Herein, we report the results of the 2018 survey on the external quality assessment (EQA) scheme for the Transfusion Medicine Program (TMP) in Korea. The proficiency testing specimens were prepared at Ajou University Hospital and were sent to the participants biannually. The average accuracy rates for ten different test items on the regular survey were as follows: ABO typing, 99.5%–99.8% (N=841); RhD typing, 99.8%–100.0% (N=827); crossmatching, 89.4%–99.6% (N=708); ABO subtyping, 94.2% and 94.4% (N=53); Rh CcEe antigen testing, all 100.0% (N=51); weak D test, 80.9% (N=207) for the first trial and not graded for the second trial; antibody screening, 99.7%–100.0% (N=304); direct antiglobulin test (DAT) using a polyspecific reagent, 98.9%–100.0% (N=264); DAT using an immunoglobulin-G monospecific reagent, all 100.0% (N=66); DAT using a C3d monospecific reagent, 97.0%–100.0% (N=67); antibody identification, 98.4%–100.0% (N=127); and ABO antibody titration, 84.6%–100.0% (N=73). There were approximately 10.5% more participants in the 2018 EQA scheme than that in 2017. Excellent survey results were obtained in the 2018 EQA scheme compared with those in 2017, except for the weak D test. The 2018 EQA scheme for the TMP should be helpful for improving the quality of the participating laboratories.
Coombs Test ; Korea ; Laboratory Proficiency Testing ; Mass Screening ; Quality Improvement ; Thymidine Monophosphate ; Transfusion Medicine

Post-traumatic stress disorder (PTSD) is a trauma-induced psychiatric disorder characterized by impaired fear extermination, hyperarousal, and anxiety that may involve the release of monoamines in the fear circuit. The reported pharmacological properties of tetramethylpyrazine (TMP) include anti-cancer, anti-diabetic, anti-atherosclerotic, and neuropsychiatric activities. However, the anxiolytic-like effects of TMP and its mechanism of action in PTSD are unclear. This study measured several anxiety-related behavioral responses to examine the effects of TMP on symptoms of anxiety in rats after single prolonged stress (SPS) exposure by reversing the serotonin (5-HT) and hypothalamic-pituitary-adrenal (HPA) axis dysfunction. Rats were given TMP (10, 20, or 40 mg/kg, i.p.) for 14 days after SPS exposure. Administration of TMP significantly reduced grooming behavior, increased the time spent and number of visits to the open arm in the elevated plus maze test, and significantly increased the number of central zone crossings in the open field test. TMP administration significantly reduced the freezing response to contextual fear conditioning and significantly restored the neurochemical abnormalities and the SPS-induced decrease in 5-HT tissue levels in the prefrontal cortex and hippocampus. The increased 5-HT concentration during TMP treatment might be partially attribute to the tryptophan and 5-hydroxyindoleacetic acid mRNA level expression in the hippocampus of rats with PTSD. These findings support a role for reducing the altered serotonergic transmission in rats with PTSD. TMP simultaneously attenuated the HPA axis dysfunction. Therefore, TMP may be useful for developing an agent for treating psychiatric disorders, such those observed in patients with PTSD.
Animals ; Anxiety ; Arm ; Freezing ; Grooming ; Hippocampus ; Humans ; Models, Animal* ; Prefrontal Cortex ; Rats* ; RNA, Messenger ; Serotonin ; Stress Disorders, Post-Traumatic* ; Thymidine Monophosphate ; Tryptophan

Here, we have reported results of the surveys on the external quality assessment scheme (EQA) of the Transfusion Medicine Program (TMP) in Korea that were carried out in 2017. The proficiency testing specimens were prepared at Ajou University Hospital, and sent to the participants biannually. The average accuracy rates (N=the number of participants) for ten different test items on the regular survey were as follows: ABO typing, 99.1%–99.9% (N=714); RhD typing, 99.3%–100.0% (N=695); crossmatching, 88.9%–98.5% (N=618); ABO subtyping, 80.7% and 96.0% (N=51); Rh CcEe antigen testing, 98.8%–100.0% (N=51); weak D test, 99.3% and 100.0 (N=150); antibody screening, 98.6%–100.0% (N=295); direct antiglobulin test (DAT) using a poly-specific reagent, 99.2%–100.0 (N=256); DAT using an immunoglobulin-G monospecific reagent, all 100.0% (N=68); DAT using a C3d-monospecific reagent, 83.6%–100.0% (N=72); antibody identification, 88.7%–99.2% (N=123); and ABO Ab titration, 84.6%–100.0% (N=73). The number of participants for the EQA for TMP in 2017 was much higher than that in 2016. Except for the case of ABO subtyping, excellent survey results for the 2017 EQA for TMP were obtained, compared to those in 2016. Thus, the EQA for TMP in 2017 should be helpful for improving the quality of the participating laboratories.
Coombs Test ; Korea* ; Laboratory Proficiency Testing ; Mass Screening ; Quality Improvement ; Thymidine Monophosphate ; Transfusion Medicine*

