Journal of the korean academy of Pediatric Dentistry
/
v.46
no.4
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pp.409-415
/
2019
Cleidocranial dysplasia (CCD) is an autosomal-dominant disease characterized by the delayed closure of cranial sutures, defects in clavicle formation, supernumerary teeth, and delayed tooth eruption. Defects in the Runt-related transcription factor 2 (RUNX2), a master regulator of bone formation, have been identified in CCD patients. The aim of this study was to identify the molecular genetic causes in a CCD family with delayed tooth eruption. The 23-year-old female proband and her mother underwent clinical and radiographic examinations, and all coding exons of the RUNX2 were sequenced. Mutational analysis revealed a single nucleotide deletion mutation (NM_001024630.4 : c.357delC) in exon 3 in the proband and her mother. The single C deletion would result in a frameshift in translation and introduce a premature stop codon [p.(Asn120Thrfs*24)]. This would result in the impaired function of RUNX2 protein, which may be the cause of delayed eruption of permanent teeth in the family.
Purpose: Congenital dermoid cysts develop during the fusion of the embryo when the ectodermal tissue gets trapped in the line of fusion. Dermoid cysts of the head are rare lesions comprised of epidermal and mesodermal elements. Furthermore, dermoid cysts in the occipital area are extremely rare. Only a few cases of dermoid cysts in the posterior scalp have been reported. Especially, A bilateral, synchronous presentation in this location has not been reported previously in the literature. Methods: All 5 cases had a gradually enlarging mass of the posterior aspect of the scalp. The cysts were mobile, noncompressible, and non-tender, without evidence of an associated sinus tract, skin dimpling, discoloration, or communication with adjacent structures. The CT scan displayed a hypodense cystic lesions about -87 to +24 HU (Housefield units, average +3.2 HU) with hypodense capsule and no postcontrast enhancement. All tumors were found just under the skin, and were well encapsulated, so they were completely removed the mass with adjacent periosteum. Results: On gross findings, all tumors were oval-or round-shaped, and when the cystic tumor was cut open it presented a greasy and caseous substance. Histologically, all specimens contain desquamated squamous epithelium and keratin in the lumen and are encapsulated and lined by keratinized stratified squamous epithelium. And, all cases of posterior mass are the presence of adnexal structures. Conclusion: Appropriate diagnosis requires not only an index of suspicion for this rare tumor a very careful history and search for skin changes. Especially, CT can reveal the exact location of the cyst, its relationship with the adjacent structures. We think that occipital dermoids divide into superficial and deep type. In our cases, because they did not have intra-cranial involvement or fistula formation, they are superficial type. This report describes the clinical and operative aspects of the superficial dermoid cysts and provides a review of the literatures.
Four external and 27 cranial characters of three subspecies of striped field mice (Apodemus agrarius) from 15 regions in far eastern Asia(South Korea, North Korea, northeastern China, and far eastern Russia) were analyzed by multivariate methods in order to determine taxonomic status of striped field mice from North Korea. Three subgroups were recognized: a large-size form from two localities in South Korea(Cheju Island, Wando Island): a medium-size form from eight localities in South Korea and North Korea(Jindo Island, Kunsan, Mt. Chirisan, Cheongju, Mt. Weoleksan, Mt. taebaksan; Haeju, Sineuiju); a small-size form from five localities in North Korea, northeastern China, and far eastern Russia (Mt. Kumkangsan, Mt. Myohyangsan; Jirin, Yichun; Vladivostok). It is confirmed that the large-size form, the medium-size form, and the small-size form are subspecies chejuensis, coreae, and manchuricus, respectively, as noted by Koh et al.(1997). It is revealed that striped field mice(the medium-size form) from western region of North Korea (Haeju, Sineuiju) is classified into A. agrarius coreae, as noted by Thomas(1908), but striped field mice(the small-size form) from eastern region of North Korea(Mt. Kumkangsan, Mt. Myohyangsan) is A. agrarius manchuricus.
