A conversion to implant assisted removable partial denture infailed fixed implant prosthesis of mandible: A case report / 대한치과보철학회지

Failure of fixed implant supported prosthesis is caused by biomechanical factors such as excessive occlusal stress and biological factors such as bacterial infections andinflammation. Implants with severe bone resorption that have worsened without being resolved due to implant complications should be removed and then new treatmentsshould be planned, taking into account remaining teeth, remaining implants, and residual alveolar. The patient of this case removed some of fixed implant prosthesis of mandible.The condition of the remaining alveolar bone was reassessed for further implant replacement and a few implants were placed. Then implant assisted removable partialdenture (IAPRD) treatment is performed using implant surveyed bridge as abutment. Through this treatment, the clinical results were satisfactory on aspect of masticatoryfunction recovery and oral hygiene management. (J Korean Acad Prosthodont 2020;58:161-8)

Psoriasis increases the risk of concurrent inflammatory bowel disease: A population-based nationwide study in Korea

Background: The epidemiology of the association between psoriasis and inflammatory bowel disease is poorly defined and remains controversial. Aim: To evaluate the prevalence of inflammatory bowel disease in patients with psoriasis compared with the general population. Methods: We searched the nationwide health claims database between 2011 and 2015 and evaluated the prevalence of inflammatory bowel disease, including Crohn's disease and ulcerative colitis. Results: Prevalence of inflammatory bowel disease, Crohn's disease and ulcerative colitis in patients with psoriasis vs the general population in 2011 were 0.16, 0.05 and 0.12% vs 0.08, 0.03 and 0.06%, respectively, which increased significantly with time between 2011 and 2015. Patients with psoriasis consistently revealed higher standardized prevalence (age and sex adjusted) of inflammatory bowel disease, Crohn's disease and ulcerative colitis compared with the general population. Subgroup analysis revealed the highest risk of prevalent inflammatory bowel disease in patients younger than 19 years (crude odds ratio 5.33, 95% confidence interval 3.74–7.59). Severe psoriasis demonstrated higher odds of inflammatory bowel disease (odds ratio 2.96, 95% confidence interval 2.54–3.45) than mild psoriasis (odds ratio 1.68, 95% confidence interval 1.51–1.88). Limitations: Limited data for doing adjustment and cross-sectional study design. Conclusions: Psoriasis patients revealed higher risk of inflammatory bowel disease. In particular, young patients and those with severe psoriasis may require closer monitoring and comprehensive management.

Changes in the tumor necrosis factor-α level after an ultrasound-guided femoral nerve block in elderly patients with a hip fracture / Alterações no nível de TNF-α após bloqueio do nervo femoral guiado por ultrassom em idosos com fratura de quadril

Abstract Background and objectives: An ultrasound guided femoral nerve block is an established analgesic method in patients with a hip fracture. Elevated cytokine levels correlate with poor patient outcomes after surgery. Hence, the aim of the study was to describe the levels of tumor necrosis factor-α after an ultrasound-guided femoral nerve block in elderly patients having a femoral neck fracture. Methods: A total of 32 patients were allocated into two treatment groups: 16 patients (femoral nerve block group; ultrasound-guided femoral nerve block with up to 20 mL of 0.3 mL.kg−1 of 0.5% bupivacaine and intravenous tramadol) and 16 patients (standard management group; up to 3 mL of 0.9% saline in the femoral sheath and intravenous tramadol). Tumor necrosis factor-α and visual analogue scale scores were evaluated immediately before the femoral nerve block and again at 4, 24, and 48 h after the femoral nerve block. All surgery was performed electively after 48 h of femoral nerve block. Results: The femoral nerve block group had a significantly lower mean tumor necrosis factor-α level at 24 (4.60 vs. 8.14, p < 0.001) and 48 h (5.05 vs. 8.56, p < 0.001) after the femoral nerve block, compared to the standard management group. The femoral nerve block group showed a significantly lower mean visual analogue scale score at 4 (3.63 vs. 7.06, p < 0.001) and 24 h (4.50 vs. 5.75, p < 0.001) after the femoral nerve block, compared to the standard management group. Conclusions: Ultrasound-guided femoral nerve block using 0.3 mL.kg−1 of 0.5% bupivacaine up to a maximum of 20 mL resulted in a significant lower tumor necrosis factor-α level.

