A Case of Adrenal Myelolipoma Associated with Hereditary Spherocytosis / 임상소아혈액종양

Hereditary spherocytosis is the most common hereditary red blood cell membrane disorder. It results from a deficiency in certain proteins that are part of the red blood cell membrane cytoskeleton. We report a case of adrenal myelolipoma in a 23-yearold patient with hereditary spherocytosis. She was diagnosed with hereditary spherocytosis at 7 months of age. As she grew older, hemoglobin level was low, so splenectomy was recommended, but continuous follow up was not possible due to the circumstances of the patient, so the splenectomy was delayed. Adrenal myelolipoma was discovered incidentally at the age of 23 with abdominal pain and cholecystitis with gallstones at the time. Myelolipoma is a benign tumor of the mesenchymal origin;its etiology remains unclear. Myelolipoma is composed of adipose and hematopoietic tissues and mainly arises from adrenal tissues. The mass is often detected during routine radiologic examinations because myelolipoma is usually asymptomatic and not generally associated with hematologic diseases. The prevalence of myelolipoma appears to be increasing due to the increased use of imaging modalities. The association of myelolipoma with hereditary spherocytosis has rarely been reported in the literature. To our best knowledge, this is the first report of adrenal myelolipoma associated with hereditary spherocytosis in the Korean population.

Significance of Evaluation for von Willebrand Disease in Premenopausal Women with Iron Deficiency Anemia and Menorrhagia / 임상소아혈액종양

Background@#In Korea, in the case of patients with significant bleeding symptoms due to unknown causes, there are very few studies that evaluate bleeding disorders, including von Willebrand disease (VWD ). VWD should be considered as an important causative factor in patients with iron deficiency anemia (IDA) and unexplained menorrhagia. This study aimed to understand the clinical characteristics of VWD and the significance of evaluation for VWD in premenopausal women in Korea with menorrhagia and ID A. @*Methods@#Premenopausal women who were diagnosed with IDA and menorrhagia from January 2009 to March 2020 were included. IDA was diagnosed by either low ferritin or transferrin saturation with microcytic anemia. Menorrhagia was evaluated based on the medical records obtained from a gynecologist. VWD diagnosis was defined as von Willebrand factor antigen ＜50% and von Willebrand factor ristocetin cofactor activity ＜50%, which were low according to the Hospital for Sick Children criteria. @*Results@#Out of a total of 120 patients, only 12 were tested for VWD, all of whom were pediatric patients. Four of the 12 pediatric patients tested were diagnosed with VWD and 4 of the 120 (3.3%) patients with IDA and menorrhagia were diagnosed with VWD. Three out of the 4 patients was diagnosed with VWD by repeat screening test. Although all parameters are not statistically significant, VWD patients tended to have ID A at a younger age (13.25 vs. 15.00 years) and were more likely to have recurrent IDA than patients without VWD. @*Conclusion@#Clinical doctors should consider VWD if patients have menorrhagia with ID A. If VWD is suspected, repeated VWD screening tests are necessary to increase the diagnosis rate. Accurate diagnosis of VWD in patients with significant bleeding may facilitate decisions for appropriate treatment.

Significance of Evaluation for von Willebrand Disease in Premenopausal Women with Iron Deficiency Anemia and Menorrhagia / 임상소아혈액종양