Kim Jae Cheol;Jang Yang Suk;Ryu Samuel;Park In Kyu
Radiation Oncology Journal
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v.7
no.2
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pp.205-211
/
1989
From July 1984 to September 1988, 27 patients with limited stage small cell lung cancer were treated with combined modality (combination chemotherapy Plus radiotherapy) at the Department of Therapeutic Radiology in Kyungpook National University Hospital. Of the 27 patients, 19$(70\%)$ achieved a complete response, 6$(22\%)$ a partial response, and 2$(8\%)$ no response. Female, performance status HO, serum enolase level below 30ng/ml, radiation dose over 4500 cGy, and 4 or more cycles of chemotherapy had a favorable effect on the rates of complete response, although there were no statistical differences according to the variables. Median survival time was 10 months and overall 1- and 2-year survival rates were $40,7\%$ and $12.2\%$, respectively. Complete response (p<0.05), performance status HO (p<0.05), 4 or more cycles of chemotherapy (p<0.05), and radiation dose over 4500 cGy had a significantly favorable effect on 2-year survival rate. Prophylactic cranial irradiation or sex had no effect on survival. The results of this study suggest that radiation treatment should be combined with combination chemotherapy in the therapeutic strategy of SCLC of limited stage.
Kim, Tae-Hwan;Hong, Subin;Kim, Minkyung;Shin, Jeong-In;Jang, Yun-Sul;Lee, Jae-Hoon
Journal of Veterinary Clinics
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v.32
no.6
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pp.530-535
/
2015
An 11-year-old intact female miniature poodle presented with a four-month history of hemorrhagic effusion. The patient was alert on physical examination, although muffled heart sounds were noted upon auscultation of the right hemithorax. The radiographic finding was pleural effusion. Ultrasonography revealed cystic changes in both ovaries and several nodules in the liver. A refractory opacity in the right lung field, as visualized with computer tomography (CT), was diagnosed as right middle lung lobe torsion with a collapsed bronchus. Five days after diagnosis, a right fifth intercostal thoracotomy was performed to remove the right middle lung lobe; the right middle lung lobe was grossly shrunken as a result of chronic lung lobe torsion. Ovariohysterectomy was also performed. Histopathologic examination revealed papillary adenocarcinoma in both ovaries and suspected metastasized ovarian adenocarcinoma cells in the lung lobe. The patient recovered favorably and had been doing well up to two months post-surgery. However, after four months, the dog presented with respiratory difficulty. The radiographic findings were pleural effusion and collapse of the right cranial and left caudal lung lobes. Malignant cells of epithelial origin were observed in the pleural effusion. The tumor cells were suspected to be metastasized cells from the previously resected lung lobe. Although cancer treatment was recommended, the suggestion was suspended and the dog was discharged from hospital. This was a case of lung lobe torsion that had occurred because of hemorrhagic effusion due to tumor. Although ovariohysterectomy and lobectomy were performed, there was a relapse of hemorrhagic effusion because of metastasized tumor from the previously resected lung lobe.
Kim, Young-Woo;Yoo, Do-Sung;Kim, Dal-Soo;Huh, Pil-Woo;Cho, Kyung-Suck;Kim, Jae-Gun;Kang, Joon-Ki
Journal of Korean Neurosurgical Society
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v.30
no.sup2
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pp.216-220
/
2001
Objective : Cranioplasty is required to protect underlying brain, to correct major aesthetic deformities, or both. The ideal material for this purpose is autogenous bone. When this is not available, alloplastic or artificial materials may be used. In this study authors compared the infection rate according to the cranioplasty materials(the frozen autologous bone vs. bone cement), and duration of the skull defect. Materials : Between May 1994 and December 1999, 111 patients with skull defect treated with cranioplasty(82 cases of frozen autologous bone and 29 cases of artificial bone material) were included in this study. There were 77 males and 34 females with a mean age of 41.4 years(range 1-85 years). 57 patients had head trauma and 54 had non-traumatic insults. According to the duration of skull defect, there were 28 cases under 1 month, 33 cases of 1-2 months, 15 cases of 2-3 months, 20 cases of 3-6 months and 15 cases over 6 months of duration. Results : Overall infection rate was 9.9%. In cases with frozen autologous bone and artificial bone material, the infection rate was 8.5% and 13.7%, respectively. The infection rate according to the duration of skull defect was 3.6%(among 28 cases) under 1 month of age, while those were 12%(4 among 33 cases) at 1-2 months, 20%(3 among 15 cases) at 2-3 months, 5%(1 among 20 cases) at 3-6 months and 13%(2 among 15 cases) over 6 months. Accoring to the underlying disease, the infection rate in traumatic cases was 12%(7 among 57 cases) and that in non-traumatic one was 3.7%(2 among 54 cases). Conclusion : From this study, it appears that skull defect should be repaired as soon as possible, because early cranioplasty can lower the infection rate. And surgeons could save the patients' cranial bone as possible as they can because autologous bone is not only cost effective in cosmatic purpose but lower the infection rate.