Resumo Justificativa e objetivos: O bloqueio do nervo femoral guiado por ultrassom é um método analgésico estabelecido em pacientes com fratura de quadril. Níveis elevados de citocinas estão correlacionados com resultados desfavoráveis para o paciente após a cirurgia. Portanto, o objetivo do estudo foi descrever os níveis do fator de necrose tumoral alfa após bloqueio do nervo femoral guiado por ultrassom em pacientes idosos com fratura do colo de fêmur. Métodos: No total, 32 pacientes foram alocados em dois grupos de tratamento: 16 pacientes (grupo bloqueio do nervo femoral; bloqueio do nervo femoral guiado por ultrassom com até 20 mL de bupivacaína a 0,5% (0,3 mL.kg−1) e tramadol intravenoso) e 16 pacientes (grupo tratamento padrão, até 3 mL de solução salina a 0,9% na bainha femoral e tramadol intravenoso). Os escores do fator de necrose tumoral alfa e da Escala Visual Analógica foram avaliados imediatamente antes do bloqueio do nervo femoral e novamente em 4, 24 e 48 horas pós-bloqueio do nervo femoral. Todas as cirurgias foram realizadas de forma eletiva após 48 horas de bloqueio do nervo femoral. Resultados: O grupo bloqueio do nervo femoral teve um nível médio de fator de necrose tumoral alfa significativamente menor em 24 (4,60 vs. 8,14, p < 0,001) e 48 horas (5,05 vs. 8,56, p < 0,001) pós-bloqueio do nervo femoral, comparado com o grupo tratamento padrão. O grupo bloqueio do nervo femoral apresentou uma média significativamente menor no escore da Escala Visual Analógica em 4 (3,63 vs. 7,06, p < 0,001) e 24 horas (4,50 vs. 5,75, p < 0,001) pós-bloqueio do nervo femoral, em comparação com o grupo tratamento padrão. Conclusões: O bloqueio do nervo femoral guiado por ultrassom utilizando 0,3 mL.kg−1 de bupivacaína a 0,5% até o máximo de 20 mL resultou em um nível significativamente menor de fator de necrose tumoral alfa.

Removal and killing of multispecies endodontic biofilms by N-acetylcysteine

ABSTRACT Removal of bacterial biofilm from the root canal system is essential for the management of endodontic disease. Here we evaluated the antibacterial effect of N-acetylcysteine (NAC), a potent antioxidant and mucolytic agent, against mature multispecies endodontic biofilms consisting of Actinomyces naeslundii, Lactobacillus salivarius, Streptococcus mutans and Enterococcus faecalis on sterile human dentin blocks. The biofilms were exposed to NAC (25, 50 and 100 mg/mL), saturated calcium hydroxide or 2% chlorhexidine solution for 7 days, then examined by scanning electron microscopy. The biofilm viability was measured by viable cell counts and ATP-bioluminescence assay. NAC showed greater efficacy in biofilm cell removal and killing than the other root canal medicaments. Furthermore, 100 mg/mL NAC disrupted the mature multispecies endodontic biofilms completely. These results demonstrate the potential use of NAC in root canal treatment.