Background@#In Korea, in the case of patients with significant bleeding symptoms due to unknown causes, there are very few studies that evaluate bleeding disorders, including von Willebrand disease (VWD ). VWD should be considered as an important causative factor in patients with iron deficiency anemia (IDA) and unexplained menorrhagia. This study aimed to understand the clinical characteristics of VWD and the significance of evaluation for VWD in premenopausal women in Korea with menorrhagia and ID A. @*Methods@#Premenopausal women who were diagnosed with IDA and menorrhagia from January 2009 to March 2020 were included. IDA was diagnosed by either low ferritin or transferrin saturation with microcytic anemia. Menorrhagia was evaluated based on the medical records obtained from a gynecologist. VWD diagnosis was defined as von Willebrand factor antigen ＜50% and von Willebrand factor ristocetin cofactor activity ＜50%, which were low according to the Hospital for Sick Children criteria. @*Results@#Out of a total of 120 patients, only 12 were tested for VWD, all of whom were pediatric patients. Four of the 12 pediatric patients tested were diagnosed with VWD and 4 of the 120 (3.3%) patients with IDA and menorrhagia were diagnosed with VWD. Three out of the 4 patients was diagnosed with VWD by repeat screening test. Although all parameters are not statistically significant, VWD patients tended to have ID A at a younger age (13.25 vs. 15.00 years) and were more likely to have recurrent IDA than patients without VWD. @*Conclusion@#Clinical doctors should consider VWD if patients have menorrhagia with ID A. If VWD is suspected, repeated VWD screening tests are necessary to increase the diagnosis rate. Accurate diagnosis of VWD in patients with significant bleeding may facilitate decisions for appropriate treatment.

Porencephaly with Skull Remodeling

No abstract available.

Successful Treatment of Acinetobacter Baumannii Meningitis with Colistin

Acinetobacter baumannii is a source of opportunistic infection in hospital and can cause central nervous system infection mainly by neurosurgical operation. Community acquired infection by Acinetobacter baumannii is very rare. A 58-year-old man was presented with fever, decreased consciousness and seizure. Acinetobacter baumannii was identified in the CSF culture, and colistin was administered intravenously. Patient's clinical symptoms improved after treatment. We report a case of successful treatment of Acinetobacter baumannii meningitis with colistin.

Mental Health Status of Correctional Officers in Correctional Institutions / 신경정신의학

OBJECTIVES: The purpose of this study was to measure the current mental health status of correctional officers by using various assessment instruments. The results of the study could help establish appropriate mental health-related solutions and policies for the officers. METHODS: The study enrolled 2571 correctional officers from 50 correctional facilities in South Korea in 2011. They were asked to answer or rate their occupational experiences within various investigative inventories and scales including the Maslach Burnout Inventory (MBI), the Korean Occupational Stress Scale (KOSS), the Job Satisfaction Scale by Davison and Cooper, the Korean version of the Beck Depression Inventory (K-BDI), and Rosenberg's Self-Esteem Scale (RSE). RESULTS: Based on MBI results, the burnout score of the correctional officers was higher than that of the other occupations. Using the KOSS data, the average occupational stress of the correctional officer was higher than that of the other occupations. The officers' average job satisfaction score was lower than that of the other occupations. The average depression level of the correctional officers derived from K-BDI data was 9.36. The average RSE score of correctional officers was lower than that for the other occupations. CONCLUSION: This research revealed that the mental health status of correctional officers is poorer than that for other occupations. This finding emphasizes the need for a system to regularly assess the mental health status of correctional officers and to find solutions that can improve their mental health status.

The Challenges of Diabetes Education for Young Diabetes Patients: A 1:1 Mentoring Program Teaching Method / 임상당뇨병

Diabetes is a chronic disease resulting from an absolute or relative shortage of insulin, and it is a disease that requires continuous management throughout the lifetime. Most past diabetes education programs used educational methods for training; however, only a small number of studies on individual education methods were conducted for diabetic patients. Along with individualized educational methods, various instructional methods for knowledge delivery and self-control have been attempted, and one of these is a one-on-one mentoring process. Through such a program, children can acquire knowledge and management of diabetes, and systematic management can be maintained through mentoring and use of diabetic resources. Also, shared cultural and life experiences between the mentor and the mentee can help the children overcome the identity crisis of adolescence to produce a suitable emotional experience. These positive effects can reduce the socio-economic costs associated with diabetes through improved management of problems that can arise in diabetics.

Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan

PURPOSE: This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. METHODS: We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. RESULTS: There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3–22.0), and Winter (OR, 5.9; 95% CI, 3.5–10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. CONCLUSION: Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation.

