate case with type 2 familial hemophagocytic lymphohistiocytosis / 소아과

Hemophagocytic lymphohistiocytosis (HLH) occurs in the primary form (genetic or familial) or secondary form (acquired). The familial form of HLH (FHL) is a potentially fatal autosomal recessive disorder that occurs because of constitutional defects in cell-mediated cytotoxicity. Here, we report a fatal neonatal case of type 2 FHL (FHL2) that involved a novel frameshift mutation. Clinically, the newborn presented with severe sepsis-like features and required mechanical ventilation and continuous venovenous hemodiafiltration. Flow cytometry analysis showed marked HLH and complete absence of intracytoplasmic perforin expression in cytotoxic cells; therefore, we performed molecular genetic analyses for PRF1 mutations, which showed that the patient had a compound heterozygous mutation in PRF1, that is, c.65delC (p.Pro22Argfs*2) and c.1090_1091delCT (p.Leu364Glufs*93). Clinical and genetic assessments for FHL are required for neonates with refractory fever and progressive multiple organ failure, particularly when there is no evidence of microbiological or metabolic cause.

Acute Spontaneous Cervical Epidural Hematoma Mimicking Cerebral Stroke: A Case Report and Literature Review

Spontaneous cervical epidural hematoma (SCEDH) is a rare disease, but can cause severe neurologic impairment. We report a case of a 68-year-old female who presented with sudden onset, posterior neck pain, right shoulder pain, and progressive right hemiparesis mimicking stroke with no trauma history. Initial brain CT and diffusion MRI performed to rule out brain lesion did not show any positive findings. Laboratory examination presented only severe thrombocytopenia (45,000/mm3). Subsequent cervical MRI revealed a cervical epidural mass lesion. We confirmed that it was pure hematoma through C5 unilateral total laminectomy and C6 partial hemilaminectomy. She achieved complete neurologic recovery with active rehabilitation. Early surgical decompression for SCEDH with neurologic impairment should be recommended for better outcome.