Isolation and characterization of a novel endo-beta-1, 4-glucanase from a metagenomic library of the black-goat rumen

ABSTRACT The various types of lignocellulosic biomass found in plants comprise the most abundant renewable bioresources on Earth. In this study, the ruminal microbial ecosystem of black goats was explored because of their strong ability to digest lignocellulosic forage. A metagenomic fosmid library containing 115,200 clones was prepared from the black-goat rumen and screened for a novel cellulolytic enzyme. The KG35 gene, containing a novel glycosyl hydrolase family 5 cellulase domain, was isolated and functionally characterized. The novel glycosyl hydrolase family 5 cellulase gene is composed of a 963-bp open reading frame encoding a protein of 320 amino acid residues (35.1 kDa). The deduced amino acid sequence showed the highest sequence identity (58%) for sequences from the glycosyl hydrolase family 5 cellulases. The novel glycosyl hydrolase family 5 cellulase gene was overexpressed in Escherichia coli. Substrate specificity analysis revealed that this recombinant glycosyl hydrolase family 5 cellulase functions as an endo-β-1,4-glucanase. The recombinant KG35 endo-β-1,4-glucanase showed optimal activity within the range of 30-50 °C at a pH of 6-7. The thermostability was retained and the pH was stable in the range of 30-50 °C at a pH of 5-7.

Symptom Interval and Patient Delay Affect Survival Outcomes in Adolescent Cancer Patients

PURPOSE: Unique features of adolescent cancer patients include cancer types, developmental stages, and psychosocial issues. In this study, we evaluated the relationship between diagnostic delay and survival to improve adolescent cancer care. MATERIALS AND METHODS: A total of 592 patients aged 0-18 years with eight common cancers were grouped according to age (adolescents, ≥10 years; children, <10 years). We retrospectively reviewed their symptom intervals (SIs, between first symptom/sign of disease and diagnosis), patient delay (PD, between first symptom/sign of disease and first contact with a physician), patient delay proportion (PDP), and overall survival (OS). RESULTS: Mean SI was significantly longer in adolescents than in children (66.4 days vs. 28.4 days; p<0.001), and OS rates were higher in patients with longer SIs (p=0.001). In children with long SIs, OS did not differ according to PDP (p=0.753). In adolescents with long SIs, OS was worse when PDP was ≥0.6 (67.2%) than <0.6 (95.5%, p=0.007). In a multivariate analysis, adolescents in the long SI/PDP ≥0.6 group tended to have a higher hazard ratio (HR, 6.483; p=0.069) than those in the long SI/PDP <0.6 group (HR=1, reference). CONCLUSION: Adolescents with a long SI/PDP ≥0.6 had lower survival rates than those with a short SI/all PDP or a long SI/PDP <0.6. They should be encouraged to seek prompt medical assistance by a physician or oncologist to lessen PDs.

Subclinical Hypothyroidism in Childhood Cancer Survivors

PURPOSE: In childhood cancer survivors, the most common late effect is thyroid dysfunction, most notably subclinical hypothyroidism (SCH). Our study evaluated the risk factors for persistent SCH in survivors. MATERIALS AND METHODS: Survivors (n=423) were defined as patients who survived at least 2 years after cancer treatment completion. Thyroid function was assessed at this time and several years thereafter. Two groups of survivors with SCH were compared: those who regained normal thyroid function during the follow-up period (normalized group) and those who did not (persistent group). RESULTS: Overall, 104 of the 423 survivors had SCH. SCH was observed in 26% of brain or nasopharyngeal cancer survivors (11 of 43) and 21.6% of leukemia survivors (35 of 162). Sixty-two survivors regained normal thyroid function, 30 remained as persistent SCH, and 12 were lost to follow-up. The follow-up duration was 4.03 (2.15-5.78) years. Brain or nasopharyngeal cancer and Hodgkin disease were more common in the persistent group than in the normalized group (p=0.002). More patients in the persistent group received radiation (p=0.008). Radiation to the head region was higher in this group (2394±2469 cGy) than in the normalized group (894±1591 cGy; p=0.003). On multivariable analysis, lymphoma (p=0.011), brain or nasopharyngeal cancer (p=0.039), and head radiation dose ≥1800 cGy (p=0.039) were significant risk factors for persistent SCH. CONCLUSION: SCH was common in childhood cancer survivors. Brain or nasopharyngeal cancer, lymphoma, and head radiation ≥1800 cGy were significant risk factors for persistent SCH.