Vitamin D deficiency in children aged 6 to 12 years: single center's experience in Busan

PURPOSE: This study investigated the prevalence and risk factors associated with vitamin D deficiency in children. METHODS: We analyzed the medical records of 330 patients from the age of 6 to 12, who visited the endocrinology clinic of the Department of Pediatrics at Pusan National University Hospital, from September, 2013 to May, 2014. According to their serum 25-hydroxyvitamin D (25(OH)D) levels, the patients were grouped into either the deficiency group (25(OH)D<20 ng/mL), or the sufficiency group (25(OH)D≥20 ng/mL). The differences between the 2 groups were compared. RESULTS: There were 195 patients (59.1%) who had vitamin D deficiency. Their mean serum 25(OH)D level was 14.86±3.20 ng/mL. The differences in sex, age, and pubertal status between the 2 groups were not statistically significant. Weight standard deviation score (SDS), and body mass index SDS, were significantly higher in the vitamin D deficiency group (P=0.002 for each), compared to the sufficiency group. Compared with Autumn, both Spring (odds ratio [OR], 9.7; 95% confidence interval [CI], 4.3–22.0), and Winter (OR, 5.9; 95% CI, 3.5–10.0), were risk factors for vitamin D deficiency. In multiple logistic regression analysis, only seasonal differences have been confirmed to have an effect on vitamin D deficiency. CONCLUSION: Vitamin D deficiency in children aged 6 to 12 years is very common. Spring and Winter are the most important risk factors for vitamin D deficiency. We suggest that it is necessary to supplement the guideline for the vitamin D intake according to our situation.

Balloon Occlusive Diameter of Non-Circular Atrial Septal Defects in Transcatheter Closure with Amplatzer Septal Occluder

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the balloon occlusive diameter (BOD) of non-circular defects in the transcatheter closure of atrial septal defect (ASD). SUBJECTS AND METHODS: A total of 67 patients who had undergone transcatheter closure of an ASD were reviewed retrospectively. A non-circular defect was defined as the ratio of the short diameter to the long diameter of the defect on the en-face image less than 0.75. The BOD was compared with the long diameter of the defect and then compared between the two groups. RESULTS: There were 22 patients with circular defects and 45 patients with non-circular defects. The difference in BOD measuring from the long diameter of the defect was quite different between the two groups and significantly smaller in non-circular morphology (0.1+/-4.0 vs. 2.3+/-2.1, p=0.006). The difference in BOD measurement from the long diameter of ASD showed a positive correlation with the ratio of the short diameter to the long diameter of ASD (b/a) (r2=0.102, p=0.008). In the non-circular morphology of ASD, the difference in BOD measured from the long diameter had a significant negative correlation with the long diameter of ASD (r2=0.230, p=0.001), whereas in circular ASD, no significant correlation was found between the difference in BOD and the long diameter of ASD (p=0.201). CONCLUSION: The BOD compared with the long diameter measured from three-dimensional transesophageal echocardiography was smaller in non-circular ASD than in circular ASD. This difference was much smaller in non-circular ASD with a large long diameter.

The First Pediatric Case of Intrathoracic Tuberculosis Lymphadenitis Diagnosed by Endobronchial Ultrasound Guided Transbronchial Needle Aspiration / 소아감염

Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) now provides an important alternative diagnostic modality in patients with intrathoracic tuberculosis lymphadenopathy. The procedure is well tolerated in the outpatient setting, provides access to the mediastinal and hilar lymph node locations commonly in tuberculosis and also allows bronchial washing to be performed at the same procedure. However, there is no report of EBUS-TBNA applied to children to diagnose tuberculosis. We report a case of EBUS-TBNA applied to children who had intrathoracic tuberculosis lymphadenopathy.