Objective : The aim of this study to investigate the benefits of patient-based 3-dimensional (3D) cerebral arteriovenous malformation (AVM) models for preoperative surgical planning and education. Methods : Fifteen patients were operated on for AVMs between 2015 and 2019 with patient-based 3D models. Ten patients' preoperative cranial angiogram screenings were evaluated preoperatively or perioperatively via patient-based 3D models. Two patients needed emergent surgical intervention; their models were solely designed based on their AVMs and used during the operation. However, the other patients who underwent elective surgery had the modeling starting from the skull base. These models were used both preoperatively and perioperatively. The benefits of patients arising from treatment with these models were evaluated via patient files and radiological data. Results : Fifteen patients (10 males and five females) between 16 and 66 years underwent surgery. The mean age of the patients was 40.0±14.72. The most frequent symptom patients observed were headaches. Four patients had intracranial bleeding; the symptom of admission was a loss of consciousness. Two patients (13.3%) belonged to Spetzler-Martin (SM) grade I, four (26.7%) belonged to SM grade II, eight (53.3%) belonged to SM grade III, and one (6.7%) belonged to SM grade IV. The mean operation duration was 3.44±0.47 hours. Three patients (20%) developed transient neurologic deficits postoperatively, whereas three other patients died (20%). Conclusion : Several technological innovations have emerged in recent years to reduce undesired outcomes and support the surgical team. For example, 3D models have been employed in various surgical procedures in the last decade. The routine usage of patient-based 3D models will not only support better surgical planning and practice, but it will also be useful in educating assistants and explaining the situation to the patient as well.
Background: Trigeminal neuralgia (TN) is characterized by brief, unilateral, sharp, stabbing, and shooting pain of the fifth cranial nerve. The objective of this systematic review with meta-analysis was to determine the effect of medications compared to placebo in adult patients with TN. Methods: Review authors identified randomized placebo-controlled trials (RCTs) from PubMed, Web of Science, Cochrane, and EMBASE up to February 2021. We assessed the inclusion and exclusion criteria as well as the risk of bias of the studies based on the Cochrane Handbook. A total of 324 unduplicated references were scanned independently and reduced to eight relevant RCTs, with 89 patients included. Medications investigated included oral carbamazepine, subcutaneous sumatriptan, lidocaine (intranasal, 8% spray on the oral mucosa or intravenous), buprenorphine (ganglionic local opioid analgesia), and oral Nav1.7, a selective sodium channel blocker. Results: Meta-analyses showed that overall patients receiving lidocaine reported a significantly lower post-treatment intensity of pain -3.8 points on a 0-10 scale (95% Cl = -4.653 to -2.873; P < 0.001). Patients who received lidocaine were 8.62 times more likely to have pain improvement than patients on placebo (P < 0.001). In one RCT, patients receiving oral carbamazepine showed a significant improvement in pain intensity of -32% compared to the placebo (P < 0.001). In one trial, patients receiving 3 mg subcutaneous sumatriptan had a significantly lower intensity of pain on average -6.1 points on a scale of 0-10 compared to placebo (P < 0.001) and a significant improvement in pain intensity of -75% compared to the improvement in the placebo group (P < 0.001). Patients who received subcutaneous sumatriptan were 10 times more likely to have pain improvement than those who received placebo (P = 0.001) in one study. Due to the unclear/high risk of bias and small sample size, the quality of the evidence for lidocaine in the treatment of TN was low. Conclusion: Further studies are needed for carbamazepine, sumatriptan, buprenorphine, and oral Nav1.7 sodium channel blockers, as only one study reported outcomes.