Effect of software version and parameter settings on the marginal and internal adaptation of crowns fabricated with the CAD/CAM system

Objective This study investigated the marginal and internal adaptation of individual dental crowns fabricated using a CAD/CAM system (Sirona’s BlueCam), also evaluating the effect of the software version used, and the specific parameter settings in the adaptation of crowns.Material and Methods Forty digital impressions of a master model previously prepared were acquired using an intraoral scanner and divided into four groups based on the software version and on the spacer settings used. The versions 3.8 and 4.2 of the software were used, and the spacer parameter was set at either 40 μm or 80 μm. The marginal and internal fit of the crowns were measured using the replica technique, which uses a low viscosity silicone material that simulates the thickness of the cement layer. The data were analyzed using a Friedman two-way analysis of variance (ANOVA) and paired t-tests with significance level set at p<0.05.Results The two-way ANOVA analysis showed the software version (p<0.05) and the spacer parameter (p<0.05) significantly affected the crown adaptation. The crowns designed with the version 4.2 of the software showed a better fit than those designed with the version 3.8, particularly in the axial wall and in the inner margin. The spacer parameter was more accurately represented in the version 4.2 of the software than in the version 3.8. In addition, the use of the version 4.2 of the software combined with the spacer parameter set at 80 μm showed the least variation. On the other hand, the outer margin was not affected by the variables.Conclusion Compared to the version 3.8 of the software, the version 4.2 can be recommended for the fabrication of well-fitting crown restorations, and for the appropriate regulation of the spacer parameter.

Efficacy of levofloxacin, amoxicillin and a proton pump inhibitor in the eradication of Helicobacter pylori in Brazilian patients with peptic ulcers

OBJECTIVES: The eradication of Helicobacter (H.) pylori allows peptic ulcers in patients infected with the bacteria to be cured. Treatment with the classic triple regimen (proton pump inhibitor, amoxicillin and clarithromycin) has shown decreased efficacy due to increased bacterial resistance to clarithromycin. In our country, the eradication rate by intention to treat with this regimen is 83%. In Brazil, a commercially available regimen for bacterial eradication that uses levofloxacin and amoxicillin with lansoprazole is available; however, its efficacy is not known. Considering that such a treatment may be an alternative to the classic regimen, we aimed to verify its efficacy in H. pylori eradication. METHODS: Patients with peptic ulcer disease infected with H. pylori who had not received prior treatment were treated with the following regimen: 30 mg lansoprazole bid, 1,000 mg amoxicillin bid and 500 mg levofloxacin, once a day for 7 days. RESULTS: A total of 66 patients were evaluated. The patients’ mean age was 52 years, and women comprised 55% of the sample. Duodenal ulcers were present in 50% of cases, and gastric ulcers were present in 30%. The eradication rate was 74% per protocol and 73% by intention to treat. Adverse effects were reported by 49 patients (74%) and were mild to moderate, with a prevalence of diarrhea complaints. CONCLUSIONS: Triple therapy comprising lansoprazole, amoxicillin and levofloxacin for 7 days for the eradication of H. pylori in Brazilian peptic ulcer patients showed a lower efficacy than that of the classic triple regimen. .

Etiologies and characteristics of children with chief complaint of short stature

PURPOSE: Short stature is a very common reason for visits to pediatric endocrine clinics. It could be the first sign of an underlying disease. The purpose of this study is to investigate the etiologies and general characteristics of subjects who visited an outpatient clinic due to short stature. METHODS: We retrospectively reviewed the medical records of 3,371 patients who visited Severance Children's Hospital with the chief complaint of short stature from 2010 to 2012. Medical history, auxological data, and laboratory tests including bone age were collected and analyzed. Chromosome studies or combined pituitary function tests were performed if needed. RESULTS: Approximately 89.4% of the subjects with the chief complaint of short stature who visited the outpatient clinic were of normal height, and only 10.6% of subjects were identified as having short stature. Of the subject of short stature, 44.7% were classified as having normal variant short stature; that is, familial short stature (23.0%), constitutional delay in growth (17.7%), and mixed form (3.9%). Pathological short stature was found in 193 subjects (54.2%). Among pathological short stature, most common etiology was growth hormone deficiency (GHD) (38.9%). CONCLUSION: A majority of children had a normal height. Among children with short stature, pathological short stature and normal variants occupied a similar percentage. GHD was the most common cause of pathological short stature and found in about 20% of the children with short stature. In pathological short stature, the height, height velocity, and IGF-1 level were lower than in normal variants.