Differences in the Determination of Cause and Manner of 127 Natural Death Cases by Postmortem Inspection and Autopsy / 대한법의학회지

Medico-legal investigations in Korea depend mostly on postmortem inspection without autopsy. Both untrained physicians and experienced forensic pathologists may not be able to determine the cause and manner of death only by postmortem inspection. The aim of our study was to show the limitations of postmortem inspection by evaluating the discrepancy between the results of postmortem inspection and autopsy after natural death. The manner of death by postmortem inspection changed to unnatural death in 9.4% of the cases (12 cases/127 cases) after autopsy. The cause of death in most cases was consumption of alcohol, agricultural chemicals, and antipsychotic intoxication. The cause of death by postmortem inspection changed in 24.3% of the cases (26 cases/107 cases) among cases of confirmed natural death after autopsy. The positive predictive value of cause of death by postmortem inspection was higher for cardiovascular disease (79.0%) and lower for chronic alcoholism and/or chronic liver disease (28.6%). This study shows that forensic pathologists could make considerable errors in determining the cause and manner of death without autopsy and emphasizes the necessity to refine medico-legal investigations in Korea by introducing the limited autopsy with toxicologic study and supportive postmortem imaging.

Asphyxial Death Using Plastic Bag / 대한법의학회지

Asphyxia due to plastic bag is not common. The manner of death may be accidental, suicidal or homicidal. We report an asphyxial death using plastic bag, giving us difficulty in determining the manner of death, suicidal or homicidal. A 32-year-old female was found dead in bathroom and her head was wrapped in a supermarket shopping bag sealed with adhesive tape around the neck. Strangely she was handcuffed behind the back of the victim. Because of no evidence of violence on the body and the presence of a suicide note at the scene, the manner of death was concluded as suicide. This case emphasizes that the interpretation of postmortem examination should be incorporated with the proper investigation of circumstances at the scene of death to determine the manner of death.

Pathogenesis of Sudden Manhood Death Syndrome / 대한법의학회지

Sudden manhood death syndrome (SMDS) is a disorder found in southeast Asia, particularly Thailand, Philippines and Japan, which causes sudden cardiac death during sleep. In Korea, SMDS cases have been occasionally encountered in forensic autopsy practice. However, the incidence and pathogenesis has been rarely studied. This study to review chronologically the proposed pathogenesis of SMDS; pathology of cardiac conduction system, sleeping and breathing disorder, K+ and thiamine deficiency, mental stress, testosterone, hyperlipidemia, and narrow circumference of coronary artery. Brugada syndrome and SMDS are phenotypically, genetically and functionally the same disorder and Brugada syndrome has been a subject of intensive study since its early description in 1992. While Brugada syndrome is an inherited cardiac disorder caused by mutations in the SCN5A gene encoding the cardiac sodium channel alpha-subunit. less than 20% of its patients are known to be associated with SCN5A mutations. Moreover, the postmortem molecular screening of SCN5A mutations on formalin fixed paraffin embbeded cardiac tissues from SMDS cases has not been rewarding due to technical problems. The role of structural heart disease and sodium channel dysfunction in the induction of electrical instability in SMDS and Brugada syndrome is still debatable.

Colorectal Trauma by Compressed Air / 대한법의학회지

A seaman died after a jet of compressed air directed to his anus while working with his colleagues in deck of the ship. He suffered from abdominal pain and distension, and died 5 hours later in the ship. Autopsy was performed and revealed rupture of the splenic flexure of the colon and partial seromuscular tearing in the rectosigmoidal region. We concluded that the cause of death was tension pneumoperitoneum by the pneumatic colonic rupture. The need for education of the workers handling compressed air appliances is emphasized.

Effect of restoration type on the stress distribution of endodontically treated maxillary premolars; Three-dimensional finite element study / 대한치과보존학회지