Premature ventricular complex (PVC) usually follows a benign course and shows good response to medical therapy. However, high burden of PVC deteriorates cardiac function and is often associated with progression into dilated cardiomyopathy (DCMP). We report a case of a young patient who recovered from DCMP after PVC ablation. The patient complained of palpitations and dyspnea on exertion. Holter examination revealed an isolated PVC burden of 29%. Despite intensive medical therapy for more than a year, symptoms aggravated and PVC burden was not diminished on follow-up Holter examination. Furthermore, the echocardiogram revealed deteriorated systolic function as well as left ventricular enlargement, indicating progression into DCMP. Surface electrocardiogram indicated PVC origin in the left ventricular outflow tract. Detailed mapping at the right ventricle and left ventricle outflow tract with the aid of 3-dimensional mapping system, demonstrated PVC origin from the left ventricular outflow tract area, between the right and left coronary cusps. Radiofrequency ablation successfully abolished all ventricular premature beats. Follow-up Holter examination revealed no PVC, and the echocardiogram showed recovery to normal systolic function and chamber size. In conclusion, ablation of PVC should be considered when it does not respond to medical therapy and is associated with deterioration of cardiac function.
Cardiac Complexes, Premature ; Cardiomyopathy, Dilated ; Catheter Ablation ; Deoxycytidine Monophosphate ; Dyspnea ; Electrocardiography ; Follow-Up Studies ; Heart Ventricles ; Humans ; Ventricular Function, Left ; Ventricular Premature Complexes

Chromosome 2q37 deletion syndrome is a rare chromosomal disorder characterized by mild to moderate developmental delay, brachydactyly of the third to fifth digits or toes, short stature, obesity, hypotonia, a characteristic facial appearance, and autism spectrum disorder. Here, we report on a patient with 2q37 deletion presenting with dilated cardiomyopathy (DCMP). Congenital heart malformations have been noted in up to 20% of patients with 2q37 deletions. However, DCMP has not been reported in 2q37 deletion patients previously. The patient exhibited the characteristic facial appearance (a flat nasal bridge, deep-set eyes, arched eyebrows, and a thin upper lip), developmental delay, mild mental retardation, peripheral nerve palsy, and Albright hereditary osteodystrophy (AHO)-like phenotypes (short stature and brachydactyly). Conventional chromosomal analysis results were normal; however, microarray-based comparative genomic hybridization revealed terminal deletion at 2q37.1q37.3. In addition, the patient was confirmed to have partial growth hormone (GH) deficiency and had shown a significant increase in growth rate after substitutive GH therapy. Chromosome 2q37 deletion syndrome should be considered in the differential diagnosis of patients presenting with AHO features, especially in the presence of facial dysmorphism. When patients are suspected of having a 2q37 deletion, high-resolution cytogenetic analysis is recommended.
Autism Spectrum Disorder ; Brachydactyly ; Cardiomyopathy, Dilated ; Chromosome Disorders ; Comparative Genomic Hybridization ; Cytogenetic Analysis* ; Cytogenetics* ; Deoxycytidine Monophosphate ; Diagnosis, Differential ; Eyebrows ; Growth Hormone ; Heart ; Humans ; Intellectual Disability ; Muscle Hypotonia ; Obesity ; Paralysis ; Peripheral Nerves ; Phenotype ; Toes

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate how fibrinogen-based collagen fleece (Tachocomb®) graft myringoplasty (FCGM), performed under microscopic guidance, improves both hearing and tympanic membrane tissue repair in patients with traumatic tympanic membrane perforation (TMP). SUBJECTS AND METHODS: Between August 2009 and March 2015, a total of 52 patients with traumatic TMP visited the department of otorhinolaryngology at a secondary medical center. Twenty-nine of these underwent FCGM under microscopic guidance in our outpatient clinic. For each patient, we recorded the location and size of the perforation, the time elapsed from the onset of TMP until the myringoplasty, and the hearing level both before and after myringoplasty. RESULTS: The TMP closed completely in all cases (29 of 29 patients). After myringoplasty, the postoperative air-bone gap (ABG) differed significantly from the preoperative ABG. Three of the 29 patients (10.3%) experienced complications. Specifically, 2 presented with otorrhea after FCGM, but conservative management led to improvement without recurrence of perforation. One patient showed delayed facial palsy 1 week after the procedure. The condition of this patient also improved and the palsy was not permanent. CONCLUSIONS: FCGM may be an effective treatment option in case of traumatic TMP. The procedure requires no hospitalization, and can be used to avoid traditional tympanoplasty.
Ambulatory Care Facilities ; Collagen* ; Facial Paralysis ; Hearing ; Hearing Loss, Conductive ; Hospitalization ; Humans ; Myringoplasty* ; Otolaryngology ; Paralysis ; Recurrence ; Thymidine Monophosphate ; Transplants* ; Tympanic Membrane Perforation* ; Tympanic Membrane* ; Tympanoplasty