Effects of ursolic acid on glucose metabolism, the polyol pathway and dyslipidemia in non-obese type 2 diabetic mice.

Ursolic acid (UA) is a pentacyclic triterpenoid compound that naturally occurs in fruits, leaves and flowers of medicinal herbs. This study investigated the dose-response efficacy of UA (0.01 and 0.05%) on glucose metabolism, the polyol pathway and dyslipidemia in streptozotocin/nicotinamide-induced diabetic mice. Supplement with both UA doses reduced fasting blood glucose and plasma triglyceride levels in non-obese type 2 diabetic mice. High-dose UA significantly lowered plasma free fatty acid, total cholesterol and VLDL-cholesterol levels compared with the diabetic control mice, while LDL-cholesterol levels were reduced with both doses. UA supplement effectively decreased hepatic glucose-6-phosphatase activity and increased glucokinase activity, the glucokinase/glucose-6-phosphatase ratio, GLUT2 mRNA levels and glycogen content compared with the diabetic control mice. UA supplement attenuated hyperglycemia-induced renal hypertrophy and histological changes. Renal aldose reductase activity was higher, whereas sorbitol dehydrogenase activity was lower in the diabetic control group than in the non-diabetic group. However, UA supplement reversed the biochemical changes in polyol pathway to normal values. These results demonstrated that low-dose UA had preventive potency for diabetic renal complications, which could be mediated by changes in hepatic glucose metabolism and the renal polyol pathway. High-dose UA was more effective anti-dyslipidemia therapy in non-obese type 2 diabetic mice.

Prevalence and Clinical Implication of Partial Tandem Duplication of the Mixed Lineage Leukemia Gene in Pediatric Acute Leukemia / 임상소아혈액종양

BACKGROUND: The mixed lineage leukemia (MLL) gene may induce hematopoiesis and leukemia. Partial tandem duplication of MLL (MLL-PTD) is associated with poor prognosis in acute myeloid leukemia (AML); however, the significance of MLL-PTD in acute lymphoblastic leukemia (ALL) has not been thoroughly studied. We evaluated the incidence, relationship with other cytogenetic abnormalities, and the prognostic role of MLL-PTD in ALL.METHODS: We reviewed medical records from pediatric patients diagnosed with ALL in Severance Hospital, Yonsei University Health System, South Korea from 2002 to 2008. MLL-PTD was detected by nested reverse transcriptase polymerase chain reaction.RESULTS: In ALL patients, 50.0% (42/84) were positive for MLL-PTD. There was no significant difference in the 10-year overall survival (10Y OS) and event-free survival (EFS) between MLL-PTD-positive (+) and MLL-PTD-negative (-) groups (69.4% vs. 76.2%, P=0.609, and 62.6% vs. 66.7%, P=0.818, respectively). The combination of high level of lactate dehydrogenase (>1,100 IU/L) and MLL-PTD(+) [MLL-PTD(+)/High LDH] was a statistically significant negative prognostic factor for 10Y OS and EFS (P=0.0226 and P=0.0230, respectively). In multivariate analysis, National Cancer Institute risk stratification and very high risk features were independent significant prognostic factors but MLL-PTD (+)/High LDH was not.CONCLUSION: MLL-PTD was observed frequently in pediatric ALL patients. MLL-PTD was not an independent prognostic factor. MLL-PTD (+)/High LDH should be evaluated further for its prognostic potential in ALL.