Cho, Hyoung Sun;Kwon, Yonghwan;Kim, Young-Ung;Kang, Jin-Su;Lee, Kichang;Kim, Namsoo;Kim, Min Su
Journal of Veterinary Clinics
/
v.36
no.1
/
pp.53-61
/
2019
Total hip arthroplasty (THA) is a successful surgical treatment for both patients with chronical lameness and dogs who are nonresponsive to medical treatments, providing excellent joint function for returning dogs to the normal gait in 80% to 98% of hip dysplasia (HD) patients. The THA surgical implant system manufactured by BioMedtrix and Kyon are today widely accepted. When comparing the BioMedtrix biological fixation (BFX) system to the BioMedtrix cemented fixation (CFX) system, the many advantages of BFX, which include longer potential implant life, decreased risk of postoperative or later infection, and better implant stability, become evident. However, BFX implies a greater risk of femoral fracture during reaming and requires a more precise surgical technique to achieve good implant fit, given the press-fit nature of cementless THA. The purposes of this study are to both describe the mistakes and complications during stem implantation for beginner surgeons with both the BFX and the CFX systems and to document the initial result of 12 implantations in canine cadavers. Given the detailed evaluations of 3 specialists, who are Diplomate American College of Veterinary Surgeons (DACVS), only 3 of 11 stems were appropriately sized. Specifically, 6 stems were anteverted rather than being retroverted; further, although 7 stems were coaxial with the femoral long axis in the frontal plane, the other stems were in the varus at the frontal plane, with the proximal medial stem adjacent to the medial femoral cortex. Moderate angulation from the cranial to the caudal directions was found in 4 cases in the sagittal plane. Additionally, 1 case of femoral fissure and 1 case of perforated femoral cortex were reported. It is not easy for surgeons performing cementless THA for the first time to achieve a good result, even though they completed an educational course about it and given that catastrophic complications often occurred during early surgical clinical cases. Therefore, ex-vivo studies are sincerely required to get an expertise by rehearsing the preparation of the femoral envelop in isolated bones. Further studies should be conducted to achieve both highly accurate implant size and correct orientation during the preoperative planning. Additionally, surgeons' learning curve should be examined in future investigations.
Background: Gintonin is a ginseng-derived exogenous ligand of the G protein-coupled lysophosphatidic acid (LPA) receptor. We previously reported that gintonin stimulates gliotransmitter release in primary cortical astrocytes. Astrocytes play key roles in the functions of neurovascular systems. Although vascular endothelial growth factor (VEGF) is known to influence the normal growth and maintenance of cranial blood vessels and the nervous system, there is little information about the effect of gintonin on VEGF regulation in primary astrocytes, under normal and hypoxic conditions. Methods: Using primary cortical astrocytes of mice, the effects of gintonin on the release, expression, and distribution of VEGF were examined. We further investigated whether the gintonin-mediated VEGF release protects astrocytes from hypoxia. Results: Gintonin administration stimulated the release and expression of VEGF from astrocytes in a concentration- and time-dependent manner. The gintonin-mediated increase in the release of VEGF was inhibited by the LPA1/3 receptor antagonist, Ki16425; phospholipase C inhibitor, U73122; inositol 1,4,5- triphosphate receptor antagonist, 2-APB; and intracellular $Ca^{2+}$ chelator, BAPTA. Hypoxia further stimulated astrocytic VEGF release. Gintonin treatment stimulated additional VEGF release and restored cell viability that had decreased due to hypoxia, via the VEGF receptor pathway. Altogether, the regulation of VEGF release and expression and astrocytic protection mediated by gintonin under hypoxia are achieved via the LPA receptor-VEGF signaling pathways. Conclusion: The present study shows that the gintonin-mediated regulation of VEGF in cortical astrocytes might be neuroprotective against hypoxic insults and could explain the molecular basis of the beneficial effects of ginseng on the central nervous system.
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