Deoxyribonucleotides (dNTPs) are important for the efficient growth of DNA viruses. Therefore, many DNA viruses have strategies for the upregulation of cellular dNTP levels. Both α- and γ-herpesviruses encode functional homologs of cellular dNTP anabolic enzymes, including the class I ribonucleotide reductase (RNR) large (R1) and small (R2) subunits, whereas β-herpesviruses modulate host cells to induce genes that increase dNTP levels. Interestingly, β-herpesviruses still express the nonfunctional RNR R1 subunit. However, it is not clear why β-herpesviruses still carry inactive R1 homologs. Recently, the R1 homologs of herpesviruses have been shown to inhibit innate immune signaling pathways. In particular, both functional and nonfunctional R1 homologs target receptor-interacting protein kinase 1 (RIP1) and inhibit RIP1-mediated signaling pathways to promote viral replication. Here, we summarize recent findings on the activity of herpesviral R1 homologs and discuss their roles in the regulation of innate immune signaling pathways.
Deoxyribonucleotides ; DNA Viruses ; Herpesviridae ; Protein Kinases ; Ribonucleotide Reductases* ; Up-Regulation

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss

Graves disease (GD) can lead to complications such as cardiac arrhythmia and heart failure. Although dilated cardiomyopathy (DCMP) has been occasionally reported in adults with GD, it is rare in children. We present the case of a 32-month-old boy with DCMP due to GD. He presented with irritability, vomiting, and diarrhea. He also had a history of weight loss over the past few months. On physical examination, he had tachycardia without fever, a mild diffuse goiter, and hepatomegaly. The chest radiograph showed cardiomegaly with pulmonary edema, while the echocardiography revealed a dilated left ventricle with an ejection fraction (EF) of 28%. The thyroid function test (TFT) showed elevated serum T3 and decreased thyroid stimulating hormone (TSH) levels. The TSH receptor autoantibody titer was elevated. He was diagnosed with DCMP with GD; treatment with methylprednisolone, diuretics, inotropics, and methimazole was initiated. The EF improved after the TFT normalized. At follow-up several months later, although the TFT results again showed evidence of hyperthyroidism, his EF had not deteriorated. His cardiac function continues to remain normal 1.5 months after treatment was started, although he still has elevated T3 and high TSH receptor antibody titer levels due to poor compliance with drug therapy. To summarize, we report a young child with GD-induced DCMP who recovered completely with medical therapy and, even though the hyperthyroidism recurred several months later, there was no relapse of the DCMP.
Adult ; Arrhythmias, Cardiac ; Cardiomegaly ; Cardiomyopathy, Dilated* ; Child* ; Child, Preschool ; Compliance ; Deoxycytidine Monophosphate ; Diarrhea ; Diuretics ; Drug Therapy ; Echocardiography ; Fever ; Follow-Up Studies ; Goiter ; Graves Disease* ; Heart Failure ; Heart Ventricles ; Hepatomegaly ; Humans ; Hyperthyroidism ; Male ; Methimazole ; Methylprednisolone ; Physical Examination ; Pulmonary Edema ; Radiography, Thoracic ; Receptors, Thyrotropin ; Recurrence ; Tachycardia ; Thyroid Function Tests ; Thyrotropin ; Vomiting ; Weight Loss

Although heart transplantation is a final therapeutic option in pediatric patients with dilated cardiomyopathy (DCMP), the shortage of pediatric heart donors is a major obstacle. In adults with DCMP characterized by cardiac dyssynchrony, cardiac resynchronization therapy (CRT) is known to be an effective treatment option. However, there is a lack of evidence on the effectiveness of CRT in infants with DCMP. Several studies have reported improvement in hemodynamics and cardiac performance following CRT in infants with DCMP. Here, we report CRT in an infant with DCMP during extracorporeal membrane oxygenation with 5 months of follow-up.
Adult ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Deoxycytidine Monophosphate ; Extracorporeal Membrane Oxygenation ; Follow-Up Studies ; Heart ; Heart Transplantation ; Hemodynamics ; Humans ; Infant* ; Oxygenators, Membrane* ; Tissue Donors