Radiation or Chemotherapy rather than Observation may be a Better Modality after Subtotal Resection for Pilocytic Astrocytoma in Children / 임상소아혈액종양

BACKGROUND: Pilocytic astrocytoma (PA) is a low-grade glioma that occurs primarily in children and young adults. The optimal postoperative treatment modality after subtotal resection (STR) of PAs remains to be elucidated. The aim of this study was to compare the efficacies of different post-STR treatment modalities and to examine the risk factors for the progression of PAs.METHODS: We reviewed the medical records of 91 pediatric PA patients in a single institute during a 30-year period. Kaplan-Meier analysis was used to assess overall survival (OS) and progression-free survival (PFS), and Cox proportional hazard models were used to calculate hazard ratios.RESULTS: The median age of 91 patients was 8.9 years (range, 0.3-17.9). GTR was perfomed, whenever possible. Patients who underwent STR afterwards received either radiotherapy, chemotherapy, or were observed without further treatment, according to clinician preference. In total group, 10-year OS was 97.4% and 10-year PFS was 57.2%. In GTR group (N=33), 10-year OS and PFS was 100%. In STR group (N=49), 10-year OS was 97.7%, while 10-year PFS was 38.6%. STR group underwent following postoperative (PO) modalities; observation (PO-Obs, N=32), radiotherapy (PO-RT, N=10), chemotherapy (PO-CTx, N=7). The 10-year PFS rate was higher in patients who received postoperative treatment (either PO-RT or PO-CTx) than in patients who received PO-Obs (62.5% vs 27.0%, P=0.039). In multivariate analysis for STR group, PO-CTx (Hazard ratio (HR)=0.20, P=0.035) and PO-RTx (HR=0.13, P=0.008) were superior to observation, respectively.CONCLUSION: Radiation and chemotherapy are better post-STR treatment modalities than observation for pediatric PA patients.

Lower Diagnostic Value of Bone Marrow Biopsy in Children with Fever of an Unknown Origin / 임상소아혈액종양

BACKGROUND: Diagnostic value of Bone marrow (BM) biopsies for fever of unknown origin (FUO) remain controversial and BM biopsies are difficult to perform in young patients. Our study aimed to elucidate the diagnostic yield of BM biopsies in FUO patients of all age, particularly for diagnosing hematological malignant diseases.METHODS: The medical records of 150 patients, hospitalized between January 1, 2008 and June 30, 2013, who underwent BM biopsies were evaluated to determine the cause of FUO. FUO was defined as fever (38.3degrees C, 101) either on several occasions during the 3 hospital days without a clear cause, after 1 week of invasive investigation, or after 3 outpatient visits. BM-specific diagnoses included those determined by BM biopsies (i.e., leukemia, lymphoma, myeloproliferative disease, myelodysplastic syndrome, aplastic anemia, and hemophagocytic lymphohistiocytosis).RESULTS: The final diagnoses of 24 patients (16%) were determined by BM biopsies; the majority included hematologic diseases and malignant neoplasms. Low hemoglobin levels, thrombocytopenia, bicytopenia, increased Lactate dehydrogenase (LDH) and ferritin levels, and ultrasonographic/computed tomographic abnormalities were significant risk factors (P<0.05). The young patient group (<18 years old) was safer from the tendency of BM biopsy diagnosis compared to adult patient group (>40 years old).CONCLUSION: Some laboratory abnormalities were related to the BM biopsy diagnostic yield. Furthermore, pediatric age was an important factor for deciding to do not perform excessive BM biopsies in FUO cases.

Prevalence and Clinical Implication of Partial Tandem Duplication of the Mixed Lineage Leukemia Gene in Pediatric Acute Leukemia / 임상소아혈액종양