The purpose of this study was to investigate the effects of four restorative materials under various occlusal loading conditions on the stress distribution at the CEJ of buccal, palatal surface and central groove of occlusal surface of endodontically treated maxillary second premolar, using a 3D finte element analysis. A 3D finite element model of human maxillary second premolar was endodontically treated. After endodontic treatment, access cavity was filled with Amalgam, resin, ceramic or gold of different mechanical properties. A static 500N forces were applied at the buccal (Load-1) and palatal cusp (Load-2) and a static 170N forces were applied at the mesial marginal ridge and palatal cusp simultaneously as centric occlusion (Load-3). Under 3-type Loading condition, the value of tensile stress was analyzed after 4-type restoration at the CEJ of buccal and palatal surface and central groove of occlusal surface. Excessive high tensile stresses were observed along the palatal CEJ in Load-1 case and buccal CEJ in Load-2 in all of the restorations. There was no difference in magnitude of stress in relation to the type of restorations. Heavy tensile stress concentrations were observed around the loading point and along the central groove of occlusal surface in all of the restorations. There was slight difference in magnitude of stress between different types of restorations. High tensile stress concentrations around the loading points were observed and there was no difference in magnitude of stress between different types of restorations in Load-3.

Stress distribution of endodontically treated maxillary second premolars restored with different methods: Three-dimensional finite element analysis / 대한치과보존학회지

The purpose of this study was to evaluate the influence of elastic modulus of restorative materials and the number of interfaces of post and core systems on the stress distribution of three differently restored endodontically treated maxillary second premolars using 3D FE analysis. Model 1, 2 was restored with a stainless steel or glass fiber post and direct composite resin. A PFG or a sintered alumina crown was considered. Model 3 was restored by EndoCrown. An oblique 500 N was applied on the buccal (Load A) and palatal (Load B) cusp. The von Mises stresses in the coronal and root structure of each model were analyzed using ANSYS. The elastic modulus of the definitive restorations rather than the type of post and core system was the primary factor that influenced the stress distribution of endodontically treated maxillary premolars. The stress concentration at the coronal structure could be lowered through the use of definitive restoration of high elastic modulus. The stress concentration at the root structure could be lowered through the use of definitive restoration of low elastic modulus.

Sudden Death Due to a Ganglioglioma / 대한법의학회지

The incidence of sudden death due to undiagnosed primary intracranial tumor is very low in forensic autopsy practice. The ganglioglioma is a well differentiated, slow growing primary brain tumor that is encountered predominantly in childhood. We report a sudden, unexpected death in a 6-year-old girl from a ganglioglioma. On autopsy, there was an intraventricular tumor with recent hemorrhage, occupying the anterior aspect of the lateral ventricle and resulting in hydrocephalus. Acute intratumoral hemorrhage appears to be the mechanism of sudden death.

Hypopharyngeal Perforation Associated with Traffic Accident / 대한법의학회지

Hypopharyngeal perforation associated with traffic accident is a rare occurrence, which can cause serious morbidity and mortality if not diagnosed and treated early. We report an autopsy case of hypopharyngeal perforation following a traffic accident. A 32-year-old man hits his chin when his car hits highway guardrail. He complained continuous neck pain and died 5 days after the accident. Autopsy was performed, and death was due to undiagnosed retropharyngeal abscess with sepsis.

Stress analysis of maxillary premolars with composite resin restoration of notch-shaped class V cavity and access cavity; Three-dimensional finite element study / 대한치과보존학회지

The purpose of this study was to investigate the distribution of tensile stress of canal obturated maxillary second premolar with access cavity and notch-shaped class V cavity restored with composite resin using a 3D finite element analysis. The tested groups were classified as 8 situations by only access cavity or access cavity with notch-shaped class VS cavity (S or N), loading condition (L1 or L2), and with or without glass ionomer cement base (R1 or R2). A static load of 500 N was applied at buccal and palatal cusps. Notch-shaped cavity and access cavity were filled microhybrid composite resin (Z100) with or without GIC base (Fuji II LC). The tensile stresses presented in the buccal cervical area, palatal cervical area and occlusal surface were analyzed using ANSYS. Tensile stress distributions were similar regardless of base. When the load was applied on the buccal cusp, excessive high tensile stress was concentrated around the loading point and along the central groove of occlusal surface. The tensile stress values of the tooth with class V cavity were slightly higher than that of the tooth without class V cavity. When the load was applied the palatal cusp, excessive high tensile stress was concentrated around the loading point and along the central groove of occlusal surface. The tensile stress values of the tooth without class V cavity were slightly higher than that of the tooth with class V cavity.