BACKGROUND: The mixed lineage leukemia (MLL) gene may induce hematopoiesis and leukemia. Partial tandem duplication of MLL (MLL-PTD) is associated with poor prognosis in acute myeloid leukemia (AML); however, the significance of MLL-PTD in acute lymphoblastic leukemia (ALL) has not been thoroughly studied. We evaluated the incidence, relationship with other cytogenetic abnormalities, and the prognostic role of MLL-PTD in ALL. METHODS: We reviewed medical records from pediatric patients diagnosed with ALL in Severance Hospital, Yonsei University Health System, South Korea from 2002 to 2008. MLL-PTD was detected by nested reverse transcriptase polymerase chain reaction. RESULTS: In ALL patients, 50.0% (42/84) were positive for MLL-PTD. There was no significant difference in the 10-year overall survival (10Y OS) and event-free survival (EFS) between MLL-PTD-positive (+) and MLL-PTD-negative (-) groups (69.4% vs. 76.2%, P=0.609, and 62.6% vs. 66.7%, P=0.818, respectively). The combination of high level of lactate dehydrogenase (>1,100 IU/L) and MLL-PTD(+) [MLL-PTD(+)/High LDH] was a statistically significant negative prognostic factor for 10Y OS and EFS (P=0.0226 and P=0.0230, respectively). In multivariate analysis, National Cancer Institute risk stratification and very high risk features were independent significant prognostic factors but MLL-PTD (+)/High LDH was not. CONCLUSION: MLL-PTD was observed frequently in pediatric ALL patients. MLL-PTD was not an independent prognostic factor. MLL-PTD (+)/High LDH should be evaluated further for its prognostic potential in ALL.

Radiation or Chemotherapy rather than Observation may be a Better Modality after Subtotal Resection for Pilocytic Astrocytoma in Children / 임상소아혈액종양

BACKGROUND: Pilocytic astrocytoma (PA) is a low-grade glioma that occurs primarily in children and young adults. The optimal postoperative treatment modality after subtotal resection (STR) of PAs remains to be elucidated. The aim of this study was to compare the efficacies of different post-STR treatment modalities and to examine the risk factors for the progression of PAs. METHODS: We reviewed the medical records of 91 pediatric PA patients in a single institute during a 30-year period. Kaplan-Meier analysis was used to assess overall survival (OS) and progression-free survival (PFS), and Cox proportional hazard models were used to calculate hazard ratios. RESULTS: The median age of 91 patients was 8.9 years (range, 0.3-17.9). GTR was perfomed, whenever possible. Patients who underwent STR afterwards received either radiotherapy, chemotherapy, or were observed without further treatment, according to clinician preference. In total group, 10-year OS was 97.4% and 10-year PFS was 57.2%. In GTR group (N=33), 10-year OS and PFS was 100%. In STR group (N=49), 10-year OS was 97.7%, while 10-year PFS was 38.6%. STR group underwent following postoperative (PO) modalities; observation (PO-Obs, N=32), radiotherapy (PO-RT, N=10), chemotherapy (PO-CTx, N=7). The 10-year PFS rate was higher in patients who received postoperative treatment (either PO-RT or PO-CTx) than in patients who received PO-Obs (62.5% vs 27.0%, P=0.039). In multivariate analysis for STR group, PO-CTx (Hazard ratio (HR)=0.20, P=0.035) and PO-RTx (HR=0.13, P=0.008) were superior to observation, respectively. CONCLUSION: Radiation and chemotherapy are better post-STR treatment modalities than observation for pediatric PA patients.

Lower Diagnostic Value of Bone Marrow Biopsy in Children with Fever of an Unknown Origin / 임상소아혈액종양

BACKGROUND: Diagnostic value of Bone marrow (BM) biopsies for fever of unknown origin (FUO) remain controversial and BM biopsies are difficult to perform in young patients. Our study aimed to elucidate the diagnostic yield of BM biopsies in FUO patients of all age, particularly for diagnosing hematological malignant diseases. METHODS: The medical records of 150 patients, hospitalized between January 1, 2008 and June 30, 2013, who underwent BM biopsies were evaluated to determine the cause of FUO. FUO was defined as fever (38.3degrees C, 101) either on several occasions during the 3 hospital days without a clear cause, after 1 week of invasive investigation, or after 3 outpatient visits. BM-specific diagnoses included those determined by BM biopsies (i.e., leukemia, lymphoma, myeloproliferative disease, myelodysplastic syndrome, aplastic anemia, and hemophagocytic lymphohistiocytosis). RESULTS: The final diagnoses of 24 patients (16%) were determined by BM biopsies; the majority included hematologic diseases and malignant neoplasms. Low hemoglobin levels, thrombocytopenia, bicytopenia, increased Lactate dehydrogenase (LDH) and ferritin levels, and ultrasonographic/computed tomographic abnormalities were significant risk factors (P40 years old). CONCLUSION: Some laboratory abnormalities were related to the BM biopsy diagnostic yield. Furthermore, pediatric age was an important factor for deciding to do not perform excessive BM biopsies in FUO cases.

The multiple faces of calcineurin signaling in Caenorhabditis elegans: Development, behaviour and aging.

Calcineurin, a well-conserved protein phosphatase 2B (PP2B), is a Ca2+-calmodulin–dependent serine/threonine protein phosphatase that is known to be involved in a myriad of cellular processes and signal transduction pathways. The biological role of calcineurin has been extensively studied in diverse groups of organisms. Homologues of mammalian and Drosophila calcineurin subunits exist in the nematode, Caenorhabditis elegans. The C. elegans counterpart of the catalytic subunit, calcineurin A, cna-1/tax-6, and the regulatory subunit, calcineurin B, cnb-1, are known to express ubiquitously in multiple tissues including neurons. The characterization of C. elegans calcineurin mutants facilitates identification of its physiological functions and signaling pathways. Genetic interactions between cna-1/tax-6 and cnb-1 mutants with a number of mutants involved in several signaling pathways have exemplified the pivotal role of calcineurin in regulating nematode development, behaviour and lifespan (aging). The present review has been aimed to provide a succinct summary of the multiple functions of calcineurin in C. elegans relating to its development, fertility, proliferation, behaviour and lifespan. Analyses of cna-1/tax-6 and cnb-1 interacting proteins and regulators of the phosphatase in this fascinating worm model have an immense scope to identify potential drug targets in various parasitic nematodes, which cause many diseases inflicting huge economic loss; and also for many human diseases, particularly neurodegenerative and myocardial diseases.

Successful Selective CD34+ Cell Infusion after Late Graft Failure of Hematopoietic Stem Cell Transplantation in Two Cases of Severe Aplastic Anemia / 임상소아혈액종양

Matched sibling bone marrow transplantation (BMT) in severe aplastic anemia (SAA) has been known as the treatment of choice in children and young adults. To overcome graft failure, second stem cell transplantation showed good results in previous studies. Here we report two cases of aplastic anemia patients with late graft failure and resulted in successful complete recovery after selective CD34+ cell boost infusion. The patients previously underwent allogeneic BMT from HLA-matched sibling donors and the engraftment was achieved although their CBC started to decrease respectively 3 months and 11 months after transplantation. Both patients received selective CD34+ cell infusion without additional conditioning therapy. Their CBC showed significant improvement and they are doing well without transfusion or complications. From this study we suggest that selected CD34+ cell boost treatment can be a promising curative treatment for late graft failure after matched sibling BMT in SAA patients.

Successful Selective CD34+ Cell Infusion after Late Graft Failure of Hematopoietic Stem Cell Transplantation in Two Cases of Severe Aplastic Anemia / 임상소아혈액종양

Matched sibling bone marrow transplantation (BMT) in severe aplastic anemia (SAA) has been known as the treatment of choice in children and young adults. To overcome graft failure, second stem cell transplantation showed good results in previous studies. Here we report two cases of aplastic anemia patients with late graft failure and resulted in successful complete recovery after selective CD34+ cell boost infusion. The patients previously underwent allogeneic BMT from HLA-matched sibling donors and the engraftment was achieved although their CBC started to decrease respectively 3 months and 11 months after transplantation. Both patients received selective CD34+ cell infusion without additional conditioning therapy. Their CBC showed significant improvement and they are doing well without transfusion or complications. From this study we suggest that selected CD34+ cell boost treatment can be a promising curative treatment for late graft failure after matched sibling BMT in SAA patients.